Medical Cannabis - The Safer Solution To Chronic Pain The Safer Solution To Chronic Pain

Published: LifeLines for Health, vol. 7 (Spring 2016)

By Dr Dustin Sulak D.O. 

Chronic pain and opiate addiction have ruined countless lives and changed the landscape of medicine.

Some patients feel let down by the medical system when they are denied effective treatment for chronic pain. Others feel injured by medical providers who have started them on opiate medications that have led to addiction and ruined their lives. Healthcare providers feel frustrated that they cannot do more to safely help patients with chronic pain, guilty for providing effective short-term treatments with harmful long-
term effects, and depleted of compassion for patients who need help.

Luckily, there is another solution that is safe, effective, and sustainable. Plus, patients can grow their own. The vast majority of medical cannabis patients use this herbal medicine to treat chronic pain.

The providers at my medical practice are amazed by the results of medical cannabis in the treatment of chronic pain. Patient after patient tells a similar story, and it goes something like this: “I cut my opiate usage drastically, I’m sleeping better, feeling happy, and enjoying my life more, all with less side effects.”

We find that whenever we see a patient who has been using opiates to treat chronic pain, after starting medical cannabis the opiate dosage decreases by at least 50% in the first week. It’s often more like a 60-80% reduction. Many patients go on to completely discontinue opiate medications over the next several months. They often reduce or discontinue other pain medications as well, including anti-depressants and anti-seizure drugs.

Scientific research tells the same story. A review article published in Life Sciences (2004) concludes, “The administration of low doses THC in conjunction with low doses of morphine seems to be an alternative regimen that reduces the need to escalate opioid dose while increasing opioid potency.” An animal study found that adding a small amount of THC increased the effectiveness of morphine by four to twelve times.

And while cannabinoids, like THC, can work together with opioids to increase potency in the pain centers of the brain, there isn’t a significant effect on the brainstem, the area responsible for decreased heart rate, slower breathing, and ultimately death in the case of an opiate overdose. This means that using cannabis with opioids is safer than using opioids alone.

Patients are starting to figure this out. A study published in the Harm Reduction Journal (2009) looked at 350 medical cannabis users, and found that 65% of them were using cannabis as a substitute for prescription drugs, 40% were using it as a substitute for alcohol, and 26% were using it as a substitute for illicit drugs. Three of the subjects used cannabis to help them quit smoking tobacco. The most common reasons for the substitution were less adverse side effects, better symptom management, and less withdrawal.

So while we all were told that cannabis is dangerous because it is a “gateway drug,” scientists are now calling it an, “exit drug” within the framework of harm reduction. The goal is to substitute a safer drug for one that’s more dangerous in patients who are unable to achieve total abstinence.

And cannabis is certainly safer than most of our current options for treating chronic pain. In 1999, the Institute of Medicine published a report that stated, “Except for the harms associated with smoking, the adverse effects of cannabis use are within the range tolerated for other medications,” and “there is no conclusive evidence that marijuana causes cancer in humans.”

Unlike opiate medications, cannabis can remain effective at a low dose for years or decades. While opiates can numb the pain and disconnect the mind from the body, increasing risk for inappropriate activity and re-injury, cannabis often does the opposite. Patients report that after using cannabis, the pain is still there but is less intense, less bothersome, and they no longer have to dwell on it. They often feel more connected to their bodies because they no longer have to retreat from the pain. Patients feel able to reclaim their lives from the constant focus on pain.

Cannabis does not have to be smoked, as many patients are using tinctures (liquid extracts) and vaporizers (smoke-free inhalation). Certified patients are able to grow their own or purchase the medicine from caregivers or dispensaries.

Choosing the Right Medicine Strains

There are hundreds of varieties (i.e. strains, cultivars) of medical cannabis, each with different medicinal effects. The unique properties of each strain arise from their synergy of the cannabinoids, terpenes, and other compounds. Finding the right medicine will likely require trying out a number of varieties, but the following guidelines can help direct your search. Most patients eventually find 2-3 different strains that work well for different purposes, such as one for daytime and one for sleep.

Medical marijuana strains fall into a few major categories: indica, sativa, hybrid indica/sativa, and high-CBD. While these terms are not scientifically accurate, they are widely used in the cannabis world. Furthermore, cannabis by one name from one producer may be vastly different than cannabis by the same name from a different producer. The best way to identify a strain that works or doesn’t work for you is to have it laboratory tested for cannabinoid and terpene content, and to remember the appearance and aroma infusing fresh buds.

CANNABIS SATIVA strains are tall plants with narrow leaves and buds often having a spicy or flowery fragrance. These varieties are generally more mentally stimulating,
more energetic and euphoric. They tend to help more with nausea and other abdominal complaints, appetite stimulation, headaches, depression and fatigue. Although side effects are uncommon and usually mild, sativa strains are more likely to cause or worsen anxiety or paranoia.

CANNABIS INDICA strains are shorter and bushier plants and often having sour or fruity fragrance. Indica varieties are typically relaxing and sedating. They tend to help more with pain, anxiety, muscle spasms, and insomnia.

HYBRID INDICA/SATIVA strains are very common, and have been bred to achieve specific qualities. Many hybrid strains are able to produce effects that provide the best of both worlds.

CBD-RICH strains have specifically been bred to produce high levels of cannabidiol (CBD). CBD has several
exciting medicinal properties, including anti-anxiety, anti-inflammatory, anti-tumor, anti-seizure, and pain relief. Strains with high levels of CBD have decreased our negligible psychoactivity, making them desirable forpatients who want the medical benefits without getting “high” or impaired. This can be ideal for patients who need to drive, work, etc.

We recommend you consult with an experienced healthcare provider to receive personalized recommendations on cannabis strains and cannabinoid ratios/dosages for your condition. If you cannot find an expert, you can network with other cannabis-using patients that have similar conditions and find out which strains work the best for them, but remember, unless you purchase from the same producer, you may end up with totally different product.

Cannabis does not have to be smoked.

The beneficial compounds in cannabis can be administered by inhaling smoke or vapors, ingesting cannabis added to food or drink, swallowing capsules, taking liquid extracts such as tinctures or oils absorbed through the mouth or swallowed, or applying cannabis lotions, salves or patches to the skin. Each delivery method will have a different medicinal effect, even when using the same strain of cannabis. There is no “best” delivery method – each has it’s benefits, drawbacks, and clinical utility in certain situations.

INHALATION: ONSET 1-5 MINUTES, DURATION 1-6 HOURS

Inhalation of cannabis has several advantages, including fast onset, ease in consuming the correct dosage, and convenience. It’s an ideal delivery for patients with nausea, vomiting, or other conditions that make swallowing difficult. Due to the rapid onset of inhaled cannabis, it’s easiest for users to judge their response and know how much the cannabis is helping and whether or not they need more. On the other hand, inhaled cannabis is more likely to cause cardiovascular side effects, has a shorter duration than other methods, and may have a higher abuse potential.

Smoking is perhaps the most traditional method of cannabis use, and while it works well for many patients,
it has some disadvantages. The heat and smoke irritate the respiratory tract and lungs and could potentially worsen conditions such as asthma and COPD. While the smoke itself contains carcinogenic (cancer-causing) substances, long term heavy marijuana smokers do not have an increased risk of lung cancer, likely due to the anti-cancer properties of cannabis offsetting the carcinogens in the smoke.

Herbal vaporizers are very popular with many medical marijuana patients, and are widely considered to be the healthiest way to inhale cannabis. Studies have shown vaporizers reduce the amount of harmful substances created compared to burning and smoking. The vaporizer heats the herb to a specific temperature between 300 and 400 degrees Fahrenheit, releasing the medicinal substances into a vapor without the plant material burning. The vapor produced is warm and non- irritating. It contains the smell and flavor of the cannabis but feels as if you are breathing air.

A wide variety of vaporizers can be found on the market today, and many require some practice before achieving optimal use. When a patient uses a vaporizer it may take a little longer for the therapeutic effects of the cannabis to be felt compared to smoking. Many novices make the initial mistake of taking too many puffs in their first use, thinking they are not getting the medicine because it does not feel like smoke in their lungs. You can avoid this mistake by following our Introduction to Cannabis and Cannabis Sensitization programs.

TOPICAL (EXTERNAL USE ON SKIN): ONSET AND DURATION VARIABLE

Cannabis or its oil-based extracts can be added to balms, lotions, salves, patches, and linaments. Many of these preparations can be made at home and applied topically to help alleviate pain, muscle spasms, inflammation, itching, and various skin conditions, including eczema. Topical use of cannabis typically does not produce psychoactive effects if used in small amounts and in small areas. Using large amounts over significant areas of the body can cause psychoactive effects. Topicals labeled “transdermals” are designed for better absorption and will have a full body effect. The efficacy of a topical preparation may depend on its potency, but many patients have found they can use flower/bud trimmings or leaves to produce an effective topical medicine.

LIQUID CANNABIS EXTRACTS: ONSET 10-45 MINUTES, DURATION 2-8 HOURS

A tincture is a liquid extraction of cannabis, often in a solution of alcohol. Sometimes the word tincture
is erroneously used to describe oil infusions, which can be used in a similar manner. Liquid extracts can be absorbed directly through the mucous membrane in the mouth for faster onset, or they can be swallowed.

This is one of the most versatile delivery methods and works well for most patients. They are convenient, discreet (minimal odor), and easy to dose correctly. The onset and duration are intermediate compared to ingestion or inhalation. Liquid medicines do have some challenges: active constituents can settle to the bottom of the bottle, the effects are variable if held in the mouth vs swallowed, alcohol tinctures can irritate the mouth and may be inappropriate for people with a history of alcohol abuse, and the taste can be bitter or spicy.

To use a liquid cannabis extract, a specific number of drops or milliliters can be placed under the tongue and
held for 1-2 minutes before swallowing. Brushing one’s teeth first can increase blood flow in the mouth and speed the onset. Because each batch of liquid medicine may have a different potency, be sure to look at laboratory testing results, or if unavailable, start each new batch with a few drops and increase slowly. When using an alcohol based tincture, add the liquid to a small amount of warm or hot water first – this will evaporate some of the alcohol and dilute the rest, preventing irritation to your mouth.

INGESTION: ONSET WITHIN 1-2 HOURS, DURATION 4-12 HOURS

Cannabis can be added to a wide range of capsules, foods, and beverages. When cannabis is swallowed, it is absorbed through the GI tract and then metabolized in the liver (known as first-pass metabolism), which changes the cannabinoids into a form with longer duration of action and somewhat different medical properties. Many users report ingestion produces a stronger psychoactive effect, while others report less psychoactivity, and most find that ingestion produces somewhat different therapeutic effects. Many patients find that once they know their optimal dose, ingestion is a superior delivery method due to convenience, long duration, and preferred effects. Ingestion can be complicated by erratic absorption from one day to the next – absorption may depend on other foods consumed, sleep, stress, and other factors that affect the motility of the gastrointestinal tract. Also, cannabis edibles may not have a homogenous distribution of cannabinoids throughout the entire product – one half of the cookie may be more potent than the other half.

It is often difficult to achieve the correct dosage when consuming cannabis edibles, mostly because it takes so long for the patient to know if he or she has taken
too little or too much. For these reasons, we emphasize caution when eating cannabis; it is a good idea to start with a very small amount, wait 2-3 hours, then repeat the dose if needed. While it is not dangerous to overdose on edible cannabis, the effects can be quite unpleasant, sometimes aggravating the symptoms a patient wishes to improve. Since so many patients make dosage mistakes with cannabis-infused foods and have unpleasant experiences, we recommend keeping novice cannabis users avoid edibles and use cannabis in the form of a medicine, not a food.

Please note that while raw cannabinoids have non- psychoactive therapeutic properties, the effects commonly associated with cannabis are due to cannabinoids that have been heated (decarboxylated). Before being consumed, cannabis must be heated to convert the cannabinoids into their active form.

RECTAL (Variable onset and duration)

Cannabis suppositories can be useful for treating conditions in the pelvis and low back, and in patients who are unable or prefer not to inhale or swallow cannabis. Some patients report that rectal administration is less-psychoactive than eating, which may be due to the avoidance of first-pass liver metabolism. The little research on rectal cannabis absorption shows wide variability in absorption, and the composition of the suppository can make a significant difference.

RAW CANNABIS PREPARATIONS (ONSET 2-45 MINUTES, DURATION 2-8 HOURS)

Almost all of the research on the therapeutic properties of cannabinoids has examined the compounds after they have been exposed to heat, an activation process known as decarboxylation. A small body of literature has shown that raw cannabinoids possess anti-inflammatory and anti- nausea properties at surprisingly low doses in rodents. Patients have reported that raw cannabinoids are also effective for treating pain and seizures.

Raw cannabis is non-psychoactive and has a higher content of terpenes, the substances that produce the aroma, which also have a broad range of therapeutic effects. While very large doses can cause some digestive upset, few other side effects have been reported from raw cannabis. Fresh raw cannabis flowers can be eaten directly (chewing for a few minutes can speed onset), prepared into an oil, or brewed into tea (boil 1g cannabis in 1 L of water for 15 minutes). This recipe of cannabis tea produces a beverage with 90% raw cannabinoids and 10% decarboxylated, and quickly loses its potency over the course of a week, even when stored in the refrigerator.

About Dr Sulak: Dr Dustin Sulak, D.O. is
a renowned integrative medicine physician based in Maine, whose practice balances the principles of osteopathy, mind-body medicine and medical cannabis. Regarded as an expert on medical cannabis nationally, Dr. Sulak educates medical providers and patients on its clinical use, while continuing to explore the therapeutic potential of this ancient yet emerging medicine. He received undergraduate degrees in nutrition science and biology from Indiana University, a doctorate of osteopathy from the Arizona College of Osteopathic Medicine, and completed an internship at Maine- Dartmouth Family Medicine Residency.

Material for this article was originally posted on www.Healer.com written by Dr. Dustin Sulak and may be viewed here http://healer.com/

Resources

Brenneisen, R., et al. “The effect of orally and rectally administered delta 9-tetrahydrocannabinol on spasticity: a pilot study with 2 patients.” International journal of clinical pharmacology and therapeutics 34.10 (1996): 446-452.

Earleywine, Mitch, and Sara Smucker Barnwell. “Decreased respiratory symptoms in cannabis users who vaporize.” Harm Reduct J 4.11 (2007).
Elsohly, Mahmoud A., et al. “Rectal bioavailability of delta- 9-tetrahydrocannabinol from various esters.” Pharmacology Biochemistry and Behavior 40.3 (1991): 497-502.

Gieringer, Dale, Joseph St. Laurent, and Scott Goodrich. “Cannabis vaporizer combines efficient delivery of THC with effective suppression of pyrolytic compounds.” Journal of Cannabis Therapeutics 4.1 (2004): 7-27.

Grotenhermen, Franjo. “Pharmacokinetics and pharmacodynamics of cannabinoids.” Clinical pharmacokinetics 42.4 (2003): 327-360.

Hart, Carl L., et al. “Comparison of smoked marijuana and oral Δ9-tetrahydrocannabinol in humans.”Psychopharmacology 164.4 (2002): 407-415.

Hazekamp, Arno, et al. “Cannabis tea revisited: A systematic evaluation of the cannabinoid composition of cannabis tea.” Journal of ethnopharmacology 113.1 (2007): 85-90.

