Clinic for Special Children
Encyclopedia
The Clinic for Special Children is a gene
research clinic located in Strasburg, Pennsylvania
. The facility, established by Dr. D. Holmes Morton
, specializes in genetic problems of the plain sects such as the Amish
and Old Order Mennonites.
The clinic treats about 600 children for 80 different genetic disorders or syndromes such as glutaric aciduria
(GA1), maple syrup urine disease
(MSUD), Crigler-Najjar syndrome
(CNS), and medium-chain acyl-CoA dehydrogenase deficiency (MCADD). Not all the children are Amish or Old Order Mennonites; about 15% of the children come from elsewhere, including Africa and Asia. About 75% of the children are treatable—and a third of those are highly treatable, many through techniques developed at the center. The center is responsible for nearly two dozen scientific papers.
derive from about 200 families who moved from the lower Rhine valley, the German Palatinate and Alsace
in the 18th century, though there have been some converts from the outside "English" world. The Amish do not allow marriage outside of the faith, therefore their gene pool
is more shallow than that of the average person. Some Amish are afflicted by heritable genetic disorders, including dwarfism
(Ellis-van Creveld syndrome
), and are also distinguished by the highest incidence of twin
s in a known human population, various metabolic disorders, and unusual distribution of blood types. Since almost all of the current Amish descend primarily from about 200 founders in the 18th century, some genetic disorders from a degree of inbreeding do exist in more isolated districts. However, Amish do not represent a single closed community, but rather a collection of different demes
or genetically-closed communities. Some of these disorders are quite rare, or even unique, and they are serious enough that they increase the mortality rate among Amish children. The majority of the Amish accept these as "Gottes Wille" (God's will); they reject any use of genetic tests prior to marriage to prevent these disorders as well as genetic testing of unborn children that would discover any genetic disorder.
There is an increasing consciousness among the Amish of the advantages of exogamy
. A common bloodline in one community will often be absent in another, and genetic disorders can be avoided by choosing spouses from unrelated communities. For example, the founding families of the Lancaster County Amish are unrelated to the founders of the Perth County Amish community in Canada.
Treating genetic problems is the mission of the Clinic which has developed effective treatments for such problems as maple syrup urine disease
, a disease which previously was fatal. The clinic has been enthusiastically embraced by most Amish, and has largely ended a situation in which some parents felt it necessary to leave the community to care properly for their children, an action which normally might result in being shunned.
when he first became aware of their special problems.
In 1989, Morton bought untillable
land from an Amish farmer and held a barn-raising. The result was a community hospital providing care, counseling, and genetic testing for disorders unique to the Amish and Old Order Mennonite
populations.
He initially did most of his own genetic testing and lab work, but now outsources DNA testing for over 30 genetic disorders in addition to the 25 extremely rare disorders he and his team screen for.
Amish and Mennonites near Middlefield, Ohio
, have raised US$700,000 towards the US$1.8 million needed to open the nonprofit Deutsch Center for Special Needs Children in Middlefield, to be headed by Dr. Heng Wang, who studied and worked with Morton.
The Lancaster County community holds several benefit auctions for the clinic each year, raising sufficient funds to cover about a third of the clinic's operating costs. Amish and Mennonite families donate quilts, furniture, baked goods, and other items to the sale. The clinic is a registered 501(c)(3) nonprofit charity.
In early 2009, the LaGrange County, Indiana Amish community began the process of establishing a Community Health Clinic for genetic research and treatment of rare disorders in the Midwest. CHC is to be modeled after and will be collaborating with The Clinic for Special Children in Strasburg, PA. On Sept. 25, 2009 a fundraiser auction was organized at Shipshewana, IN bringing in excess of $180,000 to the CHC fund. CHC is registered as a 501(c)(3) nonprofit organization. This midwestern 'Medical Home' is still in its planning stages and once completed should yield a wealth of genetic information which will be very useful in the prevention and treatment of genetic disorders.
