The term pulmonary hypertension (PH) is broad and applicable to a group of conditions. The early 1990s saw major changes in the understanding of the spectrum of disease affecting the pulmonary circulation. We have also seen the central place of issues such as understanding, capturing, and directing treatment pathways towards improving the health-related quality of life (HRQoL) for PH patients. This has led to an increased interest in assessing HRQoL with a greater sensitivity and validity in patients with PH, which since 2013 has been enhanced by the development of a new PH-specific HRQoL tool (emPHasis-10; Fig. 24.1 ).
Experiences of Living With Pulmonary Hypertension
A detailed literature review would identify only a small number of qualitative research papers. This qualitative work exploring the experiences of living with PH is currently limited, and there is one study focusing on the prediagnostic phase. There is much reporting within these studies that PH symptoms, particularly breathlessness, are unpredictable and make any planning for the immediate and long-term future difficult for patients. Breathlessness has not only been identified as the most burdensome symptom of PH but also presents patients with a limitation in activity and a need to redefine their lives. Yorke et al. obtained data from a study exploring the day-to-day impact of PH, gaining valuable insight into the lived experience from the patients’ perspectives. The lack of visible signs of having a chronic condition, which might otherwise alert people to the fact that they are ill, presented participants with a unique challenge. This often meant that they felt they were living with a concealed illness. There was a sense that other people did not appreciate how ill participants were and often felt that they were required to justify why, for example, they were frequently exhausted or short of breath. Patients also reported that PH places a heavy burden on their social and emotional well-being, describing feelings of frustration, anger, and low self-esteem, as well as feeling misunderstood and insignificant. Patients gained little pleasure from the activities they used to enjoy and some felt fearful or frightened of the future. Frequently, participants reported that their PH symptoms were unpredictable and highly variable from day to day. Living with an unpredictable fluctuating illness made such things as applying for disability benefits difficult and a source of constant frustration for many participants. Others found traveling difficult, especially if they required supplementary oxygen.
Starting PH treatment requires a period of adjustment, with participants learning to live with their life-threatening disease, and for some, beginning an invasive treatment regimen. The perceived time limit for PH drugs remaining effective made participants fearful of the future; commencing new treatment offered optimism and a sense of control. For some, the side effects of medication were viewed as “worse than the breathlessness,” and could not be “endured.” The effort required to adhere to different treatments was often viewed as causing distress, which patients often hid from family and friends. The frustration and distress experienced by patients when trying to convince their doctor that their concerns were real were identified. Similarly, disproportionate side effects that necessitated certain drugs to be stopped left participants wondering what alternatives might next be offered. This was further supported in a study by Peloquin et al., who interviewed three females in the initial stages of prostacyclin therapy and found that the therapy regimen was troublesome and impacted negatively on the patients’ perceived quality of life. Flattery et al. explored the experiences of 11 patients with PH and found that coping with uncertainties and troublesome treatment regimens were, unsurprisingly, both challenging and stressful for the patients.
It is known that the proportion of PH patients unable to work due to their PH is considerable. Therefore the socioeconomic impact of PH is likely to be high. Many patients have to give up work or reduce the number of hours they work because of the effects of PH. This can place a substantial financial burden on the patients and their families.
Within two of these studies, Armstrong et al. and Pulmonary Hypertension Association UK (PHA UK), were able to describe the patients’ experiences and the “journey” of PH from the symptoms’ onset through to their living with the condition. This journey includes not only the physical effects of PH, such as symptoms and effects on mobility, but also the psychosocial effects, such as the emotions associated with diagnosis and living with the condition. It may also include the effects on loved ones.
Each patient will have his or her individual journey, which will differ from others, depending on the severity of symptoms and other coexisting conditions, such as age and experiences. However, generally this journey can be broadly divided into four distinct sections: (1) prediagnosis phase (when patient first notices symptoms), (2) diagnosis, (3) treatment, and (4) living with the condition.
Developing a patient’s journey provides a number of significant insights, including the following:
- 1.
Barriers and challenges that a patient faces at different stages of his or her journey
- 2.
Factors that affect decision making
- 3.
Interactions that the patient has with health care professionals
- 4.
Emotional effects of PH
- 5.
Attitudes and expectations (in terms of diagnosis, treatment, and care)
- 6.
Adherence to medication
- 7.
What motivates a patient
- 8.
Effects of PH on family members, caregivers, and others close to the patient (as graphically described in Fig. 24.2 later)
Figure 24.2
The full picture of pulmonary hypertension (PH) for the patient and their loved ones.
