The psychological impact of end-stage lung disease

STUDY OBJECTIVES: End-stage lung disease is associated with poor quality of life and increased risk for psychological distress. Despite the significant number of individuals with end-stage lung diseases, the emotional health of these patients, as compared with those with other chronic organ diseases, is not well-known. The purpose of this article is to elucidate personality styles and the presence of psychopathology in a clinical sample of patients with end-stage lung disease presenting for possible lung transplantation. DESIGN: Cross-sectional survey. SETTING: Two academic medical center transplant programs. PARTICIPANTS: Two hundred forty-three consecutively referred transplant candidates. RESULTS: Cluster analysis of the Minnesota Multiphasic Personality Inventory (MMPI)-2 indicated five different personality styles. The majority of patients evidenced mild somatic and depressive symptoms. Approximately one fourth of the sample exhibited marked anxiety and mood disturbances. A small cluster also evidenced features consistent with an antisocial personality style. CONCLUSIONS: Separate and distinct personality styles that could affect quality of life, the need for adjunct treatments, and medical compliance emerged from this sample of individuals with end-stage lung disease. Results are discussed in light of prior research on other end-stage organ conditions and in relation to personality and coping theories.     

Special considerations for patients with cystic fibrosis undergoing lung transplantation

This article reviews lung transplantation in patients with cystic fibrosis (CF). Lung transplantation is commonly utilized for patients with end-stage CF. There are several characteristics of CF that present unique challenges before and after lung transplantation. There is new information available that can be utilized to predict outcomes in patients with end-stage CF, and therefore can help in decisions of referral and listing for lung transplantation. The new lung allocation score, which allocates organs to patients who are on the lung transplant waiting list in the United States, presents new challenges and opportunities for patients with end-stage CF. In addition, the effect of the presence of microbiological flora prior to lung transplantation has been better linked to outcomes after lung transplantation. It is now known that, other than those patients harboring Burkholderia cepacia in their lungs before transplantation, most CF patients can undergo transplantation successfully. Nutrition remains an important issue among CF patients, and diabetes is a common problem after lung transplantation. In contrast, liver disease does not usually present major problems but, if it is severe, can necessitate liver and lung transplantation. Mechanical ventilation prior to transplantation might not be an absolute contraindication for CF patients. CF lung transplant recipients have good outcomes after lung transplantation compared with those of other lung transplant recipients. Quality of life is dramatically improved. However, they are still prone to common complications that all lung transplant recipients are prone to, including primary graft dysfunction, acute and chronic rejection, a variety of infections and malignancies, and renal failure.     

Current trends in candidate selection,contraindications, and indications for lung transplantation

Lung transplantation is an established treatment option that can improve quality of life and prolong survival for select patients diagnosed with end-stage lung disease. Given the gaps in organ donation and failures to make effective use of available organs, careful selection of candidates for lung transplant remains one of the most important considerations of the transplant community. Toward this end, we briefly reviewed recent trends in pretransplant evaluation, candidate selection, organ allocation, and organ preservation techniques. Since the latest consensus statement regarding appropriate selection of lung transplant candidates, many advances in the science and practice of lung transplantation have emerged and influenced our perspective of ‘contraindications’ to transplant. These advances have made it increasingly possible to pursue lung transplant in patients with risk factors for decreased survival—namely, older recipient age, increased body mass index, previous chest surgery, poorer nutritional status, and presence of chronic infection, cardiovascular disease, or extrapulmonary comorbid conditions. Therefore, we reviewed the updated evidence demonstrating the prognostic impact of these risk factors in lung transplant recipients. Lastly, we reviewed the salient evidence for current trends in disease-specific indications for lung transplantation, such as chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis, emphysema due to alpha-1 antitrypsin deficiency, and pulmonary arterial hypertension, among other less common end-stage diseases. Overall, lung transplant remains an exciting field with considerable hope for patients as they experience remarkable improvements in quality of life and survival in the modern era.     

