management topics Flashcards
Aspiration
Feed position, thickening, reduce volumes
Can give only the consistency that is shown not to reflux on video fluoroscopy
Percutaneous feeds (PEG or PEJ) to bypass upper gastrointestinal tract
Behaviour problems
Prioritise problem behaviours
● List all behaviours that parents would like to change
● Select the two that are most troubling (take into account dangerousness)
● Always tackle sleep problems first as this exacerbates bad behaviour
Educate parents
● Slow process
● May get worse before better (escalation of behaviour)
● Most important aspects are the three Cs: clear boundaries, consistency, and consequences
● Triple P (positive parenting program)
Four-pronged approach ● Environmental modulation o Remove TV from room ● prioritised behaviour o Clear boundaries (if appropriate give a warning first) o consistent consequence ● Ignore other behaviours ● Praise for good behaviours o Be specific o Immediate o Sticker charts – short term goals/can’t take stickers away o Involve child in choosing a reward
Disabled child
- exploring triggers/causes
- lessen triggers
- safe environment (padding, no hard edges) and sensory spaces
Medication
risperidone and olanzepine
doperidol
Bone health
Non-pharmocological
o increase wt bearing exercise
o diet
o R/V absorption and exclude medical cause for poor bone health eg. coeliac, renal failure
● Pharmacological:
o Supplements
▪ Calcium – caltrate 600mg OD
▪ Between meals for absorption
In chronic renal failure, may give with meals also to bind phopshate and reduce
absorption of phosphate
▪ Vitamin D - ergocalciferol/cholecalciferol
▪ Calcitriol
▪ Hormonal therapy
o Prolia (SC q6m)
o Bisphosphonates (BMD)
▪ Used for osteogenesis imperfecta initially
▪ Only if have had at least one fracture (controversial)
▪ Side effects: initial low calcium, myalgia, bone pain, fevers, avascular necrosis of the TMJ
▪ Unknown long term effects (therefore refer to endocrinology)
▪ Usually given weekly – IV palmidronate/zoledronic acid (yearly),
Tonizimab (6mnthly s/c)
▪ Monitor treatment - alkaline phosphatase
● Reduce fracture risk – optimise vision (improve lighting/contrast, eliminate glare)
o Review medication (especially sedatives/altering gait/hypotension)
o Home modification
o Exercise program
Improving communication
Formal hearing and vision assessment initially
● Regular re-assessments
● Provision of hearing aids/glasses
● Environmental modification – provide contrast/increase light
● Turn off radio/television when speaking
Involve a speech therapist
Depends on fine motor abilities and memory/ability to learn
● Access via: direct touch; fist or head pointer; light pointer
Non-aided system
● Gestures – informal (facial expression/gestures) v formal
● Key word signs – usually taken from Makaton vocabulary (varies from indicating
wants to 2-3 word sentences)
Aided system ● Non-technological o Printed words o Pictures o Tactile feeling boards o Real objects ● Low technology o Communication boards/books o Switches ● High technology o Sound picture boards - simple message systems o Computer – allow producing long messages with a few selections
Constipation
● Rule out organic causes
● Dairy protein intolerance can manifest as constipation in first three years of life
● Hirschsprungs disease
● Coeliac disease, hypothyroidism, hypercalcemia & spinal cord problems
Assess problem ● Stool chart – Bristol stages ● Toileting regime ● Anxiety associated with toileting ● Soiling ● Exam – faecal loading/FTT/neuro o Rectal exam and AXR not routine (assess tone if there is a neurological problem)
Educate
● Common problem (1 in 4 children)
o “No blame” approach
● Likely to take a long time – not a quick fix
● Continue treatment 3-4 months after regular bowel movements are achieved
o Risk of relapse once stop treatment
● Emphasises bowel/worsens behavioural aspects
Behavioural:
o Regular toileting – three times per day for 3-5 mins after meals (gastro-colic reflex)
o Star charts for positive behaviour with appropriate and timely rewards
Acute disimpaction:
Movicol 1 sachet - building up to four sachets BD (<12 years = movicol 1/2)
Daily treatment:
● Softener
Parachoc = sweet, contains soluble fibre
o 1-6 years old 10-15ml, 7-12 years old 15-20ml, adults up to 40ml/day
o >6 months: lubricant/stool softener - paraffin oil
o Colourless/ odourless, so easily disguised
o Avoid in GOR/neurological impairment (aspiration risk)
o Risk of fat soluble vitamin malabsorption with chronic use
Coloxyl
o <6 months -10 drops TDS, 6-18 months – 15 drops tds, 18-36 months – 25 drops
TDS
● Bulking –> Metamucil
●Osmotic
Lactulose
o Flatulence, abdo discomfort
o Electrolyte disturbance very uncommon
o Can use >1 month
o <1 year old - 5ml daily, 1-6 years old - 10ml daily, >6 years old - 15ml daily
movicol - Osmotic stool softener
o Nausea and vomiting, diarrhoea, cramps
o Electrolyte disturbance very uncommon
o Must be dissolved in correct amount of water (62.5ml/sachet)
● Picoprep
o Caution re electrolyte shifts
Rectal
o Glycerine suppository
o Microlax
o Usually start with parachoc/lactulose +/- sennacot
Drooling/secretions
● Conservative
o Positioning and seating (physiotherapy input)
o Speech therapy input - sucking (straw)
o Child’s oral awareness (recognise feeling of wetness)
o Tell them that they are drooling
o Swallow more frequently
o Waterproof bibs/scarves/specially designed clothes
o Orthodontic devices (poor success rate)
● Medical – anti-cholernergics
o Hyoscine butylbromide patch (SAS script)
