Management Plans

Question 1

Acne

Answer 1

Assess severity and extent that it is an issue.

Simple measures:

• Eat plenty of fresh fruit and vegetables.
• Avoid comodegenic beauty products.
• Regular cleansing - OTC preparations
• Stop smoking.
• May take weeks to months to help.

Topical antibiotics:

• Clindamycin/erythromycin - best used with benzoyl peroxidase/azelic acid to decrease resistance.

Oral antibiotics:

• Oral doxycycline (minocycline partially funded).
• Use for at least 3 months.
• Sensitivity to sun, oesophageal irritation.

Hormonal:

• OCP with cyproterone (Ginette)
• May help with premenstrual flares.

Iso-retinoin:

• Topical - often combined with antibiotic.
• 10-20% local skin irritation.

Isotretinoin:

• For severe cystic acne.
• Require 2x contraception - teratogen.
• Depression.
• Dry skin and sun sensitivity.
• Monitor for pregnancy, LFT’s and lipids.

Question 2

Adherence

Answer 2

Medication, chest physiotherapy, appointments.

Important for long term care but need short term goals and reinforcement.

Consider as an issue in adolescence and any child moving out of home.

Approach:

• Explain poor adherence with medications is normal.
• Identify barriers:
  
  • Lack of understanding of long term complications.
  • Short term seen as more important than long term.
    
    • Educate.
  • Side effects.
  • Doesn’t fit in to lifestyle - many medications/complicated regime:
    
    • Simplify regime as much as possible.
    • Negotiate regime with patient.
  • Forgetful.
  • Teasing at school:
    
    • Avoid school time medications.
    • Work with school on taking meds privately.
  • Depression:
    
    • Seek appropriate treatment.
• Start increasing autonomy over illness:
  
  • Start seeing alone and discuss confidentiality.
  • Re-educate - often miss out on undertanding of issues in illnesses that start as a child.
  • Explore and correct misunderstandings around illness.
  • Educate about medications.
• Simplify regime:
  
  • Decrease number of medications.
  • Decrease frequency of dosing.
  • Tailor to daily routine (avoid school time).
  • Involve adolescent in decision making process.
  • Manage side-effects.
• Increase access to medications:
  
  • Oral and written instructions.
  • Keep by items of daily use.
  • Make part of routine.
  • Negotiate role of parents that is acceptable to adolescent.
  • Set alarms on phone.
• Ensure they have community services card.
• Focus on short term goals and follow-up:
  
  • Encourage and praise.
  • Negotiate short term goals.
  • Medication free periods.
• “Tom is now 13 years old and it would be appropriate for him to start taking over the administration of his medications. I would like to enhance compliance by increasing Tom’s ownership of the illness. I would do this by spending more appointments alone with Tom and exploring his understanding of his condition. I would ensure that he has a complete understanding of both his condition and the purpose of each medication.*
• I would review his current regime and ensure that it is as simple as possible. In Tom this would include trying to decrease his frusemide to twice daily dosing so that he doesn’t need to take medications at school.*
• I wouldalso make sure he wasn’t experiencing any unwanted effects that might prevent him from being compliant such as frequent toileting or teasing for this at school.*
• I would provide written instuctions in addition to oral instructions.*
• I would provide Tom with a short term goal such as trying his current regime for at least 1 month and assessing the impact that has on his breathlessness during sports. Regular review would be important”.*

Question 3

Aspiration

Answer 3

Contributes to recurrent pneumonia in neurologically impaired children.

From above - swallow dysfunction, or below - GOR

Investigations:

• Plain CXR or CT - changes in dependent areas.
• Aspiration:
  
  • Video fluoroscopy - only investigation correlated with decreased aspiration.
  • Milk study.
  • Saliva study.
• GOR:
  
  • pH probe.
  • Endoscopy if does not respond to conservative therapy.
  • Barium - only useful in structural cause.
  • Trial of therapy.

Management:

• Aspiration:
  
  • Feed position
  • Thickening
  • Reduce volumes
  • Percutaneous feeds to bypass upper GIT.
• GOR:
  
  • Feed thickening
  • Proton pump inhibitor ( more efficacious than H2 blockers)
  • Fundoplification (60-90%) improve.
  • Post-pyloric feeds

Question 4

Augmentative communication

Answer 4

Basics:

• Formal hearing and vision assessment initially.
  
  • Regular reassessments.
  • Provision of hearing aides and glasses.
  • Environmental modification - provide contrast, increase light, turn off radia/television when speaking.
• Involve 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 vs. formal
• Key word signs (Makaton vocabulary)

Aided system:

• Non-technical
  
  • Printed words, pictures, tactile feeling boards, real objects.
• Low tech
  
  • Communication boards/books, switches.
• Hi tech
  
  • Sound picture books, computer.

