19 JUNE - WORLD SICKLE CELL AWARENESS DAY

Sickle cell anaemia (SCA) - hereditary haemoglobinopathy associated with a disorder in the structure of the haemoglobin protein. Red blood cells containing abnormal haemoglobin S have a characteristic sickle shape, reduced stability and reduced oxygen transport capacity. Sickle cells die early, which causes a permanent lack of red blood cells and leads to chronic anaemia. In addition, deformed, inelastic red blood cells stick to the vascular endothelium and block blood flow, which can cause pain and other serious complications. In people with SCA, signs of the disease can appear during the first year of life, usually around the age of 5 months. The first signs may include painful swelling of the hands and feet.

The characteristic symptoms of the disease include:

• chronic anaemia;
• pain syndrome (pain can be in the chest, arms, legs and other parts of the body);
• frequent bacterial infections;
• swelling and pain in the joints, arthritis;
• lung function disorders;
• problems in the heart and blood vessels.

What is the danger of sickle cell anaemia?

The most common complications of sickle cell anaemia include pulmonary hypertension, stroke, splenomegaly (enlarged spleen), chronic kidney disease, and visual impairment. In severe cases, complications can lead to death.

What are the treatments for sickle cell disease?

The disease requires lifelong treatment and is poorly treatable. The only FDA-approved treatment for sickle cell disease is bone marrow or stem cell transplantation, which is used in cases of severe SCA for children who have minimal organ damage from the disease. In the future, it may be used for older people as well.

The goal of traditional treatment is to avoid crises, reduce symptoms and improve the patient's quality of life.

Patients with sickle cell anaemia should undergo regular medical examinations and receive timely vaccinations to prevent dangerous infections and their consequences. In the event of any infection or illness, you should immediately contact your family doctor.

https://www.cdc.gov/ncbddd/sicklecell/facts.html

https://medtour.help/uk/disease/serpovidnokletochnaya-anemiya/