Long Cases Flashcards

1
Q

What are key domains for developmental assessment <5 years old?

A

< 5 years
* Global developmental delay
* Gross motor - what can they do - equivalent to 2 month old
* Fine motor - unable to grasp
* Speech - non verbal 2 month old
* Social -
* Hearing -
* Vision -

Function
* Moves around
* Head control
Double incontinent

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2
Q

What are key domains for development and functional assessment > 5 years old?

A

Older >5
* Functional impairment, developmental delay
* Full scale IQ of ..
* Gross motor/Mobility- hoist, 2 hour requirement
* Fine motor/ADLs - feeding, dressing etc needs assistance with all activities of daily living
* Language/Communication -non verbal, how does she communicate
* Social - Behaviour and socialisation - aggressive, outburst, joy

  • Hearing and vision
  • Continence - Double incontinent - bladder and bowels
  • School - Mainstream, specialist, year, attendance is poor once per month, how they get there
  • Sleep
  • Constipation
  • Strengths - what they like
  • What can they do - something to do
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3
Q

Spasticity - what are key areas to assess and Mx steps?

A
  • Hypertonia and contractures secondary to upper motor neuron

Assessment
* Functional mobility
* Pain
* Contractures
* Pressure sores
* Hip dislocation
* Scoliosis
* Previously trialled treatments

Goals - to improve
* Function
* Enlocation of joints
* Comfort and care

Management
Non pharm
* Casting and AFOs (ankle foot orthoses)
* Physiotherapy - stretching exercises, hydro, swimming

Pharm
* Diazepam/ Clobazam (benzo)
* Dantrolene (Ca)
* Baclofen oral (GABA)

Injected botulism (presynaptic)

Intrathecal Baclofen (GABA)
* Dystonia and mixed tone - GMFCS IV and V

Surgical
* Tendon release
* Hip surgeries - adductor lengthening, if dislocated - femoral/pelvic osteotomies
Selective dorsal rhizotomy - sensory root without affecting motor, can worsen dystonia, patients chosen

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4
Q

Dystonia - key Mx?

A

Dystonia
* Muscles to contract involuntarily

Mx
* Baclofen
* Benzhexol
L-dopa

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5
Q

Seizures - areas to assess and key Mx steps?

A
  • Seizure types, generalised, semiology
  • Episodes of status, complications
  • Current medications and Mx
  • Complications of medications
  • Emergency Mx plan

Mx
* AEDs
* Seizure emergency management plan and education
CPR training

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6
Q

Seizure Safety and Emergency Mx plan?

A
  • Seizure first aid and Mx
  • Emergency Mx plan
  • Epilepsy Australia
  • Bracelet
  • No baths, unsupervised swimming
  • Driving
  • Contraception and pregnancy- AED and teratogenic
  • Alcohol - decrease seizure threshold

Seizure Management Plan
* Education - swimming, baths, climbing heights
* Basic first aid
* Seizure Mx plan
* Hx, medications, seizure types
* Specific - first aid, call ambulance, +/- midazolam
* Midazolam - duration of seizures, proximity to hosp, comfort level
* Liase with Neurology

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7
Q

Scoliosis - areas to assess, Mx steps?

A
  • Interested to know bracing pre surgery
  • Cobb’s angle
  • Causes - neuromuscular (CP, high/low tone), syndromes, congenital, orthopaedic
  • Scoliosis impact - chest deformity, pain, difficult ADLs, crush #s
  • Assess on x-ray - Cobb angle
  • Impact on function - standing, sitting in wheelchair, resp function

Mx
* Mx in CP is challenging - difficult to determine if benefits outweigh risks, requires expert MDT input
* Prevent - well designed chair
* Cobb angle + QOL
* < 15 degree - monitor for Cx
* 20-40 degrees - wheelchair, physio, Brace
Ø 40 - surgery
· Surgical - spinal fusion

Pre - op
· Resp - PFT, resp infection, pre-op Bipap
· Nutritional status - baseline nutritional bloods, optimise
· GI - treat reflux and saliva control
Cardio - Echo

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8
Q

Bone Health - areas to assess, key Mx steps?

A
  • Hx - diet, sunlight, medications (steroid, AEDs), puberty, conditions (malabsorption, ESRF)
  • Ex - evidence of rickets - bowing of legs, widened growth plate, rachitic rosary (on chest)
  • Ix - DEXA scan - 2 yearly, Z scores matched to bone age
  • Osteopenia 1 SD below, osteoporosis 2SD below
  • X-ray - crush #s
  • ALP, Ca, Vit D, PTH

Mx
* Diet, sun, physical activity (weight bearing)
* Ca, Vit D supplementation
* Bisphosphonate (Zoledronic acid) most common - 6 monthly
* AE - post dose fever, myalgia, rigors, vomiting
Manage fracture risk

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9
Q

Sialorrhea/ Saliva Control - areas to assess and Mx steps?

