Management issue spiels Flashcards
Aspiration
Importance: each episode can cause life-threatening respiratory compromise, and recurrent episodes can cause chronic lung disease
Points
ITSELF
- witness a feed
- ask for acute symptoms or concern for silent aspiration
- treat promptly: abx + resp support as needed
- education about aspiration symptoms (choking during feed, wet lung sounds and work of breathing) and crisis plan to present to ED
RISK FACTORS (assess and manage)
- dysphagia - modified oral diet, compensatory strategies (positioning, different teat, swallowing exercises, calm environment) or non-oral feeds
- secretions - positioning, oral motor skills training, anticholinergic medications (scopolamine, hyoscine, glycopyrrolate), procedural intervention such as salivary gland botox or ligation/resection (if severe may need tracheostomy)
- reflux
- uncontrolled seizures
SEQUELAE (assess and manage)
- chronic lung disease - consider lung function tests
Chest health
Importance: chronic lung disease affects quality of life and predisposes to life-threatening infections
ITSELF
- pulmonary function tests
- education
CONTRIBUTING FACTORS
- aspiration (appropriate acute event management, dysphagia, secretions, reflux, uncontrolled seizures)
- poor airway clearance: chest physiotherapy, mucolytic therapy, cough assist, suction
- restrictive lung disease: brace/surgery
- obstructive sleep apnoea: T&As/CPAP/BiPAP
- lifestyle (nutrition, exercise, smoking, poor oral health, vaccination): dietetics referral, physiotherapy goals, quit smoking, regular dental review, vaccination for whole family
- comorbidities: asthma: asthma action plan
Menstruation in disability
Importance: pain, discomfort, quality of life, risk of pregnancy (incl in context of higher risk of sexual abuse)
Principles - best interests of the woman, least restrictive first
OVERALL
- menstrual diary and property characterise symptoms
SEQUELAE
- hygiene: EDUCATION, menstrual hygiene products (incl continence products)
- menorrhagia: TXA
- pain: analgesia ladder
- behaviour: behavioural strategies, behavioural psychologist, antipsychotics (last resort)
- seizures: address other contributors, adjust AEDs
- sexuality: education and safety
- overall consider contraception (OCP, LARC, surgical options)
Puberty in disability
Importance: self-image and relationship to others changing - increasing autonomy
I would like to assess and manage Ollie’s
- Medical transition - individualised transition plan, referral to transition service, joint appointments, appointments alone, tailored education, referral to support groups
- Future plans - liaise with school/council/social worker/disability support groups regarding programs/resources for future learning, income, community; discuss with family re: accommodation plans
- Physical maturation incl menstruation and sexuality - education, as per above
- Exacerbation of behavioural episodes/seizures - behavioural strategies, behavioural psych re-engagement, medications last resort, seizures - evaluate for other contributors incl triggers, pain and consider adjusting medication
- Mental health issues - thorough assessment, MHCP and psych/headspace/school counsellor
Hip disease in disability
Importance: hip dysplasia reduces mobility, causes pain
ITSELF
- assess: acute episode (pain - acute OR chronic of unclear cause, limited ROM) or need for screening (regular AP pelvis xrays especially if non-ambulatory and quadriplegic)
- xray: migration percentage 30% plus refer to surgeons
- treatment: non-invasive abduction bracing, soft-tissue release, femoral or pelvis osteotomies (or salvage procedure - proximal femoral resection)
- EDUCATION
RISK FACTORS
- spasticity: treat
SEQUELAE
- pain: analgesia ladder
- ADLs, caregiving: hygiene
Scoliosis
MORE GROWTH POTENTIAL AND COBB’S ANGLE
= PROGRESSION MORE LIKELY
Importance: pain, impaired mobility, restrictive lung disease, neurological sequelae, ADLs and caregiving
ITSELF
- assess clinically - location, severity, follow on effect on shoulders/pelvis/rib prominence; whole spine xray PA and lateral looking for Cobb’s angle (less than 15 degrees monitor, more than 40 degrees surgery)
- manage: observation vs physio and bracing vs surgery (growing rods pre-adolescence, spinal fusion after 10-12yo)
RISK FACTORS
[- causes (risk factors): non-structural, idiopathic, congenital, neuromuscular, other]
SEQUELAE
- pain: exercises, physio, consider spasticity meds
- pressure sores, neuro sx, lung function
- ADLs, caregiving - dressing, hygiene
- mental health
Behavioural issues in disability
Important due to distress to patient and family, and difficulty with treatment compliance and health service engagement.
