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Practical Intestinal Rehabilitation (Part 2)

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We had an brilliant lecture given to our group by Danielle Wendel who leads Seattle Children’s Intestinal Rehabilitation team. My notes below may contain errors in transcription and in omission. In addition, the information provided is based on what is done in Seattle. However, there is not a lot of evidence for much of what is done in intestinal rehabilitation. Thus, there is variation in practice at different centers and what works for one patient might not work for another. Following my notes, I have included many of her slides (same slides as yesterday’s post).

CLABSI Pointers:

  • -At Seattle, with suspected CLABSI, usually central blood culture obtained without peripheral blood culture. (Peripheral blood cultures have not helped their team improve management)
  • -Everyone with SBS and with fever (greater than or equal to 100.4) stays for at least 48 hrs on broad spectrum IV antibiotics (choice based on local sensitivities) through the central line until it is conclusively determined if they have a CLABSI (which still carry a significant mortality risk)
  • -Sodium bicarbonate lock experience has been good (8.4% solution, 1.5 mL lock for the entire time off PN in all tunneled CVL flushed in at the end of the dwell). It has become a good substitute for ethanol locks.  Their experience will be published soon.  Since sodium bicarbonate lock does not need to be withdrawn, it has been associated with less line breakage.  Several lock solutions (KiteLock and Taurolidine) are not currently available in the U.S.  KiteLock is about to be studied in Seattle.
  • -At Seattle, all CLABSI are treated  through the line and every effort is made to salvage and/or repair lines.  Line replacement increases risk of losing central IV access.
  • -Line is removed for fungal infections
  • -The Seattle team prefers tunneled CVC

SIBO Pointers:

  • -Testing is problematic.  Breath tests are not reliable in kids with SBS.  Duodenal aspirates are often not helpful and have a number of technical difficulties; also, it is unclear whether a duodenal aspirate is representative of the bacteria in the more distal bowel.
  • -Metronidazole is their first line choice.  Gentamicin (IV formulation given enterally) is their 2nd choice.  Rifaximin is their 3rd line.  Rifaximin would possibly be used earlier in treatment except for difficulty getting covered.  When used, they crush up pills rather than have it compounded to avoid sweeteners.

Teduglutide

  • -Best to start if a patient is is > 1yo and on stable TPN (not able to wean)
  • -Make sure patient is using a tiny needle (not adult needle in package)
  • -Anticipate long-term treatment (?indefinite)

GI Bleeding Pointers:

  • This is being seen frequently. 
  • Etiologies include anastomotic ulcers and IBD-like lesions.   If a patient is not improving with standard approaches and possibly resection, could need an anti-TNF type agent.
  •  At Seattle, they are very selective about patients appropriate for a STEP procedure as this may be associated with more frequent bleeding over time due to the many staples used. Hand-sewn tapering may be a better option for many patients.
  • With the challenging decisions required for these bleeding patients, discussion with an experienced intestinal rehab center may be helpful.

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  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.

Practical Intestinal Rehabilitation (Part 1)

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We had an brilliant lecture given to our group by Danielle Wendel who leads Seattle Children’s Intestinal Rehabilitation team. My notes below may contain errors in transcription and in omission. In addition, the information provided is based on what is done in Seattle. However, there is not a lot of evidence for much of what is done in intestinal rehabilitation. Thus, there is variation in practice at different centers and what works for one patient might not work for another. Following my notes, I have included many of her slides.

Key points:

