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:

screenshot-98

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.

screenshot-120

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:

screenshot-121 screenshot-122 screenshot-125 screenshot-126 screenshot-128 screenshot-129 screenshot-130 screenshot-133

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.

Related blog posts:

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.

Related blog posts:

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.

screenshot-135

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:

screenshot-134

Briefly Noted: Crash Test Dummies

screenshot-96

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

Related blog posts:

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.

screenshot-147

 

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.

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.

I liked this t-shirt

I liked this t-shirt

Nutrition Week (Day 7) Connecting Epidemiology and Diet in Inflammatory Bowel Disease

A supplement in Gastroenterology (2017; 152: 309-462) provides a great update on a lot of topics.  These include pathophysiology articles (eg. role of Paneth cell, role of microbiome), treatment/development of fibrosis, management advances in endoscopy and biomarkers, newest treatments and emerging treatments, complementary medicine approaches, pain/psychology issues, medications in pregnancy, and detecting dysplasia.

For me, the update on epidemiology and its relationship to diet (pgs 313-321) as well as the review on diet as a trigger or therapy for inflammatory bowel disease (398-414) were most interesting.  Though, I will keep the update on complementary and alternative medicines article at my desk in case questions come about this topic

GG Kaplan, SC Ng. “Understanding and Preventing the Global Increase of Inflammatory Bowel Disease”  Gastroenterology 2017; 152: 313-321

Epidemiology:

1st case of ulcerative colitis was reported in 1859.  !st cases of Crohn’s disease reported in 1932 (BB Crohn et al. JAMA 1932; 99: 1323-29).

Olmstead County, Minnesota –cases per 100,000:

  • 1965: 28
  • 1980: 90.5
  • 1991: 132.7
  • 2001: 213.9
  • 2011: 246.7

While rates of IBD have “shown signs of stabilization…pediatric-onset IBD continues to increase steadily in incidence.”

IBD Around the World –cases per 100,000:

  • 2005 Japan: 76
  • 2005 S Korea: 42
  • 2013 India: 9.3
  • 2013 China: 3.3.  The greatest incidence is noted in areas of increased urbanization and economic advancement.
  • 2005: Brazil: 9.7

Environmental factors/associations:

  • Cigarette smoking –increases risk of Crohn’s disease in Western countries, and has protective effect against Ulcerative colitis
  • Antibiotic use –increases risk of IBD in Western countries, but may be protective in developing countries.  “Antibiotic-induced dysbiosis may not develop as easily in developing countries, owing to ubiquitous exposure to a diverse range of microbiota that rapidly repopulate the intestinal tract.”
  • Breastfeeding –protects against developing IBD
  • Vitamin D –low levels increase risk of IBD in Caucasians.
  • Fiber –a “diet high in fiber protects against Crohn’s disease.”

screenshot-111

JD Lewis, MT Abreu.”Diet as a Trigger or Therapy for Inflammatory Bowel Disease”  Gastroenterology 2017; 152: 398-414.

“The most common question asked by patient is …’Doctor, what should I eat?'”

Key points:

  • Data from studies of immigrants to higher-IBD prevalence countries show an increasing incidence of IBD, leading to the hypothesis that environmental factors such as diet affect risk of IBD.
  • In early life, breast milk, in some but not all studies, has been associated with a lower risk of childhood-onset IBD.
  • Before development of IBD, studies have shown lower risk of IBD “among people who consume more fruits and vegetables, and a higher risk in people who consume less of these and more animal fats and sugar.”
  • “There is little information about which foods induce flares.” However, for UC, “a high intake of meat, especially red and processed meat, protein, alcoholic beverages, sulfur, and sulfate increased the likelihood of a flare” based on food questionnaires.  In patients with CD, diet with higher “total fat, saturated fat, monounsaturated fatty acids, and a higher ratio of omega-6:omega-3 PUFAs was associated with disease relapses.”
  • “Only approximately half of patients have ever received advice from a dietitian.”
  • Oral iron may trigger flares in a small percentage of patients with IBD.  The authors note that adherent E coli express genes for iron acquisition and require iron for growth.

Specific Diets/Additives:  Most of these diets have been discussed in previous posts, including:

Exclusive (and Partial) Enteral Nutrition:

  • “The most widely studied dietary intervention.” It has been shown to be effective for CD.  More elemental formulas have NOT been shown to be more effective.  “EEN and PEN therapy is less likely to normalize fecal levels of calprotectin in children.”
  • “Dietary therapy reduced inflammation and led to changes in the microbiome within 1 week. Unlike TNF antagonists, however, the changes to the microbiome induced by EEN did not lead to a microbiome resembling that of healthy individuals.”

Specific Carbohydrate Diet (SCD):

  • This diet has been studied in small populations.  Suskind et al reported SCD effectiveness “in 7 children with CD…showed that fecal calprotectin level decreased from a mean of 685 mcg/g to 213 mcg/g at 2-6 after starting the diet.”  “Cohen et al used video capsule endoscopy…in 10 children with CD…Four of 10 children achieved complete mucosal healing (Lewis score <135) and 6 of 10 children achieved clinical remission.”

Low FODMAP diet:

  • While the diet may induce symptom improvement, there is no “evidence that a low FODMAP diet reduces inflammation.”

Vitamin D supplementation:

  • “Vitamin D has multiple potential beneficial effects on intestinal inflammation.” The authors review studies that report lower risk of CD in patients with higher vitamin D levels and on the reduction in relapse in a study of CD patients who were in remission and  treated with Vitamin D (1200 IU daily)

Curcumin supplementation:

  • The authors review two small studies which suggested that curcumin for patients with ulcerative colitis increased clinical remission (when used with mesalamine)

The overall advice the authors give is that patients “should be advised to eat a well-balanced diet, such as the Mediterranean-style diet, avoiding processed foods or foods that they self-identify as worsening their symptoms.  Patients who are committed to attempting to manage their disease predominantly through dietary modification should be counseled about the importance of assessing for resolution of inflammation in addition to symptoms.”

Other Related blog posts:

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  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.