o Benzhexol (artane) – cheaper and easier to obtain from chemist;
o Side effects: anti-cholinergic (sedation/urinary retention/constipation), thickened
saliva ( increasing risk of aspiration)
o Glycopyrulate – fewer side effects but more expensive
o Botox injections – ultrasound guided into parotid gland (under GA)
o Risk of facial nerve palsy/thickened saliva
● Surgical
o For severe drooling or failure of conservative therapy
o Preferred option = relocation of submandibular ducts with excision of sublingual
glands
o Side effects: dry mouth and dental problems (need careful follow up with dentists)
o 80% success rate
Enuresis
● Reassurance
● Stop blame – inherited
● Exclude encopresis, constipation, detrusor instability (try an anticholinergic)
● Don’t treat under 7 years old
Non-pharm
● Pad and bell for 8 weeks (70% cure, 30% relapse, better next time)
● Toilet before bed, undies only, alarm on, wake with alarm, child to turn off, go to toilet, clean and repeat
● When dry for 1/52, give a large glass of water to stress bladder and test
Desmopressin – ADH analogue
● Short term stop gap (school camp, sleep overs)
● Medium term solution if alarm has failed – use 3-6/12 then try again
● May have synergistic role with alarm
● Withdraw when vomiting, diarrhoeal illness – hyponatraemic seizures
Detrusor instability
● Girls 2-8
● Urgency and squatting
● High fluids before 5pm, frequent voiding through day, fibre, anticholinergic (probanthine or oxybutynin)
Hearing impairment
Management
● Multidisciplinary – monitor linguistic and social development
● Treat underlying cause
● Otitis media – decrease smoking, daycare and dummies
● Antibiotics – one month course
Non-pharm
Decrease background noise
● Use louder than normal voice
● Protect hearing – ear plugs with loud noise
Early intervention programs - speech therapy
safety - cross roads with child
Medication
● Early treatment of otitis media
Surgery
Grommets
short term symptomatic benefit – no convincing long term benefit
● May help in children with other disabilities – decreased IQ, neuro/cleft
● Perform if bilateral >30db conductive hearing loss with recurrent otitis media/ externa
50dB = amplification
● Hearing aids for conductive and sensorineural hearing loss
● If not compliant – may be ok if school performance ok
● Cochlear implants – stimulate auditory nerve – aim for <2 years
80dB = alternative form of communiciation
● Lip reading/signing
Reflux
Non-pharm
● Feed thickening - meta-analysis shows that this has some efficacy in reducing reflux episodes and also increases the caloric density of feeds
● Positioning
● Optimise volume of feeds/ method (bolus vs continuous, gastric vs jej)
● Post-pyloric feeds
● All reflux tends to improve with age
Meds
● PPIs are more effective than H 2 blockers – decrease acidity and gastric volumes
Surgery
● Fundoplication is reasonably efficacious with 60-90% of children improving after surgery - however there is a reasonably high complication and reoperation rate.
● Post-pyloric feeds
scoliosis
Monitoring
● Yearly x-rays (Cobb angle) +/- pulmonary function tests
● Cobbs measure – take angle of top and bottom of deformity
● Severity - 25-40 moderate, >40 significant
Treatment based on…
● Location – thoracic more likely to worsen than thoracolumbar or lumbar
● Maturity – brace if still young
● Potential to progress (neuromuscular, before growth spurt)
Treatments
● Observe (small curves, low risk of progression)
o Annual x-ray as a child, 5-yearly as an adult
● Idiopathic <30 watch, consider brace, >40consider surgery
● Conservative - physiotherapy – stretching and strengthening exercises
● Bracing – 25-40with growth still happening
o Prevents further but doesn’t correct
o Succeeds in 80% of cases
o Risk - pressure sores/respiratory compromise
o Requires chair that fits the curve (to minimise progression)
● Surgery if >40 /progression/affecting QOL/non-brace respondent
o Posterior spinal fusion
▪ Using bone graft from hip, several hours
▪ Most don’t need bracing after, out of hospital in one week, back to school in 2-4
weeks, back to total function in 4-6 months
o Anterior spinal fusion
▪ Deflate lung, remove rib, anterior approach
▪ Shorter recovery, better cosmetic, less segments, but need brace for several months
o Risks – death, respiratory failure, infection, protrusion of metalware
spasticity
Conservative(more effective for localised)
● Physiotherapy – stretching exercises
● Orthoses/serial casting – AFO (initially at night)
● Equipment: patient specific modifications for home/ sleep/ wheelchair. padding between legs to prevent sweeping
● Must be well-fitting or risk of pressure sores
Medical therapy
● Aims are to:
o Reduce spasticity to reduce pain and make care easier (possible)
o Prevent contractures and to improve function (unknown if current drugs do this)
● Better evidence for botox than oral drugs and has significantly fewer complications
● Botox, baclofen (oral/intrathecal), benzodiazepines, dantrolene (NB potentiates sedative effect benzos, interacts with Ca channel blockers)
o Botox 4-6 monthly
o Baclofen IT- programmable pump (continuous)
● Dystonia – baclofen/benzhexol/L-dopa
● Pain management: AS above, gabapentin
Surgical intervention
● Selective dorsal rhizotomy is the sectioning of the sensory nerve root which interrupts
the afferent arc causing spasticity, thus spasticity is reduced without affecting motor
function. Has good long term effects but which patients benefit most is unknown (i.e.
