CF: Information for Case Managers. Cindy Capen MSN, RN Pediatric Pulmonary Division University of Florida
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1 CF: Information for Case Managers Cindy Capen MSN, RN Pediatric Pulmonary Division University of Florida
2 About your speaker 25+ years in pulmonary Coordinator for CF Center and CF Newborn screening program Grandmother of 2 year old twins who are cute, cute, cute!
3 Cystic Fibrosis 101 Where we are today What s made the difference? Cost Hopes for the future
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5 A chronic life-shortening, genetic disorder that primarily affects the lungs and the gastrointestinal tract
6 Lung disease is the predominant cause of illness and death (68.3%)
7 Lower Respiratory Manifestations Persistent cough/wheeze Recurrent pneumonia Abnormal PFT s Sputum production Abnormal CXR Organisms
8 Blocked pancreatic duct Malabsorption Obstruction Bloating/Discomfort Large, greasy stools Prolapse
9 Inheriting CF Autosomal Recessive Gene Mom CF/N Dad CF/N CF/CF CF/N CF/N N/N Same risk with EVERY pregnancy!
10 1 in 3200 Caucasians 94.7% 1 in African- Americans 4% Hispanic 6.9%
11 Electrolyte Imbalance Respiratory 20.5% Abnormal Stools 7.8% Family History 13.4% CF Rectal prolapse 0.9% Nasal polyps/sinus 3.5% Mec Il/Intestinal Obst 8.8% FTT/Malnutrition 9.7% Where could You come in? Neo screening 57.5%
12 Percent of New Diagnoses by Newborn Screening Percent '90 '91 '92 '93 '94 '95 '96 '97 '98 '99 '00 '01 '02 '03 '04 '05 '06 '07 '08 '09 Year
13 Costs Families Psychosocial Time Money CF Centers Staff Care management Prior authorizations Payors More and more costly medications Equipment
14 Patients & Families Medications 2-3 hours/da Copays Access Airway clearance 1 hour/da Costly? Cumbersome? Hospitalizations Time/money/work loss/pain/sleep disturbance Clinic visits Time/money/fear Emotional drain of chronic illness/fear/restrictions
15 CF Care Centers CFF Accreditation Requirements Interdisciplinary team Facility requirements Data reporting Standards of care: know them use them! QI projects National meetings Barriers to care Prior authorizations Access to care/assistance Economy Family factors
16 Third party payors Determination of medical necessity Science Art Kitchen sink Increasing cost of new drugs/therapies Cost shifting to providers?
17 success of Mechanics
18 Patients Evaluated by a Respiratory Therapist, by CF Center 100% 80% Percent 60% 40% Lung function testing, airway clearance, correct administration of medications is pivotal 20% 0% The national rate is 89.2 percent (green bar). The range is 20 to 100 percent.
19 Patients Evaluated by a Social Worker, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 77.3 percent (green bar). The range is 0 to 100 percent.
20 Lung function and nutrition are clearly linked
21 Physicians and Nurses?
22 Family Partners still optional but recognized for their value
23 Number of Respiratory Cultures vs. Age 100% 80% 60% None Percent Four or more Three 40% Two One 20% 0% 0 to 5 6 to to to to to Overall Overall, 44.5 percent of patients get four or more respiratory cultures a year.
24 Age Specific Prevalence of Respiratory Organisms, % 80% P. aeruginosa Any Staph 60% Percent of Patients 40% H. influenza MRSA S. aureus 20% S. maltophilia B. cepacia complex 0% 0 to 1 2 to 5 6 to to to to to Age (Years) P. aeruginosa 51.7% H. influenza 16.0% B. cepacia complex 2.7% S. aureus 51.3% S. maltophilia 12.7% MRSA 23.7% Any Staph 65.8%
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26 Number of Pulmonary Function Tests vs. Age 100% 80% 60% None Four or more Percent Three Two 40% One 20% 0% 0 to 5 6 to to to to to Overall Overall, 74.4 percent of patients had two or more pulmonary function tests in 2009.
27 Patients 6 to 18 Years With 4 Visits, 1 Culture, and 2 PFTs, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 72.9 percent of patients who meet all three criteria (green bar). The range is 7.7 to 100 percent.
