Thursday, November 18, 2010
Tuesday, November 16, 2010
My Thoughts on Friday's Surgery
As Quinn's Roux En Y Jejunostomy surgery is rapidly approaching (this friday!), I find myself playing the same mind games I did when Q had her g-tube put in, and then when she had her Nissen. i feel a little lucky that Quinn is not as fragile as some children, but I still continuously second guess myself.
i recall Quinn's g-tube surgery and how nervous I was that she was going to be getting a TUBE in her stomach! Quinn was not a typical case.. as she never was and never will be. Quinn wasn't grossly underweight. She stayed on the growth charts at least, but she was ALWAYS in and out of the hospital because she was always sick and everytime she got sick, she got severely dehydrated (to the point that she couldn't even lift her head) This lead to multiple trips to the ER just for IV hydration and many, many hospital admissions. Before her G-tube, these were the bulk of her admissions. At first I regretted doing that to her, mainly because my child could eat. she only needed it for supplementation and hydration when sick. And when her first sickness came after the tube, it DID NOT keep her out of the hospital. It was a raging g-tube infection that landed her inpatient for a week. And this happened not just once, but MANY times. I was so mad at myself, however her stays in the hospital were fewer, shorter and farther between. We were at least able to keep her hydrated for a little bit until she continued vomiting everything. we realized though that she still had a very severe case of reflux. The surgery to correct it was recommended due to the fact that Quinn was still eating a good amount of her nutrition by mouth. We knew that she was refluxing what she was eating (she had many PH probes that indicated very severe reflux) and when she got fever, she projectile vomited. if she got the g/j tube, she would still end up puking unless we stopped feeding her by mouth and this was not an outcome we wanted. At this time, we were completely unaware that she potentially had a disease that would keep her tube-dependent indefinitely. We focused on the fact that the surgery could fix her and that we could eventually remove the tube and she would be able to survive like a normal child without always throwing up.
When Quinn had her surgery for reflux, she, again was not a typical case. We heard many many horrible nightmares about stories that other parents had been through. We were told by other parents that unless it was a life-or-death matter, never to have it done. but our surgeon was confident that Quinn was a good candidate and that it would help her. Quinn had her gtube and although she puked all day every day, she was somehow able to keep enough calories in her to continue to grow! Though she had a few pneumonias, it wasn't found that she aspirated. And so in Quinn's case, the Nissen was NOT a life-or-death situation. We just wanted her to be healthy and to live a normal life, without a g-tube, without puking. She was also globally delayed and all her docs felt that her reflux was so severe, it was probably contributing to her delays. By the time Quinn had her surgery, she was in the 20th percentile. So, like I said, she was not life-or-death.
I, to this day, have NEVER regretted Quinn's g-tube surgery or her Nissen surgery. These, again, were surgeries that were well processed, pros and cons were weighed and decisions were made based on other's experiences, our experiences, doctor's recommendations and consulting with family. We have always had Quinn's best interest at heart. In retrospect, we are so grateful that we did not wait too long. Given the immediate improvement in both operations, they were good decisions. Quinn was healthy enough to suffer little to no complications and for that we are so grateful. The surgery did help her! After she had her nissen, Q started thriving in therapies and now, at four, she is not at all developmentally delayed.
In short, from those decisions to now, we are almost 100% certain that Q has mitochondrial myopathy. And she has followed that path. Unfortunately, after a very healthy year in 2009, the disease has taken a road of progression and her motility has slowed down quite significantly this past year. It affects her health and daily life greatly. She can no longer eat food like a normal person. It must be pureed and she cannot have a lot of it. Her stomach does not empty well and this means taht she walks around almost every day looking like a pregnant toddler. She is on a very high calorie diet, is on a feeding tube 16 hours a day and has episodes where her stomach just decides not to work. As crazy as this all seems, she still finds ways to gain weight (yes with lots of intervention she is still gaining). but this does not come as a price to her. Quinn lives with belly pain constantly. Sometimes it is mild, but sometimes it is raging and she spends a lot of time screaming in pain, pain that I can't always make go away. She also has frogs that try to hop out of her mouth (this is her description of retching). And she is on 3 seizure medications because she is lacking the ability to absorb all of them through her stomach.
Earlier this year, Quinn had her gtube switched to a g/j tube. but, Quinn's anatomy is different and caused the g/j tube to not stay in and after multiple attempts to keep that thing in place, that is why she was referred to surgery for the Roux En Y Jejunostomy procedure. And as this day nears, I find myself with questioning thoughts. Does she really need this? what if something bad happens? What if it doesn't go as planned? what if we make things worse!? These are the same thoughts that consumed my brain when she had her gtube put in, and when she had her nissen surgery. These decisions were never abrupt, they have been thoroughly thought out and she has had many consultations with doctors, teachers, and family.
Though on days like these, the decision should be an easy one and on those days, it IS easy to say, YES, I just want her to feel better and to convince myself that she NEEDS this.
However, My Quinn is so amazing and it's moments like THESE that I can convince myself that she doesn't need it.
We are still going through with the surgery as planned. We feel that it was a well-thought out process, we are not making an abrupt decision and are confident that it will work as planned and give Quinn a better quality of life. All this week, I have been asking family and friends if t hey feel that this is the best thing for her and the answer is always yes. I have asked her GI doctor and he too feels that we should go through with the surgery. Though we have no way to know for sure how the outcome will be, if it goes as planned, it will help her.
As for the surgery, it is not an easy surgery. They are cutting her intestines, moving a portion over to make the stoma for the J tube and re-attaching the other part of the intestine to her stomach. We have been told the recovery is a minimum of 4 days in the hospital and for us to expect a week. We were also informed today that given Quinn's "history" she might have to remain intubated for a short period of time to give her body a boost for healing.
