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Fetal Alcohol Syndrome

Fetal Alcohol Syndrome

Definition

Fetal alcohol syndrome (FAS) is a pattern of birth defects, learning, and behavioral problems affecting individuals whose mothers consumed alcohol during pregnancy.

Description

FAS is the most common preventable cause of mental retardation. This condition was first recognized and reported in the medical literature in 1968 in France and in 1973 in the United States. Alcohol is a teratogen, the term used for any drug, chemical, maternal disease or other environmental exposure that can cause birth defects or functional impairment in a developing fetus. Some features may be present at birth including low birth weight, prematurity, and microcephaly. Characteristic facial features may be present at birth, or may become more obvious over time. Signs of brain damage include delays in development, behavioral abnormalities, and mental retardation, but affected individuals exhibit a wide range of abilities and disabilities. It has only been since 1991 that the long-term outcome of FAS has been known. Learning, behavioral, and emotional problems are common in adolescents and adults with FAS. Fetal Alcohol Effect (FAE), a term no longer favored, is sometimes used to describe individuals with some, but not all, of the features of FAS. In 1996, the Institute of Medicine suggested a five-level system to describe the birth defects, learning and behavioral difficulties in offspring of women who drank alcohol during pregnancy. This system contains criteria including confirmation of maternal alcohol exposure, characteristic facial features, growth problems, learning and behavioral problems, and birth defects known to be associated with prenatal alcohol exposure.

The incidence of FAS varies among different populations studied, and ranges from approximately one in 200 to one in 2000 at birth. However, a study reported in 1997, utilizing the Institute of Medicine criteria, estimated the prevalence in Seattle, Washington from 19751981 at nearly one in 100 live births. Avoiding alcohol during pregnancy, including the earliest weeks of the pregnancy, can prevent FAS. There is no amount of alcohol use during pregnancy that has been proven to be completely safe.

There is no racial or ethnic relation to FAS. Individuals from different genetic backgrounds exposed to similar amounts of alcohol during pregnancy may exhibit different signs or symptoms of FAS. Estimates state that 30-45% of women who consume six or more drinks a day throughout pregnancy will give birth to a child with FAS. The risk of FAS appears to increase as a chronic alcoholic woman progresses in her childbearing years and continues to drink. That is, a child with FAS will often be one of the last born to a chronic alcoholic woman, although older siblings may exhibit milder features of FAS. Binge drinking, defined as sporadic use of five or more standard alcoholic drinks per occasion, and "moderate" daily drinking (two to four 12 oz bottles of beer, eight to 16 ounces of wine, two to four ounces of liquor) can also result in offspring with features of FAS. Experts say a few binges early in pregnancybefore a woman may even know she is pregnantmay be enough to be dangerous, even if she stops drinking later.

Causes and symptoms

FAS is not a genetic or inherited disorder. It is a pattern of birth defects, learning, and behavioral problems that are the result of maternal alcohol use during the pregnancy. The alcohol freely crosses the placenta and causes damage to the developing embryo or fetus. Alcohol use by the father cannot cause FAS. If a woman who has FAS drinks alcohol during pregnancy, then she may also have a child with FAS. Not all individuals from alcohol exposed pregnancies have obvious signs or symptoms of FAS; individuals of different genetic backgrounds may be more or less susceptible to the damage that alcohol can cause. The dose of alcohol, the time during pregnancy that alcohol is used, and the pattern of alcohol use all contribute to the different signs and symptoms that are found.

Classic features of FAS include short stature, low birthweight and poor weight gain, microcephaly, and a characteristic pattern of facial features. These facial features in infants and children may include small eye openings (measured from inner corner to outer corner), epicanthal folds (folds of tissue at the inner corner of the eye), small or short nose, low or flat nasal bridge, smooth or poorly developed philtrum (the area of the upper lip above the colored part of the lip and below the nose), thin upper lip, and small chin. Some of these features are nonspecific, meaning they can occur in other conditions, or be appropriate for age, racial, or family background. Other major and minor birth defects that have been reported include cleft palate, congenital heart defects, strabismus, hearing loss, defects of the spine and joints, alteration of the hand creases, small fingernails, and toenails. Since FAS was first described in infants and children, the diagnosis is sometimes more difficult to recognize in older adolescents and adults. Short stature and microcephaly remain common features, but weight may normalize, and the individual may actually become overweight for his/her height. The chin and nose grow proportionately more than the middle part of the face and dental crowding may become a problem. The small eye openings and the appearance of the upper lip and philtrum may continue to be characteristic. Pubertal changes typically occur at the normal time.

Newborns with FAS may have difficulties with feeding due to a poor suck, have irregular sleep-wake cycles, decreased or increased muscle tone, seizures or tremors. Delays in achieving developmental milestones such as rolling over, crawling, walking and talking may become apparent in infancy. Behavior and learning difficulties typical in the preschool or early school years include poor attention span, hyperactivity, poor motor skills, and slow language development. Attention deficit-hyperactivity disorder is a common associated diagnosis. Learning disabilities or mental retardation may be diagnosed during this time. Arithmetic is often the most difficult subject for a child with FAS. During middle school and high school years the behavioral difficulties and learning difficulties can be significant. Memory problems, poor judgment, difficulties with daily living skills, difficulties with abstract reasoning skills, and poor social skills are often apparent by this time. It is important to note that animal and human studies have shown that neurologic and behavioral abnormalities can be present without characteristic facial features. These individuals may not be identified as having FAS, but may fulfill criteria for alcohol-related diagnoses, as set forth by the Institute of Medicine.

