Sickle Cell Disease. Sickle Cell Anemia

What is sickle cell disease?

What is sickle cell disease?

Sickle cell disease is a family of inherited blood disorders. It affects people of African ancestry.What these disorders have in common is a problem with hemoglobin, a special protein found in red blood cells.

Sickle cell disease is an inherited condition. It is not infectious—no one can “catch” it from a person who has the disease.

About 80,000 people in the United States have sickle cell disease. About 1,000 babies are born with the disease every year.

An estimated 2.5 million people in the United States have sickle cell trait, which means they have inherited the gene for sickle cell disease. People with sickle cell trait are healthy, but two people with sickle cell trait have a one in four chance of having a child with sickle cell disease.

The disease occurs in roughly one in every 500 African American births. About one in every 12 African Americans carries the sickle cell trait.

Around the world, an estimated 75 million people have sickle cell trait and nearly 200,000 babies are born with the disease every year.

Sickle cell disease is most common among people whose ancestors come from sub-Saharan Africa, South and Central America, Saudi Arabia, India, and Mediterranean countries such as Turkey, Greece, and Italy.

 

Understanding what sickle cell disease is:

The job of the red blood cells is to carry oxygen from the lungs to all parts of the body. Hemoglobin, a protein inside the red blood cells, carries the oxygen. Hemoglobin also gives red blood cells their red color. Normal red blood cells are donut-shaped and soft, so that they can squeeze through even the smallest of blood vessels.

In sickle cell disease, the hemoglobin in red blood cells is abnormal. When it gives up its oxygen to the body’s tissues, it goes rigid, causing the red blood cells to buckle. The cells lose their soft texture and round shape. Instead, they become hard and take on the shape of a crescent moon or a sickle.

The stiff, sickle-shaped red blood cells cannot flow easily through small blood vessels as normal red blood cells do. Sometimes they get stuck in the blood vessels. This is known as vaso-occlusion. When this happens, the blood cannot get to the body’s tissues to deliver oxygen and other nutrients and to remove waste products.

If a blood vessel remains blocked for some time, the organs or tissues supplied by that vessel become inflamed and may die. Many of the problems of sickle cell disease stem from this organ and tissue damage.

There are several types of sickle cell disease. The most common is sickle cell anemia.

Others types are:

• sickle-hemoglobin C disease

• sickle beta-plus thalassemia

• sickle beta-zero thalassemia

 

 

What causes sickle cell disease?

Sickle cell disease is inherited. Parents pass it to their children in their genes. Genes are the body’s map for development. We have a pair of genes for our eye color, our blood type, and each of our other features. We also have a pair of genes for our hemoglobin type. We inherit one of each pair of genes from our mother and one from our father.

To inherit sickle cell disease, a child must receive abnormal hemoglobin genes from both parents. A child who inherits an abnormal hemoglobin gene from only one parent does not get sickle cell disease. But anyone who has an abnormal hemoglobin gene can pass it to his or her children. A person who has one abnormal hemoglobin gene is said to have sickle cell trait.

 

What are the chances of having a baby with sickle cell disease?

Children inherit two hemoglobin genes—one from each parent.

• If both parents have sickle cell trait (one abnormal hemoglobin gene), the chances that their baby will have sickle cell disease (by inheriting one abnormal gene from each parent) are 1 in 4. This does not mean that if the parents have four children, only one will have sickle cell disease. It means that for each pregnancy, there is

1 chance in 4 that the baby will have the disease

1 chance in 2 that the baby will have sickle cell trait

1 chance in 4 that the baby will have neither the trait nor the disease

 

• If one parent has sickle cell disease (two abnormal hemoglobin genes) and one has sickle cell trait (one abnormal hemoglobin gene), the chances that their baby will have sickle cell disease are 1 in 2. This means that for each pregnancy, there is

1 chance in 2 that the baby will have the disease

1 chance in 2 that the baby will have sickle cell trait

 

 

Is sickle cell trait a disease?

