Haemophilia in Bulgaria - EHC

HAEMOPHILIA
IN BULGARIA
T. LISSITCHKOV
EHC – SOFIA, 03-04.09.2011

DATA FOR BULGARIA
• POPULATION – 7 500 000
• GROSS NATIONAL PRODUCT –
AROUND 10 000 EUR PER CAPITA
• HEALTHCARE BUDGET – 4.4%
• PATIENTS WITH CONGENITAL
COAGULOPATHIAS - 847
REGISTERED)

WHERE IS BULGARIA?
BULGARIA IS A COUNTRY
WITH LIMITED FINANCIAL
RESOURCES

FVIII per capita
GDP (€)
USE OF FACTOR VIII PER CAPITA AND
GDP (EUR)
FVIII GDP (€)
10,00
40000
9,00
8,00
7,00
35000
30000
6,00
25000
5,00
20000
4,00
3,00
2,00
1,00
15000
10000
5000
0,00
0
Brian O’Mahony 2009

CONGENITAL
COAGULOPATHIAS IN
BULGARIA
• ORGANIZATION
• DIAGNOSIS
• TREATMENT

ORGANIZATION OF THE
MANAGEMENT OF
CONGENITAL
COAGULOPATHIAS
• TILL 1969
• 1970 – 1995
• 1996 - 2010
• SINCE 2011
FOUR PERIODS

“GOLDEN ERA” OF THE
TREATMENT OF
HAEMOPHILIA
• SAFE PLASMA DERIVED PRODUCTS
• RECOMBINANT PRODUCTS
• PROPHYLACTIC TREATMENT “GOLD
STANDARD”
• ITI FOR INHIBITOR PATIENTS

THIRD PERIOD (SINCE 1996)
• NEW ORGANIZATION (IN MEDICAL
UNIVERSITIES) – SIX HEMOPHILIA
TREATMENT CENTERS
• NATIONAL REGISTER OF PATIENTS
• DISCONTINUE THE PRODUCTION OF
“DANGER” PLASMA PRODUCTS
• ANNUAL TENDER FOR THE
COAGULATION PRODUCTS IN THE
MINISTRY OF HEALTH

THIRD PERIOD (SINCE 1996)
• EXPERT COUNCIL IN THE MINISTRY
OF HEALTH
• GUIDELINES FOR THE TREATMENT
OF THE BLEEDINGS IN PATIENTS
WITH CONGENITAL COAGULOPA-
THIAS (1998)

GUIDELINES FOR THE
TREATMENT
PRINCIPLE APROACH
• PROPHILACTIC TREATMENT
(CHILDREN)
• TREATMENT ON DEMAND (ADULTS)
• RECOMBINANT OR PLASMA DERIVED
PRODUCTS
• IMMUNE TOLERANCE INDUCTION
(LOW DOSE REGIMEN)

GUIDELINES FOR THE
TREATMENT
TREATMENT OF THE BLEEDINGS
• WHERE TO TREAT
• HOW MUCH OF FACTOR TO USE
• HOW LONG TO TREAT
• ADJUNCTIVE THERAPY
• OTHER MEASURES

THIRD PERIOD (SINCE 1996)
EDUCATION OF PATIENTS
BOOKLETS AND BROCHURES
• HEMOPHILIA
• INHIBITORS IN HEMOPHILIA
CONTACTS WITH THE SPECIALISTS

THIRD PERIOD (SINCE 1996)
• SCREENING OF INHIBITORS
• PRENATAL DIAGNOSIS
• TREATMENT OF CHRONIC JOINT
PROBLEMS
• TREATMENT OF CHRONIC HEPATITIS
C INFECTION
• TREATMENT OF HIV INFECTION

THIRD PERIOD (SINCE 1996)
TWINING PROGRAMME
• SOFIA – PALERMO
• PLOVDIV – BONN
• BULGARIAN HEMOPHILIA
SOCIETY – DUCH HEMOPHILIA
SOCIETY

TWINNING PROGRAMME
TRAINING OF SPECIALISTS
• PALERMO
1 SPECIALIST IN HEMATOLOGY
• UTRECHT
1 SPECIALIST IN HEMATOLOGY
2 SPECIALISTS IN REHABILITATION
1 NURSE