Sublingual tacrolimus for immunosuppression in lung transplantation: a potentially important therapeutic option in cystic fibrosis

Lung transplantation has emerged as an option to prolong and increase the quality of life in patients with end-stage pulmonary lung disease. In lung transplant recipients, because of the high potential for acute and chronic allograft rejection, optimal selection, dosage and delivery of immunosuppressive medications is critical. Cystic fibrosis (CF), a multi-organ system disease that often includes pulmonary and gastrointestinal manifestations, represents the leading indication for bilateral lung transplantation. The gastrointestinal manifestations of CF, however, confound post-transplant management by causing significant variation in the rate and extent of absorption of orally administered immunosuppressive medications. Tacrolimus, a new calcineurin inhibitor, is increasingly employed as the primary immunosuppressive agent in lung transplant recipients. Unfortunately, tacrolimus itself exhibits variable bioavailability, particularly in CF transplant recipients. A novel approach to the absorption dilemma is administration of tacrolimus via the sublingual (SL) route. Little published information exists concerning the use of SL immunosuppression in transplant recipients. However, emerging evidence suggests that SL tacrolimus provides is an effective means of drug delivery particularly for CF lung transplant recipients. Preliminary results from a pilot study, demonstrate that SL delivery of tacrolimus achieves therapeutic serum levels, in lung transplant recipients with CF, over the first few postoperative months. In addition, the early postoperative use of SL tacrolimus has been associated with acceptable rates of transplant rejection and normal renal function in a cohort of 22 lung transplant recipients that included CF and non-CF transplant recipients. Potential pharmacokinetic advantages of the SL route of delivery include good permeability, rapid absorption, acceptable bioavailability and easy accessibility. From an economic standpoint, considerable cost savings could be achieved by using the SL rather than the intravenous route of drug delivery for tacrolimus. Comparative, prospective randomized trials are necessary to evaluate the long-term safety and efficacy of SL tacrolimus in lung transplant patients. Until such data are available, the use of SL tacrolimus should be considered in situations where alternative routes of delivery are unavailable or as part of ongoing research studies. Ultimately, SL tacrolimus may prove efficacious for short-term use in the early postoperative lung transplant recipients, particularly in patients with malabsorption problems such as CF transplant recipients.     

Psychological functioning of adults with cystic fibrosis

STUDY OBJECTIVES: The purpose of this study is to assess the psychological profiles of adult patients with cystic fibrosis (CF) and to investigate predictors of patients' psychological status. PATIENTS AND METHODS: Thirty-four adults with CF completed a battery of psychological testing including the Minnesota Multiphasic Personality Inventory-2, Beck Depression Inventory, and State-Trait Anxiety inventory. These were compared to health status data, including pulmonary function testing and nutritional status measures. RESULTS: As a group, adults with CF did not demonstrate significant levels of depression, anxiety, or other psychopathology. Results were not affected by age, sex, or severity of disease. Male gender predicted higher scores for depression and anxiety, and better lung functioning predicted less anxiety. Having a higher level of psychosocial support emerged as a strong predictor of better psychological functioning. CONCLUSIONS: Overall, adults with CF report relatively healthy psychological functioning. Better lung function and a strong social support system predicted better psychological functioning, which may have implications for clinical intervention.     

Lung transplantation in cystic fibrosis.

Lung transplantation has emerged in the last decade as a valid therapeutic endeavor for patients with end-stage lung disease. Although the presence of highly resistant organisms in the airway and sinuses of patients with cystic fibrosis (CF) increases the perioperative risk of infection following lung transplantation, transplant procedures can be undertaken in properly selected CF patients with acceptable morbidity and mortality at experienced centers. The introduction of a modification in the technique of double-lung transplantation has resulted in improved operative survival of CF patients undergoing lung transplantation. Whereas problems with posttransplant obliterative bronchiolitis, infection, and lymphoproliferative disorders contribute to morbidity, it is clear that lung transplantation can offer CF patients with end-stage lung disease improvement in both the quality and quantity of life. The most serious impediment to more widespread application of this therapy in CF is the inadequate number of donor organs.     

