Fetal Alcohol Spectrum Disorder

1
Fetal Alcohol Spectrum Disorder

• Dr. Brenda Stade, Mary Cunningham,
• Doug Nugent St. Michaels Hospital

2
Outline

• Introduction
• Early Identification and Assessment
• Diagnostic guidelines and assessment
• Screening
• Rational for early diagnosis
• Cognitive, Behavioral, Social Development and
  Nutrition of Children, Birth to Age 6 years
• Issues and Strategies
• Focus on Families

3
Introduction

• In Canada the incidence of Fetal Alcohol Spectrum
  Disorder (FASD) has been estimated to be 1 in 100
  live births.

4
Introduction

• Caused by prenatal exposure to alcohol.
• FASD is the leading cause of developmental and
  cognitive disabilities among Canadian children.

5
Introduction Fetal Alcohol Spectrum Disorder
Defined

• Growth Restriction
• Facial Anomalies
• CNS Dysfunction
• Prenatal Alcohol Exposure

6
Introduction

• Cost of FASD annually to Canada of those 1 to 21
  years old, was 344,208,000 (95 CI 311,664,000
  376,752,000).
• (Stade, 2004).

7
Introduction Etiology

• Alcohol readily crosses the placenta and results
  in similar levels in the mother and fetus.
• Rate of elimination is slower in the fetus.
• Most teratogenic effect during organogenesis and
  development of the nervous system.

8
Etiology

• When neuronal activity is abnormally suppressed
  during the developmental period, the timing and
  sequence of synaptic connections is disrupted,
  and this causes nerve cells to receive an
  internal signal to commit suicide, a form of cell
  death known as "apoptosis".
• Addiction Biology 2004 Jun9(2)137-49.

9
Etiology

• Teratogenesis is grossly dose related, although
  the threshold dose is still unknown and related
  to maternal/fetal susceptibility.
• Risk to fetus greatest with more than 7 standard
  drinks per week (1 standard drink 13.6 grams of
  absolute alcohol).
• Binge drinking of more than 5 ounces (142 grams)
  per occasion vs. 4 or more drinks per occasion.

10
Standard drinks 0.5 oz alcohol
12 oz (341 mL) can of beer (5 alcohol) 12 oz
(341 mL) bottle of cooler (5 alcohol) 5 oz (142
mL) glass of wine (12 alcohol) 1.5 oz (43 mL)
distilled spirits (40 alcohol) 3 oz (85 mL)
fortified wine e.g. sherry or port (18
alcohol )
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Etiology

• No safe time to drink during pregnancy.
• No known safe amount.

12
Risk Factors

• Maternal Age and Parity
• Chronicity of Alcoholism
• Socioeconomic Status
• Polydrug Use
• Ethnicity
• Fetal Susceptibility.

13
Diagnostic Guidelines
14
Important Features of Diagnostic Guidelines

• Minimize false negatives and false positives
• Precisely define diagnostic criteria
• Consider genetic and family histories
• Multidisciplinary approach.

15
Rational for Early Diagnosis

• Accurate and timely diagnosis is essential
• to improve outcomes
• decrease risk of secondary disabilities
• increase opportunities for prevention
• ensure more accurate estimates of incidence and
  prevalence.

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Canadian Guidelines for DiagnosisCMAJ, March 2005

• The Diagnostic Process
• Screening and referral
• Physical exam and differential diagnosis
• Neurobehavioural assessment
• Treatment and follow-up
• Team members
• Program director/Co-ordinator
• Physician (trained in diagnosis)
• Psychologist
• Social worker
• OT, Speech, psychiatrist, geneticist, addiction
  worker, community support workers, teachers etc.

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Canadian Guidelines for Diagnosis

• Physical Exam
• General physical to rule out other disorders
• Growth (at or below 10th percentile)
• Facial features.

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Growth Restriction

• Growth restriction is demonstrated by height and
  weight at or below the tenth (10th) percentile.
• Growth restriction may be apparent prenatally
  and/or postnatally.

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DiagnosisGrowth Restriction
20
Facial Features

• Short palpebral fissures
• Smooth or flat philtrum
• Thin upper lip.

21
Facial Features
22
Facial Features
23
Associated Anomalies

• Cardiac anomalies
• Joint and limb anomalies
• Neurotubal defects
• Anomalies of the urogenital system
• Hearing disorders
• Visual problems
• Severe dental malocclusions.

