Brain Stem Glioma

I have been contemplating writing a document like this for a few months now. I collected my thoughts together and thought of the pros and cons and the hullabaloo it may stir up. After careful consideration I decided to go ahead with it. Those of you who know me would say "why should he worry about being controversial now, it never stopped him before." If you do not want a healthy dose of reality stop reading now. Everything from here on is factual and is culminated from the similarities or many many cases of patients who have or who have passed away from having a brain stem glioma tumor over the past three years. Some of it can be related to brain tumors in general. I can only hope that by passing this information on that you as a caregiver or patient can get an idea of the ugly course that this disease takes. You most probably will not ever read this in any book, article or abstract. It is written from a caregivers point of view. I am not an MD, and this information is provided for information purposes only. Use it at your own risk and judgement. Always consult a physician and get a second opinion if possible before starting any treatment. If you want a patients point of view I urge you to read the Late Steve Depesa's web site "Living with a Malignant Brain Tumor" which can be found at http://home.earthlink.net/~sdepesa/ . Steve recently succumbed to this disease but I corresponded with him frequently while he was alive and he relied on me and my site for help with information for his site. I HIGHLY reccommend that you peruse this site. For those of you who do not know me, my experience came from caring for my daughter who died from this disease and going on to maintain in her memory one of the largest culmination of links and information for brain tumors next to Al Musella's Virtualtrials site on the World Wide Web. In fact I have been working with Al and my links were moved to a subdirectory of his site two years ago when they got too large for the space my internet provider allowed. I research daily and scour the web for new treatments, information and articles on brain tumors and post them to the Braintumor mailing list and the smaller brainstem tumor mailing list which I started and moderate. It went from 6 people two years ago to over 400 subscribers now. With this in mind you should have a pretty good idea now who I am so here we go. I will have to warn you again that some of this is really gut wrenching horrible so stop now if you do not think you can handle it.

Signs, signs, everywhere the signs.
Patients present with brain stem tumors in many different ways. It seems that there are some very common statements or characteristics that happen that I have collected over the years that are some identifying factors.

The only prior clue I had in my daughters case was that she "couldn't read the chalk board at school" and her grades were slipping. I took her to the eye doctor and was told she had perfect vision. Two days later she woke up in her bed unable to move or speak! That is a pretty extreme case to say the least. The docs at the Cleveland Clinic said it was the worst case they had ever seen. I learned later her not reading the chalk board was actually from the fact that she saw two of everything, the medical term diplopia or double vision. Unfortunately even an opthamologist can not diagnose these things in early stages. This I found is one of the most common symptoms. Ataxia or a gait in the walk is another. Patients can experience headaches, vomiting, weakness, droop in their face or loss of function on one side of their body or another. Or may experience little to no symptoms at all.

