Sickle Cell Anemia, Part 2 — Treatment, Side Effects, Restrictions, and Implications for School

Treatment

A major component of management for sickle cell anemia is prevention of its complications. The patient may receive antibiotics such as penicillin (Pen VK®) and routine vaccinations to prevent some of the most frequent bacterial infections. The patient with sickle cell anemia is at risk for eye damage; the retinas are especially vulnerable to damage from this disease. For this reason, regular eye examinations by an ophthalmologist are an important part of preventative treatment.

Control and management of symptoms is another major focus of treatment during sickle cell crises. Analgesics and adequate liquid intake are mainstays of treatment for these painful episodes. Non-narcotic medications are often effective for pain control, but some patients may require narcotics. Additional treatments may include:

Checking chest x-ray.
  • antibiotics for infections
  • partial exchange transfusion for acute chest syndrome, for severe neurological events (such as stroke) and prior to surgery
  • dialysis or kidney transplant for kidney disease
  • irrigation or surgery for priapism
  • surgery for eye problems
  • hip replacement for degenerative complications of the hip, known as avascular (ay-VAS'-kyoo-lur) necrosis
  • gallbladder removal
  • wound care
  • zinc oxide or surgery for leg ulcers
  • counseling for psychosocial complications

Hydroxyurea (Hydrea®) has been shown to effectively reduce the frequency of painful crises, the likelihood of acute chest syndrome, and the need for blood transfusions in both children and adults.

Some other medications, currently under development, attempt to induce the body to produce more fetal hemoglobin. Increased amounts of this normal hemoglobin may prevent the abnormal hemoglobin from sickling, and may increase the binding of oxygen to the red blood cells. However, none of these other drugs has been approved to treat sickle cell anemia.

Sickle cell anemia is a chronic illness, and is not considered to have a cure. However, bone marrow transplantation has been tried for a small number of patients. In this treatment, a patient's diseased bone marrow is destroyed by high doses of chemotherapy, and then replaced by another person's healthy bone marrow. It is extremely difficult to find a donor whose bone marrow will be a good "match" for the patient. Also, the chemotherapy drugs are very toxic and the entire process of receiving a bone marrow transplant can be life-threatening. Bone marrow transplant for sickle cell anemia is very expensive and is not covered by most insurance companies. Gene therapy, which replaces the gene for hemoglobin S with the normal gene for hemoglobin A, may be the best possible chance for a true cure; but this has been very difficult to accomplish in human patients.

Possible medication side effects

The medications used to treat sickle cell anemia have minimal side effects. Some antibiotics may cause stomach cramping, nausea or diarrhea. Narcotics may cause lethargy, lack of coordination and difficulty concentrating. Many treatment centers for sickle cell anemia have pain management teams who will work with the patient to find a treatment regimen that will control the pain yet create minimal side effects.

Physical, dietary and other restrictions

Adequate hydration (fluid intake) is very important in managing sickle cell anemia and preventing sickle cell pain crises. Exposure to strong sunlight can increase the body's water needs, and would require increased fluid intake.

There are no dietary restrictions for a person with sickle cell anemia. The person with sickle cell anemia should consume a well-balanced diet, complete with all necessary nutrients, vitamins and minerals. The doctor or nutritionist can provide guidance.

Any person with both sickle cell anemia and asthma should stringently follow all asthma medication regimens to avoid damage to the lungs due to complications from the sickle cell anemia such as acute chest syndrome .

Because the patient's red blood cells cannot carry as much oxygen as normal, and because they will tend to clump in a low-oxygen environment, any person with sickle cell anemia should avoid travel to very high altitudes, such as a non-pressurized airplane flight.

Implications for school

kids at school.

The management of sickle cell anemia at school has several facets. Educators must consider the physical, psychosocial, cognitive and educational issues of this diagnosis in order to ensure that the young person with sickle cell anemia is successful at school.

