It has been a long couple of weeks since I updated about Zach. Sad to say, we are in the hospital again...so much has happened, I don't even know where to begin or what to write.
We did get 2 whole weeks at home! Then he woke up sick, vomiting, chills, fever so we were admitted for probable sepsis. It turned out he grew yeast again from his line...that was only 2 weeks old!! Words can't tell you how we felt about that. He can't afford to go through lines every 2 weeks, I couldn't believe he grew the exact same thing.
So the plan was, leave the infected line in and treat with mycafungin for a week and then remove and replace the same day. His surgeon was afraid of what happened last time with losing iv's, and blood sugar issues. Zach was feeling pretty good anyway so that was hope he was getting better.
So we spent our Thanksgiving in the hospital, my mom and I ordered lunch from Cracker Barrel and had a good time with our favorite nurse, Ryanne. Then the rest of the family came up for dinner together.
Zach also had an eeg on Thanksgiving to determine if his "reaction" to medicines being put in his J tube was any seizure activity...it is not.
Then friday morning, Zach had surgery. He woke up very nauseaus and vomited in the holding room before surgery and then after surgery he continued to vomit. He was vomiting every 15-30 minutes all day and night. It was by this point just blood, and he was draining blood..then on saturday, he continued to vomit and was dehydrated....wouldn't wake up and very painful. They had him on IV zofran, IV kytril, IV reglan, phenegren for vomiting and IV tylenol, IV nubain, IV dilaudid for pain.
They did an upper GI contrast study on Saturday that showed that his stomach/duodenum wasn't emptying the contrast so his GI dr decided to scope him first thing Sunday morning. During the scope he found that his esophagus and stomach are very inflammed from vomiting so much. But he also reached a point where he couldn't get the scope through so he placed some contrast in and it and the contrast just came back into his stomach. So the GI dr thinks he has an obstruction.
But his surgeon doens't think he has an obstruction, he has had a surgery before where his body was acting like an obstruction but nothing was there. So his surgeon is leary. We know his motility is not good so it could be that,,,we just know yet.
I do know that my poor son has been sicker this weekend than ever before. He has been vomiting since friday morning and in so much pain. He spent 3 days in PIMC because he was so sick. He has been on lots of meds and none of them really helping. Today was the first day he got out of bed...it was short, about a minute but it was something.
We are still waiting to find out what we are doing. He will have a test tomorrow where they will place a tp tube into his stomach and duodenum and inject contrast right at the spot they think is obstructed. If it is then surgery will be convinced and if not then the dr's are talking about doing a celiac plexus nerve block. It should help with pain and vomiting. I am supposed to talk to the pain dr today that would do it, he hasn't done one on a child Zach's age so it is scary.
After that, I am not sure what is going to happen. I guess we need to discuss transplant again....that is a scary road and I wasn't ready for it yet but looks like it will be sooner than later.
Wednesday, November 30, 2011
Saturday, November 12, 2011
We made it a whole week!!!
I am not jinxing anything, but I am so happy that we have been out of the hospital for a whole week!!! This hasn't happened it a while...well like about 2-3 months. A-m-a-zing!!
Zach has a lot of ups and downs this week....pain, vomiting, low grade fevers, autonomic crap but through it all we are still at home.
He has started vomiting and gagging whenever I put his meds in his J tube, then that sets off his autonomic crap...spiking fever, dizzy and lethargy
He has also been having more pain. His psuedo obstruction is really showing its ugly head right now. He was on lortab every 6 hours around the clock to help, but his dr's have changed up some meds this week.
His clonidine has been changed from a liquid form to the patch form that can be worn for 3 days, then changed. Hopefully he will get a more steady stream of medicine this way and it will help more with pain. Also they have started him on Lyrica to help with autonomic dysfunction and pain. So far can't see any difference but we will see. He is still on zofran every 6 hours around the clock. He is also still on his antibiotic (vancomycin) and his fungal (fluconazole) both IV for his blood infection, but they will both end on Monday.
Overall in between his pain and vomiting episodes he has been in a good mood. He gets worn out and tired very easily but we are pushing through. He is doing good in PT, he sees her once a week to work. We have lots of appts next week and hopefully learning some news that has been pending.