Resources - Talk Link

Question 5

Behavioural problems

Answer 5

Prioritise problem behaviours:

• List all that parents would like to change.
• Select 2 most troubling (consider dangerousness).
• Tackle sleep first.
• Ignore others and reassure they will be addressed in time.

Educate parents:

• Slow process.
• May get worse before improving.
• Stabilise routines.
• Have specific time for the child each day.
• 3 C’s - clear boundaries, consistency, consequences.

4 pronged approach:

• Environmental modulation.
• Time out for prioritised behaviour:
  
  • Clear boundaries.
  • Safe but boring.
  • Min/year of age up to 5min.
  • Takes 1-2 months to work.
  • >5y removal of priviledges more effective.
• Ignore other behaviours:
  
  • Difficult.
  • Avoid eye contact.
  • Walk away.
• Praise for good behaviour:
  
  • Specific.
  • Immediate.
  • Sticker charts.

Early review.

For tantrums:

• Ensure safe.
• Completely ignore.
• +ve reinforcement the moment they stop.
• “Firstly I would get the parents to prioritise the problem behaviours by listing all the things they would like to change and choosing 2 they would like to target first. This should take in to account the dangerousness of the behaviours.*
• I would warn the parents that behavioural change is a slow process and that the problem behaviour may get worse before it gets better. I would emphasise that the most important aspect in implementing a behavioural change programme is consistency.*
• My usual approach is a 4 pronged approach including environmental modification, time out for proritised behaviours, ignoring other behaviours and praise for good pehaviour.*
• For this child, his biggest problem was running away. I would modify the environment by ensuring the property is safe and an appropriate fence erected. Time out should be used for any attempts to run away. This should be an age-appropriate amount of time (1min/year) in a safe but boring environment such as a hallway or corner. Othe behaviours that have not been prioritised such as the childs tv watching should be ignored. Parents often find this difficult and I would advise the parents to distract themselves and remove themselves from the room if they find they are getting irritated by the behaviour.*
• I would also like to emphasise the importance of praise for good behaviours. I would encourage the parents to find 2-3 opportunities to praise their child every day. Praise should be immediate. A sticker chart could also be implemented. This needs to have a short time frame with an appropriate reward which can be negotiated with the child”.*

Question 6

Bone Health

Osteopenia

Osteoporosis

Answer 6

Osteopenia = poor bone density (-1SD or 2x # on DEXA).

Risk factors:

• Immobility, reduced weight bearing.
• Diet - PEG feeds good! Dieting is bad.
• Decreased sun exposure/dark skin.
• Delayed puberty and amenorrhoea.
• Antiepileptics (valproate and phenytoin)/steroids/warfarin.
• Liver failure/fat malabsorption.
• Renal failure.

Investigations:

• Ca/PO/ALP/Vit D (+/- PTH)
• X-ray
• Bone mineral density (DEXA) - usually hip and lumbar spine (beware crush fractures falsely elevating score).
  
  • Use patient as own control.
  • Expect 10%/year increase in puberty.

Treatment:

• Supplements: calcium carbonate
• Vitamin D: cholecalciferol, calcitriol
• Hormone replacement if hypogonadal.
• Exercise programme.
• Bisphosphonates - Low BMD with 2+ long bone fractures (low trauma), and/or vertebral crush fracture irrespective of BMD, or skeletal fragility syndrome.
  
  • SE: initial low calcium, myalgia, bone pain, fevers, AVN of TMJ.
  • Unknown longterm effects - refer to endocrinology.
  • Weekly.
  • Monitor with ALP.

Osteoporosis = poor bone density and quality (-2SD or 4x# on DEXA).

Risk factors:

• Low Ca diet.
• Decreased Ca absorption - ETOH, caffeine, tannins, PO4.
• Increased Ca excretion - high salt diet, excess protein.
• Lack of sunlight/dark skin.
• Immobility.
• Prematurity.
• Malabsorption (CF).
• Chronic lung disease.
• Chronic renal failure.
• Drugs - AED (valproate and phenytoin)/steroid/warfarin
• Caffeine/ETOH

Investigation:

• Ca/PO4/ALP/Vit D/PTH
• Bone density for bone age.
• DEXA

Treatment:

• Ca
• Sun exposure.
• HRT if hypogonadal.
• Calcium - take between meals. If renal failure take with meals also to bind PO4.
• Vitamin D - cholecalciferol needs liver and kidney action to become calcitriol.
• Bisphosphanates - with endocrine consult.

Question 7

Bullying

Answer 7

Explore underlying causes and fix if able.

Increase protective factors:

• Highlight strengths and improve self esteem.
• Gain assertiveness/confidence skills through counselling.
• Ensure positive family environment - support at home.
• Liase with school - ?senior “buddy”
• Trusted adult to report to.

