Multidisciplinary ALS team: Russian experience 11th annual Nordic ALS/MND Conference Multidisciplinary ALS team: Russian experience Lev Brylev, (Russia), MD, PhD, Head of neurologic department in Moscow city hospital#12, ALS Team leader in Martha-Mary medical center "Miloserdie"  21-23 August, 2015

500 patients applied to our organization from 20 regions of Russia Research center of Neurology M-M medical center Home care City hospital Online Diagnosis Research Outpatient clinic Psychological groups Equipment and nutrition Nurse Physician Psychologist Priest Volunteer Social workers PEG Tracheostomy Ventilation adjustment alsfund.ru miloserdie.ru air4help.ru Information Fundraising 2011 - 2015 500 patients applied to our organization from 20 regions of Russia 107 are currently closely followed in Moscow

Evidance-based approach Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review) Miller R. G. , MD et al. Neurology October 13, 2009 vol. 73 no. 15 1227-1233 Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review) Miller R. G. , MD et al. Neurology October 13, 2009 vol. 73 no. 15 1218-1226 EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force. Eur J Neurol. 2012 Mar;19(3):360-75.

EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) – revised report of an EFNS task force  Flowchart for the management of respiratory dysfunction in amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). NIPPV, non‐invasive positive pressure. © IF THIS IMAGE HAS BEEN PROVIDED BY OR IS OWNED BY A THIRD PARTY, AS INDICATED IN THE CAPTION LINE, THEN FURTHER PERMISSION MAY BE NEEDED BEFORE ANY FURTHER USE. PLEASE CONTACT WILEY'S PERMISSIONS DEPARTMENT ON PERMISSIONS@WILEY.COM OR USE THE RIGHTSLINK SERVICE BY CLICKING ON THE 'REQUEST PERMISSION' LINK ACCOMPANYING THIS ARTICLE. WILEY OR AUTHOR OWNED IMAGES MAY BE USED FOR NON-COMMERCIAL PURPOSES, SUBJECT TO PROPER CITATION OF THE ARTICLE, AUTHOR, AND PUBLISHER. European Journal of Neurology Volume 19, Issue 3, pages 360-375, 14 SEP 2011 DOI: 10.1111/j.1468-1331.2011.03501.x http://onlinelibrary.wiley.com/doi/10.1111/j.1468-1331.2011.03501.x/full#f1

Noninvasive ventilation Bourke SC Lancet Neurol 2006:5:140-7 Unpublished data Evidence from a single randomised trial of NIV involving 41 participants suggest that it significantly improves and maintains quality of life and prolongs survival in people with ALS. Survival and some measures of quality of life were significantly improved in the subgroup of people with better bulbar function, but not in those with severe bulbar impairment. We believe adverse effects related to NIV should be systematically reported as at present there is little information on this subject. Cochrane Database Syst Rev. 2013 Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Radunovic A1, Annane D, Rafiq MK, Mustfa N. No study was identified that addressed the adverse effects of NIV.

Invasive ventilation 27 patients, only 3 of them had an advanced decision to go for tracheostomy 20 patients were taken home 11 are alive 9 died, mean ventilation time 412 days 7 died in ICU, mean ventilation time 40 days Mean delay in providing ventilator is 39 days Patients and their families do not make decisions in advance

Managing Dyspnoe Lorazepam for laryngospasms Morphine s\c We were first physicians, who prescribed morphine for dyspnoe in ALS patient We try to change regulations and guidelines to make it available for ALS patients Physicians are scared to “break the law”

PEG placement Neurology. Oct 13, 2009; 73(15): 1218–1226.

Gastrostomy Number of PEG 2012 4 2013 24 2014 35 35% of patients had 20% BMI decrease on the first visit 20% of patients had FVC <50% before PEG placement Number of PEG 2012 4 2013 24 2014 35 Patients and their families do not make decisions in advance

PEG availability

Survival of Irish ALS patients according to the clinic type attended, 1996–2000. B J Traynor et al. J Neurol Neurosurg Psychiatry 2003;74:1258-1261 ©2003 by BMJ Publishing Group Ltd

Facebook secret group

Online patient records

Patients’ expression of gratitude (n=58)

Survey on death experience (n=58) Just 1/3 of people spoke about death at all, and ¼ of people were discussing one's preferred way/place to die once or several times. Doctors, priests and volunteers of charity foundations are more efficient in this quality than are family members. About 2/3 of those who have never discussed death with their relatives, were confident that they have died in a way they liked. This might reveal unawareness of the relatives about the very wishes of their deceased family members. family members are reporting on their behalf

Next steps Improve palliative care Spread knowledge Сommunication skills Morphine availability Hospice for ALS patients Spread knowledge National guidelines and protocols Fundraising Participate in research Participate in international activities

Team Priest Music therapy Volunteers Social workers Psychologist Anna Hansen Natalia Semina Andrey Prokofiev Psychologist Marina Bialik Elena Timoschuk Oksana Orlova Marina Vasilieva Neurologists Elena Sergeeva Anna Kas’yanova Lev Brylev Physical therapy Anna Vorob’eva Vadim Parshikov Diana Schukina Elena Lysogorskaya Pulmonologist Music therapy Vasiliy Shtabnitskiy Alisa Apreleva Vera Fominikh Anastasiya Beltukova Alexey Vasiliev Intensive care Maria Ivanova Rita Fominikh Priest Nurses Egor Larin Antoniy Sveta Sergeeva Palliative care Ekaterina Dikhter Anna Sonkina

Special thanks Kathy Mitchel, Professor-retired Algonquin College, Ottawa Ontario, Canada Richard Sloan, Joseph Weld Hospice Medical Director-retired, UK Anna Hansen, clinical nurse specialist, Norway Per Halvor Lunde, physical therapist, Norway David M. Steinhorn, MD, Division of Critical Care, Sacramento, USA

Lev.brylev@gmail.com www.alsfund.ru