Non malignant disease Flashcards
State EF for:
HFrEF
HFmrEF
HFpEF
<40%
40-50%
>50%
Median survival for CHF exacerbation and age >65
2 years
3 prognostic tools in CHF
Seattle HF score
HF survival score
MAGGIC
Challenge with HF prognosis tools
None are good at predicting prognosis within months
Seattle heart failure score overestimates prognosis in advanced disease
Which medications should be avoided / used with caution in CHF?
NSAID (CHF, AKI, and mortality risk)
Amphetamines
Steroids (Dex is safest)
Gabapentinoids (increase edema Pregab > Gaba)
TCA’s
Antipsychotics
Which SSRI/SNRI’s have proven safety in CHF?
Sertraline
Venlafaxine
Evidence based treatments for HFrEF
- ACEi/ARB
- B-blockers
- Spironolactone
- If channel inhibitor (Ivabradine)
- Hydralazine / Nitrates (esp. if unable to tolerate ACE/ARB)
What is the major adverse effect of combining Furosemide and Risperidone?
Incr. mortality in the elderly
How does digitalis work?
Reduced sympathetic tone (augments parasympathetic tone), improves myocardial contraction
HFrEF - improved symptoms (fatigue, dyspnea), no mortality benefit
HFpEF - no benefit
Inotropes
- 2 key medications and what they do
- Outcomes and indications
Milrinone (inotrope, vasodilator) and Dobutamine (synthetic catecholamine)
Improve symptoms but increase mortality
Short term indication to increase CO and diuresis
Palliative HF
Indications for pacemakerinCHF
EF <35%
LBBB
Wide QRS
Improvements in QOL and survival
What is the consequence of overtreating hypoxia in CHF
vasocontriction -> SVR -> reduction in CO
LVAD
- time to, and duration of benefit
- indications
- morbidities
3 months to benefit, up to 2yrs
bridge to transplant (now also destination)
stroke and arrhythmia
NYHA classes of heart failure
1 - No symptoms with ordinary physical activity
2 - No symptoms at rest, symptoms with ordinary levels of activity
3 - No symptoms at rest, symptoms with less than ordinary levels of activity
4 - Symptoms at rest
List some treatments for PAH
What are the key goals of therapy
- CCB’s
- PDE5 inhibitors
- Prostacyclin pathway agonist
- Endothelin antagonist
- Reduce pulm. vasocontriction and reduce proliferation of arteriolar smooth muscle cells
What specific considerations must be taken into account when managing palliative PAH care
- RV failure v. sensitive to hypotension, incr. RV afterload and reduction in RV contractility
- Use opioid, TCA, anti-psychotic with caution
- Gut edema is common (med absorption)
- Avoid drugs with sympathomimetic and pro-arrhythmic property
Aside from diuresis, how else does furosemide work
Immediate venodilatory effect
What are indications for deactivation of ICD?
inconsistent with goals
withdrawal of anti-arrhythmic
nearing EOL
No therapy has been proven to alter the course of which type of heart failure
HFpEF
How to HFrEF and HFpEF patients compare in terms of demographics and symptom burden?
HFpEF patients are older, higher BMI, have greater comorbidities, worse QOL, and worse prognosis
What is the prognosis of ALS?
Median age of diagnosis?
Negative prognostic factors?
prognosis 20-48 mo (<2yrs in most) - 10/10 rule (only 10% live >10yrs)
median age of dx. 55
negative prognostic factors:
- age
- bulbar onset
- frontotemporal dementia
- gen mutations (SOD1)
What specific aspect must be covered in ACP for ALS patients?
intubation preference
What are the benefits of NIV in ALS?
improved QOL and symptoms
slows rate of respiratory decline
survival
how does resp failure usually present in ALS?
nocturnal hypoventilation
Optimal timing for feeding tube insertion in ALS
When FVC still >50
2 treatments for spasticity in ALS
baclofen
tizanidine
other: gaba, bzp, dantrolene, botox
european society - keppra
management of secretions in ALS
- list the 2 types and respective approaches
anterior secretions - dry it up (glyco/anticholinergics, suction, sage tea, botox, RT)
posterior secretions - thin them out (fluid, guaifenesin, cough assist device)
other - respiratory exercise (chest wall physiotherapy, chest wall oscillator)
What type of cognitive impairment is common in ALS
How does it present?
