Dealing with the Everyday

Pain is a part of everyday life with sickle cell anemia. There is no cure for the pain, but pain-relieving medicines can be taken and easy steps can be followed to ease the pain. Staying warm through blankets, hot water bottles, and massaging can help increase blood flow, increasing the amount of oxygen to the tissues (Sickle Cell Society 2005). Resting and deep breathing help both physiologically and psychologically to relax and distract the sufferer (Sickle Cell Society 2005).

People with sickle cell anemia should stop exercise if feeling overworked and should stay well-hydrated (Sickle Cell Society 2005). When sickled cells are dehydrated, the concentration of hemoglobin S increases within the cell, increasing the likelihood and speed with which the HbS molecules polymerize (Brugnara 2000). Cell dehydration is a result of membrane damage due to both waves of polymerization/depolymerization and the presence of reactive oxygen species. The loss of sodium through the damaged membrane then causes potassium, and thus water, to be lost from the cell (Brugnara 2000). The build-up of calcium in these cells then activates a potassium transport, causing more potassium and water to leave the cell (Bridges 2002).

In hydrated cells HbS is less concentrated, so it is less likely to polymerize and the cell is less likely to sickle. In dehydrated cells HbS is more likely to form chains due to an overall higher concentration.

Sickle cell anemia makes one especially susceptible to infections, so antibiotics like penicillin should be taken everyday as a preventative measure (Sickle Cell Society 2005). This increased susceptibility is often a result of damage to one of our major defenses against infection, the spleen, due to restricted blood flow (Bloom 1995). Folic acid, which aids in the formation of healthy blood cells, should be taken daily in large quantities as well (Guyton 1991).

Some less conventional everyday treatments, like massage therapy, have also proven to be effective in reducing pain and the frequency of pain crises. Massage not only warms the body, increasing blood flow and oxygen to the tissues, but it also helps “aid efficient blood flow” (Sickle Cell Society 2005). The relaxing physiological effects of massage therapy can also initiate psychological benefits to the patient (Sickle Cell Society 2005).

Hydroxyurea

The drug hydroxyurea is a cytotoxin often used in treating leukemia. It stops mitosis in the S-phase by inhibiting the enzyme ribonucleotide reductase (Charache et al. 1995). Major side effects of the drug include lower numbers of white blood cells and lower platelet counts, increasing the risk of infection (Bridges 2002). Hydroxyurea, however, has been found to be the safest drug of its kind. There is still debate as to whether hydroxyurea causes leukemia, but no conclusive data has demonstrated that is does.

The chemical structure of hydroxyurea.

In 1995 a large collaborative study was published concerning the effects of hydroxyurea on the occurrence of pain crises (Charache et al.1995). Patients with sickle cell anemia were studied from 21 different sites in the United States and Canada and were given either placebos or hydroxyurea doses based on body mass. The effects of hydroxyurea were so beneficial that the study was terminated early and all patients were given hydroxyurea. Higher doses resulted in larger increases in fetal hemoglobin concentrations; as little as a four percent increase in HbF concentrations was needed to help prevent some sickling of cells. Treatment with hydroxyurea reduced the frequency of pain crises as early as two months into the study; patients treated with hydroxyurea also suffered fewer instances of chest syndrome and required fewer blood transfusions.

The percent of cells containing hemoglobin F in patients treated with hydroxyurea increased to almost 80 percent compared to patients treated with placebos in the collaborative study. Increased F cell concentrations likely caused fewer cells to sickle and fewer pain crises to occur in patients treated with hydroxyurea. Figure adapted from Charache, et al. 1995.

As mentioned in Hemoglobin F, hydroxyurea is effective in treating sickle cell anemia through increased hemoglobin F levels in cells. The exact method of inducing hemoglobin F production is still unknown. Hydroxyurea can improve the quality of life of many patients with sickle cell anemia, by increasing hemoglobin F levels and red blood cell concentrations (Sickle Cell Society 2005).

More Intrusive Treatments

Blood transfusions are often performed if both adult and child sickle cell patients are experiencing severe pain crises. But blood transfusions can also be used as a preventative treatment. In the STOP study of 1998, children given monthly blood transfusions, in which their hemoglobin S concentrations were reduced to less than thirty percent, had much lower occurrences of strokes related to sickle cell anemia (Adams 1998).

Although a blood transfusion is an effective treatment, there are also risks and barriers to performing this treatment as frequently as needed. The most significant problem is having enough of the right type of blood to perform the procedure as necessary. The transfusion is also a temporary treatment, as the concentrations of hemoglobin S will gradually build up again, replacing the transfused normal hemoglobin. Risks associated with any blood transfusion can also be high, possibly causing blood clots, iron overload, and blood-born infections (Sickle Cell Society 2005).

Bone marrow transplants are the closest thing we have to a cure for sickle cell anemia. By replacing the patient’s bone marrow cells with cells containing genes for non-sickle cell hemoglobin, the sickling of new cells can be prevented. The obstacle here is, of course, finding enough donors for such an invasive and painful procedure, as well as finding specific donor/patient matches. If the transplant is performed while the patient is young (before organ damage has occurred from sickled cells) and a proper donor is found, then success rates can be as high as 90 to 95 percent (Sickle Cell Society 2005).