What Is the Prognosis of Hemophilia?

The prognosis for individuals suffering from hemophilia has changed dramatically over the many years that the disease has been recognized, studied and documented. The cause, types and levels of severity that present with the disease were not always understood but the toll it took on its victims was.

Perhaps the most interesting documentation of this bleeding disorder, and the effects it has on those who suffer from it, can be found amongst the pages of history. Hemophilia is also referred to as Royal Disease. England's Queen Victoria (1837-1901) was a carrier of the hemophilia gene and passed it on to her children and subsequently, her grandchildren. Some male relatives died from brain hemorrhages in their early 30s, others lived longer but were not well. Victoria's daughters, Alice and Beatrice, were carriers and introduced it to the Spanish and German Royal bloodlines when they married into the families and had children. Furthering the spread of hemophilia, Victoria's granddaughter married the Tsar of Russia. She gave birth to a hemophiliac son, Alexei. The prognosis turned out to be exponentially worse than anyone could have imagined; it affected an entire country. A palace monk, Rasputin, was the only person able to help Alexei with his pain. Rasputin won great favor with the family and slowly gained power over the family. This gain in power, coupled with the royal family's preoccupation with Alexei's health, were amongst factors leading to the 1917 Russian Revolution.

Facts

• There are three types of hemophilia: type A, type B and the rare type C. This excessive bleeding disorder can be mild, moderate or severe. Approximately 18,000 Americans have hemophilia and as many as 400 babies are born with the bleeding disorder annually. Hemophiliacs bleed longer than usual because their blood is missing vital clotting factors. They experience internal bleeding into joints of the body (hemarthroses), resulting in damage to the joints or crippling them. They may experience internal bleeding into organs. If this uncontrolled bleeding occurs often, the prognosis will be poor, and the patient may die.

Type A Prognosis

• The National Heart Lung and Blood Institute maintains that nine out of 10 hemophiliacs have type A hemophilia and seven out of 10 of these individuals have severe hemophilia. Hemophilia type A is the most common type of hemophilia. Twenty-five percent of these individuals will develop inhibitor antibodies that diminish the effectiveness of treatment. They are more susceptible to blood infections which can lead to early death. They are more likely to be crippled from hemarthroses. They face the least hopeful prognosis of all of the hemophilia types.

Type B Prognosis

• Hemophilia type B, also known as Christmas disease, accounts for 12 to 15 percent of hemophilia cases. This type is linked to inheriting a problematic x chromosome. They are less likely to have severe hemophilia and have a better prognosis. Fifty percent of type B hemophiliacs develop inhibitor antibodies. The effectiveness of treatments are compromised, as with type A, and treatment becomes complicated. These individuals also risk infection and hemarthroses. This complication can contribute to an early death.

Type C Prognosis

• Hemophilia type C occurs in 5 to 8 percent of individuals diagnosed with the bleeding disorder. Aetna InteliHealth maintains that hemophilia is close to being an exclusively male disease; however, females can be diagnosed with type C hemophilia. Type C is not linked to the x chromosome so this type can be passed from either parent to the daughter.

Considerations

• Before the 1950s, individuals with all types of hemophilia were given an extremely poor prognosis; life expectancy was 30 years at the most but many died in childhood. From the early 1950s until 1965, hemophiliacs with mild to moderate hemophilia were given a slightly better prognosis. Yet, many with the severe for died in childhood, young adulthood or were crippled due to hemarthroses. In 1965 it was discovered that the precipitation from thawed plasma was high in a clotting factor vital to hemophiliacs. By the 1970s the treatments became part of every day home life allowing hemophiliacs much more freedom and a longer life.

  According to the National Hemophilia Foundation, 50 percent of hemophiliacs contracted HIV during the 1980s due to tainted blood products. Thousands died. Stricter guidelines and testing restored the safety of the blood supply. Recent technology enables clotting factors to be created in a laboratory, bypassing the need for human donation. Children born with hemophilia today have a favorable prognosis; barring complications, they can expect to live an active and long life.