We have GI follow up, immunology and surgery follow up to get the biopsy results. The MRI has already been sent to Pittsburgh and so will the biopsy results. They will interpret everything along with the bloodwork and let us know.
And some bittersweet news as well, Zach was approved for Make a Wish. This is awesome in the fact that he will get to go somewhere that we would not be able to go without them but bittersweet that yes, he does a life altering diagnosis.
Zach has a lot of ups and downs this week....pain, vomiting, low grade fevers, autonomic crap but through it all we are still at home.
He has started vomiting and gagging whenever I put his meds in his J tube, then that sets off his autonomic crap...spiking fever, dizzy and lethargy
He has also been having more pain. His psuedo obstruction is really showing its ugly head right now. He was on lortab every 6 hours around the clock to help, but his dr's have changed up some meds this week.
His clonidine has been changed from a liquid form to the patch form that can be worn for 3 days, then changed. Hopefully he will get a more steady stream of medicine this way and it will help more with pain. Also they have started him on Lyrica to help with autonomic dysfunction and pain. So far can't see any difference but we will see. He is still on zofran every 6 hours around the clock. He is also still on his antibiotic (vancomycin) and his fungal (fluconazole) both IV for his blood infection, but they will both end on Monday.
Overall in between his pain and vomiting episodes he has been in a good mood. He gets worn out and tired very easily but we are pushing through. He is doing good in PT, he sees her once a week to work. We have lots of appts next week and hopefully learning some news that has been pending.
We have GI follow up, immunology and surgery follow up to get the biopsy results. The MRI has already been sent to Pittsburgh and so will the biopsy results. They will interpret everything along with the bloodwork and let us know.
And some bittersweet news as well, Zach was approved for Make a Wish. This is awesome in the fact that he will get to go somewhere that we would not be able to go without them but bittersweet that yes, he does a life altering diagnosis.
Friday, November 04, 2011
Central line drama, biopsy, MRI and discharge
On Sunday we found out that Zach's central line culture grew yeast....not good, that is the worst thing you want in your central line. It means you automatically lose your line, you can't clear yeast. So the doctors added in fungal meds in hopes to treat a few days with the line until his surgeon returned on Tuesday.
Sunday he ran low grade fevers all day but otherwise felt pretty good, he must have been pretty energized from the night before. Sunday night he ended up having hallucinations all night, he screamed and cryed and yelled that spiders and snakes and bugs were getting him...this lasted from midnight til 7am, then he slept some then continued throughout the day on Monday.
Monday of course was Halloween and Zach and Luke were both ninja turtles. Luke wanted to come see us instead of going to his halloween party at school him and Nana came up and all of them went to the party that the hospital had in the playroom. Right about lunchtime he started getting sick again, spiked a 104 fever, moaning, chills. So the doctors changed his meds around and put him back on Vanc, meropenum and changed his fungal med. Around 1 am Tuesday night, he started shaking really badly, spiked another fever and his stats kept dropping so we knew his line had to come out soon. His line had also starting clotting, you couldn't get any blood return on it.
So Tuesday his surgeon was back and the plan was to take Zach to the sedation room at 2pm and place him under sedation, place 2 iv's and remove his central line. They sedated him and the transport team got to work to try to find iv sites, unfortunately that is pretty hard in Zach. They had 5 transport (the team that rides on the helicopter) people and the PICU attending/anesthesia trying to place iv's and no one could get them in. Finally after about 30-45 minutes his surgeon got one. And then another was placed, both in his feet...poor boy :(
They had to run D10 in both iv's to keep his sugar levels up and still run the two antibiotics and fungal meds and fluid replacement. The next morning, the first iv had blown and transport had to come again and try to place another iv. Usually when you remove a line for infection, the plan is to wait 3-5 days for the infection to clear and then place a new line...but we didn't have the time to do that. His iv's wouldn't last and he had no access for more iv's and he can't go without due to sugar issues so they wanted to place a new one on Thursday.
Thursday morning when we got down to surgery, they took us down around 6:30 in the morning, we found that both iv's had blown. So the dr's were paged and everything was rushed trying to get him back into surgery before his sugar dropped to dangerous levels.
