Saturday, June 16, 2001.


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Sickle Cell Anemia


What's New

Unless another source is noted, WellnessWeb's What's New items are used with permission from Doctor's Guide to the Internet,

  • October 15, 1999 --Nitric Oxide May Play Role In Treatment Of Sickle Cell Anemia
    Increasing levels of nitric oxide in the blood may offer new options for treating sickle cell anemia, the most common inherited blood disorder in the country.  In a study described in this month’s Journal of Clinical Investigation, researchers at the Warren Grant Magnuson Clinical Center at the National Institutes of Health are examining how nitric oxide acts on hemoglobin, the oxygen-carrying molecule in red blood cells. Nitric oxide, a gas produced in many parts of the body, relaxes the smooth muscle cells in blood vessels. As the muscles relax, the vessels expand and allow more blood to pass through. Improving blood flow is a critical aspect in treating the painful and potentially deadly complications associated with sickle cell anemia.   The Clinical Center (CC) study demonstrates that when nitric oxide is inhaled, it binds to hemoglobin. Once attached, the gas hitches a ride through the blood stream, and may dialate vessels as it passes through.  

  • 3/2/99 -- Sickle Cell Effects On Brain Found In Very Young Children
    Sickle cell disease alters the brains of very young children long before strokes or learning disabilities announce brain damage, suggest the results of a study published in this month’s issue of the Annals of Neurology.  "It appears that patients may be suffering brain damage at an earlier age than we thought before," said R. Grant Steen, Ph.D., an assistant professor of diagnostic imaging at St. Jude Children's Research Hospital in Memphis, TN., and one of the authors of the study.   Steen and his colleagues used a form of magnetic resonance imaging (MRI) not typically employed in clinical medicine to demonstrate subtle changes in the brains of children with the disease who had not shown any manifestations of neurological damage.   The results promise to spark debate about whether risky -- but potentially protective -- measures such as blood transfusions should be employed in younger patients without severe symptoms of the disease. 

Sickle Cell Disease in Newborns and Infants

A Guide for Parents -- National Library of Medicine

What is sickle cell disease?

Sickle cell disease is an inherited disorder of the red blood cells. Red blood cells carry oxygen to all parts of the body by using a protein called hemoglobin. Normal red blood cells contain only normal hemoglobin and are shaped like doughnuts. These cells are very flexible and move easily through small blood vessels.

But in sickle cell disease, the red blood cells contain sickle hemoglobin, which causes them to change to a curved shape (sickle shape) after oxygen is released. Sickled cells become stuck and form plugs in small blood vessels. This blockage of blood flow can damage the tissue. Because there are blood vessels in all parts of the body, damage can occur anywhere in the body.

The most common types of sickle cell disease are:

Sickle cell anemia
Hemoglobin SC disease
Sickle beta-thalassemia

Purpose of This Booklet

This booklet can help you understand sickle cell disease and how it can affect your child.

The best way to help your baby is to learn as much as you can about the disease, the problems it can cause, and what you can do to care for your baby. Talk about this booklet and your baby's health care choices with your doctor and others who know about sickle cell disease. Working together, you can give your child the best possible care.

You will find a description of the kinds of problems a baby with sickle cell disease may have on below of this booklet. Remember when you read it that not all babies will have all of these problems. At the back of this booklet, you will find a list of terms often used by doctors and nurses when they talk about sickle cell disease.

Types of Sickle Cell Disease

There are several forms of sickle cell disease. The most common is sickle cell anemia. Your doctor or nurse will tell you what kind of sickle cell disease your baby has. Be sure to write down the name so that you can refer to it if your baby has to go to a new doctor or clinic.

How are babies affected?