Please send some good thoughts, vibes, prayers, whatever you do, as we gear up for this surgery. for quinn to remain healthy until her surgery day.
i recall Quinn's g-tube surgery and how nervous I was that she was going to be getting a TUBE in her stomach! Quinn was not a typical case.. as she never was and never will be. Quinn wasn't grossly underweight. She stayed on the growth charts at least, but she was ALWAYS in and out of the hospital because she was always sick and everytime she got sick, she got severely dehydrated (to the point that she couldn't even lift her head) This lead to multiple trips to the ER just for IV hydration and many, many hospital admissions. Before her G-tube, these were the bulk of her admissions. At first I regretted doing that to her, mainly because my child could eat. she only needed it for supplementation and hydration when sick. And when her first sickness came after the tube, it DID NOT keep her out of the hospital. It was a raging g-tube infection that landed her inpatient for a week. And this happened not just once, but MANY times. I was so mad at myself, however her stays in the hospital were fewer, shorter and farther between. We were at least able to keep her hydrated for a little bit until she continued vomiting everything. we realized though that she still had a very severe case of reflux. The surgery to correct it was recommended due to the fact that Quinn was still eating a good amount of her nutrition by mouth. We knew that she was refluxing what she was eating (she had many PH probes that indicated very severe reflux) and when she got fever, she projectile vomited. if she got the g/j tube, she would still end up puking unless we stopped feeding her by mouth and this was not an outcome we wanted. At this time, we were completely unaware that she potentially had a disease that would keep her tube-dependent indefinitely. We focused on the fact that the surgery could fix her and that we could eventually remove the tube and she would be able to survive like a normal child without always throwing up.
When Quinn had her surgery for reflux, she, again was not a typical case. We heard many many horrible nightmares about stories that other parents had been through. We were told by other parents that unless it was a life-or-death matter, never to have it done. but our surgeon was confident that Quinn was a good candidate and that it would help her. Quinn had her gtube and although she puked all day every day, she was somehow able to keep enough calories in her to continue to grow! Though she had a few pneumonias, it wasn't found that she aspirated. And so in Quinn's case, the Nissen was NOT a life-or-death situation. We just wanted her to be healthy and to live a normal life, without a g-tube, without puking. She was also globally delayed and all her docs felt that her reflux was so severe, it was probably contributing to her delays. By the time Quinn had her surgery, she was in the 20th percentile. So, like I said, she was not life-or-death.
I, to this day, have NEVER regretted Quinn's g-tube surgery or her Nissen surgery. These, again, were surgeries that were well processed, pros and cons were weighed and decisions were made based on other's experiences, our experiences, doctor's recommendations and consulting with family. We have always had Quinn's best interest at heart. In retrospect, we are so grateful that we did not wait too long. Given the immediate improvement in both operations, they were good decisions. Quinn was healthy enough to suffer little to no complications and for that we are so grateful. The surgery did help her! After she had her nissen, Q started thriving in therapies and now, at four, she is not at all developmentally delayed.
In short, from those decisions to now, we are almost 100% certain that Q has mitochondrial myopathy. And she has followed that path. Unfortunately, after a very healthy year in 2009, the disease has taken a road of progression and her motility has slowed down quite significantly this past year. It affects her health and daily life greatly. She can no longer eat food like a normal person. It must be pureed and she cannot have a lot of it. Her stomach does not empty well and this means taht she walks around almost every day looking like a pregnant toddler. She is on a very high calorie diet, is on a feeding tube 16 hours a day and has episodes where her stomach just decides not to work. As crazy as this all seems, she still finds ways to gain weight (yes with lots of intervention she is still gaining). but this does not come as a price to her. Quinn lives with belly pain constantly. Sometimes it is mild, but sometimes it is raging and she spends a lot of time screaming in pain, pain that I can't always make go away. She also has frogs that try to hop out of her mouth (this is her description of retching). And she is on 3 seizure medications because she is lacking the ability to absorb all of them through her stomach.
Earlier this year, Quinn had her gtube switched to a g/j tube. but, Quinn's anatomy is different and caused the g/j tube to not stay in and after multiple attempts to keep that thing in place, that is why she was referred to surgery for the Roux En Y Jejunostomy procedure. And as this day nears, I find myself with questioning thoughts. Does she really need this? what if something bad happens? What if it doesn't go as planned? what if we make things worse!? These are the same thoughts that consumed my brain when she had her gtube put in, and when she had her nissen surgery. These decisions were never abrupt, they have been thoroughly thought out and she has had many consultations with doctors, teachers, and family.
Though on days like these, the decision should be an easy one and on those days, it IS easy to say, YES, I just want her to feel better and to convince myself that she NEEDS this.
However, My Quinn is so amazing and it's moments like THESE that I can convince myself that she doesn't need it.
We are still going through with the surgery as planned. We feel that it was a well-thought out process, we are not making an abrupt decision and are confident that it will work as planned and give Quinn a better quality of life. All this week, I have been asking family and friends if t hey feel that this is the best thing for her and the answer is always yes. I have asked her GI doctor and he too feels that we should go through with the surgery. Though we have no way to know for sure how the outcome will be, if it goes as planned, it will help her.
As for the surgery, it is not an easy surgery. They are cutting her intestines, moving a portion over to make the stoma for the J tube and re-attaching the other part of the intestine to her stomach. We have been told the recovery is a minimum of 4 days in the hospital and for us to expect a week. We were also informed today that given Quinn's "history" she might have to remain intubated for a short period of time to give her body a boost for healing.
Please send some good thoughts, vibes, prayers, whatever you do, as we gear up for this surgery. for quinn to remain healthy until her surgery day.
Friday, November 12, 2010
11/12/10
Was really hoping to get the results of Quinn's pulse ox study today, but no one called me back so now we are stuck waiting until MONDAY! Grrr!
Quinn's MRI was normal though. It's actually the first MRI that was found to be normal... all the others showed paranasal sinus disease and lymphnoid issues. This one was totally clear. It was done by a different radiologist and i'm hoping to have her neuro actually LOOK at the previous MRI and compare the most recent one to be sure that the white matter thing wasn't just over looked and flagged as normal. This is surprising to me considering that her seizures have intensified since her last MRI was done. But, i decided not to worry about it. It is a good thing that her MRI was normal. Q has been on a nasal steroid for like 2 years or something like that so it's a relief that it is working. However her nasal cavity still collapses on a daily basis, it is very audible. I'll never be able to explain this, so unless you have witnessed it, you probably have no clue what i'm talking about. And no, she has never had a PET or an MRS, just MRI.
