Hemophilia is a rare genetic disease in which your blood doesn’t clot properly. As a result, when you bleed, it can take a really long time to slow down the bleeding, and it might not even stop. This disease is usually seen in younger people because it starts in early life. There was a time when the lifespan of a hemophilia patient was short. But things have changed. With the advancement of modern medicine, people with hemophilia can now live much longer. However, with growing age, there come some complications. In this article, we are going to discuss hemophilia and aging, and the complications of hemophilia in elderly people.
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About Copay AssistanceWhat Is Hemophilia?
Hemophilia is a rare genetic disorder that’s typically more common in males. People with hemophilia aren’t able to form blood clots because they’re missing certain proteins called clotting factors. These clotting factors are needed for the steps of blood clot formation. There are two main types of hemophilia: hemophilia A and hemophilia B. Hemophilia A is caused by a lack of clotting factor VIII, and hemophilia B is caused by a lack of clotting factor IX. Both types of hemophilia lead to longer bleeding times, which harms joints and muscles over time. People with hemophilia often experience symptoms like joint pain, swelling, and headaches. They may also bleed internally and have trouble moving. They must manage these symptoms for the rest of their lives with medications that replace the missing clotting factors to help prevent or control bleeding episodes.
Hemophilia and Aging
With the advancement in the medical sector, the life expectancy for people with hemophilia has improved a lot. In 1920, the life expectancy of hemophilia patients was just 11.4 years. This has now increased to nearly a normal lifespan today [1]. According to a 2011 report from the CDC, there are 18,270 people in the U.S. with hemophilia. Of these people, 2,278 (or 13%) are between 45 and 64 years old, and 530 (or 3%) are 65 years or older. The number of people with hemophilia who are over 65 is expected to grow a lot in the next decade. But living longer brings new challenges. The natural aging process can make hemophilia complications worse. Here are some of those complications:
1. Viral Disease
Many viral infections, like hepatitis B, hepatitis C, and HIV, create great challenges for older hemophilia patients. The introduction of a therapy known as HAART has improved the survival of HIV-positive hemophilia patients. But, many people also have hepatitis B and C, especially those born before 1975 [2]. This is because hemophilia patients need some blood products, and back then, there was a lack of safety measures. Hepatitis B and C can cause severe liver disease, liver failure, and liver cancer. These conditions are difficult to manage, especially in old age. Furthermore, liver damage from these viral infections increases the risk of bleeding complications. This can make treatment more complex and reduce overall life expectancy.
2. Development of Inhibitors
Inhibitors are antibodies (proteins) that your body creates against the treatment you receive. In the case of hemophilia, your body can develop inhibitors against factor VIII or factor IX therapy. These inhibitors can reduce your treatment’s effectiveness, which can lead to hemophilia symptoms coming back, such as bleeding, joint damage, and pain. Inhibitors develop in about 30% of hemophilia A and 5% of hemophilia B patients, often within their first 20 – 50 treatments. The risk of developing inhibitors increases with age and repeated treatments. By age 75, about 12% of people with mild or moderate hemophilia may have inhibitors [3]. So, as you age, managing inhibitors may require special treatments and therapies.
3. Heart-Related Complications
The good news for hemophilia patients is that hemophilia may lower the risk of heart disease due to reduced blood clotting. A European study has shown that heart disease-related deaths are lower in men with hemophilia [4]. Despite this, age, high blood pressure, diabetes, and high cholesterol still pose heart risks. If an elderly hemophilia patient develops heart problems, then managing it becomes difficult, especially with surgeries and blood thinners, which increase bleeding risk [3].
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4. Cancer
Data suggests that older people with hemophilia are at a higher risk for certain cancers, including liver cancer and lymphoma. Liver cancer is a significant cause of death in people with hemophilia, especially in those over 45 or those who got hepatitis C at an older age [2]. So, for older people with hemophilia, regular checks are recommended to catch cancer early.
5. Joint Disease
Repeated bleeding episodes over time in people with hemophilia can lead to long-term joint problems and joint disease, especially if there’s bleeding into a joint. This is more serious in older patients and can cause significant pain. Moreover, managing pain is tough due to the risks associated with common painkillers. A common solution for managing pain is joint replacement surgery. However, bleeding management during surgery can be a challenge.
6. Managing Multiple Medications and Drug Interactions
Older people often take multiple medications for various medical conditions. So, managing hemophilia in older people is complex because certain medications, including painkillers (e.g., NSAIDs like ibuprofen [Advil, Motrin]) and heart medications (e.g., aspirin, clopidogrel [Plavix]), can further raise the risk of bleeding.
Hemophilia and Aging: The Bottom Line
As people with hemophilia age, they face new health challenges. They need treatment for their hemophilia and other conditions. For them, starting early treatment, getting regular checkups, and making personalized care plans are important. This can help them live a better life.
REFERENCES:
- Angela K. (n.d.). Hemophilia and Aging. https://www.hemophilia.org/sites/default/files/document/files/nurses-guide-chapter-17-aging.pdf
- The aging patient with hemophilia: Complications, comorbidities, and management issues | hematology, ash education program | American Society of Hematology. (n.d.). https://ashpublications.org/hematology/article/2010/1/191/96370/The-Aging-Patient-with-Hemophilia-Complications
- Shapiro, S., & Makris, M. (n.d.). Haemophilia and ageing. https://eprints.whiterose.ac.uk/139926/3/BJHaem%20review%20Final%20PDF.pdf
- Schutgens, R. E. G., Klamroth, R., Pabinger, I., Malerba, M., & Dolan, G. (2014). Atrial fibrillation in patients with haemophilia: a cross‐sectional evaluation in Europe. Haemophilia, 20(5), 682–686. https://doi.org/10.1111/hae.12445