High-Touch Care for Highly Specialized Pulmonary Arterial Hypertension Patient Needs

Specialty Pharmacy TimesSeptember/October 2014
Volume 5
Issue 5

Individualized care programs can help patients with pulmonary arterial hypertension manage complex treatment regimens and overcome the challenges presented by this condition.

Individualized care programs can help patients with pulmonary arterial hypertension manage complex treatment regimens and overcome the challenges presented by this condition.

Fewer than 200,000 Americans—about 12 per 1 million adults—are living with pulmonary arterial hypertension (PAH), but the exact number is not well established because of the difficulty in properly diagnosing the disease.1 Many more people may be living with PAH who have yet to be diagnosed. For those patients who have been given the proper diagnosis, the disease poses a heavy burden both physically and financially.

Recent developments in treatment options have helped improve both life expectancy and quality of life. However, misdiagnosis is common, which further delays the onset of appropriate medical care. Despite advances in pharmacologic management, PAH has a 15% mortality rate within a year of diagnosis even with current treatment options.1 Increasing disability is common with disease progression, and treatment complexity and costs only escalate.

Due to the difficulty in making the appropriate diagnosis and choosing the right treatment options, PAH patients need specialized support and experienced clinicians to help them manage their condition and complex treatment regimens. Data from Express Scripts show that Accredo, our specialty pharmacy, can help improve patient outcomes through the individualized care and counseling provided by our specialist PAH pharmacists and nurses.

Accredo pharmacy is the only specialty pharmacy to have access to the full portfolio of PAH drugs, which is important for continuity of care as patients progress through the different levels of therapy.

History of PAH

Pulmonary arterial hypertension was first identified as a disease by Dr. Ernst Von Romberg in the in the 19th century; however, the first drug for the treatment of PAH—intravenous prostacyclin—was not ready for pivotal clinical trials until the 1990s. The 21st century has seen an explosion of treatment options for patients, most recently including the introduction of the first oral prostacyclin treatment.

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Despite the availability of oral medications, treatment regimens remain complex. The course of this disease is unpredictable, and there is a high incidence of increasing disability with disease progression. Today, PAH patients have a median survival rate of about 7 years post diagnosis. Without therapy, the expected survival rate is less than 3 years.2

Treatment Challenges

In addition, PAH patients have a high rate of hospitalization. Complicated regimens, often involving intravenous or subcutaneous administration of medication, multiple dose requirements, combination therapy with oral agents, and significant side effects mean nearly a quarter of PAH patients are nonadherent to their prescribed medication regimen. Adherence and medication availability are critical and life-sustaining. The most seriously ill of these patients cannot be without medications. Such a situation can be immediately life threatening due to rebound pulmonary hypertension.

Oral therapies, while more convenient, carry the risk of serious adverse events such as anemia or liver damage—necessitating frequent laboratory evaluation—or vision or hearing loss. Drug—drug interactions with medications commonly used to treat diabetes or HIV further complicate the use of the oral therapies.

Side effects of the inhaled or infused products include diarrhea, flushing, headache, and nausea. Because the infused medications should never be stopped due to the risk of life-threatening rebound PAH, these side effects become a part of life for patients with PAH.

Rising Costs

The cost of PAH treatment increases significantly as the disease progresses and therapy gets more complicated. Annual treatment can cost from $25,000 to $250,000 per patient, with combination therapies costing more than $350,000 a year for a single patient.3

In addition, nearly 75% of PAH medications are billed through a patient’s medical benefits, where plan sponsors may lack access to utilization details and often have little control over the spend.3 Many times, the medications billed to the medical benefit tend to be the higher-cost therapies.

Need for Specialized Support

Nonadherence can be critical, even fatal, for patients with PAH. It can also add costs for plan sponsors in the form of emergency department visits, additional hospitalizations, and other adverse events from disease progression. Accredo specialty pharmacy has up to 16% better adherence than other specialty pharmacies for both mono and dual therapy, resulting in $13,000 lower medical costs per patient.

Accredo’s PAH Therapeutic Resource Center (TRC) offers patients clinical services, including 24/7/365 access to a nurse or a pharmacist. The 500-plus field nursing staff includes 32 cardiopulmonary nurse specialists. Accredo’s field nurses provide:

  • Initial pharmacist consultation
  • Clinical assessment and risk evaluation calls
  • Clinical follow-up calls based on patient needs
  • In-home nurse education for targeted PAH therapies
  • Risk evaluation and mitigation strategy requirement adherence
  • Proactive patient calls and access to specialty-trained clinicians prior to shipment
  • Specialized nursing care
  • Access to clinicians and medications on a national scale, critical for patients who may need specialized and experienced nursing support or drug delivery within a very short period of time
  • Delivery of medications, supplies, or durable medical equipment to 90% of the continental United States within 4 hours in the event of an emergency

The Accredo TRC also has more than 45 dedicated specialty-trained PAH pharmacists who counsel patients and manage care. Accredo has therapy-specific clinical protocols and programs to close gaps in care, and also offers educational and online resources including:

  • Educational videos and booklets
  • Treatment diaries
  • Opportunities for patients and caregivers to learn more about local support services and national advocacy groups

Accredo’s specialized care has demonstrated results. Our analysis shows:

  • A patient satisfaction rate of 98%
  • Significantly lower central venous line infection rates
  • Fewer days in the hospital and shorter transition times for patients
  • Approximately 93% of PAH patients are within 75 miles of an Accredo nurse

With the PAH drug trend projected to increase by double digits during the next several years, plan sponsors need tools to rein in their drug spending. This can present a huge challenge with more than one-fourth of the drugs billed through the medical benefit. Vigilance in talking to patients frequently, pharmacist counseling, proactive nurse outreach, and in-home nurse visits are just a few examples of how Accredo remains connected to patients with PAH. This high-touch model helps to control extraneous use of health care resources by recognizing signs and symptoms of PAH that can be managed outside of the hospital setting earlier. SPT


  • McGoon MD, Humbert M. Pulmonary arterial hypertension: epidemiology and registries. Adv Pulmonary Hypertens. 2014;13(1):21-26.
  • Barst RJ. Pulmonary hypertension: past, present, and future. Ann Thorac Med. 2008; 3(1):1-4.
  • Accredo book of business data and analysis.

* References corrected since initial publication.

About the Author

Mary Dorholt, PharmD, leads Express Scripts’ specialty clinical strategy and protocol development. In this role, Mary and her team of clinical experts develop clinical guidelines for patient care and physician interaction for this complex and growing area of the pharmacy and medical benefit. She is also responsible for driving organizational research on specialty medications and the Express Scripts experience. Dr. Dorholt has a 17-year history at Express Scripts and the former Medco organization. Prior to her current role, Dr. Dorholt was responsible for provision of specialty strategic guidance to employer, government, and labor organizations. In this capacity she was also responsible for marketplace oversight and internal and external communications related to specialty drug management. Dr. Dorholt has extensive knowledge and experience in the development and implementation of specialty solutions, Medicare Part D strategies, and clinical support services. She has been a frequent speaker on specialty and Medicare topics during her tenure. Dr. Dorholt received her Doctor of Pharmacy degree from the University of Minnesota College of Pharmacy in Minneapolis. She holds bachelor’s degrees in mathematics and biology.

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