Causes Of Sickle Cell Crises (Causes)

Sickle cell disease (SCD) is caused by an inherited genetic mutation in the hemoglobin gene that affects how red blood cells develop.

 Sickle cell trait is inherited from one parent.

• Inheritance: Sickle cell disease is an autosomal recessive condition, meaning a person must inherit two mutated hemoglobin genes, one from each parent, to have the disease. 
• Hemoglobin S: The mutated gene results in an abnormal hemoglobin protein called hemoglobin S, which causes red blood cells to become rigid and sickle-shaped. 
• Sickle cell trait: If a person inherits one mutated hemoglobin gene and one normal hemoglobin gene, they have sickle cell trait and are generally healthy. They can still pass the mutated gene on to their children. 
• Chance of passing on: If both parents have the sickle cell gene, there’s a 1 in 4 chance of each child they have being born with SCD. 

Approximately 1 in 79 babies born in the UK carry sickle cell trait.

Sickle cell carriers do not have sickle cell disease themselves. However, there is a chance that they could have a child with a sickle cell disease if their partner is also a carrier.

SCD is also known as sickle cell anemia, and it usually affects people at around 5–6 months of age. Treatments include vaccination, antibiotics, pain medication, blood transfusions, folic acid supplementation, and high fluid intake. 

How Sickle Cell Affects the Body

Sickle cell disease is an inherited blood disease that affects red blood cells and can cause a variety of health problems.

Sickle cells will normally block blood flow to organs thereby depriving the affected organs of blood and oxygen. Again, the blood also is short in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including the kidneys, liver and spleen, and can be fatal.

• Painful episodes: Sickled red blood cells can get stuck in small blood vessels, blocking blood flow and causing episodes of severe pain. These episodes, also known as sickle cell crises, can last from a few hours to more than a week. 
• Anemia: Sickled red blood cells break down prematurely, leading to anemia. Symptoms include fatigue, shortness of breath, and delayed growth in children. 
• Organ damage: Sickled red blood cells can block blood flow to organs like the lungs, kidneys, brain, and spleen, depriving them of oxygen-rich blood. 
• Stroke: Sickle cell disease can increase the risk of stroke. 
• Eye problems: Sickle cell disease can damage blood vessels in the eye, especially in the retina. 
• Kidney problems: Sickle cell disease can make it hard for the kidneys to concentrate urine. 
• Liver problems: Sickle cell disease can cause liver damage when sickled red blood cells block blood vessels in the liver. 
• Gallstones: Sickle cell disease can lead to gallstones. 

How long can a sickle cell person live?

Although new treatments and optimal disease management have improved the life expectancy of persons who have Sickle cell disease, with patients now surviving beyond the age of 50 years, life expectancy is still shorter than that of the general population by about 20 years, and the quality of life is often poor.

• Life expectancyPeople with sickle cell anemia typically live to be 40–60 years old. However, some people with milder types of SCD may live as long as people without the disease. 
• Factors that affect life expectancyPeople with SCD who manage their disease well can live long lives. This includes:
  • Having strong family support 
  • Taking medications and attending appointments regularly 
  • Making lifestyle changes 
  • Not smoking 
  • Limiting alcohol consumption 
  • Maintaining a healthy body weight 
• Medical interventions: Medical interventions like newborn screening, pneumococcal vaccines, and prophylactic antibiotics have improved the life expectancy of children with SCD. 
• Stem cell transplantation: Stem cell transplantation is the only potentially curative treatment for SCD.