A
  • Drooling normal until 18 months when oro-motor control established
  • Causes - bulbar dysfunction (lower CN nerves for swallowing), cleft lip/palate, posture
  • Ix - evidence for aspiration - CXR, video fluoroscopy ?microaspiration
  • How many times wiping, suctioning
  • Mechanical device, medication, surgery

Mx
* Position, speech path, waterproof scarf/bib
* Anticholinergic - atropine drops, glycopyrolate, hysosycine hydrobromide
* SE - sedation, urinary retention, constipation, thickened saliva
Surgical - Botox, re-location of salivary glands, SE - dry mouth resulting in dental issues

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10
Q

Aspiration - key areas to assess, causes, Ix and Mx steps?

A
  • Bulbar dysfunction and GORD
  • Hx - coughing with feeding, recurrent chest infections, wheeze, drool/pooling saliva, apnoea
  • Ix - observe a feed -phase of swallowing, trigger a swallow, larynx rise with swallow
  • CXR - aspiration
  • Further Ix - liase with speech pathology and gastro
  • Video fluro - risk of aspiration
  • Barium meal/swallow
  • Milk study
  • Saliva study
  • Esophageal pH monitoring
  • BAL

Mx
* Bulbar dysfunction - thicken feeds/nil oral feeds, nasogastric, nasojejunal, saliva management
Reflux - thicken feeds, position after meals, H2 antagonist, PPI, fundoplication + PEG/PEJ

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11
Q

Fundoplication?

A

Fundoplication
* Indications - neurological disease, not responding to medical Mx, esophagitis Cx - peptic strictures, Barrett’s oesphagus, gastrostomy feeds, resp disease
* Improve - 60-90%
* Cx - suture breakdown, adhesions, oesophageal obstruction, dumping syndrome

Reflux from bottom
Waking up secretions
Vomiting - microaspiration

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12
Q

Long case structure - medical problems Hx?

A

DSIT CCF + Other

* Diagnosis - when was it diagnosed
* Symptoms - often not relevant 
* Investigations - what investigations confirmed the Dx?
* Treatment - how is it treated? (Medical and surgical) 

* Complications - need to prompt for key complications, presenting relevant negatives important 
- Enterostomy - ever been pulled out? Any infections? Any obstruction? 
- Seizures - any prolonged seizures? Had to go to ICU? 
· Course - overall getting better/worse/staying the same? 
- Helpful to work if major problem or not 
* Future - what are plans for future?  \+ Other important
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13
Q

Development + Function and Growth structure long case?

A

Development + Function and Growth
· IQ
· Gross motor and mobility:
· Fine motor and ADLs: feeding, dressing, assistance
· Social: behaviour, socialisation
· Language and Communication: non verbal, how communicates
· Hearing:
· Vision:
· Continence/stools/urine/constipation- double incontinent
· Strengths/Can do -

Feeds/Growth/Nutrition
· How feeds - any cough/gag/aspiration
· Weight
· HC
· Length
Diet

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14
Q

General health screen/Systems review screen?

A

SPET PBSM + systems
· Sleep/OSA - sleeping arrangements, naps
· Puberty
· ENT
· Teeth

· Pain & Procedures
· Behaviour
· Smoking (in family)
· Mental health

· Neuro & seizures
· Cardio
· Resp
· Abdo
· Bones
Infections

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15
Q

Medications mneumonic long case Hx?

A

Medications
AACCS
* Administration
* Alternative
* Compliance
* Cost
* Side effects

Allergies
Immunisations
· Flu, COVID, household

Equipment

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16
Q

FHx structure?

A

MMECC
* Medical problems FHx
* Miscarriages
* Ethnicity/indigenous + citizenship
* Carer/custody:
Consanguineous

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17
Q

Social Hx structure?

A

AT WFN APH
Accommodation (rental/mortgage)
· Rooms in house
· Modifications
· Single/double story
· Steps

Transport and who can drive
- Transport allowance

· Work 
· Finances
- Extra support/bursaries 
- Medication costs
· NDIS

· Ambulance 
· Private 
· Health care card

SRC DV
· Supports:
· Respite, holiday
* Crisis plan
* Drugs and alcohol
* Violence

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18
Q

Patient HEADSS structure?

