Download The Every Issue Rundown - The Royal Children`s Hospital

Aspiration pneumonia/confirming reflux
Involve speech pathologist
(I’d be interested to hear how the SP’s at RCH approach this in kids with severe C.P. e.g.
how the decision for/against fundoplication with PEG insertion is made)
Video fluoroscopy
Oesophageal pH monitoring
“Reflux index” = % time pH <4
Considered most valid measure
Upper limits of normal are 12% (if 12mths or younger), 6% otherwise
Note that abnormal results not highly sensitive nor specific in detecting whether aspiration
pneumonia is related to reflux, but patients with aspiration tend to reflux more frequently
into the proximal oesophagus
Upshot: oesophageal pH monitoring may be useful in combo
Barium contrast radiography
Considered less sensitive and specific than oesophageal pH monitoring for diagnosis of
GORD
Bronchoalveolar lavage
Occasionally used to assess for evidence of recurrent small-volume aspiration (looking for
lipid-laden macrophages)
Obviously invasive
Nuclear scintigraphy
Ingestion of technetium-labeled food or formula followed by scanning to detect the
distribution of the isotope in the stomach, oesophagus, lungs
Is this what a “milk scan” is?
Less sensitive but more specific than oesophageal manometry
Oesophageal manometry
Said to be on minimal use in diagnosis of GORD but may establish major motor disorder
(such as bulbar palsy in CP? – be interested in what the speech paths have to say on this)
Endoscopy (invasive) looking for evidence of oesophagitis from GORD
Problem behaviours
 Over praise
o be specific about what you overpraise
o can add in sticker charts (e.g. 3-6yo) = when they finish filling out the chart, they
get a reward; not allowed to take the stickers away; not to sue it more than 3-5 days
at a time
o for older children, modify this to allow them to negotiate what they want e.g.
o e.g. I really like about the way you pack up the toys
o e.g. I really like the way that you DIDN’T hit the brother
 Dedicated time
o spend 15 minutes each day with the child doing anything that they want to do
 Sleep issues
o Majority have poor sleep
o This will actually help with tackling the behaviour – THIS MAY BE THE FIRST
THING TO DO
o Know the sleep requirements for children
 Neonates 16 hrs
 1yo 14 hrs
 Preschool 12-13 hrs
 School children 11 hrs
o Nap
 Stop naps in 4yo (in Australia, 3yo in America)
o Hx
 Time of sleep
 Where do they sleep
 Quality of sleep (OSA)
o Mx
 Controlled crying (in older kids)
 Introduction of associations
 Reduce sleep association
 Positive reinforcements – sleep sticky charts (e.g. listening to stories to
mother = 1 sticker; falling asleep without mother = 2 stickers; use rewards
after that) USE ONE STICKER CHART AT A TIME
 Settling babies – Harriet Hiscock; 2 minute; don’t wait outside the bedroom;
use the timer
Temper tantrums
 2 school of thoughts – ignoring vs timeout (not good as you are giving the kid attention –
achieving this)
 Reinforce positively good behaviour i.e. when they stop
Body image
Body image
 HEADS screen/nutrition
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Remember to classify
o Restrictive eating
o Purgers
o Over-exercisers
Hx - What do you see when you look in the mirror?
No ideal weight for the anorexics
BMI charts – be careful
o BMI changes with the height
Remember
o Acne – risk of depression
o Pectus excavatum
You suspect that your long case adolescent is non-compliant with medication – how would you
approach this?
Why are they non-compliant?
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lack of understanding of long term consequences
side effects
forgetful
bullying/embarrassed at school
depression/anger at chronic illness
Non-compliance is associated with a number of factors among youths suffering with CI. Age is one
such factor, such that youths younger than age eight may not yet have developed the cognitive
maturity to understand the concept of death and thus feel no urgency to cooperate with treatment
(Rianthavorn et al, 2004). Compliance of children is usually passive. By contrast, adolescents struggling to
achieve separation and individuation may be particularly sensitive to the dependence implied by the
sick role (Wallander and Varni, 1998), inadvertently sabotaging treatment as they strive for what they
perceive to be autonomy. Non-compliance is further associated with a lack of social support, family
dysfunction, poor parent-child communication and a strained relationship with the treating
physician (Wallander and Varni, 1998; Bender et al, 1998; Bernstein et al, 2000; Hack and Chow, 2001). Additionally, just as CI
is associated with a number of psychiatric disorders (e.g. depression and Post-Traumatic Stress
Disorder), these same disorders can reciprocally drive non-compliance (Wallander and Varni, 1998; Alvin et al,
1995
). In fact compliance improves upon resolution of such disorders.
Most of those interviewed believed that compliance with prescribed medication was extremely
important, with many having formed this belief following a negative experience which they
attributed to their non-compliance.
Assess where they are in the transition process
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see them alone
do they understand their disease
are they taking responsibility for their own health
Example speil:
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I am concerned that Tom is not taking his pancreatic enzymes and this is impacting on his health.
To further assess this I would use my ‘alone’ time with Tom during a consultation to question him
on this matter. I would ask him directly if he ever forgot to take his medications, and try and
ascertain what the underlying reason might be…
1. In Tom, I felt that although he understood his illness and the consequences and needs for
medications, that he was angry at being different from the other children. I suspect this might be
contributing to his reluctance to take his medications at school. Together with Tom and his teachers
I would seek to devise a strategy where he could take his medications without other school mates
needing to be aware of it, and with minimal disruption to his lunch break.
2. I am a little concerned that Tom has recently come to the full realization of his probably reduced
lifespan as a result of cystic fibrosis, and is struggling to cope. I believe that he has stopped taking
his medications as he can see little point in the short term gain. As such, I would like to assess him
for signs of depression and help implement treatment. Specifically I would like to address Tom’s
fears for the future, in order to help him maximize his health in the present.
3. In Tom, I think that most of the time he forgets to take his medication. With Tom’s permission I
would like to reenlist his family and teachers in reminding Tom to take medication, or explore other
options such as a daily timetable or blister pack.
4. Tom has told me he specifically hates the fact that the enzymes make him feel nauseated, and
therefore he is reluctant to take them. I will look into strategies to limit this side effect, and
reinforce the need for his enzymes.
Enhancing adherence to therapy (adolescents)
1.
Blanket statement
o A common but challenging issue
o Important to adopt a long term view
o Understand that adherence often drops off during adolescent period
2.
Explore underlying reasons
o Is there a simple practical problem that can be rectified?
o Peer pressure – wanting to be normal?
o Depression – not care about illness?
o Lack of insight?
o Body image issue – dislikes side effect?
3.
Re-educate the patient
o As patients who present at children miss out on the direct education initially
4.
Involve the young person in the decision making process
o Be flexible regarding the treatment regime
5.
Agree on the most practical therapy
o Simple regime (e.g. tds to bd regime)
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o
1)
Careful selection of a therapy with the least side effects
6.
Make it practical
o Encourage making it part of daily routine (e.g. after breakfast)
o Have tablets placed next to an item of daily use e.g. clock/toothbrush
o Have enough scripts available
7.
Give effective instructions
o Repeat instructions
o Check comprehension by having them to repeat the instructions
o Write them down
8.
Encourage & praise (age-appropriate) adherence
o Negotiate a role for the parents to play & discourage parental nagging
Conflict resolution for adolescents
1.
Understand
o What triggers fighting
o Sometimes people use fighting to make themselves feel better
o Understand that the other person is a decent human being
2.
Control
o Keep control of temper & stay calm
o Try to use humour to diffuse the situation
3.
Talk to the decent side of the person
o E.g. this isn’t worth fighting about
o E.g. I have nothing against you, I don’t want to fight you about this
4.
