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Optimizing Patient Outcomes: Clinical Use of the Cystic Fibrosis Pulmonary Guidelines Michael P. Boyle, MD, FCCP John Paul Clancy, MD Kristin A. Riekert,

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Presentation on theme: "Optimizing Patient Outcomes: Clinical Use of the Cystic Fibrosis Pulmonary Guidelines Michael P. Boyle, MD, FCCP John Paul Clancy, MD Kristin A. Riekert,"— Presentation transcript:

1 Optimizing Patient Outcomes: Clinical Use of the Cystic Fibrosis Pulmonary Guidelines Michael P. Boyle, MD, FCCP John Paul Clancy, MD Kristin A. Riekert, PhD

2 Michael P. Boyle, MD, FCCP Associate Professor of Medicine Director, The Johns Hopkins Adult Cystic Fibrosis Program The Johns Hopkins University School of Medicine Baltimore, MD John Paul Clancy, MD Professor, Director, and Raymond K. Lyrene Chair in Pediatric Pulmonology The Children’s Hospital of Alabama University of Alabama at Birmingham Birmingham, AL Kristin A. Riekert, PhD Assistant Professor Co-Director, The Johns Hopkins Adherence Research Center Division of Pulmonary and Critical Care Medicine The Johns Hopkins University Baltimore, MD Faculty

3 Dr Boyle reports having no financial or advisory relationships with corporate organizations related to this activity. Dr Clancy reports having no financial or advisory relationships with corporate organizations related to this activity. Dr Riekert reports receiving grants/research support from Genentech, Inc. and Novartis Pharmaceuticals Corporation. Disclosures

4 I. Cystic Fibrosis (CF) Overview and Introduction (1 min) Michael P. Boyle, MD, FCCP II. Aggressive Treatment Strategies to Optimize Patient Outcomes (25 min) John Paul Clancy, MD III. Promoting Adherence and Increasing Life Span (25 min) Kristin A. Riekert, PhD IV. Optimizing Patient Outcomes: An Illustrative Case (Slide Presentation, 8 min) Michael P. Boyle, MD, FCCP V.Conclusion and Summary (1 min) Michael P. Boyle, MD, FCCP Agenda

5 Upon the conclusion of this activity, the participant should be able to: Evaluate current and emerging therapies and the delivery mechanism of these therapies. Recognize and identify adherence issues in patients with cystic fibrosis. The Johns Hopkins University School of Medicine takes responsibility for the content, quality, and scientific integrity of this CME activity. Learning Objectives

6 ACCREDITATION STATEMENT— The Johns Hopkins University School of Medicine is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. CREDIT DESIGNATION STATEMENT— The Johns Hopkins University School of Medicine designates this educational activity for a maximum of 1.25 AMA PRA Category 1 Credit(s) TM. Physicians should only claim credit commensurate with the extent of their participation in the activity. Accreditation & Credit Designation Statements

7 Johns Hopkins would like to acknowledge an educational grant from Novartis Pharmaceuticals Corporation which helped to make this activity possible. Educational Grant

8 Optimizing Patient Outcomes: Clinical Use of the Cystic Fibrosis Pulmonary Guidelines Michael P. Boyle, MD, FCCP John Paul Clancy, MD Kristin A. Riekert, PhD

9 Aggressive Treatment Strategies to Optimize Patient Outcomes John Paul Clancy, MD The Children’s Hospital of Alabama University of Alabama at Birmingham

10 Overview Changing the face of CF Improving longevity New challenges, new opportunities Pulmonary treatment guidelines for adolescents and young adults CFF panel (RTs, nurses, MDs, and CFF personnel) Future and emerging strategies Ion transport-active agents New classes/delivery of antibiotics Monitoring for pathogens Adherence Cystic fibrosis pulmonary guidelines Chronic medications for maintenance of lung health Flume, PA et al. Am J Respir Crit Care Med V176. pp , CF = cystic fibrosis.

11 Improving Longevity in CF Survival by birth cohort Age (Years) Percent Surviving 1980– – – –2004 Reprinted with permission from Cystic Fibrosis Foundation Patient Registry Annual Data Report. Bethesda, MD.