Brain tumors are usually found via a Magnetic Resonance Imaging (MRI) scan that is done at a major center. Tumor tissue actually lights up on the scan. Tumors in the brain stem are especially troublesome and dangerous because every autonomic function of the body is either controlled there or the nerves pass through it to the rest of the body. Surgery is very dangerous because if you constrict or cut the wrong nerve you could stop the person from breathing or their heart from beating and cause instant death. This is why there are only a handful of surgeons who are capable of operating in this area of the brain. There are basically two types of brain stem tumors. The best document I have read that tries to discern the types is by Dr. Paul Fisher. This document can be found on my site at: http://www.virtualtrials.com/btlinks/brain.htm Fisher breaks the brainstem glioma down into two basic types... atypical and typical. The typical brain stem glioma is the most common and it infiltrates diffusely throughout the pons (middle portion of the brain stem). The term diffuse can best be analogous to taking a handful of sand and throwing it in a tray of jello just before you refrigerate it. When the jello is done it is impossible to remove the sand without removing some of the jello. It is a good analogy to think of as to why these tumors are usually inoperable. You can't remove the tumor without removing necessary good brain tissue. This type is often known as a fibrillary astrocytoma or higher grade. (anaplastic astrocytoma or glioblastoma multiforme) Symptoms usually present for 6 months or less at time of diagnosis. The other type Dr. Fisher calls "atypical". The symptoms are basically the same but not the usual combination of several of them found in the typical type. Duration of symptoms is usually greater than 6 months before the tumor is diagnosed. Fisher writes, "Surgery with less than total removal can usually be performed on this type of tumor and results in quality long term survivals, without administering chemotherapy or radiotherapy immediately after surgery, even when the patient has residual tumor. " These tumors tend to carry a much better prognosis than the typical kind and since they do I am going to focus more on the typical kind because that is the kind my daughter had and it is the most deadliest. My kid has a brain tumor what now? The first thing that usually happens with the typical diffuse brain stem glioma patient is they are put on steroids. It could be decadron (dexamethasone) or some other form of hydrocortizone. (Prednisone, cortef, etc.) These drugs have horrible side effects but provide excellent results in quickly reducing the size of the tumor. And let me state here it is not the tumor that kills the patient, it is the SIZE of the tumor that kills that patient. You could live a long time with a tumor in your head if it never grew in size. If you concentrate your treatment on keeping the size reduced you are doing the best that can be expected. Steroids reduce the swelling of the tumor. They don't kill the cells though or stop them from growing. They are one of the drugs that basically just buy the patient time. They also have some nasty side effects. Patients gain enormous amounts of weight, experience bone loss, (osteoporosis), stretch marks, mood swings, headaches and more. That is just the tip of the iceburg. They create a terrible dependency and virtually shut down the bodies ability to create cortizone. This is why they need to be tapered slowly so the body can start generating cortizone on its own again. Cortizone is the chemical that is produced by our bodies in times of trauma. I like to call it the fight or flight chemical. When cortizone is not created properly or in balance in your body you go into what the medical community refers to as addisonian crisis. Your body is not creating enough cortizone and if not treated properly death could even occur. The person will possibly hiccup uncontrollably, vomit continuosly and become dehydrated quickly, shake, have fevers among other symptoms. What most patients do not realize is that even after being off of steroids for a YEAR or more their bodies could still not properly produce the correct amount of cortizone during times of trauma, (sickness/accident etc.) and they could experience this phenomena. It may be a good idea to ask your doctor to look into giving a calcium supplement to prevent the bone loss and an antidepressant or St. Johns Wort to combat the mood swings while taking these drugs. St. Johns Wort virtually eliminated the mood swings my daughter had and provides the added bonus that it contains the herb hypericin which has been found to be a Protein Kinase C inhibitor (like the touted chemo drug tamoxifen)via the studies of Dr. W. Couldwell and be effective in purer forms of stopping the growth of gliomas. If you have trouble getting off of the steroids you should consult a knowledgeable endocrinologist.

Choices in treatment....
Brace yourself. Even with modern medicine there is still no cure for brain stem gliomas. We are closer every day to finding a cure but until we do children (and adults) are going to continue to die from this disease. HOWEVER, there are survivors! The disease progresses along a pretty predictable course. You basically can not stop the inevitable unless you are one of the lucky few. But you have no way of knowing if you will be one of the lucky few unless you fight this beast with everything you can. Reoccurance of this tumor almost always occurs within six to nine months after initial diagnosis. One doctor refers to the period between radiation and reoccurance as the "honeymoon period". It is during this time that the patient is at their best and could even be mistaken as being cured or in remission. Take advantage of this time, go to Disney, spend every moment you can. It is short and deceptive.

From experiencing the deaths of many children and the rejoices of the survivors I can tell you those that have survived have done so because they have agressively treated this tumor with continuously changing non toxic/less toxic chemotherapy drugs on an alternating basis. The wait and see theory simply does not work with this type of tumor. I am sorry to say I have waited and seen many children die. I have also convinced many not to wait and see and to keep treating, some of those children have died, but some are still alive and fighting. This is not an "opinion" this is based on hundreds of patients over the last 3 years whose parents I have corresponded with.

At any rate depending on how the patient first presents with the disease and how big the tumor is at original diagnoses will determine how the doctors suggest treatment. A tumor that has grown so large to create the effect it did in my daughter there was basically no choice but to shrink the tumor fast. The only choice we had was hyperfractionated radiation. There are many methods of radiating a tumor (all discussed on Steve Depesa's site mentioned above)which all have the same effect. It virtually kills billions of cells all at once. While radiation is not reccomended for very small children, sometimes it is their only hope for survival.

The preferred method and order of treatment you will find is surgery, some type of chemotherapy, immunology or gene therapy and then finally radiation. If you eliminate even one of these steps virtually billions of cells are left alive to be replicated exponentially. If you really don't believe this read renowned Dr. Patrick Kelly's Document entitled "A Simple Explanation of Gliomas" at: http://mcns10.med.nyu.edu/intro/brain.tumor.primer.html . I am not going to use this document to go into other types or specific types of treatment. It wouldn't matter anyways because currently none of them as yet for the most part provide a cure they only buy time. However, your choice of chemo drugs can determine the quality and quantity of that bought time. And bought time gives the chance that a cure will come.