Rock, E. M., et al. “Tetrahydrocannabinolic acid reduces nausea‐induced conditioned gaping in rats and vomiting in Suncus murinus.” British journal of pharmacology 170.3 (2013): 641-648.

Verhoeckx, Kitty CM, et al. “Unheated Cannabis sativa extracts and its major compound THC-acid have potential immuno- modulating properties not mediated by CB 1 and CB 2 receptor coupled pathways.”International immunopharmacology 6.4 (2006): 656-665.

The Art of Transitioning

Published: LifeLines for Health, vol. 7 (Spring 2016)

Written by: Janet Brewer, M.Ed

The Webster definition of transition is (n) passage from one state, stage, subject, or place to another (v) a movement, development, or evolution from one form, stage, or style to another. In our lifetime, we transition multiple times as life is always in perpetual motion. Heraclitus the Greek philosopher said, The only thing that is constant is change.

As families or individuals managing hemophilia with an inhibitor, we have become experts at transitioning. Our lives change in the blink of an eye. One moment we are sitting quietly enjoying a cup of coffee and the next thing we know we are inpatient. When life is going along “smoothly” we are waiting for the other shoe to drop. The art of transitioning is an ongoing process. One that begins the moment our lives are affected by hemophilia.

Transitioning in a school setting does not happen all at once either. There are four critical stages within the educational transitioning process; entry at the early childhood to kindergarten level, then middle school, high school, and finally college and/ or employment. Each level hasits own areas of concern and set of communication and skill development.

Stage 1: BE TRUE TO YOUR SCHOOL

One of the most critical aspects of this transition stage is cultivating an open, honest and trusting relationship with the elementary team. Our children are leaving our safe, controlled home environment and moving into what I would call as a teacher, “organized chaos”. There are now many more children surrounding our child, increasing the risk of injury. This is a very stressful transition for every parent and child (as well as the school system). The first step is to meet with the school and institute an individualized health care plan (IHCP) that establishes protocols for any possible scenario to keep our children safe. There will be a lot of communication between you and your child’s teacher and/or school nurse to solidify this relationship. Annual transitions as the child advances through the grades will require reinforcement of that relationship with your school system and teachers. Miscommunications and mistakes are to be expected at each end of the relationship. The vast majority of school systems, especially teachers want to work with you for the benefit of your child’s success; it is there job. Try to remember that your child is one of at least twenty to twenty-five students s/he is responsible for every day.

Learning about hemophilia does not happen overnight; it is a gradual progression of skills. The more often you practice appropriate communication skills to collaborate with your child’s school system, the better your skills will become.

Stage 2: STUCK IN THE MIDDLE WITH YOU

Middle school is the next critical stage within the transitioning process. The positive communication skills you have (hopefully) developed by now will lay the groundwork for this chapter of multiple teachers and subjects. If your child does not have a 504 plan going into middle school, they should, as they will now be learning from a team of teachers. There may also be new members of the team who will become the point person or central advocate for your child such as a guidance counselor or school nurse. You should request that your 13-14 year old child be invited to any meetings that involve them; which is all of them. They need to begin developing and honing the skills necessary to communicate their own needs effectively with your coaching. Some teachers may be uncomfortable with this or indicate that it is not their policy but by calmly explaining that you are teaching your child skills for a lifetime of self-advocacy and transitions, they will understand. You can expect that there may be three or four meetings per year during this transition stage for parent- teacher conferences. There will also be a minimum of two 504 meetings, one at the beginning of the year and one at the end to add, delete or modify accommodations. If your child has an IEP, there will be one annual meeting with your child’s team unless they are up for a re-evaluation that occurs every three years. During a re- evaluation year, you can expect more than one meeting, as eligibility is determined. Parents have asked me if they need to attend all these meeting every year and my response is always “YES”. Yes, it is many meetings. Dependent upon the health of your child and consistent communication, you may rely upon the point person in your team to update you regarding progress or concerns if you cannot attend them all. Make it a point however, to attend the first and last semester/trimester parent-teacher conferences. These are crucial for the year’s success and setting your child up for success and expectations for transitioning into the next grade.

Stage 3: THE TIDES ARE HIGH

This now leads us to the high school transition year. These next four years will move at lightening speed; if you thought the elementary or middle school years went fast, this period of time feels like you blinked and it is over. You and your child will now be negotiating with multipleteachers, multiple semesters and multiple meetings. Communication at this stage can be very difficult between you and your child. Make friends with your child’s teachers, you will need them! Chocolate and gift cards go a long way. Handwritten notes are ALWAYS appreciated. Make it a point to get to know the athletic director or PE teacher if your child has not been excused from PE. This period of non- communication that began in middle school with your child will likely become exasperating to negotiate. The end goal

is age 18 at which point your child is now considered an adult. YEP! Trust me, they use their well-established communication skills to remind you of it frequently starting with the year they turn 17. If your child’s high school has not linked them in with your states’ vocational rehabilitative agency by the age of 16, be sure to make that connection. Don’t forget your child should still be attending meetings (which they may not like) and the school district is federally obligated to invite them. It is wise to start asking by the time they are 15 to begin this process. Dependent upon the state, the eligibility process can take up to a year, so start early. You will find more information at http://www2.ed.gov/ programs/rsabvrs/index.html. Here youcan find facts regarding your individual state’s governance over the Rehabilitation Act of 1973 and what services you or your child may be afforded. Each state is awarded different amounts of funding from the federal government so resources vary from state to state https://rsa.ed.gov/ people.cfm.

Stage 4: SCHOOL’S OUT

No matter whether your child decides on a college career, a safe trade, or a year or two of figuring it out, they will need to use their self-advocacy skills with college admissions, disability services, or a boss. Once they reach 18, parents can no longer be provided with information regarding your child so it is all up to them. They can fall back on their resource contact from vocational rehabilitation no matter what career route they choose. Vocational rehabilitation services can include assistance with obtaining a job, training and in some states college funding. It is well worth the effort to get in touch with them! Just an aside, as a mother who has done the high school/college “rodeo” three times, relax! There is already so much pressure put on young adults these days. Here in Massachusetts with many prestigious private colleges, the message often received is that you must attend a private four-year institution and sleep away from home in order to be successful. This path may be the right one for some children, but not for all. Student loan debt is at an all time high with young adults coming out of college with massive debt and no jobs to help pay it. It will most likely take your child more than 4 years to complete their degree depending upon their health, their level of interest and how many times they change their major. Try to remember that they have lived a life that makes them wise beyond their years in some ways but very naïve in others. Each generation’s lifespan extends longer and longer into old age. In this country, we work hard for a long period of time where leisure and recreational activities are just now being recognized as important to our overall health. They will follow their own path. It will probably make us crazy as parents to watch the trails they go down and what trials they bring; but they were raised to be the best advocates for themselves and we have to trust we taught them well.

How to Thrive in Today’s World and M a n a g e Your ANGER (Fall 2016 Feature)

Published: LifeLines for Health, vol. 8 (Fall 2016)

By Matthew Manela, LICSW, LADC

In today’s world we are put to the test every day. A typical day for many of us involves getting kids to school, picking them up from school, driving one to practice and then the other to Karate all while thinking about what to make for dinner and cooking dinner! This is only the beginning of the night...next comes homework, baths, making school lunches and bed! This is all after working an 8-hour day! This is a good day with no additional stressors and frustrations! Consider a not-so-good day where you hit traffic, are late for work, or forget an important appointment. How can people thrive and live their lives without becoming obsessed with anger?

First, let’s start with why we get angry. Anger is a secondary emotion that is triggered when we are afraid of something negative happening and blame someone or something for the negative situation that occurred. We then personalize the negative event and see the person or thing as causing the event. Anger serves as a response to protect ourselves from being harmed. In addition, we continue to stay angry because we are having a hard time accepting the event for what is. It is our reaction to the event that triggers and maintains our anger. The problem with anger is that, while we are angry, we are not living life to its fullest in a way that is important to us. In order to best illustrate how to cope with anger, I developed a 12-step model to manage your anger.

Step 1: Recognize that we are powerless over our initial response to anger.

The first step to anger management is to accept that we are powerless over our initial reaction to anger. Through our life experiences, we have developed automatic thoughts that are based on the events of our lives. Over time, these events have created a response to protect us from being harmed. Unfortunately, we can’t control our automatic thoughts but we can observe and notice our thoughts. It does not matter what your initial thought is, but rather, what you do with the initial thought that dictates what emotion you will experience. If your next thoughts are focused around blame and being wronged, then you will feel angry. If your thoughts are more neutral then you will feel an emotion that corresponds to your thought process. The goal in this step is to begin to observe your thoughts and your reactions to the events in your life and begin to identify patterns.

Step 2: Willingness to accept that adjusting perspective is a necessary part of changing the anger-response cycle.

My 12 step model follows the philosophy that the event is not the cause of your anger, rather, your thoughts about the event are responsible for maintaining anger. In other words, life has a series of ups and downs, no one made you angry but rather you allow yourself to continue to be angry. This may be a very different way of thinking about anger than you have previously considered. As
a result, shifting to this new way of thinking may be challenging. That is the reason Step 2 is about getting ready to adjust your thoughts and take more responsibility around your anger.

One of the most difficult parts of learning to manage your anger is making a decision to change. The reason it is so hard is because we can become obsessed over our unmet needs. When we are angry we may find it impossible to think of anything except that another is “wrong” and we are “right”. The question that I often get is “what if you are right?.” The problem with this question is that anger has nothing to do with whether you are “right.” The question you need to ask yourself is whether or not you want to reduce and/or let go of your anger. To help make this decision you will need to think about and be clear about what is important to you. You need to ask yourself ‘how do I want others to see me?’ “What do I want my relationship with this person or this company to look like?” “How do I want to be remembered?” “What are my values and do these words and actions move me towards or away from meeting my values?”

Step 3: Make an inventory on what triggers us to get angry.

Once you have made the decision to reduce your anger, the next step is to observe your thoughts, expectations, needs and events that triggered your anger. In order to understand your anger and what triggers it, it is helpful to explore whether the thought that you are having is helping you move towards, or
away from your values. You want to explore if you are trying to control the situation with your anger, or if you are just reacting to the situation? Other factors to consider are the circumstances around you. Notice if anger episodes increase when you are tired or hungry. Notice what you were doing right before an anger episode and what impulses you experience. The reason for taking an Anger inventory is because it is your thoughts and perspectives that get you angry, not the event itself. If this is true, then knowing your triggers is a necessary step for anger reduction.

Step 4: Come to a full understanding of the Fear = Anger connection.

One of the biggest anger triggers is fear. The fear that I am referencing is based on our past experiences and are triggered in the present. For example, an individual with a learning disability may have been removed from their mainstream classroom as a child. A fear of not being liked or being different may develop. As an adult, when an event triggers the feeling of being left out an individual may become angry to protect themselves from being hurt. It is important to explore and identify your pain and how your fear tricks you in to thinking you can do something about it, by blaming others. When we get angry, we are trying to control
the event or person we see as causing our anger, instead of accepting it. In this step, we need to recognize how we are avoiding our pain. We need to decide what changes will bring us closer to being the person we want to be. This does not mean that we are powerless to change the negative aspects of our situation in the future, but rather, recognizing that staying angry does not help make the pain better over the long term.

Step 5: Compassion for others and not blaming them entirely allows us to be less angry.

In this step we move from collecting information about our anger, to making changes in the way we are thinking about the situation. To do this, we need to reduce the amount of blaming we are doing and replace it with compassion. What is compassion? Compassion is looking at an event or situation the way it is and not the way that we created it to be. Looking at things objectively is a difficult process. To increase objectivity, we need to reduce judgements and be open to alternative ways to explain other’s behavior. One way to do this is to adopt the philosophy that most people ‘s intentions are generally good. The reason we judge people is because the mind has a natural instinct to see things in black and white or right and wrong. However, when challenging your thoughts you need to bring compassion to your understanding of the current situation. You need to keep in mind while you’re practicing these skills that you don’t need to believe everything that the mind dishes up. Your job in this step is to try to bring compassion to what your mind comes up with. My suggestion is to practice this skill in places that only create simple frustrations. For example, try bringing compassion the next time you are waiting in a long line in the supermarket or in a traffic jam. Try focusing only on thoughts based on the facts of the situation.

Step 6: To identify that Anger-Triggering Thoughts are based on cognitive distortions (thinking errors). We recognize that these thoughts are based on assumptions and are not valid.

The thing about stressful emotions is that they only stay with us when we are feeding them with new negative thoughts. When we feel angry, we usually add to the original thought. For example, if I become angry that my son lied to me about something, I don’t usually say “Ok that’s fine.” My next thought is usually something like this - “That little bugger, he is always doing this to me.” No matter what you say to yourself, most likely it is based on an assumption, which causes a thinking error that maintains your anger. These are called cognitive distortions. When you are using cognitive distortions, you are also fusing with your anger. That is, you see your thoughts as 100% true and are not able to separate from your thoughts. When you fuse with your thoughts, you are not open to anything except how you are “right. “In this step, you learn to identify when you are fusing with your thoughts and using cognitive distortions . The following is some examples of cognitive distortions; should statements, jumping to conclusions, magnification, and over generalization. There are others but these are the main ones that create and maintain anger.

Step 7: To challenge and accept our cognitive distortions and move forward towards our values.

In this step, we are disproving the distortion and learning to separate from our thoughts. How can we separate from our thoughts?

In order to understand this concept you need to understand that we are not our thoughts and that our thoughts are not always true or accurate. In fact, thoughts are just thoughts. I might have a thought that I want a piece of cake but that does not magically make a piece of cake appear. The same rule applies to my thought that my coworker intentionally acted in a negative manner. Just because I think it, does not make it true. It may feel true and we may really convenience ourselves that it happened but without evidence, it’s not true. So what do we do with our thoughts? First, observe your thoughts and look at the facts. Next, notice your thoughts without giving them much attention. By just noticing your thoughts instead of feeding them you will begin the process of separating from your thoughts. You will begin to accept your thought. For example, let’s say that you are angry with a coworker. Which one feels better to you, my co-worker does not trust me or I am having a thought that my co-worker does not trust me. Telling yourself you are having a thought or noticing a thought allows you to accept your thought, which opens yourself up to more possibilities. It opens yourself up to looking at the issue in a different way and helps move towards actions that are important to you.

Step 8: To assert our needs and take responsibility for our actions.

Now that we have started the process of separating from our thoughts, we need to determine if we need to communicate about them. We don’t always need to tell someone that we are angry. Sometimes it makes sense to tell someone about your anger and problem solve with the person. In the spaces below, I have identified reasons we should communicate/problem solve.

1. To reduce the likelihood that you will hold onto a resentment

2. To reduce the chances that you will get angry at the same thing over and over again

3. To help others better understand your triggers

4. To help you accept the present

5. Gain clarity of your thoughts

6. To enhance understanding of your expectations

In deciding to engage in communication and problem-solving, we need to realize that we cannot expect others to act or think like ourselves. We can only take responsibility for our own actions and communicate what would be helpful for us while understanding that other people may not agree. When you communicate with someone you need to be willing to work with the person and have an open mind. Then together, we can begin to resolve an issue. For example, you decide to communicate your anger to someone for not understanding your needs. In order for that conversation to be helpful, you will need to be open to the other person’s point of view. If the two of you share your point of views with each other you are more likely to agree on a solution that you both are happy with. One tip when talking with someone, make sure you have done the other steps first. This way, you are clear why you are angry and you are not communicating while you are having angry thoughts. If you communicate while you are still angry your communication and ability to problem- solve suffers. We have a tendency to communicate things we don’t mean when we are still angry.

