;., I._ : .-; : :. . . ..`I. .LI1 `~ 7 "7' ^- r,* ,.. ,,, `:i.. ..* e-1 :,; `: :-. r / .."`1. , Tangier Disease - .".,. :" ,, : -.- ' Tonsils By JOHN PRUITT Virginian-Pilot Staff Writer TANGIER ISLAND - Scientists believe that the discolored tonsils of a Tangier Island boy, Teddy Laird, may provide an important clue in the search for causes and cures of some heart ailments. Laird's peculiar tonsils, removeo' seven years ago when he was five, led to the discovery of a blood disorder so rare it is known internationally as Tangier Disease. Since the Teddy Laird case was discovered, 10 others have been found in the world. The disorder is marked by a near absence of alpha lipoprotein (fat and protein) in the blood. His sister, Elaine, has the second case, and there are six other known cases in America. The others are in England and Switzerland. Mrs. Peggy Laird said that except for the unu- sual tonsils, the malady has had little effect on her children. "Elaine is very seldom sick," shle said, "and Ted just has a cold now and then." STARTED IN `60 Events leading to the discovery of Tangier Dis- ease began in April 1960, when Teddy was sent to Northampton-Accomack Memorial Hospital for a tonsillectomy. "Ted would just lie around," Mrs. Laird re- called recently. "He was just sickly. It wasn't a cold. It was his tonsils and adenoids doing it." Dr. J. Thomas Edmonds, who performed the tohsillectomy, was intrigued by the abnormally large, "grayish yellow . . . lobulated (and) wrinkled tonsils." E&ntnds had a pathologist at the Nassawadox %spital make microscopic sections to be sent to the Armed Forces Institute of Pathology in Washingfon. There, Teddy's malady was diagnosed as a rare fat.1 storage disorder, possiblv Hand-Schuller-Chris- tian Disease or Niemanri-Pick Disease. FEARED WORST In late Anril 1960, Ted& and his mother left their Chesarea!te Bay island home for the National Institutes of Health in Bethesda. Vd. Teddy was re- ferred to the National Cancer Institute. He spent the next seven weeks in Bethesda. "It almost ran me crazy, because I thought he had leukemia," Glado'en Laird, Teddy's father, said. Gladden Laird, like most of Tangier's popula- tion, is a Waterman. He works with his father, crab- bing during the warm months and oystering in the winter. At the Cancer Institute, the preliminary diagno- sis of Hand-Schuller-Christian Disease was dropped, and Teddy was sent to the National Heart Institute, where rare fat storage disorders are studied. The tentative diagnosis was Niemann-Pick Disease. "One doctor said Teddy couldn't live much long- er," Teddy's 3%yearold father recalled recently. "I was really getting on edge." NAMED FOR ISLAND . Niemann-Pick Disease is a genetic disorder usually suffered by infants. Its symptoms include malnutrition, stomach and intestinal disturbances, and abnormalities in blood-forming organs. It usual- ly is fatal. The disease also is marked by nervous system disorders. Teddy suffered no nervous disorders, so that diagnosis was dropped. The malady was la- beled Tangiar Disease. In Novemiber, Dr. Donald F. Fredrickson and some of his associates from the National Institutes of Health came to examine tonsils and take blood samples of Tangier's population. They came by mailboat from Crisfield, Md. This is the only daily connection between this 2x3-mile is- land anal the mainland. The mass examination was conducted in the island medical center, then unoc- cupied because Tangier had no doctor. Elaine Laird was found to have large, oddly-col- ored tonsils like her brother's She was admitted to N.I.H. for a tonsillectomy. It was the first tonsillectomy ever performed at N.I.H., Dr. Frederickson said. Another Tangier is- !ander's tonsils were removed there. They served May Cure Hearts J Dr. Fredrickson Elaine Laird Teddy Laird as controls, for comparison to those of the L.airds. Blood samples were taken from 127 people akin to Teddy and Elaine, and from 90 others for use as community controls. Dr. Fredrickson said he found Tangier's popula- tion "`very helpful and hospitable." Tangier's closely related population proved a poor community control. Investigation revealed that only 17 of the 90 volunteers