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."
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."
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!
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.
“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.
Ivan Giron’s life has been anything but easy, but he was able to overcome his past and find his purpose, even when all the cards were stacked against him. Ivan was born in the city of Bell in Los Angeles County, CA. At 2 years old, he was diagnosed with Moderate Hemophilia A after busting his lip on a toy car. His mother was scared which caused her to be overprotective throughout Ivan’s childhood. She made him wear a helmet and didn’t want him to play any sports. Ivan convinced his mother to let him play soccer once he turned 7, but he was forced to quit once he reached high school because of excessive ankle bleeds from his hemophilia. He says his childhood was difficult because he wanted to be active so badly, and wasn’t able to do the things other kids were participating in. He was competitive, and wasn’t allowed to compete. He was frustrated and often picked on, because no one understood his situation.
Ivan’s father faced a very tough childhood himself, and because he had a hard time coping, he became an alcoholic as an adult. His father was violent, angry, and abused drugs throughout Ivan’s life. He followed fairly close to his father’s example, and joined a gang at just 15 years old. He was angry because of hemophilia and his family life. He saw the other gangs in the community earning money and making a name for themselves, and he wanted those things also. He and several of his friends started their own gang, and quickly began growing their circle. Ivan was buying guns, selling drugs, recruiting new members, and ultimately watching some of his closest friends go to jail.
At the age of 18, Ivan was shot in the right ankle.
Being a young parent isn’t an easy task, but being a young parent and having your child diagnosed with a severe bleeding disorder is something most people never imagine. This was the case for Jeff’s parents. At just 19 years old his mom gave birth to him, and having no family history his diagnosis came with quite the shock. But this didn’t determine his life. As Jeff says “it’s part of your life, not your whole life.”
Jeff was always very active, played competitive baseball, and unofficially played football. He remembers kind of doing his own “prophy” before that was really a thing. And because of this, his only real damage today is a left elbow target joint.
At around the age 15 years old, Jeff found a passion in stock car racing. Which as you can imagine, made his parents very nervous. Jeff said his moms favorite thing to always tell him was “you can do it if the hematologist says it's ok”. The reasoning behind this, is because his parents never wanted to be the reason he was limited in activities. This way the disappointment was left to the doctors and not them. Personally, as a mom of a young Hemophiliac I think this is great advice, and plan to use it in my future battles with out son.
When Jeff turned 18, he got involved with demolition derbies. His exact words were, “I was hooked after my first race! The adrenaline was unmatched!” Although this was not something his mom would have chose for him, she was on board and would bring his factor into the pit so he could infuse right before the race. Jeff also had his car painted to say “STOP! I’m a bleeder!” on the drivers door so anyone who saw it would know.
“I was an adrenaline junky, and hemophilia took a back seat.” Because of the addiction to this feeling, Jeff started only infusing when he absolutely had to. As a result, he was in a terrible ATV (alterraine vehicle) wreck that left him in the hospital for 42 days due to severe injuries and bleeding caused from his hemophilia. During this time, his pain was indescribable. He was on very high doses of IV (intravenous) pain medications. He says, “I remember just wanting out, I couldn’t wait to be able to leave the hospital.”
Leemar Yarde has hemophilia B with an inhibitor, and he lives on the tropical island of Barbados. He is one of six people with hemophilia in his family, but he's the only one with an inhibitor—in fact, the only one on the island with one. So he often meets new
challenges. Growing up in a family with two older, experienced relatives with
hemophilia has its benefits, but here's a little backstory.
Barbados is a developing country. The medical care there is free, and that is
commendable, because many people can’t afford medical care. However, the
downside is that due to the cost of some medicine, the government can only afford
either generic or very limited supplies. Budgets are often allocated to life-
threatening illnesses, and the government doesn’t view hemophilia as one. As a
result, very limited amounts of clotting factor are imported for both types of
hemophilia, and none for inhibitors.
Barbados doesn’t have a hemophilia treatment center (HTC) or even a
medical facility dedicated solely to hemophilia. What we do have is a hematology
center that caters to various blood disorders. That center has a handful of
experienced hematologists; one or two have dealt with inhibitors in different
countries. Sadly, a hematologist can only work with the tools available. Leemar remembers
having to wait from 8 am until 12 pm to see the doctor, then being told that the
hospital had no factor at that time. Still, in Barbados, their fortunate because
some of the neighboring Caribbean islands can’t afford any clotting factor.
So for Leemar, at one point all hope seemed lost...until he met “her.”
Arlo Smith was born to Tara and Adam on August 10, 2016. Tara noticed that Arlo had bruises every once in a while, during his first few months. Being a large newborn, when he was held he was held tight. A few months later, Arlo had a bruise on the back of his leg, where his leg would sit on your hip. After multiple instances, his parents decided to ask the pediatrician for a second opinion. The doctor was not concerned and agreed that he was a large baby so he would have bruises. At the six-month check-up, red flags were showed when a tennis ball bruise appeared on his stomach. When Arlo’s pediatrician examined the hematoma on his stomach, she decided to run some blood tests to appease Tara and Adam’s concerns. When the results came back, Tara explains, “The pediatrician told us, ‘It’s definitely not hemophilia.’” temporarily extinguishing the hemophilia scare.
Tara and Adam returned to the pediatrician a couple of weeks later with Arlo having even more bruises and they were recommended to a specialist at Vanderbilt hematology for further testing. They went into the appointment with a positive attitude and felt like, it was probably nothing. As the hematologist examined Arlo, he made it clear that the bruising was abnormal, and something was going on. After blood work, chest x-rays to determine if Arlo had a broken rib, and conversations about the people that were exposed to Arlo, they received the confirmation phone call with the lab results. The nurse continued to confirm that Arlo’s factor levels were less than 1%, concluding the diagnosis of severe factor IX deficiency. Tara, in a state of shock, told Adam the results. They did not have any known family history of hemophilia. Tara shakily describes that moment, “For me, I just felt like he (Arlo) had died”. Tara felt as if she was grieving the death of a dream of the life she thought Arlo would have lived without hemophilia.
The first couple of weeks, the Smith’s struggled both physically and emotionally. “It was really weird getting a diagnosis like this and then having to tell people. I’ve never had to tell people such tough news before.” Tara said. Trying to process how to tell people about the diagnosis and if they even should tell people was a learning curve. People would ask Tara and Adam questions to dig for something that would help them find the happy ending to the story. Ultimately, they learned that not everything wraps up with a little bow.