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Sickle Cell Disease

Sickle Cell Disease affects approximately 1 in 625 African American newborns in the United States. The sickle cell trait is present in 8 percent of African Americans. Newborn screening for the sickle cell gene is performed on all infants born in the state of Michigan. The results are given to you and your primary care physician usually by the time the infant is 2 weeks old. If both parents carry the sickle cell trait (each have one sickle S gene), there will be a 1 in 4 (25 percent) chance of having a child with sickle cell disease (the affected child has two sickle S genes).

Note: There are other types of sickle cell disease that are less common, including sickle cell-Hemoglobin C disease and sickle cell-beta thalassemia disease. Sickle Cell-hemoglobin SC disease, commonly known as Sickle "SC" disease, is a milder form of sickle cell disease in which there is inheritance of 1 sickle S gene and 1 hemoglobin C gene. Sickle SC Disease represents 25 percent of all the sickle cell disease cases in the United States.

Sickle Cell Pain

Sickle cell disease is one of the most common inherited diseases worldwide, and pain is the most important symptom of the disease. The pain is often described as deep, gnawing and throbbing. The skin may be tender, red and warm in the painful areas. Kids with sickle cell disease may experience a wide variety of pain ranging from mild to severe, starting as early as six months old and continuing throughout their lives.

The pain of sickle cell anemia is very frustrating. You never know when it will strike and it is often difficult to get under control.

Avoid Triggering a Pain Crisis

Most pain episodes do not have an obvious reason for their occurrence. But here are some possible causes and things that your child should avoid if possible:

Smoking and Sickle Cell Pain

Research shows that kids with sickle cell disease who are exposed to environmental tobacco smoke (ETS) at home have more than twice as many pain episodes (pain crises) as sickle cell patients who were not exposed. Sickle cell crises involve serious symptoms and often require hospitalization. Researchers in one study estimate that ETS exposure increases the risk of pain crisis by 90 percent among children with sickle cell disease. If you smoke, either quit or don’t smoke around your child.

When to See a Physician for Sickle Cell Pain

If your child has sickle cell disease, is under two years old, and has pain or fever, they should see a doctor right away. Other serious warning signs that signal a need for immediate medical treatment are:

Recognizing Sickle Cell Pain

Everyone can feel pain, even babies and young children. Sometimes children have a hard time expressing themselves and may find it hard to tell you where it hurts and what it feels like.

For this reason, doctors and nurses are using new tools to help define pain in the kids they care for. Pain charts and scales for children use pictures or numbers to describe their pain. Describing the pain can help parents, doctors and nurses understand how bad the pain is, and how to best treat it. Talking to your child’s doctors and nurses about pain is important. The more they know about your child’s pain, the more they can help. Pay attention to how your child acts. For example, when your child is in pain, he or she may be restless or unable to sleep.

The first step in treating your child’s pain is to tell your child’s doctor or nurse about it. Your health care provider will ask several questions about the pain, including where it hurts, what it feels like and how it has changed since it started.

Your child’s doctor may ask you to keep a pain diary with your child, which keeps track of when your child has pain throughout the day. This diary can also document how the pain changes after taking pain medications. If medications do not seem to work, or if your child has a bad reaction, tell the doctor and keep a list of these problem medicines for future reference.

Treating Pain at Home

Many pain crises can be managed at home. Your child will need to increase their fluid intake and should use the pain pills prescribed by his or her doctor. If you can’t take care of the pain at home with these measures, or if the warning signs listed above occur, then get medical care right away.

In addition to drinking lots of water and taking pain pills, there are many other ways to help your child fight the pain of sickle cell disease. As with other kinds of pain, it’s best to use many different approaches at once to get the best pain relief. Biofeedback, cognitive strategies, self-hypnosis and progressive relaxation all show great promise as useful treatments to add to the usual sickle cell pain treatment.

Treating Pain at the Hospital

If treating the pain at home didn’t work, you would bring your child to the hospital. At the hospital, your child will probably receive treatment similar to this:

Challenges of Pain Treatment in the ER or Hospital
A fear of addiction, by patients, families and health care workers can sometimes interfere with appropriate treatment of pain. However, there are detailed treatment guidelines that outline the right way to treat sickle cell pain.

Meperidine (Demerol)
Meperidine (Demerol) has been a controversial medicine. Meperidine should not be taken by mouth for chronic or acute pain in sickle cell disease. Intravenous (IV) meperidine should not be used as a first-line medication for sickle cell pain because it can be toxic to the nervous system at higher doses. However, if your child has a morphine or hydromorphone allergy, or when they have improved in the past using meperidine briefly, meperidine may be a good choice.

