Trust Guideline for Treatment and prevention of Low Bone Mineral Density in Adults and Children with Cystic Fibrosis

Transcription

1 Trust Guideline fr Treatment and preventin f Lw Bne Mineral Density in Adults and Children with Cystic Fibrsis A clinical guideline In: By: Fr: Key wrds: Written by: Supprted by: Assessed by: Apprved by the: Nrflk and Nrwich University Hspital Fundatin Trust Cystic Fibrsis Adult and Paediatric teams including specialist Dietitians, Cnsultants, Physitherapists, Specialist Nurses and Radilgy staff. Adults with Cystic Fibrsis Bne health, steprsis, cystic fibrsis, lw bne mineral density Darren Sills (Cystic Fibrsis specialist Dietitian) (NNUH) Bryny James (Cystic Fibrsis specialist Dietitian) (NNUH) Dr Mark Pasteur Respiratry Medicine Cnsultant (NNUH) Dr Chris Uptn Paediatric Cnsultant (NNUH) Clare Peters Dietetics Services Manager (NNUH) Prfessr William Fraser Endcrinlgy Cnsultant (NNUH) Dr Patrick Wilsn Radilgy Cnsultant (NNUH) Clinical Guidelines Assessment Panel 19 Nvember 2014 Clinical Standards Grup Effectiveness Sub-bard Guideline issued: 19 Nvember 2014 T be reviewed befre: 19 Nvember 2017 extensin granted until 30/08/2018 T be reviewed by: Cystic Fibrsis Lead Dietitian Guideline supersedes: Nne Guideline Reg. N: CFBHv1 - id Versin Infrmatin Versin n Updated by Updated n Descriptin f changes Versin 1 Bryny James N/A New dcument Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 1 f 11

2 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cntents 1. Objective Ratinale...3 Brad recmmendatins DEXA scan intervals Defining lw BMD and steprsis Risk factrs precipitating lw BMD Evaluating bne health and preventin f BMD lss Nutritin and nutritinal status Vitamin D Calcium Vitamin K Exercise Treatment with bisphsphnates and cnsideratins Clinical audit standards Summary f develpment and cnsultatin prcess undertaken befre registratin and disseminatin: Distributin list and disseminatin methd: Glssary f terms References and surce dcuments: Appendix Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 2 f 11

3 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis 1. Objective T reduce the prevalence f steprsis and lw bne mineral density (BMD) in adults with Cystic Fibrsis (CF). 2. Ratinale Adults with CF have an increased risk f lw BMD and increased risk f bne fracture. Frm recent meta-analysis the pled prevalence f steprsis is 23.5% (Paccu 2010) lcally 23% f this patient grup have a lw BMD. Brad recmmendatins: 3. Assessing bne mineral density Assessment f BMD shuld be cmpleted using Dual Energy X-ray absptimetry (DEXA). Rutine DEXA scans shuld be cmmenced frm the age f ten and shuld be repeated every ne t five years. DEXA can be initiated at an earlier age in children with significant risk factrs fr lw BMD and prir t prescribing specific treatments fr lw BMD. With DEXA, BMD may be verestimated in peple with a shrt stature. Fr yunger adults whse height is at least 1 standard deviatin belw age and sex matched healthy cntrls, Z scres shuld be adjusted fr height r statural age when reprting DEXA results. Fracture histry shuld be included in the medical recrd. Chest X-rays shuld be rutinely examined fr vertebral fractures as these are ften under diagnsed. Vertebral fracture shuld als be assessed in patients with lw bne mineral density, height lss r back pain. Z-scres can be used t assess BMD in individuals 20 years ld r yunger and in pre-menpausal wmen and in men less than 50 years ld. T-scres shuld be used in pst-menpausal wmen and men ver the age f DEXA scan intervals DEXA scans shuld be perfrmed and repeated at ne t five yearly intervals. When assessing Z/T scres the lwest scre shuld be used t prvide advice/actin: <50yrs: Z-scre -1 repeat every 5 yrs. Z-scre between -1 and -2 repeat every 2 yrs. Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 3 f 11

