Trust Protocol for the prevention and treatment of Fat Malabsorption in Adults with Cystic Fibrosis.

Transcription

1 Trust Prtcl fr the preventin and treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. A Clinical Plicy Nrflk and Nrwich University Hspital Fr Use in: Fundatin Trust Cystic Fibrsis (CF) team including specialist By: Dietitians and Cnsultants Adults and children ver 16 years f age with Pancreatic Insufficiency (PI) requiring pancreatic Fr: enzyme replacement therapy (PERT) dsage adjustment. Divisin respnsible fr dcument: Medical Pancreatic enzyme replacement therapy, Key wrds: pancreatic insufficiency, cystic fibrsis, malabsrtptin Darren Sills (Cystic Fibrsis specialist Dietitian) Name and jb title f dcument Bryny James (Cystic Fibrsis Specialist authr s: Dietitian) Name f dcument authr s Line Claire Peters Manager: Jb title f authr s Line Manager: Dietetic Services Manager Dr Mark Pasteur Respiratry Medicine Cnsultant (NNUH) Supprted by: Clare Peters Dietetics Services Manager (NNUH) Clinical Guidelines Assessment Panel Assessed and apprved by the: (CGAP) If apprved by cmmittee r Gvernance Lead Chair s Actin; tick here Date f apprval: 21/10/2015 Ratified by r reprted as apprved t (if applicable): Clinical Standards Grup and Effectiveness Sub-Bard T be reviewed befre: 21/10/2018 T be reviewed by: Cystic Fibrsis Lead Dietitian Reference and / r Trust Dcs ID N: CFfatmal - id Versin N: 2.1 Descriptin f changes: Cmpliance links: (is there any NICE related t guidance) If Yes - des the strategy/plicy deviate frm the recmmendatins f NICE? If s why? N clinical changes additin f disclaimer t fter f frnt page n 18 March 2016 Nne N/A This guideline has been apprved by the Trust's Clinical Guidelines Assessment Panel as an aid t the diagnsis and management f relevant patients and clinical circumstances. Nt every patient r situatin fits neatly int a standard guideline scenari and the guideline must be interpreted and applied in practice in the light f prevailing clinical circumstances, the diagnstic and treatment ptins available and the prfessinal judgement, knwledge and expertise f relevant clinicians. It is advised that the ratinale fr any departure frm relevant guidance shuld be dcumented in the patient's case ntes. The Trust's guidelines are made publicly available as part f the cllective endeavur t cntinuusly imprve the quality f healthcare thrugh sharing medical experience and knwledge. The Trust accepts n respnsibility fr any misunderstanding r misapplicatin f this dcument. Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 1 f 9

2 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. Cntents 1. Objective Ratinale Brad recmmendatins Diagnsis f malabsrptin Criteria fr exclusin Cmmencing PERT Adjusting PERT dsage Cmmunicating dsage change Mnitring f PERT adequacy Persisting malabsrptin Persisting bwel symptms despite ptimal treatment General infrmatin fr taking PERT Staff selectin and training Register f staff Clinical audit standards Summary f develpment and cnsultatin prcess undertaken befre registratin and disseminatin Distributin list/disseminatin methd References Surce dcuments Appendix 1- Cmpetency Assessment Appendix Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 2 f 9

3 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. 1. Objective Optimise treatment f fat malabsrptin thrugh dse adjustment f pancreatic enzyme replacement therapy (PERT). Patients with Cystic Fibrsis (CF) wh have pancreatic insufficiency (PI) need PERT. 2. Ratinale Apprximately 85% r mre adults with CF have pancreatic sufficiency (CF trust, 2010) due t an inadequacy f their wn pancreas t secrete pancreatic enzymes. Patients wh are PI need PERT in rder t prevent symptms f malabsrptin (frequent pale, ily and ffensive stls, abdminal pain and distensin), prmte a healthy weight and bdy mass index and prevent deficiencies f fat sluble vitamins and essential fatty acids (Cnway et al, 2008). 3. Brad recmmendatins 3.1 Diagnsis f malabsrptin Patients wh present with bvius symptms f malabsrptin shuld be cmmenced n PERT; cnfirmatin f pancreatic insufficiency shuld then be made by measuring faecal elastase. If clinical symptms f malabsrptin are absent, pancreatic insufficiency shuld be cnfirmed by faecal elastase befre PERT is cmmenced. Faecal elastase shuld be mnitred n an annual basis in thse patients wh are pancreatic sufficient. 3.2 Criteria fr exclusin Patients wh have pancreatic sufficiency. Knwn sensitivity t prcine prteins 3.3 Cmmencing PERT There are varius pancreatic enzyme preparatins available (refer t the British Natinal Frmulary). Patients shuld be cmmenced n standard strength preparatins initially at a dse f 10,000-20,000 units lipase per meal and 10,000 units lipase per fat cntaining snack. When cmmencing PERT the initial prescriptin shuld be cmpleted by the attending cnsultant and the dse can be subsequently adjusted by CF specialist Dietitians. 3.4 Adjusting PERT dsage The dse f PERT shuld be gradually increased until the symptms f malabsrptin are cntrlled. Increases f PERT are recmmended at 10,000-20,000 units lipase Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 3 f 9

