Your child’s blood has many important jobs. It brings oxygen and nutrients to their organs, carries cells to fight off infections, and carries waste to their kidneys and liver to be filtered out of their body. In other words, healthy blood is vital to your child’s health.
Blood is made up of:
- Plasma: the liquid part of the blood, which transports blood cells throughout your body
- Platelets (thrombocytes): cell fragments that can form clots, which help stop wounds from bleeding
- White blood cells (leukocytes): cells that help keep the body protected against infections
- Red blood cells (erythrocytes): cells that help carry oxygen to the body and bring carbon dioxide back to the lungs
When your child has sickle cell disease, their red blood cells are damaged.
What is Sickle Cell Disease?
Normal, healthy red blood cells are round and carry oxygen throughout the body. If your child has sickle cell disease, their red blood cells become hard, sticky, and shaped like the letter “C”.
These sickled red blood cells can get stuck in small blood vessels, slowing or stopping the flow of blood and oxygen to parts of the body, such as the legs and arms, lungs, heart, and brain. This decreased blood flow can cause pain (usually in the lower back, arms, legs, chest, or belly), lead to infections, and make it difficult for your child’s organs to function properly.
Sickled red blood cells also only live 10 to 20 days, compared to 90 to 120 days for normal red blood cells. Because of this, your child may not have enough red blood cells. This is called anemia. Symptoms of anemia may include:
- Shortness of breath
- Fast heartbeat
What Causes Sickle Cell Disease?
Sickle cell disease is inherited, meaning it’s passed from parent to child. It is also more common in African Americans and other ethnic groups. The cause of sickle cell disease is a defective gene, called a sickle cell gene. Your child may have one or two sickle cell genes:
- Children with sickle cell disease are born with two sickle cell genes, one from each parent.
- Children with sickle cell trait only have one sickle cell gene. Children with sickle cell trait are generally healthy, but they can pass the defective gene on to their children.
How Is Sickle Cell Disease Diagnosed?
Because sickle cell disease is a blood disorder, a simple blood test can show if your child has sickle cell disease or sickle cell trait. All children born in the United States are tested for this during their newborn screen.
How Is Sickle Cell Disease Treated?
Children with sickle cell disease are at an increased risk of infection and other health problems such as anemia, so early diagnosis and treatment are important to keep your child healthy.
Sickle cell treatment includes:
- Penicillin: This medication prevents bacterial infections. Most children with sickle cell disease take penicillin until the age of 5. In some cases, your child may need to take it longer.
- Pain medication: These medications treat pain. For mild or moderate pain, Ibuprofen or other nonsteroidal anti-inflammatory medications (NSAIDs) are used. For severe pain, your child may need opioids.
- Hydroxyurea: This medication decreases or prevents sickle cell complications.
- Immunizations: Vaccines prevent infections, such as influenza, meningitis, and pneumonia.
- Blood transfusion: Blood transfusions treat severe anemia.
- Exchange transfusion: Exchange transfusion removes and replaces the sickled blood with donor blood or plasma. This may be needed for children with severe sickle cell disease or complications.
- Stem cell transplant. A stem cell transplant replaces the bone marrow with healthy blood-forming cells (stem cells). This is a rare treatment option, but may cure sickle cell disease. Transplant is only used in severe cases.
What Are The Potential Complications Sickle Cell Disease Can Cause?
Because red blood cells have such an important job, there are a number of ways sickle cell disease can take a toll on your child’s health. These complications range from mild to severe.. Complications include:
|Acute Chest Syndrome||
When Is Sickle Cell Disease An Emergency?
If your child’s blood flow severely decreases, serious life-threatening complications can occur.
Go to the Emergency Department right away if your child has:
- Fever of 100.4°F (38.0°C) or higher
- Shortness of breath or difficulty breathing
- Trouble talking, walking, or confusion
- Weakness in their face, arms, or legs on one side of the body
- Numbness or tingling in their face, arms, or legs
- Swelling in their arms, hands, legs, feet, or abdomen
- Blurry vision or difficulty seeing
- Severe pain
- Severe headache
- An erection lasting longer than 4 hours
- Chills or shaking
- Sudden weakness
- Pale skin or nail beds
- Yellow color to their skin or whites of eyes
- Blood in their urine
How Can You And Your Child Prevent And Manage Complications of Sickle Cell Disease?
Sickle cell disease is a lifelong illness. After diagnosis, the goal is to prevent and manage complications, including pain and infections. In order to keep your child healthy, they should:
- Drink plenty of water.
- Avoid getting too hot or too cold.
- Avoid high altitudes, which can cause red blood cells to become deformed.
- Follow up with their hematologist (a physician that specializes in caring for patients with blood disorders) regularly.
- Take medications as prescribed.
- Maintain regular check-ups with their pediatrician or primary care provider.
- Avoid smoking and alcohol.
Additional Information on Sickle Cell Disease
- Centers for Disease Control and Prevention
- National Heart, Lung, and Blood Institute (National Institutes of Health)
- National Library of Medicine (MedlinePlus)
- Sickle Cell Association of America
What To Do Next
To make an appointment, contact the Children’s Hospital & Medical Center Hematology/Oncology Program at 402-955-3950.
For Referring Providers
The Physicians’ Priority Line is your 24-hour link to pediatric specialists at Children’s for referrals, emergency and urgent consults, physician-to-physician consults, admissions, and transport services. Call 855-850-KIDS (5437).
Learn more about referring patients.