This case study presents Mr Churi (not his real name), who received a bilateral lung transplant in Australia for silicosis. After introducing the patient and his history, this case study explores the pathophysiology of silicosis and the reason for transplantation. It discusses some elements of Mr Churi's postoperative recovery including his experience of type 2 respiratory failure (T2RF) and noncompliance with noninvasive ventilation (NIV); nursing care and management in this area are considered. Challenges in patient medication education are discussed and, finally, social implications during recovery after lung transplantation are explored.
Over the past three decades, lung transplantation has become a well-established treatment for a variety of end-stage pulmonary diseases (Hachem, 2018a). In Australia, lungs are the second most commonly transplanted organ; 222 lung transplants were performed in 2018, compared with 150 10 years previously (ANZOD Registry, 2019). This mirrors the international experience, in which the incidence of lung transplantation is growing along with the rate of post-transplant survival (Yusen et al, 2013). Although lung transplant survival rates are climbing, post-transplant complications persist (Gutierrez-Arias et al, 2016).
Patients can face a burden of deteriorating health that leads to a decision to transplant as well as recovery pathways after transplantation that are complicated by physical, psychological and social challenges (Graarup et al, 2017). Nurses play a key role in all aspects of recovery and ongoing patient education.
Silicosis is a rare indication for lung transplantation but, as no other treatment is available for the disease in its end stages, bilateral lung transplantation potentially offers a life-saving therapy (Mao et al, 2011; Rose, 2018).
Patient history
Mr Churi, a 52-year-old man, was diagnosed with advanced silicosis with progressive massive fibrosis.
Initially, Mr Churi presented to a GP after developing dyspnoea, which worsened over a 4-year period. He was a non-smoker with no diagnosed respiratory conditions. In addition to his reported history of dyspnoea, Mr Churi's only other medical history included diagnoses of hypertension and hypercholesterolaemia. Mr Churi was previously very fit and had worked in landscaping for 18 years. His occupational history is critical. During his work as a landscaper, he was involved in sandstone cutting and exposed on a regular basis to sand and cement dust containing crystalline silica; inhaling this has been shown to be linked directly with the development of silicosis (Rose, 2018). His worsening respiratory symptoms had led to his inability to work.
Four years after Mr Churi saw the GP, a specialist diagnosed silicosis and urgently referred him for assessment at a lung transplantation unit. Candidates for lung transplantation are usually symptomatic during activities of daily living (Hachem, 2018b). At the time of referral for assessment at a lung transplantation unit, Mr Churi's exercise tolerance had reduced from unlimited to feeling breathless after 10 m, unable to manage 100 m and unable to walk up more than five steps. He had also developed a cough productive of a cup of sputum a day and a noted wheeze. Lung function testing demonstrated a forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio of 0.72; normal is considered to be >0.80.
In the subsequent 11 months, Mr Churi progressively deteriorated. His FEV1/FVC ratio further decreased to 0.57 and he was receiving continuous domiciliary oxygen. He was supportively treated with a combination of inhaled medications to manage his respiratory symptoms, including a short-acting bronchodilator (salbutamol), a long-acting bronchodilator (vilanterol), an anticholinergic (ipratropium) and corticosteroid (fluticasone furoate). Following full assessment, Mr Churi was placed onto the lung transplant waiting list and, 3 months later, received a bilateral lung transplant.
Mr Churi had a complicated psychosocial background and, after a business failure, was under significant financial stress. At the time of his transplant, he was effectively homeless, staying with family members, estranged from his wife and the primary caregiver for his three teenage children.
Pathophysiology of silicosis
Silicosis covers a range of lung diseases caused by inhaled silicon dioxide or silica. Occupational exposure occurs in many industries where substances containing silica are broken down to dust (Leung et al, 2012). Silicosis can be classified as chronic, acute or accelerated. Chronic silicosis symptom onset is usually 10–30 years after initial exposure, whereas in acute silicosis, symptoms occur within weeks to years of exposure, and accelerated silicosis occurs within 10 years of high-level exposure (Rose, 2018). Mr Churi's case involves chronic silicosis in the context of his initial occupational exposure to silica dust and his first reported symptoms 15 years later.
