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

We're back...in treatment again.

“You take the good you take the bad you take them both and there you have…the facts of life:”

The Good:

Over the past 4 weeks (ever since mommy finished school for the year and daddy finished his PhD) Eli has been developing by leaps and bounds and has become, well the happiest, most active, most loving little boy we could imagine (yes we are attributing all of this to our (his parents) renewed and devoted attention). But seriously, it has been amazing to watch the changes taking place within our child.

To begin: he has finally found out just how fun a playground can be, slides and bridges and stairs and sand and rocks, and rocks and did I mention rocks. He now loves to climb the stairs, jump on the bridges and slide down pretty much any slide. Speaking of slides, on our recent and much needed vacation to Hawaii, Eli FELL IN LOVE with the water slide. He must have gone up and down it a million times. When I asked if he wanted to go on the bigger water slide, he grinned his biggest grin…and then was told he was too small. If you ask him, he will tell you all about it.

His imagination and desire to interact with other children has blossomed. This combination has made for some beautiful interactions between he and others as well as some challenges for us…like how to explain that Dora and Little Chick and Elmo may not make it over for dinner tonight.

As always he is singing, only now he makes up words to fit whatever is going on. He is still mesmerized by music and continues to practice playing the piano while singing any number of songs. He is running (jogging) and wants to jump so bad he can taste it! He loves to give me kisses when I hurt myself, hugs when he thinks I may be sad (his excuse) and more kisses upon request. His diet is 90% fruit and veggies and we are thinking he may as well just go Raw at this point! Soup and bread are the only other real staples and they keep me from fearing mal-nutrition.

He is, simply put, blissfully happy. He is pain-free and his hydrocephalus is better than it has ever been (since he was probably about 6 months old). His hormones are all properly regulated. Therefore, I imagine he is feeling better than he has EVER, ever felt. We could not be happier.

(The top-right is a self-portrait Eli took of himself (a.k.a self-portrait) and a little picture of Eli enjoying playing in the ocean in Hawaii...he never wanted to get out!)

The Bad:

(top picture was taken 7/8/09 compared to bottom picture taken 3/30/09 prior to collapse the tumor collapse on 5/4/09)

While often no news is good news, in this situation no news was “we are too exhausted to talk about it anymore” news.

Our most recent update of Eli’s tumor showed that it has collapsed. We received many congratulations, but as we feared it was not to last. About 3 weeks after the collapse we had another MRI to see what really happening in there. The MRI showed that that tumor had refilled/grown to about 1/3rd the original size. Not totally depressing, but not good either. Eli’s oncologist told us he wanted to wait and do another scan in 6 weeks. His hope was that the tumor would stabilize and that we could leave it alone for a while. So we went to Hawaii and put the entire thing out of our head (yea!!!).

This last Wednesday Eli went in, once again for another MRI…45 minutes later the scan revealed a tumor only slightly small then when originally diagnosed (5.1 cm verses 5.6 cm tall) . We were sad, but not shocked, not disappointed. To be totally honest with all of you, this is the reality of Eli’s disease. It may shrink and then re-grow almost overnight. Or it may disappear from all sight for 20 years only to reoccur once again. While Eli’s battle will not be a devastating one –comparatively speaking (for we will do everything in our power to prevent any additional morbidities and trauma) it will be life-long. One day, we hope very soon, the tumor will disappear from sight, but the reality of a tumor free boy will only be realized after it has failed to reoccur 25 years later. Of course the chances of reoccurrence will dwindle as the years pass…but now I am getting ahead of myself.

One light in this darkness is that we will be able to continue to try and treat the tumor using intracavitary bleomycin (chemo injected directly into the tumor without any systemic effects). We feared that when the tumor collapsed the little straw-like tube that had been surgically inserted into the tumor may have slipped out…but it is still there and the system is still closed and leak-free.

The Reality:

Eli started chemo treatments again today. He did great – cried for a few minutes, mainly in anticipation and then was his chatter-box self seconds later. He will have treatments 3 days a week for the next 3 weeks. After that we have no idea what we will do if the tumor is still “standing.” We may try for another 6-9 sessions of chemo. We may decide for a resection (cut the %$@er out). We have no idea. We are however, so thankful to have THOUGHTFUL, intelligent and compassionate physicians working with us. Doctors who treat Eli as an individual case and not just another number, another tumor surgery under their belts. As usual we will do our best to preserve his beautiful brain, while conquering the cranio. We also know that we will be working to kill this tumor for several months and years to come. We unknowingly put false hope into many of you with our last email and success story…sorry. We hope for better success in the future. Hope is important and powerful, so please keep hoping for us, for Eli. Reality is temporary, always changing…so we’ll just hope it changes fast and in the right direction.

Thank you as always for your love, support and encouragement. Every email we receive boosts our spirits…even if we fail to respond. So thank you for your unsupported support!

Our love

M, D and E

Consequences of a Brain Tumor-Life for our little boy with pan-hypopit

What is meant by a "benign" brain tumor? In our opinion it is a bit misleading. What we do know is that our son's life is not directly threatened (at least now that he has been diagnosed and is receiving treatment). This is unfortunately not the reality for so many other children diagnosed with brain tumors, most of which are malignant and very difficult to treat. With every visit to the neuo-oncology clinic, which shares space with the heme-onc clinic (that's kids with leukemia, lymphoma and other blood cancers), we are reminded how lucky we are, how lucky Eli is. Although he has scars on his head and belly and patchy hair, he has hair and it is growing. He runs around the clinic full of energy because while he is there for a chemo treatment too, his chemo is not systemic. His treatment will last 2-3 minutes, not 2-3 hours.

