Janet P. Baxter

ESPEN Guidelines on Parenteral Nutrition: Home Parenteral Nutrition (HPN) in adult patients

Home parenteral nutrition (HPN) was introduced as a treatment modality in the early 1970s primarily for the treatment of chronic intestinal failure in patients with benign disease. The relatively low morbidity and mortality associated with HPN has encouraged its widespread use in western countries. Thus there is huge clinical experience, but there are still few controlled clinical studies of treatment effects and management of complications. The purpose of these guidelines is to highlight areas of good practice and promote the use of standardized treatment protocols between centers. The guidelines may serve as a framework for development of policies and procedures.

Long-term follow-up of patients on home parenteral nutrition in Europe: implications for intestinal transplantation

Background The indications for intestinal transplantation (ITx) are still debated. Knowing survival rates and causes of death on home parenteral nutrition (HPN) will improve decisions. Methods A prospective 5-year study compared 389 non-candidates (no indication, no contraindication) and 156 candidates (indication, no contraindication) for ITx. Indications were: HPN failure (liver failure; multiple episodes of catheter-related venous thrombosis or sepsis; severe dehydration), high-risk underlying disease (intra-abdominal desmoids; congenital mucosal disorders; ultra-short bowel), high morbidity intestinal failure. Causes of death were defined as: HPN-related, underlying disease, or other cause. Results The survival rate was 87% in non-candidates, 73% in candidates with HPN failure, 84% in those with high-risk underlying disease, 100% in those with high morbidity intestinal failure and 54%, in ITx recipients (one non-candidate and 21 candidates) (p<0.001). The primary cause of death on HPN was underlying disease-related in patients with HPN duration ≤2 years, and HPN-related in those on HPN duration >2 years (p=0.006). In candidates, the death HRs were increased in those with desmoids (7.1; 95% CI 2.5 to 20.5; p=0.003) or liver failure (3.4; 95% CI 1.6 to 7.3; p=0.002) compared to non-candidates. In deceased candidates, the indications for ITx were the causes of death in 92% of those with desmoids or liver failure, and in 38% of those with other indications (p=0.041). In candidates with catheter-related complications or ultra-short bowel, the survival rate was 83% in those who remained on HPN and 78% after ITx (p=0.767). Conclusions HPN is confirmed as the primary treatment for intestinal failure. Desmoids and HPN-related liver failure constitute indications for life-saving ITx. Catheter-related complications and ultra-short bowel might be indications for pre-emptive/rehabilitative ITx. In the early years after commencing HPN a life-saving ITx could be required for some patients at higher risk of death from their underlying disease.

Survival of Patients Identified as Candidates for Intestinal Transplantation: A 3-Year Prospective Follow-Up

BACKGROUND & AIMS The US Medicare indications for intestinal transplantation are based on failure of home parenteral nutrition. The American Society of Transplantation also includes patients at high risk of death from their primary disease or with high morbidity intestinal failure. A 3-year prospective study evaluated the appropriateness of these indications. METHODS Survival on home parenteral nutrition or after transplantation was analyzed in 153 (97 adult, 56 pediatric) candidates for transplantation and 320 (262 adult, 58 pediatric) noncandidates, enrolled through a European multicenter cross-sectional survey performed in 2004. Kaplan-Meier and chi-square test statistics were used. RESULTS The 3-year survival was 94% (95% CI, 92%-97%) in noncandidates and 87% (95% CI, 81%-93%) in candidates not receiving transplants (P = .007). Survival was 80% (95% CI, 70%-89%), 93% (95% CI, 86%-100%), and 100% in parenteral nutrition failure, high-risk primary disease, and high-morbidity intestinal failure, respectively (P = .034). Fifteen candidates underwent transplantation. Six died, including all 3 of those who were in hospital, and 25% of those who were at home at time of transplantation (P = .086). Survival in the 10 patients receiving a first isolated small bowel transplant was 89% (95% CI, 70%-100%), compared with 85% (95% CI, 74%-96%) in the candidates with parenteral nutrition failure not receiving transplants because of central venous catheter complications, or 70% (95% CI, 53%-88%) in those with parenteral nutrition-related liver failure (P = .364). CONCLUSIONS The results confirm home parenteral nutrition as the primary therapeutic option for intestinal failure and support the appropriateness and potential life-saving role of timely intestinal transplantation for patients with parenteral nutrition failure.

