Presentació a Milà

1. Joan Escarrabill MD Institut d’Estudis de la Salut Barcelona [email_address] Home care in neuromuscular patients: generalists or specialized teams? Milano. Venerdì 6 febbraio 2009 “ Hot topics ” nelle Malattie del Motoneurone

3. BMJ 2002;324:1350 The evidence-based medicine is not an exclusive element for making clinical decisions It is not easy to define good clinical practice

4. Haynes RB, Devereuax PJ, Guyatt GH. BMJ 2002;324:1350

5. BMJ 2005;330:1007-11.

6. BMJ 2005;330:1007-11.

8. J Neurol Neurosurg Psychiatry 2004;75:1753-55 Scottish ALS Register n=1226 2.4 months Medical nihilism ? Riluzole & PEG use increases Patient autonomy Less aggressive therapy

9. Survival in ALS patients with bulbar involvement Farrero et al. Chest 2007;127:2132-8 NIV Tolerance NIV intolerance NIV Tolerance NIV intolerance NIV Tolerance NIV intolerance Hypercapnia Normocapnia

10. Bulbar impairment Bulbar Non-Bulbar Bourke SC. Lancet Neurol 2006;5:140-7

11. ALS: Acute chest infection Servera E. J Neurol Sci 2003;209:111-3 65 yrs old man Daytime Mouth piece Nasal mask for nocturnal use

12. Lancet Neurol 2006;5:140-7

13. Are NIV trials necessaries in ALS with non-bulbar impairement? Servera E. Sancho S. Lancet Neurol 2006;5:140-7 Ethical issues Non-bulbar patients in control group Stop studies according the results Technical issues Assessment effects of NIV Pressure vs volume ventilators Secretion management It’s mandatory to evaluate therapy “package”

14. Survival on HMV Laub M & Midgren B. Respir Med 2007;101:1074-8 n=1526 ALS

15. Kurian KM et al. J Neurol Neurosurg Psychiatry. 2009;80:84-7. 44 patients undergoing autopsy 73% respiratory causes Scottish Motor Neurone Disease Register

17. Brain 2008 ; 131 : 2729 - 2733 Sweden 1965 - 2004 6642 patients R elative risk is higher during the earlier stage of the disease , within the first year after the patient’s first period of hospitalization Suicide 3,6 21 Predicted ALS partients

18. ALS: Therapy a t the time of death NIV Tracheo Riluzole PEG Gil J et al. Eur J Neurol 2008 ; 15:1245-51

20. Mitsumoto H & Rabkin JG. JAMA. 2007;298:207-216 C are in multidisciplinary clinics is associated with enhanced quality of life by alleviating symptoms and may extend survival

21. Mitsumoto H & Rabkin JG. JAMA. 2007;298:207-216

22. Zoccolella S et al. J Neurol 2007;254:1107-12 No improvements in survival: Low rate of interventions?

23. Survival of Irish ALS patients One year mortality was decreased by 29.7%

24. Survival of Irish ALS patients with bulbar onset Prognosis of bulbar onset patients was extended by 9.6 months

25. ALS patients who received their care at a multidisciplinary clinic had a better prognosis Recruitment bias ALS clinic treated a group of fitter ALS patients General neurologists saw all ALS patients Living further from ALS clinic More disabled Increased age Bulbar onset Shorter duration of illness Hutchinson M. J Neurol Neurosurg Psychiatry 2004;75:1208-12

26. Effect of referral bias Sorenson EJ et al. N eurology 2007;68:600-602 132 subjects Tertiary center 3 years. Survival p = 0.007 18 months 29 months local population referral population

27. J Neurol Neurosurg Psychiatry 2006;77:948-50 Tertiary center Neurology clinic 1080 days 775 days The median survival from onset was 10 months longer in ALS centers 4 yrs younger PEG & NIV more often Less hospital admissions

28. Chest 2007;127:2132-8 Early systematic respiratory evaluation is necessary to improve the results Of HMV in ALS Survival in patients without bulbar involvement Protocol Pre-Protocol

29. www.has-sante.fr/ 2003 17 Reference centers Coodination Working groups Local organization

30. Evalutaion of ALS reference centers

31. Home care organized through reference centers has many limitations Complex organization, D istance, R esponse to emergenc ies In most cases the reference center coordinates care but it can not assume direct care

32. Reference centers: benefits and limits Improve skills & knowledge Distance Unnecessary referals Health professionals workload + -

35. JAMA 1998 ; 279 :1364-1370

36. Can Fam Physician 2006;52:1563-1569. “ Because ALS is a complex disease, care of ALS patients is best provided at multidisciplinary clinics that specialize in managing patients with this disorder ”

37. Paul Bonisteel MD CCFP FCFP canadian rural physician Bonisteel P. Can Fam Physician. 2007;53: 402. T he multidisciplinary team is an urban construct that works from a geographically fixed site Living 100 km from the capital city , the team means to use existing resources in the community

38. G eneral practitioner s are more accessible s and closer than the center of reference, but they can not work without the support of experts Home care of patients with ALS without the support of experts is unacceptable

40. Generalists or specialized teams: only? Generalists Specialized teams Patients

41. www.patientslikeme.com/

42. Sweden 1965 - 2004 6642 patients 40 years 3 years

43. Three stars PALS

45. Equipment

46. NIV n=345

48. Anxiety Constipation

49. PNAS 2008 ; 105 : 2052–2057 n=44 16 : Riluzole + Lithium 28 : Riluzole

51. http://alslithium.atspace.com/

52. www.its.caltech.edu/~kfelzer/SixMonthUpdate.pdf n=191 37% stopped before 6 months side effects lack of efficacy doctor’s advice

56. Generalists or specialized teams: only? Generalists Specialized teams Support network

57. Community nurse Home care General practitioner Resources in the community RRT Social worker Occupational therapist Multidisciplinary team

58. Network Reference center General practitioner Support network I nformation technology and communication Escarrabill J. Arch Bronconeumol 2007;43:527-9 Patient-centered care : accessibility vs performance

61. The “S. Maugeri” Telepneumology Programm Pulse oximetry / HR Pneumotacograph Central workstation on call Tutor nurse Vitacca M. Telemed & e-Health 2007;13:1-5 Technical elements Health professional access General support Nurse solving problems Access to pneumologist on duty 24 h/day Educational material Link with GP Telemetric monitoring

62. Community nurse Home care General practitioner Resources in the community RRT Social worker Occupational therapist Multidisciplinary team Support team Hospice

63. www.slideshare.net/jescarra Grazie per la sua attenzione !