Understanding the “Rashomon Effect”

Posted on by

An interesting commentary (GM Ronen, DL Streiner. J Pediatr 2016; 179: 17-18) discusses the “Rashomon” effect and how this can relate to studies which show differences between children with health problems and their parents’ perception of how they are doing.

“In this famous Japanese tale, set in the 12th century, a notorious bandit attacked a samurai and his wife in the woods.”  Afterwards, all of the accounts of the incident by the participants were widely discrepant. “When the tale is over, the reader realizes that even though none of the version is a truthful objective account, all must be true at least from the character’s own unique perspective.”

In medical studies with children and their parents, different versions of the truth can be due to many factors:

  • Depression distortion hypothesis –raters with depression tend to score poorer on numerous health variables
  • Disability paradox –“some persons with impairments, against all odds, are satisfied with their life and rate their health similar to typical children”
  • Parents may also be affected by the emotional impact of their child’s health problem even when the problem is well-controlled

My take: This short commentary has a lot to say about understanding why a person with a medical problem may rate their health much better or much worse than an outside observer would expect.

Penobscot Narrows Bridge, Maine

Penobscot Narrows Bridge, Maine

 

Blind Men and The Elephant: Lasting Consequences of Enteric Infections

Posted on by

Recently, Ben Gold handed me a supplement which alluded to the case of “the blind men and the elephant.”  So, of course, I wanted to know more about this.

According to Wikipedia:

In various versions of the tale, a group of blind men (or men in the dark) touch an elephant to learn what it is like. Each one feels a different part, but only one part, such as the side or the tusk. They then compare notes and learn that they are in complete disagreement. The stories differ primarily in how the elephant’s body parts are described, how violent the conflict becomes and how (or if) the conflict among the men and their perspectives is resolved.  In some versions, they stop talking, start listening and collaborate to “see” the full elephant. When a sighted man walks by and sees the entire elephant all at once, the blind men also learn they are all blind. While one’s subjective experience is true, it may not be the totality of truth. If the sighted man were deaf, he would not hear the elephant bellow.

It has been used to illustrate a range of truths and fallacies; broadly, the parable implies that one’s subjective experience can be true, but that such experience is inherently limited by its failure to account for other truths or a totality of truth. At various times the parable has provided insight into the relativism, opaqueness or inexpressible nature of truth, the behavior of experts in fields where there is a deficit or inaccessibility of information, the need for communication, and respect for different perspectives.

The rest of the supplement regarding chronic health consequences following acute enteric infections was less interesting but probably more important than learning a new anecdote.

The introduction notes that nearly 600,000 children under 5 years die from dehydrating diarrhea each year.  Many more suffer from consequences of disease-associated malnutrition with both physical and cognitive deficits.

Articles in supplement:

  • Am J Gastroenterol Suppl 2016; 3: 4-11. –details diarrhea-associated years lived with disability 51 per 100,000 in developed regions compared with 685 in developing regions.
  • Am J Gastroenterol Suppl 2016; 3: 12-23. –details the likelihood of consequences following enteric infections, including functional GI disorders, inflammatory bowel disease, celiac disease (data limited), Guillain-Barré syndrome, hemolytic uremic syndrome, chronic fatigue, and neurologic sequelae.
  • Other articles in the supplement describe changes in the microbiome, the micorbiome-gut-brain axis, and the relationship between autoimmunity and irritable bowel.

 

Nutrition Guidelines for Cystic Fibrosis

Posted on by

Wilschanski et al (JPGN 2016; 63: 671-5) provide a summary (“highlights”) of a full report (Turck D et al. Clin Nutr 2016; 35: 557-77) on nutritional recommendations for infant and children with cystic fibrosis.

What’s in here:

Table 1: criteria for adequate nutritional status including

  • Age <2 yrs: 50% for weight & height compared to healthy-age peers
  • Age 2-18 yrs: 50% BMI compared to healthy peers

Table 2: nutritional assessment and followup

  • Assess elastase-1 annually if pancreatic sufficient
  • Assess pancreatic enzyme supplementation
  • Annual blood tests: CBC/d, iron status, fat-soluble vitamins, LFTs.  Possibly: fatty acids
  • If older than 10 years, annual glucose tolerance
  • Dietary review every 3 months
  • Bone density assessment between 8-10 yrs and then every 1-5 yrs

Table 3: Energy requirements

  • Anticipate need for 110-200% compared with healthy peers

Table 4: Pancreatic enzyme replacement therapy (PERT)