Aerosolized vancomycin for the treatment of MRSA after lung transplantation

Lung transplantation is the only life-prolonging therapy available for cystic fibrosis (CF) patients with end-stage lung disease. The presence of pathogens in the airways of CF patients prior to transplantation is the major risk factor for infection in the post-transplantation period, with methicillin-resistant Staphylococcus aureus (MRSA) having a growing impact. Aerosolized vancomycin has been used successfully in the treatment of MRSA in the CF population but its use after lung transplantation has not been previously reported. We report the case of a lung transplant recipient who was successfully treated for MRSA infection with aerosolized vancomycin.     

Sublingual Tacrolimus for Immunosuppression in Lung Transplantation

Lung transplantation has emerged as an option to prolong and increase the quality of life in patients with end-stage pulmonary lung disease. In lung transplant recipients, because of the high potential for acute and chronic allograft rejection, optimal selection, dosage and delivery of immunosuppressive medications is critical. Cystic fibrosis (CF), a multi-organ system disease that often includes pulmonary and gastrointestinal manifestations, represents the leading indication for bilateral lung transplantation. The gastrointestinal manifestations of CF, however, confound post-transplant management by causing significant variation in the rate and extent of absorption of orally administered immunosuppressive medications.Tacrolimus, a new calcineurin inhibitor, is increasingly employed as the primary immunosuppressive agent in lung transplant recipients. Unfortunately, tacrolimus itself exhibits variable bioavailability, particularly in CF transplant recipients. A novel approach to the absorption dilemma is administration of tacrolimus via the sublingual (SL) route. Little published information exists concerning the use of SL immunosuppression in transplant recipients. However, emerging evidence suggests that SL tacrolimus provides is an effective means of drug delivery particularly for CF lung transplant recipients.Preliminary results from a pilot study, demonstrate that SL delivery of tacrolimus achieves therapeutic serum levels, in lung transplant recipients with CF, over the first few postoperative months. In addition, the early postoperative use of SL tacrolimus has been associated with acceptable rates of transplant rejection and normal renal function in a cohort of 22 lung transplant recipients that included CF and non-CF transplant recipients. Potential pharmacokinetic advantages of the SL route of delivery include good permeability, rapid absorption, acceptable bioavailability and easy accessibility. From an economic standpoint, considerable cost savings could be achieved by using the SL rather than the intravenous route of drug delivery for tacrolimus.Comparative, prospective randomized trials are necessary to evaluate the long-term safety and efficacy of SL tacrolimus in lung transplant patients. Until such data are available, the use of SL tacrolimus should be considered in situations where alternative routes of delivery are unavailable or as part of ongoing research studies. Ultimately, SL tacrolimus may prove efficacious for short-term use in the early postoperative lung transplant recipients, particularly in patients with malabsorption problems such as CF transplant recipients.     

Long-term outcome of lung transplantation

Lung transplantation (LTx) nowadays is an established therapeutic option for various end-stage lung diseases in despite of an optimal medical therapy in selected patients affected with various pathologies such as emphysema/chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF) and various forms of bronchiectasis, pulmonary fibrosis and pulmonary hypertension. Progress and refinement over the last two decades in both surgical techniques and medical management including especially the introduction of cyclosporine A, have been impressive. Patient survival improved significantly over time. According to the most recent data of the International Registry of Lung Transplantation (ISHLT), actuarial survival is 79%, 63%, 52% and 29% at 1, 3, 5 and 10 years respectively, with a current overall survival half-life of 5.3 years, and more than 7 years for those who survived 1 year or more. However, survival depends on different parameters such as the underlying disease, data relative to the donor, the recipient and/or the transplant procedure. Major limitation of long-term survival is still chronic allograft dysfunction, which is histologically represented by an obliterative bronchiolitis and functionally by the bronchiolitis obliterans syndrome (BOS). In uncomplicated cases near-normalization of pulmonary function is the rule, with more than 80% of survivors at 1, 3, 5 and 10 years reporting no activity limitations. Functional results after single LTx remain lower than those of the bilateral LTx, and depend on the potential worsening of the underlying disease and complications arising on the native lung. Exercise performance usually is approximately 50% of predicted, suggesting extrapulmonary causes such as side effects of immunosuppressive drugs. Quality of life improves significantly in all domains including employment status.     

