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<st1:date Year="2004" Day="15" Month="1">


CF and Lung Transplant - 2

Early Bronchoscopy Justified in Cystic Fibrosis Lung Transplant Patients - The risk of tracheobronchial aspergilosis for cystic fibrosis patients receiving lung transplantation justifies early surveillance bronchoscopy to detect the infection, especially in recipients with pre- transplant colonization. It was found that 53%(17/32) of the cystic fibrosis recipients had Aspergillus fumigatus isolated from their respiratory secretions before undergoing transplantation. In 59% of the recipients with Aspergillus (10/17), the A. fumigatus was persistently found in respiratory secretions after transplantation compared with 40% (6/15) cystic-fibrosis patients who have not been colonized pre-transplant and 28% (28/101) of the non-cystic-fibrosis recipients. (Source: Chest, March 21, 2003)


Pre- transplant mechanical ventilation and outcome in patients with cystic fibrosis - Mechanical ventilation for ventilatory failure has been considered a relative contraindication to subsequent lung transplantation. The purpose of this study was to test the hypothesis that patients with cystic fibrosis who are intubatted and mechanically ventilated before transplantation have poorer post-transplant outcomes than do patients who are not ventilated. We compared the outcomes of 8 patients with CF who underwent mechanical ventilation for  3-153 days before bilateral lung transplantation with outcomes of 24 patients with CF who did not undergo pre- transplant mechanical ventilation. Although time to extubation after transplantation was prolonged significantly ( 11 vs 4 days) for the pre transplant ventilated group, days to hospital discharge, forced expiratory volume in 1 second ( percent predicted) at 1 year after transplantation, and post transplant survival as determined using the Kaplan-Meir method did not differ statistically between the 2 groups.(Source: The Journal of Heart and Lung Transplantation, April 2003)
Update on Cystic Fibrosis: Selected aspects related to lung transplantation  Survival after lung transplantation for cystic fibrosis has improved substantially. To date, 1-year survival is as much as 80-90% and 5-year survival 60-70%. Experience of surgical techniques, periand postoperative management and long term follow-up care has grown.  High risk patients for whom transplantation was contraindicated ten years ago are today being transplanted.  In the early era of lung transplantation, mortality due to bacterial infections were high and so at that time many centrs excluded patients with multiresistant organisms from their waiting lists.  B. cepacia - One of the first reports of B. cepacia-positive patients undergoing lung transplantation came from Toronto.  Of 22 CF patients transplanted between 1988 and 1991, 15 cultured positive for B. cepacia and 7 of these died.  In contrast, only 5 of 27 patients transplanted between 1990-1993 at the University of North Carolina were B. cepacia positive, and only one died.  Post operative B.cepacia-associated complications included pneumonia, empyema, subdural empyema, and bronchiolitis obliterans due to B. cepacia.  Nutrition - It has been shown that patients with a low body mass index are at increased risk of death on the waiting list. Every effort should therefore be made to prevent further weight loss when patients are listed for transplant. Osteoporosis - The prevalence of osteoporosis in cystic fibrosis  is higher than in the general population. The risk factors include malabsorption of vitamin D and calcium deficiency, hypogonadism, lowbody weight and corticosteroid therapy. After transplantation, bone loss is increased due to immunosuppressive therapy with calcineurin inhibitors and corticosteroids.  Patients considered for transplant should undergo aggressive therapy with calcium and vitamin D supplements as well as bisphosphonates, to prevent further bone loss both before and after transplantation. Pleural adhesions - Due to repeated infection, pleurodesis for pneumothorax or other thoracic procedures are often seen in patients with CF. They are not considered a contraindication to transplant. Psychosocial issues - If the lung transplant recipient's environment is dysfunctional with lack of dedicated support personnel, transplantation is less likely to be successful. In adolescent transplant recipients in  particular, care should be taken to ensure a positive outlook on life with strong emphasis on sought-after goals as well as interpersonal relationships. At the time of referral, close attention should be focused on the psychosocial situation and if necessary, intensive psychosocial counseling and therapy should be instituted.(Source: Swiss Medical Weekly, 2003
April 18, 2004 -- Improved quality of life after lung transplantation in individuals with cystic fibrosis.-- The aim of the study was to assess the effect of lung transplantation on health related quality of life in a group of patients with CF, compared to patients with other diagnoses.  Patients in the CF group were younger, spent more days on the waiting list, and were more likely to be working or going go school than patients with other indications. Significantly better scores in the CF group compared to non-CF group were found in the dimension of mobility. Scores remained more or less stable over time in both groups. It may be concluded that patients in both groups experience major restrictions in HRQL before transplantation.  However, pretransplant non- CF patients experience more restrictions than CF patients. After lung transplant, both groups of patients showed substantial improvement in HRQL, and this improvement was maintained until 31 months after lung transplant. ( Source: Pediatric Pulmonology, 2004)
