“One of these things is not like the others…”

“One of these things is
not like the others…”
Recognizing serious (pediatric)
situations in primary care.
Tim Zager. MD
Duluth Family Medicine Residency
Essentia Health Pediatrics
One of these things is not like the others,
One of these things just doesn’t belong…
Can you tell which thing is not like the others…
By the time I finish my song?
https://www.google.com/search?q=sesame+street+one+of+these+things&biw=1280&bih=579&tbm=isch&tbo=u&so
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Objectives:
• Discuss some scenarios ripe for clinical masquerade
• Discuss some cognitive processes we use in diagnosis
• Review some pitfalls in cognition and diagnosis
https://www.google.com/search?q=wolf+in+sheep's+clothing&biw=1280&bih=579&tbm=isch&tbo=u&
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1KagXM%3A
Primary Care Challenge: How does a physician
recognize the infrequent, serious situation in
the midst of a busy, routine office day?
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https://www.google.com/search?q=one+of+these+things+is+not+like+the+other&biw=1280&bih=579&tbm=isch&t
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4 year old female
CC: asthma exacerbation x 3 weeks
History
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3 weeks of SOB, wheezing
No fever, no runny nose
Cough is “tight”, ?productive
No other ill family members
No exposure to Resp Illness
Past hx: mild intermittent
asthma + allergic rhinitis
• Seen by MD one week ago
for wheezing- Rx steroids
• ? Better but still “not good”
Exam
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Pale skin color
RR= 42
Temp= 37.8
Oxygen Sat= 91%
Persistent cough observed
Mild intercostal retractions
Exp wheezes bilaterallyrated as “moderate”
• Symmetric breath sounds
Dx: asthma
• Continue nebulized albuterol + more prednisone
• Begin azithromycin x 5 days
One week later… she is worse
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Low grade fever by report (afebrile in office)
O2 Sats= 88%
RR= 50’s
Diminished breath sounds, exp wheezing bilateral
CXR- dense RUL infiltrate + “scattered fluffy infiltrates”
Consulted Pediatric Allergy- recommended bronch
Bronchoscopy Results
• Sunflower seed removed right
upper lobe bronchus
• Suction of purulent secretions
right > left
• Gram + cocci/ negative culture
• IV Rocephin
• Out of ICU 2 days
• D/C after 4 days on PO Cefdinir
https://www.med-ed.virginia.edu/courses/rad/cxr/anatomy5chest.html
Airway foreign body removal- bronchoscopy
http://www.cai.md.chula.ac.th/lesson/atlas/Q/ImgBig/IMG0012.jpg
Foreign body- inspiratory/ expiratory films
Inspiratory Film
Expiratory Film
Left mainstem f.b.- lack of filling on left (insp)- A
- some air trapping on left (exp)- B
http://www.pediatricsconsultant360.com/sites/default/files/images/Screen%20Shot%202013-0114%20at%202.15.22%20PM.png
16 year old female
CC: 2 weeks progressive HA- now fever and chills
History:
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Exam, Imaging, Lab:
• T= 39.4 P= 112 RR= 16
Nov 27- presents to ED
BP= 132/83 Ox= 98%
24 hours fever (103) & chills
• Ill appearing, no findings
Progressive frontal HA x 2
on exam (Rt pupil > Lt)
weeks and mild nausea
“Terrible” generalized aching • WBC= 3.6 (PMN’s up)
• Hgb= 12.1 Plt= 152
Neck stiffness, photphobia
• K= 3.3 Na= 136 glu= 123
h/o scoliosis, asthma
• LFT’s = normal
School + works @ Dunkin’
• UA= normal
Donuts
• CT head= normal
No neuro sx, ST, rash
ED Course:
• Family and patient declined LP
• IV Ketorolac and Ondansetron improved Sx
• Tick panel pending
• DX- HA, viral illness, fever
