Community Is the Bonus to Hemophilia
Ashley is somewhat new to the hemophilia community but has already worn many hats including mother to a child with hemophilia, carrier of hemophilia, volunteer, and most recently, executive director of a state chapter. Even though her uncle had hemophilia, she says she had limited experience with the disorder until her nephew was born. “My family wasn’t prepared for someone to be diagnosed with hemophilia again. I guess we thought since my mom didn’t have any kids with hemophilia, it just ended there. Then I realized I could have this too; it’s something I should think about when I have kids.”
Ashley was tested when she was pregnant for her first son, Leo, and learned she is a carrier. While Leo does not have hemophilia, her youngest son, Max, does. The diagnosis process was frustrating for her each time they tested her sons. “It’s such a rare disease that people aren’t familiar with testing for it. Both times the hospital botched the test and the baby had to go back in for testing.” It was difficult to sit by and watch her babies endure multiple painful needle sticks and maintain patience when she desperately sought out answers.
“With Max, finding out was hard, even though I knew it was a possibility. Nothing really prepares you for hearing those words.” Ashley’s biggest fear was that they would never get to have a normal life. “It felt so overwhelming thinking that this would take over. I was scared that it would take over our life.” The first few days after receiving his diagnosis were the hardest and were clouded by sadness. She could not stop herself from comparing her boys and how a normal part of childhood, like bumping your head, would have such drastically different implications for Max. However, Ashley knew she could not be sad forever. “I told myself it could be worse. This might present some challenges, but we’ll get through this as a family.”
Max suffered his first bleed when he was 8 months old. Ashley noticed that he would not crawl and would only reach for things. She knew something was wrong when he began to cry when she held his ankle while changing his diaper. She immediately called her HTC, The Louisiana Center for Bleeding and Clotting Disorders at Tulane; an ultrasound revealed an ankle bleed. Max began prophy treatment, which meant weekly travel to the HTC for infusions. When Max developed inhibitors, the need for a port became imminent. “Port surgery was stressful. Putting him to sleep was stressful and knowing he’d have a cut and permanent scar. When you have a child with hemophilia, surgery is just scary.”
Max’s port surgery took place one week before his first birthday, and Ashley expected to be in the hospital for 2 to 3 days. However, due to uncontrolled bleeding at his port site, he was in the hospital for 10 days. “That was scary. In the beginning, when it wouldn’t stop bleeding, I could tell the doctors and nurses were worried. It scared me. I was worrying about my other son who was worried about his brother. All of the unknowns were scary.” Their hospital stay ran so long that Max celebrated his first birthday in the hospital.
The hospital staff was very supportive and brought presents and a cupcake for Max. However, Ashley’s worries about having a normal life with hemophilia resurfaced. “I just cried. I was trying to get myself together, but it was so overwhelming to be there for his birthday. I wondered if every birthday was going to be spent in the hospital and if every celebration would be tainted.”
Finding Inner Peace
Cassandra comes from a long family history of hemophilia. Starting with her grandmother who passed it down to her father, then herself, and now her son, she describes her journey as phases of transition from being a daughter, a patient, an educator/helper, and now a mother, all beautifully protected by a bond between herself and her father that she now shares with her son.
When Cassandra was very young, her parents explained that she bruised easily because she was a carrier for hemophilia. However, the reality of it did not sink in until she was a junior in high school. She was an athlete excelling in her sport, pole vaulting, when she started to experience shin bleeds and was diagnosed with hemophilia.
She continued to pole vault through her college years, but her disorder presented many challenges. Cassandra did not know any other athletes with a bleeding disorder and was frustrated with how quickly others recovered from injuries, while she suffered for weeks. She often felt isolated by not being able to participate in practices and spending lots of time in the training room doing physical therapy. She was never really afraid of her hemophilia because she was “a bit ignorant of it.” In all the years she watched her dad work through his disorder and infuse, she did not realize that she was also affected by it and that was why she was getting injured so frequently and not healing properly. She began to seek advice from her father on how to manage her hemophilia, even preferring to call him before calling her HTC, because she has so much trust in his knowledge and experience.
