Living With - Sickle Cell Disease - Living With

If you or your child has sickle cell disease, you should learn as much as you can about the disease. It is important that you receive follow-up care and know the warning signs of complications so you know when to get medical help. Your doctor will monitor your condition and may recommend adopting healthy lifestyle changes and taking steps to prevent and control complications of sickle cell disease.

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\n Receive routine follow-up care \n
- Sickle Cell Disease - Living With

Talk to your doctor about how often you should schedule follow-up visits. Most people who have sickle cell disease should see their doctor every 3 to 12 months, depending on their age. These visits will help to reduce the number of acute problems that need immediate care.

As part of your routine visits, your doctor may recommend the following vaccines to prevent infections.

Hepatitis. If you receive transfusions, your doctor will monitor you and give you vaccines for hepatitis.
Influenza. Get an influenza or flu shot every year at the start of flu season. This vaccination should begin at 6 months of age. Only the inactivated vaccine, which comes as a shot, should be used in people who have sickle cell disease.
Meningococcus. A child who has sickle cell disease should receive a meningococcal vaccine at 2, 4, 6, and 12 to 15 months of age. The child should receive a booster vaccine 3 years after this series of shots, then every 5 years after that.
Pneumococcus. Even though all children routinely receive the vaccine against pneumococcus (PCV13), your child\u2019s doctor may recommend a second kind of vaccine against pneumococcus (PPSV23). This second vaccine is given after 24 months of age and again 5 years later. Adults who have sickle cell disease and have not received any pneumococcal vaccine should get a dose of the PCV13 vaccine. They should later receive the PPSV23 if they have not already received it or if it has been more than 5 years since they did. Follow these guidelines even if you or your child is still taking penicillin.

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\n Learn about donating blood \n
- Sickle Cell Disease - Living With

People who have sickle cell disease cannot donate blood. If you have sickle cell trait, you may donate platelets, but you should not donate whole blood, as it will interfere with later steps involved in handling the donated blood.

Talk to your doctor to learn more about donating blood.

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\n Monitor your condition \n
- Sickle Cell Disease - Living With

To monitor your or your child\u2019s condition, your doctor may recommend the following tests.

Screening for iron overload. Your doctor will treat iron overload with chelation therapy, a medicine to reduce the amount of iron in the body and the problems that iron overload causes.
Height and weight to be sure that a child is growing properly and maintaining a healthy weight.
Oxygen saturation tests to measure how much oxygen the blood is carrying.
Blood tests to check for other health problems, such as anemia or organ damage.
Urine testing to detect early kidney problems or infections.
Transcranial Doppler (TCD) ultrasound screening to find out whether a child is at higher risk for stroke. When the test results are abnormal, regular blood transfusions can reduce the chances of having a stroke. This screening is recommended every year for children between the ages of 2 and 16 who have hemoglobin SS or hemoglobin S?0 thalassemia.
Eye examinations to detect sickle cell disease\u2013related problems of the eye. Eye exams every 1 to 2 years can help doctors find and treat problems early to prevent loss of vision. A patient should see his or her doctor right away for any sudden change in vision.
Cognitive screening to identify problems early that may be hard to notice early in life. Sometimes these problems are caused by silent strokes that can be seen only with magnetic resonance imaging (MRI) of the brain. Tell your doctor if you have difficulties learning in school, making decisions, or organizing your thoughts.

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\n Adopt a healthy lifestyle \n
- Sickle Cell Disease - Living With

Like all people, you and your child should strive to maintain a healthy lifestyle that includes:

Being physically active. People who have sickle cell disease often tire easily, so be careful to pace yourself and avoid very strenuous activities.
Getting enough sleep
Heart-healthy eating, including limiting alcohol. Drink extra water to avoid dehydration.
Quitting smoking. Visit Smoking and Your Heart and the National Heart, Lung, and Blood Institute\u2019s Your Guide to a Healthy Heart. Although these resources focus on heart health, they include basic information about how to quit smoking. For free help and support to quit smoking, you can call the National Cancer Institute\u2019s Smoking Quitline at 1-877-44U-QUIT (1-877-448-7848).

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\n Learn ways to manage pain \n
- Sickle Cell Disease - Living With

Every patient experiences pain differently. Work with your doctor to develop a pain management plan that works for you. This often includes over-the-counter medicines, as well as stronger medicines that you get with a prescription.