4 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. per meal and 10,000 units lipase per fat cntaining snack until a pattern f imprved stl cnsistency and abdminal symptms can be identified. Patients shuld be advised t titrate PERT dse based n fat intake. The ttal dsage can vary widely and shuld be individualised based n symptms f persisting malabsrptin. Aim t keep lipase intake belw 10,000 units per kg bdy weight per day t reduce the theretical risk f develping fibrsing clnpathy. Once patients reach dses f 10,000 units per kg bdy weight per day r mre the attending cnsultant shuld be advised and cautin taken when increasing dsages further. 3.5 Cmmunicating dsage change When patients PERT dsage is altered this shuld be agreed by ne f the cystic fibrsis respiratry physicians. A recrd f the dsage change is dcumented in the patient care recrd and cmmunicated t the patient s General Practitiner by the cystic fibrsis respiratry physicians in the frm f a clinic letter. Where changes are made utside f clinic settings (e.g. telephne) a letter will still be sent frm the respiratry physicians after cnsultatin. 3.6 Mnitring f PERT adequacy The PERT dse shuld be adjusted as abve t ptimise therapy and reviewed at each clinic appintment r mre frequently if required. Measurement f faecal fat micrscpy alng with symptms f malabsrptin will help determine the adequacy fat absrptin. There shuld be n fat seen n fat micrscpy. Faecal fat micrscpy shuld be checked yearly at annual review n all pancreatic insufficient patients and mre ften if symptms dictate. 3.7 Persisting malabsrptin If symptms f malabsrptin persist despite what appears t be an adequate PERT dse and methd f administratin r dses in excess f 10,000 units per kg bdy weight per day, assessment f ther factrs shuld be undertaken: Patient knwledge f enzyme titratin accrding t dietary fat Timing and methd f administratin. Taking enzymes thrughut the meals may imprve distributin f enzyme as fd leaves the stmach. Thse taking enzymes shuld be encuraged t swallw capsules whle. If enzymes are remved frm capsule, the micrspheres shuld be mixed with a small amunt f milk r ther nn-acidic fd and swallwed; they shuld nt be chewed r crushed. Enzyme strage and stck rtatin shuld be discussed. Ensure enzymes are within use by date and have nt been stred at extremes f temperature. Changing brand f enzyme may be f benefit due t differences in frmulatin Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 4 f 9

5 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. Patient adherence shuld be discussed. Cnsider using acid-reducing drug therapy 3.8 Persisting bwel symptms despite ptimal treatment Sme patients may still have bwel symptms despite malabsrptin being well cntrlled. It is imprtant that persisting symptms are investigated and a full gastrintestinal investigatin shuld be cnducted t rule ut ther causes f symptms. 3.9 General infrmatin fr taking PERT Enzymes shuld be given with all fat cntaining fds r drinks. If the capsules cannt be swallwed whle the micrspheres shuld be mixed with a small amunt f milk r fd. The micrspheres shuld nt be sprinkled n r mixed with the whle meal. The micrspheres shuld nt be chewed r crushed. 4. Staff selectin and training Registered Dietitians (Band 6 grade and abve) wh specialise in cystic fibrsis and abve shuld be able t wrk under this prtcl. The designated staff will have acquired knwledge and understanding with regards t the dietetic treatment f adult cystic fibrsis patients. They will annually update their knwledge and skills as required by the prcess f cntinuing prfessinal develpment. Thse using this prtcl shuld have an understanding f the pharmaclgy f PERT in the treatment f fat malabsrptin in cystic fibrsis. All designated cystic fibrsis Specialist Dietitians will have cmpleted the necessary training, demnstrated underpinning knwledge and been assessed as cmpetent prir t taking n this clinical practice and will be authrised t wrk accrding t this prtcl by the relevant department lead (appendix 1). 4.1 Register f staff The details f each member f staff wrking t this prtcl must be retained n a live departmental register (appendix 2) held within the department f nutritin and dietetics. It is the department s respnsibility t keep the register up t date. 5. Clinical audit standards T ensure that this prtcl is cmpliant with the abve standards, the fllwing mnitring prcesses will be undertaken: A yearly audit will be undertaken assessing the current chrt s pancreatic status and the departments practice f using PERT. The reprt will summarise: Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 5 f 9

