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September 3, 2009 – Vol. 4 • No. 1
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Children with sickle cell disease — also referred to as sickle cell anemia — can lead healthy and productive lives. With medications for pain and infection, timely immunizations and adherence to healthy habits, children can live a life filled with school, family and other activities they enjoy. This is not to minimize the severity of the disease. Sickle cell anemia — an inherited illness caused by an abnormality in the red blood cells — can be a painful and deadly disorder. It is marked by serious infections, persistent anemia, painful episodes and damage to several organs of the body.

How can I tell if my child has sickle cell disease?

Since 1990, the Massachusetts Department of Public Health has mandated newborn screening for sickle cell disease.
Fortunately, all 50 states as well as Washington, D.C., Puerto Rico and the U.S. Virgin Islands offer newborn screening for sickle cell disease. Most states, including Massachusetts, mandate the screening, which is usually performed within 48 hours of birth. Since most screening programs have been in effect for several years, cases are now caught early rather than later. The advantage to early screening is early treatment.

Helping children live well with sickle cell disease

After learning their child has sickle cell disease, parents may wonder what life will be like for him or her. Will it ever be normal? What should they do to help prepare their child for the future?

The quality of life will also depend on whether the disease is mild or severe and the effectiveness of treatments. Infections are a major problem for infants and young children, and can be fatal. Sickle cell anemia can harm the spleen, an organ that helps fight infections. In addition, pneumonia is often the cause of death in young children with sickle cell anemia.
The disease is also characterized by episodes of pain called “crises.” A crisis occurs when red blood cells clump in the bloodstream and block the flow of blood, causing pain and ultimately tissue damage. The pain can be pervasive — invading the stomach, bones, lungs and joints — and can last a few hours, days, weeks or even months.

Major advances have been made in the treatment of sickle cell anemia — from the reduction of infections to the approval by the Food and Drug Administration of hydroxyurea, an anti-cancer medicine that has proven effective in reducing crises. Although studied in adults, hydroxyurea is often offered to children as well. In some cases, bone marrow transplants have cured the disease.

There are some things you can do as a parent to keep your child healthy and minimize complications.

Prevent infections

Antibiotics, administered twice a day from the age of two months to five years, have proven successful in lowering the incidence of infections in infants and children with sickle cell.
Hand washing is one of the most effective ways to prevent the spread of infections. Wash your hands before eating or preparing food, after using the toilet or changing diapers, and after contact with someone who has an infection. Use soap and clean water or gel hand cleansers.

Food safety can prevent salmonella, bacteria especially harmful to children with sickle cell. Salmonella can be found on fruits and vegetables, in dairy and meat products, poultry and even on some pets. To prevent contamination, wash hands, cutting boards and utensils after touching uncooked foods; wash vegetables; cook meat until it is well done; and do not eat raw or undercooked eggs.

Keep up with immunizations

In addition to the regular childhood vaccines, a few extra are recommended for children with sickle cell disease. Yearly flu vaccines as well as pneumococcal and meningococcal vaccines for pneumonia and meningitis, respectively, are encouraged. Meningitis is an inflammation of the membranes that cover the brain and spinal cord, and can result in brain damage. Prior to the development of these immunizations, pneumonia was one of the leading causes of death in children with sickle cell. Some experts recommend protection against hepatitis A as well.

Have regular checkups

The Centers for Disease Control and Prevention recommends a schedule of regular visits to the doctor — every two to three months for infants from birth to age 1; at least four times a year for children from age 1 to 2; and at least yearly for children 2 and older. Children with sickle cell are followed by pediatricians as well as hematologists, who specialize in disorders of the blood.

Encourage healthy habits

Children with sickle cell should drink lots of fluids — preferably water — each day to keep hydrated and reduce the clumping of red blood cells. Although exercise is encouraged, rest is important as well. Overdoing it can lead to an attack. Extremes of temperature can cause a problem — kids need to be kept warm in the winter and cool in the summer.

Be aware of symptoms

The symptoms of sickle cell vary by severity of the disease and do not appear until 4 months of age or older. As noted in http://www.kidshealth.org and http://www.familydoctor.org, some of the symptoms to look for are:

• Anemia

• Swelling of the feet or hands

• Skin or nail beds suddenly turn pale

• Eyes or skin turning yellow

• Infection

• Pain in joints, stomach, chest or muscles

• Fever

• Episodes of pain

Know when to call the doctor

There are some symptoms that require immediate professional medical care.

• Fever above 101 degrees

• Difficulty breathing

• Chest pain

• Abdominal (belly) swelling

• Severe headache

• Seizure

• Signs of a stroke — sudden weakness, slurring of speech, numbness and tingling, sudden change in vision

Because of advances in the treatment of sickle cell disease and better understanding of the disease, life expectancy has significantly increased in the past 20 years.