The Pediatric Hematology Clinic at Gertrude’s Children’s Hospital cares for children and adolescents with diseases of the blood or blood-forming organs. These include conditions like sickle-cell anemia and bleeding disorders, among many others. We have a team of pediatric hematologists supported by experienced clinicians, nurses, pharmacists, and others
We offer complete and compassionate care to kids in a loving and supportive environment
What is sickle cell disease?
Sickle cell disease (SCD) is a common group of inherited blood disorders that interferes with how oxygen is delivered to the body tissues. A person’s red blood cells contain molecules called hemoglobin that carry oxygen to the body tissues. A normal hemoglobin is smooth, round and flexible but in sickle cell disease it has an abnormal shape that makes it easy for them to clump together and block blood vessels hence reducing blood flow. This can cause extreme pain. Red blood cells that are sickle shaped don’t survive long in the body. They are broken down when they pass through the spleen. This can lead to anemia. The sickled cells cause damage to the spleen and this weakens the body’s ability to fend off certain infections.
What is the cause of sickle cell disease?
Sickle cell is passed down through families. A person is born with it when a defective gene that leads to abnormal hemoglobin molecules being produced is passed on to him. The defective gene must come from both parents for one to have sickle cell disease. If one inherits a defective gene from only one parent one is said to have sickle cell trait. People with sickle cell trait do not display the severe symptoms of sickle-cell disease that occur those who have inherited two defective genes. There is a 1 in 4 chance of having a child with sickle cell disease if both parents have sickle cell trait.
What problems does sickle cell disease cause to my child?
People may experience the problems caused by sickle cell disease differently. Here are the common ones:
Anemia. This happens because the sickled red cells are short lived and are destroyed in large numbers the spleen. Severe anemia may make one feel dizzy, short of breath, and tired.
Attacks of pain. Also known as Pain crisis, or sickle crisis. Severe pains affecting the parts of the body whose blood supply has been blocked by the clumps of sickled cells. Hands, legs arms, thighs, forearms and legs are the commonest sites affected.
Painful swelling in the hands and feet could be could be the first warning.
When sickling affects the chest it could lead to a painful and potentially life threatening condition called acute chest syndrome. It can cause chest pain, cough, fever and difficulty in breathing.
Increased risk of bacterial infections. Sickled cells can pool in the spleen causing to swell and be painful. Pooling of blood can also lead to sudden decline in hemoglobin level which may be life threatening if not managed promptly.If the spleen pools and swells a number of times it gets damaged and this puts the child under an increased risk for bacterial infections.
Stroke. If the abnormal blood cells block blood vessels in the brain. This can interrupt blood flow to the brain and lead to severe brain damage. Those who have suffered a first stroke are more likely to get repeat episodes.
Jaundice. This is the yellowing of the eyes, mouth or skin which is caused by deposits of a yellow pigment called bilirubin. Bilirubin levels increase in the body because they are released from the abnormal red blood cells being broken down.
In males sickled cells may block the blood vessels of the penis leading to painful, persistent erection-like condition called priapism. If priapism is not promptly treated it could result in impotence.
How is sickle cell diagnosed?
When sickle cell disease is suspected tests will be done to confirm it .Hemoglobin electrophoresis is a blood test that measures different types of hemoglobin in the red blood cells and can determine if one has any of the diseases associated with sickle cell. Other tests can be done to assess the general health of the child.
How is sickle cell disease treated?
The best treatment for your child will depend on her age, state of health, the frequency and intensity of symptoms and other factors. It is important for the problem to be detected early so that complications like organ damage and infections are prevented. The are some of the treatments that may be recommended:
Certain Vaccinations to prevent infections.
Penicillin V is given regularly to prevent bacterial infections. Additional antibiotics may be prescribed bacterial infections are suspected.
Drink fluids frequently to keep well hydrated. It helps prevent and treat pain attacks.
Folic acid supplementation. Helps to prevent anemia.
Hydroxyurea may be given to reduce the frequency of pain attacks and to reduce the need for blood transfusions.
Pain medications are given during pain attacks.
Blood transfusions help to increase the levels of red blood cells in order to prevent or treat severe or frequent pains, acute chest syndrome, stroke or damage to the spleen.
The eyes need to be checked regularly to screen for damage to the blood vessels of the retina.
What kind of damage can sickle cell do to the body?
Any of the major organs (including the liver, heart, kidneys, gallbladder, eyes, bones, and joints) can be damaged when the abnormal sickle cells get stuck and clog the flow of blood to the tissue that is supplied by the blocked vessels. This can lead to loss of function in the affected organ(s).
How do I prevent pain crises in sickle cell disease?
Drink plenty of water to keep well hydrated.
Try to avoid high altitude places or situations.
Try to avoid situations with extremes of temperature.
Try to avoid physically strenous activities.
Take medications as advised by your doctor