Why Living with Pulmonary Arterial Hypertension Requires a Holistic Approach: A Patient and Clinician Perspective

Pulmonary arterial hypertension (PAH) is a rare disease caused by high pressure in the blood vessels leading from the heart to the lung. PAH affects many parts of a patient's life, which means that patients should be managed by a clinical team of different specialists, including doctors, advance practice providers, nurses, social workers, and therapists. This article is co-authored by a patient living with PAH and an acute care nurse practitioner specializing in the management of patients with pulmonary hypertension. In the first section of this commentary, the patient describes her experience of living with PAH. The specialist nurse practitioner then discusses the management of PAH, to provide a clinician perspective in the context of the patient's experiences. [GRAPHICS] PLAIN LANGUAGE SUMMARY This article describes the experience of a young patient with pulmonary arterial hypertension, or PAH, as well as the point of view of a PAH specialist nurse practitioner. PAH is a rare disease caused by high pressure in the blood vessels leading from the heart to the lung. This means the right side of the heart needs to work harder to pump blood through the lungs, which can make it weaker and eventually cause right heart failure and death. Although PAH cannot be cured and gets worse over time, effective treatment can delay worsening, reduce symptoms, and improve quality of life. Recognizing symptoms and diagnosing PAH early is important so that patients can be referred to specialists who understand PAH and can provide the most appropriate treatment. A patient's risk status shows the chance of the disease getting worse and the patient dying. The aim is to treat patients so that they stay low risk and improve their chance of living longer. Clinical guidelines give specialists advice on treating patients with PAH and say that regular risk assessment is important to guide treatment decisions. Because PAH affects many parts of a patient's life, the guidelines also say that patients should be managed by a clinical team of different specialists including doctors, advance practice providers, nurses, social workers, and therapists. Making sure that patients and carers understand PAH and the benefit of treatment is also important to help patients follow their treatment plan and actively help to manage their disease.