  • 0-1 yr: 2000-4000 units lipase/120 mL of formula/breast milk & 2000 units lipase/gram of dietary fat
  • 1-4 yrs: 2000-4000 units of lipase/gram of dietary fat (max 10,000 units lipase/kg/day)
  • >4 yrs: starting dose; 500 units lipase/kg/meal -titrate up to 1000-2500 units lipase/kg/meal (max 10,000 units lipase/kg/day)

Table 5: Fat-soluble vitamin/vitamin guidelines

Table 6: Sodium supplementation

  • 0-6 months: 1-2 mmol/kg/day –give salt in small portions throughout the day, “diluted in water or fruit juice”.  In some infants, up to 4 mmol/kg/day if increased losses (eg. due to heat, gastrointestinal losses)
  • Older children: anticipate need for additional salty foods or use sodium chloride capsules, especially when excessive sweating (eg. fever, sports, hot weather)

Related blog posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

Maine Coast, near Acadia

Maine Coast, near Acadia

 

IBD Updates -January 2017

Posted on by

L Beswick et al. Inflamm Bowel Dis 2016; 22: 2966-2976. The authors provide an algorithm for diagnosis and management of CMV in the setting of inflammatory bowel disease which primarily in the setting of steroid-refractory colitis.  Despite conferring a worse prognosis, the authors note that in most cases the virus is nonpathogenic and thus antiviral is usually ineffective.  Figure 1 outlines their algorithm, in those with high density inclusions on tissue (≥5 per biopsy) and/or high blood CMV PCR, the authors recommend treatment (including ganciclovir).  The details of this figure are too complex to easily summarize and if faced with this clinical scenario, I recommend reviewing source article.

KA Dunn et al.  Inflamm Bowel Dis 2016; 22: 2853-62. Fecal samples from 10 patients & 5 controls (age 10-16) showed that microbial diversity was lower in Crohn’s and lowest in patients who did not achieve SR.

EL Barnes, R Burakoff. Inflamm Bowel Dis 2016; 22: 2956-65. New biomarkers for diagnosing inflammatory bowel disease: biomarker signatures, gene expression analysis, protein profiling and microRNA

Does a Healthy Lifestyle Result in Better Outcomes?

Posted on by

It’s easy to become discouraged that sensible actions may not be effective due to general pessimism and sometimes conflicting medical reports.  On the positive side of the ledger, a recent study (AV Khera et al. NEJM 2016; 375: 2349-58) provides compelling data that a combination of healthy lifestyle changes make a BIG difference.

The study focused on 4 healthy lifestyle factors: no smoking, no obesity, regular physical activity, and a healthy diet.  The study examined three large prospective cohorts with a total of more than 55,000 patients.

Key finding:

  • Among participants with high genetic risk, a favorable lifestyle was associated with a 46% lower relative risk of coronary events compared to those with an unfavorable lifestyle over the 10-year study period.

In the same issue, a review of the human intestinal microbiome (pages 2369-79) notes that “dietary intake appears to be a major short-term and long-term regulator of the structure and function of gut microbiota.  Still, only a relatively small number of randomized, clinically controlled dietary interventions targeting the gut microbiota have been reported in humans and these show that energy restriction and diets rich in fiber and vegetables are associated with gut microbial changes that, in turn, are associated with a health benefit.”

My take: To enhance your odds of good health, avoid smoking, stay fit, and eat your fruits/veggies.

coldcomment

 

Care Coordination and Magical Thinking

Posted on by

One myth that has been promulgated has been that care coordination will lead to cost containment. A commentary on this topic (JM McWilliams. NEJM 2016; 375: 2218-20) explains the fallacy of this thinking.  While care coordination can improve medical care, “conflating cost containment and care coordination poses many potential dangers.”  Good care is worthy goal even in the absence of saving money.

Key points:

  • Care coordination often improves outcomes but typically involve interventions to correct underuse of care
  • For every costly complicated prevent, “a care coordination program must manage care for multiple patients…[which] is costly.”
  • Early evaluation of care coordination in accountable care organizations (ACOs) have shown the efforts “have meaningfully improved patient experiences but not rates of hospitalizations for ambulatory care-sensitive conditions.” There has not been evidence of fewer readmissions or fewer preventable hospitalizations with care coordination.
  • Other strategies to reduce cost are now being targeted, like steering patients to lower-priced providers

My take (from author): “We should coordinate care not to save money but because coordinated care is better care.”

Yosemite

Yellowstone (I took this picture!)

Primary Sclerosing Cholangitis (PSC) –Natural History Study

Posted on by

Last week, I posted an blog referencing new guidelines for peanut introduction.  A more detailed explanation of these guidelines: New Guidelines: Early Introduction of Peanut to Prevent Peanut Allergy from David Stukus (Thanks to Kipp Ellsworth for sharing this information)

_________________

A recent study (PL Valentino et al. JPGN 2016; 63: 603-09) follows “the largest reported pediatric PSC cohort” to determine the natural history.