  • Enteral nutrition is key for adaptation.  At Seattle, oral feeds rather than GT feeds are preferred.
  • Time is the thing that helps the most.  Unclear which additives help.
  • Though prediction models often look at >50 cm, it is important to counsel families, even with more bowel length, that the care is going to be quite challenging.
  • Acid suppression can be helpful in the early phase after resection especially if problematic high stoma output.
  • Iron: if given enterally, typically given everyday or every other day.  Iron dextran can be given in TPN (cannot be given with lipids).  Ferric carboxymaltose is a good choice for parenteral administration.  Due to the need for few infusions (~1-2 per year), it is safer (less line entry) and cost-effective compared to iron sucrose.
  • Seattle shares an “Emergency Care Letter” with their patients (template available in EPIC)
  • Yearly doppler ultrasound is recommended to assess vascular access.
  • If a patient has more than one thrombosis, the Seattle team recommends long term prophylaxis, though it might take treatment levels to prevent further thrombosis.
  • SMOFlipid is the most frequently used lipid at Seattle. It can be given in higher doses than Omegaven which is important nutritionally; omegaven is generally given at only 1 to 1.5 mg/kg/day and used for treatment of IFALD.
  • Long term outcomes are just as good with chronic TPN as with intestinal transplantation. So, referral generally needed based on complications like losing central access (3 of 4 upper central sites) or progressive liver disease.

Diet Pointers:

  • -In infancy, standard formula and breastmilk are preferred and thought to help with adaptation.  Some infants need elemental diets but it is not routinely given across the board  (some other institutions feel strongly about using elemental diets, but there is limited data)
  • -Kids with short bowel syndrome may tolerate volume better than concentration
  • -The Seattle program strictly restricts sweet tasting food/drink for first 3-4 years of life to help educate the child’s palate and recommends limiting these food/drink for IF patients in general.
  • -Addition of solid foods usually helps with stoma output

Nutrient Monitoring Pointers:

  • Usually best to batch them all lab tests for micronutrients [many micronutrients are affected by inflammation and this may affect timing of lab testing]
  •  At Seattle, aluminum and manganese are not routinely checked as they are contaminants in PN that cannot be removed
  • Serum thyroid testing is a marker for iodine deficiency in patients receiving most of their calories from PN (>70) which may be more frequent now that betadine is not used for dressing changes.  Their goal for urine iodine is >100 (can be treated with ultra-diluted potassium iodide which needs to be compounded by the pharmacy)
  • When testing for EFA (essential fatty acid) deficiency, lipids should be off for 4 hrs (or more).  Urinalysis is checked to monitor for chronic kidney disease. Urine sodium goal is >30 and is checked quarterly in patients with high ostomy output or excessive rectal stool output with poor growth.
  • Hypokalemia may be a sign of total body sodium depletion due to the kidneys dumping potassium to conserve sodium

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  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.

30 Million Excess Deaths (Estimated) Worldwide due to COVID-19

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“The COVID-19 pandemic caused the most severe drops in life expectancy seen in 50+ years.” per IHME

NY Times 3/11/24: The Fourth Anniversary of the Covid Pandemic

“Globally, Covid ranks among the worst killers since 1900. AIDS, for example, is estimated to have killed about 40 million people, but over a half century rather than only four years. The 1918 flu killed somewhere between 20 million and 50 million people.”

“Among high-income countries, the U.S. has had one of the highest Covid tolls. The excess-death rate here, as a study by Jennifer Nuzzo and Jorge Ledesma of Brown University notes, has been much higher than in Canada, Britain, Germany, France, Spain, Sweden, Denmark, Japan, South Korea or Australia…”

“Many Americans, especially political conservatives, were skeptical of the vaccines despite overwhelming evidence of their effectiveness. To this day, more than 30 percent of self-identified Republicans have not received a Covid vaccine shot, compared with less than 10 percent of Democrats…You can see the tragic effects of vaccine skepticism in this chart.“

“While many liberals exaggerated the value of pandemic restrictions, they were right about the vaccines. “

According to data from Washington State in 2023, the death rate for those older than 65 years due COVID-19 was nearly double in those unvaccinated compared to those who had been boosted. In the younger age group, 35-64, the rate of fatal infection was much lower but remained 5 times as high in those unvaccinated compared to those who had been boosted.

My take: COVID-19 has exacted a tremendous toll and these articles do not even focus on long COVID which afflicts so many people as well.