whether to operate only on severely affected patients)
● Monitoring for hip subluxation 6-monthly from 18 months, and early corrective surgery
on tight adductors and iliopsoas
● Muscle and tendon lengthening and transfer usually done once mature (8+ years) to
improve gait may include surgery on hamstrings, calves. Planning involves gait
analysis.
Acne
Simple measures
● Avoid precipitants (non-comodegenic beauty products)
● Don’t pick (causes scars)
● Regular cleansing - OTC cleansers (benzylperoxidase)
Topical antibiotics
● Clindamycin/erythromycin
Oral antibiotics
● Minocycline (avoid pregnancy/dry skin/sun sensitivity)
● Use for at least 3 months
Hormonal
● OCP - may help if premenstrual flares
Iso-retinoin
● Topical – often combined with antibiotic
● 10-20% have local irritation
Roaccutane
● Oral – for severe cystic acne
● Has to be prescribed by dermatologist for severe (cystic) acne
● Side effects: dry skin, sun sensitivity; teratogenicity (must be on at least two forms of contraception); depression
Bullying
Explore the underlying causes and fix if able
● Smelly PEG/drooling
Increase protective factors
● Increase self esteem
● Highlight the child’s strengths e.g. music, sports, art
● Assertiveness/confidence skills through counseling
● Increase socialisation and supports e.g. peer groups
● Ensure a positive family environment to provide support at home
● Liaise with the school – consider a ‘buddy’
● Find a trustworthy adult to report to
Reduce the bullying behaviour
● School policy - discuss with school
● Avoid situations that exacerbate it
● If really bad – may need to change schools
Compliance
When discussing medications explain that poor adherence is normal
Identify barriers
● Lack of understanding of long term complications
● Short term seen as more important than long term
● Educate
● Side effects or dislike taste
o Consider changing medication
● Doesn’t fit into their lifestyle – many medications/complicated regime
o Make regime as simple as possible and lifestyle friendly
o Negotiate regime with adolescent
● Forgetful
o Suggest reminders
o Enlist the support of others with adolescent’s approval
● Teasing at school
o Try to avoid school time medications or work with teacher to allow taking of medications privately
● Depression
o Seek appropriate treatment
Start increasing adolescents autonomy over illness
● Start seeing them alone and discuss confidentiality
● Re-educate (often they miss out on understanding as the problems begin as child or neonate) - explore their understanding of illness and correct any misunderstandings
● Educate about medications
Simplify the regime
● Reduce the number of medications (cease any that aren’t providing benefit)
● Reduce the frequency of dosing
● Tailor to their daily routine (avoid school-time medications)
● Involve the adolescent in the decision-making process (be flexible)
● Manage side effects
Increase their access to medications
● Oral and written instructions (avoid jargon)
● Keep medications by items of daily use (clock/toothbrush)
● Make it part of their routine (during ad break of favourite programme)
● Negotiate the role of parents that is acceptable to the adolescent
● Set alarms on phone (every adolescent now has a mobile phone that stays with them, turned on, 24/7)
Ensure they have health care card
● Focus on short term goals and follow up
● Encourage and praise
● Negotiate short term goals
● Medication free periods – ADHD on school holidays
School refusal
●Ensure parental support – agree on a time schedule
o Immediate if refusal period has been brief
o Graded exposure if longer (parent in class - tearoom - phone contact)
● Acknowledge reality of feelings
● Parent involvement - calm morning routines and escort to school
● School involvement:
o Try hard to keep child at same school
o Special supports (modified curriculum, reduced homework)
o Give child active tasks (i.e. lunch orders) therefore need to be there
● Child:
o Relaxation training/breathing retraining
o Social skills training
o Praise +/- graded reward system (star chart)
● Adolescents – consider alternative education options, e.g. TAFE, apprenticeship
● Monitor for mental health symptoms – anxiety, depression
● Referral to multidisciplinary mental health team for longer term school refusal
o If no improvement after two weeks or you suspect a more severe mental health
● Regular monitoring of progress and signs of relapse
Fussy eating
Dietary advice
● Consumption of excessive fluid reduces the intake of solid foods, so offer solids
before liquids
● Limit intake of juice, and carbonated drinks
● If weight is ok, offer water, if failure to thrive offer milk or formula instead
● Variety is not important
● Offer foods that are easy to handle (e.g, Cheerios, French fries, slices of banana, or peas)
Feeding times
● Children need to eat often, not constantly
● Offer something every two to three hours, to allow three meals and two to three snacks per day
● Avoid snacks right after an unfinished meal
● Children work well with schedules - try to keep mealtimes and snack times about the same each day.