Quinn has been exhausted lately. she LOOKS so worn out and has been sleeping a lot. She has dark circles under her eyes and she is pale. for some reason, she keeps getting these incredibly low grade fevers... like maybe 99 or 100 at most honestly, but they are just there and they go away. She also has cold sores on the sides of her mouth and one in the fold of her nose. I've been putting abriva on them, but one of the ones on the side of her mouth is huge and seems to keep growing. I'm ok with watching it for right now. I think it might be from the tape from the anesthesia last week.
I have actually blogged before about Quinn's issues with food. she LOVES to eat, but she just can't eat much or she will get a huge belly and scream to be vented. So the way we have been coping with this has been to just give her very tiny portions of pureed foods (usually only 2 meals and a snack) and she gets her very high cal tube feeding at 35ml/hr for 16 hours a day. this has been tolerable for right now, but she still is usually hungry no matter what. I rarely ever hear her tell me she is full. (if i never stopped ehr, she would probably eat until her stomach exploded and i'm honestly not kidding about that, she used to be suspect prader willi) anyway, she told me today that she was full and didn't want to eat anymore. Ok, imagine the shock i felt when she told me this. It was amazing. Either A) she is understanding the full feeling, finally or B) something is wrong... lol! I'm going to go with A for right now.
quinn has also had this cough for a long time, at least over amonth i know that, probably much longer. But I don't know what this cough is from. I thought it would go away, but it hasn't. To me, it TOTALLY sounds like a reflux cough because of how she reacts when this happens (lots and lots of swallowing, flaring nostriles and etc...), she also tells me that her throat hurts sometimes and that the frog is trying to get out (her description of retching) now, before I even start to imagine that it could be reflux, she just had a UGI like a month ago or so to see if she needed a pyloroplasty and we saw her barium just pile up on the wrap, but it never went past the fundo. if that thing wasn't there, she would have puked 30cc of the barium for sure. So based on that, the fundo is definitely intact. But still...
She is suppose to have her surgery for her Roux En Y separate J tube on the 19th. That's just 7 days away. We were told she would be inpatient for a minimum of four days but to expect a week. I just want her to be healthy for the surgery. I've decided to continue her oxygen for right now until we get the results of her pulse ox study because of the upcoming surgery. If we are lucky, we will be able to be out by Thanksgiving! it sucks that the surgeyr was scheduled so close to the holidays, which means we will probably have crap doctors and crap nursing care, but we just need to get this done already. It's been dragging out for far too long and I just want my baby to feel better. We may even be able to start weaning one of her seizure meds if we can give them and they are better absorbed through the J.
Thanks for reading.. just a day in the life of Quinn. :)
Quinn's MRI was normal though. It's actually the first MRI that was found to be normal... all the others showed paranasal sinus disease and lymphnoid issues. This one was totally clear. It was done by a different radiologist and i'm hoping to have her neuro actually LOOK at the previous MRI and compare the most recent one to be sure that the white matter thing wasn't just over looked and flagged as normal. This is surprising to me considering that her seizures have intensified since her last MRI was done. But, i decided not to worry about it. It is a good thing that her MRI was normal. Q has been on a nasal steroid for like 2 years or something like that so it's a relief that it is working. However her nasal cavity still collapses on a daily basis, it is very audible. I'll never be able to explain this, so unless you have witnessed it, you probably have no clue what i'm talking about. And no, she has never had a PET or an MRS, just MRI.
Quinn has been exhausted lately. she LOOKS so worn out and has been sleeping a lot. She has dark circles under her eyes and she is pale. for some reason, she keeps getting these incredibly low grade fevers... like maybe 99 or 100 at most honestly, but they are just there and they go away. She also has cold sores on the sides of her mouth and one in the fold of her nose. I've been putting abriva on them, but one of the ones on the side of her mouth is huge and seems to keep growing. I'm ok with watching it for right now. I think it might be from the tape from the anesthesia last week.
I have actually blogged before about Quinn's issues with food. she LOVES to eat, but she just can't eat much or she will get a huge belly and scream to be vented. So the way we have been coping with this has been to just give her very tiny portions of pureed foods (usually only 2 meals and a snack) and she gets her very high cal tube feeding at 35ml/hr for 16 hours a day. this has been tolerable for right now, but she still is usually hungry no matter what. I rarely ever hear her tell me she is full. (if i never stopped ehr, she would probably eat until her stomach exploded and i'm honestly not kidding about that, she used to be suspect prader willi) anyway, she told me today that she was full and didn't want to eat anymore. Ok, imagine the shock i felt when she told me this. It was amazing. Either A) she is understanding the full feeling, finally or B) something is wrong... lol! I'm going to go with A for right now.
quinn has also had this cough for a long time, at least over amonth i know that, probably much longer. But I don't know what this cough is from. I thought it would go away, but it hasn't. To me, it TOTALLY sounds like a reflux cough because of how she reacts when this happens (lots and lots of swallowing, flaring nostriles and etc...), she also tells me that her throat hurts sometimes and that the frog is trying to get out (her description of retching) now, before I even start to imagine that it could be reflux, she just had a UGI like a month ago or so to see if she needed a pyloroplasty and we saw her barium just pile up on the wrap, but it never went past the fundo. if that thing wasn't there, she would have puked 30cc of the barium for sure. So based on that, the fundo is definitely intact. But still...
She is suppose to have her surgery for her Roux En Y separate J tube on the 19th. That's just 7 days away. We were told she would be inpatient for a minimum of four days but to expect a week. I just want her to be healthy for the surgery. I've decided to continue her oxygen for right now until we get the results of her pulse ox study because of the upcoming surgery. If we are lucky, we will be able to be out by Thanksgiving! it sucks that the surgeyr was scheduled so close to the holidays, which means we will probably have crap doctors and crap nursing care, but we just need to get this done already. It's been dragging out for far too long and I just want my baby to feel better. We may even be able to start weaning one of her seizure meds if we can give them and they are better absorbed through the J.