Related blog posts:

AGA: High Quality Upper Endoscopy

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S Nagula et al. Clin Gastroenterol Hepatol 2024; DOI:https://doi.org/10.1016/j.cgh.2023.10.034. Open Access! AGA Clinical Practice Update on High-Quality Upper Endoscopy: Expert Review

The summary with nine “best practice advice” statements is not very helpful. However, Figure 2 and Table 1 are very useful.

From Figure 2 -not shown below (but in article) are Prague classification for Barrett’s and EREFS for eosinophilic esophagitis. The remaining parts of this figure include the Los Angeles classification for erosive esophagitis, the Hill classification of the gastroesophageal flap, and the Forrest classification of peptic ulcers:

From Table 1:

Table 1 also gives guidance for biopsies with peptic ulcer disease, Barrett’s esophagus, gastric preneoplasia, and for gastric polyps.

My take: When suspicious of underlying disease, this article recommends taking more biopsies and in more jars.

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How Will We Afford the Future of Medicine?

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NY Times 2/19/24, Elizabeth Currid-Halkett: The Future of Medicine Is Unfolding Before Us. Are We Nurturing It?

This essay describes the terrific response of the author’s son with Duchenne Muscular Dystrophy to Elevidys. “At $3.2 million per patient, Elevidys is the second-most-expensive drug in the world.”

“D.M.D. prevents the production of dystrophin, a protein needed to protect and repair muscle cells. It is caused by a genetic mutation on the X chromosome, thus the disease almost exclusively affects boys (one in 3,300). Over time, children with D.M.D. lose muscle mass and thus the ability to do basic things like run and walk. Eventually they lose their ability to breathe, and they experience heart failure.”

She argues that “every child afflicted with a life-threatening disease deserves the chance Eliot has been given.” The article argues that the FDA should broaden the indications to cover older children with DMD in which the data are less convincing.

While this article discusses some ways to lower costs like reducing regulatory hurdles to get approval from FDA, it mainly discusses ways to force insurance companies to cover the cost. Disappointingly, the author (a public policy professor) does not address the reality that these costs are shared by everyone. For DMD alone, if the full cost is unchanged, that’s nearly $1000 for every person in the country. Yet, there are numerous other costly genetic therapies (for spinal muscular atrophy, hemophilia, Crigler-Najjar, others) in addition to extremely expensive treatments for cancer, inflammatory bowel disease, cystic fibrosis, rheumatoid arthritis, Alzheimer’s, obesity and many others.

My take: The ability to cure severe diseases like DMD is amazing. But, there is not a plan for how to deliver/afford these expensive therapies. If we cannot provide these treatments, it will be agonizing to watch patients deteriorate while a cure is just out of reach.

Meanwhile, we are not spending enough to address our biggest drivers of poor national health including poverty, education and poor diet.

Related blog posts:

Turk’s Cap Cactus. St John

Primary Prevention of Obesity Still Needed

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SL Gortmaker, SN Bleich, DR Williams. NEJM 2024; 390: 681-683. Childhood Obesity Prevention — Focusing on Population-Level Interventions and Equity

Despite the exciting advances in obesity pharmacology, most children and adults are unlikely to benefit from these expensive therapies anytime in the near future.

This commentary’s key points:

  • “Scholars and policymakers shouldn’t lose sight of population-level strategies that can prevent excess weight gain and obesity among children in the first place.”
  • The authors identify three successful policy examples: 1. Revision of WIC food packages to improve nutritional quality at a cost about $18 per child. 2. Improving school lunch standards (2010 Healthy, Hunger-Free Kids Act) at a cost of about $30 per child. 3. Excise tax on sugar-sweetened beverages. “In California, an analysis found that such a tax would be cost-saving, would prevent 42,700 cases of obesity in children and 223,000 cases in adults statewide over 10 years because of projected reductions in consumption of sugar-sweetened beverages.”
  • “Leveraging these strategies won’t fix the problem of childhood obesity overnight, but it could (and has already begun to) slow the development of new cases, particularly among members of historically underserved populations — a major public health achievement.”

My take: There is not a simple solution for widespread obesity in children and adults. We need to chip away at this problem from every angle. It is crucial to use public policy changes as one of our tools.

