Nutrition Imbalance for Ventilated Children

Posted on by

A recent study documents a high rate of nutritional problems among a prospective cohort of 20 children on home ventilators and documents a metabolic assessment aimed at improving these problems (Martinez EE, et al. J Pediatr 2015; 166: 350-7, ed 228-29).

In these children the authors did careful nutritional assessment with anthropometry, bioelectrical impedance analysis (BIA), actual energy intake (AEI), and indirect calorimetry in the subject’s home. Indirect calorimetry was used to calculate a measured energy expenditure (MEE).

Indirect calorimetry allows measurement of energy expenditure: (From NASPGHAN Foundation N2U Course 2012, Praveen Goday: “Energy and Protein Metabolism”)

  • “When carbohydrate, protein, and fat are oxidized, oxygen is consumed and carbon dioxide is produced.”
  • “If oxygen consumption and carbon dioxide production can be measured, the energy released in the course of the utilization of these gases (or the energy expenditure can be determined.”
  • “The techniques is referred to as indirect, because gas exchange does not actually measure heat production.”

Key findings:

  • 13 were either underfed (AEI:MEE <90%) or overfed (AEI:MEE >110%)
  • 11 of 19 had suboptimal protein intake
  • 15 subjects were hypo or hypermetabolic

The authors conclude that a “majority of children on home ventilation are characterized by malnutrition, altered metabolic status, and suboptimal macronutrient intake” (especially low protein intake).  The discussion lists many of the study limitations: small number, discrepancies between some of their measuring tools, lack of long-term followup, lack of widespread availability of mobile indirect calorimetry, diverse comorbidities, and reliance of 3-day food records. In addition, the indirect calorimetry must be properly calibrated, performed when patient at baseline state, and feedings held (if on bolus feeds).

Although I think this study makes some important points, I think the ‘high-tech’ approach is overemphasized.  It would be interesting to see how (if at all) these interventions would improve a child who is followed closely by a nutritionist and a GI physician.  While precise measurement of resting energy expenditure, when performed properly, is informative, I think this information is much less helpful than serial basic measurements.

At the same time, there are many limitations on optimal nutrition in these children.  The mobility problems of many kids on home ventilators can make gaining weight problematic for care providers.  It is not practical for all caregivers to manage a 60 kg adolescent.

Recent advice from N2U () regarding children who were tube-fed/wheelchair-bound:

  • In children older than 10 years, if they are receiving 6 cans/day of commercial formula product, they are likely receiving adequate nutrients.
  • In children younger than 10 years, if if they are receiving 4 cans/day of commercial formula product, they are likely receiving adequate nutrients.
  • The newer reduced calorie formulas make it easier to provide adequate nutrients without excessive calories
  • Avoid obesity in these children.  Losing weight can be very difficult in this population.

Bottomline: Children on ventilators often are too heavy or too thin and need to be followed closely.  Whether indirect calorimetry is useful in this regard is not clear to me.

Briefly noted: A high nutrient diet appears to help treat fatigue (Nutrients 2015, 7(3), 1965-1977).  From abstract (thanks to Kipp Ellsworth): A group of 98 children (2–18 years old) with unexplained symptoms of fatigue was examined. Children in the intervention group were asked to follow the diet for three months, whereas the control-group followed their normal diet.  The dietary modifications consisted of green vegetables, beef, whole milk and full-fat butter.

From NPR: Empathy Cards “Please Let Me Be the First to Punch the Next Person Who Says Everything Happens for a Reason”

What’s More Important: Improving Mortality Rate or Survival Rate? (Hint: It is not a trick question)

Posted on by

A recent commentary by Aaron E. Carroll in the NY Times explains “Why Survival Rate is Not the Best Way to Judge Cancer Spending.”  If you don’t understand the difference between survival rate and mortality rate, then it is worth a quick read; it explains the concept of “lead-time bias” and “overdiagnosis bias.” Here’s an excerpt:

Mortality rates are determined by taking the number of people who die of a certain cause in a year and dividing it by the total number of people in a population…

Survival rates describe the number of people who live a certain length of time after a diagnosis…

Let’s consider a hypothetical illness, thumb cancer. We have no method to detect the disease other than feeling a lump. From that moment, everyone lives about four years with our best therapy. Therefore, the five-year survival rate for thumb cancer is effectively zero, because within five years of detection, everyone dies.