Thanks for reading.. just a day in the life of Quinn. :)
Monday, November 8, 2010
Well, we were discharged yesterday. I've just been busy. The doc kept asking me if we were ready to go home and I kept telling her that I thought her stomach was having a hard time, which is typical for Q, but I still was hopeful that we would be able to get her better treated, but no.Everytime i talked to the doc about it, she seemed to not care, said to just kep giving her mirilax and that she will poop. Well, she kept getting big tummy and the doc kept saying that she has normal bowel sounds and that since it decompresses everytime i vent her, she is fine. So the nurse mentioned to me about the ferral bags and told me to ask the doc about it. When i asked her about it, she made me feel like I was asking for too much and said that if she is on CCS, then her GI doc can order them but that she couldn't do it because she wasn't a CCS doctor. She said that stuff like that is like a special type of treatment for children... hmmm... So she kept asking me if i was comfortable to go home and I kept telling her that I thought her bowels weren't and she kept asking me what I wanted to do to help her and I was like... ummm you are the frickin DOCTOR! I was so done arguing with her and knew that her bowels weren't exactly stable but I just wanted to go home at this point because they were doing nothing for her in there that I couldn't do for her here. So i'm just gonna watch her.
she had a low grade fever yesterday and came home screaming looking like a pregnant toddler, but she finally started pooping and she pooped a lot. Lots of fluffy diarrhea. She was still bloating up a lot everytime i fed her so i just held her formula feed last night and seems to be so much happier today. Fever is gone thank goodness. We will start back up with our daily routine today.
I have vowed never to go back to that hospital for any kind of "observation" unless she is under the care of her GI doc or her surgeon. I feel like I can observe her at home and I can give her tylenol if she has a fever. they didn't even take her blood or culture her urine. Q was very thirsty on the day she got a fever, was bloating up really badly and was peeing brown, but they said there is no way she is dehydrated because she was drinking a lot. ummm... that makes no sense. if her stomach is not working properly, of course she can still be dehydrated.
When I asked for her new discharge paper, they said that all they did was activate the last discharge paper and a new one was not typed out, so basically, there's no true documentation of Saturday's hospital day. Of her fever, of her being on o2, of her heartrate, it just says that she was completely stable for the course of her stay and that she had no complications. all they changed was the date.
I often wonder if this will ever get any easier and I also wonder if we just need to change hospitals to get a clean slate. I'm not sure if maybe I'm flagged as an over paranoid mother or what... but that's the reason I started documenting EVERYTHING!!!! EVERYTHING that i know is out of the norm, i pop out my video phone and start recording. Sometimes I feel like i'm going to go insane, I hate that I have to push for treatment for Quinn and I am pretty certain that her outward appearance takes a front seat to how the inside of her body is behaving.
All I know is that I won't be going back to that hospital unless she is deathly ill... and obviously for her surgery but I definitely won't fight them on any treatment i'll jsut let them do what they need to do and high-tail it out of there as soon as they let us leave.
This how she was the whole way home from the hospital
How her belly looked at discharge (doc said it was soft and non-distended... lol! )
she had a low grade fever yesterday and came home screaming looking like a pregnant toddler, but she finally started pooping and she pooped a lot. Lots of fluffy diarrhea. She was still bloating up a lot everytime i fed her so i just held her formula feed last night and seems to be so much happier today. Fever is gone thank goodness. We will start back up with our daily routine today.
I have vowed never to go back to that hospital for any kind of "observation" unless she is under the care of her GI doc or her surgeon. I feel like I can observe her at home and I can give her tylenol if she has a fever. they didn't even take her blood or culture her urine. Q was very thirsty on the day she got a fever, was bloating up really badly and was peeing brown, but they said there is no way she is dehydrated because she was drinking a lot. ummm... that makes no sense. if her stomach is not working properly, of course she can still be dehydrated.
When I asked for her new discharge paper, they said that all they did was activate the last discharge paper and a new one was not typed out, so basically, there's no true documentation of Saturday's hospital day. Of her fever, of her being on o2, of her heartrate, it just says that she was completely stable for the course of her stay and that she had no complications. all they changed was the date.
I often wonder if this will ever get any easier and I also wonder if we just need to change hospitals to get a clean slate. I'm not sure if maybe I'm flagged as an over paranoid mother or what... but that's the reason I started documenting EVERYTHING!!!! EVERYTHING that i know is out of the norm, i pop out my video phone and start recording. Sometimes I feel like i'm going to go insane, I hate that I have to push for treatment for Quinn and I am pretty certain that her outward appearance takes a front seat to how the inside of her body is behaving.
All I know is that I won't be going back to that hospital unless she is deathly ill... and obviously for her surgery but I definitely won't fight them on any treatment i'll jsut let them do what they need to do and high-tail it out of there as soon as they let us leave.
This how she was the whole way home from the hospital
How her belly looked at discharge (doc said it was soft and non-distended... lol! )
Saturday, November 6, 2010
In Hospital
So, Q went in to have her MRI and we were admitted last night (11/5) for observation. She did pretty frickin good and had super awesome saturations and even a low heart rate. I just wanted to SCREAM I was so frustrated that we see 80 sats but when she goes inpatient, we deal with 90 saturations! Like... REALLY?! Quinn... really! So anyway, Q does pretty excellent throughout the night and by morning, I knew it was time to go home. So the doc is coming in to discharge us and Q's stomach is only slightly distended, but she still hadn't pooped since Wednesday (it's now Saturday) but whatever, take her home, maybe get her a suppository or enema and hopefully things will start moving. But, something was itching me. I don't know if it's because Q is notorious for being discharged a little too soon and we end up driving all the way up the mountain 1hr 45mins back home only to end up back at the children's hospital within hours, or if I was just being a worry wart, but anyway... the doc was like I noticed in her records she is o2 dependent but she has had really great sats here, could you explain that to me. So I told her that i had NO idea but that she just has low sats, it's recorded in her pedi office and on my phone. her sats are low at home but everytime we come here they are usually pretty high and perfectly normal! <--- not always, but usually. Apparently, she overheard us talking to our really awesome roommate about living in the mountains. So she asks me... do you live at an elevation over 3000 feet?.... ummm... yes, it's 3500 feet. Hello! lights come on! she said that's probably why she requires o2 up there and not really down here. yes! that makes so much more sense! I told her how crazy i was feeling that I just wanted to throw in the towel and she said that her o2 should definitely be covered because she obviously needs it at the altitude we live at. Makes sense, right?