If these steps do not work, walk away from the situation
Chronic constipation is defined as the occurrence of 2 or more of the following within the
previous 8 weeks:
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< 3 bowel motions per week
>1 episode of faecal incontinence per week
Large stools in rectum or palpable on abdominal examination
Retentive posturing and withholding behaviour
Painful defaecation
Example Speil:
One of Tom’s main issues is his ongoing struggle with constipation. As his general practitioner I
would be looking at addressing this on several levels:
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1.
An action plan for when Tom becomes acutely constipated. This would
include grading up his regular movicol satchets on a daily or second daily basis until he had a
large motion. I would aim to avoid hospitilasation and nasogastric wash out where possible.
Occasionally there is a need for enemas, but I would consider these second line.
2.
In between Tom’s episodes of acute constipation I would wish to optimize
his bowel habits. I would work in conjunction with a dietician in order to increase his fibre
and fluid intake. In addition I would have him on regular stool softeners (either lactulose or
parachoc). I would encourage regular toilet time, and an age-appropriate reward system such
as a star chart.
Degeneration in CP
1.
Consider the following causes
o Wrong diagnosis (e.g. neurodegenerative disorder, endocrine)
o Uncontrolled seizures
o Medication
o Depression
o Contractures
o Subclinical infection
2.
Consider the following investigations for an alternate cause
o MRI
o Metabolic studies
o CK
o Karyotype
o FISH for fragile X
o TFT
DEXA scans
1.
2.
3.
4.
1SD below (osteopenia) = 2x risk of fractures
2SD below (osteoporosis) = 4x risk of fractures
3SD below = 8x risk of fractures
Patients on LT steroids should have one baseline done, and then yearly after that
Diabetes & alcohol
1.
Emphasise safety
o Drink responsibly
o Drink in groups
o Alternate non-alcoholic and alcoholic drinks
2.
Getting home & recovering
o Have a designated driver
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o
Have someone wake them up in the morning to give them insulin & breakfast
Drooling
EXCESSIVE DROOLING (sialorrhoea)
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saliva is produced by submandibular (majority, watery) + sublingual (small amount, thick) +
parotid (while eating)
o ~500ml – 2L per day
innervated by parasympathetics
normal for babies to drool → until ~18 mnths when oro-motor control is established
drooling is a problem for: CP (~40%), intellectual disability, neurological impairment
cleft lip/ palate & micrognathia
nasal obstruction, dental disease
medication side effect (avoid benzodiazepines)
problem is ineffective swallowing (cf excessive production)
causes: social implications: embarrassment
skin irritation
frequent changes of clothing/ bibs
↑ risk of aspiration
management:
o
o
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parents rating – severity & frequency
assess via: speech therapist
video fluoroscopy
barium meal
nuclear med (salivogram, milk scan, gastric emptying study)
management
 conservative
 positioning & seating (physio input)
 speech therapy input - ↑ sucking (straw)
 ↑ child’s oral awareness (recognise feeling of wetness)
o
tell them that they are drooling
o
swallow more frequently
 waterproof bibs/ scarves/ specially designed clothes
 orthodontic devices (poor success rate)
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medical – anti-cholernergics
 benzhexol (artane) – cheaper & easier to obtain from chemist;
s/e: anti-cholinergic (sedation/ urinary retention/ constipation)
↑ thickened saliva (↑ risk of aspiration)
glycopyrulate – less SE’s but more expensive
 botox injections – ultrasound guided into parotid gland (under GA)
risk of facial nerve palsy/ ↑ thickened saliva
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surgical
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1)
for severe drooling or failure of conservative therapy
preferred option = relocation of submandibular ducts with excision of
sublingual glands
side effects: dry mouth → dental problems (need careful f/up with
dentists)
80% success
Starting school for the disabled child
1.
Remember parent’s wants
o Want the best start for their child in life
o Want the child to be part of the local community
o Want the child to be valued by the school
2.
Start early
o Age for starting school – eligible at 5yo, but doesn’t have to go until 6yo
o Apply for funding early (in October)
3.
Explore options available in their local area
o Government vs non-government schools (e.g. Catholic/Independent schools) –
generally govt school have more disability funding
o Normal mainstream school
o Specialist school e.g. Autism, vision impairment, hearing impairment (can be govt
or non-govt)
o Special Developmental School (SDS) – for children with severe ID (generally IQ
<50)
o Dual enrolment option
4.
Explore pro’s & con’s of each
o What is suitable depends on both the child’s needs, parental wishes, what’s
available & practical locally
o Mainstream school pro’s - mix in with normal kids, same school as siblings, more
practical for family logistically
o Mainstream school con’s – teasing & bullying of child AND siblings, inadequate
services by school e.g. PT, OT; no school bus pickup service, lack parental support
of children with disabilities
o Special school con’s - studies have shown that families start to feel that they do not
belong in mainstream society as they are segregated in the community
5.
Visit the school & talk to principal
o Do they have wheelchair access?
o Past experience of student with disability?
o Obtain educational needs questionnaire
o Obtain school’s program for students with disability
o Involve parents in school’s parent support group
6.
Role of paediatrician
o Facilitate this process
o Arrange appropriate assessments (neuropsych, vision & hearing)
o Long term – monitor progress, liase with school & advocate for the child
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See also Services
Encopresis
1.
Rule out organic pathology
o Hirschprung’s disease
o Spinal cord lesion
2.
Explain to parents
o Multifactorial but underlying cause unknown
o Could be behavioural (previous negative experience with painful defaecation),
underlying gut problem
o Shift blame away from child
3.
Specific management
o Stool softeners
o Regular toileting (twice a day, sit on the toilet)
o Reinforce behaviour with sticker charts & reward system
Bedwetting
Detrusor Instability (wetting, frequency, urgency)
1. Alarms
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Mainstay of treatment, but arduous - need motivated family
With proper explanation and use - 70% success initially
20-30% relapse rate
13x more likely to become dry if treated vs placebo
if fail keep trying – increase success rate with incr age
acc to M Harari – if fail pad and bell – put on DDAVP for 3-6/12
then try again with conditioning alarm
incr success on 2nd go?
Instructions:
o Go to toilet before bed
o Go to sleep in underpants only (ensure urine reaches mat)
o Turn alarm on (!)
o If alarm goes off:
o Wake child (often child sleeps through)
o (if not awake with shaking - pour cold water over!
o increased noxious stimulus)
o Child to turn alarm off
o Go to toilet
o Clean and repeat
Usually use for 8wks
If nearing dryness, but not completely dry at 8w - can extend to 12/52
If not working consider constipation, detrusor instability
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If child becomes dry with alarm for 1/52 - give large glass of water to drink before bed
(stress bladder). Over-learning with added challenge may decrease relapse rate
Mechanism?
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?classical conditioning
?induces circadian rhythm in ADH release
?teaching contraction of pelvic floor muscles when bladder is full
Dry bed training refers to regimens that include enuresis alarms, waking routines, positive
practice, cleanliness training, bladder training, and rewards, in various combinations. In
systematic review of 4 RCTs - there is no benefit of dry bed training over alarms alone.
Desmopressin
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Synthetic ADH analogue
Mechanism of action is not clear - probably not due to increased concentration of urine
overnight; may be due to effect on arousability
Short term stop-gap (eg school camp, sleepovers)
Medium term solution if alarm treatment has failed
~4x more likely to become dry than if not treated
High relapse rate after stopping treatment (no sustained effect if used alone)
Dose - 1 spray (10mcg) in each nostril at bedtime
Best absorbed from lower nasal mucosa
Especially kids >10yo failed alarm
?may have synergistic role with alarm
One RCT of 76 children (Acta Paediatrica 1997) suggested significantly higher remission
with combination of desmopressin and alarm(76%), vs alarms alone(46%)) with similar
relapse rates in the two groups
(side effects uncommon - local irritation, epistaxis; rarely - water intoxication, coma, death)
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need to restrict fluids after DDAVP given each night
treatment must be withdrawn during vomiting/diarrhoeal illnesses
absorption may be decreased during URTI
Detrusor Instability:
Bladder is irritable, and sensitive to concentrated urine, other irritants eg constipation
Usually girls between 21/2-8yo
Often dry until then
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Day wetting ± night
*urgency a/w squatting (walking along, suddenly dro down and wet themselves)
bladder spasms - urge incontinence
Frequent infections
Rx: (acc to Paddy Dewan)
1.
