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Just Life - An Update

Non-Tumor Life

Eli was a raspberry for Halloween...So so cute!

Eli started school this year at the Denver Waldorf School…the same school I attended from pre-school to 8th grade. He absolutely loves it and, of course, he already has many girlfriends. His teachers are the most dedicated, compassionate and engaging teachers you can imagine, and it shows in how much he is learning, growing and loving his new school. Additionally, we feel so lucky to have the support of the other Waldorf families. We decided to tell everyone a little about Eli so that there would be no secretes, uncertainties or questions like “why is that kid so small and still cannot go up stairs at 3 years old!” The response we got was just amazing. Not amazing because everyone wrote back with their sympathy, but amazing because everyone took the time to tell us little stories about Eli that touched them – like how he befriended their child, or how he always says good morning to the child AND the parent each day or just how much they loved Eli and find his spirit beautiful…We are so very very lucky to have the Waldorf family as part of our family.

Jacob started his post-doc and is very inspired (again – finally). He has decided to continue his work on the basic science of cancer therapy. This has been what he has worked on for the past 10 years and although he was thinking of looking into a career in alternative energy – circumstances confirmed his path and dedication to cancer research.

I am happy to be nearly half way through my 2nd year of med school and very relieved to have completed my neurology/nervous system block. It taught me a lot about Eli’s disease and gave me better perspective to understand his treatment – but it was tough emotionally (and academically!). Unfortunately, now I must begin preparing for the Step 1 USMLE board exam. This exam is probably the single biggest determinant of what specialty you have the OPTION of going into. While I am still unsure of what I want to specialize in (peds or OB sub-specialty) I hope to do well enough on my boards that I will have some options (like peds-ophthalmology if I so desire). I will take the exam in the beginning of April – so be wishing me luck….please!

Otherwise life is moving along. We have good days and bad days, sad days and silly days, confusing days and frustrating days and days that we wish would last forever…And those are the ones we try to remember when we have days that we wish never were.

More on the Medicine of Cranio (tumor) Treatments.

(Subject matter may be difficult for some.)

From the beginning we decided to treat Eli’s tumor differently than is standard care for such tumors. We have been able to do this for many reasons, but probably the most significant one is because...we can! What does that mean? Often the brain is in eminent danger of further damage by the tumor and the tumor must be removed quickly. We know of a case of a little boy Eli’s age who was going blind and therefore the tumor needed to be removed immediately to try to save the child’s vision. In Eli’s situation, the tumor had already done its damage before he was diagnosed. It had already completely crushed his pituitary gland and compressed his right optic nerve leaving him blind in the right eye. Fortunately, his left eye is okay and the tumor is just off to the right enough that it has spared that nerve for the most part. Therefore, we are in the unusual position of having options. Even more, we have options because Eli does not have a malignant disease that must be treated aggressively and excised immediately to give him the best hope of survival. Instead he has a disease that really, in our opinion, requires careful planning and consideration. We are fighting for the quality of his live, not the quantity.