Now, let’s assume that we develop a new scanner that can detect thumb cancer five years earlier. We prevent no more deaths, mind you, because our therapy hasn’t improved. Everyone now dies nine years after detection instead of four. The five-year survival rate is now 100 percent.

 

Ordering Tests and Good Health

Posted on by

A provocative article from the NY Times discusses the fact that more testing does not always lead to good health outcomes.  Here’s an excerpt:

A cadre of test skeptics at Dartmouth Medical School specialize in critically examining our test-based approach to well adult care. If you are confused about mammography, colonoscopy or the PSA test for prostate cancer, these folks deserve much of the blame: They have repeatedly demonstrated that these tests and many others do not necessarily ameliorate a healthy person’s health, any more than standardized testing in grade school improves a child’s intellect…

… systems that rate doctors by how well their patients’ blood pressure is managed are likely to invite trouble. Doctors rewarded for treating aggressively are likely to keep doing so even when the benefits begin to morph into harm…One study found that nursing home residents taking two or more effective blood pressure drugs did remarkably badly, with death rates more than twice that of their peers. In another, dementia patients taking blood pressure medication with optimal results nonetheless deteriorated mentally considerably faster.

Yet no quality control system that I know of gives a doctor an approving pat on the head for taking a fragile older patient off meds. Not yet, at least. Someday, perhaps, not ordering and not prescribing will mark quality care as surely as ordering and prescribing do today.

Related blog posts:

Keeping Up with Clostridium Difficile

Posted on by

It is difficult to keep up with all of the relevant publications regarding Clostridium difficile–there are so many.  This likely reflects its emergence as a frequent and important pathogen.

Recent references:

  1. Sandberg KC et al. “Disproportionate Rise in Clostridium difficile Associated Hospitalizations Among US Youth with Inflammatory Bowel Disease, 19978-2011.” JPGN 2015; 60: 486-92 (editorial 421-22).
  2. Leffler DA, Lamont JT. NEJM 2015; 372: 1539-48.

In the first study, the researchers note that there has been a 5-fold increase in inflammatory bowel disease (IBD) hospitalizations with concomitant Clostridium difficile infection (CDI).  Whereas, the hospitalization without CDI increased 2-fold.  Associated with this 5-fold increase in hospitalizations, there were increased costs and longer length of stays.  Interestingly, IBD patients with CDI had a  lower likelihood (OR 0.31) of colectomy in this study. This epidemiology yields more questions than answers.  Certainly, a significant fraction of this increase is due to the use of more sensitive PCR-based assay. In addition, many of these patients may not be symptomatic due to CDI; it can be difficult to determine if IBD symptoms are due to IBD or due to CDI. Even treatment with antibiotics like vancomycin does not fully differentiate as the response could be nonspecific.

In the second review, severe useful points were made.

Risk factors:

  • Antibiotics –this remains most important risk factor
  • Older age (especially if >65 years)
  • Possible acid suppression -not confirmed in some studies when adjusting for coexisting conditions
  • Inflammatory bowel disease
  • Immunosuppression
  • Chronic kidney disease

Diagnosis:

  • Use of DNA assays has allowed for detection of “low levels of toxigenic organisms of uncertain clinical significance.”  Thus, these assays may detect clinically-insignificant infections.
  • Endoscopy is rarely needed, but sometimes helpful in ovelapping conditions like coexistent CDI from IBD
  • Negative PCR assay has a negative predictive value of “more than 95% in average-risk groups.”
  • Testing and treating persons with solid stools is not recommended

Prevention:

  • Probiotics “have an uncertain effect on the prevention of C difficile infection, and their routine use for the prevention or treatment of active infection is not recommended.”  The authors note that initial favorable studies of antibiotic-associated diarrhea were underpowered and that more recent studies have shown mixed results.  In studies of patients with unusually high rates of CDI, probiotics were shown to confer benefit.

