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Psychosocial Aspects of Pulmonary Hypertension: A Review

Received November 8, 2006; revised February 26, 2007; accepted March 16, 2007. From the Univ. of Toledo Dept. of Psychiatry; the Univ. of Michigan Dept. of Psychiatry; and the Univ. of Michigan, Division of Cardiovascular Medicine, Dept. of Internal Medicine. Send correspondence and reprint requests to John M. Wryobeck, Ph.D., Univ. of Toledo Health Science Campus; Dept. of Psychiatry; Mail Stop 1193, 3000 Arlington Ave.; Toledo, OH 43614. e-mail: John.Wryobeck{at}utoledo.edu
© 2007 The Academy of Psychosomatic Medicine

ABSTRACT

TOP ABSTRACT INTRODUCTION Review of PAH: Diagnosis,... Importance of Adaptation to... Psychiatric Comorbidity Coping and Emotional Well-Being Psychosocial Adjustment Palliative Care REFERENCES

 
Pulmonary arterial hypertension (PAH) is characterized by progressive and sustained elevation of pulmonary-artery pressure, which ultimately leads to right-ventricular failure and death. The diagnosis carries with it an uncertain and historically very bleak prognosis. Although new oral and chronic parenteral (intravenous and subcutaneous) treatments have had a significant positive medical impact on the physical functioning of individuals with PAH, patients often struggle with new short- and long-term psychosocial challenges. The purpose of the current article is to 1) provide a brief review of PAH and its treatment; 2) summarize the limited literature examining the psychosocial adjustment of those with a PAH diagnosis; and 3) provide pertinent information extrapolated from the larger literature on chronic illness that might inform us on the psychosocial challenges faced by the patient diagnosed with PAH.

INTRODUCTION

TOP ABSTRACT INTRODUCTION Review of PAH: Diagnosis,... Importance of Adaptation to... Psychiatric Comorbidity Coping and Emotional Well-Being Psychosocial Adjustment Palliative Care REFERENCES

 
Pulmonary-arterial hypertension (PAH) is a chronic disease of the pulmonary vasculature characterized by progressive and sustained elevation of pulmonary arterial pressure that ultimately leads to right-ventricular failure and death.¹ The condition most often escapes detection, and individuals frequently suffer symptoms for several years before being appropriately diagnosed.² Historically, the average mortality was 50% in 2.8 years, and life expectancy after onset of symptoms was about 4 years.¹ Approximately 100,000 women and men in the United States have PAH.³

The development of specific treatments, including novel oral drugs and continuous intravenous prostacyclin, has improved physical functioning and survival by improving cardiac functioning and slowing the progression of the disease, but a cure remains elusive. In the context of living longer with PAH-related symptoms, patients also must learn to face their unpredictable future and manage the complex treatments that can be associated with severe adverse effects and may require significant changes in lifestyle. As a result, significant psychosocial issues (e.g., depression, anxiety, panic disorder, and marital and work-related problems) have emerged that are proving to be difficult for many patients.⁴ Few empirical studies exist that can serve to enhance our understanding of the emotional health and quality-of-life issues faced by this patient population.

Our purpose is to 1) provide a review of PAH and its treatment; 2) summarize the limited literature examining the psychosocial adjustment of those with a PAH diagnosis; and 3) provide pertinent information extrapolated from the larger literature on chronic illness that might inform us about the psychosocial challenges faced by the patient diagnosed with PAH.

Review of PAH: Diagnosis, Prognosis, and Treatment

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Mild-to-moderate degrees of pulmonary hypertension or an increase in the pressure in the pulmonary artery and its branches is most often the result of congestive heart failure or chronic lung disease. When various triggers or diseases are associated with an increase in pressure in the pulmonary capillaries or thickening and loss of vasodilator reserve of the precapillary pulmonary arterioles (<200 microns) varying degrees of pulmonary hypertension may result. The consequences of severe pulmonary hypertension include pressure load on the thin-walled right ventricle, which results in pump failure; decrease in cardiac output; and impaired venous return, which are manifested as progressively worsening shortness of breath, fatigue, congested liver, peripheral edema, and death.⁵

The World Health Organization (WHO) classification of pulmonary hypertension (PH) includes five categories, each of which has a different mechanism of causation, prognosis, and treatment options. WHO Diagnostic Class 1 is pulmonary arterial hypertension, and that is our focus. Other classifications include PH associated with left-heart failure and valvular heart disease; chronic respiratory diseases; chronic pulmonary thromboemboli; and rare disorders of the pulmonary vessels.⁶ The previous classifications of primary (PPH) and secondary pulmonary hypertension (SPH) have been abandoned.