2.
3.
4.
High fluid intake (before 5pm)
Frequent voiding during the day (2 hourly)
Better than normal perineal hygiene (showers, wiping) (Wide, Wait Wipe)
Increased fibre in diet (unprocessed bran) - aim for 1 bowel action per day
Treat constipation properly
5. 'Lifting' (get the child up to void at 10.30 - ie before you go to sleep)
6. Prophylactic antibiotics
7. Anticholinergics - Probanthine
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If unsuccesful try oxybutinin
Side effects: dry mouth, constipation, rash (hot weather), mood changes
If recurrent UTI and detrusor instability conider U/S, MCU ± DMSA
Daytime wetting:
In boys – think about PUV – organise MCU
In girls – likely detrusor intstablility
Consider ectopic ureter if constant
Example speil:
The issue that Tom and his family have identified as their major concern is his ongoing nocturnal
enuresis. Hi father also had nocturnal enuresis until the age of 10yr. I would start his management
by normalising this problem for Tom, and adopting a rewards rather than punitive approach.
Initially I would do a full paediatric assessment including a urine screen looking for any possible
underlying cause, such as constipation or detrusor instability which may be treatable. In Tom’s case
I think that this likely a genetic variant.
I would start by regular pre-bed toileting, in combination with an enuresis alarm and a rewards chart
for the number of dry nights. I would initially try this for 8 weeks, and keep in regular contact with
Tom and his family during this time as it needs a highly motivated family to increase the likelihood
of success.
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Enuresis
 Assessment
o wetting chart
o posturing/behaviour a/w wetting
o stool hx
o primary or 2o. FHx
o UTI, hx of renal abnormality
o LL neuro exam, abdo mass (bladder/stool), genital abN
o Fluid intake
 Management
o Emphasise no punishment and positive reinforcement
o Educate – common problem, long term management
o Depends on type of incontinence
 **Find out epidemiology and management*
1)
Nocturnal enuresis (abnormal after 5-7yo)
1.
2.
3.
4.
5.
6.
Assess
o Rule out medical problem e.g. DM, renal pathology, spinal cord lesion - has child
achieved continence previously?
o Assess problem – frequency (no pun), large/small volume
o Look for comorbidities – encopresis, day time enuresis, constipation, family history
o Fluid diary, voidance diary and bowel action diary
Wait and see
o 15% of children become dry/year
Motivational therapy
o Useful first line particularly in younger children e.g. use of sticker charts
o Significant improvement (>80%) occurs in >70% of patients
o Successful (>14 consecutive dry nights) in 25% with a relapse rate of 5%
Bladder training
o Ask the child to hold onto urine for successively longer period of time
o Significant improvement (>80%) occurs in 60% of patients
o Successful (>14 consecutive dry nights) in 35%
Fluid training
o Increase daytime fluid, reduce night time fluid intake
o Some authors recommend total fluid intake should be divided into 40% morning,
40% afternoon and 20% night time
Bell & alarm conditioning
o Most effective long-term therapy
o Use > 7yo and children need to be motivated; use for 14-16 wks
o Method
 Child is in charge of alarm
 Each night before he goes to sleep, should test the alarm
 With the sound in mind, should imagine in his mind what he is going to do
when alarm rings
 I.e. CHILD turns off alarm, gets up and finish voiding, goes back to
bedroom, changes bedding and underwear, wipe down sensor with a wet
cloth then a dry cloth, reset alarm and go back to sleep
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Parents may need to help wake child up
o Success rate about 65-70% (14 consecutive dry nights)
o Cochrane review: bell & alarm is marginally better than DDAVP and Imipramine
7. DDAVP
o Important that they do not have further drinks after DDAVP  risk of
hyponatremic seizures (only need to drink 200-240 mls)
o Side effects: headaches, vomiting, epistaxis & nasal congestion
8. DDAVP + Bell & alarm
o Authorised for use in >6yo if alarm failed ($70/month)
9. Imipramine (tricyclic antidepressant)
o Equivalent to DDAVP
o But less used nowadays secondary to side effects
10. Indomethacin
o Not often used but in a small trial shown to be of benefit in children >6yo
o Reduced dry nights significantly in children treated for 3 weeks
o Postulated mechanisms – removal of normal inhibitory effect of prostaglandins on
the response of vasopressin
11. Oxybutinin (anticholinergic - Ditropan)
o Used when there is urge component (esp daytime)
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Ex-prem prognosis
1.
Latest survival figures
o 23-24 weeks – 50%
o 26 weeks – 66%
o 28 weeks – 85%
o >28 weeks – 90%
2.
Of those who survive <28 weeks
o 10% had major sequale
o 25-30% ADHD
o 25-30% psych issues in adolescence
o 65% have to repeat a year at school
3.
Of those who survive <26 weeks
o Ex 23/40 = 1/3 major sequelae
o Ex 24/40 = 1/4 major sequelae
o Ex 25/40 = 1/5 major sequelae
o Of the rest – 66% will have behavioural problems
4.
Poor prognostic indicators of survival (if 1 factor positive = 20% mortality rate) – from
Lex Doyle
o G4 IVH
o Cystic PVL
o Surgery
o Steroids
Neurodevelopmental outcome in the ex-prem
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Risk factors
o Prematurity
o Low birth weight – 2/3 of <750g have IVH
o Hypoxia/RDS
o Hypotension
2. Brain imaging
o Cranial US at Day 42 for PVL (indicative of spastic diplegia)
Best indicator of cognitive outcome is receptive communication
1.
The first fit: to treat or not to treat (Michael Hayman)
Consider:
1.
2.
3.
4.
Risk of recurrence
Does treatment make a difference?
Adverse effects of treatment
Balance of all these
Risk of recurrence
10% of people will seize at some stage in their life: 50% of these during childhood/adolescence
Risk of recurrence after an untreated unprovoked seizure:
 50% will have another seizure within two years (highest risk first 6mths)
 If this is going to happen to a particular pt, 90% chance it will be within this 2yr window
(i.e. if more than 2yrs have past, chances down to 10%)
Two big factors suggest more likely to have another seizure:
1. Abnormal EEG
2. Systemic cause or abnormal neurological examination
Also consider:
3. Epilepsy syndrome (may help dictate course, prognosis etc. and therefore need to treat or not
e.g. juvenile myoclonic epilepsy will all need lifelong tx)
4. Status epilepticus: conflicting evidence, but emotional weight towards treating
Family history of epilepsy does not carry increased risk of recurrence in paediatric age group!
Focal seizure  suggests underlying pathology (so fits into point two above)
Does treatment make a difference?
1.
Seizure recurrence risk:
For tx:
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30% reduction in recurrence risk in first 2yrs
Against tx:
Majority won’t recur anyway
NNT 14 (to prevent one seizure in two years)
Long-term (at 5yrs) no difference in recurrence in treated vs untreated cohorts
2.
Risk of injury/death during recurrence
Against tx:
Little data for brain injury in humans (though convincing evidence in animals)
SUDEP risk is relatively tiny
3.
Does delay lessen chance of achieving remission?
Short term  yes
Long term  no
Adverse effects of treatment
Not insignificant in either prevalence or clinical significance
Balance
Lifestyle may be in favour of treatment (e.g. adolescent fearful of seizure at school; wanting to get
licence)
Balance with side-effects, daily need to take meds etc.