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Canadian Guidelines for Diagnosis-Neuro-behaviour
al Assessment

• Domains to be assessed by psychologist or team
• Hard and soft neurological signs
• Brain structure
• Cognition (IQ)
• Communication
• Academic achievement
• Memory
• Executive functioning
• Attention deficit/hyperactivity
• Adaptive behaviour, social skills, social
  communication.

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Early Infancy

• Tremors
• Poor suck
• Hypotonic/Hypertonic
• Irritability
• Feeding problems
• Developmental delay.

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Early Childhood

• Cognitive Problems
• Motor Issues
• Behavioral Presentation
• Sensory Dysfunction
• Speech Delay
• Hyperactivity
• Socialization Difficulties.

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Canadian Guidelines for DiagnosisMaternal
Alcohol History in Pregnancy

• Key to establishing an accurate diagnosis
• Require confirmation based on clinical records,
  self-report, reliable observation.

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Classification of FASD

• Fetal Alcohol Syndrome (FAS)
• Partial Fetal Alcohol Syndrome (PFAS) with
  confirmed maternal alcohol exposure
• Alcohol-Related Neuro-Developmental Disorder
  (ARND) with confirmed maternal alcohol exposure.

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Diagnostic Criteria

• FAS
• Evidence of growth impairment
• 3 facial anomalies
• 3 central nervous system domains impaired
• Confirmed or unconfirmed alcohol exposure.

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Diagnostic Criteria

• Partial FAS
• 2 facial anomalies
• 3 central nervous system domains impaired
• Confirmed alcohol exposure.

31
Diagnostic Criteria

• ARND
• 3 central nervous system domains impaired
• Confirmed alcohol exposure.

32
Screening
33
Screening and Primary Care Referral

• Referral of individuals to FASD diagnostic
  clinics
• Evidence of prenatal exposure to alcohol (or
  probable) with suspected or confirmed CNS
  dysfunction or
• Presence of 3 characteristic facial features with
  growth deficits with or without known prenatal
  alcohol exposure.

34
Conclusion

• Diagnosis requires a multi-disciplinary approach
• Diagnosis is complex and guidelines are well
  defined and cannot be a gestalt approach
• Confirmed prenatal alcohol exposure is required
  for a diagnosis of Partial FAS and ARND
• Screening does not equate to diagnosis.

35
Cognitive, Behavioral, Social Development and
Nutrition of Children from Birth to Age 6
36
Cognitive
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Cognition

• Attention problems and memory deficits often make
  learning difficult in the young child.

38
Cognition

• Infants and young children with FASD live with
  differing levels of cognitive abilities
• All programs to develop cognitive abilities
  should be child specific.

39
Cognition

• How does the individual child with FASD
• learn?
• Some are primarily visual learners, some are
  tactile learners, some kinesthetic, and some
  learn best by listening.
• (Mountford,A. The Golden Hoop of Life).

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Cognition Strategies

• If a child learns best through music
• If a child learns through body movement
• If a child learns best through listening
• If a child is a tactile learner
• (Mountford, A. The Golden Hoop of Life).

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Cognition Strategies

• May need to use short sentences
• Break down information and instruction
• Repetition, Repetition, Repetition
• Teach one concept at a time.

42
Cognition Strategies

• It took him four weeks at age four to learn the
  colour red. We decided in February he was going
  to learn his colours. So everyday of the month I
  dressed him in red.
• The teacher had to say X youre wearing a red
  shirt today. Show me your shirt. Its red. X
  youre wearing red pants today. Something had to
  be red.

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Cognition Strategies

• Treasure hunts
• Problem-solving activities
• Visual-spatial games
• Story building
• Math skills visual teaching.

44
Cognition

• Impacting on the development of cognitive
  skills is the childs ability to process his
  sensory world.

45
Sensitivity
46
Sensory Processing

• Many infants and young children
• with FASD have difficulty processing and
  organizing sensory information they receive from
  their own bodies and the outside world.

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Sensory Processing

• Sensory processing is a developmental process
• Takes place in the central nervous system
• Involves ability to take in information
• through the senses, organize it in our brains
  and use it to respond appropriately.

48
Sensory Processing

• The brain must properly process information from
  the senses to develop
• concentration
• organization
• learning ability
• specialization of each side of the body and brain
• self-esteem
• self-control.

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Sensory Processing

• How does sensory processing abilities impact on
  day-to-day life of a child with FASD?

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Normal Sensory Integration

• Schwab, D. (2001).