MAIN PROBLEMS
SHORTAGE OF COAGULATION
PRODUCTS
• UPDATED GUIDELINES FOR THE
TREATMENT
ACCENT ABOUT
OPTIMAL USE
EARLY TREATMENT
PROPHILACTIC TREATMENT

MAIN PROBLEMS
PRODUCTION OF PLASMA PRODUCTS
FROM BULGARIAN PLASMA
• FULLY RENOVATED FIVE BLOOD
TRANSFUSION CENTERS
• HIGH QUALITY PLASMA
• INSUFFICIENT QUANTITY
SEVERE DECREASE OF NUMBER OF
BLOOD DONATIONS
ENORMOUS LARGE USE OF FFP

PRODUCTION OF PLASMA
PRODUCTS
SOLUTIONS
• CONTRACT FRACTIONATION
• PARTIAL CONTRACT
FRACTIONATION

ACTIVITY OF BULGARIAN
HEMOPHILIA SOCIETY
• ORGANIZATION OF SUMMER CAMPS
• EDUCATIONAL BOOKLETS
• PARTICIPATION OF THE EXPERT
COUNCIL IN THE MINISTRY OF
HEALTH
• CONTACTS WITH WFH
• CONTACTS WITH EHC
• CONTACTS WITH COMPANIES

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
HAEMOPHILIA 14, 2008, 361-374

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
CENTRAL HAEMOPHILIA
ORGANIZATION WITH SUPPORTING
LOCAL GROUPS
6 HTC + MODIFIED CHTC (SOFIA)

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
NATIONAL HAEMOPHILIA PATIENT
REGISTRY
• GLOBAL REGISTER OF CONGENITAL
COAGULOPATHIAS 847
• REGISTER ACCORDING TO THE
DIAGNOSIS
• REGISTER ACCORDING TO THE
REGIONS OF THE COUNTRY

PATIENTS WITH CONGENITAL
COAGULOPATHIAS
• HEMOPHILIA A 556
• HEMOPHILIA B 67
• INHIBITORS TO F. VIII 30
• VWD 90
• RARE COAGULOPATHIAS 30
• THROMBOCYTOPATHIAS 24
• WITHOUT CORRECT DIAGNOSIS 83

RARE COAGULATION
DISORDERS
DEFICIENCY OF:
• FACTOR I 13
• FACTOR II 1
• FACTOR V 1
• FACTOR VII 7
• FACTOR X 3
• FACTOR XI 2
• FACTOR XIII 3

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
COMPREHENSIVE CARE CENTERS AND
HAEMOPHILIA TREATMENT CENTERS
• SOFIA SHAT JOAN PAVEL
NSBALHZ
• PLOVDIV
• VARNA
• PLEVEN
• STARA ZAGORA

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
PARTNERSHIP IN THE DELIVERY OF
HAEMOPHILIA CARE
• MINISTRY OF HEALTH
• NATIONAL HEALTH INSURANCE
FUND
NATIONAL EXPERT GROUP
(COMMITTEE)

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
• SAFE AND EFFECTIVE
CONCENTRATES – YES
• AT OPTIMUM TREATMENT LEVELS
NO

THIRD PERIOD (SINCE 1996)
ANNUAL QUANTITY OF COAGULATION
PRODUCTS (2009 - 2010)
(PER CAPITA)
• 1.9 U F VIII
• 0.1 U F IX
• 0.1 U VWF
• 0.05 U PCC
• 800 000 – 1 200 000 U rF VIII
• 800 -1000 MG rF VIIa
• APCC 350 000 U

THIRD PERIOD
LACKING PRODUCTS
• FIBRINOGEN
• FACTOR XIII CONCENTRATE
• DDAVP

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
HOME TREATMENT AND DELIVERY
YES

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
PROPHYLAXIS (PREVANTIVE)
TREATMENT
• CHILDREN - YES
• ADULTS - VERY RARE

