Overview of Mindfulness and Research - Based Physiological Benefits

By: Krystyn Strother

Published: Lifelines for Health Fall 2018

In past issues we’ve talked about the general benefits mindfulness can have on our general well-being, reducing stress, coping with chronic pain, but what about the physiological effects of mindfulness?

Let’s define mindfulness as a moment-to-moment awareness of your environment and experience without judgement. It is important to highlight that mindfulness, when we talk about it this way, is not a character of your personality, it is an active state that you engage in. In other words, being a “mindful” person is not the same as practicing mindfulness. For our intents and purposes, mindfulness is an intentional practice, be it 30 seconds or 30 minutes.

When our “thoughts and feelings are observed as temporary events in the mind, instead of a reflection of oneself or reality,” we are better, and more easily, able to access calmness, clarity, and concentration (Russell, et al., 2018).

The majority of research focuses on the effect mindful meditation has on the brain and body and explores the connection between the two. Below is a highlight of compelling research showing the physiological effects formal and informal mindfulness meditation practices have.

How Does Mindfulness Affect the Body?

In 2010, Hoffman et al. looked at 39 studies exploring the use of mindfulness- based stress reduction and mindfulness- based cognitive therapy and concluded that practicing mindfulness may be useful in changing physiological processes that are at the root of many clinical issues, stress. They saw a pattern that was consistent with other evidence that mindfulness meditation increased positive physiological experiences, decreased anxiety, and decreased negative physiological experiences.

Mindfulness has the ability to initiate a relaxation response in the body. When we experience a stressful situation, the brain begins a cascade of events that affect the body called the stress response. The brain, signaling the rest of the body through the autonomic nervous system, initiates a host of involuntary functions such as increased breathing and heartbeat, and elevated blood pressure. That signal from the brain triggers a release of hormones from the adrenals that intensify the physiological changes already taking place and affects the endocrine, sympathetic nervous and immune systems. Physically, the stress response shows up as increased breathing, heartbeat, and elevated blood pressure. Those hormones have other effects on the body potentially contributing to insomnia, digestive disorders, mental health issues, heart disease, fertility, and weight gain.

Think about this cascade being initiated every time you encounter something stressful. It is no wonder chronic stress has such a profound impact on our health and wellness! Mindfulness can give rise to the relaxation response by engaging the part of the nervous system responsible for getting the body back to its baseline levels after a stressful event. The heart and respiratory rates are lowered, blood pressure drops, and tension in the muscles is released. All of those stress hormones that were released-those are greatly reduced as well.

Research shows that mindfulness meditation not only promotes awareness and understanding of one’s own thought process but also has many broad-ranging benefits from weight loss, managing inflammation, and emotional wellbeing.

Manage Inflammation & Prevent Disease

Looking at recent research; a number of leading institutions are finding a link between the brain and our immune systems. Scientists at the Center for Healthy Minds University of Wisconsin-Madison have been studying the mind-body connection and the idea that the brain and the immune system work in conjunction with one another. Their theory is that the brain influences the body’s inflammatory responses, and our ability to train the brain to intervene on those responses might prevent some chronic disease.

Weight Loss & Disordered Eating

Many studies have shown that mindfulness meditation has a great effect on the area of the brain that regulates emotions and perspective, making it easier to let negative thoughts come and go without dwelling on them. Furthermore, there are a handful of studies pointing to mindfulness meditation as an “effective intervention for emotional eating.” A 2016 study conducted by UMass Medical School Center found that mindfulness meditation helped reduce emotional eating behavior.

Manage Inflammation & Prevent Disease

Looking at recent research; a number of leading institutions are finding a link between the brain and our immune systems. Scientists at the Center for Healthy Minds University of Wisconsin-Madison have been studying the mind-body connection and the idea that the brain and the immune system work in conjunction with one another. Their theory is that the brain influences the body’s inflammatory responses, and our ability to train the brain to intervene on those responses might prevent some chronic disease.

Cellular Changes

A study published in the journal Cancer by the Tom Baker Cancer Center in Alberta, Canada suggests that mindfulness meditation can influence, “key aspects of your biology.” In this particular study, researchers were looking at the effect of mindfulness on telomere length. Telomeres are protective lengths of protein at the ends of chromosomes and can be used to determine how quickly cells age. The subjects that participated in mindfulness training on a weekly basis over a three- month period showed preserved telomere length, while the subjects that did not learn or practice mindfulness had shorter telomeres. While more research is currently being done, the idea here is that folks in this study who engaged in mindfulness meditation may have more resilient cells.

Emotional Reactivity

Many of the physiological benefits of mindfulness meditation take place within the body in ways that aren’t immediately recognizable. A 2007 study reported a decrease in emotional reactivity among participants that had anywhere from one month to 29 years of mindfulness meditation practice. Mindfulness practices stimulate and increase the density of neurons in the area of the brain that regulates emotions and decreases neuron density in the region of the brain responsible for initiating the stress response. That means, the more we engage in mindfulness practices the greater our ability to regulate emotions without initiating the stress response.

Studies suggest that our minds and bodies can affect one another, and that the benefits of mindfulness meditation have profound physiological and psychological effects. More research is being done to better understand how these benefits are achieved in the body.

There are many paths to approach mindfulness and what works for you may not work for your neighbor, partner, or child. Yoga, Tai Chi, formal and informal mindful meditation are just a handful of ways to practice mindfulness. Consider mindfulness meditation as a preventative strategy to help manage how your brain responds to events taking place in the body.

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

1. Russell, L., Ugalde, A., Milne, D., Krishnasamy, M., O, E., Austin, D. W., .
   . . Livingston, P. M. (2018). Feasibility of an online mindfulness-based program for patients with melanoma: Study protocol for a randomised controlled trial. Trials, 19(1). doi:10.1186/s13063-018-2575-x

2. Hofmann, S. G., Sawyer, A. T., Witt, A. A., & Oh, D. (2010). The effect of mindfulness-based therapy on anxiety and depression: A meta-analytic review. Journal of Consulting and Clinical Psychology, 78(2), 169-183. http://dx.doi.org/10.1037/a0018555

3. https://centerhealthyminds.org/news/mind-over-matter-mapping- the-two-way-street-of-the-brain-and-immune-system?utm_ content=63584016&utm_medium=social&utm_source=twitter

4. https://www.ncbi.nlm.nih.gov/pubmed/27890474?utm_ content=63208938&utm_medium=social&utm_source=twitter

5. https://www.sciencedirect.com/science/article/pii/ S019566631630825X?via%3Dihub

6. https://www.sciencealert.com/world-first-evidence-suggests-that- meditation-alters-cancer-survivors-cells

LadyBugs at World Federation of Hemophilia in Glasgow, Scotland - May 20-24, 2018

by Janet Brewer, M.Ed

Published: Lifelines for Health Spring 2018

For the second time, CHES has presented a poster abstract at WFH on the LadyBugs Program. LadyBugs was founded in 1998 by Barb Forss, an amazing woman who received her diagnosis of severe FVII deficiency at the age of 47. She had been told her entire life that “only males have hemophilia”. Armed with her new knowledge, Barb became an outspoken advocate for women with all bleeding disorders. Although CHES has assisted Barb and LadyBugs for over a decade; we officially assumed it as a program in 2016. Similar to all of our programs, the LadyBugs Program satisfaction rating was 97% in 2017.

In 2016, CHES offered the LadyBugs program in three (3) regions within the United States, partnering with local chapters in Oregon, Texas and New York. These three programs were small, but successful, with an overwhelming request for MORE. The first and only National LadyBugs Women’s Summit became a reality on November 4-6, 2018 in Newport News,VA. Funding from Aptevo, Bioverativ, CSL Behring, Grifols, HEMABiologics, Kedrion, Novo Nordisk, Octapharma, Shire, and Soleo made this inaugural event possible. Separate tracks for vonWillebrands, FVIII/FIX and Rare Bleeding Disorders to include FV, FVII, GT, FXI and platelet storage pool were presented by Virginia Kraus, Dr. Joanna Davis (who presented Dr. Robert Sidonio’s slides), and Dr. Diane Nugent. Sessions on mindfulness, advocacy, self-infusion and self-care were offered. Saturday evening, we gathered for a paint night celebration.

Science is finally confirming what we as women have known for years, we bleed. The “family
business” of prolonged periods and anemia are NOT normal! On November 3, 2016, MASAC released a Recommendation Regarding Girls and Women with Inherited Bleeding Disorders #245 indicating that “Inherited bleeding disorders are under-recognized as causes of bleeding in girls and women. However, recent data have documented that 50% of girls and women who are carriers for hemophilia A or B have factor VIII or IX levels below 50%, putting them in the category of mild hemophilia and causing them to have an increased risk of bleeding, especially during menstruation and at the time of an accident, surgery, or delivery.” https://www.hemophilia.org/Researchers- Healthcare-Providers/Medical-and-Scientific-Advisory- Council-MASAC/MASAC-Recommendations/M

CHES is committed to increasing awareness, as well as advocating for more research and education for women with any bleeding disorder through the LadyBugs Program.

After the Shock! “Inhibitor Camp for All”

by Yasmin C. Pavri

Published: Lifelines for Health Spring 2018

School was over less than 24 hours before we left to participate in “After the Shock 2018” camp organized by CHES. We were tired from a long school/work year and were happy to take some time off from our regular routine.

We reached the camp site after almost a 2 1⁄2 hour drive. The final 40 minutes of which was very scenic. It made us excited and eager to know what the camp site would be like. Camp Zeke (located in the foothills of the Poconos) was where CHES chose to host the camp, and it was gorgeous! Scenic, serene, clean, and extremely comfortable!! Every staff member of the camp was dressed with a smile and was welcoming in nature. Great job CHES for this find.

Janet Brewer, Eric Lowe, Joan Ward, and Chris Brewer - the hard work and effort you put into pulling this camp together showed and every arrangement was flawless. Adult sessions were very well thought out and kid’s activities had so much variety. While parents were learning about developing coping skills to manage deep emotions dealing with the shock of inhibitors, kids were mentored and monitored by their family pals who were not just supervising them but were befriending them. While parents listened to guest speakers who were throwing light on new treatments for inhibitors, or how to deal with insurance and to be a great advocate for themselves and the community, or sometimes just hearing each other’s stories; kids were either fishing, doing archery, engaging in arts and crafts or even cooking some delicious desserts. There were outdoor field games and the lake had kayaking and canoeing. There was something for everyone! There was a ping pong table. There was a basketball court. There was a soccer field. And if you got tired, there was even a place to go relax and do some yoga with professional guidance. It can’t get better!!

I would also like to give a big shout out to Chad Brown, Adam Smith (with Music for the Cause), and Krystyn Strother for being more than presenters. They got so friendly with all these kids and went out of their way to know each one of them personally. One special family pal was Gabe Anspaugh! Unfortunately, my children

are older now and did not require a family pal with them all the time, but Gabe was their friend, support, helper, fellow community member and someone who related to all these young boys with hemophilia with inhibitors. He was such a positive and high-spirited person that no one around him could sit sad or feel lonely. It was like he could put life into a rock. He is the Rock Star of “After the Shock” camp, just like how Lee was to “The Painted Turtle” location when we attended Inhibitor Family Camp in 2015.

There were also these super nurses, Kathy & Brianna, who led the infusion sessions on both days making sure all our children had their factor and were ready to roll for the day. Some even got their big stick award at this camp. Hurray!!

We were a variety of families, each displaying strengths in their own ways and motivating others in the room. I personally made a new friend, a good friend in the community who I know I can share my concerns with and who can depend on me when they need a friend.

On behalf of my husband Cyrus, my children Farah & Porus, and on behalf of all those that attended this camp, we sincerely want to thank you CHES for giving us this opportunity of learning, sharing, laughing, and most of all having fun together.

We look forward to returning to this camp again next year.