A person who has sickle cell trait has one abnormal hemoglobin gene. People with sickle cell trait are healthy. They do not have a “mild case” of sickle cell disease. Having the trait does not cause health problems, and the trait never changes into sickle cell disease. But if two people with sickle cell trait have a child, the child may have sickle cell disease.

 

How can you find out if you have sickle cell trait?

Many families carry sickle cell trait (one abnormal hemoglobin gene) without knowing it. The only way to know for sure whether or not you have the trait is to have a special blood test. The most widely used test is called hemoglobin electrophoresis (electro-for-EE-sis) with complete blood count.

Your blood sample is sent to a laboratory, where it is treated by passing an electrical charge through a solution of hemoglobin. This test will reveal whether you have normal or abnormal hemoglobin.

 

Can sickle cell disease be cured?

Currently there is no cure for sickle cell disease. However, the disease can be treated. With good medical care and support, people with sickle cell disease can lead reasonably healthy and productive lives.

 

How is sickle cell disease detected?

In most countries babies of African ancestry are tested for sickle cell disease or sickle cell trait at birth. This simple blood test is performed at the same time that infants are screened for a range of other diseases. The most widely used test is called hemoglobin electrophoresis (electro-for-EE-sis) with complete blood count.

It is also possible to test for sickle cell disease or sickle cell trait before birth. Two kinds of prenatal tests can be done.

 

Chorionic villus sampling

In this test, a small piece of the placenta (the special lining that grows within the uterus during pregnancy to nourish the developing fetus; sometimes called the “afterbirth” because it is expelled from the uterus a few hours after childbirth) is gently removed through a tiny tube that is inserted into the uterus. This test can be done at nine to 12 weeks of pregnancy.

 

Amniocentesis

In this test, a small amount of amniotic fluid (fluid that surrounds, protects, and nourishes a fetus while it is growing in the womb.) is removed from the uterus through a needle. This test can be done at 15 to 19 weeks of pregnancy. For more detailed information about aminocentesis, go to aminocentesis.

 

Should you be tested for sickle cell trait?

Couples who are planning to start a family should consider being tested for sickle cell trait if

• Anyone in either of their families has sickle cell disease (or had it in the past).

• Their ancestors came from a part of the world where sickle cell trait is common, such as Africa, Central or South America, Saudi Arabia, India, Turkey, Greece, or Italy.

 

It may be helpful to consult a genetic counselor to help you decide whether to be tested and, if you decide to proceed, to help you understand the test results. Genetic counselors are trained to offer information and support about inherited diseases to individuals and families.

 

Symptoms of sickle cell disease

Many people with sickle cell disease feel well most of the time. In some people the disease causes only mild problems while in others it can cause serious problems. Most of the problems of sickle cell disease are caused by sickled red blood cells getting stuck in narrow blood vessels, blocking the flow of blood. Most people with sickle cell disease will have some of the following problems at some time in their lives.

 

• Pain. Pain is the most common problem experienced by people with sickle cell disease. Children most often have pain in the arms, legs, elbows, ankles, and knees. Adolescents and adults frequently have pain in the shoulders, hips, chest, pelvis, and spine.

 

What causes the pain of sickle cell disease?

When sickled red blood cells get stuck in blood vessels, they cut off the blood supply to nearby tissues. As a result, the tissues are starved of oxygen. When this happens, the person with sickle cell disease feels pain in the part of the body where the blood vessels are blocked.

 

What is a painful crisis?

A painful crisis is an episode of pain in a body organ or in the bones, joints, or muscles. Some people with sickle cell disease have these painful episodes less than once a year, while others may have them as many as 15 times a year. The pain may last anywhere from a few hours to several weeks. Hospitalization may be necessary for treatment of severe, prolonged pain.

 

What is hand-foot syndrome?

Hand-foot syndrome (dactylitis) occurs when sickled red blood cells block the small blood vessels in the hands or feet, causing pain, swelling, and fever. This may be the first problem that sickle cell disease causes in infants and young children. About one in three children with sickle cell disease who are less than three years old get hand-foot syndrome.

