Adequate fluid intake: One of the factors triggering red blood cells to sickle is a high HbS concentration. The more concentrated they are, the closer the molecules are to each other. As soon as these molecules make contact, they polymerize.
When fluids enter the red blood cell, they dilute HbS concentrations, which lessens the likelihood of the molecules coming into contact with each other and polymerizing and, thus, causing a sickling crisis. That is one reason why sickle cell people need to drink generous amounts of fluid every day.
Another reason is to keep the blood less viscous or sticky, which helps it to flow. Since children with sickle cell disease are usually thirstier than their healthy peers, it is easy to encourage them to drink more fluids. At other times, they should be encouraged to drink more than they may want, especially when they are experiencing pain.
Pain suggests that the blood vessels are clogging and additional fluid might help the blood to flow again. Fever is another reason to encourage more fluid intake. Fever could be caused by blood vessel blockage, or it could be as a result of a developing infection. In either case, fever causes the body to lose water through the skin.
Other situations that require an increased fluid intake include diarrhea, hot weather, dry hot rooms, and strenuous activity. The following is a fluid intake guide:
- Children weighing up to 35 pounds should consume 1 cup of fluid for every 5 pounds of body weight each day.
- Larger children should consume 1 cup of fluid for every 6 pounds of body weight each day.
Blood transfusions are often an effective means in relieving painful episodes caused by a sickle cell disease crisis. Some doctors give whole blood transfusions, while others use packed red cells only. The use of only red-cell transfusions does well in keeping the blood hemoglobin as normal as possible.
The goal of a transfusion is to increase the number of red blood cells and to maintain the level of normal red blood cells. This keeps the number of sickled cells below the critical point that may cause a pain episode. However, transfusions are not recommended as a routine means of relieving painful episodes. Not only are transfusions inconvenient, but there are other dangers attached.
Although donor blood is carefully screened, there is always a tiny possibility that it might contain hepatitis or the AIDS virus. The recipient may also develop a reaction as a result of an imperfect match to all the proteins in the donor blood. Therefore, transfusions should only be used in such life-threatening situations as when surgery is needed.
People with sickle cell disease may require surgery to repair damage caused by blood-vessel blockage. Joint replacement and nose and throat surgeries to open blocked airways are just two of the possibilities.
Cold temperatures: Wintertime can be especially difficult for sickle cell sufferers. One of the ways the body normally copes with cold is by constricting the blood vessels to the skin, thereby cutting down the loss of body heat radiating from the blood flowing close to the surface. This restricted blood flow means less oxygen is reaching these tissues and sickled cells may plug these narrowed blood vessels, bringing on a pain episode. Therefore, it is important that those with sickle cell disease dress as warmly as possible when it is cold outside.
During a painful crisis, warm compresses to painful areas may be applied. Cold compresses should never be used as this aggravates the condition. Swimming in cold water and even drinking cold water will also produce the same aggravation.
Drugs and alcohol have been known to trigger painful episodes. Therefore, those with sickle cell disease should avoid them. When they are broken down in the body, drugs and alcohol produce an acidic condition, which tends to cause the red blood cells to sickle even more.
Infection: The most important guideline for those with sickle cell disease is to avoid infection. Since their immune systems are so weak and vulnerable, even a simple cold may bring on a pain episode. Pneumonia is one of the most common complications and is responsible for a large number of deaths, especially in children with sickle cell disease.
Low oxygen: Those with sickle cell disease should also stay away from places where oxygen is low, since oxygen deprivation can cause blood cells to sickle. Therefore, it is not a good idea for them to go mountain climbing or scuba diving. Flying in commercial airplanes is safe because they are completely pressurized, but flying in some military planes, light aircraft, or other planes can be dangerous since they may not be pressurized.
Nutritional supplements: Since the red blood cells have a much shorter lifespan in the sickle cell individual, a consistent and above-average supply of nutrients must be consumed in order to keep the blood well nourished. This, in turn, will keep the other organs from breaking down as readily and will provide an added barrier against invading microorganisms that will cause infections.
Vitamin E supplements: Doctors Danny Chiu and Bertram Lubin MD, of Oakland, California, think that sickle-cell anemia patients have a deficiency of vitamin E. This lack may contribute to the sickling process, in which red blood cells bend into distorted, crescent shapes. The scientists have found that – at least in the test tube – damage to the red blood cells can be prevented by vitamin E.
Dr. Chiu speculates that vitamin E’s antioxidant effect may alter the red cell membrane’s stability, making it less vulnerable to distortion. Still more promising research, from Hoffmann-La Roche Inc. and Columbia University, showed that when patients took 450 IU of vitamin E a day, the sickling process slowed. The proportion of their irreversibly sickled cells dropped from 25% to 11%.