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About gutsandgrowth

I am a pediatric gastroenterologist at GI Care for Kids (previously called CCDHC) in Atlanta, Georgia. The goal of my blog is to share some of my reading in my field more broadly. In addition, I wanted to provide my voice to a wide range of topics that often have inaccurate or incomplete information. Before starting this blog in 2011, I would tear out articles from journals and/or keep notes in a palm pilot. This blog helps provide an updated source of information that is easy to access and search, along with links to useful multimedia sources. I was born and raised in Chattanooga. After graduating from the University of Virginia, I attended Baylor College of Medicine. I completed residency and fellowship training at the University of Cincinnati at the Children’s Hospital Medical Center. I received funding from the National Institutes of Health for molecular biology research of the gastrointestinal tract. During my fellowship, I had the opportunity to work with some of the most amazing pediatric gastroenterologists and mentors. Some of these individuals included Mitchell Cohen, William Balistreri, James Heubi, Jorge Bezerra, Colin Rudolph, John Bucuvalas, and Michael Farrell. I am grateful for their teaching and their friendship. During my training with their help, I received a nationwide award for the best research by a GI fellow. I have authored numerous publications/presentations including original research, case reports, review articles, and textbook chapters on various pediatric gastrointestinal problems. In addition, I have been recognized by Atlanta Magazine as a "Top Doctor" in my field multiple times. Currently, I am the vice chair of the section of nutrition for the Georgia Chapter of the American Academy of Pediatrics. In addition, I am an adjunct Associate Clinical Professor of Pediatrics at Emory University School of Medicine. Other society memberships have included the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN), American Academy of Pediatrics, the Food Allergy Network, the American Gastroenterology Association, the American Association for the Study of Liver Diseases, and the Crohn’s and Colitis Foundation. As part of a national pediatric GI organization called NASPGHAN (and its affiliated website GIKids), I have helped develop educational materials on a wide-range of gastrointestinal and liver diseases which are used across the country. Also, I have been an invited speaker for national campaigns to improve the evaluation and treatment of gastroesophageal reflux disease, celiac disease, eosinophilic esophagitis, hepatitis C, and inflammatory bowel disease (IBD). Some information on these topics has been posted at my work website, www.gicareforkids.com, which has links to multiple other useful resources. I am fortunate to work at GI Care For Kids. Our group has 17 terrific physicians with a wide range of subspecialization, including liver diseases, feeding disorders, eosinophilic diseases, inflammatory bowel disease, cystic fibrosis, DiGeorge/22q, celiac disease, and motility disorders. Many of our physicians are recognized nationally for their achievements. Our group of physicians have worked closely together for many years. None of the physicians in our group have ever left to join other groups. I have also worked with the same nurse (Bernadette) since I moved to Atlanta in 1997. For many families, more practical matters about our office include the following: – 14 office/satellite locations – physicians who speak Spanish – cutting edge research – on-site nutritionists – on-site psychology support for abdominal pain and feeding disorders – participation in ImproveCareNow to better the outcomes for children with inflammatory bowel disease – office endoscopy suite (lower costs and easier scheduling) – office infusion center (lower costs and easier for families) – easy access to nursing advice (each physician has at least one nurse) I am married and have two sons (both adults). I like to read, walk/hike, bike, swim, and play tennis with my free time. I do not have any financial relationships with pharmaceutical companies or other financial relationships to disclose. I have helped enroll patients in industry-sponsored research studies.

Expert Advice on Clostridium difficile and Inflammatory Bowel Disease

Link: Management of Clostridium difficile Infection in Inflammatory Bowel Disease: Expert Review from the Clinical Practice Updates Committee of the AGA Institute

Abstract: The purpose of this expert review is to synthesize the existing evidence on the management of Clostridium difficile infection in patients with underlying inflammatory bowel disease. The evidence reviewed in this article is a summation of relevant scientific publications, expert opinion statements, and current practice guidelines. This review is a summary of expert opinion in the field without a formal systematic review of evidence.