Parents/patient need to make informed decision
Growth hormone indications = height < 1st centile OR Growth velocity < 25th
centile over 1 year
 Exceptions: Pituitary surgery : over 6 months (not one year…)
Usually don’t give GH in renal disease because of excellent catch-up growth
Use of growth hormone
 Daily injections, Usually over several years
 Used to be problems with CJD – now recombinant technology
 Contra-indicated in patients with malignancy
 Side effects:
o Risk of malignancy (leukemia)
o SUFE
o Gynaecomastia
o BIH
New immigrant
o Origin specific disease surveillance
o Nutritional status eg Vit D, parasites, micronutrients
o Dentition
o Immunisation
o Advocacy to immigrant groups for English classes, vocational training etc
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Menstruation in the disabled:
Common concerns:
1. personal hygiene – complicated by contractures, immobility, bladder or bowel incontinence,
behavioural difficulties
2. sexuality –
a. for girls who are able to express sexuality appropriate expression must be supported.
Early sexual education
b. For those without decisional capacity – focus on protection from sexual abuse
3. Effects of cyclical hormonal changes
a. Increase in seizures during certain stages of the menstrual cycle.
b. Cyclical behavioural changes as part of the premenstrual syndrome – aggression,
restlessness, hyperactivity
4. Abnormal timing of puberty
a. Precocious puberty 20X more common
i. Central precocious puberty Rx with gonadotropin-releasing hormone
analogue therapy
ii. Delayed onset of puberty – can aggravate poor bone mineralisation (+
anticonvulsant effect on bone mineralisation_
Treatment:
1. Sanitary pads, nappies
2. Contraceptive management is used to facilitate hygiene and personal care, prevent
pregnancy,
a. Oral contraception – commonly used in patients with cognitive impairment. Causes a
decrease in menstrual flow. Compliance may be an issue. Risk of thromboembolism.
Dose may need to be adjusted if the patient is also on anticonvulsants.
b. Intrauterine devices – usually not recommended for patients with cognitive
impairment because they can’t report pain or discomfort. SE: menorrhagia and
dysmenorrhoea, increasing menstrual hygiene problems.
c. Mirena – progestegin-releasing levonorgestrel IUD. Causes amenorrheoa, reduces
dysmenorrhoea.
d. Long acting progestins – a/w easier hygiene. Depo-medroxyprogesterone or
levonorgestrel implants. SE: mood, behvaiourla changes, pain from injection,
fatigue, menstrual pattern irregularity.
e. Transdermal and transvaginal estrogen-progestin – transdermal contraceptive patch
weekly for 3/52. SE: amenorrhoea, bleeding and spotting, increased risk of venous
thomboembolism compared with OCP
Management of periods in disabled girl
 Assess family and patient’s concerns, e.g. distress, discomfort, heavy bleeding, nappies vs
continent, behavioural changes in premenstrual period
 OCP
o Reduce frequency of periods and timing of periods
o Potential risks, e.g. clotting
o Minipill (progesterone only – less side effects, more strict with timing of
administration) vs OCP
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Depot provera, implanon, Mirena (IUD)
Why induce periods?
Menses management
1.
2.
3.
4.
Educate – teach adolescent how to use pad, tampon (esp if able to have own toileting
function)
NSAID’s
Tranexamic acid
Contraceptives
o Options – OCP (daily), transdermal patch (change weekly), depoprovera (IM – 3
monthly), implants (3-5 years)
o Side effects – thrombosis, breast & uterine cancer, migraines
o Benefits – oestrogen reduces dysmenorrhoea, menorrhagia & ovarian cancer;
progestin reduces endometrial cancer; beneficial for bones
o Drug interactions – cyt p450 inducers increases metabolism, therefore reducing
effectiveness of OCP i.e. CARBOS (CBZ, alcohol, rifampicin, barbiturates,
oestrogen & St John’s Wort)
o Remember – need for concurrent barrier protection against STD’s
Obesity approach
 Underlying – whole family fat? What is child’s insight?
 Activity – exercise, bike rides with the family
 Food – involve dietician
 Less video
Obesity
Assessment
1. Medical risk:
Child history and exam
Child growth
Parental obesity
Family history (obesity and related e.g. type II DM, cardiovascular dx)
2. Behaviour risk:
Sedentary time
Eating
Physical activity
3. Attitudes:
Patient concern and motivation
17
Family concern and motivation
BMI is the recommended screen for body fat.
Skinfold thickness measurements are not recommended.
Waist circumference measurements are not (currently) recommended:
 Can provide indirect information about visceral adiposity, however
 Reference values for children that identify risk over and above the risk from BMI category
are not available.
Screen for medical risks (complications) includes:









Psych: Depression, social stigmatization, isolation, bullying etc.
Neuro: Idiopathic intracranial hypertension (more of an association than complication; recurrent
headaches, visual)
Resp: asthma (more prevalent, and impacts on exercise as a remedy), OSA
(snore/apnoea/nocturnal enuresis/daytime symptoms; sleep, cardiovascular consequences);
obesity hypoventilation syndrome (sheer weight on chest, abdomen compromises respiration)
CVS: BP, lipids, (OSA)
Gastro: non-alcoholic fatty liver disease, reflux, constipation (all more likely)
Ortho: SUFE, musculoskeletal stress from weight, Blount disease (tibia vara)
Gynae: PCOS (irregular menses/primary amenorrhoea)
Type II diabetes – poly/poly, sudden unexpected weight loss
Skin: acanthosis nigricans, intertrigo, furunculosis
Hypothyroidism rarely causes severe obesity: worry if cessation in linear growth, goitre (TFTs
usually unnecessary if normal linear growth velocity and no other symptoms)
Primary Cushing’s: very rare; should be short (so not likely in tall obese child); striae are violaceous
in colour
Genetic syndromes – Prader-Willi etc.
Investigations must include:
 Lipids
 LFTs (for NAFLD) – initially and then 2yrly
 ?GTT (for type II DM)
Prevention


Target all children!
Lifestyle behaviours aimed at prevention, are preferable to getting to stage where intervention is
required
Evidence exists for the following target behaviours:
1. Limiting sugary beverages;
18
2.
3.
4.
5.
6.
Lots of fruit and vegies;
Limiting TV and other screen time (AAP: none before 2yrs, no more than 2hrs after that);
Breakfast every day;
Less eating out, especially fast food;
Encouraging family meals, eating together (a/w higher quality diet, lower obesity prevalence
– but sounds like could be confounding for SES?)
7. Limiting portion size
Expert recommendations:
1.
2.
3.
4.
5.
6.
Lots of calcium;
Lots of fibre;
Balance between protein, fat and CHO;
Breast-feeding exclusively until 6mths; keep going thereafter after introducing solids;
Promoting moderate to vigorous physical activity for at least 60mins/day;
Cut down energy-dense foods
Intervention
This is the bit to have a spiel on.



Overall goals are establishment of permanent healthy lifestyle habits and improvement in
medical and psychological complications of obesity, with decreasing BMI as primary
measure.
More obese  aim to change weight more gradually
Allow 3-6mths for each stage of management to show benefit (or otherwise) before moving
to more intense level.
Stage 1 – Prevention Plus
Stage 2 – Structured Weight Management
Stage 3 – Comprehensive Multidisciplinary Intervention
Stage 4 – Tertiary Care Intervention
Stage 1: Prevention Plus
1.
2.
3.
4.
5.
6.
7.
8.