● Allow one hour without food or drink (except water) before a meal to stimulate the appetite.
● Do not make mealtime too long - 15 minutes is probably long enough for a toddler
Feeding behavior
● Try to relax; feeding/eating and mealtimes should be pleasant for everyone
● Recognise the childs cues indicating hunger, satiety, and food preferences
● Parents are responsible for deciding what food the child is offered (with consideration for the childs preferences); the child decides how much to eat
● Avoid battles over eating - encourage, but avoid forced feeding, threatening, bribing, or punitive approaches; instead, use positive reinforcement (e.g. praise for eating
well)
● Do not withhold food as a form of punishment
● Allow the child to feed himself or herself - try very small amounts at first, offer seconds later.
● Expect messiness and be prepared for easy clean-up (bibs, newspaper under high chair, etc)
growth
Management:
● Ensure intake is adequate
● Manage chronic disease
● Ensure losses are reasonable (not malabsorping)
Growth hormone principles
● Short term catch up, long term linear growth
● Achieve familial potential
Indications (given daily >6 days/wk)
● <1st centile and growth velocity <25th over 1 year
● Usually need three heights over a year, bone age
● Radiotherapy/pituitary surgery
o Male >15 years old and bone age >13 years
o Female >12.5 years old and bone age >10.5 years
o And velocity <25th centile over 6 months
● Renal
o GFR <30ml/min/m2
o And velocity <25th and height <25th
● Turner syndrome
o <95th centile on Turner syndrome charts
Side effects
● Slipped upper femoral epiphysis, benign intracranial hypertension, gynaecomastia,
reversible hypothyroid
● IGF1 – increased risk of prostate, breast, colon cancer
Stop treatment if:
● Bone age >13.5 in girls, >15.5 in boys
● >10th centile for adult height
● No response (<4cm/yr)
Immunosuppressants.
Monitor for specific adverse effects
● Mycophenolate - GI upset, leucopenia
● Tacrolimus - renal impairment, hyperglycemia, hypomagnesemia, neuro (seizures,
tremors) does not increase cholesterol, is CYP450 substrate
● Cyclosporin - renal impairment, “hyper” - tension, trichosis, kalemia, plasia of gingiva -
and hypomagnesemia
● Methotrexate - mucositis or ulcers, bone marrow suppression, heptatotoxicity
● Not:
o Lipids with CSA despite previous teaching
o Infertility unless cyclophosphamide
In practice:
● Clinical and laboratory monitoring is baseline BP and bloods (FBE, UEC, LFT), two- weekly for the first few months and then two-monthly after that
● If adverse effects - reduction in dose, changing of drug or management of known adverse effects
Monitoring for leading causes of death (proportional to degree of immunosuppression)
● Infection - especially EBV, CMV, PCP (less on prophylaxis)
● Post-transplant lymphoproliferative disorder
● Cardiovascular risk factor reduction (major cause of death in adult transplant patients)
● Sunscreen and skin cancer monitoring
● Immunisations (not varicella if immunosuppressed per previously mentioned criteria) including for family
Irritability
Differential diagnosis:
● GORD, constipation, dental, fractures, pressure sores, otitis media
Treatments:
● Behaviour - sleep hygiene, routine
● Environment - light, music
● Alternatives – melatonin, massage, acupuncture
● Treatment – diazepam, paracetamol, baclofen
Menstruation
Treatment:
● Sanitary pads, nappies (suppression is an option)
● Heavy bleeding - NSAIDs (by 30%) or tranexamic acid (by 50%)
● Contraception - reduces frequency of periods
● Reduce dysmenorrhea
● Control timing
● OCP (daily) – compliance may be an issue
o Beneficial for bones
o Risk of thromboembolism
o May require dose adjustment if on anticonvulsants
● Transdermal patch (weekly) or Depo Provera (IM 3 monthly)
o Side effects - mood, pain, weight gain, spotting
● Implants (3-5 years)
● Mirena (progesterone only device) – amenorrhea ~5 yrs
o May have spotting
● Intrauterine devices – not recommended if cognitive impairment
o Can’t report pain
o Side effects - menorrhagia/dysmenorrhea
Remember -need for a concurrent barrier protection against STDs
Menorrhagia
● Change super pads every two hours
● Education is dependent on the response to blood/toileting/intellectual capabilities
● FBC, iron, TFT, APTT, PTT, von Willebrand factor, platelet function
● Treatment – NSAIDs (reduction in 30%), tranexamic acid (reduction in 50%), OCP, Depo, implanon)
Needle phobia
Address fears
Trauma of procedure
● Use different room
● Anaesthetic cream/nitrous/midazolam/sucrose and swaddle
● Experienced personnel with equipment prepared earlier
● Consider a long line if repeated access is required
Coping strategies
Child
● Prepare: demonstrate on a doll first
● During - play therapist for distraction techniques
● Blow away the pain
Adolescent
● Prepare - plan including who will be in the room/techniques
● During - breathing exercises/hypnotherapy
● Distraction - rub on the site to close pain gate
Parents role
● Educate parents about what not to say (no bargaining/apologies/threats)
● Encourage to keep calm and their main role is as distraction
● Not to be involved in restraining the patient
Note: consider long line/port/CVC if repeated access is required.
