Intermediate and long-term outcomes of a bowel management program for children with severe constipation or fecal incontinence

Journal Pre-proof Intermediate and long-term outcomes of a bowel management program for children with severe constipation or fecal incontinence

Julie A. Kilpatrick, Sarah Zobell, Elisabeth J. Leeflang, Duyen Cao, Lija Mammen, Michael D. Rollins PII:

S0022-3468(19)30808-5

DOI:

https://doi.org/10.1016/j.jpedsurg.2019.10.062

Reference:

YJPSU 59491

To appear in:

Journal of Pediatric Surgery

Received date:

22 October 2019

Accepted date:

30 October 2019

Please cite this article as: J.A. Kilpatrick, S. Zobell, E.J. Leeflang, et al., Intermediate and long-term outcomes of a bowel management program for children with severe constipation or fecal incontinence, Journal of Pediatric Surgery(2019), https://doi.org/10.1016/ j.jpedsurg.2019.10.062

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© 2019 Published by Elsevier.

Journal Pre-proof

Intermediate and Long-Term Outcomes of a Bowel Management Program for Children with Severe Constipation or Fecal Incontinence Julie A. Kilpatrick, B.S.1, Sarah Zobell, APRN2, Elisabeth J. Leeflang, M.D.1 Duyen Cao, B.S.1, Lija Mammen, MBBS, MPH1, Michael D. Rollins, M.D.1,2 1 University of Utah School of Medicine, Salt Lake City, Utah 2 Primary Children’s Hospital, Intermountain Healthcare, Salt Lake City, Utah

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Corresponding Author:

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Julie Kilpatrick, B.S. Primary Children’s Hospital 100 N. Mario Capecchi, Suite 3800 Salt Lake City, UT 84113 [email protected]

Journal Pre-proof ABSTRACT Purpose: We sought to examine the long-term clinical success rates of a bowel management program (BMP) for children with severe constipation or fecal incontinence. Methods: A single center review was conducted of children (≤ 18 years) enrolled in a BMP and followed in a colorectal specialty clinic (2011-2017). All patients who completed an initial week

defined as no accidents and < 2 stool smears per week.

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of the BMP were included. Patients enrolled in a BMP after 2018 were excluded. Success was

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Results: A total of 285 patients were reviewed. BMP was initiated at a median age of 7 years (9

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months-17 years). Primary diagnoses included functional constipation (112), anorectal

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malformation (ARM) (104), Hirschsprung Disease (HD) (41), rectal prolapse (14), spina bifida (6),

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fecal incontinence (3) and other (5; 4 sacral coccygeal teratomas and a GSW to the buttocks). Initial bowel regimen included large volume enema in 54% and high dose stimulant laxative in

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46%. The initial Bowel Management Week (BMW) was successful in 233 (87% of adherent

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patients) patients with 17 (6%) non-adherent. 122 patients had follow-up at 12 months (72%

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success amongst adherent patients, 7% of patient non-adherent ) and 98 patients had follow up at 24 months (78% success amongst adherent patients, 10% of patients non-adherent). 21/154 (14%) patients started on enemas were later successfully transitioned to laxatives and 13/132 (10%) patients started on laxatives subsequently required enemas in order to stay clean. Clinic phone contact occurred outside of scheduled visits for adjustment to the BMP in 44% of patients. 33% of patients had surgery to aid bowel management (Antegrade Colonic Enema (ACE)=81, resection + ACE=13, diverting stoma=4). Median follow up was 2.5 years (5 weeks-7 years).

Journal Pre-proof Conclusion: Children who follow a structured BMP with readily available personnel to provide outpatient assistance can experience successful treatment of severe constipation or fecal incontinence long-term. A multi-institutional collaboration is necessary to identify factors which predict failure of a BMP and non-adherence.