• Plan= symptomatic care, follow up with PCP
5 days later… f/u with PCP
Exam, Imaging, Lab:
History:
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Fever, chills continue
HA, body aches continue
Lightheaded, faint
? Slight cough
No hx of travel
No rash
No neuro sx
No joint swelling
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T= 38.4, P= 88, RR= 24
Ill appearing but nml exam
Tick panel from ED negative
CXR= negative
WBC= 3.9 Hgb= 11.6 plt= 77
Strep, EBV= negative
Vit D= 25 (low)
Repeat Lyme, Erlichia panel=
pending
Pediatrics in Review September 2015
Pediatrics in Review September 2015
Pediatrics in Review September 2015
Office follow up plan:
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Fever & Chills (6 days) & HA (3 weeks)= ? etiology
Leukopenia + thrombocytopenia = ? Anaplasmosis
Send blood culture
Start: Doxycyline 100 bid
Ibuprofen, hydration
• Phone follow up next day: “back to normal a few
hours after first dose of Doxycycline
• (Erlichia PCR, Lyme screen = negative)
http://www.bphc.org/whatwedo/infectious-diseases/Infectious-Diseases-A-toZ/Pages/Anaplasmosis.aspx
Anaplasmosis- Clinical Features
• Vector: Ixodes ticks; Bacteria family: Anaplasmataceae
• Incubation after tick bite: 1-2 weeks (no “herald” rash)
• Usual Sx: fever, headache, chills, malaise, myalgia, nausea
• Variable Sx: arthralgia, vomiting, cough, confusion, rash
• Severe Sx: acute respiratory distress syndrome,
encephalopathy, meningitis, DIC, renal failure
• Exam: no specific findings
• Lab: leukopenia, lymphopenia, thrombocytopenia,
hyponatremia, elevated LFT’s
Anaplasmosis
Diagnosis:
• Hx tick exposure (?) Tick season & Geography
• Fever + aches + leukopenia (lymphopenia) + low plts
• PCR for Anaplasma DNA (most sensitive week 1)
• Blood smear showing morulae in PMN’s (<20% subjects)
• Four-fold antibody rise (IFA IgG) over 2-4 weeks
• Default: clinical suspicion trumps negative lab studies
Treatment:
• Doxycycline 100 bid until 3 days afebrile (usually 7-10 days)
• Children < 8: Doxycycline (yes!) 4 mg/kg/day divided bid
• Rapid recovery confirms dx (often < 24 hrs)
2 year old male- comes to ED
CC= 4 days vomiting
Exam & Lab
History:
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Vomiting q 2 hrs x 4 days
T max= 38.3
Watery diarrhea (frequent)
No blood with stool
“Stomach flu” at day care
PO intake: water + juice
Sleepy
Uncertain last urine output
Past Hx- VSD
No meds (i.e., antibiotics)
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T= 37.4 P= 156 RR= 25
Wt is 5% down from 2 mos ago
Pale, sleepy, arouses
Eyes sunken (?)
Reduced saliva in mouth
Otherwise negative exam
Na= 131 K= 3.4 Cl= 98
BUN= 12 Glucose= 53
WBC=5.6 Hgb=13.5 Plt= 180
ED Assessment & Plan
• Viral Enteritis with
moderate dehydration
• Parents instructed on fluid
& dietary management
• Discharged to home
• Pedialyte ad lib
• Follow up with PCP
Follow up next day- PCP
History:
• Vomited x 3 in 24 hrs
• Doesn’t like Pedialyte
• Very sleepy- not drinking
• Diarrhea has lessened
• Last urine- 6 hrs prior
• (4 y-o sib with vomiting)
Exam & Lab:
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T= 37.8 P= 161 RR= 24
Wt= 1 lb less
Pale, sleepy, slow to arouse
Eyes sunken (?)
Reduced saliva in mouth
Otherwise negative exam
Na= 130 K= 3.6 Cl= 99
BUN= 14 Glucose= 46
WBC=9.8 Hgb=14.9 Plt= 201
Follow up next day- PCP
Exam & Lab:
History:
• Vomited x 3 in 24 hrs
• Doesn’t like Pedialyte
• Very sleepy- not drinking
• Diarrhea has lessened
• Last urine- 6 hrs prior
• (4 y-o sib with vomiting)
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T= 37.8 P= 161 RR= 24
Wt= 1 lb less
Pale, sleepy, slow to arouse
Eyes sunken (?)
