Author Archives: gutsandgrowth

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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.

Severe Pruritus with Alagille Syndrome

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A recent study reviews the King’s College experience for managing pruritus associated with cholestasis in patients with Alagille syndrome (AGS) (JPGN 2013; 57: 149-54).

This retrospective study examined 62 patients (1995-2010). 82% (n=51) had pruritus.  Most common treatments:

  • Ursodeoxycholic acid in 40 patients. 1st line Rx in 31. Efficacy was rated as good in 20% and some efficacy in 67.5%.
  • Rifampicin in 39 patients. 1st line Rx in 8. Efficacy was rated as very good/good in 49% and some efficacy in 46%.
  • Cholestyramine in 18 patients. 1st line Rx in 9. Efficacy was rated as  very good in 17% and some efficacy in 67%.
  • Naltrexone in 14 patients. Efficacy was rated as good in 43% and some efficacy in 36%.
  • Alimemazine in 13 patients
  • Nonsedating antihistamines in 7 patients
  • Ondansetron in 5 patients
  • Phenobarbital in 1 patient.

Despite these medications, pruritus was controlled by medication in 41% (n=21).  16 patients were referred for liver transplantation and 11 of these patients have been transplanted.  These 11 patients make up 55% of those who had permanent resolution of their pruritus.

The authors proposed an algorithm for treatment:

  • 1st line: ursodeoxycholic acid 10-20 mg/kg/day divided in 2 doses or cholestyramine 240 mg/kg/day divided into 3 doses
  • 2nd line: (if needed) Add/substitute rifampicin 5-10 mg/kg/day divided into 2 doses (max 600 mg/day)
  • 3rd line: (if needed) Add/substitute naltrexone 0.25-0.5 mg/kg/day (max 50 mg/day)
  • 4th line: (if needed) Add/substitute ondansetron max 8 mg/day divided into 2 doses per day (or phenobarbital 5-10 mg/kg/day divided into 2 doses.
  • If none of these are helpful, options could include MARS (molecular adsorbent recirculation system), partial external biliary diversion, or liver transplantation.

Related blog entries:

Extensive Workup Not Needed for IBS

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Another study has shown that an extensive workup is not needed for IBS (Clin Gastro Hepatol 2013; 11 956-62).

In this study from Denmark, the authors enrolled 302 patients aged 18-50 from a primary care setting with suspected IBS.  250 patients completed the entire study including a 1-year followup.  These patients fulfilled Rome III criteria and had no alarm signals which were the following:

  • Unexplained weight loss >3 kg
  • Rectal bleeding
  • Unexplained fever or anemia
  • Family history of inflammatory bowel disease (IBD) or colorectal cancer (CRC)
  • Abnormal physical exam

Patients were randomly assigned to either an extensive diagnostic group which included blood tests (including celiac screen & lactase gene test), stool exams, and sigmoidoscopy or to a “positive strategy” which involved testing only with a blood count (CBC/diff) and C-reactive protein.

The group which underwent a more extensive workup had no cases of serious disease, like IBD or CRC identified.  11 patients were identified with lactose intolerance, 1 patient had a rectal adenoma, 1 patient had a benign polyp, and 1 patient had giardiasis.

Overall, the authors and the accompanying editorial (pgs 963-964) conclude that the positive strategy was noninferior to the more extensive evaluation.  One limitation of this study was that patients had carried symptoms compatible with IBS for an average of 7 years before enrollment.

Take-home message: this study “adds to the growing body of evidence in favor of a relatively minimal symptom-based approach to diagnosing IBS.”

Related blog posts:

Understanding IBD Therapy Risks -A Good Link

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This link was posted on the GI Bulletin Board.  It is a 6 minute internet video overview of the treatments for IBD -it would be a useful resource for most families:

http://www.youandibd.com/en/understanding-ibd/understanding-the-risks-and-benefits-of-ibd-therapies.html

Some related blog posts:

Data Supporting Miralax

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A summary of the effectiveness of polyethylene glycol for chronic constipation, fecal disimpaction, and as a bowel preparation are presented in a recent article (JPGN 2013; 57: 134-40).

