Sickle cell anemia is a genetic disease condition that is passed down through generations. Unlike the normal shape of a disc, red blood cells take on a sickle or crescent shape.
It is these red blood cells that provide the body with oxygen. The number of red blood cells in sickle cell disease is decreased. The result is less oxygen getting to tissues and problems all over the body.
In addition, sickle-shaped cells can block blood vessels in the brain, leading to strokes. Therefore, we aimed to compare long‐term blood transfusion schedules to other transfusion schedules or other ways of preventing stroke.
Causes, Incidence, and Risk Factors
In sickle cell anemia, an abnormal type of hemoglobin, hemoglobin S, is responsible for carrying oxygen. Hemoglobin carries oxygen in red blood cells. Hemoglobin S changes the red blood cells. As a result, red blood cells become small and fragile, resembling crescents or sickles. These sickle-shaped red blood cells are not able to efficiently transport oxygen throughout the body.
Abnormal blood cells provide less oxygen to your tissues. Their abnormal shape can also make them quickly stuck in small vessels and break apart. A blocked blood vessel can further limit the amount of oxygen that reaches body tissues. Typically, sickle cell anemia is inherited from both parents—the sickle cell trait results from having the sickle cell gene from just one parent. The sickle cell trait does not cause sickle cell anemia symptoms.
Those of Mediterranean and African descent are much more likely to have sickle cell disease. Other countries with this condition include South and Central America, the Caribbean, and the Middle East.
Symptoms Of Sickle Cell Anemia
The onset of symptoms usually occurs after four months of age. Individuals with sickle cell usually experience painful episodes known as crises. They can last for hours or days.
Symptoms of sickle cell anemia initially may include acute pain in hands and feet. But in sickle cell crisis, there is chronic pain in the lower back, leg, joints, or chest. Some people experience a crisis every few years. Other people experience many episodes per year. Severe form of pain crises may require hospitalization.
Vaccines against hepatitis B virus and human papillomavirus (HPV) are optional.
People with sickle cell anemia should take folate supplements. These help prevent neural tube defects such as spina bifida.
Symptoms Of Severe Sickle Cell Anemia Include:
- Extreme tiredness
- Paleness
- Rapid heart rate
- Shortness of breath
- Yellowing of the eyes and skin (jaundice)
Complications with Sickle Cell Disease
As a Result Of Abnormal Sickled Cells Blocking Small Blood Vessels, The Following Symptoms May Occur:
- Priapism (painful and prolonged erection)
- Vision problems or blindness
- Small strokes that cause mental confusion or problems with thinking
- Loss of consciousness
- Lower leg ulcers (in adolescents and adults)
Over time, the spleen stops functioning. This can result in painful enlargement due to large numbers of abnormal cells pooling inside. As a result, people with sickle cell disease may experience symptoms of frequent infection, including:
- Bone infection (osteomyelitis)
- Gallbladder infection (cholecystitis)
- Respiratory infections (penumonia)
- Urinary tract infection
Other Symptoms Include:
- Delayed growth and puberty
- Painful joints caused by arthritis
Signs and Tests
The Following Diagnostic and Monitoring Tests Are Often Performed On Sickle Cell Anemia Patients:
- Bilirubin
- Blood oxygen saturation
- Complete blood count (CBC)
- Hemoglobin electrophoresis
- Serum creatinine
- Serum potassium
- Sickle cell test
Sickle cell disease can also be diagnosed at birth. This can be done by testing the amniotic fluid or a sample of placenta. Early testing can help with timely treatment.
Treatment
Cure for sickle cell seeks to limit the number of crises, manage the symptoms, and control them. A person with sickle cell disease needs ongoing treatment even when they do not have a crisis. Getting sickle cell anemia care from health care that takes care of many patients is best.
A folic acid supplement is helpful for people with this condition. It encourages red blood cell production.
Treatment Options For Sickle Cell Crisis Include:
- Regular blood transfusions (for stroke prevention).
- Pain relief drugs.
- Plenty of fluids
Sickle Cell Anemia Treatments May Also Include:
- Hydroxyurea (Hydrea). In some people, this can reduce severe pain episodes (including chest pain and breathing difficulties)
- Antibiotics. Aid in preventing the bacterial infections that are commonly seen in sickle cell disease patients
Complications Of Sickle Cell Anemia Can Be Treated With The Following Therapies:
- Kidney transplants or dialysis
- Psychological counselling
- Gallbladder removal in people suffering from gallstones
- Hip replacement in patients suffering from avascular necrosis of the hip
- surgery for eye disorders
- narcotic addiction treatment
- leg ulcer treatment
Bottom Line
In most cases, sickle cell anemia cannot be cured with stem-cell transplant or bone marrow transplant. In addition, patients with sickle cell anemia often have difficulty finding well-matched stem cell donors.
To lower the risk of infection among people with sickle cell disease, they should receive the following vaccinations:
- Hib vaccine (Haemophilus influenza vaccine)
- Pneumococcal conjugate vaccine (PCV)
- PPV (Pneumococcal polysaccharide vaccine)
