Improving Patient Care in Pulmonary Arterial Hypertension: Addressing Psychosocial Issues

PAH is a rare, incurable, and progressive disease caused by an increase in pulmonary vascular resistance at the precapillary level.1, 2, 3 In a survey of national registries and expert centers in France and Scotland, the prevalence of all types of PAH was reported as being approximately 15 to 50 cases per million, with an incidence of idiopathic PAH of 6.5 to 25 cases per million.2, 4 There is a strong female preponderance and a higher rate of prevalence observed in high‐risk groups, such as patients with systemic sclerosis.2 The estimated 1‐year mortality rate ranges from 8% to 10% in patients with idiopathic, familial/heritable, or anorexigen‐associated PAH.5 An increase in mean pulmonary arterial pressure to at least 25 mm Hg at rest, together with a mean pulmonary capillary wedge pressure of <15 mm Hg, are the defining features of PAH.2 As PAH progresses, symptoms become exacerbated owing to restricted cardiac output through the pulmonary arteries and concomitant right heart failure, which induces common symptoms of PAH including breathlessness, fatigue, weakness, and angina.1, 2, 6

Prompt and accurate diagnosis of PAH is central to effective management, with the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines recommending therapeutic intervention even for patients who have mildly symptomatic PAH,3 the aim of which is to improve symptoms, exercise tolerance, prognosis, and quality of life.2, 3, 5, 6 As a life‐altering disease, the impact of PAH is not restricted to the effects of the symptoms on the patient. PAH has significant effects on aspects of life and daily living for both the patient and their family.2, 7 The aim of this commentary is to show that the psychological burden of PAH is often overlooked and that consideration should be given to providing both patients and caregivers with adequate psychosocial support.

The Psychological Burden of PAH

As with many other severe chronic diseases, anxiety and depression leading to impairment in quality of life commonly affects many patients with PAH.2, 7 An analysis of the REVEAL Registry: Registry to Evaluate Early and Long‐Term PAH Disease Management demonstrated that a significant proportion of patients with PAH experience depression at the time of diagnosis.8 This may be the result of the long patient journey between the onset of their symptoms and a definitive diagnosis of PAH, which could exhaust their psychological reserves.

Results from an international survey conducted by a steering committee of PAH specialists and representatives from PAH patient organizations indicate that 56% of patients and caregivers consider PAH to have a very significant impact on their daily life, with a further 23% considering there to be a moderate impact and only 11% considering their illness to have no impact on their daily life.2 In addition, the proportion of patients stating that PAH significantly impacts their daily life increases with disease progression, with 90% of those who meet World Health Organization criteria for functional class IV PAH stating that their illness has a significant impact on daily life.2

This survey identified the key areas of life impacted by PAH as: work, finances, family, and authorities.2 Overall, 85% of patients found that their ability to work was affected by their PAH, which can have a major impact on household income, resulting in families with below‐average incomes and the need to adopt a different lifestyle.2 Relationships with partners can be badly affected, with almost two thirds of patients with PAH reporting an impact on their sex life and libido, caused in part by low self‐esteem and the physical limitations that result from PAH.2 Effects on family life also include the fact that pregnancy is strongly contraindicated because of significant risks to both the mother and fetus.2, 3 Moreover, more than half of patients reported an impact of PAH on their ability to play with their children or grandchildren or to undertake leisure activities, while 77% of PAH patients reported an impact of their illness on their ability to travel or go on vacation.2 In addition, patients may have unexpressed fears that there is a genetic element to their illness and that the disease may be inherited by either their children or grandchildren. Finally, patients entering transplantation lists have to cope with the stress of waiting for an organ as well as a successful intervention.

Interactions with authorities can be an additional cause of anxiety for patients with PAH. For example, access to optimal healthcare may prove challenging and stressful for the patient.3 Moreover, personal finances can be a cause of anxiety, particularly when having to ensure that appropriate benefits and support are obtained. To minimize anxiety for the patient, it is vital that appropriate information is given in a timely fashion.3

Given the progressive, incurable nature of PAH, it is important that any patient education that is designed to raise awareness of the long‐term prognosis for PAH is provided in context and by an appropriate healthcare professional in order to minimize the risk of anxiety and depression.2 This is especially important both for patients with predictive factors indicative of actively progressive disease, as well as for those with stable chronic disease.9 Without adequate professional provision of information at diagnosis, there is a tendency for patients to undertake Internet research, which may result in the discovery of inappropriate and worrying information.2 Patients need to understand their disease in order to discuss their diagnosis with family and friends. Sometimes patients may feel that there is a lack of understanding among friends and family, resulting in feelings of isolation.3 Caregivers may also feel there is a lack of emotional and social support to assist them in caring for their loved one, and their needs should also be considered alongside those of the patient.

Improving Psychosocial Care in PAH

It is imperative that healthcare professionals ensure the provision of patient education at the time of diagnosis of PAH.2, 3 However, there is a need to ensure that appropriate information is provided at an appropriate time, and consideration should be given to the fact that the provision of information may trigger completely different psychological responses in different patients. For example, the realization that current treatment is purely “symptomatic” rather than “curative” may cause psychological repercussions.7 A full understanding of the limitations of the disease and the need to adopt a disciplined lifestyle is important. For example, daily exercise and rehabilitation can show benefits; however, excessive physical activity should be avoided.10 Therefore, information relating to the underlying pathophysiology of PAH, the available treatment options, and lifestyle or nutritional changes should be provided by the physician to the patient at the time of diagnosis.

A “drip feed” of information may prove more valuable than provision of large amounts of information at one time. This approach will enable patients to absorb the information provided without feeling overwhelmed. While it is important, and necessary, to ensure that patients are fully supported, information relating to the long‐term consequences of PAH, such as the financial, social, and functional aspects of the disease, should be provided at a later date, eg, 1 to 3 months post‐diagnosis.3

In order to fully support patients, care and treatment should be undertaken by a multidisciplinary team who are able to advise them on various aspects of the condition. Given the nonspecific nature of symptoms such as dyspnea at the onset of PAH, timely diagnosis is challenging, with other conditions such as asthma, chronic heart failure, or even lack of fitness or depression frequently considered prior to a definitive diagnosis of PAH.5 In addition, such symptoms may themselves cause the emergence of additional conditions, such as panic attacks, which require the application of appropriate management strategies, including psychological techniques.11

The association between anxiety and depression, and patient quality of life means that the timely referral of patients diagnosed with PAH to a psychiatrist or psychologist should be considered when appropriate.2 In addition, health care professionals should ensure that patients are aware of the existence of patient support organizations, which can be a good source of emotional support,2, 3 and how to contact them. This is in line with recommendations in the 2009 ECS/ERS guidelines that psychosocial support should be offered to patients with PAH.3 Encouraging patients and their families to join patient support groups can have positive effects on coping, confidence, and outlook and provide practical assistance.

Conclusions

Feelings of isolation, depression, and anxiety are common in individuals with PAH. It is the responsibility of healthcare professionals to ensure that they are aware of the challenges faced by their patients in daily living, both caused by the symptoms of their PAH and psychosocial factors. A holistic treatment approach should be adopted, so that, in addition to appropriate medication for improving symptoms and quality of life, consultation with mental health professionals for emotional support is included to help address underlying psychosocial factors.

Disclosures

Iraklis Tsangaris has received lecture fees and consultancy fees and has participated as an investigator in clinical trials sponsored by Actelion, Bayer, and Pfizer. Editorial assistance was provided by Julia Coleman of Synergy, Richmond. Editorial assistance was funded by Pfizer.