obesity
Attitudes and motivation (both patient and family), especially where there is parental obesity
Overall goals
● Establishment of permanent healthy lifestyle habits
● Improvement in medical and psychological complications of obesity
BMI as primary measure – aim for static weight gain (grow into height)
Parental participation vital as role models/authority figures and purchasers of food (create environment)
Age appropriate portions in toddlers and stroller in pre-schoolers
General measures:
● Limit sugary beverages
● Energy dense food
● Avoid dining out (especially fast food)
● Encourage breakfast every day
● Fruit and veggies (aim for 5 times per day)
● Family meals/eating together (associated with higher quality diet)
● Self-regulation of portions (parents decide what, child decides how much)
● Limit TV and other screen time (<2 hrs/day)
● Exercise (60 mins/day)
If overweight, aim is weight maintenance (decrease in BMI as gets older, height increases)
● Ensure monthly follow-up
● If no improvement in BMI or weight in 3-6 months, seek dietician involvement
● Ask what they think they could do
● Exercise, dog walks, stairs, decrease screen time
● Nutrition and drinks – healthy snacks
● Follow up
oral aversion
● Eliminate the underlying cause if possible (NG tube/O 2 tubes/nausea/GORD)
● Better to avoid food if there is an active issue (can cause long-term aversion)
● Involve a speech pathologist and dietician
Educate parents
● May take a long time to improve
Start introducing food at an early age
● Make it fun - play therapy to introduce foods of different tastes, textures and smells
(lay it on a mat on the ground and they can play with it)
● Have this done during family meals – also improve bonding of the child with family, understands the concept of meal time, and that eating is fun
Monitor growth and nutritional status
Monitor dental health
pain
Assess pain
- FLACC behavioural pain assessment tool (infants/disabled)
- FACES (4-7)
- numerical
type of pain
- acute vs chronic
- nocipathic vs neuropathic
Non-pharmacological measures
● Stress aim is to reduce the impact of pain rather than the pain
● Develop life-coping skills
● CBT - controlled breathing, sleep, exercise
● Visualisation techniques – turning pain down/cutting off painful part
● Distraction techniques
● Heat packs/cool packs
● Exercise/physiotherapy – stretching exercises
● Hypnosis
● Protective factors – supportive family/activities they enjoy
● Treat co-morbidities – anxiety/depression
● Set realistic goals and reward
Pharmacological
Analgesic ladder - paracetamol/NSAID
Includes opiates (beware addiction but never under-medicate)
Neuropathic pain - amitryptilline/gabapentin/pregabalin
opioid rotation if side effects
breakthrough: 1/6-1/8 of daily dose
steroid side effects
CUSHINGOID MAP ● Cataract/cushingoid face – moon face/interscapular adiposity ● Ulcer ● Striae/skin thinning ● Hypertension ● Infection – thrush ● Necrosis of bone (avascular) ● Growth - short stature ● Osteoporosis/obesity ● ICP (pseudo-tumour cerebri) ● DM- glucose intolerance ● Myopathy(proximal)/moon face/mood ● Adipose tissue hypertrophy/acne ● Pancreatitis/psychosis – behavioural problems
Management
● Minimise dose – get off ASAP and use steroid sparing agents
● Monitor for side effects:
● Regular growth – height and weight
● Mood and behavior
● Blood pressure
● Urinalysis/BSL
● Functional muscle strength – walking up stairs
● Annual – ophthalmology
● Bone mineral densitometry
o 1SD below (osteopenia) = 2x risk of fractures
o 2SD below (osteoporosis) = 4x risk of fractures
o 3SD below = 8x risk of fractures
General
● Diet – beware of polyphagia
o Vitamin D and calcium supplements +/- bisphosphonates
● Immunisations (pneumococcus/meningococcus/influenza)
o Avoid live vaccines (vaccinate family against varicella)
● Education – risk of addiction, remember not to stop steroids suddenly, may require increased dose when sick, medic-alert bracelet
● Pregnancy – causes neonatal cleft palate and adrenal insufficiency - avoid if possible (especially early pregnancy), safe for breast-feeding
● Drug interactions – if with OCP results in hypertension
Specific
● Antihypertensives
● Growth hormone
● Hypoglycaemic agents
substance use
Approach
● Be on their side – don’t condone it
● Be realistic and negotiate
● Adopt long term approach and involve the family
● Understand high-risk behaviour and associations with other behaviours
Assessment
● Degree – Experimentation/use/abuse/dependence/addiction
● Functional impact e.g. deterioration of academic performance, alcoholic hepatitis,
other risk taking behaviour
● Motivation stage – pre-contemplation, contemplation, readiness to change
Education/ re-education on condition
● Adolescents have often missed out on important first hand education if condition was diagnosed as a child
Emphasise safe and responsible drinking
● Drink in groups
● Know what you’re drinking and watch it at all time
● Don’t mix drinks
● Medic-alert bracelet (diabetes, renal failure, adrenal insufficiency)
● Ask them about their aspirations in life
Give strategies to say “no”
● Tackle peer pressure issue
● Do her friends know about her underlying condition?