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Sensory Processing Dysfunction
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Sensory Processing

• Hypersensitive
• Touch (Touch Processing)
• Noise (Auditory Processing
• Visual Input (Visual Processing).
• Dysfunction in Behavioural Outcomes of Sensory
  Processing.

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Sensory Processing Strategies

• Place your child first or last in line
• Wash clothes a couple of times before wearing
• Use soft bedding
• Remove tags from clothes
• Avoid
• ties under the chin
• thick seams in clothing
• clothes that are scratchy
• Avoid tickling.

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Sensory Processing Strategies

• Weighted Vests
• Deep Massage
• Bear Hugs
• Activities using a number of muscles groups.

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Sensory Processing Strategies

• Tone down the rooms effects on all senses
• Avoid decorated rooms
• Walls should be single colour and very pale
• Avoid clutter.

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Sensory Processing Strategies

• Provide a place/space where the child can have a
  quiet place to be
• Avoid crowds and places with many people, lots of
  noise and high activity level
• At daycare, preschool, and school group activity
  should avoid large groups.

57
Sensory Processing Strategies

• Group play use little mats
• Recognize why a child may refuse to participate
  in a game
• Occupational Therapy.

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Sensory Processing

• Hyposensitive
• Pain
• Hot or Cold

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Strategies Hyposensitive

• Supervision
• Avoid overdressing in summer
• Ensure dressed adequately in winter
• Ensure child monitored and receives adequate care
  when ill.

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Behaviours

• Behavioural and Emotional Responses may reflect
  the childs outcomes of sensory processing.

61
Behaviour Hyperactivity

• Due to the childs sensory processing
  difficulties he or she may have a constant need
  for activity.

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Strategies

• Fidget Items
• Short periods of sitting still
• Hammock
• Teaching during activity
• Music.

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Difficult Behaviours

• What is needed is a change in thinking from
  discipline to redirection or re-teaching
• Prevention sensory strategies,
  transitioning.

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Strategies

• Be firm but supportive
• Choose one or two critical behaviors at a time to
  work on
• Ignore minor negative behaviour
• Keep the mood positive. Give five times
• more praise to every one correction.
• Identify warning signs re melt down
• Teach child to self-monitor.

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Difficult Behaviours

• Calming strategies
• Comfort corner
• Tents and caves
• Very short time outs
• Deep pressure.

66
Crying Infancy

• Crying is an infants way of expressing his/her
  needs.
• Infants prenatally exposed to alcohol may seem
  like they are crying constantly.

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Crying Infancy

• Avoid, if you can, letting a baby get to a state
  of frantic crying.
• Get to know strategies that work best, and tell
  other caregivers how the baby likes to be handled.

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Crying Infancy

• Wrap the infant snugly in a receiving blanket
  when not sleeping
• Use a soother
• Bathing may settle some, quiet music may help
  others
• Rocking the infant up and down rather than back
  and forth has been found to be soothing for some
  infants.

69
Crying Young Child

• Crying is a method of communication for all young
  children
• In the child with FASD be alert for
• sensory overload
• inability to communicate
• mood problems.

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Crying Young Child

• Modify environment
• Ensure child can communicate needs pictures,
  sign language
• Assessment by a mental health professional.

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Health Illness
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Health and Illness

• Generally, FASD is not defined by associated
  physical disability or illness.

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Health Illness

• Some children with FASD are born with organ
  anomalies.

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Organ Anomalies

• Cardiac anomalies
• Joint and limb anomalies
• Neurotubal defects
• Anomalies of the urogenital system.
• Hearing disorders
• Visual problems
• Severe dental malocclusions.

75
Health Illness

• Zhang and others (2005) demonstrate the adverse
  effects of alcohol on immune competence and the
  increased vulnerability of ethanol-exposed
  offspring.

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Health Illness

• The infant should not be exposed to environmental
  irritants such as tobacco smoke
• Protect the infant from exposure to viruses.

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Health Illness

• Young children with FASD are particularly prone
  to upper respiratory illnesses and ear infections
• Monitoring and ensure treatment as necessary.

78
Health Illness

• Motor deficits are not uncommon in infants and
  young children with FASD.
• Infant Pre-school stimulation programs
• Occupational Therapy.

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Sleep
80
Sleep Disturbances

• Sleep disturbances among individuals with FASD
  are not uncommon.
• Younger children often have trouble falling
  asleep and waking.

81
Sleep Disturbances

• They may have trouble settling and wake often
  throughout the night.
• Night terrors among individuals with FASD can
  continue throughout life.

