Henry (Hank) Bernstein, D.O. is a Senior Lecturer in Pediatrics at Harvard Medical School and Professor of Pediatrics at Dartmouth Medical School. Formerly the Associate Chief of General Pediatrics and Director of Primary Care at Children's Hospital Boston, he currently is the Chief of General Academic Pediatrics at Children's Hospital at Dartmouth. He has extensive and varied experience as a primary care pediatrician, and is a spokesperson for the news media on a variety of pediatric health care topics, including vaccination, common childhood illnesses, and practical information for caregivers.
Can sickle cell anemia be fatal since it can cause blood clots and blocked blood vessels?
Yes, it is possible for sickle cell anemia to be fatal.
About half of all deaths related to sickle cell disease are from infection. Sickle cell disease can make it more difficult to fight infection, particularly if the sickle cells have caused damage to the spleen. The next most common cause of death in sickle cell disease is a stroke.
People with sickle cell disease have a shortened life expectancy. In a large study in 1994, half of all men with sickle cell disease survived past age 42; half of all women with sickle cell survived past 48. At least one person with sickle cell anemia has lived to age 85.
It is possible for severe complications of sickle cell to occur at any age, and people with the most severe disease can die in childhood. How often does this happen? The best way to answer that question is to learn from real numbers.
One study followed 711 children from the day they were diagnosed by newborn screening tests. They were monitored for most or all of their childhood and, when possible, until they were 18. From this group, 25 children died in childhood, most commonly between ages 4 and 6. Fifteen of these deaths were felt to be directly related to sickle cell disease. (For example, some died of infections that were made more severe due to the fact that sickle cell had damaged the child's spleen, limiting their ability to fight infection.)
These statistics translate to one childhood death for every 47 children who have sickle cell disease.