Bleeding Disorders

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Our First Year of Living with Hemophilia: a Reflection

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By Jeremy Sobotka with Ragina Auch

Ragina and I are the parents of two adorable children – Jade, age 14 and Tristan, 9. We were introduced to the world of bleeding disorders when our son was diagnosed with severe hemophilia B. We’ve learned a lot in the past few years and would like to share the experiences of our first year.

After Tristan was born, his heel was pricked for routine newborn lab work. Throughout the day, the tiny puncture bled through the bandage and booties and onto the blanket. His nurse did a complete bandage, bed and clothing change. The next day, another nurse came in and changed the bandage, put clean clothes on, and brought in fresh blankets. On the third day another nurse arrived to change everything again, and this time I asked, “Why is he still bleeding?” I mentioned that the other nurses had changed everything each day prior because of the bleeding. Apparently, the nurses had not communicated that with each other and the doctor was unaware. When the doctor was notified, a blood draw was ordered to test his platelet count and check for other bleeding disorders. Later that afternoon he came in and gave us the shocking news that Tristan had hemophilia.

Our initial reaction to learning our son has severe hemophilia B was one of fear, grief, confusion and hopelessness. Though Ragina had heard of hemophilia, she didn’t know much about it. Never having heard of it myself, I didn’t even know how spell it! I hoped Tristan would just be given some kind of medicine, and everything would be fine. When I understood it was a lifelong medical condition, I instantly felt sick to my stomach, angry, scared and helpless.

It wasn’t until the 3rd or 4th visit to the hemophilia treatment center when the doctor suggested inserting a port so Tristan could be placed on a weekly prophylactic schedule that I began to realize the lifelong impact. There really isn’t anything that can or would prepare a parent for that kind of news. Everything sounded even worse when our pediatrician said he had never had a patient with hemophilia in his 30+ years of being a doctor.

As Tristan started learning how to get around, bruises began appearing all over. As the bruising increased with his activity level, we became more interested in having a port placed so he could start prophy treatments. However, we had to wait until after Tristan’s first birthday to proceed. He was already walking, and we knew things could get worse. 

At first Ragina and I had split opinions on the decision to have a port inserted. After all, who wants to have their child go through any surgery, especially at a year old? 

After weeks of debating, we decided it would be best for him to have a port. After going through all the difficult blood draws and injections, we have been happy with our decision. For us, it’s been a night and day difference - now that we can give him his treatments and have labs drawn without a single tear! Learning to infuse him via the port was nerve racking at first, but it became our weekly evening family ritual that we did together. Jade’s job was to keep Tristan entertained as I held him while Ragina did the infusion.

He didn’t experience many bleeds before the port was placed, so we have not learned how to access a vein. At nine-years-old, Tristan’s port is still fully functioning, but we are now exploring how best to learn to infuse our son through venous access.

Since those early days, we have accepted that Tristan will have hemophilia forever, or at least until a cure is found. We have realized the best thing we can do is learn as much as possible about his bleeding disorder so we can be better prepared to teach him as he grows up. We’ve been doing our best to treat Tristan as normally as possible. At the same time, overprotectiveness kicks in and sometimes I just want to put him in a bubble. On occasion, we have to get on our daughter because she tends to play rough with him, but he is usually the one instigating the horseplay! As he’s gotten older, he’s proven to be a typical boy who loves to climb and get into things… go figure!

With my son’s first year of life, I have learned that when it comes to bad news about my family, I tend to blow it way out of proportion. I am supposed to be the rock for them to lean on and I admit I am the least qualified for the job. I envy Ragina for being able to keep her cool and be strong enough to handle everything better than I do.

Presently, even though we are not together as a couple, we continue to work together for the benefit of Tristan’s health. We have worked well together in both routine and emergent care. Jade, even years later, still enjoys roughhousing with her younger brother! 

Tristan is doing very well these days and enjoys video games, playing with his friends and family, driving an ATV, and has just signed up for 4-H. He hasn’t had many emergencies, a few stitches, but the biggest issue he continues to experience are bleeds in his ankles. We are in the process of having his dosage adjusted and getting orthopedic inserts for his shoes to hopefully help with that. You would never know by looking at him that he has hemophilia, he is just a normal 9-year-old boy living life to the fullest. 

Hemophilia has been an experience for all of us. It has taught us a whole new level of patience. Tristan didn’t understand in that first year that anything was wrong with him. Even now, his hemophilia has been so well controlled that in his eyes, the problem he has is with the port and treatment rather than bleeding episodes. I don’t want him to know any different! Kids with hemophilia are the same as any child and shouldn’t be treated differently – they still play and fight with siblings. Just remember to be a bit more cautious and alert and know they will have a few extra bruises now and again no matter how careful they are. Take each day one at a time and have faith everything will work out fine!

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In Search of Support

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By Fel Echande

My family is a mix of Costa Rican and Mexican descent, and we have lived in the USA for 24 years now. I am a commercial driver, and my wife, Laura, is a photographer. We have two sons, Fiach, 22 years-old, and Yanni, who is 17.

Our first-born son was diagnosed with severe hemophilia B at 10–months old. After consistently showing up to the pediatrician’s office with unexplained bruising, our physician suspected abuse. However, after running a battery of blood tests, it became evident that Fiach has hemophilia.

With no family history of a bleeding disorder, we knew very little about hemophilia. We had a lot to learn. We knew the diagnosis was going to be a struggle, but thanks in part to amazing support from the bleeding disorder community, our family is thriving.

Twenty-plus years ago, the internet was in its infancy. In those early days, the lack of information was challenging us. There was very little information available in Spanish.

My wife came across a book authored by Laurie Kelley, which had been translated to Spanish (no longer available in Spanish). The book proved to be a huge help for us as we struggled to find more information.

Outside of the clinical support provided by our medical team, we began to realize the importance of connecting with others in the bleeding disorders community. Becoming involved with local and national chapters was instrumental in broadening our education, comfort, and ability to deal with hemophilia.

Laura and I began meeting so many wonderful people in the community. Each friendship brought a fresh perspective and helped us see that our son could live a largely normal life. We learned so much from the individuals and families having already walked in our shoes.

Making friends with other children with hemophilia helped our son understand that he is not alone in the world. The friendships taught him to embrace life with a bleeding disorder. When things were stressful for us as parents or for Fiach as an affected child, we knew we could lean into community-based support provided by our local chapter and organizations like The Coalition for Hemophilia B.

Through our involvement we learned to be strong advocates for ourselves, for our son, and for others in the bleeding disorders community.  As the years went by, we became more knowledgeable and confident and were compelled to get involved and give back to the community that had helped us so much.

We are now very involved in volunteering. I am currently on the board of directors for the Hemophilia Association of San Diego County. My wife and I have been part of National Hemophilia Foundation’s Guias Culturales (Cultural Guides helping NHF with workshops for the Hispanic families). My wife was involved with the Hemophilia Chapter of Northern California’s The Female Factor Retreat. We are both very involved nationally with The Coalition for Hemophilia B and we also lead community support groups to help our local families.

We encourage all bleeding disorder families to get involved, get educated, and take advantage of the support provided by the organizations serving this community locally and nationally. Attend events, connect with families, and learn through our shared experiences. For those who are dealing with a new diagnosis know that it does get better. Our community is a family—don’t ever be hesitant to reach out.

Spanish Language Resources


The Coalition For Hemophilia B
Newsletters in English and Spanish

www.hemob.org/newsletter

www.hemob.org


National Hemophilia Foundation
Guias Culturales (Cultural Guides)

https://www.hemophilia.org/educational-programs/outreach/guias-culturales

HemAware en español, https://hemaware.org/es


Hemophilia Federation of America
Sangre Latina

https://www.hemophiliafed.org/join-sangre-latina/

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Time to Rethink Women and Bleeding Disorders: Stop the Unnecessary Suffering!

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By Linda “Lew” E. Wyman-Collins, BSN, RNC-NIC

As a woman with hemophilia A, a platelet disorder and Ehler’s Danlos Syndrome (EDS), the bleeds I experience are more in line with moderate to severe hemophilia. At age 65, I suffer with various medical complications and side effects from receiving a later-in-life diagnosis. I did not obtain a diagnosis or proper/adequate treatment for most of my life. The pursuit of equal or compatible treatment that my blood brothers receive has been a struggle.

Throughout my life, dental work and cleanings caused extensive bleeding. Impacted wisdom teeth caused me needless mouth pain for many years, but because of how easily my mouth bled, no one wanted to extract them. Finally at 52, I found an oral surgeon who agreed to remove them with treatment prior to procedure. I experienced senseless tooth and mouth pain for so many years. Now I treat with DDAVP nasal spray prior to all dental appointments, and the bleeding is minimal.

When I was 11 years old, I began having gastrointestinal bleeding. Physicians believed I had colitis; the treatment was dietary changes and medications. The mucosal lining of my lower intestine is permanently damaged from years of uncontrolled bleeding. Now with a proper diagnosis, I am able to effectively treat with clotting factor, desmopressin (DDAVP®) and tranexamic acid (Lysteda®).

All my life, I have bruised very easily and when my menstrual cycles started, I bled heavily with large clots for more than seven days every month. When I voiced my concern to my doctors, I was told my cycle was “normal” because my mother and grandmother also had heavy cycles and that being a fair-skinned redhead added to the bruising. In retrospect, my mother and grandmother both had undiagnosed bleeding disorders. Due to the prolonged bleeding each month, I had chronic anemia, which greatly affected my quality of life.