4 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Z-scre <-2 repeat yearly. If Z-scre <-2 and prescribed bne prtective therapy, repeat after 18 mnths. >50yrs: If significant risk factrs fr lw BMD are present r lw trauma fractures ccurring in children DEXA can be dne yearly. T-scre >-1 repeat every 5 yrs T-scre between -1 and -2.5 repeat every 2 yrs T-scre <-2.5 r prir t prescribing bne prtective therapy repeat yearly. If significant risk factrs fr lw BMD are present DEXA can be dne yearly. 5. Defining lw BMD and steprsis The term CF related lw bne density shuld be applied if Z-scre is belw -2 in either hip r spine sites (CF Trust 2007). The term steprsis shuld be applied t yung adults (adlescents and premenpausal wmen and men under 50 years f age) with a Z-scre <-2 and significant fracture histry. The term steprsis shuld als be used in lder adults and pstmenpausal wmen with a T-scre < Risk factrs precipitating lw BMD Advances in medical treatment f CF have increased the life expectancy f peple with CF greatly and hence have increased the likelihd f mre chrnic health prblems arising. The aetilgy f CF related bne disease is thught t be cmplex and multifactrial. Predispsing excrine functin can lead t vitamin D, calcium and vitamin K deficiency, pr nutritinal status and grwth. In turn, this results in the fllwing; lw bdy weight, systemic inflammatin caused by frequent infective exacerbatins, use f crticsterids, sex hrmne insufficiency, CF related diabetes mellitus and reduced weight bearing exercise. When cnsidering ptential risk factrs fr CF related lw BMD the fllwing shuld be accunted fr: pr nutritinal status/lw bdy mass index (BMI) infective pulmnary exacerbatins Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 4 f 11

5 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Pancreatic excrine functin status vitamin D insufficiency vitamin K insufficiency negative calcium balance abnrmal fatty acid status hypgnadism delayed puberty CF related diabetes gluccrticid therapy lw level f weight bearing activity and CFTR dysfunctin effect n bne cells 7. Evaluating bne health and preventin f BMD lss 7.1. Nutritin and nutritinal status Assessment shuld include energy, prtein and calcium intake at least yearly. Height shuld be recrded at every clinic visit until maximal grwth has been attained and nce yearly thereafter. In children height shuld be pltted n the apprpriate percentile chart. Weight shuld be recrded at each clinic visit and cnverted t BMI (kg/m 2 ). In adults a nrmal BMI is imprtant t minimise risk factrs. In children ver the age f tw, BMI shuld be pltted n the apprpriate percentile chart and expressed by percentile psitin r standard deviatin scres Vitamin D Assessment shuld include: serum 25-hydrxyvitamin D, calcium, phsphrus and parathyrid hrmne (PTH). Aim t avid deficiency, minimum cncentratin 50nml/L. NNUH pathlgy lab measure 25OHD 2 and 25OHD 3 using mass spectrmetry with reference ranges: Deficiency NNUH <50nml/L. Adequate NNUH 50nml/L-120nml/L. Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 5 f 11

6 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Prir t prescribing bisphsphnates, serum levels f vitamin D shuld be established. If vitamin D serum cncentratin is < 25nml/L, r calcium is belw reference range, these shuld be supplemented. If stemalacia is suspected, refer t bne specialist in endcrinlgy. Daily vitamin D shuld be advised fr all pancreatic insufficient patients. 1,000-2,000 internatinal units in infants and 1,000-5,000 internatinal units in children ver the age f ne and in adults (Sermet-Gaudelus et al 2011). This shuld be titrated accrding t serum vitamin D levels. Vitamin D 3 (clecalciferl) is favured ver vitamin D 2. Patients with vitamin D deficiency shuld have a supplement f 1,000-10,000 internatinal units daily. Vitamin D supplements may be split int daily r weekly dses (Gerge 2012). If individual des nt respnd adequately, cnsider increasing vitamin D using UVB therapy r ther vitamin D frms. IM vitamin D injectins result in 100% adherence, but biavailability is unpredictable (Natinal Osteprsis Sciety, 2013). In these cases discussin with an endcrinlgist seems prudent. Other frms f vitamin D (i.e. calcitrl r alfacaclidl) shuld be started after referral t the endcrinlgists Calcium The CF Trust (2007) recmmends dietary calcium intake shuld be arund mg daily frm the age f seven in line with UK reference nutrient intakes. Dietary advice +/- calcium supplements (calcium carbnate) shuld be used t increase intake t recmmended levels. Cmbined calcium and vitamin D supplements may have the advantage f meeting bth calcium intake recmmendatins and achieving adequate serum vitamin D cncentratins Vitamin K Assessment shuld include: vitamin K1 and PIVKA II. If the patient is n vitamin K supplements these shuld be stated n the ICE bld test request frm Pancreatic insufficient patients shuld be cmmenced n vitamin K using a dse 0.5-2mg in infants and 1-10mg daily in children ver the age f ne and adults with CF. Cnsider additinal vitamin K if lw vitamin K1 r prteins induced by vitamin K absence (PIVKA II). If lw vitamin K status, cnsider checking prlnged prthrmbin time. Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 6 f 11

