Hemophilia is a rare clotting disorder that causes blood to not clot properly. It causes more than normal bleeding when someone is injured and can be life threatening. This very rare and usually inherited disorder affects approximately 20,000 people in the United States. The severity of the disease can vary dramatically between affected individuals and the severity, from mild to severe would dictate the level of medical care one needs. It can not only be financially costly, something we will discuss in part 2, but also physically costly; which is what is discussed in part one of this article series.
The basics of hemophilia
Those that are born with hemophilia are born with little or no clotting factor. These clotting factors are proteins that work with platelets for normal blood clotting. There are two distinct types of hemophilia – A and B. Hemophilia A, the most common type affecting 80 percent of all hemophiliacs, is a low or non-existent level of clotting factor VIII (8). Low or non-existent clotting factor IX (9) is Hemophilia B, sometimes called Christmas Disease. Almost 2/3 of all babies born with hemophilia have at least symptomatic parent. In the lottery of genetics, because hemophilia is a mutation of the X gene, babies born with XY genes (males) have a much greater likelihood of being hemophiliacs than those who have XX pair of genes (females). That being said, only about 1 in 5000 males babies born have this condition.
How does hemophilia affect your health?
The physical cost of hemophilia is a decrease the quality of life for some individuals, the amount dependent upon the severity of the disorder. This can feel stifling for a child. Many parents of hemophiliac children limit the child’s activities to keep them safe, but this means that many aspects of a typical childhood are limited for such little ones which can affect their development. “About 25 percent of young people with severe hemophilia — those between the ages of 6 and 18 — have below-normal motor skills, academic performance, and emotional and behavioral problems. With appropriate education and treatment, people with hemophilia can have fulfilling and productive lives, however.”
If the hemophilia is severe, a patient may have to deal with bleeding joints, a painful condition that may develop into chronic, debilitating joint disease. A simple nosebleed or mouth bleed, which can be embarrassing and difficult to deal with for the average person, can become a medical issue for hemophiliacs. Another more serious issue is bleeding in the skull, which affects about 10 percent of those with severe hemophilia. Bleeding in the skull puts pressure on the brain and approximately 30% of patients who experience this do not survive. Due to the complications from hemophilia, it is not surprising that parents of children with this disease are so protective.
Medical care is complicated.
Any injury that causes bleeding, even a minor one, is cause for concern. Surgery can be particularly dangerous for those that suffer with hemophilia. The main treatment option for this disease is replacement therapy. A concentrate of the missing clotting factors is injected or given via IV to replace the protein that is absent or low. The concentrate can be made from human blood that is specially treated to prevent disease, including hepatitis; but some people are concerned about the risk. Before blood was regularly screened for HIV, there were multiple cases of children developing AIDS from infected transfusions. Now, precautions are in place to prevent this. The treatments for hemophilia can also be made from other concentrates not made from human blood that can be stored, mixed, and used at home. Replacement therapy is given on a regular basis helps to prevent bleeding on a prophylactic basis; and there are occasions when additional treatments must be administered due to bleeding that has occurred.
While Hemophilia is a rare disorder, those with it find that it affects almost every aspect of their life. An active life may be difficult, simply due to the dangers that are associated with excessive internal and external bleeding. However, even with the risks, most studies have found that life expectancy is only decreased by ten years, so it is a condition that can be lived with if you have proper preventative care.
If you or a family member are dealing with this disease, you may need assistance coordinating care and ensuring all of your doctors are in communication with each other as well as have all of your records on file in the event of an emergency. Curus complex care services were designed for such an instance. Additionally, if your company is paying higher premiums or drug costs association with an employee who suffers from hemophilia, Curus Navigator services may be able to help your company renegotiate these costs and save both your company and your employees money.
