Felt Angry Lately?

by Dr. Gary McClain, PhD

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

Coping With Angry Feelings at Home

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

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

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

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

Really Mad? Talk Yourself Down!

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

Count to ten. 

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

Step out of the story. 

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

Reconsider your expectations. 

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

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

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

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

Look for the humor. 

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

Have a heart. 

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

Reach out for support. 

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

Take care of yourself. 

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

Use Anger Productively in Family Communications

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

Express your feelings with the “I word.” 

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

Focus on the issue. 

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

Let your family member know what you need. 

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

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

And Help Family Members Express Their Anger

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

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

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

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

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

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

Remember:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

LEVERAGE: a Molehill Practice for a Mountainous Adventure

By: Justin Levesque

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

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

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

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

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

Taha’s Moment: Finding “Normal”

By Taha Amir

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

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

What Inhibitor Family Camp Means to the Family

By  Cheryl Cook

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

All I can say is, “thank you!”

Pain and the Power to Heal - Winter 2017 Feature

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

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

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

Pain in the Bleeding Disorders Community

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

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

Definition of Pain

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

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

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

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

Pain Management Requires a Variety of Approaches

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

Managing Physical Aspects of Pain

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

Cognition and Pain

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

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

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

Emotional Well-being

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

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

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

Pain and Relationships

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

The Power to Heal

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

Assistive Devices

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

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

  http://www.healingbuddiescomfort.org/

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

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

Distraction-Passive and Active

• Blow bubbles

• Listen to or create music

• Play games

• Read/Listen to Stories

• Watch movies

• Utilize social media

Emotional Strategies

• Calming Techniques

• Change your thoughts-
  positive self-talk and reinforcement

• Coping

• Counseling

• Grief and loss acceptance

• Humor

• Pain Education

• Set realistic goals/expectation

Movement

• Balance, coordination, ball therapy

• Change position

• Dance

• Exercise/Gentle stretching

• Mini Breaks

• Tai Chi

• Walk/pace/rocking

• Yoga

Mindfulness

• Attention Management

• Breathing

• Guided imagery

• Meditation

• Progress muscle relaxation/body scan

• Self-hypnosis

• Set realistic expectations of self/others

Physical Comfort Measures

• Acupuncture

• Acupressure

• Beverages

• Biofeedback

• Contrast baths-Epsom salts

• Cuddle

• Dim lighting, noise reduction

• Essential oils, salves, rubs/ aromatherapy

• Food

• Hot/Cold packs/pads

• Loose, soft clothing

• Massage

• RICE

• Sleep

• TENS (Transcutaneous electrical nerve stimulation)

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

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

Managing Inhibitors in Hemophilia Treatment: The Importance of Bypassing Agents

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

By: Mike Denne

Published: Lifelines for Health, Winter 2017

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

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

Bypassing Agents supplement other Factors in the blood-clotting process

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

REFERENCES:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

New Sub-Q Treatment is FDA-Approved for Inhibitors

by Eric Lowe

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

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

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

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

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

Results

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

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

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

Looking Ahead

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

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

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

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

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

When Someone You Love Hurts How Can You HELP?

by Dr. Gary McClain, PhD

Published: Lifelines for Health, Winter 2017

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

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

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

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

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

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

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

Here are some ideas to consider:

Get Informed Together

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

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

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

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

Encourage Adherence

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

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

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

When You Don’t Know What To Do...

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

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

Get specific

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

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

Watch the Silver Lining Talk

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

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

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

Give Your Loved One Their Space

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

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

Take Care of Yourself

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

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

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

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

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

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

by Ann Marie M. Minichiello

Published: Lifelines for Health, Winter 2017

The Individualized Healthcare Plan

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

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

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

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

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

Emergency Plan:

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

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

The Basics of a Well-Developed IHP:

• should always include up to date emergency contact information

• should include a review date

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

• should include six components

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

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

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

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

  5. Implementation: put the plan in practice

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

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

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

References and additional resources:

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

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

A Review of Inhibitor Family Camp at Victory Junction

Making Life-Long-Distance Friendships

By: Ashley Davis

Published: Lifelines for Health Spring 2017

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

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

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

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

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

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

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

The Timeline of Education: A New and Uncertain Turn?