Treatment:

  • Metronidazole and vancomycin remain 1st line treatments.
  • Fidaxomicin use has been limited due to expense, but has been shown to reduce recurrence of CDI in those who do not have the b1/NAP1/027 strain.
  • Alternative antimicrobials, including rifaximin, nitazoxanide and others, are “not recommended except in cases of unacceptable adverse effects.”
  • For recurrent infection, 1st line approach is retreatment with either metronidazole & vancomycin. Second recurrences are often treated with fidaxomicin or tapered vancomycin course.
  • Fecal microbial transplantation –noted to be highly effective and safe as salvage therapy. The precise components that are important are uncertain; however, “the phyla Bactteroidetes and Firmicutes are thought to comprise critical components.”  “More work is neede to understand the possible role for fecal microbial transplantation for primary CDI”

Bottomline: CDI remains an important pathogen and significantly complicates the management of IBD.

Related blog posts:

Another Big Study: No Link between MMR vaccine and Autism (Plus one)

Posted on by

In a study with 95,727 children, there was no link between receipt of the MMR (measles, mumps, rubella) vaccine and autism, even in children at high risk (eg. sibling with autism).

Here’s a summary from USA Today: No link between MMR and Autism

Original JAMA article (free & entire article)

Related blog: “Too many vaccines and autism” is debunked | gutsandgrowth

An unrelated commentary, “Social Distancing and the Unvaccinated,” (NEJM 2015; 372: 1481-83) notes that a recent ruling (Phillips v City of New York) upholds the state’s authority to bar unvaccinated children from school during outbreaks.  This practice is referred to as social distancing to lessen likelihood of further transmission. This “reiterated the Supreme Court decision in the 1905 case Jacobson v. Massachusetts, which clearly found vaccine mandates constitutional.”

GI Care For Kids: Our group has been very supportive of the Crohn’s and Colitis Foundation of America (CCFA) and especially active in staffing the yearly Camp Oasis for more than 20 years.  Throughout the year, there are a number of other events to support CCFA.  This past weekend many of us participated in “Taking Steps.”  Here are a few pictures:

Super Poopers: Ben Gold, Larry Saripkin, Dinesh Patel, Seth Marcus, and Jay Hochman

Super Poopers: Ben Gold, Larry Saripkin, Dinesh Patel, Seth Marcus, and Jay Hochman

Dr. Spandorfer’s team raised a great deal of money (50K) and he/his family were featured in the Atlanta Journal Constitution (Local Family Takes Big Steps to Raise Awareness).  His son, Jack, spoke at the event, and was honored as this year’s hero.  They also had pretty clever T-shirts

With 'Pip" Spandorfer (whose team raised $50,000)

With ‘Pip” Spandorfer (whose team raised $50,000)

Dinesh Patel and Kimberly Sheats

Dinesh Patel and Kimberly Sheats

 

 

 

Briefly Noted: E-Cigarette Use Increasing Rapidly in Kids & Gilead Profits

Posted on by

From NPR (accessed 4/24/15): The statistical findings, published in this week’s issue of Morbidity and Mortality Weekly Report, come from the CDC’s National Youth Tobacco Survey. The latest survey found that the use of e-cigarettes increased from 1.1 percent in 2013 to 3.9 percent in 2014 among middle school students, and from 4.5 percent to 13.4 percent among high school students. That translates to a total of 450,000 middle school students now using e-cigs, alongside 2 million high school students.