A

Patient
HEADSS
* Home
* Education & school, employment:
* Activities/fun, ambition:
* Drugs and alcohol:
* Suicide/mental health, body image/self esteem:
Sexuality (boyfriend, girlfriend), contraception:

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19
Q

Structure for final part?

A

GFUQ NPE
Final
· Goals and hopes
· Fears - what is biggest fear?
· Understanding of illness
· QOL - what impacts on X quality of life the most?

· Negative experiences - teams, delayed diagnosis?
· Palliation/ACP (advanced care plan) - any treatments wouldn't want Examiners asked -
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20
Q

Examination long case?

A

Examination
* Picture general - paint picture of patient
* Growth and nutrition: Weight, head circumference, height
* Puberty
* Vitals
All other relevant positives and negatives

21
Q

Interpretation of growth parameters?

A

Growth Interpretation:
* Weight < length < HC - nutritional (weight low)
* Length < weight < HC - endocrine (height low)
* HC < weight < length - neurological (HC low)
Weight = length - genetic/constitutional

22
Q

Sleep Mx?

A
  • Hx - prior to sleep, stimulation
  • During sleep - number of awakenings, settling, sleep, snoring
  • Morning -time of waking, refreshed
  • Sleep diary
  • Ix - if concerns of OSA - overnight oximetry, formal sleep study
    Mx
  • Education - regular routine, avoid stimulating activities before bed, appropriate environment, sticker charts
  • Pharm - melatonin -assist sleep onset
  • Sedating antihistamines - chloral, phenergan
23
Q

How would you manage degeneration in CP?

A
  • Consider:
  • Wrong diagnosis
  • Comorbid condition - infection, B12 defic, Fe defic, hypothyroid
  • Seizures
  • Medical AE
  • Msk disorder
  • Subclinical infection

Ix
* Basic - FBE, Fe, inflamm markers, TFTs
Re-investigate cause - MRI, metabolic, CK, microarray

24
Q

How do you optimise development for developmental delay?

A

Developmental delay - optimise development for maximal function
1. Close monitoring - regular review
2. Stable and happy family - practical support, family time, financial support
3. Keep child well + surveillance - vaccinations, nutrition, hearing/vision/sleep
4. Allied health - early intervention services to maximise functional potential, influence trajectory
5. Referral Early intervention services
Referral NDIS

25
Q

How would you manage pain and distress?

A

Assessment - Hx, Ex, Ix
Non pharm - heat pack, distraction, physio/exercise, CBT, co-morbidities, hypnosis
Pharm - analgesic ladder non opioid, opioid, adjuncts neuropathic - gabapentin, amitriptyline

26
Q

Management to optimise finances?

A

Centrelink - disability support pension, carer allowance, health care card
Social work involvement - local bursaries
Transport reimbursement
Disability parking

27
Q

What is approach to poor growth?

A

Assessment - prev growth trajectory, intake, output, nutritional bloods
Consider barriers
Indications for intervention - poor weight gain, micronutrient deficiency
Management - food fortification, oral supplements, NGT feeding

28
Q

How would you manage recurrent respiratory infections (in CP?)

A

How would you manage her recurrent respiratory infections?
1. Establish contributing cause
- Aspiration - GORD, poor saliva control
- Restrictive lung disease - scoliosis
- Neuromuscular weakness
- OSA
2. Investigations if as required
- Sleep study
- Video fluroscopy, barium swallow
3. Manage
- Manage contributing issues - aspiration, sialorrhea, OSA
- Non Pharm - Chest physiotherapy, normal saline neb, treatment intercurrent infections
4. Keep well - vaccinations, good nutrition, less contact with unwell children

? Qualify for prophylactic antibiotics

29
Q

How would you manage reflux?

A

Symptoms suggesting:
* Vomiting, distress, RESPIRATORY INFECTIONS, ALTE

Complications
* Esophagitis, poor growth, recurrent resp infections

Ix
* 24 hour esophageal pH monitoring
* Endoscopy- esophagitis, strictures, complications

Mx
Non pharm:
* Thicken feeds
* Positioning upright with feeds
* Pharm:
* H2 antagonist (raniditine)
* PPI (long term SEs - Resp infections, C diff infections, bone fractures, low Mag and B12, tubulointersitial nephritis)
* Antacid
Prokinetic (domperidone) rarely used

30
Q

What are main SEs of prolonged/high dose steroids?

