Medical Care

Fever and Infection

Infection is a life-threatening problem of sickle cell disease. Unlike other children, children with sickle cell disease have less resistance to fight infections.

For this reason, children with sickle cell disease may have more infections than other children do. They also tend to easily get more serious infections.

The child with a serious infection may not seem very sick at first. It is important to learn the early warning signs of an infection and know when to take your child right away to the doctor or hospital.

• Signs and Symptoms
  • Fever (high temperature of 101 degrees or higher) is the most common symptom of infection.
• Special Care for Fever
  • Do not give any medicine such as Tylenol before checking the temperature.
  • Use a thermometer to check for fever.
  • Do not wait for a fever to go down.

**Call the child's doctor right away or take the child to a hospital emergency department.

Special Care to Help Prevent Infections

• Penicillin
  • It is important for the child to take penicillin twice a day. Antibiotics such as penicillin help prevent dangerous infections.
  • It is now recommended that some children above the age of 5 or 6 years stop taking penicillin under certain conditions. Do not stop taking antibiotics unless you have been advised by your doctor. If your child has had his/her spleen removed or if a bacterial infection in the bloodstream has occurred in the past, he/she should not stop taking penicillin. Also, we recommend that your child receive a second vaccination with Pneumovax before stopping penicillin.
• Pneumovax
  • Pneumococcal vaccine (Pneumovax) should be given to the child by 2 years of age. We recommend that this vaccination be repeated in 3 to 5 years. It may need to be repeated later as well. This vaccine is given to children with sickle cell disease to help prevent infections caused by the bacteria called pneumococcus.
• Other Immunizations
  • Your child must also have the usual childhood immunizations (baby shots).
  • Hepatitis B vaccine is recommended for all children as part of their routine childhood vaccinations whether they are affected by sickle cell disease or not. It helps to prevent a serious viral illness that can be transmitted by blood transfusions.

Anemia

Normal red blood cells live about four months before they break down. Your body makes new red blood cells all the time to replace the older ones that are slowly breaking down. Sickle red blood cells live less than one month. Even though the body tries, it cannot make new red blood cells fast enough to replace the old ones. This causes the child’s red blood cell count to be low (anemia).

When red blood cells break down, the hemoglobin changes into a yellow colored substance called bilirubin. People with sickle cell anemia make a lot of bilirubin. As a result, their skin, eyes and urine may look yellow (jaundice).

Children with sickle cell disease usually have anemia. They also usually adjust and do not have problems from it. There are times, however, when their blood count drops lower than usual. This can happen as a result of an illness or infection.

At certain times, the child can not make blood cells quickly enough. Their blood count drops suddenly, making them tired, pale and having a poor appetite. The term aplastic crisis is used to describe the problems resulting from this sudden drop in blood count.

• Signs and Symptoms
  • Tire easily.
  • Poor appetite.
  • Paleness.

• Special Care
  • If you notice any of these signs and symptoms, call the child's doctor. Hospitalization and blood transfusion may be required.

Splenic Sequestration Crisis

The spleen is an organ on the left side of the belly under the rib cage. It helps the body fight infection. In children with sickle cell disease, the spleen may get plugged with sickle cells. When the plugging in the spleen makes it suddenly fill with blood and get bigger, the problem is called splenic sequestration crisis. Splenic sequestration crisis can happen with little notice in a few hours and is life-threatening.

• Signs and Symptoms
  • sudden weakness.
  • paleness (especially of lips, gums, nails).
  • belly pain.
  • enlargement (swelling) of the spleen.

• Special Care
  • Take your child to the emergency room immediately.
  • Immediate hospitalization and blood transfusion may be needed. 
  • You can feel the spleen normally in some children with sickle cell disease. It is important for you to know what is normal for your child.
  • Ask your doctor or nurse to teach you where the spleen is and how to feel for changes. See pictures on next page.
  
  Normal spleen and swelling spleen

Lung Problems

Pneumonia is a common lung infection in children with sickle cell anemia. If a child with sickle cell disease gets pneumonia, his/her lungs do not work as well. Trouble breathing and chest pain can result. The combined symptoms are called acute chest syndrome. Acute chest syndrome can be life-threatening.

• SIGNS AND SYMPTOMS
  • chest pain.
  • cough
  • shortness of breath.
  • fast, noisy, or difficult breathing.
  • fever.

• SPECIAL CARE
  • It is extremely important to report any of the above signs and symptoms to the child's doctor or nurse. The child with pneumonia/acute chest syndrome needs to be in the hospital. Intravenous (in the vein) antibiotics will be given to fight any infection that may be present. Transfusions may also be necessary in treating this problem.

Kidney Problems

All children with sickle cell disease pass urine frequently because their kidneys do not hold water well. Bed-wetting often happens and is not the fault of the child. Children with sickle cell disease also get kidney infections more often than other children.

• SIGNS AND SYMPTOMS
  
  • Pain when urinating.
  • Blood in the urine (hematuria).
  • Bed-wetting (when it has not happened before)
  • More frequent urination than usual.

• SPECIAL CARE
  • Make sure your child is getting enough fluid.
    
  • Bed-wetting may occur until your child realizes that he needs to get up at night to use the bathroom. Try to be supportive and understanding towards your child. It is sometimes worthwhile to wake your child up in the middle of the night to use the bathroom to prevent bed-wetting. IT IS IMPORTANT THAT YOU DON'T GIVE THE CHILD LESS TO DRINK DURING THE DAY TO TRY TO CORRECT THIS PROBLEM. THE CHILD WITH SICKLE CELL ANEMIA NEEDS EXTRA FLUIDS.
    
