Thank you to Jeff Johnson, who has severe hemophilia, for visiting Cascadia Tech recently to speak with Dental, Applied Medical Sciences, Medical Assisting and Cosmetology students about the history and realities of hemophilia and bleeding disorders, and especially how they relate to the work that our students who are studying to enter the healthcare field will be doing. He talked about his own experiences living with hemophilia, his pharmacy work managing the care and treatment for individuals living with bleeding disorders, and the evolution of hemophilia and care over the years. He also urged students to be advocates for themselves and for others, and especially for their patients, whom rely on medical professionals at every level to help them acquire the best possible care in order to attain the best possible outcomes.
March is Bleeding Disorders Awareness Month
Bleeding Disorders are more accurately viewed as clotting disorders, as these conditions cause a person's blood to be unable to form a clot, and thus unable to stop bleeding. Superficial wounds, like paper cuts, are not typically a concern for those with bleeding disorders, who suffer most from internal bleeding into joints, organs, and muscle tissue as well as intracranial bleeding in the brain, which can be fatal.
In various ways, bleeding disorders inhibit clotting, but in most cases a protein is missing in the person's blood which is required to complete the clotting cascade. Without that protein, the clotting cascade - or chain - is broken, and no clot is formed. In these incidents, the person's injured blood vessel continues to bleed until medication is administered to replace the missing protein, complete the clotting cascade, and end the bleeding. People with bleeding disorders will bleed for longer periods, but typically do not bleed faster than those without bleeding disorders.
Most bleeding disorders are often genetic (there are very rare cases of acquired hemophilia, though these constitute a very small portion of the bleeding disorders population) and affect both biological males and females, but to different extents depending on each individual’s case. Males will typically live with a more severe form of these disorders, though that is not always the case, as some females will also experience regular internal bleeding, along with heavier menstrual bleeding, more severe bleeding during surgeries and childbirth, and joint bleeding due to trauma.
It is estimated that in the United States, there are between 20,000 and 35,000 individuals with bleeding disorders, and worldwide that number is most often believed to be 400,000 though recent research suggests that due to undiagnosed individuals, increased lifespans due to improving treatments, and the recognition that females are also affected, there may be as many as one million people on Earth who live with a bleeding disorder. Worldwide, only 1 in 4 people with bleeding disorders have access to any care at all, which makes an accurate census of those with bleeding disorders difficult, as population tallying typically comes from counting those who have established care with bleeding disorders doctors. The low number of those who have access to care is also of great concern due to the fact that a person with untreated bleeding disorder only has a life expectancy of approximately fourteen years, with the majority of their life being in a crippled, painful state before their organs shut down, and their life ends in organ failure.
Treatment for bleeding disorders is extremely expensive, with some patients requiring medications that cost as much as $5,000,000 or more every year. Gene Therapy to treat Hemophilia A, one of the most common types of bleeding disorders, has recently been approved and is expected to cost $3.75 million per patient, making it the most expensive medical treatment ever devised, though in the long run still constitutes a cost reduction over time as the patient will no longer be using millions of dollars in other medications every year.
People with hemophilia suffered what is thus far the greatest medical disaster in U.S. history when, in the 1970s and 1990s, the medications given to hemophiliacs worldwide were unknowingly contaminated with both HIV and Hepatitis C. As many as 90% of the hemophiliacs living in the United States at the time were infected with these viruses via their medications, and today less than 2,000 of the 20,000 U.S. hemophiliacs who lived in the U.S. at the time are still living.