A word that we hear frequently right now is “access”—access to quality care, resources, education, community, and other people affected by a bleeding disorder. Our Blood Brothers and Sisters everywhere have these concerns.
The recent rise in the number of bleeding disorder organizations around the country (4 new chapters and member organizations) in the last 5 years is clear evidence that there are issues with access to care. Stories and experiences that those of us without easy access to the community share are further evidence. How many of us have heard of someone in the community who thought they were the only one out there like them, living alone with their bleeding disorder? Some have not even been diagnosed until much later in life.
In response to our bleeding disorders community’s demonstrated need, we formed the Care Access Working Group (CAWG) in February of 2013. Working alongside the CHOICE Project, the CAWG is finding ways to give voice to the under-served and support our member organizations’ efforts to serve everyone directly.
Our merry band of advocates originally set out to serve our Blood Brothers and Sisters in rural areas, but by the end of our first call, we realized that those in large cities have many of the same issues. So we created our first initiative, which is called Project “Reaching Everyone Directly” (RED).
Our mission is to identify solutions to the lack of access to care and connection to community experienced by all members of the bleeding disorders community, focusing on rural members. We will be accomplishing our mission through the following actions:
- Research the impact living in rural areas poses on members of the bleeding disorders community.
- Find means of access to care, education, and other support through community-based mechanisms that increase empowerment, efficacy, and community.
- Create a national network of support for those living in rural communities.
- Work to establish an “ombudsman” system for communicating with HTCs when needed.
We went to various communities looking for personal stories and have published some highlights here. In the coming months, look for more stories (names and locations have been, and always will be, changed to protect privacy). One way that you can help is by sharing your access stories, both problems and successes, with CAWG so that we can start a dialogue and, together, support each other and ensure better access for all!
Lucy was diagnosed with vWD at the age of 18. Her mother was diagnosed at the age of 32. At the time, they both lived in a big city. Late diagnosis is problematic because so much damage can already be done to joints from years without treatment. By the time she was 24, Lucy was living in a rural area and finishing up medical school. She was having complications with bleeding despite treatment. Her doctors chose to stop treating her vWD rather than looking for an additional cause of bleeding because the treatment wasn’t working (so they thought she must not have vWD after all). Instead, they put her on pain medication, which caused her to be accused of being “med-seeking.” The lack of treatment caused a great deal of joint bleeding, which led to severe arthritis and 782 days of physical therapy. In the end, she found a specialist who figured out that she had a rare platelet disorder in addition to vWD.
Eva and Eli
Living “out in the middle of nowhere” definitely has its positives and negatives…especially when you are a family living with hemophilia. Our 6-year-old son, Eli, was diagnosed with hemophilia A at birth, at a hospital that was 3 hours away. The whirlwind of emotions was overwhelming those first few days. On the drive home, as we got further and further from those doctors and nurses with their years of hematology experience, the panic set in. How can we do this without daily help? What if something happens? What if Eli needs factor? Has the hospital at home even HEARD of hemophilia? How will we know if he has a bleed that we can’t see or feel?
Eli’s first few months seemed pretty “normal.” But once he started crawling, the hemophilia really manifested. He had bleeds from high chairs, head bonks, and, of course, bruises and bleeds that just occurred without explanation. That’s when the emergency room (ER) visits started. And finding a vein on Eli was like trying to find a blond hair in a sea of milk.
Our first few trips to the ER were not fun. There were lots of questions about his bruises, lots of blown veins, lots of confusion, and lots of tears (from both Eli AND his mom, Eva). Each time we went in, we got a different doctor…a different nurse…and a different “protocol.” It was incredibly frustrating. And of course, everything seemed to happen at night…during our HTC’s “on-call” hours.
After a few months, we decided that we would call the shots at our local ER. We also decided to no longer have an “us against them” attitude. We used each ER visit as an opportunity to teach the staff. We told them about previous ER visits—what worked and what didn’t. We would ask about their family, and we made a point to get to know them. Over the next year, we became a team. We would call ahead to the ER to give them a heads-up. Soon, we realized that we knew the staff, and they knew us. They have watched Eli grow over the last 6 years, and, as funny as this may sound, the ER is now a place of comfort for us. The feeling we get when we walk in, and they give our son high fives, know which room he likes best, hand him the remote control before he even asks, and remember that he likes the purple popsicles is just…good.
Living in a rural area does not have to be scary when it comes to treatment for your child. Be an advocate for your son/daughter, and team up with your local ER. And do not be afraid to get to know them. Often, they are who you see for bleeds that need extra attention. You just might be surprised how comforting the ER will grow to be.
Janie and Joe
When members of the hemophilia treatment center (HTC) behave erroneously or unethically, there is no one to file a complaint to that could cause a change in behavior.
When my son was born, I felt unsure about blood transfusions. Because members of the HTC team disagreed with my reservations, I felt isolated and likewise, the HTC felt that I was incapable of parenting a hemophiliac. There was no one to “translate” my concerns to them. When a child’s life is in danger, neither the parent nor the doctor really wants to gently convince the other on the best course of treatment. Neither side is willing to do the most important thing: reason. The result? Both sides get angry.
Because I didn’t feel heard, I was angered by anything that had to do with the HTC, and, as a result, limited visits there. Then, one day the HTC social worker invited me to a parenting class. All barriers to attendance were removed (e.g., childcare, transportation, food, and lodging were provided for free).It was a non-judgmental atmosphere where I learned much more than how to raise my hemophiliac son better. I learned what the HTC thought about me and about parents in general. It was not always what I’d assumed. They were concerned about my skills as a parent because I didn’t articulate well to them what my thoughts, predicaments, and plans were. To this day, I believe that there are times when this HTC can be more likely to ignore the parent. But this is only because they lack access to the true desires of the patient for one reason or another.
