World Sickle Cell Awareness Day 19 June

01 Illustration Catherine Rengade 100pxWhat is Sickle Cell Disease? Sickle cell disease, also known as sickle cell anemia, is a hereditary disease, transmitted by both parents, which is characterized by deformation of red blood cells, resulting in abnormal hemoglobin called hemoglobin S or HbS. Because of this deformation, red blood cells become fragile and get easily destroyed. Their lifespan reduces from about 120 days to just twenty days. They are no longer able to pass through the small blood vessels, and sometimes block blood supply to certain organs. In the absence of proper management, the rapid destruction of the red blood cells leads to hemolytic anemia.

 

How does sickle-cell anemia present? : Symptoms can appear as early as 4 months of age, but they vary greatly from one individual to another and over time. Hemolytic anemia is the main sign but is often accompanied by painful attacks and increased sensitivity to infections. As a result, the most common symptoms of sickle cell anemia could include: 

  • pallor,
  • intense fatigue,
  • dizziness caused by anemia,
  • yellow coloration of the eyes and skin. In black people, this symptom only occurs in the eyes

  •  

    dark urine caused by the destruction of red blood cells
  • pain in the limbs, abdomen, back, chest, and sometimes,
  • the bones caused by obstruction of small blood vessels and blood flow,
  • in toddlers, a painful swelling of the feet and hands,
  • in children, it usually results in anemia, fatigue and jaundice, notably yellow color of the eyes,

  • frequent infections due to increased vulnerability of the spleen.

02 Illustration Catherine Rengade 300pxTreatment: how can one live with sickle cell anemia?
Unfortunately, there is no cure for sickle cell anemia. Current treatment is essentially meant to relieve symptoms. Regular follow up and ensuring a healthy lifestyle are recommended. A balanced diet and proper hydration are necessary. I addition to this, it is advisable to avoid factors such as extreme temperatures or altitude, that can trigger crisis. Supplementation with folic acid and iron will help stimulate red blood cell production. In case of aggravated anemia, transfusions can be per-formed.

The best treatment is prevention which consists in carrying out the recommended blood test. Two persons who decide to have a baby should do the test to know whether or not they are carriers of the Hbs or sickle cell anemia trait. That’s why premarital laboratory tests are important. In case this has not been done, it is important to screen the baby during the first week of life and presently it can be done in Cameroon.

Screening at birth is highly recommended. Detected at birth, early preventive care is insured to reduce crisis and pain in the long term. Currently in Cameroon, it is feasible in a few Centres. Since June 2015, a pilot project is ongoing in the following Centres in Yaoundé(CASS de Nkolndongo, Centre Hospitalier d’Essos, Hôpital Gynéco-Obstétrique et Pédiatrique de Yaoundé, Hôpital Catholique Deo Gratias d’Emana) and Douala (Hopital Padre Pio, Hôpital Ad Lucem Bonamoussadi, Hôpital Laquintinie and Hôpital Catholique Albert Legrand) and 18 123 newborns have been screened sofar in these Centres.

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Illustratios:Catherine Rengade