A

SHIM LEGS B

S - Skin
* Striae (stretch marks)
* Bruising
* Thin skin
* Cushingoid appearance
* Hirsuitism
* Acne
* Facial erythema

H - Hypertension + Cardiovascular
* HTN
* Fluid retention

I - Immunosuppression
* Increased risk of infections

M - Muscle and Bone
* Osteopenia and decreased bone density
* Myopathy (proximal)
* AVN and SUFE

L - Liver + GI (Ulcers)
* Fatty liver
* Peptic ulcers

E - Eyes
* Cataracts
* Glaucoma

G - Glucose + adrenal
* Hyperglycemia + diabetes
* HPA axis insufficiency

S - Short
* Growth
* Short - decreased height

B - Behaviour
* Behavioural change
* Mood and headaches
* Dysphoria/depression
* Insomnia
Mania/psychosis

31
Q

How would you manage patient on long term/high dose steroid?

A
  1. Monitor for side effects
    Clinical
    - Height, growth, weight gain
    - HTN
    - Proximal myopathy
    - Behaviour and mood change
    Investigations
    - HbA1c
    - Lipids
    - DEXA bone scan and lumbar spine X-ray
    - Opthal for cataracts
  2. Minimise dose
    - Explore alternatives
  3. Treat side effects
    - Optimise bone health - Vit D and calcium, weight bearing, bisphosphonates
    - Antihypertensives
    - Oral hypoglycemic agents
  4. Health optimisation
    - Immunisations - quality for additional immunisations
    - Diet
    - Education - steroid plan
    - Pregnancy - contraindicated
    Teenaged - CI if on OCP and has HTN
32
Q

How would you manage aspiration?

A
  1. Consider: above - bulbar dysfunction, below - GORD
  2. Thorough history - coughing, recurrent chest infections, drooling saliva
  3. Investigation
    - Observe feed
    - CXR
    - Liase with Speech Path and Gastro - video fluro, barium swallow etc
  4. Management
    * Bulbar:
    * Thicken feeds/nil oral feeds
    * NGT, NJT
    * Saliva Mx
    * Reflux:
    * Thicken feeds
    * Position
    * H2 antagonist, PPI
33
Q

How would you manage oral aversion?

A
  1. Eliminate contributing factors
    - Candida, mucositis, reflux
  2. Educate parents
    - Start food at an early age
    - Range of textures and colours - maximise palate development, identify foods they like, fun, no pressure
  3. Create MDT team
    - Speech path, play
    Monitor growth and nutrition long term
34
Q

How to optimise/manage communication?

A
  • Optimise communication - hearing, vision, support (sitting upright), enviro mod
  • AAC (augmentative and alternative communication device) - consider cognition, fine motor, vision, options - direct touch, pointer, vision directed
  • Options - non- aided - gestures, signs, aided - communication boards, low or high technology
    Involve allied health team
35
Q

How would you optimise finances and options?

A

Centrelink
· Carer allowance - not means tested
· Carer payment - income for full time carers
· Automatic for health care card
· Until 16 years old
· Disability support pension

Social work
· Local bursaries
· Condition specific

Transport reimbursement
Disability parking

36
Q

What is ECEI and NDIS and criteria and used for?

A

Early Childhood Early intervention
* < 6
* Developmental concern 2 areas
* No diagnosis

NDIS

  • Criteria - requires diagnosis, build capacity and function
  • NOT for respite, NOT related to school
  • Paed role - advocate access, planning meeting, setting goals
    Steps - apply online, assess function and needs, > 12 months - package starts
37
Q

How would you manage compliance issues?

A
  • Rapport
  • Understanding of illness and effects of medications
  • Barriers to compliance
  • Collaborate with child/family to come up with strategie

Adolescence
* Autonomy and independence
* Collaborate with teenager to come up with strategies
* Specific:
* Educate
* Take ownership of illness
* Simplify dosing regime
* Specific side effects and consider changing if having Ses
* Improve access - written instructions, scripts, financial
* Reduce forgetfulness - alarm associate with daily activity
Set goals and review reguarly

38
Q

Steroid Dependence Mx?

A
  • Minimise dose - work with treating team
  • Monitor for side effects - growth, weight, BP, mood and affect, Opthal
  • Ix - HBA1c, DEXA
  • Treat complications:
  • Vit D/Ca, bisphosphonates
  • SSRI (mood)
  • PPI
  • Health prevention:
  • Vaccines: extra vs caution with live
  • Weight Mx
  • Stress dose plan
  • Pregnancy considerations
    Drug interactions
39
Q

Medication compliance Mx?