In 7th grade I injured my knee during gym class. When I look back at that injury, it was clearly a muscle and joint bleed. As I grew older, my left knee deemed itself my target joint. It is chronically swollen with decreased range of motion; x-rays reveal the knee joint is now bone-on-bone. Steroids and gel injections only offer temporary relief, and I am now considering a knee replacement. Had my joint bleeds been treated properly when I was younger, the extensive joint damage could have been avoided or at least lessened. I now wear rib, back, knee and thumb braces due to impaired joints.

Current studies are revealing a correlation between Factor VIII deficiency and bone health, and I have been diagnosed with osteopenia. Women with a bleeding disorder are developing osteopenia and osteoporosis at an earlier age.

In 1983, when my oldest son was diagnosed with severe hemophilia A at 17 months, scientists had not yet discovered the gene where the mutation occurred. When DNA testing became available, I was tested, and the results showed I had the same gene mutation as all 3 of my children, yet despite my bleeding history, I was labeled as just a “symptomatic” carrier.

At 35, I had an inguinal hernia repair scheduled but at that time, wasn’t being seen by a hematologist. After reading in my medical record that I was a carrier, the anesthesiologist refused to clear me for surgery until I was seen by a hematologist. I went to a hemophilia treatment center and was tested and was diagnosed with a platelet disorder.

Shortly after, I was identified as having EDS and was then prescribed DDAVP via IV for any surgery or invasive procedure. Tranexamic acid and aminocaproic acid (Amicar®) helped with menstrual cycles and mucosal bleeds. In my late 40’s, I was finally properly diagnosed with a bleeding disorder and received appropriate treatment for chronic anemia; yet I was not given access to clotting factor for another 17 years.

Unfortunately, the majority of the medical community is often still under the notion that only males can have a bleeding disorder. The assumption is women are only carriers and do not need treatment. However, rather than basing care on gender, treatment should be based on documented factor levels and bleeding tendencies.

Our national organizations, non-profits, and pharmaceutical companies have done a great job at educating the community on bleeding disorders in men and women. Yet, there still remains a missing piece on prescribing proper treatment for women. Education needs to be expanded at the medical school level to instruct doctors-in-training to recognize that women can and do bleed. In the meantime, women need to advocate for themselves with a much louder voice and not allow themselves to be dismissed. In the 25 years I have been active in the bleeding disorders community on a national level, I have not seen much change in the timely diagnosis and adequate treatment for women. This needs to change.

ABOUT THE AUTHOR

Linda “Lew” E. Wyman-Collins, BSN, RNC-NIC is a mother, wife, aunt, sister, and daughter of someone with a bleeding disorder and has a bleeding disorder of her own. As a nurse, she has much experience in neonatal intensive care and was recognized as Dallas/Fort Worth Great 100 Nurses. Lew served HFA formerly on the Board of Directors, Blood Sisterhood Chair, Symposium Chair, Medical/Professional Advisory Board member, and is a founding member of HFA’s women’s group Focus on the Feminine. She has served on Texas State Bleeding and Clotting Disorders Advisory Council and is a member of Equity in Bleeding Disorders Care for Women and Others. Additionally, Lew has presented frequently at national and international conferences and has authored numerous articles on hemophilia and women with bleeding disorders in industry magazines and journals.

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Living with Hemophilia: Hope for Help

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By Ashley Gregory

Anthony and Nicholas were born in 1998, and although I had no family history of bleeding disorders, I now had twins with severe hemophilia A. I became informed about their condition and as they grew, stayed in close contact with our hemophilia treatment center. Also in 1998, NHF launched Project Red Flag, which advocated for women with bleeding disorders. I remember being curious because I had experienced puzzling symptoms throughout my time, but life would take a darker turn, and it would be several years before I could revisit this topic.

One day Anthony didn’t seem right; he was lethargic and not eating. I took him to our pediatrician who suspected the flu. I remember looking directly at her and saying, “Shouldn’t we infuse him since his head is hot, but he has no fever?” She said, “No, just take him home.” Instead, we took him to the emergency room where we learned he had a brain bleed. Despite heroic surgical efforts and finally infusing factor, my son died the next morning – he was only 11 months old. Thus began a slow tumble into despair that our family would not emerge from until well into the next decade.

During subsequent years, researchers were learning that women with the hemophilia gene exhibit unexplained bleeding symptoms and need treatment. Some doctors began successfully working with women to determine how to manage symptoms using factor replacement; they were learning that despite “normal” factor levels, women who experienced unexplained bleeding responded well to factor treatment with no adverse reactions. It was unexplained bleeding that pulled me back into a search for a diagnosis.

While volunteering at my local foundation, I heard women discuss similar untreated symptoms around inexplicable bleeding. It didn’t take long to realize there was a common problem. Women in our community, like me, were experiencing puzzling bleeding that was not addressed despite reporting these symptoms to their doctors. The concerns were usually explained away with comments like, sometimes these things happen, sounds like all the women in your family are like this, or this is just your normal. Were we being ignored, or did the doctors simply not believe us?

This led to a personal investigative journey to seek a diagnosis and treatment while, at the same time, pursuing a career in advocacy and education in bleeding disorders. I began attending local, regional and national programs that provided comprehensive education about my bleeding symptoms and brought me in contact with physicians who were successfully treating women’s bleeding issues using all the medications available to men with bleeding disorders.

I learned about lyonization, which is when one of the two X chromosomes in every cell of a female is inactivated. Lyonization can cause an effective X chromosome to stop working, allowing the other ineffective X chromosome to take over, producing lower factor levels. This led to an even greater understanding of my particular bleeding disorder as a woman with two X chromosomes. Thanks to Dr. Barbara Konkle and the My Life, Our Future genetic analysis project, I learned that some mutations present a high factor level but bleed like a severe! I finally learned that connective tissue disorders can also be present in persons with bleeding disorders, which can then exacerbate bleeding.

Empowered with this knowledge, I confidently entered the HTC near me and presented the information I had gathered, along with my symptoms and history. I trusted I would be heard and cared for. I could not have been more wrong. I even brought my mother along who had been by my side through all my pain and could attest to my history. The treatment center told me my factor levels were too high for my swollen ankles and knees to be caused by hemophilia, and my petechiae were birthmarks.

My unexplained bleeding history was useless in gaining a diagnosis here. I was also seen by the genetics specialist who told me I lacked enough markers to have a connective tissue disorder. I was left with the option to do nothing or to have a synovectomy on my right ankle to see what fluid it contained. Based on my past, I instinctively knew without infusing factor prior to the procedure, the healing process would be long and painful. I declined the surgery in pursuit of a better option. 

It was time to try a new approach. I crossed state lines and met with an expert clinician I had met at a national symposium. After a thorough medical evaluation, complete history review of symptoms, physical evaluation and lab workup, I was diagnosed with hemophilia A (symptomatic carrier) and hypermobility syndrome – a connective tissue disorder meaning my joints stretch further than normal. Aminocaproic acid (Amicar®) was prescribed for mucosal bleeding and clotting factor for muscle and joint bleeds. An emergency medical card was prepared with my treater’s name, contact number and diagnosis. A medical alert bracelet was ordered for me, and I was instructed to contact the treatment center and treat on-demand as needed.

Imagine my delight to find when I treated a bleed as my hematologist instructed, my whole body felt better; things that had hurt my entire life stopped hurting; my petechiae cleared, and the swelling in my knees and ankles subsided. Then, as the factor left my body, the pain and baffling bruising would return.

I was able to access treatment from the out-of-state HTC for a short time, and I was emboldened to treat my hemophilia the same way I was as a mother in treating Nicholas’ hemophilia. Since I had been infusing him for years, infusing myself was easy, and I kept a log of bleeds and treatments. I was amazed at the overall improvement in my energy and stamina when using factor!

Moving forward to 2022 – Through my out-of-state HTC, I was able to access free trials of factor products, but those have ended. I am no longer able to have treatment for my bleeds. The system that pays for factor for persons with bleeding disorders requires an in-state doctor to write the prescription. So far, I have not found a doctor in my state who is knowledgeable about the particular genetic mutation that causes me to have a high factor level yet bleed severely. I am now a woman without a treatment center and without treatment.

My chronic pain and suffering affects not only me, but my family as well. Because of my health, we are not living our best life. In spite of this, I am grateful for my experiences, to my sons born with hemophilia, and to Anthony who didn’t survive due to the lack of knowledge that prevails to this very day. This lack prevents his mother from treatment and medication.

I am grateful to Nicholas, who bears witness to the stark contrast of gender care in hemophilia. I have built a career advocating for those like me who are unable to access the care we know is needed. I am appreciative for all of these experiences, but I would also like to be grateful for access to treatment for all women with hemophilia. It is my hope this will be a reality soon.

TIMELINE HIGHLIGHTING A FEW SYMPTOMS THROUGH THE YEARS:

  • Age 4: my first memory of severe pain in my knees. I had no words to describe the pain and it went unattended.

  • Age 9: moved to a home in a hilly area. I had pain that brought tears and immobility. Diagnosed with pre-patellar chondromalacia and was instructed to avoid hills and stairs, and to rest, ice and elevate. The constant pain kept me sedentary.

