Models of Post-Transplant Care for Individuals With Cystic Fibrosis

There is no consensus on the best model of care for individuals with CF to manage the non-pulmonary complications that persist after lung transplant. This position paper outlines two models of post-transplant care to accomplish optimal CF care after transplant.

10 min read

Summary

This position paper outlines two models of post-transplant care to accomplish optimal CF care after transplant.
The first model incorporates the CF team into care and proposes delineation of responsibilities for the CF and transplant teams.
The second model consolidates care in one center and may be more practical for transplant programs that have expertise managing CF and have access to CF multidisciplinary care team members (e.g., located in the same institution).

McKone E, Ramos KJ, Chaparo C, et al. Position paper: Models of post-transplant care for individuals with cystic fibrosis. J Cyst Fibros. 2023 May;22(3):374-380. doi:10.1016/j.jcf.2023.02.011. Epub 2023 Mar 5.

Purpose and Background

Clinical guidance has not previously existed to describe or guide the best models of care for people with cystic fibrosis following lung transplant. Although transplanted lungs have functional CFTR and CFTR protein, CF is a multisystem genetic disorder and integrated post-transplant care is necessary. This focus of care is on the lung transplant allograft and the non-pulmonary manifestations of CF. These include:

Nutrition
Malabsorption and obesity
Vitamin replacement
Distal intestinal obstructive syndrome (DIOS)
CF-related sinus disease
CF-related diabetes
Bone health
Mental health
Cancer screening

There is no standardized method of post-transplant CF care, and the workgroup acknowledges that situations may differ depending on the availability of transplant and CF care expertise, patient preference, and center experience. The purpose of the position paper is to present two models of care that may be used by CF and lung transplant centers that share or coordinate care of lung transplant recipients with CF.

Methodology

The Cystic Fibrosis Foundation convened a workgroup comprised of international experts in CF and lung transplant care and people with CF who had undergone lung transplantation. The group conducted a focused literature search and discussed models of care across centers. A survey was developed and distributed internationally to the CF and lung transplant community. Data derived from these approaches informed the development of this position paper.

Key Points

1. Multidisciplinary CF care after lung transplant is required to manage multisystem disease.

2. Prior to transplant, the CF and transplant teams should educate the individual with CF and their support personnel about prevention, early identification, and treatment of common CF-related complications that can occur around the time of transplant:

3. There are two models of post-transplant care that can be utilized:

Model 1 — Fully integrated shared care model between the transplant center and the referring CF center
Model 2 — Transplant team manages and coordinates all aspects of transplant and CF care

4. The lung transplant team should manage the pulmonary complications of lung transplant indefinitely, regardless of the model of care utilized.

5. Timely and clear communication between the transplant team and the referring CF center is essential. Each transplant recipient should have a named primary CF or transplant physician to lead coordination of post-transplant care management.

6. The preferred model of care for each patient is determined by the CF and transplant centers in conjunction with the individual with CF. Hybrid models with components of each model may best serve some patients.

7. Factors influencing the care model selection process:

CF expertise at transplant center
Distance to transplant and CF centers
Insurance and patient financial resources
Physician and patient preference
Access to specialists with transplant and CF experience (e.g., nephrology, infectious disease, endocrine, ENT, GI)
Patient’s social support network

8. Crucial to either care model is the relationship and the trust that the CF transplant recipient develops with each team member:

Effective, streamlined communication between the referring team, the transplant team, and the transplant recipient leads to optimal, individualized care and enhances shared decision-making. Formal communication between teams is continued indefinitely after transplant for routine elements of care, such as social work, endocrinology, GI, and other subspecialty care.

9. Key themes identified by patients and families that optimize the lung transplant journey for CF patients across the continuum are:

Establishment of relationships between CF and transplant care providers
Development and exchange of discipline-specific education tools between CF and transplant teams
Inclusion of individuals with CF and their support persons in both CF and transplant quality improvement teams

10. Best practices identified include:

Regularly scheduled meetings between referring CF and transplant programs
Virtual, monthly educational teaching sessions for CF and transplant teams
- For example: Each month a different topic is selected and is presented by an “expert” in the field (CFRD, GI/nutrition, chronic rhinosinusitis, optimizing medications in the post-transplant CF patient)
A post-transplant handoff sheet
Create and provide a contact list of transplant care members to the CF care team and vice versa
Co-management of CF transplant recipient documentation describing what tests and support are required after transplant and which team is responsible

11. Regardless of the model chosen, roles and responsibilities should be clearly delineated, ensuring effective communication between team members and the lung transplant recipient.

12. Pediatric lung transplant can present additional challenges:

The number of pediatric lung transplant centers is small, so the CF referring center and pediatric lung transplant center may be geographically distant. Ongoing communication between the CF center and the lung transplant center is vital for the success of the transplant.
Adolescents with cystic fibrosis requiring transplant may be referred to adult lung transplant centers. However, outcomes for children requiring a lung transplant are better when they are transplanted at a pediatric center, and that should be the first choice when possible.

13. Maintaining knowledge of CF and transplant is critical to the success of both models of post- transplant care.

Continuing medical education with extensive and up-to-date information on CF and lung transplant in CF is available through a partnership with the Cystic Fibrosis Foundation and the Indiana University Division of Continuing Medical Education.
Guidelines for post-transplant care for individuals with cystic fibrosis were developed by a multidisciplinary working group sponsored by the Cystic Fibrosis Foundation.
The North American Cystic Fibrosis Conference and European Cystic Fibrosis Society Annual meetings provide excellent education about state-of-the-art care for patients with cystic fibrosis pre- and post-lung transplant.

Summary of Models
Model 1: Shared model	Model 2: Transplant team manages and coordinates all care
Transplant program provides care for all transplant and pulmonary related issues, including pulmonary complications and complications related to immunosuppression.	Transplant program provides care for all issues, engaging subspecialty and primary care providers as needed.
Transplant program provides care for all transplant and pulmonary related issues including pulmonary complications and complications related to immunosuppression. CF program provides care for all non-pulmonary CF-related issues.

	Pros	Cons
1. Shared model	Facilitation of shared knowledge between transplant and CF teams Reduced travel for individuals with CF living a distance from transplant center for CF-related complications For patients living a distance from transplant center with CF center but with a CF physician who has interest and experience in transplant medicine, potential to attend their local CF center and receive care for transplant-related complications in close liaison with their transplant center Continuity of care in CF center for non-pulmonary complications of CF such, as diabetes, sinus disease, and GI/liver disease Extensive education about CF and lung transplant complications and treatment is not required for all members of both teams Lung transplant team knows how to contact CF team and vice versa	More than one team managing the patient with the potential for poor communication could lead to management errors Potential need to attend more than one hospital with different physicians involved at different hospitals Unclear delineation of responsibilities could lead to overlap between transplant and non-transplant related complications of cystic fibrosis
2. Transplant team manages and coordinates all care	Continuity of care by single transplant multidisciplinary team Single point of care is convenient for individuals with CF and their families Potential to reduce cost for CF patient Single point of care facilitates communication and reduced clinic visits Efficient for medical records	Limited number of transplant centers with CF expertise prevent this from becoming a universally viable model Requires engaged local provider if transplant recipient lives a distance from transplant center. Potential inexperience of transplant center in managing nontransplant-related complications of cystic fibrosis, including the use of CFTR modulators

Unanswered Questions:

Can we effectively use data from the CF Foundation Patient Registry to determine which model leads to the best outcome for people with CF post-lung transplant?
How can we best foster education about CF in transplant teams and transplant education in CF care teams?
How can we set standards for, and enhance communication between lung transplant teams and CF care teams?
How can we best assess and ensure CF patients post-transplant are receiving comprehensive care for their CF for their lung transplant and the non-pulmonary aspects of CF?

Use of This Position Paper

The CF Foundation intends for this executive summary of this position paper to summarize the published position paper. The published position paper summarizes evidence, and provides reasonable clinical guidance based on that evidence, to clinicians, patients, and other stakeholders. Care decisions regarding individual patients should be made using a combination of this paper, the associated benefit-risk assessment of treatment options from the clinical team, the patient's individual and unique circumstances, as well as the goals and preferences of the patients and families that the team serves, as a part of shared decision-making between the patient and clinician.

This executive summary was prepared by:
Erin Lowery, MD, MS (University of Wisconsin Madison) and Michael Anstead, MD (University of Kentucky)

Share this article

Purpose and Background

Methodology

Key Points

Summary of Models

Pros

Cons

Unanswered Questions:

Further Reading:

Use of This Position Paper