Overview of Fetal Alcohol Syndrome
People with fetal alcohol syndrome (FAS) are born with it. FAS is a birth defect that has its primary effect on the brain. Some people with FAS are slightly affected and manifest only mildly dysfunctional behavior; others are severely affected, devastatingly disabled in their ability to cope with even simple day-to-day interactions. Each child with FAS has his or her own special needs, problems, and capabilities. FAS is a birth defect whether or not it is noticeable at birth. One does not outgrow FAS, although the manifestations may change with age. A bad environment (e.g., one in which there is abuse, neglect, or poverty) cannot cause FAS, just as a good environment (e.g., one with loving, caring parents) cannot fully undo it. A good environment and proper community supports, however, can protect the person with FAS from secondary disabilities, which can also be debilitating.
FAS is caused by prenatal alcohol exposure (see Figure 1.1) and is characterized by growth deficiency, a specific pattern of facial features, and some signs of CNS dysfunction.
PRENATAL ALCOHOL EXPOSURE CAN CAUSE A SPECTRUM OF EFFECTS
Prenatal alcohol exposure does not always result in FAS. Depending on the dose, timing, and conditions of exposure as well as on the individual characteristics of the mother and fetus, prenatal alcohol exposure can cause a range of disabling conditions. Some children are diagnosable with the full FAS; others have only partial manifestations, usually the CNS effects without the characteristic facial features or growth deficiency. Children who have only some of the characteristics of FAS (i.e., not enough for a full diagnosis) are often said to have fetal alcohol effects (FAE) or possible fetal alcohol effects (PFAE). These terms, although useful descriptively, have no specific differentiating criteria that would warrant their own diagnoses, even though these conditions can be just as debilitating as FAS. In 1996, the term alcohol-related neurodevelopmental disorder (ARND) was introduced by the Institute of Medicine (IOM, Stratton, Howe, & Battaglia). Focusing on the CNS characteristics (rather than on the growth deficiency and characteristic face), ARND is used congruently with FAE in this book. As these CNS effects can be caused by alcohol but are not unique to alcohol, the terms FAE, PFAE, and ARND are not appropriately used in the absence of a prenatal alcohol exposure history.
Research has shown that people with both FAS and those with FAE/PFAE/ARND experience many of the same kinds of problems growing up. Their families and communities also face many of the same kinds of challenges raising them. Therefore, this book frequently refers to these terms in the collective (i.e., FAS/FAE) in tacit recognition of the fact that it is the behavior problems caused by the prenatal damage from alcohol that present the primary challenge to treatment and management.
People with FAS/FAE, like everyone else, have a variety of talents and capabilities. They exhibit a wide range of intellectual levels and functional disabilities that probably reflect differing sites and degrees of prenatal brain damage due to different levels, patterns, and timing of prenatal alcohol exposure and individual differences in mothers and offspring. Despite their wide array of talents and abilities, many people with FAS exhibit some of the same general behavioral characteristics. They are usually trusting (even overly trusting), loving, and naive despite their years. They can also be grumpy, irritable, and rigid. As a result of their prenatal brain damage, they may have difficulty, especially as they mature, in evaluating a situation and using their past experiences to cope with the problems at hand. They seem to need more protection, supervision, and structure for a longer period of life than usual. Individuals with both FAS and FAE also vary widely in their ultimate outcomes and in the number of secondary disabilities that they acquire as they mature. In 1996, research from the University of Washington indicated that these secondary disabilities become more and more observable in people with FAS/FAE as they mature (Streissguth, Barr, Kogan, & Bookstein, 1996). Extremely high rates of mental illness as well as high rates of disrupted school experiences, trouble with the law, and alcohol and other drug problems are alarming. These secondary disabilities can be as debilitating as the primary disability — the brain damage — with which they were born. Many individuals with FAS/FAE need ongoing help across the life span — anything from a protective environment to a trusted friend, spouse, or advocate to help them stay grounded and focused.
Research, however, has also shown that there are risk and protective factors associated with these secondary disabilities. In other words, there are things that families and communities can do that can truly make a difference in what happens to children with FAS/FAE as they mature. But orchestrating these (i.e., mobilizing the protective factors and diminishing the risk factors) hinges on understanding the cause of the child's problems (i.e., getting a diagnosis).
