Integrity, Accuracy, Empathy...
CONTENTS FEATURE
8 I The Prevalence of Pain
Acute pain, chronic pain, neuropathic pain. They all lead up to the complex symptoms that are difficult to treat. In this two-part series, we will discuss its prevalence, definitions, brain science, goals, and barriers for effective treatment.
COMMUNITY CHATTER
INSURANCE CORNER
4 I Inhibitor Family Camp: What It Means to One Family
12 I Ten Things You Should Know (about your health insurance)
Learn why one family, like so many others, looks forward to this one-of-akind program.
How much do you know about your coverage? See if you can pass the test.
6 I HFA Symposia 2013: A Fresh Focus on Inhibitors Couldn’t make it this year? No worries, we have you covered.
FUN & INSPIRATION
7 I You Might Be Living with Inhibitors If...
Jeff Foxworthy might be the pro on rednecks, but we call dibs on inhibitors.
13 I How Much Will You Pay?
7 I I Am Poem
Lavang Vu is an insightful, 12-year-old young man living with hemophilia and an inhibitor. We are honored by his persevering spirit.
Discover a tool that can help you calculate your health insurance costs for 2014.
BLOODLINES
14 I Pipeline Drugs for Inhibitors: What the Pharmas are Cooking Up
Think our factor choices are far and few? “Think again,” says the crystal ball. Check out what the pharmas are cooking up in their kitchens.
15 I Clinical Trials: Just for You
Don’t spend all of your time searching for clinical trials relevant to inhibitors. Let us do that for you. Here are a few of the best ones!
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CONTENTS
Letter From the Editors Welcome to the first and only newsletter dedicated to the inhibitor community! We are thrilled to be able to bring to you information that is pertinent and specific to your lives. As individuals who live with hemophilia and an inhibitor, we understand the challenges, struggles and concerns that are very different than having “just hemophilia”. Although each one of us face issues that are individually unique, what unifies us is that sense of knowing each other’s battles and triumphs. Our summer edition of LifeLines for HealthTM features the beginning of a series on pain. Nothing unites this community more than this particular topic. We hear it over and over from families and individuals who live with it at an acute and chronic level with sometimes no real plan for relief. Finally the medical community is treating this symptom as something that is very real and just as important to recognize, but we are just at the beginning of this understanding. What we do know is that it is just as important to have a well-developed plan for treating pain, as it is to have a plan for managing an inhibitor. In our Community Chatter section, we will bring you news of events and programs specific to the inhibitor community written by community members. Our BloodLines section will bring information on new treatments, products and trials. Insurance Corner will include as much relevant information as we can provide in an environment that changes daily as we work towards full implementation of the Affordable Care Act. The Fun and Inspiration section is meant to provide the levity and triumphs we need and to celebrate, as we navigate this path in our lives. It is indeed a path; one with many twists and turns and sometimes forks in the road but we are always humbled by the grace so many of you demonstrate on your travels. Not only do you persevere, you succeed! Lastly, we want to hear from you! What do you like, what should we include? This is YOUR newsletter! We are so happy to bring it to you. Enjoy! - Janet Brewer & Eric Lowe
LifeLines for HealthTM Disclaimers The views and opinions of our writers are not a reflection of Comprehensive Health Education ServicesTM, Inc. (CHESTM), or its’ sponsors. This newsletter is designed to provide a forum for community members to express their views from an open and honest platform. It is meant to provide a sharing of knowledge and experience meant to help one another. Nothing in this newsletter is meant to replace the advice of your HTC, medical professional team or insurance provider. You are always urged to seek the opinion of a healthcare professional for treatment and your specific insurance provider for information. We take your privacy very seriously. We would never disclose your personal health information without your express written consent. We would never sell nor make available our secure database to anyone. Articles and pictures may not be reproduced, published, and/or placed on websites without the express written permission of CHESTM. In every publication of LifeLines for HealthTM, we will provide links to other websites that are not owned or controlled by CHESTM or its sponsors. We cannot be responsible for privacy practices of other website owners, nor can we be responsible for the accuracy of the information provided.
