Advances in Pulmonary Hypertension

Advances in Pulmonary Hypertension CME Section

Program Overview

Pulmonary arterial hypertension (PAH), an incurable disease, is characterized by medial hypertrophy, intimal fibrosis, and in situ thrombi in small muscular pulmonary arteries. PAH was considered a rapidly fatal illness with a median survival of 2.8 years in the 1980s when no evidence-based therapies were available. Since then the treatment of this disease has made tremendous advances, and the last 10 years have seen the discovery of new medications that have positively influenced the prognosis and survival of patients with PAH.

This self-study activity is based on 4 articles that review the
role of the multidisciplinary team in helping PH patients achieve better and longer lives.

This activity is jointly sponsored by the University of Michigan Medical School and the Pulmonary Hypertension Association and supported by an unrestricted education grant from Actelion Pharmaceuticals US, Inc, Gilead Sciences, Inc, Pfizer, Inc, and United Therapeutics Corporation.

Target Audience

This self-study activity is appropriate for cardiologists, pulmonologists, rheumatologists, and other physicians who treat patients with pulmonary hypertension.

Learning Objectives

Upon completion of this activity participants will be
able to:

  1. Discuss the state of health-related quality of life measurement in pulmonary hypertension (PH) and the various instruments used to date.
  2. Discuss the implications for pediatric patients and families of transitioning from pediatric to adult care and plan a successful transition.
  3. In collaboration with the PH team, develop a plan of care for PH patients that addresses care decisions, adherence to therapy, depression, and other issues affecting quality of life.

Self-Assessment Examination

See pages 237-238 for self-assessment questions, answer key, and evaluation form.

Faculty

Chair
Glenna Traiger, RN, MSN
Pulmonary and Critical Care
Pulmonary Hypertension CNS
University of California, Los Angeles

Contributing Authors

Ann F. Gihl, MA, RN
Procurement Manager
LifeSource
St Paul, Minnesota

Deborah McCollister, RN, BSN
Pulmonary and Critical Care
University of Colorado, Denver

Philippe Weintraub, MD
Psychiatry
University of Colorado, Denver

Traci Stewart, RN, MSN
Cardiomyopathy and Pulmonary Hypertension Treatment Programs
University of Iowa Hospitals and Clinics
Iowa City, Iowa

Michelle Ogawa, CPNP
Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford
Lucille Salter Packard Children's Hospital at Stanford
Palo Alto, California

Darci Albrecht, MSW
Vera Moulton Wall Center for Pulmonary Vascular Disease at Stanford
Lucille Salter Packard Children's Hospital at Stanford
Palo Alto, California

Agenda

Health-related Quality of Life in Pulmonary Arterial Hypertension
Ann F. Gihl, MA, RN

Depression and Pulmonary Arterial Hypertension: Should We be Screening for Depressive Symptoms?
Deborah McCollister, RN, BSN, and
Philippe Weintraub, MD

Facilitating Pulmonary Arterial Hypertension Medical Adherence: Patient-centered Management
Traci Stewart, RN, MSN

Adolescence to Adulthood: Safely Transitioning the Adolescent with Pulmonary Arterial Hypertension
Michelle Ogawa, CPNP, and Darci Albrecht, MSW

Accreditation Statement

This activity has been planned and implemented in accordance with the Essential Areas and Policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the University of Michigan Medical School and the Pulmonary Hypertension Association. The University of Michigan is ac-credited by the ACCME to provide continuing medical education to physicians.

Credit Designation

The University of Michigan Medical School designates this activity for a maximum of 2.0 AMA PRA Category 1 CreditsTM. Physicians should claim credit commensurate with the extent of their participation in the activity.

Instructions for Earning Credit

This activity is a self-study program; a self assessment examination is included on page 237 to help physicians review important points. A form is also included on page 238 for physicians to evaluate the CME activity. Completion of this activity involves reading the journal and completing the self-assessment examination and evaluation form, which may take up to 2 hours. Credits for this self-study program are available from February 20, 2010 through February 20, 2011. There is no fee for this program.

Please note that this self-study program may also be viewed online at: http://www.cme.med.umich.edu.

University of Michigan Privacy Statement

http://www.cme.med.umich.edu/privacy.asp

Sponsorship

This CME self-study program is jointly sponsored by the University of Michigan Medical School and the Pulmonary Hypertension Association.

Support

This CME self-study program is supported by an edu-cational grant from Actelion Pharmaceuticals US, Inc., Gilead Sciences, Inc., Pfizer, Inc., and United Therapeutics Corporation.

Oversight and Accreditation

Arlene Bradford, BA
Assistant Director
Office of CME
University of Michigan Medical School

Disclosures

The Accreditation Council for Continuing Medical Education and the Association of American Colleges have standards and guidelines to ensure that individuals participa- ting in CME activities are aware of relationships between authors and commercial companies that could potentially affect the information presented. To be disclosed to participants are all personal financial relationships with a commercial interest whose products are relevant to the content of this CME activity. The University of Michigan Medical School follows these national policies to ensure balance, independence, objectivity, and scientific rigor in all its CME activities. Each author was asked to complete a disclosure information form for this activity. Disclosures are reported below.

