Sunday, November 15, 2009
Just Life - An Update
Non-Tumor Life
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.
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
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
Monday, July 13, 2009
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 c
ollapse 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
Thursday, May 14, 2009
A Diagnosis - Long time coming
For a long time I have wanted to write about what it was like in the days preceding Eli's diagnosis as well as elaborate a bit on Eli's tumor. As many of you know, I had always been concerned that something was not quite right with him. I saw specialist after specialist and everyone thought he was just fine...he was just so close to normal no one ever thought he could have a tumor!
For one of my classes in medical school we were instructed to write a narrative about being a patient and what our experience was like. Needless to say I decided to write about my experience with Eli. The stream of conscious essay that follows should be taken in this context. Also, I want to emphasize that while the doctor who diagnosed Eli with a brain tumor was very sure of his illness, all the specialist we saw in the months preceding did not have all the pieces of the story. First, many of his signs and symptoms had not presented yet, and others I under appreciated and did not tell the doctors about. We do not feel that any doctor "should" have picked this up! It is a very rare condition. What we do wish, what I wish, is that a mother's earnest concern about her child was taken more seriously. If this had occurred earlier in his life, he would probably still have pituitary function! Unfortunately, a parent's concern for their child, and most often a mother's intuition, is not taken seriously...and mother's really do know best. The week before Eli was diagnosed I attended a medical school lecture that featured a mom and her daughter. The daughter had some obscure genetic abnormality that no one could figure out. The little girl was very sick. The moral of the lecture was the importance of listening to the parents about their children, what they are like and how they feel. In the 5-15 minutes that a doctor spends with a patient they cannot possible know what is going on with a child better than their parent (providing the parent is a decent observer and not crazy!) After the lecture I said to myself..."yeah I guess". Two weeks later my own instincts about my child were confirmed. I hope that in my career as a doctor I always remember this lesson, and really listen to what my patients are telling me.
The Day Eli was Diagnosed
On the day Eli was born, we welcomed the most beautiful, the most perfect little boy into our world. From beneath the newness of his skin and redness of his swollen eye lids, an ageless and ancient soul emanated. While admiring his inner resonance, we noted that his hands looked just like daddy and his nose like mommy. The nurses counted his fingers and toes, examined his skin and spin, the mundane check-up of the newborn. All was in order. He was our “perfect” baby boy.
Everyone was jealous of us. Eli was such an easy baby. He was happy, inquisitive and had an amazing calmness. With every passing day we watched and waited for the “milestones” of infanthood. The smiles came at 4 weeks, the rolling over by 8-12 week, then cooing and babbling began. He sat up unassisted by five months and at five and a half used his first sign from the sign-language we were teaching him, “milky”. We continued to watch and wait for him to really get moving, to crawl, walk, run…but they were slower to come. His vocabulary by a year was astounding 50+ words (including sign language), he loved to read, listen to music and of course…talk! But he never crawled, did not scoot, never rolled across the floor and seemed to be uncomfortable when forced to be in a quadruped position. I demanded he receive physical therapy in spite of the remarks from his doctors: “He’ll catch up. The spectrum of normal is a bell-curve. He’s perfectly normal!” But he wasn’t. I knew he wasn’t. His gross motor skills continued to lag, I wanted further evaluation and so again demanded we see an orthopedist. She found nothing really wrong, but suggested we see a neurologist. My PCP declined that request.
He remained a very easy baby and toddler. He never ran into the street (he couldn’t run), he never hit, or bit or had a temper-tantrum or even got really upset! Everyone told me it was because I was such a good mom, he didn’t have a reason to be angry or act out. I thought that was ridiculous. All children have cause to rebel, unless something is wrong with them. I again requested we see a neurologist, my new PCP, although not convinced he needed the evaluation was accommodating. The neurologist found no real problems. I was almost convinced, convinced that I was just plain wrong about any underlying medical problem. My family, our doctors (pediatrician, neurologist, ophthalmologist, orthopedist) all agreed: “He was just fine.” My husband was the only one who agreed when I said: “But he’s not like other children. There is something wrong with him!”
In June of his second year Eli started to vomit. At first we thought it was a cold, he had always been a puker. You know, some kids get fevers, some get ear infections, Eli vomits. He had bouts of emesis nearly daily for 2 weeks. It stopped and he was well for 10-14 days before he began vomiting again, this time he only vomited for 1-3 times over a 1-2 day period. Then he would be miraculously well. This pattern continued for 5 months. We brushed it off to starting preschool and being infected by all those germy little kids. He was fine. I had now been convinced I was just wrong about him.
On December 31st, 2008 while visiting family in New Jersey, Eli, now 2 years and 3 months old, started vomiting again. But this time it was different. It did not stop. “Mommy, mommy my head hurts,” he sobbed clinging to his blankie and holding it up to his temples. After a brief visit with a local pediatrician, Eli was admitted to “Holy Hell” hospital for severe dehydration. I explained Eli’s entire history, including recent episodes of polydipsia and polyuria, so extreme that in a 20 hour period he drank nearly 70 ounces of water (children his size should not drink more that 24 oz in 24 hours). This event happened two days prior to his hospitalization. Both the headaches, the previous vomiting episodes, his urinating were all ignored by the attending physician.
He was worked up for gastroenteritis, put on IV fluids and antibiotics. He did not improve. After spending the night in the hospital and spending the first 6 hours of the morning wiping bilious vomit from my son’s lips I demanded further evaluation (Why did I keep on having to demand care for my son? Why did I feel I was always being demanding?) Intussusception was next diagnosis to bat. I argued with the attending, “but he does not have abdominal pain. He said his head hurt.” She assured me that it was common for patients to have non-painful intussusception, in fact she had a case just last week. They abdominal x-ray was negative so they moved on to a diagnosis of bowl impaction. But I said “he just pooped yesterday and he has never had problems with his bowel movements.” The general surgeon assured me that feces can be passed even when there is a blockage. I looked at the scans with the doctor. He showed me that Eli’s ascending colon was collapsed and showed me the blockage that was backed up from his sigmoid to his left colic flexure. Okay I said. Let’s get this kid an enema! It did not make sense to me, I was just a first year medical student, what the hell did I know? I had NO idea what was wrong with Eli. I just knew that things were not adding up. So I called every doctor I knew. I did not trust the staff taking care of us, but did not know enough, or was not lucid enough to advocate for a transfer to another hospital.
Two hours after the enema Eli had a massive grand mal seizure. I saw it coming immediately. I thought that he had been having absence seizures all day, but the nurses told me “Oh kids do all kinds of things to manage what is happening to them. He’s okay.”