The article provides information on the biochemistry and mechanism of action along with a good number of references –49.

From the summary:

“PEG is an osmotic laxative used in children in the last few years.  It is more effective than lactulose for the treatment of chronic constipation.  It is equally effective compared with milk of magnesia and mineral oil for the long-term treatment of constipation but has a much better acceptance rate…It is a safe medication without any significant adverse effects.  Because PEG can be mixed in a beverage of the patient’s choice, it has excellent long-term patient acceptance.”

Related blog posts:

High Rates of Anxiety Develop in Kids with RAP

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From NY Times review of a recent Pediatrics study:

Children with chronic stomach pains are at high risk for anxiety disorders in adolescence and young adulthood, a new study has found (goo.gl/I2UvHP ), suggesting that parents may wish to have their children evaluated at some point for anxiety.

Researchers at Vanderbilt University tracked 332 children with recurring stomachaches that could not be traced to a physical cause — so-called functional abdominal pain — comparing them as they reached young adulthood with 147 children who had never had such stomachaches.

About half the teenagers and young adults who had had functional abdominal pain as children developed an anxiety disorder at some point, compared with 20 percent of the control group, the researchers found. The vulnerability to anxiety persisted into adulthood even if the pain had disappeared, although the risk was highest if the pain continued.

Forty percent of the children with functional abdominal pain went on to experience depression, compared with 16 percent of those who had never had these stomachaches.

The study was published on Monday in the journal Pediatrics.

“What this study shows is a strong connection between functional abdominal pain and anxiety persists into adulthood, and it drives home the point that this isn’t by chance,” said Dr. John V. Campo, chairman of the department of psychiatry at Ohio State University, who was not involved in the new study….

Chronic abdominal pain affects 8 percent to 25 percent of school-age children. The problem can lead to school absences and take a toll on families.

“Somebody might say, ‘Of course they have mental issues or they are emotionally distressed — it’s because of the pain,’ ” said Lynn S. Walker, senior author of the study and director of the division of adolescent health at Monroe Carell Jr. Children’s Hospital at Vanderbilt.

“But we found even if the pain went away, these adolescents and young adults still have anxiety,” Dr. Walker said. “So maybe we need to treat their anxiety.”

The state-of-the-art treatment for functional abdominal pain is rehabilitative, focused on getting patients to participate in daily activities despite their stomachaches. “There’s no question that there are triggers for the pain, but the problem is in the perception of the pain and adaptation to the pain,” said Dr. Samuel Nurko, director of a functional abdominal pain center at Children’s Hospital Boston.

Dr. Nurko compared the pain to a light on a dimmer switch, which psychological techniques can help children control. “You don’t take away the pain,” he said. “You ‘dim’ it to be able to cope better.”

The new study underscores the importance of screening children with the condition for anxiety or depression, the authors said. Anxious children tend to be good children who are concerned about doing their best, Dr. Walker said, and parents may be flummoxed by the suggestion that such a child could be grappling with a mental health issue.

Related blog link:

Anxiety and Functional Abdominal Pain | gutsandgrowth  This link has additional links on related material.

What it takes to adopt good ideas

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A New Yorker article by Atul Gawande highlights the importance of spending time and talking about good ideas on a person-to-person basis as the best way to help innovations take hold.  Specific innovations that are discussed include the introduction of anesthesia, the control of germs/Lister’s theories of sepsis, adoption of oral rehydration solutions, and preventing hypothermia in newborns. The link to the article and a brief video review on the Colbert report:

Staying current with PSC

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A recent article provides a useful review for primary sclerosing cholangitis (PSC) (Clin Gastroenterol Hepatol 2013; 11: 898-907).

