Clinicals - Longs Flashcards
Cerebral palsy - classification
Spastic (hemiplegia = unilateral, diplegia = LL>UL, quadriplegia)
Dyskinetic = extrapyramidal/involuntary, subtypes = choreoathetoid, dystonic
Ataxic
Hypotonic
Mixed
Cerebral palsy - investigations
Bloods rarely helpful, but consider:
- TFT
- Lactate, pyruvate,
- organic and amino acids
- karyotypes
Imaging is helpful
- MRIB, abnormal in 90% (99% if prem -> PVL)
Metabolic 4%, genetic 2%
EEG only if seizures
LP in dyskinetic CP to rule out GLUT1 deficiency, especially if seizures/refractory seizures
Cerebral palsy - mimics
4xMs
Metabolic
Muscular dystrophy
Mitochondrial
Malformation
CP - GMFCS
1 = ambulatory in all settings 2 = walks without aides but limited in community settings 3 = walks with aides 4 = wheelchair or adult assist 5 = dependent for mobility
CP - manual ability classification system
1 = handles objections with ease 2 = handles most objects with some limitation 3 = handles with difficulty and needs help 4 = only handles some easily manipulated things in adapted situations 5 = does not handle objects
CP - communication function classification system
1 = effective sender/receiver communication with familiar and unfamiliar partners 2 = effective but slower 3 = effective only with familiar partners 4 = inconsistent 5 = seldom effective communication
Management Issues - steroid dependence
Features: cataracts, Cushingoid facies, ulcer (GI), striae, skin thinning, HTN, infection, avascular necrosis, growth, osteoporosis, obesity, raised ICP (idiopathic intracranial HTN), diabetes, myopathy, mood, acne, pancreatitis, psychosis/behaviour
a. Minimise dose
i. Work with treating team: steroid sparing agents, biologics
b. Monitor for side effects
i. Growth and weight
ii. BP
iii. Mood and affect
iv. Ophthalmology review e.g. cataracts, ICP
v. Investigations e.g. BSLs and HbA1c, DEXA
vi. Functional muscle assessment
c. Treat complications
i. Vitamin D and calcium, bisphosphonates (bones)
ii. SSRI (mood)
iii. PPI (PUD)
d. Health prevention
i. Vaccines (extra, caution with live)
ii. Weight management
iii. Stress dose plan
iv. Pregnancy considerations (teratogenic)
v. Drug interactions
e. Advice from Margot
i. Medicalert bracelet
ii. General health e.g. smoking (given CVS risk factors)
iii. Don’t pad – answer questions, move on to the next one
iv. Focus on the specific patient
- assess the impact ON. THIS. CHILD. Do the steroids cause mood disturbances that are affecting relationships? Work? School? Are they causing weight gain and making the patient suicidal? Impact is key in long cases.
Management Issues - general approach
- Gather information
a. Is the diagnosis correct? Do you need correspondence from other professionals, are the tests that haven’t been done that should be done…
b. Were there elements you didn’t have time to explore today? Say so and explain what you want to do with more time / future visits and why.
c. Does the history need corroborating? E.g. behavioural issues, talk to the school.
d. Consider using follow up and reviews to allow time to gather more information, e.g. constipation/sleep/headaches can all be tracked with a diary at home and shown to you on follow up.
e. What’s been tried previously? What’s worked and hasn’t? Complications of previously tried things? - Assess IMPACT on patient and family
a. What is it about the issue that is most important to them?
b. How significantly is it impacting their lives? - Non-pharmacological options
a. Education - Pharmacological
- Monitor for side-effects and complications
a. Side effects of the issues
b. Side effects of any treatment
c. General health preventative things, like steroids and bones, or poor growth and nutritional deficiencies, etc
d. Tests, bloods, imaging, levels of drugs… - Referrals
a. Specialists
b. AH
Management issues - adherence (not compliance)
a. Review current medication burden – can we rationalise these to make it easier?
b. Explore barriers to adherence + solutions
i. Forgetting -> alarms e.g. on phone, timetable, webster packs
ii. Formulation -> ?bad taste, pills vs liquids
iii. Embarrassment -> dosing schedule not around friends/peers
iv. Complex dosing -> simplify
v. Perceived non benefit -> education
vi. Side effects -> explore alternatives
vii. Social/economical: poor health literacy, unstable living, lack of health insurance, medication costs
viii. Patient related: visual/hearing/cognitive impairment, knowledge about disease, perceived risk, perceived benefit of tx, motivation & confidence
c. Rebelling against diagnosis / seeking independence
i. Can flip this around to encourage independence in taking control of one’s medications
1. Stepwise e.g. parental control at home, patient control at work/school
d. Review unpleasant effects of noncompliance e.g. return of symptoms
e. Make plan to improve, agreed upon by patient and family
f. Regular reviews
- screen for issues related to poor adherence
g. Triangulate compliance (e.g. levels)
Manage the 3 process of medication adherence
Initiation: educate (purpose, how, SE)
Implementation: calendars, phone, pill box
Discontinuation: timely med refills, edu
h. Advice from Margot
i. Explaining the “badness” of noncompliance to teenagers is often unhelpful. Don’t berate them.