The seizure began with total body stiffening, convulsions, eye blinking and uncontrollably lip smacking. I screamed out of the room and into the intercom: “He is having a seizure, a seizure. He’s seizing.” His face was stiff, his skin taught, his neck veins and chest wall protruded from beneath the hospital gown as his body convulsed. Just the previous week I was shadowing at Children’s Hospital and witnessed a child have a seizure…it was terrible. It was nothing compared to Eli’s. I tried to remember the calm of that little boy’s mother. Her example gave me the strength to maintain my composure. While inside my blood boiled with fear and anger and I wanted to scream bloody murder, outside I remained calm and holding back my tears I gently said “Look at mommy Eli, look at mommy… I am right here.” He stared right through me. “Come back to mommy Eli come back to mommy. I’m right here. I love you bubby.” His body shook in my arms like stiff wooden boards, his neck did not bend, his eyes remained peeled open, as though he was searching for his way back to my voice. “Mommy’s right here Eli, it’s okay, you’re gonna be okay. I’m right here. You’re gonna be okay.” I do not know how many more times I said it out loud but the words “you’re gonna be okay baby” pervaded my thoughts. It was more of a prayer than anything else…and I don’t pray!
Completely untrusting of the competence of the attending and hospital staff I called my pediatrician back in Colorado. It was 11pm in Denver and therefore I got the physician on call. He happened to be the only doctor in the practice I did not know. I did not care! As Eli lay in my arms seizing, I gave him the minute-by-minute account of what was happening. He asked me if they were ordering a Chem 10, I said “are you ordering a Chem 10?” The nurses looked at me blankly and replied a “A chem 7 with the addition of magnesium and phosphate.” He asked if they had called a code to alert the hospital of the emergency…again when I asked they looked confused. My pediatrician stayed on the phone with me for the next hour. He kept me sane, informed me what should be happening, what tests to make sure they ordered, what should be done post-seizure, answered all my questions and mainly just sat there and let me breath into the phone. He saved my life that night.
Eli received two doses of ativan 5 minutes apart, but that did not stop the seizure. His lip smacking and eye blinking disappeared, but his body continued to struggle. For whatever completely unacceptable and still unfathomable reason, it took the hospital 1 hour and 32 minutes to get Eli’s phenobarb drip started. He finally stopped seizing. We were transferred to the PICU at Hackensack University Medical Center (HUMC) within the next hour. Eli’s “Chem 7 + 3” came back. His sodium was 109 (P.S...That's BAD).
Upon admittance to HUMC we were greeted by 2 pediatric intensive care doctors (PICU docs). Within the hour we had four doctors at Eli’s bedside. Finally, I thought…finally someone is going to help us…finally Eli is going to get the care he needs, the care he has been waiting 2 years to receive. Finally someone is going to take all the little pieces of Eli’s story, and put them together. So what are all the pieces and what picture do they paint. His current and past history is pertinent for the following, in no particular order of importance:
1. Gross and some fine motor developmental delay (9-12 months delayed)
2. Socially/Intellectually precocious (9-12 months ahead)
3. Low activity level. Low threshold for physical stresses/challenges.
4. Intermittent unexplained vomiting.
5. No or minimal growth in height or weight since 18 months of age (that’s almost a YEAR)
6. Progressively worsening polydipsia and polyuria over 10 months with an extreme episode on Dec 28th 2008 (5 days ago).
7. Exotropic strabismus with corrective surgery on 5/08. (Note family history for strabismus in mother and maternal grandmother.)
8. Enlarged head circumference for body size.
9. Misshapen head.
10. Blood work negative for a metabolic disorder.
11. Negative x-rays for hip dysplasia and intussesseption
12. Low muscle mass, without decreased tone (not hypotonic)
13. Hyponatremic –despite IV fluids with sodium chloride.
14. Seizure (most likely due to the 109 Na+).
15. Lastly (and not unimportant) his mother’s heartfelt belief that there was something not right about her son, and had not been right since he was 3 months old.
After a CT scan Eli was diagnosed with a large craniopharyngioma (a non-malignant brain tumor in a “malignant location”). It had destroyed his pituitary gland and compressed his right optic nerve to the point of total atrophy, the extent of which would not become apparent to us for another 4 months. I asked the intensivist caring for Eli if he had an idea before the CT that Eli had a brain tumor. He said that after hearing in detail all of Eli’s medical problems over the last two years, he knew. In a thick New York accent he replied bluntly, “Well, it was all in the patient’s history.”
For one of my classes in medical school we were instructed to write a narrative about being a patient and what our experience was like. Needless to say I decided to write about my experience with Eli. The stream of conscious essay that follows should be taken in this context. Also, I want to emphasize that while the doctor who diagnosed Eli with a brain tumor was very sure of his illness, all the specialist we saw in the months preceding did not have all the pieces of the story. First, many of his signs and symptoms had not presented yet, and others I under appreciated and did not tell the doctors about. We do not feel that any doctor "should" have picked this up! It is a very rare condition. What we do wish, what I wish, is that a mother's earnest concern about her child was taken more seriously. If this had occurred earlier in his life, he would probably still have pituitary function! Unfortunately, a parent's concern for their child, and most often a mother's intuition, is not taken seriously...and mother's really do know best. The week before Eli was diagnosed I attended a medical school lecture that featured a mom and her daughter. The daughter had some obscure genetic abnormality that no one could figure out. The little girl was very sick. The moral of the lecture was the importance of listening to the parents about their children, what they are like and how they feel. In the 5-15 minutes that a doctor spends with a patient they cannot possible know what is going on with a child better than their parent (providing the parent is a decent observer and not crazy!) After the lecture I said to myself..."yeah I guess". Two weeks later my own instincts about my child were confirmed. I hope that in my career as a doctor I always remember this lesson, and really listen to what my patients are telling me.
The Day Eli was Diagnosed
On the day Eli was born, we welcomed the most beautiful, the most perfect little boy into our world. From beneath the newness of his skin and redness of his swollen eye lids, an ageless and ancient soul emanated. While admiring his inner resonance, we noted that his hands looked just like daddy and his nose like mommy. The nurses counted his fingers and toes, examined his skin and spin, the mundane check-up of the newborn. All was in order. He was our “perfect” baby boy.
