Fetal Alcohol Syndrome
Fetal alcohol syndrome (FAS) is a disorder that can occur to the embryo when a pregnant woman ingests alcohol during pregnancy. An ingestion of alcohol does not always result in FAS. The current recommendation of both the US Surgeon General and the UK Department of Health is not to drink alcohol at all during pregnancy.
Alcohol crosses the placental barrier and can stunt fetal growth or weight, create distinctive facial stigmata, damage neurons and brain structures, and cause other physical, mental, or behavioral problems.
Surveys found that in the United States, 10-15% of pregnant women admit to having recently used alcohol, and up to 30% use alcohol at some point during pregnancy.
The main effect of FAS is permanent central nervous system damage, especially to the brain. Developing brain cells and structures are underdeveloped or malformed by prenatal alcohol exposure, often creating an array of primary cognitive and functional disabilities (including poor memory, attention deficits, impulsive behavior, and poor cause-effect reasoning) as well as secondary disabilities (for example, mental health problems, and drug addiction). The risk of brain damage exists during each trimester, since the fetal brain develops throughout the entire pregnancy.
Fetal alcohol exposure is the leading known cause of mental retardation in the Western world. In the United States the FAS prevalence rate is estimated to be between 0.2 and 2.0 cases per 1,000 live births, comparable to or higher than other developmental disabilities such as Down syndrome or spina bifida.
Fetal Alcohol Syndrome Symptoms
Growth deficiency
Growth deficiency is defined as significantly below average height, weight or both due to prenatal alcohol exposure, and can be assessed at any point in the lifespan. Growth measurements must be adjusted for parental height, gestational age (for a premature infant), and other postnatal insults (e.g., poor nutrition), although birth height and weight are the preferred measurements.
The CDC and Canadian guidelines use the 10th percentile as a cut-off to determine growth deficiency. The presence of FAS facial features indicates brain damage, though brain damage may also exist in their absence. FAS facial features (and most other visible, but non-diagnostic, deformities) are believed to be caused mainly during the 10th and 20th week of gestation.
Refinements in diagnostic criteria since 1975 have yielded three distinctive and diagnostically significant facial features known to result from prenatal alcohol exposure and distinguishes FAS from other disorders with partially overlapping characteristics. The three FAS facial features are:
- A smooth philtrum — The divot or groove between the nose and upper lip flattens with increased prenatal alcohol exposure.
- Thin vermilion — The upper lip thins with increased prenatal alcohol exposure.
- Small palpebral fissures — Eye width decreases with increased prenatal alcohol exposure.
Measurement of FAS facial features uses criteria developed by the University of Washington. The lip and philtrum are measured by a trained physician with the Lip-Philtrum Guide, a 5-point Likert Scale with representative photographs of lip and philtrum combinations ranging from normal (ranked 1) to severe (ranked 5). Palpebral fissure length (PFL) is measured in millimeters with either calipers or a clear ruler and then compared to a PFL growth chart, also developed by the University of Washington.
Ranking FAS facial features is complicated because the three separate facial features can be affected independently by prenatal alcohol. A summary of the criteria follows:
- Severe — All three facial features ranked independently as severe (lip ranked at 4 or 5, philtrum ranked at 4 or 5, and PFL two or more standard deviations below average).
- Moderate — Two facial features ranked as severe and one feature ranked as moderate (lip ''or'' philtrum ranked at 3, ''or'' PFL between one and two standard deviations below average).
- Mild — A mild ranking of FAS facial features covers a broad range of facial feature combinations:
- Two facial features ranked severe and one ranked within normal limits,
- One facial feature ranked severe and two ranked moderate, or
- One facial feature ranked severe, one ranked moderate and one ranked within normal limits.
- None — All three facial features ranked within normal limits.
These distinctive facial features in a patient do strongly correlate to brain damage. Sterling Clarren of the University of Washington's Fetal Alcohol and Drug Unit told a conference in 2002:
I have never seen anybody with this whole face who doesn't have some brain damage. In fact in studies, as the face is more FAS-like, the brain is more likely to be abnormal. The only face that you would want to counsel people or predict the future about is the full FAS face. But the risk of brain damage increases as the eyes get smaller, as the philtrum gets flatter, and the lip gets thinner. The risk goes up but not the diagnosis.
At one-month gestation, the top end of your body is a brain, and at the very front end of that early brain, there is tissue that has been brain tissue. It stops being brain and gets ready to be your face ... Your eyeball is also brain tissue. It's an extension of the second part of the brain. It started as brain and "popped out." So if you are going to look at parts of the brain from alcohol damage, or any kind of damage during pregnancy, eye malformations and midline facial malformations are going to be very actively related to the brain across syndromes ... and they certainly are with FAS.
Central nervous system damage
Central nervous system (CNS) damage is the primary feature of any FASD diagnosis. Prenatal alcohol exposure, a teratogen, can damage the brain across a continuum of gross to subtle impairments, depending on the amount, timing, and frequency of the exposure as well as genetic predispositions of the fetus and mother. While functional abnormalities are the behavioral and cognitive expressions of the FAS disability, CNS damage can be assessed in three areas: structural, neurological, and functional impairments.
