Didn't make it

We tried really hard to keep him home for Christmas, he was fighting and suffering since the tuesday before but was ok..but by Saturday it was clear that the pain meds were not enough. They had already increased the methadone that morning and told me try not to use the diluadid aaa much for fear of it making his episode worse.
We did dinner for the family that night, and presents for everyone except Santa gifts. Poor Zach wasn't even awake for more than ten minutes of it and didn't have the energy or interest in any gifts or family. By 9:00 that night (christmas eve) I was on the phone with his pediatrician, the pain was worse and he was really out of it. So we were sent to the hospital :(
Luckily we got one of our favorite nurses taking care of him and they started him on iv diluadid every hr as needed for pain. They did x rays and ultrasounds, but they revealed nothing. His gallbladder has developed some sludge but thatnis normal for being on TPN and no enteral feeds. His blood counts had dropped yesterday so he received a blood transfusion last night. They also were checking into his anemia conditions and for now have decided that it fits more with chronic disease anemia and he will just receive transfusions as needed for now. He could get iv iron infusions but they feel the benefits do not outweigh the risks right now.
As of today, he is really congested..he probably just picked up he cold that daddy had the week before christmas. He is also still puffy in his face and belly, still nauseous and has already thrown up a couple of times and still having breakthrough pain. The plan is for the dr's to call Pittsburgh and try and move him up there to finish evaluation and to help with pain/ vomiting control.

Sorry

Sorry I haven't updated in a week or so..I cannot even express how crazy it has been around here. We got home on Friday night, with a still nauseaus Zach but he was fast asleep when I pulled up in our driveway. It was so good just to be home..to sleep in our bed and to have both my boys together again.
Saturday and Sunday they were inseperable, they played and followed each other around everywhere. Monday was also not to bad for him but by monday night the pain was back and nausea was getting worse. He screamed and cried in pain all night and by tuesday morning I was waiting on his ped to call me back about what to do.
They decided to stop his feeds and hopefully they would end the pain. He started throwing up Wednesday morning and it has only gotten worse since. Yesterday his home health nurse pulled cultures and blood work, even though he isn't running a fever..infection is always still possible. I along with everyone else right now, believes it is another psuedo obstruction episode. He is still in tremendous pain, vomiting every 30 min to hour and his g tube and j tube are draining lots of air and bile.
We are trying everything to try to keep him home for Christmas, he is getting extra fluids and I have him hooked up to a GI suction machine to help with nauseau. He is also getting zofran every 8 hrs, phenegren every 4 hrs and dilaudid every 3 hrs or so on top of the methadone infusion. He isn't running high fevers, and blood work isn't pointing toward infection. So we are ok with trying to treat at home, really there isn't anything differently they would do at the hospital.
Please just keep praying he can make it through Christmas at home. Also keep my family in your prayers this holiday..we have been through a rough week. My husbands grandfather passed away on Friday, my father passed away on Monday and my husbands aunt passed away on Tuesday (the morning of his grandfathers funeral) And then with Zach being sick on top of all of this, it is just going to be a hard Christmas for all of us.

Going home and other plans

So we are finally going home tonight, probably around 8:30 or 9 pm after his last rounds of antibiotics and antifungal meds. He is going home on lots more meds and more pumps so it will take some getting used to.
He now has
*IV reglan every 6 hrs
*IV zofran every 8 hrs
*IV protonix 1 x day
*TPN 20 hrs a day
*Fluid replacement 24 hrs a day
*IV infusion methadone 24 hrs a day
*IV ethanol locks for 4 hrs in each lumen once a week
and
Elecare/pedialyte mix running enterally 1 cc/hr 24 hrs a day
Phenegren suppository every 4 hrs prn
Diluadid suppository every 1 hr prn

I think that is enough meds and stuff to keep up with for now.

His dr's met earlier this week and had big discussion about him and his plan of care. The biggest changes right now are they started him on the mix of elecare and pedialyte at 1 cc/hr 24 hrs a day to try and buy us time in preventing infections and not losing access. Everyone tells me that it is not feeds, no one is trying to feed him enterally...it is just to buy time. I am trying to think of it like that, because feeding scares me...we have been down that road several times and it never ends well.
They also discussed sending him to a rheumotologist at Duke and still going to Pittsburgh to see what they think. I already heard from insurance about Pittsburgh, they are approved so just waiting on our dates to go up there. Hopefully they will let us know soon.

no much going on

Really not much to update...we are just hanging out until he finishes antibioitcs and we get his pain meds approved for home use.
He is doing really good, no extra pain meds have been used in a couple of days..which is awesome! It is so good to actually see my happy boy back, and to be getting sleep at night. He has been in pain for 2 1/2 years now and has been waking up and screaming every night and it is so great for him and us to finally get sleep. He looks so much better now. The IV pain pump is wonderful! Hoping we can go home on it...that is the plan as long as home health allows. We might have to switch over to Kids Path, which is a kids hospice company to get approved for the pain meds but we will see. I would really hate to lose our nurse we have now, she only comes once a week to do labs and check up but she is family now. We have had her for a year and a half.
Still waiting to hear dates for Pittsburgh as well, insurance takes forever! But hopefully by the end of the week we will know something.
Yesterday, his neurologist suggested that he had a mixed connective tissue disorder. We need to see a Rhuem Dr to get diagnosed. But all of his dr's are meeting this afternoon to discuss him and try to come up with some ideas. I am praying they can have some "light bulbs" going off and come up with some good plans. I should hopefully hear tomorrow what they discussed.

