Title: A longitudinal study of factors that impact on the quality of life of intestinal failure patients treated with home parenteral nutrition
Funder: Health Education England

Abstract: Background: Home parenteral nutrition (HPN) is a lifesaving therapy for intestinal failure patients, when the oral or enteral route is unavailable. It refers to the intravenous administration of macronutrients, micronutrients and fluid. The aims of treatment are to increase survival and improve quality of life (QoL). However patients struggle with physiological symptoms (pain, fatigue, dehydration, gastrointestinal discomfort), time-consuming invasive therapy and an increased occurrence of depression and social isolation. Decreased physical health, due to multi-morbidity and complications of treatment such as intestinal failure associated liver disease (IFALD), impacts further their QoL.IFALD is the most serious complication of HPN with rapid progression leading to hepatic failure, followed by a multi-visceral transplant (the simultaneous transplantation of liver, intestine and pancreas) with high morbidity and mortality. Chronic liver disease is a major cause of poor QoL, a contributor to excess death, and a significant concern for patients and the NHS. Despite this, we do not understand the aetiology of IFALD specifically nor the extent to which IFALD affects QoL. Recommendations for the treatment and prevention of the disease are vague and require a better evidence base. Contributory factors may include sub-optimal formulation of the HPN solution; however which component(s) (calories, glucose, lipid, fatty acids and micronutrients) are present in inappropriate concentrations is unknown. Aim: To understand how HPN impacts on the QoL of patients, and the contribution played by liver disease. Plan WP1: Systematically review and evaluate the evidence for different PN solutions What are the relationships between PN solutions and differences in (a) QoL; (b) markers of health status including liver enzymes, nutritional status, inflammatory and anti-oxidant markers, and (c) clinical safety and tolerance.WP2: A survey of current practice, attitudes and knowledge. Identify (a) the importance of QoL assessment from a clinician perspective; (b) frequency and method of QoL assessment; (c) management of QoL issues Explore (a) clinician views on IFALD as a contributor to poor QoL; (b) management of IFALDWP3: Longitudinal investigation of the QoL of patients treated with HPN Quantify (a) the QoL deficit in HPN patients; (b) baseline QoL scores in new HPN patients; and (c) longitudinal changes in QoL scores Identify which factors contribute to the QoL deficit and the links between them.The primary outcome is mean change in QoL scores over 12 months. The secondary outcome is how factors including liver function, number of PN infusions per week, formulation of PN, and nutritional status impact on QoL scores.Outputs: The long-term vision is to develop a patient-led, clinical service that supports patients to live their best life. This project represents the essential first step towards achieving this vision. In a largely evidence-free zone, this research will allow us to understand the contributors to poor QoL, how to treat them, and therefore provide a framework for future intervention studies. It is intended that subject to approval of a later grant application (NIHR Clinical Lectureship), a complex intervention study will follow.

Lay abstract: Background: Food and drink provide the nutrients we need to stay alive. Intestinal failure occurs when a person's gut (intestine) is unable to process these nutrients. Treatment involves replacing food and drink with a nutritional solution. This is given directly into the person's blood through a tube into their veins ('parenteral nutrition'). Patients are taught how to administer this at home. They normally infuse it overnight, affecting sleep. In England, there are over 2500 people treated with home parenteral nutrition (HPN). Numbers are rising by 20% each year.Parenteral nutrition radically changes a person's life. It's time-consuming and invasive. Patients struggle with ongoing symptoms of their underlying condition, live with multiple health problems and consequently are required to manage a range of drug and lifestyle management regimes. This inevitably has severe consequences for their quality of life. Further impacting on this, are the life-threatening consequences of parenteral nutrition. The most serious is if the liver begins to fail (intestinal failure associated liver disease). This is often due to fat deposits within the liver. If this occurs, it can rapidly develop into the need for a liver and gut transplant. Such surgery carries high risk of complications and death. The long-term vision is to develop a patient-led, clinical service that supports patients to live their longest and best life. Previous research demonstrates that patients have poor quality of life. However, very little is known about the causes of poor quality of life, nor how to treat them and therefore patients continue to struggle. Specifically, how liver disease develops, and the extent to which it affects the quality of life of patients is poorly understood. Contributory factors are thought to include the ingredients of the parenteral nutrition solution which are often variable, perhaps owing to a lack of consensus in the clinical guidance available.This project represents the essential first step towards achieving this long-term vision. In a largely evidence-free zone, this research will allow us to understand the contributors to poor quality of life, how to treat them, and therefore provide a framework for future intervention studies.Aim: To understand how parenteral nutrition impacts on the quality of life of patients, and the contribution played by liver disease.Methods: There are three parts to the proposed research: Review and summarise the evidence for different nutritional solutions, to understand how each affects quality of life, liver function and clinical outcomes Conduct a survey of clinicians across England who manage patients on HPN to understand how quality of life is being addressed and measured Investigate the quality of life of patients treated with home parenteral nutritionQuality of life will be measured using validated questionnaires at study commencement and 12 months later. Detailed analysis will be performed to understand which factors affect scores, and the potential pathways between them. For example, length of time on treatment, dietary intake, number of nutrition infusions per week, liver function.Patient and public involvement: Ten patients with intestinal failure have been involved in designing the project so that it is relevant to themselves and their families. Six have volunteered to form a patient panel for the duration of the project.Dissemination: Panel members will be involved in sharing research findings to benefit patients, their families and the NHS: To guide future research aimed at improving the factors that affect quality of life; To guide future recommendations for home parenteral nutrition services; To improve patient experience of nutritional and medical care; To support patients to engage in research, increasing confidence and enhancing experience.

Research activity codes: 3.3 NUTRITION AND CHEMOPREVENTION;7.1 INDIVIDUAL CARE NEEDS
Health categories: ORAL AND GASTROINTESTINAL