"Honey, I'm home"
I likened my daughter's reoccurance to Jack Nicholson sticking his face in the front door window in the movie the Shining..."Honey, I'm Home!" The sheer terror of that scene is what it feels like when you hear the word reoccurance.

There are however signs of reoccurance that usually are common. They tend to be the same signs as when the tumor first reared it's ugly head in the front door. Honey, I'm home! Vomiting, ataxic gait, droopy face, heart palpitations, you name it, it could happen.

Since the brainstem controls so much ach patients symptoms at reoccurance seem to differ greatly which is why sometimes you get a distant look from the oncologist and a shrug of the shoulders. The brainstem controls so much they just can't say for sure what will happen.

You need to realize at the point where you actually notice reoccurance tumor cells have begun to grow very rapidly. So fast that there is usually little chance at stopping them. This lends credit to my don't play the wait and see game because by then it is too late. If you start agressive toxic treatments at this point your child will live a little longer but the end is terrible. I can't put it any other way. The words of one of the neurosurgeons still haunt me to this day and run over and over in my mind, "She is going to get better before she get's worse, and she will get worse." That sums up what these beasts do. They trick you into thinking that all will be fine and that maybe there is a chance. True for some there is but the numbers are few.

Going Home.
I only want to touch on this subject because each of us at some time in our life will have to deal with the fact that someone we know will die and of course are faced with our own mortality. Death comes slowly for most of these patients and it has been described by some as being peaceful and painless. Unforntunately the moment comes as a terrible surprise and you can never be truly "prepared" for it as they say. Commonly the patient's tumor has progressed so far that their body is totally shut down. Your child will be a baby once again and will require you to feed them (usually through a tube) bathe and do everything for them. They will lose most if not all bodily functions and be totally dependent on you. Towards the end they will sleep for longer periods of time and have less energy. This could go on for days, weeks or even months! It is a horrible time that many do not speak of or want to relive ever again. The patients body will slowly shut down over time. With my daughter it was completely different and not very common. Hers was totally unlike any of the other children I have seen in that she was walking, talking and moving up to the moment she died! I have written about the signs of death previously to the list and also post several documents on my website. One such document is very good and is written by a hospice organization. Basically, though people don't think about it, the signs of a persons body shutting down are common amongst most patients. This document is at http://www.virtualtrials.com/btlinks/death.cfm for future reference.

Wrapping this whole long piece up...
You can learn more things from a child by spending 5 minutes with them than you can in a lifetime. I constantly think of the book "All I ever needed to know I learned in Kindergarten". I would rename it "All I ever needed to know I learned from my child who had a brain tumor". Here is an excerpt from it:

"Most of what I really needed to know about how to live, and what to do, and how to be, I learned in kindergarten. Wisdom was not at the top of the graduate school mountain, but there in the sandbox at nursery school. These are the things I leared: Share everything. Play fair. Don't hit people. Put things back where you found them. Clean up your own mess. Don't take things that aren't yours. Say you're sorry when you hurt somebody. Wash your hands before you eat. Flush. Warm cookies and cold milk are good for you. Live a balanced life. Learn some and think some and draw and paint and sing and dance and place and work every day some." "Take a nap every afternoon. When you go out into the world, watch for traffic, hold hands, and stick together. Be aware of wonder. Remember the little seed in the plastic cup. The roots go down, the plant goes up, and nobody really knows why or how, but we are all like that." "Goldfish and hamsters and while mice and even the little seed in the plastic cup -- they all die. So do we." "And then remember the book about Dick and Jane and the first word you learned, the biggest word of all: LOOK. Everything you need to know is in there somewhere. The Golden Rule and love and basic sanitation, ecology, and politics and sane living." "Think of what a better world it would be if we all -- the whole world -- had cookies and milk about 3 o'clock every afternoon and then lay down with our blankets for a nap. Or if we had a basic policy in our nation and other nations to always put things back where we found them and clean up our own messes. And it is still true, no matter how old you are, when you go out into the world, it is best to hold hands and stick together."

Let's all stick together and pray for each other. Never give up and never give in! A cure will be found.

Copyright 1999 Jim Kenzig mailto:jimkenz@concentric.net
No portion may be reproduced without express written consent of the author of this document.

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