We welcome the first generation of men with hemophilia living into older age-and explore the medical issues that come with it.
By Heather Boerner, freelance writer
When Dan Liedl, who has severe hemophilia A, wakes up in his Morgantown, West Virginia, home every morning, he feels fine. It’s only when he starts to move that he feels every minute of his 58 years. It’s an interesting experience for a man whose adoptive mother was told her son wouldn’t live to age 21.
There’s the arthritis in his hips, ankles, knees and shoulders that tell him what the weather is going to be like that day. There are times he has to dangle his legs over the side of the bed and slowly rotate them so that the pain in his ankles drops from agonizing to just sore. And of course, there is the limp caused by the fused knee and the neuropathy from cancer treatment that remind him with every step he’s not a kid anymore. It’s a weird realization for a man who, in midlife, is studying to get a PhD in medical sociology.
“Some days my mind tells me I feel like a teenager and my body tells me I’m an old man,” Liedl said in a deadpan.Â
What he does know is that his body is older than his years, largely because he’s a member of the first generation of men with hemophilia who are living into their 60s, 70s and 80s. He and his peers have experienced the full gamut of hemophilia treatments-whole blood transfusions, cryoprecipitate and every generation of replacement factor out there. This means they also have had a large number of bleeds and the accompanying joint damage and bone density loss, often leading to mobility limitations and pain. Plus, he and his generation are dealing with something that men with hemophilia never had to face before: diseases of aging, such as diabetes, high blood pressure, cancer, joint replacements, even heart disease and organ transplants. And as they do, they-and the health care system that treats them-are having to learn how best to meet all of their needs.Â
The good news is that everything these men are dealing with can be addressed with the right medical teams and with the help of the discipline that most men with hemophilia learn early, said Richard Vogel, 65, of East Brunswick, New Jersey, who has severe hemophilia A. Vogel is a past president of Hemophilia Federation of America.
“If you don’t infuse, you’re going to bleed,” he said. “So you learn to be compliant with your prophylaxis. I know every third day I’m going to infuse. And I don’t miss a day because it’s ingrained in me that this is what I’m going to do. The same is true for high blood pressure medication [or any other medication]. That discipline is there.”Â
But there are a number of ways that having hemophilia requires different care. For instance, routine screening for colorectal cancer through a colonoscopy or a sigmoidoscopy are low risk for bleeds, but the removal of colorectal polyps to prevent colon cancer is not. Endoscopy to assist with cancer staging in the gastrointestinal tract isn’t high risk, but doing the same procedure with the addition of a biopsy with a fine needle is. The list goes on.
All of this calls for collaboration between hemophilia treatment centers (HTC) and treatment teams, said Joan Osip, RN, NP, a former HTC nurse in the Minneapolis area. And it’s why one of the first things Osip used to tell her clients who were older was that they couldn’t depend only on their HTC for all their care anymore.
While some HTCs also offer primary care, most HTCs, she said, are set up only to manage hemophilia. And smaller ones may not have the staff to add primary care or ongoing management of non-hemophilia conditions. Just like the men they care for, HTCs have to adapt to aging needs, too.
“Nurses have to be trained in primary care,” Osip said. “HTC nurses are used to managing bleeding and clotting disorders; primary care is a little bit different.”
They will also refer you to specialists such as nephrologists, oncologists and others to provide the specialized care people need as they age.
Murali Pazhayannur, 61, of Aurora, Illinois, who goes by one name, “Murali,” knows this better than most. Murali lives with severe hemophilia A and has a primary care provider. But he gets most of his care from specialists: an endocrinologist for diabetes, nephrologist for kidneys and so on.
He has also had two major surgeries in his life. In 2003, Murali had a pseudotumor removed from his hip. The result of a childhood growing up without hemophilia treatment in India, the pseudotumor started as a small bleed. But because it never stopped, the body walled
off the bleeding, creating a football-sized pseudotumor that compressed his femoral nerve and caused withering and paralysis in his lower leg. That procedure, managed by a hemophilia expert, also included various specialists because of how dangerous it was-carrying with it a 1 in 4 chance of death, he said.
“Obviously I’m still living,” he quipped.
So when Murali learned that his chronic kidney disease was threatening to force him to, in his words, be “tied to tubes” for dialysis, he got on the kidney transplant list. After his pseudotumor surgery, the transplant he received in 2015-with a kidney from his brother-seemed much less complicated. However, because he receives his care at Rush Medical Center in Chicago and was having theÂ transplant at University of Wisconsin Medical Center in Madison, he became his own care coordinator.Â
Luckily, as a database administrator who calls himself “fixated” on keeping every lab result and piece of medical data about himself, he was uniquely qualified to play the role.Â
“For the kidney, I had to collect all that information, and I had saved all of it,” he said. “Madison was asking me for X, Y and Z thing, and Rush was asking me to sign all these documents for every itty-bitty information they needed. I had all my paperwork, though. All I had to do was upload it in one place and then send the link to the Madison nurse.”