Keywords: Bowel Management, Outcomes, Fecal Incontinence, Constipation, Anorectal

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Type of Study: Single Center Retrospective Chart Review

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Malformation

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Level of Evidence: 3

Journal Pre-proof Introduction: The prevalence of idiopathic constipation in the pediatric populations is as high as 30% and may result in medically refractive constipation or fecal incontinence [1]. Approximately 25% of ARM patients suffer from fecal incontinence despite quality treatment [2] while 10% of patients with HD suffer from obstructive symptoms after surgical correction [3]. Managing these symptoms carries associated costs of up to $3.9 billion per year for families

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and health care, with deleterious effects on quality of life [4][5]. Medical management of severe constipation with diet modifications, laxatives or enemas can be successful in patients

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with and without anatomical lesions or malformations [11]. Some of these children will present

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to a pediatric surgeon after many years of failed medical management [12]. Previous reports

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suggest that patients and families have a greater chance of success, improved quality of life and

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lower cost when followed early in a BMP [13] [14] [15]. Children who progress to surgical management when less invasive therapies fail, may also experience improved quality of life

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[16][17][18][19], although up to 35% will continue to have bowel issues, requiring additional

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medical or surgical treatment [20][21].

Treatment regimens for severe constipation or fecal incontinence are highly variable with a paucity of long-term follow up data[22]. A consensus of the definition of successful treatment is also lacking among studies. We sought to examine the long-term outcomes of the bowel management program used in our colorectal specialty clinic.

Methods: After obtaining IRB approval, a retrospective chart review was conducted on patients less than 18 years of age who were prescribed a Bowel Management Week (BMW) between

Journal Pre-proof February 2011 and January 2018 at the Pediatric Colorectal Clinic at Primary Children’s Hospital in Salt Lake City, Utah. Data was collected using RedCap and filtered and analyzed in Excel. A BMW consisted of an approximately 7-day period during which patients were placed on a bowel management regimen, received daily KUBs, and frequent phone contact with the treatment team to monitor clinical response and make adjustments based on algorithms

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previously reported [12]. Documentation from the BMW and follow-up visits was recorded, noting whether treatment was successful, unsuccessful or patients were non-adherent. Success

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was defined as no soiling accidents. Fewer than two “smears” on undergarments per week was

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considered success. For children who were not toilet trained or had a stoma present, success

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was defined as a regular stooling pattern. Adherence was defined as following the prescribed

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treatment plan and only adjusting the plan under the guidance of the treatment team. Non-

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adherence was defined as a major deviation to the prescribed treatment plan, such as giving enemas only as needed when they were prescribed daily. Follow-up visits, generally occurring

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at 6-12 month intervals, documented phone communications with our clinic, emergency

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department visits, and surgical interventions related to bowel management were reviewed.

Results: Initial bowel management week A total of 285 (175 male, 110 female) patients who were prescribed a BMW were reviewed. The median age was 7 years (9 months- 17 years). Primary diagnoses included functional constipation (FC) (112), anorectal malformation (ARM) (104), Hirschsprung Disease (HD) (41), rectal prolapse (RP) (14), spina bifida (SB) (6), fecal Incontinence (3), and other (5; 4 sacral

Journal Pre-proof coccygeal teratomas and 1 GSW to the buttocks). The most common ARM diagnoses were rectoperineal and rectovestibular fistula. The most common HD phenotypes were rectosigmoid and sigmoid colon transition zone. A total of 154 patients were started on large volume enemas (124 via rectum, 30 via ACE) and 131 were started on stimulant laxatives (ExLax® or Senna®). At the end of the BMW, 233 patients (87% of adherent patients, 82% of total) were

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successful, 35 (13% of adherent patients, 12% of total) were unsuccessful and 17 (6% of total patients) were non-adherent. Primary diagnosis, initial bowel management regimen, and

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success rate amongst adherent patients at the completion of the BMW are shown in Table 1.

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BMW generally lasted a single week with the average length calculated at 7 days; however, 4%

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of successful patients required ongoing adjustments to the regimen for approximately two

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weeks. There was little difference in outcome based on patient age, primary diagnosis, or bowel

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management regimen (enema versus laxative).

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Long-term results of bowel management program

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A total of 202 patients had at least one follow-up appointment after their initial BMW. Median follow up was 2.5 years (5 weeks to 7.7 years). There were 83 (29%) patients with no follow up data available after the initial BMW. 122 (60%) patients had a one year follow up visit and 98 (50%) had a 2 year follow up visit with success rates amongst adherent patients 72% and 78% respectively (Table 2.). Success rate amongst total patients were 70% at each time point. 21/130 (16%) patients started on enemas, with at least one follow-up, were later successfully transitioned to laxatives and 5/130 (4%) with at least one follow-up were able to discontinue bowel management completely. 13/57 (23%) patients started on laxatives, with at least one

Journal Pre-proof follow-up subsequently required enemas in order to stay clean while 3/57 (5%) with at least one follow up were able to discontinue bowel management completely. 33% of patients eventually had surgery to aid bowel management (ACE=81, resection + ACE=13, diverting stoma=4).

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There was little difference in non-adherence amongst primary diagnosis although nonadherence decreased slightly during consecutive follow up visits (Figure 1). There were 267

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instances of telephone calls between appointments, 43 patients visited the ED for bowel

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management related issues, and 10 patients required manual disimpactions. Sixty patients who

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were on enemas underwent an additional BMW for a laxative trial (50% successful, 5% non-

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adherent and 27% unknown because follow-up had not yet been documented). 13/91 (14%)

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patients were able to successfully discontinue the use of an appendicostomy.

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Discussion: Constipation and fecal incontinence may have severe effects on the quality of life for patients with functional and anatomical colorectal disorders [23] . Our institution, as well as

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others have demonstrated the short-term success of a structured BM program in patients with severe constipation or fecal incontinence from a variety of etiologies[10][24][25]. The success rate amongst adherent patients in our experience after the BMW was 87% which is similar to others reported. The overall success rate was 82%, which is slightly lower. We speculate that this may be related to certain nuances of our program. Specifically, following the initial evaluation we allow patients who do not live locally to return home and complete the program remotely. This results in a less selective group of patients whose chance for success is likely

Journal Pre-proof affected by other daily activities. The benefit of this approach, however, is that families learn to incorporate the bowel management regimen into their routine from the start.

We primarily sought to assess long-term outcomes of our bowel management program. We observed that success rates decrease slightly at one year follow up but that high success rates

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can be achieved at or beyond two years of initiating a bowel management program. Additionally, success rates did not appear to be affected by primary diagnosis or treatment

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regimen used. Our long-term success rates at 1 and 2 years were 72 and 78% respectively. This

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study is unique in that it includes a number of diagnoses and provides follow up data for two or

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more years after starting bowel management. When looking at outcome over the course of

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follow-up visits (as opposed to single time points), we found that the success rate remained relatively consistent while non-adherence consistently decreased (from 23-1%). The increase in

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non-adherence observed at the isolated time points of one and two years represents patients

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who did not follow up routinely after the BMW and then returned to clinic for additional care.

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This suggests that with repeated clinical contact, patients and caregivers may better understand or develop trust in the program. Another important finding of this study was that few patients were able to discontinue the bowel management regimen (4%) during the follow up period.

Having an infrastructure in place for timely patient contact is critical as demonstrated by the number of phone calls which occurred between appointments for bowel management questions or regimen adjustment. Despite these resources, 21% of patients presented to the ED

Journal Pre-proof and 5% required manual disimpaction. While we were not able to elucidate the circumstances resulting in these events, this represents an area for improvement.

Limitations and Further Questions: This was a retrospective chart review of 287 patients and information was limited to that contained within the electronic medical record. Thus, we

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cannot comment on the reasons that some patients were non-adherent or chose not to return for follow up. From our experience, non-adherence can be driven by unpleasant side effects

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such as bloating, difficulty or inexperience with enema administration, and potentially the shift

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of responsibility from parent to teenage child, though our data did not show an age-driven

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difference in non-adherence (not shown). It is interesting to note that non-adherence is nearly

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the same in each treatment group at 6 months and the laxative treatment group nonadherence is higher than the enema group at 1 year and 2 years. Additionally, loss to follow-up

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may be affected by patient distance from clinic, a factor that was not explored in this study.

