Eli has discovered ICE CREAM!
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.
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