We want to try our best to make decision that protect his brain, and have the greatest potential to spare further damage. This is why we have decided to put off surgery as long as possible and drain the tumor to relieve pressure and further complications. So what is so bad about surgery and radiation beyond the obvious? Well, it is very VERY case specific. What I mean is that given Eli’s age, the location and aggressiveness of his tumor, the damage that has already been done and the damage that could be done by doing surgery now – it makes most sense to us and Eli’s oncologist to wait. So what are the risks and most common problems with removing these tumors? There are generally two approaches with differing risks: Option one-Complete Resection: That means cut all of the tumor out, leave none behind (or as little as possible). Why this is dangerous: The tumor has interwoven itself into the hypothalamus – like little fingers inter-digitating. Therefore to remove the tumor completely means damage to the hypothalamus. What does that mean? Most common problem is morbid obesity because the brain can no longer recognize the feeling of satiety. Difficulty regulating body temperature – predisposing him to hypo and hyperthermia because his brain will not recognize that he is too cold or too hot signaling him to make the appropriate behavioral changes (seek warmth, take off your jacket). Finally, there can be issues with aggression and anger – Hard to imagine Eli as an angry and aggressive little kid…we want to keep it that way. Okay so what about Option Two – Surgical Excision with Radiation: Generally one can avoid, to an extent, damage to the hypothalamus. This is a more conservative approach that some people favor and some do not. It necessitates radiation often to completely kill the tumor cells. Radiation to the tumor, is radiation to the brain. Although radiation today is very targeted and significantly superior to 5-10 years ago, it is not perfect. For an adult this would be less of an issue. It is an issue for Eli because he is 3. A 3 year olds brain has not finished myelinating and radiation causes problems with this process. This means that while he may not lose IQ points (after 3 years old I guess you’re as smart as you’re going to be! I have no idea – that’s just what I’ve been told), he will process things slower, think slower etc. There are also other issues and risks associated with radiation, some specific to craniopharyngiomas – email me if you want these details.

There are many who would disagree with our treatment plan. And there are many who will not understand it. But for us it makes the most sense. It is not an easy choice and there will always be questions about what is the BEST choice. Unfortunately no one knows. Because Eli’s tumor is very rare and NOT generally lethal – there is little to no research or even studies of treatment outcomes! One story I think pertinent here is about those different treatment beliefs.

When Eli was first diagnosed we spoke with one of the most highly respected pediatric neurosurgeons in the country (and possibly the world) about his approach to cranios. Let’s call him Dr. J. He told us, and I quote “With me you take all your risks up front. I think the best way to treat craniopharyngiomas is with total resection. If there are going to be any deficits, you know them immediately.” We disagree with this approach philosophically and medically. In our opinion, the risks associated with irradiating a young brain should be avoided IF AT ALL POSSIBLE. This is often not the case – and just for the record we do NOT endorse going against medical advice nor treating cancer with prayer or herbs alone…ALL modalities must be employed –chemo, radiation, surgery, love, hope etc. So make sure to talk to your doctor and make sure you understand your options and the potential risks and benefits of each option. If you want a second opinion GET ONE. Doctors understand this and often can give you names of some people they respect who treat differently. If your doctor is less than helpful and appears offended that you would seek a second opinion, then you should probably find another doctor anyway! This isn’t about their ego – it is about your child’s or your health. Okay…. back to our story and off my little tangential pedestal…We believe that the longer we can put off radiation the better. We believe that slow and thoughtful treatment is best – if it is an option.

But slow and thoughtful treatment is hard and there is an undeniable benefit of “taking all your risks up-front.” First, it feels right. Our immediate gut reaction when we hear “tumor” is to cut it out. The idea of leaving something like this around is preposterous. In fact, when Dr. J said that one of the many options (not one he recommended) was to just “draining the tumor” I said “Hell NO!” I wanted to do something. Initially I felt that the best option would be to act and act quickly. What I did not realize then, nor after we decided on a different treatment option, and am only realizing now is that the slow road in many respects is harder. It is hard to wait, to act cautiously and think critically. It is emotionally draining, physically difficult and intellectually perplexing. In some ways it is easier to get it out and then deal with the repercussions of the disease. Time for another little side-story: Just the other day I was on Labor and Delivery. There was a mom who had been on bed-rest for 4 weeks because her water-broke. She and the baby were doing well and were now 32 weeks. But then she started to have some contractions and people got worried. Her husband and family wanted to deliver the baby. Why wait? Why not get the baby out and take care of it in the NICU? Well, there are so many reasons I won’t elaborate them here – but what I realized that day is just how hard it is to wait. To be unsure of what will happen, when it will happen is exhausting. To be “done” and face the next challenges often seems like the better option.