ii. Listen to patient. I’d try and find out what’s behind it -> understand why before trying to fix it.
iii. Maintain relationship with patient, reciprocal trust.
iv. Negotiate -> stop taking this drug but keep taking that one.
v. Reward systems
vi. Start from perspective of forgetting – how often do you forget? Rather than do you take them all?
vii. Explore emotional maturity, relationship with family, opinions of parents
viii. Psychologist, if you think they will engage
Management issues - pain
a. WHO analgesic ladder
- First step. Mild pain: non-opioid analgesics such as nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen with or without adjuvants
- Second step. Moderate pain: weak opioids (hydrocodone, codeine, tramadol) with or without non-opioid analgesics, and with or without adjuvants
- Third step. Severe and persistent pain: potent opioids (morphine, methadone, fentanyl, oxycodone, buprenorphine, tapentadol, hydromorphone, oxymorphone) with or without non-opioid analgesics, and with or without adjuvants
- Involve complex pain team if considering third step
c. Review any current drugs (in the example - amitriptyline and gabapentin), review how often and doses ?rationalise ?reduce
d. Model for chronic pain from James
i. Pain
1. Address underlying cause
2. Non pharmacological
a. Reduced distress with pain, non necessarily reduce pain
b. Visualisation, distraction, CBT
c. Physiotherapy
3. Pharmacological
a. Analgesic ladder vs neuropathic pain
4. Sleep
a. Hygiene optimisation
b. Explore impacting factors (OSA, environment)
c. Pharmacological e.g. melatonin
5. Mood
a. Psychology input
b. SSRI
e. Margot
iii. Hypnosis, acupuncture
iv. Chronic pain clinic (given amitriptyline and gabapentin)
v. Physiotherapy
Steroid side effects
Cushingnoid appearance Short stature Obesity Hypertension Osteopaenia/osteoperosis Immunosuppression Striae/bruising/skin thinning Proximal myopathy Psychosis/behavioural problems Cataracts
Management issues - obesity
b. Aim to establish healthy habits
i. Whole family on board
ii. Diet
1. Food selection
2. Serving sizes
iii. Exercise
1. Target 60 minutes a day, low impact activities (e.g. can consider swimming)
2. Limit screen time
c. Achievable goals e.g. not weight loss but slowed weight gain
d. Reduce barriers
i. E.g. medication side effects
e. Enlist dietitian
f. Weight management clinic referral
g. Surgical considerations
i. Banding or bypass
h. Margot
ii. Acknowledge that we may not achieve anything with Jessica’s weight
iii. Recognise this will be hard
iv. See what Jessica and family think about Jessica’s weight – if entire family overweight, different situation
v. Non-food treats, or healthier treats if food is being used as a treat
vi. Review insight of family e.g. soft drink and fruit juice are essentially the same nutritionally, museli bars aren’t healthy
vii. Boredom -> look for other things
viii. Hunger -> healthier options
Eugene
- BMI as primary measure (i.e. you can be gaining weight more slowly and reducing BMI given kids are still growing)
- Assessment of: Risk factors: parental obesity, growth, pmhx, Dietary input: quality & quantity, Behaviours: activity VS sedentary time, attitude, motivation , Medications: steroids/valproate/olanzapine, Complications: body image/depression, bullying, endo (PCOS/DM), CVS (HTN), OSA, GORD, fatty liver, SUFE
- Investigations: Lipids, glucose, insulin, LFTs, OGTT, Genetic causes, Endocrine causes
- Nonpharmacologic: Multicomponent intervention 🡪 targeted at LIFESTYLE MODIFICATION, Dietary modifications: food pyramid, traffic light system , Physical activity: 60mins/day all or most days of the week, Behavioural strategies, Family involvement: maintenance of healthy behaviours, Goals targeting family members
- Pharmacologic: orlistat, surgical (bypass, banding)
Management issues - mood
i. Identify triggers/exacerbating factors
ii. Assess impact(***) on function, e.g. social, educational, QOL
iii. Explore symptoms/expression
1. Insomnia, hypersomnia, appetite, somatic symptoms, aggression
iv. Stratify risk, risk assessment (if didn’t have time)
1. Create safety plan
v. Non-pharmacological
1. Psychology/CBT -> mental health care plan
2. School supports -> friends/counsellor
vi. Pharmacological
1. SSRI
a. Margot
i. School
1. Academic capacity
2. Plans before illness impacted her
ii. Psychologist
iii. CAHMS, Origin, Headspace
iv. Chips, peer groups
v. What does she like
vi. Review meds - ?side effects, weight gain
Eugene:
Assessment of cause 🡪 multifactorial
- Genetic/endocrine/biochemical: ADHD, disruptive disorders, neurodevelopmental delay, ASD, learning disorder, physical illness, disability, cognitive