Your doctor may recommend these steps to manage acute pain.

Talk to your doctor about taking hydroxyurea. When taken daily, hydroxyurea has been found to decrease the number and severity of pain episodes.
When an acute crisis is just starting, drink lots of fluids and take a nonsteroidal anti-inflammatory (NSAID) pain medicine, such as ibuprofen. If you have kidney problems, acetaminophen is often preferred. If pain persists, you may need a stronger medicine.
Try alternative ways to manage pain, such as massage, relaxation methods, acupuncture, or a heating pad.
If you cannot control the pain at home, go to a sickle cell disease day hospital/outpatient unit or an emergency room to receive additional, stronger medicines and intravenous (IV) fluids. You may be able to return home once your pain is under better control. In this case, the doctor may prescribe additional pain medicines for a short course of therapy. You may need to be admitted to the hospital to fully control an acute pain crisis.

Your doctor may recommend these steps to manage chronic pain.

Take pain medicines, such as ibuprofen, daily to help to reduce the pain.
Try massage, heat, or acupuncture.
Seek help if you have feelings of depression or anxiety. Supportive counseling and, sometimes, antidepressant medicines may help.

Some patients taking L-glutamine oral powder (Endari) may have fewer hospital visits or hospitalizations due to severe pain than patients who are not taking this medicine. They may also have shorter stays when they are hospitalized. More research is needed to understand how effective L-glutamine oral powder is as a treatment and which patients may benefit from using it.

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\n Prevent and treat complications over your lifetime \n
- Sickle Cell Disease - Living With

To prevent and treat complications over your lifetime, your doctor may recommend the following.

Avoid overexertion and dehydration. Take time out to rest and drink plenty of fluids.
Avoid situations that may set off a crisis. Extreme heat or cold, as well as abrupt changes in temperature, are often triggers. When going swimming, ease into the water rather than jumping right in.
Do not travel in an aircraft cabin that is unpressurized.
If you experience priapism, you may be able to relieve your symptoms by doing light exercise, emptying your bladder by urinating, drinking more fluids, and taking medicine recommended by your doctor.
If your child attends daycare, preschool, or school, speak to his or her teacher about the disease. Teachers need to know what to watch for and how to accommodate your child.
Learn how to palpate, or feel, your child\u2019s spleen. Because of the risk of splenic sequestration crisis, caretakers should learn how to palpate a child\u2019s spleen. They should try to feel for the spleen daily and more often when the child is ill. If the spleen feels larger than usual, they should call the care provider.
Take your medicines as your doctor prescribes. Get any medical and lab tests or immunizations that your doctor orders.

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\n Learn the warning signs of serious complications and have a plan \n
- Sickle Cell Disease - Living With

Sickle cell disease can lead to serious and life-threatening complications. If you think you or someone else is having any of the following symptoms or complications, seek medical care or call 9-1-1 immediately.

Severe anemia

See your doctor or go to a hospital right away if you develop anemia symptoms from a splenic sequestration crisis or an aplastic crisis. These conditions can be life-threatening.

Some patients may have fewer hospital visits due to sickle cell crises, including splenic sequestration, if they are taking L-glutamine oral powder, than patients who are not taking this medicine do. More research is needed to understand how effective L-glutamine oral powder is as a treatment and which patients may benefit from using it.

Fever

Having a fever when you have sickle cell disease is considered a medical emergency. All caretakers of newborns and children who have sickle cell disease should take the child to their doctor or go to a hospital right away when their child has a fever.

All children and adults who have sickle cell disease and a fever of more than 101.3 degrees Fahrenheit or 38.5 degrees Celsius must be seen by a doctor and treated with antibiotics right away. Some people will need to be hospitalized.

Acute chest syndrome

If you have symptoms of acute chest syndrome, such as chest pain, coughing, fever, and shortness of breath, see your doctor or go to a hospital right away. You will need to be admitted to the hospital, where you may receive antibiotics, oxygen therapy, or a blood transfusion.

Some patients may have fewer hospital visits due to sickle cell crises, including acute chest syndrome, if they are taking L-glutamine oral powder, than patients who are not taking this medicine do. More research is needed to understand how effective L-glutamine oral powder is as a treatment and which patients may benefit from using it.