An Archer’s Dedication at Inhibitor Family Camp

Our inhibitor programs are all about pushing your boundaries to try something you may have never done before. Read about Evan Chung’s achievement at Inhibitor Family Camp at Victory Junction. This experience had such a profound effect on him, he wrote an essay about it! Way to go Evan-you demonstrate such determination! We are proud of you! -Janet Brewer

Published: Lifelines for Health Spring 2018

By: Evan Chung

I was running down a steep hill at 6:30pm. I was going to archery at Inhibitor Family Camp at Victory Junction. My dad and I came there together. My dad and I had to wait for a while. Finally, I got a spot. It was pretty windy there. I was feeling pretty excited because I just got a spot, then I tried reaching a bow, but I couldn’t reach one because I wasn’t tall, and I was only 6 3⁄4 years old. I tried many more times, but I couldn’t make it. Then my dad took one bow down, and I said I wanted a different bow. But he said it’s not my size and he also said the one he picked was the right size for me. Dad picked a blue bow.

Then came Larry the archery councilor at camp. He said to my dad “good!” I don’t know why. Then Larry gave me twelve arrows. Then he told me how to do archery. Then he said to try to get a bullseye which is when you get your arrow in the middle of your target. Once at a different camp called Camp Bold Eagle, there was archery too, but I never got a bullseye there. In fact, I never got a bullseye, ever. Anyway, I was really determined to even get one bullseye because my dad told me if I get one bullseye, I can get a gold medal from Larry.

Anyway, I shot my first arrow. Really surprising! I did better than last time! Then I shot my next arrow. Then I started going crazy because I just got my arrow in the red part of the target. My dad said, “calm down, Evan” so I calmed down, but in my thoughts, I just kept going crazy. That caused me to waste some arrows because I was going crazy. I only had three arrows left. I shot an arrow. Red part. I shot another arrow. Blue part. Worse. It was my last arrow... and I got the red part. I sighed. “Parent, pick-up!” shouted Larry. Then all of the parents went to pick up the arrows. They also pulled them off the targets.

It was almost lunch time, so I thought I’d better hurry up. But I didn’t waste any arrows. I did my first shot out of twelve arrows. Blue part, bad. Then I shot my second arrow. Red part, not that bad! Well now let’s skip to the ninth arrow. I got it in the yellow, but it was not a bullseye. I was on my second to last arrow. I took a deep breath, then set up and shot. Then... “twang!” It hit right in the middle of the bullseye! Then I started going really crazy. I shouted, “Larry! Larry! I got a bullseye!” Of course, he didn’t hear me because he was working with another kid. Then I shouted again. This time he heard me. He gave me a gold medal. It was the first medal I ever got. I felt really proud. “I did It!”, I thought. Dad said it was time for lunch. Then we ate lunch. I played with my medal. I felt really proud.

New Inhibitor Treatments: Weighing Risks and Benefits FEATURE

Published: Lifelines for Health Spring 2018

By: Dr. Jonathan Bernstein, Md

Over the past several years, hemophilia care has been turned on its head as new opportunities and ideas for the treatment of hemophilia have emerged. During this time, we have seen the greatest change in hemophilia care since Judith Graham Poole first helped to develop cryoprecipitate and observed that Factor VIII could be made cheaply from this product. These new changes include new factors, new bypass products that can be used subcutaneously, and gene therapy. Finally, we now may have two new ways to treat bleeding diseases that have no treatment at this time.

With these new treatments come choices where we must weigh the risks versus the benefits of these drugs.

Sometimes it is hard to know what the risk might be when a drug is first being used to treat patients. We can look at the research that has been done to test the new drug in a well-defined protocol and see if any significant adverse events were reported.

However, research protocols are strict and can have no variance. When a drug is available for use in the medical community, the way it is used by doctors and patients often differ from the strict rules for use that were specified by the research protocol. How many of you have not used your medication in the way you were instructed by your doctor? I know I have – but, of course, doctors are the worst patients. When patients and doctors vary in how they use a drug, this leads to increased risk of something bad happening, and possibly death. Because of our concerns when we first introduce a drug to our patients, we tend to be more conservative in prescribing these drugs. Companies are now required to do what is called “phase 4 research” to try and capture the changes in use over time of a drug or treatment.

Hemlibra

The first of these newer drugs, Hemlibra, had problems early on with one death, and the development of both microangiopathic hemolytic anemia and blood clots. These adverse events occurred when Hemlibra was used with a high dose of FEIBA. This combination of a drug that activates Factor Xa (Hemlibra) and a drug containing both Factor Xa and prothrombin (FEIBA), at high doses seems to activate compliment and cause increased clotting and the development of microangiopathic hemolytic anemia (which always seems to involve compliment activation). With further studies, Genentech, the company that developed and tested this drug, was able to eliminate these serious complications, by decreasing the dose of FEIBA, or by avoiding its use completely. Many of you know that 5 patients have died who received this drug off study under compassionate use. While it is stated that these patients’ deaths were not caused by Hemlibra, it is curious that so many deaths occurred in the Factor VIII inhibitor group, when so few were exposed to the drug. Little information has been released on the causes of deaths in these patients. We also have no data on whether we can tolerize patients while on these drugs. Without tolerance, these patients may never be allowed onto gene therapy protocols, as they might have a greater risk of negative outcomes.

Hemlibra has also solved many of the problems that patients with Factor VIII inhibitors have. Patients on this drug are able to give themselves subcutaneous (SQ) shots once a week, with few if any breakthrough bleeds. Many patients who were bleeding once or twice a week are now going from a month to years without bleeding. This decreases the damage to and joints, improves quality improves compliance (much give a subcutaneous shot), and patients more independent. deciding whether to use this must make a choice weighing risks versus benefits. Patients who are doing well on current treatment with tolerance of their inhibitors, should probably avoid Hemlibra. However, for patients with higher titer inhibitors or frequent breakthrough bleeds, this drug’s benefits will most likely be greater than the risks as long as patients follow instructions for use.

Fitusiran

Another drug on the horizon is Alnylam’s/Sanofi’s Fitusiran. This drug increases the thrombin level in the blood by using small interfering RNA (siRNA) to silence the ability of the liver to produce Antithrombin III through RNA interference. It is similar to a process that the body uses to decrease production of certain proteins. This increases the thrombin level in the blood making up for the level lost when one is deficient in a factor. It can be used in patients with inhibitors of factors VIII and IX, as well as to treat patients with factor deficiencies and platelet disorders that can cause excessive bleeding. It is also given subcutaneously. It is currently being studied in a clinical trial protocol at this time. There has been one death in a patient with hemophilia A. This is thought to have occurred when the patient had a sinus venous thrombosis, which then hemorrhaged, resulting in the patient’s death. Protocols and education were put in place to decrease the chance that this would happen again. The decision to use this drug must be decided by both the patient and the physician after balancing risks against benefits to decide if the risks are greater than or less than the benefits. In patients with factor IX deficiency and an inhibitor, this would allow bypass and improve clotting greatly. In patients withwith no treatment other than antifibrinolytics, such as Factor V patients, as well as severe platelet disorders, such as Glanzmann’s Thrombasthenia and Bernard Soulier, Fitusiran may greatly improve quality of life and outcome.

Hemlibra and Fitusiran may also both make it hard to measure Factor VIII and inhibitors as they both bypass what we usually measure to determine levels. New methods must be discovered that will allow us to find the levels in patients on these drugs.

Gene Therapy

The next set of treatments on the horizon are gene therapies for Factor VIII and IX deficiency.
Both will soon be available. Studies are ongoing with increasing success. Gene therapy will only be offered to individuals above the age of 18 for the foreseeable future. This approach has been a long time coming. However, there are still substantial risks associated with gene therapy. In the past, patients (with disorders other than hemophilia) have developed cancers when treated with a retroviral vector (viral DNA that carries the gene into the cells). Genes carried by these vectors into the DNA often inserted themselves next to genes that allowed unrestrictive division, a hallmark of cancer. The improved treatments that were then developed avoid this problem by either not inserting into the genome or having more specific insertion. This is a vast improvement. One company estimated that only one in ten thousand would insert incorrectly. However, there may be millions of these vectors, that will have to be given. This might mean that several hundred could insert incorrectly. If this happens, it is possible that years later a patient could develop a malignancy – the most likely site of cancer would be the liver as these vectors insert the changes into liver cells. Next, although less worrisome, is that with time a patient who has undergone gene therapy may need to have boosts of the vector to maintain success. However, it is possible that patients might develop antibodies to the vector so that they are no longer candidates for repeat boosts of the vector.

Some patients may not be candidates for treatment with gene therapy. If one has an antibody to the vector the body may reject the treatment. Some companies are attempting to develop several vectors that would avoid this problem. If a hemophilia patient has an inhibitor it could either cause the inhibitor level to increase, or could allow the immune system to see a constant amount of factor and tolerize these patients. I believe that we will see both occur. Plasma-Derived Factor

Finally, a trial comparing plasma Factor VIII to Recombinant Factor VIII showed that plasma
Factor VIII was less likely to cause inhibitors than Recombinant Factor VIII. However, much has changed since the study was started. New factors, some of which had longer half-lives, became available. These factors were not compared to plasma. It is believed that humanized factor VIII or extended half-life Factor VIII may develop as few inhibitors as plasma does, and these may also be as good at tolerizing patients as plasma is. If studies prove this to be the case, we may find that more patients convert to these drugs.

Thus, we now have a wealth of new treatments available with many others on the horizon. There
will be risks and benefits, as is true of all drugs, even relatively benign drugs like acetaminophen or aspirin. When patients are given a chance to treat themselves with these drugs, they must weigh the risks against the benefits and decide whether to use the drug. They must also follow the instructions given to them by their doctors and the drug companies so that they can avoid side effects as much as possible.

It’s Easy! How WE Can Report an Adverse Event

by Jane Cotter Forbes

Published: Lifelines for Health Spring 2018

There are many medications for people with bleeding disorders in the marketplace today and our pipelines are bursting with new therapies and medications. Some medications have been available or many years while others are newly FDA approved. If you or your loved ones experience a serious adverse event (AE) while using a bleeding disorders medication, it is always a good idea to voluntarily report an AE to the Food and Drug Administration (FDA) MedWatch consumer voluntary reporting program. AE’s should be reported as soon as possible to MedWatch using Form FDA 3500B by those who are consumers and medical personnel for the following situations: death, life-threatening medical events (e.g., anaphylactic reactions), AEs that require hospitalization (short or long term), disability or permanent damage, congenital anomaly and/or birth defect, and any required medical intervention to prevent permanent impairment (e.g., hemorrhage). If you are not sure if the AE is related to the medications you are using, it still is a good idea to voluntarily submit a report to MedWatch just in case. Mandatory reporting is required by law by user- facilities, importers, distributors, and manufacturers. AE’s for these organizations need to be reported to MedWatch within 24-48 hours. In considering the filing of a voluntary report for an AE to the FDA, here are some points for guidance!

IT IS EASY. First you go directly to the FDA MedWatch website [https://www.accessdata.fda. gov/scripts/medwatch/index.cfm?action=reporting. home] and click on form 3500B. Then you start filling in the blanks. When you are done, you hit send. In particular, the website asks for a lot number, an NDC number, the strength, the units, and the quantity (how many doses or vials of the medication were used).

Most of this information can be found from the container of the medication and/or on the prescription label. The website asks for a detailed description of the AE or serious problem as well any and all medical reports (if available). You will need to include a list of all other medications in use. This website also suggests not throwing the medication away because the FDA may request what remains for further inspection.

In addition, medication that comes with faulty equipment or devices such as needle breaking or ineffective transfer device to be reported. If you are not able to use the website addressed herein, you are free to call the FDA at 1-800-FDA-1088 in order to file a report. “The FDA encourages patients to report AEs as soon as possible. If the AE team gets a cluster of reports about the same drug in a short period of time, it will be able to respond more quickly.” IT TAKES ONLY A FEW MINUTES. Depending on the details of what you submit and the explanation of the AE, it will mostly likely take only 10 to 15 minutes to complete.