Hydroxyurea
The medicine hydroxyurea has been used for more than 10 years to treat sickle cell disease in adults. It is also approved for use in children over the age of 3.

Impact of Sickle Cell Pain

Sickle cell disease is a hard condition to cope with. Controlling the pain can be very frustrating. Because there are no outward signs of the pain, sometimes people don’t believe a child is suffering as much as they really are. In addition to the pain, there are many ways kids with sickle cell have to change the way they live. For example, they may not be able to go swimming with their friends if the pool is too cold, and frequent hospital stays interrupt their lives. These kinds of restrictions can really cramp a kid’s style.

You may need to work with your child’s school to be sure your child is getting the help and support needed. With proper support in school, kids with sickle cell pain can participate in most activities. Find out how to work with the school system to get the help in school that your child needs. This information includes tips for parents, school nurses and teachers.

Sickle cell disease, like any chronic condition, challenges kids and their families in all kinds of ways. When they find helpful coping strategies, kids and their families can rise to the occasion.

Sickle Cell Pain and Coping Strategies

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Alternative Treatment Options

Hydroxyurea
Hydroxyurea, a cancer drug used to treat certain types of blood cancers, has been found to benefit some patients with sickle cell disease without significant side effects. Hydroxyurea helps sickle cell patients by increasing fetal hemoglobin (Hbg F). Babies are born with high fetal hemoglobin, which protects babies with sickle cell disease from having pain crisis during the first few months (less than six months) of life. Fetal hemoglobin has “anti-sickling effects” resulting in fewer pain crises. Previous trials in adults and recently in children have shown that hydroxyurea can significantly decrease the complications of sickle cell disease including decreased pain crises, decreased acute chest syndrome episodes and overall improved lifestyle. The long-term side effects have yet to be determined conclusively.

Currently, hydroxyurea is given to patients older than 3 years old who suffer form more severe complications of sickle cell disease, including frequent pain crises and a history of acute chest syndrome. We evaluate every sickle cell disease patient individually and determine if they are eligible for the use of hydroxyurea. Please contact us for any questions regarding the use of hydroxyurea in sickle cell disease for your child or patient.

Bone Marrow Transplantation
Bone marrow transplantation has been used here at the University of Michigan and nationally to cure patients with sickle cell disease or thalassemia. Bone marrow transplantation is usually reserved for patients with more severe sickle cell disease including frequent pain crises (usually requiring medical attention), multiple acute chest syndromes, pulmonary hypertension and history of stroke.

Once a patient is deemed eligible for bone marrow transplantation for sickle cell disease, bone marrow typing (a simple blood test) studies are performed on the patient’s family (including siblings) to determine if there is a “bone marrow match.” Please contact us or visit the U-M Pediatric Blood and Marrow Transplant Program’s Web site for any questions regarding the use of bone marrow transplantation in sickle cell disease.

Sibling Matched Bone Marrow Transplantation: The University of Michigan Pediatric Blood and Marrow Transplant Program has successfully transplanted and cured patients with sickle cell disease from sibling bone marrow donors.

U-M Joins New Multicenter Unrelated Bone Marrow Transplantation Study for Sickle Cell Disease in Children: The Sickle Cell Unrelated Transplant (SCURT) research study is a Phase II, multi-site clinical trial that is accepting qualified patients. This first-of-its-kind clinical trial may provide new treatment options to children with sickle cell disease, and the University of Michigan Health System is one of the primary institutions participating in the trial.

Marrow or blood stem cell transplantation currently is the only potential cure for severe sickle cell disease. Most people with sickle cell disease who have undergone a transplant have received stem cells from family members, usually siblings. In this nationwide, multi-center clinical trial, researchers will evaluate the role of unrelated donor transplants in treating severe sickle cell disease, and the effectiveness of a less-intensive regimen of chemotherapy to prepare patients for transplant for more information and continued reading about this new study, follow this link or contact us.

Chelation Therapy
To help remove excess iron, patients undergo "iron chelation therapy," in which a drug is introduced into the body; the drug binds with excess iron and removes it through the urine or stool. For many years, the only FDA-approved iron chelator was Desferal, which has to be administered through a painful and difficult infusion process. When using Desferal, a needle is attached to a small battery-operated infusion pump and worn under the skin of the stomach or legs five to seven times a week for up to twelve hours.

In November 2005, the FDA approved an oral chelator, Exjade. This is a pill that is dissolved in water or juice and drunk once a day. Many patients now have an option in terms of chelators, and it is hoped that more options will become available in the coming years.

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