12 Basic CF Care Paradigms Coordinated Care CF Care centers Optimize: Education Nutrition Exocrine pancreas (and fat soluble vitamins) Endocrine pancreas Hepatobiliary disease GI ion transport *Pulmonary care Reproduction Other

13 Photos – compliments of Carlos Milla. Example of growth curve - Nancy CF airway obstruction CF Lung Disease – Macroscopic

14 Pulmonary Care Rationale of Treatment Strategies Disease manifestations and Targets: CFTR (1) *Ion transport (2) *Clearance (3) *Antimicrobials (4) *Inflammation (5) Reprinted with permission from Advances in cystic fibrosis therapies Rowe, SM and Clancy, JP. Curr Opin Pediatr V18(6): pp , 2006.

15 Sweat abnormalities Airway clearance Family-centered care Enzyme replacement Anti-PsA antibiotics Fat-full diet Cl - impermeability CFTR identified DNase AZM Hypertonic saline Ion transport agents CFTR modulators Nebulized atbx NSAIDs Timeline of CF Therapies From 1950’s to 2010: childhood ---> childhood and adult disease Inhaled tobramycin

16 Complexity of Current CF Pulmonary Care Recombinant DNase TOBI Hypertonic saline Inhaled colistin Inhaled gentamicin Inhaled steroids Azithromycin Anti-staphylococcal antibiotics Inhaled  2 agonists Inhaled anticholinergics Leukotriene modifiers Systemic steroids ?

17 Assessing the Evidence for Use of Current Pulmonary Therapies Systematic reviews of original research Modified systematic reviews Existing Cochrane systematic reviews Reprinted with permission from Cystic Fibrosis Pulmonary Guidelines Chronic medications for maintenance of lung health Flume, PA et al. Am J Respir Crit Care Med V176. pp , Quality of data Strength of recommendations (A > B > C > D; I)

18 Recombinant Human DNase Age > 6 yo Moderate to severe disease (FEV 1 <69%) Ten RCT, 3 cross-over, six without comparator, Cochrane review (2005) Total n = 3140 Recommendation = A (strength of evidence good, benefit substantial) Mild disease (FEV 1 = 70%–89%) Three RCT, one cross-over Total n = 520 Recommendation = B (strength of evidence fair, benefit moderate) +

19 Chronically Inhaled Tobramycin PsA + (persistent), and > 6 yo Moderate to severe disease (FEV 1 <69%) Three RCT, one RCO, two 1-arm trials, Cochrane review (2006) Total n = 679 Recommendation = A (level of evidence good, net benefit substantial) Mild disease (FEV 1 = 70%–89%) Two RCT (n = 202) Recommendation = B (level of evidence fair, net benefit moderate) Other inhaled antibiotics Recommendation = I (level of evidence poor, net benefit small)

20 Reprinted with permission from Ramsey B, et al. N Engl J Med. 1999;340(1): Inhaled Tobramycin (300 mg BID) Phase III trial, 24 weeks randomized, placebo-controlled

21 Hypertonic Saline Age > 6 yo Three RCT, one cross-over trial, and six trials without comparators Total n = 520 Two RCT, two RCO compared with recombinant DNase Total n = 284 Cochrane review (2005) Recommendation = B (level of evidence fair, net benefit moderate)

22 Anti-inflammatory Agents Inhaled corticosteroids (age > 6 yo) No asthma, no ABPA Five RCT, two RCO, Cochrane review (2006) Total n = 388 Recommendation = D (level of evidence fair, net benefit zero) Oral/systemic corticosteroids (age 6–18 yrs) No asthma, no ABPA Three RCT, Cochrane review (2006) Total n = 354 Recommendation = D (level of evidence good, net benefit negative) Oral/systemic corticosteroids (adults) No asthma, no ABPA One RCT (Total n = 20) Recommendation = I (level of evidence poor, net benefit zero)

23 Anti-inflammatory Agents (cont’d) Oral nonsteroidal anti-inflammatory drugs (NSAIDS) Three RCT, Cochrane review (2005) Total n = 145 Recommendation = B (level of evidence fair, net benefit moderate) Leukotriene modifiers Two RCO, one controlled trial Total n = 64 Recommendation = I (level of evidence poor, net benefit none) Cromolyn Two RCT, one clinical trial Total n = 44 Recommendation = I (level of evidence poor, net benefit none)