Best Practice Advice 1: Clinicians should test patients who present with a flare of underlying inflammatory bowel disease for Clostridium difficile infection.

Best Practice Advice 2: Clinicians should screen for recurrent C difficile infection if diarrhea or other symptoms of colitis persist or return after antibiotic treatment for C difficile infection.

Best Practice Advice 3: Clinicians should consider treating C difficile infection in inflammatory bowel disease patients with vancomycin instead of metronidazole.

Best Practice Advice 4: Clinicians strongly should consider hospitalization for close monitoring and aggressive management for inflammatory bowel disease patients with C difficile infection who have profuse diarrhea, severe abdominal pain, a markedly increased peripheral blood leukocyte count, or other evidence of sepsis.

Best Practice Advice 5: Clinicians may postpone escalation of steroids and other immunosuppression agents during acute C difficile infection until therapy for C difficile infection has been initiated. However, the decision to withhold or continue immunosuppression in inflammatory bowel disease patients with C difficile infection should be individualized because there is insufficient existing robust literature on which to develop firm recommendations.

Best Practice Advice 6: Clinicians should offer a referral for fecal microbiota transplantation to inflammatory bowel disease patients with recurrent C difficile infection.

 

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Heartfelt Apologies

“Resentment is like drinking poison and then hoping it will kill your enemies.”

–Nelson Mandela

A recent NY Times article explains how the right type of apology can be good medicine: The Right Way to Say ‘I’m Sorry’

An excerpt:

I admit to a lifetime of challenges when it comes to apologizing, especially when I thought I was right or misunderstood or that the offended party was being overly sensitive. But I recently discovered that the need for an apology is less about me than the person who, for whatever reason, is offended by something I said or did or failed to do, regardless of my intentions…

Nor should a request for forgiveness be part of an apology. The offended party may accept a sincere apology but still be unready to forgive the transgression. Forgiveness, should it come, may depend on a demonstration going forward that the offense will not be repeated…

Offering an apology is an admission of guilt that admittedly leaves people vulnerable. There’s no guarantee as to how it will be received. It is the prerogative of the injured party to reject an apology, even when sincerely offered…

“Apology has the power to repair harm, mend relationships, soothe wounds and heal broken hearts. An apology actually affects the bodily functions of the person receiving it — blood pressure decreases, heart rate slows and breathing becomes steadier.”

Related blog posts:

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Safe Sleep (AAP 2017)

Behind the scenes, there is a core group of pediatricians and staff at the American Academy of Pediatrics working to improve the health of children and the ability of pediatricians to be effective.

A couple items from this year’s Georgia board of directors meeting:

  1. Increasing Safe Sleep practices
  2. Working with legislators to improve access to health care
  3. Establishing strategic goals for the next few years

The emphasis on Safe Sleep follows recent AAP guidelines –see previous posts:

Right now, in Georgia, it is estimated that there are 3 infant deaths per week associated with sleep practices.  In Tennessee, following widespread adoption and promotion of safe sleep practices, this resulted in a 50% reduction in these types of infant deaths within two years.  In Georgia, the department of public health has been working on distributing inexpensive portable bassinets to Medicaid population, along with educational material.  There is a lot more to do.  In hospital nurseries infants are often NOT placed on their backs to go to sleep until shortly before discharge.

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Useful website: Charlieskids.org This website has a book called “Sleep Baby Safe and Snug” which incorporates updated recommendations on safe sleep practices.  Interestingly, the fact that the book has a picture of a pacifier has slowed distribution of this book (even when free) because this runs counter to another program (“Baby Friendly” hospital designation) to promote breastfeeding.