Manganese requirement and toxicity in patients on home parenteral nutrition

Two patients who were receiving home parenteral nutrition complained of vague neurological symptoms of such severity that they underwent full clinical appraisal. The only positive finding was that plasma manganese concentrations were greater than twice the upper 95% confidence interval of normal (7-27|nmol/l). In the light of this result all nine patients receiving home parenteral nutrition underwent evaluation for possible manganese toxicity. One other patient had serum manganese concentrations exceeding twice the upper limit (127|nmol/l). The three patients with elevated serum Mn had evidence of manganese deposition in the brain on magnetic resonance imaging scanning. In contrast two patients with normal plasma results had negative scans. Patient susceptibility appears very variable. We suggest that current amounts of trace elements provided in nutrition solutions may be a potential source of nutrient activity. The fine tuning of supply and demand may be difficult on account of a limited range of commercially available trace element solutions.

The prognosis of incurable cachectic cancer patients on home parenteral nutrition: a multi-centre observational study with prospective follow-up of 414 patients

BACKGROUND The role of home parenteral nutrition (HPN) in incurable cachectic cancer patients unable to eat is extremely controversial. The aim of this study is to analyse which factors can influence the outcome. PATIENTS AND METHODS We studied prospectively 414 incurable cachectic (sub)obstructed cancer patients receiving HPN and analysed the association between patient or clinical characteristics and surviving status. RESULTS Median weight loss, versus pre-disease and last 6-month period, was 24% and 16%, respectively. Median body mass index was 19.5, median KPS was 60, median life expectancy was 3 months. Mean/median survival was 4.7/3.0 months; 50.0% and 22.9% of patients survived 3 and 6 months, respectively. At the multivariable analysis, the variables significantly associated with 3- and 6-month survival were Glasgow Prognostic Score (GPS) and KPS, and GPS, KPS and tumour spread, respectively. By the aggregation of the significant variables, it was possible to dissect several classes of patients with different survival probabilities. CONCLUSIONS The outcome of cachectic incurable cancer patients on HPN is not homogeneous. It is possible to identify groups of patients with a ≥6-month survival (possibly longer than that allowed in starvation). The indications for HPN can be modulated on these clinical/biochemical indices.

A review of the instruments used to assess the quality of life of adult patients with chronic intestinal failure receiving parenteral nutrition at home

Home parenteral nutrition is an established method of supporting patients with intestinal failure, but this treatment may be life long and imposes severe restrictions on daily life. Impact on quality of life is an important outcome when considering the management of home parenteral nutrition patients. This paper reviews studies in which the quality of life of patients receiving home parenteral nutrition has been assessed. A systematic search of electronic databases and relevant publications was undertaken to identify generic or treatment-specific questionnaires used with home parenteral nutrition patients. Many of the thirty-four reports discovered were small studies. Nineteen used non-specific generic instruments, eight used non-validated questionnaires, four used a combination of both, and three did not use any formal tool. Few systematic patterns emerged. There are few available data on the quality of life of home parenteral nutrition patients, and there is a need for standardised, scientifically validated, treatment-specific instruments to measure quality of life in this population.

The Scottish Home Parenteral Nutrition Managed Clinical Network: one year on

The purpose of home parenteral nutrition (HPN) is to allow patients with intestinal failure a reasonable quality of life and to facilitate independence from hospital. A managed clinical network (MCN) has been established for the organisation of HPN. This multi-professional group works towards optimal patient management by adoption of nationally agreed, evidence standards and protocols, to allow equity of access to quality care. A coordinator has identified patients and practice. A MCN exists to ensure that quality care can be delivered near to the patients home. An audit of the organisation of HPN has been completed. The aims of this study were to identify the numbers of patients receiving this treatment and to assess management practices. Data was collected from 16 centres between July 2000 and August 2001. This paper discusses the practicalities of setting up the network and presents data from the first full year of audit. This will act as the baseline for continuous audit and a basis for revision of documentation.