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These may be opportunities for a future study. Our data on Spina Bifida patients was limited as

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all were lost to follow-up. We have a successful and active Spina Bifida program at our hospital where bowel management is prescribed. All Spina Bifida patients seen in the colorectal clinic are self-referrals who come for additional resources and generally follow up with the Spina Bifida clinic. Also, our definition of success allowed for a patient to have a maximum of 2 smears in the underwear per week. This definition may be considered liberal by some and conservative by others. Parent definitions of success sometimes differ from those of the treatment team. There were instances when the management plan remained the same, despite >2 smears per week because this was acceptable to the parents and/or patients. With parents

Journal Pre-proof and patients happy with an “unsuccessful” outcome and unwilling to make changes, our ability to achieve our definition of success over time was affected. Better understanding of patient and parent expectations and definitions of success is an area for future investigation. For patients who required intervention between visits, we do not have data on whether they were adherent or non-adherent at the time of their ED visits and/or disimpactions preventing us from

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determining the role this played in these outcomes. Possible confounding factors not explored in our long-term data include age, ethnicity, primary language and socioeconomic status. All of

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these had the potential to affect the prescribed treatment plan, trust in the providers, and

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resources available for patient and caregiver adherence and success.

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Conclusions: Children who follow a structured BMP with readily available personnel to provide outpatient assistance can experience successful treatment of severe constipation or fecal

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incontinence long-term. Periodic adjustments to the BMP are necessary and non-compliance

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rates may decrease over time. A multi-institutional collaboration is necessary to identify factors

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which predict failure of a BMP and non-adherence.

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Journal Pre-proof Table 1. Patient Demographics and BMW Outcomes* Enema Success

Laxative Non-Ad

Success

Non-Ad Primary Diagnosis _______________________________________________________________________________________________________ Functional Constipation (n=112) 44/50 (88%) 3/53 (5%) 43/55 (78%) 4/59 (7%) 56/64 (88%)

4/68 (6%)

34/35 (97%)

1/36 (2%)

Hirschsprung’s Disease (n=41)

19/22 (86%)

0/22 (0%)

16/18 (89%)

1/19 (5%)

Rectal Prolapse (n=14)

N/A

N/A

11/12 (92%)

2/14 (14%)

Spina Bifida (n=6)

2/4 (50%)

1/5 (20%)

1/1 (100%)

0/1 (0%)

Fecal Incontinence (n=3)

3/3 (100%)

0/3 (0%)

N/A

N/A

Other (n=5)

2/2 (100%)

1/3 (33%)

2/2 (100%)

0/2 (0%)

126/145 (87%)

9/154 (6%)

107/123 (87%)

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Total (n=285, 175M/110F)

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Anorectal Malformation (n=104)

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*Success was calculated amongst adherent patients

8/131 (6%)

Journal Pre-proof Table 2. Long Term Outcomes* by Primary Diagnosis and Treatment Regimen 6 Month Successful

1 Year

Non-Ad

2 Years

Successful

Non-Ad.

Successful

Non-Ad. Primary Diagnosis _______________________________________________________________________________________________________ Functional Constipation 26/31 (84%) 3/34 (9%) 30/35 (86%) 3/38 (8%) 27/32 (84%) 3/35 (9%) 33/46 (72)%

3/49 (6%)

30/42 (71%)

4/46 (9%)

Anorectal Malformation

20/25 (80%)

1/26 (4%)

Hirschsprung’s Disease

5/11 (45%)

0/11 (0%)

17/25 (65%)

2/28 (7%)

9/11 (82%)

3/14 (21%)

Rectal Prolapse

6/6 (100%)

0/6 (0%)

3/4 (75%)

0/4 ( 0%)

1/1 (100%)

0/1 (0%)

1/1 (100%)

0/1 ( 0%)

1/1 (100%)

0/1 (0%)

1/1 (100%)

0/1 (0%)

1/1 (100%)

0/1 (0%)

Fecal Incontinence

0/1 (0%)

Other

N/A

0/1 (0%) N/A

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Treatment Regimen _______________________________________________________________________________________________________ 38/47 (81%)

3/50(6%)

60/77(78%)

4/81 (5%)

43/58 (74%)

5/63 (8%)

Laxative

19/27 (70%)

1/28 (4%)

26 /37 (70%)

4/41 (10%)

26/30 (87%)

5/35 (14%)

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Enema

57/74 (77%)

4/78 (5%)

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*Success was calculated amongst adherent patients

82/114 (72%)

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Total

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________________________________________________________________________________________________________ 8/122 (7%)

69/88 (78%)

10/98 (10%)

Figure 1