Understanding sickle cell
What it is, in plain language.
Written for families in Uganda who have just been given a diagnosis and were not given much else. If you only read one section, read the one on pain crises.
Sickle cell is inherited, not caught
Sickle cell disease is passed down from parents to children through genes. You cannot catch it from someone, you cannot give it to someone by touch, and nothing a parent did or failed to do caused it. It is not a curse and it is not a punishment. Anyone who tells you otherwise is wrong, and the belief does real harm to children who grow up hearing it.
Red blood cells normally look like soft round discs and slip easily through the smallest blood vessels. In sickle cell disease the cells become stiff and curved, like a crescent. Stiff cells get stuck, block the vessel, and starve the tissue behind the blockage of oxygen. That blockage is what causes the pain. The cells also break apart sooner than they should, which is why anaemia comes with the condition.
Trait and disease are different things
Everyone carries two copies of the haemoglobin gene, one from each parent.
- Sickle cell trait means you inherited one sickle gene and one normal one. You are a carrier. You are generally healthy, you will usually have no symptoms, and you can live a completely normal life. You can pass the gene on.
- Sickle cell disease means you inherited a sickle gene from both parents. This is the condition that causes crises, anaemia and organ damage.
When both parents carry the trait, each pregnancy carries roughly a one in four chance the child will have the disease, a one in two chance the child will have the trait, and a one in four chance the child will have neither. Those odds reset with every pregnancy. Having one healthy child does not protect the next one.
Sickle cell trait gives some protection against severe malaria. That is why the trait is common across malarial regions, including much of Uganda. It is also why it has not disappeared over generations.
Pain crises
A crisis is an episode of severe pain caused by blocked blood vessels. It can settle in the arms, legs, back, chest or abdomen. In small children it often shows first as painful swelling of the hands and feet. Crises vary enormously between people and between episodes. Some pass at home in a day. Some need hospital admission and strong pain relief.
What tends to bring one on
- Dehydration, including on hot days and after exercise
- Cold, and sudden changes of temperature, including cold water and rain
- Infection of any kind, including malaria
- Physical exhaustion and stress
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During a pain crisis
- Give fluids. Water, plenty of it, and keep giving it.
- Keep them warm. Blankets, warm room, no cold water.
- Give the pain medicine you were prescribed, at the dose your clinician gave you. Do not wait to see whether it passes on its own.
- Rest. No exertion, no school, no work.
- Go to hospital immediately if there is fever, chest pain or difficulty breathing, a suddenly swollen or tender belly, weakness on one side of the body, drooping face or trouble speaking, a change in vision, a painful erection lasting more than two hours, unusual paleness or floppiness in a child, or pain that the usual medicine is not touching.
Fever is an emergency. In a person with sickle cell, infection can become life threatening within hours. Do not wait until morning.
Getting tested
A simple blood test tells you your status. Testing matters at three moments in particular.
Newborns and young children
Early diagnosis is the single biggest factor in survival. A child who is diagnosed early and started on daily folic acid, preventive antibiotics and vaccinations has a dramatically different outlook from one diagnosed after years of unexplained illness.
Before marriage or children
If both partners carry the trait, you deserve to know that before you plan a family, not after. Knowing does not decide anything for you. It only means the decision is yours and it is informed.
Anyone with repeated unexplained pain or anaemia
Many adults in Uganda live undiagnosed, treated for malaria again and again while the real cause goes unnamed.
If you are not sure where to go, ask us. We can point you to a facility near you that tests, and tell you roughly what it will cost. Get in touch.
Living well with it, day to day
Sickle cell is lifelong, but the difference between good routine care and no care is enormous. Most of what helps is unglamorous and cheap.
- Drink water constantly. More than feels necessary, especially in heat and during illness.
- Take folic acid every day. Your body is replacing red cells faster than usual and needs it.
- Prevent malaria seriously. Sleep under a treated net every night, and follow whatever preventive medicine your clinician has prescribed. Malaria is far more dangerous with sickle cell.
- Keep vaccinations current, and for young children, take the preventive antibiotic exactly as prescribed.
- Stay warm and dry. Avoid cold water, cold nights without cover, and getting soaked.
- Treat fever as urgent, always.
- Ask about hydroxyurea. It is a daily tablet that reduces the number of crises for many patients and is available in Uganda. It is not right for everyone and needs monitoring, so it is a conversation with a clinician, not a decision to make alone.
Children with sickle cell should go to school. Adults with sickle cell work, marry, have children and build careers. The condition sets constraints. It does not set a ceiling.
On stigma
Some families hide a diagnosis. Some children are told not to mention it at school. Some parents are blamed, usually mothers, for something no one chose. This silence is one of the reasons sickle cell stays badly understood in Uganda, and it makes life much lonelier for the person living with it.
You do not owe anyone your medical information. But a teacher who knows why a child misses school, and a workplace that knows why someone needs water and warmth, generally makes life easier rather than harder.
This page is general information, not medical advice, and it cannot account for your particular situation. Every treatment decision belongs with you and a qualified clinician who knows your case.