4 more things to know about sickle cell disease

I have an earlier introductory blog post about sickle cell disease, focusing on what it’s about, and why organs are damaged.  Here are 4 other things you may or may not know about the disorder:

There is a health benefit, in malaria-endemic parts of the world. This is why sickle cell trait/disease has persisted at high levels among some populations.  Individuals with the sickle cell trait are less likely develop severe malaria.  Given how endemic malaria in across equatorial Africa, it is not surprising that a somewhat malaria-resistant population was selected out (as much as 30% of this population has the sickle cell trait).  This would also explain the occurrence of sickle cell trait among the African-American population (7 – 10%), historically descended partly from West and East African ancestors (remember the Trans-Atlantic slave trade).

Chronic illness can affect quality of life and lifespan. Studies among patients with access to excellent health care in the USA have shown that life expectancy may be shortened, with the average person surviving to the 60s (although many of course can do a lot better).  Individuals with sickle cell disease are at risk of infection, complications related to crises, pain, and organ damage.

For those who do have sickle cell disease, treatment revolves around a few issues.  There is much that can be done to help things so all hope is not lost.  Main goals in treatment include:

  • reducing the fraction of sickle cells and abnormal red cell pigment (hemoglobin) with a medicine like hydroxyurea, or using a procedure called exchange transfusion.  Exchange transfusion removes sickle red cells and replaces them with healthy red cells from another person.
  • watching for, and treating organ damage if it occurs
  • treating pain
  • counseling
  • supporting with blood transfusion, folic acid and others

The only possible way to cure sickle cell disease is through a bone marrow or stem cell transplantation.  This is a rather high risk process that is best reserved for children as a result (who might tolerate it better than adults).  The process does not always work to cure sickle cell disease and side effects can be potentially long-term and life-altering.

Have you encountered sickle cell anemia? Feel free to share your thoughts.  If you found this helpful, please share freely with your friends. Hover over the bottom right and click “Follow” to subscribe to my blog.

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