Decrease bullying behaviour:

• School policy.
• Avoid situations that exacerbate (walking to school).
• If really bad ?change schools.

Question 8

Constipation

Answer 8

Rule out organic causes:

• Dairy protein intoleranc can manifest as constipation in first 3 years of life.
• Hirschsprungs disease.
• Coeliac disease, hypothyroidism, hypercalcaemia and spinal cord problems.

Assess problem:

• Stool chart - Bristol stages.
• Toileting regime.
• Anxiety with toileting.
• Soiling.
• Activity.
• Examine for faecal loading.
  
  • Rectal exam and AXR not routine - assess tone if neuro problem.

Educate:

• Common problem (1/4)
  
  • No blame approach.
• Likely to take a long time to improve.
• Continue treatment 3-4 months after regular BM achieved.
• Risk of relapse on stopping treatment.
  
  • Avoid enemas - unpleasant, exacerbates fissures.
• Behavioural:
  
  • Regular toileting - 3x daily for 3-5min after meals.
  • Star charts with timely and appropriate rewards.
• Lifestyle:
  
  • High fibre diet (uncertain evidence).
  • Increased fluids.
  • Excercise.
  • Inappropriate emphasis on fluid/diet places blame.
  • Position - feet up on stool so that knees are higher than hips, lean forward and place elbows on knees.
• Acute disimpaction:
  
  • Movicol: 1 sachet –> 4 sachets BD.
  • Picosulphate drops if cannot tolerate movicol volumes.
• Daily treatment
  
  • Infants <1m: coloxyl drops.
  • Infants 1-12m: Molaxol or lactulose.
  • Children: Molaxol or paraffin oil.

Question 9

Education

Missed School

School Refusal

Answer 9

Missed School

• Extra exam time.
• Psychometric testing.
  
  • WPPSI - <6y
  • WISC - 6-16y
  • WAIS - >16y
• In hospital:
  
  • Central Regional Health School.
  • Online learning and Zoom.
  • School visits if able.
  • Visits from friends/teachers.

School Refusal

1-5% of all school children.

Peaks at 5-7y, 11y and 14y.

Differs from truancy - do not attempt to conceal. Overt anxiety around going to school.

Outcomes:

• Poor academic performance.
• Family difficulties.
• Worsening peer relationships.

Long term consequences:

• Employment difficulty.
• Increased risk of psychiatric illness.

Poor prognostic features:

• Longer periods of refusal >3y.
• Adolescence.
• Depression.
• Low IQ.

Assessment:

• Determine extent of problem - number of days.
• Consideration of predisposing, precipitating and perpetuating factors.
  
  • Stressors:
    
    • Bullying/teasing.
    • Poor self image/esteem.
    • Poor grades.
    • Seperation anxiety.
    • Chronic fatigue.
    • Truancy (different to school refusal – stays home to enjoy pleasurable activities).
    • Unwell parent.
  • Pattern - after holidays, start of week.
  • Parental responses and parent child relationship.
• Discuss with school.
  
  • Behaviour and functioning.
  • Academic performance.
  • Attendance record.
• If somatic complaints.
  
  • Throrough examination and judicious investigations.
  • Reassure likely due to emotional upset.

Management:

• Ensure parental support - agree a time schedule.
  
  • Immediate return if refusal period has been brief.
  • Graded exposure if longer.
• Acknowledge reality of feelings.
• Parent involvement - calm morning routines and escort to school.
• School involvement - try hard to keep at the same school, special supports (modified curriculum, less homework), active tasks (need to be there).
• Child - relaxation training/breathing retraining, social skills training, praise and graded reward system (star chart).
• Adolescents - consider alternative education.
• Monitor for mental health symptoms.
• Referral to multidisciplinary mental health team for longer term school refusal.
  
  • If no improvement after 2 weeks or suspect more severe mental health.
• Regular monitoring of progress and signs of relapse.

Question 10

Encoporesis

Answer 10

Diagnose and treat constipation.

Regular toileting.

Nappies/continence pads - continence nurse/public health nurse.

High health needs application for teacher aide to assist with toileting/changing.

Psychology input in severe cases.

Question 11

Enuresis

Answer 11

Diagnose and treat constipation.

Rule out UTI.

Consider renal tract abnormalities:

• Constant dribbling.
• Recurrent infections.
• Dysuria.
• Poor urinary stream.
• Neurology - leg weakness.

High fluid intake.

Avoid caffeinated drinks/

Regular toileting/timed toileting.

Double voiding (PU count to 20 and then try again).

Spare underwear/change of clothes at school or panty liners.

Pelvic floor exercises.

Nappies/incontinence pads or pants ??funding.

Public health nurse input.

Investigations - MSU, USS further as indicated.

Can use oxybutynin in overactive bladder.

Question 12

Fussy Eating

Answer 12

Normal.

