Erum Naqvi obtained her Ph.D. in Molecular Medicine from Hannover Medical School (Germany) after completing her Masters in Biomedical Science and Bachelors in Microbiology from University of Delhi (India). She has several years of experience as a science writer.
Patient education is crucial throughout the course of pulmonary arterial hypertension (PAH), with patients interested in getting high-quality and individually tailored information at the appropriate times. At the time of diagnosis, patients are more interested in getting information about the disease and its treatment. At a later stage, they may be more interested in other patients’ stories, experiences, testimonials, or information about the effects of PAH on their lives, such as the effects on travel or sexual relations.1
Importance of Patient Education
Effective patient education, ie, individually tailored information delivered or communicated to patients in a timely manner, is important since it may encourage patients to become more proactive, involved, and engaged with managing their condition. This engagement makes it easier for the patients to cope with PAH, as well as take care of their condition. Informed patients who receive comprehensive guidance have better outcomes in terms of symptom management and disease progression.1
Patients with PAH need information regarding PAH and its physical and personal impacts, available treatment options, effects on emotional health, and relevant socioeconomic factors. In addition, patients need an understanding of their treatments and practical advice on managing titration procedures and complex drug delivery systems, such as intravenous and subcutaneous pumps.2
Relatives of patients with idiopathic PAH and heritable PAH should be advised of the availability of genetic testing as well as counseling.3
Heavy physical exertion that leads to distressing symptoms should be avoided, and physical activity should be limited to a symptom-free level.3
Patients in functional classes (FC) III and IV and those with arterial blood oxygen pressure consistently < 8 kPa (60 mmHg) should be considered for in-flight oxygen administration.3
It is essential to inform female patients about the risks associated with pregnancy in PAH and advise them about suitable methods of family planning.3
Since PAH often restricts the ability of patients to carry out their daily activities, leaving them feeling frustrated or angry, it is crucial to provide information about physical restrictions and their impacts on patients, as well as ways to manage the condition.
Reduced ability to work causes financial stress on many patients with PAH, often causing frustration, anger, or low self-esteem. Many patients with PAH suffer from depression, and thus information about the effect of PAH on mental well-being should be provided.3
Patient Education Material
Several organizations and programs provide a wealth of information to patients with PAH, as well as their family members and caregivers. The Pulmonary Hypertension Association4 helps patients find medical specialists and facilities with expertise in PAH and provides access to support groups. Some additional organizations that help patients with PAH find support are the PAH Initiative,5 American Thoracic Society,6 MedlinePlus,7 National Organization for Rare Disorders,8 American Lung Association,9 and Genetic and Rare Diseases Information Center (GARD).10 RareConnect11 is a safe platform that offers patient-hosted online communities, allowing patients and caregivers affected by PAH to connect.
PAH Patient Associations
Patient associations, such as the Pulmonary Hypertension Association12 and European Pulmonary Hypertension Association,13 are important tools for patient education that may contain resources in many languages for health care providers and patients. A survey of patients with PAH found patient associations are one of the most useful sources of information, with 66% of patients using them.1 PAH patients are encouraged by clinical PAH guidelines, including the latest European Society of Cardiology/European Respiratory Society guidelines, to contact patient associations.13
- Graarup J, Ferrari P, Howard LS. Patient engagement and self-management in pulmonary arterial hypertension. Eur Respir Rev. 2016;25(142):399-407. doi:10.1183/16000617.0078-2016
- Farber HW, Gin-Sing W. Practical considerations for therapies targeting the prostacyclin pathway. Eur Respir Rev. 2016;25(142):418-430. doi:10.1183/16000617.0083-2016
- Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67-119. doi:10.1093/eurheartj/ehv317
- Resources. Pulmonary Hypertension Association. Accessed June 30, 2021.
- PAH Initiative homepage. PAH Initiative. Accessed June 30, 2021.
- Pulmonary hypertension. American Thoracic Society. Accessed June 30, 2021.
- Pulmonary arterial hypertension. MedlinePlus. Updated August 18, 2020. Accessed June 30, 2021.
- Pulmonary arterial hypertension. National Organization for Rare Disorders. Accessed June 30, 2021.
- Pulmonary arterial hypertension (PAH). American Lung Association. Accessed June 30, 2021.
- Pulmonary arterial hypertension. Genetic and Rare Diseases Information Center. Accessed June 30, 2021.
- RareConnect homepage. RareConnect. Accessed June 30, 2021.
- Pulmonary Hypertension Association UK homepage. Pulmonary Hypertension Association. Accessed June 30, 2021.
- Pulmonary Hypertension Association Europe homepage. Pulmonary Hypertension Association. Accessed June 30, 2021.
Article reviewed by Harshi Dhingra, MD on July 1, 2021.