what is
DIPG?
Diffuse intrinsic pontine glioma (DIPG) is a brain tumor found in the pons which is part of the brainstem. The term diffuse means that the tumor grows into other surrounding tissues which causes cancerous cells to mix with healthy cells. It is impossible to perform surgery to remove a DIPG tumor without damaging healthy tissue. DIPG is typically diagnosed in childhood between the ages of 5 and 9. Unlike many other types of cancers or tumors, there has been very little progress in finding effective treatments or improving current therapies. Unfortunately, this results in very low survival times following diagnosis. With continued funding of research towards treatment, We Love You Connie Foundation hopes to make DIPG a curable disease.
Who Does it Affect
DIPG tumors are almost exclusively found in children, mainly those between the ages of 5 and 9, but can occur at any age in childhood as well as very rarely in adults. Boys and girls are equally affected by DIPG. It is estimated that approximately 300 children in the United States and 30 in Canada are diagnosed annually with DIPG or roughly 1 in a Million of children are diagnosed with DIPG globally relative to the global population. DIPG tumors are the most common brainstem tumor found in childhood and are the leading cause of brain tumor death in children.
Signs & Symptons
The brainstem is responsible for controlling many critical life functions, including blood pressure, breathing, and more. Due to this, many of DIPG’s early symptoms will appear in the facial region, affecting eye muscles and movements. The tumor will grow quickly and therefore symptoms appear suddenly and worsen rapidly. Symptoms of DIPG include:
- Eye and eyelid movement problems such as difficulty looking to one side, double vision, and drooping eyelids.
- Facial weakness or drooping on one side of the face.
- Sudden hearing problems and deafness.
- Headaches.
- Limb weakness.
- Problems with coordination and difficulty standing or walking.
- Nausea and vomiting.
The symptoms listed above do not definitively indicate brain tumors, but anyone with neurological symptoms similar to those mentioned should seek professional evaluation by a physician.
Prognosis
Unfortunately, the prognosis for DIPG continues to be very bleak. In the absence of any effective therapies, DIPG is a fatal diagnosis. The average survival time after diagnosis is 6-9 months. 95% of children will pass away within 2 years of initial diagnosis. Less than 5% of diagnosed children will survive longer than 5 years. With continued funding of DIPG research, there is hope for a cure and improved treatment options.
Radiation is currently the main treatment option for DIPG patients, as it is the only form of treatment that has proven to benefit the patient at all. For roughly 70% of patients, radiation will cause the tumor to shrink, which provides temporary relief from many of the symptoms associated with DIPG. Even though the tumors shrink during radiation, the treatment is incapable of increasing chances of survival, and the tumor simply grows again within weeks. DIPG patients who do not undergo radiation have a typical survival rate of 5 months - radiation therapy is estimated to extend the patient’s life about 3 months.