Dietitian’s Role in HPN Kirstine Farrer, Consultant Dietitian – Intestinal Failure Intestinal Failure Unit, Hope Hospital, Salford, Manchester kirstine.farrer@srht.nhs.uk Thank you for inviting me I am going to deliver this presentation from a dietetic perspective, highlighting the team approach we have at Hope and finish with a short case study. If you are interested is this area I would suggest reading the BAPEN document HPN in the UK 2003 So why is it needed?

Home Parenteral Nutrition Nutritional &/or water/electrolyte imbalance that cannot be corrected by enteral feeding Acute (type 2) or chronic (type 3) intestinal failure PN feasible at home Patient Family Healthcare professionals Text book definition of HPN. It can be short or long term – by short I mean 2yrs, we have a lady who has been on HPN 25years. At Hope the first patient was initiated on HPN in 1978, since then we have had approx 500 patients What type of diagnostic groups? We tend to see a considerable number of ………

How to create IF and create the need for HPN!

This is typical of the type of patients admitted to the Intestinal Failure Unit. As you can see she looks frightened, disorientated and in shock but at this point her physical needs are the greatest. HPN has become a life saving modality however the best clinical outcomes are achieved by a dedicated NST

HPN - indications Type 2 Intestinal Failure – short term Pending spontaneous recovery (e.g. some fistulas) intestinal reconstructive surgery death (some cancer patients) Type 3 Intestinal Failure - long term intestinal adaptation? small bowel transplantation? What type of diagnostic groups?

IF Aetiology Admitted patients (1999-2006)

All Treated Patients Regional Data (2005/6)

HPN centres Hope 132 St Mark’s 105 Royal Victoria, Belfast Cardiff/Swansea/ Wrexham Oxford Cambridge (SB Tx) Royal London Southampton Bristol Dudley (DGH) Leicester Liverpool Newcastle Nottingham Sheffield Leeds Sorry if I have left anyone off Also Scotland has its own clinical network – 125 patients Patient and public involvement is crucial for CG. What do the patients think of the service

The Patients’ Views (PINNT) Mean Distance for Routine check: 84 (240) & for emergencies:71(400) Average cost/trip: £16.48 (£230) Accompanied: >50% Local care out of Hours: 63% Dissatisfied: 30% Would like service nearer home – 78% Travel To & From HPN Centres This survey relates to the 98 respondents from 200 circulated in 2001.   HPN patients may have to travel long distances for routine follow up or in emergencies, often at night. A recent survey of HPN patients conducted by the patients’ group PINNT, has highlighted these problems.   Frequency of Visits Four to six visits per year was the most common response, but three people attended 12 times a year for HPN related issues, whilst six people made more than 12 visits. National Referral Centre Attendance 47.9% attended a national referral centre and 52.1% attended other hospitals. (Under present arrangements, care is in 1 centre only in most cases). Distance of Travel For both routine and emergency cases, people travelled a wide range of distances. Some people travelled in excess of 300 miles for routine or emergency visits, with one travelling over 480 miles for a routine visit and two travelling in excess of 400 miles in an emergency. Mode of Transport 77% travelled by car, 10.5% by hospital transport. Travel by public transport is inadvisable when you are feeling ill. One person travelled by plane. Cost The average cost was £16.48, which is a lot for someone on restricted income. One person incurred £230 per hospital visit. Some people reported the need to be accompanied to hospital, whether for routine or emergency trips, which can incur greater costs. Emergencies All but nineteen respondents said they had not had an emergency. However, two people reported eight emergency trips to hospital. Service Preference 78% said they would prefer closer more local services but 30% expressed dissatisfaction with present local services. Until local services are better supported and able to respond to urgent calls with a consistent high standard of care, patients will continue to lack confidence in them. This means greater dependency on the major centres. The Scottish MCN has approached this problem by attempting to bring all units with HPN patients up to the same standards of care within a properly organised and supported network. Hope Hospital Travel Data The above patient survey findings are supported by independent data from Hope Hospital Intestinal Failure Unit. In 1998, 16% travelled >150 miles, and 45% travelled >50 miles. 21% were older than 60 years.

Outcome New patients Hope St Mark’s UK Wales % Discharged home HPN 24 3 55 43 S/c fluids 5 Artificial enteral 4 9 Oral alone 12 1 27 22 42 Died Transfer (hospital)

Demographic Details 132 HPN patients at Hope mean 50 years (range 18-78 years) 79 female Mean length of time on HPN 5.4 years (range 0 -25years) Mean No of nights on HPN=5 Hope data from July 2004 As established HPN patients become older, or older patients are referred for HPN, the support required will increase. This has implications for carers and commissioners of care. Ethical dilemmas will be inevitable.  