The prototypical patient with PAH is a young-to-middle-aged woman (>75% are women)⁵ who notices mild fatigue and breathlessness that she initially attributes to stress, weight gain, and lack of exercise.⁷ Her doctor usually confirms that impression. Over 6 months to a few years, there is a progression of other symptoms that are often difficult to characterize by the primary-care physician; these may include peripheral edema, chest pain, and lightheadedness. By the time these symptoms are taken seriously and the patient is referred to physicians with expertise in diagnosis and treatment, the prognosis is limited, and there are few treatment options. The reduction in heart function and associated medical conditions each affect physical functioning and outcome. Together, these limit a patient’s quality of life and add to the uncertain future regarding life expectancy and the ability to continue functioning in their professional (as a contributing and effective employer/employee) and personal (spouse, parent, and community member) roles. Until she is referred to a center specializing in PH, the patient is given a very bleak picture by her physician, which is often augmented by the patient’s use of the Internet as an interim source of information. The burden and emotional response to the disease is very much related to the associated diseases, the resulting change in functional/social roles, and the availability of social support and health insurance.^4,8

Pulmonary arterial hypertension can be idiopathic (IPAH; formerly PPH), of which 10% is familial or associated with other diseases.^9,10 The more common associated disorders include the connective-tissue diseases (scleroderma, systemic lupus erythematosis, rheumatoid arthritis); congenital heart disease with right-to-left shunting; portal hypertension with or without cirrhosis; drugs, including cocaine and diet pills; sickle-cell disease and other hemolytic anemias; and human immunodeficiency virus (HIV), with or without the autoimmune deficiency syndrome.^11–14

There is incomplete penetrance of the gene, so fewer than 50% of family members are affected, but the disease appears earlier in subsequent generations,^15,16 a fact that is not lost to patients and family members worried about siblings and children. The psychological distress of the patient who knows that family members have died of PAH at a young age is palpable. Similarly, there is the added distress of the parent, worried that they may be passing the disease to their young children.

Patients with symptoms suggestive of PH first undergo a noninvasive test, the echo-Doppler to determine the presence of PH and exclude left-heart and valvular disease.¹⁷ If there is significant PH, pulmonary-function testing identifies those with lung disease, and a radioisotope lung scan or computer tomographic angiography is used to identify those with pulmonary emboli. If each is excluded, the conclusion is pulmonary arterial hypertension, and blood testing is performed to identify associated diseases or triggers.¹⁷ By this time, the patient has usually seen one or two specialists, who suggest the condition is rare and outlook bleak. When PAH is suspected or concluded, a right-heart catheterization is needed to confirm the diagnosis and determine the best therapeutic options.¹⁷ The testing itself can be frightening and often takes several weeks of appointments to complete.

The advances in treatment have improved quality of life and survival in PAH as well as other associated disorders. But the life expectancy of those with symptoms during modest activity, such has climbing a flight of stairs (>WHO Class II) is relatively poor, and response to treatment is very unpredictable. The mean survival in PAH has improved from about 2 years to over 6–7 years with the introduction of novel therapies.^18,19 Prognosis correlates highly with initial functional class, effort tolerance in the 6-minute hall walk, and clinical and hemodynamic response to treatment.¹⁷

It was the introduction of intravenous prostacyclins in the late 1990s that initiated advances in the treatment of PAH and improvement in symptoms, quality of life (QoL), and survival.²⁰ But the PAH-specific treatment brought new problems for patients and caregivers. The cost of the intravenous treatment averaged more than $100,000 per year. The first available was epoprostenol (or Flolan^TM), which is administered via a central venous catheter, using a continuous infusion pump.¹⁷ This requires the patient or support person to prepare the medication each morning, place that medication into the infusion pump, and keep it cool with ice-packs. Most patients improve with epoprostenol for at least months-to-years, but they are dependent upon it for survival. Because of the very short, 4-minute half-life of the drug, the patient is informed that any sign of problems with the pump or catheter must be attended to immediately, with the possible consequence of death. Treprostinil (Remodulin^TM) is a newer-generation prostacyclin that can be given intravenously or subcutaneously.¹⁷ Although there is considerably less experience with this drug, it is easier to administer and has a 4-hour half-life. Adverse effects of the prostacyclins include jaw pain, headache, diarrhea, nausea, flushing, and a skin rash, each of which usually reflects excessive dosing. The in-dwelling catheter results in bacteremia in about 10 to 14 cases per 100 cases yearly, and this can be fatal.²¹

