Sickle Cell Awareness

This blog was translated from Dutch/Nederlands. 

This month September is National Sickle Cell Awareness Month in America. I was made aware of this earlier this month by a White House tweet (@WhiteHouse). This special month was set by the United States Congress and 'declared' by the president. The American sicklecell disease patients' organisation has a special website about the activities and points of interest.

I immediately forwarded the link to a Dutch friend of African descent who now lives in America and who suffers from this disease. In my circle of friends I know several people with sickle cell anemia. It is an inheritable disease common in West Africa. Due to a gene abnormality, the red blood cells are not closed as a circle, but semi-circular and open, in the shape of a sickle. Such a semi-open blood cell is less able to transport an oxygen cell and this abnormality leads to a severe form of anemia.

The disease is accompanied by severe pain attacks. I have witnessed this once. Another time I was going to meet a friend at her house but I received a phone call on my way that I had to travel on to the Academic Medical Centre in Amsterdam. There I found her rather cheerful again on an examination stretcher. The pain attack was over, but she still needed to gain strength. Earlier, in high school she had passed out in class one day due to anemia and she was rushed to hospital by ambulance. The attacks are controlled with pain relief and, if necessary, oxygen, fluid infusion or blood transfusion. Healing is not possible. The disease can eventually lead to serious damage to the organs and be life-threatening in the long term.

There is an evolutionary and practical link between sickle cell and malaria. The malaria parasite attacks the red blood cells and becomes deadly, but due to the sickle shape and the shorter lifespan of the red blood cells, the parasite is less likely succesful in a sickle cell sufferer. Conversely, by surviving malaria this gene abnormality in West Africa is more strongly passed on to subsequent generations. Only recently someone pointed out this connection to me and that immediately made a lot clear to me. The first time, years ago, that a friend from Sierra Leone texted me that she had malaria, I was shocked. For us Westerners, malaria can be deadly within days. Before, during and after my visit to Sierra Leone I had to take malaria tablets every day. I know of a Dutch family where a few days after a holiday in Africa the mother unexpectedly fell ill at home during the night and died very soon after. Malaria is a deadly disease for us. But my African friend sent me a happy photo from the hospital a few hours after her first disturbing message. She was feeling much better the same day.

Afterwards I received apps and photo's from Sierre Leone countless times. "Uncle Ytzen, I am sick". When I ask what's wrong, most of the time it's malaria. Like our "flu". I almost always get a picture of the patient on a stretcher in the hospital or at home on the couch with an IV (intravenous drip). Mother or child have rushed from work or school to hospital or home, but after the "drip" they feel a lot better the same day and often they can go back to school or work the next day. The bitter and sad thing is that this dear African friend, who texted fairly carefree about her malaria, died a few years later of complications from another disease common to West African women, namely fibroids, benign tumors in the womb. Due to serious blood loss she did not survive an operation. The average life expectancy in Sierra Leone, one of the poorest countries in the world, is now about 54 years. (*)

Awareness of poverty, inadequate health care and genetic diseases still is very important. 

Sources: 
National Sickle Cell Awareness Month
Declaration president Donald Trump (PDF 4p)

Wikipedia: Sickle cell anemia
Wikipedia: Sikkelcelanemie (Dutch)
Het Sikkelcelfonds: Wat is sikkelcelziekte (Dutch)

(*) Just today (September 25th) the yearly statistics for life expectancy in the Netherlands were published: appr. 80 years for men and 83 years for women.