The two most common types of short bowel Jejuno-colic anastomosis Ileum and some of jejunum resected leaving jejuno-colic anastomosis Jejunostomy Colon, ileum and some of jejunum resected, leaving a jejunostomy These are the two most common types of patients on HPN Tend to find if pts are on HPN they absorb<35% of oral intake Jejunocolic anastomosis Jejunostomy

Fistuloclysis/Distal Feeding – a new HETF indication?

Spot the surgeon in the MDT

Multidisciplinary Team for HPN Clinic – the patient’s journey Dietitian Nurses (Psychologist) Pharmacist Biochemist Clinical Director Surgical and Medical Registrars It is a journey – nobody gets seen by a Dr until they have seen me first, dietetic/triage nurse! I then liase with the other members of the team Patient is then given a room and all the other members of the MDT “visit” Appears chaotic if you visit – but very effective

Nutritional Requirements Nitrogen 0.17-0.2gN/kg/day 150-350g Glucose / day <1g fat /kg lipid / day Fat soluble vitamins 1-2/ week Water soluble vitamins Minerals HPN patients should be stabilised on the regimen prior to discharge – no net catabolism similar to free living individuals – physical activity is the main differentiating factor. Hypoglycaemia can be caused if glucose infusions are suddenly stopped. Oversecretion of endogenous insulin at high infusion rates rebound hypo, a slower rate will reduce hyperinsulinaemia. Lipid bags I.e. The 3 in one bags are limited to 1-2 /week, which also limits FSV. HOWEVER little evidence to suggest pts are clinically deficient. Could query the adequacy of micronutrient preparations, however the amount in each daily dose is > the amount expected to be absorbed from the RNI and since its given IV its inevitably > the reqs for most individuals. SHENKIN Note we only change over to a glucose and fat regimen when the patient is not septic or diabetic. Iron def anaemia can be corrected by venefur – we don’t add additional iron, affects stability of PN – some units do, esp paeds units. We do not add extra iron to our TPN solutions apart from the tiny amount in Additrace(20 micromoles) as the stability can be quite difficult when you add extra iron to the TPN. Other centres such as Leeds do add extra iron to their solutions particularly in paediatric bags. The solution most centres use is iron(Fe 3+) chloride solution 0.1mg/ml and from memory they add around to 2ml to the bag. We try not use it as it reduces the stability of the TPN so it may increase the frequency of deliveries the patient has to have at home. We prefer to use Venofer or Cosmofer for patients with low iron but normal Hb and for patient with low iron and low Hb. For Venofer we give a course of around six infusions of 200mg in 100ml over an hour. The number of infusions will depend on how low the iron is and that is repeated as required depending on results. We will always give the venofer and cosmofer in hospital due to the risk of anaphylactic shock even after repeated infusions. Cosmofer is the single total dose iron infusion and is non formulary at our Trust but we have used it in patients who have had difficulty coming to the hospital for repeated infusions. Also safer and more effective if micronutrients are added immediately before the infusion. This is where the pharmacist plays a integral part of the team, work very closely together.

HPN – dietetic monitoring Weight Height BMI MAC TSF MAMC Comments What do I do? Do SRD routinely measure MAMC It is open to intra/interobservor error, but its simple, cheap and will raise your profile in the team. We are under more pressure to provide evidence for health gain and improved clinical outcomes – JLS believes it’s a useful adjunct to monitoring HPN

Nutritional Status of HPN Patients Weight Median 60kg (Range 41-94kg) BMI Median 22.5 (Range 15-30) TSF Median 11mm (Range 2.8-22) MAC Median 27cm (Range 18-42cm) MAMC Median 23.6cm (Range 17.2-34) Based on all our HPN patients end of July 2005 Also need to focus on oral intake

Oral Intake Actively encouraged – psychological and physiological reasons Improves QOL / social interaction Decreases biliary sludge and promotes intestinal adaptation - may result in a reduction of HPN. HPN is associated with hepatobiliary complications from mild elevation of LFTs to hepatic steatosis and intrahepatic cholestasis , < frequent when patients receive oral feeding ie stimulates GI, therefore stimulating biliary secretion GUGLIELMI 2001 Also avoid excessive energy and cyclical PN may help Can use MCT/LCT lipid emulsions