PROBLEMS WITH GETTING A DIAGNOSIS
A diagnostic evaluation is the starting point for understanding, treating, and managing any medical condition. Yet, surprisingly, despite 2 decades of research and clinical observations, it remains difficult for families to find diagnosticians and clinicians specializing in FAS/FAE. An appropriate diagnosis and an understanding of that diagnosis can help families set realistic expectations, plan for some successes in their children's lives, and build a network of support and structure (see the discussion of an advocacy network in Part III). Diagnostic information about FAS not only helps people to better understand the syndrome's accompanying challenges but also facilitates appropriate treatment, intervention, and planning.
Experienced parents and teachers know that the everyday behaviors of children with FAS/FAE can be "unexpected," "perplexing," and even "unusual." Yet, because these particular behaviors have not yet been clearly linked to structural anomalies in the human brain and because they have not yet been described in the authoritative listing of mental disorders (Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition [DSM-IV], American Psychiatric Association [APA], 1994), many professionals do not yet have the nomenclature to fully understand the syndrome and its implications or really "hear" what parents are saying. Although the field has grown dramatically since the mid-1980s, much confusion still exists, and there remains a gap between scientific knowledge and general clinical information. Often, parents have had to advocate for children whom they suspected had been prenatally affected by alcohol — searching out diagnosticians willing to diagnose this often unheard-of condition, searching out teachers willing to be instructed in appropriate educational methods, and searching out mental health and social services professionals willing to offer their children the extra support and understanding their condition requires.
As I was packing for an extended trip, the frantic mother of a 16-year-old boy with FAS called. She and her husband had just returned from a parent conference with Max's school psychologist. According to her report, the psychologist had indeed heard of FAS, but he thought that children with FAS simply had mental retardation. Therefore, he concluded after examining this adolescent that Max couldn't possibly have FAS because with a performance IQ well into the normal range and a verbal IQ at the lower end of normal, he clearly wasn't mentally retarded. Furthermore, the psychologist was positive that Max had only attention-deficit/hyperactivity disorder and a conduct disorder and warned his parents against putting labels such as FAS on him because they would thwart his development.
The mother begged me to give Max some test that would prove that their son had the brain damage associated with FAS, so that he could get the help and support they knew he needed. Although we did work out a plan, her quest has no easy solution. The tests have not yet been developed, and the research has not yet been done. Understanding the personal consequences of FAS/FAE on children and families and on society at large should accelerate both prevention and intervention efforts once the magnitude of the problem is recognized.
INCIDENCE OF FAS
It is important to outline what is known about prenatal alcohol exposure, which causes FAS/FAE. Alcohol is a teratogen (i.e., any agent or chemical that causes a birth defect). In fact, alcohol is the most frequently ingested teratogen in the world. Alcohol is an addictive but entirely legal drug. According to the Assistant Secretary of Health and Human Services (National Institute on Alcohol Abuse and Alcoholism [NIAAA], 1990), more than 10 million adults in the United States are addicted to alcohol. An additional 7 million abuse alcohol but are not addicted. Many of these 17 million are women in their childbearing years.
Thousands of experiments with laboratory animals have shown conclusively that alcohol can cause birth defects in almost any species, even when it is not coupled with other drug use. Among humans, prenatal alcohol exposure can and does affect children of all races and socioeconomic backgrounds. Researchers at the IOM (1996) estimate that between 0.5 and 3.0 of every 1,000 infants are born with some degree of FAS. (For additional information, see Cordero, Floyd, Martin, Davis, & Hymbaugh, 1994; Dehaene, 1995; Dehaene et al., 1991; May, Hymbaugh, Aase, & Samet, 1983; NIAAA, 1990.) If this estimate is accurate, 2,000'12,000 of the projected 4 million children born in the United States each year will have FAS.
The most accurate estimates of incidence come from a small number of studies that used expert diagnosticians. Large variations can exist from one study population to another, depending on the rate of maternal alcohol abuse and the study design. The NIAAA (1990) estimated that 1.9 in every 1,000 children are born with FAS or ARND, a rate that is nearly double the incidence of Down syndrome and almost five times that of spina bifida, two of the most commonly recognized birth defects. The NIAAA (1990) also estimated that FAE (as a clinical categorization) occurs three times more often than FAS.
On some Indian reservations, where alcohol abuse is common among women, FAS has been reported in 1 in 100 children (May et al., 1983). In one small Native American community, the incidence of FAS was 1 in 8 (Robinson, Conry, & Conry, 1987). At that frequency, FAS is a community catastrophe that threatens to wipe out any culture in just a few generations. However, FAS is not a Native American problem or a problem of poverty per se. It is an alcohol problem, and it is our problem.