In 1991, Streissguth and others reported some of the first long-term follow-up studies of adolescents and adults with FAS. In the approximate 60 individuals they studied, the average IQ was 68, with 70 being the lower limit of the normal range. However, the range of IQ was quite large, as low as 20 (severely retarded) to as high as 105 (normal). The average achievement levels for reading, spelling, and arithmetic were fourth grade, third grade and second grade, respectively. The Vineland Adaptive Behavior Scale was used to measure adaptive functioning in these individuals. The composite score for this group showed functioning at the level of a seven-year-old. Daily living skills were at a level of nine years, and social skills were at the level of a six-year-old.

In 1996, Streissguth and others published further data regarding the disabilities in children, adolescents and adults with FAS. Secondary disabilities, that is, those disabilities not present at birth and that might be preventable with proper diagnosis, treatment, and intervention, were described. These secondary disabilities include: mental health problems; disrupted school experiences; trouble with the law; incarceration for mental health problems, drug abuse, or a crime; inappropriate sexual behavior; alcohol and drug abuse; problems with employment; dependent living; and difficulties parenting their own children. In that study, only seven out of 90 adults were living and working independently and successfully. In addition to the studies by Streissguth, several other authors in different countries have now reported on long-term outcome of individuals diagnosed with FAS. In general, the neurologic, behavioral and emotional disorders become the most problematic for the individuals. The physical features change over time, sometimes making the correct diagnosis more difficult in older individuals, without old photographs and other historical data to review. Mental health problems including attention deficit, depression, panic attacks, psychosis and suicide threats and attempts, and overall were present in more than 90% of the individuals studied by Streissguth. A 1996 study in Germany reported more than 70% of the adolescents they studied had persistent and severe developmental disabilities and many had psychiatric disorders, the most common of which were emotional disorders, repetitive habits, speech disorders, and hyperactivity disorders.

Diagnosis

FAS is a clinical diagnosis, which means that there is no blood, x ray or psychological test that can be performed to confirm the suspected diagnosis. The diagnosis is made based on the history of maternal alcohol use, and detailed physical examination for the characteristic major and minor birth defects and characteristic facial features. It is often helpful to examine siblings and parents of an individual suspected of having FAS, either in person or by photographs, to determine whether findings on the examination might be familial, of if other siblings may also be affected. Sometimes, genetic tests are performed to rule out other conditions that may present with developmental delay or birth defects. Individuals with developmental delay, birth defects or other unusual features are often referred to a clinical geneticist, developmental pediatrician, or neurologist for evaluation and diagnosis of FAS. Psychoeducational testing to determine IQ and/or the presence of learning disabilities may also be part of the evaluation process.

Treatment

There is no treatment for FAS that will reverse or change the physical features or brain damage associated with maternal alcohol use during the pregnancy. Most of the birth defects associated with prenatal alcohol exposure are correctable with surgery. Children should have psychoeducational evaluation to help plan appropriate educational interventions. Common associated diagnoses such as attention deficit-hyperactivity disorder, depression, or anxiety should be recognized and treated appropriately. The disabilities that present during childhood persist into adult life. However, some of the secondary disabilities mentioned above may be avoided or lessened by early and correct diagnosis, better understanding of the life-long complications of FAS, and intervention. Streissguth has describe a model in which an individual affected by FAS has one or more advocates to help provide guidance, structure and support as the individual seeks to become independent, successful in school or employment, and develop satisfying social relationships.

Prognosis

The prognosis for FAS depends on the severity of birth defects and the brain damage present at birth. Miscarriage, stillbirth or death in the first few weeks of life may be outcomes in very severe cases. Major birth defects associated with FAS are usually treatable with surgery. Some of the factors that have been found to reduce the risk of secondary disabilities in FAS individuals include diagnosis before the age of six years, stable and nurturing home environments, never having experienced personal violence, and referral and eligibility for disability services. The long-term data helps in understanding the difficulties that individuals with FAS encounter throughout their lifetime and can help families, caregivers and professionals provide the care, supervision, education and treatment geared toward their special needs.

Prevention of FAS is the key. Prevention efforts must include public education efforts aimed at the entire population, not just women of child bearing age, appropriate treatment for women with high-risk drinking habits, and increased recognition and knowledge about FAS by professionals, parents, and caregivers.

KEY TERMS

Cleft palate A congenital malformation in which there is an abnormal opening in the roof of the mouth that allows the nasal passages and the mouth to be improperly connected.

Congenital Refers to a disorder that is present at birth.

IQ Abbreviation for Intelligence Quotient. Compares an individual's mental age to his/her true or chronological age and multiplies that ratio by 100.

Microcephaly An abnormally small head.

Miscarriage Spontaneous pregnancy loss.

Placenta The organ responsible for oxygen and nutrition exchange between a pregnant mother and her developing baby.

Strabismus An improper muscle balance of the ocular musles resulting in crossed or divergent eyes.

Teratogen Any drug, chemical, maternal disease, or exposure that can cause physical or functional defects in an exposed embryo or fetus.