IT IS IMPORTANT. It is the fundamental way by which American people can alert the FDA of any
and all AEs after having taken a pharmaceutical medication. This information is gathered, reviewed, analyzed, and a determination is readily made if further action is needed. All AE reports are kept in the FDA database.

IT IS APPRECIATED. The FDA truly welcomes this information, for it provides ready and immediate feedback on medications which they have approved. It is an essential feedback mechanism. The FDA MedWatch system also allows for consumers or patients to report voluntarily and independently from their doctors. The FDA “understands that for a variety of reasons, you may not wish to have the form filled out by your health care provider, or your health care provider may choose not to complete the form.

IT IS REWARDING. There is a genuine sense of personal fulfillment in having participated in this safety program for not only the American people, but for people worldwide, who might also have access to such medications. It is a tangible means by which we can protect ourselves from harmful medications.

IT IS FOR EVERYONE. All Americans using prescription drugs approved by the FDA are encouraged to report all adverse events or serious problems.

SO WHAT HAPPENS AFTER AN AE REPORT IS FILED?

The FDA reviews all of the submitted MedWatch reports for safety signals - similar adverse events

occurring with a particular drug. Some examples include:

• A new serious AE which is not listed in the drug’s package insert

• An increase in the reporting rate of an event already occurring with a drug

• A new report of how an FDA approved drug interacts with other medications.

• A product’s name, packaging, or labeling leading to an AE needs to be adjusted, relabeled, and added safety information needs to be included.

Depending on the information found during the FDA’s review of the safety signals and the severity of the AE, the FDA may decide to:

Update the drug’s package insert or labeling

• Place restrictions on the drug’s distribution

• Release a drug safety communication to inform the public

• Withdraw the drug from the market

A Mitigating WHAT?

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

Published: Lifelines for Health Spring 2018

Child Find

Child Find is part of the 504 law in which a school district is responsible for evaluating students who have a suspected disability to determine if they need related services and accommodations under a 504 plan. This extends to students in private schools and who are homeschooled. The district is responsible for evaluating and offering appropriate services and accommodations in the public school setting if a private school student is identified as having a disability. Parents whose children don’t attend public schools may not understand what kind of help and services are available.

Section 504 of the Rehabilitation Act of 1973

Section 504 was initially enacted as one part of the American’s with Disabilities Act. It PROTECTS students with a disability and requires public institutions who receive federal funding. A plan is developed to facilitate EQUAL access to a disabled student as a non-disabled student.

Section 504 was enacted to protect qualified individuals from discrimination based solely on their disability. Whether a particular student is protected under Section 504 requires a determination that the student is an
“individual with a disability” and that the student is “qualified” under Section 504. A student is “disabled” under the Section 504 regulations if the student meets any one of the three criteria listed in 34 CFR §104.3(j)(1). An individual is considered disabled under Section 504 if the individual:

1. has a physical or mental impairment which substantially limits one or more major life activities (SEE table 1 for “Major Life Activities”),

2. has a record of such an impairment, or

3. is regarded as having such an impairment.

“Substantially limits” is measured against their same age, non-disabled peers in the general population without the benefit of medication or other mitigating measures such as accommodations.

Table 1 (Major Life Activites)

“Major life activities, as defined in the Section 504 regulations at 34 C.F.R. 104.3(j)(2)(ii), include functions such as caring for one’s self, performing manual tasks, walking, seeing, hearing, speaking, breathing, learning, and working. This list is not exhaustive. Other functions can be major life activities for purposes of Section 504. In the Amendments Act (2008), Congress provided additional examples of general activities that are major life activities, including eating, sleeping, standing, lifting, bending, reading, concentrating, thinking, and communicating. Congress also provided a non-exhaustive list of examples of “major bodily functions” that are major life activities, such as the functions of the immune system, bleeding disorders, normal cell growth, digestive, bowel, bladder, neurological, brain, respiratory, circulatory, endocrine, and reproductive functions.”

Children that have bleeding disorders have the right to a “free and appropriate public education (FAPE)” regardless of the type of disability or the severity. A 504 plan lasts for one school year and MUST be reviewed/ updated annually. A 504 plan protects your child and ensures that the school and school staff are implementing all accommodations your child requires!

What should be in a 504 plan?

The plan should outline what accommodations a student needs
to access academics (instructional supports), the physical building space, transportation, testing (standardized and regular classroom), behavior etc. This is to ensure that students with
a disability have equal access to all education as their non-disabled peers. For students with bleeding disorders, it is imperative that part of the 504 plan addresses self-management of health conditions in the school setting or school-sponsored events (field trips, dances, field day etc.). Additionally,
if self-management is unattainable, the 504 plan should address how the student will have access to health management and by whom. It is also important that the plan address how the plan will be monitored and who the school contact person is.

Many districts across the United States find it easier to say to a family that their child with bleeding disorders
should not participate in physical education or recess. This is a civil rights violation! Your child has
access to all the activities that their non-disabled peers have access to. It is recommended that a 504 plan
specifically addresses physical activity as one part of the plan. For example, a student with a bleeding disorder might consider joining a swim team but not a football team, or can play golf but not soccer. It’s imperative that the 504 plan be a specific as possible!

The 504 Plan would define the signs and symptoms of your child’s bleeding disorder (i.e. external bleeding, swelling, tingling etc.). Additionally, it should address an emergency care plan for the school about what should happen and who is responsible in the case of a bleed. There should be a coordinated plan between home and school regarding specificity around what to do with a bleed. There should be listed contact numbers for Hemophilia Treatment Center’s (if used) and/or pediatrician, hematologist and parents along with any other pertinent members of the Team. It should discuss any accommodations necessary for the student to access the building (wheel chair accessible, elevators etc.) and who is responsible for keeping teachers and staff (including substitutes) up-to-date on the students’ 504 plan and/or health care plan. Often times, a 504 and health care plan overlap. Some districts insert the health care plan into their 504 document and vis a versa.

Mitigating Circumstances

The Americans with Disabilities Amendment Act of 2008 updated eligibility based on positive mitigating circumstances. They ruled that the determination of a disability must focus on whether or not the student would be substantially limited in performing a major life activity REGARDLESS of what the mitigating circumstances are. Mitigating circumstances are things like medication (i.e. Factor), medical equipment/devices, mobility devices etc. For comparison, refer to table 1 on pg. 30. The law also notes that it can take into consideration the negative mitigating circumstances which is considering the side-effect or burdens of the mitigating circumstances and whether there remains a need for the accommodation.

Post-Secondary 504 Information

ADA rights carry into the college setting. However,
it is the responsibility of the student to self-identify and request appropriate services to staff. It stays as confidential information and can not be used against the student. Generally, there is a Disability Services office on all college campuses. One thing to keep in mind, is that unlike 504 plans in public schools, there is not a written plan. The college student needs to advocate for himself/herself and provide documentation that can help the disability services center provide the accommodations needed. Although it is not a written plan per se, students have the rights to the accommodations needed!

The Access to Marketplace Insurance Act: Allowing Charities to Be Charitable

by: PSI, Mandy Herbert & Jim Romano

Published: Lifelines for Health Spring 2018

Patient Services Incorporated (PSI) is leading the effort to pass H.R. 3976, The Access to Marketplace Insurance Act, and protect the valued safety net of patient assistance. This legislation is in response to a regulation issued in 2014, during the implementation of the Affordable Care Act (ACA), that allowed Marketplace insurance companies to deny charitable third-party premium and cost-sharing assistance. The rule essentially provided a permission slip to health insurance providers to prohibit assistance by charities looking to help patients in need in order to shift sicker patients off their plans.

Since 2014, the impact of this regulation has been considerable, with approximately 90 insurance plans in 43 states having implemented the prohibition. This rule allows insurers to evade the reforms of the ACA removing the pre-existing condition exclusions and provides an open route to discriminate against
patients with higher healthcare costs simply because they receive assistance. Taking these discriminatory practices, a step further, health insurance providers have also attempted to expand this prohibition into other markets including the Medicare Supplemental Insurance Market.

In response to this misguided policy, PSI created the Marketplace Access Program (MAP) coalition to bring together leading patient advocacy groups and patient assistance organizations dedicated to protecting charitable assistance for individuals suffering from chronic and life-threatening illnesses. The focal point for our coalition’s work is H.R. 3976, federal legislation that would require insurers to accept assistance from non-profits, places of worship, and local civic organizations. PSI has worked closely with H.R. 3976’s sponsor, Congressman Kevin Cramer (R- ND), to develop this legislation and stop this harmful policy from continuing.

We have made great progress on the bill, garnering over 130 cosponsors since introduction in October.

PSI would welcome any assistance from readers who would like to get involved. If interested, please contact PSI at jromano@uneedpsi.org or (804) 521-7908 for more information.

Clinical Trials: For Inhibitor Patients

by Ann Marie M. Minichiello

Published: Lifelines for Health Spring 2018

As research and medical advances progress, it is important to recognize that this momentum is not possible without the involvement of patients. Without clinical trials, new drugs and treatment options cannot be made available to those who would benefit from them. Presently, there are six clinical trials recruiting patients with hemophilia and inhibitors. The following studies are all “interventional” (clinical trials) with the primary purpose of treatment. The information on each of the six trials begins with its official title and drug being used.

1. Individualized ITI Based on Fviii(ATE) Protection by VWF (INITIATE) with Wilate® began on June 1, 2017 and is estimated to be completed by December 1, 2022. Requiring 120 participants, the primary goal of this trial is to compare the outcome of individualized lot selection to random lot selection using Wilate® for immune tolerance induction (ITI). The principal investigation site for this study is the University of California, Davis. (Sponsor: UC Davis)

2. A Non-controlled, Open-label, Multicenter,Study of Efficacy of rFVIIIFc for Immune Tolerance Induction (ITI) in Severe Hemophilia A Subjects With Inhibitors Undergoing the First ITI Treatment , utilizing Biological: rFVIIIFc, began June 30, 2017 and is estimated to conclude December 2020. There are 30 participants needed for this study to describe the time to tolerization using rFVIIIFc within 12 months of ITI. There are 9 sites recruiting. (Sponsor: Bioverativ Therapeutics, Inc.)

3. A Phase III Study on the Safety, Pharmacokinetics and Efficacy of Coagulation Factor VIIa (Recombinant) in Congenital Hemophilia A or B Pediatric Patients From Birth to <12 Years Old With Inhibitors to Factor VIII or IX: PerSept 2, using Biological: Coagulation FVIIa (Recombinant), began in December 2015 and was estimated to conclude August 30, 2017. Requiring 24 participants, the goal of this study is to assess the safety, efficacy and pharmacokinetics of 2 separate dose regimens of Coagulation Factor VIIa (Recombinant) for the treatment of bleeding episodes. The principal investigation site is the University of Colorado, Denver, with 2 additional sites. (Sponsor: LFB USA, Inc.)

4. ATLAS-INH: A Phase 3 Study to Evaluate the Efficacy and Safety of Fitusiran in Patients With Hemophilia A or B, With Inhibitory Antibodies to Factor VIII or IX is utilizing Fitusiran, recombinant Factor VIIa and FEIBA. This study began January 8, 2018 and is estimated to

   be completed in July 2019. Requiring 54 participants, the goal is to determine the frequency of bleeding episodes in patients receiving Fitusiran for prophylaxis, compared with patients who continue with their regular medication. There are 5 sites recruiting. (Sponsor: Alnylam Pharmaceuticals)

5. A Phase IV, Multicenter, Single-Arm, Open-Label Study of Emicizumab (Hemlibra) Prophylaxis in Patients With Hemophilia A and Inhibitors Undergoing Minor Surgical Procedures with Emicizumab began March 31, 2018 and is estimated to conclude June 28, 2019. 30 participants are needed for this study to evaluate whether participants receiving Emicizumab prophylaxis can safely undergo minor surgical procedures without prophylactic bypassing agents. There are 3 sites presently recruiting. (Sponsor: Genentech, Inc.)