Whats new

Its been a long couple of days..waiting for answers.
Dr H came by a couple of days ago and said that they heard from Pittsburgh and that the dr's up there needed to discuss Zach and decide whether or not they still wanted to transplant him. All of his mito tests so far have came back negative, but with his biopsy showing some sort of myopathy they were worried.

This afternoon, Dr H emailed me with a wonderful email from the transplant surgeon in Pittsburgh saying that they are bringing Zach up there, they are just waiting on insurance approval!!

Zach is so lucky to have such wonderful dr's that care about him so much. Dr Y is so sweet and told me yesterday that even if Pittsburgh didn't want him, he still does...aww, love him!! His entire office and him care so much for my boy. And here, he has the best GI docs..Dr H is great and even the ones we don't see often, want to make sure he is comfortable. His surgeon is awesome, Dr P is so caring...he is really worried about Zach and wants him transplanted asap.

And on the pain note...we are working on that. The pain team came by yesterday and put Zach on methadone. They started with a bolus of methadone then put him on an infusion to try to find the right dose. They also increased his dilaudid frequency to every hour as needed.

Today, they discussed what would be best to go home on. He is thinking about sending him home on a PCA pump with a daily infusion of dilaudid and a button with the ability to give extra dilaudid boluses as needed for breakthrough pain.

They think he definitely needs to be on an infusion of meds to get the greatest results and then the extra meds for extra pain.

We will remain inpatient for the weekend at least. Dr L, his neurologist will be coming by on Monday to see him again. He is trembling at times, and she can give us her opinion on his biopsy and other symptoms. And hopefully by then, the pain team will have figured out the best home meds.

And then on Wednesday all of his dr's are getting together to discuss Zach and hopefully come up with some good ideas.

We are having some good days now...he played really well today. Him and his favorite nurse, Ryanne laughed and played today. They squirted each other with water syringes and got each other in trouble. He is so lucky to have a nurse that loves him so much and goes far above her "nurse" duty to care for him. 

"Lator Gator" Ryanne!

nothing to update

Nothing really to report today...GI came by this morning and they brought up the idea of the obstruction being due to his mickey button in his J tube. We have been down this road before, so I do not believe this is the problem AT ALL! They did an xray today to see if the contrast from yesterdays endoscopy made it past the button and yes it did, so wonder what tomorrow's big idea will be.
He is doing good today, cultures are still negative.

Update

Zach had an endoscopy today around lunchtime to rule out the possible obstruction. They also did some contrast while he was under and got some flouroscopy pictures. Of course they didn't find an obstruction...it would have been so much easier and so much better if he would have just had an obstruction.
I didn't see GI after the endoscopy so not sure where we go now. Hopefully Dr Hill will be by tomorrow morning to enlighten me.

His autonomic garbage keeps acting up more and more....he is very clammy and sweating like crazy, causing his central line dressing to not want to stick and any tape I put on it isn't helping. He is actually kinda melting the monitor leads that on his chest, we have to keep replacing them everyday.

Still no talk about when we get out of here. He is still on 2 weeks of antibiotics and fungal treatment but that doens't stop us from going on....his GI uncertainity is what henders us.

gut bugs

So things were looking good infection wise, we were still trying to figure out the obstruction issues and then....he spiked a 103.7 fever. After an interesting experience with some residents who didn't know enough to satisfy me, he finally had some cultures and blood work drawn. And less than 12 hours later he grows 2 different gram neg bacterias.

The next day, he was no longer running any fevers but his heart rate was very tachy and he became very swollen, so we were transferred down to IMC again :( This time it was uneventful except for the test with the tp tube that was supposed to reveal if he had an obstruction. Thats a long story but to sum it up...it was unsuccessful.

The two gram neg bugs were identified as a type of E coli called Citrobactor and then a psuedomona. Both gut bugs. So now he is on antibiotics again, this time a different one because the bugs he grew this time are smarter and more advanced than past ones...they are becoming antibiotic resisitant. Not good news at all!
But he is doing better, no fevers and playing again. We finally got negative cultures starting on saturday so now 14 days of antibiotics and also extending the micafungin treatment the full length of the antibiotic treatment.

On another note....both his surgeon and the Id docs think he needs to go to transplant ASAP but that also brings the problem of you can't get listed or stay listed with infections. His GI doc came by late this afternoon to give us an update on what they are doing. They have spoken to Pittsburgh and also believe he needs to go there but now Pittsburgh is hesistant about transplanting him due to not knowing the underlying condition.

So bascially we just sit and wait for Pittsburgh to make a decision. His GI doc would like to just transfer Zach up there from here so that they can help him.

So I am left wondering what happens now, what if they don't accept him, where do we go from here? I am hoping we get some answers soon. It looks like we will either be here or there for Christmas probably...there is no talk of us going home right now.

This is the condensed version..we are processing a lot right now, and my thoughts are kinda random. I will try to update again when we know more.

Where do I begin

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.

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.

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.

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.

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

 

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!

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.

Thank you

I am truly thankful for all the wonderful people that care for my baby boy.

He has the best Pediatrician in the world, he drops everything to talk to me or meet me after hours to look after Zach. He is always on call for Zach and I can not say enough how much it means to have a Dr that stands up for you and your child and fights for your child like he has. I completely trust him with Zach's care.