I knew Joe had an inhibitor long before he was diagnosed by the HTC. Despite several trips to the HTC, they didn’t want to test him for inhibitors. He got worse with each visit. He almost died three times. Here’s why:
- I had no access to education on inhibitors. I had no one to talk to about this besides the HTC team, who felt that I did not need information on inhibitors.
- I had no words to explain what I really felt to a medical team.
- I wavered in getting my son the treatment he needed and doubted I had the right to do so.
- I felt that I owed the HTC the right to tell me how to treat my son because they’d saved his life. They’d saved his life several times. I felt a sense of loyalty to them that caused me to ignore what I knew and follow their conclusions to an extent.
- I didn’t know about the Bioethical Committee. I thought that the HTC was the end of the line for complaints.
I researched inhibitors all I could and slowly became sure that Joe had one. During HTC visits, they would check Joe by touching his injuries and documenting his response or lack thereof. I saw him trying not to show pain. They thought he was pretending and not responding to what was supposedly hurting him. I described bruising. They said that they didn’t see it, so they weren’t sure that it was from hemophilia. I took pictures, which got us into the exam rooms again. Nurses would “check” Joe and say things like, “Maybe you should take his video game access away and he’d feel better,” or, “He’s probably having issues with wanting to go to school, that’s all.” This environment made me defensive.
After weeks of this, we were there after-hours for physical therapy, and Joe needed a wheelchair. Knowing there’d be one in the HTC, I stopped by. The lights were on, so I went in and saw a hemophilia nurse. I literally begged her to look at my son and to test for an inhibitor. Previously, when under review, the swelling and bruises didn’t show because I had been treating him frequently for bleeds—unknowingly making the inhibitor stronger. I’d now been reduced to allowing him to swell on HTC visit days so that I could have a better chance of having proof that what I was seeing was real. I swear to you, just writing that breaks my heart. It’s oxymoronic: I had to allow him to hurt to stop him from hurting. Still, I was thankful that I’d done that on this day. That nurse stood looking in awe at the bruising and immediately drew Joe’s blood.
The next day, Joe couldn’t walk. Skipping factor for one morning the previous day had caused so much pain that he couldn’t sleep, so I gave him a treatment that night. It seemed to have not worked well after an hour or so. Because of his consecutive days of not eating and sleeping due to his fear of causing or aggravating a bleed by having to use the restroom, I was worried and called the clinic. Again, they saw nothing.
Then they had an official meeting with me. I sat with specialists and medical personnel as they said that it was highly unlikely that he’d have an inhibitor because he was moderate (1.6% factor), and my father never had an inhibitor. They thought I was being duped by my son. For weeks I hadn’t slept for more than an hour at a time. I was at the end of my rope. My whole body shook, and I yelled responses throughout much of the conversation. I explained that my father never even received care until he was 30! I knew inhibitors were transient from my personal research and told them that they had no way of knowing whether my father experienced this or not. They stated that there was no physical problem and even intimated that Joe was being abused either sexually or physically. I don’t remember exactly what happened after that. I remember indescribable feelings and rushed thoughts and police. And shame, seeing personnel scatter as I slammed open the door to find huge guys with guns and handcuffs. I remember being escorted by security into the lobby of the HTC, where my son was playing with toys, and the doctor (who had kindly watched my son during the meeting) trying to be polite to me. Joe showed no signs that he knew what was happening, and I was grateful for that.
When I got home, I felt more fear because I thought that they might let Joe die—that I might let him die. I knew I would not survive this loss. I had already lost children, but those deaths were sudden. Though I still experience guilt, I know that it is irrational; I had no time to save them. But I had time to save Joe—months. And if he died, there would be no reasoning the guilt out of my mind. I had no idea of what to do next.
Shortly after I treated him again (which didn’t help), the HTC nurse who had secretly done the inhibitor test called me. She said that Joe needed to come back in. Just minutes after that, another nurse called and said, “The doctor says that she really heard your plea, and she’s changed her mind. She wants to test Joe for an inhibitor.” It was like a weight had been lifted. He would survive! But I knew the real reason we’d received that sudden call and why the doctors had had a sudden change of heart: that first nurse had tested his blood already, and he had already been diagnosed.
On the way to the HTC, I thought, what if it’s not an inhibitor? I wanted a solution! If it was an inhibitor, I knew that they would know what to do. It was, and they did. And in 4 months, we were clear of our inhibitor. The HTC never admitted that they were wrong and they never apologized.
Many of these problems could have been solved through access to education of inhibitors, access to understanding of patient rights, access to HTC ethical commitments, and access to proper/useful communication techniques.
Janie and Joe’s story (yep, there’s more) will be continued in our next article!
Jill Packard, MS is the mother of two sons, ages 6 and 9, who have Hemophilia A. Jill is most passionate about her family, connecting people, and building sustainable communities. Founding and current President of the Hemophilia Alliance of Maine, Jill is also passionate about supporting rural communities where she attempts to shine a light on health care equity disparities while looking towards holistic, patient-centered care.
Lori Long lives in the Southwest and has hemophilia B. She is also raising a son (age 10) with hemophilia B. She is passionate about building community and believes strongly in community strength making a difference in the world (even if it’s just her little corner of it). Her interest in serving rural communities stems from the number of blood brothers and sisters she has in her area who live without phone, Internet, and even water service. Her interest in serving in general is inspired by her father, who died at the age of 68 from a head bleed due to a lack of access to factor.