Overview Of Sickle Cell Anemia
Sickle Cell Anemia, also known as Sickle Cell Disease (SCD), is a hereditary disease that causes red blood cells to be malnourished and under developed within a person’s body. Red blood cells are typically shaped like a disk, but under this condition they appear more as a sickle or crescent shape.
Commonly Associated With
Anemia – sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell anemia
Causes Of Sickle Cell Anemia
Sickle cell disease originates from an irregular hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.
Hemoglobin S alters red blood cells where they then become fragile, noticeable by their shape like crescents or sickles.
Abnormal red blood cells deliver less oxygen to the body’s tissues, causing the body itself to then become more fragile.
These red blood cells can also easily become stuck in narrower blood vessels and then fracture into break into pieces. This would interrupt a healthy blood flow and significantly decrease the amount of oxygen flowing to body tissues.
Sickle cell disease can be inherited from both parents. If a person has the sickle cell gene from only one parent, you will then show a sickle cell trait. Typically, people with sickle cell traits do not have symptoms of sickle cell disease.
Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East. Although, it is relatively possible for anyone of any geographical descendance to have this condition, based on how it is passed down in one’s genes.
Symptoms Of Sickle Cell Anemia
Symptoms usually do not make themselves prominent until after an affected person is 4 months old.
Almost all people with sickle cell disease experience episodes of drastic pain called crises. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.
People can experience one episode every few years, though, while others might experience many episodes each year. Any crises can be severe enough to require a hospital stay.
When the anemia becomes more severe, symptoms may include:
- Fatigue
- Paleness
- Rapid heart rate
- Shortness of breath
- Yellowing of the eyes and skin (jaundice)
- Younger children with sickle cell disease have attacks of abdominal pain.
The following symptoms of sickle cell anemia can occur as a result of small blood vessels becoming blocked by abnormal cells:
- A painful and prolonged erection (priapism)
- Poor eyesight or blindness
- Problems with thinking or confusion caused by small strokes
- Ulcers on the lower legs (in adolescents and adults)
Over time, the spleen might stop functioning. As a result, people with sickle cell disease may have symptoms of infections such as:
- Bone infection (osteomyelitis)
- Gallbladder infection (cholecystitis)
- Lung infection (pneumonia)
- Urinary tract infection
Other signs and symptoms of sickle cell anemia include:
- Delayed growth and puberty
- Painful joints caused by arthritis
- Heart or liver failure due to too much iron (from blood transfusions)
Exams & Tests
Tests commonly done to diagnose and monitor people with sickle cell disease include:
- Bilirubin
- Blood oxygen saturation
- Complete blood count (CBC)
- Hemoglobin electrophoresis
- Serum creatinine
- Serum potassium
- Sickle cell test
Treatment Of Sickle Cell Anemia
People with this condition should take folic acid supplements. Folic acid helps make new red blood cells.
Treatment for a sickle cell crisis includes:
- Blood transfusions (may also be given regularly to prevent stroke)
- Pain medicines
- Plenty of fluids
Other treatments for sickle cell disease may include:
- Hydroxyurea (Hydrea), which helps reduce the number of pain episodes (including chest pain and breathing problems) in some people
- Antibiotics, which help prevent bacterial infections that are common in children with sickle cell disease
- Medicines that reduce the amount of iron in the body
- Newer therapies to reduce the frequency and severity of pain crises have been approved
Treatments that may be needed to manage complications of sickle cell anemia include:
- Dialysis or kidney transplant for kidney disease
- Counseling for psychological complications
- Gallbladder removal in people with gallstone disease
- Hip replacement for avascular necrosis of the hip
- Surgery for eye problems
- Treatment for overuse or abuse of narcotic pain medicines
- Wound care for leg ulcers
- Bone marrow or stem cell transplants can cure sickle cell disease, but this treatment is not an option for most people. People with sickle cell disease often cannot find well-matched stem cell donors.
People with sickle cell disease should have the following vaccinations to lower the risk for infection:
- Haemophilus influenza vaccine (Hib)
- Pneumococcal conjugate vaccine (PCV)
- Pneumococcal polysaccharide vaccine (PPV)