ITSELF
- behaviour: type, triggers, patterns of escalation
POTENTIAL CAUSES (assess and treat)
- environmental (stimuli, communication difficulties)
- physical (pain, seizures, neurological decline/expected symptoms, meds, thyroid/B12/electrolytes)
- psychiatric (mood, anxiety, psychosis)
(BEHAVIOURAL TECHNIQUES
- behavioural psych
- consider medications: antipsychotics, beta-blockers, SSRIs, mood stabilisers)
SEQUELAE
- family’s mental health
disorder - psych
Spasticity
Importance: pain, mobility, ADLs and caregiving
ITSELF
- assess: affected joints, ROM
- treat: non-pharm: physio stretches, orthoses, casting
- pharm: localised - botox; generalised - baclofen, benzos, dantrolene; refractory - intrathecal baclofen
- surgery: selective dorsal rhizotomy, muscle/tendon lengthening/release, osteotomy
SEQUELAE
- pain (and effect on sleep)
ADLs, caregiving (eg dressing, hygiene)
bone health (because mobility) and hip dysplasia
(??- medication side effects)
I am concerned about Ollie’s spasticity due to his significant mobility impairment and pain, and resulting impact on quality of life, function, and caregiving difficulty.
I would like to assess the involved joints and limitation in mobility; and the extent of resulting pain, functional impairment, caregiving difficulty, and assess for associated hip dysplasia and decreased bone mineral density.
I would like to liaise with the rehab team, orthopaedic surgeons, physiotherapists and orthoptists regarding non-pharm strategies such as physiotherapy exercises, orthoses and casting. Medication regimen adjustments such as adding local(/generalised) agents such as botox injections(/baclofen, benzos, dantrolene) or consider intrathecal baclofen if refractory. I would also consider the role of selective dorsal rhizotomy, muscle/tendon lengthening or release and affected joint osteotomy.
I would also treat complications such as pain, decreased bone mineral density and hip dysplasia.
Reflux
Importance: can cause failure to thrive, contribute to aspiration, cause symptomatic pain/irritation, ??lead to malignant transformation
ITSELF
- assess: reflux episodes, severity/frequency; consider barium swallow, oesophageal ph /impedance, scope
- manage:
conservative: (infants) thickening, smaller more frequent feeds, avoid overfeeding, avoid seated or supine positioning???, avoid tobacco smoke, CONSIDER EXCLUDING CMPI; (older kids) weight loss, avoid chocolate/caffeine/spicy food, avoid smoking and tobacco diet changes
pharm: PPI (or H2RA);
surg: fundoplication if refractory and severe; consider postpyloric feeds
SEQUELAE
- resp symptoms - assess aspiration, lung function
- failure to thrive - monitor and supplement if necessary (and look for additional contributors)
Obstructive sleep apnoea
Importance: impacts on behaviour and schooling and at chronic/severe end, can cause FTT, metabolic syndrome, heart failure
ITSELF - OSA symptoms (snoring, apnoeas, EDS), overnight sats monitoring; sleep study RISK FACTORS - predisposition: T+As, anatomical, neuromusc, obesity OVERALL management - T+As, CPAP/BiPAP, weight loss SEQUELAE - metabolic syndrome - monitor and treat - behaviour - behavioural strategies - school - liaise with school - socialising - FTT
Sleep problems
Importance: impacts on behaviour, schooling, family
ITSELF
Assessment
- BEARS (bedtime problems, EDS, awakenings during the night, regularity and duration of sleep, snoring) - sleep diary
- elaborate on specific problem - insomnia (sleep-association, limit-setting, anxiety-related), delayed sleep phase, parasomnias
