PLAN Care Guidelines

Care guidelines for PLAN now under development
By Patricia Wood

 December 2022

 Hoffnungsbaum eV

Consensus guidelines for treating and managing a form of NBIA known as PLAN, or PLA2G6-associated neurodegeneration, are now underway, thanks to support from four organizations funding the project.

This is the third NBIA disorder for which researchers have created best practices. The other two are more common forms of NBIA, PKAN and BPAN.

PLAN is a broad spectrum of symptoms, and based on an individual’s age of onset and symptoms, they may be classified as having one of three subtypes: INAD, aNAD or PLA2G6-related dystonia-parkinsonism.

Four organizations collaborated to support “Best practices in the care and management of people with PLAN.” Along with NBIA Disorders Association, the others are INADcure Foundation, a U.S. based nonprofit dedicated to helping those with INAD and other subtypes of PLAN, and sister NBIA organizations AISNAF in Italy and Hoffnungsbaum e.V., in Germany.

Dr. Susan Hayflick, with the NBIA research group at OHSU, is the principal investigator of the PLAN consensus guidelines underway.

Dr. Susan Hayflick of OHSU is the principal investigator, working with colleagues Dr. Jennifer Wilson as lead writer and Allison Gregory, MS, as project leader. This group also developed the best practice guidelines for PKAN and BPAN.

The main objective of this project is to counsel clinicians on the best possible and most acceptable way to address diagnosis, management or treatment of PLAN and its three subtypes. Given that INAD is the most common form of PLAN, a greater portion of the guideline will likely focus on this subtype.

Best practice in the following areas will be addressed: diagnostic evaluation, initial management, pharmacologic and surgical management, monitoring for disease complications, emergency management, educational management, nutrition, psychosocial support and any additional areas identified by participants.

Other leading experts in PLAN will also be asked to contribute, along with select parents, caregivers and the funding patient advocacy organizations. Members of the larger patient and family community and other patient advocacy organizations will be invited to review and comment on a final draft of the guideline during a two-week period.

The project will require about 12 months to submit a draft publication. The total cost is $50,308, comprised of personnel costs that cover the time and efforts of the project team with their professional expertise, project leadership and coordination. Other costs are publication fees for free public access to the paper and travel costs to present findings at two meetings within a year of publication.


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