Sickle Cell Disease
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What is sickle cell disease in children?
Sickle cell disease (SCD) is a blood disorder that a child is born with. It's passed
down through a parent’s genes. Children with SCD make an abnormal type of hemoglobin.
This is the protein in red blood cells that carries oxygen to all parts of the body.
Healthy red blood cells with normal hemoglobin are round and move easily through blood
vessels. When a child has SCD, the red blood cells are hard and sticky. They are shaped
like the letter C (and like a farm tool called a sickle). These damaged red blood
cells (sickle cells) clump together. They can’t move easily through the blood vessels.
They get stuck in small blood vessels and block blood flow. This blockage can cause
pain. It can also damage major organs.
Sickle cells die sooner than healthy cells. Normally the spleen helps filter infections
out of the blood. But sickle cells get stuck in this filter and die. Having fewer
healthy red blood cells causes anemia. The sickle cells can also damage the spleen.
Without a healthy spleen, children are more at risk for serious infections.
There are several complex types of the sickle cell gene. Some don’t cause symptoms
or severe problems, but others do. Talk with your child’s healthcare provider about
the specific form of sickle cell your child has.
Most children with SCD will start showing symptoms during the first year of life,
often around 5 months.
What causes sickle cell disease in a child?
Sickle cell is present at birth. It is inherited when a child has 2 sickle cell genes,
1 from each parent.
A child who has only one sickle cell gene is healthy. But they are a carrier of the
disease. If 2 carriers have a child, there is a chance their child will have sickle
Once parents have had a child with sickle cell disease, there is a 1 in 4 chance that
another child will be born with sickle cell disease. There is also a 1 in 2 chance
that a child will be a carrier, like the parents.
Which children are at risk for sickle cell disease?
Having a family history of SCD increases a child’s risk for the disease. SCD mainly
affects people whose families came from Africa, and Hispanics whose families are from
the Caribbean. But the gene has also been found in people whose families are from
the Middle East, India, Latin America, and Mediterranean countries. It has also been
found in American Indians.
What are the symptoms of sickle cell disease in a child?
Most children with SCD will start to have symptoms during the first year of life,
often around 5 months. Each child’s symptoms may vary. They may be mild or severe.
Symptoms can include:
Anemia. This is the most common symptom. Having fewer red blood cells causes anemia. Anemia
can make a child pale and tired.
Yellowing of the skin, eyes, and mouth (jaundice). This is a common symptom. Sickle cells don't live as long as normal red blood cells.
They die faster than the liver can filter them out. The yellow color is caused by
a substance (bilirubin) that is released when the red blood cells die.
Pain crisis, or sickle crisis. When sickle cells move through small blood vessels, they can get stuck. This blocks
blood flow and causes pain. This sudden pain can happen anywhere, but most often occurs
in the chest, arms, and legs. Babies and young children may have painful finger and
toe swelling. Blocked blood flow may also cause tissue death.
Acute chest syndrome. This is when sickle cells stick together and block oxygen flow in the tiny vessels
in the lungs. This can be deadly. It often occurs suddenly, when the body is under
stress from infection, fever, or fluid loss (dehydration). It looks like pneumonia
and can include fever, pain, and a violent cough.
Splenic sequestration (pooling). The spleen becomes enlarged and painful when sickle cells get stuck and build up there.
Fewer red blood cells are able to move. This can cause a sudden drop in hemoglobin.
It can be deadly if not treated at once.
The symptoms of SCD may look like other disorders or health problems. Always see your
child's healthcare provider for a diagnosis.
How is sickle cell disease diagnosed in a child?
Most states check newborn babies for abnormal hemoglobin as part of routine newborn
screening tests. State newborn screening includes tests for all newborns in the first
few days of life. These tests identify serious, life-threatening diseases.
SCD may be found as part of newborn screening. Your family history, your child's medical
history, and a physical exam are all included in the diagnosis. If the screening test
shows SCD, a blood test called hemoglobin electrophoresis may be done. It can tell
if your child is a carrier of sickle cell. It can also tell if your child has any
of the diseases linked to the sickle cell gene. Other blood tests may also be done.
How is sickle cell disease treated in a child?
Treatment will depend on your child’s symptoms, age, and general health. It will also
depend on how severe the condition is.
Early diagnosis and preventing further problems is important in treating this disease.
Your child's healthcare provider will refer you to a hematologist. This is an expert
in blood disorders. Other specialists may also be involved in your child's care.