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Near Leduck Island off St John

When Is It Safe To Replace a Central Line in the Setting of Candida Infection?

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D Katz et al. JPGN Reports 4(4):p e358, November 2023. | DOI: 10.1097/PG9.0000000000000358 Open Access! Early Central Venous Catheter Replacement After Candida in Pediatric Intestinal Failure Patients.

Background:

  • The Infectious Diseases Society of America (IDSA) recommends the removal of both short- and long-term catheters in patients with catheter-related bloodstream infection (CRBSI) due to fungi (1). 
  • In addition to source control, antifungal treatment and blood culture-confirmed clearance are recommended for all cases of CRBSI due to candida…In contrast, little is known about the optimal timing for the replacement of a CVC in the setting of candidemia (5–7). As a result, practices greatly vary between practitioners.

Methods: This was a retrospective, single-center review of children with intestinal failure (IF). Patients were divided into early (<7 days after their first negative culture), and late (≥7 days after their first negative culture) CVC replacement following uncomplicated candidemia. 

Key findings:

  • Early replacement occurred in 18 encounters and late replacement in 21 encounters. The median time to CVC replacement or exchange in the early group was 4 days, compared to 10 days in the late group (P < 0.001).
  • The median duration of the hospitalization in the early group was 12 days compared to 21 days in the late group (P = 0.011).
  • None of the patients were reinfected with candida within 30 days.

My take: This small study provides reassurance that earlier replacement of  CVCs after clearance of uncomplicated candidemia is beneficial.

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Income and Health Outcomes in Pediatric Short Bowel Syndrome

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Clarification: Yesterday’s post on the safe use of polyethylene glycol (Long Term Use of Polyethylene Glycol (PEG 3350)) noted the labeling indicates “‘to not use these medications for more than 7 days.” However, Ben Enav pointed out that the label also states the following in bold: “do not take more than directed unless advised by your doctor.” The actual label is shown below.

———

SA Gutierrez et al. J Pediatr 2024; 265: 113819. Neighborhood Income Is Associated with Health Care Use in Pediatric Short Bowel Syndrome

Methods: The authors used the Pediatric Health Information System (PHIS) database to evaluate associations between neighborhood income and hospitalization data for children with short bowel syndrome (SBS). This included 4289 children with 16,347 hospitalizations from 43 institutions.

Key findings:

  • 2153 of the 4289 (50%) patients were readmitted during the study period (2006-2015)
  • Children living in low-income neighborhoods were more likely to be Black, Hispanic, have public health insurance, and live in the Southern U.S.
  • Children from low-income neighborhoods had a 38% increased risk for all-cause hospitalizations (rate ratio [RR] 1.38), an 83% increased risk for CLABSI hospitalizations (RR 1.83) and increased hospital length of stay.
  • 2.4% of patients in this cohort experienced 10 or more CLABSI hospitalizations

One of the study’s limitations is that ‘there is no singular ICD-9 code for SBS.’

My take: It is speculation about the reasons why children in low income neighborhoods have higher rates of hospitalizations and CLABSI hospitalizations. It could be that more parents in these households have less time and resources to manage a child with SBS. It is possible that these households have more chaotic environments. Regardless of the reason, it takes a lot of work and meticulous care to prevent CLABSI hospitalizations in children with SBS.

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A lot of Turk’s Cap Cacti along the Ram Head Trail, St John

Long Term Use of Polyethylene Glycol (PEG 3350)

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A Bautista-Casasnovas et al. JPGN Reports 4(4):p e353, November 2023. Open access! Multicentre Study Into the Use of Polyethylene Glycol With Electrolytes Over at Least 6 Months to Treat Constipation in Paediatric Populations

Background: PEG 3350 with electrolytes (PEG+E) is the most widely used osmotic laxative in Europe, and it is normally prescribed for short or limited periods in children, such that there is little information regarding its long-term use (≥6 months).

Methods: This was a retrospective, observational, descriptive, longitudinal, and multicentre study was carried out on 74 children diagnosed with functional constipation.