Inhibitor Family Camp
What it means to one family
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y family and I have had the fortunate opportunity to experience some of the most amazing journeys of our lives as a result of, not only our son Tristan’s severe hemophilia, but also his severe hemophilia with an inhibitor. I always refer to Tristan’s disorder as a blessing in disguise. In our travels, we have found Comprehensive Health ServicesTM (CHESTM) and their wonderful staff, who like us, have family members who have experienced the trials, tribulations, and complications of hemophilia and inhibitors. They have brought the possibility of a place where families can escape the emotional, mental and physical roller coaster of everyday life, living with this disorder. With their true heartfelt dedication, they have created a place for families to feel relaxed and accepted during such trying times, and for that, I am truly grateful they have given us the opportunity to attend Inhibitor Family Camp. Two years ago, in my search for fun family things to do within the bleeding disorder community, I came across CHESTM and Inhibitor Family Camp. As I read up on it I thought to myself, “Wow, really, a camp for families with children who have hemophilia and inhibitors? That’s unbelievable!” The minute I saw this, I had to figure out how to get our family there and I was on a mission. I can’t even describe how excited I was. When I got the notification that we were accepted and learned that we would make the trip to Victory Junction in North Carolina, I was overjoyed with excitement and anticipation. We were going to be with other families who, like us, were going through the same things we were. It brought a tear to my eye, thinking about it and wondering what the possibilities could be. I thought, “Maybe we would meet some really amazing families, and kids, and just bond and feel accepted and normal. Maybe we could share our experiences and bounce ideas off of each other. It’s going to be a wonderful experience, I just know it!” Sure enough, we met some of the most wonderful staff, volunteers, counselors, and families. Victory Junction (founded by the Petty racing family) was a wonderful place to allow children with life-threatening disorders to just be, and enjoy camp like a normal child. We literally left there feeling like we had an extended family and many memories to cherish. This year, when the opportunity arose to go to Inhibitor Family Camp at The Painted Turtle in California, there was no question that I had to make it happen. Tristan had a crazy beginning of the year, with his first ever port infection and an eighteen-day hospital
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stay, along with multiple elbow and ankle bleeds. I was determined to get a reprieve for us. We so needed the support and the break from the monotony of treatments and sleepless nights. So, without hesitation, we made the 6-hour flight from the east coast to the west coast and the two-hour journey by bus, through the foothills of the Angeles Mountains to The Painted Turtle in California. If I can say one thing, the place was breathtakingly beautiful. The cabins and scenery made things feel like we could release the stress and just go with it and we did! From the minute we arrived, it felt like home. A weight was lifted off my shoulders for once. We were welcomed with open arms by the volunteers, staff, nurses, and instantly felt accepted.
The kids were so excited to try activities like horseback riding, archery, boating, fishing, wood shop, arts and crafts, and teen bonding sessions. I was just excited to be able to relax for once and share experiences at the evenings’ parent café sessions. Horseback riding became a challenge for Tristan as he ended up with the inevitable elbow bleed, at camp no less, and was not cleared to ride. He was extremely sad and angry because he was not able to do what his sister and everyone else was doing, which broke my heart. The camp staff saw this and made him feel extra special by bringing him into the horse stalls to meet and pet the horses. He even got to meet a movie horse, which warmed my heart and turned me into a blubbering mess! The staff was so attentive to Tristan’s
feelings and found a way to make him feel special. I could tell he felt so much better about himself, which meant the world to me. One afternoon after education sessions, camp provided its very own Pinewood Derby. Each family made pinewood cars to race. The kids’ cars were amazing! We were entertained all weekend by “Pun”, who was hilariously funny. I don’t think I had laughed that hard in a really long time! At the camp talent show our family, jammed to Ronny James Dio’s “Rainbow in the Dark,” while wearing goofy outfits. I don’t remember the last time my kids and I had so much fun. There were dance sessions and singing during meals. YouTube would have loved our camp version of the “Harlem Shake.” At the end of our long weekend as we watched a slide show from the weekend’s activities, all I could do was cry as I thought, “These pictures are beautiful and those people up
there are our family and are some of the most amazing people I have ever met. We will miss them so much.” I tried so hard to hug everyone as we left that day feeling more relaxed and connected then I have ever felt.
As a single mom of a child with a serious life-threatening disorder, I want to make sure my children get to experience everything life has to offer, despite our life’s many hurdles. Life is too short. To our family, camp is a way to feel normal and to find solace within our much smaller, hemophilia community. Camp is a way to meet new friends, re-unite with old ones, network with other families going through what you may be or will go through, teach and learn from each other and socialize in an environment that feels safe. We are like one big family from beginning to end. That’s what Inhibitor Family Camp is all about. It means that you get to be normal among friends and your inhibitor “family.” Jessica Herren is the parent of two children, Tristan and his sister Taylor. They reside in PA.
Editor’s note: Since the April Inhibitor Family Camp session, The Painted Turtle was significantly affected by the Powerhouse wild fires starting on May 31. Over 30,000 acres were destroyed in the surrounding area, including 90% of the camp’s grounds. Fortunately, the majority of the camp’s buildings were spared, but summer camp sessions were forced to be cancelled. We keep The Painted Turtle in our thoughts and prayers.
COMMUNITY CHATTER
HFA Symposia 2013
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A Fresh Focus on Inhibitors
ost of us dealing with an inhibitor know what it is like to attend a hemophilia event and walk away feeling isolated and confused. We show up with thoughts of ITT, Bethesda Units, immune suppression, orthopedic complications and pain management in our heads, while everyone else seems preoccupied with sports and physical activities. We get commiserating looks from parents who assert that their children “will never have to know what it is like to have a bleed” while ours are sitting close by bandaged up, on crutches or in a wheelchair. We tend to withdraw and stop talking because we don’t want to “scare” other families with our stories or just because we are afraid we will burst into tears. Invariably, we end up gravitating towards others with inhibitors. Many of us stop attending events altogether. In April, the Hemophilia Federation of America held their Annual Symposium. Upon request, an inhibitor rap session was added to the agenda. This was the first time HFA had included anything specific to inhibitors. It was an open agenda session, meant to give individuals and families dealing with inhibitors a chance to get together and share concerns. There were approximately 30 people in attendance, representing about 10 different patients. Matthew Compton, HFA President, Corey Dubin, COTT (Committee of Ten Thousand), Dr. Christopher Walsh, Associate Professor Mount Sinai Department of Hematology, and a representative from
the Center of Disease Control were also in attendance. The Inhibitor Journey is different for each of us. Some are just starting out and suddenly trying to deal with hemophilia and an inhibitor diagnosis. Some tolerize quickly and early in life. Some have had an inhibitor most of their lives and have given up on ITT while just trying to get by the best they can. Some develop an inhibitor later in life and are suddenly thrust into a whole new realm of hemophilia. Some struggle through multiple ITT and immune suppression treatments to eventually end up victorious. Some consider themselves to be tolerized but never really respond to traditional treatment very well and continue to think of themselves as inhibitor patients. The HFA meeting included patients and families at all different points of this journey. As the meeting progressed, we were asked to share our concerns; a long list quickly developed. Emotions ran high and the pain and frustration was palpable as we began to describe the challenges we faced. Some expressed physical concerns; like how to find informed treatment providers, orthopedic complications, and venous access issues. Some had emotional needs; including feelings of anger, guilt, frustration and sadness. My concern was that prevention and treatment for inhibitors had not advanced in many years. The feelings of isolation came out, as many of us agreed that the hemophilia community in general seemed to be either unaware
Debbie Porter lives in Southern California with her husband and three children. Her son, Matthew is 19 years old and has severe hemophilia and an inhibitor. Debbie has been involved with hemophilia for most of her life. She has served on the Board of Directors for the Hemophilia Foundation of Southern California and has represented patient interests on consumer panels and advisory boards. Currently, she has started a campaign called RID (Reduce Inhibitor Development) to raise awareness of inhibitors in hemophilia and to advocate for better information, research and the implementation of solutions. For more info on RID, visit https://www.facebook.com/nomoreinhibitors
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or unconcerned with inhibitors, while the number of inhibitor patients seems to be quietly growing in number. It quickly became apparent that much more than a one-hour, once a year session was needed to begin to address all of these concerns. HFA representatives indicated that they would take our list into consideration and evaluate how future inhibitor programs could be offered. Some parents suggested that separate organizations could be started to provide education, support and treatment guidance to inhibitor patients. My family shared that we had started a campaign to raise awareness about inhibitors and were encouraging further research into why inhibitors develop and strategies to reduce inhibitor development. Cory Dubin from COTT offered to start a working group to review and evaluate the concerns and develop strategies to move forward with addressing the various needs. I think most of us attending the meeting left feeling drained and confused. We had come to the meeting hoping to find support and information. We came from different points of the journey, the needs and concerns were many; and we didn’t all agree on the same priorities. I left wiping away tears, but I realized at least this time we had been offered a meeting during a hemophilia community event and had felt safe to state our fears and openly voice our concerns. Even though it became clear to me that we still have a long road to travel, at least it felt like some steps had been taken. Hopefully next year I will be able to sit back in a room at HFA with my inhibitor family and know that our concerns have been heard and we no longer need to feel alone in the hemophilia world.
COMMUNITY CHATTER
I Am Poem
You Might Be Living with by Eric Lowe, adult with inhibitors Inhibitors If...
by Lavang Vu, April 9,2013
I am a caring person who understands hardships I want to help kids who are sick I hear kids thanking people for gifts I see kids recovery from their sickness I want to take a pill instead of getting poked to get medicine I am a funny person who likes to talk
About the Author Lavang Vu
Severe Hemophilia A Inhibitors Sixth Grade Fort Worth, Texas Talents
I pretend to be a doctor Piano, flute, and singing I feel the pain when the needle goes in Interests I was touched by the nurse’s care Reading and coin collecting I worry about children who are sick I cry when a child is in pain I am a caring person who understands hardships I understand that this is God’s plan for me I say never give up I dream of finding a cure for hemophilia and other incurable diseases I try to help those in need I hope I can get a job that helps other people I am a caring person who understands hardships
10. The ER knows you by your first name. 9. You build onto your house just for factor storage. 8. You’re on your 3rd healthcare policy just this year alone. 7. Your grandma can move faster than you. 6. You own more ice packs than the hospital, your factor supplier, and Amazon combined. 5. Getting around “without” an assistive walking device just feels weird. 4. You have more ace wraps in your sock drawer than socks. 3. You haven’t seen Child Protective Services at your doorstep for over a year, and you’re thinking, “What’s taking them so long?” 2. You actually use your medicine cabinet to store… well, medicine. 1. Your blood type is PAC-MANTM Think you can do better? We’d love to hear them! Email or write to us using the contact info on the back of this newsletter, and you just might get published.
