Sickle Cell Anemia Symptoms

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About Sickle Cell Anemia

Sickle cell anemia is an inherited blood disease. That means you are born with it and it lasts a lifetime. Sickle cell anemia affects the red blood cells. Normal red blood cells are smooth and round like doughnuts. They move easily through blood vessels to carry oxygen to all parts of the body.

In sickle cell anemia, the red blood cells become hard, sticky, and shaped like sickles or crescents. When these hard and pointed red cells go through the small blood vessels, they tend to get stuck and block the flow of blood. This can cause pain, damage, and a low blood count or anemia.

Anemia is a shortage of red blood cells in your blood. In sickle cell anemia, this shortage of red blood cells occurs because sickle cells do not last very long. It is hard for your body to make new red blood cells fast enough to keep up. Normal red blood cells last about 120 days in the bloodstream. Sickle cells die after only about 10 to 20 days.

Symptoms

The signs and symptoms of sickle cell anemia are different in each person. Some people have mild symptoms. Others have very severe symptoms and are often hospitalized for treatment.

The most common symptoms or signs are related to:

Anemia
Pain when sickle-shaped red blood cells block the flow of blood to an organ
Other more specific symptoms.

The general symptoms or signs of anemia are:

Fatigue (feeling very tired)
Paleness
Yellowing of the skin and eyes (jaundice)
Shortness of breath.

Causes

People with sickle cell anemia inherit two genes, one from each parent, that are variant (different from normal). The variant genes are call sickle cell genes.

The sickle cell genes tell the body to make the variant hemoglobin (hee-muh-glow-bin) that results in deformed red blood cells. Hemoglobin is the protein in red blood cells that carries oxygen to all parts of the body.

Children who inherit sickle cell genes from both parents will have sickle cell anemia. Children who inherit the sickle cell gene from only one parent will not have the disease. They will have sickle cell trait. Persons with sickle cell trait:

Generally have no symptoms
Live normal lives
Can pass the sickle cell gene on to their children.

Treatment

Although there is no cure (other than bone marrow transplantation) for sickle cell anemia, doctors know a great deal about how to treat it.

People with sickle cell anemia need regular medical care. There are doctors and clinics that specialize in treating people with the disease.

The goal of treatment is to relieve pain, prevent infections, and control complications if they occur. The treatments include:

Medications
Blood transfusions
Specific treatment for complications.

Treatment for Pain

Painful crises are the leading cause of emergency room visits and hospitalizations. The usual treatment for acute pain crises is giving pain-killing medications and increasing fluids. The pain-killing medicines most often used are:

Acetaminophen (a set a mee' noe fen)
Nonsteroidal anti-inflammatory drugs (NSAIDs)
Opioids, like morphine, oxycodone, hydrocodone, and others.

The treatment of patients with mild-to-moderate pain usually begins with a NSAIDs or acetaminophen. If pain continues, an opioid may be added. Moderate-to-severe pain is treated with opioids. The opioid may be used alone or together with a NSAIDs or acetaminophen.

Preventing Infections

Infection is a major complication of sickle cell anemia. In fact, pneumonia is the leading cause of death in children with the disease. Other infections common in persons with the disease include meningitis, influenza, and hepatitis.

To prevent infections in babies and young children, treatment methods include:

Daily doses of penicillin. Treatment may begin as early as 2 months of age and continue until the child is at least 5 years old.
Vaccinations for pneumonia, meningitis, influenza, and hepatitis.

Children with sickle cell anemia should have a flu shot each year.

If a child with sickle cell anemia shows early signs of an infection, such as fever, it is very important to get treatment right away.

Adults with sickle cell anemia should also have a flu shot every year. They should also be vaccinated for pneumonia.

Natural Remedies

In a preliminary study, individuals with pulmonary hypertension (a life-threatening complication of sickle cell anemia) received L-arginine in the amount of 100 mg per 2.2 pounds of body weight, three times per day for five days. L-arginine treatment resulted in a significant improvement in pulmonary hypertension, as determined by a 15% decline in the pulmonary artery systolic pressure. Longer-term studies are needed to confirm these preliminary results.

References for Sickle Cell Anemia Article

Morris CR, Morris SM Jr, Hagar W, et al. Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 2003;168:63–9.
Osifo BO, Adeyokunnu A, Parmentier Y, et al. Abnormalities of serum transcobalamins in sickle cell disease (HbSS) in Black Africa. Scand J Haematol 1983;30:135–40.
al-Momen AK. Diminished vitamin B12 levels in patients with severe sickle cell disease. J Intern Med 1995;237:551–5.
al-Momen AK. Diminished vitamin B12 levels in patients with severe sickle cell disease. J Intern Med 1995;237:551–5.
National Institutes of Health

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