7 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Menadil sdium phsphate is preferred at NNUH (as it is water sluble and, therefre, thught may be better absrbed. It is als less cstly). Phytmenadine shuld be reserved fr thse wh have nt respnded adequately t menadil sdium phsphate despite gd cmpliance Exercise Children and adults shuld be encuraged t exercise fr minutes at least three times weekly. Weight bearing activity shuld be encuraged including high impact (e.g. jumping r skipping) and resistance activities. Exercise prgrammes shuld be individualised. Fr thse nt used t ding regular exercise, the prgramme shuld begin with lw impact exercises such as gentle jgging. As fitness and muscle strength imprve, the impact aspect f the prgramme can be increased. When admitted t hspital, patients shuld be encuraged t cntinue their usual exercise prgramme, where pssible. 8. Treatment with bisphsphnates and cnsideratins Bisphsphnates shuld be cnsidered when (Sermet-Gaudelus et al 2011): i. In adults Z-scre is <-2 and there is evidence f bne lss (>4%/yr) n serial measurements despite ptimisatin f treatment. ii. iii. iv. In children and adlescents after cnservative treatment fr ptimal BMD has failed. Patient have had lw trauma fracture Awaiting r undergne rgan transplant and Z-scre<-1.5 in adults and <-2 in children and adlescents v. Cntinuus sterids fr three mnths r lnger and a Z-scre belw -1.5 and <-2 in children and adlescents Befre starting bisphsphnates, vitamin D deficiency (<25nml/L) shuld be crrected and calcium intake ptimised. Bisphsphnates shuld nt be used in patients with stemalacia, r if vitamin D <10nml/L and PTH is increased (r the patient has hypcalcaemia). Bisphsphnates shuld be taken n an empty stmach and thse taking them shuld be advised t remain in an upright psitin fr at least 30 minutes r ne hur after taking the tablet and befre taking any fd, drink r ther medicine (MHRA, 2012) If n ral bisphsphnate calcium supplements shuld nt be taken at the same time as will reduce absrptin f bisphsphnate Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 7 f 11

8 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis DEXA scans shuld nt be repeated mre frequently than 18 mnths after cmmencement f bisphsphnates and: If a further decrease in BMD is fund despite ral bisphsphnates cnsider IV bisphsphnates. Cautin shuld be taken in renal impairment Females shuld be advised f cntraceptin Bisphsphnates shuld be avided in thse with esphageal disease (severe reflux and varices) Patients shuld be cunselled n the pssibility f stenecrsis f the jaw (fr further infrmatin see appendix 1). The need t cntinue bisphsphnate treatment fr steprsis shuld be reevaluated peridically based n an assessment f the benefits and risks f treatment fr individual patients, particularly after 5 r mre years f use (MHRA 2011). 9. Clinical audit standards A yearly audit will be undertaken assessing the current chrts verall bne health and practice will be cmpared t this guideline. The reprt will summarise: Prprtins f the chrt wh have nt had r have utdated DEXA scans, DEXA Z Scre categries belw -2, -1 t -2 and abve -1 BMI categries underweight, belw ideal, nrmal, verweight and 30 - bese. Vitamin D status Deficient, Insufficient and Sufficient. Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 8 f 11

