Do We Need the Term "FAE"?

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16. American Academy of Pediatrics Committee on Drugs. Guidelines for monitoring and management of pediatric patients during and after sedation for diagnostic and therapeutic procedures. Pediatrics. 1992;89: 1110-1115

428 COMMENTARIES

like a lollipop. However, the candy matrix of the Oralet was attached to a stick-handle as a safety precaution! If a patient becomes sleepy before a dose

is completed, the Oralet can be easily removed from the child’s mouth by the stick-handle. Oralet will be kept in locked narcotic cabinets in hospitals and will not be available in pharmacies. Children will have no opportunity to overdose on it inadvertently.

Finally, like all procedures performed under seda-tion, rigorous monitoring as described in the

Amen-ican Academy of Pediatrics conscious sedation

guidelines must be strictly employed.’6 At a mini-mum this requires individuals who are specifically trained in using this drug and in airway manage-ment.’6 Additionally, all patients will need to be monitored (pulse oximetry as a minimum) before, during, and after the procedure, indeed, until

con-sciousness returns to baseline conditions. Further-more, the company that will be marketing this drug has placed stringent criteria on the settings in which the fentanyl Onalet can be used.

Hopefully, the release of this drug heralds the beginning of a new era in pediatric pain manage-ment in which drugs that are specifically designed for and tested in children will be routinely used to alleviate or eliminate pain. Our patients deserve no less.

MYRON YASTER, MD

The Johns Hopkins Hospital Division of Pediatric Anesthesia Baltimore, MD 21287

REFERENCES

1. Schechter NL. The undertreatment of pain in children: an overview. Pediatr Cli,, Norti, Am. 1989;36:781-794

2. Beyer JE, DeGood DE, Ashley LC, Russell GA. Patterns of postoperative analgesic use with adults and children following cardiac surgery. Pain. 1983;17:71-81

3. Schechter NL, Allen DA, Hanson K. Status of pediatric pain control: a comparison of hospital analgesic usage in children and adults.

Pediatrics. 1986;77:1 1-15

4. Zeltzer LK, Jay SM. Fisher DM. The management of pain associated with pediatric procedures. Pediatr Cliii North Am. 1989;36:941-964 5. Zeltzer LK, Altman A, Cohen D, LeBaron 5, Munuksela EL, Schechter

NL. American Academy of Pediatrics Report of the Subcommittee on the Management of Pain Associated with Procedures in Children with Cancer. Pediatrics. 1990;86:826-831

6. McGrath PA, de Veber LL. The management of acute pain evoked by medical procedures in children with cancer. IPain Symptom Manage. 1986;1 :145-150

7. Anand KJ, Carr DB. The neuroanatomy, neurophysiology, and neuro-chemistry of pain, stress, and analgesia in newborns and children. Pediatr Cli,, North Am. 1989;36:795-822

8. Maxwell LG, Yaster M, Wetzel RC, Niebyl JR. Penile nerve block for newborn circumcision. Obstet Gynecol. 1987;70:415-419

9. Beyer JE, Wells N. The assessment of pain in children. Pediatr Clin North Am. 1989;36:837-854

10. Cote CJ. Sedation for the pediatric patient. A review. Pediatr Clin North Am. 1994;41:31-58

11. Smith C, Rowe RD, VIad P. Sedation of children for cardiac catheter-ization with an ataractic mixture. Can Anaes Soc J.1958;5:35-40

12. Mitchell AA, Louik C, Lacouture P. Slone D, Goldman P. Shapiro S. Risks to children from computed tomographic scan premedication.

JAMA. 1982;247:2385-2388

13. Rosen DA, Rosen KR. A palatable gelatin vehicle for midazolam and ketamine. ILetterl. Anesthesiology. 1991 ;75:914-91 5

14. Weldon BC, Watcha MF, White PF. Oral midazolam in children: effect of time and adjunctive therapy. ISee commentsl. Anesth Analg. 1992;75: 51-55

15. Darling HS, Lambert GH. Therapeutic orphans. ILetter; response]. Pc-diatrics. 1992;89:689

Do

We

Need

the

Term

“FAE”?

ABBREVIATIONS. FAS, fetal alcohol syndrome; FAE, fetal alco-hol effect.

Fetal alcohol syndrome (FAS) was first recognized

as a distinct clinical entity by Jones and Smith in 1973.’ In their first reports,2’3 all affected children had been born to severely alcoholic women, and had in common problems in three major categories:

1. prenatal and/on postnatal growth deficiency; 2. abnormal brain function reflected in mental deficit;

and

3. a distinctive pattern of mild facial dysmorphology.

