A Supplement to CareManagement CE FOR CCM & CDMS APPROVED FOR 2 HOURS OF CCM, CDMS AND NURSING EDUCATION CREDIT
Accessing Pulmonary Arterial Hypertension (PAH) Medication—It Takes a Village! J. Wesley McConnell, MD1; Sally McConnell, RN1; Kimberly Robinson, APRN1; Susan Raspa, RN, BSN2; Janis Pruett, EdD, RN, MSN, FNP-BC2 Kentuckiana Pulmonary Associates; 2Actelion Pharmaceuticals, Inc, a Janssen Pharmaceutical Company of Johnson & Johnson
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ulmonary arterial hypertension (PAH) is a rare, reasonable option.3 These different options entail differchronic disease that often leads to disability ent combinations of possible therapeutic effects and side and premature death. In PAH, proliferation effects.3 For example, some PAH medications (treprostiof the endothelium nil, epoprostenol) are adminFIGURE 1 THE PATIENT AT THE CENTER of the pulmonary istered through the parenteral arterioles and smooth muscle route, requiring titration and Clinic or cell hypertrophy cause narrowmay necessitate central line PAH Center ing of the vessel lumen. This placement with concomitant Physician, leads to increased pulmonary potential for local and systemic Nurse Practitioner, Physician Assistant, vascular resistance, right veninfections. Some newer oral Nurse, Staff tricular remodeling and dysmedications (selexipag, orenfunction, and ultimately, right itram, riociguat) require titraheart failure. The average tion to therapeutic levels over Specialty Pharmacy Resource life expectancy after diagnoweeks to months. Patient input Organizations: Case Manager sis is approximately 7 years,1 and buy-in are important in sePatient PHA, Foundations, Nurse Manufacturers Hub up from 2.5 years2 a couple lecting and prescribing theraPharmacist of decades ago. In part, this peutic options and may play a progress is due to the availrole in medication adherence. ability of newer pharmacoThe patient is not alone through logic agents. However, even Health Plan the PAH journey. Numerous Case Manager with multiple treatments availresources exist, including proPharmacy able, the 1-year mortality rate fessionals in centers or clinics, 1 is 15%. Currently there are 13 health plans, and specialty FDA-approved drugs available pharmacies, as well as the (Table 1) with four new agents Pulmonary Hypertension Association (PHA), patient supapproved in the last 4 years. Given the severity of the port groups, and manufacturer-sponsored educational disease, timely access to these important medications materials and programs. Patients who are technologiis critical. cally savvy have an advantage because they are not only able to access the myriad online educational resources, The Patient and Provider Team but can find out what financial resources become availThe patient must be an integral part of the treatment able and apply quickly for them, often ahead of others team practicing patient-centered care (Figure 1). Guiding who may not have access to computers. Patients who clinical decisions with patient-centered care demonaccess the varied resources can partner most effectively strates respect for the patient.3 Achieving shared deciwith the health care team. sion-making requires developing a good relationship between the patient and care team, sharing information, Pharmacologic Treatment and supporting patients in expressing their preferences Currently, there are FDA-approved drugs targeting three and views in the decision-making process.4 Some deciknown pathways that contribute to the development of sions are straight forward (ie, a hip fracture needs repair), PAH. Selection of appropriate drugs must be individualbut for most medical decisions, there is more than one Made possible by an educational grant from Actelion Pharmaceuticals, Inc, a Janssen Pharmaceutical Company of Johnson & Johnson | March 2018
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