Study characteristics:

This retrospective study followed 120 children (1-21 yrs) with a median age of 14 years.  27% had autoimmune sclerosing cholangitis (ASC), 63% had PSC; 24% (n=29) of entire cohort had exclusive small duct PSC. Median followup was 3.7 years.

Key findings:

  • 81% of PSC patients had inflammatory bowel disease; most (72/97) had ulcerative/indeterminant coliits. 40/72 had pancolitis.
  • PSC-IBD was more common than ASC-IBD (85% vs 68%).
  • 10-year transplant-free survival in this cohort was 89%; there were 6 liver transplants.
  • The rate of cirrhosis was lower in the group who had IBD preceding PSC (15% vs 31%,P=0.05).
  • PSC is clinically silent in the majority of patients; 64% presented with abnormal chemistries and no other symptoms.
  • ERCP therapeutic intervention was low, 3% for stenting and 7% for balloon dilatation.

The authors speculate that one reason for milder PSC-IBD disease could relate to the fact that IBD patients undergo frequent chemistries.  In those without IBD, subacute PSC could be present for a much longer period before detection.  The authors note that PSC in children presents as a milder disease with only 10% having cirrhossi compared with 30% in studies with adult patients.

My take: We have a lot to learn about PSC including which patients are likely to develop clinically significant liver disease and whether most patients benefit from treatment.

Related blog post: Should we care about subclinical PSC? (This post has links to others related to PSC)

Thunder Hole, Acadia Nat'l Park

Thunder Hole, Acadia Nat’l Park

Can the FDA stop snake oil salesmen?

Posted on by

A recent commentary (C Robertson, AS Kesselheim. NEJM 2016; 375: 2313-5) examines how the issue of “free speech” may undermine the FDA’s ability to regulate ineffective or dangerous medications. This has been discussed in a previous blog:

Can the FDA prohibit free speech?

In a previous case, Caronia had promoted sodium oxybate for a wide range of nonapproved uses; some of these uses “were likely to cause patients substantial harm.”

Yet, the 2nd Circuit court reversed a lower court in ruling that Caronia’s sale pitches were protected free speech.  This decision “subverted decades of presumptions about how the government could oversee the behavior of the pharmaceutical and medical device industries.”

The authors hope that an upcoming case to the 1st circuit will uphold the FDAs ability to assure that patients are protected and that the use of drugs is driven by science and not marketing.  If manufacturers are allowed to promote a wide range of uses for drugs with narrow indications, there will not be an incentive to determine if these medications are safe and effective.

My take: If the principles of free speech are extended to promoting bogus claims about pharmaceuticals and medical devices, this would be a huge blow to medical science.

Acadia Natl Park

Acadia Natl Park

Better Hydration May Lead to Better Outcomes For Hemolytic Uremic Syndrome due to E coli

Posted on by

According to a recent meta-analysis of 8 studies (1511 children), better hydration may reduce the risk of bad outcomes for hemolytic uremic syndrome (HUS): From JAMA Pediatrics: Shiga Toxin-Producing E coli, Hydration Status and Outcomes of Patients Infected With Shiga Toxin–Producing Escherichia coliA Systematic Review and Meta-analysis

Reference: JAMA Pediatr. Published online November 28, 2016. doi:10.1001/jamapediatrics.2016.2952

Results: A hematocrit value greater than 23% as a measure of hydration status at presentation with HUS was associated with the development of oligoanuric HUS (OR, 2.38 [95% CI, 1.30-4.35]; I2 = 2%), renal replacement therapy (OR, 1.90 [95% CI, 1.25-2.90]; I2 = 17%), and death (OR, 5.13 [95% CI, 1.50-17.57]; I2 = 55%). Compared with putatively hydrated patients, clinically dehydrated patients had an OR of death of 3.71 (95% CI, 1.25-11.03; I2 = 0%). Intravenous fluid administration up to the day of HUS diagnosis was associated with a decreased risk of renal replacement therapy (OR, 0.26 [95% CI, 0.11-0.60]).

Conclusion from abstract: Two predictors of poor outcomes for STEC-infected children were identified: (1) the lack of intravenous fluid administration prior to establishment of HUS and (2) a higher hematocrit value at presentation. These findings point to an association between dehydration and adverse outcomes for children with HUS.

This study is in agreement with a prior study referenced on this blog: Changing Paradigm in Hemolytic Uremic Syndrome

acadia5