I am so thankful for the entire Piedmont Pediatrics office as well, they are wonderful and they all care and love Zach more than anyone could ask. I am especially thankful for Tonya, Kathy, Heather and Leigh Ann (even though you are no longer there). Thank you so much for everything!

And as much as we hate being in the hospital, when we are there I know the nurses that take care of him, love him and truly want to see him better. He has all the nurses charmed with his big blue eyes and little smile. They all spoil him, we could not ask for better care when we are there. I wish I could just take them all with us to Pittsburgh so they could look after him there too. Thank you so much Ryanne, Kelly, Stephanie, Brittany, Lindsay, Tracy, Mindy, Leslie, Molly and Holly for all you do for Zach and for all the love you have for him. I could not ask for better nurses!!!

I have so much thanks for his home nurse and friend, Becky. She is so much than just his home health nurse. I can call her anytime for advise or questions about Zach. Even if she isn't on call she will come see him. She loves him and Luke so much and is always there to help me.

And a very special thank you for my mom, Benita. You are the best Mom and Nana anyone could ask for, I couldn't imagine going through this without it and I am so thankful for all you do for us and the boys. Luke and Zach are very lucky to call you Nana.

Again...really again, we are in the hospital

Zach woke up with a fever Thursday morning...well actually both my boys woke up with fevers :( Luke had just a low grade fever with a really stuffy nose and allergies so it was obvious and nothing a little benedryl and tylenol can't fix. Zach on the other hand was not obvious and his fever was 103 so of course we wind up at his peds office when they open. Then it is straight to get blood cultures drawn and a cbc done.

I wait for the ped to call telling me they think Zach needs antibiotics and go to Brenners. I feel like saying blah..blah...blah, at this point I really stop listening after I hear hospital and think I knew it, I knew this morning when he rolled over and touched my arm with his leg and it was burning up that we would be at Brenners by the end of the day.

So, we are.....luckily nothing is growing so far on the cultures, no more fevers since yesterday!! He feels good, still tired but he is still having blood sugar issues. Last night his blood sugar dropped to 36 while the TPN was paused to give meds. They had to give a bolus of extreme sugar water to bring it up.

Today we are waiting on the endocrinologist to come by and discuss what he/she thinks it might be and why. The GI thinks this could all be related to autonomic/mito issues :(  They also switched his TPN back to 24 hours a day so, sorry liver....please hang in there.

Good news!! I got the word today that Pittsburgh is fully approved by insurance and Zach goes on Monday!!
The catch.......as long as he doesn't grow anything on his cultures!! So praying, praying, praying and more praying that nothing grows.

What?

So today has been a whirlwind of craziness. First off, lets start by saying on Monday when his weekly labs were drawn for his TPN, they found his blood sugar level was 40. He has also been very fatigued the past couple of days...sleeping anywhere from 16 hours to 22 hours a day. So the dr's decided that he needed to
have his blood sugar checked every 3 hours for a 24 hour period. And they wanted an insulin and glucose pulled from his line this morning.

So this morning, his nurse came to draw his insulin and glucose and we did the first of many blood sugar checks. It went fine, he actually slept through it...but then again, all he did today was sleep. He slept for 19 hours today! That has been the story all weekend, he wakes for about an hour and then he sleeps for 3 or 4. this is certainly not our Zach.

For the first 2 checks his blood sugar was fine....79 and 124. And then when he got unhooked from his TPN and I checked it about 10 minutes later, it was 48! Low again, so by this point his lab results from this morning were back and his nurse had already called his ped about his results and his sleepiness.

Apparently, his ped talked to an endocrinologist and they decided that with his insulin level and glucose levels, his body doesn't have any reserves so he is using up the glucose faster than he makes more and so he can't handle changes in blood sugar. So they increased the glucose in his TPN and we will try that for 2 days and then recheck his level on Friday.
Hopefully this is why he has been sleeping all the time.

And on the Pittsburgh note, I heard from them today but no news.....the nurse said the dr is still reviewing his files and trying to decide what type of appt he needs. He is talking with the transplant director as well and together they will decide who he needs to see. So they don't have an exact date yet...but hopefully soon.

It's Ok

While reading another blog, I came across this post. It is hard to explain your feelings some times and people just don't understand what it is like living day by day and never knowing what tomorrow brings or if we will be back in the hospital. If the roles were reversed, I can't say I would understand either. I never understood until I was placed in this role and that's ok..I wouldn't change a single moment I get to spend with Zach....even if it is not knowing what's next.

*It's OK to say that you are sorry, or that you are sad. It's OK to empathize and tell me that this must be hard, or that this must hurt, or that you wish this weren't happening. It's also OK to say that you don't know what to say!

* It's OK to cry, and it's OK if I cry. Trust me - you aren't making me cry, and you aren't making me sad. I cry every day and I'm already sad. It can be helpful to cry with someone else who cares about Zach and about our whole family, and it is healing in a way to know that other people care deeply about him as well.

* It's OK for me to NOT be sad all of the time, and I need to feel safe smiling and laughing when that is what I want to do. I have so, so much for which to be thankful, and so much that makes me smile. I have 2 wonderful boys that need happiness and smiles and laughter admist all this turmoil.

