Topic: Diseases & Conditions
This handout was written to answer some of the questions most often asked about sickle cell disease. Feel free to ask your doctor or nurse practitioner to go over any information you do not understand.
What is sickle cell disease?
Sickle cell disease is an inherited disorder of hemoglobin. Hemoglobin is the red color in the blood cells that carries oxygen to all the parts of the body. Normal hemoglobin is called hemoglobin A and sickle hemoglobin is called hemoglobin S. Sickle cell disorders can occur by themselves or in combination with other hemoglobin disorders.
There are three main types of sickle cell disease (hemoglobins):
1. Hemoglobin SS (also called sickle cell anemia)
2. Hemoglobin SC
3. Hemoglobin S-beta-thalassemia
There are other kinds of disorders and your doctor may know more about them. Hemoglobin S tends to form crystals. The crystals make the red blood cells stiff and sickle-shaped. Sickled red cells tend to get stuck in small blood vessels. This blocks the blood flow through these vessels.
What problems are cause by the lack of blood flow to different areas of the body?
1. Infection: The spleen is an organ that removes bacteria (germs) from the blood. Sickled cells tend to clog the spleen so bacteria are not removed properly. Bacteria can grow quickly in the bloodstream. A certain bacteria called pneumococcus may cause very serious infections in children with sickle cell disease. A fever may be the only sign of a pneumococcal infection. Without treatment a child can die within 12 hours of the start of the fever. To lessen the risk of pneumococcal infection, several things are recommended:
a. Penicillin - Children with sickle cell disease are given penicillin twice a day until 2 years of age. Children with hemoglobin SS are given penicillin until 5 years of age. The penicillin must be given even if the children do not appear to be ill. Liquid penicillin must be kept refrigerated. You must get a fresh supply of liquid penicillin every two weeks. After age two or age five for those with hemoglobin SS disease, penicillin is not given regularly unless your child’s hematologist feels it is necessary.
b. Immunizations - Children with sickle cell disease need to have the same immunizations as other children. They should also have pneumococcal and influenza vaccines. The pneumococcal vaccine is given at 2 and 5 years of age and every 5 years after that. The influenza vaccine is given every year in the fall. Your child should receive these vaccines if they were not already given in the past.
c. Fever management - A temperature over 101°F in children with sickle cell disease is an emergency. You should call your child’s doctor right away. Usually, immediate medical care and IV antibiotics are needed.
2. Pain episodes: Pain episodes (also called vasoocclusive crisis) are usually due to blockage of small blood vessels in the bones or muscles, but can affect any part of the body. Pain episodes can often be treated at home with ibuprofen and /or Tylenol® with codeine, rest, fluids, warmth and massage.
In infants, pain episodes may cause swelling of hands and/or feet. This is called hand/foot syndrome.
3. Aplastic crisis: Normal red blood cells live for four months. Sickled cells live for only two weeks. If new red blood cells are not being made, the amount of hemoglobin in the blood drops quickly. This causes severe anemia (a very low hemoglobin level). This is called an aplastic crisis. It is usually due to a certain viral infection called parvovirus. Severe anemia can be life threatening since oxygen is not carried properly throughout the body. Dizziness when standing, paleness and shortness of breath can be signs of severe anemia.
4. Sequestration crisis: Young children with sickle cell disease may have a sudden enlargement of the spleen, called a sequestration crisis. Red blood cells are caught in the spleen causing severe anemia. Dizziness with standing, paleness, shortness of breath and/or pain or swelling in the abdomen could mean sequestration.
5. Priapism: Priapism is a prolonged painful, nonsexual erection of the penis. This is due to sickled cells clogging up the blood vessels leading away from the penis. Priapism can lead to permanent impotence and therefore needs to be treated immediately.
6. Chest syndrome: Chest syndrome means there is sickling in the lungs. It may be caused by infection in the lung or by sickled cells clogging up blood vessels in the lungs. Chest syndrome can become a serious illness very quickly and may be fatal. Chest pain, fast breathing and/or shortness of breath may be signs of chest syndrome.
7. Stroke: A stroke is caused by sickled cells clogging the blood vessels in the brain. First signs and symptoms can be vague and nonspecific. Weakness on one side of the body and trouble speaking are the most common signs of a stroke. Weakness is often noticed first as a limp, which is not due to pain. These symptoms often go away within a few hours, but could be a warning sign that a serious stroke will occur. If stroke is suspected, seek medical attention immediately.
When should I call the doctor?
You should call the doctor right away for any of the following:
• Fever of 101°F
• Pain that does not go away after giving medicine (Ibuprofen/Tylenol with Codeine) at home
• Pain that is unusual for your child
• Dizziness with standing
• Chest Pain, shortness of breath or fast breathing
• A painful erection of the penis that does not go away
• Weakness on one side of the body
• Trouble speaking
Call anytime you are worried about your child’s condition. You may call the CMC hematology clinic during business hours at 937-641-3111 (8:00 am – 5:00 pm Monday through Friday). Any other time, call 937-641-3000 and ask to speak to the hematologist on call.
When should my child see the doctor?
He or she should see a doctor at the following times:
• Regular check-ups with his or her family doctor or pediatrician
• When he or she is ill
• A once a year visit at the comprehensive sickle cell clinic. Call 937-641-5013 to schedule an appointment.
What causes sickle cell disease?
All forms of sickle cell disease are inherited; children are born with sickle cell disease or trait. They inherit genes for the disease from their parents.
A child who inherits both normal and sickle hemoglobin have sickle cell trait (hemoglobin AS). Sickle cell trait is not a disease and does not change to a disease. When both parents have sickle cell trait, there is a 25% chance that each baby they have will have sickle cell disease (hemoglobin SS) a 50% chance that each baby will have sickle cell trait (hemoglobin AS) or a 25% chance that each baby will have normal hemoglobin (hemoglobin AA).
The inheritance of other forms of sickle cell disease can be explained by your doctor. Sickle cell trait, hemoglobin C trait and beta-thalassemia trait usually cause no problems, but there is a risk for sickle cell disease and other blood disorders in offspring.
This general information may not always apply to every family. If both parents are tested, more specific genetic information can be given to your family. Testing and genetic counseling are recommended before people with sickle cell disease have children.
Derechos de autor(c) de The Children's Medical Center, ano 1999. Este material unicamente tiene fines educativos. No puede ser reproducido, distribuido ni modificado sin previa autorizacion de The Children's Medical Center of Dayton, One Children's Plaza, Dayton, Ohio, 45404-1815. Llame al 937-641-3666 para solicitar autorizacion o para obtener un juego maestro para copias. Para obtener mas informacion puede visitar www.childrensdayton.org (consulte la seccion de informacion legal).
La informacion contenida en este material es unicamente informacion de tipo general. No debe considerarse como completa. Para obtener mas informacion acerca de los complementos para leche materna, por favor pidala a su doctor.
The information contained in this handout is for general information only and should not be considered complete. For specific information about bathing your baby, please ask your doctor or nurse practitioner.
Additional information may be located in the Family Resource Center, 2nd floor, near the Outpatient Surgery Center. Hours of the center vary; please contact the Family Resource Center at 937-641-3700.
Copyright(c) The Children's Medical Center of Dayton. This material is for educational purposes only. It cannot be reproduced or distributed without permission from Dayton Children's.
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