Sickle-Cell, Part 2 — Treatment, side-effects, and restrictions
Treatment
A major component of treatment for sickle cell anemia is in prevention.
Antibiotics and vaccines are given to prevent frequent bacterial infections.
Supplementation with folic acid, which is essential to producing cells,
is important due to the rapid turnover in red blood cells. The retinas
of a person with sickle cell disease are at risk of damage, so eye examinations
by an ophthamologist are an important part of preventative treatment.
Control and management of symptoms is a major focus of treatment for
sickle cell anemia. Pain control is indicated during crises. Analgesics
and adequate liquid intake is the basis of treating painful episodes.
Non-narcotic medications are often effective, but some patients may require
narcotics. Additional treatments include:
- Antibiotics for infections
- Partial exchange transfusion for acute chest syndrome, prior to
surgery or for neurological events (such as stroke)
- Dialysis or kidney transplant for kidney disease
- Irrigation or surgery for priapism
- Surgery for eye problems
- Hip replacement for avascular necrosis of the hip
- Gallbladder removal
- Wound care
- Zinc oxide or surgery for leg ulcers
- Counseling for psychosocial complications
Hydroxyurea (Hydrea) has been effective in reducing the frequency of
painful crises, incidences of acute chest syndrome, or need for blood
transfusions in adults. Other new drug agents are being developed to manage
sickle cell anemia. Some of these attempt to induce the body to produce
more fetal hemoglobin, thus reducing the amount of sickling or increasing
the binding of oxygen to sickle cells. However, thus far there are no
other drugs available that have shown success in the treatment of sickle
cell anemia.
Sickle cell anemia is a chronic illness, and one that 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. Finding a “match”
for the person’s bone marrow type is extremely difficult. The chemotherapy
drugs are also very toxic and the process of receiving a bone marrow transplant
can be life-threatening. Bone marrow transplant is very expensive, and
not covered by most insurance companies as a treatment for sickle cell
anemia. Gene therapy, or replacing the Hemoglobin S with normal Hemoglobin
A, may be the best possible chance for a cure, but has been very difficult
to accomplish in humans.
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 have side-effects of lethargy, lack of coordination and difficulty
in concentration. Many treatment centers for sickle cell anemia have pain
management teams who will work with the patient to find a pain control
regimen that will result in minimal side effects.
Physical/dietary/other restrictions
Adequate hydration (fluid intake) is very important in the prevention
of sickle cell pain crises. In addition, exposure to sun may impair proper
hydration. There are no food restrictions for persons with sickle cell
anemia. A person with sickle cell anemia should eat a well-balanced diet
that is complete in necessary nutrients, vitamins and minerals. Exposure
to extreme temperatures (hot or cold) should be avoided, as should very
strenuous physical activity. Due to the difficulty of proper oxygenation
of the blood, a person with sickle cell anemia should avoid environments
with low oxygen content, such as high altitudes and non-pressurized airplane
flights. Emotional stress and known sources of infection should also be
avoided in an attempt to reduce the risk of sickle cell crises.
Sickle-Cell, Part 2 — Treatment, side-effects, and restrictions
For more information, please contact:
Kathy Davis, MSEd, PhD
kdavis2@kumc.edu
(913) 588-6305