Although some cellular mechanisms are understood to be involved in silicosis, the complete pathogenesis remains unknown (Bhattacharjee et al, 2016). Initially, inhaled particles of silica are deposited in the alveoli of the lung (Pollard, 2016). The physiological role of macrophages in the lungs is to clear particles from the alveoli (Grossman, 2014). It is understood that, after being deposited, silica particles cannot be cleared from the lungs so, when alveolar macrophages ingest these particles, an inflammatory response is triggered. This response initiates the production of fibroblasts and collagen. Collagen engulfs the silica particles and creates the parenchymal nodules that can be seen with radiography and characterise silicosis (Pollard, 2016). Silicosis is characterised by small nodules of a few millimetres in size, commonly situated in the upper lobes of the lungs. As collagen and fibroblasts proliferate, fibrosis develops and the lungs' capacity for respiration diminishes (Palabiyik et al, 2013). A fibrotic lung is stiff and noncompliant, making inflation difficult, which causes increasing dyspnoea and decreasing exercise tolerance because of hypoxaemia (Grossman, 2014). In a minority of cases, including Mr Churi's, chronic silicosis may advance to progressive massive fibrosis (PMF) (Rose, 2018). In PMF, individual parenchymal nodules coalesce to form nodules that may be greater than 10 mm in size (Gibbs and Attanoos, 2018).
In addition to the diagnostic radiological appearance, symptoms of simple silicosis may be a chronic cough and dyspnoea on exertion (Rose, 2018). As silicosis progresses and becomes advanced or end stage, as it did in Mr Churi's case, fibrosis becomes diffuse, gas exchange is impaired and T2RF, with its characteristic elevated PaCO2 and reduced PaO2, may ensue (Mao et al, 2011). Clinically, this may manifest as worsening radiographic pulmonary signs (Figure 1), increased sputum production and the presence of a wheeze. PMF presents with more severe symptoms of cough and dyspnoea than simple silicosis (Rose, 2018).
Transplantation as the treatment option
Silicosis carries a poor prognosis; it is associated with significant premature mortality (Mao et al, 2011). Treatment options are limited to supportive therapies, such as smoking cessation, vaccinations against respiratory infections such as influenza and pneumococcus, bronchodilators to treat airflow limitations and the use of supplemental oxygen. Experimental treatments have been proposed, such as inhibition of cytokines, but have not yet provided a definitive mainstream treatment option. Individuals such as Mr Churi, who progress to advanced silicosis and have no contraindications, may be considered for lung transplantation (Rose, 2018).
The International Society of Heart and Lung Transplantation (ISHLT) developed a consensus document for recipient selection for pulmonary transplantation (Weill et al, 2015). Broadly, an individual should be considered for transplant if they have physiologically and clinically severe lung disease for which medical treatment is not available or effective. The document specifies that candidates should meet three criteria to be considered for transplant: a high risk of death within 2 years without transplantation; a high likelihood of surviving 90 days after a transplant; and a high chance of surviving 5 years after a transplant.
Although transplantation may be the only intervention available for patients with advanced silicosis, transplantation itself is still a relatively uncommon treatment for silicosis so survival specifically after lung transplantation for this condition is not well researched (Singer et al, 2012). Nonetheless, a recent study (Hayes et al, 2015) supported transplantation for silicosis as an effective treatment for patients meeting the ISHLT transplantation criteria. The study looked at 7227 lung transplant recipients and found that long-term survival for individuals with silicosis was not significantly different from that of the rest of the lung transplant population. However, this research was limited by its population size, with only 24 of the 7227 patients receiving a transplant for silicosis.
Postoperative recovery journey
Lung transplantation offers a life-saving treatment but the patient's postoperative journey to recovery is complex and demanding (Lundmark et al, 2016). The recovery pathway after lung transplant surgery can vary significantly, from a 1–2 week postoperative hospital stay with no complications to a protracted hospital stay with a need for extensive rehabilitation because of physical deconditioning or other complications.