So I guess what I am getting at is that sometime you will be given options…and sometimes you won’t. We have them now, but when Eli was first diagnosed he was in really really bad shape – and we did not have many options. His head had filled up with CSF fluid because the natural drainage path was blocked by the tumor. This is why he was having headaches, vomiting incessantly and was so severely sick – it wasn’t the tumor per se, but the physical presence that was causing so many problems. The PICU doctors wanted to put in a shunt to drain the fluid out of his brain and into his abdomen (called a V-P shunt. Yes, Eli has one). There was an option to remove the tumor and put the shunt in at the same time – again standard of care for some people and situations. But we were unsure about doing either! We did NOT understand the severity of the situation. We called many doctors in NYC and Denver. Finally, we were told that we had to do the shunt NOW. We did not really understand, but we did sense that there was an urgency and emergency and a fear in the doctors to get it done (mind you this all took place in a matter of 2 hours from the time we were told he had a brain tumor). Eli went into surgery. The anesthesiologist was this amazingly compassionate woman. She looked at me and told me she would take care of him like she was his mother, and the way she looked into my eyes, tears welling in her eyes too, I know she would. Needless to say everything went well. When he got out of surgery the neurosurgeon said that the pressure in his brain was so high that when they drilled the hole and placed the catheter into his brain, CSF fluid shot 10 feet across the room! What I know now (after my lovely neurology block)…Eli was at risk for herniation. What does that mean? It means that after a point the pressure in the brain has nowhere to go – so it pushes itself through the only hole it can (the foramen magnum – ‘cuse it’s big!) at the base of your skull where the brain connects to the spinal cord – there is nothing worse. One could say we had no option. We had to do what the doctors said was best, because we did not know what was best, and there was no time to deliberate or educate ourselves. We had to trust our doctors and their advice. This is most often the case with most people, most of the time. We are luck that they were (and are) wonderful, compassionate and brilliant physicians.

Okay, I think I've said enough for now!

As always thank you for your love and support. Thank you for remaining involved in Eli’s story and our lives. We are constantly reminded and made stronger by your thoughts, prayers and wishes – so thank you.

Meagan, Jacob and Eli

Figuring out the Tumor Drainage Schedule - Email Update

Figuring It All Out

Over the past few months we have been trying to figure out what to do and how we want to continue to treat Eli’s tumor. Our last update discussed how we were going to try another round of intra-cavitary bleomycin in an attempt to weaken/kill the tumor. After 9 more rounds there was no change in the tumor status. Therefore, we decided to totally drain the tumor under propofol (anaesthesia – propofol is very safe – don’t believe the Michael Jackson hype!). Our hope was that the tumor would refill slowly because the chemo had damaged its ability to produce fluid. We waited 2 months and had another look by MRI – against our hopes the tumor had completely refilled to its beginning size. At that point we knew the chemotherapy treatments were a failure. Because of the toxic effects of the chemo and the non-response there is no reason to try more. So now what!!!

We decided that the best way to treat the tumor is to drain it under anaesthesia as needed. What does “as needed” mean? Essentially, we will drain the tumor when it refills to about its starting size (maybe a little smaller). So how frequently will that be? Well, we are still figuring that out. We last drained the tumor 2 weeks ago (Nov 3rd) and did another MRI on Nov 12th. At that time it was about ½ full. We will have another MRI on the 24th and check the status again. By doing MRI’s ever few weeks we can see how quickly it refills so that we know how frequently we need to drain it to keep the tumor size minimal, but at the same time not have him going under anesthesia every week. Most likely we will need to drain it every 6-10 weeks. We will continue with this approach for as long as we can in order to avoid surgery and radiation. For more info on why we have chosen this path please visit the blog. So that is where we stand today – still figuring out how to fight this thing while preserving as best we can his cognitive, emotional, vision and endocrine functions.

As always thank you for keeping us in your thoughts and checking-in on us! It makes us so happy to get your emails and see your comments on the blog.

All our love to you and yours,
Meagan, Jacob and Eli