The availability of plasma-derived factor concentrates, starting with cryoprecipitate in the mid-1960s for hemophilia A, and factor IX complex for hemophilia B in the late 1960s and early 1970s, began the process of bringing hemophilia lifespans to today’s near-normal levels. Unfortunately, those life-giving products came with the risk of infectious diseases that also limited the lifespans of people with hemophilia.
We might have known more about aging with hemophilia by now, but the AIDS crisis in the 1980s wiped out a huge part of our community. Our hemophilia elders are a sparse, but lively, group. We’re now in an era of safe, highly effective products with a healthy middle-aged population who can expect to find out what happens as they age. The Hemophilia Treatment Center (HTC) network, in addition to providing care, is also a resource for keeping track of the health of patients over their whole lifespan. That information is invaluable for researchers looking to see what happens to patients under many conditions, including as they age.
One of the primary principles in medicine is that every person is different. That’s why we have to study a large number of subjects in order to find out what the typical or average behavior is. I have a friend in the hemophilia B community who had a heart attack. He survived and is now probably doing better than before his heart attack. Should we use him as a model for how to treat a heart attack in a person with hemophilia? No, his case is one good piece of data, one piece of evidence, but we need much more before we really know what happens to most people and the best way to treat them.
So, let’s start with heart disease, the number one killer of Americans. Since a heart attack is caused by a clot blocking one of the coronary arteries that provide blood to the heart, many people have proposed that having hemophilia should tend to protect patients from heart attacks. However, heart attack clots are mainly caused by platelets, and most people with hemophilia have normal platelets. The research shows that we can’t really guess about these things. From the few studies that have been done, it appears that people with hemophilia have just as many heart attacks, but they tend to survive them more than does the general population.
As I’ve gotten older (I’m 72 and don’t have hemophilia), I’ve come to realize that the greatest danger is not dying but being debilitated. It’s quality of life. You might survive a heart attack, but if it damages your heart so you can’t participate in your normal activities, that can significantly reduce your quality of life. You not only want to live a long time; you also want to be as healthy as possible.
The typical hemophilia patient brings a number of conditions with them into old age. One of the most important is high blood pressure (HBP). We don’t know why, but people with hemophilia have a greater tendency to develop HPB, which can cause a number of issues including heart disease, stroke, dementia, kidney disease, eye problems and more. HBP is called a silent killer because people often don’t know they have it until it causes a problem. It’s important to know your blood pressure, and if elevated, get treatment.
Another thing some hemophilia patients bring into old age is infectious diseases, especially those old enough to have been treated with plasma-derived products. Infections with HIV, the AIDS virus, and the hepatitis C virus are common in older people with hemophilia. We also don’t know that much about AIDS in the elderly; people with AIDS are only more recently living into old age. We do know about hepatitis C, and it presents its own set of challenges.
People with hepatitis C tend to develop liver cirrhosis and liver cancer. Now that we have fairly easy treatments for hepatitis C, people are encouraged to get tested and treated. The longer you wait, the more potential damage to your liver. Even after treatment some people are left with significant liver damage that might still lead to liver cancer. Hepatitis C is also a risk factor for diabetes, another common disease of aging.
One obvious issue many hemophilia patients bring into old age is mobility concerns. Many people have damaged joints from repeated bleeds. It doesn’t get any better as you get older. Some of this can be treated, but the important goal is to prevent further damage. Keeping up with your prophylaxis to prevent future bleeds is key, as is keeping active. We know that exercise is instrumental in strengthening your joints.
Mobility issues can lead to a higher risk of falls and other injuries. Falls are often the beginning of the end for elderly people. In addition, there is some indication that people with hemophilia tend to have low bone density, potentially allowing bones to fracture more easily. Accidents are the fourth leading cause of death (they were third before COVID came along). Accidents become more prevalent in older people because of both physical and mental decline.
Aging can also interfere with treatment. Patients may have more problems finding and accessing veins for intravenous infusion. This might seem like a small thing, but it can have a significant effect on an older person with hemophilia who needs factor. Vision and coordination problems may also interfere. The difficulties may also lead to anxiety, missed infusions, and reduced independence. The development of more treatments that can be given subcutaneously will be a big improvement.
The community has begun to focus more on mental health, and that can be a special need for older people, with or without hemophilia. Retirement can bring the loss of a sense of purpose. Reduced independence can bring the need for additional help. Loss of friends and family members can result in loss of caregivers and loss of companionship. Depression, anxiety, and potentially dementia can undermine not only one’s mental health, but also their physical health.
After all that, there are conditions affecting everyone with or without hemophilia as they age, for instance, cancer, diabetes, and COPD. We don’t yet know whether hemophilia influences such conditions, but even without additional complications, they are serious.
All of this also applies to carriers and women with hemophilia. Unfortunately, they face a double burden. Not only do we not know much about women aging with hemophilia, we don’t even know a lot about women aging without hemophilia. Women have been ignored for far too long. We are beginning to understand, for instance, that heart disease works differently in women. The symptoms of a woman having a heart attack can be quite different than those in a man. These differences can delay getting treatment and lead to greater damage to the heart.
Aging with hemophilia is one of this community’s next adventures. A 2018 editorial in the medical journal Haemophilia [Haemophilia, 2018, 24, 15-16] ends with this statement: “Haemophilia care is no longer about treating bleeds but rather about treating the individual as a whole with all their accumulating comorbidities.”
So, what should the aging person with hemophilia do? Pay attention! Many of the conditions listed above are easier to treat when they are discovered early. Have an annual physical exam, and don’t delay asking your doctor about any new symptoms you develop. One of the big advantages for hemophilia patients is the HTC network. They are dedicated to comprehensive care and encourage annual visits. Beyond that, take good care of yourself. Eat healthy (lots of fruits and vegetables), exercise, get enough sleep, stop smoking, and try to reduce stress.
