Sickle Cell Anaemia: myths and facts

In my first blog post on Blood: A mode of transport for the body, I identified the important role played by red blood cells in transporting oxygen from the lungs to the cells in the body. They can perform this role because they contain a protein called haemoglobin. Red blood cells are disc-like shaped and this helps them to move easily through blood vessels.

An abnormality of haemoglobin in the red blood cells can occur, which results in the red blood cells becoming rigid and sickle-like in shape making it difficult and painful to travel through blood vessels. This condition is referred to as sickle cell anaemia.

Normal red cells and sickle red cells. Image Courtesy: National Heart, Lung, and Blood Institute

Sickle cell disease is a genetic disease. It arises in an individual that inherits two abnormal genes haemoglobin S from each parent. It is possible for an individual to inherit a normal haemoglobin A gene from one parent and an abnormal haemoglobin S from the other parent. Such an individual is termed to have the sickle cell trait and often called carriers as they can pass on the abnormal haemoglobin S gene to their offspring. However, carriers do not suffer from sickle cell anaemia.

Inheritance pattern for sickle cell disease. Image Courtesy: National Heart, Lung, and Blood Institute

It is vital to note that in a situation where both parents are carriers of the abnormal haemoglobin S gene, there is always a 25% chance that each child they have will inherit two haemoglobin S genes, thereby having the genetic material that cause sickle cell anaemia. Both genders can have sickle cell anaemia. A simple blood test is used to diagnose the disease and most children are tested at birth.

The World Health Organisation (WHO) estimates over 300, 000 babies are born worldwide with severe forms of these diseases; with majority in low and middle income countries with inadequate healthcare facilities and resources to provide comprehensive healthcare management and facilitate a good quality of life. Sickle-cell disease is prevalent in Sub-Saharan Africa, parts of the Indian sub-continent and within the black communities in other parts of the world. In the UK, it is estimated that around 15,000 people have sickle cell disease with around 270 babies with sickle disease born every year.

The symptoms of sickle cell disease usually begin in early childhood, from as early as 5 – 6months. These can vary from person to person, but it is mainly reflected as acute pain crisis. These can lead to increase fatigue due to low oxygen levels in the body because of low haemoglobin to transport oxygen around the body. Persistent symptoms can expose the patient to further complications including acute chest syndrome, chronic pain, eye problems, heart problems, including coronary heart disease and pulmonary hypertension, infections, joint problems, and severe anaemia. Most times, an individual that has sickle cell anaemia disease does not know what triggers the crisis.

A key strategy in managing sickle cell anaemia is to reduce the occurrence of a sickle cell crisis by avoiding possible triggers. These include drinking lots of water to avoid dehydration and dressing warm to avoid getting cold.

Treatment of a sickle cell anaemia patient may include prescribing over the counter painkillers – paracetamol, stronger painkillers – morphine, antibiotics – penicillin to treat infections, vaccinations against potential infections, folic acid to treat anaemia. In severe cases, blood transfusion and hydroxycarbamide can be used.

The only cure that has been developed to treat sickle cell anaemia disease is stem cell or bone marrow transplants. However, due to associated risks and the challenge of finding a matched donor, these are rarely used to treat the disease.

There is ongoing research for the potential use of genetic therapies to help develop new treatments or find a cure for sickle cell disease. These are still at early-stage inquiries and will have associated potential risks when fully developed.

In writing this blog post, the principal aim is to create awareness about sickle cell anaemia disease. More public communication and engagement campaigns, resources, events and strategies used the more likely members of the public will know more about the disease particularly sickle cell anaemia trait carriers. Some campaigns within the African community in the UK are run along marriage counselling sessions to sensitise intending couples to the implication of the disease. An example is the event – JB4Marriage, Just Before Marriage – held by RCCG Living Waters Parish, South Wimbledon.

Image Courtesy: JB4Marriage by RCCG Living Waters Parish

An event like this shows the collaboration between science, faith groups and the government. Organising events of this formats in conjunction with local government officials will create more urgency and relevance with the authorities and might result in increase funding for research into sickle cell anaemia.

The Sickle Cell Society in the UK works to create a forum to identify, celebrate and promote the reality that individuals with sickle cell anaemia can live a fulfilled and impactful life.

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