In most children, the pain of hand-foot syndrome is mild and the swelling goes down within a couple of days. It can usually be treated at home with extra fluids and over-the-counter pain medication. If the child has severe pain or a fever, he or she may need to be treated in the hospital with stronger pain medicine and intravenous fluids. Hand-foot syndrome almost always goes away without any lasting effects.

 

• Infections. People with sickle cell disease are more prone to infections than other people. Babies and young children can become very sick from infections if they do not get prompt treatment.

 

Why do people with sickle cell disease get infections?

Most infections in people with sickle cell disease are caused by problems with the spleen The spleen is the largest lymph node in the body. Lymph nodes, found throughout the body, help the body to fight off infections. When sickled red blood cells clog the spleen, it cannot do its job.

 

[PICTURE of human body showing location of the spleen and other lymph nodes]

 

What kinds of infections can people with sickle cell disease get?

Three kinds of bacteria are responsible for many infections in people with sickle cell disease.

• Streptococcus pneumoniae (also known as pneumococcus)
• Salmonella
• Haemophilus influenzae (H. influenzae)

All of these bacteria can cause serious infections of the blood (septicemia) or the lungs (pneumonia). Salmonella can also infect the bones (osteomyelitis). Any of these infections can be life-threatening.

A common virus called the parvovirus can infect developing red blood cells in the bone marrow. This stops production of new red blood cells, causing severe anemia.

How can I tell if my child with sickle cell disease has an infection?

The most important sign of infection is fever. Take your child to the doctor right away if he or she has a fever of more than 101^˚F.

A cold can easily turn into pneumonia in a child with sickle cell disease. Signs of pneumonia include:

• fever

• frequent coughing

• rapid breathing

• shortness of breath

• tiredness

• chest pain

 

Young children are most at risk for serious infections with the pneumococcus bacteria. Daily penicillin pills, from infancy until at least age 5, can prevent most of these infections.

• Anemia.  Anemia is the medical name for a shortage of red blood cells. Problems commonly caused by anemia include fatigue, pale skin, and shortness of breath. For more detailed information about anemia, go to anemia.

 

What causes anemia in people with sickle cell disease?

Sickle cell disease makes the red blood cells rigid and sickle-shaped instead of soft and round. The body recognizes that the rigid, sickle-shaped cells are abnormal and destroys them at a faster rate than normal. The average life span of a sickled red blood cell is about 10 days, compared with 120 days for a normal red blood cell. The body tries to make extra red blood cells in the bone marrow, but often cannot make enough to replace the cells it destroys.

 

Why do people with sickle cell disease feel easily tired?

Red blood cells carry oxygen to the muscles to serve as fuel for activity. Because people with sickle cell disease have fewer red blood cells than normal, their muscles get less oxygen. For this reason, they tire more quickly than people with normal blood counts.

 

What is an aplastic crisis?

A young child with sickle cell disease may stop making red blood cells for a short time. This is called an aplastic crisis. Signs of an aplastic crisis include pale skin, fast breathing, a rapid heartbeat, and less activity than normal. Take your child to the doctor right away if he or she shows these signs. An aplastic crisis is treated with blood transfusions.

 

• Organ swelling. Sickled red blood cells can cause a lot of blood to become trapped in an organ. As a result, the organ stops working and may swell up. Swelling of an organ is called sequestration. Children with sickle cell disease may experience swelling of the spleen. Swelling of the liver is more likely to occur in older children and adults. These problems are usually treated by an exchange blood transfusion that removes the sickled cells and supplies normal red blood cells.

 

What is splenic sequestration?

In infants and young children, sickled cells can cause the spleen to swell up suddenly, trapping a lot of blood cells. As a result, the blood count quickly drops, and this can lead to heart failure.

In some cases, swelling of the spleen is related to an infection. Parents can help prevent this problem by learning to feel for their baby’s spleen and calling the doctor right away if the spleen is enlarged.