Everyone was jealous of us. Eli was such an easy baby. He was happy, inquisitive and had an amazing calmness. With every passing day we watched and waited for the “milestones” of infanthood. The smiles came at 4 weeks, the rolling over by 8-12 week, then cooing and babbling began. He sat up unassisted by five months and at five and a half used his first sign from the sign-language we were teaching him, “milky”. We continued to watch and wait for him to really get moving, to crawl, walk, run…but they were slower to come. His vocabulary by a year was astounding 50+ words (including sign language), he loved to read, listen to music and of course…talk! But he never crawled, did not scoot, never rolled across the floor and seemed to be uncomfortable when forced to be in a quadruped position. I demanded he receive physical therapy in spite of the remarks from his doctors: “He’ll catch up. The spectrum of normal is a bell-curve. He’s perfectly normal!” But he wasn’t. I knew he wasn’t. His gross motor skills continued to lag, I wanted further evaluation and so again demanded we see an orthopedist. She found nothing really wrong, but suggested we see a neurologist. My PCP declined that request.
He remained a very easy baby and toddler. He never ran into the street (he couldn’t run), he never hit, or bit or had a temper-tantrum or even got really upset! Everyone told me it was because I was such a good mom, he didn’t have a reason to be angry or act out. I thought that was ridiculous. All children have cause to rebel, unless something is wrong with them. I again requested we see a neurologist, my new PCP, although not convinced he needed the evaluation was accommodating. The neurologist found no real problems. I was almost convinced, convinced that I was just plain wrong about any underlying medical problem. My family, our doctors (pediatrician, neurologist, ophthalmologist, orthopedist) all agreed: “He was just fine.” My husband was the only one who agreed when I said: “But he’s not like other children. There is something wrong with him!”
In June of his second year Eli started to vomit. At first we thought it was a cold, he had always been a puker. You know, some kids get fevers, some get ear infections, Eli vomits. He had bouts of emesis nearly daily for 2 weeks. It stopped and he was well for 10-14 days before he began vomiting again, this time he only vomited for 1-3 times over a 1-2 day period. Then he would be miraculously well. This pattern continued for 5 months. We brushed it off to starting preschool and being infected by all those germy little kids. He was fine. I had now been convinced I was just wrong about him.
On December 31st, 2008 while visiting family in New Jersey, Eli, now 2 years and 3 months old, started vomiting again. But this time it was different. It did not stop. “Mommy, mommy my head hurts,” he sobbed clinging to his blankie and holding it up to his temples. After a brief visit with a local pediatrician, Eli was admitted to “Holy Hell” hospital for severe dehydration. I explained Eli’s entire history, including recent episodes of polydipsia and polyuria, so extreme that in a 20 hour period he drank nearly 70 ounces of water (children his size should not drink more that 24 oz in 24 hours). This event happened two days prior to his hospitalization. Both the headaches, the previous vomiting episodes, his urinating were all ignored by the attending physician.
He was worked up for gastroenteritis, put on IV fluids and antibiotics. He did not improve. After spending the night in the hospital and spending the first 6 hours of the morning wiping bilious vomit from my son’s lips I demanded further evaluation (Why did I keep on having to demand care for my son? Why did I feel I was always being demanding?) Intussusception was next diagnosis to bat. I argued with the attending, “but he does not have abdominal pain. He said his head hurt.” She assured me that it was common for patients to have non-painful intussusception, in fact she had a case just last week. They abdominal x-ray was negative so they moved on to a diagnosis of bowl impaction. But I said “he just pooped yesterday and he has never had problems with his bowel movements.” The general surgeon assured me that feces can be passed even when there is a blockage. I looked at the scans with the doctor. He showed me that Eli’s ascending colon was collapsed and showed me the blockage that was backed up from his sigmoid to his left colic flexure. Okay I said. Let’s get this kid an enema! It did not make sense to me, I was just a first year medical student, what the hell did I know? I had NO idea what was wrong with Eli. I just knew that things were not adding up. So I called every doctor I knew. I did not trust the staff taking care of us, but did not know enough, or was not lucid enough to advocate for a transfer to another hospital.
Two hours after the enema Eli had a massive grand mal seizure. I saw it coming immediately. I thought that he had been having absence seizures all day, but the nurses told me “Oh kids do all kinds of things to manage what is happening to them. He’s okay.”
The seizure began with total body stiffening, convulsions, eye blinking and uncontrollably lip smacking. I screamed out of the room and into the intercom: “He is having a seizure, a seizure. He’s seizing.” His face was stiff, his skin taught, his neck veins and chest wall protruded from beneath the hospital gown as his body convulsed. Just the previous week I was shadowing at Children’s Hospital and witnessed a child have a seizure…it was terrible. It was nothing compared to Eli’s. I tried to remember the calm of that little boy’s mother. Her example gave me the strength to maintain my composure. While inside my blood boiled with fear and anger and I wanted to scream bloody murder, outside I remained calm and holding back my tears I gently said “Look at mommy Eli, look at mommy… I am right here.” He stared right through me. “Come back to mommy Eli come back to mommy. I’m right here. I love you bubby.” His body shook in my arms like stiff wooden boards, his neck did not bend, his eyes remained peeled open, as though he was searching for his way back to my voice. “Mommy’s right here Eli, it’s okay, you’re gonna be okay. I’m right here. You’re gonna be okay.” I do not know how many more times I said it out loud but the words “you’re gonna be okay baby” pervaded my thoughts. It was more of a prayer than anything else…and I don’t pray!
Completely untrusting of the competence of the attending and hospital staff I called my pediatrician back in Colorado. It was 11pm in Denver and therefore I got the physician on call. He happened to be the only doctor in the practice I did not know. I did not care! As Eli lay in my arms seizing, I gave him the minute-by-minute account of what was happening. He asked me if they were ordering a Chem 10, I said “are you ordering a Chem 10?” The nurses looked at me blankly and replied a “A chem 7 with the addition of magnesium and phosphate.” He asked if they had called a code to alert the hospital of the emergency…again when I asked they looked confused. My pediatrician stayed on the phone with me for the next hour. He kept me sane, informed me what should be happening, what tests to make sure they ordered, what should be done post-seizure, answered all my questions and mainly just sat there and let me breath into the phone. He saved my life that night.
Eli received two doses of ativan 5 minutes apart, but that did not stop the seizure. His lip smacking and eye blinking disappeared, but his body continued to struggle. For whatever completely unacceptable and still unfathomable reason, it took the hospital 1 hour and 32 minutes to get Eli’s phenobarb drip started. He finally stopped seizing. We were transferred to the PICU at Hackensack University Medical Center (HUMC) within the next hour. Eli’s “Chem 7 + 3” came back. His sodium was 109 (P.S...That's BAD).