FTD
apathy, disinhibition, decr. executive function, loss of insight and empathy
Management for pseudobulbar affect (pharmacological)
quinidine + dextromethorphan
some evidence for the use of SSRI, TCA
(inappropriate expression INCONGRUENT with true mood)
2 variants of ALS
Bulbar (20%) - starts with cranial nerve dysfunction
Spinal (80%) - starts with asymmetric tetraparesis
What respiratory intervention should be avoided in ALS
supplemental O2
medication approved for spasticity in MS that may be used “off label” in ALS
cannabis
How do ALS patients die
Mostly whilst sleeping
- resp failure
- autonomic failure
What are the 4 parkinson plus syndromes
- DLB
- CBD
- MSA
- PSP
Key distinguishing features of dementia with lewy bodies
- cognitive impairment (fluctuating)
- hallucinations (early in course of disease)
- neuroleptic sensitivity
- also more likely to have paradox. agitation with benzo.
Key distinguishing features of progressive supranuclear palsy
- supranuclear gaze palsy (esp. downward)
- early dysarthria
- early dysphagia
Key distinguishing features of multiple systems atrophy
Prominent and severe autonomic dysfunction (orthostatic hypotension, sexual dysfunction, neurogenic bladder)
Key distinguishing features of corticobasal degeneration
myoclonus
dystonias
apraxia (difficulty with skilled movements)
alien limb phenomena
How does the disease course and prognostic outlook of PD plus syndromes compare with PD
PD plus syndromes experience quicker progression, worse prognosis
Less responsive to dopamine
Key side-effects of dopamine / dopamine agonists
neuromotor - dyskinesias
neuromotor - motor fluctuations (wearing off, dose failures, sudden off time)
psychiatric - impulse control disorder, psychosis, delirium
autonomic - orthostatic hypotension
GI - nausea and vomiting
Failure to wean off levodopa will result in what?
parkinsonian crisis / parkinsonism-hyperpyrexia syndrome - a neuroleptic-like malignant syndrome (life threatening)
What factors predict 6-12mo mortality in PD?
- dysphagia
- weight loss
- reduction in medication required due to a neuropsychiatric effect
What are 3 common nighttime symptoms in PD and related disorders?
Sundowning / night-time hallucinations
Restless legs syndrome
REM sleep behaviour disorder
Medication approach to sundowning / hallucinations in parkinsons:
Withdraw culprits in following order:
- anticholinergics, dopamine agonists, COMT inhibitors, levodopa
may trial low dose seroquel
Treatments for REM sleep d/o:
Which PD like d/o is this common in?
1) Melatonin
2) Clonazepam
Cholinergics (cholinesterase inhibitors)
Dopamine agonists
More common in lewy body dementia
Treatments for RLS:
- rule out Fe deficiency (treat if ferritin low)
- dopamine
- benzodiazepines
- gabapentin
Management of dysautonomia (orthostatic hypotension) in PD
Hydrate and increase Na intake
Compression stockings
Reduce levodopa
Midodrine
Fludrocortisone
What is the role of NG feeding in patients with acute stroke?
What are the outcomes?
Short term measure to support nutritional intake (end points: regained ability to swallow or long term PEG)
Outcomes - improved recovery, does not reduce risk of aspiration
What are the benefits of feeding tubes in ALS
- nutrition
- medication administration
- prolong survival
Non motor symptoms of parkinsons
- delayed gastric emptying
- constipation
- urge incontinence
- erectile dysfunction
- depression
- anxiety
- cognitive impairment
- REM sleep behaviour d/o
Symptoms/signs of parkinsonism hyperpyrexia syndrome
Lab findings
Treatment
- rigidity (can lead to rhabdo)
- mental status change
- fever
- autonomic instability (labile BP, tachycardia)
labs: elevated CK, AKI, transaminitis, myoglobinuria, leukocytosis
treatment: supportive care, resume dopaminergics, benzodiazepines PRN
(classic NMS therapy bromocriptine, dantrolene, amantadine has poor evidence in this situation)
Symptoms/signs of serotonin syndrome
Treatment
- mental status change
- fever
- hyper-reflexia, clonus (including ocular), myoclonus, tremor
- n/v/diarrhea
- rigidity
- flushed skin and diaphoresis
Treatment: benzo, supportive care, cyproheptadine
How does MS present?
Highly variable
- gait abnormalities
- dysphagia and dysarthria
- ataxia
- psychological distress
What are the most common causes of pain in MS?
1) Central neuropathic
2) MSK = spasticity / spasm
Top agents for spasticity in MS:
baclofen
tizanidine
botox
cannabinoid
List prognostic scores for ischemic and hemorrhagic stroke
What are their pros and cons?
Ischemic - NIHSS scale (score >16 strong predictor of poor outcome at 3mo.)
Hemorrhagic - ICH score (validated for 30 day mortality)
Scores mortality and morbidity, but not QOL / recovery to patient centric goals
What is the greatest prognostic challenge in stroke?