Potential complications after a lung transplant that may be experienced include infections because of immunocompromise, primary graft dysfunction, surgical problems of both airway and vascular anastomoses or the pleura, alloimmune responses causing organ rejection, phrenic nerve and diaphragmatic dysfunction, and malignancy such as post-transplantation lymphoproliferative disease or skin malignancies (Habre et al, 2018; Ahya and Kuwat, 2020). These are in addition to more generally experienced postoperative surgical complications such as postoperative bleeding, pain, dysrhythmias and acute or chronic renal failure (Ahya and Kuwat, 2020).
Immediately following his bilateral lung transplantation surgery via a ‘clamshell’ thoracotomy incision, which extends across the entire anterior chest, Mr Churi was transferred from the operating theatre to the intensive care unit (ICU). His immediate postoperative recovery was complicated by a reperfusion injury to his lungs. Reperfusion injury is a pathological process that can occur when oxygen supply to the lung has been compromised followed by a period of reperfusion, such as during lung transplantation surgery. Mr Churi required support of veno-venous extracorporeal membrane oxygenation (ECMO) for seven days after his surgery. ECMO is used for prolonged mechanical cardiopulmonary support, and can be veno-arterial or veno-venous (Bartlett, 2021). Veno-venous ECMO provides primarily pulmonary support. Mr Churi was then weaned off sedation and mechanical ventilation support and extubation was carried out. Mr Churi was subsequently transferred to the ward area to continue his recovery.
During this period of Mr Churi's recovery, his respiratory status became increasingly compromised. An arterial blood gas analysis demonstrated T2RF, with arterial blood levels showing elevated carbon dioxide and decreased oxygen. In conjunction with this, Mr Churi developed a lower respiratory tract infection, with the causative organism likely acquired from the donor anastomosis site as identified from donor lung swab microbiology testing.
Following lung transplantation, there is loss of normal cough reflex because of intraoperative nerve disruption, which is compounded by a reduced ability to cough owing to postoperative pain. Additionally, these patients are immunosuppressed, which renders them highly susceptible to respiratory infections. These factors combined put lung transplant recipients at a high risk of respiratory failure (Ahya and Kuwat, 2020), which Mr Churi experienced. To combat this, he received treatment that included targeted intravenous antibiotics, adequate analgesia and regular chest physiotherapy. These further variations from an anticipated smooth recovery pathway brought significant challenges for nursing care and management. A recent study of 156 lung transplant recipients with acute respiratory failure concluded that these patients are at a high risk of death and that NIV is a viable intervention (Wiles et al, 2018).
Mr Churi was treated on the ward with bi-level positive airway pressure for a number of days, a form of NIV intended to improve gas exchange in the lungs and support the work of breathing for a patient with fatigued and ineffective respiratory muscles (Credland, 2013). Mr Churi continued to deteriorate and was subsequently readmitted to the ICU. Banga et al (2014) have reported that acute respiratory failure is the most common cause of readmission to ICU for lung transplant recipients.
It is well established that high patient compliance with NIV is associated with better survival (Rousseau and Pietra, 2016); however, rates of noncompliance with NIV therapy in the clinical setting have been reported to be as high as 20-30% (Shah et al, 2018). Interestingly, there is a significant gap in the literature around the impact of nursing care in relation to patient compliance with NIV. A qualitative study on patient experience of NIV found that feelings of fear, anxiety and physical discomfort were associated with the therapy (Goldman et al, 2016).
The success of NIV depends on the patient's tolerance of the treatment and their level of comfort (Sanchez et al, 2014). It could be argued that the patient's tolerance and comfort is greatly influenced by the nurse providing the treatment.
Mr Churi's patient notes—both general documentation and nursing reports—showed he was frequently noncompliant with NIV therapy before he was readmitted to the ICU. This was despite reported reassurance and encouragement by the nurses, which included telling him about the reason for and benefits of NIV in treating T2RF.