- Psychological/social/socioecomical: martial disharmony (divorce, separation), domestic violence, physical & sexual abuse, school difficulties, social isolation
Management
- GP mental health plan
- CAMHS
- Headspace
- Psychology/psychiatry referral
- Medications: Counselling about how they work, Consider side effects
Assessment of complications: Psychiatric morbidity, Suicidality, Medical comorbidities, Social/educational/QOL
Management issues - menorrhagia/dysmenorrhoea
a. Assess true menorrhagia
i. ?Correct use of pad/tampon
ii. ?Clots, leaking
b. Consider exacerbating conditions
i. TFTs (hypothyroidism linked with bleeding diathesis), coags, vWD, platelet function
ii. USS ?uterine anomalies
c. Treat side effects
i. Iron replacement
d. Pharmacological
i. Antibleeding – NSAIDs, tranexamic acid
ii. Contraception
1. OCP
2. Implanon/IM depoprevera
3. IUD – Mirena
ASSESS IMPACT
Management issues - school absenteeism
a. Explore barriers
i. Workload
ii. Mood
iii. Adopting sick role
b. Clear health plan
i. Written down, emergency instructions, clear expectations of work/abilities
c. Graded exposure approach
i. Agreed upon attendance goals
ii. Positive reinforcement
iii. Agreed upon aim in terms of when to be back full time
d. Modifications
i. Work/access/uniform/exams
e. Student support groups
f. Promote access to friendship groups
g. Margot:
i. Negotiate – probably fits with graded exposure?
ii. What type of school?
iii. Get permission from family to speak with school and get their opinion
iv. ?Secondary gain staying home
v. ?Bad sleep/wake cycle making it harder to get up
vi. ?Separation anxiety
vii. Working with school is best
viii. If she really likes music and music is on Thursdays -> try and get them to go on Thursdays
ix. Work out the goals and hopes
Eugene
- Paediatric role is to:
- Optimise health so that they can attend school
- Coordinate inpatient and outpatient visits to minimise disruption to school - Provide support – 5 steps:
- Share information: decide how much for adequate care vs confidentiality
- Chronic illness plan: staff roles in emergency, contacts
- Facilitate good communication btw school and family. Best way of assessing how child is going. Home-school communication book
- Minimising sequelae of missed school: Sending home school work, email contact, visiting teaching service, in-hospital education. Ronald McDonald learning program: educational catchup program
- Catering for disability. Physical mods to allow access. Special consideration
- Assessment of special services. Education support officer. Psychologist. Social work. Speech. Other allied health. Student welfare
- Access to special programs. Program for students with disability (supplementary funding program). Provides additional resources if meet eligibility for 7 categories (Physical disability, Visual impairment, Severe behavioural disorder, Hearing impairment, Intellectual disability, ASD, Severe language disorder with critical education)
Management issues - aspiration
1° ASPRIATION
- Give your impression of the main contributors in your case
- Review previous investigations
- Assessment of swallow: Observe a feed. Speech evaluation. Video fluoroscopy. Barium study
- If indicative of aspiration mx would involve
- Modification of feeds, as guided by speech path (thickener reduces symptoms and hospitalisations). Continuing to provide some oral feeds is preferable to preserve oromotor skills and avoid aversion.
- Mx of salivation: oral appliances, meds, surgery
- Neck posturing
- Pacing and timing of feeds
- Suction
- NBM 🡪 NG
- Surgery 🡪 PEG/PEJ
Consider parents wishes. May be appropriate to continue some oral intake for quality of life weighed against risk of aspiration.
2 ° ASPRIATION
- Give your impression of the main contributors
- Most likely to be GORD - Review previous investigations
- Assessment of reflux
- Barium meal
- Oesophageal pH monitoring - If indicative of significant reflux mx would involve
- Conservative: Thickened feeds. Upright positioning post feeds
- Medications: PPI, Promotility drugs (CI: prolonged QT)
- Surgical: Fundoplication
Factors affecting respiratory decline in CP
- Primary aspiration
- Secondary aspiration
- Strength of cough/airway clearance
- Reduced with decreased mobility
- Mx: PT, suction, nebs, immunisations - Scoliosis
- Central and obstructive sleep apnoea
- Central hypotonia + dystonia 🡪 narrow airway
- Ix: PSG, ENT
- Mx: NPA, CPAP
Management issues - dystonia
Definition:
Movement disorder characterised by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures or both.
Typically patterned, twisting and may be tremulous.