Resources

PERIODICALS

Committee of Substance Abuse and Committee on Children with Disabilities. "Fetal Alcohol Syndrome and Alcohol-Related Neurodevelopmental Disorders." Pediatrics 106 (August 2000): 358-361.

Cramer, C., and F. Davidhizar. "FAS/FAE: Impact on Children." Journal of Child Health Care 3 (Autumn 1999): 31-34.

"Fetal Alcohol Syndrome Is Still a Threat, Says Publication." Science Letter September 28, 2004: 448.

Hannigan, J. H., and D. R. Armant. "Alcohol in Pregnancy and Neonatal Outcome." Seminars in Neonatology 5 (August 2000): 243-54.

"Prenatal Exposure to Alcohol." Alcohol Research and Health 24 (2000): 32-41.

ORGANIZATIONS

Fetal Alcohol Syndrome Family Resource Institute. PO Box 2525, Lynnwood, WA 98036. (253) 531-2878 or (800) 999-3429. http://www.fetalalcoholsyndrome.org.

Institute of Medicine. National Academy Press, Washington, DC. http://www.come-over.to/FAS/IOMsummary.htm.

March of Dimes Birth Defects Foundation. 1275 Mamaroneck Ave., White Plains, NY 10605. (888) 663-4637. resourcecenter@modimes.org. http://www.modimes.org.

Nofas. 216 G St. NE, Washington, DC 20002. (202) 785-4585. http://www.nofas.org.

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Fetal Alcohol Syndrome

Fetal alcohol syndrome

Definition

Fetal alcohol syndrome (FAS) is a set of physical and mental birth defects that can result from a woman drinking alcohol during her pregnancy. The syndrome is characterized by brain damage, facial deformities, and growth deficits. Heart, liver, and kidney defects are also common, as well as vision and hearing problems. These infants generally have difficulties with learning, attention, memory, and problem solving as they get older.

Description

Although there is a wide range of effects that result from in utero alcohol exposure, the diagnosis of FAS is recognized as the most severe birth defect that occurs. Fetal alcohol effect (FAE) is a term used to describe alcohol-exposed individuals whose condition does not meet the full criteria for an FAS diagnosis. The term alcohol-related neurodevelopmental disorders (ARND) is used for individuals with functional or cognitive impairments linked to prenatal alcohol exposure, including decreased head size at birth, structural brain abnormalities, and a pattern of behavioral and mental abnormalities. Alcohol-related birth defects (ARBD) describes the physical defects linked to prenatal alcohol exposure, including heart, skeletal, kidney, ear, and eye malformations.

FAS is the leading known preventable cause of mental retardation and birth defects. It affects one in 100 live births or as many as 40,000 infants born each year in the United States, and it is felt that the incidence is significantly under-reported. An individual with FAS can incur a lifetime health cost of over $800,000. In 2003, FAS cost the United States $3.9 billion in direct costs with indirect costs at approximately $1.5 billion. Children do not outgrow FAS. The physical and behavioral problems can last a lifetime. The syndrome is found in all racial and socio-economic groups. It is not a genetic disorder, so women with FAS or affected by FAS have healthy babies if they do not drink alcohol during their pregnancy.

Causes and symptoms

Alcohol is readily absorbed from the gastrointestinal tract into a pregnant woman's bloodstream and circulates to the fetus by crossing the placenta. Here it interferes with the ability of the fetus to receive sufficient oxygen and nourishment for normal cell development in the brain and other organs. The consumption of alcohol directly contributes to malnutrition because it contains no vitamins or minerals , and it uses up what the woman has for metabolism. Studies suggest that drinking a large amount of alcohol at any one time may be more dangerous to the fetus than drinking small amounts more frequently. The fetus is most vulnerable to various types of injuries depending on the stage of development in which alcohol is encountered. During the first eight weeks of pregnancy, organogenesis (the formation of organs) is taking place, which places the embryo at a higher risk of deformities when exposed to teratogens. Since a safe amount of alcohol intake during pregnancy has not been determined, twenty-first century authorities agree that women should not drink at all during pregnancy. A problem is that many women do not realize they are pregnant until the sixth to eight week. Therefore, women who are anticipating a pregnancy should abstain from all alcoholic beverages.

Unlike many birth defects which are identified at birth and then treated, FAS and FAE are usually overlooked at birth and treated later by mental health specialists, and often unknowingly. Possible FAS symptoms include:

  • growth deficiencies: small body size and weight, slower than normal development, and failure to catch up
  • skeletal deformities: deformed ribs and sternum; curved spine; hip dislocations; bent, fused, webbed, or missing fingers or toes; limited movement of joints; small head
  • facial abnormalities: small eye openings; skin webbing between eyes and base of nose; drooping eyelids; nearsightedness; strabismus ; failure of eyes to move in same direction; short upturned nose; sunken nasal bridge; flattened or absent groove between nose and upper lip; thin upper lip; cleft palate (opening in roof of mouth); small jaw; low-set or poorly formed ears
  • organ deformities: heart defects, heart murmurs , genital malformations, kidney and urinary defects
  • central nervous system handicaps: small brain; faulty arrangement of brain cells and connective tissue; mental retardation (usually mild to moderate but occasionally severe); learning disabilities; short attention span; irritability in infancy; hyperactivity in childhood; poor body, hand, and finger coordination