82
Sleep Strategies

• Establish rituals for saying good night
• Start a calming bedtime routine an hour before
  bedtime
• A light snack before bed may be beneficial for
  some children.

83
Sleep Strategies

• Decrease sensory stimulation in the bedroom
• White noise when the child is in bed may be
  calming to some but distracting to others
• Night-lights help some young children but for
  some can lead to night terrors.

84
Sleep Strategies

• Start young to promote the child sleeping in his
  or her own bed
• Melatonin may be beneficial
• Childproof the house for night wanderers
• As much as possible wake the child in the same
  predictable way every morning.

85
Nutrition
86
Growth and FAS

• Substantial literature on the association between
  maternal alcohol consumption during pregnancy and
  decreased neonatal weight, length and head
  circumference.
• Unsure of the effect of alcohol on growth
  parameters later on in life.
• McFadyen, K. (2005)

87
Studies Growth and FASD

• Russell (1991)
• Differences in head circumference and ht at 6
  years
• Sampson (1994)
• No detectable differences from 8 mos to 14 years
• Day (2002)
• 1st trimester exposure predicted significant
  reductions in wt, HC, and length
• 2nd trimester exposure predicted significant
  reductions in wt and skinfold thickness.

88
Nutrition and FASD

• Infants and young children with FASD must have
  there growth followed regularly
• Those with poor growth/growth restriction should
  be followed by a dietician
• Motor dysfunction resulting in poor suck and
  swallow requires OT intervention
• Picky eaters requires patience, persistence,
  and imagination.

89
Essential Fatty Acids
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What we know.

• Essential fatty acids (EFA) are necessary for the
  formation of healthy cell membranes, proper
  development and function of the brain and nervous
  system -
• Omega 3 and Omega 6 fatty acids must be provided
  from food as they cannot be synthesized by the
  body.
• McFadyen, K. (2005)

91
ESSENTIAL FATTY ACIDS
OMEGA 3 FATTY ACIDS
OMEGA 6 FATTY ACIDS
Green leafy vegetables, flax, flaxseed oil,
canola oil, walnuts, Brazil nuts, fish oil, fish,
tofu, and eggs
Vegetable oils (soybean, safflower, and corn
oil), nuts and seeds
92
What we know continued

• Some evidence indicates that
• fatty acid deficiencies or imbalances may
  contribute to the negative sequelae of some
  childhood
• neuro-developmental disorders.
• McFadyen, K. (2005)

93
EFA Supplementation

• There have been no studies to date looking at EFA
  supplementation and children with FASD.
• Some studies have demonstrated the benefits of
  EFA in children with other neuro-developmental
  disorders but other research have found no
  effect.

94
Thoughts..

• Pregnancy stresses maternal EFA status because
  the mother must supply fatty acids needed for
  fetal and placental growth.
• Alcohol can disturb placental transport.
• Alcohol increases fatty acid catabolism
  resulting in ???

95
What we do not know.

1. Whether supplementation of essential fatty acids
   may benefit in children with FASD
2. Optimal dosage of fatty acids
3. Optimal composition (Omega 3 and Omega 6 fatty
   acids)
4. Dose response relationship
5. Duration or treatment.

96
In the End

• Encourage the young child with FASD to eat a
  variety of foods from the four food groups.
• To increase intake of EFAs offer fish, eggs,
  nuts, seeds and use vegetable oils.
• Monitor growth.
• McFadyen, K. (2005)

97
Social Skills Friendships
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Social Skills and Friendships

• Social skill development should begin early for
  children with FASD.
• Distractibility, aggressiveness and, and
  impulsivity can interfere with social
  development.

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Social Skills and Friendships

• Social skills program
• Practice, model, rehearse social skills.
• Foster activities that the child likes and is
  good at
• Brief activities in small groups.

100
Social Skills and Friendships

• Invite other children to the home and adapt the
  situation so it is fun for the other children
• Educate young children that they may learn or
  respond to situations or stimuli somewhat
  differently than others.

101
Caregivers
102
Strategies for Caregivers

• Keep remembering they are not willfully trying to
  make you exhausted or crazy
• Forgive yourself when you lose your temper
• Allow yourself to grieve
• Advocate for their needs
• It will make you feel better about them and
  yourself.

103
Strategies for Caregivers

• Do something for yourself every day
• Find someone you can talk to
• Try to get in as many breaks as possible
  friends, family, respite
• Monitor yourself for signs of increased stress
  and depression.

104
Thank-You

• Thank-you for listening!

105
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