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


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.

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 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.

Saturday, October 08, 2011

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.

Friday, October 07, 2011

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 long as he doesn't grow anything on his cultures!! So praying, praying, praying and more praying that nothing grows.

Tuesday, October 04, 2011


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.

Sunday, October 02, 2011

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!

Saturday, October 01, 2011

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.