Dr P was placing a new central line, taking a muscle biopsy from his thigh and Dr H was doing an endoscopy to check why he has had so much blood from his g tube and then he was going for his MRI with spectroscopy. He finally got done and back to his room about 1pm. But the fun didn't end there, the first time we flushed his central line, his chest got really red and streaky looking and everyone freaked out. It looked like it had blown the vein. So the surgery residents and Dr P came up and determined that it was good, he just had a reaction to local anesthesia they had used. *note to self, allergic to lidocane*
Today went well, Zach is pretty sore in his leg from the biopsy. He is fine if he just sits still but if he trys to walk or put any weight on that leg he crys. So we have him on tylenol every 6 hours to help. I also had a consult with the pallative care nurse today at the hospital. She works with the enhanced care team which is over chronically ill children and complex medical cases. She is going to be over Zach's case now, which I am hoping will be a big relief for me. She will coordinate all of his doctors and keep them talking and help with getting all his info to the Pittsburgh neuro dr and so on. I am really hoping that is helps a lot.
We got to go home this afternoon, he is on vanc and the fungal med for another 10 days at home. We also have several follow up appts, GI is in 2 weeks and we go back to see Dr P in 2 weeks to get biopsy results. They are also sending the MRI study and biopsy results to the Pittsburgh dr for him to evaluate. We still have about 3-4 more weeks on the bloodwork to come back so we are still in a waiting game. For now we just pray for no more infections and pray for good days to enjoy.
Sunday he ran low grade fevers all day but otherwise felt pretty good, he must have been pretty energized from the night before. Sunday night he ended up having hallucinations all night, he screamed and cryed and yelled that spiders and snakes and bugs were getting him...this lasted from midnight til 7am, then he slept some then continued throughout the day on Monday.
Monday of course was Halloween and Zach and Luke were both ninja turtles. Luke wanted to come see us instead of going to his halloween party at school him and Nana came up and all of them went to the party that the hospital had in the playroom. Right about lunchtime he started getting sick again, spiked a 104 fever, moaning, chills. So the doctors changed his meds around and put him back on Vanc, meropenum and changed his fungal med. Around 1 am Tuesday night, he started shaking really badly, spiked another fever and his stats kept dropping so we knew his line had to come out soon. His line had also starting clotting, you couldn't get any blood return on it.
So Tuesday his surgeon was back and the plan was to take Zach to the sedation room at 2pm and place him under sedation, place 2 iv's and remove his central line. They sedated him and the transport team got to work to try to find iv sites, unfortunately that is pretty hard in Zach. They had 5 transport (the team that rides on the helicopter) people and the PICU attending/anesthesia trying to place iv's and no one could get them in. Finally after about 30-45 minutes his surgeon got one. And then another was placed, both in his feet...poor boy :(
They had to run D10 in both iv's to keep his sugar levels up and still run the two antibiotics and fungal meds and fluid replacement. The next morning, the first iv had blown and transport had to come again and try to place another iv. Usually when you remove a line for infection, the plan is to wait 3-5 days for the infection to clear and then place a new line...but we didn't have the time to do that. His iv's wouldn't last and he had no access for more iv's and he can't go without due to sugar issues so they wanted to place a new one on Thursday.
Thursday morning when we got down to surgery, they took us down around 6:30 in the morning, we found that both iv's had blown. So the dr's were paged and everything was rushed trying to get him back into surgery before his sugar dropped to dangerous levels.
Dr P was placing a new central line, taking a muscle biopsy from his thigh and Dr H was doing an endoscopy to check why he has had so much blood from his g tube and then he was going for his MRI with spectroscopy. He finally got done and back to his room about 1pm. But the fun didn't end there, the first time we flushed his central line, his chest got really red and streaky looking and everyone freaked out. It looked like it had blown the vein. So the surgery residents and Dr P came up and determined that it was good, he just had a reaction to local anesthesia they had used. *note to self, allergic to lidocane*
Today went well, Zach is pretty sore in his leg from the biopsy. He is fine if he just sits still but if he trys to walk or put any weight on that leg he crys. So we have him on tylenol every 6 hours to help. I also had a consult with the pallative care nurse today at the hospital. She works with the enhanced care team which is over chronically ill children and complex medical cases. She is going to be over Zach's case now, which I am hoping will be a big relief for me. She will coordinate all of his doctors and keep them talking and help with getting all his info to the Pittsburgh neuro dr and so on. I am really hoping that is helps a lot.