Differentiate between fussy and malnourished, and fussy and thriving.

Fussy and thriving:

Dietary advice:

• Exessive fluid intake reduces solid intake, offer food before fluid.
• ​If weight ok offer water, if FTT offer milk or formula.
• Variety is not important.
• Offer foods that are easy to handle.

Feeding times:

• Eat often not constantly.
• Offer a snack every 2-3h, allow 3 meals and 2 snacks/day.
• Avoid snacks directly after unfinished meal.
• Children work well with schedules, keep meals/snack times the same each day.
• Allow 1h without food/water before a meal to stimulat appetite.
• Do not make meal time too long (15min for toddler).

Feeding behaviour:

• Relax, mealtimes should be pleasant.
• Recognise hunger cues, satiety and food preferences.
• Parents decide what is offered child decides how much to eat.
• Avoid battles, encourage but avoid forcing, threatening, bribing or punitive approaches. Use reinforcement instead.
• Do not withold food as punishment.
• Allow child to feed themselves, small amounts first, seconds later. Expect messiness and be prepared for easy clean-ups.

Question 13

Future directions

Answer 13

Prognosis:

• Check understanding, education, information, contact with other families.
• Families expectations of what child can achieve.
• ?expectation that siblings will become future carers.

End of life plans:

• Slowly introduce idea.
• Need both parents.
• Involve palliative care.
• Optimal timig of discussion (around severe illness).

Transplant:

• Immunisation.
• Bloods.
• Donor selection (family v. non-family).

Genetic Counselling:

• Discussion with geneticist.
• Understand implications for future children/patients children.
• For family if wanting more children.
• For young person if wants family.
• Timing of testing for patient and siblings.
• Options for future children - prenatal testing, antenatal testing (what would they do if positive).

Question 14

GOR in CP

Answer 14

2/3 with CP have GIT motility disorders.

Symptoms include:

• Constipation (delayed transit, poor muscle tone, inadequate feeding, prolonged imobility).
• Swallowing disorders (dysfunction of oral/pharyngeal phase of swallowing from corticobulbar dysfunction).
• Vomiting/regurgtation (delay in gastric emptying, abnormal oesophageal motility, GORD).
• Abdominal pain (oesophagitis, constipation).
• Respiratory symptoms (2º chronic pulmonary aspiration 2º GORD).

Often FTT due to GORD/impairment of swallowing.

Work-up for GORD:

• Endoscopy + biopsy.
  
  • 40-50% will show oesophagitis, 40-50% will be normal and the remainder will have H. pylori, eosinophlic oeophagitis or Barretts oesophagus.
• pH studies.
• Barium studies.
• Nuclear medicine milk/saliva scan.

Management:

• Acid suppression
• Anti-reflux surgery
  
  • Uncomplicated in 70%.
  • Complications included gagging, wretching, dumping, bloating and cyclic vomiting.
  • Re do needed about 3%.
• No evidence to support efficacy of prokinetics or thickened feeds.

Patients with funoplification have similar rate of aspiration as those with gastrojejunal feeding.

Dietician - assess diet and increase calories

SLT - altering food consistency

PT - postitioning during feeding

Post-gastrostomy there is usually improvement in weight, height and skin fold thickness and a decrease in chest infections but no change in hospitalisation rates.

Question 15

Health Promotion

Answer 15

Dental referral - encourage tooth brushing.

Smoking cessation.

Immunisation - advice, education.

Flu vaccination - child and family.

Exercise and diet - Active families/green prescription.

Question 16

Medications

Answer 16

• Blister pack for ease and to aide compliance.
• Review medications - dose changes, meds that can be stopped.
• Yellow cards.

Question 17

Menstruation/Vulnerability

Answer 17

Significant stress to young person and family.

Concerns re: sexuality, pregnancy and vulnerability.

Appropriate management can significantly improve QOL.

Discuss prior to menarche.

Hormonal changes can exacerbate underlying medical and behavioural issues.

Challenges: hygiene, behaviour, mood, pain, heavy or irreguar bleeding, cyclical exacerbation of medical disorders.

Education:

• Puberty and menstruation.
• Abuse and keeping themselves safe - books from library, teachers, CCS, IHC library.
• Managing personal hygiene - if managing toileting hygiene should manage menstruation, specifics how to carry pads, where to change and dispose, managing a calendar and anticipating menstruation.
• Sexuality and relationship issues.

Managing menstruation:

• Many will cope given the right tools.
  