24 Macrolides (chronic) PsA + (persistent), and > 6 yo Two RCT, one crossover trial, one clinical trial, Cochrane review (2005) Total n = 296 Recommendation = B (level of evidence fair, net benefit substantial)

25 Day % Exacerbation-free Placebo Azithromycin OFF Reprinted with permission from Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. Saiman, L. et al. JAMA. Oct 1;290(13): , Day Placebo Azithromycin * * * OFF FEV1 (% predicted) Azithromycin in PsA+ CF Patients

26 Anti-staphylococcal Antibiotics Patients with CF (most studies in children, including < 6 yo) Use of prophylactic antibiotics in Sa (+) patients Three RCTs, one crossover trial Cochrane review (2006) Total n = 306 Recommendation = D (level of evidence fair, net benefit negative)

27 Bronchodialators Patients > 6 yo  2 adrenergic agonists Fourteen RCO (mix of nebulized/MDI) Total n = 257 Recommendation = B (level of evidence good, net benefit moderate) Anticholinergics Five RCO Total n = 79 Recommendation = I (level of evidence poor, net benefit none)

28 Summary (patients over age 6 yrs) Class A recommendations (substantial benefit) Recombinant DNase Inhaled tobramycin (PsA +) Class B recommendations (moderate benefit) NSAIDs (ibuprofen) Macrolides (azithromycin) Bronchodialators (  2 adrenergic receptor agonists) Hypertonic saline (7%) Class D recommendations (no benefit or potential harm) Oral corticosteriods ( 6–18 yrs, chronic) Inhaled corticosteroids Anti-staphylococcus antibiotics (chronic) Class I recommendations (insufficient information) Leukotriene antagonists, oral corticosteroids (adults), anticholinergics, N acetyl cysteine, and cromolyn

29 Pediatric CF Care Trends – Influence on Young Adult Care Relationship between PsA infection and lung function Inverse PsA eradication strategies Various antibiotic approaches Development of a ‘new’ young adult CF population Lack chronic (mucoid) PsA infection Careful monitoring Aggressive intervention for new infections Starner T and McCray P. Ann Intern Med. 2005;143:

30 Time Uninfected Lung Function 100 % Transient Bacterial Infection Eradication Chronic Bacterial Infections Mucoid/Biofilm Bacterial Infection Inflammation Irreversible Lung Damage Adapted from Starner T and McCray P. Ann Intern Med. 2005;143: Slide compliments of France Foundation. Infection with Pseudomonas aeruginosa and Progression of CF Lung Disease

31 Reprinted with permission from Cystic Fibrosis Foundation Patient Registry Annual Data Report. Bethesda, MD. prevention management Prevalence of Respiratory Infections in CF Patients Over Ages

32 Reprinted with permission from Li Z, et al. JAMA. 2005;293(5): No PsA Nonmucoid PsA only Mucoid PsA +/- Nonmucoid PsA Age-Specific Prevalence of P. aeruginosa from Birth to Age 16

33 Reprinted with permission from Li Z, et al. JAMA. 2005;293(5): Pa(-) Non-mucoid Pa (+) mucoid Pa (+) Association of Mucoidy with Decline in FEV1 % Predicted

34 Available at: Accessed March Randomized, double-blind, placebo- controlled, safety and efficacy study 54 US Centers 300 CF patients 1–12 years old Primary Outcome: Proportion of recurrent Pa positive cultures (18 months) Time to occurrence of pulmonary exacerbations QT: quarterly treatment PsA +: treatment only when quarterly cultures are + for P. aeruginosa Month Tobramycin + Ciprofloxacin QT PA+ QT PA+ Tobramycin + Placebo QT PA+ QT PA+ Slide compliments of France Foundation. Early Pseudomonas Infection Control (EPIC) Phase 4 – closed to enrollment

35 Summary – Pulmonary Treatment Guidelines Many options available Address many aspects of CF lung disease Strength of recommendations varies Key features: Pseudomonas aeruginosa status Chronic management Emerging eradication strategies Few head to head comparisons Burden of care increasing (accelerating) Optimal outcomes ► adherence strategies

36 Promoting Adherence and Increasing Life Span Kristin A. Riekert, PhD The Johns Hopkins University

37 Composite Medication Possession Ratio (MPR) Reprinted with permission from Riekert KA, et al. Pediatr Pulmonol. 2007;S30:405.