9 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Bisphsphnate use Oral IV 10. Summary f develpment and cnsultatin prcess undertaken befre registratin and disseminatin: The authrs (Darren Sills and Bryny James) drafted this guideline n behalf f the CF 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. 11. Distributin list and disseminatin methd: Trust Intranet Cystic Fibrsis Team 12. Glssary f terms DEXA Dual Energy X-ray absptimetry Bne mineral density (BMD) refers t the density f bne minerals (e.g. calcium) T scre bne mineral density f an individual cmpared with a healthy, 30 year ld adult. Z scre bne mineral density f an individual cmpared with the average, age matched, bne mineral density. PIVKA II Prteins induced by vitamin K absence 13. References and surce dcuments: Cnwell, L.S., and Chang, A.B. (2012). Bisphsphnates fr steprsis in peple with cystic fibrsis (Review). Cchrane Database f Systematic Reviews. Issue 4. Cystic Fibrsis Trust (2007). Bne mineralisatin in cystic fibrsis. UK Cystic Fibrsis Trust Bne Mineralisatin Wrking Grup. Cystic Fibrsis Trust: Brmley Gerge. S. (2012) Diagnsis and Management f Vitamin D Deficiency in Adults in Primary Care. NHS Nrflk and Waveney. Medicines and Healthcare Regulatry Agency (2011). Bisphsphnates: atypical femral fractures. Drug Safety Update, vl 4 issue 11: A1 (fund at accessed nline 21 st January 2013) Natinal Osteprsis Sciety (2013). Vitamin D and Bne Health: A Practical Clinical Guideline fr Patient Management. Natinal Osteprsis Sciety: Bath Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 9 f 11

10 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Paccu, J., et al (2010). The prevalence f steprsis, stepenia, and fractures amng adults with cystic fibrsis: a systematic literature review with meta-analysis. Calcified Tissue Internatinal, 86: pp1-7. Sermet-Gaudelus, I. et al (2011). Eurpean cystic fibrsis bne mineralisatin guidelines. Jurnal f Cystic Fibrsis. 10 (supplement 2): pp S16-S23. Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 10 f 11

11 Trust Guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Appendix 1 Oral health management f patients prescribed bisphsphnates (SDCEP, 2011). The risk f stenecrsis f the jaw is substantially greater fr patients receiving intravenus bisphsphnates in the treatment f cancer than fr patients receiving ral bisphsphnates fr steprsis. Risk factrs fr develping stenecrsis f the jaw that shuld be cnsidered are: ptency f bisphsphnate (highest fr zlendrnic acid), rute f administratin, cumulative dse, duratin and type f malignant disease, cncmitant treatment, smking, cmrbid cnditins, and histry f dental disease. All patients shuld have a dental check-up (and any necessary remedial wrk shuld be perfrmed) befre bisphsphnate treatment, r as sn as pssible after starting treatment. During bisphsphnate treatment patients shuld maintain gd ral hygiene, receive rutine dental check-ups, and reprt any ral symptms. Bisphsphnates: atypical femral fractures (June 2011). Atypical femral fractures have been reprted rarely with bisphsphnate treatment, mainly in patients receiving lng-term treatment fr steprsis. The need t cntinue bisphsphnate treatment fr steprsis shuld be reevaluated peridically based n an assessment f the benefits and risks f treatment fr individual patients, particularly after 5 r mre years f use. Patients shuld be advised t reprt any thigh, hip, r grin pain during treatment with a bisphsphnate. Discntinuatin f bisphsphnate treatment in patients suspected t have an atypical femral fracture shuld be cnsidered after an assessment f the benefits and risks f cntinued treatment. Scttish Dental Reference Scttish Dental Clinical Effectiveness Prgramme (2011). Oral health management f patients prescribed bisphsphnates. NHS educatin fr Sctland: Dundee. Authr: Darren Sills, Bryny James Date f issue: Nvember 2014 Valid until: Nvember 2017 extensin granted until 30/08/2018 Guideline Ref N: CFBHv1 id Dcument: Trust guideline fr Bne Health and Treatment f Lw Bne Mineral Density in Adults with Cystic Fibrsis Cpy available frm Trust intranet Page 11 f 11