Genetics
Genetics , a discipline of biology, is the science of genes, heredity, and variation in living organisms....
research clinic located in Strasburg, Pennsylvania
Strasburg, Pennsylvania
Strasburg is a borough in Lancaster County, Pennsylvania, United States. It developed as a linear village along the Great Conestoga Road, stretching about two miles along path later known as the Strasburg Road...
. The facility, established by Dr. D. Holmes Morton
D. Holmes Morton
D. Holmes Morton is an American physician specializing in genetic disorders of Old Order Amish and Mennonite children. In 1989 he established the Clinic for Special Children in Strasburg, Pennsylvania to focus on these diseases....
, specializes in genetic problems of the plain sects such as the Amish
Amish
The Amish , sometimes referred to as Amish Mennonites, are a group of Christian church fellowships that form a subgroup of the Mennonite churches...
and Old Order Mennonites.
Glutaric acidemia type 1
Glutaric acidemia type 1 is an inherited disorder in which the body is unable to break down completely the amino acids lysine, hydroxylysine and tryptophan...
(GA1), maple syrup urine disease
Maple syrup urine disease
Maple syrup urine disease , also called branched-chain ketoaciduria, is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia...
(MSUD), Crigler-Najjar syndrome
Crigler-Najjar syndrome
Crigler-Najjar Syndrome or CNS is a rare disorder affecting the metabolism of bilirubin, a chemical formed from the breakdown of blood. The disorder results in an inherited form of non-hemolytic jaundice, which results in high levels of unconjugated bilirubin and often leads to brain damage in...
(CNS), and medium-chain acyl-CoA dehydrogenase deficiency (MCADD). Not all the children are Amish or Old Order Mennonites; about 15% of the children come from elsewhere, including Africa and Asia. About 75% of the children are treatable—and a third of those are highly treatable, many through techniques developed at the center. The center is responsible for nearly two dozen scientific papers.
Amish and genetics
The vast majority of the nearly 200,000 Amish in AmericaUnited States
The United States of America is a federal constitutional republic comprising fifty states and a federal district...
derive from about 200 families who moved from the lower Rhine valley, the German Palatinate and Alsace
Alsace
Alsace is the fifth-smallest of the 27 regions of France in land area , and the smallest in metropolitan France. It is also the seventh-most densely populated region in France and third most densely populated region in metropolitan France, with ca. 220 inhabitants per km²...
in the 18th century, though there have been some converts from the outside "English" world. The Amish do not allow marriage outside of the faith, therefore their gene pool
Gene pool
In population genetics, a gene pool is the complete set of unique alleles in a species or population.- Description :A large gene pool indicates extensive genetic diversity, which is associated with robust populations that can survive bouts of intense selection...
is more shallow than that of the average person. Some Amish are afflicted by heritable genetic disorders, including dwarfism
Dwarfism
Dwarfism is short stature resulting from a medical condition. It is sometimes defined as an adult height of less than 4 feet 10 inches , although this definition is problematic because short stature in itself is not a disorder....
(Ellis-van Creveld syndrome
Ellis-van Creveld syndrome
Ellis–van Creveld Syndrome is a rare genetic disorder of the skeletal dysplasia type.-Symptoms:...
), and are also distinguished by the highest incidence of twin
Twin
A twin is one of two offspring produced in the same pregnancy. Twins can either be monozygotic , meaning that they develop from one zygote that splits and forms two embryos, or dizygotic because they develop from two separate eggs that are fertilized by two separate sperm.In contrast, a fetus...
s in a known human population, various metabolic disorders, and unusual distribution of blood types. Since almost all of the current Amish descend primarily from about 200 founders in the 18th century, some genetic disorders from a degree of inbreeding do exist in more isolated districts. However, Amish do not represent a single closed community, but rather a collection of different demes
Deme (biology)
In biology, a deme is a term for a local population of organisms of one species that actively interbreed with one another and share a distinct gene pool...
or genetically-closed communities. Some of these disorders are quite rare, or even unique, and they are serious enough that they increase the mortality rate among Amish children. The majority of the Amish accept these as "Gottes Wille" (God's will); they reject any use of genetic tests prior to marriage to prevent these disorders as well as genetic testing of unborn children that would discover any genetic disorder.