Reduced saliva in mouth
Otherwise negative exam
Na= 130 K= 3.6 Cl= 99
BUN= 14 Glucose= 46
WBC=9.8 Hgb=14.9 Plt= 201
Glucose Homeostasis
• Serum glucose levels during fasting are maintained by
mobilizing glycogen stores and gluconeogenesis.
• Brain energy metabolism depends on glucose and ketone
bodies- free fatty acids available to other tissues during
fasting cannot cross blood brain barrier
• Younger children have higher relative brain mass
• Glucose production and utilization in young children is 3 fold
higher than adults but declines 30% after 30 hrs of fasting
• Complex counterregulatory hormone system and substrate
shift preserves brain metabolism
• Mild hypoglycemia symptoms= autonomic response:
sweating, anxiety, nausea, tachycardia
• More severe hypoglycemia= lethargy, confusion
Hypoglycemic Counterregulation
http://emedicine.medscape.com/article/921936-overview
Etiology of Pediatric Hypoglycemia
• Inborn Errors of Metabolism: enzyme defects in fat and
amino acid metabolism
• Hyperinsulinism: endogenous or exogenous
• Medical conditions increasing glucose use: sepsis, shock
• Hepatic disease: Reye Syndrome, hepatitis
• Other: Ketotic Hypoglycemia of childhood, ingestions
(sulfonylureas, ethanol, salicylates)
Hypoglycemia in Infants & Children. Up to Date
Etiology of Pediatric Hypoglycemia
• Inborn Errors of Metabolism: enzyme defects in fat and
amino acid metabolism
• Hyperinsulinism: endogenous or exogenous
• Medical conditions increasing glucose use: sepsis, shock
• Hepatic disease: Reye Syndrome, hepatitis
• Other: Ketotic Hypoglycemia of childhood, ingestions
(sulfonylureas, ethanol, salicylates)
Hypoglycemia in Infants & Children. Up to Date
Ketotic Hypoglycemia of Childhood
• Most often seen between ages 15 mos and 5 years
• Remits by age 8
• Factor: increased brain share of total body energy needsglucose dependent
• Factor: lower relative hepatic glycogen stores
• Factor: glucose levels cannot be sustained until shift to
ketone metabolism and gluconeogenesis
• Remits at age when brain to body ratio decreases to near
adult level
• Diagnosis depends on eliminating other causes of
hypoglycemia
Hypoglycemia in Infants & Children. Up to Date
Data from Haymond, MW, Sunehag, A, Endocrinol Metab Clin North Am 1999; 28:663
Establishing Dx of Ketotic Hypoglycemia
• Appropriately decreased insulin levels (≤2 microM/mL)
• Decreased alanine on quantitative plasma amino acids
• Elevated GH, cortisol, FFA, and ketones
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Negative urine reducing substances.
Normal lactate and pyruvate
Normal thyroxine (excludes hypopituitarism)
Normal free/ total carnitine + acylcarnitines
• No response to administration of glucagon at time of
hypoglycemia, but normal response after an overnight fast
Hypoglycemia in Infants & Children. Up to Date
Treatment & Course:
• Provisional dx: viral enteritis, dehydration, ketotic
hypoglycemia
• IV D5/ NS @ 20 ml/ kg bolus over 20 minutes was
followed by same fluid at calculated maintenance rate +
deficit – WOKE UP!
• Fingerstick glucose normalized at end of boluseliminating need to piggyback D10W
• Child tolerated PO intake and diet rapidly and was
discharged next day
• (Standard Tx = oral therapy- 0.3 gm/kg of rapidly
absorbed carb- i.e., 11 g CHO in 4 oz of apple juice)
• (IV Tx = 0.25 gm/kg dextrose= 2.5 ml/kg of D10)
http://medhacker.com/2011/07/16/virtual-patient-tests-before-a-real-patient-experience/
How do physicians keep
diagnostic skills tuned?
Traditional CME focus: sit
back and absorb info on
declared topic
Real life in clinic:
• Variable groups of clues
• Full picture- in flux
• Free association thinking
• Context: real people with
potentially serious disease
How do we arrive at a diagnosis?
Recognize pattern?
Multiple tries??