Effects of sinus surgery in patients with cystic fibrosis after lung transplantation: a 10-year experience  (ADDED 9/22/04)
Chronic infectious rhinosinusitis with Pseudomonas aeruginosa is common in cystic fibrosis and may result in allograft infection after lung transplantation. Sinus surgery followed by nasal care may reduce these adverse effects. Sinus surgery was performed in 37 patients with cystic fibrosis after transplantation.  Sinus surgery was successful in 54% and partially successful in 27% of patients. Successful sinus management led to a lower incidence of tracheobronchitis and pneumonia (P =0.009) and a trend toward a lower incidence of bronchiolitis obliterans syndrome (P =0.23). Sinus surgery followed by daily nasal douching may control posttransplant lower airway  colonization and infection. In the long term, this concept may lead to less bronchiolitis obliterans syndrome by decreasing bronchiolar inflammation.Colonization of both the upper and lower airways with Pseudomonas aeruginosa is a well-known phenomenon in patients with cystic fibrosis (CF) (1). Patients with CF have a significantly higher risk of acquiring and harboring P. aeruginosa in the lung allograft compared with transplant recipients with other underlying diseases (2). P. aeruginosa-induced inflammation may be associated with the occurrence of posttransplant bronchiolitis obliterans syndrome (BOS), leading to progressive, irreversible graft dysfunction and death. Rigorous treatment for chronic infectious rhinosinusitis may be essential to reduce negative impact on the transplanted lung.  Surgery consisted of an endoscopic fronto-spheno-ethmoidectomy in which all sinuses and aired cells were largely opened. On the second day of surgery, daily nasal douching with isotonic saline solution was started. Success of sinus surgery and postoperative control of the infection of the sinuses were defined as the presence of three or less sinus aspirates with significant bacterial growth as previously defined. Partial success was defined as the presence of four to five sinus aspirates with significant bacterial growth and minimal intervals of 6 weeks during which no bacteria could be cultured. Failure was defined as the presence of more than five sinus aspirates with significant bacterial growth and intervals of less than 6 weeks with no growth. Our study shows that surgery and postoperative management of the infection of the paranasal sinuses were successful or partially successful in more than 80% of lung transplant recipients with CF. The remaining patients developed persistent colonization of their sinuses, mostly by P. aeruginosa, showing a trend toward a higher incidence of episodes with detectable bacterial strains in BAL during their postoperative course.(Source: Transplantation:  15 January 2004)
</st1:date>
Study finds lung transplants increase survival of cystic fibrosis patients by more than four years - (Added 10/4/04) -Lung transplantation increases the survival of patients with cystic fibrosis by almost 4.5 years on average, according to a study presented at the American Thoracic Society International Conference in on May 24. "Initially the risks from lung transplantation for cystic fibrosis patients are quite high, but the risk drops over time, and it becomes worth the risk in the long run," said study co-author Roger D. Yusen, MD, MPH, Assistant Professor of Medicine in the Divisions of Pulmonary and Critical Care Medicine and General Medical Sciences at Washington University School of Medicine in St. Louis. Lung transplants are not common--approximately 1,000 are performed each year in the and only 150 of them, or 15%, are performed on CF patients.  The study included 247 patients, who were followed through 2002. Most were Caucasian, reflecting the CF population as a whole, and the average age at the time of placement on the waiting list was 28. All the patients had severely impaired lung function and severe symptoms when they were placed on the waiting list. Of the 247 patients in the study, after one year, 84% of patients were alive while waiting for a transplant, and an equal percentage were alive after having a transplant. After two years, 67% of those waiting for a transplant were alive, compared with 76% who had a transplant. After five years, 55% of transplant patients were alive. Though transplantation had a significant early death risk compared with continued waiting, the risks significantly decreased over time. Within 15 months, the survival benefit from transplantation became apparent. Based on an average survival of 5.7 years after transplant, the researchers determined that lung transplantation improved the life expectancy of patients with CF by almost 4.5 years. "Next, we hope to see if transplanted patients are not only living longer, but enjoying a better quality of life than those who remain on the waiting list," Dr. Yusen said. (Source: American Thoracic Society 2004)
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