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

Published: Lifelines for Health Spring 2017

Important Events in Public Education:

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

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

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

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

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

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

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

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

Recent Education Bills Passed:

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

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

New Bills introduced:

What is H.R. 610?

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

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

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

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

Thinking behind H.R. 610:

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

Nuts & Bolts of Choices in Education Act:

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

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

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

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

Effect of H.R. 610 Special Needs Students:

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

Summary:

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

Clinical Trials: Optimistic Caution

Published: Lifelines for Health Spring 2017

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

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

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

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

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

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

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

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

Clinical trial thoughts to examine:

1. What phase trial am I participating in?

2. What is the inclusive/exclusion criteria?

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

4. Carefully read the Informed Consent Form that contains:

   1. Purpose

   2. Details

   3. Duration

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

   5. Key contacts

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

6. What are the risk/benefits?

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

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

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

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

    1. Quality of Life?

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

11. What are your feelings on taking risks?

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

    13. Read.

    14. Talk to friends, family, clergy.

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

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

    17. Trust your gut.

    18. Read some more.

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

Engaging Uncertainty

Published: Lifelines for Health Spring 2017

by Krystyn Strother

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

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

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

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

Mindfulness

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

Be Confident

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

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

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

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

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

Read more about Krystyn at krystynstrother.com

"HOME" Coming

by: Crystal James

Published: Lifelines for Health Fall 2018

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

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

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

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

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

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

Leverage 2018 Walking the Wire

by Gabriel Anspaugh

Published: Lifelines for Health Fall 2018

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

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

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

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

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

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

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

FEATURE: Understanding Complex PTSD Trauma

Published: Lifelines for Health Fall 2018

By: Charlie P. Gilbert II, LCSW

“The” Inhibitor Traumas

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

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

The Brain’s Response

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

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

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

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

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

Overcoming the Trauma

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Anxious? How About Running Toward Your Anxiety?

by Dr. Gary McClain, PhD

Published: Lifelines for Health Fall 2018

Living with a chronic condition gives you all kinds of reasons to feel anxious. Do I need to go through a list with you? Probably not. Humans don’t do well with uncertainty. And chronic conditions can bring all kinds of uncertainty to your life. Having to face a test or a procedure. Adjusting to changes in your daily life. Thinking about the future. And stress. All of this can lead to anxious feelings. So, what do you do about those anxious feelings? Suffer until they hopefully go away on their own? Try a technique and hope it works? You’ve got more options than that. Coping with anxiety begins with how you think about anxious feelings as well as preparing yourself for the anxiety that might come your way.

Here’s how to get started: Accept that anxiety is part of life

When I was in training to be a therapist, one of my teachers said this at the beginning of a lecture on working with anxious clients: “You’re anxious. So what!” Look around you at the world news, your community, your family and friends. You could say that any given day brings lots of reasons for anyone to feel anxious. That doesn’t mean you have no choice but to be continuously overwhelmed with anxiety. Not at all. The point is that unless you’re comatose, you’re probably going to experience some anxious feeling from time to time, or often. It’s normal, and you’re not alone.

Lean in to your anxiety

Here’s a mindfulness exercise for you. Visualize yourself hiding in your house with all the doors and windows locked and anxiety standing outside on the porch, pounding on the door to get in. And then, imagine opening the door and saying: “Come on in, anxiety. Let’s sit down and talk.” If the anxiety is not going away on its own, you might as well see if the two of you can come to an understanding.

Look for the lesson

Is there something you can learn from those anxious feelings? In other words, could some of that anxiety be a message from your mind that it’s time to take better care of yourself or to make a change for the better in your life? Successful people often talk about how they use their anxiety to motivate themselves to work a little harder to be excellent. Clients living with chronic conditions often tell me that feeling a little bit anxious helps motivate them to stay compliant with their treatment and self-care routine. This isn’t to say that’s all that anxiety is good for you. But if there’s lesson there, then learning it might help you to reduce some of that anxiety.