All four diagnostic systems allow for assessment of CNS damage in these areas, but criteria vary. The IOM system requires structural or neurological impairment for a diagnosis of FAS. During the third trimester, damage can be caused to the hippocampus, which plays a role in memory, learning, emotion, and encoding visual and auditory information, all of which can create neurological and functional CNS impairments as well.
As of 2002, there were 25 reports of autopsies on infants known to have FAS. The first was in 1973, on an infant who died shortly after birth.
In 1977, Dr. Clarren described a second infant whose mother was a binge drinker. The infant died ten days after birth. The autopsy showed severe hydrocephalus, abnormal neuronal migration, and a small corpus callosum (which connects the two brain hemispheres) and cerebellum.
"The 4-Digit Diagnostic Code" ranking system distinguishes between levels of prenatal alcohol exposure as ''High Risk'' and ''Some Risk''. It operationalizes high risk exposure as a blood alcohol concentration (BAC) greater than 100 mg/dL delivered at least weekly in early pregnancy. This BAC level is typically reached by a 55 kg female drinking six to eight beers in one sitting. (which may cause light sensitivity, decreased visual acuity, or involuntary eye movements).
- Occasional abnormalities — Ptosis of the eyelid, microophthalmia, cleft lip with or without a cleft palate, webbed neck, short neck, Tetralogy of Fallot, coarctation of the aorta, Spina bifida, and hydrocephalus.
Fetal Alcohol Syndrome Diagnosis
Several diagnostic systems have been developed in North America:
- The Institute of Medicine's guidelines for FAS, the first system to standardize diagnoses of individuals with prenatal alcohol exposure,
- The University of Washington's "The 4-Digit Diagnostic Code," which ranks the four key features of FASD on a Likert scale of one to four and yields 256 descriptive codes that can be categorized into 22 distinct clinical categories, ranging from FAS to no findings.
- The Centers for Disease Control's "Fetal Alcohol Syndrome: Guidelines for Referral and Diagnosis," which established general consensus on the diagnosis FAS in the U.S. but deferred addressing other FASD conditions, and
- Canadian guidelines for FASD diagnosis, which established criteria for diagnosing FASD in Canada and harmonized most differences between the IOM and University of Washington's systems.
Fetal alcohol syndrome is the only expression of FASD that has garnered consensus among experts to become an official ICD-9 and ICD-10 diagnosis. To make this diagnosis (or determine any FASD condition), a multi-disciplinary evaluation is necessary to assess each of the four key features for assessment. Generally, a trained physician will determine growth deficiency and FAS facial features. While a qualified physician may also assess central nervous system structural abnormalities and/or neurological problems, usually central nervous system damage is determined through psychological, assessment. A pediatric neuropsychologist may assess all areas of functioning, including intellectual, language processing, and sensorimotor. Prenatal alcohol exposure risk may be assessed by a qualified physician or psychologist.
The following criteria must be fully met for an FAS diagnosis: The Royal College of Obstetricians and Gynaecologists conducted a study of over 400,000 women, all of whom had consumed alcohol during pregnancy. No case of fetal alcohol syndrome occurred and no adverse effects on children were found when consumption was under 8.5 drinks per week. A review of research studies found that fetal alcohol syndrome only occurred among alcoholics; no apparent risk to the child occurred when the pregnant women consumed no more than one drink per day. A study of moderate drinking during pregnancy found no negative effects and the researchers concluded that one drink per day provides a significant margin of safety, although they did not encourage drinking during pregnancy. A study of pregnancies in eight European countries found that consuming no more than one drink per day did not appear to have any effect on fetal growth. A follow-up of children at 18 months of age found that those from women who drank during pregnancy, even two drinks per day, scored higher in several areas of development. An analysis of seven medical research studies involving over 130,000 pregnancies found that consuming two to 14 drinks per week did not increase the risk of giving birth to a child with either malformations or fetal alcohol syndrome.
In the United States, the Surgeon General recommended in 1981, and again in 2005, that women abstain from alcohol use while pregnant or while planning a pregnancy, the latter to avoid damage in the earliest stages of a pregnancy, as the woman may not be aware that she has conceived. While these elements are important to consider when working with FAS and have some usefulness in treatment, they are not alone sufficient to promote better outcomes.
An understanding of the developmental framework would presumably inform and enhance the advocacy model, but advocacy also implies interventions at a systems level as well, such as educating schools, social workers, and so forth on best practices for FAS. However, several organizations devoted to FAS also use the advocacy model at a community practice level as well.
Neurobehavioral approach
The neurobehavioral approach focuses on the neurological underpinnings from which behaviors and cognitive processes arise. The idea to ''try differently'' refers to trying different perspectives and intervention options based on effects of the CNS damage and particular needs of the patient, rather than ''trying harder'' at implementing behavioral-based interventions that have consistently failed over time to produce improved outcomes for a patient. This approach also encourages more strength-based interventions, which allow a patient to develop positive outcomes by promoting success linked to the patient's strengths and interests. (rather than a mental health condition, which is considered a secondary disability).