6. A Phase III, Multicenter, Open-label Study of the Efficacy and Safety of B-Domain Deleted Recombinant Porcine Factor VIII (BAX 802) in Subjects With Congenital Hemophilia A With Factor VIII Inhibitors Undergoing Surgical or Other Invasive Procedures with Biological: Antihemophilic Factor (Recombinant), Porcine Sequence (BAX 802), began December 22, 2016 with an estimated completion date of June 1, 2018. There are 15 participants needed for this study to evaluate the efficacy and safety of BAX 802 in patients who are undergoing surgical or other invasive procedures. There is 1 site recruiting in the U.S. (Sponsor: Baxalta, part of Shire)

The safety and validity of a study is the responsibility of the sponsor and investigators. As with any clinical trial, please consult your healthcare provider (HTC) before participating. For more information, please visit www.clinicaltrials.gov.

PTSD and Chronic Health Conditions

by Krystyn Strother

Being diagnosed with a medical problem can be distressing, even traumatic. The experience of trauma is entirely subjective but it can lead to post-traumaticstress disorder (PTSD). Let’s first look at the definition of PTSD and Trauma:

Trauma is defined by the American Psychological Association (APA) as an emotional response to a terrible event.1 That’s a pretty broad definition and can be entirely subjective.

PTSD or Post Traumatic Stress Disorder, is described as an intense response to trauma and can create severe distress and can limit one’s ability to function in life.2 PTSD from chronic pain or illness is very real and if you think about it, makes a lot of sense.3 Chronic illness and pain are all at once traumatic to the body, brain and emotions, and the level to which people experience symptoms are vast. Some folks dowell, while others struggle to get out of bed each day.

One of the tools that we possess to cope with trauma is to work directly with the brain using mindfulness. Science has shown us, through a huge number of studies, that the brain does not become static once it reaches maturity. It continues to change based on our experiences and actions by way of neuroplasticity. They gray matter in our brain can get bigger or smaller; neural connections can become stronger or weaker. Every time you learn a new dance step you are creating a new neural pathway.

We often hear about neuroplasticity in relation to using techniques to heal from injury, cope with chronic pain, deal with stress and anxiety. While creating new neural pathways does help us deal with these things, it can also work against us when we have negative experiences. When trauma is encountered, the brain creates new pathways and changes its structure in response to the event in a way that does not serve us well.4

There is a significant amount of data that supports using mindfulness as treatment for those affected by trauma and PTSD.5 Practicing mindfulness reverses the neurological patterns taking place with trauma and PTSD by decreasing areas of the brain that are more active in stressful situations and increasing activity in areas of the brain that regulate our emotion and decision making.6 Do a google search for “mindfulness brain scan” to see side by side images of brain function before and after practicing mindfulness.

It is important to realize and reiterate that fear or anxiety around procedures, infusions, or other pokes and sticks is nothing to be ashamed of, and trying to get yourself or your child to accept an “it’s no big deal” attitude is often not the answer, especially if there is trauma and PTSD involved. Fortunately, there are tools that we can use to help our kids, and ourselves, become more comfortable. Practicing together as a family will help those with fear, anxiety, or trauma feel less alone and supported.

1. Progressive relaxation

   A. Tense the muscles of your body (arms, upper body, legs) and hold for a count of 10.

   B. Release the tension and relax to the count of 20.

2. Belly breath

A. Relax your face and place one hand on your belly. Take long, slow, deep breaths in through your nose and out through your mouth. Feel the belly rise as you breathe in and gently drop as you breathe out. Don’t force the breath, let it be easy. Do this for 5 or more breaths.

3. Fear wave

a. This is helpful for those that may have a fear of needles or experience anxiety around pokes and sticks. This can also be used as a template for other procedures.

We begin by noting what it is that makes us the most anxious. Rather than tackle the big stick first we can start with small things. Use the chart below and have your kiddo rank what scares them the most. This one is filled out as an example.

Begin with the least difficult situation. You can visualize this situation as an island out at sea. You slowly paddle out in your boat or surfboard (your kid’s watercraft of choice) toward this point. If anxiety begins, hold where you are in the water and try a few rounds of progressive relaxation or belly breath. The anxiety doesn’t have to go away completely but it will begin to lessen and become more manageable. Continue moving towards the feared situation. Each bout of anxiety or fear is like a wave. It builds and intensifies and then it will gradually reduce. Staying with the fear allows us to see how anxiety makes us feel and shows us that it is only temporary. Ride the waves of the sea until you find yourself at the shore of this particular island.

When your child feels confident with one situation, move onto the next. Eventually, with visualization practice, there will be smooth sailing towards these situations. This practice changes the way the brain reacts to the actual fear inducing situations when they arise. Overcoming your fear will take some time and practice, but in the long run, it will make life less stressful and you will feel less anxious.

Rewarding Surrender

by Stephanie Shea

Published: Lifelines for Health Fall 2019

When I first heard about the MUMS event (Moms’ ‘Uninhibited Meeting), I made up a zillion reasons why I wouldn’t be able to go. The dates weren’t right with tentative summer plans we had already made, it was too close to other events we had already committed to, my son might need me (his port had been acting up), a friend had mentioned she may potentially come to town that weekend....yada, yada, yada. Excuses. All of them.

The truth was, I was scared to leave my 5-year-old son. My husband is amazing and completely capable of taking care of our two boys, daily factor infusions and all. But if something did happen to my boy, I would want to BE there - to reassure and hug and comfort and do all those things that us mamas do. But it’s often all of those things that us mamas do that can lead to burnout and feeling depleted. And when we’re exhausted and
not taking care of ourselves, it’s often the case that we don’t have anything left to give to those we love around us.

Being a caregiver for someone with a chronic condition such as hemophilia can be frustrating
at times and exhausting. It’s often emotionally, physically, and mentally draining. It may limit your social life and can cause financial problems. Burnout occurs when the stress and burden from these negative effects become overwhelming, negatively affecting your life and health.

When I received the email about the MUM event,
I was definitely experiencing burnout. Before I had children, I was a professional counselor/therapist,
so I was very aware of the signs and symptoms. Yet I still had a hard time agreeing to the trip. My husband finally -lovingly- nudged me to commit to it (and I’m so grateful he did!)

The event was held at a beautiful hotel in downtown Santa Fe. When I first arrived and checked in to my room, I felt at a loss. What do I do with myself when I don’t have two little humans to look after?! I realized my predicament and committed to sinking into that uncomfortable space for the weekend in hopes of not only recharging my mama batteries but rekindling my relationship with myself.

The event started with a welcome session before dinner as everyone began trickling in from different places all over the U.S. Since I was one of the first to arrive, I took the time to chat with some of the other moms. And then I had a very happy surprise when I met someone with whom I had only ever connected with online. She was my first ever hemophilia mom friend and she reached out to me online through social media mere days after my son was first diagnosed (when my head was still spinning from the shock). She was a huge support to me then and I was thrilled to finally meet her in person. And I had no idea she was going to be there, such an awesome surprise!

Connecting with other moms at the event was definitely the highlight of the weekend for me. Hemophilia can be such an isolating condition to live with and when you throw an inhibitor into the mix, it can feel even more isolating within an already isolated community. I feel fortunate because I do have a wonderful group of friends and tons of support outside of the hemophilia community. Yet, sometimes I still feel isolated. When we’re going through a lot of hemophilia related issues, it tends to be forefront on my mind. And if I don’t talk about it to the people who I spend my time with, I don’t feel authentic or as if I’m able to truly connect. And although I have friends who will listen and provide support, there’s a part of me that knows they will never truly understand what I’m experiencing.

That is not the case with hemophilia moms and especially other mamas of kiddos with inhibitors. I often feel an automatic connection, a knowingness that we truly get what it’s like to walk in the others’ shoes. I also often learn more from other members of the community than I do from medical professionals and this weekend was no exception. Connecting with other mothers has been so incredibly healing for me and the MUM event was invaluable for that.

We had a “rap” session that was a bit of a free for all in terms of subject matter. We talked about the things that we felt were necessary to talk about. We talked about the biggest challenges that we face in raising a child with intense medical issues. We discussed new treatments and how we feel about them. We spoke about how we make decisions for our children and how we advocate for them. We talked about how we manage (or don’t manage) the stress we face in our lives. We spoke about anything and everything related to mothering a child with an inhibitor and it was very satisfying and therapeutic.

After dinner on the first night, most women walked a few blocks into downtown Santa Fe and checked out the sights. There was live music and wine-tasting and art and antique cars and all sorts of interesting things to see and explore. But the best part of the night? And the best part of the entire event? Simply being with other mothers who know what it’s like to have the extra challenges of raising a child with hemophilia and inhibitors. Thank you CHES for allowing me this opportunity!

Taking Time On

by Donovan Guerrero

Published: Lifelines for Health Fall 2019

Action. The willpower that it requires to take risks is vital to growing as a person. Sometimes I struggle with this. I think it is imperative for young adults like myself to do this to gain independence.

This past September I was blessed with an opportunity to apply for a program called LeverageSM. I had heard very little about this camp specially designed for adults with inhibitors. My mother actually filled out part of the application without my knowledge. I was shocked to get a call from Eric at CHES, never having met him, asking if I’m still interested in the program. I was unfamiliar with the two facilitating organizations, GutMonkey and CHES. Within three weeks of that phone call, I was packing my gear and preparing to go to Portland, Oregon.

The first lesson that I learned was the elephant-rider- path-theory (a concept that explains the conflicting struggles within all of us. Developed by the Heath brothers in a book called Switch, it’s the idea that everyone has a figurative elephant along with a rider inside him/herself. The elephant (relative to our emotions) is often controlled by the rider (our thought of logic.) But at times, our elephant (or emotions) can derail from its path by outside factors. And at this point, it’s our rider’s responsibility to motive our elephant to return to its path.) This lesson resided with me the most. There was not one significant moment that I could point to as being a pivotal moment during this program. I think it was more of an accumulation of different challenges and tasks that I can focus on. To name a few in particular, would have to be the rock-climbing tower, building didgeridoos, and the high ropes course. I felt as though these were the biggest barriers I overcame. From the burning of my forearms on the tower, frustration of my didgeridoo cracking all over, and recovering from the splits on the stirrups obstacle on the high ropes course. My elephant wanted to give up – tell me to stop climbing, stop trying, stop caring. But the elephant-rider-path-theory really pushed me to succeed.

Personally, I think about when I do great things or go to amazing places, I always end up asking myself the same question when I return home: “What’s next?” For example,
I like setting goals. One goal I made a few months prior was to get my boating license so I can operate a boat that has 25 horsepower or more. I’ve almost completed the course currently and hoping to pass the test soon after. My current boat does not require a boating license since it only has 7.5 horsepower. I did it to learn the “rules of the road” so to speak and so I’m already ahead of the game when I get a big boat in the future. When the time comes, I’m sure I will evaluate my decision and think to myself, “Yay, you passed the test!” Then when I buy a nice boat, I will think, “Well, now I guess I should take a fishing trip to Alaska for salmon, or Florida for smallmouth bass, or even the Great Lakes for perch and crappie.”

It’s that drive that I have to learn and share. For me, I want to keep setting goals and I don’t want to meet them,
I want to surpass them. To go above and beyond. Like the difference from getting an “A” or a “B” on a test. I’m at a point in my life where I graduated high school and I’m not attending college this year. I want to go next year but I wanted to take time off and become a more mature person, learn more on my own and gain independence, and work to save up for college. All of these facets would contribute to working on my mental health as well. What I really want to do is take the remainder of the year head on. Take a “gap year” on. Leverage definitely gave me the support and the confidence to do that. I have big plans ahead of me. Taking action to put them in motion is the crux of the issue. I want to be a leader in some fashion.

Thank you to Takeda pharmaceuticals for making this event possible. Thank you to CHES and GutMonkey for putting on a great Leverage 2019.

