Care guidelines for PLAN are now in the works

from | April 17, 2023

Translation of an English article by Patricia Wood

Thanks to the support of four organizations funding the project, consensus guidelines for treatment and care in a form of NBIA known as PLAN or PLA2G6-associated neurodegeneration are now underway.

This is the third NBIA disorder for which researchers have developed best practices. The other two are more common forms of NBIA, PKAN and BPAN. PLAN encompasses a wide range of symptoms, and depending on the age of the affected person and their symptoms, they can be classified into one of three subtypes: INAD, aNAD, or PLA2G6-related dystonia-parkinsonism.

Four organizations have joined together to support "Best Practices in the Care and Treatment of People with PLAN." In addition to the NBIA Disorders Association, they are the INADcure Foundation, a U.S.-based nonprofit organization that advocates for people with INAD and other subtypes of PLAN, and NBIA sister organizations Associazione Italiana Sindromi Neurodegenerative da Accumulo di Ferro (AISNAF) in Italy and Hoffnungsbaum e.V. in Germany.

Photo by Dr. Susan Hayflick of OHSU.

Dr. Susan Hayflick of OHSU is the lead investigator, working with her colleagues Dr. Jennifer Wilson as lead author and Allison Gregory, MS, as project director. This group also developed the care guidelines for PKAN and BPAN.

The main goal of this project is to advise clinicians on the best and most acceptable approach to diagnosis, care, or treatment of PLAN and its three subtypes. Because INAD is the most common form of PLAN, a larger portion of the guideline will likely focus on this subtype.

Best practices will be covered in the following areas: diagnostic assessment, initial treatment, pharmacological and surgical care, observation for complications of illness, emergency management, educational support, nutrition, psychosocial support, and any other areas identified by participants.

Input will also be sought from other leading PLAN experts, as well as selected parents, caregivers, and funding patient organizations. Members of the larger patient and family community and other patient organizations will be invited to review and comment on the final draft of the guideline within two weeks.

The project will take approximately 12 months to submit a draft for publication. The total cost is $50,308, which consists of personnel costs covering the time and labor of the project team with its expertise, project management, and coordination. Other costs include publication fees for free public access to the paper and travel costs to present the results at two meetings within one year of publication.

Translated from: December 2022 Newsletter of the NBIA Disorders Association, p. 4/5:

Translated with (free version)

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