Péče o dě) s hemofilií v ČR Data registru ČNHP. Blatný J, Smejkal P., Ovesná P. Za centra zastoupená v ČNHP

Rozměr: px

Začít zobrazení ze stránky:

Download "Péče o dě) s hemofilií v ČR Data registru ČNHP. Blatný J, Smejkal P., Ovesná P. Za centra zastoupená v ČNHP"

Luboš Bláha
před 7 lety
Počet zobrazení:

1 Péče o dě) s hemofilií v ČR Data registru ČNHP Blatný J, Smejkal P., Ovesná P. Za centra zastoupená v ČNHP

2 Systém péče v ČR Český národní hemofilický program (ČNHP) Ročně se jedná o cca ¾ mld Kč pro cca 1000 lidí Systém center (2 úrovně) 4 Komplexní (CCC): 2xFN Brno, FN Motol a ÚHKT 7 Léčebných (HTC): Ostrava, Hradec Králové, Olomouc, Plzeň, Úsa n L., Č. Budějovice, Liberec Zásadní odlišnost center: Ortopedie/chirurgie (vč. elek)vní), inhibitory CCC

3 Úhrada péče o hemofilii v ČR (2013) V rámci DRG ALFA (většina diagnóz/výkonů) Hrazeno plně bez omezení Chir/Int. dg HLAVNÍ, Hemofilie VEDLEJŠÍ! V rámci DRG BETA/GAMA Hrazeno plně do výše stropu (výjimečně na chir. prac.) MIMO ÚV (tzv. balíčky, např TEP) Individuální jednání ZZ a ZP Snaha od r 2014 eliminovat tento způsob úhrady (?)

4 Úspěchy ČNHP Vytvoření formální sítě center a jejich akreditace za účas) odborné veřejnos) a VZP První audit 2011 Re- audit vždy á 2 roky (věstník MZd) Úspěšné jednání S NRC a MZd směřující k vyněa úhrady léčby hemofilie z DRG a z paušálů Úzká spoluráce v rámci projektů Evropské komise EUHASS, EUHANET a sam související pozi)vní PR české hemofilické péčé

5 The current status of care for persons with haemophilia and von Willebrand s disease registered within CNHP registry Jan Blatný, Petra Ovesná, Petr Brabec on behalf of Centres contribudng to common database of the CNHP (Czech NaDonal Haemophilia Programme)

6 Sample size N=972 All valid persons N=644 Haemophilia N=49 Hereditary deficiency of other clozng factors N=279 Von Willebrand s disease * N = 233 Adults N = 411 * Persons under 19 years old in 2012

7 Par)cipa)ng centres in CNHP Valid persons Paediatric centres N % FN Motol Dpt. of Pediatric Haematology and Oncology FN Brno DN Dpt. of Pediatric Haematology FNHK Dpt. of Pediatric Medicine UnL Pediatric Dpt. Haematology FN Ostrava Dpt. of Pediatric Medicine FN Olomouc Dpt. of Pediatric Medicine FN Plzen Pediatric Dpt CB Pediatric Dpt Valid persons Adult centres N % FN Brno OKH FN Ostrava Blood centre FN Olomouc Haemato- Oncology Dpt FN Plzen UKBH CB OKH FNHK OKH KN Liberec OKH UnL OKH Plzen Health Centre Haematology UHKT Praha Kolin Haematology and Transfusion Dpt Pelhrimov - OHT 2 0.3

8 Age All N=644 Age at diagnosis (years) N 477* Mean 7.6 Median (min - max) 2 (0 69) Current age (years) N 644 Mean 30.5 Median (min - max) 26 (0 90) 40,0 35,0 30,0 33,1 25,0 20,0 17,4 18,8 % of persons 25,0 20,0 15,0 10,0 5,0 22,4 13,4 10,9 8,4 4,2 2,5 2,5 2,5 % of persons 15,0 10,0 5,0 13,5 12,9 9,6 10,1 12,7 5,0 0,0 0, >65 age (years) * Missing informa7on on year of diagnosis in 167 persons. age (years)

9 Type and severity of haemophilia I N=233 Type of haemophilia Haemophilia A (N=204) Haemophilia B (N=29) Severity of haemophilia (N=216) Mild (N=101) Moderate (N=48) Severe (N=84) 12,4% 36,1% 43,3% 87,6% 20,6%