Upon admittance to HUMC we were greeted by 2 pediatric intensive care doctors (PICU docs). Within the hour we had four doctors at Eli’s bedside. Finally, I thought…finally someone is going to help us…finally Eli is going to get the care he needs, the care he has been waiting 2 years to receive. Finally someone is going to take all the little pieces of Eli’s story, and put them together. So what are all the pieces and what picture do they paint. His current and past history is pertinent for the following, in no particular order of importance:
1. Gross and some fine motor developmental delay (9-12 months delayed)
2. Socially/Intellectually precocious (9-12 months ahead)
3. Low activity level. Low threshold for physical stresses/challenges.
4. Intermittent unexplained vomiting.
5. No or minimal growth in height or weight since 18 months of age (that’s almost a YEAR)
6. Progressively worsening polydipsia and polyuria over 10 months with an extreme episode on Dec 28th 2008 (5 days ago).
7. Exotropic strabismus with corrective surgery on 5/08. (Note family history for strabismus in mother and maternal grandmother.)
8. Enlarged head circumference for body size.
9. Misshapen head.
10. Blood work negative for a metabolic disorder.
11. Negative x-rays for hip dysplasia and intussesseption
12. Low muscle mass, without decreased tone (not hypotonic)
13. Hyponatremic –despite IV fluids with sodium chloride.
14. Seizure (most likely due to the 109 Na+).
15. Lastly (and not unimportant) his mother’s heartfelt belief that there was something not right about her son, and had not been right since he was 3 months old.
After a CT scan Eli was diagnosed with a large craniopharyngioma (a non-malignant brain tumor in a “malignant location”). It had destroyed his pituitary gland and compressed his right optic nerve to the point of total atrophy, the extent of which would not become apparent to us for another 4 months. I asked the intensivist caring for Eli if he had an idea before the CT that Eli had a brain tumor. He said that after hearing in detail all of Eli’s medical problems over the last two years, he knew. In a thick New York accent he replied bluntly, “Well, it was all in the patient’s history.”
Our Little Boy is BACK!!!
The shunt surgery went very smoothly (our Neurosurgeon is god!!!) and Eli is doing great. We have not seen him feeling this good in months. His energy level is way up and even when he is tired he stays up talking and singing in bed. He is also just to happy. It is a clear indication of just how bad he felt before.
Currently he is way into baking! His khaaleh (auntie) Marji sent him a recipe for no-kneed-bread and he has been enjoying making that every other day (literally). We are also growing flowers and herbs- he loves watching the little seeds sprout into leaves and flowers. He is amazingly patient with the entire growing process too...so optimistic. We have been trying to sprout an avocado seed for 3 weeks now. Jacob and I have given up, but everyday Eli says "It's gonna grow soon", too endearing.
He is still enjoying chocolate ice cream, chocolate pudding, chocolate cake, chocolate whatever...his Papa (my dad) is thrilled. Papa is also thrilled because Eli appears to have a liking for GOLF!!!
We are very hopeful that Eli is on the road to recovery and we will be spending substantially less time at the hospital now. The collapsing of the tumor while WONDERFUL, is just the beginning. Now it is a 25 year wait-and-see game. Essentially, there is about a 20% chance that all the tumor cells will die and not regrow. However if just one little cell survives it can regrow into a tumor. This can take up to 25 years. Therefore, Eli will be getting MRI's every 3 months for the next few years and then 1 MRI per year for the next few decades! Only time will tell. If the tumor does regrow we will have to intervene again with either radiation or surgery. Hopefully IF this happens it will be a long long long time.
For now we are excited to enjoy a summer of freedom and fun. I am particularly thrilled to be able to spend more time with him.
We wish all of you a happy and safe summer.
Love,
Meagan, J and E
Currently he is way into baking! His khaaleh (auntie) Marji sent him a recipe for no-kneed-bread and he has been enjoying making that every other day (literally). We are also growing flowers and herbs- he loves watching the little seeds sprout into leaves and flowers. He is amazingly patient with the entire growing process too...so optimistic. We have been trying to sprout an avocado seed for 3 weeks now. Jacob and I have given up, but everyday Eli says "It's gonna grow soon", too endearing.
He is still enjoying chocolate ice cream, chocolate pudding, chocolate cake, chocolate whatever...his Papa (my dad) is thrilled. Papa is also thrilled because Eli appears to have a liking for GOLF!!!
We are very hopeful that Eli is on the road to recovery and we will be spending substantially less time at the hospital now. The collapsing of the tumor while WONDERFUL, is just the beginning. Now it is a 25 year wait-and-see game. Essentially, there is about a 20% chance that all the tumor cells will die and not regrow. However if just one little cell survives it can regrow into a tumor. This can take up to 25 years. Therefore, Eli will be getting MRI's every 3 months for the next few years and then 1 MRI per year for the next few decades! Only time will tell. If the tumor does regrow we will have to intervene again with either radiation or surgery. Hopefully IF this happens it will be a long long long time.
For now we are excited to enjoy a summer of freedom and fun. I am particularly thrilled to be able to spend more time with him.
We wish all of you a happy and safe summer.
Love,
Meagan, J and E
Thursday, May 7, 2009
Like the weather in Colorado - Eli's health is always changing!
Well, that was a short vacation!!!
Eli had a wonderful day yesterday. He was feeling great. He walked all the way to the park with his Meme, played on the playground and was feeling happy. Then in the evening we went for dinner, and he played in a little fountain by the restaurant for an hour, just splashing around, making foot-prints and chatting with the other kids. We were so happy to see him finally feeling better. "We have our little boy back" we thought to ourselves. Well, it was not to last.
This morning Eli awoke from a poor nights sleep and promptly vomited after eating breakfast. We knew right away what was going on.
One of the major risk factors of the tumor collapsing was having the VP shunt (that drains fluid off his brain and into his abdomen) fail...and it did. When the tumor collapsed it displaced the position of the shunt and now it is not working. So we are back in the hospital for another shunt revision. Hopefully this one will last a while.
So, not much else to say but, #$@%^&%.
Thank you all for your emails and thoughts...they really keep us going!!!
Wishing all you mom's a Happy Mother's Day.
Meagan and Jacob
Eli had a wonderful day yesterday. He was feeling great. He walked all the way to the park with his Meme, played on the playground and was feeling happy. Then in the evening we went for dinner, and he played in a little fountain by the restaurant for an hour, just splashing around, making foot-prints and chatting with the other kids. We were so happy to see him finally feeling better. "We have our little boy back" we thought to ourselves. Well, it was not to last.
This morning Eli awoke from a poor nights sleep and promptly vomited after eating breakfast. We knew right away what was going on.
One of the major risk factors of the tumor collapsing was having the VP shunt (that drains fluid off his brain and into his abdomen) fail...and it did. When the tumor collapsed it displaced the position of the shunt and now it is not working. So we are back in the hospital for another shunt revision. Hopefully this one will last a while.
So, not much else to say but, #$@%^&%.
Thank you all for your emails and thoughts...they really keep us going!!!
Wishing all you mom's a Happy Mother's Day.