Liver transplantation in cystic fibrosis.

Liver disease is the second most common cause of death in patients with cystic fibrosis (CF). Improvement in surgical techniques, medical management, and imaging modalities has broadened the range of options for treatment of these patients. Medical management with ursodeoxycholic acid and nutritional support may help decelerate the progression of liver disease. A timely evaluation of CF patients with liver involvement for transplantation is important. Such evaluation should not be delayed until signs of hepatic decompensation occur. Combined lung-liver transplant can be considered for patients with advanced pulmonary disease. Pretransplant management of portal hypertension with a portosystemic shunt procedure is an option for patients with well-preserved synthetic liver function. Improvement in lung function after liver transplantation and no significant risk of pulmonary infection with immunosuppressive therapy have been reported. Review of individual center experiences have shown satisfactory survival and improved quality of life for CF patients undergoing liver transplant.     

Double-lung transplant for advanced chronic obstructive lung disease

We have achieved repeated success with unilateral lung transplantation for pulmonary fibrosis and have developed an en bloc, double-lung transplant procedure for patients with advanced lung disease of an obstructive or infective nature. Six such procedures have now been performed for end-stage emphysema, and all recipients are alive and well 5 to 15 months later. A seventh transplant for primary pulmonary hypertension was unsuccessful. All recipients were judged to have a life expectancy of 12 to 18 months on the basis of the degree of disability and the documented rate of disease progression. We feel the double-lung procedure is more appropriate than the combined heart-lung transplant for patients requiring replacement of both lungs when right heart function is adequate or deemed recoverable. With this procedure, the recipient is able to retain his or her own heart, avoiding the liabilities associated with cardiac transplantation. Furthermore, the donor heart is available for a separate recipient, and this sharing of the heart and lungs greatly increases the supply of transplantable lungs for patients with end-stage lung disease. Ischemia of the donor airway has been a source of complication, including the one death to date, but this appears to be a surmountable problem.     

Quality of life and health status in interstitial lung diseases

PURPOSE OF REVIEW: The aim of this review is to provide information on the influence of interstitial lung disease on patients' health status and quality of life, with an emphasis on which aspects might be affected by interstitial lung disease. RECENT FINDINGS: The quality of life of sarcoidosis patients with current symptoms and patients with idiopathic pulmonary fibrosis is mainly impaired in the domains of physical health and level of independence. Concerning health status, sarcoidosis patients report reduced physical functioning, especially when they have current symptoms. The health status of idiopathic pulmonary fibrosis patients is impaired in almost all aspects. Subjectively assessed dyspnea is related to health status. A relationship between treatment and health status has not been found. SUMMARY: Results from the existing studies show that both illnesses have a substantial impact on patients' life. Studies aiming at measuring quality of life or health status in other interstitial lung diseases are scarce or nonexistent.     

Impact of pulmonary rehabilitation on quality of life and functional capacity in patients on waiting lists for lung transplantation

OBJECTIVE:

To investigate the impact of a pulmonary rehabilitation program on the functional capacity and on the quality of life of patients on waiting lists for lung transplantation.