If a child has one splenic sequestration, he or she is more likely to have another. In children over two, the doctor may suggest surgery to remove the spleen to prevent this problem from happening again. Children under two will be treated with blood transfusions until they are old enough for surgery.

 

What is sickle chest syndrome?

Sickle chest syndrome is a swelling of the lungs caused by sickled red blood cells. This condition is also called acute chest syndrome. This potentially life-threatening problem, which causes fever and chest pain, is most likely to occur in adolescents and adults.

The lungs are the source of the oxygen that the red blood cells carry to the body’s tissues. When sickled red blood cells get trapped in the lungs, the lungs can hold less oxygen. A “vicious cycle” can result: Blood oxygen levels can fall, causing more sickled red blood cells to get trapped in the lungs, which further reduces the lungs’ capacity for oxygen.

Sickle chest syndrome is usually treated by an exchange blood transfusion. In this type of transfusion, the sickled blood is removed and replaced with healthy blood. People who are severely affected may need to be on a ventilator for a few days to help them breathe.

• Kidney problems. The kidneys help the body to retain fluid and get rid of wastes. Sickle cell disease can cause a number of kidney problems, including infections, blood in the urine, and problems with urination.

 

Kidney and bladder infections

People with sickle cell disease are very prone to infections. Sometimes these infections affect the kidneys and the bladder.

Signs of a kidney or bladder infection include:

• fever

• urine that smells foul or looks cloudy

• needing to urinate frequently or urgently

• burning or pain while urinating

• pain in the abdomen or back

 

Blood in the urine

Sickled red blood cells that clog the kidneys can causing bleeding in the urine. When this happens, the urine may be brownish or bright red in color.

 

Problems with urination

• Frequent urination and bedwetting. Sickled red blood cells can damage the kidneys, making it harder to hold onto urine. People with sickle cell disease may need to pass urine frequently. More than one in three children with sickle cell disease continue to wet the bed at least three times a week after their fourth birthday. The medical name for bedwetting is enuresis.

• Dehydration. Because they retain less fluid, children with sickle cell disease can easily become dehydrated when they are sick. Tiredness and passing urine less often than usual are signs of dehydration.

• Other health problems. Sickle cell disease can cause problems in many of the body’s organs and tissues. Not everyone with sickle cell disease will have all of these problems. In people who do experience these problems, they can vary in severity from mild to serious.

 

Delayed growth

Because sickled red blood cells break down more quickly than normal cells, people with sickle cell disease have fewer red blood cells than other people. This may cause children who have sickle cell disease to grow more slowly than other children. As a result, they are often shorter and slighter in build than other children their age. They may go through puberty at a later age than many of their friends and school mates.

In most cases, children with sickle cell disease keep growing after other children stop. Most of them eventually grow to the size and height they would be expected to reach. Children who are very thin or small for their age may need extra food or a special diet to help them grow. However, special diets will not help most children with sickle cell anemia to grow faster.

 

Jaundice

Sickled red blood cells have a much shorter life span than normal red blood cells—about ten days compared with 120 days for a normal cell. This rapid breakdown of red blood cells can cause yellowing of the skin and eyes, which is known as jaundice. Jaundice may also be a sign of gallstones.

 

Gallstones

Gallstones are another problem that can occur because sickled red blood cells have a much shorter life span than normal red blood cells. The dead cells collect in the gall bladder forming a thick sludge or gall stones. About one in every three children with sickle cell disease is likely to develop gallstones by the age of seven.

Gallstones are not harmful unless they get stuck in the gall bladder duct. This can cause a serious infection. Eyes that are very yellow can be a sign that gallstones are stuck in the duct. Pain in the right side of the abdomen can be a warning sign that a person has gallstones. The usual treatment for gallstones is surgery to remove the gall bladder. For more detailed information about gallstones, go to gallstones.