  • Age 10: my menstrual cycle began with extreme pain, heavy clots, bruises under my eyes and sheer exhaustion. Soaked through sanitary pads and ruined sheets. At school, it seemed I was in the bathroom more than in class. Treatment for this would never come. I spent my menstruating years suffering the effects of anemia.

  • Age 15: cut my ankle on a jagged piece of wood. The wound kept oozing and reopening, taking a year to fully heal.

  • Age 16: worked long shifts standing on a hard restaurant floor, in constant pain, fatigued, with swollen knees and ankles. When sitting, I would draw my legs up under my body to prevent my ankles from dangling as the pain was unbearable. Tired and hurting all the time. By the time I became sexually active, I bled with intercourse regardless of my cycle.

  • Age 21: diagnosed with gout in my toe. I now recognize this was a bleed.

  • Age 25+: with each of my first 3 pregnancies, I experienced anemia, 2nd trimester spotting, petechial hemorrhaging around my face during childbirth and prolonged postpartum bleeding. 2nd pregnancy brought throbbing pain behind my left eye leading to a spinal tap that would not clot causing a week-long leak of cerebrospinal fluid. 3rd pregnancy resulted in prolonged healing of c-section incision.

  • Age 27: wisdom teeth extraction bled for weeks.

  • Age 28: at the ER with excruciating knee pain. Was told there was nothing to be done.

  • Age 32: sons Anthony and Nicholas were born via C-section. A bleed at the incision caused excruciating pain; bleeding and severe bruising in my groin and down my legs.

  • Age 35: diagnosed with fibromyalgia.

  • Age 45: diagnosed with tendinosis (Related to connective tissue disorder).

  • Age 53: finally diagnosed as a symptomatic carrier of hemophilia A and connective tissue disorder reaffirmed. Was prescribed factor, Amicar® and physical therapy.

  • Age 56: free factor trials end; I no longer have access to treatment.

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New Hemophilia Classifications For Women

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By David Clark, Ph.D.

We now recognize women can also have hemophilia, and it is imperative to define diagnostic criteria that apply to them. This is needed for insurance coverage of their treatment as well as their own recognition and self-respect. Imagine if you had to limp around on your damaged joints from doctor to doctor to find one to take you seriously. Too many women in our community have had just that experience. Now, we can give names to their conditions.

An international group of twelve hemophilia treaters and patient advocates has taken on this project under the Scientific and Standardization Committee (SSC) of the International Society on Thrombosis and Haemostasis (ISTH). The project was mainly supported by the NHF, HFA, and the Coalition for Hemophilia B from the U.S., as well as other hemophilia organizations around the world. There was no commercial support. The results were published in an article in the Journal of Thrombosis and Haemostasis on July 31, 2021. [See the complete citation at the end of this article.]

The results are shown in the table below. The same classifications are used for both hemophilia A and B. For factor levels up to 40%, women receive exactly the same diagnoses as their male counterparts. They are classified as severe/moderate/mild based on their factor levels. Above 40%, the tables turn. Men with factor levels above 40% are not considered to have hemophilia in many countries. However, women who are carriers with factor levels above 40% can still have a bleeding diagnosis.

The first thing to recognize is that the term “carrier” is now being returned to its proper definition. Carrier is a genetic description – it does not define a bleeding disorder. A woman is a carrier because she carries a mutated factor VIII or IX gene on her X chromosome that she can pass on to her offspring. She may or may not have a bleeding disorder. Carriers can have normal levels of factor VIII or IX.

Next, we need to discuss the international standard of 40% upper limit for hemophilia. In the U.S., we commonly use 50% as the upper limit for hemophilia and the lower limit for the range of normal factor levels. We recognize men with clotting levels up to 50% may still have mild hemophilia and may need treatment. In the rest of the world, men with levels of 40 – 50% are not considered to have hemophilia.

This gets more complicated because we know women can bleed even at levels up to 60%. We don’t know why they still bleed, but the study’s authors have recognized this and have given women two more categories. If a carrier has a level above 40% and does not have bleeding symptoms, she is classified as an “asymptomatic carrier.” However, if a carrier has a factor VIII or IX level over 40% (with no upper limit) but still has bleeding symptoms, she is classified as a “symptomatic carrier.”

This fuzziness in the over 40% levels could lead to situations where it is now the men who could have trouble getting treated. Going by the international classification, a man with a 50% factor VIII or IX level would not be considered to have mild hemophilia, even if he has bleeding symptoms. Yet, if he were a woman with a 50% level and bleeding symptoms, she would be a symptomatic carrier who might have a better chance of being treated. 

In addition, all of the categories are just approximations. It is the best we can do with our current state of knowledge. We know that about 15% of people (men and women) do not bleed according to their category of mild, moderate or severe, as determined by their factor level. For instance, some people classified as severe bleed like moderates. Some people classified as mild bleed much more heavily.

Another term seen is obligate carrier. This is also a genetic description, not a bleeding diagnosis. If you are genetically female (have two X chromosomes) and your father has/had hemophilia, you are an obligate carrier. That means you carry (have inherited) your father’s mutated factor VIII or IX gene. That’s just how genetics works. You may or may not bleed. Of course, the genetics can always mess up – that’s how we get hemophilia in the first place. However, it is extremely unlikely that when your father passes along his mutated factor VIII or IX gene, there is another mutation that actually fixes the gene.

One interesting point in the article is the estimate that for every male with hemophilia, there are 1.6 female carriers. Since many of these female carriers might have bleeding problems, there may actually be more women with hemophilia than men. Tell that to your doctor who says women don’t get hemophilia!

This is all based on averages, and no one is average! That’s why you always have to talk to your doctor about your individual case. No one should bleed, no matter their factor levels.

ABOUT THE AUTHOR

David Clark, PhD. is an independent consultant to the biotechnology, plasma, and tissue industries. He has 35+ years of experience in the development and manufacturing of plasma and tissue products, including factor VIII and factor IX concentrates, primarily with the American Red Cross. Dr. Clark holds a Ph.D. in chemical engineering from Cornell University.

REFERENCE

van Galen Karin PM, et al., A New Hemophilia Carrier Nomenclature to Define Hemophilia in Women and Girls: Communication from the SSC of the ISTH, Journal of Thrombosis and Haemostasis, 19(8), 1883-1887, 2021. https://pubmed.ncbi.nlm.nih.gov/34327828/

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His Blood... the Ink Inside My Veins

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By Sawsen Jamaleddin

The aroma of ginger and garlic swirls in and out of my nostrils, tap dancing inside a wok of hot oil, overpowering the sterility of bleach that has inhabited our house for the past year. The two women in our small kitchen are dressed in matching blue uniforms. They are stirring and whispering as the flame of the oven rises and relaxes against the hum of the microwave vent. The one with brown curly hair notices me and smiles. The other is so tall that I question the ceiling. But I have stopped questioning their presence. Hunger is stronger than my curiosity. Fear is better than knowing.

The tall one moves the wok back and forth with a grace that reminds me of a swaying palm tree. “How was school?” she asks, sprinkling small conversation between us, her eyes as warm as the heat fogging up the windows. I have no words to respond. At ten years old, my tongue refuses to work with my mind. Years of a lisp and speech therapy and I still can’t form the question that is on my mind. What is wrong with my father? I know she knows what I don’t.

She was there the day I opened my parent’s always-closed bedroom door and ran inside with a set of BIC pens that I bought for my father’s upcoming 43rd birthday. When I neared his bed, I did not recognize the skeletal body in front of me. Brown eyes that resembled mine opened, disoriented, turning into droplets of despair when he blinked at me. His dark skin looked faded in the light of sun, the cascading rays revealing an IV that protruded from his arm. He resembled a prisoner of war, starved, resigned. All I wanted to do was bandage the pain, cover his bones with my skin, drape my eyes from seeing, from imprinting the image of my father in front of me.

“Who is she?” he asked staring at me. Shock stood numbly between us. The set of pens fell from my hands, unwilling to write the tragic chapter unfolding.

It was then that I noticed movement in the corner, the tall woman making her presence known, as she stood up from the opposite side of the room and rushed to pick up the set of BIC pens off the floor. She put the jumbled pens in my palm and ushered me out of the room.

Weeks later, my father lost the war of life.

The kettle sputters and seethes. I turn the stove off and pour the water into a floral cup that has survived my move into wifehood. I open the white canister to get a tea bag when I suddenly feel a rush of liquid run down my legs. The vinyl tile in the small kitchen has become a pool of amniotic fluid. At twenty-two years old, I am going into labor for the first time. It seems that my unborn daughter is as patient as boiling water.

“Ahmad,” I call out to my husband of less than a year, “it’s time!” He sprints out of the room, then stands frozen by the murky water in front of him, a look of excitement and fear crisscross across his face. The ride to the hospital took less than ten minutes, but once we got there, labor seemed endless. After more than twenty-four hours of what-the-hell-was-I-thinking screaming, along with spurts of meconium leaking from my womb, an emergency c-section was the only option to deliver my baby safely.

Once the epidural was given, everything became hazy. All I remember before the blackness is hearing the doctor’s panicked voice in the background, “She’s losing a lot of blood.” When I woke up, it took me a while to orient myself to where I was. My husband was seated in a chair facing the window. My mother was seated beside him. My newborn daughter was in a bassinet beside my bed. I tried to move my arms to pick her up, but my limbs felt like spaghetti. Pain ricocheted throughout my body. I stifled my scream when a nurse came in and handed me pain medication. Then the doctor walked in.

