Sickle cell disease

What is sickle cell disease?

The sickle cell anemia or anemia Sickle cell disease is an inherited disease of the red blood cell. The hemoglobin of people with sickle cell anemia is called hemoglobin S (HbS). Under conditions of low oxygen concentrations (deoxygenation), HbS forms rigid fibers, changing the shape of the red blood cell which becomes "sickle".
The sickle-shaped red blood cell is less deformable so it gets stuck in the blood vessels causing pain (painful crises) or "breaks" into the circulation (anemia).
Painful crises or anemia can lead to hospitalization and are at the root of the health problems that affect patients with sickle cell anemia.

How is it transmitted?

If only one parent carries the Hb S gene, the children will have a 25% chance of being a carrier (AS).
If both parents carry the HbS gene, the children will have a 25% chance of having sickle cell disease (SS), 50% of being carriers (AS) and 25% of being healthy.
If one of the two parents is affected by sickle cell disease (SS), the child will be sick only if the other parent is also a carrier of HbS or b-thal. Otherwise the child will be a Carrier (AS).

How are the red blood cells of the patient with sickle cell disease?

In conditions of low oxygen tension (deoxygenation) they deform becoming "sickle" and create obstructions in the blood vessels.

What are the main problems?

The main problems are anemia and painful crises.

What triggers a crisis?


  • Intense exercise
  • The altitude
  • The rapid transition from a warm to a cold environment or exposure to cold in the absence of adequate protection
  • Dehydration caused by inadequate introduction of fluids, vomiting, diarrhea or infections and fever.


How can I avoid the pain?


  • Hydrating (2-3L / day) with particular attention in case of physical activity, fever, high temperatures, long journeys
  • Covering yourself with warm clothing in case of low temperatures 
  • Relaxing and breathing deeply while maintaining a prolonged exhalation


If it doesn't pass what do I do? When should I go to the hospital?


  • In case of severe pain that does not subside after 2 days
  • If there is vomiting or diarrhea
  • If the fever is high (> 38 ° C)
  • If you have difficulty breathing or cough
  • If the eyes turn yellow
  • If severe headaches, drowsiness or mental confusion appear


Are there any chronic therapies?


  • Hydroxyurea which increases the synthesis of fetal hemoglobin and reduces sickle red blood cells
  • The transfusion of healthy red blood cells or the exchange of healthy red blood cells

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