Step 9: To forgive or accept others and that our past resentments cause us to replay the same anger triggers over again.

In this step we are working towards accepting a situation for what it is and/or we are forgiving the person or thing involved. The word forgiveness is tricky because most people see forgiveness as saying that “what you did is Ok.” For our purposes, forgiveness is the process of accepting the situation and letting go of the event and being able to move forward. Forgiveness is for you and not the other person. It helps you move on and live a valuable life. In addition, forgiveness is not the same as forgetting.

For example, those who were alive for 9/11 know and remember the event like it was yesterday. I am sure you can tell me where you were when you heard the news. Although I have not forgotten, I have accepted the event and no longer hold on to the anger from that day.

The question I am often asked is “How do we forgive?” By the time you get to forgiveness you are already on your way. You have already acknowledged your anger, identified the hurt, fear, and judgement, and separated, and communicated your thoughts. The next part is making a decision to accept the situation for what it is by using the skills previously discussed and move closer to your desired view of yourself.

Step 10: The power of positive thinking and being in the present can help us maintain a more serene lifestyle.

Now that you have accepted the situation, it is now important to maintain your progress. In this step you are working on keeping your thoughts in the present and focusing your attention on the event and situation that you are participating in. Just like any other muscle you need to exercise your brain to learn to stay in the present. You will find if you practice staying present when you are not angry, then when you are tested, you will find it easier to work the anger management steps.

Another skill learned in this step is to increase your flexibility and your openness to possibilities. This takes practice. You can practice these skills in places that don’t cause too much anger. Perhaps the next time you are in the supermarket and you are annoyed by the cashier, you can practice identifying as many possibilities of why the line is moving slow. See if you can come up with positive and negative reasons. The purpose of the exercise is to open your mind up to the possibilities. It is not to be right. The better you get at different possibilities the better you will be at being flexible in the future. It is important not to compare yourself to other people, life is not a competition. Comparing yourself to others makes it hard to stay positive about the present. Instead, focus on what you like about your situation instead of what you don’t like.

Step 11: To continually take your inventory in order to maintain your hard work.

This step focuses around the maintenance of your newly learned anger management skills. In order to maintain your hard work, you need to keep paying attention to your thoughts and feelings on
a regular basis. Although, it is not possible to pay attention to your thoughts all the time, it is important to keep your values in the front of your mind and to stay focused on who you want to be, what you want your relationships with others to look like, and doing things that are important to you. Paying attention to your values allows you be open to different choices and possibilities. You also want to be aware of your triggers, thoughts, expectations and values. In this step you are focusing on what is important to you. The goal of this step is doing these things without feeding the anger tiger inside. It is important to be aware of what your barriers are to becoming the person you want to be. It is important to remind yourself of your values everyday so you can ask yourself, “Is this behavior and action towards or away from living the life I want to live?”

Step 12: Practice these principles and make a commitment towards your values

The final step is to help remind you to practice the principals on a regular basis in all areas of your life. It is important to practice these principles so when you get angry, (and you will get angry) you can handle your reaction and turn it around much faster. It’s important to remind yourself that it is up to you whether you will be driving yourself towards your values, or whether you will go on a detour. If there are barriers blocking your values, then you need to strengthen those skills by practicing. Practicing will help you to remove the barriers.

One thing to remember is that anger management is not an abstinence model. You will get angry but practicing will help maintain the speed that you accept the event or increase your ability to move quickly, let go of the anger, and go back to living a life that brings you enjoyment.

Here are the skills for a quick review:

1. Reduce judgments

2. Bring compassion to different situations

3. Accept events for what they are

4. Reduce expectations of others to think and act in the same way as you do

5. Practice flexibility when things don’t go as planned because they won’t

6. Things don’t need to be done perfectly or “correctly”

7. Open your mind to other viewpoints and possibilities

8. Stay in the present

9. Forgive/accept people and situations for you and not others

10. Keep your values and your inventory in the front of your mind

Anger can be an overwhelming emotion and it can be difficult to change the way we respond when we feel wronged. However, by practicing these 12 steps and learning how to manage your emotions, things might feel a little easier. In addition, these steps will help you to respond to things in a way that will help you achieve what is important to you including how we respond to others and how we see ourselves. These 12 steps are not a “cure all” and things do “stink” sometimes but by practicing these steps it may seem a little more possible to live a more productive and satisfying life.

Matt Manela is a Licensed, Independent, Clinical Social Worker, and a Licensed Alcohol and Drug Counselor who has developed, researched, and implemented his 12 Step Anger Management program. He received his bachelor’s degree from the University of Rhode Island and his master’s degree from Widener University. Matt has had positions as a clinical supervisor, clinical director, and full time private practice clinician. He is currently working at Mclean Hospital as a family therapist in a residential program for adolescents managing OCD and anxiety disorders. Matt has a small private practice where he conducts his successful anger management program. He is also an adjunct professor at Massasoit Community College.

Felt Angry Lately?

by Dr. Gary McClain, PhD

Published: LifeLines for Health, vol. 8 (Fall 2016)

Coping With Angry Feelings at Home

Felt angry lately? If you’re human, the answer is probably yes. So the better question is: What did you do about it? Something? Nothing? The wrong thing?

It’s only human to get angry. In fact, you’ve been getting angry since before you were even aware you could get angry; like that time when you were a few days old and you howled until the milk was delivered. Anger is just one more emotion along a whole range of emotions that are part of being human.

Here’s what’s ironic. While anger is an emotion that comes naturally, it is also the emotion we often have the most trouble feeling and expressing. And the emotion we often have the most trouble dealing with in others. We live in a world where we often witness anger that seems to get out of control. This leads to outbursts and hurt feelings or worse.

Life challenges bring up a lot of emotions, including anger. Living with a chronic condition, or living with someone who is living with a chronic condition, can certainly bring up a lot of anger. Whether the anger gets expressed or not, and what happens if it does, is complicated, to say the least.

Really Mad? Talk Yourself Down!

Here are some ideas on how to handle your own angry feelings:

Count to ten. 

Yes, this is one of the oldest tricks in the book. You’ve probably even seen it on a sitcom or two. But I can tell you from personal experience that it works. Counting to ten gives you a chance to think, and to calm yourself down, before you react in a way you might regret later. While you’re doing that countdown, don’t forget to breathe.

Step out of the story. 

Ask yourself if there is an old story behind your anger. If you hear yourself saying something like “this always happens to me” or “I never get what I want” then that’s a clue to a story you may be trying to repeat. React in the moment, to the current situation, and not to a situation in the past that this might remind you of. By staying in the moment, you will be more likely to keep that reaction from blowing up into something that it isn’t.

Reconsider your expectations. 

What were you expecting (to happen, receive, hear)? And were your expectations realistic? Sometimes anger is the result of not having our expectations met. Having realistic expectations is a great way to avoid the disappointment that can lead to angry feelings. And remember: you’re not in control of everything. Isn’t that a relief?

Get clear with yourself on why you’re feeling angry. 

Often, you can identify what is making you angry. Something that happened or didn’t happen. Something that someone else did or said. Situations that feel out of your control. So take a step back and look inside to

see if you can identify what’s pushing your anger button. Other times you just feel bad about the challenges that come with a chronic condition, and just want to vent. Whatever the reason is, be aware of it.

Look for the humor. 

Recognizing the humor in a situation goes a long way toward balancing out that rage-rush that anger can bring on, as well as help you to keep your perspective. How about having a good laugh about how life just seems to get in the way of our plans?

Have a heart. 

Chronic conditions bring up all kinds of emotions. Accept your own feelings – all of them – and let yourself be human. Give yourself a break, and you’ll be that much more able to give others a break, too. Patience! We’re all in this together.

Reach out for support. 

Talk to a friend or family member who can listen without judging you for your feelings or trying to tell you what to do. Vent if you need to. Let those angry feelings out.

Take care of yourself. 

Are you placing the needs of others before your own needs? Not taking care of yourself can leave you feeling emotionally or physically depleted, and especially vulnerable to resentment that leads to angry feelings. Take time for yourself every day. Take a walk, listen to music, do something you enjoy. Feeling angry is only human. You don’t have to avoid feeling angry, and you don’t have to be controlled by your anger. You have a choice!

Use Anger Productively in Family Communications

Now... let’s talk about anger that gets directed toward other family members. When one member of the family is living with a chronic condition, everybody is living with it. Day-to-day interactions at home can get tense sometimes. And when that happens, tempers can flare. Here’s how to communicate when you’re angry:

Express your feelings with the “I word.” 

Start out the conversation with your partner or other family member by expressing how you feel. It can be helpful to use the “I” word and not the “you” word. Make the conversation about how you feel, not how you assume the other person is feeling. Take responsibility.

Focus on the issue. 

Talking about your anger will be a lot more productive if you focus on the specific issue you are angry about. A bad day when you had plans. Something they said or did that caused you to feel hurt. Another change you have to make to accommodate your or a family member’s chronic condition. But not everything that has made you mad over the last 10 years. Caution: consistently talking about angry feelings as they arise will help you to avoid the big build-up that can lead to an explosion.

Let your family member know what you need. 

Sometimes we just feel angry because of the challenges of life. No one knows this better than someone who is living with a chronic condition. You may need a little time to vent and get some support. Other times, the issue may be one that needs to be addressed with action. Your family member can’t read your mind. So tell each other what you need. The best way to promote peace is for everyone to be aware of each other’s hot buttons, and what everybody can do to either avoid pushing them, and how to get back on track after they have been pushed.

Attend to any collateral damage. For example, if your kids had to watch their parents hollering at each other. That can be scary for kids. A few hugs may be in order, along with giving them an opportunity to talk about what it was like for them, followed by some reassurance. If friends or family members were within earshot (not to mention the neighbors), you may want to reach out to them as well.

And Help Family Members Express Their Anger

And when you are on the receiving end of anger, here are some ideas that might help:

Listen. When your partner or family member directs anger toward you, your first reaction may be to put up a barrier or to become defensive. Try to remain calm and open. One way to do this is to remind yourself that they may be having a bad day and need to vent. But it may also be possible that they need to say something that, once you become aware of it, can promote more peace and harmony in your relationship. Listen and learn.

Help the other person identify the issue. Your partner or family member may not be crystal clear about what he or she is angry about. One way to help keep the anger from escalating, and potentially help to identify what is bothering them, is by asking a simple question: “How can I help you?” This shows you are listening and care. And by asking it, your family member may be able to identify what’s at stake, or to realize they simply need a listening ear.

Talk. Listen. Understand. Don’t expect to solve. Anger doesn’t always lead to a solution. Sometimes the lack of a solution is what leads to the anger in the first place. Be okay with sharing feelings and understanding each other. Be okay with acknowledging each other’s anger. Recognize what is in your control and what is not in your control, what can and can’t be changed, where you agree, and where you agree to disagree.

It’s not okay to make someone else feel like a punching bag. And you don’t have to feel like one. Sure, chronic conditions introduce lots of challenges and frustrations. At some point, it may be necessary to gently let your partner know you are willing to help, and listen, but that you aren’t there to be verbally abused. Repeat as needed.

Caution: Unexpressed anger can lead to depression. Depression is sometimes referred to as anger turned inward. Create an environment at home in which it’s okay to talk about feelings. Watch your children for signs of depression! Symptoms can include withdrawing, loss of interest, changes in eating or sleeping habits. It may be time to sit down and have a talk.

Remember:

Compassion is a boomerang. Show love to yourself by feeling how you feel and not denying your own
emotions. Show love to your family members by being willing to hear how they are feeling, even when the conversation isn’t so easy to listen to. That’s compassion.

How about starting the conversation with: “I love you.”

Gary McClain, PhD, LMHC, is a therapist, patient advocate, and author, specializing in helping clients deal with the emotional impact of chronic and life-threatening illnesses, as well as their families and professional caregivers. He works with them to understand and cope with their emotions, to learn about their lifestyle and treatment options, to maintain compliance with medical regimens, to communicate effectively with the medical establishment, and to listen to their own inner voice as they make decisions about the future. His email is: gary@JustGotDiagnosed.com. He welcomes your questions and comments.

How to Approach a 504, (or any other meeting at your child’s school)

Published: LifeLines for Health, vol. 8 (Fall 2016)

by Lisa Cosseboom, M.Ed. & C.A.G.S School Psychologist & Special Education Evaluation Team Chairperson

For parents, the process of going into the school for a meeting produces anxiety, if not some level of fear. So often, it is one or two parents entering a room, quite often filled with multiple educators, including teachers, special educators, nurses, specialists, counselors etc. The process can be daunting and intimidating. Acronyms like IEP, LRE, FAPE are thrown around and parents frequently don’t know what those acronyms mean. As an Evaluation Team Chair, I see parents’ frustration, or even worse them disconnect from the meeting process altogether. This often produces tension amongst the Team members and parents feel that they need to come to a meeting with their defenses up and ready to battle! Complicating matters is that often a school team is uninformed about the bleeding disorder community and the impact a bleeding disorder has on a child’s education.

When parents come to a meeting with an initial sense of anger or frustration, the emotional outcome of the meeting is not going to be productive or what is in the best interest of the child and is most likely to fracture the entire team process. As the person who facilitates the IEP meeting, I generally can sense the direction the meeting is going to go when I introduce myself to the parents to bring them into the meeting room. Disposition and attitude of the parents are generally evident upon this first greeting as is the attitude and disposition of the school team. It is extremely important that the school based team and the family work together in the education process to strengthen the partnership between home and school. Research demonstrates that children perform better in school when parents are more informed and involved in their education process. Additionally, this involvement fosters relationships with the teachers, nurses and specialists that help keep them informed of your child’s current medical/emotional needs!

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Some things for parents to keep in mind when approaching a 504 or IEP Team meeting:

1. Approaching a meeting making demands will not be productive, even if the demands are legitimate or reasonable. It is important to model the behavior that you want drawn out in your child’s plan.

2. It is important to recognize that having the mentality of you vs. them is not useful. The Team approach should be a “we” approach. The parents are part of the Team!

3. When parents are asked about their thoughts or concerns, it is just as important to comment on the positive as well as the concerns. Positive comments go a long way to help facilitate a positive atmosphere!

4. Always keep in mind that the Team is there to focus on the child, not any one person’s agenda.

5. It is important for parents to be knowledgeable in the process. If you are unsure of the regulations
of an IEP or 504 meeting, don’t be afraid to reach out to your school for clarification.

6. Provide information about your child’s bleeding disorder to the school-based team ahead of time so that they may make the time to read and research!

School Teams are invested in the process and want things to be positive and work together collaboratively with families. It is vital that all parties come to the table with the child and the child’s needs in mind in relationship to school functioning and success. Most educators go into the education field because of their love of children and their love of watching children grow and succeed. They work hard, long hours, for little pay and just want to make a difference for children and families. Approaching meetings positively will almost always yield positive results!

LEVERAGE: a Molehill Practice for a Mountainous Adventure

By: Justin Levesque

Published: Lifelines for Health, Winter 2017

Some things in life never change, like living with severe hemophilia. Treatment options have come and gone and of course, there’s the promise of new therapies on the horizon. But on a fundamental level, I’ll always have these mutated genes to keep me company. It wasn’t until recently though that the perception of my bleeding disorder was turned upside down with the sudden development of a high-titre inhibitor at twenty-five (I’m now thirty-one). Things were really rough those first few years. With lots of bleeds and a dash of emotional defeat, I only exacerbated the impact of my perceived epic misfortune by isolating from others, imposing limitations upon myself, and no longer seeking out life’s great adventures.