Don’t get anxious about getting anxious

Nobody likes being anxious. But I often find with my clients is that they dislike anxiety so much and are so afraid of being overwhelmed by their anxious feelings, that when they feel anxiety coming on, or worry that they might get anxious later, they get anxious about the anxiety. They do this by worrying about how bad their anxiety might be. They create anxious stories about what might happen.

They get anxious about how they’re going to cope with the anxiety. And guess what? They end up creating more anxiety for themselves. Watch out for negative self-talk that helps to create more anxiety.

Talk to yourself

When anxious thoughts and feelings start building up in your mind, talk yourself off the ceiling. Remind yourself that it’s normal to be anxious sometimes. Ask yourself what you can do for yourself to reduce your anxious feelings. When your mind tosses those scary stories and images at you, talk back to them: “Bad things can happen but so can good things. I can’t predict the future.” When you stop fighting anxiety, you free yourself up to focus your energy on what you can do about it.

Calm yourself down

Think about what helps you when you’re feeling anxious. Imagining a relaxing place like the beach? Taking a walk? Listening to music? Reading a book? It might help to put a tool kit together with the calming techniques that work for you, and have them ready for when you feel anxious.

Be proactive

Take a look at recent days when you have felt less anxious to find clues for how to avoid anxiety. Was there something you did or didn’t do that day that might have helped you to have a more relaxed outlook? Were you compliant with medication? Following your diet? Staying active? In touch with supportive people? One of the best ways to cope with anxiety is to not set yourself up to feel that way by building anxiety prevention into each and every day. Again, apply the lessons that anxiety can teach you. As they say, an ounce of prevention is worth a pound of cure.

Talk to somebody

Get some perspective. Sit down with someone who can listen without judging you or telling you what to do. Talk about what’s making you feel anxious. Sometimes just saying it out loud can help you to see what’s real and what’s being manufactured by your imagination. Ask for some encouragement. And maybe some accountability if you want to make some changes for the better.

And reach out for help if you need it

If you are feeling overwhelmed by anxiety, if it is interfering with your ability to do what you need to do
to take care of yourself, if it is getting in the way of your relationships, or if it is making it hard for you to make decisions, then it may be time to reach out to a mental health professional. Talk to your doctor if you aren’t sure where to start. Don’t go through this alone.

Anxious feelings are part of being human. Lean in to your anxiety. Listen for the lesson anxiety can teach you. And take good care of yourself.

Keep Calm

Withdraw if you need to. Move yourself out of the center of the action. Physically, mentally, or both. Step back. You can even say something like, “I need a moment.”

Use your breathing. That initial emotional rush can be overwhelming. Notice how you’re breathing. Little short breaths? Not breathing at all? Take a series of deep, calming breaths. In through your nose, out through your mouth.

Do things that calm you down. Listen to music. Take a walk. Read. Do some relaxation exercises. Anything that helps you to stay calm, that helps you feel connected to your center. Calming yourself down is essential if you are experiencing symptoms of PTSD.

Don’t fight your feelings. Okay, so you’re having a lot of feelings. The worst way to cope with emotions is by pushing them down or trying to pretend they aren’t there. Your feelings are your feelings. Good feelings, feelings you aren’t so proud of. Give yourself permission to feel them all.

Remember that feelings may not represent reality. The feelings of the moment can make the world around you look pretty foggy. You may see things that aren’t real, like what someone else’s motives look like, or where a situation seems to be going. All the more reason to take a pause until the fog dissipates.

Try to be patient with yourself. Emotions are part of being human. Our way of coping with strong emotions is hard wired into us, based on years of practice. Some of us shut down, some of us wear our emotions on our sleeve. Learning how to cope with emotions in a healthier manner takes practice. And keep in mind, if you’re living with a chronic condition, you have that much more on your plate. So be patient with yourself.

Most of All, Stay Supported...

If you’re living with a bleeding disorder and an inhibitor, you already know the value of staying connected to your support network. If you are experiencing strong emotions, and especially if you suspect you are experiencing PTSD, it is important to get emotional support, professional support, family support, friends, and other members of the inhibitor community. The same if you are a caregiver. If you are a family member of someone you suspect may be experiencing PTSD, it’s important to get support for both your loved one and for yourself. Don’t go through this alone!

