Sickle Cell Anemia in Children

Sickle cell anemia is a condition in which red blood cells have an abnormal “sickle” shape. Red blood cells carry oxygen through the body. Sickle-shaped red blood cells do not live as long as normal red blood cells.

They also clump together and block blood from flowing through the blood vessels. This condition prevents the body from getting enough oxygen. Sickle cell anemia causes organ damage and pain. It also increases the risk of infection.

What are the causes?

This condition is caused by a gene that is passed from parent to child (inherited). Two copies of the gene causes the disease. One copy causes the “trait,” which means that symptoms are milder or not present.

What increases the risk?

This condition is more likely to develop if your child’s ancestors were from Africa, the Mediterranean, South or Central America, the Caribbean, India, or the Middle East.

What are the signs or symptoms?

Symptoms of this condition include:

• Episodes of pain (crises), especially in the hands and feet, joints, back, chest, or abdomen. They can be triggered by:
  • An illness, especially if there is dehydration.
  • Doing an activity with great effort (overexertion).
  • Exposure to extreme temperature changes.
  • High altitude.
• Fatigue.
• Shortness of breath or difficulty breathing.
• Dizziness.
• Pale skin or yellowed skin (jaundice).
• Frequent bacterial infections.
• Pain and swelling in the hands and feet (hand-food syndrome).
• Prolonged, painful erection of the penis (priapism).
• Acute chest syndrome. Symptoms of this include:
  • Chest pain.
  • Fever.
  • Cough.
  • Fast breathing.
• Stroke.
• Decreased activity.
• Loss of appetite.
• Change in behavior.
• Headaches.
• Seizures.
• Vision changes.
• Skin ulcers.
• Heart disease.
• High blood pressure.
• Gallstones.
• Liver and kidney problems.

How is this diagnosed?

This condition may be diagnosed with:

• Blood tests. These check for the gene that causes this condition
• A prenatal screening test. This test is done in the first trimester of pregnancy. It involves taking a sample of amniotic fluid.

How is this treated?

There is no cure for most cases of this condition. Treatment focuses on managing your child’s symptoms and preventing complications of the disease. Treatment may include:

• Medicines, including:
  • Pain medicines.
  • Antibiotic medicines for infection.
  • Medicines to increase the production of a protein in red blood cells that helps carry oxygen in the body (hemoglobin).
• Fluids to treat pain and swelling.
• Oxygen to treat acute chest syndrome.
• Blood transfusions to treat symptoms such as fatigue, stroke, and acute chest syndrome.
• Creams and ointments to treat skin ulcers.
• Massage and physical therapy for pain.
• Regular tests to monitor the condition, such as blood tests, X-rays, CT scans, MRI scans, ultrasounds, and lung function tests. These should be done every 3–12 months, depending on your child’s age.
• Hematopoietic stem cell transplant. This is a procedure to replace abnormal stem cells with healthy stem cells from a donor’s bone marrow. Stem cells are cells that can develop into blood cells, and bone marrow is the spongy tissue inside bones.

Follow these instructions at home:

Medicines

• Give your child over-the-counter and prescription medicines only as told by the health care provider. Do not give your child aspirin because it has been linked to Reye syndrome.
• If your child was prescribed an antibiotic medicine, give it to your child as told by the health care provider. Do not stop giving the antibiotic even if your child starts to feel better.
• If your child develops a fever, do not give him or her medicines to reduce the fever right away. This could cover up a problem. Notify your child’s health care provider immediately.

Managing pain, stiffness, and swelling

• Try these methods to help ease your pain:
  • Applying a heating pad.
  • Preparing a warm bath.
  • Distracting your child, such as with TV.

Eating and drinking

• If your child is breastfeeding and breastfeeding is not possible, use formulas with added iron.
• Have your child drink enough fluid to keep urine clear or pale yellow. Increase fluids in hot weather and during exercise.
• Feed your child a balanced and nutritious diet that includes plenty of fruits, vegetables, whole grains, and lean protein.
• Give vitamin and nutrition supplements as directed by your child’s health care provider.

Travelling

• When travelling, keep these with your child:
  • Your child’s medical information.
  • The names of your child’s health care providers.
  • Your child’s medicines.
• If your child has to travel by air, ask about precautions you should take.

Activity

• Have your child get plenty of rest.
• Have your child avoid activities that will lower oxygen levels, such as vigorous exercise.

General instructions

• Do not smoke around your child.
• Make sure your child wear a medical alert bracelet.
• Have your child avoid:
  • High altitudes.
  • Extreme heat, cold, or temperature changes.
• Tell your child’s teachers and caregivers about your child’s condition, what symptoms to look out for, and how to manage symptoms.
• Keep all follow-up visits as told by your child’s health care provider. This is important.

Contact a health care provider if:

• Your child’s feet or hands swell or have pain.
• Your child has joint pain.
• Your child has fatigue.

Get help right away if:

• Your child develops symptoms of infection. These include:
  • Fever.
  • Chills.
  • Extreme tiredness.
  • Irritability.
  • Poor eating.
  • Vomiting.
• Your child feels dizzy or faints.
• Your child develops new abdominal pain, especially on the left side near the stomach.
• Your child develops priapism.
• Your child’s arms or legs get numb or are hard to move.
• Your child has trouble talking.
• Your child’s pain cannot be controlled with medicine.
• Your child becomes short of breath or breathes rapidly.
• Your child has a persistent cough.
• Your child has chest pain.
• Your child develops a severe headache or stiff neck.
• Your child feels bloated without eating or after eating a small amount.
• Your child’s skin is pale.
• Your child suddenly loses vision.

Summary

• Sickle cell anemia is a condition in which red blood cells have an abnormal “sickle” shape. This disease can cause organ damage and chronic pain, and it can raise your child’s risk of infection.
• Sickle cell anemia is a genetic disorder.
• Treatment focuses on managing symptoms and preventing complications of the disease.
• Get medical help right away if your child has any symptoms of infection.