*Please don't tell me that God doesn't give us more than we can handle. I just don't see that in His Word. I do see that His strength is made perfect in our weakness. I believe with all of my heart that He DOES give us way more than we can handle, and then He steps in and carries us. I am not superwoman. I am not stronger than you or more capable than you. I'm not living this life because I am some kind of hero - I'm living this life because I have no other options. It can be very awkward when people try to put me on a pedestal and assume that I am more than who I really am. I'm just a wife and mommy, just like you. I get tired and I get discouraged. I get impatient sometimes. I feel whiny sometimes. I get overwhelmed sometimes.

*We always appreciate it when people offer to help, but please understand that if you say, "Call me if you need anything," or "Please let me know if there is something I can do," you probably aren't going to hear from us. :-) It is hard, uncomfortable, and honestly unrealistic for us to call people whenever we need something. It doesn't cross our mind to start calling friends with a wish list, and even if we know you want to help, we don't want to ask for something you didn't really mean to offer.

If you want to help, there are plenty of ways to do so, but we are usually so tired and overwhelmed that it works best if you simply call/email and tell us that you want to bring a meal, bring a restaurant or grocery store or Walmart gift card, that you want to put gas in one of our vehicles. It would even help if you said, "I want to meet a need for you. I have this much $$ or I have this much time, and I want to know what I can do to help with that $ or that time." Even that gives us permission to really ask, because we know that what we ask falls in the parameters of how you really meant to help. "Either or" offers are always a blessing and very easy to accept.

I'm not saying that to solicit these things. I'm saying it because we get lots of general "Please let me know what we can do" remarks. We KNOW that you mean them, and we know that your intentions are good, but specific offers are much easier to accept. If you ask if we could use one of the things I just listed, I'll say yes, but I won't call you to tell you that we need gas, or that I'm so tired that I can barely think about making dinner.

*It's OK to ask questions. Again, it won't make us sad and it certainly won't offend us. When someone asks about Zach's meds or care needs or asks for more explanation about what is going on with him, we feel less isolated because we feel like maybe now someone understands a little bit better. Please understand, though, that if you ask "How's Zach doing," we won't mind AT ALL, but we may get a deer in the headlights look and be unsure how to answer. Chances are that you'll get a short, basic answer like, "He's hurting," or "He's tired," or "He's ok right now," simply because we don't know how much you really want to know. We also don't know how much you really know already - we get thousands of hits on his blog  or facebook many days, but only a handful of comments, so we don't even know who's been reading and keeping up with things. Again, we'd rather have you ask how he's doing than to ignore the situation, but please don't be hurt if you get a short generic answer - and please feel free to ask for more details!

Not so good day

Zach has been awake for all of 3 hours today....he is just not doing well today at all. During the 3 hours he was actually awake, he was horrible. He was very tempermental and irritable. I don't even know what to do anymore, nothing makes him happy. He just wants to be held all the time or lay in the bed. I feel so bad for him and really want him to feel better.
I think he actually smiled and was in a good mood for about 30 minutes. Not long but at least he smiled. He also had more dried blood output from his G tube overnight which is still a mystery.
Maybe tomorrow will be better.

Quick update

We are home, Zach got discharged tonight around 8pm after his last dose of antibiotics. We are praying all goes well this weekend and we will not be making anymore trips back to hospital for a while. We are supposed to hear from the Dr at Pittsburgh on Monday for him to tell us when they want Zach there. Hoping it is very soon, we are just playing russian roulette with this central line....we are lucky with the last two infections, he hasn't lost a line.
Also tomorrow his new pediatric walker will be delivered. This will hopefully allow him to move around more and build his endurance and muscle strength back up. When PT came today, she tried to have him walk around some and after about 20 steps or so he was breathing really heavy..he just doesn't have the energy or endurance anymore to move around.
Please help us pray that baby Zach makes it to Pittsburgh this week and stays infection and fever free :)

Changes

So plans have changed...again...instead of going to Cincinnati, since insurance wasn't approved for transplant or GI consult we have decided to go to Childrens Hospital of Pittsburgh instead. I have heard it is a great hospital and insurance approves...which means a lot. The dr's and hospital social worker are all working on getting him up there. He finished his antibiotics tomorrow, and will stay over the weekend to be observed then hopefully early next week we will drive up for the consult and admit to get him evaluated. The thing about Pittsburgh I am looking forward to is they have a Mitochondrial center as well, so at least we can get that testing done while we are there. His genetic dr here has done some broad testing but no specific Mito tests yet but since it is a big possibility, I think the sooner the better would be nice on the testing.

He has been doing pretty good today, only a few meltdowns....I am really beginning to wonder if he has this disorder called sensory processing disorder...he seems to really struggle with some sensory issues and gets a overloaded a lot causing big meltdowns. He also doesn't like stuff on his hands, has to have his sleeves pulled down on his shirts, over sensitive to loud noises, is easily distracted and much more but I will ask OT about it when they come by. His dr ordered PT and OT to come by and check out his issues with walking and muscle pains right now. He doesn't want to walk anymore and if he does he is very wobbly so hopefully they will have some insight.

And, yet again..I have said it thouand of times...I love his his Pediatrician and his entire office!! I can't tell you why but just believe me, they are the best and most caring people around. I will never know how to repay them for all they do for our family. They truly love Zach and his brother.