Often initiated or worsened by voluntary action and associated with overflow muscle activation.
Seen in:
Cerebral palsy
Neurodegenerative disorders e.g. Wilson’s disease
Mx: Aim is for as normal life as possible, multidisciplinary approach
Conservative: positioning, equipment, PT
Medical: levodopa, baclofen, benzhexol, gabapentin, botox injections
Surgical: deep brain stimulation
Status dystonicus:
Increasingly frequent or continuous severe generalised dystonic contractions that may be refractory to standard medical treatment 🡪 can be fatal
Cx: respiratory failure, pain, rhabdomyolysis and AKI
Mx: benzodiazepines, clonidine
Management issues - talking about prognosis
Important for THREE important reasons:
- Helps makes appropriate decisions about chronic disease management and screening interventions
- Avoids missing opportunities to fulfil personal goals and ambitions due to unneeded interventions
- Allows appropriate sharing of decision making about care with family
When done well results in:
- Better QOL
- ↓non-beneficial medical care
- Enhanced goal-consistent care
- Positive family outcomes
- ↓ costs
APPROACH:
- Establish what patient and family know about illness and prognosis
- Determine how much they want to know
- Determine how ready they are to engage in discussions
- Deliver clear information
- Respond appropriately to emotion
- Refer appropriately to palliative vs bereavement services
Management issues - disclosing information
- Quite and private space
- Involvement of senior person who is familiar with family
- Prepare family about seriousness of disclosure
- Have both parents ideally. Parents should know BEFORE others
- Provide appropriate supports form somebody NOT involved in giving information
- Check parental understanding – should never be taken for granted. Start off with summary. Give early opportunities to clarify, ask questions, raise concerns
- Present simply
- If possible, offer follow up
Management issues - sialorrhoea
Aetiology: ineffective swallow
- Clarify history and exacerbators:
- CP, intellectual disability, neurological impairment
- Cleft lip, micrognathia
- Dental disease 🡪 poor healthy assoc. with drooling
- Nasal obstruction
- Medication SE (benzos)
- Parent’s perception of severity and frequency - Complications:
- Social
- Skin irritation
- Eating and drinking
- Communication issues 🡪 articulation
- Dehydration
- Aspiration - Assessment
Speech assessment - Management
- Address aggravating factors
- Conservative:
- PT: positing, seating
- Behaviour: self recognition, self wiping, swallowing, eliminate mouthing behaviours e.g. sucking hands
- Oral hygiene: brushing teeth, floss, dental visits, avoid sugary snacks
- Appliances: help oral awareness, motor control and tongue position in mouth 🡪 dentist and speech
- Conservative:
- Medical:
- Anticholinergics: benzhexol, benztropine, glycopyrulate
- Botox
- Medical:
- Surgical:
- Salivary gland ligation/diversion
- Surgical:
Management issues - oral aversion
Impacts:
Growth and nutrition
Dentition
Approach
- Treat the underlying cause
- Do not give food if actively giving may cause long-term aversion - Multidisciplinary approach
- Speech: oromotor dysfunction
- OT: sensory issues
- Paediatrician
- Dietician - Educate parents
- Want to avoid major aversion issues
- Importance of growth and nutrition in chronic illness
- Dentition
- Family meal times, child-parent bonding - Educate child
- Observe a feed
- Introduce range of textures, colours 🡪 helps palate development
- Make meal times fun 🡪 PLAY THERAPY - Induction of hunger
- Monitor growth, nutrition, development and dental health
- Consider PEG
Management issues - sleep
- Optimise identified risk factors
- ?Oximetry or PSG
- ?Behavioural contributors inadequately managed
- ?Pain ?Constipation
- In CP think about GORD, hip dislocation, pressure injuries, seizures, dystonia
Think about reversible/pathological causes: OSA, Parasomia, Life stressors, Anxiety/depression
- Assuming the above have been appropriately addressed 🡪 GOOD SLEEP HYGIENE
- Sleep hygiene:
- Bed is for sleep only
- Routine
- Healthy diet
- Limit/reduce caffeine
- Sunlight
- Exercise (PT input)
- Sleep position (bedding 🡪 OT)
- Other: sleep diary, reward good habits
- Sleep hygiene:
- Follow up
- ?Referral to sleep services
- Raising children network - Failing the above consider melatonin
- Assess impact on family and provide support if needed
Management issues - transition
- Introduce team early
- Assessment of where child is with:
- Understanding of disease (progression, follow up etc)
- Own self care - Education
- Medical: disease, genetics, meds
- Adolescent issues: sex, drugs, employment, pregnancy
- Psychosocial + educational/vocational. Post school activities, role in society, driving, voting
- Transition: expectations about process - Encourage/promote communication, decision making, assertiveness, selfcare, self determination and self advocacy
- E.g. medications, refills, make appointments
- Give option to see specialists by themselves
- Give opportunities to express thoughts and make informed decisions - Involve interested and capable adult services
- Medical and allied health
- 4-12 month joint care (flexible) prior to transition
- Ensure 2 way communication and allow for feedback - Facilitate a coordinated process with a coordinator/case manager
- Primary health care involvement
In general, transition will be to/via GP. Some centres, like RCH, have a specific transition team that you can refer to.