METHODS:

Patients on lung transplant waiting lists were referred to a pulmonary rehabilitation program consisting of 36 sessions. Before and after the program, participating patients were evaluated with the six-minute walk test and the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). The pulmonary rehabilitation program involved muscle strengthening exercises, aerobic training, clinical evaluation, psychiatric evaluation, nutritional counseling, social assistance, and educational lectures.

RESULTS:

Of the 112 patients initially referred to the program, 58 completed it. The mean age of the participants was 46 ± 14 years, and females accounted for 52%. Of those 58 patients, 37 (47%) had pulmonary fibrosis, 13 (22%) had pulmonary emphysema, and 18 (31%) had other types of advanced lung disease. The six-minute walk distance was significantly greater after the program than before (439 ± 114 m vs. 367 ± 136 m, p = 0.001), the mean increase being 72 m. There were significant point increases in the scores on the following SF-36 domains: physical functioning, up 22 (p = 0.001), role-physical, up 10 (p = 0.045); vitality, up 10 (p < 0.001); social functioning, up 15 (p = 0.001); and mental health, up 8 (p = 0.001).

CONCLUSIONS:

Pulmonary rehabilitation had a positive impact on exercise capacity and quality of life in patients on lung transplant waiting lists.     

Quality of life in elderly patients with chronic nonspecific lung disease seen in family practice

We studied the quality of life of elderly patients with chronic nonspecific lung disease (CNSLD) in family practice. We also investigated the relationship between patients' somatic condition and their quality of life. Seventy patients, aged 40 years or older, with a diagnosis or symptoms of CNSLD completed the Sickness Impact Profile (SIP) and the list of daily activities (DAL). Pulmonary function (FEV1, IVC) and respiratory symptoms were assessed. The results indicated that patients were more impaired in their physical and psychosocial functioning than healthy control subjects. Most lung function parameters showed no correlation with the SIP scores. The respiratory symptoms of wheezing and dyspnea were related to patients' quality of life. Patients with chronic obstructive pulmonary disease (COPD) were more restricted in their daily functioning than patients with asthma. Since the relationship between patients' somatic condition and their quality of life is weak, we recommend comprehensive care that encompasses psychosocial as well as somatic interventions.     

Psychosocial issues in the assessment and management of patients undergoing lung transplantation

This review examines psychosocial issues among lung transplant patients from the time of assessment through the posttransplant period. Although psychological factors are recognized as being important in the transplant evaluation, no standard approach to psychological assessment currently exists. Lung transplant candidates often experience high levels of psychological distress while awaiting transplant, and both pretransplant and posttransplant psychological functioning have been found to predict posttransplant quality of life, adherence to treatment, and, in some cases, medical outcomes. Given the limited long-term survival following transplantation, improving psychosocial functioning is essential for enhancing outcomes among lung transplant recipients. This review summarizes the extant literature on the psychosocial factors in lung transplantation and highlights several innovative efforts to improve psychological outcomes in this challenging patient population.     

Health-related quality of life and psychological correlates in patients listed for liver transplantation

Purpose Given the increasing waiting time for liver transplantation, and the amount of possible stressors associated with it, assessment of psychological well-being and health-related quality of life (HRQoL) in these patients is warranted in order to optimize pretransplant care. Patients and methods Patients with chronic liver disease (n = 32) awaiting transplantation completed a series of questionnaires measuring HRQoL, depression, anxiety, coping, and self-efficacy. Comparisons were made with other patients with liver disease with and without cirrhosis, and a healthy norm population. Relationships among these psychological variables were explored and subgroup analyses were performed to assess possible differences in coping strategies. Results Compared to other patients with liver disease without cirrhosis, liver transplant candidates had statistically significantly lower HRQoL scores on the subscales of physical functioning (P < 0.001) and general health (P < 0.001). Their HRQoL did not differ from patients with liver disease with cirrhosis. Overall, patients awaiting liver transplantation had significantly reduced HRQoL (P < 0.001) and increased depression scores (P < 0.001) compared to healthy controls. Levels of depression, anxiety, self-efficacy, and coping did not differ between liver transplant candidates and other patients with liver disease. Depression correlated significantly with HRQoL. Patients without depression made significantly more use of active coping strategies than patients with elevated depression levels. Conclusions Patients awaiting liver transplantation are not experiencing worse physical and psychological HRQoL than other liver patients with cirrhosis of the liver. Therefore, there is currently no indication to increase the level of psychosocial care for liver transplant candidates.     