Babies with sickle cell disease may have:

  • Anemia (a low number of red blood cells). People with anemia may tire easily.
  • Aplastic crisis. Babies with sickle cell disease may stop making red blood cells for a short time. Signs include paleness, less activity than normal, fast breathing, and fast heartbeat. A baby with these signs must be seen quickly by the doctor.
  • Hand-and-foot syndrome. Babies with sickle cell disease may have pain and swelling in their hands or feet.
  • Painful episodes (mostly in the arms, hands, legs, feet, or abdomen). This happens when sickle cells plug blood vessels and block the flow of blood. Doctors call this a painful episode, event, or crisis.
  • Severe infections. The child with sickle cell anemia is at great risk for serious infections -- such as sepsis (a blood stream infection), meningitis, and pneumonia. The risk of infection is increased because the spleen does not function normally.
  • Splenic sequestration crisis. The spleen is the organ that filters blood. In children with sickle cell disease, the spleen can enlarge rapidly from trapped red blood cells. This condition is called splenic sequestration crisis and can be life-threatening.
  • Stroke. This happens when blood vessels in the brain are blocked by sickled red blood cells. Signs include seizure, weakness of the arms and legs, speech problems, and loss of consciousness. A baby with any of these signs must be seen quickly by a doctor.

Who is affected?

In the United States, most people who have sickle cell disease are African Americans. About 1 in 375 African-American children has sickle cell disease. Hispanic Americans from the Caribbean, Central America, and parts of South America also may have the disease. Sickle cell disease is also found in individuals from Turkey, Greece, Italy, the Middle East, or East India.

What causes sickle cell disease?

All forms of sickle cell disease are inherited. Children inherit genes for the disease from their parents.

Genes are substances within the father's sperm and the mother's egg that determine all of the physical characteristics of a baby. Children inherit the genes for hemoglobin from their parents. Persons who inherit both normal and sickle hemoglobin have sickle cell trait. Sickle cell trait is not a disease and does not change to disease. The individual sperm or egg from a person with sickle cell trait may contain either a gene for normal hemoglobin or a gene for sickle hemoglobin.

When both parents have sickle cell trait, for each pregnancy, the chances are: 1 in 4 that the baby will have only normal hemoglobin.
2 in 4 that the baby will have both normal and sickle
hemoglobin (sickle cell trait).
1 in 4 that the baby will have only sickle hemoglobin (sickle cell anemia).

The inheritance of other forms of sickle cell disease can be explained by your doctor.

How do I know if my baby has sickle cell disease?

All newborn babies should be tested for sickle cell disease. Many States have screening programs that test babies born in the hospital within a few days of birth. A blood sample is taken from the baby's heel for the sickle cell test, as well as screening tests for several other medical conditions.

If the test shows your baby might have sickle cell disease, the doctor will do the test again to make sure. The doctor may ask one or both parents for blood samples to test. If your baby has sickle cell disease, the doctor will tell you as soon as possible.

What if my baby has sickle cell disease?

If your baby has sickle cell disease, the doctor will help you find the best medical care for your child. This care could be provided by your family doctor, a pediatrician (children's doctor), or a pediatric hematologist (children's blood specialist), or a special sickle cell clinic. You also may want to see a counselor who can talk with you about your chances of having another baby with sickle cell disease.

Sickle cell disease is not just a medical problem. You may have many concerns about your baby and your family--for example, how to cope with your feelings and how to pay the medical bills. Your doctor or nurse can talk with you about your concerns. They also can help you find a local social service agency to assist you. In many areas there are sickle cell support groups, as well as community organizations that offer testing, education, and support to families affected by sickle cell disease.

How can I help my baby?

The best way to help your baby is to learn as much as you can about the disease and to make sure your baby gets the best health care possible. The child with sickle cell disease has special needs and must have regular medical care to stay as healthy as possible. The doctor or nurse will explain how often to bring your baby for medical care and what you can do if your baby becomes ill.

By 2 months of age, your baby should start taking penicillin by mouth twice each day. It is very important to give the medicine exactly as the doctor tells you. This will help prevent life-threatening infections. Penicillin should be continued until at least 5 years of age.

Also, by 2 months of age, your baby will get a shot to protect against H. influenzae, a type of bacteria that causes an infection which can be dangerous to people with sickle cell disease. The baby also will need a shot to protect against hepatitis B, a liver disease. At age 2, your child should receive pneumococcal vaccine. Your child should have all the other shots that children normally receive.

Here are some of the most important things you need to know about caring for a baby with sickle cell disease:

If your baby has a fever (over 101 degrees), you must get medical help right away. A fever in a child with sickle cell disease can be a sign of serious medical problems. Always take your baby's temperature when your baby appears sick. Your doctor will tell you what to do if your baby has a fever.