Multiplying Health Conditions
Still, aging with hemophilia brings with it a lot of non-hemophilia conditions. For Murali, “it would be easier if you asked me for a specific ailment and then I could give you the medication. I don’t know if I can rattle off all the medications.”
Like Murali, Liedl and Vogel also have a lot of chronic conditions: diabetes and high blood pressure for all three. Vogel has high cholesterol and kidney problems. Murali no longer has kidney problems, but now he takes immunosuppressants every day to keep his body from rejecting his kidney. Liedl has had pancreatitis and had his gallbladder removed. Now, he’s also got narrowing and hardening of the bile ducts on his liver. Both Vogel and Liedl acquired HIV and hepatitis C from tainted blood products in the ’80s. (Both men are now cured of hepatitis C.)Â
The privilege of growing older, it seems, comes with a laundry list of medications, specialist appointments and prior authorizations from insurance. Oddly, one side effect of having uncontrolled bleeds for so many years before factor replacement and prophylaxis is that their blood never got a chance to build up blood clots-and that has been associated with men with hemophilia having lower rates of heart attacks and strokes. But as bleeds become less frequent, Osip said she doesn’t expect that protection to endure.
And when blood clots do form-Osip said she’s worked with men who have had heart stents placed or other heart-related issues-some require aspirin, an almost unimaginable situation for people with hemophilia decades ago.Â
“You have to use it, but they’re probably not taking it as long as people without hemophilia,” Osip said. “And you probably have to be on prophylaxis.”
And then there are the multiple medications that men must begin to juggle. Data out of Europe suggest that so-called polypharmacy-taking lots of medications-may be less common for people with hemophilia than those without. But that doesn’t mean drug-drug interactions can’t happen, she said. And they can send a person to the hospital. That means that periodically primary care providers should also check a person’s medications to make sure they still need all of them and at their current dosage-while also doing lab work to look at blood sugar and cholesterol. They should also check that their patient is taking them at the right time of the day and with or without meals as necessary.Â
“Primary care will monitor the drugs you should be on and tweak it as it needs to be tweaked,” Osip said.Â
Quality of Life: The Final Frontier
Understanding your medications isn’t the end of the story on aging with hemophilia, Osip said. There’s more to life than being disciplined in taking your medicines and following up on appointments. Indeed, there’s joy, and there’s sex.Â
Trouble enjoying life and sex can increase with age in general. For instance, a Canadian study found that nearly 2 out of 5 men in their late 40s with hemophilia reported erectile dysfunction, with odds of erectile dysfunction rising with age. This isn’t something to just give up on, Osip said.Â
“Having sex is important,” she said. “It brings all kinds of great dopamine and serotonin.”Â
She pointed out that, like many other things associated with age, sometimes sex needs to be adjusted to make it work for your current body. There’s even a book, “Sexual Health in Hemophilia: Preventing Joint and Muscle Injuries,” written by an HTC physical therapist.Â
“The thing about aging is that you start to lose stuff that, as a younger adult, you don’t think about,” Liedl said. “One of the things I lost when I went through cancer treatment was I became impotent. It’s just another thing you’ve lost.”
Another big issue to consider as people age: mental health. Dana Francis, MSW, a social worker at University of California, San Francisco, HTC, has facilitated groups so guys could talk about hemophilia and life. But he said he didn’t dare call them “support groups” for years.
At one group in 2018, he said, “Somebody said the D word-depression,” he said. “By the time we’d gotten around the circle, half of them had said the same thing.”
But the same stoicism that keeps men persevering through bleeds can prevent them from acknowledging or getting help for the depression and anxiety that can come from loss of mobility, isolation and aging.
“It feels really good to not be alone,” Francis said he tells the men he works with. “It feels good to not be isolated. It feels good to have other guys you can confide in. What we’re all trying to do here is expand our humanity beyond the narrow limits of what we’ve been taught it means to be a man.”
For Liedl, the solution to loss isn’t isolation; it’s teaching himself to be curious about new things. Like sex, for instance. You learn to be intimate in other ways.
“Why say goodbye [to things you’ve lost] when you can change and do something new?” he said. “Do something else that thrills you. In my life, I’ve been an air traffic controller. When I lost that job, I went back to school; I became a therapist. When I got cancer, I went back to school, and now I’m getting my PhD so I can be a university professor. There’s always opportunities out there. You just have to find what makes you happy.”