Short bowel syndrome and the impact on patients and their families: a qualitative study.

BACKGROUND: Short bowel syndrome (SBS) is a malabsorptive condition that can result in intestinal failure (SBS-IF). Many patients with SBS-IF require home parenteral nutrition (PN) for survival. However, PN has profound effects on patients and their family members. The present study aimed to understand the lived experience of SBS-IF for patients and their families. METHODS: In-depth semi-structured qualitative interviews were conducted with 15 patients with SBS-IF and five adult family members living with someone with SBS-IF. A patient-centric approach was taken, with a patient steering group providing input and guidance to develop the interview guide. Key concepts were identified using thematic analysis of interview transcripts. RESULTS: Patients' lives were dominated by having SBS-IF. They described physical impacts that included patient-reported signs and symptoms and physical restrictions comprising of restrictions on daily life, actives of daily living and physical functioning. In addition, they encountered emotional impacts with a plethora of negative feelings and social impacts, such as difficulties socialising and maintaining relationships. Patients coped by adapting their life around SBS-IF, having support and adopting an attitude of gratitude and acceptance. Family members were also affected and, along with patients, appreciated the respite of a night off from infusions. CONCLUSIONS: Patients and families face many difficulties with SBS-IF. Healthcare professionals can support patients by facilitating them explore what others have found beneficial; adapting their life around PN, viewing PN with acceptance and trying to cultivate gratitude. Further research into the support required for families may be beneficial.

Factors Associated with Health-Related Quality of Life in Children with Intestinal Failure.

OBJECTIVE: To evaluate disease-specific and age-related factors contributing to health-related quality of life (HRQOL). in children with intestinal failure. STUDY DESIGN: A prospective study of HRQOL was performed in a regional intestinal rehabilitation program. Parent-proxy Pediatric Quality of Life Inventory surveys were administered annually to families of 91 children with intestinal failure over a 6-year period. Survey data was stratified by age and compared with pediatric HRQOL data in healthy and chronically ill populations. Linear mixed-effect models using multivariable regression were constructed to identify associations with HRQOL. RESULTS: A total of 180 surveys were completed by 91 children and their families. HRQOL scores were lowest for children ages 5-7 years (P < .001) and 8-12 years (P < .01), and these changes were primarily related to school dimension scores. In multivariable regression, age of 5 years and older and developmental delay were independently associated with lower HRQOL scores. The trend toward lower HRQOL scores parallels reference data from healthy and chronically ill children, although patients with intestinal failure scored lower than both populations at school age. CONCLUSIONS: Children with intestinal failure experience lower parent-proxy HRQOL scores in the 5-7 and 8-12 year age groups primarily related to school dimension scores. Multicenter data to validate these findings and identify interventions to improve QOL for children with intestinal failure are needed.

Neonatal Intestinal Failure Is Independently Associated With Impaired Cognitive Development Later in Childhood.

OBJECTIVE: The impact of pediatric intestinal failure (IF) on neurodevelopment beyond infancy has not been systematically studied. Our aim was to evaluate cognitive and motor impairment and to identify risk factors for adverse outcomes among children with IF. METHODS: We conducted a cross-sectional single-center study at the Helsinki University Children's Hospital. Patients with IF with >60 days of parental nutrition (PN) dependency aged between 3 and 16 years (n = 40) were invited to participate. The cognitive and motor skills were evaluated using validated tests: Wechsler Preschool and Primary Scale of Intelligence, 3rd edition, Wechsler Intelligence Scale for Children, 4th edition, and Movement Assessment Battery for Children, 2nd edition. RESULTS: All the patients attending the study tests (n = 30, males = 24) were included. Their median age, gestational age, and birth weight was 7.5 (range 3-16) years, 35 (interquartile range [IQR] 28-38) weeks and 2238 (IQR 1040-3288) grams, respectively. Median duration of PN was 13 (IQR 5-37) months and 9 patients were currently on PN. Median intelligence quotient was 78 (IQR 65-91) and 10 (35%) patients had an intelligence quotient under 70 (-2 standard deviation). Significant motor impairment was detected in 10 patients (36%) and milder difficulties in 8 (28%). Adverse cognitive outcome was associated with neonatal short bowel syndrome, number of interventions under general anesthesia, and length of inpatient status, whereas adverse motor outcome was associated with prematurity. CONCLUSION: Clinically significant cognitive and motor impairments are alarmingly common among neonatal patients with IF. We recommend early neurodevelopmental follow-up for all children with IF.