Consume 5 or more servings of fruits and vegies per day
No sweet drinks
TV/computer <2hrs/day
>/=1hr physical activity/day
Breakfast every day
Limit meals outside the home
Family meals at least 5-6x/wk
Allow child to self-regulate meals (avoid overly restrictive behaviours) – someone put this
as parents decide what is eaten, kid decides how much is eaten
19
Aim is weight maintenance (decrease in BMI as age, height increases)
Monthly follow-up
To stage 2 if no improvement in BMI or weight in 3-6mths
Stage 2: Structured Weight Management
1. Develop structured diet plan (with dietician) – balanced macronutrients, small amounts of
energy-dense foods;
2. Structured meals (B/L/D +/- 1-2 snacks)
3. Supervised active play of >/= 1hr play
4. Screen time <1hr
5. Increased monitoring of food/activity (pt/parent/family)
6. Positively reinforce behaviours (more so than weight goals)
Stage 3: Comprehensive Multidisciplinary Intervention
1. Referral to specialist MD team
2. Planned negative energy balance diet
3. Structured behavioural modification program (inc. monitoring and forming short-term diet,
physical activity goals)
4. Home environment “training” for parents/family!
5. Frequent follow-up (weekly say for 8-12wks)
In this cohort, aiming for weight maintenance or gradual decrease until BMI <85th centile
Stage 4: Tertiary Care Intervention


For kids >11yrs with BMI >95th, significant comorbidities and no success Stages 1-3; OR
Kids >99th centile with no improvement stage 3
1. Above elements
2. Might consider medications (sibutramine, an SSRI; orlistat – causes fat malabsorption;
approved by FDA, ?efficacy, ?available in Aust)
3. Very low-calorie diet
4. Surgery: gastric bypass or banding
Oral aversion
1.
2.
Important particularly in children who are on TPN since a young age
Assessment
o Underlying reasons e.g. prolonged parenteral nutrition, mucositis, nausea, GORD
o Identify particular foods that child does like
o Observe child during a feed – is the child interested? How is food being offered (is
there play involved)?
o Involve a speech pathologist early
20
3.
4.
5.
Educate parents
o Important as this may lead to MAJOR ORAL ADVERSION PROBLEMS in later
childhood, and growth & nutrition crucial for chronic illness
o Also important for dental health
Start introducing food at an early age
o Make it fun - play therapy to introduce foods of different tastes, textures and smells
(lay it on a mat in the ground and they can play with it)
o Have this done during family meals – also improve bonding of child with family,
understands concept of meal time, and that eating is fun!
Follow this up
o Over the long term; monitor growth & nutritional status
Oral aversion
 Particularly in the kids with TPN
 Play therapist important
st
 In the 1 12 months – put objects in the mouth of different taste, textures, smells (involve
speech pathologist) = if this is not done can have major oral adversion problems
 Involve them in family meals – good time for parent & child bonding
 At risk of dental hygiene
2)
3)
Osteoporosis
1.
Underlying factors
o Medical – fat malabsorption (coeliac disease, CF), hypogonadism in men,
hypothyroidism, Down
o Antiepileptic – phenytoin, valproate
o Delayed puberty & amennorhoea
o Immobility & lack of weightbearing exercise
o Inadequate sun exposure
o Poor diet – calcium & vit D
2.
Investigations
o BMD – remember “z” scores are matched to age, sex, height, weight and normal
pubertal timing
o Bone age – consider doing to aid “z” score interpretation
o Bloods – Vitamin D level, Ca, PO4, ALP
3.
Management
o Increase sun exposure
o Dietary
 Increase calcium - i.e. dairy products - fat-reduced milks are usually
enriched with skim milk powder containing more calcium, yoghurt, cheddar
cheese
 Increase vitamin D (dietary vit D only contributes 10% to body) – milk,
margarine, butter, oily fish
o Drugs
21
Vitamin D supplement – ergocalciferol
 Calcium supplements
 Hormonal therapy
 Bisphosphanates
Remove fracture risk
 Optimise vision - Improve household lighting & contrast, eliminate galre
 Review meds – esp sedatives, drugs altering gait or causing hypotension
 Appropriate home modification – rails for toilet, involve OT, reduce steps –
have ramps
 Walking aids; Consider hip protectors?
Physiotherapy - increase exercise (exercise programs)

o
o
1)
2)
Poorly controlled diabetic adolescent
1.
Blanket statement
o Important time in diabetic management
o Difficult time for the adolescent
o Beware insulin requirements of >2 units/kg/day (though often increased insulin
requirements in adolescents)
2.
Why difficult control?
o Practical issues – needles, supply, who does it, embarrassment
o Insulin allergy and they aren’t telling you – change to human insulin, desensitising
or steroids (if systemic symptoms)
o Insulin resistance (RARE) – anti-insulin antibodies; change to highly purified
insulin
3.
Education and encouragement
o Accept that adherence is not 100% and encourage positive things
o Re-educate as children who are diagnosed in early years miss out on education part
and need to be re-educated in the teen years
4.
Role models & routine
o E.g. Wasim Akram
o Would basal bolus regime be better – esp for the adolescent
5.
Negotiation
o Parental issues and independence
o Negotiate a role for the parents in the management of this child
22
RADIATION, DOSE AND EFFECTS
BACKGROUND
1. In Australia, the level of natural or background radiation exposure is
approximately 2.5mSv per year, but there are wide variations depending on
geological and geographical location.
2. E.g. the dose rate from cosmic rays increases by a factor of around 3 in going from sea
level to an altitude of 2km.
3. An around the world flight in a commercial jet airliner represents a dose of around
0.2mSv.
4. A flight from Australia to the UK is equivalent to around 3 chest x rays.
DOSE LIMITS FOR PATIENTS
1. There are no formal dose limits for patients. However, there should always be a net
benefit from undergoing a radiological procedure.
2. “ALARA” principle: keep doses As Low As Reasonably Achievable whilst achieving a
clinical diagnosis.
RADIATION DOSE FROM CT
1. This varies from patient to patient depending on the size of the body part imaged, sex
and age of patient, the type of procedure and the type of CT equipment and its operation.
2. The quantity most relevant for assessing the risk of cancer detriment from a CT
procedure is the “effective dose”. Effective dose allows comparison of the risk estimates
associated with partial or whole body radiation exposures and it also incorporates the
different radiation sensitivities of the various organs in the body.
RADIATION DOSE COMPARISON
Diagnostic
procedure
CXR (PA)
Skull
Lumbar spine
IVU
Barium meal
Barium enema
CT head
CT abdo (avg)
Typical
effective dose
(mSv)
0.02
0.07
1.3
2.5
3.0
7.0
2.0
10.0
Number of CXR
for equivalent
effective dose
1
4
65
12.5
150
350
100
500
Time for equivalent effect
dose from background
radiation
2.4 days
8.5 days
158 days
304 days
1 year
2.3 years
243 days
3.3 years
23
Age of Consent for Sex
Age of consent for sex is 16 for heterosexual and homosexual intercourse in Vic, NSW, ACT, NT
and WA. In SA and Tas age is 17. QLD it is 16 for vaginal sex and 18 for anal sex
In ACT, VIC – sex is legal younger than this if there is not more than 2 years between the
consensual partners (eg 10 year old girl and 12 year old boy) and the youngest person is over 10. 3
years in Tassie.
Sex in the other states is usually legal at a younger age if the older person ‘believed’ the partner to
be of consenting age.
School refusal
Occurs in 1-5% of all school children, peaking at 5-7 years, then 11 and 14 years.
Outcomes:
 Poor academic performance
 Family difficulties
 Worsening peer relationships
Longer term consequences:
 Academic underachievement
 Employment difficulties
 Increased risk of psychiatric illness
Poor prognostic features:
 Longer periods of refusal > 3 years
 Occurence in adolescence
 Depression
 Lower IQ
Assessment:
 Consideration of predisposing, precipitating and perpetuating factors in the child, family and
school eg
o Fear of loss of parent
o Needing to protect a parent
o Excessive fear of situations within the school eg bullying, tests, changing of teachers
 Thorough examination to reassure the child and parents about physical symptoms
 Judicious use of investigations relevant investigations to allay concerns.
 Information from school about the child’s behaviour, social functioning, academic progress
and records of attendance. Diary of attendance, associated events, triggers, activities to
recognise patterns in behaviour and emotions.
