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PAH: Multidisciplinary Care and Medication Benefits


Charles Burger, MD: Patients with pulmonary arterial hypertension really require a team approach to their care. It’s a very narrow area of medicine. Diagnosis can be complex and the treatment regimens are quite clearly complex. Because of the expense of the medications, authorization and approval is required through the third-party payer. Then, of course, how do you manage the dosing of these medications and the side effects of these medications, which range from oral, to inhaled, to infusion? All of them have side effects. It is a very unusual patient that tolerates these medications without anything in the way of a side effect. Typically, the benefit outweighs the side effects. But, specific expertise with the nurse coordinators, or the pulmonary hypertension center coordinators, in managing these side effects is crucial to educating the patient and ensuring compliance with treatment. The worst case scenario would be that the patient isn’t properly educated or managed by the nurse coordinators, additional therapies are added to the initial choice of medication if the medication isn’t being used properly, or the patient is noncompliant and you don’t know that or you didn’t manage it early on. You’re adding to the side effect profile by adding therapies and you’re adding to the expense by adding therapies when a very tightly controlled single drug might have done the trick, had the patient been properly educated and there had been access to managing the various side effects along the way. So, that team approach is extremely important in managing these medications and the side effects of them.

The specialty pharmacies really can provide an additional layer of support for these patients, and not only with supply of the medications and educating the patients about the logistics of administering the medications, but hotlines for managing problems with the delivery systems and the side effects. It’s very important that the specialty pharmacists and the specialty home nurses work together in a coordinated fashion to support the prescribing clinician, the nurse coordinator, and the pulmonary hypertension center so that they’re all communicating appropriately, have the same objectives with delivery of the medications, and have a consistent message in terms of managing the side effects and ensuring compliance.

Overall, the efficacy studies for the currently approved pulmonary arterial hypertension medications across the board seem to have a positive impact on reducing the need for hospitalization from complications of pulmonary arterial hypertension, and reducing the need for surgical interventions and surgical treatment of disease, specifically the need for transplant or atrial septostomy. And, in that way, very firm hard endpoints, I think, are now available showing the clinical benefit of these medications. In an individual clinical setting, decisions still have to be made about the types of therapies, the number of therapies, and the dosing of the therapies. That really can only be individualized based on the patient’s severity of pulmonary arterial hypertension and the risk factors.

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