It’s a longer story than this space allows but eventually, having an inhibitor (and everything that comes with it) became integrated into everyday life and I got back to sustaining a creative practice of making artworks and photographs. Most photographers dream of the opportunity to travel to remote places and make pictures. While it’s a dream I also shared, I was always deeply worried about my ability to do so; this fear now bigger since I developed an inhibitor.

Last year I came across a “Call For Applicants” to The Arctic Circle artist residency program. The Arctic Circle residency is a highly competitive program that brings together thirty international artists of all disciplines, scientists, architects, and educators who collectively explore remote and fascinating destinations aboard an ice-class Tall Ship and takes place in the international territory of Svalbard, a mountainous Arctic archipelago just 10 degrees from the North Pole. On a whim, I applied thinking the chances were pretty slim. But a few months later, I was beyond stunned to learn of my acceptance and that I was now invited to go north in June 2017.

Of course, I was excited. So excited. And while I still couldn’t believe it, I bombarded myself with questions about the reality of my body in the Arctic: Can someone with a inhibitor go tot heArctic? Should I disclose my disorder to the program beforehand and potentially jeopardize the opportunity? What if the activities are beyond my physical capability but everyone else is doing it? Do I push myself and risk a bleeder catastrophe? Will I miss out on seeing parts of the Arctic landscape if I don’t do every adventure presented to me?

It was about this time that I was encouraged to participate in CHES’s Leverage inhibitor adventure program co-facilitated by GutMonkey at YMCA Camp Collins in the heavily wooded forests of Portland, Oregon. Leverage is described by CHES as, “Introducing a revolutionary, new program for young adults with inhibitors. This 5-day adventure delivers heart-pounding excitement that puts your inner strength to the test! Find out what you’re made of, and develop new skills that can help you become who you want to be.”

It probably sounds silly but I initially declined because “That’s too.. adventurous for someone with an inhibitor.” I’m not sure why my brain was OK with winging it in the Arctic while scoffing at the Pacific Northwest but the absurdity of my rationalization was thankfully pointed out to me. I prepared to fly to Oregon just a few weeks shy before heading North. It was, hands down, the best decision I could have made.

At Leverage, you’re surrounded by other folks with a bleeding disorder and an inhibitor just like you. It couldn’t be more relaxed and no one has to explain why you’re different than a “normal” bleeder. There’s structured adventurous activities (more on that later) and plenty of free time to explore the camp’s beautiful and serene natural environment. To top it off, GutMonkey and CHES’ supportive staff brings truly impactful educational opportunities that speak authentically to the difficulty of the lived bleeder experience and provide new skills and techniques to navigate that Inhib Life.

When it came time for adventure, there was a wide gamut of activities for us to participate in. I don’t use that word lightly, participate. From learning to throw a tomahawk to floating 60 feet in the air (they call it the Flying Squirrel) to rapelling down the side of a building with nothing but open ravine below you, the only thing that mattered was to participate. What I learned was that participation didn’t always mean being the person strapped in or required to use their body in some performative way. Participation was also helping to keep the ropes organized, shouting “You got this!” and just being present for your fellow campers.

If something was too beyond my comfort zone, I was affirmed and supported in my choice to pass on a particular activity. I relearned how to say, “No thanks” and be OK with that choice. No one judged me there but I also realized if anyone did, or rather, if anyone else in the world were to, that it didn’t matter. I was doing what I had to do for me. It was both practice and a practice. I got to try all this “saying no” stuff out, to hear the words coming out of my own mouth and gain comfort in their sound. And further, it was a mindset that developed while at Leverage. To be present in my own body, to pursue and be OK with my own motivations as reconciled with my known limitations. I was asked to push myself within reason but not become some kind of unattainable superhero.

I can’t stress enough how important all of these lessons would become and how often I would think back to this week in the woods while I explored the frozen Arctic Ocean. While some folks hiked every glacier they set their eyes on, I resolved to climb one. While some folks lugged every single piece of heavy gear to shore, I rotated one piece of photo gear for each landing where I could really focus on using that tool. While some folks didn’t think at all about their bodies and found themselves injured and quite sore, I was always listening to my body with an acute awareness to make choices accordingly and with confidence.

Thank you CHES. Thank you GutMonkey. Without Leverage, I’d have been frozen with fear instead of finding myself in the middle of a glacier just shy of the North Pole.

Taha’s Moment: Finding “Normal”

By Taha Amir

Published: Lifelines for Health, Winter 2017

I am a 32-year-old male with severe hemophilia A. I moved to the United States, from Saudi Arabia when I was about 7 years old. Within that year, the hematologist told my parents that I had inhibitors - and they were lost, confused, and devastated. My parents knew what hemophilia was because my mom’s brothers also had it. But inhibitors were unheard of. The hematologist said that my body does not accept the “regular” factor, and I needed a bypassing agent called FEIBA. My brother was born in 1992, and he also has hemophilia, but not inhibitors, thankfully. To me, he was considered a “normal” person.

As a kid growing up with hemophilia with an inhibitor, I always stood out. I had bruises, swollen joints, and I walked with a limp. If I wasn’t walking, I was either using a wheelchair, crutches, cane, or a walker. I felt left out because the other kids were playing and walking better than me.

My pediatric hematologist suggested that I attend a hemophilia camp, which I did. The first time I went, I was nervous and scared because I wasn’t in my safety net and away from my parents for the first time. Even at the hemophilia camp, I stood out. I was still “different” from them because I had inhibitors. The “normal” hemophiliacs were still better than I was. Despite my struggles at camp, I attended a couple times after that because I was accepted. It felt like they knew me!

As a teen, I really didn’t attend any hemophilia events because it seemed like I was always bleeding. My younger brother, who also has hemophilia without inhibitors, was able to do more than I could. I felt as if I was the only one who had a different kind of hemophilia, which I did.

As I graduated high school, I decided to change my outlook on life. I attended a hemophilia conference, and met some amazing friends whom I still keep in contact with. At one of these events, I met the co-founder of CHES. She introduced herself and what CHES does for the hemophilia community - especially the inhibitor patients/community. One of the programs they offer is called Momentum, a men’s inhibitor retreat.

So far, I have attended Momentum twice. In July, I had started Immune Tolerance Therapy (ITT) in April 2017. By the time I went to the event, my inhibitor level was 0. Even though it had been 0 for a few months, the hematologists still hadn’t declared tolerization. I still have to do daily infusions of factor for the next few months.

Each time I have gone to the Momentum event, I have learned something new and met some amazing men who actually understand my struggles. The retreat is a three- day event over the weekend. The first day was dinner and a small informative talk by Dr. Dustin Sulak about the uses of medicinal cannabis for both acute and chronic pain. The second day started off with breakfast, a few more educational talks from treatment products to pain management, followed by lunch and more great information like infusion tips and tricks and practical career paths. One of the hot topics of this year’s retreat was health insurance and the current changes by NHF’s Michelle Rice. In the evening, the group went out for dinner and entertainment.

Most of the previous events that I’ve attended were primarily catered to “just normal hemophiliacs.” At Momentum, I feel accepted and better about myself. It has really changed the way I look at events for hemophilia with inhibitors.

What Inhibitor Family Camp Means to the Family

By  Cheryl Cook

Published: Lifelines for Health, Winter 2017

How do I even begin to tell anyone what Inhibitor Family Camp means to our family? How do you explain that it was a life-changing event?

My son is a mild hemophiliac, but we didn’t even know what an inhibitor was until he was diagnosed with one.
Our life changed in a minute. I heard about this camp from someone who had been there before, so I looked into it. We registered and “yeah!” we heard we were going!!

However, I was told by many that Jake should not go. His needle phobia required five (5) adults to hold him down for every infusion. We had health care companies that refused to work with us. We were giving infusions everyday. He was a mess; I was a mess. After much thought, I called to let CHES know we would not be coming to camp this year.

There was this voice on the other end of the phone, (I could barely hear because of the tears I was crying - saying we would not be coming.) It was another mom. The mom who said this camp was something she knew people needed; this was Janet Brewer (of CHES.) She told me to get Jake on that plane. She knew what I was going through and assured me that she would be there. She had survived through these same times and would help us get through them. She said she believed this would be something that Jake would never forget. She was so right. That weekend CHANGED Jake’s life!

When we got back from camp, none of his doctors or nurses believed this was the same kid. He was, but something had changed. I am still not sure what exactly happened to my 7-year-old that weekend, but he had
a different attitude and outlook. He was better able to accept this inhibitor diagnosis. He said it was because he was able to hang out with kids who “got” it. He said he felt that if they could do it, he could do it. We still have some stumbling blocks, but each time we go, it gets better.

The second year we were able to go, my son learned to self-infuse! Wow, what one year can do! The third year, he was excited about seeing his friends. He looks forward to hearing that registration has opened. He looks forward to seeing who is going to be there. He gets to go to Inhibitor Family Camp and be a kid; be with other kids, just like him, and no one is looking at his port. No one is wondering why he has it, or what is wrong with him? He’s able to just be a kid.

I asked him to describe what this camp means to him, and he turned around and said one word, ”JOY!”

All I can say is, “thank you!”

Pain and the Power to Heal - Winter 2017 Feature

By: Georgia Panopoulas, PhD, LP - Pain Psychologist and Janet Brewer, M.Ed

A few days ago, I came across an interesting article in the Minneapolis Star Tribune, and a very timely article, in retrospect. The author of the article titled “Opioids: Epidemic is the symptom, not the disease,” argued that “one of the reasons we have an opioid epidemic is because we have a pain epidemic.”

There is no arguing that pain, both acute and chronic, is prevalent in the United States and around the globe. Although trends in the prevalence of pain disorders are generally lacking, one study found that for chronic, debilitating low back pain (LBP), prevalence increased from 3.9% in 1992 to 10.2% in 2006. Increases were seen across all adult ages, for females and males, and across race. Reasons for the observed increase in prevalence rates were unclear, however, researchers suspected that increasing rates of obesity, changes in psychosocial and physical work demands, increase prevalence of depression, and increases in symptom awareness and reporting contributed to these increases in prevalence rates.

Pain in the Bleeding Disorders Community

Would these findings stand true within the bleeding disorders community? Witkop et al. (2017) argued that as life expectancy of persons with hemophilia (PWH) increases, pain and specifically joint pain is a major problem affecting adult PWH. Their study (the Hemophilia Experiences, Results and Opportunities study; HERO) found 85% of participants experienced pain during the 6-month period prior to completing the survey. Twenty percent of PWH (77% with Hemophilia A, 23% with Hemophilia B, and 9% with inhibitors, median age of 34 years) reported experiencing acute pain only, 34% reported experiencing chronic pain, and 32% reported experiencing both acute and chronic pain. Ankles (37%), knees (24%), and elbows (19%) were identified as the most painful joint. By comparison, 11.2 % of the general population in the United States experiences chronic pain, with LBP being the most common, followed by headache or migraine, neck pain, and facial pain.

In 2014, the Food and Drug Administration (FDA) sponsored a public forum titled “Patient-Focused Drug Development for Hemophilia A, Hemophilia B, von Willebrand Disease, and Other Heritable Bleeding Disorders” in an effort to learn from those affected about their treatment options and treatment concerns. A major topic of concern was pain management (both acute and chronic).

Definition of Pain

For the purpose of this article, pain is defined by the International Association for the Study of Pain (IASP) as: “An unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage.” IASP also added that pain is a subjective experience. Acute pain typically lasts less than 3 to 6 months. In PWH, acute pain is common during joint or muscle bleeds.

Chronic pain is defined as pain that persists longer than 1 month beyond the normal healing period or that is associated with a pathological process (e.g., arthropathy) that causes continuous or recurrent pain over months or years.

Chronic Pain Syndrome (CPS) effects approximately 25% of people with chronic pain. CPS occurs when people have symptoms beyond pain alone, like depression, anxiety, sleep disturbance, which interfere with daily functioning.

Why is pain so difficult to treat? The pain experience is subjective by nature. Contrary to 17th century thought that pain was either physical or psychic in origin, physical and mental aspects of pain as inseparable was the focus of medical research by the early part of the 19th century. By the 20th century, the Gate Control Theory of Pain emphasized the role of sensation, affect, and cognition “interacting with one another to provide perceptual information that ultimately influences the motor mechanisms characterizing pain,” (Olson, 2013). Further, as pain evolves from acute to chronic, it alters neural pathways, impacting mood and interfering with sleep.

Pain Management Requires a Variety of Approaches

As such, treatment requires an interdisciplinary approach focusing on physical, cognitive and emotional aspects of pain, in addition to focusing on pain and its impact on relationships. Opioids alone cannot successfully treat pain. In fact, successful treatment with opioids is considered to be 40-50% relief in pain (according to a scale of pain from 0-10, 0 representing “no pain,” and 10 representing “the worst pain imaginable”). Thus, successful treatment using opioids alone would result in a decrease of pain from 10/10 to 5-6/10. Opioids cannot eradicate the pain, much less “treat” the cognitive, emotional, and relational aspects of pain. Additionally, opioids are not without their negative side effects which include sedation, dizziness, nausea, vomiting, constipation, physical dependence, tolerance and respiratory depression. Long term use can result in allodynia and hyperalgesia (thus pain increases over time). From a psychological perspective, opioids and marijuana are dissociative drugs, numbing physical and emotional distress, making it more difficult for individuals to stay connected with loved ones and to find purpose and meaning in life.

Managing Physical Aspects of Pain

Managing the physical aspects of pain may require use of factor (in the case of acute pain, or chronic pain with acute exacerbation, or flare), RICE (rest, ice, compression, elevation), breathing exercises, relaxation exercises, gentle stretching, etc., in addition to certain medications. The goal is to manage the physiological arousal that often results from pain, but that only serves to feed right back into the pain experience. Pain is perceived by the brain as a potential threat or danger to the system. As such, pain activates the sympathetic nervous system (SNS), the fight or flight response, resulting in increases in blood pressure, heart rate, fuel availability, adrenaline, oxygen circulation to the organs, blood clotting, pupil size, while decreasing fuel storage, digestion, and salivation. Rest, which is essential for healing, becomes more difficult. Activation of the parasympathetic nervous system (PNS) is essential to counteracting the effects of the SNS. Activation of the PNS can be accomplished through meditation, massage, deep breathing, being in nature, playing with children or animals, yoga, tai chi, mindful movement. Finding the balance between relaxation and reactivation can strengthen the relaxation response and serve as an immediate defense against the effects of pain.

Cognition and Pain

Managing the cognitive aspects of pain may at first seem trivial, unimportant. Can pain management be as simple as mind over mood? Of course not. And in fact, we discussed previously that it’s not mind over mood, but mind and mood. Our pain is based on our perceptions, our perceptions are based on what we know, and what we know is interpreted based on what we have experienced in the past. Not knowing if our pain is a result of a bleed can be very disconcerting. Do we treat? Do we ignore the pain and push through? Are we thinking realistically about the situation or are we engaging in distorted, unrealistic, or irrational thoughts like:

*(Adapted from Margaret Caudiull’s Managing Pain Before It Manages You)

If you recognize any of these thought patterns, you are not alone. But once you recognize them, try to change them, not by thinking positively (for positive thinking can be as distorted as our negative thinking), but by thinking realistically/ rationally/with the evidence.

