Eli is feeling very good, growing stronger and more nimble each day, making friends, running, jumping and playing more and more like a boy his age each day. He is doing better than ever. He has grown three inches in the last year and the growth hormone has visibly redistributed his mass from chubby baby fat into lean muscle.
Unfortunately, what we are seeing on the outside is not representative of what is going on inside, in his head. Eli's latest MRI shows that the solid portion of the tumor is beginning to fill up the hollow part of the cyst (at least from what we can see by MRI). Therefore, we can no longer continue to drain the cyst to make the tumor smaller because soon there will be little fluid to drain off. This leaves us in the position of having to remove the tumor earlier than we hoped. Eli will be having surgery to remove the tumor the beginning of April. Depending on how things look after his surgery, this may be followed by a 6 week radiation regimen to kill any tumor remaining after the surgery.
July 2009 (top) vs February 2010 (bottom):
We were very much hoping not to have to do this until Eli was considerably older, but unfortunately surgery appears to be the only option remaining at this point. Our entire reason for draining the cyst was to avoid surgery and radiation because of the potential to harm his little brain. Older kids and adults fair far better with radiation treatment so we think it is best to try to avoid it until we absolutely have to.
Clearly we are very unhappy about this turn of events. We have struggeled to seek other options and potential reasons and/or ways of dealing with these new complications. But from our review of the literature, speaking to others and seeing how these tumors are being treated around the world, there are no other good alternatives.
We know how strong and resilient Eli is. And we know how much he is loved. And this gives us great strength to know that he will rise above this challenge as he has the others.
Thank you as always, again and everyday for the thoughts and prayers that you send to Eli. We are ever greatful for your love and support.
If you would like to learn more about the treatment options and the potential side effects please read below.
So what are the complications of sugery vs radiation and the different combinations currently being used? There are many different treatment paths, but unfortunately, little data exists on what path is actually best (i.e. what path results is minimal additional morbidities (blindness, obesity, IQ drop etc) while also avoiding relaspe and tumor regrowth). Furthermore, what treatnment path is best most probably depends on the child's age, the size and relative location of the tumor and their current pre-tx deficits. For example, Eli has already lost vision in one eye and has no pituitary function. For some kids they are totally blind by the time the tumor is recognised or have other deficits. So, in my opinion, there are many considerations to be made when deciding the best care-plan.
Surgery Only vs Surgery and Radiation:
There are many who advocate to do an aggressive surgical resection and avoid radiation entirely. While others contend that a conservative resection followed by radiation treatment has a better outcome.
So what are they disagreeing on?
The complications commonly seen with a total resection are hypothalamic dysfunctions. This is because the tumor tends to grown little finger-like projections into the hypothalamus. In order to remove the tumor in total, portions of normal brain must also be removed. Now, sometimes the invasion is minimal - sometimes it is not.
So what is hypothalamic damage?
The most common type is called hypothalamic obesity. It results what a portion of the brain involved in satiety (hence called the satiety center) is damaged. This leads to uncontrollable eating. They child simply cannot stop. They always feel hungry and the overwhelming urge to eat is stronger than any power of conscious mind. This can be a very debilitating disease. It often results in multiple gastric-bypass surgeries and significant stigma and health problems. Damage to the hypothalamus can also damage the bodies ability to regulate body temperature. In other words, when you go for a run, sit in the sun or go to the sauna your body temperature rises. This temperature increase is received by your brain as a signal to turn on "cooling" responses. These are generally to sweat, open skin pores, cause increases in blood to the skins surface so that is can be cooled by its proximity to the out-side world etc. Conversely, when you play in the snow, swim in your favorite lake buck-naked or otherwise get cold, your brain senses this decrease in temperature and causes your skin pores to close, for you to shiver (yup that uncontrollable shaking-teeth-chattering-thing is cause by your brain is trying to get you to warm up by making you move) etc. So, if this temperature sensing center is damaged then your body doesn't know that you're overheating or freezing. It does not make the necessary physiological adjustments to regulate your body temperature. Clearly this can be a problem. Finally, damage to the hypothalamus that is seen due to cranio resections (just to clarify that there are many other areas of the hypothalamus that do a million other things) is rage control. Pretty simply your brain helps control you from throwing total tantrums and becoming mad with rage! Loss of this center can cause random temporary uncontrollable fits. Not so fun.
Well, what about radiation? What are its risk factors!
Most notable - in a YOUNG patient (i.e. this is not so much a problem if your over 15 and even a 7 year old suffer's less loss than a 3 year old) they will suffer some degree of IQ (memory, processing time, concentration, overall intelligence) drop. The drop may be so mild that it is not apparent. It also may be more severe such that the child has problems learning new tasks, remembering new information etc.
The second problem that is seen more when cranio's are irradiated it vasculopathies (that's fancy for problems with the blood vessels in the brain). These can be short term or not cause any problems for 40 years!
Finally, subsequent tumors! As with many treatments for cancer. What we use to treat them can also induce additional tumors! Shitty huh!
So that's it more or less!!! We hope that Eli will not suffer any of these fates. That his tumor will be easily resectable and the removal will not cause any damage and he will never need radiation. As with all children - we wish him to have the fullest life. Be happy and eventually healthy. Sometimes the best path to attain these outcomes is uncertain - not only for parents, but even from the best minds in the field.
Thanks for listening. We hope this information is helpful.
Meagan
