We welcome the first generation of men with hemophilia living into older age鈥攁nd 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鈥檚 only when he starts to move that he feels every minute of his 58 years. It鈥檚 an interesting experience for a man whose adoptive mother was told her son wouldn鈥檛 live to age 21.

There鈥檚 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鈥檚 not a kid anymore. It鈥檚 a weird realization for a man who, in midlife, is studying to get a PhD in medical sociology.

鈥淪ome days my mind tells me I feel like a teenager and my body tells me I鈥檓 an old man,鈥 Liedl said in a deadpan.

What he does know is that his body is older than his years, largely because he鈥檚 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鈥攚hole 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鈥攁nd the health care system that treats them鈥攁re having to learn how best to meet all of their needs.

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.

鈥淚f you don鈥檛 infuse, you鈥檙e going to bleed,鈥 he said. 鈥淪o you learn to be compliant with your prophylaxis. I know every third day I鈥檓 going to infuse. And I don鈥檛 miss a day because it鈥檚 ingrained in me that this is what I鈥檓 going to do. The same is true for high blood pressure medication [or any other medication]. That discipline is there.鈥

Data on people aging with hemophilia are just starting聽to emerge and aren鈥檛 yet definitive. There are the hemophilia-related conditions that Liedl described as well as an increased rate of osteoporosis or osteopenia among men with hemophilia compared to men their age without hemophilia.

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鈥檛 high risk, but doing the same procedure with the addition of a biopsy with a fine needle is. The list goes on.

As a result, the National Hemophilia Foundation鈥檚 Medical and Scientific Advisory Council, known as MASAC, recommends changes in how someone doses prophylaxis factor and other hemophilia treatments on the days before, on the day of and sometimes for a week after treatment. And the risks of breakthrough bleeds increase the older a person gets, according to the guidelines.

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鈥檚 why one of the first things Osip used to tell her clients who were older was that they couldn鈥檛 depend only on their HTC for all their care anymore.

鈥淎s you get older, you need primary care,鈥 Osip said. 鈥淚f your blood pressure is increasing or you need to start on cholesterol meds, that routine maintenance is probably better for primary care.鈥

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.

鈥淣urses have to be trained in primary care,鈥 Osip said. 鈥淗TC nurses are used to managing bleeding and clotting disorders; primary care is a little bit different.鈥

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, 鈥淢urali,鈥 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鈥攃arrying with it a 1 in 4 chance of death, he said.

鈥淥bviously I鈥檓 still living,鈥 he quipped.

So when Murali learned that his chronic kidney disease was threatening to force him to, in his words, be 鈥渢ied to tubes鈥 for dialysis, he got on the kidney transplant list. After his pseudotumor surgery, the transplant he received in 2015鈥攚ith a kidney from his brother鈥攕eemed much less complicated. However, because he receives his care at Rush Medical Center in Chicago and was having the聽transplant at University of Wisconsin Medical Center in Madison, he became his own care coordinator.

Luckily, as a database administrator who calls himself 鈥渇ixated鈥 on keeping every lab result and piece of medical data about himself, he was uniquely qualified to play the role.

鈥淔or the kidney, I had to collect all that information, and I had saved all of it,鈥 he said. 鈥淢adison 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.鈥

Multiplying Health Conditions

Still, aging with hemophilia brings with it a lot of non-hemophilia conditions. For Murali, 鈥渋t would be easier if you asked me for a specific ailment and then I could give you the medication. I don鈥檛 know if I can rattle off all the medications.鈥

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鈥檚 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 鈥80s. (Both men are now cured of hepatitis C.)

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鈥攁nd 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鈥檛 expect that protection to endure.

And when blood clots do form鈥擮sip said she鈥檚 worked with men who have had heart stents placed or other heart-related issues鈥攕ome require aspirin, an almost unimaginable situation for people with hemophilia decades ago.

鈥淵ou have to use it, but they鈥檙e probably not taking it as long as people without hemophilia,鈥 Osip said. 鈥淎nd you probably have to be on prophylaxis.鈥

And then there are the multiple medications that men must begin to juggle. Data out of Europe suggest that so-called polypharmacy鈥攖aking lots of medications鈥攎ay be less common for people with hemophilia than those without. But that doesn鈥檛 mean drug-drug interactions can鈥檛 happen, she said. And they can send a person to the hospital. That means that periodically primary care providers should also check a person鈥檚 medications to make sure they still need all of them and at their current dosage鈥攚hile 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.

鈥淧rimary care will monitor the drugs you should be on and tweak it as it needs to be tweaked,鈥 Osip said.

Quality of Life: The Final Frontier

Understanding your medications isn鈥檛 the end of the story on aging with hemophilia, Osip said. There鈥檚 more to life than being disciplined in taking your medicines and following up on appointments. Indeed, there鈥檚 joy, and there鈥檚 sex.

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鈥檛 something to just give up on, Osip said.

鈥淗aving sex is important,鈥 she said. 鈥淚t brings all kinds of great dopamine and serotonin.鈥

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鈥檚 even a book, 鈥淪exual Health in Hemophilia: Preventing Joint and Muscle Injuries,鈥 written by an HTC physical therapist.

鈥淭he thing about aging is that you start to lose stuff that, as a younger adult, you don鈥檛 think about,鈥 Liedl said. 鈥淥ne of the things I lost when I went through cancer treatment was I became impotent. It鈥檚 just another thing you鈥檝e lost.鈥

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鈥檛 dare call them 鈥渟upport groups鈥 for years.

At one group in 2018, he said, 鈥淪omebody said the D word鈥攄epression,鈥 he said. 鈥淏y the time we鈥檇 gotten around the circle, half of them had said the same thing.鈥

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.

鈥淚t feels really good to not be alone,鈥 Francis said he tells the men he works with. 鈥淚t feels good to not be isolated. It feels good to have other guys you can confide in. What we鈥檙e all trying to do here is expand our humanity beyond the narrow limits of what we鈥檝e been taught it means to be a man.鈥

For Liedl, the solution to loss isn鈥檛 isolation; it鈥檚 teaching himself to be curious about new things. Like sex, for instance. You learn to be intimate in other ways.

鈥淲hy say goodbye [to things you鈥檝e lost] when you can change and do something new?鈥 he said. 鈥淒o something else that thrills you. In my life, I鈥檝e 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鈥檓 getting my PhD so I can be a university professor. There鈥檚 always opportunities out there. You just have to find what makes you happy.鈥

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