● Do they accept her to say no?
● If bad friends - explore over long period of time
o Challenge why they are friends (if making fun of you)
o Find a good role model
Offer supports
● QUIT line/alcoholic anonymous – 24 hours service
● Youth Drug & Alcohol services
● Involve psychologist, counsellors (school/community/youth workers)
Encourage other healthy activities
● Youth group involvement – camps, abseiling recreational courses
● Music/art groups - opportunity to meet new friends
● Peer support groups for youths with chronic illnesses
Transition
● start early 13-14yrs
● Aim for completion post final year of school or 18 years
● Be flexible about timing
o Should be when geographically stable and no other major stressors
o Slower with intellectual impairment
● Preparing
o Flag
o Check lists/ targets to progress discussion (preparing for adulthood checklist)
o Assess awareness of condition, consider giving access to pt record/ transition packs
o Move to adolescent speaking for themselves and having time without carer
o Discuss adolescent issues
o Allow them to decide who to be transitioned to
● Be aware of barriers
o Patient factors: readiness, motivation, parental and adolescent anxieties, capacity
o Condition: increasing complexity, increasing difficulty with transition
o Doctor
o Service availability
● Models:
Transition care service of Agency for clinical innovation (14-25)
o Joint clinics
o Member of paeds team accompanies on 1st adult clinic
o SW accompanied visits to adult hospital/ inpatient area
o Paeds review post 1 adult OPD to ensure needs met/ discuss concerns
o Assign key contact to take responsibility for smooth transition/ f/u if DNA
▪ normally a specific health care provider
o Can visit adult physician as a trial before committing
● Address adolescent issues - genetics/sex/drug issues/employment
● Involve social workers
Recurrent UTI
● Simple measures: improve perineal hygiene (wipe front to back)
● Fluid intake/treat constipation
● Behavioural modification – regular toileting
● Prophylactic antibiotics: if in nappies (<50% compliance)
o Controversial but evidence shows no effect
● Alternative: home dipsticks with script (but always take sample to check resistances)
● If intermittent catheterisation - check sterile technique, regular cultures, frequency (residual volume)
body image issues
Explore and address the underlying causes
● Drooling/depression – treat medically
● School bullying/no friends – tackle these as priorities
● PEG/scars/striae – wear clothing that hides
Enhance protective factors
● Highlight the child’s strengths e.g. music, sports, art
● Increase socialisation and supports e.g. peer groups
● CHIPS – look good, feel great programme
● Does the child have a role model to use as motivator?
● Liaise with school – consider a ‘buddy’
● Try not to label the child as different (everyone has things that makes them unique)
Advanced care / resus
Very confronting issue
● Requires good rapport
● Broach in advance when well/stable over many sessions for a patients whose resus
would likely result in poor quality of life
● When it won’t change the outcome (unnecessarily invasive) i.e. terminal
Establish the parents views
- any aims for child such as making a milestone or a holiday
- assess readiness for discussion
Educate
● What is an arrest/what is CPR?
● Ensure parents have reliable information
● Allow time to correct any misbeliefs obtained from Google/other parents
Offer treatment options from nothing to full resuscitation
Paediatrician roleis to make recommendations and guide parents
● Involve all those involved in patient care
● Aim to optimise the patients quality of life (pain free/suctioning/transfusions)
focus on what will be done, not what we wont do.
● Advanced care directive should be documented clearly in the notes and correspondence
● Revisit frequently, especially if there is a change in condition
● Support the child (play therapy/psychologist)
● Continue to support parents through bereavement and grief
● Maintain a presence
● Normalise emotions
● Make practical arrangements for when the child does pass away (i.e. transport to hospital)
● N.B. quality of life = subjective
● You can say that a patient is pain-free and in a loving and caring environment
“voicing my choices” plan for adolescents
- document that helps to go through aspects of advanced care plan and how they want to be remembered
Transplant
Monitor for rejection
Highest in first year
Manifests as graft failure - eg oliguria, hypertension, rising creatinine in renal
Graft inflammation - pain, fever
May be related to compliance, especially adolescent patients
Monitor for complications of immunosuppression
Growth and growth hormone
cognitive testing
Important to individualised learning plan and for child to reach their best potential (academic and employment). Also how we interact with the child (ie making care plan, talking about illness)
If no formal testing can look at school report
Normal child (rely on expressive language) - Wechsler Intelligence Scale for Children - 5th ed. ages 6-16yrs and 11m
- Wechsler Preschool and Primary Scale of Intelligence – Fourth Edition (WPPSI-IV, Australian Standard) for children aged 2 years and 6 months to 7 years.
