Stormie Jones
Encyclopedia
Stormie Dawn Jones was the world's first recipient of a successful simultaneous heart
and liver
organ transplant
. On February 14, 1984, Drs. Thomas E. Starzl and Henry T. Bahnson replaced the six-year-old's heart and liver at the Pittsburgh Children's Hospital in Pittsburgh, Pennsylvania
. Stormie had a condition which raised her blood cholesterol
to 10 times normal levels. The condition, a severe form of familial hypercholesterolemia
,
and the resultant high levels of low density lipoprotein
that damaged her organs, gave her two heart attacks when she was six years old. The case showed that the liver controls blood cholesterol and that high cholesterol is controllable, and was part of the research on cholesterol and the liver that won Joseph L. Goldstein
and Michael S. Brown the Nobel prize in medicine
in 1985. Stormie died on November 11, 1990. Her death was related to rejection of the heart transplant she had received in 1984.
There were very specific reasons for performing a combined heart and liver transplant in this young girl. Due to her inherited condition, Stormie's liver was unable to remove cholesterol, i.e. LDL-cholesterol, from her bloodstream. As a result, her LDL-cholesterol levels became very high and led to her having two heart attacks by age six. On the other hand, the transplanted liver, being normal and healthy, was able to clear the LDL-cholesterol from her blood. Indeed, after the transplant, Stormie's LDL-cholesterol declined by 81 percent - from an astounding 988 to a near-normal 184 mg per deciliter. Since she was going to require life-long immunosuppressant
therapy anyway to prevent rejection of her transplanted liver, and since her heart had been severely damaged by her previous heart attacks, it was decided to also perform a heart transplant.
Heart
The heart is a myogenic muscular organ found in all animals with a circulatory system , that is responsible for pumping blood throughout the blood vessels by repeated, rhythmic contractions...
and liver
Liver
The liver is a vital organ present in vertebrates and some other animals. It has a wide range of functions, including detoxification, protein synthesis, and production of biochemicals necessary for digestion...
organ transplant
Organ transplant
Organ transplantation is the moving of an organ from one body to another or from a donor site on the patient's own body, for the purpose of replacing the recipient's damaged or absent organ. The emerging field of regenerative medicine is allowing scientists and engineers to create organs to be...
. On February 14, 1984, Drs. Thomas E. Starzl and Henry T. Bahnson replaced the six-year-old's heart and liver at the Pittsburgh Children's Hospital in Pittsburgh, Pennsylvania
Pittsburgh, Pennsylvania
Pittsburgh is the second-largest city in the US Commonwealth of Pennsylvania and the county seat of Allegheny County. Regionally, it anchors the largest urban area of Appalachia and the Ohio River Valley, and nationally, it is the 22nd-largest urban area in the United States...
. Stormie had a condition which raised her blood cholesterol
Cholesterol
Cholesterol is a complex isoprenoid. Specifically, it is a waxy steroid of fat that is produced in the liver or intestines. It is used to produce hormones and cell membranes and is transported in the blood plasma of all mammals. It is an essential structural component of mammalian cell membranes...
to 10 times normal levels. The condition, a severe form of familial hypercholesterolemia
Familial hypercholesterolemia
Familial hypercholesterolemia is a genetic disorder characterized by high cholesterol levels, specifically very high levels of low-density lipoprotein , in the blood and early cardiovascular disease...
,
and the resultant high levels of low density lipoprotein
Low density lipoprotein
Low-density lipoprotein is one of the five major groups of lipoproteins, which in order of size, largest to smallest, are chylomicrons, VLDL, IDL, LDL, and HDL, that enable transport of cholesterol within the water-based bloodstream...
that damaged her organs, gave her two heart attacks when she was six years old. The case showed that the liver controls blood cholesterol and that high cholesterol is controllable, and was part of the research on cholesterol and the liver that won Joseph L. Goldstein
Joseph L. Goldstein
Joseph L. Goldstein from Kingstree, South Carolina is a Nobel Prize winning biochemist and geneticist, and a pioneer in the study of cholesterol metabolism.-Biography:...
and Michael S. Brown the Nobel prize in medicine
Nobel Prize in Physiology or Medicine
The Nobel Prize in Physiology or Medicine administered by the Nobel Foundation, is awarded once a year for outstanding discoveries in the field of life science and medicine. It is one of five Nobel Prizes established in 1895 by Swedish chemist Alfred Nobel, the inventor of dynamite, in his will...
in 1985. Stormie died on November 11, 1990. Her death was related to rejection of the heart transplant she had received in 1984.
There were very specific reasons for performing a combined heart and liver transplant in this young girl. Due to her inherited condition, Stormie's liver was unable to remove cholesterol, i.e. LDL-cholesterol, from her bloodstream. As a result, her LDL-cholesterol levels became very high and led to her having two heart attacks by age six. On the other hand, the transplanted liver, being normal and healthy, was able to clear the LDL-cholesterol from her blood. Indeed, after the transplant, Stormie's LDL-cholesterol declined by 81 percent - from an astounding 988 to a near-normal 184 mg per deciliter. Since she was going to require life-long immunosuppressant
Immunosuppressant
An immunosuppressant is any substance that performs immunosuppression of the immune system. They may be either exogenous, as immunosuppressive drugs, or endogenous, as ,e. g., testosterone...
therapy anyway to prevent rejection of her transplanted liver, and since her heart had been severely damaged by her previous heart attacks, it was decided to also perform a heart transplant.