 

Eye problems

Because people with sickle cell disease are short of healthy red blood cells, their body tissues often do not get enough of the nourishment that circulating red blood cells normally provide. Over time, this can result in tissue damage. Two or three out of every ten people with sickle cell disease will have eye problems caused by damage to the retina the part of the eye that receives and processes visual images. In some cases, this damage can be severe enough to cause blindness. Regular checkups by an eye doctor can help to ensure that eye problems are identified and treated before they seriously damage the eyesight.

 

Stroke

Sickled red blood cells may block a blood vessel in the brain, causing a stroke. Stroke is a rare complication of sickle cell disease, occurring in fewer than one out of ten children with the disease. The following may be signs of a stroke:

 

• fainting

• dizziness

• severe headache

• difficulty speaking

• numbness in the face

• sudden weakness in an arm, leg, or the whole body

 

Take your child to the hospital right away if he or she has any of these signs. Getting treatment as quickly as possible can keep a stroke from getting worse. For more detailed information about stroke, go to stroke.

 

Priapism

Sickled red blood cells can get stuck in a blood vessel in the penis, causing a painful erection known as priapism. This problem can occur in boys as young as four, although it is more common in adults. There are two forms of priapism.

• In stuttering priapism, the penis repeatedly becomes hard and painful for several hours at a time.

• In fulminant priapism, the penis stays erect and painful for more than 24 hours.

 

If priapism occurs repeatedly or is prolonged, it is usually treated with blood transfusions. Prolonged priapism can damage the penis and cause impotence. For more detailed information about impotence, go to impotence.

 

How is sickle cell disease treated?

Sickle cell disease is treated in two ways:

• by trying to prevent the problems the disease can cause, and

• by treating problems caused by the disease when they occur

 

How can the problems of sickle cell disease be prevented?

• A healthy lifestyle. People with sickle cell disease can take steps to reduce the occurrence of blood vessel blockages caused by sickled red blood cells.

• Do not smoke.
• Always keep warm.
• Drink plenty of fluids.
• Eat a healthy diet.
• Get plenty of rest.
• Avoid stress.
• Drink very little or no alcohol.
• Get regular medical checkups.
• Treat any infection as soon as it occurs.

 

Regular, moderate exercise can also help to prevent blood vessel blockages. But take care not to become too fatigued from exercise. People with sickle cell disease should avoid swimming in cold water or doing strenuous activities such as long-distance running and jobs that involve heavy physical labor.

 

• Penicillin. All children with sickle cell disease should take penicillin pills twice a day, beginning when they are about two months old and continuing until they are at least five years old. This is the most important step that can be taken to prevent life-threatening pneumococcal infections. Vaccination offers additional protection against these infections, but children who have been vaccinated should still take penicillin.

 

• Vaccinations. It’s important for all children to get all their childhood shots, but it’s particularly important for children with sickle cell disease. This is because they are more likely than other children to get serious infections. By the age of two months, babies with sickle cell disease should be vaccinated against H. influenzae. They should also get a shot to protect them from hepatitis B, a liver disease. At age two, children with sickle cell disease should receive pneumococcal vaccine.

 

How are the problems of sickle cell disease treated?

• Treatment of pain. Mild pain can be treated by taking over-the-counter pain medication and drinking plenty of fluids (to avoid or overcome dehydration). Warmth, rest, massage, and physical therapy can also help to relieve pain. Severe pain may need to be treated in the hospital with strong pain medications and intravenous fluids.

 

• Blood transfusions. Many of the problems caused by sickle cell disease can be treated with blood transfusions. Transfusions help anemia by boosting the number of normal red blood cells in circulation. Regular transfusions can prevent additional strokes in children who have already had a stroke. When organs become swollen because they are clogged with sickled cells, an exchange blood transfusion is usually performed to remove the sickled cells and replace them with healthy red blood cells.

 

• Surgery. Gallstones that get stuck in the gall bladder duct can cause a serious infection. If this happens, emergency surgery is needed to remove the gall bladder. Sometimes the doctor will recommend surgery to remove the gall bladder to prevent gallstones from getting stuck in the gall bladder duct.

A person can survive quite well without a gall bladder, although some people who have had their gall bladder taken out may experience diarrhea.