Peak recovery doesn’t occur for months (majority at 3mo, but noted up to 12mo)
Early recovery at 1 week is a good sign
List some important / distinctive points to consider when engaging in ACP for stroke patients
- burden on family could be high (patient not able to communicate, sudden event etc.)
- seek to understand the minimum acceptable outcome from QOL perspective
- determine QOL facets and cherished critical abilities
- consider level of functional recovery, but also adaptation to new health status
- beware of the “disability paradox” - we underestimate disabled people’s QOL
what are common under-recognized symptoms in stroke
- pain (central and MSK) - 50%
- CPSP (central post stroke pain - esp. sensory cortex and thalamic lesions)
- Spasticity
- depression - 30%
- cognitive impairment - 80%
- fatigue
How do you distinguish PD from LBD
Motor symptoms are present for >12mo in PD before onset of dementia
2 scales that measure dementia severity
CDR - clinical dementia rating
FAST - functional assessment staging scale
List 1 dementia prognostic tool
ADEPT
Dementia prognosis for those diagnosed after age 65
4-8yrs
6 mo. mortality in advanced dementia patients with hip#
50%
Mortality of pneumonia in someone with advanced dementia
40-50%
What is the general approach to behavioural disturbance in patient with dementia
search for an unmet need
how does depression present in dementia?
atypical
refusal to eat
aggression
vocalization
evidence for use of SSRI’s in dementia
questionable
Cholinesterase inhibitors show benefit in which types of dementia
AZ
DLB
CCS criteria for CRT pacing in heart failure patients (in sinus)
NYHA Class II-IV
and
EF <35%
and
LBBB with QRS>130
What variables does the Seattle Heart Failure score evaluate?
clinical
pharmacological
laboratory
device
Which variable is consistently associated with poor prognosis in HF and how can this be further refined?
HF hospitalizations, refined further by age
When should discussions about ICD deactivation be held?
- prior to implantation
- annual HF reviews
- after major milestones
- nearing EOL
Review the sequential escalation of bronchodilator therapy in COPD
1) SABD PRN + LAMA or LABA
2) “ + LAMA/LABA
3) “ + LAMA/LABA/ICS
ICS risks
pneumonia
thrush
ICS indications
severe COPD
bronchial hyper-reactivity/asthma
recurrent exacerbations
What is the evidence for PDE-4 inhibitors in COPD
- reduce exacerbation rates
- bronchodilation
- anti-inflammatory
Role of prophylactic abx. in COPD
Macrolides (azithro)
Prevent exacerbations in non-smokers
What are the 4 aspects of pulmonary rehab?
Key outcomes?
exercise (supervised - intensive outpatient exercise program)
education
nutrition
psychosocial
outcomes: better symptom control, QOL, function, psychosocial health
Criteria for O2 in COPD
PO2 <55 or SaO2 <88%
OR
PO2 55-60 + PHTN, polycythemia, CHF
Pathophysiology of ILD is characterized by:
- cellular proliferation
- interstitial inflammation
- fibrosis
How are ILD’s classified?
- known associations (CTD, dusts, drugs etc.)
- granulomatous (sarcoid, hypersensitivity etc.)
- idiopathic (interstitial pneumonias)
Describe the various trajectories of ILD
reversible
- self-limited (RB-ILD) or with risk of progression NSIP, DIP, COP)
stable residual disease (NSIP)
progressive with potential for stabilization (NSIP)
progressive and irreversible (IPF)
What are the key treatments for IPF?
Indications?
What are the outcomes?
nindetanib and pirfenidone
mild-mod IPF - significant reducing dz. progression, impr. survival
severe IPF - less helpful
no improvement in function, symptoms, QOL
Management of IPF exacerbations
supportive care
steroid (weak evidence)
abx. coverage
IPF - what kind of hypoxia is common?
exertional (more so than COPD)
IPF
survival w/o transplant?
survival post-transplant?
2-4 year survival
post-transplant 4.5yrs
10 non-pharmacological management strategies for COPD and ILD
- smoking cessation
- exercise training
- pulmonary rehab
- breathing exercise (purse lip, diaphragmatic etc)
- gait aids
- chest wall vibration
- neuromuscular electrical muscle stimulation
- fan
- energy conservation / management
- positioning - recovery (upright, forward)
- oxygen
- non-invasive ventilation
review mmrc scale for dyspnea
1 - trouble with strenuous activity only
2 - SOB hurrying on level ground, walking up hill
3 - walks slower than average on level ground
4 - stops walking after few mins or 100 yards on level ground
5 - unable to leave house, breathless when dressing/undressing
COPD diagnosis PFT
FEV/FVC <0.7
COPD severity by FEV criteria
Mild - FEV1 >80
Mod - FEV1 50-80
Severe - FEV1 30-50
Very Severe - FEV<30
PFT findings for ILD
reduced FVC
reduced DLCO
3 qualities of breathlessness / dyspnea
air hunger
chest tightness
work / effort
prognostic factors in HIV
age
race
baseline cd4
gender - women, black men
transmission risk group - msm
CD4 count
PJP risk in HIV
TB and pneumococcal risk in HIV
<200
<400
What are the AIDS defining malignancies
kaposi sarcoma
non-hodgkins lymphoma
cervical ca
Why are HIV patients at higher risk of other chronic disease?