If your baby is sick, you must get medical help right away. Any sign of illness in a child with sickle cell disease can be serious. Your baby needs to see the doctor quickly if the baby:

  • Is breathing fast or having a problem with breathing
  • Coughs frequently
  • Is cranky and cries more than usual
  • Screams when touched
  • Is very tired or has little energy
  • Is very weak
  • Vomits
  • Does not want to eat
  • Has diarrhea
  • Has fewer wet diapers
  • Has pain or swelling in the abdomen
  • Has swollen hands or feet
  • Has pale blue or grey lips or skin

If any new doctor or health care provider sees your baby for any reason, explain that your baby has sickle cell disease.

A good diet is very important for all babies. Ask your doctor or other health care provider about the right foods and liquids for your baby. Make sure your baby drinks plenty of liquids. Find out if your baby also should have vitamins or iron.

Make sure your baby does not become overheated or chilly. Keep your baby warm. Cold baths or cold air can slow the baby's blood flow and cause problems.

Questions To Ask

You should always feel free to ask any question about sickle cell disease and how it affects you and your family. Here are some questions you may want to ask the doctor, nurse, counselor, or social worker. There is room at the end of the list for you to write your own questions.

  • What does my baby have? How did he or she get it?
  • What do I have? How did I get it? How will it affect me and my family?
  • How often does my baby need to see you?
  • What medicine does my baby need? What do I need to know about giving it?
  • What should my baby eat and drink?
  • Is there anything my baby should not do?
  • How can I tell if my baby gets sick?
  • What should I do, and who should I call, if my baby gets sick?
  • What other help is available to my family?

Additional Resources

To learn more about sickle cell disease and how to cope with it, contact:

National Association for Sickle Cell Disease
3345 Wilshire Boulevard
Suite 1106
Los Angeles, CA 90010-1880
Telephone: 1-800-421-8453

Additional organizations that can provide help include:

California State Department of Health
Childrens Medical Services Branch
Sacramento, CA 95814
Telephone: 916-654-0499
Cincinnati Comprehensive Sickle Cell Center
Children's Hospital Medical Center
Cincinnati, OH 45229
Telephone: 513-559-4200
Clinical Center Communications
9000 Rockville Pike
Building 10, Room 1C255
Bethesda, MD 20892
Telephone: 301-496-2563
Education Programs Associates
1 West Campbell Ave, Building D
Campbell, CA 95008
Telephone: 408-374-1210
Howard University
Comprehensive Sickle Cell Center
2121 Georgia Ave
Washington DC 20059
Telephone: 202-806-7930
March of Dimes
Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(For faster service, look in the telephone book for a local March of Dimes chapter in your area)
Mid-South Sickle Cell Center
Le Bonheur Children's Medical Center
Memphis, TN 38103
Telephone: 901-522-6792
Mississippi State Department of Health
Genetics Division
P.O. Box 1700
Jackson, MS 39215
Telephone: 601-960-7619
National Maternal and Child Health Clearinghouse
8201 Greensboro Drive
Suite 600
McLean, VA 22102
Telephone: 703-821-8955
New York State Department of Health
Newborn Screening Program
Wadsworth Center for Laboratories and Research
P.O. Box 509
Albany, NY 12201-0509
Telephone: 518-473-7552
Northern California Comprehensive
Sickle Cell Center
San Francisco, CA 94110
Telephone: 510-428-3651
Texas Department of Health
Newborn Screening Program
1100 West 49th St
Austin, TX 78756-3199
Telephone: 512-458-7111

This is not a complete list. Check with your state or local health department or sickle cell agency for other sources of information.

Common Sickle Cell Terms

Your doctor, nurse, or other caregiver may use these terms in talking with you about sickle cell disease and your child.