Why do we get great results – dedicated team of nurses, at clinic know the patients from admission and Jackie leads psychological support services for patients

Biochemistry Monitoring 3/12 FBC, bone, liver profiles Cu, Zn, Se, Vitamin D status CRP, ESR Ferritin Coagulation Screen Common to have to increase Cu, Zn and Se in high output stoma patients Bone disease - High risk esp IBD/ steroids/ malabsorption Time related

Incidence of HPN related MBD Incidence is unknown but reports range from 40-100%. Analysis has shown a reduced bone formation rate in most patients No clear answer- most likely to be a combination of causes General and lifestyle factors- age, menopause, alcohol and tobacco Other drugs- corticosteriods, heparin, tinzaparin. Underlying disease- Malabsorption of Ca, Mg and Vit D in IBD Many patients are asymptomatic Common Symptoms are: Bone pain- mainly in the lower joints Back pain Fractures particularly vertebral In the 1980s Aluminium contaminated feeds caused MBD – pain in long bones and weight bearing joints and a decrease in bone formation. Changing to a balanced crystalline AA soln reduced the aluminum infused. Vit D toxicity and those with decreased Parathormone levels bone disease can be corrected by removing vit D. After removing Vit D the serum PTH and 1,25 hydroxy-vit D increase to normal levels. Acidosis – in some patients there is an excessive excretion of calcium and phosphorus, Ca balance can be improved by increasing the amount of PO given and so increasing renal tubular absorption of Ca. Most recent theory for bone disease is an altered response to PTH in HPN patients. TPN may alter the effect PTH on the bones to favour resorption. ? Caused by altering the body’s diurnal cycles. Reports of parathormone levels are variable usually low and the normal diurnal nocturnal rise of PTH is prevented by PN

Summary of Clinical Trials Pironi et al (2002)- Bone Mineral Density, 165 patients, MBD seen in 84% of pts, 35% had bone pain and 10% had fractures.symptoms. No difference seen between primary diseases. Age of starting TPN and BMI main factors to predict morbidity. The studies suggest anywhere from immediately up to three years Shaffer et al- 52 pts- review of bone scans in HPN pts. Average significant reduction of bone mass was ~ 2years. Guidelines recommend baseline scan and then yearly. (ASPEN) This was a multicentred retrospective study published in Clincial Nutrition in 2002 - 9 European centres participated 165 pts: 76 men and 89 females. Duration on HPN 55months men and 66 for woman. Bone Mineral Density was measured by Dual energy xray absorptiometry DEXA and categorised using the WHO criteria. Looked at BMD Z scores number of Standard deviations from normal values corrected for age and sex Stepwise regression analysis demonstrated the lumbar spine Z score positively associated with the age of starting HPN and the femoral neck Z score positively associated with the BMI What can you do to prevent it? Amend the TPN formula Ensure adequate amounts of calcium, phosphate and magnesium. Ensure minimal amounts of aluminium given Match sodium to losses as excess causes hypercalcuria Match glucose and protein to requirements Monitor for metabolic acidosis and swap to acetate solutions if necessary Vitamin D In TPN- fat bags only 200units If Vit D levels low start- Orally- alfacalcidol best absorbed in short bowel (dose initially 1 microgram daily) Injection- Ergocalciferol injection 300,000uints once every three months Removal of Vit D only consider if low serum PTH and 1,25-dihydroxyvitamin D normal 25-hydroxivitamin D Bisphosphates Poor Oral absorption so most effective if given IV Most commonly used is pamidronate 60mg in 500ml NaCl 0.9% over 2 hours, three monthly Side effects- hypotension and allergic type reactions particularly if given too quickly Flu type reactions- give 1g paracetamol At least two doses in hospital before home therapy Evidence Good evidence for benefit in osteoporosis patients Little evidence in TPN patients K. Haderslev et al (2002)- 20 pts- improved BMD with three monthly clodronate infusions, inhibited bone resorption B Messing et al (2002)- 20 pts-improved BMD with regular pamidronate infusions Zolendrenic Acid New generation of bisphosphonate 2-3 times more potent than pamidronate Dose: 4mg given over 15 mins Positive results in osteoporosis patients- no trials in HPN pts In osteoporosis can be given yearly Non formulary at Hope PTH Teriparatide First parathyroid hormone derivative- described as a “bone forming agent” Licensed for postmenopausal osteoporosis Dose is 20micrograms sc daily for 18 months Given with Vit D and calcium supplements Bisphosphate therapy should be stopped. Hope- on formulary for Dr O’Neil only Alfacalcidol 1ug £120.00 Ergocalciferol Inj £28.00 Pamidronate 60mg £150.00 Zolendronic Acid £195.00 Teriparatide £5,320.00