Destiny Caithamer was a shy kid with low self-esteem. In an attempt to pull her daughter out of her shell, her mom, Celine Caithamer put Destiny in her first beauty pageant, where she was scouted by a photographer. Today the 25 year-old is an international model and pageant queen. Her Facebook page is decorated with gorgeous pictures from countless professional photoshoots and magazines. She is living a life that most girls could only dream of and many would envy. However, this is only part of her story and what lies beneath the surface makes Destiny feel less glamorous and more like an “alien: unique, misunderstood, and a bit of an anomaly.”
When Destiny started having unexplained bruises at the age of 5 months and was vomiting blood, her mom knew it was time to seek answers. It was initially suggested that she might have had leukemia. After a couple of visits to the Comprehensive Center for Bleeding Disorders in Milwaukee, the family was invited in for an educational session.
“They gave me all these words that I didn’t know, like von Willebrand Disease, factor VIII. I didn’t know about the clotting cascade. It was all new. It was all foreign,” says Celine. Destiny was diagnosed with von Willebrand Disease, as well as factor VIII deficiency. “I was relieved that it wasn’t leukemia. Second thing I thought of was that I was completely ignorant on the issue. I couldn’t even pronounce the words they were using.”
Destiny’s normal childhood, despite her bleeding disorder, turned into shock and confusion when she started school as she struggled to fit in. Her peers did not understand her disorder and thought because she had bruises on her legs that she was dirty. Some kids refused to play with her. It was difficult for her to be cast aside by her peers and she said she “always felt alienated.”
In third grade, Celine had someone go to school to talk to Destiny’s classmates about her disease. Afterward, the teacher had everyone put their hands on the table and told the class to look around for the student wearing the special bracelet while attempting to explain the disease. At a very young age, Destiny already knew the value of embracing her disorder even when others shunned her because of it. “Kids thought it was a scary, bacteria, or an illness they could catch. The way the teachers were describing it, if I were able to explain it to the class, I would have been able to explain it in my way, a way that kids my age would understand.”
Life was normal for Brandee. She lived near the coast and enjoyed water sports, softball, and dance. When she was 10 years old, her brother died of a platelet disorder, idiopathic thrombocytopenic purpura. Two years later, she experienced her first menstrual cycle which lasted more than 30 days. After a year of tests, she was diagnosed with von Willebrand’s disease. Brandee had always bruised easily, but no one thought much about it. “I got put on the back burner because my family was always dealing with my brother’s issues. I wasn’t constantly in my mom’s care long enough for her to see if something was wrong.”
After losing a son to a bleeding disorder, Brandee’s mom was most fearful of her diagnosis and worried about losing her daughter. “She kept me very close.” Brandee’s reaction was quite different. Being a teenager, initially, her diagnosis seemed insignificant. “You’re young and you don’t really care.”
That attitude soon changed. Her bleeds were very difficult to control and she had to be infused so frequently that she would often go to school with an IV in her arm, which she tried to cover with clothes. Eventually, it became obvious that her disorder would be much easier to manage at home and Brandee started to homeschool. Suddenly, her “normal” childhood was anything but normal, and she was missing being around her friends. Even worse, everyone was placing limitations on the activities in which she could participate, and she did not understand why things like riding jet skis, playing paintball, softball, and dance, activities in which she had always participated, were now off-limits.
For the next 10 years, Brandee would try to find the right treatment to control bleeds associated with her menstrual cycle, spending lots of time in the ICU for transfusions. At times, she could not walk three feet without passing out from the extreme blood loss. One surgeon who has done multiple port placements on Brandee says that she seemed “very annoyed and mad at the world”. Although she did not agree with that statement at the time, in hindsight she says her doctor’s perception was correct. “I was hoping that it would just go away; that I could just put it on the back burner and that it would go away. I just really wanted to be normal and just be a kid.”
Advocate and Friend
Blanca Ramirez is a self-described advocate and friend to the bleeding disorders community, a role she has been passionate about for the last several years. However, this role presented itself through the course of life experience as she learned to navigate the diagnosis and treatment of herself, and again with her daughter, Cynthia.