Since the primary birth defect in FAS and FAE involves central nervous system damage in utero, these newborns may have difficulties with feeding due to a poor suck, have irregular sleep-wake cycles, decreased or increased muscle tone, and seizures or tremors. Delays in achieving developmental milestones such as rolling over, crawling , walking, and talking may become apparent in infancy. Behavior and learning difficulties typical in the preschool or early school years include poor attention span, hyperactivity, poor motor skills, and slow language development . A common diagnosis that is associated with FAS is attention deficit-hyperactivity disorder. Learning disabilities or mental retardation may be diagnosed during this time. Arithmetic is often the most difficult subject for a child with FAS. During middle school and high school years, the behavioral difficulties and learning difficulties can be significant. Memory problems, poor judgment, difficulties with daily living skills, difficulties with abstract reasoning skills, and poor social skills are often apparent by this time. It is important to note that animal and human studies have shown that neurologic and behavioral abnormalities can be present without characteristic facial features. These individuals may not be identified as having FAS but may fulfill criteria for alcohol-related diagnoses, as set forth by the Institute of Medicine.

In 1991, Streissguth and others reported some of the first long-term follow-up studies of adolescents and adults with FAS. In the approximate 60 individuals they studied, the average IQ was 68 (70 is the lower limit of the normal range). However, the range of IQ was quite large, as low as 20 (severely retarded) to as high as 105 (normal). The average achievement levels for reading, spelling, and arithmetic were fourth grade, third grade, and second grade, respectively. The Vineland Adaptive Behavior Scale was used to measure adaptive functioning in these individuals. The composite score for this group showed functioning at the level of a seven-year-old. Daily living skills were at a level of nine years, and social skills were at the level of a six-year-old.

In 1996, Streissguth and others published further data regarding the disabilities in children, adolescents, and adults with FAS. Secondary disabilities (those disabilities not present at birth and that might be preventable with proper diagnosis, treatment, and intervention) were described. These secondary disabilities include: mental health problems; disrupted school experiences; trouble with the law; incarceration for mental health problems, drug abuse, or a crime; inappropriate sexual behavior; alcohol and drug abuse; problems with employment; dependent living; and difficulties parenting their own children. In that study, only seven out of 90 adults were living and working independently and successfully. In addition to the studies by Streissguth, several other authors in different countries have as of the early 2000s reported on long term outcome of individuals diagnosed with FAS. In general, the neurologic, behavioral, and emotional disorders become the most problematic for individuals. The physical features change over time, sometimes making the correct diagnosis more difficult in older individuals, without old photographs and other historical data to review. Mental health problems, including attention deficit, depression, panic attacks, psychosis, suicide threats and attempts, were present in over 90 percent of the individuals studied by Streissguth. A 1996 study in Germany reported more than 70 percent of the adolescents they followed had persistent and severe developmental disabilities, and many had psychiatric disorders, the most common of which were emotional disorders, repetitive habits, speech disorders , and hyperactivity disorders. (Some of the above information derives from Ann Streissguth's book, Fetal Alcohol Syndrome: A Guide for Families and Communities, which appeared in 1997.)

Diagnosis

FAS is a clinical diagnosis, which means that there is no blood test, x ray, or psychological test that can be performed to confirm the suspected diagnosis. The diagnosis is made based on the history of maternal alcohol use and detailed physical examination for the characteristic major and minor birth defects and characteristic facial features. It is often helpful to examine siblings and parents of an individual suspected of having FAS, either in person or by photographs, to determine whether findings on the examination might be familial and if other siblings may also be affected. Individuals with developmental delay or birth defects may be referred to a clinical geneticist for genetic testing or to a developmental pediatrician or neurologist for evaluation and diagnosis of FAS. Psychoeducational testing to determine IQ and/or the presence of learning disabilities may also be part of the evaluation process.

Treatment

There is no treatment for FAS that will reverse or change the physical features or brain damage associated with maternal alcohol use during the pregnancy. Most of the physical birth defects associated with prenatal alcohol exposure are correctable with surgery. Children should have psychoeducational evaluation to help plan appropriate educational interventions. Commonly associated diagnoses as attention deficit-hyperactivity disorder, depression, or anxiety should be recognized and treated appropriately. The disabilities that present during childhood persist into adult life. However, some of the secondary disabilities already mentioned may be avoided or lessened by early diagnosis and intervention. Streissguth has describe a model in which an individual affected by FAS has one or more advocates to help provide guidance, structure, and support as the individual seeks to become independent, successful in school or employment, and develop satisfying social relationships.

Prognosis

The prognosis for FAS depends on the severity of birth defects and the brain damage present at birth. Miscarriage, stillbirth, or death in the first few weeks of life may be outcomes in very severe cases. Major physical birth defects associated with FAS are usually treatable with surgery. Some of the factors that have been found to reduce the risk of secondary disabilities in FAS individuals include diagnosis before the age of six years, stable and nurturing home environments, never having experienced personal violence, and referral and eligibility for disability services. The long-term data help others understand the difficulties that individuals with FAS encounter throughout their lifetimes and can help families, caregivers, and professionals provide the care, supervision, education, and treatment geared toward their special needs.