He congratulated us and then explained I had lost a lot of blood during surgery. “I’m wondering if you have von Willebrand disease?” he questions me. Von-what? I thought. It was the first time I had heard that word. I shook my head no.

“You lost a lot of blood. I would like you to have a blood transfusion,” he advised. My mother stood up. “No,” she shook her head adamantly, “No blood transfusion.” Why not? I wondered for a fleeting second until brain fog and pain clouded my curiosity that I said nothing. The doctor sighed, “We’ll try an iron infusion instead.” My mother nodded for the both of us.

Depending on who you ask, childbirth seemed like a small pinch compared to a wisdom tooth extraction. Once my teeth were extracted, strong painkillers were prescribed, and I devoured them like M&Ms until I became aware that the more I took, the more my mouth bled. When the pain finally eased without medication, gauze pads still had traces of blood more than a month after the procedure.

During the follow-up appointment, I asked the dentist if the ongoing mouth bleeding was normal. “Everyone is different,” he answered. Instead of demanding he investigate my concern, I walked out with more gauze. It took another month for the bleeding to stop. I have never had another tooth extraction. However, I did have three more children. Motherhood is a dizzying merry-go-round.

A year after my daughter was born, my nephew also made his entrance, granting me the title of Aunt. It was a year of new beginnings, but it was also a year of numerous hospital visits. I couldn’t understand the unexplained bruises that riddled my nephew’s small body. It reminded me of a past life, one I couldn’t quite put my finger on.

After a light fall from a bunk bed when he was five years old, an egg-shaped bump on my nephew’s kneecap formed, making it hard for him to walk. My sister took him to the hospital to get some answers. After waiting nervously for some news, my mother called to share the diagnosis. “The doctors think he has hemophilia,” she said reluctantly. Although it was the first time consciously hearing that word, it felt oddly familiar. “Your father had hemophilia,” she explained, but didn’t elaborate.

That night I googled the word: Hemophilia. Bleeding disorder. I searched for the cure. Incurable. The next day my younger brother and I drove to the hospital together to visit my nephew. “You know Dad had hemophilia, right?” He asked, not waiting for my answer. Something about the way he braced his hands across the headrest made me sit up straight and pay attention to what he was about to say. Although he was younger than me by two years, he was closer to my mother, and I knew whatever he was going to say was going to be insider information. “You know how Dad died, right?” I said nothing. This question was something I’d been wondering about since the day he took his last breath. “He got HIV from a tainted blood infusion.”

Staring out at the twirling hands of a turbine while my father drove, his hands clutching the steering wheel so tightly his olive-colored knuckles looked as faded as the clouds that traveled with us. I never asked where we were going. I was simply happy to miss a day of school, excited to bask in my father’s presence since he was always on the go, even when it seemed like his limp made it hard for him to go the distance. For as long as I remember, my father always walked that way. It was more pronounced when he was standing for a long time. He never complained and I never questioned it, thinking he was just born that way.

But there are things I wish I had questioned then, things that my young mind wondered about, like the vials of medicine in his closet that were neatly stacked beneath his coats, the times he spent a few days in the hospital and came home without an explanation. Or the nurses who used to go into my parent’s room before I went to school, and when I would return, the smell of Asian food would waft through the house – sometimes I wonder if that is where my love for Chinese food came from.

Was I trying to hold on to the time my father was alive? To the moments when I didn’t need answers because he was still alive, and that was all that mattered? Or was I too afraid to wander into the truth for why there were nurses around the clock, and instead, chose to comfort myself with the food they fed me and my siblings? Sometimes I wish I could pause and rewind the years to get the information that medical records no longer contain. I wish I could ask my father just one question: Can you tell me all about you so I can know more about me?

After more tests, my nephew was diagnosed with severe hemophilia. He needed clotting factor. It was then that I examined my lifetime of symptoms. The excessive bleeding. The unexplained bruises. The joint pain. I requested genetic testing for myself as well as testing for my factor VIII level, along with my children. My children were cleared genetically, and their factor levels came back normal. But I held the mutation gene. My factor level came back at 42. I knew then that I too needed factor. I also knew that I too had hemophilia. Not only was it factored in my blood, but in my joints, the result of years without proper diagnosis and treatment.

However, getting diagnosed as a woman with a predominantly male bleeding disorder is like trying to convince a giraffe it is of average height. Or like trying to convince your blood to clot by just shouting at it. The struggle to be heard and be taken seriously is absurd, but painfully real.

Sitting in various waiting rooms has given me a newfound appreciation for HGTV and the Food Network. It has also given me an abundance of patience that seems necessary when dealing with a bleeding disorder. The last hematologist I visited when I was thirty-six told me it was a “mystery of life” why my joints ached. She gave me the classic carrier status associated with being the daughter of a severe hemophiliac. She discarded the results of my genetic mutation, discarded my low factor VIII level, discarded my low ferritin level, and basically told me my symptoms were imaginary.

If only that were the case, then I’d will my blood to clot, I’d will my restless legs at night to stop shaking, heck, I’d will Starbucks to deliver a few shots of espressos on the spot. And I’d request a lot of ice. Because my chronic anemia demands a cold wake-up call in the morning. The truth is if I were a boy, all these symptoms would ensure a quick diagnosis. Hemophilia. And swift treatment. Factor. But my double X-chromosome warrants a shoulder shrug and the concerned-for-my-mental-health stare from various hematologists.

During the pandemic, I learned about the Hemophilia Federation of America and joined my first virtual session. It was then I realized that my story was like many women with bleeding disorders. We have been made to feel like we don’t matter. We are often overlooked and underheard. When I finally received a referral to get diagnosed, I was ecstatic. But the catch was that the clinic was hundreds of miles away from my house. I knew it was the only option for me as a woman to get diagnosed.

Once I arrived at the clinic, I felt reassured. After asking me a few rounds of questions and performing lab work, I was sent home with hope. Once the results came in, I was relieved. Mild hemophilia. It was the truth I had waited for; it was the written proof validating years of pain.

But the reality is that getting diagnosed is not enough. Treatment must be implemented. Words of reassurance that I am fine from healthcare professionals do not stop the bleeding. They do not change history. The Hemophilia Holocaust took my father. If more care isn’t taken, more lives are at risk by not getting the proper treatment.

At thirty-nine years old, my joints ache. My body hurts. You can often find me resting in bed, reading a book, or complaining how depleted of energy I am. I feel a lot of my exhaustion is due to having to fight twice as hard with medical professionals, to warrant a proper diagnosis for my bleeding disorder and to receive treatment. It should not have taken hundreds of miles and numerous hospital visits to find a doctor who acknowledges women with bleeding disorders.

It is time that a woman is taken seriously. She knows her body. If she is seeking help from a medical professional, it is not because she likes to watch paint dry while watching DIY home remodeling shows, no matter how nice the shade, or watch how to cook pasta while wearing an adult diaper, praying the blood doesn’t leak through her clothes until she can meet with a doctor, only to leave empty-handed searching for the nearest restroom to assess the damage.

It is time the past stops bleeding into the present. It is time a woman is given the red-carpet treatment in the bleeding disorders community because she bleeds just as much as a man, if not more.

ABOUT THE AUTHOR

Sawsen Jamaleddin is American by birth and Palestinian by heritage. Sawsen earned her Bachelor of Arts in educational studies from Western Governors University and is a substitute teacher. She lives with her husband and four children. She enjoys writing and traveling and is excited at finally being able to connect with women who share her history in the world of bleeding diagnosis. Sawsen was reintroduced to her family history of hemophilia when she was 29 years old. She is passionate about advocating for women in the bleeding disorders community.

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How to Avoid Access-to-Care Issues When Initiating New Coverage

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By Justin Lindhorst

New Year, New Plan 2 (1).jpg

The beginning of the year, for many, can mean initiating new health insurance coverage. When initiating new coverage, individuals living with a chronic health condition sometimes experience challenges that can delay access to prescribed therapy.

This article outlines how specialty pharmacies like BioMatrix can help patients avoid access-to-care issues, provides tips and best practices patients can implement, and includes links to helpful resources.

Specialty Pharmacy Support

Anyone who has a chronic health condition can identify a time they’ve had to spend a significant amount of time on the phone with their health insurance plan. Unfortunately, patients share it’s not entirely uncommon to be told one thing by one customer service representative, only to be told something entirely different by another.

Specialty pharmacies can help break down barriers to care and cut through red tape by conducting a thorough benefits investigation, facilitating access to financial support programs, and working with your insurance plan and medical team to provide support for prior authorizations and appeals.

Specialty pharmacies such as BioMatrix employ staff who are very well versed in promptly identifying and resolving coverage issues specific to your health condition. The first tool at their disposal is conducting a comprehensive benefits investigation. The benefits investigation provides a detailed outline of coverage specific to your therapy, including whether it is covered under the medical or pharmacy benefit, whether the medication requires prior authorization, your financial responsibility, and what specialty pharmacy service providers are available under the plan.

After the benefits investigation is complete, the specialty pharmacy can refer you to available and appropriate patient assistance programs to reduce financial barriers to care. They can also work together with your medical provider and health plan to obtain timely prior authorization for service and assist when and if an appeal is necessary.

Asking your provider to send a referral to BioMatrix or another reputable specialty pharmacy is a good first step in avoiding coverage issues.

Maintaining Access to Care: Tips and Best Practices

There are also steps you can personally take to avoid access issues. Following the guidelines below can go a long way in resolving potential barriers.

