French Settlement Disease, French Settlement, Livingston Parish, Louisiana File prepared by D.N. Pardue ********************** ********************************************** Copyright. All rights reserved. http://usgwarchives.net/copyright.htm http://usgwarchives.net/la/lafiles.htm ********************************************** From the French Settlement Historical Register, published by the French Settlement Historical Society, French Settlement, Louisiana 70733. The LaGenWeb Archives thanks them for contributing this information. Vol. 2, December 1977 Rarely are major scientific and medical discoveries isolated events. They grow out of a body of research that generally spans some decades. For example, the Salk polio vaccine was declared safe and effective in 1955, but the research that led to the successful development of the vaccine began in 1938 and involved many scientists trying various methods. Similarly, a long-term program is the prospect for developing a means to control the condition we have named French Settlement Disease (FSD). That disease is unique to the descendants of settlers in Livingston Parish of Louisiana. The disease is one of a group of disorders known collectively by the medical name, hereditary spastic paraplegia. Understand each word of that phrase -- hereditary spastic paraplegia -- and you will understand the basic concept of this disease. 1. Hereditary - received from parents of ancestors 2. Spastic - muscles are stiff and the movements awkward 3. Paraplegia - slight or incomplete paralysis of the lower part of the body FSD, therefore, is an inherited defect in which certain chemical reactions in the body do not proceed normally, resulting in serious disturbances in the function of the specialized nerves that control not only body movement but also speech and other processes. In the past few years, we have focused our efforts on FSD and several similar diseases. Strides have already been made in accumulating the know- ledge needed to control FSD. But no cure is available yet, and one is not likely soon. This leaflet gives an up-to-date report on current progress and hopes for the future. What causes the disease? Until recently that question would have been difficult to answer. However, we now have evindence that FSD occurs when a specific chemical )an enzyme) is missing in the body. An enzyme is a protein that aids in the body's chemical processes. Some enzymes convert starch into sugar. Others help the body get rid of excess sugar. Still others are necessary for the normal production and utilization of fats (sometimes called lipids.) Literally thousands of different enzymes are necessary for life. Although the search for the specific FSD-related enzyme continues, that enzyme has not yeat been identified. Why Do Some People Lack the Enzyme? A body is made of cells, the smallest living units, and cells contain chromosomes, the "instruciton code units" that tell each cell how to develop and function. Each chromosome contains thousands of genes, the carriers of heredity. Some genes control the body's manufacture of a particular enzyme. Patients with FSD either lack the special genes that control the manufac- ture of the enzyme, or those genes do not function normally in producing this enzyme. Here is how the trouble begins: The enzyme is a necesary part of a complex series of chemical reactions in which lipids (fats) are utilized in the normal functions of the body. Because of a defective or absent gene, a person with FSD cannot manufacture the enzyme, and his body may not form various fatty substances or may form them abnormally. One type of fatty substance, called myelin, occurs in high concentrations in the brain and other nervous tissues. The right amount of myelin is necesary for life; too little can disturb body functions and lead to malfunction of muscles or other tissues. In FSD, for example, the affected person lacks the enzyme needed for control of the amount of myelin in his nerve and brain cells. Without that enzyme the cells malfunction. Who Are the Victims of This Disease? Although all races and ethnic groups fal victim to some form of hereditary spastic paraplegia, the victims of FSD are residents of Livingston Parish and the surrounding area. We believe one of the defective genes producing FSD arose spontaneously in the population of Germany. All the affected persons are distantly related, being descendants of a young couple who migrated from Germany in 1741, settling near French Settlement. Since that time, 10 generations have passed and many offspring have been born. Now Literally hundreds of persons living in the area carry the FSD gene. Can the Disease Be Transmitted From An Affected Person To One Who is Not Related? How Is the Disease Transmitted? No, it cannot be "caught" from a person who has it. The disease can only be transmitted from parent to child. The defective gene is always present at birth; it does not develop in later life. A child must inherit two FSD genes, one from each parent, to actually have the disease. But a child who has only one defective gene, from only one parent, is a "carrier". A carrier is normal and as healthy as anyone else, except for two things -- 1. A specific blood test may eventually show that he or she has only half the amount of the particular enzyme that a noncarrier has. 2. A carrier should be aware of his/her carrier state, in case marriage to someone who is also a carrier is considered. If two carriers marry, the risk with each pregnancy that their child will have the disease is 25%, that the child will be totally free of the disease is another 25%, and that each child will be a carrier, like the parents, is 50%. Can A Child With FSD Be Born If Only the Father Or Only the Mother Is A Carrier? Such a couple cannot produce a child with FSD, but the odds are then one-in-two that their children will be carriers. Carriers are, of course, normal in appearance and function. But they are capable of passing on the gene that causes the disease. Both parents must be carriers for a child to be affected with FSD. Who Is Most Susceptible To Having a Child With FSD? The chance of having a child with FSD is greatest if both parents were born in Livingston Parish because the possibility of their being carriers is increased by the increased likelihood that they may be des- cendants of the original progenitor. Can Carriers of FSD Be Detected Now? No, not at present. But this is on area of research we are actively pursuing. How Early Can FSD BE Detected? Usually not before the age of 20. What Are the Hopes For the Future? In our research, we are concentrating on: 1. Finding the basic defect. We may then be able to treat persons who are now affected. 2. Identifying the defective-gene carrier and hopefully preventing extension of the disease. What Will It Cost To Accomplish This? We estimate that it will take at least $25,000 to support laboratory personnel and to purchase supplies and special equipment for a minimum of 10 years. What Can We Do Now? We can do two things: 1. We can assure that research continues at the swiftest possible pace. 2. We can assure that every potential carrier has the opportunity to determine whether he or she is, in fact, a carrier. Are Contributions Tax-Deductible? Yes, they are 100% tax deductible. Where Should Donations Be Sent? Louisiana Ethnogenetic Disease Association 1542 Tulane Avenue New Orleans, Louisiana 70112 ***Preparer's Note: This is not a plea for donations to this organization. The copy was taken word for word from the French Settlement Historical Register, Dec. 1977**** Source: Dr. Henry Rothschild, PhD. Professor of Medicine & Anatomy L.S.U. School of Medicine New Orleans, Louisiana 70112