Impact of Short Bowel Syndrome on Quality of Life and Family: The Patient's Perspective.

BACKGROUND: Short bowel syndrome (SBS) has an impact on children and their families not only physically, but also emotionally, mentally, and socially. This study aimed to evaluate quality of life and family impact in patients with SBS, using the Pediatric Quality of Life Inventory (PedsQL) measurement model. MATERIALS AND METHODS: PedsQL questionnaires were administered to patients with SBS followed at the Pediatric Surgery of Royal Manchester Children's Hospital. The scores were compared between two groups differing in age (children <5 y vs. >5 y) and with known-groups from literature published by Varni et al. RESULTS: Forty-three patients were sent the questionnaires, and 30 (70%, 17 < 5 and 13 > 5) responded. Family Impact Module failed to distinguish between families of children younger and older than 5 years of age. In contrast, it distinguished between families with SBS children and the ones with children in a long-term care facility. Healthcare satisfaction was worse in families of children over 5 years regarding information received, inclusion of the family, and communication. When compared with normal population, SBS children scored worse on Generic Core Scales for all domains except for emotional functioning. CONCLUSION: Although advances have been made on the treatment of children with SBS, and improvements have been reached on home PN, this condition is still significantly affecting the quality of life of children and their families. The future quality control of medical care must have greater focus on psychosocial and emotional functioning, aiming for the best possible quality of life.

Health-related quality of life and neurodevelopmental outcomes among children with intestinal failure.

Treatment results of pediatric intestinal failure have improved markedly during the last decades. With improved survival the attention is turning to other essential outcomes including quality of life and neurodevelopment. So far, relatively few studies with limited number of patients and variable methodology have addressed these issues. Based on these studies using generic health related quality of life tools, children with intestinal failure demonstrate decreased physical health, while PN-dependence is also associated with compromised emotional functioning. Impairments of social functioning are frequently observed among older children and parents. Few recent studies on neurodevelopment imply significant impairments in motor and mental skills among children with intestinal failure despite small sample sizes and limited follow-up times. Development of a disease-specific survey designed for the pediatric intestinal failure population could better reveal the health issues with greatest impact on quality of life. Robust studies with appropriate methodology on neurodevelopment in pediatric intestinal failure with extended follow-up times are urgently needed. Quality of life and neurodevelopment requires greater attention from medical professionals managing children with intestinal failure.

Parenteral approaches in malabsorption: Home parenteral nutrition.

Severe malabsorption of fluids and nutrients leads to intestinal failure (IF) where intravenous supplementation of nutrients and fluids is necessary to maintain health and/or growth. Long-term treatment of IF implies the start of intravenous support in the outpatient setting (home parenteral nutrition, HPN). Although HPN has proven lifesaving for many patients for more than four decades this strategy remains associated with complications that compromise the quality of life. Many problems relate to the presence of the venous access device and concern infections or vascular occlusion due to thrombosis. Patient training remains key to prevent these complications. Also metabolic problems may arise that involve liver function or composition or bone mineralization. While intestinal transplantation remains inferior to HPN as alternative treatment strategy in terms of survival, promising developments include the introduction of hormones that promote intestinal adaptation, mixed lipid emulsions that decrease liver problems and catheter lock solutions that prevent infections.

Psychosocial impact of intestinal failure: a familial perspective.

Intestinal failure is a complicated long-term condition that can have profound physical and psychosocial consequences for a patient's life. Hospitalisation can be long, with many distressing and unpleasant medical procedures being endured. Patients often have to deal with large volumes of unmanageable faecal fluid from stomas or fistulae and usually require home parenteral nutrition (HPN) for the remainder of their life. The literature focuses on how patients often have to tolerate long periods of suffering, which can be the basis for significant emotional anguish. It also addresses how the condition can contribute to myriad psychosocial difficulties, which can have devastating effects on their body image and quality of life. However, there is a lack of information regarding the effects of this condition on the family, who not only have to provide ongoing support for the patient, but also have to attempt to function normally while coping with their own fears and emotions. This article reviews the experiences of two patients, father and daughter, living with intestinal failure; it discusses their experiences and reveals how they cope with the physical aspects. It also addresses how the condition can effect psychosocial disturbances, not only for them but for those living closely with them. The article addresses ways that nurses could develop their relationship with the patient, enabling them to recognise and act early on signs of emotional malady.