Blanca was a child living in a small town in Mexico when she started experiencing unexplained bruises and bleeds. She used her clothing to cover the bruises, but the bleeds could not be hidden. She says, “I couldn’t have friends because they thought I was contagious. They just didn’t understand.”
Her initial diagnosis was anemia, but the nose bleeds and heavy periods eventually proved that to be incorrect. Her bleeds were so severe that she would get dizzy and weak and her biggest fear was that she would die from a bleed. There was no factor available at the time and transfusions were still the norm for treating bleeds. However, the clinic in her town did not carry blood and the closest hospital for which to receive a transfusion was nearly four hours away. She did her best to manage her bleeds and her pain. She says, “The pain is there, but I don’t really pay attention to it anymore. We women, we learn to deal with the pain.”
Some years later, her daughter, Cynthia, who was a physically active child, experienced symptoms similar to her moms, with lots of bruising and an abnormal number of nosebleeds. In 1991, when Cynthia was five years old, she was diagnosed with von Willebrand’s disease. Her family had no knowledge of this disorder and the specialists and doctors in their small town in Texas could offer them little help. They endured many nights of nosebleeds and long hours at hospitals searching for answers from doctors who had no experience in treating bleeding disorders or awareness of effective treatment. This was exacerbated by the lack of moral support they received and understanding from their community. Blanca says, “von Willebrand’s disease was affecting our lives in a critical way.”
One fateful trip to the hospital would soon change their fortune. Doctors were again baffled to find treatment and they were scheduled to spend the night in the hospital when they received a call from Dr. Howard Britton of Santa Rosa’s Children’s Hospital informing them that he was sending a helicopter to transfer them to his hospital for treatment. Blanca says, “Dr. Howard Britton was our saving angel. Our lives changed from that moment on.”
Kathy MacKay met David in 1985 when she moved to Nashua, NH. She referred to him as "the boy next
door." They sparked up a relationship pretty quickly, and that is when she learned he had hemophilia.
She infused him three times early on and stated that she did not know if he asked her to do that because he just wanted an excuse to come over or if he really did need her help. She said that she always ac-
cepted David's Hemophilia.
They had read about the Ryan White story in the news and knew about the issues with the blood supply.
Kathy asked David if he had been tested for HTLV-III or now know as HIV. He said he tested negative. So
she did not think much more of it because the blood supply was allegedly safe. A little under a year later,
he was tested again, and at that time, he tested positive. His doctor then offered her a chance to ask
questions because they were both concerned. Kathy immediately made an appointment to address her
David's doctor said he wasn't concerned about him progressing to AIDS at the time because of his infrequent infusions. Also, they found out he had been exposed to Hepatitis B and C, then referred to as non-A, non-B. Again, the doctor told them there was little to worry about. Kathy remembers him saying "all hemophiliacs have been exposed to Hepatitis, and it only affects a small percentage of patients. It is an acceptable risk." Kathy heard what she wanted to hear from the doctor. Kathy reflects, "I wasn't smart enough then to really think about what that meant. I heard what I wanted to hear about HIV, so I think in my head, I just accepted what the doctor said." This was before the internet, and doing research was not easy.
As time went on, David was doing well, and he proposed to Kathy. They married in August of 1987.
Every once in a while, his T-cells would dip a little bit, and they would get nervous, but they always
bounced back up. David was asymptomatic and never met the criteria for an AIDS diagnosis. Because
their future was looking bright, they decided to start a family. They had two boys: Ryan, who is now 27, and Dustin, who is now 31. They took all the necessary pre- cautions to minimize the risks of Kathy contracting HIV. They made it through both pregnancies, and neither Kathy nor the children contracted HIV. Kathy had to be tested frequently. "I lived my life being tested for HIV, but I lived my life."
In the summer of 1994, David had a bad hip injury and could not work his summer job as a camp counselor. He was a special education teacher and was really looking forward to working at summer camp that year. Due to his bleeds and a series of respiratory infections, it caused him to face Hemophilia and HIV's circumstances even more. It was during this time that David started to have issues with depression. For the most part, he kept his feelings to himself. In retrospect, Kathy wishes that David could have shared his fears and worries more openly.
