Dr Pop

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Dr Pop

  1. 1. National Cystic Fibrosis Center Timişoara Clinic II Pediatrics – University of Medicine and Pharmacy “Victor Babeş” Timişoara IMPORTANCE OF THE NATIONAL CYSTIC FIBROSIS NETWORK- THE EXPERIENCE OF NATIONAL CYSTIC FIBROSIS CENTRE TIMIŞOARA
  2. 2. IMPORTANCE OF THE ISSUE  THE MOST FRECQUENT DISEASE WITH AUTOSOMAL RECESSIVE TRANSMISSION OF POPULATION WITH CAUCASIAN ORIGIN, WITH CHRONIC PROGRESSIVE EVOLUTION AND POTTENTIALY LETAL. ONE OF 2000-2500 NEWBORN IS SICK(HAS THE DISEASE)  INCREASE FRECQUENCY OF CARRIERS 1:25  IN THE ABSENCE OF AN EARLY DIAGNOSIS (NEWBORN OR AT LIST IN INFANCY) AND CORRECT MANAGEMENT, SURVIVAL RATE DON`T OVERCOME TODDLER AGE. RESPIRATORY DISEASE IS THE ONE WHO INFLUENCE THE GRAVITY AND DISEASE`S PROGNOSIS .
  3. 3.  CURENTLY THE LIFE EXPECTANCY AND THE QUALITY OF LIFE FOR PATIENT WITH CYSTIC FIBROSIS HAS BEEN SIGNIFICANTLY IMPROVED , ON ONE HAND BECAUSE OF ACHIEVEMENT IN DISEASE`S KNOWLEDGE (AFTER THE DISCOVERY OF THE GENE AND DEFECTIVE PROTEIN DISEASE– CFTR), ON THE OTHER HAND THROUGH DEVELOPMENT OF A COMPLEX SYSTEM OF TREATMENT AND CARE. UNDER AEGIS OF CYSTIC FIBROSIS WORLDWIDE, AND WHO HEALTH PROGRAMS ARE ELABORATED AND GIVES SUPPORT MATERIAL FOR IMPLEMENTATION AND LOGISTIC SERVICE FOR DEVELOPING COUNTRIES.
  4. 4. CF IS A COMPLEX AND COSTLY DISEASE , ESPECIALLY IN ADVANCED EVOLUTION, THEREFORE ANY MESURE TREVENTING THE DISEASE PROGRESSION SHOULD BE APPLIED WITHOUT ANY LIMITATION.  IT MUST BE ASSURED ALL ORGANIZATIONAL INFRASTRUCTURE AND CONDITIONS TO ALLOW AN OPTIMAL DISEASE MANAGEMENT  TEAM THERAPY, ORGANIZED IN SPECIAL CENTERS , IN COLLABORATION WITH NATIONAL ORGANIZATIONS (GOVERNMENTAL AND NON-GOVERNMENTAL) OR INTERNATIONAL ASSOCIATIONS (ESCF, CFW, SMAC, IPG / CF ETC), DIFERENTIATED FOR CHILDREN AND ADULTS
  5. 5. EXTRAPOLATION – ROMÂNIA IS AN EUROPEAN COUNTRY WITH PREVALENT CAUCASION POPULATION COMMON ISSUES WITH OTHER EUROPEAN COUTRIES ECONOMIC ISSUES INVOLVED CYSTIC FIBROSIS  AN IMPORTANT ISSUE OF PUBLIC HEALTH  IS NECESSARY THAT HEALTH MINISTRY TO ACCEPT THIS REALITY
  6. 6. PATIENT FAMILY, FRIEND, SCHOOL, JOB PRIMARY CARE TEAM REGIONAL CENTER, NATIONAL ORGANIZATIONS, NON-GOVERNMENT– ASSOCIATIONS GROUPS RESEARCH ORGANIZATIONS, INTERNATIONAL ORGANIZATIONS  THEREFORE IS IMPERATIVE TO ORGANIZE THE CYSTIC FIBROSIS NETWORK-THE SO-CALLED CF PYRAMID
  7. 7. ORGANIZATION OF CYSTIC FIBROSIS NETWORK IN ROMANIA
  8. 8. CLINIC II PEDIATRICS TIMIŞOARA: Pioneering for over 40 years with two major objectives : - improving disease diagnosis; - expansion of knowledge at national level
  9. 9. YEARS `60: THE BEGININGS  first diagnosed children  first reports on the clinical and biological evolution of these children introduction of sweat test by pilocarpinic iontophoresis - diagnosis methodology at national level, with a device with innovating design, own patent
  10. 10. 1987: FOUNDATION OF THE CYSTIC FIBROSIS CENTRE TIMIŞOARA Providing the infrastructure of the diagnosis, surveillance and treatment according to European Guidelines Achieving over 80 years of neonatal screening Care team of children with CF includes: CF Specialist physioterapist dietician psichologist CF nurse patient geneticist social worker Family doctor