- Wechsler Individual Achievement Test (WIAT) to assist in determining a child’s academic abilities.
- Bracken School Readiness Assessment
Disabled child
- Peabody Picture Vocabulary Test 4 (PPVT 4)
measures receptive language
ages 2yrs 6m and above
Raven’s Standard Progressive Matrices (RSPM)
designed to test non-verbal, abstract and cognitive functioning
age 6 and up
developmental testing
Bayley Scales
Age range 1-42months
Purpose to identify developmental delay, not to diagnose specific disorder
3 scales – motor, mental, behaviour
Griffith developmental assessment
Birth-8yrs
Adaptive behaviour assessment system
Used to assess functional skills necessary for daily living
Age 0-89
Autism scales
Modified Checklist for Autism in Toddlers – Revised
2-stage parent screening tool to assess for Autism Spectrum Disorder (ASD). identify children 16 to 30 months of age who should receive a more thorough assessment for possible early signs of autism spectrum disorder (ASD) or developmental delay
The Child Autism Rating Scale (CARS)
Brief assessment suitable for use with any child over 2 years of age. CARS includes items drawn from five prominent systems for diagnosing autism; each item covers a particular characteristic, ability, or behavior.
The Autism Diagnostic Observation Schedule (ADOS)
A semi-structured, standardized assessment of social interaction, communication, play, and imaginative use of materials for individuals suspected of having ASD. The observational schedule consists of four 30-minute modules, each designed to be administered to different individuals according to their level of expressive language.
Autism Detection in Early Childhood (ADEC)
The Autism Diagnostic Interview – revised (ADI-R)
A clinical diagnostic instrument for assessing autism in children and adults. The instrument focuses on behavior in three main areas: reciprocal social interaction; communication and language; and restricted and repetitive, stereotyped interests and behaviors. The ADI-R is appropriate for children and adults with mental ages about 18 months and above.
The Social Communication Questionnaire (SCQ).
Weight management
diet and activity history including screen time
8 healthy habits for healthy kids
- drink water instead of sugary drinks
- Aim to eat at least 5 serves of vegetables and 2 serves of fruit every day (For 2-3 year olds, eat 21⁄2 serves of vegetables and 1 serve of fruit)
- Start each day with a healthy breakfast
- Know your portion/serve size
- Choose healthier snacks and fewer treat foods
- Limit screen time (no more than 1 hour a day for 2–5 year olds, and up to 2 hours a day for children 6 years and older)
- Be active for at least 1 hour a day, every day
- Get enough sleep (Recommended over 24 hours: 10-13 hours for 3-5 years; Recommended per night: 9-11 hours for 5-13 years; 8-10 hours for 14-17 years)
Respite and relieving burden
assess carers willingness to accept respite
Assess barriers for respite
- previous experiences
- trust issues
- making a care plan with the parents to help address specific concerns - signs of illness, management plans
formal and informal respite
- formal respite. In home, centre based (northcott childrens respite centre), community access, disease specific camps
- emergency respite
- informal –> grandparents, friends
- activities –> swimming, dance class
- school
Consent
fact sheet from council for intellectual disability
child can consent for medical treatment from 14 (gillicks principal) (can only refuse from 18)
parent can give consent up to 16yrs of age
over 16 it is person responsible 1. guardian 2. spouse 3. non-paid carer 4. close friend or relative if no person responsible to guardianship tribunal
Mistrust of medical services
explore reasons for mistrust
apologise that they had that experience
emphasise team based approach for childs care and frequently explore parents priorities
- ED management
social work
MDT meetings
culturally appropriate care
acute presentation/deterioration in chronic illness/life limiting illness
1 on 1 discussion with carers, involve social workers
discuss what your expected outcomes would be for the child with certain interventions
MDT with intensivist, palliative care. senior medical at your service
ethics board is the ultimate end point if you dont agree
investigation for intellectual diasability
first level screening (fragile x, metaboilc, mixroarray),(first line: 1-2% find cause,
next whole exome sequencing 50-60%
failure to thrive
endocrine
- TSH, GH, cortisol
gastro
- malabsorption –> faecal elastase (pancreatic)
chronic disease
- inflam markers
- too much expenditure for intake
- cardiac, resp, renal
intake
nutritional studies
diagnostic uncertainty
difficulties for family
- future family planning
- diagnosis for future planning
- funding availability
review current investigations
add some that need to be done
post bone marrow transplant
complications of preparative regimens
total body radiation
- endocrine - central and periphery thyroid/gonadal
- cardiomyopathy
- lung disease
- neurocognitive
- solid tumours - particularly thyroid
- impaired fertility
chemo - cardiomyopathy - renal - neuropathy - regular blood tests - risk of malignancy skin check annually. -and skin safety
steroids
complication of blood transfusion
- iron overload and ferritin
- be cognisant of risk of infection
oral aversion
cause
- lack of stimulation leads to sensitivity
- can sometime be due to oral pain or vomiting giving negative re-inforcement
management
- speech pathology assessment to adress specific oromotor concern
- investigate for causes of negative re-inforcement such as reflux, poor dentition.
- positive feeding environment and no force feeding. positive reinforcement usually works better.
- meal enviroment: sit in high chair or at table for meal times
- encourage teethers/chew toys and Practice and play with pretend foods and toys, bringing them to your mouth, outside of meal time
- use bottle –> sippy cup –> thicken feed through same to increase exposure
- gradualy increase exposure to other textures
Care structure
important to have a 3 tiered care approach
primary - GP - first port of call - immunisation secondary - gen paed - development - care coordination tertiary - specialist
increase social development
school
- daycare or normal school
- may need extra carers to be able to attend/extra funding
- may need reschedualig of care needs
- medical team to develop care plan to make care easier at school
other groups
- ie swimming
- play group
- mothers group
management of poor sleep
assess issue
rule out medical cause
- Stick to the same bedtime and wake time every day, even on weekends.
- Beds are for sleeping only.
- A comfy, cozy room. - cool, quiet and comfortable
- Alarm clocks are for waking up. - not for staring at while waiting to go to sleep
- Bedtime routine. A predictable series of events should lead up to bedtime. This can include brushing teeth, putting on pajamas, and reading a story from a book.
- Quiet, calm, and relaxing activities. No screen time before bed
- How to relax. meditation apps
- exercise and active in the day
- Avoid caffeine.
- Bedtime checkups should be short and sweet.
- Maintain a sleep diary
grief in intellectually disabled
acknowledge difficulties with communication
assessment
- looking at way this child has had behaviour, mood, emotional changes since the event
- idenitifying the carer who might be able to idenitfiy this change
- a symptom diary may help - mood, behaviour, sleep, appetite
- familial mental health
investigation
- assessment with psychologist
treatment
- aids to cmmonuication
- -> stories - books
- –> online
- ->
- psychology
- sleep
- healthy activity - exercise
- suporting family
- maintain a routine
- help child to recognise what they are feeling
- medication maybe appropriate
how to manage grief/palliative diagnosis in neurocognitive child
assess
- assess baseline understanding
- assessing current behaviour and feelings/emotional - aknowledging and normalising
investigate
- psychology
- palliative care
treatment
- psychology
- routine
- planning for the future/end of life
general health screening
development / educational nutrition and growth hearing and vision dental immunisation mental health sleep
immunosuppression - health promotion
reduce infection
- family vaccinated
- personal vaccinations
- emergency plan in case of fever
- prophylactic antibiotics or immunoglobulin
developmental oppportunity
- family base daycare vs large centre
- other large centres - including pools
food
- no soft cheeses or oysters
- undercooked meat
dental care
line care
- flushes - port monthly, central line weekly
- removing if not using
- abx locks
approach to parents who are not motivated to manage a medical condition
behavioural changes
- barriers to change
- -> uderstnading of risk and priorities - educate family
- -> timing –> alarms, written plans
- -> environemnt - is school hard
- -> identifying the person in charge of management: if the child may be age inappropriate
- -> motivation to change
- build rapport and trust
- frequently address
Child protection concern
-give the fmaily a chance to adopt changes and if i am concerned then report to child protection
Child protection concern
assess
- barriers to care
- previous methods tried
utilise a medical admission to further clarify issues
MRG, CWU or hepline report
child who tends to abscond
assess triggers
- senosry - noise, lights etc
- emotional - anxiety
mx
- appropriate carers present
- when little may be able to have a lead
- improve communication
- sensory dampening –> tents, weighted blanket
- safe environment - avoid dangerous roads, pools etc
procedural trauma and anxiety
Address fears
Trauma of procedure
-Use different room
-Anaesthetic cream/nitrous/midazolam/sucrose and swaddle
-Experienced personnel with equipment prepared earlier
-Consider a long line if repeated access is required
Coping strategies
Child
-Prepare: demonstrate on a doll first
-During - play therapist for distraction techniques
-Blow away the pain
Adolescent
-Prepare - plan including who will be in the room/techniques
-During - breathing exercises/hypnotherapy
-Distraction - rub on the site to close pain gate
Parents’ role
-Educate parents about what not to say (no bargaining/apologies/threats)
-Encourage to keep calm and their main role is as distraction
Not to be involved in restraining the patient
Note: consider long line/port/CVC if repeated access is required
resillience program - CHIRP Child illness and resillience program in Newcastl
child life therapy
Specific management issues for renal case
• Fluid and electrolytes • Growth and nutrition • Bone disease • Anaemia Blood pressure
causes of growth failure in renal disease
○ Protein and energy def ○ Water and electrolyte disturbance ○ Acidosis ○ CKD bone mineral disorder ○ Hypothyroidism
Life skills
executive functioning - keeping to a schedule Home safety - cooking - locks Transport - walking route - public transport Money management - debit card etc
chronically immunosuppressed - how to keep them well
monitoring for medication side effects Infection -emergency management plan in case of fever, ED management plan -exposures to large groups, foods, swimming pool -line care -compliance with immunisation and prophylactic antibiotics Mental health Growth Hearing and vision dental Development Education Social