When Kathy is asked, "what was more of a challenge," hemophilia or the viruses, Kathy said it was the viruses that really made her upset. "My life had never been perfect. I always faced challenges along the way.” it was just a challenge, but the viruses could have been prevented. Kathy goes into greater detail on how the blood products became contaminated and how the psychological effects impacted the people in her upcoming memoir, "Unacceptable Risk."
You Cannot live in fear
Ed grew up with three brothers that all had been diagnosed with hemophilia. Each of the three brothers lived with it and learned to have a full life despite their bleeding disorder. He was the middle child. His mother was two years old when Ed's grandfather, her father, died from hemophilia. He also had two cousins that had hemophilia. He stated, "I was never alone." They were a family that believed life should be lived and did not let Hemophilia limit their lives.
Ed and his brothers hung out together. When they developed a bleed, they would stay on the couch with ice unless it was really bad, and then they would go to the hospital. Ed stated his parents were cool. The boys would play sandlot baseball and only had one rule, "if you get hurt, you get the ice packs and go to the couch. No Complaining." He recalls one time that he ran into the front door, and one of his brothers ran into the back door, and both of them were holding their noses and discussing how they got their injuries. They always had a sense of humor related to their hemophilia.
When factor became available, Ed learned to self-infuse at camp. This was life-changing. They could address the bleed without disrupting the family to go to the hospital for cryoprecipitate and have much quicker relief. Since Ed was so efficient at self infusing, he started to infuse his brothers. At that time, the doctor wanted the nurses to do the infusions, but they got a neighbor that was a nurse to sign the form from the doctor, and then he was able to infuse himself and his two brothers.
Ed played ice hockey for two years and didn't tell his parents. His greatest fear, other than his mom finding out, was losing a tooth. This was because "the blood would ooze out while I was sleeping, and I would wake up with my room looking like a Quentin Tarantino movie." And he was afraid of dying from a lost tooth. His hockey career ended after a bad fall on the ice. His younger brother, Michael, was a heavyweight wrestler in high school. After breaking the school record, Michael's pediatrician told the school he needed a letter stating he wouldn't get injured.
Ed picked up guitar while waiting for his bleeds to resolve. This had provided one healthy joint and has given him a life long hobby and pleasure. Ed still plays guitar in a band called Breaking all the rules. The band plays in local sports bars and night clubs. A much more sustainable hobby than Ice Hockey. Ed says, "I wouldn't have missed out on any of my adventures."
Becoming a hemo wife
In 2011, we started dating. Then in 2015 we were married! All throughout our years of dating, his hemophilia was never an issue because we lived in different states and were rarely together during his bleeding episodes. A few weeks into our first year of marriage, Kane had an accident at work and was unable to walk for a few days because of a bleed. He was fired from his job shortly after for missing too many days without a doctor’s note. This was the first bleed in all of the years knowing each other that I was able to witness. This is when his hemophilia became real for me. After our long struggle with getting his health and medical record on track, we are now managing his hemophilia, instead of his hemophilia managing our lives!
Being the wife to a hemophiliac has shaped my career path to patient advocacy in the legal field. Sharing the challenges we have faced during these last five years of marriage on podcasts and similar outlets, has only solidified my passion for our hemo family. This is what keeps me excited to see where our hemophilia journey leads us!
July 01st, 2020
Getting diagnosed with Hemophilia wasn’t easy for Larry. He was told he wouldn’t live long and this crushed him, as it does so many others. Because of this, he didn’t really focus much on his future. Larry just lived life with no fear and not many goals for himself.
The neighborhood Larry grew up in was not a very good neighborhood and he eventually became a product of his environment. With not much hope of living a long life he found happiness in using and selling drugs. He lived on the streets, constantly getting into fights over drugs and other things. This all came to a stop when he was arrested and sent to prison at the age of 22. Larry spent seven years in prison. During this time, he realized that his hemophilia, in fact, wasn’t going to kill him. Larry decided, then, he wanted to change his life and become a better person. He went to college to get a degree.