Camp Fosters Healing, Friendship, & Play

by Stephanie Shea

Published: Lifelines for Health Fall 2019

In June, we had the opportunity to attend CHES' After the ShockSM inhibitor camp. It was a hard decision for my family as it happened to fall on the same weekend as our local chapter’s annual camp. Although my 5-year-old son Dakoda’s inhibitor isn’t “active”, we decided to go to After the Shock as we felt we might relate more with this community. And I’m so glad we did. One of the biggest blessings that we have found on our hemophilia journey has been the community....and the love and support we get from them. At times, we have felt isolated among the “normal” hemophilia population. Not all, but many that I’ve connected with, seem to infuse factor prophylactically a couple to a few times a week with no issues and they have been fortunate enough not to experience any major traumas.

Those of us that have experienced inhibitors know that it’s an entirely different ballgame for us. Although Dakoda’s inhibitor has been considered partially tolerized for almost 2 years, his half-life is very short, so he still needs daily infusions, aka, needle pokes. Daily infusions can definitely take their toll on a family, not to mention the task of emotionally healing from past traumas (head bleeds, prolonged bleeding from ports, joint bleeds, etc.). Most families with inhibitors know this path all too well.

On the first day of camp, we learned that every family was assigned a camp “buddy” who would stay with the kids for most of the day, even while parents went to educational classes. On the first day, my husband and I were on the tennis court, watching Dakoda and his little brother Phoenix “play” tennis. We started talking with a father who we’ve met before at other events. Dakoda and Phoenix wanted to run to the upper fields to play soccer with their camp buddy.

The other father we were talking with could clearly see the anxiety on our faces (as we have very rarely let Dakoda be with anyone other than us or one or two very trusted family members). The father of the boy was several years older than Dakoda and he shared his own experiences with anxiety. He encouraged us to let go and trust that Dakoda would be ok without us. During the conversation, my husband and I both teared up and were greatly touched. It set the stage for us to loosen our grip for the rest of the weekend and it felt like a turning point in both of our personal healing journeys.

Everyone in the inhibitor community seems to just “get it”. They get that we’ve all seen our children in pain, we’ve all seen them suffer and we’ve all been through a lot of trauma, which often results in fear and anxiety.

Aside from the immense emotional value we received from talking and sharing with other parents, the camp itself was incredible. Set on a lake in the

Pocono mountains in Pennsylvania, the scenery of Camp Zeke was breathtaking. We were surrounded by lush green trees and grass and it was so nice to be immersed in nature. We have attended many national and local events in the hemophilia and inhibitor community, and they have all taken place indoors, mainly in small windowless classrooms. The difference at After the Shock was staggering. The kids were able to be how kids are meant to be - running carefree and playing in nature. Even when the parents were in educational sessions or talks, the rooms had huge windows overlooking the lake and giant trees, giving us the sense that we were in a forest.

There's so much to do at Camp Zeke, that there was never a dull moment. There was the lake, of course and
all the activities that came with it (kayaking, fishing, swimming) with an outdoor pool, nature trails, a full theater, art studios, and so much more!

The educational sessions were great too! In addition to Current Treatment of Inhibitors and separate “rap sessions” for the Moms and Dads, there was also Aqua Therapy and Yoga for Relaxation. My personal favorite though was

The Bio-psychosocial Effects of a Chronic Condition. The speaker was knowledgeable about trauma as well as the intensity and daily challenges that come with managing a chronic condition. I feel that both my husband and I greatly benefited from this session. We took home tools and techniques that have enhanced our lives, especially in times of stress and anxiety.

When recalling the weekend, we spent at After the Shock, there was a special incident that stood out for me. Dakoda wanted to play basketball, but a group of older kids were in the middle of a game on the court. Dakoda just hopped on and started “playing”. Instead of having him move off the court and wait for a turn like many kids their age would, they slowed down the pace of their game and included him. When I witnessed this, my heart expanded, and I teared up again. I know that most kids with hemophilia and inhibitors have probably been excluded at some point in their lives and instead of doing the same thing to a younger kiddo, they made the point to include him (despite it diminishing the speed and flow of their game!) Every child at camp seemed to exhibit such kindness and compassion and it made me so grateful to be a part of this community.

The final night was special, with a talent show and a wooden wish boat launch to close out the weekend. My boys have already asked me when we can go back to the camp in Pennsylvania. And they’re not the only ones, I can’t wait until next year!

Momentum, Men's Inhibitor Retreat: The Old Guard

Published: Lifelines for Health Fall 2019

by Kenneth Martin

Many, and I do mean many, years ago I would go to the original Camp Bold Eagle and events through the local chapter, and my brother and I would be the lone people with an Inhibitor. There was talk about new treatments and I would ask, “Does it work for inhibitors?”, and the answer was always the same - “No.” Not until the early 90’s was there specific treatment for inhibitor patients. Even then the effectiveness of those treatments for me were barely measurable. I participated in trials and studies and again, the results did not include an opportunity to change things for myself.

I was formally diagnosed with an inhibitor at age 5, but it was Dr. Jean Lusher’s opinion that I had always had an inhibitor to Factor VIII. I had been dealing with, living with, succeeding with (to an extent), my inhibitor for years. I was married with two boys, working full time and had a life outside of hemophilia; even with having over 40 bleeds a year.

Due to the dark ages in hemophilia in the late 1980s and 1990s, I retreated from the community to concentrate on my family which was fine by me because there wasn’t anything available for me or my specific condition for nearly twenty years. Then new products, new treatment regimens for people without inhibitors became the norm in the community. Mysteriously, the number of inhibitor patients increased. So much so that in 2005, there was an actual program created JUST FOR INHIBITOR PATIENTS! My wife and I attended in 2006 and met a lot of other patients who were dealing with being newly diagnosed with an inhibitor and how it was changing their life. I told them that I had never remembered not having an inhibitor.

From that early program, other resources have evolved. CHES is one of them. It allows for adults to interact and relate their experiences, troubles, triumphs to each other and most importantly... just listen. I actually cherish these new opportunities to meet and become friends with others who are going through the same things that I am going through. Unfortunately, there aren’t many that have been there before me to rely on anymore, but there are a few who are in similar situations in their life like myself. Programs like Momentum help me, help others and provide an opportunity for me to receive what I might need at a given time. Whether it be new ways of doing things, new ways of keeping myself healthy, both physically and mentally, this program teaches, helps, and nurtures my well-being to keep me moving forward in my life.

Biopsychosocial Aspects of a Chronic Illness 2019 FEATURE

Published: Lifelines for Health Fall 2019

By: Anna Maria Bell, LICSW, LCSW-C, LISW

Having a chronic medical diagnosis, can be extremely challenging. Along with those challenges, caregivers and those managing the diagnosis often face periods of isolation, depression, and anxiety. Because of this fact, taking care of your mental health becomes as essential, as taking care of your physical health.

Mental health encompasses many things including emotional, psychological, and social well-being. Your mental health will affect how you handle stress, relate to others and make choices. Mental health may have
a biological factor or be caused by life experiences including trauma and abuse. As we explore the literature surrounding chronic conditions and mental health, namely depression and anxiety, we see a substantial cause and effect scenario. Some research suggests that due to constant issues with managing pain, the cost, as well as the stress and worry of managing the condition causes mental health complications. Other research asserts that there is an increased likelihood for a chronic condition to develop and manifest, if a person is diagnosed with mental health concerns as a result of not taking care of one’s medical needs including non-compliance with medication, attending doctor’s appointments and following medical recommendations.

Living with a chronic condition can become all consuming. Taking the time to manage multiple appointments, infusion regimens (often including their own, their children or loved ones), managing the pain and subsequent doctor’s appointments involves many hours of planning and juggling. Because of this fact, paying attention to the signs of depression and anxiety often take a back burner to day-to-day responsibilities, leaving some undiagnosed and most definitely untreated.

Take for example the chronic condition of hemophilia. Because of the genetic predisposition for it, caregivers have little to no adjustment time before their role as caregiver begins. (According to Schwartz, Powell & Eldar-Lissai, 2017), since people are born with hemophilia, family members are initiated into the caregiving role as soon as the family becomes aware of the diagnosis. Because of this, caregivers need to equip themselves to be able to manage multiple co- morbidities, while also taking care of themselves mentally because caregiving is physically, emotionally, and financially demanding. (Miravitlles, Pena- Longobardo, Oliva-Moreno & Hidalgo-Vega, 2015).

Depression and Anxiety

Depression and anxiety present themselves in different ways. Coping with a disorder causes the person to have thought processes that, if gone untreated, can lead to mental health complications. Some of those thought processes may include:

• What did I do to deserve this (why me?)

• I am not equipped enough to handle this

• I feel unfulfilled due to this diagnosis

• My body is flawed

• Why is my life so complicated?

Without proper coping mechanisms these thoughts can manifest into the symptomatology of depression and anxiety.

Anxiety is the way people respond to certain objects, stimuli or situations. These responses often involve feeling dread and fear. With a chronic condition, this fear and dread can often be centered around effect management, including quality of life issues as well as life expectancy.

With the genetic component of some chronic conditions, fear and dread is often fueled by familial examples of family members living, coping and managing it. If these “examples” have not been positive in nature or have resulted in failing health, issues with pain management, increase in bleeding episodes, multiple hospitalizations, multiple surgeries, and an overall decrease in quality of life, then the perspective on living with the condition can cause fear and dread leading to anxiety symptoms.

Depression is the persistent feeling of sadness or loss. It often manifests itself with a wide range of behavioral and physical symptoms including thoughts of suicide. These symptoms can be broken up into several categories including mood, behavioral, sleep, physical, and cognitive symptoms.

In persons managing a chronic condition, the feelings of loss of control over their own body, along with physical complications from managing it can often result in feelings of isolation, agitation, apathy, hopelessness thereby presenting as depression. As the focus of managing the disorder takes precedence, it often prevents the affected individual or their caregivers, from seeking out treatment, for depression and anxiety.

A team led by researchers at the Munson Medical Center, and supported by Pfizer, conducted a survey to better understand the incidence and impact of anxiety and depression among hemophilia patients. The study recruited 200 participants, of whom 80.5% had hemophilia type A and 19.5% had hemophilia type B, between 2013 and 2014 at national or state conferences for people with hemophilia in the United States. Among the participants, 68% had hemophilia classified as severe. The survey revealed that 186 (93%), of the participants experienced symptoms consistent with depression, and 184 (92%), had anxiety.

Among these persons, 52 (28%), reported moderate-to- severe depression, while 23 (14%) had moderate-to-severe anxiety. What is most significant about this study is that more than half of the respondents indicated no history of “diagnosed” depression. This adds to the argument that this population, although exhibiting symptoms, often moderate to severe, are still going undiagnosed and thereby untreated. With this knowledge the researchers suggest that patients and caregivers managing chronic conditions, in this case Hemophilia, should undergo routine screenings at their HTC’s or doctor’s appointments. Most HTC’s have social worker’s on staff who are available and licensed to complete these assessments. As medical models
are expanding their focus, including the “whole person” philosophy of mind/body wellness, hopefully the stigma surrounding mental health diagnosis can be removed and these screenings will become common practice.

Complications of Pain Management

More than 25 million U.S. adults have some level of daily pain, and 10.5 million have considerable pain every day. Managing hemophilia often involves treating pain by utilizing medication. Often these medications are prescribed as a daily course of treatment. For more than 25 years, opioids for the treatment of acute pain have been strongly encouraged, in the past 2 decades, the treatment of chronic pain using long-term opioid therapy has become more common. Although prescribed with intentions of increased quality of life and freedom from constant pain, this regimen of pain management has resulted in unintended consequences. The dependency on these medications have caused addiction rates in this population to skyrocket as well as all the complicating factors associated with addiction. These include financial hardships, family conflicts, an increase in rates of depression and sadly an increase in suicidal rates.

