Most people have never heard the term “childhood dementia.” That silence is part of what makes it so devastating. Collectively, childhood dementias take more young lives than childhood cancer, yet they remain largely invisible.
Childhood dementia isn’t a single disease. It describes a group of rare, mostly genetic conditions that cause children to progressively lose skills, memory, and abilities they had already gained. They share the trajectory of dementia, even though their causes differ from adult Alzheimer’s. Worldwide, an estimated 1 in 2,900 children is affected, across more than 70 distinct disorders. In the United States alone, researchers estimate roughly 1,077 new diagnoses each year.
What BPAN is
BPAN, beta-propeller protein-associated neurodegeneration, is the form of childhood dementia Isa lives with. It is caused by a change in the WDR45 gene. In almost all cases that change is spontaneous, or “de novo,” meaning it is not inherited from a parent and could happen to any family.
BPAN belongs to a family of conditions called NBIA, neurodegeneration with brain iron accumulation, in which iron gradually builds up in the brain and neurons progressively die.
What BPAN does
At the cellular level, BPAN disrupts autophagy, the brain’s recycling system, the process cells rely on to clear out and reuse their own waste. The effects unfold in two phases. In early childhood, it slows development and can cause seizures. Later, often in adulthood, it brings a steep, Parkinson’s-like decline.
There is currently no approved treatment that stops or reverses BPAN. Today, care focuses on managing symptoms. Changing that is the central purpose of the research the Foundation funds.
Why a rare disease holds universal lessons
Here is the part that surprises people. The same failures that drive BPAN, in autophagy and brain metabolism, are implicated in far more common adult diseases, including Parkinson’s and Alzheimer’s.
So research into Isa’s rare disease doesn’t only help a few hundred children. It can illuminate the biology behind diseases that affect millions. That is exactly why we fund it, and why progress against BPAN could echo well beyond it.
How you can help
The first step is simply knowing the term, and saying it out loud. From there: explore the science we fund, find resources for families, or give to push childhood dementia into the light.
Incidence figures: Childhood Dementia Initiative analysis, Brain, 2023.