Management issues - needle phobia
- Address the fears actively
- Assess reason for phobia
- Distinguish between physical VS mental distress 🡪 helps target approach - Assessment of complications
- Psychosocial impact: fear, anxiety, PTSD - Involve a procedural support team
- Preparing child AND family for medical procedures
- Coaching kids to utilise coping skills to ↓ the perception of procedural pain
- Multidisciplinary approach: medical (paed, anos), nurse, play therapy - Procedural Pain Management Plan
BEFORE
Planning
- Assess requirement (other modality, long term access)
- Child’s likes and dislikes
- Assess previous procedural experiences
- Establish procedural short term plan
- Communicate the procedural support plan
- Refer children at high risk of procedural pain or distress e.g. immunisation clinic
- Consult with appropriate procedural support team
Preparation
- Prepare children, family, environment support team
- Demonstrate
Pharmalogical
- Topical
- Nitrous
DURING Physical - Positioning Psychological - Attention to breathing - Breaking down steps - Giving control - Coping behaviours - Distraction 🡪 PLAY THERAPY
AFTER
Promote resilience and recovery
E.g. achievement beads
Management issues - distress/agitation in ASD/DD
Vulnerable group
- Behaviour and communication are significant barriers to accessing care
- Higher incidence of behavioural problems
- More likely to be admitted to hospital for medical illness and complaints
- Assessment part 1: gathering info to best mx communication and behaviour
- Level of intellectual disability and physical disability
- Functional capacity, skills and abilities
- Communication: What is their comprehension? What is their ability and methods? Aides?
- Routines: stress with changes to routine
- Sensory sensitives: sound, texture, touch, smell
- Intensive interests
- Existing disruptive behaviours: current mx plans? - Assessment part 2: exacerbating factors
- Organic cause: seizure, OT, GORD, constipation, pressure sores, teeth
- Pain: hip dislocation, fracture
- Changes in medications, SE
- New psychiatric symptoms - Address underlying factors
- Behaviour: sleep hygiene, routine
- Environment: light, music
- Alternatives: aromatherapy, massage, acupuncture
- Meds: melatonin, diazepam, paracetamol, neurofen, anti-depressants - Involve a multidisciplinary team
- Speech: assess and mx strategies for communication
- OT: can help promote supportive physical and sensory environment for those with sensory sensitivities
Management issues - bad behaviour
- Clarify history
- Events surrounding behaviour 🡪 multi-informant data collection (school, home, child alone)
- Antecedent: events preceeding/triggers
- Behaviour: exact behaviour
- Consequences: current strategies use to manage - Examination
- Observe child. Parent child relationship. Dysmorphic features. Developmental exam. - Assessment of contributing factors
- Child: sleep, low Fe/nutrition, dev delays, comorbid ADHD, depression, impaired communication
- Family: disadvantaged, family stress/trauma, parenting practices, insecure child-parent relationship, parental mental health - Impression
- Medical VS developmental VS behavioural - General approach if impression is behavioural
- Get parents to pick TWO specific behaviours
- Establish goals, set realistic expectations and assess capacity to implement behavioural management plan
- Remember the 4 Cs & that it will get worse before it gets better: Calm attitude, Clear boundaries, Consistency, Consequences
- 4 pronged approach: 1) Environmental modifications: safety; 2) Time out for prioritised behaviour: 1min/age, >6yo get privileges; 3) Ignore other behaviours: will address this later; 4) Praise good behaviours: star chart etc
Management issues - fussy eating
- Differentiate if fussy & malnourished VS fussy & thriving
- Growth chart, genetic potential, developmental milestones
IF FUSSY & THRIVING
- Reassure: very common in toddlers, child’s eating is unpredictable
- Educate:
- Feeding behaviour: Make meals fun, Give praise when trying new foods, Support independence, Encourage self feeding from young, Ignore fussy eating, Avoid pressure/trick/bribes
- Dietary advice: Parents decide which foods, kids will decide how much, Offer healthy foods repeatedly and regularly, Limit juice/sugar, Easy to handle food
- Feeding time: Three meals + 2-3 snacks, Schedules, 1 hour without food prior to main meals, Limit meal time to 15 minutes
Management issues - bone health
- Identify risk factors: Immobility, Poor nutrition, Steroids, Dark skin, Delayed puberty, Medications, Renal failure, Chronic disease
- Investigate
Bloods: Ca, PO4, ALP, vit D, PTH
Imaging: XRAY, BMD (z scores) - As a paediatrician, modify risk factors:
Optimise chronic disease
Optimise growth 🡪 nutritional intake, puberty/hormonal therapy
Sunlight exposure
Weight bearing exercises/optimise exercise
Reduce risk of fractures
Environmental mods, OT, safe falling
Bisphosphonates
Ensure adequate vitamin D and calcium
Review meds: Esp. steroids!!!