  • Modibody, Thinx.
  • Written information.
• Treat for heavy bleeding, pain or irregular cycle.
• Consider pain esp. in non-verbal - trial of NSAIDs should be considered on days of heavy bleeding (decreases menstrual flow 20-30%).
• Tranexamic acid - decreases flow 50%. Safe to use with NSAIDs, rarely causes thrombosis.
• Hormonal methods:
  
  • Medication interactions esp. antiepileptics and contraindications.
  • Route of delivery.
  • Frequency of administration.
• COC safe to use continuously (skip sugar pills).
• POP can be used continuously (nnot reliable contraceptive in teens).
• Depo-provera can decrease bone density in long term use consider DEXA 2 yearly. Trial oral 2/52 prior to ensure tolerated. Markedly reduced blood loss.
• Mirena inserted under GA. 97-98% decrease in blood loss.

Perform FBC, ferritin and coags if heavy menstrual bleeding.

https://www.rch.org.au/clinicalguide/guideline_index/Menstrual_management_in_adolescents_with_disabilities/#parentresources

Question 18

Nocturnal enuresis

Answer 18

No drinking before bed.

PU before bed.

Rule out organic cause.

Bed alarm >7y.

DDAVP for overnight stays/camps.

Question 19

Obstructive Sleep Apnoea

Answer 19

Causes:

• Obesity, hypotonia, T+A’s, tracheal/subglottic stenosis, cleft palate/pierre-robin (anatomical issues), neuromuscular weakness (SMA, myopathy).

Diagnosis:

• Polysomnography = gold standard (Auckland/Wellington).
• Other centres then for overnight oximetry (Massimo) - then refer to sleep study only if inconclusive.
  
  • Oximetry is useful if the result is very abnormal.
  • If result is normal it doesn’t always rule in/out OSA.
  • Desat events up to 4% can be normal
  • Often on CPAP for life unless OSA simply due to obesity (and lose weight) or subsequently have T+As.

Management:

• ENT for consideration of T+A’s - may need ICU post-op.
• Other surgery - mandibular distraction, maxilomandibular advancement, tracheostomy, nasal surgery.
• Medical - intranasal steroids, monteleukast (mild residual OSA following T+A’s).
• Weight loss.
• CPAP/BiPAP.
  
  • BiPAP - Duchenes and other diseases causing weakness (metabolic).
  • Check overnight study (1-2y if stble, more frequent if progressive).
  • Regular machine downloads so techs can analyse data.
  • Family need to know who to contact if something goes wrong.
  • Electricity provider needs to know power dependent medical condition.
  • Sickness plan.
• Improving CPAP compliance:
  
  • Play therapist.
  • CPAP book “The Magical Mask”.
  • “Ramp function on CPAP - gradually increases pressure.
  • Motivation - explain to older children they will feel better.
  • Trial different mask options.
  • Trial in hospital to help set routine + expectation.

Question 20

Pain/irritability in the disabled

Answer 20

Identify and treat underlying cause.

Consider:

• Infection - ears, throat, urine.
• Dental carries/abscesses.
• GOR
• Constipation.
• Fracture/displaced hips.
• Untreated spasticity/contractures.
• Increased seizures.
• Medication S/E.
• Sleep deprivation –> sleep hygeine.
• Depression –? environmental modification (music/light)
• Parents –> lack of sleep/grief –> respite.
• NAI –> underdiagnosed in this group.

Question 21

Scoliosis

Answer 21

SCOLI

Syndromic - NF-1, Marfan syndrome

Congenital

Orthopaedic - compensatory (leg length)

Low tone/high tone

• Neuromuscular
• Central - cerebral palsy
• Peripheral - SMA
• Myopathic - DMD

Idiopathic

Results in:

• Chest deformity - respiratory reserve, pneumonia, cor pulmonale.
• Pain/sitting discomfort/difficulty with dressing/hygeine.
• Posture - pressure sores, loss of balance, impact on walking function.
• Crush fractures.

Risk to mobility:

• 0% GMFCS I
• 40% GMFCS III
• 90% GMFCS V

Monitoring:

• Yearly x-ray (Cobb angle) +/- PFT

Severity:

• 25-40% moderate, >40% severe.

Treatment based on:

• Location - thoracic more likely to worsen then thoracolumbar or lumber.
• Maturity - brace if still young.
• Potential to progress (neuromuscular, before growth spurt).

Treatment:

• Observation - small curves, low risk of progression.
  
  • Annual x-ray as a child, 5yrly as an adult.
• Idiopathic - <30 watch, consider brace, >40 consider surgery.
• Conservative:
  
  • PT - stretching and strengthening.
  • Boston brace - only 15% respond.
  • Bracing - 25-40º with growth ongoing - prevents further but doesn’t correct, succeeds in 80%.
  • Risk of pressure sores, respiratory compromise.
  • Chair that fits curve to minimise progression.
• Surgery:
  
  • If >40º, progression, affecting QOL, non-brace respondent.
  • Posterior spinal fusion - using bone graft from hip.
    
    • Most don’t need bracing.
    • Out of hospital 1/52, back in school 2-4/52, back to total function 4-6/12.
  • Anterior spinal fusion - deflate lung, remove rib, anterior approach.
    