Parental concerns

Prevention of FAS is the key. Prevention efforts must include public education efforts aimed at the entire population, not just women of child bearing age, appropriate treatment for women with high-risk drinking habits, and increased recognition and knowledge about FAS by professionals, parents, and caregivers.

KEY TERMS

Cleft palate A congenital malformation in which there is an abnormal opening in the roof of the mouth that allows the nasal passages and the mouth to be improperly connected.

Congenital Present at birth.

Intelligence quotient (IQ) A measure of somebody's intelligence, obtained through a series of aptitude tests concentrating on different aspects of intellectual functioning.

Microcephaly An abnormally small head.

Miscarriage Loss of the embryo or fetus and other products of pregnancy before the twentieth week. Often, early in a pregnancy, if the condition of the baby and/or the mother's uterus are not compatible with sustaining life, the pregnancy stops, and the contents of the uterus are expelled. For this reason, miscarriage is also referred to as spontaneous abortion.

Organogenesis The formation of organs during development.

Placenta The organ that provides oxygen and nutrition from the mother to the unborn baby during pregnancy. The placenta is attached to the wall of the uterus and leads to the unborn baby via the umbilical cord.

Strabismus A disorder in which the eyes do not point in the same direction.

Teratogen Any drug, chemical, maternal disease, or exposure that can cause physical or functional defects in an exposed embryo or fetus.

Resources

BOOKS

Armstrong, Elizabeth M. Conceiving Risk, Bearing Responsibility: Fetal Alcohol Syndrome and the Diagnosis of Moral Disorder. Baltimore, MD: Johns Hopkins University, 2003.

Fetal Alcohol Syndrome No. V: Index to New Information. Washington, DC: A B B E Publishers Association, 2005.

Golden, Janet. Message in a Bottle: The Making of Fetal Alcohol Syndrome. Cambridge, MA: Harvard University Press, 2005.

Kleinfeld, Judith, et al. Fantastic Antone Grows Up: Adolescents and Adults with Fetal Alcohol Syndrome. Fairbanks, AK: University of Alaska, 2000.

PERIODICALS

Committee of Substance Abuse and Committee on Children with Disabilities. "Fetal Alcohol Syndrome and Alcohol-Related Neurodevelopmental Disorders." Pediatrics 106 (August 2000): 35861.

Hannigan, J. H., and O. R. Armant. "Alcohol in Pregnancy and Neonatal Outcome." Seminars in Neonatology 5 (August 2000): 24354.

ORGANIZATIONS

Fetal Alcohol Syndrome Family Resource Institute. PO Box 2525, Lynnwood, WA 98036. Web site: <www.fetalalcoholsyndrome.org>.

March of Dimes Birth Defects Foundation. 1275 Mamaroneck Ave., White Plains, NY 10605. Web site: <www.modimes.org>.

National Institute on Alcohol Abuse and Alcoholism. 5635 Fishers Lane, MSC 9304, Bethesda, MD 208929304. Web site: <www.niaaa.nih.gov/>.

National Organization on Fetal Alcohol Syndrome (NOFAS). 900 17th Street, NW, Suite 910, Washington, DC 20006. Web site: <www.nofas.org>.

Linda K. Bennington

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"Fetal Alcohol Syndrome." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Retrieved August 16, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/fetal-alcohol-syndrome

Fetal Alcohol Syndrome

FETAL ALCOHOL SYNDROME

Fetal alcohol syndrome, or FAS, refers to a consistent pattern of birth defects found in some individuals whose mothers drank alcohol during their pregnancy. It is the most devastating outcome of prenatal alcohol exposure. Fetal alcohol effects (FAE) refers to a condition in which fewer of the elements of FAS are present.

FAS is permanent and cannot be reversed or cured, although some aspects may change as a child grows or be ameliorated with proper environments. Small physical size often remains throughout life, beginning with low birth weight and short length at birth. Some characteristics may seem to change as the child grows; for example, some of the characteristic facial features of FAS can become less obvious. However, other problems worsen with age. For example, academic difficulties may not be noticeable until early school age, and some behavioral problems are manifested during the teenage years.

Multiple mechanisms may be involved in the way alcohol affects the fetus. Alcohol interferes with the development and function of nerve cells and can result in cell death. Alcohol consumption can act indirectly by affecting blood flow from the mother to the fetus. In that respect, acetaldehyde, a by-product of the metabolism of alcohol, may be a contributing factor to FAS, although alcohol is the primary cause. No single mechanism has been found to be the sole cause; instead, there appear to be numerous mechanisms, sites, and risk factors.

ETIOLOGY OF FAS

For well over a century, artists and popular writers have depicted disabilities among the children of alcoholic mothers, but, until the 1960s, medical professionals believed that the placenta acted as natural barrier to toxic substances. It is now known that alcohol is a teratogen that is, it causes malformations in the developing embryo. Scientific knowledge changed when French (Lemoine et al., 1968) and American researchers (Jones and Smith, 1973; Ulleland, 1972) reported on patterns of malformations in infants born to mothers who drank excessively. Since then, over 6,000 journal articles have reported research describing the prenatal effects of alcohol, with the cumulative evidence leaving little doubt regarding the adverse outcomes of heavy alcohol exposure. Longitudinal studies following children and adults with FAS since the 1970s have been descriptive of the physical, cognitive, and behavioral characteristics. Other animal and human studies have examined specific aspects, such as precise areas of brain damage, and the effects of moderate alcohol use.