38 Emerging Adulthood (aged 18–25 years) Identity exploration Role transitions Changes in residence, relationships, work, finances, becoming a parent, etc. Feeling “in-between” adolescence and adulthood Greater acceptance of responsibility for one’s self Greater autonomy in decision making

39 Social Support and Adherence Odds Ratio Functional Support Practical Emotional Family Functioning Cohesiveness Conflict Family Structure Married Living with someone vs. alone Except for conflict, odds ratio=odds of being adherent if score on variable is high. DiMatteo MR, et al. Health Psychol. 2004;23(2):

40 Adherence Typologies

41 Erratic Adherence Patient understands and agrees with therapy but has difficulty consistently maintaining regimen

42 "I try to take my medicine regularly but I'm too... " Busy Forgetful/disorganized Stressed/family chaos Out of medicine Regimen too complex

43 Unwitting Nonadherence Patient and provider mistakenly believe that the patient is adherent

44 "But I thought I was taking my medicine right..." Misunderstands regimen Poor device technique Language barriers Cognitive deficits

45 Poor Knowledge Many patients and family members do poorly on a standardized measure of CF knowledge Average score parents = 79% Average score teens = 61% For example: How many knew that fat contains more calories that carbohydrates or proteins?  16% of parents  19% of teens Quittner, A. L., Drotar, D, and Ievers-Landis, C. Improving Adherence in Adolescents with Cystic Fibrosis: Comparisons of Family Therapy & Psychoeducation. Paper presented at the Society of Pediatric Psychology National Conference on Child Health Psychology, Charleston SC

46 Poor MDI/DPI Technique % Good Technique Patients28–68 1 House staff43–65 2,3 Physicians65 3 Nurses4–47 2 Respiratory Therapists85–92 2,3 Pharmacists62 4 MDI=metered dose inhaler, DPI=dry powder inhaler. 1. Fink JB, Rubin BK. Respir Care. 2005;50(10): Interiano B, Guntupalli KK. Arch Intern Med. 1993;153(1): Guidry et al. Chest. 1992;101(1): Kesten S, et al. Chest. 1993;104(6):

47 “Intelligent” Nonadherence Patient deliberately alters or discontinues therapy

48 "I don’t need to take my medicine because I..." Don’t think the therapy makes a difference to my health Feel fine/better = don’t need it Am concerned about side- effects Fear addiction/drug resistance

49 Beliefs About Asthma Medication 1,2 Parents of children Adult patients Hard to take meds when feel fine Hard to remember to get refills Nothing I can do to stop an attack Even if take meds, will still have sx Benefits of meds make side effects worth it Taking meds makes me worry more about asthma Sx = symptoms. Riekert KA, et al. Am J Respir Crit Care Med. 2003;167:A155. Riekert KA, et al. Am J Respir Crit Care Med. 2004;169:A328.

50 Additional Risk Factors Depression OR=3.03 (95% CI, 1.96 – 4.89) 1 Poor integration of CF into personal identity Healthy versus sick versus “normal” 2,3 1. DiMatteo MR et al. Arch Intern Med. 2000;160(14): ; 2. Badlan K. Health Soc Care Community. 2006;14(3): ; 3. Lowton K, Gabe J. Sociol Health Illn. 2003;25(4):

51 What Can the CF Clinician Do?

52 Assessing Adherence Objective measures (eg, medication refill history) are most valid Patient/family-report Overestimates adherence HIGH SPECIFICITY FOR REPORT OF NONADHERENCE!!! Open-ended and direct questions better POOR = Any problems with your medicines? GOOD = How have you been taking your dornase alpha? BETTER = Which, if any meds, have you been taking?... How do you use [name med]?