    • Shorter recovery.
    • Better cosmetic result.
    • Less segments but need brace for several months.
  • Risks: death, respiratory failure, infection, protrusion of metalware.

Question 22

Seizures

Answer 22

• Education - school and carers.
• Avoid known precipitants.
• Crisis plan.
• Safety.
• Avoid alcohol and strobe lights at parties.
• Review old investigations to see classification and prev. trialled antiepileptic.
• Monotherapy but hard if refractory.
  
  • Ensure max dose.
• Monitor for drug interactions.
• Medicalert bracelet.
• Medications decrease recurrence but do not have any affect on long term outcome.

Generalised:

• Absence (3Hz spike and wave) - sodium valproate –> lamotrigine.
  
  • Childhood absence epilepsy -ethosuximide –> sodium valproate –> lamotrigine.
  • Juvenile absence epilepsy - sodium valproate –> lamotrigine.
• Tonic-clonic - sodium valproate –> levetiracetam –> lamotrigine.
• Myoclonic - sodium valproate (lamotrigine may exacerbate).
  
  • Benign myoclonic epilepsy,
  • Severe myoclonic epilepsy of infancy (SMEI/Dravet) - hard to treat.
  • Juvenile myoclonic epilepsy.
• Infantile spasms/West syndrome - hypsarrythmia hydrocort (steroids) otherwise vigabatrin –> sodium valproate.
• Lennox-Gastaut –> steroids

Focal:

• Aware - carbemazepine –> lamotrigine.
• BECTs - no treatment –> carbemazepine.
• Chidhood occipital epilepsy (Panayiotopoulous) - lamotrigine or carbemazepine.
• Landau-Kleffner - pulsed steroids –> ethosuximide –> benzodiazepines.
• Temporal lobe epilepsy –> carbamazepine

Investigations:

• BSL
• Na, Ca, Mg
• Urine metabolic screen
• LP
• EEG - multiple afebrile seizures with convincing story (i.e. not functional).
• MRI - if significant focal EEG changes, neurological abnormalities, worsening epilepsy, epilepsy surgery.

Treatment:

• Only start if reasonable chance of recurrence or for absence or JME.
• Stop if no seizures for 2 years and not a life long seizure disorder.

Principles:

• Treat seizure not EEG.
• Start low go slow.
• Stop slowly.
• Push to clinical effect OR toxicity OR high level.
• Change one at a time.

Surgery:

• Only after AED trial has failed.
• Significant impact on life.
• Localised seizures, lesion identified.
• Not in critical cortex.

Vagal stimulation.

Ketogenic diet.

Question 23

Sialorrhoea

Answer 23

Submandibular - majority, watery.

Sublingual - small amount, thick.

Parotid - while eating.

Parasympathetic innervation.

Normal until ~18m.

Problematic in:

• Cerebral palsy (40%).
• ID.
• Neurological impairment.
• Cleft lip/palate and micrognathia.
• Nasal obstruction.
• Dental disease.
• Medication SE: clonazepam, benzos, sedative.

Problem is ineffective swallowing rather than too much production.

Social Implications:

• Embarrassment.
• Skin irritation.
• Frequent changes of clothing/bibs.
• Increased risk of aspiration.

Assessment:

• Parental rating - severity and frequency.
• SLT
• Video fluoroscopy.
• Barium meal.
• Nuclear medicine.

Management:

• Conservative:
  
  • Positioning and seating.
  • SLT input - increased sucking (straw).
  • Increase childs oral awareness.
    
    • Tell them they are drooling.
    • Swallow more frequently.
  • Waterproof bib/scarves/clothing.
• Medical:
  
  • Anticholinergics - benzatropine/glycopyrolate
  • Botox injections - risk of cranial N palsy/thickened saliva.
• Surgical:
  
  • For severe drooling or failure of conservative therapy.
  • Relocation of submandibular ducts with excision of sublingual glands.
  • Side effects: dry mouth –> dental problems.
  • 80% success.

Question 24

Poor sleep

Answer 24

Sleep hygiene:

• Consistant bedtime.
• Put to bed awake.
• Teach to fall asleep without you.
• no nighttime drinks.
• Avoid parents bed before their own.
• Be boring in the evening.
• Reward good behaviour.
• “goodnight phrase”.

Melatonin

• Evidence not conclusive.
• Funded in neurodevelopmental disorders.

Overnight respite if nothing works.

Question 25

Adrenal crisis

Answer 25

High risk:

• Primary adrenal deficiency:
  
  • CAH/Addisons
• Secondary (pituitary) ACTH deficient conditions:
  
  • Hypopituitarism (congenital/idiopathic often associated with…)
  • Septo-optic dysplasia/midline CNS abnormalities.
  • CNS tumours involving suprasellar/pituitary region.
• Recent treatment with high dose prolonged steroids which have been discontinued.