Later psychological studies revealed a pattern of

behavioral aberration, which is quite common in

af-fected children, but has not been shown to be unique to FAS.4’

As is usually the case with newly described din-ical syndromes, diagnosticians soon began to

real-ize that they were encountering children with

some, but not all the classical signs of FAS. Typi-cally, the maternal history indicated moderate to

severe gestational alcohol abuse and the child

showed developmental delay and behavioral

ab-normalities, but the characteristic facial anomalies

were absent and growth and development were

variably affected. Because a diagnosis of FAS de-manded the presence of all three primary diagnos-tic criteria, (growth deficiency, CNS dysfunction, and physical characteristics)6 a term was needed to refer to children with what seemed to be form fruste FAS, and references to “suspected fetal alcohol effects” began to appear in the literature.79 This

was not intended to be a diagnosis, but only a

“bookmark,” suggesting that the abnormalities

seen in the child were compatible with those

caused by prenatal alcohol exposure, but that the pattern was not sufficiently complete to permit definition of FAS.

Unfortunately, within a few years after its intro-duction, the designation fetal alcohol effect (FAE) began to be applied more or less indiscriminately to children with a variety of problems, even those with simple growth deficiency on isolated behavioral

ab-erration, based almost entirely on the knowledge (or suspicion) that their mothers drank alcohol during pregnancy. Not only clinicians, but concerned

teach-ens, social workers, and foster parents, seeking

expla-Received for publication Feb 4, 1994; accepted Jul 8, 1994.

at Viet Nam:AAP Sponsored on September 1, 2020

www.aappublications.org/news

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COMMENTARIES 429 nations for the problems in children under their care,

seized upon the “diagnosis” of FAE. Some health agencies followed suit, accepting FAE as a medical condition making a child eligible for financial assis-tance and educational intervention. In the absence of definitive laboratory tests or other means of confirm-ing that in utero alcohol exposure is the cause of the child’s problems, the “diagnosis” of FAE can be nei-then proven nor refuted, yet it is used to make deci-sions ranging from insurance coverage to sentencing for capital crime.

Although the general criteria for diagnosing FAS

have been clearly delineated and are widely

ad-cepted, clinicians and investigators remain frustrated by the lack of a meaningful definition of FAE. The original Fetal Alcohol Study Group of the Research Society on Alcohol in their 1980 report suggested

that FAE encompassed “Any conditions thought to

be secondary to alcohol exposure in utero.”1#{176}Clearly, such a definition allows for wide divergence in in-terpretation and has little relevance in the clinical setting. In fact, the known “effects” of prenatal alco-hol exposure are those making up FAS. Each of the individual components is nonspecific, and only their combination with each other allows definition of the diagnosis.

For example, the diagnostic physical features of FAS all are minor anomalies or structural variants

that can be found as isolated characteristics in

normal individuals and families. Each of these

nonspecific variants fits the criteria for polygenic (or multifactorial) inheritance: they show a wide spectrum of variability and occur with higher fre-quency in more closely related individuals, but do not follow a classic Mendelian inheritance pattern. The significance of such minor physical features lies in their association with one another to form a recognizable pattern that helps define a specific

syndrome. The dysmorphic characteristics seen in

FAS, when combined with growth and mental/

behavioral aberrations, paints a unique picture that has been reported only in children prenatally

exposed to alcohol. No such consistent pattern

exists for FAE.

The term and concept of FAE does have validity in one application; in human population studies and animal research in which the independent variable is maternal alcohol consumption during pregnancy. If measurable differences can be found when a group of offspring with documented prenatal alcohol expo-sure is compared with an otherwise identical but unexposed population, it is justifiable to suggest that alcohol caused that difference. Important contribu-tions have been made using these techniques in the areas of growth”2 and intellectual development.13 It must be emphasized, however, that such effects rep-resent statistical correlations and not final proof of alcohol as the causative agent.

The evaluation of a specific patient in the clinical setting is quite a different matter. The greatest concern centers on individuals with behavioral dif-ficulties and learning disorders. Hyperactive chil-dren and adolescents with conduct disorders often

are suspected of FAE, even in the absence of

knowledge about maternal alcohol intake, because such behavior forms a part of FAS. As yet, how-ever, a specific psychological/behavioral

pheno-type unique to those prenatally exposed to alcohol

has not been defined, and attribution of the aber-rant conduct to in utero alcohol exposure remains problematic.