Related blog post: Tobacco 21 & ENDing Combustible Tobacco Use | gutsandgrowth

 

Also, from NY Times, Gilead is making a lot of money on its Hepatitis C medications ,$4.55 billion in 1st quarter:

Sales of Gilead Sciences’ drugs to treat hepatitis C reached $4.55 billion in the first quarter, far exceeding already lofty Wall Street expectations but likely to focus attention once again on the overall costs to the health care system of the medicines.

Gilead said on Thursday that its new drug, Harvoni, had overall sales in the quarter of $3.58 billion, of which $3.02 billion was in the United States. This was the first full quarter of sales for Harvoni, which was approved in October.

Sales of Sovaldi, the older hepatitis C drug, fell to $972 million in the quarter from $2.27 billion in the first quarter of 2014 because it was supplanted by Harvoni. Combined, hepatitis C drug sales in the first quarter were double that of a year earlier.

University of Chicago

University of Chicago -Midway

Medical Marijuana -Update

Posted on by

While medical marijuana is not a frequent concern of many pediatric gastroenterologists, our nurses have been getting questions with the recent passage of legislation.  In Georgia, as in many states, marijuana is allowed for certain medical conditions. “Georgia’s medical marijuana law [Haleigh’s Hope Act] does not legalize the production or sale of marijuana, it simply decriminalizes its possession by certain qualified individuals.” –GeorgiaCann Website

in Georgia the patient must suffer from one of these qualifying illnesses:

  1. Cancer, when such diagnosis is end stage or the treatment produces related wasting illness, recalcitrant nausea and vomiting.
  2. Amyotrophic Lateral Sclerosis (ALS), when such diagnosis is severe or end stage.
  3. Seizure disorders related to diagnosis of epilepsy or trauma related head injuries.
  4. Multiple Sclerosis, when such diagnosis is severe or end stage.
  5. Crohn’s Disease
  6. Mitochondrial Disease
  7. Parkinson’s Disease, when such diagnosis is severe or end stage.
  8. Sickle Cell Disease, when such diagnosis is severe or end stage.

While I will not be recommending medical marijuana for my patients, here is a link for How to Legally Obtain Medical Marijuana Oil in Georgia (thanks to AM for information).

Also, Georgia Department of Public Health -Low THC Oil Registry Page

Related blog posts:

From CNN:

University of Chicago

University of Chicago

“This Is A Stick Up — Your Money or Your Life”

Posted on by

When I read a recent Hepatology editorial (Hepatology 2015; 61: 1106-8), I could not help think of the aforementioned title of this blog.

Here’s the scoop:

The two most commonly used medications for Wilson’s disease are trientine (Syprine) and D-penicillamine (Cupramine). For about 20 years, the original manufacturer of these medications kept the consumer cost at ~$1 per 250 mg tablet.  Currently the cost of Syprine is ~$200 per 250 mg tablet and Cuprimine costs ~$55 per 250 mg tablet.  This 200-fold increase translates into a yearly cost of ~$300,000.

How did this happen?

  • Little competition
  • Profit motive
  • Patients are reluctant to protest (they need this medication to be manufactured)

Why is this outrageous?

This increase in cost was not driven by any new discovery or research innovation.

Are there options?

Zinc is inexpensive and may be an option after initial period of chelation/normalization of liver biochemistries.  Zinc needs to be taken two to three times per day and “well away from meals for best absorption.”

Bottomline: These medication prices are outrageous.

Briefly noted:

  • “Molecular pathophysiology of portal hypertension”  Hepatology 2015; 61: 1406-15. Terrific review with excellent figures.
  • “Ezetimibe for the treatment of Nonacloholic Steatohepatitis” (MOZART trial) Hepatology 2015; 61: 1239-50. This randomized double-blind, placebo-controlled trial with 50 patients (biopsy-proven NASH) showed that Ezetimbe was not significantly different from placebo in histologic response rates, serum aminotransferases, or in magnetic resonance elastography findings.
  • Van Biervliet et al. “Clinical Zinc Deficiency as Early Presentation of Wilson Disease” JPGN 2015; 60: 457-9. Case report.