FUN & INSPIRATION
Prevalence of Pain By ANGELA LAMBING, MSN, NP-C
MICHELLE WITKOPF, DNP
P
ain is the most common reason people seek healthcare [1]. During their lifetime, 90% of Americans will suffer pain of some kind. Over 25 million people will experience acute pain associated with an injury or trauma in a one year time span. As aging occurs, the incidence of pain and the need for treatment is expected to rise. Chronic pain (also known as persistent pain) is defined by the International Association for the Study of Pain (IASP) as “pain that persists beyond normal tissue healing time, which is assumed to be three months� [2]. Besides being the leading cause of disability, persistent pain affects a person’s overall quality of life including their ability to function and socialize, as well as impacting their family life [3, 4]. Persistent pain in both the pediatric and adult population is often unrecognized and under treated [5, 6]. Pain in the pediatric population remains poorly managed due to the misconception that children (including infants and young children) do not feel pain in the same way as adults [7]. On the opposite end of the lifespan, older patients are known to be at risk for poor pain management due to inadequate assessment, intolerance of many medications, the interactions of drugs, and multiple co-morbidities [7]. Those who suffer from pain are not the
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only ones who bear the burden. Society as a whole suffers when a person experiences pain, including those with a bleeding disorder. When a person experiences and is disabled by pain, even for a short period of time; family, friends, schools, employers, and many others bear the brunt of the impact. Although direct costs can often be measured in missed days from work, school, and decreased productivity, the direct costs to the person in terms of pain and suffering are immeasurable. Quality of life suffers, often leading to serious dysfunction with the family and social life. An interdisciplinary pain management approach with the underlying goal of helping the patient live a full life is key to success. Treatment does not focus only on the pain. A multidisciplinary team approach requires the patient to be an active participant. The team of health care providers work directly with the person in pain using a variety of measurements, interventions, and strategies for selfmanagement designed to offer a complete program. Interdisciplinary treatment programs concentrate on biological, psychological, and social factors that serve to sustain and even intensify pain. The treatment in these programs involves the active participation and coordination of medical specialists from different disciplines
Summer 2013
with an emphasis on pain management. Team members generally include: physicians, psychologists, counselors, physical therapists, case managers, occupational therapists, nurses, behavioral health and other health professionals. Treatment typically consists of physical therapy, cognitive behavioral therapy, and instruction in self-regulatory skills, augmented with group educational meetings. The objectives include offering skills to reduce muscle strain and sympathetic nervous system stimulation by recognizing and re framing ideas about pain and coping. The patients are guided in conquering fear and avoidance of behavior associated with pain and in improving physical strengthening and conditioning. [8]. Due to joint bleeds and/or dysmenorrhagia, pain is a common occurrence and a known complication in the bleeding disorders population. At a young age persons with hemophilia (PWH) begin experiencing pain. This is often a combination of acute pain secondary to bleeding episodes as well as persistent (chronic) pain as they develop hemarthrosis. The associated pain may accumulate after years of hemarthrosis, especially if they have inhibitors or as they age. The use of factor replacement prophylaxis over the past decades has increased the life expectancy for a PWH dramatically. Multiple co-morbidities of aging have accompanied this increase in life span, including persistent pain [9]. PWH frequently experience acute pain concurrently with persistent pain, thus are unusual in their presentation compared to the general population [3, 10, 11]. This leads to the difficult to treat phenomena of acute or persistent pain for which a multi modal approach is recommended [2,6,7].
Multimodal therapy is the use of different classes of drugs with varying mechanisms of action to produce a synergistic effect while minimizing dosing and potential side effects and maximizing efficacy. It also includes utilizing alternative modalities of therapy such as non-pharmacologic strategies along with pharmacologic strategies. Overall, pain is an under treated phenomenon. This is true within the general population as well as the bleeding disorders population [1, 12]. Untreated and inadequately treated pain has many biopsychosocial as well as financial ramifications. Acute pain: • • • • • • • •
Persistent pain; [16]
Pain that has lasted less than 3 months Cause is often obvious, such as a surgical intervention or trauma Generally results from disease, inflammation, or injury to tissues Onset is quick (ex: after trauma or surgery) May be accompanied by anxiety or emotional distress Cause can usually be diagnosed and treated Usually analgesic medication can be reduced in a short period and the patient will return to his/her baseline pain status Self-limiting; in rare instances can become chronic
Hemophilia Pain Hemophilia pain often starts early in a child’s life, initially presenting as acute pain due to bleeding or to procedures associated with bleed management (ex: intravenous infusions) [11]. Pain in a bleeding joint can result in pressure against the synovial lining (joint lining) and adjacent tissue and from the inflammation caused by trapped blood. Repeated bleeding into the same joint can result in the development of a target joint and progression to hemophilic arthropathy (destruction of the bone), which may cause significant persistent pain. [3]. In some instances, a different type of pain emerges that persists long after bleeding episodes have subsided and appears to be nerve based. The long-term consequences of persistent pain adversely affect family dynamics and may be linked to limited job opportunities, low self-esteem, loss of independence, and poor quality of life. Pediatric hemophilic pain can be especially challenging.
