Hemophiliacs Hope for Positive Change with Health Care Legislation The Lantern Emily Tramte

What is Hemophilia? A rare bleeding disorder in which an affected person’s blood does not clot normally due to low factor levels. It is an inherited disease, meaning the disorder is passed on from parents to children through genes. The disorder is carried on the X chromosome, so fathers will always pass it on to daughters but sons have a 50/50 chance of receiving it from mothers. Men are usually the ones affect by the disorder. Women are known to be carriers of the disorder but are symptomatic. There are 14 clotting factors in a person’s body, which are proteins needed to work together with platelets to from blood clots. Someone with Hemophilia A is missing Factor VIII (8) and Hemophilia B is missing Factor IX (9). Three degrees of severity: Severe hemophilia = less than 1% Moderate hemophilia = 1 – 5% Mild hemophilia = 5 – 40%

History of Hemophilia Known as the “Royal Disease” and is traced back to European Royalty. In 1958, average life expectancy of a person with hemophilia was only 27. This was mostly due to the lack of treatment possibilities. At this time, treatment was limited to whole blood transfusions and icing joints. Soon after, researchers developed clotting factor that is prepared from blood plasma, but it wasn’t easy to get. This alone rose the life expectancy by over 35 years. As treatment improved, the hemophilia community faced new challenges in the form of blood borne diseases such as Hepatitis C and HIV/AIDS. Prior to 1985, there was no law in the U.S. to screen blood. This meant that people receiving factor treatments were at risk to contract these diseases. As a result, in the late 70’s and early 80’s many hemophilia patients contracted these diseases and ended up passing away. It was estimated that over 50% of the hemophilia population was affected, if not more. This lead to better ways of treating hemophilia patients, which is the next slide.

Treatment of Hemophilia Treatment has evolved from whole blood transfusions to heat-treated factor to using synthetic recombinant factor that is inject directly into the veins to increase factor levels for a specific time period. The biggest concerns are internal injuries/bleeding and damage done to joints, so treatment has evolved to battle this. The goal of treating is to raise factor levels up to that of a normal person’s levels. There are two main ways to accomplish this, administer factor or use nasal spray called Stimate. Stimate is less effective and raises levels only slightly, for more minor bleeding episodes. Factor will raise factor levels to normal clotting ability. Run into the problem of inhibitors, or antibodies, to the factor product. Two ways to treat, episodically or prophylaxis. Prophylaxis is treating in order to prevent future bleeds. This is the desired treatment among all hemophiliacs but it is also the most costly. Factor also doesn’t last long due to a short half life, so infusing is frequent.

Costs of Treatment Average costs of Stimate nasal spray are around $600-$650, but as stated previously, it isn’t the best way to treat hemophilia. Costs of factor are much higher. $1 per unit. The number of units per person is based off the weight and activities of the patient. So using me as an example, I weight roughly 160lbs and am relatively active so my dosage is 3000 units +/- 10%, meaning I could have a range of 2700 to 3300 units. This would cost, at the most, $3300. Patients that have inhibitors pay even more because they a special type of factor as well as medicine to bypass the inhibitor. Costs can be close to $7000 and up. Last slide said prophylaxis was costly, this is because a person is infusing multiple times a week, usually 3-4. This means their medicine costs 3300 x 4 = $13,200. These costs are for me. Think of someone that’s taller and larger. These aren’t the only costs though, hemophilia leads to other medical problems like arthritis and can lead to brain damage, and these expenses can start to pile up on patients.

Hemophilia and Insurance Paying for the medicine to combat hemophilia was and still is very expensive, and health insurance didn’t help hemophiliacs much. The biggest concern was the lifetime caps on health insurance. Some caps were $1 million and others $2 million. Think about it, if you have someone infusing 3-4 times per week and costing, let’s say $15,000/week with 52 weeks in a year. That’s $780,000 in one year. That’s using very low costs of a patient too, many pay much more per week. When lifetime caps were used up, out-of-pocket expenses started to increase. This led to many patients not getting the proper treatment and developing many problems. The passage of the Affordable Care Act (ACA) in 2010 provided the biggest relief for hemophiliacs. This addressed lifetime caps by, pre-existing conditions, young adult coverage, and other needs important to people with bleeding disorders.

The Economics

In 2008, President George W. Bush signed into law the Genetic Information Nondiscrimination Act (GINA) to protect Americans against discrimination based on their genetic information when it comes to health insurance and employment. As we have talked about in class, people would get discriminated when purchasing health care insurance based on conditions they may have or other circumstances. With hemophiliacs at risk for arthritis and other medical problems, coverage wasn’t likely. We also talked about how the body can be seen as a capital good. We need it to provide for us but it can wear out over time. With hemophilia, a person’s body will wear out faster if they don’t have the proper medicine, decreasing the capital good that the body is worth.