There is an increasing consciousness among the Amish of the advantages of exogamy
Exogamy
Exogamy is a social arrangement where marriage is allowed only outside of a social group. The social groups define the scope and extent of exogamy, and the rules and enforcement mechanisms that ensure its continuity. In social studies, exogamy is viewed as a combination of two related aspects:...
. A common bloodline in one community will often be absent in another, and genetic disorders can be avoided by choosing spouses from unrelated communities. For example, the founding families of the Lancaster County Amish are unrelated to the founders of the Perth County Amish community in Canada.
Treating genetic problems is the mission of the Clinic which has developed effective treatments for such problems as maple syrup urine disease
Maple syrup urine disease
Maple syrup urine disease , also called branched-chain ketoaciduria, is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia...
, a disease which previously was fatal. The clinic has been enthusiastically embraced by most Amish, and has largely ended a situation in which some parents felt it necessary to leave the community to care properly for their children, an action which normally might result in being shunned.
History
In the 1980s, Morton took a special interest in Amish children with rare metabolic diseases. Morton was a pediatrician at the Children's Hospital of PhiladelphiaChildren's Hospital of Philadelphia
The Children's Hospital of Philadelphia is one of the largest and oldest children's hospitals in the world. CHOP has been ranked as the best children's hospital in the United States by U.S. News & World Report and Parents Magazine in recent years. As of 2008, it was ranked #1 in the nation for...
when he first became aware of their special problems.
In 1989, Morton bought untillable
Tillage
Tillage is the agricultural preparation of the soil by mechanical agitation of various types, such as digging, stirring, and overturning. Examples of human-powered tilling methods using hand tools include shovelling, picking, mattock work, hoeing, and raking...
land from an Amish farmer and held a barn-raising. The result was a community hospital providing care, counseling, and genetic testing for disorders unique to the Amish and Old Order Mennonite
Old Order Mennonite
Old Order Mennonites is a branch of the Mennonite church. Although the term strictly refers to one particular group, it is often used to refer to those groups of Mennonites who practice a lifestyle without some elements of modern technology.-Overview:...
populations.
He initially did most of his own genetic testing and lab work, but now outsources DNA testing for over 30 genetic disorders in addition to the 25 extremely rare disorders he and his team screen for.
Amish and Mennonites near Middlefield, Ohio
Middlefield, Ohio
Middlefield is a village in Geauga County, Ohio, United States. The population was 2,233 at the 2000 census.-Geography:Middlefield is located at ....
, have raised US$700,000 towards the US$1.8 million needed to open the nonprofit Deutsch Center for Special Needs Children in Middlefield, to be headed by Dr. Heng Wang, who studied and worked with Morton.
The Lancaster County community holds several benefit auctions for the clinic each year, raising sufficient funds to cover about a third of the clinic's operating costs. Amish and Mennonite families donate quilts, furniture, baked goods, and other items to the sale. The clinic is a registered 501(c)(3) nonprofit charity.
In early 2009, the LaGrange County, Indiana Amish community began the process of establishing a Community Health Clinic for genetic research and treatment of rare disorders in the Midwest. CHC is to be modeled after and will be collaborating with The Clinic for Special Children in Strasburg, PA. On Sept. 25, 2009 a fundraiser auction was organized at Shipshewana, IN bringing in excess of $180,000 to the CHC fund. CHC is registered as a 501(c)(3) nonprofit organization. This midwestern 'Medical Home' is still in its planning stages and once completed should yield a wealth of genetic information which will be very useful in the prevention and treatment of genetic disorders.