10 Type and severity of haemophilia II N=233 Haemophilia A (N=204) Mild (N=89) Moderate (N=40) Severe (N=75) Haemophilia B (N=29) Mild (N=12) Moderate (N=8) Severe (N=9) 36,8% 43,6% 31,0% 41,4% 19,6% 27,6%

11 Age at diagnosis according to N=233 Age at diagnosis (years) type and severity of haemophilia Haem A Typ hemofilie a věk při diagnóze N = 214 Haem B median 10 th 90 th percen)le Age at diagnosis (years) Mild Moderate Severe Inhibitor Haemophilia A Haemophilia B Age at diagnosis (years) Mild* Moderate* Severe* Inhibitor N Mean (0 17) 1 (0 13) Median (min max) 3 (0 13) 1 (0 17) 0 (0 11) 0 (0 2) * including persons with inhibitor

12 Actual age according to type and N=233 Current age (years) severity of haemophilia Haem A Typ hemofilie a věk při diagnóze N = 214 Haem B median 10 th 90 th percen)le Current age (years) Mild Moderate Severe Inhibitor Haemophilia A Haemophilia B Current age* (years) Mild* Moderate* Severe* Inhibitor N Mean (0 18) 9 (2 18) Median (min max) 10 (0 18) 11 (0 18) 9.5 (0 18) 5 (1 11) *Current age = age reached in year 2012 * including persons with inhibitor

13 Hepa))s experienced N=233 Experienced hepadds Yes (N=2) No (N=230) Not known (N=1) 98,7% 0,9% 0,4% N=2 One child has hepa77s C, type of hepa77s not specified in one child. Data from last annual report of each person.

14 HIV All N=644 HIV Posi)ve (N=2) Nega)ve (N=494) Not known / not available (N=148) 76,7% 23,0% N=2 Both HIV- posi7ve persons are adults. 0,3% Data from last annual report of each person.

15 Data from year 2012 sample size Dětša pacien): aktualizace údajů za rok 2010 All valid persons N = 644 Persons with annual report in 2012 N = 584 Persons examined in 2012 N = of them with inhibitor N = 233 Adults N = 411 reported in 2012 N = 215 Adults reported in 2012 N = 369 examined in 2012 N = of them with inhibitor Adults examined in 2012 N = of them with inhibitor

16 Persons with haemophilia All N=453 with inhibitor inhibitor was recorded in 8 persons in year 2012 other 1 person has recorded inhibitor in 2011 (data from 2012 are not available) currently 9 persons with inhibitor (5 children and 4 adults) + 6 in other centre

17 Frequency of bleeding requiring N=201 treatment in % 80% 30% 70% % of persons 25% 20% 15% 10% 5% % of persons 60% 50% 40% 30% 20% 10% 0% >20 Bleeds per year 0% >20 Bleeds per year Haemophilia A Haemophilia B Frequency of bleeding Mild* Moderate* Severe* Inhibitor N total N valid Mean / (0 49) 1 (0 26) Median (min max) 0 (0 12) 2 (0 30) 6 / 5 (0 49) 11 (3 32) Frequency of bleeding is missing in 19 children. * without inhibitor mean and median of frequency of bleeding in children with severe haemophilia without inhibitor on permanent prophylaxis

18 Loca)on of (any) bleeds in 2012 N= (66.8%) children experienced bleeding requiring treatment at least once in year; 840 bleeds were recorded in total, 50 bleeds required hospitaliza)on. 65 (33.2%) children recorded no bleed during year Informa)on on frequency of bleeding is missing in 5 examined children. Overall 143 persons have recorded loca)on of any bleeds*. Joints (N=86) Muscles (N=50) Subcutaneous (N=57) Tooth extrac)on (N=27) Urogenital tract (N=2) GIT (N=0) CNS (N=1) Other (N=54) Surgery (N=15) Without bleeding (N=65) 18,9% 1,4% 0,0% 0,7% 10,5% 35,0% 39,9% 37,8% 33,2% 60,1% * all recorded bleeds, regardless of requirement of treatment 0% 20% 40% 60% 80% % of persons

19 Treatment N=201 Immunate Fanhdi Immunine Octanate Octanine HaemocDn Feiba Not known Advate Kogenate Recombinate Other FIX NovoSeven Other FVIII Not known Plasma- derived factors N = 77 Recombinant factors N = (67.2%) children received drugs in 2012 (11 of them more different drugs). Plasma- derived factors were administered in 77 (38.3%) children, recombinant factors in 63 (31.3%). Five children were treated with both plasma- derived and recombinant factor number of persons

