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
How would you manage pain and distress?
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
Management to optimise finances?
Centrelink - disability support pension, carer allowance, health care card Social work involvement - local bursaries Transport reimbursement Disability parking
27
What is approach to poor growth?
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
How would you manage recurrent respiratory infections (in CP?)
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
How would you manage reflux?
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
What are main SEs of prolonged/high dose steroids?
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
How would you manage patient on long term/high dose steroid?
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
How would you manage aspiration?
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
How would you manage oral aversion?
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
How to optimise/manage communication?
* 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
How would you optimise finances and options?
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
What is ECEI and NDIS and criteria and used for?
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
How would you manage compliance issues?
* 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
Steroid Dependence Mx?
* 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
Medication compliance Mx?
* 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
T21 main systems involved?
* 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
T21 screening?
* 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
Turner Syndrome - manifestations?
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
Turners - screening?
* 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
Extra immunisations prem?
· 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
Consequences of Prematurity?
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
Respiratory optimisation in prematurity?
· 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
Mental health in teenager?
* 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
Substance use and smoking Mx?
* 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
Mx of transition?
* 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