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
SPECIALIST SERVICES AND
EMERGENCY CARE
• YES
• NEW ORGANIZATION

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
MANAGEMENT OF INHIBITORS
• TREATMENT OF BLEEDINGS – YES
• IMMUNE TOLLERANCE INDUCTION
NO AS A REGULAR PRACTICE

EUROPEAN PRINCIPLES OF
HAEMOPHILIA CARE
EDUCATION AND RESEARCH
EDUCATION
• DOCTORS – MU HOSPITALS
• DOCTORS – DISTRICT HOSPITALS
• NURSES – MU AND DISTRICT
HOSPITALS
• PATIENTS AND THEIR FAMILIES –
EDUCATION AND TRAINING

EDUCATION OF
HEALTHCARE STAFF
• TRANSFUSION THERAPY (2000)
• TRANSFUSION HEMATOLOGY (2003)
• CLINICAL HEMATOLOGY (2004)

EDUCATIONAL BROCHURES
• HOME TREATMENT OF CONGENITAL
COAGULOPATHIAS
• GENETIC CONSULTATION FOR
CONGENITAL COAGULOPATHIAS
• MODERN REHABILITATION AND
PROPHILAXIS OF HAEMOPHILIACS
• VON WILLEBRAND DISEASE
• CHRONIC VIRAL HEPATITIS

EDUCATIONAL BROCHURES
• JOINT PROBLEMS IN HAEMOPHILIA
• COAGULATION FACTOR
CONCENTRATES
• MILD HAEMOPHILIA
• RARE COAGULATION DISORDERS
• BLOOD PRODUCTS AND CJD
• GENE THERAPY

EDUCATIONAL BROCHURES
• PATHOLOGICAL INHIBITORS OF
COAGULATION
• PHYSIOLOGICAL INHIBITORS OF
COAGULATION
• AMAZING ADVENTURES OF
ALEXANDRO
• COMPREHENSIVE CARE ABOUT
PATIENTS WITH CONGENITAL
COAGULOPATHIAS

RESEARCH
• CLINICAL TRIALS
HAEMOPHILIA A AND B
VON WILLEBRAND DISEASE
INHIBITORS TO FACTOR VIII
PD FVIII 3
RF VIII 4
RF IX 4
RF VIIa 4
APCC 1

CLINICAL TRIALS (2009)
• FACTOR VIII
• R FACTOR VIII
• FACTOR IX
• VWF
• FEIBA
1 840 000 U
78 000 U
660 000 U
360 000 U
750 000 U
THE OVERALL COST 1 615 000 EUR

RESEARCH
• COMPREHENSIVE HAEMOPHILIA
TREATMENT CENTER – SOFIA (2007-
2008)
• IMPROVEMENT OF HAEMOPHILIA
CARE IN BULGARIA (2009-2010)
• EUROPEAN HAEMOPHILIA SAFETY
SURVEILANCE SYSTEM (2008-2011)
(EUHASS)

THE GOAL OF THE FIRST
PROJECT
FORMATION OF CHTC ON THE BASE OF
BLOOD COAGULATION DEPARTMENT
OF NCHT (HAEMATOLOGICAL
HOSPITAL JOAN PAVEL – SOFIA)
WITH THE GENERAL TASK TO SOLVE
THE PROBLEMS OF THE INDIVIDUAL
PATIENT WITH CONGENITAL
COAGULOPATHIA

THE GOAL OF THE SECOND
PROJECT
IMPROVEMENT OF THE DIAGNOSIS
AND TREATMENT OF CONGENITAL
COAGULOPATHIAS ON THE BASIS OF
THE EXISTING ORGANIZATION IN
THE COUNTRY

ASSESSMENT OF THE
PROJECT

SECOND PROJECT
PARTICIPANTS
• UMBAL SVETY GEORGI
• UMBAL SVETA MARINA
• UMBAL DR G. STRANSKI
• RCTH STARA ZAGORA
• UMBAL ALEXANDROVSKA

SECOND PROJECT
PARTICIPANTS
• SHAT JOAN PAVEL – SOFIA
• NSBALHZ - SOFIA
• NATIONAL GENETIC
LABORATORY
• CLINICAL LABORATORY
DEPARTMENT – MU SOFIA

PROJECTS IN
COAGULATION DISORDERS
SUPPORT
• MINISTRY OF HEALTH
• BULGARIAN HAEMOPHILIA SOCIETY
• WORLD FEDERATION OF
HAEMOPHILIA
• EUROPEAN HAEMOPHILIA
CONSORTIUM

MAIN TASKS
• CONSULTATIONS OF PATIENTS WITH
THE TEAM OF SPECIALISTS ABOUT
COMPLICATIONS OF THE DISEASE
AND COMPLICATIONS OF THE
TREATMENT
• TRAINING OF MEDICAL STAFF
• TRAINING OF PATIENTS
• UPDATING OF NATIONAL REGISTER

MAIN TASKS
• STANDARDIZATION OF
COAGULATION DIAGNOSTICS
• PUBLICATION OF INFORMATIVE
BROCHURES

MAIN RESULTS
ORGANIZATION OF MULTYDISCIPLINARY
TEAM WITH SPECIALISTS IN
HEMATOLOGY
HEPATOLOGY
RHEUMATOLOGY
ORTHOPEDICS
REHABILITATION AND PHYSIOTHERAPY
GENETICS

MAIN RESULTS
• REGULAR CONSULTATIONS OF
PATIENTS WITH SPECIALSTS OF
MULTYDISCIPLINARY TEAM
• REGULAR SCREENING OF
INHIBITORS

BENEFITS OF THE
PROJECTS
• EARLY DIAGNOSTICS OF THE
COMPLLICATIONS
• IMPROVEMENT OF THE TREATMENT
• PLANING OF ELECTIVE SURGERY
• ORGANIZATION OF REHABILITATION
• UP-DATE OF NATIONAL REGISTER
• GENETIC CONSULTATIONS

FOURTH PERIOD
NEW ORGANIZATION OF THE
TREATMENT
• HOME TREATMENT – NATIONAL
HEALTH INSURANCE FUND
• TREATMENT IN THE HOSPITALS –
MINISTRY OF HEALTH

FOURTH PERIOD
MAIN PROBLEMS
• OPTIMAL ORGANIZATION OF THE
TREATMENT
• COMPREHENSIVE CARE FOR
PATIENTS
• PROBLEMS OF ADULT PATIENTS
• NATIONAL HAEMOPHILIA CO-
ORDINATION GROUP

FOURTH PERIOD
OPTIMAL ORGANIZATION OF
THE TREATMENT
• NATIONAL HEALTH INSURANCE
FUND
• OPTIMAL RESOURSES FOR THE
TREATMENT

FOURTH PERIOD
COMPREHENSIVE HAEMOPHILIA
TREATMENT CENTERS
TREATMENT OF ADULT PATIENTS
• CHRONIC LIVER DISEASE
• ELECTIVE ORTHOPEDIC SURGERY
• CANCER
• HIV INFECTION
• CARDIOVASCULAR PROBLEMS

FOURTH PERIOD
NATIONAL HAEMOPHILIA
CO-ORDINATION GROUP
• IN THE MINISTRY OF HEALTH
• IN THE NATIONAL HEALTH
INSURANCE FUND

FOURTH PERIOD
NATIONAL HAEMOPHILIA
CO-ORDINATION GROUP
• NATIONAL HEALTH INSURANCE
FUND
• MINISTRY OF HEALTH
• BULGARIAN HAEMOPHILIA SOCIETY
• EXPERTS

CONCLUSIONS
BEST CLINICAL EFFICACY AND
ECONOMY EFFICIENCY OF LIMITED
RESOURCES ARE RESULT OF:
• OPTIMAL ORGANIZATION
• GUIDELINES FOR THE TREATMENT
• EDUCATION OF DOCTORS, NURSES
AND PATIENTS
• INFORMATION WITH BOOKS AND
BOOKLETS

CONCLUSIONS
LOOKING FOR ADDITIONAL SOURCES
FOR TREATMENT, TESTING AND
CONSULTATIONS WITH SPECIALISTS
• CLINICAL TRIALS
• NATIONAL AND INTERNATIONAL
PROJECTS