20 Type of treatment All N=453 % of persons 100% 80% 60% 71,9% 73,5% 40% 34,1% 20% 0% Home treatment (N=325) Prophylaxis (N=151) type of treatment 19,9% 6,6% Type of prophylaxis (N=151) Temporary (N=30) Permanent (N=111) Not available (N=10)

21 Bleeding requiring treatment according to prophylaxis Frequency of bleeding per year Typ hemofilie a věk při diagnóze median 10 th 90 th percen)le , no yes no yes no yes no yes Frequency of bleeding Mild* Moderate* Severe* Inhibitor N Mean Median (min max) 0 (0 12) 1 (0 30) 2 (1 4) 7.5 (0 49) 5 (0 25) 15 (3 32) 11 children on permanent prophylaxis 0 (0%) 9 (19.1%) 51 (72.9%) 1 (20%) % of drugs (FVIII and FIX) consumed by children on permanent prophylaxix 0 % 9 % 70 % 4 % N=201 permanent prophylaxis * without inhibitor

22 Bleeding requiring treatment according to centres Moderate Severe Child centres N Mean Median Min Max Severity FN Motol Dpt. of Pediatric Haematology and Oncology FN Brno DN Dpt. of Pediatric Haematology FNHK Dpt. of Pediatric Medicine Typ hemofilie a věk při diagnóze 1,0 2,0 4,5 5,0 5,0 6,0 N=201 Frequency of bleeding in children with haemophilia (A and B) without inhibitor on permanent prophylaxis Moderate Severe Moderate Severe Moderate Severe UnL Pediatric Dpt. Haematology FN Ostrava Dpt. of Pediatric Medicine FN Olomouc Dpt. of Pediatric Medicine 1,5 2,0 3 4, Severe Moderate Severe Moderate FN Plzen Pediatric Dpt. CB Pediatric Dpt. 2,0 2,0 5, Severe Moderate Severe

23 Bleeding requiring treatment according to centres Moderate Severe Child centres N Mean Median Min Max FN Motol Dpt. of Pediatric Haematology and Oncology FN Brno DN Dpt. of Pediatric Haematology FNHK Dpt. of Pediatric Medicine UnL Pediatric Dpt. Haematology FN Ostrava Dpt. of Pediatric Medicine FN Olomouc Dpt. of Pediatric Medicine Typ hemofilie a věk při diagnóze 0,0 1,0 1,0 1,0 2,0 2,0 2,0 3,0 5,0 5,0 7,0 N=201 Frequency of bleeding in children with haemophilia (A and B) without inhibitor regardless of prophylaxis % on premanent prophylaxis FN Plzen Pediatric Dpt. 5, CB Pediatric Dpt. 2,0 3,

24 FVIII FIX Drug Consump)on of drugs Total annual consumption Všichni pacien) Number of treated persons Average annual consumption per treated child Number of examined children Average annual consumption per examined child Immunate IU IU IU Fanhdi IU IU IU Octanate IU IU IU Haemoctin IU IU IU Advate IU IU IU Kogenate IU IU IU Recombinate IU IU IU Other recombinant IU IU IU FVIII celkem IU IU IU Immunine IU IU IU Octanine IU IU IU Other plasma-derived 26 Other recombinant FIX celkem IU IU IU apcc Feiba IU IU rfviia NovoSeven 629 mg mg Plasma-derived factors - TOTAL* IU IU IU Recombinant factors - TOTAL* IU IU IU TOTAL CONSUMTION * IU IU IU plasma- derived factors = Immunate, Fanhdi, Octanate, Immunine, Octanine, Other plasma- derived recombinant factors = Advate, Kogenate, Recombinate, BAX 326, Other recombinant *TOTAL CONSUMPTION = all men)oned drugs excluding Feiba and NovoSeven N=201

Podobné dokumenty

Hemofilie v ČR Výsledky a úhrada léčby

Hemofilie v ČR Výsledky a úhrada léčby Jan Blatný, Petra Ovesná jménem a pro Centra sdružená v Českém národním hemofilickém programu Demografie osob s hemofilií v ČR (ČNHP data) Sample size - Haemophilia