Be Proactive

Don’t wait until you are critically low on medication to place your first order with your new health plan. Placing your order in a timely manner will give your medical provider and specialty pharmacy enough time to resolve any issues before it potentially disrupts your care.

Reach Out to a Social Worker

If you are experiencing or anticipate coverage issues, social workers can be a great source of support. Many medical providers supporting patients with rare or chronic health conditions engage social workers as part of a multidisciplinary care team. Ask your provider if there are social workers available to assist with coverage or other issues.

Keep Detailed Records

When you need to contact your insurance provider regarding any issue or concern, it’s very important to document every call. Keep notes on the following: date and time of the call, the reason for the call, name of the person you spoke with, the result of the call, reference number, and any impact on your health resulting from the issue/call.

Document Medical Necessity

Work with your medical provider to document the medical necessity of your treatment. Having robust documentation on file can speed the resolution of issues related to prior authorization, denials, step therapy, or appeals. Ask your provider to write a letter on official letterhead identifying your diagnosis, the therapy you have been prescribed and why, any previously failed treatments, and the consequences of not having access to your prescribed therapy.

Include medical records, clinical evaluations, or other supplemental documentation supporting your diagnosis/treatment. Keep a copy for your personal records, and request a copy be kept on file with your specialty pharmacy.

Check for Copay Accumulators

If you are using a manufacturer or other third-party copay assistance program, determine if your plan is using a copay accumulator. Verify with your specialty pharmacy whether payments are being received from the copay assistance program you are enrolled in.

Once you’ve verified payments are being made, check your Explanation of Benefits (EOB). If the payments from your assistance program are not being applied to your out-of-pocket costs, your plan may be using an accumulator program.

Understand the Appeal Process

Every plan’s process for an appeal varies. Check with your insurance provider to determine their specific appeal process. Don’t hesitate to involve your medical provider and specialty pharmacy for additional assistance.

Understand the denial of the claim by investigating the explanation of benefits statement (EOB). There is often a code noted on the EOB if there is a denial of coverage or a letter with codes and a key code to decipher what the denial was based upon. Identifying the code will allow you to see if it was a true denial or a basic miscoding by the provider or insurance.

Keep all documentation including referrals, doctor’s notes, medical history, medicines or prescriptions, and notes from all contact with providers or insurance regarding the claim. The burden of proof is in your hands.

Organize Your Paperwork

If you have the aforementioned documents, you might also need to write a letter to the insurance company (written appeal). Keep documentation of your claim number, insurance information, provider/services information readily available. Request a reference number and employee name for every phone call to your insurance plan. 

Be cognizant of the appeal timeline (30/60/90 days in many cases). Be sure to file all paperwork and make calls within the timeline allowed. If your appeal is denied a second time you may be able to file another appeal. Some aspects of the ACA mandate states allow an external review process for denied claims.

Follow up on every call, text, email, and mail document you submit. Be sure it has a record of receipt.

Navigating new coverage can be challenging for patients who require specialty medication. Avoid any potential disruptions in care by leveraging every resource at your disposal. Work with your specialty pharmacy and medical providers early in the month to identify and resolve issues before they become an emergency. 

Follow the guidelines as outlined above any time you reach out to your health plan. Working together at the start of a new plan can make all the difference in maintaining uninterrupted access to the therapy and services keeping you healthy.

Useful Links

Centers for Medicare and Medicaid Services: Official website for the Centers for Medicare and Medicaid Services (CMS): https://www.cms.gov/

E-Health Insurance Glossary: eHealth provides a list of common insurance terms on their website: https://www.ehealthinsurance.com/health-insurance-glossary/terms-c/

Employee Benefits Security Administration: https://www.dol.gov/agencies/ebsa

National Association of Insurance Commissioners (NAIC): NAIC is a standard-setting and regulatory support organization. Their website includes a map that will allow you to determine the insurance commissioner in your state: https://content.naic.org/

Patient Advocate Foundation: The Patient Advocate Foundation (PAF) is dedicated to improving healthcare access. Their website offers education, assistance, and resources related to healthcare coverage: https://www.patientadvocate.org/

United States Department of Labor: Includes information on ERISA and COBRA plans: https://www.dol.gov/general/topic/health-plans

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

Reference

National Hemophilia Foundation. (2021). Comprehensive Medical Care. https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/comprehensive-medical-care

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Gender Equality in Bleeding Disorders

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By Danielle Nance, MD

Bleeding symptoms in women have been documented since ancient times and, in modern literature, recognized since the early 1900s.

Women with a genetic mutation for hemophilia have a 40-60% chance of having bleeding episodes during their lifetime requiring treatment and an 80% chance of having heavy menstrual bleeding. Due to X chromosome inactivation, one normal X chromosome is not always enough to produce a full amount of factor.

Even in the same family, the severity of bleeding symptoms in women varies a lot because of X chromosome inactivation, and we know now that bleeding symptoms don’t correlate with factor levels the same way they do in men. Treating bleeding symptoms in women when their factor levels are 30-50% is not always reimbursed by insurance. This can be confusing to treating physicians who were taught to treat based on “the numbers.” Treatment with intravenous medication is seen as invasive and therefore seen as “too difficult” or too expensive to use unless the bleeding is severe.

In men, even a minor bleed is no longer tolerated. Bleeding symptoms in women are often minimized or even dismissed by medical providers. As we understand more about bleeding symptoms and access to medical treatment becomes more widespread, more and more women are being offered treatment for their bleeding symptoms. 

Why should any bleeding be endured in women? Women have the increased burden of bleeding from their ovaries, uterus and reproductive organs during pregnancy, delivery and postpartum.

This issue is dedicated to women who bleed and celebrates the stories of those who have courageously talked about their personal symptoms and challenges. As women, we can help improve care for all by continuing to report bleeding symptoms and insisting on getting imaging with CT scans, MRIs, and ultrasounds to document pain and find out if the discomfort and swelling are from bleeding.

As a physician who treats women with bleeding disorders, I ask that each woman keep a calendar and write down symptoms of bleeding, especially the ones that disrupt home, work and leisure activities. Bring the calendar with you to your clinic appointment. If treatment is refused, be brave enough to ask why and ask for more studies so you can learn about your body and how to better care for yourself. Not all pain is from a bleed, and not all pain needs to be treated. Pain provides a signal for investigation. The more we know about symptoms, the better we can work through them towards better health.

May joy be with you in your journey.

ABOUT THE AUTHOR

Dr. Danielle Nance is a hematologist at Banner MD Anderson Cancer Center in Gilbert, Arizona. As a physician of more than 17 years, Dr. Nance shares, “I seek to bring accessible, expert care to each of my patients. I believe in advocacy for patients with rare diseases, access to care and insurance, and improving the patient experience.”

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Patient Navigation: Understanding Surprise Billing

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This article defines “surprise billing”, offers scenarios where a surprise bill may occur, and also provides brief information on the No Surprise Act.

What is “surprise billing”?

Specific to healthcare, a surprise bill refers to a “balanced bill” where a provider bills you, the patient, for the difference between their charge and the amount paid by your health plan. A surprise bill is usually the result of an out-of-network charge. When a cost is in-network, the provider and the insurance plan have a previously agreed-upon arrangement for what the provider will charge and what the insurance will pay. With an out-of-network cost, no such agreement exists: the provider will charge what they feel is appropriate, and the insurer will pay what they feel the service is worth. When these two figures do not align (usually with the provider asking for more than the insurer is willing to pay), the patient is billed by the provider for the balance difference. It will then become an unexpected or “surprise” bill to you, the patient.

In what scenarios might you receive a surprise bill?

There are numerous situations that might lead to a surprise bill, but a majority of these are related to emergency (ER) services. In the ER, providers often have to act quickly, and they may be using contracted help—such as an X-ray that is read by an outsider, third-party source. In another scenario, you might have a blood draw where the phlebotomist is in-network, but the lab result is farmed out to a service that happens to be out-of-network. Another common scenario may involve a procedure where a specialized provider involved in the procedure (like an anesthesiologist) may be out-of-network, even if the procedure facility and the primary physician/surgeon is in-network.

What can I do if I get a surprise bill?

Recently passed legislation protects patients from surprise billing. The No Surprise Act offers consumers billing protections when getting emergency or non-emergency care from out-of-network providers at in-network facilities. It also provides out-of-network protection from air ambulance services.

The No Surprises Act, however, does not protect you if your provider (ER, hospital clinic, or other facility) is out-of-network.

What can BioMatrix do to help with a surprise bill? 

If you think you are a victim of a surprise bill, we can determine if the bill in question falls under the patient protections of the No Surprise Act. We can also help guide you to a resolution.

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Patient Navigation: Understanding Step Therapy Mandates

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This article defines “step therapy,” provides helpful information on the patient impact of step therapy programs, and includes resources to learn more about step therapy.

What is step therapy?

Step therapy is a utilization management technique for drugs that prevents the patient from accessing prescribed treatment and instead mandates a therapy as dictated by the insurance plan. In laymen terms, it means trying “less expensive” drug options before “stepping up” to a more costly drug therapy. Step therapy plans could dictate that a patient begin treatment with a cost-effective drug before progressing to a more costly drug therapy if the initial treatment is proven ineffective. Some providers refer to this as a “fail first” plan.

How does step therapy impact me?