(From Pulmonary Hypertension Association UK [PHA UK]. The impact of living with pulmonary arterial hypertension. Results of two research projects commissioned by the Pulmonary Hypertension Association UK. 2011.)
- 9.
Support that the patient may need (practical, psychological, and educational)
Summary
Living with PH presents numerous challenges. PH can have a profound effect on all aspects of a patient’s life and the lives of those close to the patient. This relates to the ability to perform daily tasks, the ability to work or attend education, and the emotions associated with having a serious life-limiting condition ( Fig. 24.3 ). Everyday tasks, such as shopping, doing the housework, and even getting out of bed, can be difficult.
After being diagnosed with PH, patients are faced with the reality that it is a life-limiting condition. Dealing with this and the uncertainty of prognosis can lead to a range of issues for the patients and their family members and caregivers. This includes dealing with the reality of having a terminal illness, including the practicalities of the end of life, such as making a will and advanced care planning. Health care professionals play an important role in ensuring that patients and family members have the support they need to address issues associated with the end of life. Because prognosis for patients with PH is difficult to calculate approximately and depends on a number of factors, the end of life may need to be discussed when a patient is ostensibly well. However, patients should be made aware of the unpredictability of their illness and the importance of thinking about end-of-life issues, independent of the severity of their PH.
Adult Congenital Heart Disease in Nursing
International health care systems are witness to a growing population of adults with congenital heart disease (ACHD) due to advances in diagnostic, medical, and surgical techniques. ACHD accounts for 66% of the entire CHD population in 2010. It has been estimated that there are 135,000 adolescents and adults living in England with CHD with a 10% estimated annual growth rate based on live births. The increased life expectancy, with over 90% of infants reaching adulthood, has led to a demonstrable impact on clinical outcomes and patient longevity. Challenges related to the management of arrhythmia, heart failure, and PH are a sequela of palliative surgeries on long-term functioning and health related quality of life.
Nurse Education
Nurse education and training acknowledge the growth within this complex specialty underpinned by a competency framework. Clinical competence will ensure patients and their carers receive expert care throughout the trajectory of this lifelong condition. ACHD nurses have the opportunity to contribute and advance practice with new demands for expanding roles in health assessment, nurse-led clinics, and enhanced counselling skills with a focus on individualized, shared care.
There are many challenges in facilitating a shared care model with healthcare resources expected to meet optimal care standards promoting a cohesive, high quality expert tertiary service with shared responsibility provided by primary care. The primary driver is unlikely to incorporate cost alone but to follow indicators of quality outlined by commissioners and guidelines with strategic aims to improve patient experience.
Expectations of patients are changing. There is a greater emphasis on patient experience that acknowledges the value of quality of life, alleviation of fear and anxiety, and impact of illness on patients, their families, and their finances.
The global phenomenon of nursing shortages represents our greatest obstacle. The Royal College of Nursing London staffing report 2015 shows a nurse vacancy rate of 17% and an overwhelming reliance on agency nurses and recruitment from overseas. However, clinical nurse specialists are well-placed to provide support for a knowledgeable, dedicated nursing workforce and offer leadership in education and development, enabling succession planning. Time allocated for junior nurses to learn from the experienced ACHD specialist nursing team, attend outpatient clinics, and have direct contact with ACHD patients complements experiential learning with access to funded ACHD postgraduate academic qualifications previously not deemed economically viable.
An understanding of the altered anatomy of CHD, of prior surgery, and of the effect of disease on patient wellbeing can positively affect patient safety, quality of care, and healthcare outcomes. It promotes early recognition of acute deterioration in a patient with univentricular physiology and atrial arrhythmia. It also helps avoidance of dehydration, understanding of oxygen requirements, administration and provision of bubble traps or air filters in intravenous lines to prevent paradoxical air emboli in those with septal defects and/or cyanosis, and recognition of the significance of blood pressure assessment for patients (eg, coarctation of aorta and Blalock-Taussig shunts).
The role of the specialist and advanced nurse has been reported to have a positive effect on patient satisfaction. A service evaluation found ACHD nurses to be knowledgeable, informative, clear, and helpful. Respondents were satisfied with the time given to discuss individual concerns and they knew whom to contact if their conditions changed. This service evaluation provided insight into the value of the nursing service to the patient’s experience with emphasis on expertise, knowledge, advice, care coordination, accessibility, and responsiveness.
Psychological functioning, quality of life, and health perceptions are pertinent issues in gaining understanding of the long-term effects of living with CHD and of treatment demands. Specialist nurse services are well established in their current form. Advice and support are provided from diagnosis to end-of-life care and incorporate a multidisciplinary approach ( Fig. 24.4 ).