 Parent and teacher behaviour checklists and mental health scales to identify problems and
compare severity of behaviours at home and school
Management:
 Introduce school return strategy immediately if refusal period has been brief or gradually
(graded exposure) if longer.
24






2)
Acknowledge reality of feelings, work together with child to plan school return and deal
with anxieties through problem solving, relaxation training, breathing retraining, social
skills training
Work with parents – plan calm morning routines, clear instructions, escort to school, allow
the child to stay in contact with parents by phone
Work with school – arrange special supports eg modified curriculum, reduced homework,
remedial tuition, encourage reinforcers eg access to garden, privileges, rewards
Monitor for mental heath symptoms –anxiety, depression
Referral to multidisciplinary mental heatlh team for longer term school refusal.
o Behaviour management, child therapy, CBT, pharmacological therapy, sometimes
admission
Regular monitoring of progress and signs of relapse
School refusal
1.
2.
3.
4.
5.
6.
Explore antecedent factors e.g. bullying, learning disorder, ADHD, autism, chronic illness
Liase with the school (counsellors)
Try hard to keep child at that school
Graded intervention/exposure e.g. week prior to school opening, practice putting on
uniform, then drive to school, then drive past gates etc
Praise the child each step along the way
Adolescents – consider alternative education options e.g. TAFE-like courses,
apprentinciship
School refusal
 Hard to manage
 Takes a long time and
 Explore antecedent factors
o e.g. school bullying, mental illness (learning disorder, autism, adhd) – note that
learning disability should be assessed by the school (ADVOCATE FOR THIS BY
CALLING UP THE SCHOOL YOURSELF IF CONCERNS)
o e.g. chronic illness & having the chance to socialise with friends
 Parents often are reinforcing the school refusal
 Main thing - Keep the child at school & not lose ground
 Behavioural intervention – reward for going to school
 May require graded intervention/exposure
o Week before going back – practice putting on uniform, practice driving there, then
going pass school gates…etc
o Slowly grade up
 Involve the school right from the start – involve the school counsellor; remember to
obtain consent from adolescent/family
 Adolescents
o Usu in depression, OCD
o Hence treat underlying problem
o Alternative schooling options e.g. enrolling in TAFE-like courses
25
Scoliosis management
1.
2.
3.
Identify risk factors (same as hip dislocation)
o GMFCS 1 – 0%
o GMFCS 2 – 15%
o GMFCS 3 – 40%
o GMFCS 4 – 70%
o GMFCS 5 – 90%
Assessment
o X-ray – look at Cobb angle
Treatment
o Cobb <15 degrees
 Monitor for complications – sitting posture, discomfort, resp compromise,
functional issues e.g. dressing & hygiene
o Cobb 20-40 degrees
 Wheel chair with side-supports
 Physio – stretching exercises against the direction of the curvature &
strengthening exercises
 Brace – uncertain regarding prevention or delaying surgery; pros: improves
postures; con’s: pressure sores, incompatibile with sitting
o Cobb >40 degrees
 Scoliosis surgery – anterior thoracotomy/thoraco-abdominal approach; or
posterior approach
 Indications – Cobb >40-50 degrees, progression of scoliosis, affecting QOL,
>10yo, stable medically
 Risk – death, resp failure, infection, pull-out of metalware, protrusion of
metal wear
Intractable seizures
1.
Clinical assessment
a) Right diagnosis?
 Undiagnosed metabolic, neurodengerative, organic pathology,
neurocutaneous d/o?
b) Adolescents
 Poor adherence? – depression? (esp in adolescents)
 Substance abuse/illicit drugs?
26
Pregnancy???
Drug issue
 Drug interaction? Cyt p450 decreases drug effectiveness; CARBOS – CBZ,
alcohol, rifampicin, barbiutares (PHB, PHT), oestrogen, St John’s worts
 Drug side effect? – e.g. hyponatremia from CBZ
 Drug correct? E.g. CBZ worsens GTCS
Precipitant
 Unwitnessed recent head injury/fall?
 Subclinical infection?
 Environmental stimulus e.g. television
Are they seizures?
 Pseudoseizures, behavioural manifestation

c)
d)
e)
2.
Investigations
a) If adherence issue – consider drug level
b) Electrolytes
c) Repeat imaging (MRI), EEG (+/- video EEG if unclear)
d) Revisit past investigation incl metabolic studies
3.
Treatment
a) Meds – consider prev treatment; what max dose; why stopped? Tried
leveteriacetam?
b) Ketogenic diet
c) Vagal nerve stimulator
d) Surgery
GET UPDATED VERSION! Services/Aids and Assistances for Families
1. Financial Assistance
Carers Payment v’s Carers Allowance
Administered by centrelink
Carers Allowance is about $90/fortnight and is what most families are entitled to. It is not means
tested. There are a whole heap of conditions/syndromes that automatically are entitled (eg non
mobile CP, downs, autism, diabetes, cancer etc) or if you don’t have one of these then the doctor
completes a functional assessment about them (essentially parent need to be providing >14 hrs
/week of care to this child above that which would be needed for a normal child).
Carers Payment is much more difficult to get – you need to be significantly disabled to qualify –
essentially full care needs. It provides a ‘income’ for full time carers whose children are so disabled
they require full time care. (you can also get it if you have 2 children with moderate care needs). It
is means tested.
You can get the carers allowance in addition to the carers payment.
27
If you are entitled to either of the above payments you will also automatically get a health care card
in the child’s name – this allows you to get cheap scripts, discounts, free ambulance transfer etc.
You can get a health care card if you don’t qualify for either of the above (eg lesser chronic medical
problems).
This all changes when a child turns 16 which can be a stressful time for some families – the parents
no longer get the money, instead the child gets it. However rules as to who is entitled is different at
this age.
Case Management/Care Packages - Department of Human Services (VIC)
A case manager’s role is to support/advocate for the family and coordinate services/supports.
Care packages come with a case manager and have titles such as Early Choices/ Making a
Difference and Family Choice in Victoria. They are attached with brokerage funding.
Often a long waiting list and can be difficult to get
Need to have a severe disability to get funding
Different states will have different programs – mainly need to understand that there are large
amounts of funding that can be accessed for children with significant difficulties which is then
allocated to things like equipment/consumables/carers etc
Continence Aids Assistance Scheme – provides $470/yr to children over the age of 5 to pay for
nappies/paps/catheters etc. Need to fit into specific illness criteria (permanent and severe
incontinence caused by neurological or other condition). Federal program
There is also a number of state based programs that provide a similar amount of money (see stomal
fact sheet)
Aids and Equipment Program (may have different names in other states)
Helps with costs associated with aids, equipment and home modifications
Home Enteral Nutrition (HEN) Program
Provides enteral feeds and equipment free of charge. Some states do not have this scheme and
people have to pay for their enteral feeds so it is worth asking about.
Transport Costs
1. Victorian Patient Transport Assistance Scheme
VPTAS provides partial reimbursement to assist with travel and accommodation costs incurred by
rural patients and if appropriate, their escorts, when travelling long distances or staying away from
home to receive specialist medical treatment
Most states will have an equivalent
2. Disability Parking Permits
Allocated through local councils – 2 types – one for those with sig intellectual or ambulatory
disabilities, the other for thoses who require rest breaks.
3. People with disabilities are entitled to half price taxis in the multipurpose taxis.
28
4. Vehicle modification is usually the financial responsibility of the family – can be expensive!
5. Special schools usually pick up children on their own bus – however the route can be long
and slow (often a 2 hour circuit on the way to school)– the first kid may be picked up before
7am and dropped off after 5pm – makes for a long day and not well supervised for kids that
need assistance (eg will anybody see them fitting in the back corner)
6. Red Cross can provide free transport to the hospital for appointments for people how do not
have a car and cannot use public transport
Other –
Are they registered for medicare safety net (this means that once they have paid $1000 of out of
pocket costs in a year per family toward health care, 80% of any costs incurred there after will be
reimbursed)
Money can often also be available from local organisations eg Rotary, variety club to assist with
specific costs
2. Respite
Really important to ask about respite – how much, where and can they actually access the number
of hours they are entitled to! Can be a very long waiting list.