Step therapy is more prevalent in commercial plans and may or may not impact you depending on your particular health plan. If your health plan implements a step therapy mandate, you may have to utilize other medication before receiving approval for a more expensive therapy. You will have to document the ineffectiveness of the preferred, less expensive therapy before being permitted to switch. In some limited cases a doctor’s intervention may allow an override of the step therapy program.

For many chronic and rare conditions, a less-expensive (often “generic”) medication may not exist. In this scenario, a health plan may prioritize the most cost-effective medication as the starting step for medication. The health plan may dictate medication choices based on cost savings and may even deem some of the products as interchangeable. Significant documentation is encouraged in order to appeal to use another medication. This can become problematic especially if you change to a new health plan which has instituted a step therapy program; despite perhaps a long history of using a particular medication, the plan could dictate a medicine change.

For government programs such as Medicare and Medicaid, step therapy programs are less prevalent, but caution must be taken when selecting plans. Because Medicare Advantage (MA) plans are managed by commercial payors, they may be more at risk of implementing step therapy programs, but the MA plans must adhere to Part B guidelines for factor medications and must ensure they do not disrupt ongoing Part B drug therapies for beneficiaries. Under new policy guidelines, step therapy can only be applied to new prescriptions or administration of Part B drugs for beneficiaries who are not actively receiving the affected medication. This means that no beneficiary currently receiving drugs under part B will have to change their medication.

What can BioMatrix do to help with a problem with step therapy?

If you’re facing step therapy, our access team at BioMatrix can help you navigate the process. We can help you identify, collect, and submit the documentation required by your health plan and can help file for an exception in order to maintain or return to your original therapy.

Where can I learn more about step therapy?

The Alliance for Patient Access has created a succinct overview for understanding step therapy. Scan the QR code or visit the link to watch.

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Patient Navigation: Patient Assistance Programs (PAPs)

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This article defines patient assistance programs (PAPs) and provides helpful information for who may be eligible.

What are patient assistance programs (PAPs)?

Patient assistance programs (PAPs) help people without health insurance and those who are underinsured. These programs are often managed by pharmaceutical companies, nonprofits, and government agencies. PAPs may cover free or low-cost medicines if you don’t have insurance, have lost your insurance, or are underinsured and can’t afford your medicine.

Pharmaceutical manufacturers often sponsor PAPs that provide financial assistance or free product for eligible individuals. PAPs may also provide assistance to Part D enrollees and interface with Part D plans by operating “outside the Part D benefit” to ensure separateness of Part D benefits and PAP assistance.

Who should enroll in PAPs?

Anyone who is without insurance, is underinsured, and is unable to pay for their medication should consider a PAP. PAPs were created to ensure continued access to life-saving medicines. 

Are PAPs the same as copay assistance programs like copay cards?

No. PAPs are intended only for the uninsured or the underinsured. PAP programs provide drugs free or at a discount to patients. Copay assistance programs are for commercially-insured individuals and work in tandem with the patient and the insurance plan to help cover a medication’s out-of-pocket cost.

What can BioMatrix do to help connect patients to these resources?

BioMatrix can provide information on available PAPs related to your medication and can connect you to those resources.

Stay informed on the latest trends in healthcare and specialty pharmacy.

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Patient Navigation: Understanding Medicare Eligibility

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This article defines Medicare eligibility and provides helpful information for navigating Medicare eligibility.

Am I eligible for Medicare?

You are eligible if you are 65 or older, under 65 with a disability, or have end-stage renal disease.

When and where can I sign up for Medicare?  

Most people sign up for Part A and B at age 65. You may begin the enrollment process 3 months before your 65th birthday. If you are starting your Social Security benefits and are approved, Part A coverage will be granted automatically, but you will have to enroll in Part B by filling out Form CMS-40B.

You can enroll in Medicare by visiting Social Security and signing up online at www.ssa.gov/benefits/medicare/. You can also enroll by calling Social Security at 1-800-772-1213. 

For persons under 65, everyone eligible for Social Security Disability Insurance (SSDI) is also eligible for Medicare 24 months after being awarded disability benefit entitlement. 

Thereafter, the general enrollment period and when changes may be made to plans is Jan. 1 – March 31 every year.

What if I am still working at 65?    

If you or your spouse are still working at a job with more than 20 employees and have insurance, you can wait until you or your spouse stops working (or lose health insurance if that happens first), and you won’t pay a late enrollment penalty for Part B. Your job-based insurance pays first, and Medicare pays second. If you or your spouse’s employer employs fewer than 20 persons, you need to verify with the employer if you need to sign up for Part A and B because your job-based insurance may not cover the costs for services. If you or your spouse are still working and have non-job-based insurance (Medicaid or ACA Marketplace), the rules vary on coverage with Medicare, and you will need to answer a few questions to determine your coverage. 

What if I return to work but am an under-65 disabled Medicare recipient?  

Social Security’s Ticket to Work program allows beneficiaries an opportunity to resume their working careers. If you earn under $1350 during the Trial to Work period, your benefits will remain intact for those 9 months. If you return to work and begin earning more than $1350, you will lose your SSDI benefits, but you will retain premium-free Medicare Part A and B coverage for up to 93 months. After 93 months, beneficiaries will then have the opportunity to purchase Part A and B coverage if they continue to have a disability. 

What can BioMatrix do to help with my Medicare eligibility?  

BioMatrix can check your Medicare benefits and let you know about your eligibility and anticipated coverage. From there we can provide additional information based upon your needs. 

Stay informed on the latest trends in healthcare and specialty pharmacy.

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Patient Navigation: Understanding Medicare Coverage

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This article outlines Medicare coverage including Original Medicare Parts A and B; prescription drug coverage Part D; Medigap/Supplemental Insurance coverage; and Medicare Advantage.

What is Medicare Part A and B, and how much will I pay?

Part A is considered hospital insurance and covers in-patient care. There is no premium if you have 10 years of qualified work. For hospitalizations, a per episode deductible is in place for $1600 in 2023. Once met, days 1-60 are covered at 100%; then Medicare sets the coverage amount at days 61 and beyond.

Part B is considered medical insurance and covers physician services, outpatient care, home health care, and most infusion drugs, including factor. The average premium is $165 for 2023 and the deductible is $226. At that point, Part B pays 80%. Factor therapies fall under Part B coverage.

What is Medicare Part D and how much will I pay?

Part D is the prescription drug plan. Plan costs are based on income and range from $0-$77.90 with the average plan costing $43/mo. Your out-of-pocket costs depend upon your prescription as Medicare uses a tier system to determine patient cost. The yearly deductible before Medicare begins paying anything is $505 for 2023. When catastrophic coverage begins (at $7400 in shared costs), you will pay 5% of drug cost for the rest of the year.

What is Medigap and how much will I pay?

Medigap is supplemental coverage for costs not paid for by Medicare Part A or B. This includes the 20% of Part B but does not include the 5% for Part D plans. It also covers foreign travel, which Medicare does not cover. These plans are state based and managed by private insurers. Available plans are A-N and fluctuate in cost from $50-$500/mo. depending on coverage options.

What is Medicare Advantage, or Part C Medicare, and how much will I pay?

Medicare Advantage plans are managed by private companies and offer inclusive Part A and B coverage. Many add prescription drug coverage (Part D), and some include perks like gym and health behaviors discounts. These will be familiar and are run very similar to private insurance plans. Premiums, copays, and deductibles vary from plan to plan. The average premium in 2022 was $18/mo. The average out-of-pocket cost for Medicare Advantage subscribers was $4,972.

Should I choose original Medicare or Medicare Advantage?

This depends. Medicare Advantage can have lower out-of-pocket costs, but their networks are limited and may not include all the providers you need. It’s also more difficult to change to other plans once an Advantage plan is selected; certain penalties may apply, and you will lose guaranteed issue for Medigap plans should you wish to return to Original Medicare. You have to assess your healthcare needs, the access to providers in the Advantage network, and the savings you may (or may not) have with an Advantage plan.

What can BioMatrix do to help with Medicare?

BioMatrix can check your Medicare benefits and let you know what your anticipated costs will be based on your plan choices. From there we can provide additional information based upon your needs.

Where can I learn more about my Medicare benefits?

You can call Social Security at 800-772-1213 or access the handbook here.

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Patient Navigation: Understanding Medicare Coverage Gaps

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This article explains the coverage gaps in Medicare Part B and Part D plans, including the “donut hole.”

What are the primary coverage gaps within Medicare?

Each Medicare plan comes with a deductible which must be met before Medicare begins paying. Part B has a coverage gap of 20% where Medicare only pays 80% for provider services (including factor therapies) once the deductible is met. Part D has a gradient of limited coverage once the deductible is met and until the subscriber enters catastrophic coverage.

What is the Part B coverage gap?

In Part B plans, Medicare covers 80% of costs for providers, including procedures and Part B infusion therapies like factor. To cover these costs, you may be eligible for a Medigap/Supplemental Insurance policy. Depending on your income level and resources, you may also be eligible for assistance through the Qualified Medicare Beneficiary (QMB) program or the Specified Low Income Medicare Beneficiary (SLMB) program.

What is the Part D coverage gap?

Often called the “donut hole,” this is a euphemism to describe a coverage gap where, after Medicare has paid a portion of shared costs, Medicare temporarily limits its coverage. During that stage of coverage, you may pay more for drugs than you had previously. When the coverage gap (donut hole) ends, Medicare will require a flat 5% shared cost on prescription drugs.