Management issues - short stature
- Is this an issue?
- Falling centiles/poor height velocity
- Pubertal development not in order
- Psychological impact: bullying, self confidence, body image
- Genetics? 🡪 T21, Turners, Noonan, Russel silver, Prader Willi
2. Investigate Mid parental heights, Bone age Delayed 🡪 constitutional vs endocrine (hypothyroidism, GH def, hypopit) Advanced 🡪 precocious puberty Pubertal staging Genetics Others: coeliac, UEC, TFTs
- Assessment of aetiology (IS NICE):
I diopathic (constitutional),
S keletal (dysplasia, OI), spinal defects (scoliosis, kyphoscoliosis)
N utritional (malabsorption), nurturing deprevation
I atrogenic (steroids, radiation)
C hronic disease, chromosomal (genetic)
E ndocrine (hypopituitary, GH def, hypothyroid, cushings, pseudohypopara) - Optimise underlying aetiology
Encourage exercise 🡪 muscle increases bone length
Consider GH replacement 🡪 specific indications (height < 1st centile for bone age and sex + growth velocity < 25th centile for bone age and sex)
Reduced steroids
Management issues - pubertal delay
- Is there pubertal delay
- Boys: no testicular enlargement by age 14
- Girls: no breast buds by age 12 - Differentiate between 1° (gonadal) VS 2° (extra-gonadal) cause of pubertal delay
- Is puberty totally absent OR stalled
- Are there any underlying medical causes? (Poor nutrition, chronic illness, hypothyroid)
- Are there any congenital abnormalities
- Family hx of delayed puberty, mid parental height
- Normal sense of smell? - Examination
Height, weight, arm span 🡪 ?eunuchoid
Dysmorphology
Tanner staging - Investigation
Bone age
Baseline bloods: FBE, ESR, UEC, LFT; Endo bloods: LH, FSH, testosterone; Other bloods: prolactin, IGF-1, TFTs, ferritin (Fe overload) - Interpret
High LSH, FSH 🡪 karyotype (1°)
Low LH, FSH 🡪 brain MRI, genetics - Management
If 2°then initially serial observation
?psychological support if significant impacts
?short-term testosterone or oestrogen if distressing
Management issues - scoliosis
- Consider cause (SCONI)
Syndromic : NFI, Marfans, fibrous dysplasia, OI, MPS
Congential: hemivertebrae, bony abnormality
Orthopaedic: limb length discrepancy (compensatory)
Neuromuscular: Central: CP, spina bifida, cerebellar; Peripheral: SMA; Myopathic: DMD
Idiopathic: adolescent idiopathic scoliosis - Assess severity
Spine XRAY -> Cob angle - Assess for complications
Chest deformity: resp reserve, pneumonia, restrictive lung disease
Pain/sitting discomfort/ADLs/hygiene
Posture: pressure sores, loss of balance, walking
Crush fractures
Cosmetic, psychological impact
4. Management Observation: small curves Conservative: - PT: stretching, strength exercises - Bracing: Prevents progression, does NOT correct, Education about adherence Surgical: Cob angle ~50◦
Management issues - sibling rivalry
- Assessment of child’s emotions
- Encourage conversation
- Normalise and validate feelings
- Encourage empathy for siblings with illness - Involve sibling in care of patient
- Positivity in involvement, praise - Encourage normality in siblings life
- Respite for parents - Encourage time spent together as a family unit
- Find special time alone for child
- Capitalise on moments together
- Set dates - Support parents so that they can find time for the siblings
- Formal supports (respite)
- Engagement with a social worker
- Informal supports - Positive reinforcement
- Connect parent/child to resources
- Very Special Kids sibling camps
Management issues - poor growth
- Establish if there is poor growth
- Growth trends over time
- Mid parental heights
- Condition specific growth carts - Consider differential -> use the growth chart interpretation!!
- Weight < length < HC = NUTRITIONAL/MEDICAL; Inadequate intake/absorption, Excessive energy expenditure, Psychosocial factors
- HC < weight < length = NEUROLOGICAL
- Length < weight < HC = ENDOCRINE
- Weight=length=GENETIC/CONSTITUTIONAL - Investigations
- Healthy 🡪 none
- Specific findings 🡪 specific investigations
- Non specific findings 🡪 FBE, ESR, CMP, LFT, TFT, MCS stool/urine, consider karyotype - If nutritional then:
- Multidisciplinary approach
- Inpatient: observation, NG, supplementation, dietetics involvement
- Outpatient: dietetics follow up, feeding times, feeding behaviour
Management issues - parental/marital problems
Implications: A family unit that is not cohesive has impact on care of child and communication.
