Sickle cell disease

Sickle cell disease or “Drepanocytosis” is a disease of the Hemoglobin in which the mechanism of delivery of the oxygen is affected in the red blood cell throughout the body. In this disorder, an atypical hemoglobin called “Hemoglobin S”, can distort red blood cells into a sickle or in a crescent shape.

The disease is manifested in the early childhood with characteristic signs and symptoms due to a low number of red blood cell (anemia), repeated infections and periodic episodes of pain. Symptoms vary from person to person being mild for some, but others may require hospitalization for pain control or more serious complications.

The red blood cells sickle and break down prematurely causing the anemia which can be responsible of shortness of breath, while the red blood cells sickle, they break down prematurely, leading to anemia, fatigue, shortness of breath, and finally delayed in growth and development.

Often jaundice can be present due to the rapid breakdown of the red blood cells causing vaso-occlusive crisis, responsible of the painful episodes, following the entrapment of the red blood cells in the small vessels. Such mechanism will impede on the blood flow and deprive the nearby tissues and organs from an adequate nutrition. The occlusions of the vessels. promote organ damage or failure especially in the lungs, kidneys, spleen, brain and bone. A serious complication of this disease involves the vessels supplying the lungs creating a central or a pulmonary hypertension leading to heart failure. We have debated the problem with college athlete with the Sickle cell Trait and the NCAA requirements for sport participation, in the already published AMHE Newsletter (# 237, sept 3, 2018).

Sickle Cell Disease is hereditary and manifests itself in acute crises. If in the past, it was considered like a disease strictly found in black, it can be found now all over the world and is even seen in places where you would not expect it to be present.

Sickle cell Disease (SCD) is an autosomal recessive disorder that results in hemolytic anemia related to an abnormal hemoglobin and a low erythrocyte level. SCD is characterized by vascular occlusive episodes, visceral sequestration and aplastic/hemolytic crises, occurring commonly in bones. We will soon, in a near future, expose the orthopedic manifestations of the disease in one of the AMHE Newsletter to come. But it may be relevant today to only mention some of the orthopedic manifestations like the “hand and foot syndrome”, Osteonecrosis or “Avascular Necrosis”, Acute and Chronic Osteomyelitis, Septic arthritis, Rhabdomyolysis etc.

Today, it is my privilege to have Rita Bellevue MD one of our elder, specialist in the treatment of this disease, bring you some pearls in the history and treatment of such phenomenal disease. The AMHE hopes soon to be able to open an active clinical center in Darbone, Haiti, near Leogane to support the need for a materno-infantile population as well as the one suffering from Sickle cell Disease.