Treatment may include:
Pain medicines. These are used for pain crises.
Drinking plenty of water daily (8 to 10 glasses). This helps prevent and treat pain crises. In some cases, IV (intravenous) fluids may
Blood transfusions. These are used to treat anemia, chronic pain, acute chest syndrome, and splenic sequestration,
and to prevent stroke.
Vaccines and antibiotics. These are used to prevent infections.
Folic acid. This helps prevent severe anemia.
Regular eye exams. These are done to screen for an eye condition called retinopathy. Have your child’s
eyes checked each year.
Stem cell transplant. Transplants can cure some children with SCD. Studies of this treatment are ongoing.
Talk with your child’s healthcare provider. Transplants are only done at certain medical
Hydroxyurea. This is a medicine that can reduce the number of sickle cells in the blood. It reduces
complications, painful episodes (crises), and hospital stays.
There is ongoing research with newer medicines available. Discuss these options with
your healthcare provider.
What are possible complications of sickle cell disease in a child?
Complications of SCD include:
Long-term anemia. This may lead to delayed healing and delayed growth and development.
Pain crisis, or sickle crisis. In severe cases, your child may need treatment in a hospital.
Acute chest syndrome. Over time, many episodes of acute chest syndrome can cause lasting (permanent) lung
Splenic sequestration (pooling). When red blood cells build up in the spleen, it becomes enlarged and painful. The
spleen can be damaged and scarred after many episodes of splenic sequestration. By
age 8, many children with SCD have had their spleen removed. Or they may have lasting
damage from repeated splenic sequestration. The risk for infection is a major concern
for children without a working spleen. Infection is the major cause of death in children
younger than age 5.
Stroke. If the blood vessels to the brain are blocked, a stroke can occur. Serious long-term
problems may result. A child who has had a stroke is more likely to have another one.
Infections. Babies and children with SCD have a higher risk for infections.
Priapism. The sickle cells block the blood vessels in the penis, causing great pain. If not
treated right away, this can lead to the inability to have an erection (impotence).
SCD can affect any major organ. This can cause:
How can I help my child live with sickle cell disease?
Advances in preventive care and new medicines have reduced the life-threatening problems
of sickle cell. But it is still a severe, chronic, and sometimes fatal disease. Your
child should be carefully managed by specialists. How your child is managed depends
The type of sickle cell your child has
How severe the disease is
How often your child has complications
How well you and your child follow preventive efforts
You may not be able to fully prevent your child from having complications of SCD.
But helping your child live a healthy lifestyle can reduce some of the problems. Make
sure your child has regular eye exams and gets stroke screening tests. Also talk with
your child’s healthcare provider about making sure your child:
Eats a healthy diet
Gets enough sleep
Drinks plenty of fluids
Be careful about certain exposures that may trigger a crisis for your child. These
Swimming in cold water
Help your child prevent infections by:
Staying away from people who are sick
Washing his or her hands often
Having all advised vaccines such as pneumococcal
Having all advised screenings such as hepatitis C
When should I call my child's healthcare provider?
Call your child's healthcare provider or get medical care right away if your child
Sudden pain, mainly in the chest, belly, arms, or legs
Fever greater than 100.4°F (38°C), or as advised by your child's provider
Symptoms of an enlarged spleen
Sudden loss of vision
Symptoms of severe anemia
Key points about sickle cell disease in children
Sickle cell disease (SCD) is an inherited blood disorder that is present at birth.
This means it is passed down through a parent’s genes.
With SCD, the red blood cells have an abnormal C shape. They get stuck in small blood
vessels and block blood flow.
This blockage can cause pain and lead to infection. It can also damage a child’s major
organs, and cause a stroke.
Newborns are checked for SCD as part of a group of screening tests.
Most children will start to have signs or symptoms during the first year of life.
Tips to help you get the most from a visit to your child’s healthcare provider:
Know the reason for the visit and what you want to happen.
Before your visit, write down questions you want answered.
At the visit, write down the name of a new diagnosis, and any new medicines, treatments,
or tests. Also write down any new instructions your provider gives you for your child.
Know why a new medicine or treatment is prescribed and how it will help your child.
Also know what the side effects are.
Ask if your child’s condition can be treated in other ways.
Know why a test or procedure is recommended and what the results could mean.
Know what to expect if your child does not take the medicine or have the test or procedure.
If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.
Online Medical Reviewers:
- Adam Levy MD
- L Renee Watson MSN RN
- Raymond Turley Jr PA-C