Investigations:

• Na, K, glucose, Ca, FBC +/- capillary gas.
• BP, HR, temp.
• Fluid balance.

Management:

• Hydrocortisone IV/IM
• Continuous preferred but can give 4 hourly.
• Do not need additional mineralocorticoid treatment when on high dose steroid (except dexamethasone).
• Severely unwell: IVF bolus then maintenance, bolus hydrocortisone 75-100mg/m², then infuse 55-100mg/m²/day until well.
• Moderately unwell:
  
  • Tolerating oral - 50mg/m²/day in 4-5 divided doses.
  • If unable to tolerate oral treat with IV as severe.
• Slightly unwell (no fever, vomiting, looks well): continue usual medications.

All families to which this applies should have a plan for sick days and medications to manage at home if needed.

Question 26

Tracheostomy

Answer 26

24h carer often needed (funding).

Suctioning at home/school.

Skin cares:

• Granulation tissue - pumifucort and silver nitrate sticks.
• Chain/tie around neck to hold in position - ties can get wet/sweaty and break/cause skin irritation.

Overnight humidification and day time attachment for humidification:

• Need to change attachment when get wet with secretions.

Often grow pseudomonas/Staph - treat only if symptomatic (cough, secretions etc).

Emergency tracheostomy kit - take everywhere.

Parental education.

Action plans:

• When to seek help - increased secretions/fever.
• What antibiotics.
• Who is consultant in charge/outreach nurse.

Open letter - if able to do so.

Question 27

Transition

Answer 27

Start early (12y)

Discuss adolescent issues

• Drugs, sex and driving.
• Appointments with young person alone.

Focus on strengths.

Enhance autonomy and self reliance.

Make sure young person has knowledge/understanding of condition.

Ensure medical summary available to GP and other health professionals.

• Diagnosis clearly documented.
• Health passport - communication abilities, main issues to be aware of.

Review clinical status in depth.

• Referrals to be actioned prior to leaving paeds.
• New outstanding aspects of care.
• New information on established medical issues.

Anticipate future medical needs.

• Preventative care.
• Acute care.
• Chronic disease management.
• Who will provide care for these?
• Identify specific issues - screening and treatment guidelines, diagnostic issues - tell GP and patient about these guidelines.

Teach health system literacy.

• Difference between paed and adult systems.
• Need good GP relationship - not about to retire.

Discuss guardianship/power of attorney.

• Arrange prior to transition.
• ?SW can help with this.

Long term care plans.

• Respite.
• Carer supports.

Discuss with GP about being long term primary health professional.

• May need double appointments for complex issues.
• Annual appointments at least.

Clinics.

• Arrange follow-up after first adult appointment.
• Attend first adult clinic with young person.
• Stagger change of services.

Question 28

Vulnerability and relationship boundaries

Answer 28

• Young people with disabilities may have different concepts about intimacy and appropriate and inappropriate touching.
• May be more reliant on others assistance with personal care, sense of private v. public may be distorted.
• May not have had as many opportunities to interact with others.

Circle concept:

• Used to discuss relationships and appropriate behaviour.
• Diagram - fill in themselves and put up in their room.
• Inner most circle: me/self - only share if you chose to.
  
  • Represnts personal space and privacy.
  • Used to discuss the right to say no and the need to have permission to enter.
• Next circle: - people you love in a special way, can cuddle and kiss us but only if we want to.
  
  • Relatives/friends - people you have known for a long time that you like to be around.
• Next circle: Friends and aquaintances, might shake your hand or wave.
  
  • Don’t know them well and they don’t know us well, don’t usually touch each other.
  • Can include people who we have gotten to know because they help us like Dr’s, nurses’s, carer’s and teachers. May need to touch us to help us or make us better but are not people we hug or kiss.
  • People we work with or go to school with or people good friends have introduced.
• Final circle: Strangers, people we don’t know and who do not touch us.

Can also refer to IHC library for further information on vulnerability.

“One of the issues most worrying Katherine’s parents is her lack of understanding of relationship boundaries. In my practice, this is something that is seen commonly in young people with disabilities. Katherine appears to have a distorted perception of intimacy, partially due to her need for assistance with personal care, and also she hasn’t had many chances to interact with other young people due to her immobility and the fact that she attends a special school.