The correlation between addiction and mental health has been researched and proven over the years. As the communities of people who suffer from chronic conditions battle to find ways to live a “normal” life as pain free as possible, it is important to closely monitor dependency and to also seek out treatment from certified addiction counselors who help to develop alternative methods and strategies to managing pain, free from addiction. Some emotional/behavioral symptoms of drug dependency include:

• Loss of control over the amount and frequency of use

• Craving and compulsive using

• Continued use in the face of adverse consequences

• Missing work/school

• Isolating/secretive about activities

• Relationship/marital problems

• Irritability/argumentative

• Defensiveness

• Inability to deal with stress

• Confusion

• Blaming others for their problems

Survivor’s Guilt

Survivor guilt is a mental condition that occurs when a person believes they have done something wrong by
surviving a traumatic event when others did not, often feeling self-guilt. In the hemophilia community, survivor’s guilt is often not spoken about. At most national conferences they honor those who have passed on from complications associated with hemophilia, at “Remembrance Services”. These services are well attended, full of amazing and beautiful stories of love, sacrifice, and ultimate loss of loved ones. Some of the stories are about fathers, sons, moms, brothers and sisters. Then there are those stories of 5 uncles and a dad, 6 siblings and a father, whole generations of families who have fought the valiant fight and lost the battle with disease. Then you hear the remaining family discuss, or “why not me, when I lost my dad and all my uncles?” These are poignant examples of survivor’s guilt. This guilt adds to the symptoms of depression and anxiety. Often resulting in self-loathing, increased sadness and feelings of isolation.

As those suffering with chronic conditions deal with this survivor’s guilt, the desire to attach and form relationships often is compromised due to the feeling of inevitable loss or that they may be next so why get close to anyone. Survivor’s guilt may also induce non-compliance with treatment regimens with a subconscious desire to “be with the lost loved one” or “I am going to be next so why not speed up the process”. These feelings may appear extreme but are realistic to the person who is experiencing them. Survivor’s often have both depression (sadness, apathy) while also experiencing anxiety (fear and dread), at the same time. As a survivor who is living with a chronic disorder, managing these feelings through therapeutic intervention is paramount.

Suicide

Suffering debilitating pain every day may be so unbearable that some decide to take their own lives. In a study, led by Dr. Emiko Petrosky from the U.S. National Center for Injury Prevention and Control, in 8.8 percent of suicides in 2014, there was some type of history of chronic pain. This was a significant increase from previous studies in 2003 which only indicated a 4.7 percent history of chronic pain. Among suicide victims with chronic pain where toxicology results were available, opioids were much more likely to be present at the time of death than in those without pain. In addition, if a suicide note was present, more than two-thirds mentioned a pain condition as well as long suffering from the pain as a direct contributor to the suicide crises.

These startling statistics highlight the importance of mental health intervention for anyone attempting to manage a chronic condition. It also highlights the need to improve pain treatment, not only for the direct effect on management but also as a “method to raise hope” in persons coping with chronic pain due to a chronic condition. For those who may have or have had thoughts of suicide, it is imperative that you seek out intervention from someone who is licensed to provide services in crises situations. Some of the warning signs or things to look out for include:

• Inability to perform daily tasks like bathing, brushing teeth, brushing hair, changing clothes
• Rapid mood swings, increased energy level, inability to stay still, pacing
• Suddenly depressed, withdrawn; suddenly happy or calm after period of depression
• Increased agitation, verbal threats, violent, out-of-control behavior, destroys property
• Isolation from school, work, family, friends
• Paranoia
• Increased or new substance usage
• Constant sleeping or Insomnia

Common warning signs of suicide include:

• Giving away personal possessions

• Talking as if they’re saying goodbye or going away forever

• Taking steps to tie up loose ends, like organizing personal papers or paying off debts

• Stockpiling pills or obtaining a weapon

• Preoccupation with death

• Sudden cheerfulness or calm after a period of despondency

What to do if someone is in crisis/at risk of suicide:

If safe, keep them talking. Ask things that require more than a yes and no response like:

• Tell me what emotions you are feeling?

• What happened to make you feel this way?

• What can I do to help you right now?

• “Let’s go get something to eat or spend the day together.”

  (Diverting their attention is extremely beneficial.) Remember, asking questions without judgement is essential. Here are some things to ask:

• Ask them if they have a therapist or counselor they would like to reach out to.

• Tell them you would be happy to assist them to get to the hospital or crisis clinic and are willing to stay during the assessment process (this is a scary step for some people so having someone there with them is extremely beneficial)

• Ask them if they are thinking about completing suicide. An example of what to say could be: “Based on what you’ve shared with me, I’m wondering if you’re thinking about suicide?” If they answer yes, find out more details and ask them if they have a plan such as, “Do you have a plan for how you would do it?”

Never say things like:

• “We all go through tough times like these. You’ll be fine.”

• “It’s all in your head. Just snap out of it.”

• “You think you have problems, well look what happened to me.”

• “Get some sleep and things will look differently tomorrow.”

• “Grow up and stop feeling sorry for yourself.”

What can I do if I recognize my Family or Friends have Mental Health Challenges?

• Remember only 44% of adults with diagnosable mental health problems and less than 20% of children and adolescents receive needed treatment. Do something!!

• Friends and family can be important influences to help someone get the treatment and services they need.

• Treat people with mental health diagnoses with respect, compassion, and empathy

• Don’t define them by their diagnosis or use labels such as "crazy"

• Express your concern and support

• Remind your friend or family member that help is available and that mental health problems can be treated

• Ask questions, listen to ideas, and be responsive when the topic of mental health comes up

How to start a conversation with Adults about Mental Health

• Can you tell me more about what is happening? How you are feeling?

• Can we talk about what you are experiencing? If not, who are you comfortable talking to?

• Have you had feelings like this in the past or are these new feelings? When did you notice the change?

• I’m here to listen. How can I help you feel better?

• I’m worried about your safety. Can you tell me if you have thoughts about harming yourself or others?

How to start a conversation with children or adolescents about Mental Health

• Communicate in a straightforward manner. Don’t beat around the bush.

• Speak at a level that is appropriate to a child or adolescent’s age and developmental level. The developmental level is the most important.

• Discuss the topic when your child feels safe and comfortable

• Watch for reactions during the discussion and slow down or back up if your child becomes confused or looks upset (nonverbal cues are important)

• Listen openly and let your child tell you about his or her feelings and worries

Treatment Options

Seeking treatment through therapeutic intervention should not be an after-thought, but viewed as a holistic approach to wellness. It should not be a surprise or something you, “just get to when I have time”, especially if you are living with a chronic condition. Developing coping strategies and having a safe and non-judgmental place to share the emotions, the loss, the sorrows of managing and having a chronic condition, is extremely beneficial. It is important to remember that therapy is not a “one size fits all” but instead in this day-and-time, there are many different modalities and options for treatment that are available for you to consider. If the idea of “talk” therapy does not appeal to you, why not try art therapy or music therapy? The most important thing to remember is that seeking treatment should not be stigmatized but instead viewed as a way to live a healthier, more productive, and fulfilled life. Here are some things to consider when seeking therapy:

• Therapy is not a “one size fits all”.

• If one therapist just doesn’t feel right and you are unable to develop a trusting relationship, don’t be discouraged, try someone else.

• If Traditional “talk” Therapy is not comfortable there are plenty of therapy modalities. Try as many as necessary until you find the one that “fits” you and helps you with your concerns.

• Choosing a therapy modality can be difficult but so worth it. Finding a therapist that specializes in a particular area (i.e. play therapy, art therapy, dance therapy, telephone/ video health) can really lead to great success and make a huge difference when seeking help.

The most important thing to remember is that seeking help is beneficial. There is no shame in reaching out to a professional. Managing a chronic condition is extremely difficult but with the right coping strategies and support network, you can live, thrive, and have a fulfilled life.

References

Miravitlles, M., Pena-Longobardo, L., Oliva-Moreno, J.& Hidalgo-Vega, A. (2015). Caregivers’ burden in patients with COPD. International Journal of Chronic Obstructive Pulmonary Disease. 10. 347-356. doi: 10.2147/COPD.S76091

Pertrosky, E., Webb, R., Kontopantelis, E., Doran, T., Qin, P., Creed, F., & Kapur, N. (2014).

Suicide Risk in Primary Care Patients with Major Physical Disease. Archives of General Psychiatry 69(3). 256-264.

Schwartz, C., Powell, V., & Eldar-Lissai, A. (2017). Measuring Hemophilia Caregiver

Burden: Validation of the Hemophilia Caregiver Impact Measure. Quality of Life Research. 26. 2551-2562.

DOI: 10.1007/s11136-017-1572-y.

Witkop, M., Lambing, A., Nichols, C., Munn, J., Anderson T., & Tortella, B. (2019). Interrelationship Between Depression, Anxiety, Pain, and Treatment Adherence in Hemophilia: Results from a US
Cross-sectional Survey. Patients Preferences and Adherence. 13.1577-1587. Doi: 10.2147/PPA.S212723.

Anna Maria Bell is a graduate of The Catholic University of America Master’s Program in Social Work with a concentration in Gerontology and a graduate of Capella University with a Master’s Degree in Public Service Leadership. She is currently ABD at Walden University where she is pursuing a PhD in Human Services. She is Clinically Licensed to Practice in the District of Columbia, Maryland, Virginia and the State of Ohio. She provides counseling to various populations including couples, children, adolescents, women, and geriatric populations. Anna joined the Hemophilia Foundation of America consultancy staff in 2015 as a facilitator/speaker, conducting trainings, seminars and facilitations. Anna conducts various workshops throughout the Washington DC Metropolitan Area.

Stressing Out? How about using the 80/20 Rule?

Published: Lifelines for Health Fall 2019

by Dr. Gary McClain, PhD

It’s only human nature to have high expectations for how we want life to look like day to day and what we want to accomplish. In a word: perfection. But life has a way of getting in the way of our intentions. Nobody knows that better than someone who is living with a bleeding disorder.

A question for you: Have you taken a look at your expectations lately? Starting with what you are expecting yourself to accomplish?

My clients often talk to me about how their lives aren’t measuring up to their expectations. They feel let down by others, not getting enough of what they need. They feel that they are letting others down by not being able to give enough. Or they feel they have let themselves down by not doing enough.

Here are a couple of examples:

A client I’ll call Joe is balancing a full-time job, part-time college, and the responsibilities that go along with a bleeding disorder. To say that he pushes himself hard to be the best person he can be is an understatement. He recently told me about an experience he had that brought home to him how trying to do everything can lead to stress.

“I had a rough week at work, and I had a test to study for over the weekend. On Friday night, a friend called and said she was inviting a few friends over to help her set up her and her roommate’s new apartment. She told me how she really wanted me to be there.

“Again, I had a lot to do and I also needed to get some rest. But I said yes anyway.”

“How did that turn out for you?” I asked.

“I spent the day with her and her friends, and I was exhausted that evening. I also banged my knee helping her move a couch, which I shouldn’t have been doing, and ended up with a bleed. Along with a lot of stress. So, I did pretty badly on my test.”

A client I’ll call Connie, is a mother of a child with a bleeding disorder. She told me about her own struggles with attempting to be perfect.

“I have a full-time job. I am a single parent. On Friday night, my son had a bleed that took us to the emergency room. He was fine but it was a hard night and a late night, to say the least.

“I got a call early Saturday morning from my mom. She told me that she and one of her sisters were planning to stop by during the afternoon. She laughed and told me she had bragged to my aunt about my amazing chocolate chip cookies.”

“Nothing like additional pressure,” I said.

“Exactly,” she answered. “So, I rushed around to make sure the bathroom and the living room were spotless and got the cookies in the oven. I was so stressed. I don’t know how I managed to keep my smile on while they were here. Needless to say, I collapsed after they left.”

Are my clients’ experiences anything like yours?

Sure, it’s only human to expect to always perform at your best. To think you need to always be available to other people. As well as to have high expectations of how others should feel or act towards you. Especially people who are close to you, friends and family, or the healthcare professionals that you work with.

One of the most frustrating things about living with a bleeding disorder is adjusting to the constant challenges to maintain your own self-care or watching over (AKA enforcing) the self-care of a child with a bleeding disorder. A whole boatload of responsibilities and demands, none of which you asked for. Along with the need to rely more on others to do their part, assuming you let anyone else help out (more of that later).