Emotional Well-being

The importance of managing emotional aspects of pain cannot be overstated. The prevalence rates of depression and anxiety in PWH are indeed higher than what we would expect in the general population and higher and/ or comparable to rates of emotional distress in persons with other chronic pain conditions (e.g., lower back pain, headache/migraine, neck pain, facial pain). Sadness, anger, fear are real and justifiable emotional responses to chronic illness and chronic pain, but can be managed. First identify what you are feeling. I like having a cheat sheet of “feeling words.”

Ultimately, however, our feelings fall into one of four categories: mad, glad, sad, and scared. What are you feeling? What do you attribute the feelings to? Something that just happened or something that happened in the past? Can you do anything about the situation that is contributing to how you feel? If not, can you allow yourself to feel without trying to change the feeling (i.e., mindfulness)? Can you allow yourself to feel anger, sadness, grief and loss, forgiveness toward yourself and others, and to accept these feelings as they are, real and valid? Or do we too quickly assign judgment to these feelings?

Keep in mind that feelings of grief, sadness, loss apply to both the parent/caregiver and the person with pain.
Guilt can be a source of great pain for mothers given that hemophilia is a x-linked chromosomal disorder. “It is my fault my son suffers because I gave it to him”. Or, as the person in pain, “I am a burden to my wife/children because I can’t do the things I once did which falls on them.”

Pain and Relationships

As much as we would like to think that pain only impacts the person with pain, we know otherwise. Pain impacts those we love deeply. Family and friends want to help, they want to “fix it.” They can’t. They become frustrated and, at times, it seems as if they are frustrated with the person who has pain. Their response to the person with pain can be perceived as “punishing.” Or maybe overly “helpful.” The research suggests that these responses are common, but not helpful especially when attempting to manage chronic pain. So, what are family members to do? “Distracting” responses can be most helpful. What does that mean? Offer understanding, validation. But more than that, I encourage family and friends to ask, “What do we need to do to get through this.” The “we” refers to the person in pain along with the family member or friend. This, of course, is a work in progress and requires communication, willingness to try new things, and reflection. When it all gets overwhelming, there is the propensity to shut down and pull away. Parents watching their children withdraw is painful. Trying to help seems to make it worse because the child may not want to talk about it. Ultimately, we may seek counseling and/or use mood enhancing medications to get us through. As much as we’d like to put the bleed, or the flare, or the crisis behind us, it would serve us better if we talked about how we got through, what helped, what didn’t, and what we might want to do different next time, because there will always be a next time.

The Power to Heal

In 2016, I attended the NHF Inhibitor Summits. Dr. Tyler Buckner and I presented several sessions on pain management (an overview of strategies for managing acute and chronic pain). Dr. Buckner presented information about various classes of medications including but not limited to opioids and medical cannabis. The utility of these medications, as indicated previously, was variable at best.But the participants, those living with inhibitors, shared their favorite pain coping strategies with the group and, once again, I left the Summits truly believing that I learned more from the Summit participants than they learned from me. The following is a list of pain coping strategies identified by participants (including PWH, parents/ caregivers, siblings, significant others):

Assistive Devices

• Poke program*check out on our website in reference section

• Smart phone apps, i.e. - Healing Buddies Comfort Kit

  http://www.healingbuddiescomfort.org/

• Virtual reality (see related articles in what’s new on page 42)

• Buzzy (see related articles in what’s new on page 46)

Distraction-Passive and Active

• Blow bubbles

• Listen to or create music

• Play games

• Read/Listen to Stories

• Watch movies

• Utilize social media

Emotional Strategies

• Calming Techniques

• Change your thoughts-
  positive self-talk and reinforcement

• Coping

• Counseling

• Grief and loss acceptance

• Humor

• Pain Education

• Set realistic goals/expectation

Movement

• Balance, coordination, ball therapy

• Change position

• Dance

• Exercise/Gentle stretching

• Mini Breaks

• Tai Chi

• Walk/pace/rocking

• Yoga

Mindfulness

• Attention Management

• Breathing

• Guided imagery

• Meditation

• Progress muscle relaxation/body scan

• Self-hypnosis

• Set realistic expectations of self/others

Physical Comfort Measures

• Acupuncture

• Acupressure

• Beverages

• Biofeedback

• Contrast baths-Epsom salts

• Cuddle

• Dim lighting, noise reduction

• Essential oils, salves, rubs/ aromatherapy

• Food

• Hot/Cold packs/pads

• Loose, soft clothing

• Massage

• RICE

• Sleep

• TENS (Transcutaneous electrical nerve stimulation)

• Management-anti-inflammatory, gluten, soy, soda, sugar free diet

  This list is far from exhaustive! But these strategies are tried and true. Will we eradicate the pain? Can we eradicate the pain? Should we eradicate the pain? Remember, pain is not all in our head, but that’s where pain messages are processed and where we can begin to alter neural pathways and change the suffering.

Managing Inhibitors in Hemophilia Treatment: The Importance of Bypassing Agents

Bypassing Agents are a Factor option with a long history of use in treating hemophilia patients with inhibitors

By: Mike Denne

Published: Lifelines for Health, Winter 2017

Treatment of inhibitors is one of the greatest challenges in hemophilia today

Up to 33% of people with severe hemophilia A develop an inhibitor, or antibody, to treatment, while 3% to 13% of patients with moderate to mild hemophilia develop an inhibitor.1,2 (Inhibitors are less common among people with hemophilia B, affecting only 1% to 6% of individuals.1) These inhibitors stop, or block, Factor VIII (FVIII) or Factor IX (FIX) function in your blood-clotting process. Bypassing Agents offer an alternative treatment approach because they go around, or bypass, the need for FVIII or FIX.3

Bypassing Agents supplement other Factors in the blood-clotting process

This chart is a simplified visualization of Bypassing Agent activity in the blood-clotting process.

REFERENCES:

1. DiMichele DM. Inhibitor treatment in haemophilias A and B: inhibitor diagnosis. Haemophilia. 2006;12(suppl 6):37-42.

2. Hay CRM. Factor VIII inhibitors in mild and moderate-severity haemophilia A. Haemophilia. 1998;4(4):558-563.

3. DiMichele DM. World Federation of Hemophilia website. Inhibitors in hemophilia: a primer.

   http://www1.wfh.org/publication/files/pdf-1122.pdf. Published 2008. Accessed October 5, 2017.

4. Lenting PJ, van Mourik JA, Mertens K. The life cycle of coagulation factor VIII in view of its structure and function. Blood. 1998;92(11):3983-3996.

5. Berg JM, Tymoczko JL, Stryer L. Many enzymes are activated by specific proteolytic cleavage. In: Biochemistry.

   5th ed. New York, NY: WH Freeman; 2002. https://www.ncbi.nlm.nih.gov/books/NBK22589/. Accessed September 19, 2017.

6. Johari V, Loke C. Brief overview of the coagulation cascade. Dis Mon. 2012;58:421-423.

7. Retzios AD. The new and improved (?) activated factor VII molecules. Bay Clinical R&D Services. 2001:1-41.

8. Shapiro AD, Hedner U. Advances in bypassing agent therapy for hemophilia patients with inhibitors to close care gaps and improve outcomes. Ther Adv Drug Saf. 2011;2(5):213-225.

9. Ananyeva NM, Lee TK, Jain N, Shima M, Saenko EL. Inhibitors in hemophilia A: advances in elucidation of inhibitory mechanisms and in inhibitor management with bypassing agents. Semin Thromb Hemost. 2009;35(8):735-751.

10. Leissinger CA. Prevention of bleeds in hemophilia patients with inhibitors: emerging data and clinical direction. Am J Hematol. 2004;77(2):187-193.

11. Wolberg AS. Plasma and cellular contributions to fibrin network formation, structure and stability. Haemophilia. 2010;16(suppl 3):7-12.

12. Colvin BT. Physiology of haemostasis. Vox Sang. 2004;87(suppl 1):S43-S46.

13. Hoffman M, Monroe DM. A cell-based model of hemostasis. Thromb Haemost. 2001;85:958-965.

14. Hoffman M, Dargaud Y. Mechanisms and monitoring of bypassing agent therapy. J Thromb Haemost. 2012;10:1478-1485.

15. Astermark J, Donfield SM, DiMichele DM, et al. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FENOC Study. Blood. 2007;109(2):546-551.

16. Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388:187-197.

17. Hedner U. Recombinant activated factor VII: 30 years of research and innovation. Blood Rev. 2015;29(suppl1):S4-S8.

18. Hemophilia Federation of America. Treatment of hemophilia. http://www.hemophiliafed.org/bleeding-disorders/ hemophilia/treatment/. Accessed September 20, 2017.

19. Santagostino E, Escobar M, Ozelo M, et al. Recombinant activated factor VII in the treatment of bleeds and for the prevention of surgery-related bleeding in congenital haemophilia with inhibitors. Blood Rev. 2015;29(suppl1):S9-S18.

20. Mehta R, Parameswaran R, Shapiro AD. An overview of the history, clinical practice concerns, comparative studies and strategies to optimize therapy of bypassing agents. Haemophilia. 2006;12(suppl 6):54-61.

21. National Hemophilia Foundation. Medical and Scientific Advisory Council (MASAC) recommendation regarding the use of bypassing agents in patients with hemophilia A or B and inhibitors. MASAC document #167. 2006.

22. NIH Clinical Trials Registry. Ongoing and complete clinical trials using bypassing agents in hemophilia patients with inhibitors. https://clinicaltrials.gov/ct2/results/ details?term=hemophilia+a+with+inhibitors%2C+hemophilia+b+with+inhibitors&recr=Closed&cond=hemophilia +a+with+inhibitors%2C+hemophilia+b+with+inhibitors. Accessed September 19, 2017.

©2017 Shire US Inc., Lexington, MA 02421.
All rights reserved. 1-800-828-2088.
SHIRE and the Shire Logo are registered trademarks of Shire Pharmaceutical Holdings Ireland Limited or its affiliates. S34496 10/17

New Sub-Q Treatment is FDA-Approved for Inhibitors

by Eric Lowe

On November 11th 2017, Genentech announced that the study drug, widely known as ACE910 (emicizumab), had been FDA-approved for prophylaxis treatment

in children and adults with hemophilia A and inhibitors to prevent or lessen bleeding episodes. This approval snapped a streak of nearly 20 years for the inhibitor population on newly-approved treatment products.

Almost 1 in 3 hemophilia A patients that are treated with factor VIII replacement therapy tend to develop inhibitors against factor VIII, which can lead to a higher risk for bleeds and long-term joint damage. The FDA-approved drug, now named Hemlibra, bypasses that problem as it is a bispecific factor IXa- and factor X-directed antibody. This means its use can bring together the two proteins, which will activate the natural clotting cascade. Hemlibra also requires only once a week use as an injection under the skin.1

Hemlibra, has had positive results from two studies, HAVEN 1 (NCT02622321) for patients 12 years of age or older with hemophilia A with inhibitors, and HAVEN 2 (NCT02795767) for patients younger than 12 years with hemophilia A with inhibitors. Results indicated that Hemlibra significantly reduced bleeds in both adult and pediatric patients with hemophilia A.

“People with hemophilia A who develop inhibitors face significant challenges preventing and controlling bleeds and typically require infusions of medicine multiple times a week, which can be especially difficult for young children and their families,” Dr. Guy Young, professor of pediatrics at University of Southern California Keck School of Medicine, said in a press release. “This new medicine has been shown to reduce the frequency of bleeds compared to the currently available medicines and only needs to be injected once a week. This could make a meaningful difference for these children.”1

Results

Phase 3 of the HAVEN 1 study (in patients 12 or older) showed an 87% bleed-reduction when using Hemlibra compared to those who did not treat prophylactically, and a 79% bleed-reduction was reported for those who did treat prophylactically with a different bypassing agent (BPA) before switching to Hemlibra.

Similarly, the HAVEN 2 study (in patients younger than 12) also showed an 87% bleed-reduction when beginning prophylactic treatment with Hemlibra, and a 99% bleed- reduction when changing their prophylactic treatments from a BPA to Hemlibra. Common side effects of treatment were injection site reactions, headache, and joint pain (arthralgia).1

One death has occurred in the HAVEN 1 study back in February 2017. The patient received bypassing agents (BPA) after experiencing a serious rectal hemorrhage.2 The patient’s repeated use of the BPA in combination with the remaining residual amounts of Hemlibra in the patient’s system caused threat of serious thrombotic events. After discontinuation of the (BPA), the patient showed improvement. But Roche said the hemorrhage therapy was complicated because the patient declined blood transfusions. The trial investigator determined that the cause of death was the rectal hemorrhage, not emicizumab.2 You may read more details on this using the #2 resources link below.

Looking Ahead

Hemlibra’s availability in the US is currently emerging and evolving.

HAVEN 3 and HAVEN 4 studies are currently underway to evaluate the treatment’s efficacy and safety at other doses and schedules. HAVEN 3 (NCT02847637) is being conducted in patients 12 and older for those with hemophilia A without inhibitors with doses given once every 1-2 weeks.

HAVEN 4 (NCT03020160) is being conducted in patients 12 years or older, with or without inhibitors, with doses given every four weeks after an initial one-month “loading” period.

1. https://hemophilianewstoday. com/2017/11/17/fda-approves-hemlibra- by-genentech-to-treat-hemophilia-a- patients-with-factor-viii-inhibitors/?utm_ source=Hemophilia+News&utm_ campaign=58c1c52d86-RSS_EMAIL_ CAMPAIGN&utm_medium=email&utm_ term=0_ab10fdd11a-58c1c52d86-72167405

2. https://hemophilianewstoday.com/2017/02/24/ death-of-hemophiliac-in-clinical-trail-raises- more-concern-about-emicizumab/

When Someone You Love Hurts How Can You HELP?

by Dr. Gary McClain, PhD

Published: Lifelines for Health, Winter 2017

A while back, a politician said something to the effect of, “I feel your pain.”

These days, that line is generally used in a humorous sense. But if you have a partner or child who is living with pain, I suspect that line conjures up all kinds of thoughts and feelings for you. Most of them not very funny.

That’s because watching someone you love struggle with pain is anything but humorous. As a partner, as a parent. You know how hard things are for them, you can see it in their face, the way they move, and in the way they speak.

You want to say something to make them feel better. You might find yourself saying to your partner: “You’re gonna be just fine. And soon.” Or to your child: “”Where does it hurt? Let me make it better for you.”

And the way in which your loved one responded may not have been so gratifying. They may felt frustrated, or angry, because you were promising something you clearly couldn’t deliver on. Sure, you were just trying to be helpful. But it didn’t feel that way to them.

I think it is not an overstatement to say that having a loved one with chronic pain can really make you feel helpless.

Of course, you can’t take their pain away. Even if that’s what you wish you could do. But between doing absolutely nothing, and ridding them of their pain, there are actually a lot of things you can do to help a partner or child living with chronic pain.

Here are some ideas to consider:

Get Informed Together

Make it known that you are on stand-by. Let your partner or child know that you are standing by to help in not only gathering information, but making sense of it, whenever they are ready. Kids are especially in need of reassurance, so feel free to repeat as needed.

Do your own information-gathering. It’s not realistic to remain uninformed even if your loved one is locking you out of this process. Find information on your own so that you can educate yourself on how to be a better healthcare partner. And active information-gathering is a great way to help you cope with your own helpless feelings.