Iloprost (Ventavis^TM) is a novel prostacyclin delivered by inhalation 6 to 8 times per day.¹⁷ Iloprost can be a very effective therapy, particularly when used in combination with oral agents. Although symptoms of breathlessness and fatigue are reduced, the frequency and time required for treatments limit its ability to improve QoL. During the past few years, novel oral treatment options have become available and can be used alone or in combination with another of the prostacyclins. Sildenafil (Revatio^TM), the phosphodiesterase-5 inhibitor that is used for erectile dysfunction, is effective in PAH and improves QoL and WHO functional class.²² It has minimal side effects and is simple to use, but it costs $12,000 to $15,000 yearly. The endothelin-antagonist class of drugs (Tracleer^TM, Thelin^TM) is similarly effective, but can be toxic to the liver and so requires monthly liver-function testing; it is potentially teratogenic (sperm, egg, and embryo), requiring double birth-control protection and regular pregnancy testing.¹⁷ The cost is about $35,000 annually. Although each of the therapies for PAH may improve pulmonary hemodynamics, exercise capacity, and survival, they may not have a positive impact on measurable QoL, especially on mental and emotional dimensions.^23–25

The cost of treatment and the disabling nature of the disease can have a major impact on the relationship of the patient with the spouse or significant other, children, parents, friends, and employers, and on patients’ overall financial security.^4,8 Clinical interaction with patients indicate that spousal support is very helpful, but marital conflict and divorce are not uncommon. The great majority of women and men seek medical disability and/or social security disability. Needless to say, the availability of insurance coverage for treatment has a major impact on psychosocial response to the disease and its manifestations.⁸

Importance of Adaptation to Chronic Illness

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PAH cannot presently be cured, so factors influencing psychosocial adjustment to the illness must be considered along with those affecting medical outcome. Studies from the broader literature on chronic illness support the notion that failed coping (i.e., a disruption of psychological homeostasis precipitated by the failure of usual coping strategies²⁶) not only contributes to poor psychosocial outcomes such as depression²⁷ but can have an effect on medical outcomes, as well. Failure to adequately manage stressors or to adjust emotionally to illness have been associated with vulnerability to disease and worsening of disease course.^28–36 In particular, the literature shows depression to be a risk factor for both the development and progression of ischemic heart disease^37,38 and diabetes,³⁹ and it is associated with other chronic illnesses, such as respiratory disease,⁴⁰ cancer,⁴¹ and arthritis.⁴² The risk of experiencing psychological symptoms is increased as the number of medical problems and functional disability increase.^43,44 Managing stress and coping well with chronic illness are important to both physical and emotional well-being, but their importance may be greater in those with PAH for two significant reasons: First, a laboratory study examining the effects of a mental stressor on individuals with severe PAH found the stressor to increase both pulmonary artery pressure and pulmonary vascular resistance, suggesting that active pulmonary vasoconstriction was associated with mental stress in this population and has the potential for serious medical outcome.⁴⁵ Second, a possible survival advantage has been reported for PAH patients treated for depression with selective serotonin receptor inhibitors (SSRIs).⁴⁶ Although this study needs to be validated, because of the pleiotropic effects of the SSRIs, the mechanism of benefit may be the antidepressant effect or other effects, including anti-platelet effects and vascular remodeling.

Qualitative studies examining the emotional experiences and coping strategies of individuals living with PAH report dissatisfaction with emotional and social QoL⁴⁷ and a greater need to cope with uncertainty and learning how to better live with the consequences of their disease.⁴ Also, individuals with PAH struggle to manage emotions related to years of being misdiagnosed, a lack of understanding of the disease by non-PAH specialists, the refusal of some insurance companies to offer reimbursement for treatment, and the turmoil the illness can create in a family, especially one with young children.⁸ These are sizeable personal and social burdens, and, when one adds the physical burdens of PAH, it is easy to see that failed efforts at coping with the illness experience can result in both psychological and physiological disturbance.