Genetics, Insurance and waiting

Two weeks ago, the genetic doctor ordered some broad DNA bloodwork to check for abnormalities. He was supposed to have his follow up appointment next week but hopefully we will not be here so his GI doctor went ahead and asked the genetic dr to come by.

We had not met him yet, he just ordered the bloodwork last time but didn't come by because he was out of the office so I was very happy to finally get to meet him and ask him questions. His nurses came by first and went through all of our family history and zachs medical records, my pregnancy with him and my other sons health. Then Dr C came in and did his physical exam and went over some of the bloodwork that he has had done and said we are still waiting on more bloodwork to come back. But what has come back has only told us that he has the correct number of chromosomes.

He told us that with Zach having so much going on and with it being progressive and our history of maternal issues being passed down, Zach has a high probability of having some sort of Mitochrondrial disorder. He wants him to have a muscle biopsy taken the next time he is under anesthesia for any reason. And he already has his DNA stored so they can do further testing without having to draw more blood. He wants to wait on the micro array to come back and whatever else he ordered first then try to narrow down from there.  Unfortunately this could put a damper on him being approved for an intestinal transplant if he ends up going down that road.

On another note, We found out today that our insurance company does not have Cincinnati in their network for intestinal transplant centers so if we went there (if he ends up having to have one) we would have to pay for the entire thing out of pocket. The only hospitals they approve are Pittsbourgh in PA, Georgetown in Washington D.C and one in Fl. So that was a shock today, to say the least!

His GI doctor said for now lets at least get him to Cincinnati to be seen by the top GI's first and if they decide he does need a transplant then we will transfer to another hospital, one of the approved ones. But we are still just waiting on the green light to just to get seen by the GI doctors. And since it has taken all week, we can't go till next week if we are given the green light. 

The dr from there called me yesterday to see what my expectations were, and let me know what they were going to do. He said they may or may not repeat tests and there is a good chance they won't find any answers since he has already had an extensive workup here and no answers have been found.

Today, Zach has been having more pain and is draining a lot of dried blood/coffee ground looking stuff from his G tube and we have had to start draining his J tube again due to the pain and leaking. He also threw up last night so that is unexplained. He has been very moody and irritable, it is heartbreaking. We tried to take him outside to the rooftop playground earlier and he took 2 wobbly steps and then crawled back up into Nana's arms and wouldn't get down saying he was hurting.

So we are stuck waiting again for answers....I have heard the Mito wait is long, it is not one that we want to be on but I know we are not alone, there are a lot of special kids on this road as well and we pray for them.

Looking forward

 

We are looking forward to this coming week, he is definitely being transferred up to Cincinnati Childrens Hospital. We are just waiting on the day, and the transportation to get taken care of. I believe we will be taking the Angel Med Flight, since he is stable but he is still on 3 IV antibiotics, replacement fluids and TPN. He is also still under isolation for his rhinovirus...which is simply a cold but we can't risk him getting anyone else sick or him catching anything else while his body is under so much stress already.

Lately, Zach has been experiencing more autonomic dysfunction symptoms, he has been getting flushed in his face and ears really easily..every time he gets excited. He also has not gone to the bathroom in a week...7 days!! But it doesn't seem to be bothering him so we will jut wait and see what happens there. His liver enzymes are showing elevations again, so they are decreasing his lipids in the concentration...lipids are the makeup of fats so less fat but that is better for his liver. He has also been having more leg pains or muscle pains and cramps over the past week or so. And he won't walk, well maybe 2 or 3 steps and that is it. When he does, you can tell he is having difficulty....its like he doesn't bend his knees and he is really wobbly so tomorrow they will also check a CPK to see any muscle inflammation.

We are not going to be here for his immunology or genetic follow up appts so they are going to have them come by and see us before we transfer. I am really anxious to actually talk to the genetic dr. I haven't met him yet, he just ordered a bunch of labs a couple of weeks ago so hopefully they are back and we have some answers there.

Tomorrow is also Daddy's birthday, he gets to spend it at work and us in the hospital so not much fun there but we love him and wish him the best birthday!!!

 

 

Yesterday, daddy and Luke came and picked me up at the hospital to spend the day together. We went to Mt Airy and watched the Mayberry days parade with all the old Andy Griffith memorabilia. That was cool, then we made our way to Pilot Mountain...or as Luke calls it Moleberry Hill. That was really nice, it is so beautiful there and it was nice to get out and get some sunshine and see my babies. I am really going to miss them when we transfer.

 

Long over due update

So I know it has been a couple of weeks since I updated, and  a lot has happened and is still happening. We got discharged last thursday the 15th only to be readmitted on tuesday the 20th.

They sent him home with intentions to send him to Cincinnati Childrens hospital outpatient and let them direct admit him so that he can see their motility specialists and intestinal failure team. He was stable when we left, no fevers and feeling pretty good. We got to spend a good weekend at home, he wasn't himself but we know he has been through a lot in the past month so to be expected. His legs have gotten really weak and wobbly and he has begun to complain about leg pains.

Sunday morning he woke up and was very shaky so his pediatrician had us get some bloodwork at the local hospital to check his CBC and some blood cultures. We were thinking it could be blood sugar issues but by the time we checked it was fine. Monday, his nurse came and drew labs again and this time his blood sugar was low, 60 so we have ordered a glucometer to help keep track of them. This could be all related to the TPN but who knows.