You, your chronic condition, and your mental health. You’re dealing with a lot. And one stressful situation followed by another can take a big toll on your mental health. If it’s all starting to wear you down, do the bravest thing in the world and ask for HELP. PTSD, like other mental health conditions, is treatable.

Shining Light on School System Disagreements

by Lisa Cosseboom, M.Ed. & CAGS School Psychologist & Special Education Evaluation Team Chairperson

Published: Lifelines for Health Fall 2018

For this article, we asked our inhibitor families to share stories of challenges they faced with their local school systems. We thank you for sharing! We applaud your tireless efforts to access equals rights to a free appropriate public education for your child. It can be a frustrating landscape to navigate! Our school psychologist, Lisa Cosseboom shares some solutions for challenging situations.

1. My child is in a wheelchair temporarily. Can the school suggest he or she stay home until the wheelchair is no longer needed? NO! You cannot exclude a child from school based upon a temporary disability or otherwise. If it were a permanent condition, the school district must provide wheelchair accessibility including transportation, handicap bathroom access, elevator, ramps etc. Additionally, the school must provide alternative/modified physical education during which the child is unable to participate fully.

2. As part of an agreement with our public school, my child attends a private school at their expense as it met his needs better than the public system. We have been trying to get a 504 plan for him before he enters high school, but the school refuses to write one.

   Unfortunately, unless that private school is receiving federal funding in any capacity (i.e. Title 1 funding) they are not required to follow the Americans with Disabilities Act regulations. If he were entering a public high school, the school district would be required to hold a 504 eligibility meeting.

3. My child is in a private school. What accommodation or services can be provided for my child there?
   Unless receiving any type of federal funding, the school is not required to provide accommodations and/or services. However, you can certainly request a meeting with administration to see if they would be willing to entertain providing accommodations as needed. Sometimes, approaching a school with a positive attitude and reasonable request will work. (I.e. you get more with sugar than spice.)

4. My child sees the adjustment counselor at our school for anxiety issues related to his bleeding disorder. Can the school system charge me personally for those services?

   Absolutely NOT! Services provided in the public school system, by a public school employee are covered by your taxes! If your child is seeing someone outside of the public schools for counseling in school by an agency, then your insurance would cover the cost.

5. The ADA coordinator at our elementary school invalidated my child’s 504, indicating that absences and injuries would be evaluated on a case by case basis to prevent abuse of services.
   This is a clear civil rights violation. Public schools are required to provide appropriate accommodations to a qualified student who has a disability so that child can participate and access their education. Any child who has a disability that “substantially limits one or more major life activities” which includes education. They cannot “invalidate” a 504 plan because of injury or absences. I would suggest you meet with the principal or higher administrator in the district. If possible, I would suggest you acquire an advocate.

6. The school my daughter attends, put a 504 on hold for my daughter because she doesn’t go to the nurse when she has a bleed and can manage on her own. How can I get the school to put a 504 in place?
   Regardless of your daughter’s independence in caring for her bleeding disorder, she should have a plan in place. A meeting should be held for qualification and you will need to provide medical documentation. There could be times that your daughter may need to access the nurse without question from teachers or scrutiny.

The Future's So... BRIGHT

by Janet Brewer, M.Ed

Published: Lifelines for Health Fall 2018

The bleeding disorder community is currently experiencing one of the most rapid growth periods since the 1990’s when recombinant products hit the market. Corporate buyouts bring changing companies, some of whom are completely new players to the bleeding disorder community. New products that provide the convenience of subcutaneous administration are here, with more coming, gene therapy for FIX may be just around the corner, with FVIII not far behind. Women’s bleeding symptoms are finally being recognized. It is truly a dizzying pace and we do this for a living! With each of these advances however, the benefit-risk profile for any and all new products and treatments must be first and foremost in our minds.

So, what is going on?

Gene Therapy

A cure in our lifetime was the refrain when my brother was born in 1970. Multiple attempts at gene therapy have occurred in the last 4 decades, but it is clearly looking to become a treatment option in the next five years, especially for Hemophilia B. Companies such as Spark and BioMarin are on the leading edge.