Key issues in addressing the clinical and humanistic burden of short bowel syndrome in the US.

BACKGROUND: The purpose of this analysis was to provide a concise report of the literature on the burden of intestinal failure associated with short bowel syndrome (SBS-IF) in adults, focused on clinical and humanistic issues important to clinicians and payers. SCOPE: A literature search was performed using the National Library of Medicine PubMed database ( http://www.ncbi.nlm.nih.gov/pubmed ) with the search term 'short bowel syndrome' limited to adult populations and English-language reports published from January 1, 1965, to January 18, 2013. Citations were assessed for relevance and excluded articles focused on single case studies, colon fermentation, absorption of medications with PN/IV, surgical technique, mesenteric artery complications/surgery, and transplantation focus. Additional hand searches were performed using the terms 'short bowel syndrome' AND 'cost', and 'home parenteral nutrition' AND 'cost', along with the exclusion criteria described above. FINDINGS: Despite advances in management in recent decades, SBS-IF continues to carry a high burden of morbidity and mortality. In the absence of sufficient intestinal adaptation following resection, many patients remain dependent on long-term parenteral nutrition and/or intravenous fluids (PN/IV). Although potentially life saving, PN/IV is costly, invasive, and associated with numerous complications and deleterious effects on health and quality of life. Surgical interventions, especially intestinal transplantation, are costly and are associated with substantial morbidity and high mortality. New therapies, which show promise in promoting intestinal rehabilitation and reducing dependence on PN/IV therapy, are the subject of active research. CONCLUSIONS: Overall, the available literature suggests that although SBS-IF affects a relatively small population, the clinical and humanistic burden is significant, and there is an unmet need for effective therapeutic options that target the underlying problem of inadequate absorptive capacity of the remaining intestine. Consequently, many patients with SBS-IF remain dependent on long-term PN/IV support, adding to the burden imposed by the underlying disorder.

Measuring oral sensitivity in clinical practice: a quick and reliable behavioural method.

This article aims to offer a behavioural assessment strategy for oral sensitivity that can be readily applied in the clinical setting. Four children, ranging in age and with a variety of developmental and medical problems, were used as test cases for a task analysis of tolerance to touch probes in and around the mouth. In all cases, the assessment was sensitive to weekly measures of an intervention for oral sensitivity over a 3-week period. Employing an inexpensive, direct, specific to the individual, replicable, reliable, and effective measure for a specific sensory problem would fit better with the edicts of evidence-based practice. The current method offered the initial evidence towards this goal.

Impact of infantile short bowel syndrome on long-term health-related quality of life: a cross-sectional study.

BACKGROUND/PURPOSE: Studies on the long-term effects of short bowel syndrome (SBS) on the quality of life are scarce. Therefore, we determined health-related quality of life (HRQoL) in children and adolescents with a history of infantile SBS compared with that of same-aged controls drawn from the general population. METHODS: Cross-sectional assessment (2005-2007) of HRQoL in children with a history of infantile SBS treated in their first year of life, born between 1987 and 2002, using generic HRQoL measures was performed. Children aged 5 to 18 years and their parents filled out the Pediatric Quality of Life Inventory. Differences in scores between subjects and controls were analyzed using independent sample t tests. RESULTS: Thirty-one children with a history of SBS (19 girls and 12 boys; mean age, 11.8 ± 4.2 years) participated, giving an overall response rate of 70%. The children and their parents reported significantly lower HRQoL than 275 healthy age-matched children and their parents (P < .05). CONCLUSIONS: Children with a history of infantile SBS have a lower HRQoL than healthy age-matched controls. This difference lasts beyond childhood and way beyond the period of intestinal rehabilitation early in life. These findings highlight the need for targeted interventions to address these dimensions of impaired HRQoL.