Psychiatric Comorbidity

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Most healthcare professionals working with individuals diagnosed with PAH recognize the emotional distress associated with the illness and its treatment, but the literature provides little information about the actual psychiatric health of this patient population. To our knowledge, only one study, done by Lowe and colleagues,⁴⁸ was designed specifically to examine psychiatric comorbidity in a population of individuals with PAH. Individuals diagnosed with PAH from a PAH patient organization were matched to a patient group with inflammatory rheumatic disease and a general primary-care group. The PAH group did not differ from the comparison groups except on panic disorder and the presence of panic attacks. In the PAH group, 10% had a panic disorder, with 26% reporting the presence of panic attacks; 16% had a major depressive disorder; and 7%, other depressive disorders. When compared with the rates in the general population of between 1.5% and 3.5% for panic disorder and 2% to 9% for depression,⁴⁹ the rates reported in this sample of PAH patients are high.

In developing a QoL measure designed specifically for PAH patients (Cambridge Pulmonary Hypertension Outcome review [CAMPHOR]),⁵⁰ a validation sample of 89 PAH patients were shown to have a mean mood score of 2.8 (standard deviation [SD]: 2.3; range: 0 to 7) where lower scores reflected better mood. Only 6% of the sample scored the maximum 7. A descriptive study of PAH patients responding to the Hospital Anxiety and Depression Scale (HADS) reported that 20.5% and 7.5% of the sample fell into the anxiety and depression groups, respectively.⁵¹ Together, these studies suggest that anxiety, particularly panic, is a significant problem in this population, followed by depression. Although these studies provide us a glimpse into the psychiatric health of this patient population, they are incomplete.

One limitation is the method of selecting the patient population. In the study examining the psychiatric comorbidity of PAH patients, Lowe and colleagues⁴⁸ used a sample of individuals with PAH who belonged to a patient organization for those with this condition. It is possible that these individuals were not typical of the average PAH patient in that their emotional/social functioning may have been better because of their participation in the organization, and, as a result, they were better able to cope with their situation. By developing and utilizing a social resource to help in their adjustment to illness (membership in this PAH association) they may have already demonstrated an active coping style that is associated with better emotional well-being.⁵² This suggests that the prevalence of panic and depression found in this sample group may underestimate the true prevalence rate in the general population of PAH patients.

Also, not all studies have examined emotional well-being by stage of illness; for example, NYHA (New York Heart Association) functional class I–IV, which is similar to the World Health Organization (WHO) functional class. When the prevalence of psychiatric illness in the sample of patients belonging to a PAH association was examined by functional class, 17% of NYHA Class I and 62% of those in NYHA Class IV were found to have a psychiatric illness.⁴⁸ The prevalence rate of 62% from this particular sample is a large number if one assumes there is a higher level of social support and active coping processes, given that they belong to a PAH patient-support organization. It gives one pause to wonder what that rate might be in a population of individuals who have not sought or had available these outside social resources.

The high rates of panic disorder and presence of panic attacks found in PAH patients is not insignificant. Similarly high rates of panic have been reported in those with chronic obstructive pulmonary disease (COPD).⁵³ Despite their different etiologies, the similarity between COPD and PAH is the experience of shortness of breath, a major symptom in panic. Rates of anxiety and panic disorder in COPD populations range from 13% to 33% for severe anxiety and from 8% to 32% for panic disorder.^54–57 Psychotherapy and psycho-education are important interventions in this patient population, but it is not known how many PAH patients are offered or seek out mental health care. One study found that of 58 PAH patients with a psychiatric disorder, fewer than 25% reported receiving any treatment for their psychiatric illness.⁴⁸

Coping and Emotional Well-Being

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People cope in an attempt to maintain a sense of consistency, stability, and meaning while going through change. Coping has been defined as the active cognitive and behavioral adjustments made by an individual to manage both external and internal demands that are seen to be overwhelming and threatening to their sense of self.⁵⁸ This suggests that, for the PAH patient for whom reduced physical functioning is the norm, it is not the decrease in functioning that affects emotional well-being, but the impact of that change on the patient’s ability to engage in activity that provides meaning and allows them to fulfill roles that they view as important.