We got to go home this afternoon, he is on vanc and the fungal med for another 10 days at home. We also have several follow up appts, GI is in 2 weeks and we go back to see Dr P in 2 weeks to get biopsy results. They are also sending the MRI study and biopsy results to the Pittsburgh dr for him to evaluate. We still have about 3-4 more weeks on the bloodwork to come back so we are still in a waiting game. For now we just pray for no more infections and pray for good days to enjoy.
Saturday, October 29, 2011
Another holiday in the hospital
Tuesday morning, Zach woke up like he always does...he was happy and playful for about 30 minutes and then got tired so he decided to take a nap. No problem, he tried to sleep in the car while I took Luke to school. I could tell he was starting to feel bad though, he was moaning and starting to look pale.
I put him back in my bed when we got home and proceded to get ready for the day...he had PT and OT later on. About an hour later I looked at him and he was completely pale, his lips were pale, his hands and feet were frozen and mottled. He was running a fever and shaking and throwing up. So off to the peds office, on the way there he looked really bad and I was actually scared.
His lips were really pale and his eyelids kinda looked bluish. I probably should have just called an ambulance but I trust his ped the most. When we got there he immediately got some blood cultures and vitals and gave him a shot of rocephin in his thigh. We then left for Brenners ER while he called the Dr on call. When we got to the ER they were waiting on him and started fluids and antibiotics. He was starting to go into septic shock but the two fluid bolus helped to perk him up. He spent the first 24 hours in ICM which is right outside picu, it is intermediate care.
He looked much better the following days and hasn't been running fevers until tonight when he spiked a102 fever. There has been much debate over him keeping his central line, he is growing a bug he has grown before in the same line so they are thinking of pulling it since we can't keep it bug free. This makes his 3 rd infection in two months with the same line and the 5 th since April. His surgeon is out of town til Monday and when he gets back he will make the decision about the fate of the line. Like I said he spiked a fever tonight so that's not a good sign.
He is also getting another blood transfusion as we speak, his blood count had dropped to 7.4 so that explains his paleness. He fell asleep before it even started but he had to get Tylenol for the fever and benedryl for the pre med so he was tired.
This week he has his MRI with spectroscopy and muscle biopsy and GI also wants to scope him again, which is good since he has been bleeding from his stomach for about month now. They went ahead and increased his protonix again and are looking into what else they can give. He is allergic to carafate so can't have that. And they are also going to start Flagyl once a month for bacteria overgrowth.
Hopefully that will help to cut down on him translocating bacteria from his gut to his line. But we will be here for another week at least which means Halloween and our wedding anniversary will be spent in here. I shouldn't be surprised, it seems like we spend every holiday and birthday here. We decorated his room today and he is going to wear his costume on Monday so we will still make the best of it.
Tuesday, October 18, 2011
What is Mito and what does it mean for us???
With our new diagnosis (it is still an unconfirmed diagnosis but all dr's are strongly feeling this is the diagnosis our son will have), we are learning what it is and what it means to us..
When we tell someone Zach has a Mitochodrial disorder, most people have no idea what that means...
and they ask if he will outgrow it?
or if there is treatment?
Sadly, we say NO
There is no treatment...There is no cure
....and we are trying to cope with that.
What is Mito??
"Mitochondria exist in nearly every cell of the human body, producing 90 percent of the energy the body needs to function. In a person with mitochondrial disease, the mitochondria are failing and cannot convert food and oxygen into life-sustaining energy. When mitochondria are not functioning correctly then the organs in our body begin to suffer"
Mitochondrial Disease is often difficult to explain and understand, theres such a wide spectrum of symptoms and severity.
This is why its so undiagnosed, misdiagnosed and considered "rare" when its really not.
How is it affecting Zach??