• • • • • • • • •
Severe discomfort that can extend past 6 months Causes may be unknown Treatment is focused on pain reduction, increased function, and improved quality of life Can be classified as a disease process itself Can be worsened by environmental and psychological factors May coexist with other chronic pain conditions May not see the actual injury or physical responses such as changes in heart rate, blood pressure, grimacing and crying out Maintenance of daily function is important Considered non-life threatening
Pathophysiology of Pain Pain Pathway The science of pain is a complex process. Painful stimuli are transmitted from the point of injury through nerves to the spinal cord and ultimately to the brain. However, as that process occurs, there are another complex set of processes that modulate the individual pain experience. Transduction is the process where afferent (moving towards the brain) nerve endings (A-beta, A-delta and C fibers in the skin and tissue) participate in translating noxious stimuli into nociceptive (painful) impulses. A-beta fibers are myelinated (covered like a sheath) and large in diameter. They respond mostly to light touch and movement – such as vibration. A-delta fibers are fast, and myelinated but small in diameter while C fibers are slower and unmyelinated (no sheath). Both A-delta and C fibers respond to painful (noxious) stimuli. Any fiber that responds to painful stimulation is classified as a pain fiber, also known as a nociceptor.
Transmission is the movement of impulses from the nerves to the brain. The primary afferent nerves (mostly A-delta and C fibers) in the periphery (any part of the body that is not attached to the central nervous system) send and receive chemical and electrical signals to their counterparts in the spinal column. The intensity and quality of pain is determined by the sensory parts in the brain. The emotional response is determined by the frontal cortex and the limbic system. This is where emotions such as anger, frustration, anxiety, and depression are attached to the painful sensation. Modulation is the process of dampening or amplifying the pain signal. This process probably takes place at multiple levels but primarily in the spinal cord (these specific areas are known as the dorsal horn). The dorsal horn is rich in opioid receptors (mu, delta, and kappa). receptors pick up the message of pain and transmit to the brain. Modulation can be activated by
FEATURE
Pathophysiology of Pain many modalities including the use of opioids (both natural opioids found within our bodies called endogenous and those opioids we take in as medications called non-endogenous), neurotransmitters such as serotonin and norepinephrine, electric stimulation, stress, and suggestion. This is where it gets complicated -the major ascending tract (going up to the brain, also called the spinothalamic tract (STT) ) ultimately divides in two different pathways as it approaches the part of the brain called the thalamus. This lends to the sensory/discriminative aspects of pain perception and/or the affective/motivational aspects of pain perception.
Perception is the conscious awareness and subjective experience of pain. This is the final result of the interaction of transduction, transmission, modulation, psychological and other characteristics of the individual. The brain perceives the pain and understands it differently and uniquely in each individual person based on their history, the culture, their race, and their ethnicity. GATE CONTROL THEORY This is a model of pain modulation that ties all four of the above steps together. It was initially proposed by Melzack and Wall in 1965 [18]. The authors proposed the existence of an internal capacity to decrease or intensify the degree of perceived pain through the adjustment of incoming
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impulses at a theoretical neurological gate located in the spinal cord. The integration of these inputs (sensory neurons, segmental spinal cord level and the brain) differentiates between types of fibers carrying pain signals, determining whether the gate will be opened or closed. The “Gate” can be manipulated by psychological variables (emotions, thoughts, distraction, and stress reaction), pharmacologic measures (altering the transduction, transmission and modulation) or psychological intervention. Gate control theory is often used to explain phantom or persistent pain. NORMAL (NOCICEPTIVE) PAIN Normal pain is often classified as somatic (bone, joint, muscle, skin or connective tissue) or visceral (organs, such as the GI tract) pain. Nociception is when pain receptors (nociceptors) are damaged or irritated and they transmit the signal of pain to the brain. Nociceptive pain is usually time limited in that as the tissue heals the pain resolves (except for arthritis pain). This pain is often described as well localized, constant, aching and throbbing in quality. It usually responds well to opioids ABNORMAL PAIN Abnormal pain occurs in the context of a nociceptive system that has been altered by tissue damage or other processes. There are several types of abnormal pains including inflammatory pain, central sensitization, allodynia, and hyperalgesia. Hyperalgesia has several subclasses. Inflammatory pain is a sensation resulting from injury to a somatic tissue (e.g., skin, muscle, bone) customarily followed by an inflammatory reaction (ex-acute injury). The inflammatory response releases substances that cause “sensitization” of the peripheral pain fibers (nociceptors). Often these pain fibers will develop a lower threshold for firing resulting in more frequent
Summer 2013
