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More information on sickle cell

Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells. The most serious type is called sickle cell anaemia. Sickle cell disease mainly affects people of African, Caribbean, Middle Eastern, Eastern Mediterranean and Asian origin. In the UK, it’s particularly common in people with an African or Caribbean family background. People with sickle cell disease produce unusually shaped red blood cells that can cause problem because they don’t live as long as healthy blood cells and they can become stuck in blood vessels. Sickle cell disease is a serious and lifelong condition, although long-term treatment can help manage many of the problems associated with it.

Normal capillary and sickle cell anemia.jpg

https://en.wikipedia.org/wiki/Sickle-cell_disease

Sickle Cell Anaemia (HbSS)

Sickle cell disease is an inherited blood condition. Babies with sickle cell disease are usually well at birth but may start to develop symptoms from around 4 months of age. The symptoms of SCD are:

  • Chronic anaemia: long term decreased number of red blood cells and/or the amount of haemoglobin they carry.
  • Infections: people with SCD, especially infants and children, are more likely to experience infections such a flu, meningitis, and hepatitis.
  • Sudden pain crisis: this happens when the sickle blood cells obstruct a blood vessel. This can be triggered by different situations, such as intense exercise, stress, infections, sudden temperature changes and not drinking enough water.
  • Hand-Foot Syndrome: swelling in the hands and feet, often along with a fever, is caused by the sickle cells getting stuck in the blood vessels and blocking the blood from flowing freely through the hands and feet.
  • Acute Chest Syndrome (ACS): blockage of the flow of blood to the lungs can cause acute chest syndrome. ACS is similar to pneumonia; symptoms include chest pain, coughing, difficult breathing, and fever.

Because of the sickling, the patients suffer a chronic haemolytic anaemia and the usual 120 half life of erythrocytes falls to 20 days for patients with sickle disease. The chronic haemolysis results in a rapid production of erythrocytes and a FBC that contains reticulocytes, occasional nucleated erythrocytes, target cells and sickle cells. As the spleen becomes compromised with repeated infarctions, Howell-Jolly bodies are also seen.

Several factors can lead to crises in sickle cell disease: hypoxia, dehydration, vascular stasis, fever, cold and acidosis. Anything that leads to hypoxia will promote sickling. Therefore, patients with respiratory compromise due to infection or chronic respiratory diseases are prone to develop sickle crisis.

There is no single best treatment for all people with SCD. Treatment options are different for each person depending on the symptoms. Treatments can include receiving blood transfusions, maintaining a high fluid intake (drinking 8 to 10 glasses of water each day), receiving intravenous therapy (fluids given into a vein) and medications to help with pain. Nowadays, with the proper treatment, most people affected with SCD live normal long lives.

 

Other types of sickle cell disease

The different forms of sickle cell disease are:

Severe sickle cell disease: symptoms and management similar to that in sickle cell anaemia.

  • HbS/ß thalassaemia

  • HbS/OArab

    Moderate sickle cell disease:

  • HbS/C

  • HbS/DPunjab

  • HbS/Lepore

    Mild sickle cell disease: clinically non relevant

  • HbS/E

  • Hbs/HPFH