The exact mechanisms for functional problems of primary disabilities are not always fully understood, but animal studies have begun to shed light on some correlates between functional problems and brain structures damaged by prenatal alcohol exposure.
- Impaired motor development and functioning are associated with reduced size of the cerebellum
- Hyperactivity is associated with decreased size of the corpus callosum
Functional difficulties may result from CNS damage in more than one domain, but common functional difficulties by domain include: (This is not an exhaustive list of difficulties.)
- Achievement — Learning disabilities
- Adaptive behavior — Poor impulse control, poor personal boundaries, poor anger management, stubbornness, intrusive behavior, too friendly with strangers, poor daily living skills, developmental delays
- Attention — Attention-Deficit/Hyperactivity Disorder (ADHD), poor attention or concentration, distractible
- Cognition — Mental retardation, confusion under pressure, poor abstract skills, difficulty distinguishing between fantasy and reality, slower cognitive processing
- Executive functioning — Poor judgment, Information-processing disorder, poor at perceiving patterns, poor cause and effect reasoning, inconsistent at linking words to actions, poor generalization ability
- Language — Expressive or receptive language disorders, grasp parts not whole concepts, lack understanding of metaphor, idioms, or sarcasm
- Memory — Poor short-term memory, inconsistent memory and knowledge base
- Motor skills — Poor handwriting, poor fine motor skills, poor gross motor skills, delayed motor skill development (e.g., riding a bicycle at appropriate age)
- Sensory integration and soft neurological problems — sensory integration dysfunction, sensory defensiveness, undersensitivity to stimulation
- Social communication — Intrude into conversations, inability to read nonverbal or social cues, "chatty" but without substance
Secondary disabilities
The secondary disabilities of FAS are those that arise later in life secondary to CNS damage. These disabilities often emerge over time due to a mismatch between the primary disabilities and environmental expectations; secondary disabilities can be ameliorated with early interventions and appropriate supportive services.
- Music, playing instruments, composing, singing, art, spelling, reading, computers, mechanics, woodworking, skilled vocations (welding, electrician, etc.), writing, poetry
Fetal Alcohol Syndrome History
Historical references
Anecdotal accounts of prohibitions against maternal alcohol use from biblical, ancient Greek, and ancient Roman sources imply a historical awareness of links between maternal alcohol use and negative child outcomes.
In Gaelic Scotland, the mother and nurse were not allowed to consume ale during pregnancy and breastfeeding (Martin Martin).
The earliest recorded observation of possible links between maternal alcohol use and fetal damage was made in 1899 by Dr. William Sullivan, a Liverpool prison physician who noted higher rates of stillbirth for 120 alcoholic female prisoners than their sober female relatives; he suggested the causal agent to be alcohol use. This contradicted the predominating belief at the time that heredity caused mental retardation, poverty, and criminal behavior, which contemporary studies on the subjects usually concluded. though later researchers have suggested that the Kallikaks almost certainly had FAS. General studies and discussions on alcoholism throughout the mid-1900s were typically based on a heredity argument.
Prior to fetal alcohol syndrome being specifically identified and named in 1973, a few studies had noted differences between the children of mothers who used alcohol during pregnancy or breast-feeding and those who did not, but identified alcohol use as a possible contributing factor rather than heredity.
Dr. Paul Lemoine of Nantes, France had already published a study in a French medical journal in 1968 about children with distinctive features whose mothers were alcoholics, Researchers in France, Sweden, and the United States were struck by how similar these children looked, though they were not related, and how they behaved in the same unfocused and hyperactive manner. He reasoned that doing so would encourage prevention, believing that if people knew maternal alcohol consumption caused the syndrome, then abstinence during pregnancy would follow from patient education and public awareness.
Fetal Alcohol Syndrome Education
Common areas of concern in the classroom
- Distractibility
- Problems with concrete thinking
- Easily frustrated
- Poor fine and gross motor skills
- Poor attention
- Lack of organizational skills
- Poor peer relations
Strategies for teachers
- Place the child near the front of the room to help him or her focus.
- Allow the student to take short breaks when necessary.
- Give the child extra time. Allow them enough time to prepare for the next activity, they do not do well rushed.
- Have them perform one task at a time. They struggle with multi-step directions. To make sure they understand, have them repeat the instructions. Walk them through a new activity first.
- Because their handwriting is often poor, a computer may be a better way for them to complete their assignments.
- Math skills are difficult. Using manipulatives makes learning easier.
- Behavior problems become more apparent as children enter grade school. This may result in an outburst or fight.
- Punishment is not always the best answer since FAS/FASD children may not understand why they are being punished. Try defusing the situation as calmly as possible and moving into a new activity.
- Using visuals, concrete examples and hands-on learning makes school easier.
- Encourage success and reward positive behavior with praise or incentives. Positive reinforcement should be immediate to help with understanding.
Children with FAS/FASD can learn, they just need to use different paths to get there. If one technique is not successful, try something new.
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