When examining the nursing management of Mr Churi's NIV therapy, it is essential to consider local guidelines that provide recommendations for best practice. The New South Wales Agency for Clinical Innovation has published guidelines on the care of patients receiving NIV for acute respiratory failure (Sanchez et al, 2014). These guidelines recommend that patient comfort and pain should be assessed and documented every 2 hours at a minimum. They also recommend that, within the first 24 hours of NIV initiation, a clear plan for nursing care while the patient is receiving NIV should be documented and, specifically, that this should include psychosocial support and spiritual needs. Although Mr Churi's medical records demonstrate that his NIV therapy was regularly assessed, the records are less specific in noting his comfort levels at a frequency commensurate with the guidelines, providing instead a summary statement over 6–8 hour periods.
As Mr Churi continued to decline NIV, he became progressively tachypnoeic, hypercapnic and hypoxaemic over a number of days until his eventual readmission to ICU for respiratory management. Nurses play an active role in the administration of NIV and, as such, there is potential that his admission to ICU may have been prevented by improved practice. In the alleviation of suffering, nurses have a responsibility to support their patients, listening to their concerns and providing empathy and reassurance (International Council of Nurses, 2012; Burton, 2013). To promote health, nurses have a challenging role in empowering and educating patients to help them make informed decisions about their care (Manley et al, 2011). In Mr Churi's case, there was a pivotal nursing responsibility to listen to his anxieties, offer empathy and educate him on the importance of persisting with the NIV to prevent respiratory failure from worsening.
Patient education
Despite significant clinical complications in the postoperative journey after lung transplantation, patients are expected to play an active role in their recovery and they need continual support to do so. A qualitative study by Lundmark et al (2016) of 15 lung transplant recipients explored the patient's perspective of the postoperative journey to recovery. Participants identified a number of positive and negative factors that influenced their post-transplant recovery. Support from health professionals was said to be positive and complications and setbacks were articulated as negative.
Mr Churi returned to the ward following a 5-day admission to ICU for T2RF and expressed feeling disheartened and distressed by the ‘setback’. At this point in the journey, support and patient education is paramount. Organ transplantation requires a complex programme of medication administration for vital therapies such as immunosuppression and includes difficult aspects like daily dosing schedules (Mellon et al, 2017). Thus the nurse's role as an educator is critical at this point in the patient's journey after a transplant. In Mr Churi's case, nurses explained to him that although his recovery may have varied from the predicted clinical pathway, recovery from lung transplantation is a difficult and demanding process that requires patient involvement in care (Lundmark et al, 2016).
Mr Churi voiced difficulties with learning to self-administer his medications, often expressing fatigue as an influential factor. These sentiments were also described by participants in Lundmark et al's study (2016). Nurses involved in Mr Churi's care spent significant time encouraging him and explaining that he was not alone with his difficulties in developing competence in self-administration of medication.
The methods used in patient education in the authors' lung transplant unit include education and supervised self-administration of medications by multiple members of the healthcare team. The specialist team includes lung transplant physicians and the surgical team, a pharmacist, a transplant clinical nurse consultant and clinical nurse specialists (CNSs) as well as the nurses responsible for Mr Churi's day-to-day care.
All patients are issued with a lung transplant handbook into which their daily medication regimen is written. This handbook is updated regularly, as medications and dosages are prescribed and adjusted. Patients and their families or carers initially experience flexibly timed personal sessions with the CNSs in which medications are explained, including the rationale for them and side-effects. They are also given practical tips for handling and storing medications, which are tailored to individual needs and circumstances. Ultimately, patients progress to self-administration under direct supervision of the nurse. Through this incremental and reinforced learning, Mr Churi ultimately developed confidence and accuracy in the self-management of his medication regimen.