IOM Report- September 2015
Diagnostic Errors:
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“Blind spot in the delivery of Quality Health Care”
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(1) “Failure to establish an accurate and timely
explanation of the patient’s health problem(s)”
(2) “Failure to communicate that explanation to the
patient.”
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Contribute to 10% of patient deaths
Responsible for up to 17% hospital adverse events
Leading cause for paid malpractice claims
• “Improving the Diagnostic Process is
a moral, professional, and public
health imperative.”
Graber, et al. Interventions to Reduce Diagnostic Errors in Ambulatory Care
AHRQ presentation 2010
Heuristic… or Bias??
• Anchoring and adjustment – Describes the common
human tendency to rely too heavily on the first piece of
information offered (the "anchor") when making decisions
• Availability heuristic – A mental shortcut that occurs when
people make judgments about the probability of events by
the ease with which examples come to mind.
• Representativeness heuristic –judging a situation based
on how similar the prospects are to the prototypes the
person holds in his or her mind.
• Familiarity heuristic assumes that the circumstances
underlying the past behavior still hold true for the present
situation
https://en.wikipedia.org/wiki/Heuristic
Systems Factors
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Communication and coordination of care issues (transitions)
Teamwork/Supervision
Technology/equipment related issues
Organizational features:
– Safety culture
– Policy, processes and procedure related issues
– Leadership, management, or personnel problems
– Inadequate resources or available expertise
– Training issues
Diagnostic Error- Graber
Diagnosis in the Clinic Setting:
Where can gaps arise?
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Self
Cognitive pace of the office
schedule (“thinking fast and
slow)
Morning vs afternoon
Tuned Heuristics (protocols,
patterns)
Biases
Info gathering/ sorting misses
(WYSIATI)
(Medical Knowledge Base}
Extrinsic Factors
• Disruptions to work flow in
the office
• EMR complexity
• Patient variablesrecollection + biology
• Office schedule entropy
• System Factors
Diagnosis: Intuitive vs. Analytical?
• Pt schedule: “easy” vs. “hard”
• “Fast thinking”: simple AOM
• “Slow thinking”: 10 weeks of
abdominal pain + weight loss
• Brain prefers simple solutions and
“connecting the dots”- even when
there are info gaps
• Primary Care- fast pace
• How do we recognize the need to
shift gears- what about fatigue?
• Slow thinking is energy intensive
http://www.nytimes.com/2011/11/27/books/review/thinkingfast-and-slow-by-daniel-kahneman-book-review.html?_r=0
Modeled after Kahneman. Thinking, Fast and Slow. 2011
Heuristics & Biases
• Heuristics are simple, efficient rules which people often use
to form judgments and make decisions. They are mental
shortcuts that usually involve focusing on one aspect of a
complex problem and ignoring others. These rules work
well under most circumstances, but they can lead to
systematic deviations from logic, probability, or rational
choice theory…
• resulting errors are called "cognitive biases". These have
been shown to affect people's choices in situations like
valuing a house, deciding the outcome of a legal case, or
making an investment decision. Heuristics usually govern
automatic, intuitive judgments but can also be used as
deliberate mental strategies when working from limited
information.
https://en.wikipedia.org/wiki/Heuristics_in_judgment_and_decision-making
WYSIATI
“What you see is all there is”
• How does one evaluate quality
and quantity of data?
• When is there enough data to
establish a diagnosis?
• How does one avoid “jumping to
conclusions”, yet not delay Tx?
• How does one find diagnostic
“coherence” amid evolving and
incomplete data?
• Are we curious enough?
• Do we monitor and test our DX ??
Modeled after Kahneman. Thinking, Fast and Slow
… and in the end… do we…?
Go down the rabbit hole?
Connect the dots?
“We look for medicine to be an orderly field of
knowledge and procedure. But it is not. It is an
imperfect science, an enterprise of constantly
changing knowledge, uncertain information, fallible
individuals, and at the same time lives on the line….
“There is science in what we do, yes, but also habit,
intuition, and sometimes plain old guessing…
The gap between what we know and what we aim
for persists. And this gap complicates everything we
do.”
- Atul Gawande, MD
Gawande: Complications: A Surgeon’s Notes on an Imperfect Science
Discussion: