Short bowel syndrome (SBS) refers to the malabsorption of fluids, nutrients, and electrolytes seen following extensive bowel resection (1). It should be regarded as a multisystemic disease. It has an estimated incidence of 24.5/100,000 live births (2). Malabsorption results in a plethora of symptoms, including diarrhoea, dehydration, fatigue, and failure to thrive. The management of SBS focuses on controlling symptoms, supplementing nutritional intake for the promotion of growth, and avoiding further complications, the most life-threatening of which relate to the use of parenteral nutrition (PN). The degree of intestinal resection and the condition of the remaining bowel determine the route of nutritional supplementation required, although immediate management typically involves a period of PN (3).
Illness can be a substantial burden, with negative repercussions on the patient's physical, emotional, and social well-being. The effect of illness on these aspects of daily life has now been termed “health-related quality of life” (HRQoL). Historically, success following surgery has been measured according to mortality, complication, and reoperation rates, although HRQoL is becoming recognised increasingly as an important marker of a successful surgical outcome (4).
Because patients with SBS are frequently children, the burden of care falls largely on the caregivers to manage their nutritional intake and their daily needs. The high level of care required and the frequent visits to hospitals interfere with daily life as well as being emotionally and physically draining for both child and caregiver. The introduction of home parenteral nutrition (HPN) has had a positive effect on the lives of patients and caregivers and is associated with improved survival rates, a gain in patient strength and body weight, and the requirement of fewer hospital visits (5). The transfer of nutritional management to the home environment also encourages the development of family relationships and is associated with improvements in the ability of the family unit to adapt to and cope with situations effectively (6); despite these advantages, parents of HPN-dependent patients are still reported to have a low quality of life (QoL) (7). Jeppesen et al (8) used the Inflammatory Bowel Disease Questionnaire to assess the effect of the use of HPN on HRQoL, and found that patients requiring HPN had a significantly lower HRQoL compared with controls in the domains of both systemic symptoms and emotional and social function.
In children with SBS, surgical options such as autologous gastrointestinal reconstruction (AGIR) and intestinal transplantation have been introduced hoping to reducing PN dependence and improving the QoL of patients and their caregivers. No study to date, however, has adequately assessed the effect of autologous reconstructive procedures on the QoL of these patients or their caregivers. AGIR in our unit involves initial controlled bowel expansion of the remnant proximal bowel if possible (9) followed by longitudinal intestinal lengthening and tailoring (LILT), as described by Bianchi (10). We have added the serial transverse enteroplasty procedure (STEP) (11) to our armamentarium. These procedures are done in the context of a multidisciplinary approach to the management of SBS. The LILT procedure is technically more challenging than the STEP procedure but is useful in the severe short bowel state because a STEP procedure can be done after a LILT procedure if needed. This would be part of a sequential bowel lengthening programme. It is technically difficult to do a LILT after a STEP and the authors would recommend the use of the LILT first where feasible in children with the severe short bowel state. Occasionally, bowel lengthening may not be feasible and other forms of reconstruction (eg, reverse segments) or anastomosis are used. Because AGIR is frequently performed in the first years of life, patients undergoing AGIR cannot be expected to reliably assess the effect of the procedure on their own QoL; however, because these patients are completely reliant on caregivers, caregivers’ perception of their own and their child's HRQoL and their satisfaction with the surgical outcome are desired measurements of the success of AGIR.
Our pilot study aimed to evaluate caregivers’ perception of the effect of the AGIR on the lives of their child and themselves. We aimed to examine how closely AGIR met caregiver expectations; to determine the effect of AGIR on parental QoL and the perceived HRQoL of their child by comparing preoperative, postoperative, and long-term scores of subjective interpretations of QoL; and to assess the perceived effect of surgery on the daily care and medical needs of their child.
During spring 2010, all of the children who had undergone AGIR at Manchester Royal Children's Hospital between January 1999 and June 2010 were reviewed. Thirty-two children were identified. Seven were assessed outside our centre (5 outside the UK) and were not included in the study. One patient moved to another city and was not contacted. Two children died. Twenty patients were contactable and their caregivers were invited to participate in a questionnaire-based survey.
No validated instruments exist for the specific assessment of QoL in patients with SBS; however, this questionnaire was adapted from a questionnaire by O’Neill et al (12) that assessed the effect of antigastroesophageal reflux procedures on caregivers’ perception of their child's physical well-being and the QoL of their child and themselves by comparing preoperative, postoperative, and long-term values. Changes from the O’Neill et al questionnaire included removing those aspects that related to neurologically impaired children. Questions regarding pulmonary conditions were also removed. We then added questions regarding total PN and weight gain because these are paramount problems in children with SBS. Similarly, we wished to assess the effect of AGIR on these aspects of the daily lives of patients with SBS and their caregivers.
Patients were mailed the questionnaire and were either sent back completed or filled in during short bowel clinics. Completed questionnaires were analysed using SPSS version 18 (SPSS Inc, Chicago, IL). Friedman nonparametric test and 2-way analysis of variance were used to evaluate differences between preoperative and postoperative/long-term values to assess whether surgery had a positive effect on the physical condition of the child and the QoL of both child and caregiver. Comparisons between 6 months postsurgery and long-term postsurgery (last 6 months) were also made to assess whether improvements continued beyond the postoperative period. P ≤ 0.05 was considered statistically significant.
Consent from the caregiver and the patient, where applicable, was either implied by the return of the completed questionnaire or obtained during the clinic. The National Research Ethics Service deemed the study as service evaluation (Ref 04/02) and the study was also supported by the Manchester Medical School.
Thirty-two children were identified. Seven were done outside our centre (5 outside the UK) and were not included in the study. One patient moved to another city and was not contacted. Two children died. Twenty patients were contactable. Of the 20 patients sent the questionnaire, 1 failed to return their questionnaire. All of the 19 patients who returned their questionnaires were included in the study. Table 1 delineates the diagnosis, type of surgery, and bowel length of each of the children.
The first question in our study asked parents to define up to 5 aims they wanted to achieve from surgery and rate how satisfied they felt that these expectations were met (Table 2). “Survival” and a “better quality of life” were the most common aims chosen by parents. Generally, parents found that the surgery met their specific aims well, although 1 parent felt the surgery resulted in only a mild improvement in their child's symptoms. The second part looked at the parents’ overall expectations from the surgery, turning out either as expected or better than their expectations (Table 3). Parents were also given the opportunity to comment on the overall outcome of their child's surgery. Several comments praised the outcome of the surgery and the support of the multidisciplinary team. One parent commented, “Information given and answers to our questions before and after surgery gave us a great sense of support.” Other parents expressed a wish for a referral for their child to a plastic surgeon following negative comments made about the scarring left after surgery.
We asked caregivers to rate their child's preoperative, postoperative, and recent bowel habits, physical comfort, ability to enjoy life, and developmental progress (Table 4). These were divided into 3 stages. The preoperative stage was regarded as 6 months before surgery and the postoperative stage was 6 months after surgery. The most recent stage was placed during the last 6 months before the questionnaire (on average 4.38 years, ranging from 0.59 to 11 years after surgery). Parents perceived a significant improvement in the child's bowel habit in the 6 months following surgery, although beyond that period, further improvement was limited. Eighty-nine percent of caregivers reported their child's recent bowel habits as either good or excellent.
There was also statistically significant improvement seen in the child's physical comfort, his or her ability to enjoy life, and his or her developmental progress in the 6 months following surgery. These positive changes were also perceived to continue beyond the postoperative period.
Since their child's surgery, parents and caregivers have found continuing improvement in the ease of caring for their child, and thus there was improvement in their own and the child's QoL (Table 5). Correspondingly, they perceived that the time spent in caring for the child's needs and in the frequency of visits and calls to health care providers (Table 6) has become increasingly more manageable.
Parents have also reported that in the 6 months following surgery, more quality time was spent with their child, they were better able to enjoy their child, and they experienced less frustration towards their child. During this time, they also felt more optimistic about their own and their child's future and less concern regarding their ability to look after their child compared with their attitudes before surgery.
Management of children with SBS is complex and requires multidisciplinary input from specialist teams, but it is the parents who are inevitably responsible for the daily care of their child, which revolves around managing their fluid and nutritional intake, managing their trips to the toilet, and meticulously planning the day to make the most of their time (13). This daily routine can be demanding for both parent and child. Despite its advantages, caregivers of patients receiving HPN still report low mood, which they associate with frequently having to miss out on activities to accommodate its use, and concern about the risk of infection of their venous line. Therefore, to be considered successful, surgical options should not only improve survival rates but also reduce PN dependence and its negative effect on the QoL of both child and caregiver. In our study, when parents were asked to define their expectations from surgery, almost as many parents replied that they wished for a better QoL for their child as survival (Table 2), illustrating the importance of measuring QoL following surgery. AGIR is now widely accepted as a treatment option for SBS, but its effect on QoL remains inadequately assessed.