The meaning of food and eating among home parenteral nutrition-dependent adults with intestinal failure: a qualitative inquiry.

Using content and interpretative phenomenological analysis, we explored the meaning of food and eating from the perspective of adults receiving home parenteral nutrition (PN). The aim of this research was to obtain a deeper understanding of how issues related to food and eating influence quality of life (QOL). Semistructured telephone interviews were conducted between May 2006 and January 2007 with 24 adults with intestinal failure and home PN dependency. The analysis revealed themes relevant to eating behaviors, hunger and thirst, strategies for dining in restaurants, and a perception of wasting money because of malabsorbed food. Three patterns of eating emerged: eating for survival, eating for health benefits, and eating for socialization. A proposed model illustrates how these eating patterns are linked to QOL. Being able to eat and enjoy food is an important ingredient for good self-reported QOL. Measurements of QOL for this population may be enhanced with inclusion of a food and eating domain. The social and emotional context of food and mealtimes is an important component to address in the nutrition care plan for PN-dependent adults.

An exploration of quality of life and the experience of living with home parenteral nutrition.

INTRODUCTION: The aim of this research was to achieve a deeper understanding of the experience of adults living with home parenteral nutrition (PN) and to define their quality of life (QOL). METHODS: The research design was qualitative, using content and interpretative phenomenological analysis. The sample included adults with intestinal failure, stratified by length of home PN dependency. Sampling continued until data saturation was achieved. A second reviewer independently coded a subset of narratives (kappa = 0.684). RESULTS: Participants included 24 adults receiving home PN because of short bowel syndrome (95.8%) and pseudo-obstruction (4.2%). Twenty-five percent received PN for < 2 years, 20.8% for 2-5 years, 25% for 5-10 years, and 29.2% for > 10 years. Respondents viewed home PN as a "lifeline" and "nutritional safety net." QOL was defined as "enjoying life"; "being happy, satisfied, or content with life"; and "being able to do what you want to do, when you want to do it." Participants described their QOL as "good" to "wonderful." Lifestyle was affected by health, stamina, diarrhea, having an ostomy, and the amount of flexibility there was with the infusion schedule. There was a strong desire to achieve normalcy in life among all participants. CONCLUSIONS: Qualitative methodology provides new insights and richness of data regarding adults receiving home PN. The positive description of QOL in this study contrasts with the published nutrition literature. It is important for healthcare practitioners to understand and discuss the realities of home PN and lifestyle adaptation.

Quality of life in short-bowel syndrome: impact of fatigue and gastrointestinal symptoms.

OBJECTIVE: Patients with short-bowel syndrome (SBS) have impaired health-related quality of life (QoL). However, comparisons of QoL data with the data on other chronic gastrointestinal diseases are not available. The aim of this study was to assess QoL in SBS patients compared with that in the general population and with patients with inflammatory bowel disease (IBD). The potential relation between fatigue and gastrointestinal symptoms and impaired QoL in these patients was also investigated. MATERIAL AND METHODS: Four validated questionnaires were used to measure aspects of QoL (SF-36), psychological distress (hospital anxiety and depression scale, HAD), fatigue (fatigue impact scale, FIS), and gastrointestinal symptoms (gastrointestinal symptom rating scale, GSRS) in 26/28 patients (93%) attending a SBS clinic (median age 62 years, 15 F/11 M) at a tertiary referral center. Persons from the general population (n=286) as well as patients with IBD (n=41) of similar age and gender distribution as the SBS group acted as controls. RESULTS: SBS patients had significantly lower SF-36 physical and mental component summaries than those in the general population as well as significantly lower SF-36 physical (p<0.05) but not mental (p>0.05) component summaries compared with those of IBD patients. Fatigue and gastrointestinal symptoms were more severe in SBS patients than in IBD patients (p>0.05). The SF-36 physical component summary was independently related to the physical FIS dimension (beta=-0.4, p=0.004), the GSRS eating dysfunction dimension (beta=-0.31, p=0.025), and opiate use (beta=-0.28, p=0.031), regardless of diagnosis (SBS or IBD). CONCLUSIONS: Patients with SBS show poor QoL compared with that in the general population and also impairment of mainly physical health compared with that in patients with IBD. Fatigue and gastrointestinal symptoms are more severe in patients with SBS, which has an impact on QoL.

