Treating a Rare Disease Takes a Rare Commitment

Nov 22, 2016
As a specialist pharmacist focused solely on PAH treatment, providing patients with the education and overall support they need is the most important part of my job.
  • Pulmonary Hypertension

RPh Computer Article

Pulmonary arterial hypertension (PAH) – high blood pressure in the pulmonary artery to the lungs – is currently incurable. However, patients can live for years with this condition, but only if it carefully managed with proper adherence to a prescribed therapy. PAH is not common – affecting 15 to 50 people per million people – and many cases are from an unknown cause. However, there is a greater risk of acquiring PAH with certain medical conditions, such as HIV, connective tissue disease and congenital heart defects, just to name a few. There also is a higher risk associated with the use of some older weight-loss drugs.

For these reasons, and many more, specialized care from a team of professionals with deep understanding of the disease can literally make the difference between life and death.

Better Understanding Through Patient Counseling

Along with the physician, nurses, PAH support groups and the patient’s family members and caregivers, pharmacists play an important role in ensuring an appropriate treatment plan.

One of the most significant parts of an initial consultation I provide patients is about potential side effects and drug interaction concerns, because most PAH patients are on a complex polypharmacy regimen. Patients also may have questions about the inhalation system and device maintenance. Often, my role is to educate patients about how new inhaled therapies can improve their current condition.

Starting a New Form of Therapy

Once diagnosed, many patients start treating the condition with one or more oral therapies available in pill form. Recently, several new oral therapies have been approved for PAH. Other kinds of PAH therapies include inhaled medications and infused medications.

Oral medications are a general first-line treatment and can help slow the progression of the disease for those who are diagnosed early. These drugs may improve the patient’s tolerance for exercise and PAH symptoms. However, PAH is a progressive disease and over time some patients may begin to experience worsening of symptoms, such as shortness of breath with less physical ability, fluid retention, increased fatigue and chest pain.

When oral therapy is no longer sufficient, the next step is generally an inhaled prostacyclin therapy, which is a potent vasodilator. It dilates the blood vessels in the lungs, which decreases the pulmonary pressures and allows the patient to have less shortness of breath.

Transitioning to an inhaled therapy can be complex and physically and emotionally taxing. Patients have to learn how to use an inhalation device, administer the treatment properly and manage multiple treatments each day. Patients often need extensive counseling to understand their disease and therapy requirements and to help stay adherent. They are often nervous and have many questions about their treatment. A specially trained pharmacist or nurse from the Accredo PAH Therapeutic Resource Center can help.

As a specialist pharmacist focused solely on PAH treatment, providing patients with the education and overall support they need is the most important part of my job.

Goals of Healthier Outcomes

To get patients more engaged in their own care, I frequently ask them to set a small goal they would like to achieve on their new therapy. We discuss steps to achieving that goal, including the help of Accredo’s 24-hour hotline if the patient ever needs pharmacist support. Here are some other common questions and tips for newly diagnosed PAH patients starting an inhaled therapy:

  • When starting a new therapy, talk to your specialist pharmacist about potential medication interactions and side effects.
  • Specialist pharmacists also can help if you need multiple treatments each day or need to make up a dose.
  • Stay adherent even if you don’t feel better right away. Sometimes the medications can take several weeks to take effect, and nonadherence will likely cause your symptoms to worsen.
  • Get help with troubleshooting or device replacement if you have any issues with administering your medication.
  • Carefully record and report side effects.
  • Always make sure you have an adequate supply of medication on hand.
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