The physical aspects of sickle cell anemia must be addressed at school. A comprehensive evaluation should be conducted to see if the student would qualify as a student who is Other Health Impaired (OHI) under the Individuals with Disabilities Education Act (IDEA). The following accommodations and adaptations may be warranted for a child with sickle cell anemia:

  • Special bus transportation, in which the student can be picked up and dropped off at home, is an appropriate accommodation so the student would not need to wait for the school bus in very hot or very cold weather.
  • Provide rest time, when the child is lethargic at school.
  • Modify the physical education program to accommodate the limitations imposed by this diagnosis, such as limiting strenuous physical activity and avoiding activities that involve collisions (e.g., football and basketball).
  • Ensure proper hydration by allowing the student to have a water bottle available at the desk.
  • Provide frequent bathroom privileges.
  • Monitor the student closely for changes in physical status and cognitive abilities.
  • Allow student to visit nurse with any complaints of pain or other symptoms.

Psychosocial concerns for the student with sickle cell anemia may be significant. Due to frequent absences, pain crises and limitations on physical activity, a young person with sickle cell anemia will flow in and out of school, and may struggle with issues of "fitting in", feeling excluded by peers and keeping up socially. In order to encourage healthy psychological, emotional and social adjustment, the school should consider:

  • Educating peers, with the consent of the student and parents, about sickle cell anemia. The hospital school teacher is a good resource for offering a presentation, suggesting materials, or assisting with the education of educators, peers or other interested community members.
  • Encourage peers to stay in contact with their friend during absences. E-mails, text messages, phone calls, and even cards and letters can serve to bridge the gap when the student with a chronic illness must be away from school and activities.
  • Identify strong peers to mentor the student with sickle cell anemia upon returning to school. Having a friend to "fill in the gaps" on what was missed during an absence may make all the difference in providing a smooth re-entry.
  • Help the student find areas of expertise and ways to excel in the school environment. Mentoring support could be included in the IEP to help the young person develop or maintain a strong self-concept.
  • Consider including social work or counseling services as part of the IEP. Having someone at school that the student can talk to or who can act as an advocate for the youngster with sickle cell anemia can be a significant asset.
Academic Progress.

Finally, cognitive status and academic progress must be monitored continually for the young person with sickle cell anemia. Research has shown that students with sickle cell anemia may be victims of "silent strokes". These are small strokes that may remain clinically undetected, but which accumulate to cause significant cognitive decline. The student may simply have increasing difficulty in school, without realizing that there is a physical cause. In order to ensure that the student with sickle cell anemia continues to progress, educators should:

  • Evaluate for potential classification as OHI.
  • Create an IEP that allows for the possibility of ongoing change and fluctuation in ability and physical stamina.
  • Provide for a great deal of flexibility when the child has been absent. Give plenty of time to complete missed work.
  • Provide tutorial assistance if the student has been absent or is showing difficulty with specific learning material.
  • Provide careful monitoring to determine if student's performance declines.

KU Kids Healing Place

During treatment for sickle cell disease, there are many challenges for the child and family. In addition to the essential medical treatments, it is important to care for the psychological, social and spiritual needs of the patient and the family. This type of care, called palliative care, previously had been reserved for only the sickest patients. However, research has demonstrated that palliative care helps maintain normalcy throughout treatment, recovery and life. Today, every child with sickle cell disease receives the benefit of palliative care, along with the family members. The KU Kids Healing Place (KUKHP), through the Department of Pediatrics at the University of Kansas Medical Center, focuses on treating all aspects of the child and family, ensuring that each receives the care needed to maintain psychological, social and spiritual well-being during treatment for sickle cell disease and on into life. The entire treatment team will work to ensure that the needs of the child are recognized and addressed both in the hospital and in the community, including school, sports, worship and activities. KUKHP partners with school professionals, peers, clergy, coaches, scout leaders and many others to ensure that the child continues to grow and achieve in all environments.


For more information

Part 1 — Introduction, Incidence and Symptoms

The Sickle Cell Disease Association of America, Inc.

http://www.sicklecelldisease.org/

Sickle Cell Disease Association of America, Inc.
Uriel Owens Chapter, Kansas City, Kansas

http://www.sicklecellkck.org/

Sickle Cell Information Center

http://scinfo.org/

Contributed by:

Mukta Kumar, MD
Assistant Professor
Section Chief
Division of Pediatric Hematology and Oncology
Department of Pediatrics
University of Kansas Medical Center

Kathy Davis, MSEd, PhD
Associate Professor
Project Director, Connected Kansas Kids
Director, KU Kids Healing Place
University of Kansas Medical Center