ASSESSMENT
- Assess the nature the problem once rapport has been built
- Safe and confidential space to disclose difficulties?
- Is the relationship salvageable? - Screen for domestic violence
- Child protection implications
- Emotional, Physical, Financial - Destigmatize relationship breakdown
INTERVENTIONS
- Formal supports
- Couples counselling
- Respite to allow parents to spend time together
- Relationships Australia
- School – after hours care
- Cleaning services - Informal supports
- Engage extended family - If there has been relationship breakdown
- Must clarify the current custody/ guardianship arrangements
PARENTAL MENTAL HEALTH
- Impacts on: Child’s health, Compliance and attendance, Child neglect/abuse, Impacts of other children, Increase risk of children having social, behavioural and mental health issues themselves
What things can we as a paed suggest?
- Encourage own regular GP 🡪 MENTAL HEALTH PLAN
- Consultant-liaison psychiatry
- Link to support groups
- Encourage the use of respite
- Ensure full access for financial supports
- Family therapy
Management issues - participation
- Evaluation of participation with child, family and multidisciplinary team
- Paediatrician, OT, PT, therapist, carer
- Emphasis in developing life skills, employment and independence - Recognition that well formed decisions need to taken into account:
- Overall health status
- Individual activity preferences
- Availability of appropriate programs and equipment - How can we help increase participation for the child?
- > Maximise function and independence
- Behavioural management
- Facilitate improvement in impairments
- Educational services
- Family counselling - What about increasing participation in an isolated family?
- Aim to bring them back into the mainstream
- Social parent support groups
- Disability and advocacy groups
- Parent to parent programs
- Respite
- Home care
Management issues - advanced care planning
When can you withhold resus?
- Parents wishes and medical team agree
- Brain dead child, Permanent vegetative state
- Child suffering
- Unbearable situation
- Progressive, irreversible illness
- Preparation
- Collaborative approach involving child (if possible), family and all health professionals
- Empathetic approach over several appointments
- Develop rapport with family - Establish parent’s views
- Understanding and expectations of illness progression
- Perception of patient’s quality of life
- Religious, cultural and personal beliefs - Discuss treatment options
- Make recommendations and guide parents
- Careful documentation
- Ongoing assessment - Discuss what level of resus will be given
- Oxygen/fluids/antibiotics vs intubation & CPR
- If a plan is agreed to and documented, assure that parents can still change their mind - Prepare and support parents & extended family
- Provide comfort and listen to parents
- Acknowledge what is happening and emotions
- Normalise emotions
Management issues - constipation
- Consider cause
- Functional: pain -> apprehension, retention, hard stool and withholding/behavioural
- Medical: CM allergy, coeliac dx, CF, hypercalcaemia, hypothyroid, medications
- Surgical: Hirschsprungs, meconium ileus, malformation, spine - Assessment
- Frequency, consistency, size, age of onset, timing of bowel actions
- Functional symptoms: WH behavioural, painful/frightening precipitant, straining, toilet refusal, cross legs
- Previous treatment: adherence
- Complications: faecal or urinary incontinence, bullying, trained familial relationships, poor growth - Investigations
- Bloods: TFTs, ceoliac screen, CMP, stool sample
- Imaging: AXR, barium enema
- Biopsy - Management
- Address cause if medical/surgical
- Three pronged approach: - Behaviour: Foot stools, Toilet sits 5 mins x 3/day, Stool chart diary
- Praising child/positive reinforcement
- Diet: Increase dietary fibre, Increase fluid intake, Avoid excessive cows milk
- Medications:
- Diet: Increase dietary fibre, Increase fluid intake, Avoid excessive cows milk
- 1st line: stool softners (paraffin), iso-osmotic (Movicol/osmolax), coloxyl, lactulose
- Rectal meds: try to avoid
- Aim: one soft stool/day, may take 6weeks to 6 months, must keep going with aperients
- If severe may need inpatient admission and bowel washout
- Consider referral to continence clinic
- Rectal meds: try to avoid
- Education:
- Reduce anxiety
- No blame game
- Written action plan for escalation of aperients
Management issues - chronic fatigue syndrome
- Gather information
- correspondence from other professionals involved, including from the school
- are there organic contributors, e.g. poor sleep or OSA that could be addressed, hypothyroidism, etc
- explore mental health ?depression
- there is a full panel of bloods the chronic fatigue clinic do! - Assess impact on patient on family
- school absenteeism
- social
- work - Validate symptoms. Explain diagnosis and explore understanding. Involve patient in the discussion***.