  11. 11. IN ROMÂNIA – 80`s: pilot study coordinated by CMV Timişoara and Clinic II Pediatrics: - “ MUCOTEST” : own design, similar to tests overseas, but much cheaper - - 4 counties in western country ; over 10 000 newborn tested - frequency 1: 2054 newborns At an average of 210 000 births per year should be an incidence of approximately 100 new cases of CF / year 2002 - Pilot study with IRT was initiated - abandoned due to cost and reduced infrastructure
  12. 12. Discrepancy between the epidemiology and practical books Causes:  insufficient knowledge of the disease in general and in particular of the specific symptoms in new born: meconium ileus , bile- plugg syndrome , right upper lung lobe atelectasis etc..; unfortunately, in Romania, many children with CF die of other diagnoses  low compliance of decisional organization  impossibility of extending screening by the IRT dosage nationally because of high costs of reagents;
  13. 13.  Knowledge of the disease`s warning signs can successfully replace screening, thereby the patient to be referred immediately to a specialized center for sweat test is the optimum. Sino-pulmonary symptoms Gastrointestinal symptoms Others -chronic cough -meconium ileus -family history of CF -neonatal cholestasis -deceased brothers as -recurrent respiratory -chronic diarrhea newborn disease -steatorrhea -salty taste of sweat -recurrent atelectasis -recurrent pancreatitis -salt crystals on the skin - bronchiectasis -heat shock -respiratory chronic -hipoprotrombinemia infections -weight stationary or very -nasal poliposis slow growth –recurrent pasinusitis
  14. 14. 2. POSTNATAL DIAGNOSIS: For Romania, a country with low economic potential, is currently the only feasible alternative for improving the situation of the disease, involving :  disease knowledge;  early diagnosis;  application of correct management. organizational framework and specific infrastructure NATIONAL HEALT PROGRAMME SPECIALISED SERVICIES to allow  consistency at national level of various activities of primary and secondary prevention;  knowledge at any time of the epidemiological situation  reduce financial hospitals pressures .
  15. 15. 1990: APRIL: FOUNDATION OF ROMANIAN CYSTIC FIBROSIS ASSOCIATION(ARM) based in Timişoara and association's periodic publishing – “Buletin ARM” ↓ NGO: promotes the interests of patients with CF and their families
  16. 16. 1996: INTERNATIONAL PHISIOTHERAPY COURSE ON CYSTIC FIBROSIS FOR EAST EUROPEAN COUNTRIES (Timişoara 23-28 September) under CFW aegis : - attendees from 5 europeane countries; - lecturer from UK and Sweden; - course with the largest scale to date
  17. 17. 1997: Ministry of Health attribute country coordinator on this issue for Cystic Fibrosis Center Timişoara : - nationally better record of the case ; - possibility of drawing up the National Registry of CF patients - By this time - evidence for only 240 patients, of which 25 adults that is well below the real frequency
  18. 18. 2001: CF in children has been introduced for the first time in a national health program, CF Centre being a coordinating technical institution Technical rules are developed for implementing the National Health Program. From the program several medical centers were equipped with electronically sweat tests devices, with automatic reading. Delegate doctors on cystic fibrosis issue are appointed in universitary centers.
