Sickle Cell Disease Affects Brain Function

Before medical breakthroughs more than doubled the lifespan of children diagnosed with sickle cell disease, from when they were once expected to barely live into their 20s, researchers now find decades of living with it leads to poor brain function.
Researchers from Oakland Children’s Hospital publishing their stud in the Journal of the American Medical Association, found adults with sickle cell disease had lower IQ scores than control group patients. While, the study did not look into the reasons for the low scores, the authors suggest anaemia caused by the disease could be a factor.
However, researchers found the cognitive problems to be reversible, as no correlating brain damage to explain the IQ results was found. Already, doctors are conducting follow-up studies for determining whether blood transfusions could help patients with memory or cognitive problems.
While, sickle cell has changed from a paediatric disease with increased life spans, quality of life remains to be improved, as the biggest concern now is not the survival of the child, but the impact of chronic organ dysfunction and age.
An inherited disorder, in sickle cell the red blood cells instead of being round, have a distinctive sickle shape, which makes it difficult for the cells to move through blood vessels leading to anaemia. The disease can cause severe pain, stroke and organ failure, andsickle cell disease is generally diagnosed at birth.
Over the past decade, medical advances have made treatment more successful, despite which people with sickle cell disease still die by the time they are 50 years of age. However, longer life spans mean doctors are beginning to discover the long-term effects of living with the disease.
It has long been known that sickle cell disease places the patient at a higher risk for strokes, which in turn cause brain damage and affect cognitive function. However, some sickle cell disease patients with no obvious signs of brain damage, no history of strokes and normal MRIs, seemed to have trouble making appointments or following medical instructions.
The study looked at 141 sickle cell patients and 44 healthy subjects, aged 19 to 55 years, with none of the participants taking medications or having any serious underlying health problems like diabetes, depression or damage to the lungs or kidneys, which might affect theirbrain function.
Given an MRI and an IQ test that included tests for testing their memory, attention level, language skills and ability to follow instructions, the patients were found to have normal IQ range of 85 to 115, with around a third found to be below the normal IQ range.
Hydroxyurea, a cancer pill available generically, is effective in stopping the severe pain, including preventing the need for blood transfusions, which means patients should begin taking the drug at an early age.