Then on Tuesday afternoon when he woke up from his nap, he had a temp of 103.5 rectally....really not good so off the the pediatrician again :(  We got there and he couldn't find any easy answers so he called the GI on call at Brenners and they decided he could stay home and wait for test results. So we had to go back to the local hospital and get labs drawn again. This time they ordered a new test called procalcitonin which shows elevated when bacteria sepsis is a high probability

.
An hour later the pediatrician called and said it was really elevated so he has to be admitted. So off to Brenner's again. We got checked in and to our room around 8pm tuesday night. And sure enough the next day, his cultures came back positive and he also has tested positive for rhinovirus. So poor baby, he can't catch a break.

His nap that led to his fever

Not himself, but glad we had a 4 day home vacation

He is feeling better now, no more postive blood cultures but still on IV antibiotics for 10 days. And we finally made some progress with moving forward to Cincinnati. After a long email to his primary GI doctor about how nothing has been done and nobody seems to follow anyone elses plans, we made progress. Cincinnati has all of his records and are reviewing them and coming up with their plan of action.

He is going to be seeing their motility specialist, Dr Kaul and their intestinal rehab/transplant specialist, Dr Koccoshis. Hopefully they will be able to come up with some plan for Zach. The GI on call this morning informed that he should be transferring up there next week. The financial person here at Brenner's is working on his insurance information and we just have to wait for Cincy to say we are ready for him. I am not sure if we will be going on the helicopter or what, but that should be interesting. 

And we are still waiting on immunology labs and genetic labs to all come back, maybe they will give us some insight to why he is having these issues. 

A lot to take in

It has been an interesting last couple of days to say the least. All of his doctors finally got together on Wed morning, his surgeon, his primary GI and 2 other GI doctors that have taken care of him a lot. They all discussed him and what was next, what could be causing the issues and where do we go from here. They finally came to the agreement that his previous roux en y surgery was not causing any issues and that he did not have any mechanical obstructions anywhere in his intestines.


Everyone is pretty much in agreement that it is his small intestines that are just not working right. No matter what the motility test showed. He had one abnormal AD manometry study and 1 normal study so it could be intermittent but right now it is acute. They also decided to do some colonic manometry to see if he was having issues with his colon and could be pushing some stuff backwards. The other opinions were doing an illeostomy to bypass some of his intestines to see if that would help and the idea of an intestinal transplant.
They had a cancellation yesterday so he got to go down to have the colonic manometry placed in surgery. That test ran yesterday afternoon and most of today. It was pretty disgusting, actually...poop everywhere so that was fun. Still waiting on the results of that, probably will get them tomorrow. I am pretty sure it will be normal...he seems to not have a problem with having runny diapers.


Today, one of the GI doctors was also telling me about a child she had in fellowship that had similar issues like Zach and he went on to have an intestinal transplant. So that is a big possiblity. She doesn't think the illeostomy would work, if his small intestine is the problem then it won't help to just attach it to his skin. She is also going to talk with his primary GI about a trial run of cisapride. She said the med used to work wonders for motility issues and they took it off the market because 2 children died while taking it (they had undiagnosed heart issues) but still, scary. He would have to have a full cardiac workup first. But since the medicine is just coming back on the market, they are only doing limited trials and might not be doing it here.
If he did have to have an intestinal transplant they reccomend Pittsburg as the number 1 choice. I have never been there so at least a change of scenery.


They pulled half of his immunolgy labs this morning and are going to pull the other half tomorrow morning. His blood count has taken a huge hit with all the blood they have been taking and so he might have to get another transfusion soon. And she said today that his labs are showing that the TPN is already starting to damage his liver...which is not good at all, he has only been on it since April and it is vital to his survival.
So as you can tell, we have a lot to take in...there is no signs of going home right now, which is fine because going home scares me.  He is still having lots of pain and they can treat it better here. Also they are going to send genetics by to do an assesment on him. This is very helpful, if he has some sort of genetic issue it could change the course of action for eveything. Well I think that is everything for now. Please keep praying for him. He is feeling better right now which is good but even through his good days he is very sick on the inside.

He tries so hard to always feel good and always smile and laugh. He is such a wonderful 2 1/2 year old and has stolen every nurse's heart up here. He has gotten so rotten, but we love him.

Part 3

Zach had surgery on thursday to look for a possible obstruction in his small intestines. The surgeon couldn't find anything so he just placed a longer j tube...about 10 inches long into his intestines to see if that helps. The first day he was very sore and was on morphine every 2 hours as he recovered. Luckily the surgeon used the same incision he had used for his roux en y so still only 1 long scar. He was also very nauseus the first day but they had to take out all of his intestines and then put them back in so his body had to adjust. He lost lots of fluid during surgery and whole day he drained dried blood from his g tube. Which is all normal they tell me. Yesterday he was still draining bile from his g tube so they were holding off trying out the new tube just yet. Last night it also started leaking bile from around the stoma, which is the problem we were having to begin with so obviously the tube didn't solve that. We will have to wait and see what happens when we add feeds back in. We are still waiting on him to use the bathroom first before we can start feeds and it has almost been a week and nothing. This coming from a child that has 4-5 dirty diarrhea diapers a day so to me something isn't working in there.
Tomorrow we should get to see our regular doctors again so maybe they can answer some more questions or bring more insight.

surgery

They couldn't get the longer tube placed in his j tube spot today. It was blocked somewhere and so he is having surgery tomorrow sometime. They might have to remove some intestines, won't know for sure until he goes in and sees what it is or how bad it is. Just pray for Zach. This will be the 3 rd time under anesthesis in 3 days. :(

