Best Way to Deal With Sickle-Cell Anemia in Children

Many children who have sickle-cell anemia wet their beds. In 1995, T. Ernesto Figueroa and a group of researchers at the South Florida Health Sciences Center found that about 30 percent of the center’s 91 children who had sickle-cell disease had been wetting the bed since infancy. Sickle-cell anemia is an inherited disease of the red blood cells. For years people thought that the disease occurred only in people of black African descent, but we know now that it also occurs in other groups, including people from parts of Italy, Greece, India, Arabia, and else­where.

Sickle-cell anemia begins early in life. Symptoms in about half of its victims start showing between the ages of six months and two years. In others, symptoms start showing later.

What is this disease? In a person with sickle-cell anemia, the he­moglobin (oxygen-carrying substance in the red blood cells) is defec­tive; it does not get enough oxygen. This causes its molecules to com­bine with each other in a process called “polymerization.” The red blood cells lose their flexibility to the point of becoming rigid, and, instead of round, they become sickle-shaped. A round shape permits easy flow, but the sickle shape, together with the rigidity, causes the red blood cells to clump together and block the flow of blood in the small blood vessels. The tissues that are normally supplied by the blocked vessels are dam­aged or destroyed because they are cut off from the oxygen and nutrients normally carried by the bloodstream. The process causes weakness and a great deal of pain. Any child with sickle-cell anemia needs the care of a physician.

In virtually all cases, the disease destroys the ability of the kidneys to concentrate urine, and the output of urine becomes greater than nor­mal. The individual must awaken at night to urinate or wet the bed. (D. R- Readett reported that parents of sickle-cell children who bedwet consider these children more difficult to awaken than sickle-cell chil­dren who don’t bedwet.)

What symptoms alert you to the presence of this disease? Among the earliest is swelling of the fingers and toes, then thickening of the bones of the hands and feet. The symptoms of anemia are present: pale­ness, weakness, fatigue, fainting, shortness of breath, and palpitations of the heart (when the heart tries to compensate for the anemia by pumping blood faster than normal).

A child with sickle-cell anemia is small for his age, and several times a year endures “sickle-cell crises,” periods of severe pain in the abdomen, bones, joints, or muscles, accompanied by fever. These crises often require hospitalization.

Although there is still no cure, if your child suffers from this dis­ease, the pain and other related problems can be treated. Blood transfu­sions and various drugs are used. You may want to inquire about hydroxyurea, a substance that was reported by a number of researchers, including S. Charache at Johns Hopkins School of Medicine, to be helpful in reducing the frequency of sickle-cell crises.

You can ask your child’s physician to try giving the synthetic hor­mone desmopressin to control your child’s bedwetting. In 1995, T. Ernesto Figueroa and his associates reported in the Journal of Urology that they gave desmopressin to 10 children with sickle-cell anemia, and six of them experienced a complete or partial relief from bedwetting.

And you may wish to look into the possibility of bone marrow transplant. A. Ferster and a group of researchers in Belgium reported in the British Journal of Haematology on the curative effects of bone marrow transplants in five children who had severe forms of the disease. An­other researcher, Y. Beuzard, reported in 1992 in the French journal Revue du Practicien that bone marrow transplants had cured 30 patients.

There is still hope for a significant cure: At least one biotechnical company is working on the development of a gene therapy for sickle-cell anemia.

An additional note: If your child has sickle-cell anemia and day­time symptoms of urinary urgency in addition to nighttime frequency or bedwetting, then, besides other measures, you may want to ask the doctor to look for other possible urinary problems as well. Your child may have a urinary problem that is separate from the disease.