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or "Diffuse Intrinsic Pontine Glioma"

About DIPG
This, the worst of all possible brain stem tumors, is rare with about 125 cases reported in the USA in a year and it affects the young, mostly between ages 5 and 10. So for a two-year old it is even rarer. 

The name says it all:
"Diffuse" -- it is spread among the nerves and tissues of the brain stem.
"Intrinsic" -- it is wrapped amongst them, making surgery or accurate and selective radiation virtually impossible. "Pontine" -- it is located in the pons (middle) of the brain stem.
"Glioma" -- the tumor arises from the glia, the tissues that support the neurons (the thinking cells).

The brain stem is the lowest section of the brain, transmitting signals from the brain to the spinal cord and converting instructions from the brain to the motors of the body. Tumours in the brainstem can be located in the cervicomedullary junction, the midbrain, the tectum or the pons. The pons is the most difficult to reach.

While radiation therapy can shrink the tumour and bring a temporary respite, an irremediable regression follows. The median survival rate is nine months.

St Jude's Hospital in Tennessee, leads research in this field and sums up the current desperate lack of knowledge:

In their quest for a cure, DIPG children must move from one experimental protocol to another, enduring treatments with side-effects unacceptable with any other diagnosis. The cruelty of this disease cannot be denied. Sparing their cognitive abilities, DIPG slowly robs children of their motor functions resulting in partial paralysis, loss of voice and sight and finally ending with an inability to eat and breathe. It is both heart wrenching and painful as they are fully aware of their decline often until their last day.

We currently DO NOT know what causes brain tumors. The major causes seem to be chromosomal and genetic abnormalities. Research so far has statistically proved very few instances of possible environmental causes for childhood cancer. The Children's Oncology Group (COG) continues to conduct epidemiology, cytogenetic, and microbiology studies in their quest for answers.

Charlie responded well to initial radiation and recent clinical experience suggests that the risk of re-radiating is less than once thought.

One alternative is proton therapy, or particle beam radiation, which permits a degree of focus and accuracy beyond the scope of conventional radiation. It can target tiny clusters of cells within a tumor.  Being so focused, it can work with lower dosages of radiation and in theory be used more safely for re re-radiation, reducing the risk of necrosis which happens when radiated cells contaminate and destroy adjacent and vital cells. However there is a still high risk, and still no definitive clinical proof that treatment produces better results than conventional radiation. While the Massachusetts General Hospital in Boston turned Charlie down, the CDH Proton Center in Chicago indicated it is willing to take the risk and try the treatment, preferably after another two or three months, provided Charlie's condition remains stable.

However, the conclusion now, at least for the time being, is that so much cell damage may already have been done by the original radiation that even with accuracy of the proton beam any damage to surrounding cells could be fatal.  So on balance the prevailing belief is that positive results from low dosage conventional re-radiation may be achievable with less risk than proton.

It has been described as the William Tell dilemma:  Tell is invited to shoot the apple off his son’s head with an arrow. Success means the child is completely unharmed, but the smallest error in aim can mean instant death. The alternative is the shotgun.  The pellets will certainly knock the apple of the boy’s head and as just as certainly do some harm, but probably not fatal.




And, finally, a passing thought, as a postscript:
Don't give up!                                      Free Frog!

though, of course, it's never that simple.


To learn more:

Just One More Day - a site dedicated to DIPG suffers

Ped-Onc Resource Center - a site by and for parents with children with cancer