Updates part 2

So Zach's infection is looking a lot better, no more positive blood cultures since friday. No more fevers since late saturday night. That is so good, for once his infection cleared without having to pull his line. On another note, sunday when he nurse gave him his meds, they immediately came back out around his j tube stoma. Which has never happened before, it has been leaking excessivley since tuesday of last week but I had never seen it do this. So they scheduled him for a ct scan on monday morning to check his abdomen.
Monday morning came and they decided that a ct was not the best option, but another test called a double contrast study would work better. So he went down to have that done at 1pm. They injected barium contrast then air into his j tube and watched it move...or lack there of. I knew something was up when the dr left to go get another dr, the barium was just not moving down his intestines like it was supposed to. So they took xrays at 2, 2:30, 3 and then 4:30. I had to go to Zach's benefit last night so my mom (nana) talked to the dr's when they came by. They said they see a possible partial obstruction on the xrays. Apparently even after that long, barium was still in his stomach (which shouldn't have been there to begin with) and in his small intestines and it should have passed all into his large intestines at the point. So they were going to talk to his surgeon.
This morning they came by and had talked to surgery and decided it would be best to place a small scope into his j tube site and see if they could see the obstruction first before performing a big surgery. So he had that done this morning at 11.
This test showed that his roux en y was possibly the issue. The GI docs think that it is too anterior and the balloon of this button is blocking his intestines forcing formula back into his stomach and pooling around the tube. He also couldn't get the scope into the stomach portion of the roux en y so that might also be a problem . The plan is to place a longer j tube in tomorrow in intervential radiology under sedation. This tube will bypass the roux en y and hopefully make a difference. If not, if he still has pain, or vomiting or leaking then the surgeon will go in and look.
He has also expecting a visit from immunology to check on his dropping wbc. They have been trending downwards this week, it is at 5. He is also showing a big dip in his hemoglobin, it is now at 8.9. And this morning his blood sugar was 41 which is really low so that is all new. It came back up but it is very strange and new issue So we will see what else this week holds. He will be here through at least monday due to the iv antibiotics.

Updates

Friday morning Zach woke up with a 103 fever, so off to the dr we went. Unfortunately there was no easy explanation for what was causing the fever, so that means blood cultures. I got to the ER at Brenner's with him around 10, he was supposed to be a direct admit but the ER gets blood cultures faster so we went there. After about 5 hours they finally moved us up to a room.
Within 8 hours the cultures came back positive, and hours later had even more bacteria growing. Right now, he has 3 different types of bacteria growing in his blood. So that is not good at all. He is on 2 really strong antibiotics and we hoping they take care of it.
This morning his fever spiked to 105 which is scary, so they tried to get an iv started to give extra fluids and his lipids so they wouldn't have to stop his TPN to run his vancomycin but his veins wouldn't thread. So not sure what they are going to do about that.
They have him on vitals every hour and hooked up to the heart and oxygen monitors. They are also checking his blood sugar and just keeping a close eye on him for now. If he worsens he gets transferred down to PICU.  Please just keep praying!!

Monday

Zach went to his GI appt today with Dr. Hill. No news about anything, there were no specific findings on the autonomic testing and no word yet on Johns Hopkins. He also put him on a new medicine, propranolol, I haven't filled it yet. I always like to do my own research first and I am glad I did. This med is usually used for blood pressure issues and circulatory problems. Yes, it can help with migraines as well which they are hoping it would help his daily vomiting  and pain. But it says don't use if you have raynauds syndrome...he has and it says a serious side effect would be very cold and blue fingers and toes...he already has so I definitely do not want to make it worse. Plus, it is a med that you cannot stop abruptly so if he did have a reaction, we would be in trouble.
So I am going to call his Ped in the morning and check with him on what I should do.

On another note, I hope everyone saw Zach's article in the Greensboro News and Record on Sunday. He was front page of the Guilford Record section. It is a very nice article and tells about our upcoming benefits for Zach.

Huge Indoor Yard Sale
Friday 4pm to 8pm and Saturday 7am to 1pm
@ Bonnie Kay Seafood
222 Spur Road  Greensboro, 27406
and
Spaghetti Dinner and Silent Auction
Monday 6pm until
@ Bonnie Kay Seafood
$7/person includes salad, spaghetti, roll and drink

Please come out and support our Baby Zach!!

Backwards again :(

We started our sunday morning out with Zach throwing up all over the kitchen, so that was not good. Unfortunatly, it didn't end there....he has thrown up every morning since Sunday and yesterday when his nurse came to check him and change his central line dressing, she weighed him and he lost 1 lb and 1 oz. Not good at all.
I don't really think it is all do the vomiting though, because he is not throwing up a lot of formula...just stomach stuff. I just don't think his body is absorbing enough of the formula to add the calories. So I talked with the GI doctor's nurse today and they are going to increase his TPN...not sure if that means more hours or higher amount. We get our TPN delivery tomorrow so I assume I will find out then.
He goes back for his follow up appt with Dr Hill, his GI on Monday so hopefully they will have some test results from the autonomic testing and some news on Kennedy Krieger/Johns Hopkins.

