Hemophilia is classified as mild, moderate, or severe, depending on the level of clotting factor in the blood. About 70 percent of those with hemophilia have the severe form.
The most important life-threatening complications of the disease are bleeding in the skull and hemorrhages in soft tissue around airways or other internal organs.
About 10 percent of those with severe hemophilia have bleeding in the skull, and 30 percent with the condition die. There is an up to 8 percent risk that a person with the disease will develop what doctors call intracranial bleeding during their lifetime. And a third of all hemophilia deaths is due to bleeding in the skull.
People with hemophilia can also develop chronic, debilitating joint disease.
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.
Preventive measures and early treatment have significantly improved the long-term outlook, or prognosis, for people with severe hemophilia. Two major developments have led to people with hemophiliacs having better lives these days. One is clotting-promotion medicines known as antihemophilic factor concentrates, which prevent serious bleeding. The other is comprehensive care. The two have combined to keep the disease from worsening in many patients, and to increase their life expectancy and improve their quality of life.
What is the life expectancy of someone with haemophilia?
Life expectancy in hemophilia varies, depending on whether patients receive appropriate treatment. Many patients still die before adulthood due to inadequate treatment. With proper treatment, life expectancy is only about 10 years less than healthy men.
Overall, the death rate for people with hemophilia is about twice that of the rate for healthy men. For severe hemophilia, the rate is four to six times higher. In most cases, the hemophilia mortality rate depends on whether a person has other diseases.
Outlook with hemophilia
The life expectancy of people with hemophilia was low before scientists developed factor concentrates. Until the 1960s, life expectancy for those with severe hemophilia was only 11 years. The hemophilia death rate spiked in the 1980s from the impact of the human immunodeficiency virus (HIV) and therapy-associated hepatitis infections. Today, the mortality rate of men with hemophilia is still twice that of healthy men.
Although joint deterioration remains a chronic complication in hemophilia, prompt and proper  treatment can greatly reduce the risk of life-threatening bleeding episodes and the severity of long-term damage to joints.
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