Clinical Pearls Help Specialty Pharmacists Improve Pulmonary Arterial Hypertension Outcomes

Still a mystery in terms of cause and cure, pulmonary arterial hypertension (PAH) is a rare and progressive disease, but, fortunately, treatments for the condition have improved. During a session at Asembia’s Specialty Pharmacy Summit 2019, experts discussed specialty pharmacy’s potential to improve outcomes for patients with PAH. For the approximately 30,000 patients with a diagnosis, this is great news.

Matthew Casciano, PharmD, BCPS, tackled the triad of changes—vasoactive mediator imbalance/endothelial dysfunction, remodeling, and in situ thrombosis—that accumulate in PAH and eventually lead to disease progression. Right heart dysfunction is a primary concern. 

“To understand how right heart dysfunction develops in PAH, one first needs to have an understanding of how PAH progresses over time,” Casciano said. “The changes associated with progression are irreversible at later stages and include stiffening of the arteries, restricted blood flow, and advanced vascular lesions and clot formations.” 

Patients who do not receive treatment usually face death within months or years. However, with treatment, survival has improved from 2.8 years in the 1980s to more than 7 years today. 

“Almost half of cases are idiopathic, with the next-largest cause appearing to be connective tissue or collagen disorders, then congenital heart disease,” Casciano said. 

He talked about medications that have been linked to PAH, indicating that the number of cases is comparatively small but preventable. 

Patients with PAH experience significant changes in their lives after diagnosis and rely on multiple daily doses of oral medications and sometimes continuous infusions of intravenous specialty agents to manage the exhaustion, weakness, and arrhythmias that are common to the disease. Patient response tends to be somewhat unpredictable, depending on disease severity at diagnosis and underlying cause. 

Treatment for PAH has advanced significantly with the introduction of PAH-targeted drug therapy, which improves exercise capacity and quality of life and extends survival time. Christopher Ogurchak, PharmD, MSPBA, CSP, augmented Casciano’s treatment overview with a discussion of topics near and dear to the specialty pharmacist’s heart: access, adherence, and outcomes. 

Focused care provided by specialty pharmacists can dramatically improve the adherence and outcomes of patients with PAH and optimize their quality of life.  “Optimal medication management lowers total medical costs,” Ogurchak said. “Good evidence shows that adding a specialty medication, which is without a doubt an expensive proposition, decreases overall spend by about 15%.” 

He acknowledged that targeted medications have adverse drug effects (ADEs) and that those ADEs can frequently affect adherence to the treatment regimen. “Identifying ADE-related nonadherence is half the battle,” Ogurchak said. “Once pharmacists do that, they need to provide education and recommendations in digestible, patientfriendly language.”

He discussed ADEs using a model that attributed them to prostacyclin, endothelin, or nitric oxide, giving management options for each. 

Specialty pharmacists have an advantage because they interact with patients with PAH more frequently than other clinicians. This makes them touch points for patients and opens doors for the pharmacist to continually assess and act. 

“Pharmacists need to leverage motivational interviewing because expressing empathy, supporting self-efficacy, avoiding argument, rolling with resistance, and developing discrepancy pay with large rewards,” Ogurchak said. 

Cost is often an access barrier, with some medications costing more than $100,000 annually, and Ogurchak cited looming patent expirations and the likelihood of generic options as relief on the horizon. He also discussed mechanisms for finding financial assistance. 

Many products used in PAH are subject to an FDA-mandated Risk Evaluation and Mitigation Strategy (REMS). Quite often, stakeholders describe these programs as a headache and burdensome. The additional steps from a REMS program can make obtaining critical REMS drugs difficult for some patients, whether at the hospital or in their homes. Ogurchak said that the specialty pharmacist is crucial in the balancing act of access and safety, and he reviewed the necessary steps at every level for treatment with bosentan, ambrisentan, macitentan, and riociguat. 

The presentation finished with a flourish as Ogurchak presented a table of the most often used medications with lists of clinical pearls. Casciano covered drugs and biologics currently under review. Many of them are old drugs that may be repurposed for this new use, whereas others are novel treatments. 

The future looks promising for patients with PAH, Ogurchak noted, and pharmacist involvement remains critical and life changing.

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