Parenteral Nutrition + Liver Disease Shaffer and Lumen (2002) Retrospective Study of 107 case notes. Median duration of HPN was 40 months. The main underlying diagnoses were Crohns Disease (40%) and ischaemic bowel (28%). Derranged LFTs were defined as 1.5 times above the reference range, at least 6 months after initiation of HPN. Persistently abnormal LFTs were present in 39% adult patients on long-term HPN. 40 pts raised ALP Length of SB <100cm was the only significant variable for deranged LFT. It doesn’t lead to decompensated liver disease. Clinical Nutrition 2002; 21(4): 337-43.

Parenteral Lipid and Hepatic Dysfunction Lipid emulsion >1g/kg  increased hepatic dysfunction (retrospective).1 MCT/LCT emulsions rather than LCT alone  more efficient oxidative metabolism  reduce hepatic dysfunction?2 (In practice, only  lower serum bilirubin).3 Olive oil-based lipid emulsion (case report: improvement in LFTs possibly due to vitamin E content?).4 Ann Intern Med 2000; 132: 525-32 JPEN 1991; 15: 601-3 Clin Nutrition 1998; 17: 23-9 Clin Nutr 2004; 23: 1418-25

Preventing & Treating TPN-associated Liver Disease Encourage oral intake! Avoid Dextrose Overfeeding (<40kcal/kg/d) Reduce Fat Calories (<1g/kg/d) Cyclical PN Choline Liver/Small Bowel Transplant ? Total intravenous calorie intake The absence of oral intake induces loss of enteric stimulation, increased mucosal permeability and potentially bacterial translocation Ie eat small amounts and frequently Avoid excessive macronutrient supply V Ursodeoxycholic Acid (UDCA) Metronidizole Infliximab JPEN 2002; 26(5): S43-8.

HPN – case study 35 year old male – Mr W Admitted to IFU in May 2002 Diagnosis – mesenteric volvulus Remaining small bowel – 30cm of jejunum and 10cm of ileum, brought out as a jejunostomy and mucous fistula

HPN Case Study Social history – engineer, married, wife just had a baby and interests include running marathons! Physical examination – thin and jaundiced Alk phos 160, ALT 480 and bilirubin 76 Otherwise haemodynamically stable

Dietetic Assessment Weight 56.6kg (Weight history – 63kg) BMI 19.6 MAC 26cm TSF 10.6mm MAMC 22.7cm Nutritional Requirements – 2100kcals and 11gN Not eating – “too scared” increased his output

Dietetic Plan Low fibre diet Glucose-saline drink 1litre /day Restrict hypotonic fluids Commence on PPN until line cultures come back clear (3L, 1800kcals and 9gN)

PLAN HPN training and home, trained quickly, home by 24th July. Drugs on discharge – loperamide 6mg qds, codeine PO 60mg qds and omeprazole 80mg bd Reconstructive surgery at a later date to close jejunostomy

HPN px on discharge 2/7 fat 3.5L bag 2200kcals, 11gN, 244mmol Na, 50mmol K, 9mmol Ca 11mmol Mg, 27.7mmol PO, additrace and cernevit 5/7 glucose bags – 3.5L 2200kcals, 13gN, + same electrolytes + additrace

HPN – dietetic monitoring 19.5.02 20.8.02 19.11.02 16.1.03 2.12.03 Weight 56.6 54.4 60 67 70.8 Height 1.72 BMI 19.6 18.4 20.3 23 23.8 MAC 26 25.5 27.5 29 30.5 TSF 10.6 10 13.8 15.8 MAMC 22.7 22.4 23.2 24 Comments 7/7 PN Increase kcals + N Surgery 25/11 5/7 PN 2/7 PN Discuss trend views from May 02 – Dec 03 BMI MAMC Remember the girl at the beginning

This is typical of the type of patients admitted to the Intestinal Failure Unit. As you can see she looks frightened, disorientated and in shock but at this point her physical needs are the greatest.

This is the girl back on the ward for a check up – medically and nutritionally stable, put will still need psychological support People are moved not by events but by the views they take of them. As you can see her physical needs are now much less and she looks fit and healthy but her psychological problems were far greater.

‘ No man is really an accomplished physician or surgeon who has not made dietetic principles and practice an important part of his professional education’ SIR HENRY THOMPSON, F.R.C.S, 1897