If a child is having serious problems caused by a swollen spleen, the doctor may recommend surgery to remove the spleen. A person does not need a spleen to live, but without one he or she has a higher risk of getting infections. A child whose spleen is taken out should keep taking penicillin twice a day until adulthood to prevent infections.

 

• Medication. In 1998 the U.S. Food and Drug Administration approved the drug Droxia (hydroxyurea) to treat people with sickle cell disease who are over 18 and who have suffered three or more painful crises in the previous year. The drug is not currently approved to treat children. Studies are now under way to test its safety and effectiveness in young people.

In adults, Droxia has been shown to reduce the frequency of painful crises and sickle chest syndrome, as well as the need for blood transfusions. A side effect of the drug is a drop in the blood count, which can increase the risk of infections and bleeding. People taking the drug must have a blood test every two weeks to ensure that their blood count is not too low. They may have to stop taking the drug from time to time to allow their blood count to increase to an acceptable level.

Droxia seems to work by stimulating red blood cells to produce fetal hemoglobin. Fetal hemoglobin is a form of hemoglobin that we all make before birth, but most people stop making soon after birth. Some people with sickle cell disease continue making fetal hemoglobin after birth, and these people often have less severe disease. Fetal hemoglobin seems to prevent red blood cells from becoming rigid and sickle-shaped.

Droxia is not a new drug. Under the name Hydrea, it has been used for many years to treat certain kinds of leukemia and other cancers.

 

• Bone marrow transplantation. Some children with severe sickle cell disease can be cured by a bone marrow transplant. In this complex surgery, the sick child’s bone marrow is removed and replaced by healthy bone marrow donated by a brother or sister. The donated bone marrow must match that of the child with sickle cell disease. Only about one in five children with the disease is likely to have a brother or sister whose bone marrow is a close enough match.

In a study published in 1996, 22 children ages three to 14 received bone marrow transplants from a matched brother or sister. Nine out of ten children survived and seven out of ten were cured. Before the transplant surgery, all of the children had had at least one stroke, several painful crises, or several episodes of sickle chest syndrome.

Before a bone marrow transplant, patients must take strong drugs that destroy their bone marrow. Then they take more drugs to prevent their bodies from rejecting the healthy transplanted marrow. Sometimes these drugs are not enough to stop rejection.

A bone marrow transplant is major surgery that requires several weeks of hospitalization. The drugs given to prevent rejection of the transplanted bone marrow leave the patient at high risk for serious infection. For this reason, the patient must stay in a special germ-free hospital room.

 

When to call the doctor?

Call the doctor right away for any of the following:

• Pain that does not respond to fluids, warmth, rest, and over-the-counter pain medication.

• Pain that goes on for a much longer time than usual.

• Pain in the chest or abdomen.

• Pain accompanied by a fever, swelling, or redness.

• Sudden paleness, rapid breathing, a fever of more than 101^˚F or a heart rate of more than 100 beats a minute.

• Any suspicion of infection.

• Frequent coughing, weakness or severe fatigue, loss of appetite, or diarrhea.

• Swollen hands or feet.

• Swelling in the abdomen.

• Severe headache.

• Numbness or inability to move any part of the body, strange facial expressions, or abnormal movements.

• Pale blue or gray lips or skin.

 

What is the long-term outlook for people with sickle cell disease?

With a healthy lifestyle and good medical care, many people with sickle cell disease can stay in reasonably good health much of the time. They can expect to live to their mid-40s or longer.

The course of the disease varies in different people. Some people may go for years with few problems, then go through a period when they have many problems, and with time pass into a less severe phase again. In a family in which several people have sickle cell disease, some individuals may be severely affected and others only mildly. The reasons for this are not well understood.

Research continues to try to develop better treatments for sickle cell disease and, ultimately, a cure. The best hope of a cure may be gene therapy (an experimental way of treating or preventing disease by altering a person’s genes) to correct the defect that causes abnormal hemoglobin.

