What is Sickle Cell Disease?

Before I went to Zambia I had only heard of Sickle Cell Disease (SCD) mentioned in a classroom lecture, simply because my education happens to be in nursing. My experience in working with people with SCD is entirely from Zambia, a developing country with a poor health systems infrastructure. One could think that in America care for people with SCD will be much more advanced; after all, people here have access to healthcare and medications. However, I have read about people with SCD in America being labeled as drug-seeking when they show up in the ER in a pain crisis, and that SCD is uncommon enough that not all healthcare professionals are fully versed on the needs of a person in crisis. Whether a person with SCD lives in an underdeveloped country or a developed one, treatment of the disease seems to be a bit precarious.

What it is: SCD is an inherited blood disorder that affects the hemoglobin in red blood cells. Under stressful, or low oxygen, situations the sickle hemoglobin can cause normal red blood cells – which are usually soft and shaped like a disc – to become ‘C,’ or sickle, shaped. The sickled cells are hard and sticky and get stuck in small blood vessels, they also rupture easily. This is called a vaso-occlusive crisis, or pain crisis, and causes pain, achy joints, headache, fatigue, jaundice, and other complications. A vaso-occlusive crisis can be life-threatening.  People with SCD will have it their entire life. There is no cure, although there has been some success with Bone Marrow Transplants.

How it is passed on: SCD is not contagious. It is autosomal recessive genetic disorder. Both parents have to be a carrier of the gene – Sickle Cell Trait – before any of their children could be born with SCD. If both parents are carriers of the gene each pregnancy has a 25% chance the baby will have SCD, a 50% chance the baby will be a carrier of the gene, and a 25% chance the baby will have neither the gene nor the disease. SCD is most common in areas where malaria is common, such as Africa, but SCD does not discriminate based on skin color. It simple needs the gene to be passed on. The WHO estimates that “approximately 5% of the world’s population carries trait genes for haemoglobin disorders…” Anyone can have SCD.

How it is managed: The WHO also lists simple strategies for managing SCD, such as: “high fluid intake, healthy diet, folic acid supplementation, pain medication, vaccination and antibiotics for the prevention and treatment of infections, and a number of other therapeutic measures.” Some of these strategies may not be so simple in developing countries where diagnosis is poor and access to medications and supplements is challenging at best, but hydration, healthy diet, and hygiene to decrease the chance of infections can be promoted within a cultural and environmental context realistic in rural communities. I has always been my opinion that when people know what they can do, they will try.

For more information on Sickle Cell Disease the CDC has a great page!

*’Maana hi Ng’ovu’ means ‘Knowledge is Power’ in Lunda, a Zambian tribal language.

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