28 Influenza Immunization: Documented as Done in Patients 6 Months of Age, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 71.5 percent (green bar). The range is 0 to 98.6 percent. The CDC s Advisory Committee on Immunization Practices recommends influenza vaccination for all patients with CF 6 months of age 2.
29 Glucose Screening in Non-Diabetic Patients 14 Years, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 74.7 percent (green bar). The range is 14.3 to 100 percent. The CFRD guidelines recommend screening patients 14 years of age with at least a random blood sugar measurement 3.
30 Fat Soluble Vitamins Measured, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 78.2 percent (green bar). The range is 0 to 100 percent.
31 Median Weight-for-Length Percentiles for Patients < 2 Years, by CF Center Goal Percentile The national median weight-for-length percentile is 47.8 (green bar). The range is 9.5 to A center must have five patients < 2 years old to be represented. The goal established by the nutrition guidelines committee is 50th percentile weight-for-length by 2 years of age 7.
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33 Percent Taking Pancreatic Enzymes in Patients < 2 Years, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 84.4 percent (green bar). The range is 23.1 to 100 percent. A center must have five patients < 2 years old to be represented.
34 Oral or Tube * Supplemental Feeding in Patients 2 to 20 Years With BMI < 50th Percentiles, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 72.2 percent (green bar). The range is 16.7 to 100 percent. The nutrition guidelines committee recommends the use of nutritional supplements in addition to usual dietary intake for children with growth deficits 7. * Gastrostomy, nasogastric, jejunostomy, and parenteral feeding.
35 30 Median Total Duration (Hospital and Home IV) of Treatment for a Pulmonary Exacerbation in Patients < 18 Years, by CF Center Days The national median length of treatment for a pulmonary exacerbation in patients < 18 years is 15 days (green bar). The range is 5.5 to 26.3 days. A center must have five patients with an exacerbation treated by IV therapy to be included in this graph.
36 100% Dornase alfa (Pulmozyme ) Use * in Patients 6 Years, by CF Center 80% 60% Percent 40% 20% 0% The national rate is 77.5 percent (green bar). The range is 20.5 to 100 percent. Of all patients taking Pulmozyme at any encounter during the year, 13.7 percent use it twice a day [BID use by disease severity: severe (< 40) is 27.6 percent, moderate (40-69) is 19.2 percent, mild (70-89) is 11.5 percent, and normal ( 90) is 7.1 percent]. * At any encounter in the year.
37 Chronic Inhaled Tobramycin (TOBI ) Use * in P. aeruginosa Positive Patients 6 Years, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 68.7 percent (green bar). The range is 18.2 to 100 percent. * At any encounter in the year.
38 Chronic Macrolide Use * in Eligible Patients 6 Years, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 67.5 percent (green bar). The range is 9.1 to 100 percent. Patients were considered eligible if they met the selection criteria used in the U.S. Azithromycin trial. 15 * At any encounter in the year.
39 Hypertonic Saline Use * in Patients 6 Years, by CF Center 100% 80% 60% Percent 40% 20% 0% The national rate is 46.2 percent (green bar). The range is 0 to 93.3 percent. * At any encounter in the year.
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42 Case management Where to get the medications What equipment is needed Where tests can be done and how to order them Funding sources What is most necessary?...if you have to choose Resources for food, travel, copays Coordinating tests (annual labs, bone scans, lung functions, xrays/ct, GI testing, referrals, home care, pharmacy services, audiology) Cohorting clinics Solving school issues Summer activities Helping families find a balance
43 Building blocks of success? Blood work Vitamins zithromax Nutrition Supplements Clinic Visits Tuneups Cultures Nurses Nutritionist Social Worker Physicians Respiratory Therapists Pulmozyme TOBI Hypertonic Saline Airway Clearance Exercise Enzymes
44 You have to be really careful that a chronic illness doesn t become your whole life From the parent of a child with a chronic illness
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46 Predicted age of survival 38.3 years 47.4% of patients are over 18 years Range of ages is 1-74 years
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49 Simpler drug delivery A pill to treat the defect
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51 The drug has been shown to be remarkably effective at diminishing symptoms and limiting disease progression. The yearly cost for a prescription of Kalydeco is slated to be $294,000.
52 Building blocks of success? Vitamins Nutrition Supplements Blood work Clinic Visits Nurses Nutritionist Social Worker Physicians Enzymes
53
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