But then I begin to think about Eli's future and the "chronic" condition of this "acute" illness. Like many childhood cancers Eli will recover. The tumor will one day disappear. And for more and more children every year their tumors and blood cancers will be cured (not just remission). All of these children, as they grow will deal with the fear of reoccurance, for some the chances are greater than others. For Eli, he will require an MRI every year or so, well...forever! But on top of managing the tumor, Eli will have to deal with something that I feel is much more difficult and life altering - being pan-hypopituitary.

The most accurate description I have come across to describe what a benign brain tumor really is went something like this; "A craniopharyngioma is a benign tumor in a malignant location." Why malignant? Because the brain if full of vital organs and a tumor in such a place is just "bad" and causes "worsening conditions" that are unrelated to the actual cancer itself. When you think about it, the brain is the most protected organ we have. And the pituitary gland, the gland that Eli's tumor totally destroyed, it probably the best protected organ in the entire body.

Anatomy of the Pituitary - Beginning from the outside and moving in:
First, the pituitary is surrounded by the skull, which is made up of hard calcified bone as opposed to soft bone. Next it is surounded by brain tissue (undoubtable a vital organ) but provides cushion and sheilding to the pituitary. Finally the pituitary sits in a bony structure called the sella turcica or hypophyseal fossa. It is essentiall a little bowl that sits just between your eyes about 1/2-1 inch back in the head. The top of the bowl which holds the pituitary is finally not exposed to the air like a bowl of snap-krackly-pop, but protected again by a thick (relatively speaking) layer of dura mater akin to like a thousand layers of cellophane. With all these protective mechanisms, it becomes clear that a tumor in this location is a bad, bad thing.

So what does it mean to be pan-hypopit? It means that Eli does not have any pituitary function? So what? Well this is what he is missing, how it is replaced and how these deficiencies affect him.

1. ADH (Anti-diuretic hormone, also known as vasopressin): Without this hormone which is made in the hypothalymus and secreted by the posterior pituitary you cannot control your urine output and without replacement (or massive re-hydration) you will pee-yourself-to-death.
Replacement:
Eli take Desmopressin twice a day. It is a little pill that we crush up and mix with cherry syrup (yum! - aka Eli had to be brave!). It essentially decreases his urine output to very little. When the meds start to wear off we all know because he starts to drink massive amounts of water and ask to go "potty" every 30 minutes. As he grows older he will be able to compensate for his urine loss by drinking a lot, and will not necessarily have to be on the medication (if he so chooses). But right now his body cannot compensate for the loss.
2. Hydrocortisone (cortisone, glucocorticoids): Also secreted by the pituitary. This hormone is responsible for responding to stree: illnesses, falls, test-anxiety etc. I calls into action a whole array of other hormones and processes that I don't fully understand (we do Endocrin next year).
Replacement:
Again twice a day in the same manner. The only difference with this medication is that if he gets a bad cold, has a bad allergic reaction or a serious trauma, his body does not compensate by increasing its natural levels of hydrocortison, so we either augment this with aditional doses of his oral medication, or in severe cases we give his injections of cortisone. Thankfully, that has not happened yet.
3. Thyroid Stimulating Hormone (TSH). Replaced by Synthroid/thyroxine. Again I do not know what the details of this hormone, but it is responsibel for regulating metabolism, food intake, muscle building, fat metabolism etc. More importantly it is essential for normal growth of the brain, especially during the first 3 years of life, but also for later development. Deficiency can lead to growth delays and mental insufficiencies.
Replacement:
Once a day...and this is kinda cool, the dose is dependant on his body surface area (weight x hight essentially). Since his hight and weight has not really changed in the past year and will not change for about another year it is easy to regulate the dosing. Why????
4. Growth Hormone (GH): Release from the anterior pituitary, GH is responisble for, surprise, helping you to grow (not only taller which is the commonly understood affect, but it also helps your internal organs and brain grow and develop). It is also important for building muscle and regulating fat metabolism (ie preventing fat-ness). There are many other benefits to GH which is why it is the new rave in anti-aging. Hence while I thought that it was only really necessary during childhood and through adolesence, it is actually important for health throughout your life. Therefore Eli will need to be on GH replacement forever...just like all his other medications.
Replacement:
This cannot start until the tumor is more or less gone or on its way out! Because GH causes pretty much every cell in your body to grow. Therefore, we hope to start GH in 6-12 months. Until then he will remain my "little mouse" (currently he weights 26.4 lbs (he gained a whole pound over the past 3 months...huge deal) and is about 33 inches tall. When he was 18 month he was 33 inches tall and weight 24 lbs).
Beginning treatment will be exciting and a good thing, but also will be very hard and sad...it requires injection (very much like diabetic insulin injections) everyday for ever. So it will be analygous to being a little kid diagnosed with insulin-dependant diabetes.
5. FHS and LH --> Testosterone and Sperm: When the time comes for Eli to go through puberty, he will need a little help. Because his pituitary does not secrete LH (Leutinizing Hormone), there is no signal to tell his little testicles to make testosterone. Therefore, he will not develope male sexual characteristics and all that happens when adolescents go through puberty (like deepening voice and facial/body hair - hey maybe he won't be as hairy as his dad!!!).
Replacement:
Injections of testosterone...forever.

I think that is actually it. Clearly he has quite a regiment. He has become very compliant and does not even fuss when it is time for his meds, although the taste still makes him squirm! As with everything in life, he will become accustomed to the new treatments and they will become routine, like brushing his teeth. Hopefully, because he is so young, he will not know any different and in the long run that may make management easier.

Of couse we will keep you posted as to when new treatments start and how he is managing them. If you have any suggestions for teaching a child about giving themselves and receiving dailing injections we would love the advice (I am a little nervous). Also, I am sure as I learn more about his condition there will be more of these little anatomy and physiology sessions...hope I didn't bore you.