DIAGNOSIS AND DESCRIPTION OF FAS/FAE

FAS requires a medical diagnosis. Both Astley and Clarren (1997) and the Institute of Medicine (Stratton et al., 1996) have written criteria for diagnosis. Each includes as criteria: (1) known prenatal alcohol exposure; (2) growth deficiency;(3) characteristic facial features such as narrow upper lip, short palpebral fissures (eye openings), and indistinct philtrum (grove above upper lip); and (4) central nervous system involvement. The diagnosis of FAE requires confirmation of maternal alcohol use, along with fewer other criteria. Both sets of criteria also consider a diagnosis of FAS and FAE without confirmation of maternal alcohol use, which is less certain since many of these outcomes can have other causes. The term "partial FAS" has been suggested as a replacement of FAE, although others realize a continuum of effects, and prefer the term "FAS/FAE." Related terms are "alcohol-related birth defects" (ARBD), which refers to any defect caused by alcohol, and "alcohol-related neurodevelopment disorder" (ARND), which refers to neurodevelopmental problems. These conditions may not warrant a diagnosis of either FAS or FAE.

FAE should not be considered less severe, since the behavioral or learning problems can cause lifelong difficulties. FAE often goes undiagnosed in the absence of the more readily identifiable physical characteristics.

BEHAVIOR AND COGNITIVE OUTCOMES

Extensive and serious behavioral and cognitive abnormalities are associated with FAS/FAE. These characteristics result from prenatal brain damage and cannot be reversed, although with proper care many problems can be lessened. For example, many children with FAS/FAE become uncontrollable with too many audible and visual stimuli, including bright colors, competing noises, and many people around them. Altering the environment can help reduce these problems. Another common characteristic is the inability to learn from past experiences, and parents have found that pictorial reminders of daily routines help reduce frustrations for both the child and caregivers.

Some outcomes of prenatal heavy alcohol use are noticeable at infancy, including sleep disturbances and fine motor dysfunction. During pre-school years, fitful sleeping and lack of coordination persist, and other problems develop, especially attention deficit disorder, hyperactivity, and impulsivity, which may result in an individual being more accident-prone. Hypersensitivity to touch is also common. Social problems often seen in children with FAS/FAE include an inability to distinguish friends from strangers, difficulty in forming friendships, and being overly friendly with adults. Overly talkative behavior is characteristic and is often confused with good language abilities, but there may be little meaningful content. Many children have low thresholds for frustration, have frequent temper tantrums, and demand constant attention and supervision. These characteristics, and others, are commonly described in children with FAS/FAE, although every child may not have these characteristics. For school-aged children, the most frequently reported and specifically studied behavioral characteristics are attention deficit, hyperactivity, and impulsivity, which Mattson and Riley (1998) have called the "hallmark features" of FAS/FAE.

Another serious consequence of prenatal heavy alcohol exposure is the very high prevalence of mental retardation. However, some children with FAS/FAE have IQs within the normal range, although those with the most severe facial abnormalities and growth retardation are most likely to have learning problems. The range of IQ scores is higher amongst those with FAE than those with FAS. Many children have difficulties with language and mathematics. For adolescents and adults, the earlier cognitive and behavioral problems persist and new problems arise.

People with FAS/FAE are often accused of lying, although more often their stories change in order to please the listener. Typically, they seem unable to appreciate the consequences of their actions. They are often accused of behaviors such as stealing, although in reality they may take things because of an inability to see a connection between an item and its owner. Abstract reasoning and problem-solving skills also pose difficulties.

Understanding these common characteristics allows those working or living with people with FAS/FAE to realize that they are not necessarily prone to stealing or lying, but that they have problems with reasoning, understanding concepts, and language. Secondary problems arise from these difficulties. A U.S. study found that 60 percent of people with FAS over age eleven had been in trouble with the law, and a study of the Canadian criminal justice system found that 23 percent of youths remanded for forensic assessment were found to have FAS. These rates are well above the estimated worldwide incidence rates of FAS.

PUBLIC HEALTH BURDEN

The FAS incidence rate has been derived from a number of countries and is estimated to be 0.97 per 1,000 live births in the general population. The incidence of FAE is estimated to be ten times higher than FAS. The rates vary depending on the community, with some isolated, disadvantaged communities having much higher rates. FAS/FAE is a leading cause of birth defects, and may be the most common cause of mental disabilities, more common than Down syndrome (1 per 600 live births) and spina bifida (1 per 700 live births).

Beyond numbers of cases, there is a public health burden relating to cost. Estimates have been in the millions of dollars when health care, special schooling, and other costs are tallied in caring for children with FAS.

RISK FACTORS

Not all children whose mothers drank heavily during pregnancy have FAS. The extent and type of alcohol-related disabilities depend on the amount, pattern, and timing of exposure, the length of time during which the mother drank, nutrition, and other maternal health factors. Heavy alcohol exposure can come through daily drinking or drinking large amounts at one time. This refers to the pattern of drinking, and binge drinking (5 or more drinks at any occasion) is particularly risky for the fetus. Multiple maternal factors increase the likelihood of FAS, including older age, greater parity (having had previous children), and being a cigarette smoker. Poverty is considered to be a major determinant of the occurrence of FAS, and as Abel (1995) notes, "FAS is not an equal opportunity birth defect."

