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