Let your loved one know you are getting informed. You don’t need to surf the Web in secret. Gently inform your loved one that you want to be as educated as possible and are doing your own research.

Offer to share your information. As you get educated, offer to share what you are learning. Use statements like: “I just found out about an interesting new treatment. Do you want to know about it?” or “I saw a list of foods that might help you. Any interest?” If you are a parent, your information-sharing may be needed to help explain any limits you need to set.

Encourage Adherence

Look for teachable moments. The long lectures get old after awhile. You might instead be alert for moments when you can gently point out your loved one’s lack of adherence. For example, if you notice they have missed a medication dosage, you can quietly remind them and ask if they would like you to help them remember. Parents may find using teachable moments especially helpful because younger kids and teens can be especially resistant to feeling lectured.

Use some “patient” education. If you find yourself scolding your loved one, or getting angry, or giving orders, you may also find that you’re not making a whole lot of progress. Instead, assume good intention on their part rather than a desire to cause you frustration and annoyance. Who knows? Your loved one may get the overall concept of self-care but not be sure how to actually make it happen. Kids often need additional guidance in establishing self-care routines. How about asking if you can make a suggestion before you launch in with one? And then, focus on what they could have done and not what they didn’t do.

Choose your battles. As you have probably learned already, when people feel pushed into doing something, they become more resistant. That’s true for your partner and it’s true for your children. So if, out of your own desire to help, you are turning everything into a battle of wills, your partner or kids may put up a wall. Be mindful of what is most important – like taking medication on schedule – versus what be less important. Use your judgment here.

When You Don’t Know What To Do...

Ask. It’s sad to me that so many people don’t take the time to ask questions. They assume to know already. Or they just don’t think about what their loved one needs. The simple question – “What can I do for you?” – shows how much you care. By asking it, you’re already one step toward helping your loved one feel more supported.

Listen. With an open mind. You might be surprised at what you hear from your loved one. Maybe you’ll learn that a simple gesture would help him/her have a better day. But you may also learn that your partner or child needs a lot more from you than you expected. You might hear about how overwhelmed they feel at times, or a lot of the time. A few disappointments or resentments might come up. Be open to what your loved one has to say.

Get specific

I have found that both caregivers and patients avoid talking about the support process itself, and what they are expecting of each other. Family members are often afraid that if they bring up the subject of support, their partner or child may assume that they are feeling burdened with their support tasks, or that they don’t want to help them anymore and are trying to find a way to break the news. In turn, your loved one may fear burdening you, but also doesn’t want to be made to feel incompetent by receiving too much care. Keep in mind that grown-ups, younger children, and teens can all feel disempowered because “hovering” over them can be interpreted as not trusting them to do what they can do to take care of themselves. Furthermore, when needs and expectations are not clear, this can result in missteps that can lead to tension.

Figure out a path forward. Talk about how you see your role — what you can and want to give, and what you think they need — as well as your partner’s or child’s expectations. While defining your role is going to be a work in progress, open communications can help you to build a solid foundation for moving forward, and for protecting your own health and well-being. Keep talking!

Watch the Silver Lining Talk

Don’t be a positive thinking police bully. Being told to “think positive” or to “stop feeling that way” is a tactic of the positive thinking police. Jumping in with a “yes but” and showering your loved one in rainbows and puppy dogs is another tactic. Denying feelings doesn’t make them go away.

Your loved one has the right to feel bad. Be sensitive to what your loved one needs to hear and doesn’t need to hear. They are living with this chronic pain. Some days are going to be harder than others. On a bad day, go into asking questions and listening mode. Keep in mind, you may be asked to step aside and just be quiet. When your partner says, “I’ll be okay, honey,” or your child says, “please leave me alone for awhile,” keep an eye out but back off.

Let your loved one know he/she doesn’t have to sit all alone with negative feelings. Be someone who can listen without telling them how they should be feeling or judging them. Encourage them to vent! When you release feelings into the light of day, they lose their power over you.

Give Your Loved One Their Space

Individuals living with chronic pain are facing a wide range of emotions. Many of these feelings are uncomfortable — a feeling, like fear or anger, may be so uncomfortable that they may not be able to even acknowledge feeling this way, let alone begin to express these feelings to someone else. Your loved one may feel so emotionally overwhelmed that they may shut down. And that’s scary for you to witness. Gently ask your partner or child how he or she is feeling, not only physically but emotionally, let them know you are here to listen. Remind them as needed, but don’t push.

Be willing to step aside. Keep in mind that, at least initially, some individuals are more comfortable opening up to people who are not their family members, and with whom they are less involved on a daily basis, like
a counselor or support group member. Don’t take this personally. Your partner may feel the need to protect you from his or her feelings, and so might your child. Give your loved one space to cope in a way that works for them.

Take Care of Yourself

Take ownership of your own helplessness. Human beings love being in control, and we love it so much that we tell ourselves we have control even when it is obvious that we don’t. And in a caregiving situation, that need to be in control can result in running ourselves into the ground trying to meet every possible need of someone we love to the point that we are running on empty. What parent hasn’t felt this way, right? In the process of depleting ourselves, we also risk alienating the people we care about by taking their own sense of control away from them.

Find your own support system. If you totally deplete yourself, you aren’t going to be helpful to anyone. Yes, I know you’re superhuman, but you’re still human. Find a safe place to talk about your own emotions — your fears and frustrations — and to get feedback and advice. Don’t be afraid to ask for help in coping with being a caregiver. And don’t be afraid to talk about how you feel, even the feelings that you aren’t so comfortable with, like anger. A trusted friend, a family member, a counselor, or a member of the clergy can be beneficial.

Express your own emotions. You may not feel comfortable admitting to emotions like fear, out of concern that they may come across as having a negative or pessimistic attitude. While it is realistic not to sound alarms, expressing your own concerns can help to create an atmosphere of honesty. If fear, for example, has become the “elephant in the room,” getting it out in the open can relieve the tension that results from talking around the emotions that are most likely on everybody’s mind. While you want to encourage and support your partner or child, he or she most likely wants to do the same thing for you. Open the door for both of you.

And recognize where you don’t have control. Here’s the hardest one of all. When you love someone, you also want the best for them. But nobody likes to be told what to do. Even if they probably need to be told what to do. Be a support, a cheerleader, and throw in some tough love when you need to. What you can do is be a supportive partner or parent. But you can’t make the pain go away. So focus on the goal of helping to motivate your partner or child to do everything they need to do to take the best possible care of him/ herself. Be a team!

You and your loved one. Here is some final advice that is guaranteed to make every day a better one. Begin and end the day with three words: “I love you.”

The Individualized Healthcare Plan: What’s It All About?

by Ann Marie M. Minichiello

Published: Lifelines for Health, Winter 2017

The Individualized Healthcare Plan

The Individualized Healthcare Plan (also known as IHP or IHCP) is a common tool used for students with special health needs. When you send your child off to school, you want to know that his or her medical needs will be met while there, and an IHP is just the place to start. A child with a bleeding disorder should have this plan in place and it should be developed by your school or district nurse. According to the National Council of State Boards of Nursing (2005), the development of an IHP must be the responsibility of a licensed nurse. However, in the absence of a school nurse, it is recommended that there still be a plan developed by caregivers, student, healthcare providers and designated school personnel to ensure continuity of care in the school setting.

The IHP is a document based on the nursing process and in essence, is a variation of a nursing care plan. It includes a nursing assessment, diagnosis, interventions, and student outcomes. It is meant to outline the health services that your child will receive in school. It should include how, when and where these services will be provided at school. It should be reviewed and updated annually or as often as the student’s health status changes. An IHP is considered a standard for good nursing practice.

Although an Individualized Healthcare Plan is a standard of school nursing care and should be in place for any student with mild to severe health care needs, there is no law that states your child has the right to this plan.

It is designed to address only medical and nursing issues that do not impact student learning. It does not address access to education in any way; it is strictly a formalized plan to address the medical issues of a student. It does however, provide documentation of the nursing process and can be used in a legal proceeding, should the school nurse’s conduct or performance ever result in such a case. That being said, if a legally protected plan is what you want for your child, then an IEP or 504 Plan would be the appropriate and necessary route to pursue.

If it is determined that your child is eligible for special education services, then an IHP can be included in the health portion of an IEP. An IHP should also be part of a 504 plan that your child qualifies for due to a medically related health condition. When the Americans with Disabilities Act of 1990 (ADA) was first written, it was considered to be fairly strict, but certain health conditions were not always viewed as disabling. A health condition has not always guaranteed qualification as having a disability under the 1990 Americans with Disabilities Act. For instance, it could have been argued that an individual with a bleeding disorder who successfully treated prophylactically might not have been considered to have a disability because his or her medical condition did not substantially limit a major life activity. In 2008, the ADA Amendments Act sought to expand the meaning of a disability and make the law more inclusive of those who may not have qualified previously as having a disability. As a result of this legislation, a school district must consider the effect of the disability without the use of “mitigating measures” such as medication (i.e. worst case scenario). As a result of this change, those with a medically related health condition have the right to a legally binding agreement with the school by having a 504 plan which should include an IHP for specific nursing care procedures.

Emergency Plan:

Just as an IHP can be added to, or part of a 504 Plan, so too can (and should) an Emergency Care Plan (ECP) be part of the equation. An ECP is a document that requires a plan for the worst-case scenario - the emergency situations - that none of us like to think about, but that we always have in the back of our minds as parents of children with special health needs. Unlike the IHP which should be written in “nursing language,” the ECP should be clear and succinct so it is can be read quickly and easily in an emergency situation. It should be in layman’s terms, because it is not just for the school nurse but for any school staff member to implement in an emergency. It can even be formatted like a basic chart:

An IHP and an ECP are both plans that strictly address the medical issues that may arise for the student during the school day. Unlike an IEP or 504 Plan, the IHP and ECP do not have a standardized format. The format may vary by school district or even by school. While considered standard, good nursing practice, you may not find that this type of plan is the “norm” in your school district or with your school nurse. In such a case, it is important for parents to advocate for this plan for your child at school. Your healthcare provider can assist you by acting as a liaison with the school or district nurse or by giving you a plan that you can discuss with the district’s health team. In the end, an ECP is the bare minimum of what should be in place for any child with special health needs. An IHP is a more lengthy and detailed plan that focuses on nursing procedures for your child and can incorporate an ECP. However, if you feel that your child requires more accommodations (and a legally binding agreement) that a nursing care plan does not offer, then you should seek out an evaluation for services provided under a 504 Plan.

The Basics of a Well-Developed IHP:

• should always include up to date emergency contact information

• should include a review date

• should always be signed by a parent/guardian and school nurse

• should include six components

  1. Assessment: gives the background information on the student/health issue

  2. Nursing Diagnosis: the school nurse’s analysis/summary of the health issue and how the nurse can contribute for care of the student

  3. Goals/Outcomes: clear, concise outcomes for student healthcare plan

  4. Nursing Interventions: details how the nurse will meet the goals

  5. Implementation: put the plan in practice

  6. Evaluation: reviewing student goals and whether they have been attained; review interventions and adjust as needed based on status of the student’s health

• The Emergency Care Plan should be part of the IHP - it must include what emergent scenarios could arise as a result of the student’s health condition and what actions must be taken in response - for any member of the school staff (not just the school nurse)

  Remember, there is no standardized format for the IHP. You may see many different versions of this type of document. Your medical provider must submit written orders for any medical treatment that may be performed at school.

References and additional resources:

http://oley.org/?page=IHP_IEP_Difference

https://www2.ed.gov/about/offices/list/ocr/504faq.html

A Review of Inhibitor Family Camp at Victory Junction

Making Life-Long-Distance Friendships

By: Ashley Davis

Published: Lifelines for Health Spring 2017

This was our first year attending Inhibitor Family Camp at Victory Junction. We were beyond excited.

We really didn’t know what to expect. We knew there would be rap sessions and fun activities, but we had no idea the dynamic relationships we would create.

Upon arriving, we met our awesome crew chief who stayed with us all weekend and helped us out with anything we needed. Our six year old son warmed up to her pretty quickly and was never torn about having to leave us to go hang out with her and the other children. He normally does not want to leave our side if he’s around people he doesn’t know well, so we weren’t sure how he was going to feel when we headed off to rap sessions and he had to go a separate way with the kids. However, our crew chief was so good with him, and he had no hesitation about going off to have some fun without mom and dad! This was great for us, since we hardly ever leave his side at home. We needed this time to talk and empathize with other parents dealing with the same things.

The emotional and physical aspect of being a caregiver is often very overwhelming. Understandably, nobody back home – even those closest to us – really knows what it feels like on a daily basis. Being able to meet other parents, and for me other mothers, was extremely humbling. For once, we were able to be around people who really, truly get it. This was the biggest takeaway for me, personally. I needed desperately to find that connection. I was able to make lifelong friendships and find people to connect with, even if hundreds of miles away.

In the same way, our son was able to finally meet other boys going through the same things he does each day. He was able to do fun activities and participate in things we wouldn’t normally do back home. All of the boys were so welcoming and made him feel like they had always been friends.

During infusion time, our son was able to build up enough courage to stick his dad for the first time. Some
of the older boys rallied around him and gave him their support, which was so cool to watch. These kids shouldn’t have to worry about things like this, but they embrace it so well and run with it, and then encourage the others to run with it as well. Amazing.

We are so grateful for the opportunity that CHES provided us to attend Inhibitor Family Camp. Without their generosity and willingness to host, we would not be able to do something like this. They made it such a special weekend for us. We each had our own personal takeaways, as well as family experiences to take home. Thank you CHES!

The Timeline of Education: A New and Uncertain Turn?

by Lisa Cosseboom, M.Ed. & C.A.G.S School Psychologist & Special Education Evaluation Team Chairperson

Published: Lifelines for Health Spring 2017

Important Events in Public Education:

April 11, 1965: President Lyndon B. Johnson signs the Elementary and Secondary Education Act (ESEA) which expanded the federal role in k-12 education. Title 1 was implemented that enabled the Federal Government to assist with providing funding to school districts to help disadvantaged students.

1968: Congress expands on ESEA to provide programs for immigrant children, neglected children and passed the Bilingual act.

1973: The Rehabilitation Act becomes law in which the Section 504 ensures civil rights for people with disabilities requiring school districts to accommodate for students with disabilities to access buildings, programs and activities.

1974: Equal Educational Opportunities Act passes. This Act requires school districts to take action and overcome barriers which would provide equal protection for students.

1975: The Education of All Handicapped Children Act (PL-94-142) becomes Federal Law. This law provided that handicapped children and adults ages 3-21 be educated in the “least restrictive environment” to the maximum extent appropriate, meaning that they are educated with children who are not handicapped and that special classes, separate schools or other removal of children from their regular educational environment, occurs only when the severity of the handicap is such that education in regular classes cannot be achieved.

1978: President Jimmy Carter reauthorized ESEA and changed Title 1 rules allowing school- wide Title 1 programs when 75% or more of the students are low-income.

1979: President Ronald Regan reauthorized ESEA and changed funding into one block grant and reduced regulatory requirements by states.

1988: Student testing and accountability takes hold and regulations require districts to test annually and to create improvement plans.

Recent Education Bills Passed:

House Joint Resolution 58: Passed House on 2/7/17, Senate on 3/8/2017: Rejected a Department of Education regulation that imposed a new federal standard for the education and preparation of teachers which linked teacher preparation to eligibility for federal grants.