Screen Shot 2015-04-19 at 8.55.40 PM

Chronic Pancreatitis in Pediatrics -Descriptive Study

Posted on by

“Genius is one percent inspiration and ninety-nine percent perspiration.” – Thomas A. Edison

I thought about this saying as I was reading an editorial titled: “Understanding Pediatric Chronic Pancreatitis: Inspiration and Hard Work Required” (Pant C, Sferra TJ. J Pediatr 2015; 166: 798-800). The editorial was reviewing the article “Pediatric Chronic Pancreatitis Is Associated with Genetic Risk Factors and Substantial Disease Burden” (Schwarzenberg SJ et al. J Pediatr 2015; 166: 890-6).

The study comes from the International Study Group of Pediatric Pancreatitis: In Search of a Cure (INSPPIRE) consortium.  None of the findings in the study are particularly surprising; nevertheless, a descriptive study of the patients in the registry who had strictly defined chronic pancreatitis (n=76) is still an important early step in improving our understanding of this dreaded problem.

Chronic pancreatitis required either:

  • Abdominal pain consistent with pancreatic pain with imaging findings suggestive of chronic pancreatic damage
  • Evidence of exocrine or endocrine pancreatic insufficiency and imaging findings suggestive of chronic pancreatic damage
  • Histology (surgical biopsy) findings suggestive of chronic pancreatitis

Key points:

  • Two-thirds of patients with genetic testing had identified genetic mutations: PRSS1 (n=33), SPINK1 (n=14), CFTR (n=11), chymotrypsin C (CTRC) (n=2).  Mutations in more than 1 gene were noted in 9 patients, including 6 of the 11 with CFTR mutations.  Several newer mutations, like calcium-sensing receptor and carboxypeptidase A1, were not evaluated in any of the patients.
  • Pancreas divisum was present in 15 patients; however, 8 of 15 of these patients had an identified genetic mutation as well.
  • Radiographic findings of chronic pancreatitis were most commonly ductal abnormalities and pancreatic atrophy. This is in contrast to adults in which pancreatic calcifications are common.
  • The researchers also document severe disease burden with patients reporting a median of 3 emergency dept visits and 2 hospitalizations in the previous year. In addition, 70% (n=47) had missed 1 day of school in the past month and 34% had missed 3 or more days.
  • Medical treatment (eg. pancreatic enzymes) was ineffective in the majority of patients.
  • 43% had undergone ERCP and two-thirds noted improvement from this intervention
  • Surgical procedures were performed in 39% and were helpful in the majority.  Total pancreatectomy with islet autotransplantation was the most common surgery in this cohort and was helpful in 20 of 21 patients.
  • The authors recommend avoidance of CT scans due to concerns of accumulating excess ionizing radiation exposure.

Take home message: For me, this study helps define the problem.  As a practical matter, it would be helpful to have a genetic panel to check for the lesser frequent mutations if PRSS1, SPINK1, and CFTR are normal.

Related blog posts:

Stool Color Cards -Not Flashy but Effective

Posted on by

A recent report (Gu YH et al J Pediatr 2015; 166: 897-902) shows the cumulative experience of 19 years of using stool color cards to detect biliary atresia.  Cards were distributed to all pregnant women (Tochigi Prefecture, Japan) prior to or during the postnatal one-month health check.

Key findings:

  • 34 patients detected among 313,230 live births. Reported sensitivity: 76.5% and specificity 99.9%.
  • Mean age for performance of Kasai was 59.7 days.
  • Improved long-term retention of native liver: 88% at 5 years, 77% at 10 years, and 49% at 15 years.

Limitation: Children in Western countries have had lower success rates following Kasai procedure, so it is unclear whether stool cards would be as effective in different regions.

Take-home message: Detecting biliary atresia earlier will improve outcomes.  Stool color cards should be an easy low-tech option.  Other options would include stool color apps and checking bloodwork.

Image below from Screenshot from John Pohl’s twitter feed:

Embedded image permalink

Related blog posts: