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.”
Thursday, May 14, 2009
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
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