So, she hands us our discharge papers, we sign our discharge papers, Pull Q's IV, get Q dressed, pack up and start heading out the door, but when I pick up Q she is sweating, not waking up and hot. So I said.... hmmm... nurse, could you come take her temp? So she did and sure enough, Q had a fever. So, her discharge got delayed for a few hours. So we didn't give her tylenol because the doc thought it was just a fever from anesthesia (yes the anesthesia that she had 20 hours ago). So we wait about 2 hours and Quinn's HR is in the 160s, her breathing is very fast and her o2 sats start dropping. We put her on o2 and that seemed to make things better, placed her on some icepacks because she said she was really cold, but she was burning up and just wanted a blanket. and let her sleep all day long. By 630, her fever was only going up and so the nurse gave her some tylenol and informed us that we weren't going anywhere tonight. her tummy is distended and is hurting her.
So the attending for nightshift comes on at about 8pm and said that the day doc had asked that she check on us to see if we were ready to be discharged... i'm like... what? Doc, did you really read her chart? Did you even speak with the nurses? Do you know that she has had a fever all day, is now on o2 and is finally resting easy on tylenol? Have you felt her stomach? Because it's a bit distended... And she goes and touches her stomach and says... oh, yeah, it's probably best if you stay the night.... uh... yeah... gggrrrr!
So right now, i'm a little frustrated because for one, I feel like since I have a hospital at my house, I should automatically be able to take care of her, which I should, but since we are already at the hospital, I'd really just like to make sure nothing serious is brewing! They haven't even taken her blood or cultured the urine that was clear this morning but is now dark brown. And I don't care what her bowel sounds are right now, her stomach is acting up and I'd like for her to poop before we leave. I jsut think it's dumb that I have to basically hold the doctor's hands in order to get her as stable for discharge as she was upon admission.
If she has a fever by morning or needs round the clock meds to keep it down, i'm going to ask that they take blood and culture her urine and to be sure she poops before we leave... suppository or enema... whatever as long as we do not have to come back after we leave.
Plus, i need her HEALTHY for her surgery. It's been scheduled for the 19th of this month! So we need to have a really healthy Quinn. if it doesn't get done on thsi date, chances are it won't get done till 2011.
So, she hands us our discharge papers, we sign our discharge papers, Pull Q's IV, get Q dressed, pack up and start heading out the door, but when I pick up Q she is sweating, not waking up and hot. So I said.... hmmm... nurse, could you come take her temp? So she did and sure enough, Q had a fever. So, her discharge got delayed for a few hours. So we didn't give her tylenol because the doc thought it was just a fever from anesthesia (yes the anesthesia that she had 20 hours ago). So we wait about 2 hours and Quinn's HR is in the 160s, her breathing is very fast and her o2 sats start dropping. We put her on o2 and that seemed to make things better, placed her on some icepacks because she said she was really cold, but she was burning up and just wanted a blanket. and let her sleep all day long. By 630, her fever was only going up and so the nurse gave her some tylenol and informed us that we weren't going anywhere tonight. her tummy is distended and is hurting her.
So the attending for nightshift comes on at about 8pm and said that the day doc had asked that she check on us to see if we were ready to be discharged... i'm like... what? Doc, did you really read her chart? Did you even speak with the nurses? Do you know that she has had a fever all day, is now on o2 and is finally resting easy on tylenol? Have you felt her stomach? Because it's a bit distended... And she goes and touches her stomach and says... oh, yeah, it's probably best if you stay the night.... uh... yeah... gggrrrr!
So right now, i'm a little frustrated because for one, I feel like since I have a hospital at my house, I should automatically be able to take care of her, which I should, but since we are already at the hospital, I'd really just like to make sure nothing serious is brewing! They haven't even taken her blood or cultured the urine that was clear this morning but is now dark brown. And I don't care what her bowel sounds are right now, her stomach is acting up and I'd like for her to poop before we leave. I jsut think it's dumb that I have to basically hold the doctor's hands in order to get her as stable for discharge as she was upon admission.
If she has a fever by morning or needs round the clock meds to keep it down, i'm going to ask that they take blood and culture her urine and to be sure she poops before we leave... suppository or enema... whatever as long as we do not have to come back after we leave.
Plus, i need her HEALTHY for her surgery. It's been scheduled for the 19th of this month! So we need to have a really healthy Quinn. if it doesn't get done on thsi date, chances are it won't get done till 2011.
Thursday, November 4, 2010
My Thoughs on MNGIE
Ok, so... hmmm.... thanks a lot Quinn for giving me such frickin random drama. She has a way of making me feel so insane sometimes! After a few days of sats in the 80s, the past 36 hours, she has been in the high 90s, consistently. I was starting to doubt the pulse ox and thought maybe it had broke or something with her constant readings of 80%, but now i have NO doubt in my mind that it is at all broken because now we are taking her saturations, same as we always do and they are in the high 90s. so, what the heck, Quinn? We were suppose to get a bad blood gas reading so we can stop running into insurance poopiness, but we can't get a bad blood gas reading when you are saturating in the 90s. and we can't get treatment for when you have bad sats if you are going to stay in the 90s for over 24 hours. UGH! I'm so tired of the games. so, with this, i have NO idea what's going to come of getting her oxygen approved. It's absolutely ridiculous and is seriously stressing me out. I know this sounds wrong on all levels. but i really hope she is just doing the Mito thing and having random days of feeling well. soon enough, she will tank again... because i'm so done thinking that maybe she will just get better and that maybe she really doesn't need her o2. We have done this 2 times already and she fails the o2 wean.