PUBLIC HEALTH MESSAGE

Various strategies have been used to decrease the use of alcohol during pregnancy, ranging from warning signs in places wherever alcohol is sold to midwives assisting those most at risk to improve health during pregnancy. Despite recognition of this serious birth outcome, many physicians still fail to recognize alcohol use in their patients and fail to diagnose FAS/FAE. Some medical professionals believe that until there are better treatment facilities for substance-abusing pregnant women, there is little value in identifying problem drinking. Public health messages note that women should either reduce heavy alcohol use during pregnancy or, if heavy drinking continues, delay becoming pregnant. The important aspect of FAS/FAE is that it is entirely preventable.

M. Anne George

(see also: Alcohol Use and Abuse; Congenital Anomalies; Maternal and Child Health; Pregnancy; Prenatal Care )

Bibliography

Abel, E. L. (1995). "An Update on Incidence of FAS: FAS Is Not an Equal Opportunity Birth Defect." Neurotoxicology and Teratology 17:437443.

Astley, S. J., and Clarren, S. K. (1997). Diagnostic Guide for Fetal Alcohol Syndrome and Related Conditions: The 4-digit Diagnostic Code. Seattle: University of Washington.

Fast, D. K.; Conry, J.; and Loock, C. A. (1999). "Identifying Fetal Alcohol Syndrome (FAS) among Youth in the Criminal Justice System." Journal of Developmental and Behavioral Pediatrics 20:12671271.

Jones, K. L., and Smith, D. W. (1973). "Recognition of the Fetal Alcohol Syndrome in Early Infancy." Lancet 1:12671271.

Lemoine, P.; Harousseau, H.; Borteyru, J. P.; and Menuet, J. C. (1968). "Les enfants des parents alcooliques: Anomalies observées. A propos de 127 cas." Ouest Medical 21:476482.

Mattson, S. N., and Riley, E. P. (1998). "A Review of the Neurobehavioral Deficits in Children with Fetal Alcohol Syndrome or Prenatal Exposure to Alcohol." Alcoholism: Clinical and Experimental Research 22:279294.

Stratton, K.; Howe, C.; and Battaglia, F., eds. (1996). Fetal Alcohol Syndrome: Diagnosis, Epidemiology, Prevention, and Treatment. Washington, DC: National Academy Press.

Streissguth, A. P.; Barr, H. M.; Kogan, J.; and Bookstein, F. L. (1996). Understanding the Occurrence of Secondary Disabilities in Clients with Fetal Alcohol Syndrome (FAS) and Fetal Alcohol Effects (FAE): Final Report. Seattle: University of Washington School of Medicine, Department of Psychiatry and Behavioral Sciences, Fetal Alcohol and Drug Unit.

Ulleland, C. N. (1972). "The Offspring of Alcoholic Mother." Annals of the New York Academy of Sciences 197:167169.

U.S. Department of Health and Human Services, National Institutes of Health, National Institute on Alcohol Abuse and Alcoholism (2000). 10th Special Report to the U.S. Congress on Alcohol and Health. Washington, DC: Author.

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fetal alcohol syndrome

fetal alcohol syndrome (FAS), pattern of physical, developmental, and psychological abnormalities seen in babies born to mothers who consumed alcohol during pregnancy. The abnormalities include low birthweight, facial deformities, and mental retardation, and there appears to be an association with impulsive behavior, anxiousness, and an inability on the part of the affected children to understand the consequences of their actions. When some but not all of these abnormalities are present, they are referred to as fetal alcohol effects (FAE). FAE has been observed in children of mothers who drank as little as two drinks per week during pregnancy. FAS affects 1 to 2 babies per 1,000 born worldwide. Many require constant lifelong supervision and end up institutionalized because of dysfunction in the family. FAS was first defined as a syndrome in 1973, although it has been observed for centuries. See also alcoholism.

See M. Dorris, The Broken Cord: A Family's Ongoing Struggle with Fetal Alcohol Syndrome (1989).

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Fetal Alcohol Syndrome

Fetal Alcohol Syndrome

Fetal alcohol syndrome (FAS) is a birth defect caused by a mother's alcohol intake during pregnancy. The symptoms of FAS are mental retardation, poor growth, facial defects, and behavioral problems. It is one of the leading causes of mental retardation in children. The effects are lifelong. Fetal alcohol effects (FAE) is a less severe set of the same symptoms. FAS is found in infants of all races and ethnic groups. Since it is not known how much alcohol a pregnant woman must drink to cause the syndrome, it is recommended that women not drink alcohol at all during pregnancy.

see also Alcohol and Health; Pregnancy.

Sheah Rarback

Internet Resource

National Organization on Fetal Alcohol Syndrome. <http://www.nofas.org>

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Fetal Alcohol Syndrome

Fetal Alcohol Syndrome

The Leading Known Cause of Mental Retardation

What Are FAS and FAE?

What Are the Symptoms of FAS and FAE?

Living With FAS and FAE

Resources

Fetal alcohol syndrome is a group of physical and mental birth defects that can affect the children of mothers who drink alcoholic beverages during pregnancy.