Meagan and Jacob
Tuesday, May 5, 2009
Hooray! He finally caught a break.
The tumor has collapsed!!
Collapsed mood = collapsed tumor. Whew!
Over the past week we were becoming increasingly concerned about Eli. His chemo-treatments were getting harder for him, seemed more painful and he had headaches afterward. Additionally, because he had been back at school, he had contracted a cold, had vomiting and diarrhea for 4-5 days and that was followed by cough and general runny nose and congestion. All of these factors would make anyone sad and depressed, and we began to see such signs in Eli. We weren't quite sure what was going on but it was really starting to seem like he had reached his limit and was becoming depressed. He cried for almost no reason, he was easily distracted and had lost interest in activities he used to enjoy. One evening, after telling us all day that he did not feel good, was tired, was sad, he said, "Mommy I feel terrible." Wow!!! What 2 year old uses such words? We were really concerned and when we went in for chemo on monday we talked to the social workers about books we could get and things we could do to help improve his mood.
Well, as is usually the case with Eli, there was an underlying physical reason for his mood: we didn't know it yet, but the tumor in his head was beginning to collapse and was causing him some real discomfort. When we went in for his treatment on Monday we talked about our concerns with his oncologist, Dr. Foreman, and we decided to continue with treatment and start drawing a little more fluid off the tumor cyst to try to drain it. As we had seen recently, when the fluid was being drawn off the tumor, Eli was clearly VERY uncomfortable. Then as Dr. Foreman began to draw off the extra fluid, we saw blood in the line! There was a stunned silence - other than Eli crying. What the hell was going on? Then things got really scary. Eli started slurring and saying his mouth hurt and he got very pale and and suddenly weak. Everyone went in to crisis mode and we quickly went down to get the CT scan in the picture above. The CT was done as a precaution to make sure that there was nothing terribly wrong with his brain. We had no idea what had happened, nor did we have any idea how encouraging the results of the scan would be.
When we got back upstairs the nurse practitioner gave us the wonderful news that the tumor had collapsed! We had unexpectedly already drained all of the fluid and the cyst had completely deflated. The picture above shows 4 slices through Eli's head comparing a CT on 4/15/2009 (left) to the one on monday 5/4/2009 (right). I think it is quite obvious that the enormous egg which just weeks ago inhabited his skull has been thoroughly squashed. This seems to explain why he had been so uncomfortable during this most recent round of chemo: the shrinking tumor was messing with his brain (pulling and moving things around) and causing him substantial discomfort.
We are so relieved and happy. The tumor is not gone, but the bulk of it, which has caused so much damage, has probably been eliminated. This was the best outcome we could have hoped for (at least up to this point) for this treatment. We know it is unlikely that this will cure him of the tumor but it is a very slow-growing (non-malignant!) tumor and it will give Eli some time for his brain to develop before he has to have surgery or radiation to remove what is left.
Now we just wait and get the tumor scanned every so often to see if it's growing. No more chemo (no more "counting with Dr. Foreman," as Eli says), we won't be at the hospital 3 times a week and hopefully we can live relatively normally for awhile. Eli will have an MRI in three weeks to give us a really good picture of what is really left of the tumor. We will have a better idea then of how long we'll wait before the next scan and what the likelihood is that the cyst will re-grow anytime soon.
Eli is happy and seems to be feeling much better today. Hopefully he will continue to feel better and better. Maybe we can take the summer off from the hospital and really enjoy being back in Colorado, together.
Meagan, Jacob and Eli
Collapsed mood = collapsed tumor. Whew!
Over the past week we were becoming increasingly concerned about Eli. His chemo-treatments were getting harder for him, seemed more painful and he had headaches afterward. Additionally, because he had been back at school, he had contracted a cold, had vomiting and diarrhea for 4-5 days and that was followed by cough and general runny nose and congestion. All of these factors would make anyone sad and depressed, and we began to see such signs in Eli. We weren't quite sure what was going on but it was really starting to seem like he had reached his limit and was becoming depressed. He cried for almost no reason, he was easily distracted and had lost interest in activities he used to enjoy. One evening, after telling us all day that he did not feel good, was tired, was sad, he said, "Mommy I feel terrible." Wow!!! What 2 year old uses such words? We were really concerned and when we went in for chemo on monday we talked to the social workers about books we could get and things we could do to help improve his mood.Well, as is usually the case with Eli, there was an underlying physical reason for his mood: we didn't know it yet, but the tumor in his head was beginning to collapse and was causing him some real discomfort. When we went in for his treatment on Monday we talked about our concerns with his oncologist, Dr. Foreman, and we decided to continue with treatment and start drawing a little more fluid off the tumor cyst to try to drain it. As we had seen recently, when the fluid was being drawn off the tumor, Eli was clearly VERY uncomfortable. Then as Dr. Foreman began to draw off the extra fluid, we saw blood in the line! There was a stunned silence - other than Eli crying. What the hell was going on? Then things got really scary. Eli started slurring and saying his mouth hurt and he got very pale and and suddenly weak. Everyone went in to crisis mode and we quickly went down to get the CT scan in the picture above. The CT was done as a precaution to make sure that there was nothing terribly wrong with his brain. We had no idea what had happened, nor did we have any idea how encouraging the results of the scan would be.
When we got back upstairs the nurse practitioner gave us the wonderful news that the tumor had collapsed! We had unexpectedly already drained all of the fluid and the cyst had completely deflated. The picture above shows 4 slices through Eli's head comparing a CT on 4/15/2009 (left) to the one on monday 5/4/2009 (right). I think it is quite obvious that the enormous egg which just weeks ago inhabited his skull has been thoroughly squashed. This seems to explain why he had been so uncomfortable during this most recent round of chemo: the shrinking tumor was messing with his brain (pulling and moving things around) and causing him substantial discomfort.
We are so relieved and happy. The tumor is not gone, but the bulk of it, which has caused so much damage, has probably been eliminated. This was the best outcome we could have hoped for (at least up to this point) for this treatment. We know it is unlikely that this will cure him of the tumor but it is a very slow-growing (non-malignant!) tumor and it will give Eli some time for his brain to develop before he has to have surgery or radiation to remove what is left.
Now we just wait and get the tumor scanned every so often to see if it's growing. No more chemo (no more "counting with Dr. Foreman," as Eli says), we won't be at the hospital 3 times a week and hopefully we can live relatively normally for awhile. Eli will have an MRI in three weeks to give us a really good picture of what is really left of the tumor. We will have a better idea then of how long we'll wait before the next scan and what the likelihood is that the cyst will re-grow anytime soon.
Eli is happy and seems to be feeling much better today. Hopefully he will continue to feel better and better. Maybe we can take the summer off from the hospital and really enjoy being back in Colorado, together.