In other news. A fellow mito friend of mine who lives in my county invited me to go to this UMDF meeting thing and meet with Dr. C from the Cleveland Clinic. He is a very well known and respected mito doctor. I was really excited about going. even though walking in there, I was still incredibly doubtful that anything he had to say would have anything to do with my child. I walked in there expecting to feel more relieved that she didn't have mito. I definitely did not expect to feel so absolutely certain that Quinn has Mitochondrial Myopathy. I had a lot of questions.
the way they described mito made my heart flutter as although they were speaking of a car, that was exactly how it is for Quinn! Basically, picture an Escalade. On the outside it looks perfect, nice rims, no dents, beautiful. Everything works wonderfully, but over a short amount of time ,this headlight starts to dim and so, you take it to the headlight doctor and he replaces the headlight. So the car is back to being good as new. the headlight is shining brightly for a little while, but shortly after, the headlight starts to dim again, and so you think ti's maybe a battery issue, so you take it to the battery doctor and he replaces the battery and says ok... problem fixed. The car is running like new again, headlight is working beautifully for a short while, but then, it starts to go out again. So then they think that maybe it's an alternator issue. so you take it to the alternator doctor, then he fixes it up and replaces the alternator and again, the car runs smoothly for a short while, but the underlying problem is still there. The problem is not the headlight, the battery or the alternator. the problem is that somehow, somewhere, the gas is not getting to the parts of hte car it needs to get to, and so then it develops symptoms, just as a child with mito. A child like Quinn. Quinn's body has worked exactly like that! first, it was her breathing, take her to the lung doctor, ok, we fixed the problem, then it was her heart, take her to the cardio... ok we fixed the problem, then it was her GI system, take her to the GI doc, ok, we fixed the problem. Then neuro, take her to the neuro, ok they fixed the problem. But now, she is still having breathing issues, heart issues, GI issues and neuro issues. The underlying issues are not in those body systems, but in her mitochondria. She cannot produce the energy to keep these systems running like they should. And the only thing that these lung, heart, stomach and brain doctors can do is to treat her within their specialty. They aren't looking at the fuel taht isn't getting to these body systems.
I learned that these doctors who are well known have people coming from all over the country, people like me, who have taken their children to doctor after doctor after doctor and have come up with nothing.
So, after a few people started asking questions and listening to others' situations and how he replied to them, i just couldn't believe that much of these stories were just like mine! and I had a lot of questions.
A little history about HOW quinn came to the "clinical" diagnosis of mito and why we are still seeking out a better doctor, hopefully Dr. B in CHLA will be this better doctor.
Last year, Quinn had her blood taken for the TYMP gene, which is the gene responsible for the Mitochondrial disease, MNGIE. Expecting nothing, I never rescheduled a follow up and figured that nothing was found. Much to my surprise, I was called multiple times to come back in because the doctor needed to speak with me about some test results. by April, 2010, i couldn't even remember the tests that she had last year. I was shocked when the doctor told me that they may have found out waht is wrong with my daughter that would explain her issues to a T! Quinn has a mutated TYMP gene. All that was left was to check her thymadine levels, if these were found to be high, that would indicate that the enzyme was not being broken down and therefore she would be diagnosed. I asked the doctor... "what if the thymadine level comes back normal?" He wouldn't even go there, he said given the mutation and the symptoms, there is a very small percent chance that it would come back normal. we discussed the disease in detail as the doctor was very dead-set that this was what she had. A few months later, the test comes back, and it is normal. I was dumbfounded, shocked and sunk into a very dark place... back into the undiagnosed category.
Dr. H came and talked to me and said that because of this, she could not be diagnosed. I asked him what she has then and if she has mitochondrial disease and he specifically told me that he could not tell me that answer. He said that he wasn't going to say it was and he wasn't going to say it wasn't. Just that it could be we just haven't found out where yet. He discharged us and told me to take her home, love her and hope she gets better. he said if she gets worse, to bring her back. Well, this was before her stomach started having pseudo-obstructions.. so yes, in many ways, she actually has gotten worse, but i haven't taken her back. The last appointment was so discouraging and I really didn't want anyone to discourage me anymore. And so we lived with this.
A few months later, i get a letter in the mail from her special kids insurance stating that she has been approved for follow-up and treatment for mitochondrial disease.... WHAT!?! i call the insurance company and they said that they got a packet of paperwork with a diagnosis of mito. So I just decided to go see Dr B in CHLA.
Basically, i gave Dr. C a brief explanation of all this... specifically i wanted to know what the chances were that she has all these symptoms of Mito and actually DOES have a gene mutation for MNGIE, but she is still undiagnosed... how could this be? he said that mito in itself is still in its infancy, but MNGIE is so rare there's only a handful of patients who have even tested positive for this gene. He told me what he would do if Q was his patient is really not much different than what San Diego did... except he would have written a protocol letter and stated that she was highly suspected of having this disease and the precautions to take with a child with mito, and told us to come back in a year. so basically, the answer still remains, she may or may not have it, but technology just hasn't caught up with testing for this particular portion of mito.
so, i honestly don't know whether or not to say that she does or doesn't have MNGIE. I hate being in the gray area so much. I hope Dr. B can help clear this up a little better for me.
Ok, here's the thing, I've done ALOT of research before on MNGIE when I was told that she had this disease. but I've done even more research now because of how Dr. C explained to me that there's still so much more research to be done. Even though I was told that Q couldn't be diagnosed with MNGIE because her thymidine levels were found normal, i'm still not convinced that she does not have this particular form of mito. for one thing, what are the chances that she would clinically fit this diagnosis perfectly, actually HAVE a mutated gene, and still not have the disease. that to me is a little strange. I came up with these conclusions:
A) the blood was handled wrong, which is a very possible hypothesis considering that the first time they took it, the sent it to the wrong lab and it was handled incorrectly, we had to drive all the back to San Diego to have it drawn again. Also, it's such a specialized test that is RARELY if ever ordered. Not many people with Quinn's symptoms actually HAVE a mutated TYMP gene. There's actually only 70 reported cases of MNGIE from what I've read.
"B) We didn't take it from the most diseased tissue. "It is important to examine the most significantly affected tissue and to measure TP activity and plasma thymidine in order to arrive at an accurate diagnosis in this condition."quote taken from Here
C) Quinn is on MediCaid, which is notorious for only approving the cheapest labs. This hypothesis could possibly be off the wall, but i wouldn't dismiss it.
D) the doctor probably did not do enough research to look further into her symptoms. the most clinically appropriate for MNGIE was Quinn's MRI. Her MRI show's a "hallmark" for this disease. I'll go into more detail in a paragraph or so.
Basically, MNGIE is the RAREST form of mito... mito which is rare in itself (though we are finding that it's probably more prevalent that we think) Still finding actual gene mutations for mito is more rare than diagnosing off clinical symptoms <-- my thoughts, not quoted. So, I feel that due to the fact that Quinn has such symptoms for MNGIE and that she has the gene, i think that even though her thymidine levels were found to be normal (if they were actually handled correctly) it should still be looked further into.