House Joint Resolution 57: Passed House on 2/7/2017, Senate on 3/9/2017: This resolution overturns a regulation by the Department of Education that placed federal restrictions on systems developed by states to hold schools accountable to parents and taxpayers for their performance.

New Bills introduced:

What is H.R. 610?

Termed “Choices in Education Act of 2017” according to Congress.gov, this Act “Repeals the Elementary and Secondary Education Act of 1965 (ESEA) and limits the authority of the Department of Education” to nothing but the power to award block grants to qualified states.

The repeal of ESEA would essentially eliminate every education act noted in the timeline...

including Every Student Succeeds Act (ESSA) under Obama and No Child Left Behind under Bush. The more recent ESSA promotes equality in education and provides federal protections for disadvantaged and disabled students. The block grants would distribute federal funding to eligible states to award in the form of vouchers for eligible students to use in school choice. Additionally, this Act repeals

the rules surrounding established nutrition standards (availability of fruits, vegetables, reduction of sodium etc.) for the national school lunch and breakfast programs. This Act was introduced to the House Committee on Education and the Workforce on 1/23/2017.

Thinking behind H.R. 610:

Proponents behind the Choices in Education Act believe that this Act would provide better competition between public and private schools and therefore increase the quality of public education through the spirit of competition. They feel that disadvantaged students would have access to private or religious schools through the voucher system where they would not have had access freely because of their poverty levels. Families who tend to be financially well-off, tend to live in identified communities that are known for quality education, or can afford to send their children to private schools.

Nuts & Bolts of Choices in Education Act:

Title 1 is federal funding provided to public school systems based on the number of disadvantaged students that are enrolled in their districts. The funds are distributed in public schools to assist in educating the disadvantaged. H.R. 610 would remove Title 1 funds to public schools and transform this funding into the voucher system that would follow an eligible child to whatever school they are attending (including religious, private, charter schools or students being home-schooled). Many states have laws in place separating church from state and the voucher system could allow for public funding to be funneled to religious organizations.

The proposal is assuming that states would contribute approximately 110 billion dollars into the voucher system which would ultimately provide approximately $12,000 per year to each student who qualifies for the voucher system. Additionally, the vouchers are geared towards students whose family fall in the poverty level and may not cover the complete cost of private schools and would require the families to pay the difference. The very families that have met poverty levels. There are a lot of proposals on how this voucher system would work, but none seem very clear. States hold local control over education policy and regulation, and would need to “buy-in”, literally and figuratively, with the federal proposal. The Act appears to be the beginning of an attempt to privatize education and dismantle public education. Essentially, it reallocates federal funding leaving public school districts scrambling to make cuts and find funding.

Voucher programs have existed on a smaller scale in this country for a long time. Some of the research points to inconclusive or contradictory results. Some states have reported better success of student on vouchers in testing and graduation and some have noted no increase in testing scores or graduation.

The second component of H.R. 610 introduces the “No Hungry Kids Act.” While on the surface this seems like a positive Act, it is actually removing the previous “Healthy, Hunger-Free Kids Act which purpose was to improve child nutrition through the school lunch and breakfast programs. The Healthy Act was requiring the schools to increase availability of fruits, vegetables, whole-grains, low-fat milk and reduce levels of sodium, trans-fats and saturated fats in school breakfast/lunch The prosed act would prohibit the USDA from rationing calories to children and remove the previous Healthy act requirements.

Effect of H.R. 610 Special Needs Students:

The Elementary and Secondary Education Act of 1965 has been reauthorized every 5 years since its inception and has changed names several times. Whatever name it was at a given time did not matter to special education students, as it always provided protection. The ESEA provided that schools who receive federal funding must provide support for students with disabilities. Under the new proposal, federal funding in the form of grants, would not require the schools receiving the vouchered students to provide services. Private and religious schools are not required to provide special education services. Removing funding from public schools that offer a spectrum of services and place it in the hands of private or religious schools further impacts public education and decreases funding for special education services.

Summary:

If H.R. 610 passes, essentially it removes federal funding to public education and repeals the ESEA which protects programs for special education students, students in poverty, gifted students, ESL programs, rural education and school safety. Providing a free and appropriate education to all and ensuring special education students access to the curriculum and accommodations will cease to exist. The goal of H.R. 610 is to privatize education and defund public education. To dismantle fifty years of progress in education and rely on inconsistent research of school vouchers is a dangerous path and may leave many disabled, under-privileged and middle-class students abandoned.

Clinical Trials: Optimistic Caution

Published: Lifelines for Health Spring 2017

It is an incredibly hopeful time in the bleeding disorders community! Longer acting products, gene therapy, and subcutaneous injections all seem to be within our reach. The plethora of new treatments truly boggle the mind. Keeping up with the changes of names for manufactures alone is bewildering! Baxter became Baxalta, which then became Shire. Biogen became Biaverativ, Emergent became Aptevo. While multiple new companies are offering emerging therapies in our community such as Spark, Dimension and uniQure. Deep hope has been ignited again for the first time since recombinant product became available. Treatment promises that may yield fewer infusions per month, subcutaneous injections could make self-infusion and ports a thing of the past. Gene therapy has significantly prolonged FIX activity levels, which substantially increases the length of time one may be infusion-free. Each advancement comes with the opportunity to participate in a clinical trial. This decision however, comes with excitement, responsibility, uncertainty and at times - false hope.

Hemophilia has been in my family for over 7 decades and I have heard “a cure in our lifetime” since 1970. I have personally seen the excitement and uncertainty as we seek new treatment that will make our lives more predictable, better, maybe even.... normal? When carrying my first son in 1988, knowing that I was a carrier, all
I knew was that I was carrying a son and he had a 50/50 chance of having hemophilia. Like every newborn, he was miraculous! A preciously beautiful son who looked normal in every way. I dared to hope that he didn’t have hemophilia. Three days postpartum, cord blood results indicated that he did in fact have severe FVIII deficiency. As a family member of one affected by hemophilia, at least I had some knowledge of what life with a chronic condition might look like. Nothing prepared me for being the mom of a child with a bleeding disorder.

In 1992, I was anticipating the arrival of another beautiful child who would be blessed with hemophilia. Prenatal testing had evolved by that point, that via amniocentesis, we were prepared. Once I learned that he was a boy, I knew in my heart he had it. In truth, I was grateful to have two (2) sons that shared the same disorder. Boundaries and rules would be the same for each. There wouldn’t be one who could pummel the other, while the other one couldn’t pummel them back. My salvation was that I was the one who would infuse them! “Choices have consequences” was our family motto.

In 1991, while pregnant with my second son, I was approached about participating in a clinical trial for recombinant factor. He was the last previously untreated patient (PUP) in the world to be enrolled. Looking back at that time, I realized I knew very little about what adverse events could be anticipated. As a community, we were most worried about HIV and Hepatitis. The scramble to be sure that factor products held no human element of plasma was the goal. My first son was tested for HIV every year until his 6th birthday. The anxiety waiting for those results would reach a crescendo.

With a family member impacted by HIV/Hep C, my first concern was that my sons would also be affected. Signing up for a clinical trial seemed like the best possible choice I could make. Decreased risk of HIV/Hep C was paramount. The trial lasted for five years. It required multiple blood tests at a HTC nearly 2 hours from our home. I look back on that time making those long trips, multiple pokes, the fabulous nurse who bribed him with trucks-one for each hand with a sense of pride that we were doing our part to contribute to science and ground breaking research that would benefit others.

Fast forward to 2002, my second son has developed an inhibitor at the age of 10. THIS was a totally different diagnosis than “plain old hemophilia”. An inhibitor was life changing for our family. Our family was now coping with the challenges of an adverse event. In under 50 years we have seen the development of factor in 1970, (a huge improvement over fresh frozen plasma or cryoprecipitate). Factor then evolved from being plasma derived to recombinant, and then multi-generational factors that eliminated any element of human blood. In that time, our community lost thousands of lives to AIDS and Hepatitis C. Those who survived, now manage as many as three chronic illnesses-hemophilia, HIV and Hep C. Recombinant factor saved my sons from HIV/Hep C, which I will be forever grateful for. Yet, in the last 25+ years, we have seen a 25% or higher rise in inhibitors affecting those with hemophilia A and B from mild to severe. Since 2012, inhibitors are the biggest threat to our community today; finally, surpassing HIV/Hep C.

Now in 2017, there are multiple new treatments in clinical trials that heightens our feelings of hope that finally, THIS might be the one to change our lives. It is both an exciting and perplexing time in the bleeding disorders community. On average it takes 14 years and billions of dollars for a new product to be developed. Choosing to participate in a clinical trial can be a challenging decision. At the 2016 Inhibitor Summits, Dr. Tarantino’s and Dr. Kruse- Jaress’ presented on the “Knowns and Unknowns” of current therapies in clinical trials. There is still so much we do not know when it comes to choosing to participate in a clinical trial. Choices have consequences, with positive and negative effects. If you read the adverse effects of aspirin or acetaminophen, the list is lengthy. When participating in a clinical trial,every symptom is reported. On a trial and have a headache? It will be reported. The headache may have nothing to do with being on a trial, but it could be, so it must be documented. It is probably fair to say that every medication has side effects. This is a big decision, one that may not affect just you. As a parent, you are deciding for your child something that will affect them for the rest of their lives. As an individual, you may have loved ones who could be affected by your decision. Only you can decide what is best for yourself or your family. It is all about making the most informed choice you can possibly make, and your ability to trust in it. So, what can you do as an informed potential participant?

Answer: Arm yourself with as much knowledge as you possibly can.

Clinical trial thoughts to examine:

1. What phase trial am I participating in?

2. What is the inclusive/exclusion criteria?

3. By participating in this trial, does it preclude me from ever participating in another?

4. Carefully read the Informed Consent Form that contains:

   1. Purpose

   2. Details

   3. Duration

   4. Required procedures such as lab work and how often needed

   5. Key contacts

5. Is there a patient advisory committee included with this trial and may I participate?

6. What are the risk/benefits?

7. Is my current HTC an Investigational Site? If not, is there still a way I could enroll?

8. Will I be reimbursed for travel if participating in an Investigational Site far from my home?

9. Will study drugs be included free of charge? For how long? Will my insurance company pick up remaining costs of lab draws, office visits, etc.?

10. What is the purpose behind wanting to participate in this trial?

    1. Quality of Life?

    2. A feeling of duty/determination to further research for others?

11. What are your feelings on taking risks?

12. Avail yourself of resources such as clinicaltrials.gov, PubMed https://www.ncbi.nlm.nih.gov/pubmed/, or Wiley Online Library wiley.com. Trial abstracts are available on those sites and if you would like to read the entire journal articles, ask your HTC provider for them or purchase if available.

    13. Read.

    14. Talk to friends, family, clergy.

    15. Write down your questions before visiting your health care professional. They want you to make a well- informed decision as well.

    16. Take your time. There are plenty of opportunities in the pipeline.

    17. Trust your gut.

    18. Read some more.

    19. Be prepared that this may or may not produce the desired affects you were anticipating.

Engaging Uncertainty

Published: Lifelines for Health Spring 2017

by Krystyn Strother

“The quest for certainty blocks the search for meaning. Uncertainty is the very condition to impel man to unfold his powers.” - Erich Fromm

As uncomfortable as it may feel, uncertainty is at the foundation of our lives. We want to know, always, especially when it comes to our loved ones and our future. These unknown prospects can leave us feeling unsettled. Yet, the reality that every living being on this earth faces is one of uncertainty. Our jobs are not guaranteed, our cars will inevitably need fixing, and the sun shines even when rain is in the forecast. When we cling to an expectation of a certain outcome, we set ourselves up to suffer even more if it doesn’t happen.

Uncertainty is so upsetting, causing stress and anxiety, that many of us try to avoid or control it altogether. Often, people will say that we need to cope with these feelings, but what does that really mean? How do we cope? Certainly, not by putting your head down and hoping for something different. Moving through moments of uncertainty requires engaging with it and this is where a mindfulness practice can be helpful, if not essential.

Mindfulness teaches us to disassemble reactionary thoughts into manageable parts and pieces. It gives us a buffer between event and reaction so that we can form a productive response. Rather than focusing on the negativity, we learn to pay attention to our experience with curiosity and without judgement. This is engagement. A sitting back in your seat when those uncomfortable feelings come up and extending a chair and invitation for them to sit down right across from you.

Mindfulness

The subtle art of positive, internal manipulation. There are things that you can and cannot control. For example, you cannot know what the outcome will be regarding our nation’s current healthcare debate. You can, however, take note of what you are able to control when you experience any negative or stressful thinking around this topic. You can, for the most part, control your environment. Are there ways, in these moments of stress and anxiety, that you can alter your environment to find a sense of grounding? Open a window, take a deep breath, turn on a light, or adjust your posture. When you’re thinking about a moment, you can control your response, which influences everyone around you. The problem with dwelling upon a moment that is out of your control is that you are too overwhelmed by your expectations or fears of the future that you lose sight of what is taking place in the present.

Be Confident

Engaging with your uncertainty will only give you more insight and a better understanding of what you are really feeling. A better understanding of what we are feeling and why, gives us more access to our experience, and while we can’t control everything, we can learn to ground ourselves in the moment. This helps us to feel better prepared to tackle whatever comes our way. The only constant in life is that it will involve change, and try as we may to control the future, sometimes all we can do is trust that whatever happens, we can adapt and make the best of it.

Uncertainty is inevitable. And no matter how hard we try, controlling it simply doesn’t work. Instead, practice acceptance, control what you can and relinquish the rest. Mindfulness, at its best, teaches us how to be open to both. Practicing mindfulness cultivates comfort with discomfort.

Krystyn Strother is the former program director at HUSH Meditation, strategic designer/author of the HUSH meditation curriculum, is a certifiedmeditation instructor, co-founder of NOMAD, “Adventures in Wellness”, and yoga instructor.

Krystyn’s yoga classes range from Vinyasa to Yin. In addition to her regularly scheduled classes, Krystyn guest teaches at several yoga teacher training programs throughout the country, speaks at conferences on mindfulness and stress reduction practices, teaches specialized workshops, facilitates yoga + adventure retreats, and conducts continuing education classes for currently registered RYTs.

Krystyn holds a certificate of completion in the Yoga of Awareness For Chronic Pain, an evidence- based program sponsored by the Department of Anesthesiology at OHSU.

Read more about Krystyn at krystynstrother.com

"HOME" Coming

by: Crystal James

Published: Lifelines for Health Fall 2018

Inhibitor Family Camp to my family and I means love and acceptance. We live in a world that’s harsh and sometimes rather distant to the reality of what hemophilia is. Camp is a place where we as parents can watch our children be carefree and the children themselves can build relationships amongst their peers and build confidence within themselves.

Families are able to see within what can be missing as a whole when we step out of our normal environments. We get a chance to escape the everyday routine of life and let the enjoyment of being a structured family unit flourish as one.

I feel as though IFC gives my boys a sense of self confidence. They get a chance to meet peers
older than them such as camp assistants and counselors that also have the same disorder that they battle every day. Meeting these older peers with the same challenges, gives them a sense
of self-fulfillment. It provides the boys’ positive reinforcement that growing up with hemophilia is okay and they can do anything in this world that they want.

I also think Inhibitor Family Camp is a place where you can feel relieved within the hemophilia community. All of us as families were brought to this amazing place for the same reason. As crazy as life can be dealing with the horrors of this disorder every day, day in and day out, camp seems to be a place where these horrors don’t exist. All of the attending families are comfortable with one another. These children from all different walks of life join together as one and we become one big happy family for a whole entire weekend.