Meagan, Jacob and Eli
Thursday, April 23, 2009
Developmental Milestones
Eli has a made a monumental leap forward in his development. Every child crosses this milestone at some point, but like many other things for Eli, it had been delayed.
Eli has discovered ICE CREAM!
Eli has discovered ICE CREAM!
Thursday, April 16, 2009
Back on track - started bleomycin treatment again.
After a CT scan to check out Eli's head, he was given the go-ahead to start treatment again.
We started the first of 9 doses yesterday and will go every MWF for the next three weeks.
The plan is to be slightly more aggressive with the drainage of the tumor this round in hopes that the tumor tissue has been weakened by the first round of chemo. Our hope this round is that by taking off a teaspoon of fluid each day we can shrink the tumor somewhat. We don't want to go too fast because the space created when removing the fluid has to be filled-in and we don't want to collapse it too fast or it could cause a hemorrage. Our eventual hope (probably next round) is that the cyst of the tumor will collapse completely and be unable to re-form.
We started the first of 9 doses yesterday and will go every MWF for the next three weeks.
The plan is to be slightly more aggressive with the drainage of the tumor this round in hopes that the tumor tissue has been weakened by the first round of chemo. Our hope this round is that by taking off a teaspoon of fluid each day we can shrink the tumor somewhat. We don't want to go too fast because the space created when removing the fluid has to be filled-in and we don't want to collapse it too fast or it could cause a hemorrage. Our eventual hope (probably next round) is that the cyst of the tumor will collapse completely and be unable to re-form.
Sunday, April 12, 2009
"Mommy I think I lost my eyes!"
Thus spoke Eli after wearing his newly adorned "pirate" patch. Clearly (no pun intended), he could not see very well!
As many of you know Eli has always had "eye issues". Initially he had strabismus (look it up of you want), I have it a little, my mom had it a lot, so we figured it was genetic. Now in retrospect we know it was the tumor causing the problems. We used to have to patch Eli in hopes of strengthening the eye muscles so that he would not loose his vision due to the added effort of using a weak eye. Well we are at it again, in hopes of saving any vision he has in his right eye.
Over the past months, because of the pressure the tumor has placed on the optic nerve, he is loosing vision at a rapid rate. We hope it has stabilized since the pressure has been relived due to the decrease in hydrocephalus (head full of water/CSF) which give the tumor more room. You may be asking why pressure hurts a nerve. Well, a nerve, in this situation is like a big blood vessel. If you pinch it, you loose the flow or nutrients/blood to the eye and it atrophies and dies, as with any tissue or organ in your body when blood flow is restricted (hence why we should not smoke, watch our blood pressure watch our weight and get exercise so as to keep all our little and big arteries unobstructed).
When we went to the doctor to talk about his vision, he said that the only real way to know how much vision, if any, Eli has was to patch his "good" eye and see what he could do/see with the "bad" eye. Furthermore, if it turns out Eli does have some vision left in his eye, we will need to continue to patch it for a while. By patching it, it will force Eli to USE IT. As he uses it, it will get stronger and stronger. The vision will not get better, but his ability to USE the vision he has in that eye will remain. If we do NOT patch him, he will loose the ability to use whatever vision the eye has because the muscles and other nerves that control that eye will weaken and atrophy from NON-USE alone. Sound weird? Imagine you split your arm because it hurts. After a few months you remove the splint because your arm is feeling better. But now your arm is weak, and it is awkward to use it. You have already been compensating with one arm fine over the past months. So, slowly, because it is "easier" to use the one arm than both you forget you have two arms and use only one. That's ridiculous you say (El's says all the time). But, if you apply that extreme example to the eye, which you have less voluntary control over (you don't think about moving your eyes), you can see how easy it would be.
Now before any of you get all worked up and depressed, let me tell you this. If Eli does end up loosing the sight in his eye, he will be no different and it REALLY will NOT affect his life. Case in point (as told to us by our truly wonderful ophthalmologist Dr. King):
So at worst! Eli will be among this list of distinguished and successful people.
Citation for the photo: http://www.medical-look.com/systems_images/Optic_nerve.gif
Friday, April 10, 2009
Another Surgery! Can't this kid get a break?
Eli's VP shunt failed yesterday and he had to have surgery to fix it today. He just got out of surgery a few minutes ago and everything went very well. The catheter from his brain to the valve was clogged and had to be replaced. He should be waking up soon so I may have to cut this short. Assuming he recovers from the surgery as quickly as he has in the past, we will likely be able to go home tomorrow.
Yesterday Eli woke up and promptly threw up after not sleeping well all night. We thought it was just regular kid stuff type sick but we started to get worried when he wasn't feeling better after throwing up everything he ate all day. At about 8pm I checked the valve on his shunt and the bulb was stuck in the "in" position. The valve is under the skin on his head and to test it you push in on the bulb (an m&m sized lump on his head) and if it squeezes in and pops out quickly the shunt is working. When I pressed it, it was already stuck in the depressed position. All I could think was "Oh F**K!" When his shunt was put in at Hackensack in January one of the doctors told us "The only sure thing about shunts is that they fail. They all fail, whether that takes ten years or ten weeks." Apparently this one was more of the ten weeks variety. Hopefully this newly fixed one will last a bit longer.
Eli is doing great otherwise and has been very strong and in good spirits througout this hospital stay. He has been his usual happy and talkative self. He even thanked the nurses for putting in his IV! Such a sweet boy.
Yesterday Eli woke up and promptly threw up after not sleeping well all night. We thought it was just regular kid stuff type sick but we started to get worried when he wasn't feeling better after throwing up everything he ate all day. At about 8pm I checked the valve on his shunt and the bulb was stuck in the "in" position. The valve is under the skin on his head and to test it you push in on the bulb (an m&m sized lump on his head) and if it squeezes in and pops out quickly the shunt is working. When I pressed it, it was already stuck in the depressed position. All I could think was "Oh F**K!" When his shunt was put in at Hackensack in January one of the doctors told us "The only sure thing about shunts is that they fail. They all fail, whether that takes ten years or ten weeks." Apparently this one was more of the ten weeks variety. Hopefully this newly fixed one will last a bit longer.
Eli is doing great otherwise and has been very strong and in good spirits througout this hospital stay. He has been his usual happy and talkative self. He even thanked the nurses for putting in his IV! Such a sweet boy.
Monday, April 6, 2009
Back to School, Singing Like a Star and Potty Trained
Although the idea of going back to school was met with some resistance (from all parties), we are so happy to say that he is loving it. On his first day back he had an entire conversation with another little boy about dinosaurs. I have no idea what they talked about, Eli doesn't know that much about dinosaurs, but their conversation was enough to entertain the teachers! He really enjoys the socialization, especially when all the other parents come to pick-up their children and he can talk to them too.