Living long with short bowel syndrome: a historical case of twenty-nine years of living with home parenteral nutrition.

BACKGROUND: This paper traces the 29-year survival of Robert Thomas, who received home parenteral nutrition (PN), and contrasts his oral narrative with the clinical history of PN. METHODS: Interviews, chart review, review of the literature, and historical analysis. RESULTS: Bobby Thomas was part of an early group of patients scattered throughout the country who, with their medical team, provided the foundation for more successful survival with home PN. They learned together and taught numerous nutrition support clinicians the intricacies of patient management. The importance of the patient to the teaching function of new and experienced practitioners is highly critical. Patients like Robert Thomas gave practitioners firsthand evidence of both the tenacity of the human spirit and the complexity and difficulties of chronic illness and its treatments. CONCLUSIONS: While Bobby struggled with the complications and difficulties that came with the disease and the treatment keeping him alive, his own experiences over 29 years, as told to his medical team during his treatment and to informed interviewers before he died, tell a story that is both intersecting and parallel to the medical history. Pioneering patients like Bobby Thomas confirm the possibility of survival. They also, through their own negotiations to maintain a sense of control, can live lives they themselves help define.

The SF-36 instrument for the follow-up of health-related quality-of-life assessment of patients undergoing home parenteral nutrition for benign disease.

INTRODUCTION: The non-disease-specific SF-36 instrument was applied to detect changes in quality of life among patients at a single center who were receiving home parenteral nutrition (HPN) for intestinal failure. METHODS: All HPN patients completed the questionnaire during routine visits. The SF-36 examines eight domains (scores: 0% to 100%): physical functioning (PF), role-functioning physical (RP), body pain (BP), general health (GH), vitality (VT), social functioning (SF), as well as role-functioning emotional (RE) and mental health (MH). Patient responses were standardized for the sex- and age-matched group scores of the healthy population by calculating the Z-score: (healthy population mean score - patient raw score)/healthy population standard deviation (reduced value: Z-score < -1). A difference in a domain score was considered significant in the individual patient if it was greater than the 90% confidence interval for healthy subjects and if it produced a change in Z-score category. General, sociodemographic, underlying disease and HPN-related factors were collected. RESULTS: For 31 enrolled adult patients, the baseline mean Z-score was reduced in five domains: PF, RP, BP, GH and SF. Twenty patients underwent a second SF-36 assessment 10 +/- 2 months later: the HRQOL worsened in eight and improved in 10 (no. worsened domains > no. improved or vice versa). The worsened subgroup showed a decrease in body mass index (P =.104), an increase in the number of HPN infusions per week (P =.065), and a greater incidence of intestinal motility disorders (P =.161), of liquid oral diet (P =.027), and of nycturia (P =.028). CONCLUSIONS: The SF-36 instrument reflecting the generic HRQOL assessment may be a suitable tool for patients on HPN.

Quality of life and concerns in patients with short bowel syndrome.

BACKGROUND & AIMS: the life situation in patients with short bowel syndrome was described, with focus on aspects of quality of life, worries, concerns and coping. METHODS: Twenty-eight patients (19F/9 M) participated. Mean age was 54. Eight were on home parenteral nutrition (HPN). Quality of life was recorded using a visual analogue scale. Health-related quality of life (HRQOL) was assessed using Short Form 36 (SF -36) and compared with matched controls. Concerns were assessed using Rating Form of Inflammatory bowel disease patient concerns (RFIPC). Coping strategies were investigated using Jalowiec coping scale (JCS 40). RESULTS: Greatest concerns were fear of being a burden, having surgery and loss of energy. HRQOL was significantly reduced compared to controls. Patients' receiving HPN rated quality of life lower than those without HPN. The presence of a stoma appeared not to influence quality of life negatively but stoma patients expressed more concern. Confrontational coping style was most frequently used. CONCLUSION: HRQOL was low both in the HPN group and the non-HPN group. Having a stoma did not affect quality of life negatively. Fear of being a burden was the most frequently expressed concern, in the whole group as well as in subgroups. This information should be integrated into the care of these patients.