Taking it easy

Zach came home from the hospital on Monday. He is on half TPN and half J tube feeds,  it works out to being 24 hours of J tube feeds and 12 hours of TPN. So far, it seems to be working, he has already gained back a couple of pounds.

He went in Wednesday for some autonomic testing. We were greeted by Dr Fortunato (love him, he is so sweet) and another Dr, I think he was a nerve or neurogical dr...not quite sure. They hooked Zach up to a blood pressure cuff, this big pulse ox machine and chest monitors. He had to sit still for about 10 minutes while they monitored everything. Dr Fortunato was so good with Zach, he really didn't have to be in there with us but he sat and talked to Zach the whole time. They also drew about 55mls of blood to check some neurological markers.

This test is supposed to show if his nervous system has some issues with keeping everything on track. He said it might show something, it might not but they just wanted to try it. They might start him back on some different medicines for nerve problems.

Dr Fortunato is also supposed to talk to the Dr's at Johns Hopkins first of next week so hopefully they can get something worked out with Zach going up there.

On another note, Zach started running a low grade fever Wednesday. Yesterday it went up to 100 degrees so I took him into his Ped's office. He said his throat was kinda red, so he could have a little virus or it could be that his bone marrow is going crazy trying to make new blood cells from all the blood they took and that can cause a fever. Last night and today it is still hovering around 100.4-101 so we are just riding it out for now. He isn't acting sick but if it goes up higher, he will have to have some blood cultures drawn to check for infection. Since he has such a history of blood infections with his central lines, we have to monitor him closely and really at this point....any fever scares me. We have to keep this line in, he needs it to survive.

So we are going to stay at home, away from kids and all the sick people that go out.

another update :(

Zach is still in the hospital, hopefully he will be going on Monday. His dr wants to do some autonomic nervous system testing on Wednesday that he will have to come back for but it should be outpatient. They may give them some more clues and may lead to some more meds for him to try. We are still waiting on the word from John Hopkins as to when we go up there.
He is doing good on half TPN and half J tube feeds, he is already gaining some weight back. He is still having lots of pain and nausea but they are not trying to fix that right now. This visit was to just get the line back in for nutrition. He is on TPN over 12 hours at night and on his J tube feeds over 24 hours.

Update

Zach's nurse came Wednesday morning for his weekly weigh in and check up visit. He weighed in at 24.2 lbs which is a little over a pound loss since last thursday when she weighed him. So she called and reported his weight to his Ped dr. and I called and left a message that we needed to talk. Over the past week, Zach had just gotten so blah...he just laid around all day, had no energy, was sick on his stomach, extremely pale and cold. So his Ped and I talked and he decided to let his GI doc know the current weight and they could discuss it. His GI doc decided it was time to admit him and have another central line placed for extra calories.
We were admitted around 5:30 pm Wednesday night at Brenner's. The plan was to place a line today on thursday. He had surgery today around 12 noon and it was fast, only took about an hour to get the new line in. Luckily, it went into one of the old spots and they didn't have to place it in his neck.
He is going to have half of his calories through TPN and half through his J tube feeds. The GI doctor is also working on getting him into Kennedy Krieger, which is a division of Johns Hopkins Hospital in Baltimore. We just have to wait til they get his appt scheduled. At least the TPN will keep his weight under control while we wait on his appt.
He should be able to go home hopefully by monday. We have lots to get ready before his benefit at the end of the month. We are hoping to raise enough money to last us while we have to stay in Baltimore.
Please keep praying for Zach, and please pray for Brent, a 5 yr old at UNC childrens that is very sick right now. He has mito and an acute HLH attack and needs lots of prayers right now so he can get better.

Hurry up and wait.

We had a follow up yesterday with Dr Hill, which is Zach's primary GI dr. It has been a while since he has seen him, we have been seeing the other dr's on call and such. First off, he knew very little about all the changes that have taken place with his meds and symptoms. So, that was the start...getting all the meds straight in the computer, yes it has been confusing, he has been on several different meds over the past month with little success so I understand. Second, he plainly told us he was stumped as well. Zach's symptoms/body doesn't follow any textbook and it is very confusing. All the tests that he has and blood work ups that he has are all normal so nothing makes since. Yet, his symptoms are real and they have seen them which again is confusing. He said that going to UNC childrens will probably not be the best choice, he doesn't think they can help us at this point. He thinks we need a more extensive hospital. He is thinking about sending him to either Cincinnati Childrens Hospital (yes, we have been there before but it was for a specific EE program and not as a general GI patient so we didn't see all the dr's) or sending us to NIH because they take on a lot of bizarre cases. He is also going to talk to his team this week about taking apart his roux en y surgery. The problem with that is how to feed him when his J tube is gone. His stomach cannot tolerate very  much....actually not more than an ounce at a time and sometimes not even that much so we can't use his stomach for nutrition.
The reasoning behind taking apart his surgery is that sometimes, yes rarely, you can have a complication with surgery that actually makes you worse (all he bile backup and worsened pain) so if that is the case we have to find a surgeon that would do it. His surgeon doesn't think that is the problem but who would admit that anyway. Not that it is his fault if that is the problem, there is no way to predict how your body is going to act to something like that. But again, how do we provide nutrition if it is gone?
And he also took another thyroid panel and immune test and if those come back weird then he will try to move up his immunologist appt. (right now it is scheduled for sept 23) He should let us know within the next couple of days what the plan is. We have to find out fast because he has already lost 4 lbs since the end of June.