Because it is caused by alcohol, FAS is completely preventable. Yet, it continues to swell the ranks of people with mental retardation. In fact, FAS is the most common known cause of mental retardation (NIAAA, 1987). In one large study conducted throughout Sweden, more cases of mental retardation resulted from alcohol-related birth defects than from all known genetic causes of mental retardation combined (Hagberg, Hagberg, Lewerth, & Lindberg, 1981).
PROBLEMS WITH STUDYING FAS POPULATIONS
Studying children affected by alcohol involves special problems. Alcohol is a legal drug, openly sold and advertised throughout the United States. Alcohol is primarily a social drug. It is used in American society to celebrate or commemorate every occasion from marriage to death. Just as the alcoholic often uses denial to ward off awareness of impending alcoholism, society seems to use denial to avoid facing the fact that alcohol can be damaging to the next generation. It is more comfortable and easier to wage a war on illegal drugs than to face the not-so-hidden dangers that alcoholic beverages pose to the fetus.
To further complicate the issue, infants and children with FAS/FAE often appear physically quite "normal," which can prevent them from obtaining the help and services they need. Unlike many birth defects, which are identified at birth and often treated surgically, FAS and FAE are usually overlooked at birth and treated later by community professionals — often unknowingly. Because damage to the brain can occur at lower doses of alcohol than those that produce gross physical anomalies or low birth weights, the brain is the organ in the body most vulnerable to the effects of prenatal alcohol. Unfortunately, we don't yet have the technology to see the brain in living people in a manner that distinguishes the subtle disruptions that shape our misperceptions and guide our maladaptive responses.
In fact, the individual features of FAS are subtle enough that many people with this birth defect pass through life undiagnosed. Certainly, most occurrences of FAS are not recorded on birth certificates, making it useless to try to obtain these data from the usual birth defects surveillance registries that were set up to measure major congenital defects (e.g., club foot, cleft lip or palate). Consider for a moment the difficulty in detecting these subtle features in a newborn in the midst of a busy urban obstetric practice, especially when there is little time to talk with mothers about their drinking. Little and colleagues (1990) have shown a 100% failure rate in detecting FAS in one of the largest maternity hospitals in the country. Unless occurrences of FAS/FAE are associated with gross physical malformations, they often remain undetected in the newborn baby.
FAS and FAE are usually "hidden" birth defects that primarily affect the brain, conditions about which neither medical science nor society has much understanding beyond the general awareness that heavy prenatal alcohol use is bad for the outcome of pregnancy. For example, the 1990 National Health Survey of more than 13,000 representative respondents found that although 89%'92% of all women knew that heavy drinking during pregnancy could increase the chances of miscarriages, mental retardation, low birth weight, and birth defects, only 29% of women of childbearing age could correctly identify FAS as a type of birth defect. Most thought it meant being born addicted to alcohol (Dufour, Williams, Campbell, & Aitken, 1994).
The effects of FAS are difficult to fathom — subtle disruptions in the proliferation and migration of the brain cells that provide the architecture for later problem solving and subtle deviations in the neurochemical balance that permits the transport of messages from one part of the brain to another. When these processes are disrupted, it is difficult to store, retrieve, and transform past experiences into knowledge in order to modify future behaviors — a source of great frustration for those with FAS and a cause of dysfunctional and maladaptive behaviors.
Dysfunctional behavior is difficult to treat effectively, even when its causes are understood. When dysfunctional behavior occurs in children with undiagnosed FAS/FAE, the cause remains unknown and the children's behavior remains an enigma. As a result, the problem behaviors of people with alcohol-related birth defects are being regularly dealt with in schools, community mental health centers, and alcohol and other drug abuse treatment programs as well as through juvenile justice and adult corrections facilities. By failing to diagnose these people as having FAS/FAE and by failing to understand that their unpredictable and often bizarre behaviors stem from the organic brain damage with which they were born, human services providers run the risk of actually causing more of the problem behaviors they are trying to ameliorate.
Much research is needed, particularly on recommended practices for treatment, education, job training, and management; policy change is also needed to reduce barriers to existing services that people with FAS/FAE need but for which they often do not qualify. Every dollar spent on education, prevention, and intervention will reap benefits in future savings. Once the problem is fully understood, effective action is possible. Already, much is known that can benefit the lives of people with FAS/FAE and their families; there is no need for families or communities to wait another minute before putting this knowledge into action. This book seeks to provide the understanding necessary for effective action.
Excerpted from chapter 1 of Fetal Alcohol Syndrome: A Guide for Families and Communities, by Ann Streissguth, Ph.D.
Copyright © 1997 by Paul H. Brookes Publishing Co. All rights reserved. No part of this excerpt may be reproduced or reprinted without permission in writing from the publisher.