In Part D, you will first pay the deductible, $480. At that point, Medicare will begin sharing the cost based on their drug tier cost-sharing formula. Medicare will share the cost until $4660 has been reached, and the donut hole begins. From there, you will pay 25% of the drug cost until the shared cost reaches $7400. At that point, you are in “catastrophic coverage”, and Medicare will pay 95% of drug costs; you will be responsible for 5% for the rest of the year.

What can BioMatrix do to help with the coverage gaps in Part B and Part D?

BioMatrix can check your Medicare benefits and let you know what your anticipated costs will be based on your plan choices. From there we can provide additional information based upon your needs. By looking at your income and resources, we can identify support plans you may be eligible for.

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Patient Navigation: Medicare Coverage Resources

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This article outlines the four Medicare savings programs and provides helpful information for determining eligibility.

What programs exist to help cover my Medicare costs?

Medicare offers 4 general programs to help patients cover their out-of-pocket costs, which include premiums and cost-sharing for provider services and medications. Program eligibility is based on income and resources (checking and savings accounts, stocks, and bonds).

  • Qualified Individual Program (QI): Beneficiaries earning less than $1549/mo. and resources under $8400 may be eligible for the Qualifying Individual program (QI). This helps pay for Part B premiums only. States approve this on a first come, first serve basis. Ineligible if you qualify for Medicaid. Can pay for up to 3 months retroactively.

  • Qualified Medicare Beneficiary Program (QMB): Beneficiaries are eligible for QMB with incomes less than $1153/mo. and resources under $8400. This program covers premiums for Part A and Part B, deductibles, coinsurance, and copayments for services Medicare covers. It does not offer retroactive payment.

  • Specified Low-Income Medicare Beneficiary (SLMB): Beneficiaries are eligible for SLMB with incomes less than $1379/mo. and resources under $12,600. This covers Part B premiums. Can pay for up to 3 months retroactively.

  • Qualified Disabled Working Individual Program (QDWI): Disabled beneficiaries are eligible for QDWI with individual incomes less than $4615/mo. and resources under $4000. This pays for Part A premiums only.

What other resources exists to help cover my Medicare costs?

Several non-profits offer resources.

  • Accessia Health (800-366-7741) helps cover Medicare premiums.

  • The PAN Foundation (866-316-7263) helps cover premiums and out-of-pocket costs

  • The Assistance Fund (855-845-3663) helps with copayments, coinsurance, and deductibles.

How can I cover my out-of-pocket costs if I am ineligible for any of the Medicare Savings programs?

The primary step would be to see if you are eligible for a Medigap/Supplemental Insurance policy. This covers Part A and B costs not supported by Medicare.

What can BioMatrix do to help with Medicare coverage resources?

BioMatrix can check your Medicare benefits and let you know what your anticipated costs will be based on your plan choices. By looking at your income and resources, we can identify support plans you may be eligible for. We can also investigate your state rules regarding Medigap coverage plans and determine your eligibility.

Where can I learn more about the Medicare savings programs?

You can learn more about Medicare savings programs here at this link.

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Patient Navigation: Understanding 504 Plans

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This article defines 504 accommodation plans and indicates who should consider them.

What is a 504 accommodation plan?

A 504 accommodation plan is part of a federal law that allows children with special needs such as chronic health conditions to better access learning experiences at school. It is different than an IEP plan. A 504 plan provides additional assistance in the classroom as well as modifications and services that ensure a child’s access to education is equal to the other students. The law was designed to protect the rights of individuals with disabilities in programs and activities that receive federal financial assistance from the U.S. Department of Education. Section 504 provides: “No otherwise qualified individual with a disability in the United States . . . shall, solely by reason of her or his disability, be excluded from the participation in, be denied the benefits of, or be subjected to discrimination under any program or activity receiving federal financial assistance.”

The Section 504 regulations require a school district to provide a “free appropriate public education” (FAPE) to each qualified student with a disability who is in the school district’s jurisdiction, regardless of the nature or severity of the disability. Under Section 504, FAPE consists of the provision of regular or special education and related aids and services designed to meet the student’s individual educational needs as adequately as the needs of non-disabled students are met.

Who should consider a 504 accommodation plan?

Anyone with a chronic condition who receives education from any federally-funded program should consider setting up a 504 accommodation plan. This provides a safety net for the student and ensures minimal disruptions in a student’s education regardless of their condition, which may result in time missed or other school-related disruptions.

What can BioMatrix do to help with 504 accommodation plans?

BioMatrix can provide help with understanding the 504 accommodation plan and work with you and your school to put a 504 plan in place.

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I’m Not Giving Up Yet!

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By Edward Burke

My maternal grandfather had severe hemophilia A. He was injured while working on a construction site. The doctors thought he was having an appendicitis attack, but he was actually bleeding into his abdominal cavity and consequently passed away at age 35. This was when my family became aware of the hemophilia in our lineage.

Since my mom knew she was a carrier of hemophilia, she explained to my dad that if they had sons, there was a 50/50 chance of them having severe hemophilia. Sure enough, all three of us had it! When we were young, we would often wake up at night with an elbow hurting or blood everywhere from a tooth coming in. My parents had their hands full.

I was the first to self-infuse in my family after I learned at hemophilia camp. I was always giving my brothers their doses as well!

My brother Michael was four years younger than me. He went out for high school wrestling. At a match, he made a 13 second pin in the heavyweight division, making the sports page of our local paper. Our pediatrician, who never treated us for hemophilia, saw the news article and called the school. He alerted them that my brother should not be allowed to wrestle due to his hemophilia. Instead of being applauded and congratulated, Michael was thrown off the team. 

For two years in my early 20s, I was secretly part of a men’s ice hockey league. Mom didn’t know it, but my dad did, and would come watch me play. He would remind me,  “Don’t tell your mother!” While my mom watched TV, I would drag my bag of hockey pads and sticks upstairs. One night when I came home, she decided to investigate what all the noise was about. When she saw me with my gear, she said, “What’s this?” I told her I had good news and bad news. She asked, “What’s the bad news?” I told her I played ice hockey. She said, “Oh really? What’s the good news?” I told her, “I’m team captain, and we’re on a championship run!” She looked at my father to ask if he knew about this, but he was out of the room and up the stairs! He knew when she saw the hockey equipment he had better run! During a game, my knee twisted up, and it just wouldn’t stop bleeding. That was the last time I played. The injury resulted in my first knee replacement.

My older brother James passed away at 24 years old in June of 1980 following an accident. My father was destroyed – he died of a broken heart just two years later.

Michael and I were diagnosed with HIV at our Philadelphia treatment center in 1985. My mother was already crying when we walked into the room. We were given the news that we tested positive for HIV and that we had about seven years to live. I asked, “So you’re saying by 1992, my life will be over?” The doctor explained those were the statistics. In a way he was right… in October 1992, I got married! Joking aside, thirty years with my wife and she has seen a lot of this too, having attended many blood brother funerals. Sadly, my youngest brother, Michael, passed away at the age of just 35 in 1997 – his death tagged as hemophilia-related AIDS.

My mom is currently 88! She is still very much a hemophilia mom. Everything her sons went through continues to weigh heavy on her heart.

Sixty years of life with hemophilia and I have a pretty good pain threshold, but the bleeds hit hard. One time I was driving my friend to the airport and almost took my car out of the lane because of a breakthrough bleed in my elbow. The bleed was so bad I couldn’t move my hand to steer the wheel. Taking a weekend trip to attend a meeting or event and I feel the effects of aging. I was in Nashville, returned home and waited three days for my knees and ankles to get back to normal just from being on foot and walking around the city.

As we age, we learn things like pain may feel like a bleed, but it’s not a bleed—it might be arthritis or just a muscle ache. It feels like I wake up with something every day. I can still bleed into my artificial joints! The pain is sometimes intolerable.

Living long enough to have arthritis is not something I had banked on. I’ve had all these target joints and some just don’t work as they should—like my right pointer finger that doesn’t bend like it’s supposed to.

But the most difficult part of growing older is when we lose a friend. I try to stay close with the friends who are still here. Everyone young enough not to have been exposed to tainted clotting factors should watch the movie And the Band Played On. It will serve as a reminder that the “hemophilia holocaust” shouldn’t have happened to our community. Even with advances in medical treatment, HIV/AIDS and hepatitis C continue to claim the lives of people in our community who were exposed to tainted blood products in the 1980s.

One misconception is that people with hemophilia have “thin” blood so they can’t have heart problems – WRONG! My second AFib (Atrial Fibrillation) put me in the emergency room. My daughter was outside the ER yelling at the medical staff to let her in with my clotting factor because although she didn’t know what was wrong with me, she knew I needed to get factor. The cardiologist put me in the ICU and told the nurse to start blood thinners immediately. All I could say was, “HOLD ON, I AM A BLOOD THINNER! I DON’T CLOT!” My cardiologist told me, “Hemophilia really puts a kink in the armor and changes how we can help you.”

In addition to AFib, I have psoriatic arthritis. It’s difficult for a cardiologist or rheumatologist to treat with their go-to medications. For example, the medication I take for the psoriatic arthritis treats the symptoms, but it also lowers the immune system. That complicates things when combined with my HIV medications. As we grow older, other medications may be problematic when combined with medications we take for hemophilia and HIV.