Two additional achievements...he has been accepted onto the show American Idol. It appears they require contestants to be potty-trained and since Eli has pretty much mastered that he can now pursue his stardom...But really, he loves to sing, generally sings on pitch (does not have the greatest range yet) and learns new songs after three attempts...which is equally amazing since he often has no idea what the words mean! We are all finding it so fun to sing together...one little happy family!
Two additional achievements...he has been accepted onto the show American Idol. It appears they require contestants to be potty-trained and since Eli has pretty much mastered that he can now pursue his stardom...But really, he loves to sing, generally sings on pitch (does not have the greatest range yet) and learns new songs after three attempts...which is equally amazing since he often has no idea what the words mean! We are all finding it so fun to sing together...one little happy family!
Thursday, April 2, 2009
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 missi
ng, 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.
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 missi
ng, 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.
Tuesday, March 31, 2009
Beads of Courage
I just wanted to show off my beads of courage. I get a bead each time I get a poke, scan, treatment, procedure, etc, etc, etc... there are far too many etceteras and far too many beads but they are pretty and fun to play with. They remind me what a brave boy I am.
There are some new movies of me on youtube check them out:
http://www.youtube.com/user/goldngump
Monday, March 30, 2009
Today's MRI
Eli's MRI today went well. He did very well and was feeling great this evening, albeit a bit groggy. He was very brave, although it was very hard for him not to eat until 1:30 in the afternoon. He's normally had about 3 meals by then so it was quite a hard thing to do. It was yet another long and stressful day at the hospital. The MRI was so long that he had to be anesthetized for it; it's always hard for us to watch him be put under and sit in the waiting room, waiting, waiting. At least today it wasn't waiting for a surgery; he was in much better shape after this.
The scan today was twofold in purpose 1. to determine what effect the bleomycin treatments are having on his tumor and 2. to visualize growths under the skin on his leg and back that we have wondered about for a while now, but were not particularly concerning before his craniopharyngioma diagnosis. No one is really worried about them but we thought they should get checked out and depending on how they look, maybe biopsy them.
The MRI results show little to no change in the tumor, which is not unexpected. No one expects the tumor to be gone already (although it would have been nice); the average number of bleomycin treatments is 3. As we progress further into the treatments and the wall of the tumor becomes less and less strong, we will get more aggressive with the draining of the cyst in hopes of collapsing it in on itself. Based on what we saw today we are going to start with another 3 week round of bleomycin in 2 weeks and get another MRI in about 6 weeks. We are hopeful and are trying to be patient, we know this is going to take a while but sometimes all the waiting is hard. I understand why people opt for surgery to get it out right then and there, at least then it's not so unknown. The neuro-oncologist is going to consult another doc about the growths, they don't look scary based on the MRI, but he doesn't know what they are.
As for Eli's general health and well being, he is doing amazingly well. He is headed back to school on wednesday, hopefully that won't be too hard on him. I know he will like being back in school, especially now that he is feeling so much better, but it will be a hard adjustment. It is good for him too, he loves other kids and the singing and art. He will also benefit from the physical challenges posed by school; they will help him to catch up to his peers in terms of the gross motor delays he has from the hydrocephalus. The doctors all say he will be just fine in terms of motor development, he just needs some time and extra help to catch up. Eli has had a good month, he has really blossomed in terms of movement and strength. He loves to jump on his trampoline, do headstands (with his feet still on the ground) and he is almost to the point where he can run and go up and down stairs on his own. These are all major feats where he had made little to no progress in the last year.
We have finally been able to have some quality time together as a family and that has gone very far in terms of keeping our spirits high. We took a mini vacation, just the 3 of us, to Manitou springs and had a great time. We went to the Cheyenne Mt zoo, cave of the winds and garden of the gods. Eli had a great time just hanging out with his Mommy and Daddy (we probably could have been anywhere) and so did we! This trip has inspired us to use some frequent flyer miles that are going to expire and take a trip to Hawaii!! We booked tickets to the big island for June - it cost us $22; now we have to figure out how to get a place to stay for that cheap. Any ideas?
The scan today was twofold in purpose 1. to determine what effect the bleomycin treatments are having on his tumor and 2. to visualize growths under the skin on his leg and back that we have wondered about for a while now, but were not particularly concerning before his craniopharyngioma diagnosis. No one is really worried about them but we thought they should get checked out and depending on how they look, maybe biopsy them.
The MRI results show little to no change in the tumor, which is not unexpected. No one expects the tumor to be gone already (although it would have been nice); the average number of bleomycin treatments is 3. As we progress further into the treatments and the wall of the tumor becomes less and less strong, we will get more aggressive with the draining of the cyst in hopes of collapsing it in on itself. Based on what we saw today we are going to start with another 3 week round of bleomycin in 2 weeks and get another MRI in about 6 weeks. We are hopeful and are trying to be patient, we know this is going to take a while but sometimes all the waiting is hard. I understand why people opt for surgery to get it out right then and there, at least then it's not so unknown. The neuro-oncologist is going to consult another doc about the growths, they don't look scary based on the MRI, but he doesn't know what they are.
As for Eli's general health and well being, he is doing amazingly well. He is headed back to school on wednesday, hopefully that won't be too hard on him. I know he will like being back in school, especially now that he is feeling so much better, but it will be a hard adjustment. It is good for him too, he loves other kids and the singing and art. He will also benefit from the physical challenges posed by school; they will help him to catch up to his peers in terms of the gross motor delays he has from the hydrocephalus. The doctors all say he will be just fine in terms of motor development, he just needs some time and extra help to catch up. Eli has had a good month, he has really blossomed in terms of movement and strength. He loves to jump on his trampoline, do headstands (with his feet still on the ground) and he is almost to the point where he can run and go up and down stairs on his own. These are all major feats where he had made little to no progress in the last year.
We have finally been able to have some quality time together as a family and that has gone very far in terms of keeping our spirits high. We took a mini vacation, just the 3 of us, to Manitou springs and had a great time. We went to the Cheyenne Mt zoo, cave of the winds and garden of the gods. Eli had a great time just hanging out with his Mommy and Daddy (we probably could have been anywhere) and so did we! This trip has inspired us to use some frequent flyer miles that are going to expire and take a trip to Hawaii!! We booked tickets to the big island for June - it cost us $22; now we have to figure out how to get a place to stay for that cheap. Any ideas?
Wednesday, February 25, 2009
Starting Therapy...
We started bleomycin therapy this week and will continue 3 days a week for the next three weeks. Everything is going well so far and Eli is feeling just fine.