A
  • Rapport
  • Explore understanding, listen, why
  • Barriers to taking - forgetting, taste, embarrassment, complex dosing, perceived non benefit, Ses
  • Collaborate with child/family to come up with strategies
    Reminder system, different forms, dosing schedule, not around friends/peers, simplify, education, explore alternatives
40
Q

T21 main systems involved?

A
  • ID and behaviour
  • Cardiac - AVSD, VSD, ASD, TOF
  • Gastrointestinal - intestinal atresia, Hirschprung’s, coeliac
  • Endocrine - short stature, hypothyroidism, obesity
  • Eyes - cataracts, nystagmus, strabismus
  • Ears - otitis media
  • Neuro - atlantoaxial instability
  • Haematological - leukemia
  • Sleep - OSA
  • Gonadal - undescended testes, hypogonadism
    Dysmorphology - upslanting palpebral fissure, epicanthal folds, small low set ears, flat nasal bridge, protruding tongue, short neck
41
Q

T21 screening?

A
  • Audiology and ENT (yearly)
  • Dental (6 monthly)
  • Opthal (annual then every 2-3 years)
  • Cardio - Echo
  • Atlanto-axial instability
  • OSA - sleep study if required
  • FBE - annual
  • TSH - annual + symptoms
    Coeliac
42
Q

Turner Syndrome - manifestations?

A

Manifestations
* Short stature
* Delayed puberty - 1/3 will have delayed puberty
* Ovarian failure/infertility
* Cardio - bicuspid aortic valve, co-arctation, AS, risk of dissection
* Autoimmune - coeliac IBD, hyper/hypothyroidism, diabetes
* Msk - scoliosis, low bone density
* Renal - horseshoe kidneys
* Ears - middle ear otitis media
* Eyes -
Neck webbing

43
Q

Turners - screening?

A
  • Growth - consider growth hormone
  • Puberty - delayed, bone age, consider estrogen (not in puberty 12-14 yrs)
  • Autoimmune - coeliac, thyroid, HBA1c
  • Cardiac - Echo BP monitoring
  • Renal - USS
  • Hearing - annual
  • Dental
  • Psychosocial - fertility counselling
    Cosmetic - webbing neck
44
Q

Extra immunisations prem?

A

· RSV Ig (Palivizumab) - < 29 weeks and < 12 months old - not in 2nd year of life based on prem
· VLBW may have lower response to Hep B - may need extra
< 28 weeks extra dose pneumococcal

45
Q

Consequences of Prematurity?

A

Brain
· Neuro - Grade III/IV IVH, CP evolving - diagnosed 2 years

Chronic lung disease
· Home O2
· Smoking at home
· Long term reduced lung function- small airway disease

Metabolic bone disease
Renal disease

Immunisations
· RSV Ig (Palivizumab) - < 29 weeks and < 12 months old - not in 2nd year of life based on prem
· VLBW may have lower response to Hep B - may need extra
· < 28 weeks extra dose pneumococcal

Growth
· BW
Growth restricted

46
Q

Respiratory optimisation in prematurity?

A

· Minimising resp infections - smoking, hand hygiene, avoiding contact those unwell
· Optimise nutrition - higher calorie, monitor feeds, dietician
· Vaccination and consider pavalizumab and extra pneumoccal
Further Ix - pulmonary HTN, OSA

47
Q

Mental health in teenager?

A
  • Hx - mood, triggering factors, strategies tried
  • Risk assessment - acute risk suicidal ideation, harm to others
  • Ex - mental state exam
  • Consider side effects medications - pred/immunosuppression
  • Ix - FBE, Fe, TFTs,
  • Mx
  • Non pharm - GP with mental health care plan, psychoeducation, CBT
    Pharm - SSRI, SNRI
48
Q

Substance use and smoking Mx?

A
  • Assessment - reason for marijuana use, understanding of risks, feelings around quitting
  • Motivational counselling - health impact, financial, downsides of marijuana, positives of quitting
  • Collaborate with him establish Mx plan - goals, contract
  • Regular review
  • Support if relapses
    Adolescent team input
49
Q

Mx of transition?

A
  • High risk time - often transition from school to uni, home to outside etc
  • Begin discussions and planning - early adolescence 15 yrs old, involve local transition service, location of future care with accessibility
  • Key specialities required - future care, documentation, formal handover
  • GP - find and establish relationship
  • Independence of Michael - lead consultations, time away from parents
  • Transition checklist - understands medications, doses, SEs, reasons, own medical Hx, how to access medical services
    Create transition plan - meetings required, documentation, formal handover