Here are some of the slides from Dr. Freed’s presentation on safe sleep practices:

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Word of Caution with New Hepatitis C Medications

From NY Times: Are New Drugs for Hepatitis C Safe? A Report Raises Concerns

An excerpt:

Drugs approved in recent years that can cure hepatitis C may have severe side effects, including liver failure, a new report suggests. The number of adverse events appears relatively small, and the findings are not conclusive. But experts said the report was a warning that should not be ignored…

The report will be published online on Wednesday [1/25/17] by the Institute for Safe Medication Practices, a nonprofit in Horsham, Pa., that studies drug safety. Its findings are based on the group’s analysis of the Food and Drug Administration’s database of reports from doctors around the world of adverse events that might be related to medications.

In October, the F.D.A. identified the first major safety problem caused by the nine antiviral drugs. In 24 patients, the drugs wiped out hepatitis C — but also reactivated hepatitis B infections that had been dormant. Two of those patients died, and one needed a liver transplant. The agency said there were probably additional cases that had not been reported.

As a result, the agency required that a boxed warning, its most prominent advisory, be added to the labeling of the newer antivirals, telling doctors to screen and monitor for hepatitis B in all patients taking the drugs for hepatitis C. Infection with both viruses is not common, and how the reactivation occurs is not known. The problem was not detected during premarket testing of the drugs because patients who currently had hepatitis B or who had a history of it were not allowed into the studies…

The other cases of liver failure are a separate problem. He said it was important for doctors prescribing the newer drugs to test patients’ liver function thoroughly first, because liver disease can be deceptive

My take: Overall, these newer Hepatitis C medications represent a tremendous achievement.  However, as with most medications, rare serious problems can occur and in some cases may be preventable.

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From Twitter Feed-Funny Church Signs

From Twitter Feed-Funny Church Signs

February Briefs

JM Powers et al. J Pediatr 2017; 180: 212-6. This retrospective study details a protocol for using intravenous ferric carboxymaltose (FCM) (Injectafer) in children.  This product has become available for adults in U.S. since June 2013; it had been available in Europe since 2009. In this retrospective study, 72 pediatric patients received FCM for iron deficiency anemia (off-label); there was a good safety profile and a good response with hemoglobin increasing from 9.1 to 12.3 (4-12 weeks post infusion).  FCM is a relatively costly IV iron formulation, but can be given over 15 minutes.

L Peyrin-Biroulet et al. Clin Gastroenterol Hepatol 2017; 15: 25-36.  This systemic review with more than 2800 patients showed that TNF antagonists were effective for extraintestinal manifestations of inflammatory bowel disease, including cutaneous disorders (eg.. pyoderma gangrenosum, erythema nodosum), hematologic problems (eg anemia), ocular disorders, and rheumatologic symptoms( eg. arthralgias/arthritis).

AE Mikolajczyk et alClin Gastroenterol Hepatol 2017; 15: 17-24. Useful review of the GI/Liver manifestations of autosomal-dominant polycystic kidney disease. “There is not a role for therapy [for the liver] in asymptomatic patients.” Other problems reviewed included pancreatic cysts, hernias, and diverticular disease. Related posts:

T Rajalahti et al. JPGN 2017; 64: e1-6.  Among 455 patients <18 with Celiac disease, anemia was noted in 18%. This resolved in 92% after one year of a gluten-free diet.  Anemia is associated with more severe histological and serological presentation. Related posts:

FL Cameron et al. JPGN 2017; 64: 47-55. This retrospective review of 93 children treated with infliximab and 28 children with adalimumab provides data on growth after anti-TNF therapy.  This study shows that anti-TNF therapy is more likely to be associated with growth improvement when used at earlier stages of puberty.

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Chattahoochee River

Chattahoochee River

Deadly Market Forces in Narcotics

Lately, I’ve been shocked and dismayed by the frequent headlines about the number of overdoses and deaths due to narcotics throughout our country.  A timely article (RG Frank, HA Pollack. NEJM 2017; 376: 605-7) addresses one aspect of this threat to public health that I was not aware of previously.