  19. 19. 2003: FIRST NATIONAL CYSTIC FIBROSIS CONGRESS WITH INTERNATIONAL PARTICIPATION – Timişoara 7-9 mai
  20. 20. 2004  Translated by MV for patients and their families "The manual for Cystic Fibrosis Patients and their Parents" by WHO and ICF (M) A, under the European Initiative undertaken by the Action for Cystic Fibrosis .  Foundation of National Working Group for Cystic Fibrosis
  21. 21. 2005:  Organization of basis national network  In National CF Centre Timişoara, in collaboration with the Department of Molecular Biology of the Biochemistry Department - UMF Timisoara is performed the genetic methodology for prenatal and postnatal genetic diagnosis
  22. 22. 2006:  MS Order No 247 / 16 03 2006 declares CF Center Timişoara as National Center and approve the organization of Regional Centers of Cystic Fibrosis
  23. 23. RESULTS  In recent years has increased interest and expansion of concerns regarding cystic fibrosis on a national scale : -diagnosis is completed earlier and genetic diagnosis is also performed; - standards of care have been improved - Improved quality of life of CF patients and increased the life expectancy (50 adults showing)
  24. 24. The survival rate, depending on diagnosis time ( trend lines) 18 16 14 12 10 8 6 4 2 0 diag 5 years 10 years 0-1 an 1-3 ani 3-5 ani 5-8 ani peste 8 ani
  25. 25. REDUCTION OF MINIMAL DRUGS CONSUMPTION/YEAR - patient with good clinical condition, no admission necessary : 55 000 – 60 000 lei / an (including Pulmozyme treatment) - patients with frequent admissions (one /quarter): plus 25 000 – 30 000 lei (without medication)
  26. 26. QUALITY OF LIFE Report episodes of admission for respiratory exacerbation : - diagnosed late: 4-8 admissions/ year - diagnosed on time: 2-3 admissions / year, usually for clinical , biological and pulmonary function assessment Bronchiectasis occurrence: - among patients diagnosed over 10 ani is a rule Pulmonary function evaluation (FEV): - in patients diagnosed in time: decrease in steps with long periods of secure respiratory parameters; - those diagnosed in advanced stages of disease ( FEV< 60%), the disease evolution is fast and progressive descendent , especially if they are chronically infected with Pseudomonas
  27. 27. ADULT WITH CF CONCERN:
  28. 28.  In România there is no organizational framework to enable taking care of these patients  Abroad, in addition to specialized medical centers for the CF adult , there are national associations of adults with cystic fibrosis  Since 2003 in România also there is an adult's association - AAFC - based in Focsani. However, as a positive sign, a part of the medication, it is currently free for adults also
  29. 29.  Levels of care are the same for adults as for children with CF: local, regional and national center  Team care for adults with CF will be completed with gastroenterologist, pulmologist, surgeon, gynecologist, endocrinologist (Diabetolog) etc
  30. 30.  ADULTS WITH CF HAVE MANY DIFFICULTIES REGARDING: •THE DECISION TO INDEPENDENT LIVE • CAREER CHOICE AND JOB SELECTION IN THE SITUATION OF LIMITS IMPOSED BY THE DISEASE • FOUNDATION OF A FAMILY • STERILITY A ND PREGNANCY MATTER • SOCIAL ADAPTATION • IMPACT OF DISEASE AGGRAVATION In the face of these complex problems, interdisciplinary care team must be a highly professional support because QUALITY OF LIFE IS AS IMPORTANT AS HIS PROLONGATION
  31. 31. PATIENT FAMILY, FRIENDS, SCHOOL, JOB PRIMARY CARE TEAM REGIONAL CENTERS, NATIONAL ORGANISATIONS, NON-GOVERNMENTALS GROUPS RESEARCH ORGANIZATIONS, INTERNATIONAL ASSOCIATIONS

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