Many times, I have been wakened in the middle of the night. It’s my daughter calling. Every three or four weeks, she wakes up from a bad dream and has to check on me.  I tell her I have no plans to go anywhere anytime soon and that I feel fine. It’s heartbreaking to know your daughter is so concerned about your health that it keeps her up at night.

In my retirement, to stay busy and make a little extra money, I signed up with a talent agency and have been doing movie parts and voice overs, and I play guitar and have done performances. I enjoy trying to entertain others. My dream for retirement was to keep playing and entertaining, but it has become more difficult as my left hand’s ability decreases as the arthritis increases. I often ask myself, “Is it a bleed or is it arthritis?”

These are the things we deal with as we age, but we must keep living life to the fullest! I am still active in the bleeding disorders community and will participate in a hemophilia walk in the fall. I enjoy spending time with my wife and watching my much-too-beautiful 21-year-old daughter grow into a mature young woman.

My advice to the younger set is to always stay positive! Be active, and stay engaged in the bleeding disorder community. Make friends. Reach out to your community brothers and sisters when you’re having a problem—they will always be there for you! My close blood brothers and I have been saving each other’s lives and sanity for years!

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Aging Gracefully

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By Bob Murdock

One of the most ironic comments a doctor has ever made to me was about five years ago. She said, “Good news – bad news. The good news is hemophilia has become a very manageable and treatable disease. Most likely, you are not going to die from a bleeding episode and will live a normal life span. Bad news, now that you’re going to live a longer life, you will most likely die from a heart attack, cancer, stroke, etc.—medical issues that affect most adults.” My response was, “So basically nothing has really changed.”

I feel blessed to be a part of my generation of hemophiliacs because we have witnessed an incredible amount of progress in the treatment of our bleeding disorder. Advances we were never sure we’d see are here. As a result of living longer, I have developed moderate/high blood pressure and had an AFib event for which I now take daily medications. 

I also never thought I’d see the day I would have to modify my diet because a cardiologist told me to eat healthier, exercise, and stay active. The diet part stinks because I had to cut back eating the things I truly enjoy. I’ve had to learn the term everything in moderation. I used to eat whatever I felt like and went happily on my way. 

All that said, I feel very fortunate to have lived long enough to have these issues to deal with. I am able to live a relatively pain-free life and can do most of the things people my age can do. All is good in my world!

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Growing Older Is a Complex Subject

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By Joe Markowitz

Let’s start with the growing old part. I was born in 1950, and some of my first memories of doctors were from being treated in emergency rooms. However, my first memory of a meaningful conversation with a doctor was when I was visiting a hematologist in Yonkers, New York, with my parents. He told me about a hemophilia patient who died from licking an envelope. I have not licked one in about 60 years. The other bit of news he shared was that I’d probably die in my 20s.

So, doctor, I guess you were wrong. I’m still here at 71.

There are two aspects of growing old with hemophilia that I feel are worth discussing. Hemophiliacs of my generation generally have arthritis and experience pain and mobility issues. However, with the highly effective products now available, the stress caused by hemophilia is no longer related to bleeding. The stress is from the decades-long degeneration of joints that bled back 50 years ago, mostly before college. 

Surgery, unimaginable several decades ago, is now fairly routine as long as the expertise of your hemophilia treatment center is behind you. I’ve been lucky enough to have bilateral knee and one hip replaced, so I am no longer experiencing joint pain. The surgeries were accomplished with no bleeding, but lots of factor, including during the post-surgery physical therapy period.

Then there’s the second aspect of growing old, the aspect unaffected by hemophilia. As we age, our bodies seem to find new ways to torment us. As a kid, I thought having hemophilia meant I wouldn’t have any other bad medical things happen to me. What I didn’t understand is that at about the same time you get on Medicare, your body wants to take advantage of the great insurance and dumps new ailments on you.

Look around at men in their 70s, and you can be sure each one has at least one of these conditions: high blood pressure, high cholesterol, enlarged prostate, vision and hearing problems, arthritis or diabetes. These conditions are not unique to hemophiliacs but the result of living a long life.

Having hemophilia, with or without a co-morbidity, complicates the medical intervention for some of these conditions. For instance, if a person with hemophilia has AFib, aspirin and blood thinners are not the go-to treatment. Many arthritis medications have caused stomach bleeds that can be made more complicated with a bleeding disorder.

So maybe growing old with hemophilia requires more caution and planning than most people need. But we ARE growing older and leading full and happy lives. And that is certainly a great, previously unexpected outcome!

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Growing Older: Taking It Day-by-Day

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By Warren P. Ingram

In the late 1950s and into the 60s, good medical care was not always equal for people of color in the south. Wisely, my parents decided to become career military in the Air Force so they could get the care I would need.

When I was 3, my father received orders to deploy to Okinawa, Japan. It was while living in Okinawa that my memories of bleeding episodes began. Treatment consisted of fresh frozen plasma and long hospital stays.

My parent’s first major scare with me happened at about 5 years old. I had been playing outside, fell and bumped my head. Later that day my mom reported I was not myself, becoming lethargic and incoherent. They brought me to the USAF Military hospital where I went into a coma lasting 3 days due to a brain hemorrhage. On the 4th day, my mother recounts that upon her arrival she found me sitting up in my hospital bed eating a bowl of cereal. Her prayers had been answered.

In 1964, we moved to Otis Air Force Base on Cape Cod, Massachusetts. I continued to receive care at military hospitals until my father retired from the USAF in 1970. Between 1964 and 1970, I averaged two weeks per month in the hospital to receive fresh frozen plasma along with many casts and braces. It was later determined the casts and braces were not an appropriate treatment as they caused many issues for my knees.

My target areas were knees, hips, elbows, wrists and shoulders. At 13 years old, I started on home infusion treatment which changed my life. I was given independence, and my life seemed almost as normal as someone without hemophilia.

At 18, it was determined I would eventually need hip replacements. As a teen, working was a challenge. I learned early on that physically challenging jobs were not going to work for me, especially if I had to be on my feet. I also learned I needed a job that offered generous health benefits. In 1983 I found a great opportunity with AT&T that provided a good salary and great benefits. 

In 1985, I found out I had contracted HIV through contaminated factor VIII. This was devastating news. I was also experiencing a lot of bleeds at that time, so bad that I had to use crutches to go to work. This led me to have a bilateral hip replacement in 1986. The surgery was a great success. I no longer bled into my hips and regained a lot of mobility.

During these years, however, I lived with a lot of stress and anxiety because there was no good treatment for HIV. I saw it as a death sentence. Except to get the necessary treatment, I did not want to associate myself with hemophilia and didn’t have any relationships with other hemophiliacs.

Eventually, I was transferred to Alpharetta, Georgia for a new position with AT&T. I was doing well in my new surroundings, but HIV was still in uncertain territory. I decided to join a support group at my local chapter. This proved to be life-changing; I soon met other people like myself who were not only dealing with hemophilia but also with HIV. These friendships led to my involvement in becoming an advocate for the bleeding disorders community.

Because of my HIV status, I didn’t believe I would ever get married—but in 1998, I met the love of my life, Sharon. We were married the same year and raised two daughters and a son together.

I was diagnosed with hepatitis C in 1995 and lived with it until it was cleared in 2018. In 2002, I went on disability because of the on-going joint issues I was having, mostly with my knees and hips. That same year, my right knee was replaced and then my left in 2005.

In 2006, I was diagnosed with hereditary hemorrhagic telangiectasia (HHT), a condition that causes abnormal connections to develop between arteries and veins. This caused me to have such bad nosebleeds that at one point I was having weekly blood transfusions receiving 2-3 units at a time. I was tired and weak all the time. When I was first diagnosed, there was no real treatment for HHT. Today an intravenous drug is available and every couple of months I receive an infusion to prevent the blood vessels from bursting.

2016 saw a right hip revision, followed by a left hip revision in January 2017 with another that same year in August.

At 65, past bleeding episodes have taken a toll. It has become difficult to do the day-to-day things most people take for granted. I now must use a power chair, rollator or scooter—take your pick!

I always assumed there would be issues with my lower body, but my younger self would not have imagined my troubles in aging would involve elbows, shoulders, fingers, and back. These joints bothered me in my youth but stopped in my later teens. However as I approached 60, I began to lose range of motion in my elbow and shoulders, which interferes with doing simple things. For example, since my elbow won’t bend properly, my right hand can’t reach my mouth to eat.

Through the years, Sharon and I have led a variety of workshops at NHF annual meetings. One of our favorite workshops centered on talking with younger couples about our experiences and how we dealt with my hemophilia.

At one particular session, Sharon mentioned that I watch a lot of television. As a young child, my parents wouldn’t allow me to play for fear of an injury, so I spent a lot of time watching TV—it became my friend. As an adult, it’s much the same way. There were probably ten guys at that session who said they were the same way. This was an eye opener for the wives when they realized they weren’t alone and that television was their husband’s coping mechanism.

I don’t have the drive or desire to do a lot of things that I used to enjoy. Living with a lot of pain is a challenge, but I’m still here! With all the trials I have in front of me, I lean on my faith for strength and always try to look at the glass as half full rather than half empty.

I enjoy a lot of things as an aging adult—especially being married to my wife. When Sharon came into my life, she took on being an advocate with me and for me. She is my partner in every way and my biggest support.

My best advice for aging with hemophilia is to try to stay connected with people and events in the community. You can still learn a lot and the friendships are uplifting and immeasurable!

Be blessed and stay encouraged!

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