- Create a plan
- rehab approach
- limited activity with gradual increase
- treat/optimise any contributors e.g. sleep hygiene, diet, returning to physical activity
- start with school/work, commit to a certain amount, modifications and accommodations as able
- increase over time
- “pacing” -> achievable goals, don’t expect premorbid function, modified schedule at school - Referrals/other teams
- mental health/psychologist/counsellor
- OT for neurocognitive assessment to get a better sense of any specific deficits, important information for school etc
- referral to chronic fatigue clinic
Cerebral palsy checklist
Seizures Vision Hearing Dentition Sialorrhea/oromotor function
Aspiration risk/recurrent check infection Reflux Nutrition/feed assistance Constipation Continence
Scoliosis
Hip subluxation
Contractures
Pressure sores
Trisomy 21 checklist
Cardiac disease (AVSD, VSD, ASD)
Hearing loss
Ophthalmological disorders
Dental
Developmental issues (nearly all have mild-mod ID) Behavioural and psychiatric issues (most commonly ADHD, autism)
Obesity Thyroid (hypothyroidism) Coeliac disease OSA Leukaemia Infections Seizures Diabetes mellitus Constipation
Atlantoaxial instability
Orthopaedic (DDH, scoliosis)
Cystic fibrosis checklist
Lungs - FEV1 - microbiology (Pseudomonas, Burkholderia) - ABPA - haemoptysis - pneumothorax - transplant Sinuses
Cor pulmonale
GI
- mec ileus
- DIOS/constipation
- CFRLD
- pancreatic insufficiency
- CFRD
- rectal prolapse
- small bowel bacterial overgrowth
Immunisation
Growth, nutrition, weight loss (non compliance, inadequate intake, inadequate PERT, chest infection, CFRD, coeliac, IBD)
Bones
Fertility
Management issues - emergency planning
Resuscitation directives Crisis management at home/plans (seizures, hypoglycaemia, asthma, adrenal crisis stress dosing, PEG displacement, tracheostomy) - written plan - CPR training - medicalert bracelet
Management issues - communication in disabled child
“Augmentative and alternative communication devices (ACC)”
Communication depends on vision, hearing, fine motor skills, memory, cognition, concentration
Basics
- asses hearing and vision formally and regularly
- hearing aids, glasses
- environment modification
SP/OT/rehab
Non-aided
- gestures (informal vs formal)
- key word signs
Aided
- communication books/boards, printed words on cards, pictures
- tactile feeling boards
- low technology
- high technology message systems
Communication resource centre and funding schemes
Management issues - irritability
Differentials:
- GORD
- constipation
- dental
- fractures
- pressure sores
- otitis media
- in CP: seizures, hip dislocation, UTI, medications, dystonia
Manage contributors:
- sleep
- environment (light, music)
- non pharma: massage, acupuncture, aromatherapy
- pharma: melatonin, diaz, paracetamol, baclofen
Management issues - minimising impact of hospitalisation
Therapists: social work, play therapy, diversional therapy Finances - travel and transport allowance - on site hospital accommodation - parking/disabled permits - food vouchers School - encourage attendance as able, ?day leave, continue curriculum in hospital - can friends visit?
Day leave/weekend leave/special outings
HITH
Maximise equipment available at home to minimise comorbidity
Keeping them well
- immunise
- no household smokers
- vision/hearing/dentition surveillance
- parental health literacy
Management issues - precocious puberty
Boys more likely than girls to have serious pathology
Separate from pubarche and adrenarche/thelarche
- adrenache <8 girls <9 boys (pubic hair, skin sebum changes, mood, acne), benign but diagnosis of exclusion (ddx CAH, tumours, central precocious puberty, exogenous androgens, Cushings syndrome)
- pubarche <8 girs <9 boys, can be central or peripheral
- thelarche benign
Ix
- DHEAS, 17OHP, adreostenodione, testosterone
- bone age XR
- gonadotrophins, oestradiol
- adrenal USS
- refer to endo for stimulation tests
Rx
- serial review
- pubertal suppression
- GnRH agonists
Management issues - rural issues
Access to health care
- AH
- subspecialists
- support groups
Transport
- SW, financial support
- cluster appointments, telehealth
Accommodation
Social supports/contingency plans
- care for other children
- work e.g. family business
Emergency/specific issues
- more likely to delay presentation
- increase training for parents
- increase supplies at home
- document emergency plan
- AV cover
- phone coverage
- delivery of equipment and supplies
Management issues - transplant
(Only gen paeds stuff, specialists would be involved)
Renal most common
Monitor for rejection Monitor for complications of immunosuppression Growth and nutrition Vaccinations - 5 yearly pneumovax - flu - COVID - avoid (or care) with live vaccines if haven't completed schedule yet