No one is saying yet that hemophilia will be cured. Currently, gene therapy — which uses a virus to deliver a new gene to cells — can only be used once. If it stops working, the patients lose the benefits. For now, “we are anticipating that this is a once-in-a-lifetime treatment,” said Dr. Steven Pipe, director of the hemophilia and coagulation disorders program at the University of Michigan and a lead investigator of a clinical trial conducted by the biotech company BioMarin (https://www.nytimes. com/2018/08/13/health/hemophilia-gene- therapy.html)

Many questions exist however, regarding its long term-safety and efficacy. The FDA has
a draft document that’s currently open for comments, entitled Human Gene Therapy for Hemophilia at:

https://www.fda.gov/downloads/ BiologicsBloodVaccines/ GuidanceComplianceRegulatoryInformation/ Guidances/CellularandGeneTherapy/

The document covers considerations for product development, consideration for FVIII/FIX activity measurement assessed by different clinical laboratory assays, expedited programs, communication with the FDA, considerations for preclinical studies, and considerations for clinical trials to include:

• Efficacy Endpoints

• Study Population

• Study Design

• Study Monitoring

• Statistical Considerations

• Patient Experience

Increased patient monitoring efforts that are well- established and enforced with clear cut guidelines for what constitutes an adverse event with proper reporting procedures, should be part of the approval process. Our community has a well-documented history of what can go wrong when products that are considered “life changing” are introduced into our treatment regiments.

Hemlibra

For many in the inhibitor community, Hemlibra has proven to be a game-changer in care. There is no doubt it has decreased bleeding and consequently increased quality of life for inhibitor patients where nothing else has worked. The convenience of subcutaneous treatment after years of port therapy and complications has led to unprecedented recommendations for its use.

Genentech announced the release of Hemlibra to hemophilia A patients without an inhibitor on October 4, 2018. This comes less than a year after its approval on November 18, 2017 for the treatment of hemophilia A patients with an inhibitor.

During Dr. Stacy Croteau (Boston Children’s Hospital) and Dr. Lindsey George’s presentation, Update on New Therapies and Gene Therapy Trials in Hemophilia for physicians at NHF, October 18th-21st, 2018, they explained, emicizumab/Hemlibra is a hot topic. The HAVEN 3 noninhibitor patient study also shows that “emi” (emicizumab) is better than treating on demand. However, there were adverse events occurring in at least 5 percent of participants including injection site reaction, headache and diarrhea. Dr. Croteau also concludes that there is “significant laboratory monitoring challenges and (a) lack of understanding of effective hemostasis provided by emi alone and that more information is needed concerning those who have had major trauma or surgery.”

Dr. Glen Pierce concludes that, “questions remain; while it mimics FVIII in bringing FIX and X together, it doesn’t simulate other functions of FVIII.”

The American Thrombosis and Hemostasis Network (ATHN) announced the launch of ATHN7: Hemophilia Natural History Study Overview at NHF this October. The study’s intent is to follow a group of people over time to collect health information from patients to understand how bleeding and clotting disorders like hemophilia develop and to design effective treatments. According to Dr. Tyler Buckner, one of the lead researchers at Hemophilia and Thrombosis Center, University of Colorado, “ATHN 7 is looking to find out whether non-factor replacement products are safe when used as a preventative measure for patients who have inhibitors as well as for those who don’t. We’re also going to be evaluating the effectiveness of non-factor products*, bypassing agents, or coagulation factor replacement products when used for prevention and for surgery in patients with hemophilia.” - NHF Daily

Want to participate? Hemophilia A or B patients of any severity with or without an inhibitor are eligible through their hemophilia treatment center over a multiple year period. *For the purposes of this study, “non-factor products are those that became commercially available in the US after January 1, 2017.”

My Life Our Future

Genentech announced the release of Hemlibra to hemophilia A patients without an inhibitor on October 4, 2018. This comes less than a year after its approval on November 18, 2017 for the treatment of hemophilia A patients with an inhibitor.

