Palliative care studies review in Russia in 2010-2013

Gleb Levitsky Md PhDDirector

Russian Charity ALS Foundation

GREETINGS FROM NATALIA RYBAKOVA – THE QUEEN OF RUSSIAN PALS

PALLIATIVE CARE STUDIES 2010-2013

• EMOTIONAL PROFILE IN PALS AND THEIR FAMILY MEMBERS

• COGNITIVE FUNCTIONS• NIPPV• SIALORREA

EMOTIONAL PROFILE IN PALS AND THEIR FAMILY MEMBERS

• 118 PALS• 97 family members

• Eye-to-eye consultation, HAM-D (eye-to eye, by phone and email), FTD Score (for PALS only)

Together with:Vadim Gilod Md PhD Dept Suicidology, Moscow City

Hospital # 20Dr Andrey Levitsky, private psychiatrist and narcologist,

visits at home

PALS FM0

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Mental disordersHealthy

MENTAL DISORDERS IN PALS AND THEIR FAMILY MEMBERS IN RUSSIA

PALS – 85% FM-52%

Spectrum of situation-mediated mental disorders in PALS and their FM

PALS FM0

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DepressionAnxietyDepression/Anxiety

PALS FM0

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TDHealthy

TOXIC DEPENDENCIES IN PALS AND THEIR FAMILY MEMBERS IN RUSSIA

PALS – 49% FM-80%

Cognitive change in PALS

• 28% frontotemporal cognitive changeMedium log rank 1,47 [1,92; 1,26]• 4,2% frontotemporal dementiaMedium log rank -0,4 [1,07; -3,09]

Overall 32,2%

Coincidences of ALS and schizophrenia, schizoaffective and bipolar disorder were described. These cases have very poor prognosis.

Together with Professor of Neurology Oleg Levin MD PhDDept of Neurology Qualification Improvement Academy

Withdrawal of cholinolytic cocktail

Patient H., 62 years old, bulbar ALS, rapid progression by ALSFRSR

• ALSAQ40 total 207,5 ->310• emot. 22,5->55• HAMD 7 ->13• CNSLS 7/9 -> 7/14• FTD Log rank 1,9 -> 5,39• Montreal Cog 19 -> 26

More mistakes in graphic tasks Less mistakes in graphic tasks

Good recognition, but mistakes spelling No mistakes in spelling

CHOLINOLYTIC COCKLAIL FOR SIALORREA WITHDRAWAL OF COCKTAIL

Management of mental disorders

• Withdrawal of cholinolytics for sialorea (change to Dysport or Irradiation)

• Cholinomimetic (Galantamin in low dose)• atypic neuroleptic (s) , may be in drops• antidepressant (s)• hypnotic • gopantenic acid

Patient R., 55 years old

• Lumbar onset ALS with bulbar involvement, duration 5 years, artificial ventilation and PEG

• Therapy for 3 monthsRisperidon 1-2 drops 3 times a dayHaloperidol 1.5 mg ¼ tab 3 times a dayPipophesin 25 mg 3 rimes a dayGopantenic acid syrup 10% 5 ml 3 times a dayDiazepam 5 mg 2 times a dayResult: HAMD 30->13 CNSLS 20/39 -> 13/19

NIPPV studies

N=37Males 22 Females 15

Age range 36-72• Spinal ALS N= 22

FVC >50% FVC <50%• Bulbar ALS N=15FVC > 65% FVC < 65%

S-mode of NIPPV (the lowest expenses)80% of PALS used ipap 10 epap 4 cm and increased

pressures only once

Together with Professor of Pulmonology Sergey Babak MD PhD Centrosoyuz Hospital

Duration of ALS in PALS adapted and not adapted to NIPPV

30.3±11.7 and 20.7±11.7 months р=0.033

Series10

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Duration of SPINAL ALS in PALS adapted and not adapted to NIPPV

24.8 ± 7.1 and 16.1 ± 5.3, р=0.041

Spirometry in spinal ALS on NIPPV

Spirometry in ALS on NIPPV

Patient Sch., 54 years, cervical onset of spinal ALS, slow progression by ALSFRSR

FVC 70% in March 2012 and FVC 66% in September 2012 after Ipap 12 Epap 6 cm 4 hours per day

Patterns of adaptation to NIPPV

• Gradual adaptation• Forced adaptation• Inadaptation/ Desadaptation

GRADUAL adaptation

DAY NIGHT

FORCED adaptation

DAY NIGHT

Inadaptation / desadaptation

DAY NIGHTDAY NIGHT

DAY NIGHT

NIPPV in bulbar ALS

There are prerequisites to think that in bulbar ALS NIPPV may prolong survival when it is started in patients with FVC above 65%, but there are few patients for analysis

Orbicularis oris muscle paresis prevents from exact measurement of FVC by spirometry or body pletismography

Early tracheostomy with NIPPV like VIVO-40 (BREAS) is another option to prolong life in bALS

COMPULSORY ALS MANAGEMENT

If a PALS in Russia has FTD/FN cognitive impairment and moderate to severe

depression and declines aggressive treatments a neurologist and 3 psychiatrists register his incapacity to make decisions himself, he is recognized dangerous for himself and for

emotional well being of his relatives, and early tracheostomy and PEG are performed in a

compulsory manner

Sialorea in ALS

• Cholynolytic cocktail (Atropine drops, Atrovent solution, Amytriptiline)

• Scopoderm Plaster• Buscopan• Dysport (250 U in dysarthria vs 500 U in

anarthria)• Irradiation of parotids 6-7 Gr (single)*

*Together with Lev Epifanov MD PhD Dept of Radiotherapy Moscow City Hospital # 14

Sialorea in ALS

• Cholynolytic cocktail (Atropine drops, Atrovent solution, Amytriptiline)

1) Moderate effect on sialorea2) Decline of cognitive function

• Scopoderm Plaster1) Excellent effect on sialorea2) Decline of cognitive function

Sialorea in ALS

• BuscopanBenefits- does not enter CNS, no central

cholinolytic effectDisadvantages – not recommended for long-

term use1) Moderate effect on sialorea in mild and

moderate bulbar ALS2) No cognitive decline

Sialorea in ALS

• Dysport 1) Excellent effect on sialorea in 250 U +

Amitripyiline 25-37.5 mg in dysarthric bALS used each 3-6 months

2) Excellent effect on sialorea in 500 U in anarthric bALS each 3-6 months

3) No cognitive decline• 500 U in dysarthric bulbar ALS can exceed

anarthria!

Sialorea in ALS

• Irradiation of parotids 6-7 Gr (single)

Excellent effect on sialorea

Can not be combined with Dysport

Follow-up studies are required to determine the interval of repeated procedure

THANK YOU! СПАСИБО!

Gleb Levitsky MD PhD DirectorRussian Charity ALS Foundation

+ 7 926 182 18 99 alsrus@gmail.comhttp://alsportal.ru, http://r-health.ru