Acute chest syndrome.
A serious condition caused by infection or trapped red blood cells in the lungs. Fast or difficult breathing, chest pain, and coughing are signs of acute chest syndrome in the child with sickle cell disease. A child with acute chest syndrome usually will have to go to the hospital for treatment.
A reduced number of red blood cells. Anemia occurs in persons with sickle cell disease because sickled red blood cells do not live as long as normal red blood cells. A child with sickle cell disease cannot make red blood cells fast enough to keep up with the rapid breakdown, so the person with sickle cell disease has fewer red blood cells than normal and is anemic.
Aplastic crisis.
Occurs when a child's bone marrow temporarily stops producing red blood cells. A child with aplastic crisis may appear pale and be tired and less active than usual.
Tiny blood vessels where sickle-shaped blood cells may get trapped and cause problems.
The biological units that are passed from both parents to a child. Genes determine all of the child's characteristics -- for example, hair, eye, and skin color, foot size, height -- and whether the child will have sickle cell disease or another inherited disease.
Haemophilus influenzae.
A type of bacteria that causes infection and can lead to serious problems in the child with sickle cell disease. Babies must receive a special vaccine beginning at 2 months of age to protect them from this condition.
Hand-and-foot syndrome.
Pain and swelling of the hands and feet caused by sickle-shaped red blood cells that plug blood vessels in the hands and feet. Often this will be the baby's first problem caused by sickle cell disease.
A molecule found in red blood cells that carries oxygen from the lungs to other parts of the body.
Pain event or painful episode.
Pain caused by plugging of blood vessels by sickled blood cells. Pain is most often felt in the arms, legs, back, and abdomen. The pain may last only a few hours or as long as a week or two. The pain may be mild or so severe that pain medicine is needed. The number of pain events a person has may vary greatly.
The presence of infection in the blood stream.
Sickle cell anemia.
The most common form of sickle cell disease. Other types of sickle cell disease include hemoglobin SC disease and sickle beta-thalassemia; there are also other, less common types of sickle cell disease.
Sickle cell disease.
A group of inherited disorders in which anemia is present and sickle hemoglobin is produced.
Sickle cell trait.
The condition in which a person has both normal and sickle hemoglobin in the red cells as a result of inheriting a normal hemoglobin gene and a gene for sickle hemoglobin. Sickle cell trait is not a disease and does not change to sickle cell disease. Persons with sickle cell trait may pass the sickle gene to their children.
Sickled cells.
In children with sickle cell disease, hemoglobin molecules in red blood cells stick to one another and cause the red cells to become crescent or sickle shaped. Sickled cells cannot pass easily through tiny blood vessels.
Splenic sequestration crisis.
Occurs when a large portion of the child's blood becomes trapped in the spleen. Early signs include paleness, an enlarged spleen, and pain in the abdomen.
Streptococcus pneumoniae.
A bacteria that causes a very serious type of pneumonia in children with sickle cell disease. Twice daily doses of penicillin by mouth, starting at about 2 months of age, can help to prevent this life-threatening infection in children with sickle cell anemia and sickle beta0-thalassemia.

For More Information

The information in this booklet was taken from the Clinical Practice Guideline on Sickle Cell Disease: Screening, Diagnosis, Management, and Counseling in Newborns and Infants. The guideline was written by a panel of experts sponsored by the Agency for Health Care Policy and Research. Other guidelines on common health problems also are being developed.

For more information about guidelines, or to order extra copies of this booklet, contact:

Agency for Health Care Policy and Research
Publications Clearinghouse
P.O. Box 8547&127
Silver Spring, MD 20907

Or call 1-800-358-9295 (for callers outside the US, only: 301-495-3453) weekdays, 9 a.m. to 5 p.m., Eastern time.

Department of Health and Human Services
Public Health Service
Agency for Health Care Policy and Research
Executive Office Center, Suite 501
2101 East Jefferson Street
Rockville, MD 20852
AHCPR Publication No. 93-0564
April 1993


The Center for Sickle Cell Disease
Howard University
2121 Georgia Avenue NW
Washington, D.C. 20059
Phone: 202-806-7930

Sickle Cell Disease Association of America
3345 Wilshire Boulevard, Suite 1106
Los Angeles, CA 90010-1880
(310) 216-6363; 800-421-8453; FAX: (310) 215-3722

The Georgia Comprehensive Sickle Cell Center
PO Box 109
Grady Health System
80 Butler St.
Atlanta, Georgia 30335

Sibling Donor Cord Blood Program at Children's Hospital Oakland serves families nationwide who wish to save cord blood for their children, when there is a specific medical indication for banking (such as sickle cell anemia.)

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