During Dr. Stacy Croteau (Boston Children’s Hospital) and Dr. Lindsey George’s presentation, Update on New Therapies and Gene Therapy Trials in Hemophilia for physicians at NHF, October 18th-21st, 2018, they explained, emicizumab/Hemlibra is a hot topic. The HAVEN 3 noninhibitor patient study also shows that “emi” (emicizumab) is better than treating on demand. However, there were adverse events occurring in at least 5 percent of participants including injection site reaction, headache and diarrhea. Dr. Croteau also concludes that there is “significant laboratory monitoring challenges and (a) lack of understanding of effective hemostasis provided by emi alone and that more information is needed concerning those who have had major trauma or surgery.”

Dr. Glen Pierce concludes that, “questions remain; while it mimics FVIII in bringing FIX and X together, it doesn’t simulate other functions of FVIII.”

The American Thrombosis and Hemostasis Network (ATHN) announced the launch of ATHN7: Hemophilia Natural History Study Overview at NHF this October. The study’s intent is to follow a group of people over time to collect health information from patients to understand how bleeding and clotting disorders like hemophilia develop and to design effective treatments. According to Dr. Tyler Buckner, one of the lead researchers at Hemophilia and Thrombosis Center, University of Colorado, “ATHN 7 is looking to find out whether non-factor replacement products are safe when used as a preventative measure for patients who have inhibitors as well as for those who don’t. We’re also going to be evaluating the effectiveness of non-factor products*, bypassing agents, or coagulation factor replacement products when used

for prevention and for surgery in patients with hemophilia.” - NHF Daily

Want to participate? Hemophilia A or B patients of any severity with or without an inhibitor are eligible through their hemophilia treatment center over a multiple year period. *For the purposes of this study, “non-factor products are those that became commercially available in the US after January 1, 2017.”

Until the end of 2017, My Life Our Future (MLOF), originally supported by ATHN, Biogen, Bloodworks Northwest and NHF gathered genotypic data from 9,000+ hemophilia A and B patients and carriers. Your support of this invaluable data collection contributed to the MLOF Research Repository, the largest hemophilia scientific resource of its kind in the world. Over 4,000 genetic variations in hemophilia were discovered as a result of this research, which will hopefully answer questions such as, which variants increase the risk of inhibitor formation, or why bleeding severity varies from person to person? Some of the research projects that will be explored using this repository include: Inhibitor Development, Bleeding Specific to Hemophilia A and Hemophilia B, FVIII clearance, and Carriers.

Doctors Hong Yang and Zuben Suana of the FDA’s Center for Biologics Evaluation and Research (CBER) will utilize MLOF data to create a mathematical model to predict the body’s immune response to treatment. Once validated, it could be used to evaluate new FVIII drugs, project the risk of the development of antibodies, and help determine appropriate treatment for newly diagnosed patients. Eventually, it may also help inform development of specific treatments for people with different types of hemophilia A. - NHF Daily October 13, 2018

It is truly an exciting time in the bleeding disorders community! Since 2017, CHES has had the great fortune to collaborate with Dr. Diane Nugent, CHOC and her team from The Center for Inherited Blood Disorders in Orange, California and Hematology Advanced Diagnostic Lab (HADL) to offer whole gene molecular sequencing for our FVII patients at the Factor 7 Retreat initiated in 2012. The overarching goal was to provide molecular diagnosis and variant identification for patients and family members with FVII deficiency to ascertain why bleeding symptoms and severity levels do not correspond. The discoveries made as a result of this project have led to abstract presentations at both World Federation of Hemophilia (WFH) and International Society of Thrombosis and Hemophilia (ISTH) 2018. This preliminary research clearly indicates that the factor VII levels do not correlate with the bleeding phenotype and that there are mutations that are more closely associated with increased bleeding, regardless of factor level.

So, what does this have to do with hemophilia and inhibitors you may ask? There is still so much we do not know in the world of bleeding disorders and many think we are just scratching the tip of the iceberg. We are caught up in a wave of new science, new studies, new products which are all very exciting! New advancements also create multiple unknowns. In this time of significant change, continue to do your research, reflect on our community’s history, ask questions and don’t hesitate to speak up if a decision just doesn’t feel right for you. The choice to use any product or protocol is yours, alone. After all, it is your own or your child’s body you are making decisions about and those choices can have benefits or consequences for a lifetime.