Several unfortunate consequences may result from inappropriately using the term FAE:

I. Presupposition that alcohol is the major (or only) cause of the child’s problems may end the search for other possible causes such as psychosocial deprivation and abuse;

2. Educators and care providers may base their ex-pectations for the child’s performance on that of children with FAS;

3. Women are stigmatized for having damaged

their children by drinking during pregnancy when it is by no means certain that they have done so;

4. Clinicians become frustrated by the imprecision of the “diagnosis” of FAE and thus disregard any possible contribution of alcohol exposure to their patients’ problems; and

5. Efforts to learn the real magnitude of the problem

of prenatal alcohol damage are frustrated by

overdiagnosis.

On the other hand, foster and adoptive parents

have raised the objection that without a “diagnosis” such as FAE, a child may not quality for special education programs, Social Security payments, and other benefits. Some state and local social service agencies do indeed require a medical diagnosis from a specified list as justification for provision of ser-vices. We believe that it is unfair that a mother must be stigmatized to obtain services for her child; such decisions should be based on demonstrated patient need, rather than arbitrary categories of diagnosis. The results of specific physical, psychological, and behavioral testing form the best basis for such deci-sions, and pediatricians armed with this information can be effective advocates for their patients in help-ing to obtain appropriate services as well as helping

change the present inadequate qualification

categories.

We propose abandoning the clinical use of the

term fetal alcohol effect with its implications of cau-sation, and urge simple recording of the verifiable conclusions concerning the individual patient. For

patients referred because of suspicion of FAS, a

three-axis scheme seems appropriate. The first entry refers to exposure status, the second to presenting problems, and the third to definitive diagnosis. If prenatal alcohol exposure has taken place, but FAS cannot be substantiated, the exposure still should be

indicated, and any nonspecific abnormalities or

problems noted.

Thus, the problem list for a growth-deficient new-born with no other physical stigmata whose mother admits to drinking during pregnancy would read: 1) prenatal alcohol exposure; 2) intrauterine growth re-tardation; and 3) diagnosis deferred.

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430 COMMENTARIES

In a similar fashion, an adolescent for whom

gestational alcohol exposure is suspected but not

confirmed might have a problem list reading: 1)

questionable prenatal alcohol exposure; 2) normal growth, microcephaly, and learning deficits; and 3) inadequate evidence to define the possible

contribu-tion of alcohol to these problems.

Obviously, if information later came to light that would add further specificity, the “diagnosis” could be changed as needed.

This paper is simply a call for accuracy and a more conservative approach to diagnostic terminology. A diagnosis that implies causation should not be ap-plied unless the relationship can be proven. Until it is known which features in individuals prenatally ex-posed to alcohol are uniformly and exclusively caused by that exposure, we suggest reporting only objective descriptors unless the “full” fetal alcohol syndrome can be confirmed.

JON M. AsE, MD Department of Pediatrics

University of New Mexico School of Medicine Albuquerque, NM

KENNETH L. JONES,MD Department of Pediatrics

University of California School of Medicine San Diego, CA

STERLING K. CLARREN, MD

Department of Pediatrics

University of Washington School of Medicine Seattle, WA

REFERENCES

1. Jones KL, Smith DW, Ulleland CN, Streissguth AP. Pattern of malfor-mation in offspring of chronic alcoholic mothers. Lancet. 1973;1:

1267-1271

2. Jones KL, Smith DW. Recognition of the fetal alcohol syndrome in early infancy. L.ancet. 1973;2:999-1001

3. Jones KL, Smith DW, Streissguth #{192}Y,Myrianthopoulos NC. Outcome in offspring of chronic alcoholic women. Lancet. 1974;1:1076-1078

4. Streissguth AP. The behavioral teratology of alcohol: performance, be-havioral and intellectual deficits in prenatally exposed children. In: West J, ed. Alcohol and Brain Development. New York, NY: Oxford University Press, mc; 1986:3-44

5. Streissguth AP, Barr HM, Sampson PD. Alcohol use during pregnancy and child development: a longitudinal, prospective study of human behavioral teratology. In: Greenbaum CW, Auerbach JG, eds.

Longitu-dma! Studies of Children at Psychological Risk: Cross-National Perspectives.

Norwood, NJ: Ablex; 1992:174-200

6. Sokol RJ, Clarren SK. Guidelines for use of terminology describing the impact of prenatal alcohol on the offspring. Alcohol dim Exp Res. 1989; 13:597-598

7. Clarren SK, Smith DW. The fetal alcohol syndrome. N Engl I Med.

1978;298:1063-1067

8. Smith DW. Fetal drug syndromes: effects of ethanol and hydantoins.

Pediatr Rev. 1979;1:165-172

9. Smith DW. Fetal alcohol syndrome and fetal alcohol effects. Neurobehav

Toxicol Teratol 19813:127

10. Rosett I-IL. A clinical perspective of the fetal alcohol syndrome. Alcohol

ClinExp Res. 1980;13:118

11. Kyllerman M, Aronson M, Sabel KG, Karlberg E, Sandin B, Olegard R. Children of alcoholic mothers (growth and motor performance com-pared to matched controls). Acta Paediatr Scand. 1985;74:20-26

12. Day NL, Robles N, Richardson G, et al. The effects of prenatal alcohol use on the growth of children at three years of age. Alcohol dim Exp Rca. 1991;15:67-71

13. West JR, ed. Alcohol and Brain Development. New York, NY: Oxford University Press; 1986

Sudden

Infant

Death

Syndrome

and

Subsequent

Siblings

ABBREVIATION. SIDS, sudden infant death syndrome.