About her MRI, which strikes me as too on-target for it to NOT be MNGIE: I found This to be the most informative literature and couldn't BELIEVE how appropriately Q fits this diagnosis. so basically, what is so incredibly interesting to me about the asymptomatic leukoencephalopathy. In this article it states that absence of leukoencephalopathy is very unlikely in MNGIE. Originally when she had her brain MRI, this was noted, specifically in the impression quote: "subtle diffuse prominence of the cerebral white matter"... originally, the doc said that given that she basically wasn't retarded, it was probably just something to watch for now since she didn't show symptoms of leukoencephalopathy, however in MNGIE patients, it is shown to be asymptomatic, but a "hallmark" of the disease. Her MRI also explains thatt he "hemispheric white matter and corpus callosum appear prominent"... and in this article it says, " Relative sparing of the corpus callosum is reported in some individuals" Now i'm trying to figure out what "relative sparing" means because I have no idea if it can relate to what was reported in her MRI with her corpus callosum appearing prominent... Can anyone clear that up for me?
I know for certain that upon discharging us, her doctor did not know about this MRI thing, probably didn't even know she had an MRI and probably didn't think to look further. He likely saw the test results and was like... welp that's not it, bye! i'm not saying that this doctor is incompetent at all, I'm taking into consideration that MNGIE itself is so rare and that for a child like Quinn, who has a mutation, who presents with the right clinical symptoms of the disease, who they were so certain had MNGIE before the thymidine levels were taken, should not be brushed off so easily because of a normal test result and that it is perhaps something that needs to be better researched.
so, when we finally get our appointment for Dr. B in CHLA and when we FINALLY get to see him, I really hope he can clear all this up for me and explain to me HOW this child does not have MNGIE. Oh believe me, i will be quoting my resources and presenting to him my research.
this made me feel better about telling people, doctors, teachers that my child has mitochondrial myopathy. I still would like a solid diagnosis. However, I am hoping that Dr. B will be able to provide us with a better diagnostic approach.... better than San Diego.
Now that you have gotten through my book, thank you for reading my thoughts on this subject. I look forward to any comments anyone reading this might have.
Please keep Eithene In your thoughts and prayers. This child has endured far too much in her 4 years of life and is still struggling after spending pretty much an entire year in the hospital. She is a fellow Mito Warrior.
Please also keep Kyle In your thoughts and prayers. He too has spent too much time in the hospital and is still struggling with lung issues after an emergency surgery. These two need our constant thoughts and prayers.
In other news. A fellow mito friend of mine who lives in my county invited me to go to this UMDF meeting thing and meet with Dr. C from the Cleveland Clinic. He is a very well known and respected mito doctor. I was really excited about going. even though walking in there, I was still incredibly doubtful that anything he had to say would have anything to do with my child. I walked in there expecting to feel more relieved that she didn't have mito. I definitely did not expect to feel so absolutely certain that Quinn has Mitochondrial Myopathy. I had a lot of questions.
the way they described mito made my heart flutter as although they were speaking of a car, that was exactly how it is for Quinn! Basically, picture an Escalade. On the outside it looks perfect, nice rims, no dents, beautiful. Everything works wonderfully, but over a short amount of time ,this headlight starts to dim and so, you take it to the headlight doctor and he replaces the headlight. So the car is back to being good as new. the headlight is shining brightly for a little while, but shortly after, the headlight starts to dim again, and so you think ti's maybe a battery issue, so you take it to the battery doctor and he replaces the battery and says ok... problem fixed. The car is running like new again, headlight is working beautifully for a short while, but then, it starts to go out again. So then they think that maybe it's an alternator issue. so you take it to the alternator doctor, then he fixes it up and replaces the alternator and again, the car runs smoothly for a short while, but the underlying problem is still there. The problem is not the headlight, the battery or the alternator. the problem is that somehow, somewhere, the gas is not getting to the parts of hte car it needs to get to, and so then it develops symptoms, just as a child with mito. A child like Quinn. Quinn's body has worked exactly like that! first, it was her breathing, take her to the lung doctor, ok, we fixed the problem, then it was her heart, take her to the cardio... ok we fixed the problem, then it was her GI system, take her to the GI doc, ok, we fixed the problem. Then neuro, take her to the neuro, ok they fixed the problem. But now, she is still having breathing issues, heart issues, GI issues and neuro issues. The underlying issues are not in those body systems, but in her mitochondria. She cannot produce the energy to keep these systems running like they should. And the only thing that these lung, heart, stomach and brain doctors can do is to treat her within their specialty. They aren't looking at the fuel taht isn't getting to these body systems.
I learned that these doctors who are well known have people coming from all over the country, people like me, who have taken their children to doctor after doctor after doctor and have come up with nothing.
So, after a few people started asking questions and listening to others' situations and how he replied to them, i just couldn't believe that much of these stories were just like mine! and I had a lot of questions.
A little history about HOW quinn came to the "clinical" diagnosis of mito and why we are still seeking out a better doctor, hopefully Dr. B in CHLA will be this better doctor.
Last year, Quinn had her blood taken for the TYMP gene, which is the gene responsible for the Mitochondrial disease, MNGIE. Expecting nothing, I never rescheduled a follow up and figured that nothing was found. Much to my surprise, I was called multiple times to come back in because the doctor needed to speak with me about some test results. by April, 2010, i couldn't even remember the tests that she had last year. I was shocked when the doctor told me that they may have found out waht is wrong with my daughter that would explain her issues to a T! Quinn has a mutated TYMP gene. All that was left was to check her thymadine levels, if these were found to be high, that would indicate that the enzyme was not being broken down and therefore she would be diagnosed. I asked the doctor... "what if the thymadine level comes back normal?" He wouldn't even go there, he said given the mutation and the symptoms, there is a very small percent chance that it would come back normal. we discussed the disease in detail as the doctor was very dead-set that this was what she had. A few months later, the test comes back, and it is normal. I was dumbfounded, shocked and sunk into a very dark place... back into the undiagnosed category.
Dr. H came and talked to me and said that because of this, she could not be diagnosed. I asked him what she has then and if she has mitochondrial disease and he specifically told me that he could not tell me that answer. He said that he wasn't going to say it was and he wasn't going to say it wasn't. Just that it could be we just haven't found out where yet. He discharged us and told me to take her home, love her and hope she gets better. he said if she gets worse, to bring her back. Well, this was before her stomach started having pseudo-obstructions.. so yes, in many ways, she actually has gotten worse, but i haven't taken her back. The last appointment was so discouraging and I really didn't want anyone to discourage me anymore. And so we lived with this.