“premature aging” - immune dysfunction
potentially lifestyle related
Which diseases are more common in HIV patients?
all cancers
renal failure (HIV nephropathy)
liver failure (coinfection HCV, NAFLD)
hypogonadism
dementia
frailty
psychiatric
most common s/e of HAART
GI upset
What correlates best with prognosis in late stage HIV
age and function (not cd4 or viral count)
Does fentanyl dose need adjusting in renal failure
Yes - parent chemical can build up
Consider using 50-75% of the usual dose
Does methadone dose need adjusting in renal failure?
theoretically no (fecal excretion thought to compensate)
BUT, suggested caution and 50-75% usual as starting point
What are the top causes of fatigue in renal failure
anemia
uremia
dialysis (either too burdensome, or inadequate)
hyperparathyroidism
outline management of pruritis in renal failure
renal mgmt - hyperphos, hyperparathyroid can provoke
skin issues
pharmacotherapy -
gabapentin (most evidence),
anti-histamine,
opioid antagonist, SSRI (sertraline)
other - UVB light, tacrolimus
Which benzodiazepines are preferred in renal failure?
short acting (lorazepam, midazolam)
reduce doses
(midazolam recommendation of 25% if GFR<10)
No survival advantage of dialysis seen beyond what age?
80
survival advantage of dialysis is offset by what in the the elderly?
days in hospital
what has been postulated to slow rate of renal decline in late stages?
stopping ACEi/ARB
low protein diet (controversial)
What is the typical survival post discontinuation of dialysis?
days to short weeks (usually <2 weeks)
(depends on residual function)
which analgesic is contraindicated in dialysis?
baclofen
what is the median survival for compensated vs. decompensated cirrhosis?
12yrs
2yrs
2 prognostic tools for cirrhosis
child-pugh c
meld (meld-na. meld 3.0)
child pugh c criteria
bili
inr
albumin
encephalopathy
ascites
meld na criteria
bilirubin
inr
cr
na
need for dialysis
SAAG criteria for portal hypertension
> 11g.l
treatment for ascites in liver failure
na restriction <2g/d
diuretics - spironolactone:lasix 100:40 ratio
(resistance if not managed by 400/160 dose)
treatments for diuretic resistant ascites
paracentesis
indwelling catheter
TIPS
pharmacological treatments for pruritis in liver failure
cholestyramine
rifampin
opioid antagonist
SSRI (sertraline)
antihistamines - weak evidence
ursodiol - only recommended in PBC
what is the distinguishing factor between overt (Stage II-IV) hepatic encephalopathy and covert hepatic encephalopathy?
disorientation and asterixis
list 6 consequences of stigma in healthcare
decreased QOL
isolation
depression
distrust
reduced adherence to treatments
reduced access to care
max dose of acetaminophen in liver failure
2g
which opioid half life is greatly prolonged and should be used with caution in liver failure?
oxycodone
describe uremic caliphylaxis
calcification of small blood vessels -> ischemia
presents as painful skin / visceral lesions
most common in ESRD (dialysis > non-dialysis patients)
poorly understood d/o
RF: female, hypercoagulable states, DM, hyperPTH, hypoalbumin
clinical presentation of calciphylaxis
early - pain, purple discoloration (livedo reticularis)
later - SC nodules, plaques, ulcerations
greatest affected areas - fatty tissue
diagnosis of calciphylaxis
clinical
imaging shows calcification
biopsy deferred usually (poor healing)
Treatments for calciphylaxis
underlying conditions - DM and hyper PTH
supplements - stop Ca and vit D
diet - low phosphate
medical - calcimimetic, incr. dialysis frequency
symptoms - pain control and wound care
other - O2 therapy and hyperbaric O2
What is the typical prognosis of CJD?
Age of onset?
Incubation period?
<1 year from symptom onset
most die within 6mo.
onset age 60
incubation 10 years prior to symptom onset
What should trigger clinical consideration for CJD?
non-reversible dementia over days to months
often present with ataxia, emotional lability, concentration challenges, disordered sleep and hallucinations