Camp is also a place where we learn how to deal with the mental aspects of living with a child that has hemophilia with an inhibitor. I find it amazing that we get the chance to challenge our intellect and obtain knowledge on how to deal with the harsh reality of what hemophilia can do to your family members and the siblings living with it as well. We need that aspect of teaching for us as parents because it can be tough having to deal with this disorder and having other children.

For me, altogether Inhibitor Family Camp means - we grow substantially as a family. We gain confidence as well as independence. We gain memories and friends for a lifetime. We look forward to the year passing so that we can make these memories grow with many more. If I truly had to sum up camp to one word, I would say camp is like home because (in my eyes) home is where the heart is, and Inhibitor Family Camp is full of just that. Love for a lifetime. I will always love for my family to be a part of this experience. Thank you.

Leverage 2018 Walking the Wire

by Gabriel Anspaugh

Published: Lifelines for Health Fall 2018

I was diagnosed with inhibitors when I was 15 years old. Up until that point I lived my life pretty normally. Yes, I had hemophilia, but I wasn’t going to let it get in the way of how I lived my life. However, the first little bit of time with inhibitors was quite tough on my mental state. What could I do, and what couldn’t I do? And could I even get back to where I was? As time went on, I again discovered what I could do and what would be more difficult for me.

Back in February, I was on a slackline at a rock-climbing gym. (I know, a hemophiliac with an inhibitor at a rock-climbing gym - sounds like the beginning of a bad joke, right?) Moving on, a slackline is essentially a tightrope that has a lot of slack, so it’s more wobbly. I was determined to prove that I could do this. I ended up rolling my ankle pretty severely. This injury put me in the hospital for a week and in a wheelchair for many more. As I was going through this, it was pretty hard for me to look at the positive side of this situation since there really wasn’t one when losing the ability to walk.

However, there was an upside to this accident. Eight months later, this life lesson came full circle when I attended an educational, adventure, camp program for individuals with inhibitors called Leverage in Oregon. As I was at Leverage, one activity we did was a high ropes course. The amazing instructors had suggested to us that we each find a specific goal to accomplish. I wanted to complete every element of that course.

My first instinct was to go straight for the most difficult ones to complete. In my nature of being a very physically, active person, these tasks were fairly easy for me. But I knew that the last element was going to be a challenge. It was a slackline with ropes hanging

at separate points to hold onto. My experience with a slackline from less than a year prior was the most difficult for me to accomplish. It’s funny how such an easy obstacle to complete was the most difficult for me to overcome. That horrible fall, the time spent in the hospital, and that debilitating wheelchair were all running through my mind throughout the entire experience. I watched all of my blood brothers (who I had grown to know and love) go through this obstacle with

no problem and just keep going. However, when I stepped out and was about a quarter of the way through this element, I actually had to stop, take a knee, breath, and wrap my mind around everything. I knew I couldn’t fall because I had a safety line. And I knew that the likelihood of injury was low. But why was this so hard for me? Once I caught my breath enough to keep going, I just had to keep my eyes on the trees ahead and try not to focus on the memories of what was the worst injury of my life. Once I got to the next platform, I had to fight off tears as I realized that I just conquered something that had crippled me for a better part of the year.

So, what was one of the biggest things that I took away from Leverage 2018? ...the memory of conquering something that was once so disabling to me.

FEATURE: Understanding Complex PTSD Trauma

Published: Lifelines for Health Fall 2018

By: Charlie P. Gilbert II, LCSW

“The” Inhibitor Traumas

Do you remember yours? That first time that it really hit you that your child had a life-threatening inhibitor. Maybe it was when the Doctor or Nurse told you in the HTC clinic that the “normal” treatments to stop the bleeding weren’t working and that it was clear that your child’s body was rejecting the known factor solutions. Or it could have been when you nervously waited in the chaotic Emergency Room with dozens of other parents while a Doctor somewhere behind a curtain in another room tried to figure out what to do with your child who couldn’t stop bleeding, swelling and crying. And then there was the time that you with others had to hold down your child, kicking and screaming, to infuse or draw labs. The abject powerlessness of a parent to protect or save their child is painful in itself. You probably recall the de-personalization that you felt when it seemed as though you were outside

for that unsuspecting moment when they trigger the anger, panic, powerlessness, fear or hurt that comes from your exposures to the earlier threat to your child’s existence and your impotent role as a parent. It might be on another visit to the hospital years later when someone minimizes the effects of a bleed. Or it could be when the school assumed that your child couldn’t participate in the class musical or other event.

The Brain’s Response

So what will it take to recognize the lasting effect these events have had on your mind? We know that the most traumatic events and stressors lead to a cascade of neuroendocrine responses in the body. These changes alert and prepare us to take on the stresses and challenges we’re facing. But, if constantly repeated, they also lead to permanent changes in our brain structure in which we develop high arousal levels leading to anxiety, fear, hyper- vigilance, avoidance, and compulsive, intrusive thoughts and memories. Some clinicians will find these symptoms giving rise to a diagnosis of depression or PTSD. In Depression they observe a loss of hope and the inability to find enjoyment. Acute Inhibitor care at a very young age can also result in traumatic exposures which isolate family members, change their roles and may produce an anxious attachment style in the child, leading to future difficulty forming interpersonal relationships.

In the case of PTSD, clinicians look for a traumatic event. Here however, we find in inhibitor care and treatment a series of small and repetitive traumas that over time coalesce into an ongoing chronic stressor leading to physical, developmental and interpersonal negative outcomes. These include avoidance of medical care and treatment or withdrawal or hypersensitivity to other threats in the environment.

We know, from the research of the ACEs (Adverse Childhood Experiences) events in children’s lives, that abuse, neglect and other severe trauma, experienced or witnessed predicts very negative outcomes later in adult life. In this study of the early lives of more than 17,000 adults, the CDC and Kaiser Permanente Health Appraisal Clinic found that ten forms of childhood trauma were associated with negative psychological and physical outcomes. Unfortunately, the forms of trauma most studied failed to capture the inevitable and repeated traumatization of children and their families by medical and surgical procedures. In response to this oversight, professional bodies have begun to look at new terminology to describe this problem. Complex trauma and Complex PTSD are two of the newer terms created to recognize the various symptoms of this developmental trauma.

The changes that occur in the brain following these occurrences become permanent and represent considerable risk to health and life for parents and children going forward. Parenting also affects children’s’ brain development in lasting ways. As children grow with the ever-present threat of severe physical outcomes of a momentary lapse in judgement, they take their cues from the coping practices of their parents. In effect, they surrender their own coping choices to the dominant style of their parents’ management of the stressors that develop out of the child’s inhibitor.

This is also quite evident in our other children, the siblings of the affected child, when they witness the overpowering acuity of inhibitor related activity. Priorities shift, plans are given up, goals are set aside, events cancelled, hours are spent in clinical offices or emergency rooms. Needed attention shifts from the unaffected siblings to your child with the inhibitor. The brothers and sisters get to see their significant adults at their most stressed and find in that experience meaning about their own role in the family. At the same time the older and younger sibs are required to take on adult responsibilities and more mature coping responses. Their childhood can fade away into a “parentified” identity with accompanying expectations from
other family members. The siblings help with the caregiving, sacrifice their own events and happiness, and can become surrogate adults in the home. Of course, it’s quite possible that they will reject the role entirely and become the focus of resistance to the family process. Their acting out can send a powerful message about their complaints. The stressors experienced by siblings, while lacking a diagnostic terminology, lead to many of the same symptoms and developmental impediments found in PTSD. More than a few siblings of children with inhibitors have shared that in crisis they become “invisible.” Some even hide there.

Overcoming the Trauma

So what, if anything can we do about this? How can we help our traumatized children, siblings, partners, and ourselves to avoid the negative outcomes of the repeated traumatizing exposures? There are certainly a great many approaches to reduce, mitigate and overcome the symptoms and risks of the trauma. In the summer issue of Lifelines for Health Dr. Gary McClain told us about the effects of repeated traumatic exposures on the individual with an inhibitor and on his caregivers as well. He encouraged us to improve our coping, manage our anxiety, remain calm and get support.

I’d like to add several important ideas that could help us to address the symptoms and improve outcomes for our affected children, siblings, partners and for ourselves. The following approaches are designed to be useful for anyone experiencing the effects of repeated and chronic traumatic exposures and needing to overcome adversity. Some of these you may find are helpful while others don’t make sense to you at this time. Some of you may have tried and found them not helpful. Others you might see as difficult or moving you out of your normal responses. I would ask you not to discard them as they may be more effective at another time or with another member of the family. Here are “Charlie’s Challenges.”

First let’s remember the lesson of why we have families. A family is the laboratory for the biopsychosocial development of its members. Each person in our family is on a trajectory of development towards happiness, satisfaction, cognitive/ intellectual development, interpersonal effectiveness and meaning in life. We can get there and help our family members to get there by understanding and supporting each other. There are some rules for accomplishing this. One is that we need to understand the meaning of events, emotions, and behavior not just in ourselves and family members but in others around us. This requires us to set aside our emotions for a moment and consider the significance of what is happening, what we and our family members are feeling. The poet, Maya Angelou told us that people don’t really remember what we said or did but “...how we made them feel.” If we lose our “cool,” we become reactive and not reflective. Aside from saying things that serve to alienate others, we set a problematic example for our children and impede their acceptance and appropriate response to the stressors.

Our children are smart and they often know exactly how we’re feeling even if we didn’t verbalize it directly. But they more frequently act out their feelings rather than articulate them. We must help them to find the words and sometimes we give them the words by recognizing and validating their feelings. They often need help to really see and understand what is going on around them, especially in threatening environments such as schools, emergency rooms and clinics. To understand their risk, they will take their cue from their parents and other adults behavior. If they’ve got an angry, upset or fearful parent they will adapt quickly to fight, flight or freeze. Their arousal level will peak with uncertainty about what to do next. In that setting they may mistake or over exaggerate the threat that exists and they won’t be able to see any positive benefits of treatment and compliance. They won’t recognize or adopt the process of reflection and choosing the best approach. So, we must protect them from our negative emotions and model the self-regulation that will instill confidence and serve them well in this and future crises.

But reflection serves us as well. An important aspect of coping with stress is recognizing and accepting events and their meaning to us. As we learn to identify our triggers we can adopt an improved “reflective functioning,” that simultaneously engages our emotional and cognitive brain structures. Rather than respond immediately to each insult and stressor that appears in our environment we need to step back and ask ourselves, “What is this?” and “Do I own this?” This will help us to modify our perception of the problem and reduce its power. If we can adopt this approach we can avoid the outlay of emotional energy on the past and move on to a more productive approach of understanding and coping with the stressors. These changes can re-train the brain to be less responsive to triggers and reduce our need to be overly vigilant.

Some parents have pursued other behavioral and cognitive approaches that have proven helpful in defusing their emotional outbursts. Quite a few people have discovered that they need a “time out” in response to re-experiencing the thoughts and emotions from the original trauma. They are inclined to focus on a breathing exercise to clear their mind and control their physiological response. Others may go directly to meditation and find that doing so routinely, before entering a stressful venue, helps to reduce their anxiety.

The old cliché of “living in the moment” rather than the “painful past” gives us a chance to think clearly and positively. Sometimes we’ll find that we “own” only a part of the stressor and that a painful part belongs to some other person or is part of a different problem. Yes, we certainly learn from the past, but if our
negative emotions focus only on the past, they cloud our vision of what will make a significant change in coping with the issue. At other times we may find that we need to take responsibility for our actions
and emotions and this requires making amends to our loved ones. The act of offering apology and asking for forgiveness can lift a burden from ourselves and from our partners and children.

Moving towards a more effective outcome, we know that decision- making is improved by positive thinking. Problem solving is enhanced and positive affect reduces psychological distress and alters neuroendocrine, inflammatory, and cardiovascular activity. It leads to experiencing less pain and improved social relations. Other research points to positive affect reducing the likelihood of adolescent substance abuse and risky behaviors. Helping our loved ones to find the positive in a difficult situation can make a big difference in their feeling of security and hope for a better future.

Another critical aspect of ownership is that as parents, we can’t take ownership of health problems and behavior from our children.

It can be awfully hard for parents to not take complete responsibility for our children’s health and treatment issues but doing so takes away the motivation to be effective managers of their own lives. It deprives them of the feelings of competence and confidence in directing their own position in the family and in the world in general. Yes, we love and want to spare them all the mistakes in judgement, but they find out who they are by participating in the process. As Billy Joel reminds us about mistakes, “...they’re the only things that you can truly call your own.” One of the hardest parts of parenting is supporting our children even when they chose poorly and against our best advice.

Helping our children overcome the trauma experience is often foremost on parents’ minds. One mindful approach for parents to adopt that has proven effective is PACE. Developed most recently by Dan Hughes and Jonathan Baylin, it incorporates Playfulness, Acceptance, Curiosity, and Empathy. When discussion of the event or stressor is experienced by children in a less- serious or lighthearted manner they feel less threatened and the de- escalation allows for more objectivity. Acceptance is a means by which judgement is set aside and parent and child can work together to understand the problem and adapt a response.

Curiosity serves to facilitate a pattern of discovery and understanding of the stressor and encourages the child to explore and make meaning of their own inner lives. Finally, a consistent empathic approach to our stressed-out children helps to reduce defensive posturing and enables children to feel visible and valued.

Another benefit of letting go of the painful past is that we can avoid re-enactment of the trauma or re-traumatizing members of the family. If we visit that crisis every time we or they are triggered by some offense or challenge, the original insult is re-enforced and brought to foreground of our emotional coping responses. This tears away at our resilience and blocks our adaptation to effective management of the issue. The same is true for our children, as they relive all the emotional baggage and hurt that originally caused them pain. Rather, we try to avoid the baggage of over analyzing, blaming, and fixing that we are inclined to do to protect our loved ones from their pain and their development of an emotional boundary against it. As we and our children battle with the challenges of life we develop a pattern of coping with stressors that leads to a mature repertoire of adaptive mechanisms that will carry us towards that success that we want and need. If we can remove that baggage from our relationships, we will be much more likely to develop the “challenge orientation” and independence that equips us with strength, determination and motivation to be successful.

One last look at our response to the trauma of the inhibitor experience, involves training ourselves to listen more carefully with empathy even when we are hearing painful and negative comments. We can easily get
caught up in the rolling catastrophe of an ER visit when a physician, nurse, or child welfare advocate want to second guess causation or a remedy for a problem we’ve been coping with on a daily basis. If we hear this often enough we respond emotionally and tend to stop listening and block the sometimes unspoken meaning of it. It might be coming from a sibling of our affected children in the form of a complaint about fairness or it could be a silent acceptance of their secondary status in the family. We have to remember that all feelings are real and listening for the meaning of comments rather than just the exact details of the spoken words will help us to keep on the path of open communication. When we develop a curiosity for the meaning behind other’s statements and behavior we can begin to truly understand their motivation and needs. We are in effect “PACEing” ourselves.

Our brain’s ability to adapt and develop new connections is referred to as plasticity and it continues throughout our lives. Those triggers and traumatic stress symptoms which have been created in our minds can be altered. If we take on the challenges to develop different responses to the stressors of having an inhibitor in the family, we can reduce the power of the symptoms and retrain our minds to overcome any adversity. Change is coming and if we get behind it in a positive way we’ll have more positive and resilient outcomes.