People with sickle cell disease can help themselves by learning as much as they can about the disease so that they can recognize problems early and seek treatment before the problems become serious. Support and counseling can help individuals and their families to cope with the stresses of living with a serious chronic disease.

If possible, individuals may wish to seek treatment at a comprehensive sickle cell center. Doctors, nurses, and other health care staff at these centers have special training and expertise in caring for people with sickle cell disease.

 

Frequently asked questions

Q: My fiancé has a first cousin who has sickle cell disease. We are planning to have a family. What is our risk of having a baby with this disease?

A: Sickle cell disease occurs when a baby inherits abnormal hemoglobin genes from both parents. Having one abnormal hemoglobin gene is known as sickle cell trait. People with sickle cell trait are healthy, but when two people with sickle cell trait have a child, there is one chance in four that the child will have sickle cell disease. If one parent has the trait and the other does not, they cannot have a child with sickle cell disease. However, the gene for sickle cell trait can be passed on to the child.

In the United States, sickle cell trait is most common among people of African ancestry. About 1 in every 12 African Americans carries the trait, but many are unaware of it. If someone in your fiancé’s family has sickle cell disease, it is possible that your fiancé carries the trait. The only way to know for sure whether or not you have the trait is to have a special blood test.

If you are concerned, consider consulting a genetic counselor about whether both of you should have the blood test. Genetic counselors are trained to offer information and support about inherited diseases to individuals and families.

 

Q: We are planning to take a trip with our 4-year-old daughter, who has sickle cell disease. What precautions should we take?

A: Ask your doctor to give you a letter that states what kind of sickle cell disease your child has, what medication she takes what her normal hemoglobin level is, what other problems she has, and who to call in an emergency. Take the letter with you when you travel with your child. Also talk with your doctor about where to take your child if she needs medical care during your trip.

Be sure to take your child’s penicillin and any other medications she takes with you on your trip. While you are traveling, whether by car or plane, make sure your child drinks plenty of fluids.

Be extra careful if you are driving or hiking at an altitude higher than 5,000 feet. At such high altitudes, there is less oxygen in the air. Give your child extra fluids and make sure that she rests if she feels tired. If she starts to feel sick, take her to a lower elevation. Note that traveling in a commercial plane should not cause altitude sickness because the air is pressurized.

 

Q: Is it okay for my 8-year-old son who has sickle cell disease to play games with his friends?

A: Sickle cell disease need not prevent your child from playing with his friends as long as he takes a few simple precautions: resting when he feels tired, drinking extra fluids when he’s active, and dressing for the weather (for example, putting on a jacket when it’s cold). Your child should not go swimming in cold water.

Talk to your doctor about whether it’s wise for your child to go skiing or backpacking at a high altitude. Also talk to the teachers, coaches, or other adults who supervise your child’s activities. Tell them that he needs to drink extra fluids and rest when he gets tired.

 

Q: Our doctor is recommending that our 1-year-old daughter with sickle cell disease start taking a folic acid supplement. What is folic acid and why does my child need to take it?

Folic acid is a vitamin that helps the body to make new red blood cells. It can be helpful for some children with sickle cell disease. The dose that is usually recommended is 1 mg a day. The tablet can be crushed and mixed with milk, juice, or food. Sometimes doctors will recommend that children with sickle cell disease take other vitamin and mineral supplements, such as vitamin E, zinc, and iron.

 

Additional sources of information

Sickle Cell Disease Association of America, Inc.

200 Corporate Pointe, Suite 495

Culver City, CA 90230-8727

Phone: (310) 216-6363 or (800) 421-8453

Fax: (310) 215-3722

E-mail: scdaa@sicklecelldisease.org

Web site: http://www.sicklecelldisease.org

 

National Heart, Lung, and Blood Institute Information Center

P.O. Box 30105

Bethesda, MD 20824-0105

Phone: (301) 592-8573

Fax: (301) 592-8563

E-mail: NHLBIinfo@rover.nhlbi.nih.gov

http://www.nhlbi.nih.gov/health/health-topics/topics/sca/