Zach suffers from Intestinal failure due to dysmotility and pseudo obstruction, Gastroparesis, EE, Sucrose Iso maltase def, Autonomic dysfunction (temp issues, bradycardia, low b/p, unknown fevers, abmormal sweating, flushing), Raynauds syndrome, Ataxia, Intermittent ptosis, Muscle pain/cramps and Muscle weakness and Fatigue, for now...
He will progress and get worse...
We know, that this may be the best he is..
These may be the best days of his life..
We know this is our life now
Hospital stays, ER trips, surgeries, TPN, line/blood infections, fevers, the bad days, the sleeping all day, the falling down, Dr appts, labs, dressing changes....and so much more
And we know one day
he will have questions....and what do you say?
How do you parent knowing the future you want and dreamed of for your child is not really their future?
You pray, and you turn to God
You teach them and show them LOVE, you give and take and you change because they will change you.
They are not ours to keep.....
but we cherish them while they are in our care.
Mitochondrial disorders are cruel...
Every 30 minutes, a child is born who will develop a mitochondrial disease by age 10.
Each year, 1000 to 4000 children in the United states are born with a mitochondrial disease.
80% of children diagnosed before the age of 5 won't survive to be 20.
Right now the statistics show Mito is as frequent as all childhood cancers combined and, sadly, just as deadly
THERE IS NO CURE, NO SURGERIES TO HEAL, NO REMISSIONS
Each year, 1000 to 4000 children in the United states are born with a mitochondrial disease.
80% of children diagnosed before the age of 5 won't survive to be 20.
Right now the statistics show Mito is as frequent as all childhood cancers combined and, sadly, just as deadly
THERE IS NO CURE, NO SURGERIES TO HEAL, NO REMISSIONS
so one way or another whether it happens slowly or quickly... we're forced to watch our children fall apart.
PLEASE help spread
Mitochondrial Disease Awareness.
http://www.umdf.org/
blog it, share it!
Friday, October 14, 2011
It's been a long week....
Ok, so I didn't update that we were in Pittsburgh or about anything that happened this week...really I was going to and actually tried to a night or so but just decided not to. It was a long week, exhausting, frustrating and overwhelming on a lot of aspects.
The hospital was HUGE! The city was way too crowded, too confusing and really not for me, I will stick with my small town (I consider Greensboro small). He got admitted on Monday and not much excitement for the day, just a lot of infomation taking and going over his story again to several dr's.
Tuesday he had an upper GI with contrast through his J tube, several xrays to watch it go down and some basic blood work. He did fine, they said the xray showed no dilation and so the GI docs were convinced he did not have pseudo obstruction. We also saw Neurology, the resident did the initial consult and took all the infomation then the whole team came back with the Dr for his opinion. He told us with Zach's history, the progression of his problems, the number of problems, and the motility tests and all the symptoms...he highly suspected some sort of Mitochondrial disorder :( We already had this in the back of our minds but still....
He said all of his symptoms fit with this diagnosis and he wants Zach to get a brain MRI and his muscle biopsy. He also ordered lots of bloodwork.
Wednesday, they tried to do an upper GI with contrast through his G tube but he was still full of contrast so they couldn't. We met with the transplant surgeon who thinks he indeed has pseudo obstruction, says it is intermittent and says 9 out of 10 times you don't see it on xray. But based off his motility study and the actual pseudo obstruction episode he had a month ago.....he says he has it. He actually laughed at the fact that the GI thought the contrast would be gone. The down side is he said that Zach can't be listed for transplant (although he needs one or will need one) without knowing what kind of mitochondrial disorder he has. He explained that he likes to give benefits for a transplant and right now he doesn't have any for Zach. He can't say doing a transplant would allow Zach to live any longer than he would without one.
So that means....depending on how progressive his Mitochondrial disorder is, it isn't worth doing a transplant right now.
On Thursday, they tried again to do the upper GI and still couldn't due to him not moving the contrast out of his intestines...no big surprise really. So we got to leave that afternoon and drove home. We got home around 11 pm and got to sleep in our bed..that is me and Zach sleep in our bed and Luke and daddy sleep in their bed.