Rehabilitation and palliative care in lung fibrosis

The idiopathic interstitial pneumonias are a heterogeneous group of diffuse parenchymal lung diseases with varying degrees of inflammation and fibrosis, like interstitial pulmonary fibrosis. Functional exercise tolerance and quality of life have been shown to be significantly affected in patients with lung fibrosis. Moreover, interstitial pulmonary fibrosis is a progressive disease with poor prognosis and limited response to conventional pharmacological treatment like immunosuppressive agents. So, in patients with lung fibrosis there seems a clear indication to refer them for comprehensive pulmonary rehabilitation programmes and to initiate palliative care in an early phase of the disease. In the current review we will present a rationale for pulmonary rehabilitation in patients with lung fibrosis and the effects of this type of non-pharmacological intervention on exercise capacity and quality of life. In addition, we will discuss possibilities for palliative care in these patients.     

Effects of a telephone-based psychosocial intervention for patients awaiting lung transplantation

STUDY OBJECTIVES: To test the efficacy of a tailored telephone-based intervention consisting of supportive counseling and cognitive behavioral techniques for individuals awaiting lung transplantation on measures of quality of life and general well-being. METHOD: Patients were randomly assigned to either a telephone-based special intervention (SI; n = 36) for 8 weeks (average session length, 16.3 min) or a usual care (UC) control condition (n = 35) in which subjects received usual medical care but no special treatment or phone calls. At baseline, and immediately following the 8-week intervention, patients completed a psychometric test battery. SETTING: Duke University Medical Center, Pulmonary Transplantation Program. PATIENTS: Seventy-one patients with end-stage pulmonary disease listed for lung transplantation. Primary outcome measures: Measures of health-related quality of life (both general and disease-specific), general psychological well-being, and social support. RESULTS: Multivariate analysis of covariance, adjusting for pretreatment baseline scores, age, gender, and time waiting on the transplant list, revealed that patients in the SI condition compared to the UC reported greater general well-being (p < 0.05), better general quality of life (p < 0.01), better disease-specific quality of life (p < 0.05), and higher levels of social support (p < 0.0001). CONCLUSION: A brief, relatively inexpensive, telephone-based psychosocial intervention is an effective method for reducing distress and increasing health-related quality of life in patients awaiting lung transplantation.     

Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease

Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy.     

Psychiatric disorder and quality of life in patients awaiting lung transplantation

STUDY OBJECTIVE: To examine the relationship between psychiatric comorbidity and quality of life in patients awaiting lung transplantation. SETTING: Duke University Medical Center/Lung Transplantation Program. PARTICIPANTS: One hundred patients with end-stage pulmonary disease listed for lung transplantation. MEASUREMENTS AND RESULTS: Twenty-five percent (n = 25) of the sample met diagnostic criteria for at least one current mood or anxiety disorder. Controlling for age, gender, ethnicity, percentage of predicted FEV, and lung disease diagnosis, patients with a current psychiatric diagnosis reported poorer general quality of life (p < 0.0001), poorer disease-specific quality of life (p < 0.0001), greater shortness of breath (p = 0.01), more symptoms of psychological distress (p < 0.0001), lower levels of social support (p < 0.0001), and fewer positive health habits (p < 0.04) than their counterparts without a psychiatric diagnosis. CONCLUSIONS: Psychiatric comorbidity affects a significant portion of patients awaiting lung transplantation and is associated with decreased health-related quality of life.