Patient characteristics:  The mean (±SD) duration of the symptoms of constipation before starting PEG+E treatment was >1 year (15.6 ± 8.4 months). Fecal disimpaction was necessary in 49 children (66.2%) .

Key findings:

  • The mean PEG+E dose used was 1.0 (±0.8) g/kg.
  • The mean duration of PEG+E use was 18.6 (±13.4) months (range 8–73 months), and 59.45% (n = 44) of the patients took the treatment for more than 1 year.
  • 81% (n = 60) of the patients achieved 4 or more weekly bowel movements after having taken PEG+E for at least 3 weeks.
  •  All clinical symptoms (abdominal pain, gassiness/bloating) were reduced considerably, with the resolution of the anal fissures, bleeding, and soiling in all patients.

Polyethylene glycol (the active ingredient in Miralax) is considered a first-line treatment for pediatric constipation and for fecal impaction. “However, caregivers may be hesitant to administer medication over long periods due to a fear of a rebound effect or addiction (23). Indeed, early withdrawal of laxatives is the commonest cause of recurrence (4,5), highlighting the need for longer follow-up studies (8).”

My take: It is helpful to have long term studies of PEG 3350 showing its effectiveness and safety, especially as the medication labels state to not use these medications for more than 7 days.

Related blog posts:

Clarification: However, Ben Enav pointed out that the label also states the following in bold: “do not take more than directed unless advised by your doctor.” The actual label is shown below.

Delayed Commentary

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I was a little disappointed (aka first world problem) that this commentary appeared in the February print edition of The Journal of Pediatrics about 4 months after the publication of the analyzed study. This blog commented on this study in October: Disparities Are Abundant in Pediatrics -4 Studies on IBD, SUID, Specialty Referrals and in the NICU re: J Smith et al. J Pediatr 2023; 260: 113522.

DJ Spencer. J Pediatr 2024; DOI:https://doi.org/10.1016/j.jpeds.2023.113839. Open Access! Understanding Health Outcomes in Pediatric Inflammatory Bowel Diseases: Contributing Factors that Aren’t so Black and White

“In this issue of The Journal, Smith et al report the results of an historical cohort analysis of 519 children and adolescents with newly diagnosed IBD (2013-2020)… Smith et al ask the question of whether the greater rate of complicated disease in Black patients is related more to delayed diagnosis or access to therapy rather than inherent race-based differences in response to treatment.”

Key points:

  • “In this study, Smith et al importantly identified no difference in initiating standard medical therapies based on race. Specifically, they report no difference in initial corticosteroid usage, time to initiation of maintenance therapy, or time to initiate antitumor necrosis factor therapy. In patients receiving biologics, both Black and White patients received similar loading doses and frequency of therapeutic drug monitoring.”
  • “Despite comparable disease presentation and approach to medical therapy in this study cohort, Black patients strikingly remained only one-half as likely to reach corticosteroid-free remission at 12 months compared with White patients (OR 0.52, 95% CI 0.3-0.9).”
  • ” Black patients were less likely to be seen in gastroenterology specialty clinic for follow-up, more likely to present to the emergency department, and more likely to be hospitalized.”
  • “This study described poorer outcomes in Black patients despite similar treatments. However, the authors fail to arrive at a definitive answer as to why this is the case.”

My take: Black patients, even when offered similar IBD treatment, clearly experience inferior outcomes. While access and social determinants of health are important, there may be biological/phenotypic factors (eg. more aggressive disease) that are involved as well. More studies are needed. This editorial is a helpful review -the timing of the editorial in the print edition many months later, though, is a head-scratcher.

Unrelated topic: CDC COVID-19 Recommendation

The Centers for Disease Control and Prevention announced new isolation guidance for Covid-19 this week. At the start of the pandemic, people were recommended to stay home for 10 days after testing positive. At the height of the Omicron wave, that was revised to 5 days. This week, isolation time was revised to 24 hours without a fever and symptoms improving, which is similar to the recommendations for other illnesses.

St Johns Honeymoon Beach

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