The present study investigated the effect of AGIR on caregivers’ perception of the physical well-being and medical needs of their child using several domains taken from the O’Neill et al questionnaire. This measure also provided a means for parents to rate QoL aspects relating to both their child and themselves. The O’Neill et al article was chosen because it was designed to investigate parameters of QoL following surgery in children, which we have applied to the use of the AGIR in SBS. One of the questions asked caregivers to rate their child's pre- and postoperative bowel habits. Published results following AGIR typically do not report the effect of surgery on bowel habits, although it could be reasonably assumed that because of increases in intestinal length and absorption seen post-AGIR (14), patients should see a corresponding improvement in their bowel habit. Caregivers perceived that bowel habit improved in the 6 months following AGIR (Table 4). This is presumably attributable to the lengthening effect of AGIR, although it is most likely enhanced by intestinal adaptive changes that occur postintestinal resection. After the 6-month postoperative period, there was limited further improvement seen, which could indicate that further adaptive intestinal changes occurring were also limited beyond this period.
Following surgery, caregivers reported improvements in the ease of caring for their child, their ability to enjoy their child, the quality of time spent with the child, and their level of frustration towards their child postoperatively. Medically, they also believed that they were spending a decreasing amount of time caring for their child's medical needs and attending hospital visits. Caregivers noted a consistent improvement in their child's comfort, their ability to enjoy life, and their development. This perceived improvement in the child's ability to enjoy life is likely to be influenced by many factors, including the improvement in bowel habit and physical well-being and achieving their developmental milestones, allowing them to play with other children and enabling them to do things other children of their age can do.
It is acknowledged that the present study would have benefited from the use of a standardised QoL measure that is relevant to paediatrics such as the PedsQL (15). The measures used, however, allowed limited assessment of specific QoL-related factors and gave an indication of parental and child QoL according to parental subjective interpretations of this concept. The measures chosen also indicated that parents place importance on QoL as an outcome from surgery. We hope to build on this pilot by repeating the study in a larger sample size and including the use of a standardised QoL measure.
A limiting factor of the present study is that the assessment of the child's medical requirements was subjective and may have been influenced by several personal factors. Unfortunately, because the present study was conducted anonymously, an objective assessment of the number of visits made to health care professionals could not be made to compare parental perception of medical needs to medical records. Furthermore, O’Neill et al proposed that improvements in the parent–child relationship following surgery could be attributable to the advancing age and development of their child. Parents did notice consistent improvements in their child's development following surgery, which may in part account for their ability to enjoy their child more. In our study, however, the statistical improvement in aspects of the parent–child relationship seen was attributable to perceived improvements in the first 6 months after surgery, which is more likely to result from the success of the surgery than the advancing age of the child.
The small number of subjects in our cohort does limit the confidence in our findings; however, the fact that all of the patients were included and not specifically selected makes our cohort more likely to be representative of the outcomes seen following AGIR. Furthermore, the statistical significance was so high in each domain that we can reasonably reject the null hypotheses and assume that AGIR has a positive effect on QoL in the domains that we specified.
The design of our questionnaire was a retrospective assessment of certain QoL domains. Because AGIR was performed for some patients several years before the present study, some parents may have had difficulty recalling accurately the medical needs of their child around the time of their surgery. In general, patients seemed to be able to recall this period adequately; however, if the present study were to be repeated, we would consider a longitudinal study, although this may not be a feasible option because with such a small cohort, any patients lost during this time period would limit the sample size and significantly affect results. We considered the effect that social desirability bias, which is the bias of respondents to portray themselves or others in a favourable way, may have had on our study. Caregivers appeared to avoid the tendency to portray themselves in a better light because responses were honest and described their enjoyment of their child, the quality of time spent with them, and their feelings of frustration towards their child as all subjectively poorer preoperatively. We hope that by making the study anonymous we have avoided any tendency for caregivers to improve scores because of bias towards their surgeon.
AGIR has been used in our unit as a treatment option for SBS for the past 30 years and has undergone several modifications in the context of an intestinal rehabilitation programme. Of importance in determining the success of surgery is the knowledge that it has a positive effect on the general well-being of the child and the QoL of patients and their caregivers. From our results, we have managed to gather several impressions; as perceived by parents, AGIR was a success and met their expectations well. Parents believed that the surgery had a positive effect on both the physical condition and the daily life of their child, and following AGIR they saw improvement in several aspects of their relationship with their child. These results indicate that AGIR can lead to a significant improvement in the lives of both these children and their caregivers across the domains measured and, as such, should be considered as part of the management of children with SBS.
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Keywords:Copyright 2012 by ESPGHAN and NASPGHAN
quality of life; short bowel syndrome; bowel reconstruction