Stroke

If you think someone may be having a stroke, act F.A.S.T.

F\u2014Face: Ask the person to smile. Does one side of the face droop?

A\u2014Arms: Ask the person to raise both arms. Does one arm drift downward?

S\u2014Speech: Ask the person to repeat a simple phrase. Is their speech slurred or strange?

T\u2014Time: If you observe any of these signs, call for help immediately. Early treatment is essential.

Read more about the signs and symptoms of a stroke.

If imaging studies reveal an acute stroke, you may need an exchange transfusion. This procedure involves slowly removing a certain amount of your blood and replacing it with blood from a donor who does not have sickle cell disease or sickle cell trait. Afterward, you may need to receive monthly transfusions or other treatments to help to prevent another stroke.

Priapism

If you experience priapism that lasts for 4 hours or more, go to the hospital to see a hematologist and urologist.

Some patients may have fewer hospital visits due to sickle cell crises, including priapism, if they are taking L-glutamine oral powder, compared with patients who are not taking this medicine. More research is needed to understand how effective L-glutamine oral powder is as a treatment and which patients may benefit from using it.

Return to Signs, Symptoms, and Complications to review possible complications from sickle cell disease.

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\n Transition to adult care \n
- Sickle Cell Disease - Living With

The move from pediatric care to adult care is an important step in treatment. Talk with your teen\u2019s healthcare team about creating a plan to help your teen transition to adult care. This period has been shown to be associated with greater numbers of hospital admissions and medical problems. There seem to be many reasons for this.

Some of the increased risk is directly related to the disease. As people who have sickle cell disease get older, they often develop more organ damage and more disabilities.
The shift in care usually occurs at the same time that adolescents are undergoing many changes in their emotional, social, and academic lives. The transition to more independent self-management may be difficult, and following treatment plans may become less likely.
Compared with pediatrics, there are often fewer adult sickle cell disease programs available in a given region. This makes it more difficult for a person who has sickle cell disease to find appropriate doctors, particularly doctors with whom they feel comfortable.

To improve use of regular medical care by people who have sickle cell disease and to reduce age-related complications, many sickle cell disease teams have developed special programs that make the transition easier. Such programs should involve the pediatric and adult care teams.

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\n Pregnancy \n
- Sickle Cell Disease - Living With

Pregnant women who have sickle cell disease are at greater risk for problems. If you are pregnant or are planning to become pregnant, see an obstetrician (a doctor who specializes in pregnancy and childbirth) who has experience with patients who have sickle cell disease and with high-risk pregnancies and deliveries.

Pregnant women who have sickle cell disease need more frequent medical visits. Your doctor may prescribe certain vitamins and will be careful to prescribe pain medicines that are safe for your baby.

You may need to have one or more blood transfusions during pregnancy to treat complications, such as worsening anemia or an increased number of pain or acute chest syndrome events.

You should not use hydroxyurea during pregnancy.

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\n Take care of your mental health \n
- Sickle Cell Disease - Living With

Living with sickle cell disease can be very stressful. At times, you may feel sad or depressed. Talking about how you feel with your or your child\u2019s healthcare team may help. Your doctor may recommend:

Medicines or other treatments, such as antidepressants, or other treatments that can improve your quality of life
Joining a patient support group, which may help you or your child adjust to living with sickle cell disease. You can see how other patients manage similar symptoms and their condition. Talk with your doctor about local support groups or check with an area medical center.
Support from family and friends, which can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

Living With - Sickle Cell Disease - Living With

\n \n Receive routine follow-up care \n - Sickle Cell Disease - Living With

\n \n Learn about donating blood \n - Sickle Cell Disease - Living With

\n \n Monitor your condition \n - Sickle Cell Disease - Living With

\n \n Adopt a healthy lifestyle \n - Sickle Cell Disease - Living With

\n \n Learn ways to manage pain \n - Sickle Cell Disease - Living With

\n \n Prevent and treat complications over your lifetime \n - Sickle Cell Disease - Living With

\n \n Learn the warning signs of serious complications and have a plan \n - Sickle Cell Disease - Living With

\n \n Transition to adult care \n - Sickle Cell Disease - Living With

\n \n Pregnancy \n - Sickle Cell Disease - Living With

\n \n Take care of your mental health \n - Sickle Cell Disease - Living With