Help for Slow-Growing Children

By Vogel, Marta | FDA Consumer, March 1987 | Go to article overview

Help for Slow-Growing Children


Vogel, Marta, FDA Consumer


Help for Slow-Growing Children

"I guess it was may motherly instinct that told me somethingwas wrong,' Chicago homemaker Mary Andrews says of her son Deno's growth problem. "Up until 2 1/2 he grew normally. But after that my sisters and in-laws would give me hand-me-downs [from their same-age children] and they would still fit him. I noticed that all the other kids were much taller.'

When Deno was 4 1/2 and still not growing as fast as his playmates, his mother decided to see if the problem might be due to some medical condition.

The diagnosis on Deno was not encouraging. Doctors toldAndrews that her child had a deficiency of human growth hormone (hGH)--his pituitary gland did not release enough of the protein that made him grow. They predicted that he would never reach more than 4 feet high.

"I was totally depressed,' says Andrews of that diagnosis10 years ago. "I had never heard of anything like this.' At the time, the treatment for Deno and thousands of other growth-deficient children in this country was thrice-weekly injections of human growth hormone (somatropin) taken from the pituitary (a small gland at the base of the brain) of cadavers. The yearly treatment for one child required hormone from 25 to 50 pituitary glands. Because of severely limited and expensive supplies, children with hGH deficiency received the hormone only until they had reached certain heights-- 5 feet 6 inches for boys and 5 feet 4 inches for girls.

Then, in April of 1985, three cases of an extremely rare,invariably fatal infection, Creutzfeldt-Jakob disease, were diagnosed in patients who had been using the hormone for five to 12 years. Experts theorized that some batches of the cadaver-derived hormone had contained the virus that causes the disease. (The virus is known to be harbored in the brain tissues of diseased patients.) Since there was no way to guarantee that the virus would be totally removed during the purification process of growth hormone from pituitaries, distribution of the hormone was suspended.

But in October 1985, after three and a half years of clinicaltesting at 13 U.S. medical centers, the Food and Drug Administration approved a genetically engineered form of human growth hormone providing virtually limitless supplies to the 10,000 to 15,000 American children in need of the growth stimulant.

"There is nothing but enthusiasm,' says Denise Orenstein,executive director of the Human Growth Foundation, a Bethesda, Md.-based nonprofit organization made up mainly of parents of children deficient in growth hormone. "This is the first time there has been an unlimited supply.'

The biosynthetic hormone was developed by the San Franciscocompany Genentech. Marketed under the trade name Protropin, it was only the second genetically engineered pharmaceutical, after insulin, to be approved in the United States.

(Genetic engineering, also known as recombinant DNAtechnology, is the process of introducing into a microorganism or other cell, DNA that contains genes for producing a specific protein. The DNA is inserted into the "host' cell in such a way that the cell treats it as its own, following the instructions coded in the gene to make the desired protein, in this case human growth hormone.)

Just what causes hGH deficiency remains a mystery. Occasionallya tumor destroys the pituitary gland, or there is an abnormality of the chromosome that contains the gene for growth hormone. Environmental and untritional influences play some kind of role; during protein deficiency and exercise, growth hormone production increases. Recent research indicates that the problem, in many cases, lies in a part of the brain called the hypothalamus, which releases a substance known as growth hormone-releasing factor. This substance stimulates the release of growth hormone from the pituitary gland.

How well the hypothalamus carries out its task may be affectedby stress, brought on by a variety of conditions--nutritional deficiencies and psychological or physical disturbances, for example. …

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