Keywords

for searching the Internet and other reference sources

Alcohol-related developmental disabilities

Alcohol-related neurodevelopmental disabilities

The Leading Known Cause of Mental Retardation

In the United States, the law requires that every bottle or can of beer, wine, or hard liquor that is sold must include the following information on its label: (1) According to the Surgeon General, women should not drink alcoholic beverages during pregnancy because of the risk of birth defects; (2) Consumption of alcoholic beverages impairs your ability to drive a car or operate machinery and may cause health problems. The first warning refers to the risk of fetal alcohol syndrome (FAS), the leading known cause of mental retardation in the United States. The good news is that this condition is 100 percent preventable as long as a woman does not drink alcohol while she is pregnant.

What Are FAS and FAE?

FAS is a group of physical and mental birth defects that can affect the children of mothers who drink alcohol during pregnancy. Typically, the worst cases are seen in the offspring of mothers who drank heavily. In general, the more the mother drank, the more severe the babys physical and mental problems are likely to be. The term fetal alcohol effects (FAE) often is used to refer to a less severe form of the syndrome. Since there is no proof that even small amounts of alcohol are safe for unborn children, doctors recommend that women completely avoid alcohol while they are pregnant. Both FAS and FAE have serious long-term consequences for affected children and their families.

According to the American Academy of Pediatrics, there may be as many as 10,000 to 12,000 new cases of FAS each year. In addition, there are probably many more cases of FAE. The exact number is not known, however. Since FAE does not cause the obvious physical defects seen in FAS, it can be difficult to diagnose correctly. To confuse matters further, mothers who drank during pregnancy may hide this fact from their doctors or lie about how much they drank. Experts believe that many children who now are thought to have learning disabilities may actually have undiagnosed FAE.

What Are the Symptoms of FAS and FAE?

Just as alcohol affects the brain of an adult who drinks, it also affects the brain of a developing fetus (FEE-tus), or unborn baby, who is exposed to it before birth. The most serious consequence of a mothers alcohol use during pregnancy is impaired brain development in the fetus, leading to mental retardation. Newborns with FAS often have small brains, indicating poor brain growth while they were inside their mothers, and they typically are small for their age. Other physical defects associated with FAS are narrow eyelids, a flattened midportion of the face, abnormal creases on the palms of the hands, heart defects, hearing and vision problems, and joint abnormalities. These problems are permanent.

Children with FAE may not have these physical defects. However, children with either FAS or FAE are likely to have behavioral and emotional problems. FAS and FAE can cause learning difficulties and slow down a childs development of speech, motor skills, and coordination. Children with these conditions often are impulsive, inattentive, and disorganized. They may have little understanding of the consequences of their behavior. They also may have trouble with following directions, solving problems, listening to people in authority, and socializing with peers. Sometimes these symptoms do not become evident until a child is 3 or 4 years old.

Children with FAS may have attention disorders, mental retardation, and skeletal problems. Many also have distinctive facial characteristics, such as widely spaced eyes, a shortened or flattened nose, and abnormalities in the shape and placement of the ears. David H. Wells/Corbis

Living With FAS and FAE

Life for children and young people FAS or FAE is often very difficult. Parents, caregivers, teachers, and other adults in authority need to be consistent and firm, setting predictable routines for the young person to follow. Adults may find that they have to repeat instructions again and again, making sure the child knows what is expected. Clear consequences for inappropriate behavior and rewards for appropriate behavior can be used to help the child make better choices. These children also tend to learn best when tasks are broken down into small pieces and new concepts are taught through concrete examples. In addition, medication may be part of the treatment plan.

Many children with FAS or FAE attend special education classes, just like other students who have learning disabilities or mental retardation. This gives them a chance to work with a specially trained teacher that would not be possible in the regular classroom.

See also

Attention Deficit Hyperactivity Disorder

Mental Retardation

Resources

Books

Dorris, Michael. The Broken Cord. New York: HarperCollins, 1990. At age 26, writer Michael Dorris adopted a young Native American child with FAS. This book provides compelling insight into what it is like to live with the condition.

Kleinfeld, Judith, and Siobhan Wescott. Fantastic Antone Succeeds! Experiences in Educating Children with Fetal Alcohol Syndrome. Fairbanks, AK: University of Alaska Press, 1993. This book features personal stories from children and families affected by FAS and FAE, as well as practical advice for parents and teachers who work with these young people. A sequel, Fantastic Antone Grows Up: Adolescents and Adults with Fetal Alcohol Syndrome, was published in 2000.

Organizations

National Organization on Fetal Alcohol Syndrome, 216 G Street Northeast, Washington, DC 20002. This national nonprofit group aims to raise public awareness of FAS. Telephone 800-666-6327 http://www.nofas.org

U.S. National Center on Birth Defects and Developmental Disabilities, Fetal Alcohol Syndrome section. This section of the U.S. Centers for Disease Control and Prevention website provides the latest information and statistics on FAS. http://www.cdc.gov/ncbddd/fas

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fetal alcohol syndrome

fetal alcohol syndrome (FAS) (fee-t'l) n. head and facial abnormalities and possibly mental retardation in a fetus due to intrauterine growth restriction caused by maternal over-consumption of alcohol during pregnancy.

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