What is DIPG?

Diffuse intrinsic pontine glioma, or DIPG, is an aggressive, rare, inoperable brain cancer. While it can affect people of any age, the vast majority of DIPG cases develop in children between ages 4 and 11. About 300 U.S. children develop DIPG each year. The disease accounts for about 10 percent to 15 percent of childhood brain cancers, according to defeatDIPG.org.

The pons

DIPG occurs when a tumor forms from glial cells, which protect and support nerves. DIPG tumors grow in the pons, an area at the top of the brainstem that acts sort of like an air traffic controller, passing messages between and coordinating the actions of the nervous system and the brain.

The pons controls eye movement, facial expressions, chewing and swallowing and helps sounds move from the ears to the brain. As part of the brainstem, it also affects many basic functions, such as breathing, walking, sleeping, heart rate and blood pressure.

Growing out of control

DIPG tumors grow between normal nerve cells. Some other types of tumors start as compact knots or balls that get larger over time, but a DIPG tumor is more like an ivy plant that finds its way into cracks and crevices, damaging everything it touches.

Symptoms vary depending on where the tumor is located, but most cases are diagnosed after a caregiver notices a sudden case of “lazy eye,” trouble chewing or other problems with the facial muscles.

As the disease progresses, sufferers have increasing difficulty with muscle control, making it hard for them to chew, swallow and walk, and eventually, to breathe.

High stakes

In the board game Operation, players use tweezers to remove plastic body parts from tiny metal compartments. If the tweezers touch the surrounding metal, a loud buzzer sounds and the player’s turn ends.

Treating DIPG is a bit like playing Operation, with life-or-death stakes. Until recently, doctors thought it was too risky to even biopsy the tumors because any damage to the surrounding healthy tissue could have dire consequences.

Brain surgeons can’t operate because there is no safe way to untangle the tumors from the vital parts of the brain they affect. Too much radiation can have serious consequences, and DIPG usually does not respond to chemotherapy.

Terminal diagnosis

Doctors are now able to biopsy some tumors, but usually not until they are in an advanced stage. Researchers continue to work toward a cure. There are hard-to-get experimental treatments and some recent studies show promise, but it is still considered a fatal condition.

“Treatment” consists mostly of targeted radiation sessions that temporarily stop tumors from growing. However, the “honeymoon” generally lasts only five to nine months before the tumor starts growing again. The disease often progresses very rapidly.

Only 10 percent of DIPG patients live for two years after diagnosis. Less than one percent make it to five years. On average, DIPG sufferers die within nine months of diagnosis.