Major uncertainties still exist in regard to

mecha-nisms for sudden infant death syndrome (SIDS) and proposed strategies for prospective identification

and intervention. Beginning in part with the report

by Steinschneider in 1972,1 the “apnea hypothesis” stimulated extensive SIDS research efforts, and home

monitors were advocated for the prevention of SIDS. A 1994 report, however,2’ recounted the indictment of Waneta E. Hoyt for the murder of her two children who were included in the Steinschneider report as illustrative of the apnea hypothesis for SIDS and indicative of a biological risk for SIDS in subsequent siblings. A report in Science concluded that a guilty verdict will render invalid the hypothesis of a

con-genital abnormality in breathing control causing

SIDS and further suggested that Steinschneider erred in not considering these infants victims of fatal child abuse (filicide). This conclusion, however, is incon-sistent with the extensive medical/scientific research

accomplishments related to SIDS that have been

achieved during the past 22 years.

The new study by Steinschneider et al4 concludes that documented monitoring in subsequent siblings of prior SIDS victims results in a lower total cost/ patient for home monitoring. To understand the im-portance of this report and place it in the appropriate perspective, however, we need to first review our current knowledge of SIDS pathophysiology, risk

Received for publication Sep 16, 1994; accepted Sep 19, 1994.

Reprint requests to (C.E.H.) Dept of Pediatrics, MedicalCollege of Ohio, P0 Box 10008, Toledo, OH 43699-0008.

The CHIME Steering Committee consists of: Terry M. Baird, MD, Dept of Pediatrics, Case Western Reserve University, Cleveland, OH; LeeJ. Brooks, MD, Sleep Disorders Center, Rainbow Babies and Children’s Hospital, Cleveland, OH; Theodore dolton, ScD, Boston University School of Public Health, Boston, MB; Michael J. Corwin, MD, MPH, Dept of Pediatrics, Boston University MedicalCenter, Boston City Hospital, Boston, MB; David Crowell, PhD, Dept of Psychiatry, Kapiolani Medical Center for Women and Children, Honolulu, HI; Sally L. Davidson Ward, MD, Div of Neona-tology and Pediatric Pulmonology, Childrens Hospital of Los Angeles, Los Angeles, CA; Toke T. Hoppenbrouwers, PhD, Dept of Pediatrics, LAd and USC Medical Center Women’s Hospital, Los Angeles, CA; David R. Huf-ford, Jr. MD, Dept of Pediatrics, Pediatric Pulmonary Center, The Toledo Hospital, Toledo, OH; Carl E. Hunt, MD, Dept of Pediatrics, Medical College of Ohio, Toledo, OH; Thomas C. Keens, MD, Div of Neonatology and Pulmonology, Children’s Hospital of Los Angeles, Los Angeles, CA; George Lister, MD, Dept of Pediatrics, Yale University School of Medicine, New Haven, CT; Michael R. Neuman, MD, PhD, Dept of Obstetrics and Gynecology, CWRU/MetroHealth Medical Center, Cleveland, OH; Ran-gasamy Ramanathan, MD, University of Southern California, Women’s Hospital Medical Center, Los Angeles, CA; Susan Schafer, NICHD Preg-nancy & Perinatology Branch Study Coordinator, National Institutes of Health, Bethesda, MD; Jean M. Silvestri, MD, Dept of Pediatrics, Rush-Presbyterian-St. Luke’s Medical Center, Chicago, IL; Larry Tinsley, MD, Dept of Pediatrics, Kapiolani Medical Center for Women and Children, Honolulu, HI; Debra W. Weese-Mayer, MD, Center for SIDS Research and Disorders of Respiratory Control, Rush-Presbyterian-St. Luke’s Medical Center, Chicago, IL; and Manan Willinger, MD, Pregnancy &Perinatology Branch, CRMC, NICHD, NIH, Bldg 6100, Rockville, MD.

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1995;95;428

Pediatrics

Jon M. Aase, Kenneth L. Jones and Sterling K. Clarren