We had a bit of a scare after monday's treatment when Eli got sick and threw up a few times monday night and tuesday morning. We were worried that the medicine was leaking and that maybe the treatment wasn't going to work.
We had a few CT scans to confirm that the system (the catheter stuck into the hollow cyst of the tumor) was still sealed and everything looks great. He feels great today too so it looks like it was just a normal kid throwing up thing and nothing else.
He was so cute, yesterday after he started feeling a bit better he kept telling everyone he saw at the hospital "I puked." He has been a real trooper and incredibly brave through all of it, Mommy and Daddy are very proud.
We had a bit of a scare after monday's treatment when Eli got sick and threw up a few times monday night and tuesday morning. We were worried that the medicine was leaking and that maybe the treatment wasn't going to work.
We had a few CT scans to confirm that the system (the catheter stuck into the hollow cyst of the tumor) was still sealed and everything looks great. He feels great today too so it looks like it was just a normal kid throwing up thing and nothing else.
He was so cute, yesterday after he started feeling a bit better he kept telling everyone he saw at the hospital "I puked." He has been a real trooper and incredibly brave through all of it, Mommy and Daddy are very proud.
Thursday, February 12, 2009
Here's what the tumor looks like.
Here's an MRI cross-section through the middle of the tumor at the beginning of January. The tumor (the big white egg) hasn't changed since then but the hydrocephalus in the ventricles (the black space just above the tumor) has gotten much smaller - see below.
This is a video from the same MRI that scans from one side of his head to the other.
These are pics from approx the same place in Eli's head on Jan3 -vs- Feb11. They show the reduction in cerebrospinal fluid in the ventricles (black spaces) of his brain over the last month since the shunt was installed to drain excess fluid into his abdomen and relieve the pressure that was causing many of his symptoms. The pictures look slightly different because one is an MRI and the other a CT. The white line in the bottom left of the right image is part of his shunt tube.
Let's start the bleomycin!
Today was a good day! Today we found out that we will be able to try and treat Eli's tumor the way we hoped: Intracavitary Bleomycin (a.k.a. inject medication into a reservoir that drains into a catheter that is placed in the center of the tumor and then shrink the thing from the inside out). This approach is more novel, but potentially has less risk for damage to his brain...which is a pretty wonderful thing and we want to keep it intact. Most of these tumors are removed by surgery or what is called a radical resection. Essentially they try and remove every last cell (which can result in them removing a little brain tissue), because to leave any behind will eventually result in regrowth of the tumor. So we hope to avoid this approach.
After three weeks of anticipation we are very happy to report that we will beginning the actual "treatment" in 10 days. Unfortunately, we are far from out of the woods yet, but now we have a path and hopefully the path will be clear and true and end at the annihilation of the tumor.
As for "non-medical" stuff, Eli is continuing to do great. He is becoming more and more active, having tantrums (yup-his first! and we are so happy to see them), going to the park, the zoo, the museums, the library and loving all the new things he is learning and people he is meeting. We hope that he will start back at school in March as the chemotherapy he will be getting for the tumor will not be systemic and therefore will NOT cause him to have any side effects or problems normally associated with chemotherapy.
As for mommy and daddy, we are hanging in there...Dad will be getting back to writing his dissertation this week and he hopes he will be finished in two months. Mom is back in school, attending most classes and so far has passed her first two exams. We are all looking forward to Spring Break, when we hope that we can actually take one...a break!!!
For those of you who are interested we will be starting a blog of Eli and his tumor. Because this disease is so rare we hope that charting Eli's course will help others who are diagnosed in the future with this disease to learn about treatments, presentations, emotional effects and the bright future that amazing medical care can provide. When it is up and running we will email you with the address. We will also uploaded images of Eli's tumor if you want to have a peek!
Until then we have attached the image taken today. It is of the tumor filled with a contrast dye. This was the test done to ensure that the catheter was sealed in the tumor and that no medication would leak out into the surrounding brain tissue (that would be BAD). So this picture, while kinda scary and sad, also carries a message of hope! So try and look at it that way. And if you are confused at what you are looking at...it is the big white thing in the middle. Black is water or air, white is bone or contrast dye and grayish is brain matter/tissue. And the little white half-circle on the lower left side is an outline of his shunt (the thing that drains the fluid out of his head and into his abdomen...he likes to push on it!)
I think that is all for now. Thank you again for your love and support...and for all the great pictures. Eli loves looking at them!
All our best to you and yours,
Love
Eli, Jacob and Meagan
After three weeks of anticipation we are very happy to report that we will beginning the actual "treatment" in 10 days. Unfortunately, we are far from out of the woods yet, but now we have a path and hopefully the path will be clear and true and end at the annihilation of the tumor.
As for "non-medical" stuff, Eli is continuing to do great. He is becoming more and more active, having tantrums (yup-his first! and we are so happy to see them), going to the park, the zoo, the museums, the library and loving all the new things he is learning and people he is meeting. We hope that he will start back at school in March as the chemotherapy he will be getting for the tumor will not be systemic and therefore will NOT cause him to have any side effects or problems normally associated with chemotherapy.
As for mommy and daddy, we are hanging in there...Dad will be getting back to writing his dissertation this week and he hopes he will be finished in two months. Mom is back in school, attending most classes and so far has passed her first two exams. We are all looking forward to Spring Break, when we hope that we can actually take one...a break!!!
For those of you who are interested we will be starting a blog of Eli and his tumor. Because this disease is so rare we hope that charting Eli's course will help others who are diagnosed in the future with this disease to learn about treatments, presentations, emotional effects and the bright future that amazing medical care can provide. When it is up and running we will email you with the address. We will also uploaded images of Eli's tumor if you want to have a peek!
Until then we have attached the image taken today. It is of the tumor filled with a contrast dye. This was the test done to ensure that the catheter was sealed in the tumor and that no medication would leak out into the surrounding brain tissue (that would be BAD). So this picture, while kinda scary and sad, also carries a message of hope! So try and look at it that way. And if you are confused at what you are looking at...it is the big white thing in the middle. Black is water or air, white is bone or contrast dye and grayish is brain matter/tissue. And the little white half-circle on the lower left side is an outline of his shunt (the thing that drains the fluid out of his head and into his abdomen...he likes to push on it!)
I think that is all for now. Thank you again for your love and support...and for all the great pictures. Eli loves looking at them!
All our best to you and yours,
Love
Eli, Jacob and Meagan
Monday, February 9, 2009
Day 38: Start My Blog
Hi, I am Eli. I am 2 years old and I was diagnosed with a craniopharyngioma, a "benign" brain tumor on January 2, 2009.
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