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

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

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

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

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

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

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

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

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

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

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

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

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

LEVERAGE: a Molehill Practice for a Mountainous Adventure

By: Justin Levesque

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

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

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

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

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

Taha’s Moment: Finding “Normal”

By Taha Amir

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

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

What Inhibitor Family Camp Means to the Family

By  Cheryl Cook

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

All I can say is, “thank you!”

Pain and the Power to Heal - Winter 2017 Feature

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

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

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

Pain in the Bleeding Disorders Community

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

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

Definition of Pain

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

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

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

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

Pain Management Requires a Variety of Approaches

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

Managing Physical Aspects of Pain

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

Cognition and Pain

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

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

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

Emotional Well-being

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

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

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

Pain and Relationships

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

The Power to Heal

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

Assistive Devices

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

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

  http://www.healingbuddiescomfort.org/

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

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

Distraction-Passive and Active

• Blow bubbles

• Listen to or create music

• Play games

• Read/Listen to Stories

• Watch movies

• Utilize social media

Emotional Strategies

• Calming Techniques

• Change your thoughts-
  positive self-talk and reinforcement

• Coping

• Counseling

• Grief and loss acceptance

• Humor

• Pain Education

• Set realistic goals/expectation

Movement

• Balance, coordination, ball therapy

• Change position

• Dance

• Exercise/Gentle stretching

• Mini Breaks

• Tai Chi

• Walk/pace/rocking

• Yoga

Mindfulness

• Attention Management

• Breathing

• Guided imagery

• Meditation

• Progress muscle relaxation/body scan

• Self-hypnosis

• Set realistic expectations of self/others

Physical Comfort Measures

• Acupuncture

• Acupressure

• Beverages

• Biofeedback

• Contrast baths-Epsom salts

• Cuddle

• Dim lighting, noise reduction

• Essential oils, salves, rubs/ aromatherapy

• Food

• Hot/Cold packs/pads

• Loose, soft clothing

• Massage

• RICE

• Sleep

• TENS (Transcutaneous electrical nerve stimulation)

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

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

Managing Inhibitors in Hemophilia Treatment: The Importance of Bypassing Agents

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

By: Mike Denne

Published: Lifelines for Health, Winter 2017

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

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

Bypassing Agents supplement other Factors in the blood-clotting process

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

REFERENCES:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

New Sub-Q Treatment is FDA-Approved for Inhibitors

by Eric Lowe

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

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

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

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

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

Results

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

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

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

Looking Ahead

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

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

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

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

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

When Someone You Love Hurts How Can You HELP?

by Dr. Gary McClain, PhD

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

Here are some ideas to consider:

Get Informed Together

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

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

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

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

Encourage Adherence

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

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

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

When You Don’t Know What To Do...

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

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

Get specific

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

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

Watch the Silver Lining Talk

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

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

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

Give Your Loved One Their Space

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

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

Take Care of Yourself

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

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

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

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

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

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

by Ann Marie M. Minichiello

Published: Lifelines for Health, Winter 2017

The Individualized Healthcare Plan

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

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

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

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

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

Emergency Plan:

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

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

The Basics of a Well-Developed IHP:

• should always include up to date emergency contact information

• should include a review date

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

• should include six components

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

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

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

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

  5. Implementation: put the plan in practice

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

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

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

References and additional resources:

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

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

A Review of Inhibitor Family Camp at Victory Junction

Making Life-Long-Distance Friendships

By: Ashley Davis

Published: Lifelines for Health Spring 2017

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

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

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

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

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

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

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