53 Pediatric Adherence with Inhaled Steroids Coutts JA, et al. Arch Dis Child. 1992;67(3):

54 Good Communication is Key Establish rapport Open-ended questions Reflective listening Normalize nonadherence Identify motivation to change

55 Strategies for Improving Adherence Erratic Adherence Query barriers and problem-solve Simplify and tailor regimen Behavioral strategies Self-monitoring (eg, diaries) Cueing (eg, toothbrush, pillbox) Reminders (eg, cell phone) Linking to established habits or pleasurable activities Reinforcement Encourage accessing social support

56 Strategies for Improving Adherence Unwitting Nonadherence Provide and review written treatment plan at each visit Ask patient to repeat dosing instructions Review device technique Provide CF education Encourage accessing social support

57 Strategies for Improving Adherence “Intelligent” Nonadherence Include patient in decision-making Provide personalized feedback on relationship between adherence and health outcomes Provide CF education Link therapy with personal goals

58 Look for “Turning Points” Often occurs during emerging adulthood First hospitalization Going away to college Beginning of a new romantic relationship Becoming a parent Can serve as a teachable moment Elicit these events during clinical encounter

59 Summary Emerging adults are at risk for nonadherence Time of significant yet normative life changes Query patient adherence every visit Be skeptical of self-report, but remember, a report of any nonadherence is true Identify factors contributing to nonadherence Match counseling strategies to identified barriers

60 Optimizing Patient Outcomes: An Illustrative Case Michael P. Boyle, MD, FCCP The Johns Hopkins University School of Medicine

61 Case History Ryan, 19-year-old young man with CF Genotype dF508/dF508 Baseline FEV 1 75% – 80% of predicted Chronically infected with mucoid P. aeruginosa

62 Case History “My parents always remind me how grateful I should be that I only have mild CF” “I find just staying active is the most important thing to keep me healthy”

63 Case History Other medications: Inhaled dornase alpha once a day Azithromycin 250 mg orally each day Pancreatic enzymes with each meal Vitamins First hospitalization last year to receive intravenous antibiotics for an exacerbation Subsequently prescribed chronic inhaled tobramycin for 28 days every other month

64 Case History Now in first semester at local University Missed last scheduled clinic visit because preparing for a math exam – now over 4 months since last visit Parents noted increased night time cough and encouraged him to be seen FEV 1 today 74% of predicted States: “I’m fine – the cough bothers my parents more than it bothers me”

65 Treatment Options A.Oral antibiotic and add hypertonic saline B.Oral antibiotic, add hypertonic saline and extra daily session of airway clearance C.Assess treatment adherence and tell him he needs to do better job of taking meds D.Something else?

66 “Intelligent” Nonadherence Patient deliberately alters or discontinues therapy

67 Ryan’s Actual Regimen: Enzymes, vitamins, azithromycin, occasional dornase alpha and “getting to the gym” Which, if any, of the medications have you been taking?

68 "I don’t need to take my medicine because..." convinced he only has “mild” CF feels fine = doesn’t need it doesn’t think the prescribed therapy is the most effective way to maintain health

69 Strategies for Improving Adherence “Intelligent” Nonadherence Include patient in decision-making Provide personalized feedback on relationship between adherence and health outcomes Provide CF education Link therapy with personal goals

70 Treatment Reviewed graph of FEV 1 values for last five years showing decline Obtained chest CT and reviewed images with Ryan so he could see presence of bronchiectasis, mucous plugging, and cystic changes

71 Treatment Prescribed oral ciprofloxacin and inhaled tobramycin, and proposed an experiment assessing FEV 1 before and after use of medication Scheduled follow-up in three weeks to reevaluate clinical status, support his decision for adherence, and provide further education

72 Follow-up Visit Taking medications FEV 1 79% of predicted Symptoms resolved Determined to make medications more of a priority in the future.

73 Summary: Improving Patient Outcomes Treatment Guidelines Early, aggressive therapy to optimize and maintain lung function Particular attention to initial P. aeruginosa Close monitoring of nutritional status and growth

74 Summary: Improving Patient Outcomes Understanding the Role of Adherence Assessing adherence Recognizing different types of nonadherence Utilizing treatment strategies specific for the type of nonadherence


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