firing than when in a normal state. When this occurs, even normally nonnoxious stimuli (light touch or contact with clothing) will cause pain. After tissue healing, the pain generally resolves. However, in states of ongoing inflammation or injury, such as hemophilic joint bleeding, where inflammation may resolve but leave permanent anatomic changes, (ex: joint damage produced by hemarthropathy) persistent pain may result even though inflammation disappears or becomes unnoticeable. Central sensitization is the consequence of continual or frequent and excessive pain signals from the periphery (remember this is any part of the body not attached to the central nervous system) overwhelming the central nervous system. It is often referred to as “wind-up”. This bombardment of pain signals causes long-term changes in the central nervous system leading to persistent amplification of those pain signals. Central sensitization is one suggested mechanism where a normally harmless stimulus produces pain. Allodynia and Hyperalgesia are types of central sensitization. Allodynia is the phenomenon of normally harmless/painless stimuli (such as light touch) producing pain. Hyperalgesia is an exaggerated response to normally painful stimuli. In hyperalgesia the distinction between primary and secondary is important when deciding on treatment. To effectively treat central sensitization pain, hypersensitivity must be addressed during the clinical assessment of the patient. Therapy that targets the mechanisms of hypersensitivity, if present, rather than mechanisms of nociception, must be used to try to alleviate symptoms. Primary hyperalgesia occurs at the site of injury. It is characterized by a lower pain threshold, spontaneous pain, and increased sensitivity. It usually features thermal (temperature either hot or cold) and mechanical
(touch) hypersensitivity. Secondary hyperalgesia is hyperalgesia occurring outside the area originally injured. It is thought usually to be a consequence of central sensitization. Neuropathic pain is pain due to damaged or dysfunctional nerves. The pathophysiology of neuropathic pain can have both peripheral and central mechanisms that generate abnormal signals with abnormal excitability. Dysfunctional pain is pain and abnormal sensitivity not associated with noxious stimulus, tissue damage, inflammation, or identifiable lesion to
the nervous system. This may include fibromyalgia, tension-type headaches, migraines, and/or irritable bowel syndrome. Individuals with these syndromes share a number of common characteristics, including hypervigilance to sensory stimuli, exaggerated experience of a diverse array of sensory stimuli, high prevalence of associated conditions and in some cases abnormal biomarkers (example: high levels of pain transmitters called substance P in spinal fluid) . Referred pain is the perception of pain in a body part in which it did not
originate. The mechanism of referred pain is thought to be convergence of primary afferents from different locations onto the same spinal cord neurons. The spinal nerves serve both deep tissue structures as well as skin structures leading to mislocation of the perception of the pain signal. Research continues to evolve on identifying the different types of pain and response to treatment associated with specific type of pain. All of these factors are considerations when treating hemophilic pain.
Pain Assessment GOALS OF THERAPY The first step in pain assessment in the bleeding disorder patient is to identify the goals of therapy which should include: • •
Increase function Minimize side effects
A functional pain goal is a goal based on achieving an activity or quality of life issue. This provides a clear direction as to the planned desired improvement. Keep in mind, if a person with hemophilia has been experiencing persistent pain for many years, a goal of “no pain”, or 0/10 may not be a realistic goal. The goal should be a reduction of the current pain level or, preferably, improvement in the level of functioning [19]. Development of measurable goals can incorporate the “SMART” mnemonic [20] Specific, Measurable, Achievable, Realistic, Time-based –SMART. Measurable outcomes are necessary to provide a clear goal. Suggested measurable outcomes (goals) include: pain intensity decreased, reduction of medications as pain improves, improved sleep, improved functional activities, an increase involvement in social activities [15]. It is important to use a pain scale that works for the individual person and use it consistently. Some people find that when using a numeric rating scale (0-10) decreases in pain can be visualized, whereas others may find a decrease in the percentage (%) of pain may be easier to visualize. Other Pain Resources You will find links to more than 35 important health associations and organizations that can provide additional information on pain as it relates to specific conditions and disease states, as well as links to sites and organizations devoted to substance abuse. http://www.partnersagainstpain.com/hcp/pain-resources/information.aspx
BARRIERS Barriers to effective pain management should be explored as potential barriers can affect goal achievement. Identification of potential barriers can provide an opportunity for increased education in the arena of pain management. Patient perceived barriers to the use of opioids often include worry about harmful effects, fear of addiction, misunderstanding between psychological addiction vs. physical tolerance/dependence vs. tolerance, fear of side effects (including but not limited to drowsiness, nausea, constipation, and potential mental cloudiness), immune system harm, save pain medication until they “really need it”, pain is “part of being a person with hemophilia”, hesitant to report pain as may mean progression of disease, “Good patients” do not complain, pain will distract provider from addressing underlying problems, inability to afford medication, lack of insurance, restrictive insurance formulary, fear of how perceived by others, family & friends perceptions (potential negativity, stigma, and distain; pressure from family/friends to halt use of prescribed pain management), colleagues perceptions, fear of reprisals, drug testing, and afraid to use sick time (15, 23, 24, 25, 26, 27). Provider barriers include lack of education regarding pain management strategies, lack of knowledge regarding pain experience, fear of patient addiction/tolerance if opioids are utilized, fear of patient diversion of opioids, and fear of prosecution if patients utilize opioids for pain management (15, 23, 26). System or institutional barriers include pharmacy may not have medication in stock, restrictive hours of pharmacy, pharmacist knowledge, cost of medications, provider attitudes and pharmacist attitudes (15, 23). All citations for this article can be found on: LifeLinesForHealth.org *Look for our winter issue of LifeLines for Health for a variety of approaches and concerns regarding the management of pain.
FEATURE
10 Things You Should Know
(about your health insurance)
W
e asked insurance specialist, Andy Matthews of Specialty Therapeutic Care what every individual should know about his/her health insurance coverage. See if you have answers to all ten of his top picks.