6 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. Pancreatic status f the ppulatin Numbers f patients exceeding 10,000 units/kg/d Number f patients with persisting symptms f malabsrptin Number f patients underging/undergne further gastrintestinal investigatins fr persisting malabsrptin The audit results will be sent t the wider cystic fibrsis team wh will review the results and make recmmendatins fr further actin. Additinally as part f the natinal CF Trusts registry reprts, PERT usage and BMI are recrded and can be cmpared against ther centres and against the natinal median. 6. Summary f develpment and cnsultatin prcess undertaken befre registratin and disseminatin The authrs (Darren Sills and Bryny James) drafted this prtcl n behalf f the cystic fibrsis team wh have agreed the final cntent. During its develpment it was has been circulated fr cmment t thse listed in the supprted by sectin. This versin has been endrsed by the Tri-Hspital Clinical Guidelines Assessment Panel 7. Distributin list/disseminatin methd Trust intranet 8. References Cnway SP et al. (2008). Cystic Fibrsis in Children and Adults. The Leeds Methd Of Management. Revised Editin, Number 7) Cystic Fibrsis Trust. (2010) 9. Surce dcuments British Natinal Frmulary Fieker A, Philptt J and Armand M (2011) Enzyme replacement therapry fr pancreatic insufficiency: present and future. Clinical and Experimental Gastrenterlgy; 4: Keller J and Layer P. (2005) Human pancreatic excrine respnse t nutrients in health and disease. Gut; 54 (Supp 6), v1, ) Littlewd JM and Wlfe SP. (2000) Cntrl f malabsrptin in Cystic Fibrsis. Paediatric Drugs; 2(3): Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 6 f 9

7 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. Sinaasappel M et al (2002) Nutritin in patients with cystic fibrsis: a Eurpean Cnsensus. Jurnal f Cystic Fibrsis; 1 (2), Stallings VA et al. (2008). Evidence-based practice recmmendatins fr nutritinrelated management f children and adults with cystic fibrsis and pancreatic insufficiency: results f a systematic review. Jurnal f the American Dietetic Assciatin; 108: UK Cystic Fibrsis Trust Nutritin Wrking Grup (2002) Nutritinal management f cystic fibrsis. Cystic Fibrsis Trust Walters MP et al (1990) Clinical mnitring f steatrrhea in cystic fibrsis. Archives f Disease in Childhd; 65: Wuthuyzen-Bakker M, Bdewes FAJA and Verkade HJ (2011) Persistent fat malabsrptin in cystic fibrsis; lessns frm patients and mice. Jurnal f Cystic Fibrsis; 10: Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 7 f 9

8 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. 10. Appendix 1- Cmpetency Assessment Trust Prtcl fr the Management f Fat Malabsrptin in Adults with Cystic Fibrsis. Name f persn t be assessed:. HCPC N: Assessment criteria: Demnstrates knwledge f the legal aspects regarding the prescribing and administratin f medicines and understands the difference between prescriptin nly medicines, pharmacy medicines and general sales list medicines. Can state their prfessinal respnsibilities and that f ther multi-disciplinary team members with regard t the prtcl and in their prfessinal cde f cnduct. Assessr Can explain the individual indicatins fr adjusting dses f PERT against the prtcl. Can demnstrate that advice given is recrded accurately. Demnstrates the knwledge f the maximum dses that may be advised in the prtcl. Can discuss the side effects and cautins/warnings f PERT in the prtcl. Observed giving advice n PERT adjustment n at least fur different ccasins Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 8 f 9

9 Trust Prtcl fr the Preventin and Treatment f Fat Malabsrptin in Adults with Cystic Fibrsis. 11. Appendix 2 Departmental Recrd f Signatries This is the departmental list f all thse wh have read and agreed t act within the parameters f this prtcl. Each individual has kept a signed cpy f the prtcl fr his / herself. Print Name Sign Date (dd/mm/yyyy) Authr/s: Darren Sills, Bryny James Date f issue: 21/10/2015 Valid until: 21/10/2018 Prtcl Ref N: CFfatmal v2.1 - id Dcument: Fat Malabsrptin in Adults with Cystic Fibrsis. Cpy f cmplete dcument available frm Trustdcs Page 9 f 9