A few months later, i get a letter in the mail from her special kids insurance stating that she has been approved for follow-up and treatment for mitochondrial disease.... WHAT!?! i call the insurance company and they said that they got a packet of paperwork with a diagnosis of mito. So I just decided to go see Dr B in CHLA.
Basically, i gave Dr. C a brief explanation of all this... specifically i wanted to know what the chances were that she has all these symptoms of Mito and actually DOES have a gene mutation for MNGIE, but she is still undiagnosed... how could this be? he said that mito in itself is still in its infancy, but MNGIE is so rare there's only a handful of patients who have even tested positive for this gene. He told me what he would do if Q was his patient is really not much different than what San Diego did... except he would have written a protocol letter and stated that she was highly suspected of having this disease and the precautions to take with a child with mito, and told us to come back in a year. so basically, the answer still remains, she may or may not have it, but technology just hasn't caught up with testing for this particular portion of mito.
so, i honestly don't know whether or not to say that she does or doesn't have MNGIE. I hate being in the gray area so much. I hope Dr. B can help clear this up a little better for me.
Ok, here's the thing, I've done ALOT of research before on MNGIE when I was told that she had this disease. but I've done even more research now because of how Dr. C explained to me that there's still so much more research to be done. Even though I was told that Q couldn't be diagnosed with MNGIE because her thymidine levels were found normal, i'm still not convinced that she does not have this particular form of mito. for one thing, what are the chances that she would clinically fit this diagnosis perfectly, actually HAVE a mutated gene, and still not have the disease. that to me is a little strange. I came up with these conclusions:
A) the blood was handled wrong, which is a very possible hypothesis considering that the first time they took it, the sent it to the wrong lab and it was handled incorrectly, we had to drive all the back to San Diego to have it drawn again. Also, it's such a specialized test that is RARELY if ever ordered. Not many people with Quinn's symptoms actually HAVE a mutated TYMP gene. There's actually only 70 reported cases of MNGIE from what I've read.
"B) We didn't take it from the most diseased tissue. "It is important to examine the most significantly affected tissue and to measure TP activity and plasma thymidine in order to arrive at an accurate diagnosis in this condition."quote taken from Here
C) Quinn is on MediCaid, which is notorious for only approving the cheapest labs. This hypothesis could possibly be off the wall, but i wouldn't dismiss it.
D) the doctor probably did not do enough research to look further into her symptoms. the most clinically appropriate for MNGIE was Quinn's MRI. Her MRI show's a "hallmark" for this disease. I'll go into more detail in a paragraph or so.
Basically, MNGIE is the RAREST form of mito... mito which is rare in itself (though we are finding that it's probably more prevalent that we think) Still finding actual gene mutations for mito is more rare than diagnosing off clinical symptoms <-- my thoughts, not quoted. So, I feel that due to the fact that Quinn has such symptoms for MNGIE and that she has the gene, i think that even though her thymidine levels were found to be normal (if they were actually handled correctly) it should still be looked further into.
About her MRI, which strikes me as too on-target for it to NOT be MNGIE: I found This to be the most informative literature and couldn't BELIEVE how appropriately Q fits this diagnosis. so basically, what is so incredibly interesting to me about the asymptomatic leukoencephalopathy. In this article it states that absence of leukoencephalopathy is very unlikely in MNGIE. Originally when she had her brain MRI, this was noted, specifically in the impression quote: "subtle diffuse prominence of the cerebral white matter"... originally, the doc said that given that she basically wasn't retarded, it was probably just something to watch for now since she didn't show symptoms of leukoencephalopathy, however in MNGIE patients, it is shown to be asymptomatic, but a "hallmark" of the disease. Her MRI also explains thatt he "hemispheric white matter and corpus callosum appear prominent"... and in this article it says, " Relative sparing of the corpus callosum is reported in some individuals" Now i'm trying to figure out what "relative sparing" means because I have no idea if it can relate to what was reported in her MRI with her corpus callosum appearing prominent... Can anyone clear that up for me?
I know for certain that upon discharging us, her doctor did not know about this MRI thing, probably didn't even know she had an MRI and probably didn't think to look further. He likely saw the test results and was like... welp that's not it, bye! i'm not saying that this doctor is incompetent at all, I'm taking into consideration that MNGIE itself is so rare and that for a child like Quinn, who has a mutation, who presents with the right clinical symptoms of the disease, who they were so certain had MNGIE before the thymidine levels were taken, should not be brushed off so easily because of a normal test result and that it is perhaps something that needs to be better researched.
so, when we finally get our appointment for Dr. B in CHLA and when we FINALLY get to see him, I really hope he can clear all this up for me and explain to me HOW this child does not have MNGIE. Oh believe me, i will be quoting my resources and presenting to him my research.
this made me feel better about telling people, doctors, teachers that my child has mitochondrial myopathy. I still would like a solid diagnosis. However, I am hoping that Dr. B will be able to provide us with a better diagnostic approach.... better than San Diego.
Now that you have gotten through my book, thank you for reading my thoughts on this subject. I look forward to any comments anyone reading this might have.
Please keep Eithene In your thoughts and prayers. This child has endured far too much in her 4 years of life and is still struggling after spending pretty much an entire year in the hospital. She is a fellow Mito Warrior.
Please also keep Kyle In your thoughts and prayers. He too has spent too much time in the hospital and is still struggling with lung issues after an emergency surgery. These two need our constant thoughts and prayers.
Monday, November 1, 2010
Ballerina Quinn
I wanted to share this video of Quinn's dance recital from last year. This was her super healthy year (2009), before she got sick in January 2010 and started having a lot of seizures.
On a different note, we are still battling insurance for o2. And I do believe that she is having drop-seizures as a result of her not being on o2. We have an appointment with the pediatrician today.
Ballerina Quinn
On a different note, we are still battling insurance for o2. And I do believe that she is having drop-seizures as a result of her not being on o2. We have an appointment with the pediatrician today.
Ballerina Quinn
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