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Medical Zambia

Sickle Cell Anaemia

I have seen more children with sickle cell disease during this stay in Zambia than my other two tours. It is a miserable, dreadful disease.

It is a genetic disorder of haemoglobin which alters the biconcave disc shape of red blood cells into a crescent or sickle. Being an autosomal recessive condition, a sufferer needs to inherit a sickle gene from both parents to be severely affected (HbSS). Only having one gene (sickle cell trait, or HbS) causes no problems (unless the oxygen level of the blood is reduced) and confers some protection against malaria. (This may be why evolutionary pressure has not eliminated the disease.) Paradoxically, patients with sickle cell disease (HbSS) are more susceptible to the dangerous form of falciparum malaria which can be fatal. 

Fetal haemoglobin (HbF) helps to protect babies for the first six months of life, but as HbF disappears, the affected child starts to become symptomatic. The abnormally-shaped red cells clog up the micro blood supply to bone marrow, causing acute and long-standing pain in the bones. The deformed red cells cannot revert to normal and they burst or haemolyse. The child becomes anaemic with less than 50% of the amount of haemoglobin as a child without sickle cell disease.

The sickling blood cells are filtered out by the spleen, which grows in size and then becomes destroyed as its capillaries clog up. The spleen is the location of immunological memory cells, which are lost when the spleen infarcts. This makes the child more susceptible to bacterial infections, especially streptococcal, meningococcal and salmonella (typhoid) infections.

Sickling cells also damage the lungs, causing chest pains and difficulty breathing. A stroke can result from sludging of red blood cells in the brain.

The child’s growth falters and the child is often stunted. Leg ulcers are a common complication which are very slow to heal.

Sickling crises can occur spontaneously but may be precipitated by lack of oxygen, dehydration or altered body temperature.

At the health centre, on Fridays, the laboratory runs a batch of tests for sickle cell disease. A drop of blood is mixed with a reducing agent to take away oxygen, which causes the cells to change into their classic sickle shape, seen under the microscope. Unfortunately, we have run out of reagent to do this test, so we have to send suspected children to the nearest district hospital, an hour away by bush taxi.

There is no cure for sickle cell disease apart from a stem cell transplant – but this is not an option in Zambia. We can correct the anaemia by blood transfusion, but this may only last a few weeks. We avoid giving iron tablets because of the risk of iron overload from frequent transfusions. Sadly we don’t have any stocks of folic acid, which helps red cell production. At the time of writing, we don’t even have paracetamol to treat painful crises. We do have polyvalent pneumococcal vaccine.

This is another condition where we can make the diagnosis but offer only limited symptomatic treatment with no hope of a cure. Most of our patients will not reach adulthood. It makes me feel depressed and impotent.

By Dr Alfred Prunesquallor

Maverick doctor with 40 years experience, I reduced my NHS commitment in 2013. I am now enjoying being free lance, working where I am needed overseas. Now I am working in the UK helping with the current coronavirus pandemic.

11 replies on “Sickle Cell Anaemia”

Your excellent review of SS anemia and its effects reminds me how lucky we are to have medical intervention allowing most everyone to live with this painful disease. How difficult for you to see this condition daily knowing ‘only if’ you had this, that, and the other to assist these patients in their suffering. I hope you and your team are able to replenish your supplies many times over in the near future and hurray for those who have the opportunity to travel and treat where needed. dennyho MT(ASCP)

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Sorry to disappoint you, Dennyho, but the drug situation here is getting worse. I am currently trying to find a way to treat a dozen (these are the patients I know about) with severe enduring mental illness. The nearest psychiatric facility is 100 km away. Can you imagine going that distance with a disturbed relative on public transport to get their monthly prescription of drugs?

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Your closing sentence broke my heart. To you the consultation may feel like a failure, but for them it may be the first time someone has offered comfort and hope. I admire you so much for helping these patients.

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Marian, there is no gofundme page. Zambia is not in extreme poverty, but the government chose not to fund health care sufficiently. There is a mentality which dismisses the lack of basic drugs with the phrase, “if you’re ill you need to pay for your treatment, not to rely on the government”. But I see patients in dire poverty, old folks with no means of support, young children, who can’t buy medicines. If we donate medication it lessens the pressure on the government to fund the service adequately. I saw a lady today who had been beaten up by a man. She couldn’t see out of a damaged eye. To get a police report and treatment, she was directed to the nearest hospital, costing $3, which she didn’t have. No money means no treatment, possibly losing the sight in her eye, and no medical report which reduces her chance of justice. Grim being poor in Africa.

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