  • Fentanyl, which is a powerful synthetic opioid, is much cheaper to produce than heroin.  In addition, fentanyl can result in death much more quickly as well.
  • Presumably due to its lower cost, suppliers ‘cut’ heroin with the drug.  As a consequence, fentanyl is increasingly responsible for opioid deaths. The authors estimate that from 2012 to 2014, the number of deaths due to fentanyl doubled to 5544 and that “41% of the roughly 7100 heroin-related deaths during this period involved fentanyl.”
  • Fentanyl has been found in multiple counterfeit illicit drugs.  For example, in a recent analysis from Canada, “89% of seized counterfeit OxyContin tablets” had fentanyl present.
  • Naloxone can reverse fentanyl overdoses but needs to be given more quickly and sometimes multiple doses are needed.

My take: The presence of fentanyl in illicit drugs means that even experimenting once could be fatal.

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Related blog posts:

 

Narcotic Slippery Slope

In a recent article (NEJM 2017; 376: 663-73), ML Barnett show that opioid-prescribing patterns of emergency physicians may increase the risk of long-term use. By focusing on variation of prescribing practices among physicians at the same hospitals and with a sample size of ~380,000 patients, the authors provide convincing data that starting opioids even for an intended brief period can have lasting consequences. This study focused on medicare beneficiaries (average age ~68 yrs) who received narcotics from either higher-frequency or lower-frequency physician prescribers.

In their discussion, the authors state “if our results represent a causal relationship, for every 49 patients prescribed a new opioid in the emergency department who might not otherwise use opioids, 1 will become a long-term user.”

My  take: Starting a narcotic may be the first step in a long treacherous road.

Related blog posts:

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Briefly Noted: Crash Test Dummies

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Here’s the link: Crash Test Dummies Fatter too!

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Atul Gawande: “Tell Me Where It Hurts”

In a recent New Yorker article (Jan 23, 2017, pgs 36-45,LINK:  “Tell Me Where It Hurts” –thanks to Stan Cohen for this article), Atul Gawande provides a compelling narrative on the ‘heroism’ of incremental care.

He starts his narrative with the story of Bill Haynes who had had severe migraines for four decades, but eventually improved under the care of Elizabeth Loder (John Graham Headache Center).  Over the course of four years, her ‘systematic incrementalism had done what nothing else had.’

Dr. Gawande explains that chronic illness is commonplace but “we have been poorly prepared to deal with it.  Much of what ails us requires a more patient kind of skill.  I was drawn to medicine by the aura of heroism–by the chance to charge in and solve a dangerous problem.”

Despite the appeal of dramatic medical moments, bigger impacts are noted with more subtle care. “States with higher ratios of primary care physicians have lower rates of general mortality, infant mortality, and mortality from specific conditions such as heart disease and stroke.”  One of the ways mortality is reduced is getting seen sooner for medical problems. Having a relationship with a physician “has a powerful effect on your willingness to seek care for severe symptoms.”

A parallel narrative in this piece regards the Silver Bridge tragedy in 1967.  This bridge which connected Gallipolis, Ohio and Point Pleasant, West Virginia, over the Ohio River, keeled over and resulted in 46 deaths and dozens more who were injured. This tragedy sparked attention towards infrastructure and trying to address problems before a critical collapse occurs.  “We will all turn out to have –like the Silver Bridge and the growing crack in its critical steel link–a lurking heart condition or a tumor or a depression or some rare disease that needs to be managed. This is a problem for our healthcare system.  It doesn’t put great value on care that takes time to pay off.”

Other points:

  • Incremental medicine is “at odds with our system’s allocation of rewards…the lowest-paid specialties: pediatrics, endocrinology, family medicine, HIV/infectious disease, allergy/immunology, internal medicine, psychiatry, and rheumatology.”
  • “More than a quarter of Americans and Europeans who die before the age of seventy-five would not have died so soon if they’d received appropriate medical care for their conditions.”
  • “Data indicate that twenty-seven per cent of adults under sixty-five…[have] conditions that make them uninsurable without protections” form the Affordable Care Act.

My take: Fixing an aging bridge may not be as exciting as building a new one –unless you are the aging bridge or depend on that bridge.