This can be provided by a number of different services – often sourced through regional carer
respite services. Can be in the own home or at a centralised place. Volunteers or paid staff.
Most councils provide respite services under the federal Home and Community Care program –
often it is a few hours a week to allow parents to go to a movie, get the groceries done etc.
Sometimes they also provide assistance with house cleaning etc. There is sometimes a small
payment (income based) required by the family.
People with case management usually have some respite included in their package.
A number of other associations also provide respite (different in every state eg villa maria)
Respite can be emergency, regular or for a few weeks a year for holidays.
Other options include
Interchange (national) – volunteers provide respite for disabled kids – often on a regular basis (eg
once weekend a month)
Very Special Kids – respite for kids with a terminal/life threatening illness
There is also a 24 hr emergency respite number which can be used in cases of emergencies
Family and friends are also an important source of respite.
3. Support Groups
Most of the major illness have specific support groups
Eg Heartkids, Cystic Fibrosis Australia etc
Can be a great source of support and information for families.
Illness specific organisations eg vision Australia, Yooralla (disabilities) etc
Other organisations such as carers victoria, association for children with a disability etc
4. Early Childhood Intervention Services - run by specialist children services (SCS) (branch of
Department of Human Sevices – Victoria – similar programs will exist in other states)
For pre-school aged children,– helps children with disability or developmental delays to access
therapy and support the families. Usually in a centralised place, occasionally can occur in
mainstream places (eg at the child’s normal kindergarten or in the home).
29
Often a very long waiting list in most areas (some regions better than others) so if you think they
are going to need the services then put their name down early.
5. School
Children with a disability have the right to attend their local neighbourhood school. Alternatively
they may qualify for a special developmental school or. Families need to make their own choices
on what they feel will be best for their child. Funding is available for appropriate assistance if the
child is going to attend a mainstream school. Usually children should start school at the appropriate
age (5 years). Funding runs out at the end of the year that they turn 18.
It may be worth considering getting things like neuropsychological testing (eg weschler etc) in
some circumstances prior to starting school so that areas of deficiencies can be clearly highlighted
and the most appropriate teaching assistance for their specific problems can be given
6. Other things
Does the family have a care plan/card for the hospital if they are frequent attenders?
Also things like
Make a Wish (wishes for kids with life threatening illnesses)
Starlight – wishes for kids with terminal, chronic or critical illnesses if referred by a health
professional
Companion Card – free entry to events for carers of people with a disability (also free public
transport
GP STUFF
If a GP has completed an enhance primary care plan for any patient with a chronic or terminal
illness that is complex and requires multidisciplinary care then they can then refer them on to allied
health services for upto 5 medicare rebatable sessions per year (eg physio, dental, psychological,
speech, dietician). (there are special medicare rebates for a GP to establish a care plan and have case
conferences for patients with complex health needs). Paediatricians cannot do this – this must be
done by the GP.
Other
Mental Health –
Last year a new medicare rebate was introduced whereby any patient with an ‘assessed mental
disorder’ can be referred by a paediatrician, psychiatrist or their GP to psychology for upto 12
private sessions rebatable per calender year (review after 6 sessions). In addition are also entitled to
12 group sessions per year. This includes mental disorders such as ADHD, OCD, eating disorders,
anxiety etc.
Sleep hygiene
1.
Screen healthy children with BEARS
o Bedtime problems (any problems going to falling asleep?)
o Excessive daytime sleepiness, mood disturbances, irritability, behavioural
problems
o Awakenings (frequency? triggers? trouble getting back to sleep?)
30
o
o
o
o
Regularity and duration of sleep
Snoring
Normal sleeping time (for birth – 6yo; age + number of hours to sleep ~15-16) Birth (16 hrs), 2yo (13 hrs), 6yo (9.5 hrs), 18yo (8hrs)
Normal napping – 1yo (2/day), 2yo (1/day), 5yo (0.5/day)
2.
Assessment
o Problem with sleep initiation? Sleep dissociation problem?
o Rule out organic pathology & precipitants – e.g. OSA, depression, drugs
o Sleep history – when they sleep & wake up? Schooling vs non-school days?
Number of naps?
o When do they wake up? What do they do to get back to sleep? Activities – during
day & night, just before sleep.
3.
Sleep hygiene for infants
a) Teach the infants to “self-soothe”
 Thus, bring the baby to bed drowsy but not asleep
 This will avoid dependence on parental presence to sleep
b) Regular bedtime and bedtime routines
c) Appropriate transitional objects (e.g. blanket, soft toy)
2.
Sleep hygiene for children
a) Regular sleep-wake cycle
 Do set a regular bedtime and bedtime routine
 Do keep to this in both school & non-school nights (no more than 1 hour
difference)
b) Sensible activities
 Do encourage outdoor activities during the day time (and time out of the
room)
 Do make the 1 hour prior to bed quiet time (avoid stimulating activities)
 Avoid using the room as a place for punishment/time out
 Avoid having a TV in the room
c) Appropriate environment
 Do have a quiet and dark bedroom
 Do have a comfortable temperature in the bedroom
d) Appropriate food
 Avoid caffeine containing products several hours before sleep
 Avoid going to sleep hungry (a light snack is good; heavy meal can interfere
with sleep)
e) Sticker charts
 1 sticker for listening to parent’s bedtime stories
 1 sticker for falling asleep without parents
3.
Sleep hygiene for adolescents (as above AND)
a) Think of depression, illicit drug use
b) Do use bed only for sleeping (not for reading, studying, TV, etc)
c) Consistent sleep-wake cycle (esp with parties)
d) Avoid long daytime naps - take short ones if required (avoide entirely if difficulty
initiating sleep at night)
e) Avoid alcohol & smoking before sleep
31
f)
Avoid sleeping pills unless prescribed by a doctor
Monitor for side effects (CUSHINGOIDMAP)
C cateracts
U ulcers
S striae
H HTN, hyperactivity, hallucinations, hirsuitism
I Infections, irritability
N Necrosis of bone (avascular)
G Growth retardation
O osteoporosis
I Increases ICP, insomnia
D Diabetes Mellitus, depression, dizziness
M Myopathy
A Adipose tissue hypertrophy (obesity), Acne
P Pancreatitis
1. yearly ophthalmology
2. monitor sugars and HbA1C
3. Blood pressure
4. Monitor growth – height and weight
5. DEXA scans (at start then 6 –12 monthly)
6. Functional muscle assessment – walking up stairs
7. Monitor mood
Immunization:
1.
Avoid live vaccines
2.
Ensure extra vaccines – pneumococcal, influenza, meningococcal
3.
Vaccinate – the family against varicella (not the child)
Diet:
1.
2.
Beware polyphagia and obesity
calcium
Specific measures
1. antihypertensives
2. APD for osteopenia
3. GH (indications see clinical exam)
4. Hypoglycaemic agents
Education:
1. Advise re: Addisonian risk and need for increasing dose and not stopping sudddenly
2. Medi-alert bracelet
Pregnancy:
1. PNL causes cleft palate and neonatal adrenal insufficiency
2. Avoid if possible: bad in early pregnancy, OK in later pregnancy
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3. PNL OK for BF’ing
Drug interactions: OCP and PNL = HTN, other???
Stoma’s in kids (remembered from Tute)
98% are temporary (bowel/bladder/gastrostomy) – eg for hirschsprungs, NEC, imperforate anus,
neurogenic bladders etc.