  • During the day, your child will also need to use the bathroom more often. This may become a problem when the child starts school. Have your doctor or nurse contact the school to give information about Sickle Cell Disease. Also, a letter giving permission for the child to use the bathroom as needed, and drink extra fluids during the school day may be helpful.
    
  • Infections should be treated with antibiotics.

Stroke

When there is a blockage of blood vessels supplying the brain, a stroke results. Stroke in sickle cell disease is uncommon but a stroke may be life-threatening.

• SIGNS AND SYMPTOMS
  • Problems talking.
  • Weakness of one side of the body.
  • Loss of, or blurred vision.
  • Fainting and dizziness.
  • Severe headache.
  • Coma, child won't wake up.

• SPECIAL CARE
  • Immediate hospitalization for observation, tests, and treatment. Treatment includes blood transfusion.
  • Sometimes, the child who has a stroke may return to normal within several days. However, there may be physical weakness or disabilities requiring special therapy.

Pain Crisis

Almost all children with sickle cell disease will have a sickle cell crisis at some point in their lives. Some children have them very often. A fever or infection, exposure to extreme heat or cold, physical exhaustion, and usual stress or anxiety may also trigger the pain. However, most often there is no obvious reason why a child gets a painful crisis

• SIGNS AND SYMPTOMS
  
  • Pain differs from one person to another and varies from mild to very severe. The pain can happen in any part of the body.
  • Infants and young children who can't say that they hurt may cry a lot and be irritable.
  • Swollen and painful hands and feet (The most common first type of crisis in an infant or young child).
  • Abdominal (belly) pain
    • Pain, swelling of the belly.
    • Problem breathing.
  • Fever of 101 or higher thermometer reading.

• SPECIAL CARE
  
  • If your child has abdominal pain, call your child's doctor to make sure the problem is not serious.
  • Speaking to your child's doctor is especially important if your child has not had a pain crisis before and you are not sure what to do.
  • Check for fever before giving Tylenol. If the child has a fever of 101 degrees or more, call the child's doctor right away.
  • During a painful crisis, it is important for the child to drink a lot of fluids. Children with sickle cell disease lose a lot of fluids because they have to urinate (pee) a lot. When too much water is lost, the child becomes dehydrated (loss of too much water). Dehydration can make sickle cell disease worse by causing the red blood cells to sickle faster.
  • Encourage the child to drink liquids like juice, fruit drinks and water.
  • Discourage the child from drinking fluids that contain caffeine, like soda, tea, and coffee.
  • Give pain medications as instructed by your doctor.
  • Bed rest.
  • If your child does not get better with medication or develops a fever or seems to be getting worse, call your doctor.

• Aseptic Necrosis
  When the blood vessels supplying bone get blocked by sickle cells, the bone can become damaged. This causes pain which will not tend to go away like a crisis. There is no infection (aseptic), but there is a gradual breakdown or destruction of bone (necrosis). This is called aseptic necrosis. This problem happens most commonly in the hip bone but may affect the knee and shoulder joints as well. Most children with sickle cell disease do not develop aseptic necrosis. There are no ways to prevent it from starting but rest and using crutches may delay the process from worsening over time.

• Eye Problems
  Children with sickle cell disease may develop eye problems which can occasionally lead to blindness. The most common eye problems happen very slowly and rarely occur before the age of ten years. Eye problems are more common in children with hemoglobin S-C disease.

  After ten years of age, children should be seen every one or two years by an eye doctor (an ophthalmologist) who is familiar with sickle cell disease. If your child has sudden problems with vision, call your child's doctor immediately!

• Priapism
  A prolonged unexpected erection of the penis is called priapism. This problem happens when sickle cells block the circulation of the penis. Young boys as well as teenagers can have this problem.

  If priapism lasts more than a couple of hours, notify the child's doctor. Adolescents may find it embarrassing, so they may not tell you. Talk to them and tell them this problem might happen. Tell them to let you know right away if it does. The child may have to be hospitalized.

• Skin Problems
  Some patients develop open skin sores (ulcers), especially around their ankles. If your child has an open cut which is not healing normally, notify your child's doctor. This problem does not happen often and it usually affects teenagers and young adults with sickle cell disease.

What is The Stop Study?

For the past few years, doctors have been using an ultrasound instrument, called a Transcranial Doppler, to measure blood flow in the vessels that supply the brain. The test is similar to the sonogram used to detect a fetal heartbeat or to look for gallstones.

These doctors have found that children with sickle cell anemia who have a high rate of blood flow in the brain, are at an increased risk for having a stroke. This is the first time that we have had a way of measuring the risk of a serious problem before it happens.

The equipment and the training needed to perform this study is only available at a few hospital centers throughout the country. As soon as it is available locally, your doctor will contact you and arrange for your child to have the study done.

The results of this study are very important. If your child is found to be "at risk", your hematologist (sickle cell doctor) will most likely start your child on a chronic transfusion program where he or she will receive monthly blood transfusions. This would be done to lower the amount of sickle hemoglobin in the blood and hopefully avoid a life-threatening stroke. Please ask your sickle cell doctor for more information about this very latest treatment of sickle cell disease.