I know, not the traditional way but right now until daddy finishes making Zach's new bed that will fit right beside ours, that is the way we sleep.
So for the acomplishments of the week in Pittsburgh....
He will start PT and OT therapies for several reasons. He has a new diagnosis of SPD which is sensory processing disorder and needs OT to help with that. And he is also losing coordination in his legs, probably mito progression so he will get PT for that.
He will get a brian mri and muscle biopsy to help determine the type of mito.
He is diagnosed with CIPO so at least we are all in agreeance as to the issue of the gut.
He does qualify for a transplant..if needed and able to get later down the road but for now he still has all of his central line spots open (which is a huge sigh of relief) so it is not dier for transplant right now. Which is good too, since success rates for transplant are only 85 % at 5 years and I am not ready for those odds.
His pediatrician and GI dr here are setting up appts with neuro, a mito dr at UNC, the biopsy, the mri and the therapies. And we still have a follow up with immunology. I did find out today that his microarray came back normal which determines he does not have a problem with his chromosomes so it is pointing more to mito.
So now we wait for results and more tests...not a lot of good answers but at this point we just want to know.
The hospital was HUGE! The city was way too crowded, too confusing and really not for me, I will stick with my small town (I consider Greensboro small). He got admitted on Monday and not much excitement for the day, just a lot of infomation taking and going over his story again to several dr's.
Tuesday he had an upper GI with contrast through his J tube, several xrays to watch it go down and some basic blood work. He did fine, they said the xray showed no dilation and so the GI docs were convinced he did not have pseudo obstruction. We also saw Neurology, the resident did the initial consult and took all the infomation then the whole team came back with the Dr for his opinion. He told us with Zach's history, the progression of his problems, the number of problems, and the motility tests and all the symptoms...he highly suspected some sort of Mitochondrial disorder :( We already had this in the back of our minds but still....
He said all of his symptoms fit with this diagnosis and he wants Zach to get a brain MRI and his muscle biopsy. He also ordered lots of bloodwork.
Wednesday, they tried to do an upper GI with contrast through his G tube but he was still full of contrast so they couldn't. We met with the transplant surgeon who thinks he indeed has pseudo obstruction, says it is intermittent and says 9 out of 10 times you don't see it on xray. But based off his motility study and the actual pseudo obstruction episode he had a month ago.....he says he has it. He actually laughed at the fact that the GI thought the contrast would be gone. The down side is he said that Zach can't be listed for transplant (although he needs one or will need one) without knowing what kind of mitochondrial disorder he has. He explained that he likes to give benefits for a transplant and right now he doesn't have any for Zach. He can't say doing a transplant would allow Zach to live any longer than he would without one.
So that means....depending on how progressive his Mitochondrial disorder is, it isn't worth doing a transplant right now.
On Thursday, they tried again to do the upper GI and still couldn't due to him not moving the contrast out of his intestines...no big surprise really. So we got to leave that afternoon and drove home. We got home around 11 pm and got to sleep in our bed..that is me and Zach sleep in our bed and Luke and daddy sleep in their bed.
I know, not the traditional way but right now until daddy finishes making Zach's new bed that will fit right beside ours, that is the way we sleep.
So for the acomplishments of the week in Pittsburgh....
He will start PT and OT therapies for several reasons. He has a new diagnosis of SPD which is sensory processing disorder and needs OT to help with that. And he is also losing coordination in his legs, probably mito progression so he will get PT for that.
He will get a brian mri and muscle biopsy to help determine the type of mito.
He is diagnosed with CIPO so at least we are all in agreeance as to the issue of the gut.
He does qualify for a transplant..if needed and able to get later down the road but for now he still has all of his central line spots open (which is a huge sigh of relief) so it is not dier for transplant right now. Which is good too, since success rates for transplant are only 85 % at 5 years and I am not ready for those odds.
His pediatrician and GI dr here are setting up appts with neuro, a mito dr at UNC, the biopsy, the mri and the therapies. And we still have a follow up with immunology. I did find out today that his microarray came back normal which determines he does not have a problem with his chromosomes so it is pointing more to mito.
So now we wait for results and more tests...not a lot of good answers but at this point we just want to know.
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