Table 2.
CFF recommendations for the care of PwCF after lung transplantation.
| Category | Recommendation | % Vote |
|---|---|---|
| General Care | CF Lung Transplant Recipients should follow up with a multidisciplinary CF care team within 6–12 months of transplant to resume extra-pulmonary CF care. Communication between the transplant and CF care teams is essential for coordination of care | 100% |
| General Care | CF and Transplant programs should operationalize infection prevention and control policies across all services as indicated by the CF Foundation’s Infection Prevention and Control Guidelines | 95% |
| Infectious disease | Non–invasive CF-specific bacterial, fungal, and AFB respiratory cultures should be obtained by the transplant or CF center every 3 months in actively waitlisted transplant candidates, and clinicians should review prior pathogen history to guide the peri-operative antibiotic regimen | 100% |
| Infectious disease | An intraoperative CF bacterial, fungal and AFB culture of the native lung should be obtained at the time of lung transplantation | 100% |
| Infectious disease | In CF Lung Transplant Recipients with multidrug resistant pathogens, susceptibility-driven antimicrobials should be administered when the recipient has a susceptible antibiotic choice with acceptable toxicity. In the absence of a susceptibility-driven perioperative choice, previously effective regimens should be considered | 100% |
| Infectious disease | For CF Lung Transplant Recipients, there are insufficient evidence to recommend for or against routine intraoperative pleural and tracheal irrigation with antimicrobial agents to decrease infections after transplant | 100% |
| Infectious disease | Perioperative and/or early posttransplant inhaled antibiotics for bacterial pathogens isolated prior to transplant should be considered as a complement to systemic antimicrobials in CF Lung Transplant Recipients | 100% |
| Infectious disease | There is insufficient evidence to recommend for or against the use of inhaled antibiotics for prevention of recolonization or chronic lung allograft dysfunction (CLAD) | 100% |
| Infectious disease | There is insufficient evidence to recommend for or against the routine collection of sputum for bacterial, fungal or AFB cultures in asymptomatic CF Lung Transplant Recipients | 95% |
| Sinus Disease | In individuals with CF and asymptomatic chronic rhinosinusitis (CRS), the CF Foundation recommends against pre-transplant prophylactic sinus surgery for the prevention of lung graft colonization. | 100% |
| Sinus Disease | CF Lung Transplant Recipients should be screened for symptoms of CRS at least annually | 100% |
| Sinus Disease | CF Lung Transplant Recipients with moderate or severe symptomatic CRS should be seen in consultation with an otolaryngologist experienced in CF for consideration of optimal topical therapies and endoscopic sinus surgery | 100% |
| Nutrition and GI Complications |
CF Lung Transplant Recipients should receive ongoing consultation with a dietitian with CF expertise in order to obtain individualized nutritional therapy to achieve an established BMI or weight-for-length goal | 100% |
| Nutrition and GI Complications |
In CF Lung Transplant Recipients, vitamin D supplementation should be continued, but a combination of vitamin A,D,E,K supplements should be discontinued after lung transplantation; fat soluble vitamin levels should be measured by 3 months after transplant, and levels should be repleted and followed individually as needed | 100% |
| Nutrition and GI Complications |
Symptoms should be assessed daily in hospitalized patients, particularly within the immediate post-operative period and with any opiate medication administration, for early signs of obstipation and obstruction that might herald emergence of distal intestinal obstruction syndrome (DIOS) | 100% |
| Nutrition and GI Complications |
In CF Lung Transplant Recipients who develop DIOS, early enteral lavage should be considered. Refractory DIOS should be managed in coordination with experts in CF gastrointestinal complications to reduce risk for prolonged obstruction and potential need for operative management | 100% |
| Nutrition and GI Complications |
For CF Lung Transplant Recipients who experience new or worsening symptoms of gastrointestinal dysmotility, a gastroenterologist and a dietitian with CF expertise should be consulted to guide the approach to symptom control and potential interventions | 100% |
| Nutrition and GI Complications |
CF Lung Transplant Recipients should undergo liver enzyme monitoring for CF Liver Disease (CFLD) at least annually, and when levels are elevated, patients should receive non-invasive imaging techniques for initial evaluation | |
| Diabetes and Bone Health |
CF Lung Transplant Recipients who do not have Cystic Fibrosis-Related Diabetes (CFRD) should be screened with an oral glucose tolerance test (OGTT) at 3–6 months after transplant and then annually–following the recommended screening guidelines for CFRD | 95% |
| Diabetes and Bone Health |
CF Lung Transplant Recipients who have CFRD should be treated with insulin and should undergo intensive self-blood glucose monitoring (SBGM) and individualized close clinical follow-up in addition to lifestyle modifications. Furthermore, an endocrinologist with expertise in CF and transplant associated DM should be consulted when possible | |
| Diabetes and Bone Health |
For CF Lung Transplant Recipients, bone density should be assessed with dual energy X-ray absorptiometry (DEXA) at 6–12 months after transplant | 100% |
| Mental Health and Family Planning |
CF Lung Transplant Recipients should have mental health screening and consultation for depression, anxiety, and post-traumatic stress disorder (PTSD) within 6 months of transplant and then should resume annual screening per the International Committee on Mental Health (ICMH) Depression and Anxiety Guidelines | 100% |
| Mental Health and Family Planning |
Caregivers of CF Lung Transplant Recipients should be screened for depression, anxiety, and PTSD within 6 months of transplant and should be referred for further assessment if necessary | 90% |
| Mental Health and Family Planning |
Females with CF who are post-lung transplant and are considering pregnancy should assess carefully their individual risks through shared decision making with maternal fetal medicine and transplant providers | 100% |
| Mental Health and Family Planning |
Females with CF who are post-lung transplant should avoid pregnancy for at least the first 2 years after transplantation because of the increased risk of acute rejection, accelerated chronic rejection, and death | 100% |
| Pharmacology and Therapeutics |
There is insufficient evidence to recommend for or against the use of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators for CF Lung Transplant Recipients | 100% |
| Pharmacology and Therapeutics |
There is insufficient evidence to recommend for or against the use of induction immunosuppression for CF Lung Transplant Recipients | 100% |
| Pharmacology and Therapeutics |
CF Lung Transplant Recipients should have close monitoring of calcineurin inhibitor drug levels because of altered pharmacokinetics | 100% |
| Pharmacology and Therapeutics |
Reduced renal function is common in CF Lung Transplant Recipients, and serum creatinine is often a poor surrogate for renal function. Therefore, medications should be dosed carefully according to the estimated glomerular filtration rate (GFR) of the patient, and when available, therapeutic drug monitoring should be implemented | 100% |
| Pharmacology and Therapeutics |
There is insufficient evidence to recommend for or against the routine use of airway clearance, dornase alfa, or hypertonic saline among CF Lung Transplant Recipients | 100% |
Note. Reprinted from Table 1 in [34]. Creative Commons user license link: https://creativecommons.org/licenses/by/4.0/ accessed on 10 April 2023.