Here’s what I’ve learned from my clients, as well as in my own experience with friends and family dealing with chronic conditions: Expecting 100% can be a set-up for a letdown.

Behind the science and technology of healthcare are humans. And humans aren’t perfect. Healthcare professionals aren’t always so responsive. Delays are going to happen, along with the restrictions and inconveniences of managed care.

Yes, you’re human. With good days and bad days, good intentions, and your own limitations.

Your family members are also human. And that means they aren’t always so supportive, because they aren’t able to or don’t know how to, or just won’t. Or because you won’t let them.

So, here’s an idea to consider:

Given that we are dealing with imperfect beings, what if, instead of expecting perfection, you expected imperfection? What if you started to ask yourself what parts of your life need to be functioning

at 100%, and where you might begin to loosen up on your expectations? Averaging out your expectations to, let’s say, around 80%?

Adjusting your expectations for yourself, and the people around you, to a more realistic 80 instead of 100 percent would mean allowing for the human factor in yourself and others. It might also mean a whole lot less disappointment. Not to mention a whole lot less stress.

And what if you embraced this idea so much that you instituted a new rule at your house? The 80/20 rule. With you and your family all adjusting expectations for themselves and each other accordingly.

Ready to give the 80/20 rule a chance? Here are ideas for how to loosen up your grip and giving yourself, and others, some breathing room.

Focus your energy on what needs to be in place to take the best care of yourself and your child. Taking care of your health is priority number one – medication, diet, rest, and anything else you need to do to manage your condition. As well as the health of your child with a bleeding disorder. So, if you’re looking for a starting place, this is one aspect of your life where striving for 100% makes sense. After all, your self-care is the cornerstone of your life.

Take a look at your priorities. Assuming you aren’t superwoman/man, maintaining 100% self-care and care for your children may require making some adjustments in the other areas of your life. Eighty out of 100 doesn’t have to mean an across the board cut but doing some reallocating to take the pressure off yourself. This might mean taking a look at your to-do list, the one you’ve written down or the one you keep in your head and making some decisions on what needs to be at the top and what needs to be at the bottom, what can be postponed. Think of it this way: When everything you have to do is fighting for top priority, you turn your mind into a battleground. How’s that for stress?

Say no to the go, go, go. Let’s start with your family. Sure, you want to give 100% to your family. But parents often tell me that they run themselves ragged with work around the house, trying to participate in community or school activities, being constantly available for extended family events, while also trying to have real quality time with their children.

If that’s you, then it might be time to look at where you can tighten up and where you can lighten up. For example, Joe could have just said to his friend, “Sorry, but I need to sit this one out.”

Watch out for that four-letter word: NEED. What do you “need” to commit to? Something that you really want to do or something that you feel like you should do? Do you need to go to that meeting or commit to that weekend activity? If you look at your commitments from the 80/20 perspective, you might find that good enough, and not perfect, can leave you with more balance in your life. Less stress. Here’s a way to look at what’s needed and what’s not. At the risk of repeating myself: Maintaining your optimal health and the health of your children definitely falls into the need category, and therefore front and center in that 80% of your life that gets your best effort. No, no slacking there. However...

Say yes to a little mess. Could your house be a little less spotless, with the laundry waiting an extra day, the lawn waiting a few days? 80 out of 100 might mean a less than perfect house, but also give you more time to spend enjoying your family. While taking better care of yourself by getting a little
more rest and a little less stress? And what if you asked other family members to help out, giving up some of your control and giving them a chance to give you a hand? Is there an aunt or uncle or a grandparent who would jump at the chance to come over and help out for an afternoon? For example, could Connie have asked her mom to pick up some cookies on the way to her house? Or to postpone her visit until Sunday?

Lighten up, for crying out loud. And while we’re on the topic, are you demanding 100 out of 100 from the people around you? Keep in mind that expectations for other people can lead to disappointment. Other people aren’t always going to act the way we think they should or hope they would. Everybody has days when they aren’t on their A-game, when they don’t feel good, when they aren’t so supportive. Instead of expecting the people of your life to be at 100, how about cutting them some slack, too? Given that you are dealing with human beings here, 80 out of 100 isn’t so bad. Patience helps.

And what about you? Try going a little easier on yourself. When you hear that voice of self-criticism start to zero in on what you should have don’t and didn’t, or do perfectly, remind yourself of the 80/20 rule. Give yourself credit for doing the best you can.

Speaking of should... Every time the word “should” creeps into your self-talk, ask yourself: “Who says?” Asking this question is a way to assess when the need to be perfect – and to live up to unrealistic expectations for yourself and others – is interfering with your well-being. In other words, stop “should-ing” all over yourself. (With the holidays on their way, you might want to build the “Who says?” test into your daily routine.)

Loosen up on that tight grip. Show some compassion toward yourself, and it will be a lot easier to show compassion toward others. Think 80/20! If you’re taking better care of yourself, you have that much more to give to others, and to give it joyfully and not out of exhaustion. And remember, we are all in this together.

Making a CHOICE for Inhibitor Treatment

Published: Lifelines for Health Fall 2019

With so many changes and advances in the bleeding disorders community, it can leave many of us confused
and uncertain about some of our treatment choices. You may have even asked yourself or your medical care team, "what products can I try?", or "which ones will even work for me?". Most healthcare professionals agree that inhibitor eradication/tolerization is still the preferred choice of care over bypassing agents, new or old. We've hand-picked two of our dearest friends (and their families) to share with you their experiences in inhibitor tolerization (IT.)

Taha Amir

Janet: What was your historic inhibitor B/U?

Taha: From about the age of 7 or 8 to until the age of 33 I had inhibitors. I believe my highest inhibitor BU was when I was a teen, which was about 1,000 BU.

Janet: How many times have you tried IT and with what? Units per kilo/frequency?

Taha: Ever since I was a kid, my hematologist always wanted me to try Immune Tolerance. In the early 1990’s, there was not enough data and research about IT treatments and my parents were a little scared and nervous, so they decided not to go forward.. The first time I tried IT was when I was 33 years old. My BU had been under 5.0 for a couple of years, so my hematologist thought it would be a good idea for me to try IT. However, this came with several disclaimers. The first disclaimer was that I had a very slim chance of eradicating or getting rid of the inhibitor because of how long I have had them and my age. The second disclaimer was that I had to be 100% compliant with the treatment. However, he said that if I did not try, then I would not know the outcome.

Janet: What choices for IT were presented to you by your care team?

Taha: My hematologist first suggested I try a plasma-derived FVIII with vWF complex product, because it had good success with other patients. He made me an appointment for the following month, so I could come into his office and get baseline bloodwork done.

When I had the second visit, he suggested using a recombinant FC Fusion extended half-life product. My partner and I were confused as
to why, because the previous few times, he recommended for me to be on a plasma-derived FVIII with vWD complex product.

Janet: How else did you educate yourself about inhibitor treatment choices?

Taha: Fortunately, we knew of a few people who guided us in our decision making, and we were able to talk to people who were using the product successfully.

Janet: Ultimately, why did you make your current product choice?

Taha: In the end, I chose the plasma derived, FVIII product because it seemed to work for the people I trusted, and I had to start somewhere.

Janet: How is your current regiment working for you?

Taha: So far, this is definitely my “go to” factor for IT. I started off with 100 IU per kilo, every day, for about six months. Then my hematologist lowered the dose to 75 IU per kilo, every day, for about 3 months. I was still being monitored very closely to make sure that my inhibitor did not come back. Currently I am on 25 IU per kilo, every other day and it is working great! I have been able to do more activities that I was not able to do before. If it continues

to go well, my hematologist will consider lowering the frequency to three times a week.

Janet: Would you consider participating in a clinical trial for emerging products?

Taha: I know there are many emerging trials coming out or in trials, but personally I would not consider them at this point. I am grateful to the people who do get on the trials, because without them there would not be better treatments or cures.

The Walker Family

Janet: What was your child’s historic inhibitor B/U?

The Walker's: Our son’s highest titer was 120 BU. Most of the time while on ITT his titer has been under 10 BU. However, each time he failed IT, his titer went historically higher.

Janet: How many times have you tried IT and with what? Units per kilo/frequency?

The Walker's: Our son, J, has tried IT many times with various different products. We initially started out with 100U/kg of the recombinant product he developed his inhibitor on when he was 2.5 years old. We tried a couple rounds of IT with that for 4-5 years, then moved to a plasma derived FVIII- vWF complex at 100 U/kg based on the Factor VIII. His titer was measuring 0 BU for several years on that, but during a growth spurt at age 9, his high titer came back. He began bleeding almost continually, and we took a break n IT by treating prophylactically with a bypassing agent.

After his titer slowly came back down below 10 BU close to a year later, we restarted IT with the PD FVIII- vWF complex again. It came down but spiked back up again around age 12 during puberty. After age 12, he was on a constant inhibitor titer roller coaster ride. Although we consistently infused daily, his titer was continually changing. We went through different periods of doing prophy with two bypassing agents to attempt to control bleeding while still doing IT. Based on other patients’ experiences we heard at the summits, we decided to try a combination of our previous FVIII-vWF complex and rFVIII together in the same syringe.

The reasoning behind this combination was to get more FVIII into the bloodstream than vWF, as the vWF would bind to multiple FVIII molecules and protect it. We used 200U/kg FVIII at this time. It was much less fluid volume but still did not tolerize the inhibitor. After his titer went up again and discontinuing FVIII to allow his titer to drop, we restarted IT when he was 14 with a recombinant PEGylated (extended half-life)product at 200U/kg, but his peak titer was not much higher than the rFVIII product we were using before, so we quickly switched for the final IT try to a Fc fusion extended half-life product at 200u/kg. He was using this for IT for a year and it did work in bringing his titer down.

Janet: What choices for IT were presented to you by your care team?

The Walker's: Our care team wanted us to return to his original recombinant VIII product when he was age 2.5. We were offered to be on a randomized IT study where inductees were given either daily treatment or 3 times a week treatment. Ultimately, we chose not to go on it, because we felt strongly that J should be on daily treatment.

After not making progress with that we and our care team easily concluded that we needed to switch to a plasma derived product. We let them choose for us because we did not have any strong opinions about one being better than the other after much research. When it stopped working for him, we were given more freedom to choose the next product. Multiple times we were pushed to try rituximab as an immunosuppressant however, we declined due to low long-term success rates and the high risk of side effects.

We also developed an individualized, independent care plan of how to treat bleeds with our care team, so that when J started a bleed, we knew the procedure. If after treating for the first couple of days we were not seeing improvement, we would contact them for further help.

Janet: How else did you educate yourself about inhibitor treatment choices?

The Walker's: We researched by reading the latest studies via medical journals and talked to pharmaceutical manufacturing representatives and as many medical professionals as we could. The most helpful way we educated ourselves was by going to the Inhibitor Summits and Inhibitor Family Camps, where we could talk to other families and hear what was working for them.

It's important to know that each individual reacts differently to medications, and results can vary; especially when it comes to IT since it’s an attempt to manipulate the immune system. CHES reminds you to speak with your medical care team before changing, discontinuing, or beginning a new regimen. If your doctor isn’t open to discussing or considering certain treatment options, it may be helpful to refer them to another treatment center or doctor who has experience with that specific product or treatment method. And remember, CHES makes no claims nor prescribes any medical advice, as we are strictly an education company. It is our intention to inform others of all options so they can make the best decisions for themselves.

Janet: Ultimately, why did you make your current product choice?

The Walker's: We could see that J’s quality of life was deeply affected being tied to lengthy, daily bypassing agent infusions that were not preventing bleeds and joint damage. Although his titer had historically gotten to a manageable level where we could treat with Factor VIII, his titer was never completely gone and his half-life never went over 6 hours to indicate complete tolerance. So, for us, although IT helped us manage the inhibitor for many years, it wasn’t the ultimate end to his battle with inhibitors.

We felt that trying the bi-specific, antibody product would give him the best chance to experience fewer bleeds, less joint damage throughout his life span and improve his quality of life. We were not able to access the Phase 3 trials, but switched shortly after it was approved for inhibitor patients.