1
What are my benefits and do I have a choice of providers? • Do I have a HMO, PPO? • Do I have a PBM or designated pharmacy?
is my in network benefit compared to my out of 2 What network benefits?
factor paid for? 3 How• is myMajor medical - This is the part of your policy that pays for expensive items like hospitalization, lab work, MRIs, CAT
•
• • •
• •
Scans etc. Since factor is so expensive, it sometimes could be billed in either place: medical or pharmacy. More and more insurance companies are now trying to move factor into the pharmacy part of your policy. Many families satisfy their out of pocket expenses by placing a factor order at the annual start of their policy. This is advantageous especially if your insurance is billing factor under your major medical benefit. Pharmacy - The pharmacy part of your policy is set up to pay for what is says, pharmacy items: ie pills and simple medications. The more and more people are on longer term medications, most insurance companies and employers use what they call PBM’s/ Pharmacy Benefit Managers who manage the pharmacy benefits of either employees or members of an insurance company. What is changing is when factor is switched to the pharmacy side (rather than major medical), many times most people are hit with higher out of pocket costs than they have been paying in the past. What is the difference in co-pays if I use my major medical compared to pharmacy benefit? Most people have a maximum out of pocket of $1500.00 to $5000.00. If they are on some type of hardship program through their home health care or specialty pharmacy, then those out of pocket expenses are satisfied for the year other than a few small co-pays you might incur for some pills. If moved to the pharmacy like my insurance is doing, I will now have to pay my $2500.00 maximum out of pocket that have not had to pay because it was satisfied with my first factor order under major medical. Then each time I order factor, I will be charged with a $125.00 to $250.00 co-pay every month. These co-pays will start to add up and are changes that everyone needs to be aware of. Do I even have an option of using my major medical and not pharmacy benefit? You should always ask if your factor could be billed through major medical, if possible. Every situation is different, but at least look into it to see what is best for you.
of COBRA if I leave or am laid off? 5 4 Do I• haveIf theso, amoption I prepared to pay for it? •
Have I contacted PSI for assistance or other organizations that could help?
plans, how does this change my benefits 6 IfandI dodoeschange it give me more or less choices on: • •
Factor Providers In Network doctors and hospital (i.e. your HTC)
are the best companies to work for, and do I know 8 What where to find them? Do a Google search for Fortunes’ Top 100 Best Companies to Work For.
you know that many manufacturers now have co-pay 10 Did assistance programs in addition to compassion factor use
Do I have a plan B if I lose my job or insurance? • Does my state have a high-risk pool? • What are the benefits? • Can I get private insurance on my own possibly through exchanges soon?
do I contact with my employer in case I have 7 Who questions or have a problem? other assistance is available? 9 Lastly,• what State programs like Medicaid? • • •
Federal programs like Medicare? Could I qualify? What manufacture programs am I eligible for if I lose my insurance?
programs that will help pay for some of these costs? • Baxter’s The CARE program is open to all hemophilia A and inhibitor patients, regardless of their current therapy or insurance coverage. • Call 888-229-8379 • https://rma.parexel.com/Baxter/care-webform.html?source=BXOWCRE122 • It is important you enroll in these programs BEFORE you need them.
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Please see our winter edition of LifeLines for HealthTM for other manufacturer programs.
As you know, Living with an Inhibitor can be complicated & difficult. Let AHF give you a few extra hands. AHF is the homecare that specializes in providing services to children and adults Living with Inhibitors AHF once again received a 100% satisfaction rating from their family of clients. We earned our reputation by providing the highest quality of services around and providing endless support to the bleeding disorders community. AHF_Hemophilia fb.com/AHF-Hemophilia-Services Independently Owned
Family Operated
(800) 243-4621 | AHF@AHFinfo.com | www.AHFinfo.com
How Much Will You Pay?
You may be eligible for financial assistance for your health insurance
In October 2013, the health insurance exchange will open in every state providing subsidies to those who (1) don’t qualify for Medicaid coverage and (2) fall under certain other guidelines, such as annual income. You must also purchase health insurance on your own and not through your employer to qualify for the subsidy. Below is a chart that illustrates the annual income restrictions followed by Medicaid and the health insurance exchange. As an example, a mother and her child living together is a family size of 2. By using this chart, the mother knows that she
would not qualify for Medicaid coverage if she were to earn more than $20,628.30, (or 133% above the federal poverty level/FPL). If she is not insured by her employer, and she earns less than 400% of the FPL, she would be eligible for a subsidy. According to the Kaiser Family Foundation’s online subsidy calculator, this single mother could receive approximately $3,135 in annual subsidies while earning $30,000 per year, which would cover 64% of the plan’s premium. To try out the calculator yourself, visit: http://kff.org/interactive/subsidy-calculator/
Visual provided by public policy analyst, Mary C. Garvey, of the National Hemophilia Foundation.
INSURANCE CORNER
Pipeline Drugs for Inhibitors What the Pharmas are Cooking Up Please refer to the top of page 15 for an explanation of each of the 4 trial phases mentioned below. Name: NNC 0078-0000-0007
Name: OBI-1
Company: Novo Nordisk
Company: Baxter
Product Type: Recombinant factor VIIa
Product Type: Porcine recombinant factor VIII Description: This is an alternative treatment for patients with hemophilia A and inhibitors. Where It Stands: A Phase III trial was initiated in January, 2012. Results showed the product is well tolerated and effective. Inspiration received orphan drug and fast track designations from the U.S. FDA for review of OBI-1. The company had planned to submit a biologics license application in the first half of 2013.
