What is DIPG?
It wasn't supposed to be this way
DIPG, Diffuse Intrinsic Pontine Glioma, is said to be the worst of all pediatric brain cancers. It is an aggressive brain tumor and is inoperable because of its location. The tumor is located in the pons of the brainstem which controls all bodily functions…breathing, swallowing, vision, walking, etc. It is considered terminal upon diagnosis.
DIPG strikes healthy, happy children between the ages of 4 and 12. These brain tumors are extremely rare. It strikes suddenly with no warning. There have been children as young as 9 months old to teenagers diagnosed with DIPG; adults can rarely be stricken. It is said about 300 new cases of DIPG are diagnosed each year. Many researchers believe that finding the cure for DIPG will lead to the cure for other pediatric brain cancers. Sadly, only about 4% of all cancer research funding goes to fund ALL pediatric cancer research.
How is DIPG treated?
Very, very few options. First option is to do nothing and just make memories as long as you can. Second option is radiation. Sadly, the only other option is to find a DIPG Clinical Trial and hope your child is accepted into the trial. There are not many DIPG Clinical Trials in the country and the ones we do have are funded mainly by private donors.
Diffuse Intrinsic Pontine Glioma, often just called DIPG, is a neurologically devastating, and in most children, lethal pediatric cancer that develops in the structural tissue of the brainstem, without truly effective therapy.
There are more than 300 cases in the U.S. each year, but its relative rarity actually makes fighting DIPG more challenging. There is little federal research funding for the study of “rare pediatric” diseases, and many oncologists don’t have specialized experience with DIPG or its treatments.
Understanding what Diffuse Intrinsic Pontine Glioma (DIPG) means will help you understand just why this cancer is so hard to beat.
Diffuse – Threaded through healthy brain tissue, a DIPG tumor is not a solid mass. It can’t simply be removed with surgery.
Intrinsic – It’s due to causes within the brain itself. Some studies suggest DIPG tumors start in childhood because kids’ brains are developing so much, so quickly. There’s no research linking DIPG to genetic or environmental causes.
Pontine – The tumor grows in the pons, the part of the brainstem that puts breathing, swallowing, and other basic bodily functions on autopilot. It can also, less frequently, arise in other parts of the brainstem or extend into other areas. The location makes the tumors all the more debilitating and treatment all the riskier.
Glioma – The tumor starts in glial cells, the white matter “glue” that supports and insulates neurons throughout the brain.
Even as outcomes for children with other types of cancer have vastly improved, long-term survival rates for DIPG have been stalled for more than 50 years. Most DIPG patients die within a year of their diagnosis. Less than 10 percent see two birthdays beyond their diagnosis. In the interim, DIPG patients suffer debilitating side effects as the growing tumor presses on the brainstem and makes seeing, walking, and even breathing increasingly difficult. Yet, precisely because it’s one of the most challenging and lethal cancers, the Children’s team is committed to finding new, more effective treatments for DIPG. We also believe that if we conquer DIPG, we’ll unlock cures for many other types of cancer in the process.
Though children of all ages and even a few adults have been diagnosed with DIPG, most new cases are in children four to ten years old. Some research suggests this is because of rapid brain development in those early elementary years. Symptoms first appear when the tumor puts pressure on nerves in the brainstem that control facial, eye, and muscle movement. Parents might first notice that their child is suddenly clumsy, has developed a “lazy eye," or has facial weakness. As the DIPG tumor grows, most patients suffer some combination of:
Problems seeing, such as double vision, drooping eyes, or a “lazy eye”
Drooping on one side of the face
Trouble with movement, particularly on one side
Difficulty talking, chewing, or swallowing
Hearing problems, including sudden deafness
Nausea and vomiting caused by brain swelling
Jace Courageously Fought For 347 Days