- mx: sleep hygiene: sleep-wake cycle and bedtime routine, food (avoid caffeine, going to bed hungry), environment (quiet, dark bedroom, good temp, not used for anything else, no screens in bedroom), activity (sun and exercise during day, 1hr quiet time before bed, no screen time before bed)
IF infants consider self-soothing and mothercraft clinics
(specific mx below)
CONTRIBUTING FACTORS
- mental health (depression)
- (adolescents) substance use
SEQUELAE
- behaviour
- schooling
- mental health
- family
(Management targeted to problem
- sleep phase delay: 15min earlier awakening
- night terrors: scheduled waking
- nightmares: reassurance, de-arousal strategies)
Seizures
Importance: uncontrolled seizures lead to risk of SUDEP, possible life-threatening event if swimming etc, ongoing neurodevelopmental injury
ITSELF - semiology, frequency - seizure diary CAUSES - triggers - avoid - pain - analgesia - neurological decline - electrolyte derangement - treat OVERALL - consider referral to increase/change AEDs SEQUELAE - AED side effects
ALSO
education: seizure emergency plan, safety advice
Bone health
Importance: risk of recurrent fractures and resultant pain, immobility, deformation
Assessment
ITSELF
- hx of fractures, recent DEXA
RISK FACTORS
- vitamin D and calcium deficiency - optimal nutrition and dietary maximisation, consider supplementation
- decreased physical activity - increased load bearing physical activity
- medical conditions (genetic conditions, chronic disease, immobility, meds…)
OVERALL
- (vit D supp and Ca dietary maximisation even if not deficient)
- also consider bisphosphonates
SEQUELAE
- pain, deformation, immobility - prompt treatment of fractures and analgesia as necessary
Immunosuppressants
SUNSCREEN!
Assess for side effects
- bone marrow suppression, GI disturbance
- lung: pulm fibrosis (CPA) or pneumonitis (AZA/MTX)
- GI: hepatotoxicity (AXA, MTX, CSA), pancreatitis (AZA)
- renal: impairment (CSA/TAC)
- endo: pre-diabetes (CSA/TAC)
- neuro: seizures, tremors (TAC)
- gingival hyperplasia, hypertrichosis (CSA)
- impaired fertility (CPA)
- alopecia (CPA, AZA)
- oncogenesis (CPA)
Management priorities
- Monitor for and treat side effects (BP, FBC, EUC, LFTs)
- Prevent - infections (prophylaxis vax), cancer (SUNSCREEN)
- Prevent cardiovascular morbidity
Antiepileptics
Carbamazepine: SJS, agranulocytosis, hypoNa
Lamotrigine: SJS, hypersensitivity
Levetiracetam: somnolence, behavioural
Valproate: weight gain, pancreatitis, liver failure, teratogen, hair loss
Phenytoin: hirsuitism, gum hypertrophy, aplastic anaemia, osteoporosis, serum sickness
Topiramate: kidney stones, glaucoma, metabolic acidosis, hyperthermia, weight loss
Phenobarbitone: behaviour, cognitive, rash
Vigabatrin: vision, weight gain, psychosis
CYP inducers: carbamazepine, phenytoin, phenobarbitone, topiramate
CYP inhibitors: valproate
SEIZURE THRESHOLD LOWERERS: antipsychotics, antidepressants, stimulants (and some abx, atopy meds, anaesthetics)
Transition
- Individual transition plan - address services, timeline, barriers
- Preparation (from 14yo) - education (condition, services, transition), empowerment (self-management, decision-making), support (groups, forums) - adolescent appropriate language, confidentiality and technology
- Transition service
- Teamwork and communication - joint transition clinics and/or period of joint care, written information to everyone, named contacts within each team
- Follow up - at least 12 months after incl plan for failed transition