A study by Davis et al (2014) investigated lung transplant patients' recommendations for ideal lung transplant education. It determined that information on medicines and immunosuppressants was highly desired, and the preferred time frame for receiving this information was in the hospital phase following transplantation surgery. They also determined that the preferred method of obtaining this information was by website or other online resources, closely followed by discussion with a transplant professional.
Examining nursing management in this case, Mr Churi was well supported by nurses regarding patient education on setbacks and competency in medication administration that was in keeping with published information on lung patient education.
Family implications
Beyond the clinical and personal challenges of the post-transplant pathway to recovery lie family implications. It is important to consider Mr Churi's family circumstances in this context.
By the time Mr Churi had received lung transplantation, his eldest son had finished school and become his carer. His three children lived 5 hours away from the transplant centre. While Mr Churi was in hospital recovering from transplant surgery, the eldest son was working and caring for his younger siblings, who were attending school. Occasionally, he would visit his father in hospital and the hospital social worker helped to organise transportation for these visits. This highlights there is a key need to ensure adequate family support is provided especially in the context of young carers.
During his recovery and following significant setbacks, Mr Churi struggled with not only learning new skills including complex medication regimens but also finding the motivation to engage in physical rehabilitation. A clinical psychologist became involved in Mr Churi's emotional recovery from transplant surgery as his anxieties worsened. The psychologist reported that Mr Churi voiced much distress around a recent visit from his son. His son had witnessed his lack of engagement in physical activities and left, expressing anger, frustration and disappointment with his father. Mr Churi voiced feeling abandoned by his son after this. Lundmark et al's (2016) study identified that social support from family is a positive factor in the process of recovery for transplant recipients and, furthermore, feeling abandoned by family has a negative influence on recovery.
As nurses persisted with encouraging and educating Mr Churi on the importance of regaining physical strength and independence, particularly with regard to hygiene, he became gradually more engaged. He later reported to the clinical psychologist that after his son saw this improvement, he was able to continue supporting his father and Mr Churi felt motivated by that support.
During the social recovery after transplant, emotional support from family is critical, alongside support and guidance provided by health professionals including nurses (Forsberg et al, 2018). To meet the needs of the transplant recipient during their recovery, it is essential to offer a holistic approach to care and consider the social implications of the recovery, particularly for patients who have children at home (Ivarsson et al, 2013).
The stresses that present during the transplantation journey can have a considerable psychological impact both the recipient and their family (Williams et al, 2012). Ågren et al (2016) recommended that health professionals should help lung transplant patients and their families to use coping mechanisms and offer support both before and after transplantation, with interventions focused on strengthening the patient both physically and mentally. They also pointed out that information and communication are important in improving psychosocial support. In addition, they suggested information and communication technologies, such as video technology and text messages, are powerful tools that could be used in continuing to support both patients and their carers after discharge who are a significant distance from the transplant centre.
Mr Churi remained in hospital as an inpatient for 85 days following his lung transplant in both acute care and rehabilitation areas of the hospital. He was discharged to stay in local hospital-owned community accommodation to enable frequent outpatient reviews, initially weekly, then fortnightly, as required in the first few months following lung transplantation. When review frequency was decreased to monthly or less often, Mr Churi was reunited with his children in a home close to his supportive family network.
Conclusion
Based on the above case study and research presented, it can be appreciated that lung transplantation is a complex therapy. It can be a life-saving option for individuals diagnosed with a variety of progressive pulmonary diseases. Silicosis is a rare but viable indication for lung transplantation.
Lung transplantation has become increasingly successful over the last 30 years. However, the transplant recipient's pathway to recovery remains complex and is likely to involve clinical complications as well as social, physical and emotional challenges. Mr Churi's case illustrates these hardships. His journey to recovery involved overcoming significant clinical setbacks, including a readmission to ICU for respiratory failure.
The case study explores the personal challenges of finding the motivation to engage in physical therapy to regain independence and in learning new complex skills such as medication proficiency, with a fatigued body and mind. Importantly, this case study indicates the clear need to treat the patient as a holistic individual, with consideration of their physical, psychosocial and psychological influences.