Development and validation of the disease-specific Short Bowel Syndrome-Quality of Life (SBS-QoL™) scale.

BACKGROUND & AIMS Subjects with short bowel syndrome (SBS) have impaired quality of life (QoL). No disease-specific instrument has been available to measure treatment-induced changes in QoL over time. Therefore, the aim was to develop and validate an SBS-specific QoL scale. METHODS Classical test theory and Food and Drug Administration (FDA) guidance were applied for development and validation of the SBS-QoL™. Procedures included item generation and raw scale construction. Factor analysis, construct validity and internal consistency were assessed in a non-interventional observation, test re-test reliability and responsiveness in a randomised clinical study. RESULTS The SBS-QoL™ comprises 17 items including two subscales. Subjects assessed the scale as easy to handle and comprehensible. Good construct validity was shown by comparison with the Home Parenteral Nutrition-Quality Of Life questionnaire as an external scale, which yielded moderately high correlation (r ≥ 0.7). High internal consistency was demonstrated (Cronbachs alpha: 0.94). Also the test re-test reliability was high (r ≥ 0.95), indicating reliable reproducibility of results. The Responsiveness Index (1.84) indicated the ability of the scale to detect changes in QoL over time. CONCLUSIONS The SBS-QoL™ is an easy to handle and comprehensible SBS-specific subject-reported QoL scale. It is valid, reliable and sensitive with excellent psychometric characteristics to measure treatment-induced changes in QoL over time in subjects with SBS.

Screening for psychosocial distress in patients with long-term home parenteral nutrition

BACKGROUND & AIMS Long-term home parenteral nutrition (HPN) may cause distress and negatively affect quality of life (QoL). The HPN version of the Distress Thermometer and Problem List (DT/PL) was developed to evaluate distress during HPN. This study validates the DT/PL, examines referral wish for additional care, assesses opinions on the DT/PL, and studies risk factors for distress and referral wish. METHODS Dutch and Scottish patients completed questions on socio-demographic and HPN-related general characteristics, the DT/PL, referral wish, the Hospital Anxiety and Depression Scale, and opinions on the DT. RESULTS The HPN version of the DT/PL seemed valid and the PL sufficiently reliable. Cut-off score appeared to be 6. Consequently, 45% of patients were diagnosed as clinically distressed. Fifty-three percent had a referral wish. Emotional and physical problems were most strongly associated with distress. Not being able to work related to elevated distress. Female gender and co-morbidity related to referral wish. Opinions on the DT were generally positive. CONCLUSION The DT/PL appears to be a good instrument to regularly gain insight into distress and referral wish in HPN patients. Use of the DT/PL facilitates support to patients who most need and want it, thus improving quality of care and QoL.

Development of quality of care interventions for adult patients on home parenteral nutrition (HPN) with a benign underlying disease using a two-round Delphi approach

BACKGROUND & AIMS HPN patients with benign diseases deserve professional care as they have to deal with complex techniques and risk potentially dangerous complications. The aim was to highlight main outcome quality indicators and to develop a set of key interventions to direct multidisciplinary teams in providing qualitative care. METHODS A two-round Delphi approach was used to build consensus on the most important outcome indicators and on 59 interventions identified in existing guidelines on HPN. Comments and interventions newly identified in the first round were co-evaluated in the second round. RESULTS 29 experts from 9 countries completed the two-round Delphi approach. The outcome indicators rated as the most important are 1) incidence of catheter-related infections, 2) incidence of readmission and quality of life (shared second place) and 3) incidence of dehydration. Sixty eight of a total of 89 interventions were considered as important for the quality of care, of which 46 are based on published guidelines and 22 were newly suggested by the Delphi panel. CONCLUSIONS Using a two-round Delphi approach, consensus was reached for the majority of interventions concerning HPN patients with benign diseases. This set of 68 interventions could be of use as a starting point for quality-improvement programs.