Sickle Cell Anemia
GENERAL INFORMATION:
What is sickle cell anemia? Sickle cell anemia is an inherited blood disease that affects the red blood cell (RBC). The RBC is soft and round, can squeeze through small blood vessels, and contains hemoglobin. Hemoglobin is a special protein that helps carry oxygen to all parts of the body. With sickle cell anemia, the RBC contains an abnormal hemoglobin called hemoglobin S (HbS). HbS sticks together when the oxygen is low or absent. This makes the RBC change shape from round to sickle shaped. Sickle cells may get stuck in the small blood vessels and prevent blood flow. The decreased blood flow causes lack of oxygen and damage to body organs.
What causes sickle cell anemia? Sickle cell anemia is caused by an error in the gene that makes healthy hemoglobin. Normally, hemoglobin (HbA) is the hemoglobin made in the body. With sickle cell anemia, the abnormal HbS gene is made. A child needs to get two of the HbS genes (one from each parent) to have sickle cell anemia. If only one HbS gene is inherited, a child may only have sickle cell trait.
What are the signs and symptoms of sickle cell anemia? Your child may have episodes of severe pain known as sickle cell crisis. This may last for hours or days and affects your child's arms, legs, back, chest and abdomen (belly). Your child may also have one or more of the following:
- Feeling more irritable or tired than usual.
- Fever or cough.
- In males, prolonged and painful erection.
- Paleness of the skin or nails.
- Swelling of the hands, arms, legs, or feet.
- Trouble breathing or shortness of breath.
- Yellowing of the skin or the whites of the eyes.
What causes a sickle cell crisis? Sickle cell crisis, including the pain, may occur at any time. The following may trigger a sickle cell crisis:
- Change in air pressure, such as airplane travel, or lack of oxygen from living in a high altitude area.
- Dehydration (losing too much body fluid).
- Emotional stress or too much physical activity.
- Infections, such as a common cold.
- Sudden changes in body temperature, such as becoming very cold.
What problems can sickle cell anemia cause?
- Delayed (slower than normal) growth and sexual development.
- Gallbladder stones.
- Infections.
- Spleen, kidney, liver, or lung damage.
- Stroke.
How is sickle cell anemia diagnosed? Sickle cell anemia may be diagnosed by the following:
- Amniocentesis: This procedure removes a sample of the fluid that is around a baby while his is still inside the expectant mother's uterus (womb). The fluid is called amniotic fluid. The sample will be sent to a lab to check for sickling of red blood cells.
- Hemoglobin electrophoresis: This blood test uses an electrical current to find and measure different types of hemoglobin in the blood. Caregivers will learn if your child has an abnormal hemoglobin S (HbS).
How is sickle cell anemia treated? Your child may have any of the following, depending upon his signs and symptoms:
- Blood transfusion: This is donated whole blood, or parts of blood your child may get through an IV. You may be worried that your child will get AIDS, hepatitis, or West Nile Virus from a blood transfusion. The risk of this happening is rare. Blood banks test all donated blood for AIDS, hepatitis, and West Nile Virus. If you refuse a blood transfusion for your child, his condition may get worse, and he may die.
- Bone marrow transplant: This is also called a BMT. With a BMT, your child's diseased bone marrow is replaced with healthy marrow from another person (donor). A BMT may cure your child's illness, but it can cause other serious problems.
- Medicines:
- Antibiotics: Antibiotics may be given to help your child fight an infection caused by a germ called bacteria.
- Pain medicine: Caregivers may give medicine to decrease your child's pain. Tell a caregiver if your child's pain does not go away or comes back after taking this medicine. Pain medicine can have side effects. Tell a caregiver if your child has trouble breathing, is very sleepy, or has an upset stomach. Tell a caregiver if your child is allergic to any medicine.
- Pneumococcal vaccine: When your child is between 2 and 5 years old, he may receive a pneumococcal vaccine (shot) to prevent pneumonia.
- Antibiotics: Antibiotics may be given to help your child fight an infection caused by a germ called bacteria.
- Oxygen: Your child may need oxygen to help him breathe easier. Your child may need a nasal cannula (small tubes placed in the nose) or mask. Many children do not like having these on their face, so caregivers may place the mask next to your child's face. Some children are placed in an oxygen tent or plastic hood. Do not take off your child's oxygen without asking your child's caregiver first.
- Surgery: Caregivers may remove part or all of your child's spleen. If your child has gall stones, his gallbladder may also be removed.
How can a sickle cell crisis be prevented?
- Avoid stress: Help your child to decrease or avoid stress. Ask caregivers how your child can learn to relax, such as with deep breathing exercises or meditation. Biofeedback and muscle relaxation exercises may also help your child relax. Encourage your child to talk about things that upset him.
- Avoid sudden changes of air pressure or lack of oxygen: Always travel in airplanes with normal cabin pressure when flying with your child. Talk to caregivers about how to make your child more comfortable when travelling. You may also need to avoid areas with high altitude, such as the mountains.
- Avoid temperature extremes: Exposure to extreme heat or cold can start the formation of sickle cells. Keep your child's body warm in the winter and cool in the summer.
- Drink liquids: Your child needs liquids to keep healthy. Ask your caregiver what kind of liquids and how much liquids your child should drink. Drinking liquids decreases the chance that sickle cells will form.
- Exercise regularly: Work with caregivers to plan a good activity program for your child. Encourage your child to be active, but he should avoid activities that may cause injury, such as playing football.
- Diet: To make new red blood cells, your child needs to eat healthy foods. Ask your child's caregiver for information about a healthy diet.
Where can I get support and more information? Sickle cell anemia is a life-changing disease for your child and your family. Accepting that your child has sickle cell anemia is hard. You and those close to you may feel guilty, angry, depressed, or frightened. As parents, you may blame yourself and think you have done something wrong. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. You may also want to join a sickle cell anemia support group. This is a group of people who have sickle cell anemia. Ask your caregiver for the names and numbers of support groups. Contact the following for more information:
- Harvard Information Center for Sickle Cell and Thalassemic Disorders
Phone: 1-617-768-8880
Web Address: http://sickle.bwh.harvard.edu
- Sickle Cell Disease Association Of America
231 East Baltimore St., Suite 800
Baltimore, MD 21202
Phone: 1-410-528-1555
Phone: 1-800-421-8453
Web Address: http://www.sicklecelldisease.org
- The Sickle Cell Information Center
PO Box 109
Atlanta, GA 30303-0109
Grady Memorial Hospital
Atlanta, GA 30303
Phone: 1-404-616-3572
Web Address: www.scinfo.org
CARE AGREEMENT:
You have the right to help plan your child's care. To help with this plan, you must learn about your child's health condition and how it may be treated. You can then discuss treatment options with your child's caregivers. Work with them to decide what care may be used to treat your child.
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