Glenna Traiger, Ann F. Gihl, Philippe Weintraub, MD, Traci Stewart, Michelle Ogawa, and Darci Albrecht have indicated no relevant financial relationships to disclose.

Deborah McCollister in the past 3 years has received payment from Actelion Pharmaceuticals and Gilead. She has served as an advisor to Actelion Pharmaceuticals.

Arlene Bradford, BA, has no relevant personal financial relationships to disclose.

CME Reviewer

Kevin M. Chan, MD
Assistant Professor of Medicine
Division of Pulmonary and Critical Care Medicine
University of Michigan Health systems
Ann Arbor, Michigan

Dr Chan has no relevant personal financial relationships to disclose.

Self-Assessment Examination

See answer key on next page

1. Select the most accurate statement from the following choices:
      a. Adolescents with chronic disease, like PAH, have rated themselves to have higher body dissatisfaction than their healthy peers
      b. Adolescents with chronic disease, like PAH, have rated themselves to have lower body dissatisfaction than their healthy peers
      c. Adolescents with chronic disease, like PAH, are at lower risk for depression than their healthy peers
      d. Adolescents with chronic disease, like PAH, have higher self-assessed scores of emotional well-being compared to their healthy peers
      e. None of the above


2. The current practice of transitioning adolescent patients with chronic disease is most accurately represented by which of the following statements?
      a. More than 50% of surveyed pediatric cardiology centers provide a structured transition program for their patients
      b. Among surveyed CF programs, 50% consistently perform readiness assessments of their adolescent patients prior to transition
      c. More than 25% of pediatric CF programs provide educational materials relating to transition of care
      d. The majority of adolescent patients with chronic disease are transitioned after the age of 21 years
      e. Fifty percent of surveyed pediatric CF programs have a specifically designed transition program to support the adolescent's development of readiness skills

3. All of the following should be conducted in the months just prior to the transition of care except:
      a. Pediatric and adult care teams meet with patient and caregiver to review the patient's medical and social history and future expectations once transitioned to the adult practice
      b. Pediatric provider begins discussion of transition of care with the patient and caregiver
      c. Review need for further follow-up with other specialists, if applicable
      d. Verify that health insurance coverage after the time of transition has been established

4. Which of the following statements about HRQoL is most correct?
      a. HRQoL is routinely assessed by the healthcare team
      b. HRQoL is determined by physical symptoms and degree of disability alone
      c. HRQoL represents a person's perceived satisfaction with those components of quality of life most likely affected by health status
      d. HRQoL has been studied extensively in PAH

5. The research on quality of life in PAH indicates:
      a. The quality of life tools have all confirmed that quality of life is impaired in PAH
      b. Therapy not only improves symptoms of PAH, but also improves quality of life
      c. Exercise may improve both mental and physical dimensions of quality of life
      d. It is important to use a wide variety of tools to assess quality of life to adequately capture the experience of those living with PAH
      e. a, b, and c
      f. a and b
      g. All of the above
      h. None of the above

6. Quality of life in PAH can be summarized by the following statements:
      a. Little is known about quality of life in PAH and the variables that improve quality of life
      b. The majority of tools that have been used to study quality of life in PAH have not been designed to study this disease
      c. Different etiologies of PAH that have been studied have shown the same degree of perceived quality of life
      d. All of the above

7. Factors that contribute to medication nonadherence include all of the following except:
      a. Forgetting to take medications as ordered
      b. Financial barriers of high copays or gaps in insurance coverage
      c. Understanding the benefit of medication on the PAH disease state and the risks of nonadherence
      d. Comorbid conditions of depression, anxiety, and cognitive dysfunction

8. Interventions to improve medication adherence:
      a. Should be addressed by the pharmacist alone
      b. Are delivered by the multidisciplinary PAH team in collaboration with the patient and family
      c. Should address barriers to adherence such as patient and provider factors, cultural, social, and financial barriers
      d. b and c

9 Which one of the following is required to diagnose major depressive disorder?
      a. Depressed or irritable mood, insomnia or hypersomnia, loss of interest or pleasure in life, and symptoms that cause significant clinical distress or functional impairment
      b. Depressed or irritable mood, insomnia or hypersomnia, and symptoms that cause significant clinical distress or functional impairment
      c. Depressed or irritable mood and symptoms that cause significant clinical distress or functional impairment
      d. Depressed or irritable mood, loss of interest or pleasure in life, and symptoms that cause significant clinical distress or functional impairment

10. All of the following statements are true except:
      a. Patients may be easily screened for depression with the Patient Health Questionnaire (PHQ-8), with a score ≥10 having a high sensitivity of identifying major depressive disorder
      b. Most PAH patients with significant clinical depression can have resolution of symptoms by participating in a support group
      c. A system to ensure appropriate evaluation, treatment, and follow-up should be in place for patients who are identified on screening to have symptoms of clinically significant depression
      d. Treatments for depression include medications, psychother- apy, cognitive-behavioral therapy, and interpersonal therapy