by Anne-Sophie Carret, MD

Clinical Associate Professor, University of Montreal
Pediatric Hemato-oncologist, Sainte-Justine University Hospital Centre, Montreal

 

Diffuse intrinsic pontine glioma is a tumour found in the brainstem, the part of the brain which connects the cerebellum to the spinal cord. Most brainstem tumours infiltrate the brainstem in a diffuse fashion and, consequently, are inoperable. “Glioma” is the designation for any tumour which comes from glia, tissue that supports the neurons and ensures their proper functioning. The brainstem contains all the connection fibres between the brain and the spinal cord, but also important structures necessary for eye movement, face sensitivity and motricity, swallowing and breathing control.

 

Median life expectancy is nine months from diagnosis.  Fewer than 30% of children survive 1 year from diagnosis, and fewer than 10% survive 2 years, making this tumour one of the most devastating in pediatrics. Because this is a rare disease, creating and setting up an international registry are essential in order to better understand it and support research. Each year in Quebec, an estimated one or two children are diagnosed with DIPG.

 

Until now, the standard treatment for DIPG has consisted of six weeks of radiology, which usually alleviates the symptoms. Unfortunately, after treatment, the tumour recurs within six to nine months, often spreading quickly and fatally.

 

In the ongoing quest for recovery, children suffering from DIPG are often subjected to more than one experimental treatment, enduring many undesirable side effects which can be trying for them and their loved ones.

 

While intellectual faculties remain intact, DIPG gradually destroys motor functions, causing partial paralysis, difficulty speaking, problems swallowing and breathing. What makes this particularly painful is that the children are conscious of their inevitable deterioration. The search for a cure starts here and now...