This blog has previously discussed PSC (links below); however, the above reference is succinct and covers the key issues.  A couple of points that I found particularly helpful:

Cancer surveillance:

  • Cholangiocarcinoma (CCA): recommends “consider annual imaging (MRCP or Ultrasound) along with serum CA19-9 levels” to monitor for cholangiocarcinoma.  If there is a dominant stricture, proceed with ERCP with brushings. In pediatrics, the age to start screening is less clear, usually not presenting until beyond the late teen years, though CCA has been diagnosed in one case report at 14 years of age (NEJM 2003; 348: 1464).
  • Gallbladder cancer (30-40-fold higher risk than general population): If gallbladder polyp identified that is ≥0.8 cm, recommends cholecystectomy.  If smaller, may also want to remove if normal synthetic function; otherwise repeat imaging in 3-6 months.
  • Colon cancer: colonoscopy every 1-2 years in those with coexistent IBD (70% of patients with PSC have IBD).

Diagnosis: 44-56% of patients are asymptomatic at time of diagnosis, picked up due to abnormal serum liver tests or on cross-sectional imaging.

Small-duct PSC: occurs in the setting of features of PSC (histology, biochemistry) without abnormal cholangiogram.  This represents 11-17% of PSC patients and is difficult to identify in patients without IBD. Over time, 25% will develop large-duct PSC. Small-duct PSC does not appear to result in increased risk of CCA.

Overlap syndrome with autoimmune hepatitis: patients with typical PSC but with 5- to 10-fold aminotransferase elevations should be suspected of having an overlap syndrome and may benefit from treatments directed at autoimmune hepatitis.  Other features often include histology with an interface hepatitis and the presence of auto-antibodies. This situation is more common in children and young adults.

Immunoglobulin G4-Related sclerosing cholangitis: this occurs most commonly in conjunction with autoimmune pancreatitis.  Since steroids can be effective, IgG4 levels should “be tested in all patients with suspected PSC, and, if elevated to consider an evaluation for IgG4-related disease.”

Medical management: “to date, there are no medical therapies that have been proven to alter the natural course of PSC.”  The discussion notes that standard doses of ursodeoxycholic acid (UDCA) may have protective effects against colorectal cancer in patients with coexisting IBD.  Higher doses of UDCA have been associated with a 2-fold risk of increased disease progression. Specific treatments for dominant strictures, pruritus, metabolic bone disease, and malabsorption are discussed.  In patients with cholestasis, monitoring fat-soluble vitamins is important.

Related blog posts:

Tell me about your mother

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The following except from the New York Times (nyti.ms/1cvUprv ) by Haider Javed Warraich provides some useful advice when families members ask “What would you do if this were your mother?”  In pediatrics, the question is similar: “What would you do if this were your child?”

The patient was an elderly woman, admitted to our unit just a few hours earlier, with a breathing machine keeping her alive. We proceeded with the meeting as we were trained to do. We kept our elbows off the table, maintained eye contact (but not too much) and gave the family an update of where we stood.

A healthy family meeting, we’d been told, involved us speaking for about half the time, with the family speaking for the rest – venting, questioning, grieving and hoping, in no particular order. This meeting, though, was dominated by long periods of silence that unearthed the dull, low-pitched drone in the background.

The son, quiet for most of the meeting, broke the silence and, with a hint of anger and a big dollop of frustration, asked the one question I had dreaded being asked the most: “Doc, give it to me straight. If this were your mother, what would you do?”

While the patient-doctor interaction varies widely across cultures and continents, this question seems to be a universal constant…

From a patient or family member’s perspective, though, this question helps them make sense of the confusion, desolation and powerlessness that so often defines the hospital experience, which usually involves a full-on assault of numbers, jargon and ‘expert’ opinion. They are confronted with difficult choices, like whether they want to go ahead with a particular high-risk procedure or wait for the tincture of time to kick in…

Yet I still find this question hard to answer. See, my mother is the sort of person who spends two hours each day on the treadmill, even during vacations, so that she can eat to her heart’s content. Often described as a “fighter,” any additional moment she can spend with her children or future grandchildren would be worth the extra mile. My father, on the other hand, is someone who avoids getting his blood sugar level tested to evade medications and dreams of spending his last days in the quiet serenity of the village he grew up in. Thus my answer to the question would be very different, as it would be for anyone, depending on which parent you asked me about.