The contributions of chronic illness-related stressors to an individual’s sense of self and ability to adjust are many. For example, individuals with chronic illness often cope with some level of stigma, which can affect how they see themselves, whether they remain socially involved or become socially isolated, and, ultimately, how well they cope with their illness.⁵⁹ Individuals must also cope with limited independence,⁶⁰ side effects from and adherence to medications,⁶¹ impaired activities of daily living, and fears related to disease progression. One of the more challenging aspects of chronic illness for both individuals and their families can be the perceived randomness associated with worsening symptoms.

The theoretical model often cited in connection with coping with threatening events is the cognitive adaptation model.^58,62 The model describes two processes: appraisal and coping. Appraisal is how an individual views an event, whereas coping refers to thoughts and behaviors used to regulate distress (emotion- versus problem-focused coping). Although there are a number of measures available to assess these constructs, none have been used with PAH patient populations. However, some QoL assessments offer a rough measure of coping-related constructs (e.g., role functioning: the ability to function in various roles; social isolation: withdrawal from social resources; emotional mastery: feelings of having control over illness). There have been a number of PAH descriptive/treatment outcome studies that have reported on QoL measures.^51,63–65 Examining these studies provides some useful information on the coping strategies of individuals with a diagnosis of PAH.

A descriptive study using the Medical Outcomes Study–Short Form (SF–36), a measure of psychological and physical symptoms (physical functioning, emotional functioning, social functioning, and mental health composite scores) found all functioning dimensions to be significantly lower in the PAH group, as compared with United States population norms. Taichman and colleagues⁶³ reported no differences in the mental health composite score on the basis of type of PAH treatment provided (calcium channel antagonists, intravenous epoprostenol, or bosentan). Another descriptive study reported a significantly greater emotional reaction (psychological symptoms present as the result of the illness [mean: 27 SD: 25]) and social isolation (withdrawal from social interaction [mean: 25 SD: 27]) to be higher in the PAH group than in a healthy-control group (mean 12 and 5, respectively), but these scores were relatively low, given the possible range of 0 to 89 for emotional reaction and 0 to 100 for social isolation.⁵¹

A study of 41 patients with severe PAH (NYHA classes III and IV) given continuous intravenous infusion of epoprostenol, were compared with a control group (N=40) receiving conventional treatment. The treatment group showed significant improvement in emotional functioning and mastery (feeling of control over their disease), whereas no improvement was found for social isolation.⁶⁴ A study of 233 patients (NYHA Class II, III, and IV) receiving a continuous subcutaneous infusion of treprostinil reported no improvement in emotional functioning, as compared with a placebo-control group (N=236), and a study comparing (NYHA Class III and IV) iloprost inhalation (N=101) with placebo (N=102) reported no improvement in emotional functioning, anxiety, or depression symptoms.⁶⁵

Limited conclusions can be drawn from these studies concerning the well-being of PAH patients. The studies were not specifically designed to better understand the emotional and social well-being of PAH patients, and the results are applicable only to patients willing and able to participate in controlled trials; these patients may have better social support and coping skills. There is no uniformity in QoL measures used or how anxiety and depression were operationalized. The PAH populations studied came from different NYHA functional classes and were receiving a variety of different medical treatments, each of which are likely to have varying outcomes on emotional well-being (due to the differing efficacy and methods of administration). Finally, no information was collected on the mental health or psychiatric treatment received by individuals in these samples. Because of the low prevalence of PAH and the resulting difficulty for any one PAH center to study large numbers of PAH patients over time, funding is necessary for multicenter studies to investigate behavioral and social-science questions outside of clinical trials.

Psychosocial Adjustment

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Successful psychosocial adaptation can be described as the ability to function within given interpersonal, social, and cultural norms, given new personal, social, and environmental demands created by one’s illness.⁶⁶ Factors affecting adaptation include psychological functioning (mood, self-concept, coping style), disability-related consequences (level of functioning, ability to remain engaged in role activities or to redefine one’s roles, employment), and social systems (partner/spouse, family, friends, and community).⁶⁷ In addition to the impact of psychiatric and coping-related factors that have been discussed so far, there are also social factors.

