Palliative use of NIV in end of life patients: neuromuscular disease

Transcription

1 Palliative use of NIV in end of life patients: neuromuscular disease JIVD MARCH ANITA SIMONDS ROYAL BROMPTON & HAREFIELD NHS FOUNDATION TRUST, LONDON SW3 6NP UK

2 Topics covered Inherited neuromuscular condi1ons: Use of pallia1ve care resources Duchenne MD Other congenital NMD Type 1 Spinal muscular atrophy Motor neurone disease/amyotrophic lateral sclerosis End stage oncology disease with respiratory failure Goals with NIV

3 General points Pallia1ve care medicine and end of life care are not the same thing Pallia1ve care and ac1ve management eg resuscita1on, ITU admission are not incompa1ble End of life care and ac1ve measures are usually not compa1ble but focus on symptom pallia1on should always con1nue NIV may extend life, palliate symptoms and add to pa1ent autonomy and choice, but should not protract death

4 Rising prevalence of life-limiting conditions 40,000 children 32:10,000 Prevalence doubled over last 10 years BeTer survival US cohort 39% NMD Fraser L et al Pediatrics 2012; 129:e

5 Trends in neuromuscular referrals over time to hospice care Fraser LK et al Pall Med 2011;26:924-29

6 Progressive neuromuscular disease referrals 450 cases a year neuromuscular disease pts =37% of total referrals Four main issues: End of life care, symptom management, transi1on to home, respite and family support

7 Survival trends in Duchenne MD Jeppesen J Neuromusc Dis 2003;13:804-12

8 Duchenne MD: survival by interventions Ishikawa Y et al Neuromusc Disord 2011;21:47-51

9 Duchenne MD Analysis Royal Brompton Hospital Survival Analysis of patients treated with NIV 1.0 Cohort treated with NIV (n=126) 0.8 Survival Age (years) Chatwin et al 2009

10 New Non-pulmonary complications in prolonged DMD survivors: symptom management and palliative care u Malnutri1on/dysphagia =15 u Nephrolithiasis 6 u Diabetes mellitus 2 u Deep venous thrombosis 2 u Gallstones 1 u Inflammatory bowel disease Birnkrant Ped Pulmon 2006 RBH series Bowel problems: intermittent obstruction, severe constipation, volvulus Urinary difficulties: urinary retention, calculi Nutritional : most need PEGs Orthopaedic, pain control issues Cardiac complications Autonomic dysfunction

11 Indications for non-invasive ventilation To prolong life by trea1ng ven1latory failure To treat symptoma1c nocturnal hypoven1la1on To manage, reduce frequency of acute chest infec1ons To reduce hospital admissions To palliate symptoms of breathlessness To facilitate hospital discharge and end of life care at home

12 Paediatric non-invasive ventilation : diagnostic categories Royal Brompton N=496

13 Growth in paediatric NIV and transition to adult care: Royal Brompton Hospital Paediatric patients started on NIV N= Transitioned to adult service cumulative number of patietns who transition to adult services paediatric patients alive cummulative number of patients initated on ventilatory support

14 Survival probability Age (years) Survival with non- invasive ven1latory support Chatwin, Simonds PLoSOne: 2015 In Press CMD (n=28) (l ); myopathy (n=22), open circle ( ); SMA type II (n=49) triangle ( ); DMD commenced on NIV < 17 years old (n=88) (r ); DMD commenced on NIV > 17 years old (n=63) ( ).

15 Outcome: Bottom line 40% Paediatric NIV pts have transi1oned to adult care 24% have died 9% were able to discon1nue ven1latory support

16 Thomas NH,.Dubowitz V. The natural history of type I (severe) spinal muscular atrophy. Neuromusc Disord 1994;4:

17 (0.006) Group NIV and MI- E Number of respiratory decompensa4ons treated at home (episodes/pa4ents per year) Number of respiratory decompensa4ons needing admissions (episodes/ pa4ents per year) 42 (0.02) 43 (0.023) TIPPV 80 (0.007) 71 (0.006) Total Death or ventilation > 24 hrs/day Oskoui M et al Neurol 2007; 69:

18 Sleep disturbance Nocturnal swea1ng Morning headaches NIV and symptom control in NMD Appe1te Concentra1on Total score (Mellies et al., Neuromuscular Disorders, 2004)

19 Perception of QOL in SMA Type I (Bach et al., Am J Phys Med Rehabil, 2003)

20 Respiratory Support in SMA type I: A Survey of Physician Practices and Attitudes (Hardart et al., Pediatrics,2002) Postal survey was sent and respondents were: 57% intensivists, 39% physiatrists and 34% neurologists NIV was offered and recommended by 70% offered but not recommended by 22.5%, neither offered or recommended by 7.5% of physicians Intuba1on was offered and recommended by 39%, offered but not recommended by 49%, neither offered or recommended by 12% Tracheostomy was offered and recommended by 27%, offered but not recommended by 49% and neither offered or recommended by 24%