did not have at least one relative common to the Laird children. No other islanders were found to have the large, nniouelg-colored tonsils associated with Tangier Disease. CONGENITAL However, each of the Laird children's parents and one grandoarent were found to have a deficient amount of alpha lipoprotein. The condition had been observed in blood samples from Teddy and Elaine. Researching Tangier Disease, Dr. Fredrickson said, "has been a tremendous help in unraveling This indicated to researchers that Tangier Dis- ease "no doubt" is genetically determined, and not necessarily confined to Tangier Island, Dr. Fred- rickson said. "It just happened that the first case was found on Tangier." He labeled the discovery "very exciting." relationships between heart disease and lipopro- teins. "Alpha lipoprotein is much higher in the blood of women than in men. Alpha lipoprotein (absent in Tangier Disease) levels are clearly related to the level of female sex hormones. "Women, before menopause, have about one quarter the likelihood of having a heart attack as men the same age. "It is not known why women have this special protection, and doctors still think it possible that this may be related to the higher alpha lipoprotein of women." 5 The importance of the abnormality of Tangier Disease (absence of alpha lipoproteins) to the re- search of heart disease is this: TANGIER CLUES "CholesteroI is transported by aIpha and befa lipoproteins. Arteriosclerosis is associated with the _ _ _ _ _ _ - ._ _. _. _ laying cbwn of cholesterol in the walls of the blood vesse!s. In the same manner rust inside a pipe will cut down the bore of the pipe, this accumulat- ed cholesterol cuts down the flow in the vessels." Dr. Frederickson said the discoveries made in research of Tangier Disease are "pieces to a great puzzle." The doctor said there is "no evidence as yet that Tangier Disease leads to more arteriosclero- sis." Eight of the IO people who have Tangier Dis- ease have beep to the National Institutes of Health, and blood samples from the others have been sent there. CHILDREN HELPED Except for the cases of Teddy and Elaine Laird, they all were discovered "because of low cholesterol levels," Dr. Fredrickson said. Mrs. Laird and her seafaring husband take their children to NLH. for yearly examinations. some of the secrets of how fats are transported in the blood." "We've had many a doctor to lcok at us," Gladden Laird said. While admitting that the research has been a costly procedure, Dr. Fredrickson said it has "re- paid us many fold in new information about body fat transport." Mrs. Laird said Teddy, now 12, and Elaine, 13, appear to be in almost perfect health. "Ted's just like a cricket," she said, "and I don't worry about Elaine at all." This is "particularly important," he said, "be- cause some abnormality in the way the body trans- ports fat through the blood is one of the causes of arteriosclerosis (hardening of the arteries). And ar- teriosclerosis causes more deaths than any other disease." She said she has seen a definite improvement in her children since they had the tonsillectomies. "Ted's a lot livlier. He can play longer, and he's more active," she said. MAY BE `KEY' Elaine, Mrs. Laird said, "eats real good, ana' she's in real good health." "I believe they helped them up there," Laird said. This new information about transportation of fats, the doctor said, "could be one of the keys to the solution of the No. I health problem in America and much of the world." He said researchers are trying to determine the chemical content of alpha lipoprotein. Studying Tan- gier Disease, he said, "has been an important factor in developing modern explanations of how lipoprc- teins do their job." Dr..Frerickson explained "one of the possible" Laird had high praise for Dr. Fredrickson. "There's not a better person in the world," he said, "I'd do anything under the sun for him." Dr. Fredrickson said he feels a "deep affec- tion" for Tangier's 900 residents. He said that for Teddy's or Elaine's offspring to inherit the disorder, both parents would have to be carriers of the gene producing Tangier disease. The chances of this, Dr. Fredrickson said, are slim.