• My aim for Katherine would be for her to understand approproate levels of affection and to apply this knowledge in her everyday life, in particular in helping her develop friendships and also in keeping her safe.*
• One method I would use in discussing the issues is the circle concept. This involves 4 levels of intimacy and relationships - the inner most circle of self , then special people such as friends or family, then acquaintances and finally strangers, with corresponding levels of intimacy from a cuddle for a close relative, to a wave for an acquaintance and no physical contact with a stranger.*
• I would get Katherine to think of people in her life and where they might fit in, using a diagram if necessary. I would also talk about the importance of her right to say no and the need for her to give permission to enter her inner circle. It would be important for Katherine to understand the difference between intimate touching and helpful touching. For exmple - her carer at school would be classified as an acquaintance who might need need to touch her to help her with personal care but isn’t a person she should hug or kiss. It might be important to rediscuss these concepts with Katherine over time to ensure her understanding”.*

Question 29

Intellectual Impairment

Answer 29

Grey matter - dementia (grey hair, demented)

White matter disease - spasticity, blindness, deafness.

Aetiology:

• Syndrome - Fragile X, Down syndrome
• Neurocutanous - NF1, TS, SWS
• Neurodegenerative - leukodystrophy
• Metabolic - Tay-Sachs, Wilson, Gaucher, Niemann-Pick
• Infectious - TORCH
• Genetic - Huntingtons

Investigations:

• Karyotype
• FBC
• LFT
• TFT
• Lead
• CK
• Fragile X
• Guthrie
• Blood metabolic screen
• Urine metabolic screen
• Hearing

Question 30

Irritability

Answer 30

Differential:

• GORD
• Constipation
• Dental
• Fractures
• Pressure sores
• Otitis media

Treatment__:

• Behaviour - sleep hygiene, routine.
• Environment - light, music.
• Alternatives - melatonin, massage, acupuncture.
• Medications - diazepam, paracetamol, baclofen.

Question 31

Marital Issues

Answer 31

• Consider even if no disharmony.
  
  • Child with chronic disease has significant impact on parents mariage.
• Explore community services for mariage counselling (relationship counselling).
• Involve church groups (reationship counselling)

Question 32

Sibling jealousy/rivalry

Answer 32

• Jealousy - attention given to sibling.
• Resentment - compromises they have to make.
• Sadness/helplessness - unable to help.
• Fear - may also become unwell.
• Guilt - about negative feelings.

Acknowledge and talk about it:

• Normalise and validate feelings.
• Lots of positive reinforcement.
• May need play therapist.

Minimise impact:

• Safe rules for all children.
• Family education re: behavioural strategies at home.

Involve in care:

• Make it a positive family activity.
• Praise for getting involved.

Ensure protected alone time:

• Capatilise on individual moments together.
• Use another significant adult.
• Respite.

Sibling support groups.

Family therapy.

Question 33

Sleep hygiene

Answer 33

Normal sleeping time = 16 - age.

Napping should be unnecessary after 3y.

Lack of sleep affects mood, concentration, behaviour.

Assessment:

• Problem with sleep initiation, sleep association problem.
• Rule out organic pathology and precipitants.
  
  • OSA, depression, drugs.
• Sleep diary
  
  • When they sleep vs. wake.
  • School vs. non-school days.
  • Number of naps.
• Activities
  
  • During day and night.
  • Just before sleep.

Sleep hygiene for infants:

• Teach to self-soothe.
  
  • Bring to bed drowsy but not asleep.
  • Should fall asleep in own bed.
• Regular bedtime and routine.
• Appropriate transitional objects.
• Controlled crying.
• Sleep clinics - mother baby units.

Sleep hygiene for children:

• Regular sleep-wake cycle.
  
  • Regular bedtime and routine.
  • Maintain bith school and non-school nights.
  • If get up firmly and simply return to bed.
• Sensible activities.
  
  • Encourage outdoor activities.
  • 1hr prior to bed = quiet time.
  • Avoid using bedroom as punishment/time-out.
  • Avoid TV in room.
• Appropriate environment.
  
  • Quiet and dark room.
  • Comfortable temperature.
  • Security object/monster spray.
  • Leaave room while still awake.
• Appropriate food.
  
  • Avoid caffeine several hours before sleep.
  • Avoid going to sleep hungry.
• Sticker charts.
  
  • 1 sticker for listneing to parents bedtime story.
  • 1 sticker for falling asleep withoutparents.

Sleep hygiene for adolescents:

• As above +
• Think of depression and illicit drug use.
• Biological shift of internal clock - difficulty with sleep initiation.
• Avoid daytime naps.
• Use bed only for sleeping.
• Avoid alcohol/smoking before sleep.

Managing sleep in the disabled child:

• 40% of ID have problems
• Sleep deprivation may worsen seizure control as well as behaviour/mood.
• Behavioural therapies as for normal children.
• May need additional medications.
  
  • Melatonin - helps circadian rhythm
    
    • Decreases core temp.
    • Enhances sleep in those with primary sleep disorders.
    • Increases sleep by 10%.
  • Anticonvulsants - should be modified to aide sleep.
    
    • Phenobabrbitone/clonazepam sedative.
    • Lamotrigine delayed sleep onset.