Counseling Sheet

Agatha M. Thrash, M.D.
Preventive Medicine

Red blood cells are usually round discs that are thin in the middle. A person who has sickle cell anemia will produce a certain percentage of the cells in a crescent shape, or some other abnormal shape. The reason the cells are distorted is that the hemoglobin molecules are structurally abnormal producing an elongated shape and pressing the red blood cell capsule into a curve or sickle shape.

Two and a half million Black Americans have sickle cell disease of one form or another. That is almost one of every 10 Blacks in America who have the disease. Occasionally a Puerto Rican or person of Mediterranean origin may develop sickle cell trait or disease.

The disorder is genetically determined and occurs in two forms: the "sickle cell trait" and the "sickle cell disease." The "disease" is severe, disabling, and chronic, manifested by bouts of acute episodes called "crises," and by chronic breaking down of the red blood cells. The crises are produced by plugging of small blood vessels by the crescent shaped red blood cells. When hemoglobin forms an elongated shape it is of the so-called hemoglobin S variety rather than hemoglobin A which most adults have. It is of interest that there are other forms of hemoglobin in addition to S and A; hemoglobin F is produced in the unborn baby and during the first year of life. It is a normal type of hemoglobin only during that period and produces disease when it appears at other times of life. Hemoglobin C and other abnormal hemoglobins may also produce anemia; they are less common than hemoglobin S.

The diagnosis of sickle cell anemia or trait is made by determining the amount of hemoglobin in the blood, and by a simple test to reduce oxygen in the blood which increases the amount of sickling. If all things are ideal, even a heavy sickler may show no sickling at times. When the environment deteriorates even slightly, sickling begins. If only a few red blood cells form the crescent shape and the hemoglobin is not very low the disorder is spoken of as sickle cell trait, but if the sickled red blood cells are numerous and the hemoglobin is quite low, the disorder is spoken of as sickle cell disease. When the crises occur, any area of the body can be involved, including bones, hands or feet, spleen, lungs, brain, or gastrointestinal tract.

How can one treat genetic disorders such as sickle cell disease? The disease should be studied and every attempt made to minimize the load and correct deficiencies of the body. Recent reports are indicating that one of the best ways is to consume a low salt diet. In this way the sickling molecules in the red blood cells can be redistributed, as with low sodium the red blood cells may swell slightly with water, dispersing the hemoglobin molecules better and preventing the abnormal shape. From infancy the sickler should be taught to enjoy food completely without salt. The family can join in this crusade as it will not harm the relatives of a person with sickling to be totally salt-free. Read labels, allow no salt, and buy no junk foods. All dairy products are quite high in salt, and should be avoided.

Sickle cell patients should drink large amounts of water and should eat much raw food with its extra amount of water. They should not use concentrated foods as these tend to dehydrate the red blood cells and make the blood more viscid or thick and heavy. Concentrated foods include all animal products - meat, milk, eggs, and cheese - also all other foods that have a high calorie concentration per unit volume of food - nuts, wheat germ, oil, margarine, mayonnaise, fried foods, free sugars, and most refined foods.

Regular exercise is good for sickle cell patients, as the appetite for food is better controlled and the appetite for water is promoted. Exercise causes oxygen to perfuse all tissues better, including red blood cells. While it should not be overdone, exercise is an important adjunct to any treatment routine for sickle cell anemia.

All other diseases should be fought earnestly, as an infection or a degenerative disease can precipitate a sickle cell crisis. All over-the-counter drugs should be avoided, but particularly those that cause contraction of blood vessels or an acid condition. That would include all the opiates, LSD, and other hard drugs. Since sickle cell patients are already suffering under the handicap of a chronic disease, even prescription drugs should be avoided as completely as possible to protect the patient from the extra burden that any drug can give. For pain, the sickle cell patient should be treated not with pharmaceuticals, but with hot fomentations, ice packs, poultices, heating pads, hot foot baths, massage, passive and active exercise, and other physical therapeutic measures rather than with drugs. If jaundice develops the patient should be treated with charcoal tablets by mouth, 8 tablets in the middle of the morning and in the middle of the afternoon to reduce the amount of bile being reabsorbed into the bloodstream from the gastrointestinal tract. Splenic infarcts are a frequent source of abdominal pain in the child. These may be treated with alternating hot and cold packs to the left upper abdominal quadrant, by charcoal poultices at night, and by heating compresses.

Pulmonary signs of coughing, chest pain, and shortness of breath may be due to pulmonary infarcts and can be treated with moderately hot chest packs maintained for 20 to 50 minutes, ending with a brief, mildly cold application, and followed by a heating compress to the chest or a charcoal compress. A heat lamp to the chest can often be helpful, as can a heating pad.

Bone pain should be treated with massage, hot or cold compresses, and by passive or active exercise. It should be remembered that fresh air is a must in the treatment of sickle cell disease. When red blood cells lose their oxygen they are much more susceptible to sickling.

The diet should be well-balanced and chiefly of fruits, vegetables, and whole grains. All other food items should be taken sparingly or omitted, as the influence on the water content of the red blood cells and the capsule must be carefully guarded by preventing increased osmolarity of the plasma. Heavy and concentrated foods give heavy and concentrated plasma with loss of fluid from the red blood cells to the plasma. Since sickled cells themselves make the blood more viscous, nothing should be done that will promote heavier plasma with dissolved sugars, drugs, lipoproteins, and dispersed fats. Only whole grains should be used, and a high fiber diet should be aimed for. Whole grains and legumes contain vitamin E which seems to benefit sickle cell patients. A diet high in folic acid should be taken, which means plenty of greens and other vegetables. Rest should be abundant, particularly in children. Adults need around 8 hours of sleep and some may require a bit more. A half-hour nap taken just before dinner can be very good. Since a rhythmical pattern has been noted in the development of sickle cell crises, a Herculean attempt should be make to keep the patient on a regular schedule 365 days in the year. Watch out for irregular mealtimes, bedtimes, arising times, exercise times, and elimination times. Exercise should be described as vigorous though not violent.

A person with sickle cell disease should stay away from alcohol entirely. Red blood cells do poorly when exposed to alcohol. They tend to cluster together and impede flow into capillaries. Avoid getting chilled as the slowed circulation can reduce the oxygen content of the red blood cells, and the low tissue oxygenation can cause a crisis.

The person with sickle cell disease should avoid areas of decreased oxygen tension. That would include hiking or camping at high altitudes and flying in unpressurized airplanes. If at all possible one should avoid surgery because of the stress of anesthesia. Dentists should not use nitrous oxide or any other form of general anesthesia. A warm climate at or near sea level represents the best geographical location for the patient with sickle cell disease, and a rural setting is by far the most conducive to health.