Description: The purpose of this trial was to confirm the efficacy and safety of this rFVIIa in patients with congenital hemophilia and inhibitors. The product aimed to have increased clotting activity compared to Niastase. Where It Stands: In October 2012, a Phase III trial was stopped by the company because of the appearance of antibodies to the drug in some patients. Novo will not continue development of vatreptacog alfa. Name: CSL689 rVIIa-FP
Name: PF-05280602
Company: CSL Behring
Company: Pfizer / Catalyst Biosciences
Product Type: Recombinant factor VIIa
Product Type: Recombinant factor VIIa Description: The purpose of this trial is to study the safety, tolerability and pharmacokinetics of this product, a recombinant factor VIIa variant (813d). Where It Stands: A Phase I trial started in August, 2011 and is recruiting participants. Name: LA-rFVIIa Company: Novo Nordisk Product Type: Recombinant factor VIIa Description: The aim of this clinical trial is to investigate the safety and pharmacokinetics of long-acting activated recombinant human factor VII (LA-rFVIIa) in patients with hemophilia. Where It Stands: A Phase I/II trial has been completed.
Name: LR769
Company: The Children’s Hospital of Philadelphia
Product Type: rFVIIa Description: This is a novel recombinant form of human factor VIIa (rhFVIIa) for patients with congenital hemophilia A or B and inhibitors. Where It Stands: rEVO announced in June 2013 that it had completed the treatment and follow-up phase of a key dose ranging study of LR769. rEVO will be working to initiate a Phase III trial later in 2013. Source: Canadian Hemophilia Society at hemophilia.ca
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Where It Stands: A Phase I trial was completed in 2013. The product was granted Orphan Drug Designation (ODD) by the European Commission. This could entitle CSL Behring to exclusively market in Europe for a period of 10 years if the product at the stage of license application fulfils the orphan drug requirements.
Name: Factor Xa
Company: rEVO Biologics and LFB
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Description: This is a recombinant fusion protein linking coagulation factor VIIa with albumin for the treatment of inhibitors in hemophilia A and B. A greater than 8-fold increase in half-life was observed in pre-clinical studies.
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Product Type: Recombinant factor Xa Description: The altered shape of factor Xa is intended to make it safer and more efficacious compared to the wild-type factor, but much longer lasting in blood circulation. This could lead to a new class of bypass therapy for hemophilia. Where It Stanads: Pre-clinical
Clinical Trials
Just for You
What Does Each Phase Mean? Each phase has a different goal, and is designed to answer a different question.
I Can See Your Future Official Title: NCT00178607 Purpose: Up to one fourth of patients with severe hemophilia A develop an inhibitor but at present it is not possible to predict which patients will develop such antibody. The ability to predict an inhibitor development at an individual level would greatly improve therapeutic approach to this serious problem. Subjects: Males with hemophilia A and an inhibitor titer of 0.6 or higher Link: http://clinicaltrials.gov/ct2/show/NCT00178607
I’m Everyone’s Type Identifier Number: NCT01439971 Purpose: This study hypothesizes that the study CONTENTS drug, PF-05280602 (at the selected doses) will be safe to administer to subjects with severe Hemophilia A or B with or without inhibitors and will demonstrate evidence of hemostatic activity. This is supported by the pre-clinical findings in hemophilic animal models. Subjects: Males ages 18-64 with hemophilia A or B, with/without inhibitors Current Phase: 1 Link: http://clinicaltrials.gov/ct2/show/NCT01439971 ?recr=Open&cond=%22Hemophilia%22&rank=34
A New Factor 7 Flavor Identifier Number: NCT01757405 Purpose: To determine the efficacy and safety of a new factor 7 product (currently named rFVIIa BI) as part of a six-month on-demand treatment regimen in hemophilia A or B subjects with inhibitors. Subjects: Males ages 12-65 with hemophilia A or B, with inhibitors Current Phase: 3 Link: http://clinicaltrials.gov/ct2/show/NCT01757405 ?recr=Open&cond=%22Hemophilia%22&rank=5
BLOODLINES
Phase 1: Clinical trials are designed to test the safety of a new drug or medical procedure. They are usually quite small, and may only involve a few dozen people. Phase 2: Clinical trials are designed to further test the safety of the new treatment, and learn if it is effective. They usually involve several hundred people. Phase 3: Clinical trials are intended to confirm whether a new treatment is effective, and to gather information about its side effects. Phase 3 trials may involve 1000 people or more. Phase 4: Clinical trials are usually undertaken after a new drug has been approved for use by health and regulatory agencies. Further information is gathered about the drug’s long-term risk and benefits, and the best ways to use it.
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CHESTM Mission To Inspire awareness and selfreliance for patients with chronic health conditions, their families, and their communities.
Editors in Chief Janet Brewer, M. Ed. Eric Lowe
Publication Designer Eric Lowe
Contributing Writers: Jessica Herren Angela Lambing, MSN, NP-C Andy Matthews Debbie Porter Lavang Vu Michelle Witkopf, DNP
Contributing Editors: Kathy Arnaldo Amy Massey
Contributing Content and Production: Tara Baughman Mary C. Garvey Joan Ward
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