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22 Pa4ent Resuscita4on status: For intuba4on and ven4la4on? Total number of intuba4ons Referred for management to facilitate discharge Non protocol extuba4on success Protocol extuba4on success Admissions managed with non- invasive aids Home mechanical insuffla4on / exsuffla4on? NIPPV use at discharge ajer ini4al ini4a4on NIPPV use at present or prior to death A Y 3 Y Y Nocturnally Nocturnally B Y 1 Y N Nocturnally 24 hours a day C N 0 Y N/A N/A 0 N 18 hours a day 24 hours a day D Y 2 N 0 Not atempted 0 N Acclima1sa1on to NIPPV Nocturnally E Y 0 Y N/A N/A 2 Y 18 hours per day 16 hours per day F Y 0 N N/A N/A 2 Y Acclima1sa1on to NIPPV Nocturnally G Y 2 Y Y Nocturnally Nocturnally H N 0 N N/A N/A 0 N Nocturnally 23 hours per day I Y 2 N Y Nocturnally 16 hours per day J Y 2 N N When unwell with a respiratory tract infec1on Nocturnally K N 0 N N/A N/A 1 Y 18 hours a day 20 hours per day L Y 1 N N Acclima1sing to NIPPV Nocturnally M Y 4 Y Y Nocturnally Nocturnally

23 Management of children with SMA1 should ideally be goal directed, in conjunc1on with the parents wishes In children with less severe respiratory failure ven1latory support alters the natural history of the disease A increase in survival is usually seen with ven1la1on, enteral feeding and MI- E Some SMA II children may only need cough augmenta1on Some parents may not wish to use ven1latory aids NIV may be used to facilitate discharge for child to die at home with family

24 Challenges in progressive inherited neuromuscular disease Understanding outcomes: longer survival in some condi1ons, greater uncertainty, longer dura1on for pallia1ve interven1ons Shared an1cipatory decision- making Simultaneous ac1ve care and pallia1ve support Symptoms: breathlessness, poor feeding, aspira1on, choking New symptom range in children/young adults living longer Major requirement for respite care for families as children living longer Inequali1es, and need for prac1cal, financial and social support loom large

25 RCT NIV in Motor Neurone Disease (ALS) Bourke et al Lancet Neurol 2005; 5 140

26 RCT of NIV in MND Bourke SC Lancet Neurol 2006:5:140-7 Survival Qol SAQLI All patients All patients Mod bulbar weakness Severe bulbar Mod bulbar Severe bulbar No survival advantage in severe bulbar patients but qol improved

27 NIV in MND/ALS: Quality of life } Bourke et al AJCCRM 2001: Assessment pre, 1, 3, 5 month aler star1ng NIV } Generic: Improvements in GWbS (p=0.039), SF36 emo1onal limita1on, health percep1on } Specific: Improvements Epworth SS, SAQLI, CRDQ dyspnoea, fa1gue & mastery } Improvements at 1 month maintained at 5 months despite disease progression } Indices of sleep- related symptoms most responsive } Lyall et al Neurol 2001: NIV increased Vitality domain (SF36) by 25% for up to 15 months despite disease progression

28 Advance directives No direct evidence Advance Direc1ves improve qol, however increase autonomy, and improve physician pt communicagon. Vast majority of pa1ents with MND/ALS approve of advance direc1ves Physicians more likely to follow specific direc1ves supported by discussion with pa1ents than generali1es Pa1ents want to start discussion earlier than physicians, but tend to want physician to ini1ate discussion Preferences for life- sustaining measures may change over 1me (eg for ven1la1on), so discussions need to start early aler diagnosis and con1nue

29 Withdrawal of NIV NIV may be withdrawn with 1trated opiate infusion (SC) - principle of double effect Usually ensure eyelash reflex absent before removing mask and discon1nuing NIV May add low flow O2 2 l/min to avoid terminal fiqng due to hypoxia and hypercapnia Involve family as much as they wish Can be managed in recipient s home or hospice or in hospital according to personal preference

30 RCT NIV in end stage oncology patients with respiratory failure

31 Goal setting in NIV : a summary Extend survival Improve quality of life Treatment of an acute and reversible exacerba1ons (?preven1on of exacerba1ons) Ceiling of care in do- not- intubate (DNI) pa1ents Relief of dyspnoea Control of symptoms of sleep disordered breathings eg headaches, sleep fragmenta1on Allow effec1ve treatment of other symptoms eg. opiates for pain without exacerba1ng respiratory failure, CO2 narcosis or over- seda1on Improve concurrent resp/cardiac symptoms eg. COPD/ cardiac failure pulmonary oedema Buying 1me to resolve affairs, say goodbye Provide pagent with sense of control/autonomy over endstage disease