With his new outlook on life, and getting a degree, Larry was still told that he would never be anything. He couldn’t do anything with this degree and no one would hire him because of his hemophilia. This returned him to the hopelessness that engulfed him growing up with Hemophilia. He went back into his old ways and ended up back in the criminal justice system. Larry got lucky this time around and beat his case in court and avoided going back to prison.
After this Larry decided to move back to Michigan to be with his dad. He needed to get away from his past to be able to have a new start at life. Over the next year, Larry self-isolated, self-medicated and began drinking heavily. He was barely getting by, even though he had a job doing Upholstery. He wasn't happy. The upholstery business became too much for him physically and he decided to go back to school. He knew there had to be something better for him.
Leaning on Faith
“Your life with Hemophilia has a purpose. You are fearfully and wonderfully made by God, who loves you. Turn your struggle into your story and use it to impact others.” -Lindy
Before becoming a mom to Lincoln, Lindy & her husband, Ben struggled to get pregnant. They were almost to the point of defeat when they received a prophetic word at church on Mother’s Day, 2014. Her pastor said to the congregation, she felt like she was talking to someone directly and that “this time next year, you will be holding a son.” Lindy found out two months later she was pregnant, and Lincoln arrived precisely one month before Mother’s Day 2015. She knew that her son would do great things and have an amazing testimony to share because he was truly a gift from God that she had prayed for.
When Lincoln was about 4 months old, they started noticing some abnormal bruising on his body. At first, they shrugged it off and thought he would be an easy bruiser like she was. But when the bruising increased in size and frequency, she started pressing their doctor for answers. After lots of appointments and tests without answers, they finally decided to change doctors. In December of 2015, at 8 months old, Lincoln was diagnosed with Severe Hemophilia B. A surprise diagnosis as there was no history of the disorder in her family.
Lindy remembers that for several months following the diagnosis, she thought they’d gotten it wrong because they were dealing with none of the things she was reading about the condition. Outside of the bruising, Lincoln was a healthy and happy baby boy! The Hemophilia diagnosis came to reality when Lincoln fell at 15 months old, and they were unable to get his mouth to stop bleeding. She remembers taking him to the hospital for his first factor infusion and how scary of an experience it was for them. Then, in October 2016, at 18 months old, Lincoln developed his first joint bleed in his ankle. That’s when they began pushing for a prophylactic treatment plan but ran into several obstacles with both his HTC (Hemophilia Treatment Center) and their local hospital. For 6 weeks they were sent to the ER 8 times due to reoccurring bleeds in the ankle. By late November they were finally set up to receive weekly factor infusions at the local hospital. His prophylactic treatment was going great for about ten months. Then, in September 2017, Lincoln fell in the bathtub and hit his mouth causing a bleed in his gums. After dealing with this mouth bleed for over a week and not receiving adequate help or support from their HTC, they decided to change to a new doctor and treatment center. Their new HTC immediately made them feel like they were at home. They felt loved and truly accepted for the first time in Lincoln’s hemophilia journey. Lindy addressed some issues they had been having with Lincoln’s care with their new hematologist. She told the new doctor that Lincoln had been having more frequent breakthrough bleeding, and he explained that he believes Lincoln was under-dosed. It was agreed to change his medication to try out a longer-acting factor. Luckily, they were still going to an infusion center to receive his factor because, during the first infusion with the new medication, Lincoln experienced an allergic reaction to this new medication. This caused him to go into anaphylactic shock. His nurse, who had done his infusions for over a year, immediately knew what was happening, and Lindy accounts for her fast reaction to helping save Lincoln’s life. After the injection began, Lincoln started pointing to his tongue and coughing, and he was holding his stomach, saying it hurt. Lindy said, “something is wrong, he’s having a reaction!” His nurse immediately pulled the needle and picked Lincoln up, and they all ran across the hospital to the ER so that he could get the treatment he needed STAT. While running across the hospital, Lincoln was turning red and vomiting. Lindy remembers thinking that this was the scariest moment of her life, up to that point. There would be many more scary moments in his journey in the months to come.