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
10
20
30
40
50
60
70
80
90
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
5
10
15
20
25
30
DepressionAnxietyDepression/Anxiety
PALS FM0
10
20
30
40
50
60
70
80
90
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=15
FVC > 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
5
10
15
20
25
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35
0
5
10
15
20
25
30
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 [email protected]://alsportal.ru, http://r-health.ru