So I have come to believe that the right answer to the question, “If this were your mother, doctor…” is: “Tell me more about your mother.”

This response gives patients’ families the chance to think about their loved ones, about what they would value and what they would consider a good life, what they would think was worth fighting for if they were available to answer the question for themselves….And then, slowly, the family started sharing stories of the woman we had met only a few hours before, unconscious and intubated. She loved being independent, would hate for people to open doors for her or hold her hand as she tried to get up, they told us. She loved the sun, the beach. She loved walking, loved being out and about. She would never, ever want to go to a nursing home…

We then told them that based on a combination of her vital signs and lab values, as well as our clinical judgment, that while we could hope for some progress, it would likely not be enough to allow her any real shot at experiencing life outside a nursing facility again…

They turned to us and asked us to make her comfortable, and to turn off the breathing machine.

Related blog post:

Advice for doctors after the death of a child | gutsandgrowth

Global Disease Burden

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In 1991, the World Bank and the World Health Organization launched the Global Burden of Disease Study.  A recent article reviews the key findings (NEJM 2013; 369: 448-57).

The goals of the study are to compare the burden of one disease with others; as such, it is “necessary to consider the age at death and life expectancy of persons affected by each disease and to take account of the degree of disability (eg. discomfort, pain, or functional limitations.”  A comprehensive measure of disability, disability-adjusted life-years or DALYs, was used for comparisons.

The study examined 291 types of diseases and injuries as well as 67 risk factors in 187 countries, looking at the years 1990, 2005, and 2010.

Findings:

  • In 2010, there were 2482 million DALYs which is a decrease of 0.6% from 1990.  On the basis of population growth, DALYs would have increased by 37.9% without improvements in disease burden.
  • Major causes of death in 2010: Ischemic heart disease-far ahead #1 (21.1% of deaths, 7850 thousand DALYs), Stroke (6.5% of deaths, 2574 thousand DALYs), Lung/airway cancer (6.1% of deaths, 3033 thousand DALYs), Alzheimer’s (5.9% of deaths, 2022 thousand DALYs), COPD (5.8% of deaths, 3659 thousand DALYs).
  • Global DALYs in 2010 (top ten -starting with #1): Ischemic heart disease, Lower respiratory tract infections, stroke, diarrhea, HIV-AIDs, Malaria, Low Back pain, Preterm birth complications, COPD, and road-traffic injury.
  • Top risk factors (starting with #1): High blood pressure, tobacco smoking (including 2nd-hand smoke), household air pollution, diet low in fruit, alcohol use, high body-mass index, high fasting plasma glucose level, childhood underweight, exposure to outside pollution, physical inactivity, diet high in sodium

Since 1990, there has been a shift.  “In general, communicable, maternal, neonatal, and nutritional conditions decreased in absolute terms.”  The main exceptions were HIV and malaria. Noncommunicable diseases, especially diabetes, have been increasing in terms of percentage and absolute numbers.

Another important change has been a relative increase in disability compared with premature death.  In addition, of the “top 25 causes of years lived with disability, only COPD, diabetes, road-traffic injury, ischemic heart disease, and diarrhea are also among the tope 25 causes of years of life lost.”  “What ails most persons is not necessarily what kills them.”

Bottom-line: While collecting this type of data has many potential limitations, the broad picture it provides should help inform policymakers with priorities for research and intervention.  This data also allows the US to benchmark its efforts compared to other countries.  For example, according to the authors, currently the US has the best global performance with respect to stroke and the worst with respect to lung cancer and Alzheimer’s disease; however, “data and analyses are lacking to elucidate the drivers of these changes in relative performance.”