Some specific types of stoma:
Malone –quite common. Is an appendicostomy. Used for bowel washouts in kids with
megacolon/severe constipation. Does not have a bag attached– essentially looks like an umbilicus in
the RIF. A catheter is then inserted directly into this to allow the family to give a 2nd daily bowel
washout (infuse a couple of litres of saline in over about 45 minutes and hey presto – you will have
a bowel action (out the anus). Needs to have a catheter or something put in it every 1-2 days or it
will close over. Sometimes they have a button – this is called a chard button. If this comes out it is
no big deal – most parents can reinsert, otherwise you have 24 hours until reinsertion needed.
Mitrafonoff – appendicovesicostomy (stoma to allow direct access to the bladder). Good for kids
with ectopic bladder, spina bifida in whom CIC’s are difficult etc. Usually encourage 4 hourly
catheters (overnight put on drainage usually).
Bladder augmentation surgery presents an increased risk of bladder cancer (and hence need 2 yearly
biopsies). There is a very small but possible risk of later cancer following a mitrofanoff.
Risk of stenosis with time with a malone and mitrofanoff.
Gastrostomy –
-if they dislodge they need to be reinserted within 4 hours.
-often have buttons attached eg Mickey, Bard etc
-Can stay in 18/12 before needing review.
-If blocked try coca cola
-If you have an associated fundoplication may need to vent the peg to reduce gas.
-Don’t treat redness around PEG with steroid creams. They are rarely infected. Hypergranulation
tissue can be treated with kenacomb or silver nitrate.
Bowel Managment
Kids socially tend to start becoming upset/teased by soiling in about grade 1 or 2 (usually OK in
prep)  usually aim to address bowel regime/soiling issues prior to school entry. Initial step is
timed toileting (5mins BD) and laxatives/enemas. Surgical management 2nd line
Stomal Management
If you have a stoma you can do all sports except boxing and scuba diving. Orthotics can make a
variety of protective belts/devices to wear to cover your stoma. Usually just pop a bandaid over the
top when swimming.
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There are a heap of products that the stomal therapist can use to stop leaking/gas/smells etc 
hence if the family are having problems with any of these things they can almost always be fixed by
a referral to the stomal therapist.
Catheter Management
If management of bladder issues is by CIC usually 4 hourly catherteristations (or at least 3x /day) is
recommended (depends on bladder size/function etc). Should be performed cleanly. There are a
variety of different catheters that can be used from single use/pre lubed disposable to multiple use.
Also come in a variety of sizes (one girl had one that fit nicely in each of her shoes so she didn’t
need to carry anything at school!!). Botox injected directly into the bladder can reduce spasticity if
needed.
Kids should be encouraged to self catheterise as early as possible (obviously depends on other
comorbidities) – Judy’s youngest patient who can self catheterise is 3 years old.
Think about problems that may contribute to difficulties with self catheterisation (eg subluxed hips
or joint contractures which make hip abduction difficult; poor fine motor control etc).
Financial –
A. Continence
There are 2 schemes for continence aids offered by the government (one state, one federal)
1. Federal – Continence Aids Assistance Scheme – This is NEW (previous to the election it
was only available to over 16’s so lots of kids may not yet know about it and may be
entitled). Families get $470/yr for children over 5 yrs who meet either A or B
a. Permanent/severe loss of bladder/bowel function due to eligible neurological
condition (eg CP/SB/MS/paraplegia)
b. Permanent/severe incontinence which impacts on quality of life and will last >2yrs
and is unlikely to improve with treatment.
2. State – Continence Support Service (the equivalent in other states is highly variable – great
in QLD, terrible in some other states)
Children 5-15 yrs get $480/yr for continence products and assistance from a continence nurse
(but there is a waiting list of upto 3 years to get it!!!!)
B. Stomal
Any child who has a stoma will get their stomal supplies funded through the stomal association
C. Other
Victoria Aids and Equipment Program – supplies some continence products (eg catheters, anal
plugs, reusable undies/bed protectors etc) upto $1200 annually. Needs to be prescribed on script by
a doctor.
Tracheostomy care
1.
Background
o What size? Recent upsize/downsize? Spare at home?
o Changed by parents every 2 weeks
o Home suction? Mobile suction?
o Contigency plan if falls out?
o Ambulance cover?
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o
2.
Parents trained in CPR?
Surrounding issues
o Getting it out as soon as possible (and need for reconstructive surgery)
o Speech problems – delayed articulation
o Feeding problems are great
o ENT problems – OME, conductive deafness
o Infection – croup
Transition
 Introduce early – 12 month process. Final year of schooling OR 18yo. Don’t introduce at a
time when there are other stressors.
 Encourage independence for own cares – meds, hx, diagnosis, medicare, appointment
making in the period prior to transition
 Start seeing them alone for at least some of the consultations
 Address genetic/sex/drug issues
 Offer transition clinic if available
 Period of joint care – appointment with adult medical team followed by appointment with
paeds for feedback
 Prepare summary of medical hx and current issues to give to adult team
 Agree on date and place of transfer
Barriers to transition




Reluctance of health care providers to “let go”
Reluctance of families to leave the paediatric system due to
o Close relationships developed with health professionals
o The need for families to repeat explanations of the patient’s condition to new health
care providers
Reluctance of adult physicians to take onboard patients due to lack knowledge, training or
interest
Lack of resources
o Lack of quality care for severely disabled young people in adult settings
o Lack of adult hospital resources
Why do we need to have transition


Paediatric hospitals are not physically set up to look after adults (e.g. equipments)
Paediatric health care providers are not trained in assisting in the areas of job vocation,
unemployment, sexual health/family care
Problems with bad transition


Increase rates of young people dropping out of the health care system
Increased deterioration of preventable conditions
Management of transitions

Important principles
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Transition should occur within a developmental context
o Important to promote young person’s increasing capacity for self care
o Support ongoing involvement of family
o Flexibility about timing of transfer is important
Need leadership by senior medical staff
Need a coordinated approach
Need effective systems
Need involvement of multidisciplinary team (nursing staff, allied health, administrative
staff)
o




Transition goals



Ensure the safe & planned transfer of patients approaching adulthood
Ensure the continuous and high quality of care of patients transferred to the adult system
Ensure mechanisms exist for advice and support to hospitals receiving transitioned
patients where required
UTI is common – 10% children at some stage
More common if structural anomaly
Traditional understanding of pathogenesis was that anomaly (especially VUR) meant more prone
UTI  renal parenchymal damage  long-term problems such as BP and CRF
This suggested we should look for abnormalities (including invasive investigations such as MCU,
nuclear scans, even IVP) and try to intervene:
 prophylactic antibiotics
 anti-reflux surgery (e.g. reimplantation)
however
Increasing appreciation since especially the late 90s that:
Many renal parenchymal defects are present before UTI
VUR (even higher grades) spontaneously improves
Anti-reflux surgery has little effect (on preventing UTI)
Prophylactic antibiotics don’t affect UTI rate
No documented improvement in terms of rates of end-stage renal disease despite aggressive
investigation and treatment (and this is our main end-point)
Recent studies:
JAMA 07 – antibiotic prophylaxis not associated with risk of recurrent UTI (and actually rate of
resistant organisms)
Pediatrics June 08 – multicentre RCT in kids younger than 2.5yrs with VUR grades II-IV, showed
no difference b/w groups on prophylaxis and those not in terms of:
rates of having at least one episode of pyelonephritis;
presence of renal scars in the two groups
If our intervention that is based on (invasive) investigation doesn’t affect outcome, then raises
question as to whether we should be investigating in first place.
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Mike South, RACP Congress this yr: turns out that trends in Australia over last 14yrs have been for
same number of renal U/S (not invasive) but a 65% drop-off in number of the invasives (MCU;
radiation via nuclear scans; IVP).
Thus role for prophylactic antibiotics in VUR is (increasingly) contentious.
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