Janet: How is your current regiment working for you?

The Walker's: Our current weekly injection regimen works amazingly. He has had approximately 3 bleeds in the past year and a half, where previously he had bleeding issues 2-4 times a month and now he rarely bleeds. Overall, he has spent much less time on crutches and using his wheelchair. Technically his inhibitor titer has increased in numbers now that we stopped IT, but we are better able to manage the bleeding and daily life issues. We feel he has progressed immensely in regards to his quality of life and ability to be an independent, fully functioning, young man.

Janet: Would you consider participating in a clinical trial for emerging products?

The Walker's: Yes, we would consider it and make the best choice for our individual situation based on the current information available.

It's important to know that each individual reacts differently to medications, and results can vary; especially when it comes to IT since it’s an attempt to manipulate the immune system. CHES reminds you to speak with your medical care team before changing, discontinuing, or beginning a new regimen. If your doctor isn’t open to discussing or considering certain treatment options, it may be helpful to refer them to another treatment center or doctor who has experience with that specific product or treatment method. And remember, CHES makes no claims nor prescribes any medical advice, as we are strictly an education company. It is our intention to inform others of all options so they can make the best decisions for themselves.

American with Disabilities Act (ADA) & Section 504 Overview & Reasonable Accommodations

by Janet Brewer, M.Ed & Lisa Cosseboom, M.Ed., CAGS

Published: Lifelines for Health Fall 2019

Section 504 of the Rehabilitation Act of 1973: The 504 section of this law was enacted to provide people with disabilities protection against discrimination by any program, activities or schools (as well as colleges and universities) that receive federal funding. The Office of Civil Rights (OCR) are responsible for enforcing institutions to provide reasonable accommodations for “qualified individuals.”

American with Disabilities Act (1990): This amendment in 1990 widened the breadth of coverage for people with disabilities. The act notes that its purpose is “to provide a clear and comprehensive national mandate for the elimination of discrimination against individuals with disabilities.” Part of this act prohibits a “public entity” such as a public school and non-parochial private schools from discriminating against students with a disability.

What is considered discriminatory?

• Denying a qualified student with a disability the opportunity to participate in or benefit from the aids, benefits, or services that are afforded other students.

• Affording a qualified student with a disability an opportunity to participate in or benefit from the aids, benefits, or services that are not equal to that afforded other students.

• Providing aids, benefits, or services to a qualified student with a disability that are not as effective as those provided other students.

• Providing different or separate aids, benefits, or services to a qualified student with a disability unless necessary to provide aids, benefits, or services that are as effective as those provided others.

• Aiding or perpetuating discrimination by providing significant assistance to an agency, organization, or person that discriminates on the basis of a disability.

• Denying qualified persons with disabilities the opportunity to participate as a member of a planning or advisory board because of their disability.

• Limiting a qualified student with a disability from the enjoyment of any right, privilege, advantage, or opportunity enjoyed by other students.

How is a disability determined?

The law states that a person must:

• Have a physical or mental impairment which substantially limits one or more major life activities (those basic activities that the average person in the general population can perform with little or no difficulty)

• Has a record of such impairment

• Is regarded as having such an impairment

The term “substantially limits” one major life activity can be episodic or in remission and still be considered a disability and have accommodations in place for when it is active; and the determination must be made without regard to the ameliorative effects of mitigating measures.

What does this mean?

The 504 Team must identify mitigating measures being used by a student and determine how the disability would impact the major life activity in the absence of the mitigating measure. For example, a student with an inhibitor who happens to not be actively bleeding, or is currently managing any bleeding disorder with infusions (any type of medication is a mitigating factor), the

Team must determine whether the impairment would substantially limit a major life activity if removed. Each mitigating measure must be identified and used to determine what the impairment would look like if the mitigating measure was removed. Additionally, the Office of Civil Rights has determined that health plans and emergency plans are considered mitigating measures.

Examples of mitigating measures:

• Medication, medical supplies, equipment, or appliances; low-vision devices (which do not include ordinary eyeglasses or contact lenses); prosthetics, including limbs and devices; hearing aids and cochlear implants or other implantable hearing devices; oxygen therapy equipment and supplies; use of assistive technology;

• Reasonable accommodations or auxiliary aids or services; and

• Learned behavioral or adaptive neurological modifications.

The purpose of a 504 plan is to provide access or remove barriers to participation. It provides students the same rights and services as their non-disabled peers. For students with bleeding disorders, a major life activity could include walking, writing, sitting or standing. Under a 504 plan a student can access related services as determined by the Team. These include the following:

Speech & Language Therapy:

• Occupational Therapy

• Physical Therapy

• Counseling

• Rehabilitative Counseling

• School health services

• Transportation

Reasonable Accommodations under a 504 plan:

• Schedule of Physical Education activities

• Extra set of books at home

• Extended travel time around building

• Extended time for homework/make-up work

• All medically related absences are excused with no loss of credit

• Field trips to include a nurse

• Permanent pass to the school nurse

• Medications, needles and supplies maintained in the nurse’s office

• Staff in-service

• Tutoring due to absences

• Physical education credits for participating in outside physical therapy

• Audio or video of class lectures

• Regular email contact from teachers regarding missed assignments

• Note takers/Copies of teacher notes

• Homework posted on the school district internet (Class Dojo/Google Classroom)

• Permanent pass for school elevator

• Walkie-Talkies for recess

• Medically-related absences excused with no loss of credit

• Individualized Health Care Plan

What about after-school programming?

• Medications, needles and supplies maintained in the nurse’s office

• Staff in-service

• Tutoring due to absences

• Physical education credits for participating in outside physical therapy

• Audio or video of class lectures

• Regular email contact from teachers regarding missed assignments

• Note takers/Copies of teacher notes

• Homework posted on the school district internet (Class Dojo/Google Classroom)

• Permanent pass for school elevator

• Walkie-Talkies for recess

• Medically-related absences excused with no loss of credit

• Individualized Health Care Plan

Any program that is operated or funded by a school or contracted by a school is required for ensuring reasonable accommodations for children on a 504 or Individualized Health Plan. The school district, family and after-school program should work to ensure consistency between the school plan and what accommodations the student needs in the after- school setting.

Parents should plan on advocating for their child in the school system as bleeding disorders are rare and school staff may be unfamiliar with their needs or what accommodations will be needed. After reasonable requests for 504 eligibility, if a family has a complaint contact the Office for Civil Rights at:

https://www2.ed.gov/about/offices/list/ocr/qa-complaints.html

A Journey of Faith

Published: Lifelines for Health Spring 2019

In November 2018, I had the opportunity to attend the ‘Mom’s Uninhibited Meeting (MUM) offered by CHES. It was by chance that I saw the social media post and figured I wouldn’t be able to attend with everything going on in my life, but thankfully, I did! I had no idea what to expect. My weekend started as I boarded the plane to Nashville. It was difficult being away from my family for the first time since my son had been born. Although, after the evening session that day, I immediately felt that I had made the right decision. The first night was special because it set the stage for the rest of the weekend. Even though I had never met any of these lovely women, it was easy to talk with them and share experiences since we all knew we had at least one thing in common. That night, I felt emotionally drained because the words that were shared struck a chord with me. The weight of my feelings was put into words that I could not or dared not articulate, or even think about, but benefited deeply from hearing them spoken.

My son was diagnosed with severe Hemophilia A in the early summer of 2018. About a month after that diagnosis, we found out he had developed inhibitors. To say we were overwhelmed is an understatement. The information that we had to take in seemed impossible to digest. Right when we thought we had a handle on it, something new would come up. Having the opportunity to learn and take things in that weekend opened my eyes to how much I still need to learn but also, more importantly, that my family has a choice when it comes to my son’s treatment and we can and should ask questions.

The educational sessions provided were eye opening. Since every mom had a different story and had been on their journey for different amounts of time, it was even more helpful. Each had different questions and stories that sparked questions for me and helped me learn even more than what was taught during the sessions. These things all changed my perspective on my son’s treatment. It has enabled me
to have a stronger voice when speaking with his doctors and it encouraged me to continue to champion for his best interest. Thanks to the help of Kathy Byrne, I was even able to stick my own vein! Having never been a fan of needles, it really was a milestone for me, and it encouraged me that someday I can learn to do the same for my son.

I think that every mom with a child who has an inhibitor should attend the MUM program. For the moms that are just beginning on this journey like I was, it was a great opportunity to learn, connect, and share. For moms that are further along in their journey, it was a great opportunity to guide and support those of us who are just getting started. I can’t thank Janet, and the CHES team enough for offering such a wonderful weekend. I am very grateful for the opportunity that I had to experience this MUM weekend.

Amy and her husband, Oscar have been married for 4 years and live in Orange County, CA with their 2-year-old son Hudson. She works at the Disneyland Resort as a Food and Beverage manager.

Momentum, Men's Inhibitor Retreat: Brotherhood

Published: Lifelines for Health Spring 2019

Between managing my own health (including a daily ITT regimen), partially running a business, raising two small children who have recently been dually-diagnosed with bleeding disorders, cheer practice, dance practice, and being married to a spouse who also works, “overwhelmed” has become a word of persistency in my life.

Momentum (CHES’ men’s inhibitor retreat) was created to improve the quality of life for adults living with active inhibitors. As one of the creators of Momentum, I can humbly say that I benefit just as much as the average Joe. Or in this case, the average Bro. That’s what these meetings become to most of us. It’s not just a place of resources to better our lives, it’s also a brotherhood.

As Janet and I were prepping for Momentum last summer (along with the rest of the CHES team), she asked me if I was ready for this. “This” meaning acting as the lead facilitator of the program, as she (who usually takes on this responsibility for most CHES programs) would not be attending. After 10 years of doing this, I still feel the nerves of most people’s biggest fear - public speaking. But to Janet’s question, I replied, “absolutely, these are my people.” When I speak to this group, I’m not hosting guests or friends. I’m strengthening the bonds of my brothers as well as my own.

Some of these guys I’ve known for years, and some were new acquaintances for me, but we all have opened arms to each other. There are no judgements, no cliches’, no negativity, hatred, or disrespect - only brotherly love and acceptance. We feel we understand each other better than anyone else can. And although we may not see or speak with each other more than a few times a year, the connection picks right back up where it left off.

Serving my overwhelmed state was the opener to the weekend, which was both happily welcomed and received as Emily Taylor sat us all down in a circle for some mindfulness techniques and theory of pain exercises.

Although I don’t recall the specific methods of the session, I do remember leaving in a calm, relaxed state.

As someone who has just surpassed their 11th anniversary of ITT, vein preservation is a big concern. Like almost everyone else in this group, I can jokingly say I could be a phlebotomist tomorrow if I wanted to. But CHES’ acclaimed Kathy Byrne, who has decades of infusion experience under her belt, had tips that even I had not known of. Just changing to a 27 gauge needle has really proven to provide more mileage out of my veins. And marking a hard-to- find vein with a fingernail impression has bumped my “sticking average” up to nearly 100%. But I’m not going to reveal all of her secrets here, as I hope to see each and every (qualifying) person who reads this article at a CHES program.

Dr. Jonathan Bernstein of Connecticut Children’s Medical Center in Hartford gave us the updates on the latest and greatest products for inhibitor folks, as well as some that we either overlooked or had forgotten. After all, some products may not be new and shiny, but that doesn’t mean doctors and researchers aren’t reinventing treatment methods around them. Personally, the variety of products and the multitude of concerns and unanswered questions surrounding them are yet another overwhelming factor in my life, so I have chosen the wait-and-see method while maintaining ITT.

To close the programs off, we hopped in the pool as Chad Brown, ex pro-am wake boarder with hemophilia A and professional coach as president/CEO of the Wingmen Foundation, gave us some lessons on low-impact, water aerobics in a playful yet beneficial manner.

I look forward to reconnecting with all of our community friends and family each year at our programs, but Momentum is really something extra special to me. As I said, “my people”.