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Patterns and Puzzles with Very Early Onset Inflammatory Bowel Disease

A recent review (S Chandrakasan, S Venkateswaran, S Kugathasan [corrresponding author]. Pediatr Clin N Am 2017; 64: 139-160) provides a surprisingly easy read on very early onset (VEO) inflammatory bowel disease (IBD) (Thanks to Kathleen McNamara for sharing).  Because of the myriad of genetic defects (>50 monogenetic defects), the topic of VEO-IBD can be quite confusing.  The authors of this summary make a number of key points.

  • VEO-IBD is increasing in incidence
  • Many patients with VEO-IBD have an underlying primary immune defect.  Identification of these underlying disorders may allow targeted therapy.
  • In some patients, hematopoietic stem cell transplantation could result in definitive cure
  • VEO features: more often involves colon and often severe course with poor response to conventional immunosuppressives

Besides idiopathic IBD, the differential can be subdivided into subgroups:

  • T-cell defects (IPEX and IPEX-like) (gene defects: FOXP3, LRBA CTLA4, STAT1, STAT3, STAT5B, CD25, CTLA4, )
  • Defects in IL-10 signaling (IL10RA, IL10RB, IL-10)
  • Hyperinflammtory/autoinflammatory disorders (XIAP/SAP (BIRC4), Mevalonate kinase deficiency (MVK), PLCG2, Familial Mediterranean Fever, Familial HLH Type 5: STXBP2, Hermansky-Pudlak: HPS1, HPS4, HPS6)
  • Defects in neutrophil function/phagoycte function (chronic granulomatous disease (CGD) genes: CYBB, CYBA, NCF1, NCF2, NCF4,, Leukocyte Adhesion Defect (LAD) ITGB2, GSD type 1bSLC37A4, congenital neutropenia G6PC3)
  • Epithelial barrier defect (X-linked ectodermal dysplasia and immunodeficiency (NEMO), TTC7A, ADAM17, dystrophic epidermolysis bullosa (COL7a1), Kindler syndrome (FERMT1), mutations in guanylate cyclase c, telomere biology defects like  DKC and RTEL1 )
  • T/B cell defects (X-linked agammaglobulin (BTK), SCID/Omenn (RAG1, RAG2, IL-7Ra, IL-2RG), CVID, IL21, Wiskott-Aldrich (WAS) HIES, HIMS)

The authors provide some vignettes of a typical presentation of each subset along with some commentary.  For example, with T-cell defects: “mutations in FOXP3 result in either absent or decreased Treg cell numbers or a qualitative defect…results in broader immune dysregulation, resulting in multisystem autoimmunity with autoimmune endocrinopathy, autoimmune cytopenia, autoimmune hepatitis, and severe eczema.”  Other IPEX-like mutations include gain of function in STAT-1/STAT-3, LRBA deficiency, and CTLA-4 haploinsufficiency.

The authors recommend an initial immune evaluation in VEO-IBD:

  • CBC
  • peripheral smear evaluation
  • immunoglobulin levels, lymphocyte subsets with T-cell
  • B-cell, and NK-cell enumeration
  • CD45RA/RO enumeration and B-cell panel for class-switched memory B cells
  • neutrophil oxidative burst
  • T regulatory cell (CD4+CD25+FOXP3+) cell enumeration.

Due to the increasing complexity of the immune evaluation, the necessity of a pediatric immunologist is apparent.  In addition, the role of genetic panels that test for all of these disorders simultaneously is becoming routine.  Genetic testing can help improve diagnosis and allow for early targeted intervention.  With the emergence of new defects, selecting the right lab with an up-to-date panel is another caveat.

Examples of targeted therapies include the potential role of anakinra for CGD, abatacept for LRBA deficiency, toclizumab (IL-6 receptor blocking antibody) for STAT3 gain-of-function mutation, and sirolimus for Treg disorders.  Hematopoietic stem cell transplantation is an established therapy for IL-10 signaling defects.

My take: Collaboration with immunology is an important consideration in young children with IBD.

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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.

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