A hardship for many with chronic illness, and particularly for those individuals diagnosed with PAH, is the adjustment to interpersonal and social issues unique to the symptoms and treatment of their illness. Although the typical patient with a diagnosis of PAH experiences some degree of physical limitation requiring either frequent resting and/or significant limitations in physical functioning, clinical observation would indicate that few of these individuals actually "appear" sick. In the early stages of their illness, their disease is hidden in that they appear well to others but frequently "act" sick. This can create perceptions that the individual "is not very sick" or that they might be exaggerating their symptoms in order to gain sympathy or other secondary gains such as being relieved from family, work, or social role responsibilities. These issues can be difficult to cope with in the early stages of the disease, particularly if patients themselves have not fully accepted that they are ill. Such struggles frequently lead to reduced social functioning. Social interactions are important for gaining and maintaining social acceptance, but for many individuals with a PAH diagnosis, physical limitations can lead to increased social isolation.

The reduced physical activity and exercise capacity found in this population is likely to affect more than social functioning. There is evidence that exercise benefits those suffering from depression and anxiety.^68,69 Studies have consistently shown sedentary lifestyle to be associated with overall mortality and found that exercise improves physical well-being, aerobic capacity, and strength, and improves immune functioning in persons with heart disease.⁷⁰ In a small pilot study assessing the role of exercise in PAH, Mereles and colleagues⁷¹ randomized 30 patients to an exercise training program or a control group. Intervention consisted of 3 weeks of in-hospital-training, followed by 12 weeks of at-home training. After 15 weeks, the exercise group improved on measures of exercise capacity and QoL, with the control group unchanged or worsening in those areas. Exercise training was safe and well tolerated for this group of PAH patients. The demonstration of a benefit from an intense, albeit very costly, protocol for exercise training in PAH should prompt the evaluation of more practical and less-costly protocols. The duration of benefit and willingness of patients to continue exercise also needs to be assessed. It is not expected that exercise will alter the progression of the pulmonary vascular disease. Rather, increasing strength and aerobic capacity by training muscle groups allows for physical activity to be performed at a lower cardiovascular workload. The recognition that patients can contribute to their wellness beyond taking medications may also serve to reduce anxiety and depression associated with their illness.

Palliative Care

TOP ABSTRACT INTRODUCTION Review of PAH: Diagnosis,... Importance of Adaptation to... Psychiatric Comorbidity Coping and Emotional Well-Being Psychosocial Adjustment Palliative Care REFERENCES

 
Palliative care has been described as the active, interdisciplinary care of individuals whose disease is not curable and where a more holistic approach (physical, psychological, social, and spiritual) is used in caring for them.⁷² The issue of palliative care has not been well addressed in the PAH literature and in fact, has received little attention outside the domain of cancer care.⁷³ Recently, there has been increased attention to addressing the needs of terminally ill patients with heart disease, COPD, and other non-cancer health conditions.^74–77 It has been suggested that special attention should be paid to patients who "reasonably might die" and/or experience multiple hospitalizations due to acute medical complications associated with their illness, so that healthcare staff might be more proactive in providing information that is needed for rational end-of-life decision-making.⁷⁶ The appropriate timing for this communication remains to be established, but it is important that healthcare staff provide opportunities to discuss these issues with PAH patients before crisis hits and when the patient is ready to examine end-of-life issues (functioning, complications, QoL, and care needs).

There are two relatively unique end-of-life issues concerning the individual diagnosed with PAH. The first is the question of the appropriateness of cardiopulmonary resuscitation (CPR). Hoeper and colleagues⁷⁸ report that in a sample of 132 PAH patients where CPR was attempted, it was unsuccessful in 79% of the sample, and an additional 13% died within 7 days. An additional issue for the PAH patient nearing the end of life is the refusal of the hospice to assume care because of the cost of intravenous prostacyclin treatment, which, if discontinued, would hasten death to within minutes to hours. The decision to stop the intravenous prostacyclin is comparable to removing a conscious patient from a ventilator, and so, without access to hospice care, the patient and family are left with few choices and limited support at the time of greatest need.

In summary, the state of mental health care for patients with PAH is in its infancy. To move the science forward, there must be a concerted effort to design research that will help us better understand whether the nature of the depression and anxiety experienced by these patients is qualitatively different from that in other patient populations, in order to determine whether standard treatments are effective in this population and whether treatment of their anxiety and/or depression might prolong and/or improve life. PAH is a devastating disease, one requiring significant physical, emotional, and social adjustment in order to live with it successfully, and these patients deserve to receive more holistic treatment, but, as a first step, we must better understand their illness experience.

REFERENCES

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