Pulmonary Hypertension

Shortness of breath, chest pain, fatigue, fainting-all are common symptoms seen regularly in medical practices across the country. Patients with these symptoms often are diagnosed with asthma, COPD, heart disease or sleep apnea. Yet when these patients are admitted to a hospital, enroll in pulmonary rehabilitation or receive home respiratory therapy, some of them still don’t improve, even while following their treatment plans. In these cases, the attentive respiratory therapist may play a critical role by considering these common symptoms as possible signs of a rare but serious disease.

Pulmonary hypertension, or high blood pressure in the blood vessels between the heart and lungs, affects 20,000 to 30,000 U.S. patients, adults and children, male and female, of all ages and from diverse backgrounds. It is characterized by shortness of breath with exertion and can prevent patients from working, caring for their homes and families and pursuing daily activities.

Because PH symptoms mimic those of widespread chronic illnesses, PH is difficult to diagnose. Yet early diagnosis is essential, as the life expectancy of patients who go untreated is 2.8 years. The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry), the largest registry of patients with this illness, found that the mean time from symptom onset to diagnosis by right heart catheterization was 2.8 years. This registry also showed that among patients whose pulmonary arterial hypertension (PAH) was confirmed by right heart catheterization, 73.6 percent were at advanced stages of the illness at the time of diagnosis. According to the New York Heart Association’s Functional Classification of the extent of heart failure, this large percentage of newly diagnosed patients already showed marked limitation of physical activity. Anecdotal evidence from patients also suggests that repeated misdiagnosis is common.

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For many years, we had no treatments for PH, so the timing of a correct diagnosis had no impact on patient outcomes. Today’s situation is very different. Diagnosing patients early has become far more crucial now that nine treatments for this rare disease have become available over the past two decades.

With knowledge about PH, respiratory therapists working in the hospital, pulmonary rehabilitation and home care settings may discuss with primary or referring physicians the possibility of screening a patient for PH. It is interesting to note that the goals of pulmonary rehabilitation largely parallel those of PH treatment-to assess, educate, provide therapeutic exercise, respond to psychosocial needs and develop long-term adherence to treatment for the patients served.

Early Diagnosis Campaign
In 2011, the Pulmonary Hypertension Association (PHA) published an international white paper, “Pulmonary Arterial Hypertension: Recommendations for Improving Patient Outcomes,” calling delayed diagnosis one of the biggest barriers to better patient outcomes. In response, PHA has launched an early diagnosis campaign to increase healthcare professionals’ knowledge of PH and their ability to suspect the disease, screen for it and team with PH specialists for optimum care.

The initiative, Sometimes it’s PH, is working through associations of healthcare professionals to raise the profile of this issue and to educate allied health professionals and physicians to distinguish PH from illnesses with similar symptoms. The campaign’s signature image, a zebra, plays off the lesson taught to medical students learning to diagnose: When you hear hoof beats, think horses, not zebras. But PH is like a zebra among horses-similar, yet very different.

Sometimes it’s PH is inviting a variety of professional associations to feature early diagnosis of PH in educational programming, conference activities, publications and other member communications. The campaign also will seek media coverage of early diagnosis concerns. In addition, PHA is mobilizing PH patients to advocate for early diagnosis by sharing their personal stories of misdiagnosis on the campaign’s website, www.SometimesItsPH.org.

Opportunities for Professionals
Although this early diagnosis initiative is very new, PHA has a long history of creating other opportunities for respiratory therapists to learn, network and tap into informational resources for themselves and their patients. In fact, PHA has also been working with the American Association for Respiratory Care to develop a relationship that will benefit the patients we serve.

PHA manages the PH Professional Network (PHPN), a membership group for allied health professionals. PHA also holds major conferences, alternating each year between its PHPN Symposium for allied health professionals and the PHA International Conference and Scientific Sessions. This year’s PHPN Symposium, to be held Sept. 26-28, offers CE-accredited programs on the latest advances and research in PH. The international conference is both a medical meeting and an educational and networking opportunity for patients, families and caregivers. Opportunities for allied health professionals include a PH Fundamentals course taught by respiratory therapists, nurses, nurse practitioners and others. Medically-led sessions invite patients and families to bring their questions on diverse subjects-from clinical trials to oxygen supplementation-to panels of professionals who answer in patient-friendly language.

Year round, PHA also offers webinars, publications, advocacy and leadership opportunities for professionals and patients on its website, www.PHAssociation.org. An expanding collection of educational materials, including PHA’s quarterly clinical journal Advances in Pulmonary Hypertension and free continuing education credits, can be found in the PHA Online University, www.PHAOnlineUniv.org.

PHA has always believed that patient-to-patient support is invaluable in handling the isolation and fear this illness can bring. The organization connects patients through 245 face-to-face support groups, monthly telephone support groups, nightly online chats, e-mail groups, a Facebook page and an online mentor program.

Much progress has been made in understanding and treating PH, but our professional community has much more work to do to diagnose, treat and support PH patients.

Gerilynn L. Connors, BS, RRT, MAACVPR, FAARC, is clinical manager, Respiratory Care Outpatient Services, Pulmonary Diagnostics & Pulmonary Rehabilitation, Inova Fairfax Medical Campus, Falls Church, Va. She also chairs the Practice Committee of the PH Professional Network and the AARC Continuing Care and Rehabilitation Section and is president- elect of the Virginia Association of Cardiovascular & Pulmonary Rehabilitation.

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