Medical Update, where do I even start. . .

I guess I can start with the EMG (Electromyography) he had in November,

which was a big fat FAIL.

EMG appt.

See that one and only blip on the screen, that was the only little shock Jack got this day because he started to scream.  I really didn't have much hope that this would be completed anyway since Jack is so hypersensitive to pain and touch. Plus he had this test done back when he was 4 yrs old and it was very uncomfortable back then for him.  But Dr. Richardson thought he might be able to tolerate it OK...WRONG. But it was worth a try.

Dr. Richardson is another Neuro that works with his Mito Dr. at Seattle Children's. I really like him and he was great with Jack. He felt he had some symptoms of a neuropathy to warrant the testing. Jack continues to have a "pins and needles" sensation in his feet. An EMG is the best test to determine if he has a nerve problem. We discussed him having a sedated EMG at a later date but then we decided to see how his Pain Clinic appt. would go first and get their opinion on his foot pain and other pain. I certainly didn't want to rush into another sedation.

In the mean time, we are still having many days at school like this

and this...

and this.

I seriously could make a 2012, 12 month calendar of Jack sleeping in the nurses clinic! Its quite a pathetic room, looks more like a prison cell than a nurses clinic. But remind you, the school was built in the 20's, its suppose to have that old school charm to it--or just old school ;p 

Ill often get a call saying Jack has been sleeping in the clinic for an hour, "do you want to come get him?" He is either too fatigued to continue to sit at his desk or he comes in with tummy pain and ends up falling to sleep.

Despite adding a modified school schedule to his IEP, starting at 11am instead of 9:10am, he still has days like this. So we decided to cut back the tube feed he gets at lunch and have him drink his chocolate milk throughout the school day rather than the same time as his feeding, getting awfully full and uncomfortable. Jack fights us though about it, he doesn't like to sit at his desk and drink in front of his classmates for some reason--in fear of looking different I'm guessing. But we're working on it.

Speaking of his IEP, the PT said his hand strength has declined since she tested him in June. Jack is now using an ALPHASMART so he doesn't have to do much writing in class now. Its really helped and he can even print out his little stories and give it to the teacher. He also can carry it to other parts of the school too, its been a big help to keep him up to speed with his classmates. The IEP went OK, I wont get into it, same ol' stuff and all too boring.

NEUROPYSCH testing

Jack had his Neuropsych testing done at Seattle Children's in November.

That makes for one tired boy.

But to my surprise he was quite alert that day (thank goodness!) and performed really well for them. It was a long day, 3 hours of testing with breaks.

I also had to sit with a psychologist while he was in the other room being tested. For some reason I was nervous, feeling like I was going in for a job interview. But she was nice and basically asked me a million questions, along with the many pages I had to fill out beforehand. You know questions like.... Sets Fires, smokes or chews tobacco? Not true, Somewhat true OR Very true? Eh, these Neuro psych questions were quite silly for a young child :p

All in all, there should of been a long couch for me to lay on to tell her my life story, I nearly lost it a couple times talking about Jack and what just might lie ahead for him in his future. She seem to understand Mito well as she sees many of Dr. S's patients there. So it was nice to be talking to someone that "got it"

We then came in another day of testing the following week for about 2 hours. The Psychologist then went over some of the results with me from the first week of testing.

Neuropsych Test Results

They tell me Jacks Full Scale IQ score was within the Low Average range (FSIQ =89)  His verbal comprehension index was within the Low Average range (VCI=87)  His Perceptual Reasoning Index was within the Average range (PRI-100) His Working Memory Index was within the Low Average range (WMI=86) and his Processing Speed Index was within the Average Index (PSI=94)

"Hand motor Output performances identified intact unilateral hand motor coordination, but very weak grip strength, slow finger agility, and poor bilateral hand motor coordination. Jack was not able to easily and effectively regulate his bilateral hand movements in response to an external model."

"Visual-spatial performances test suggested constructional DYSPRAXIA (or deficits in constructing figures on paper consistent with cerebral impairment) and school testing with a measure of visual-motor integration last September indicated below average visual-motor integration"

"Language performances did not indicate impairment. Verbal intelligence fell within the Low Average range as indicated above."

"Conceptual reasoning fell within acceptable limits for age"

"Generative Fluency for verbal and non verbal material fell within average limits to above average limits. Jack was able to establish and maintain set when integrating and implementing a novel set of task directions to perform complex tasks"

"Planning fell above average."

"Cognitive flexibility was quite good, Jack was quick and alert."

"Memory performances were high average, except that recall and reproduction or abstract visual-spatial designs was low average, associated with the constructional dyspraxia."

In summary:

"Neuropsychology testing identified primarily intact abilities, consistent with intelligence, but identified constructional dyspraxia and low motor coordination on visual-motor task and low bilateral hand motor coordination. Jack did not demonstrate significant deficits, did not present with a profile of a clear acute decline, and did not show evidence of significant executive function of generative fluency deficits."

"Psychosocial adjustments is significantly compromised by multiple disease complications, including loss of bowel and bladder control, abnormal sleep patterns and marked fatigue during the day, and significant issue as Jack mostly sleeps at home during the day and has missed a significant amount of school."

Theres much more with recommendations but that's more than you want to read!

So I would say I'm pretty OK with what the results were, the important thing is there NO real deficits or decline at this time. Yes, he is in the LOW average range for many things but overall a good report I felt. This gives us a good baseline at where he's at at age 8. At least we have something to compare it to now as his gets older.

Oral Surgeon Appt.

Jack had been on a waiting list since summer to be seen at the Children's Oral surgery/Dentist clinic in Seattle. Apparently they only have a few Oral Surgeons and they only come to the Clinic 5 times a month! And with the Holidays, even less. So our original appt was suppose to be in October, but the day before they call and tell me there was a mistake in scheduling and said they had to cancel--OH NO you're not :( I was very disappointed since we had been waiting for 3 months already. So the quickest they could get him is early December, crap. I did not want to wait this long since his teeth are already crowding and they need to be pulled, 6 in all!

So here comes December and the morning of, they call me and tell me there was an emergency at the hospital and they had to cancel all her appts for the day. I seriously was going to cry, so I made a big stink on the phone, which isn't like me at all but seriously? they already canceled on me once! So she said to come in and they would see what we could do. I was able to get in after some waiting, whew. Glad I let my Ma-Ma BEAR out that morning!

The appt went well and Jack cooperated, whew. The Oral Surgeon was very nice and didn't seem stressed at all even though he had twice as many patients that day to cover the other Dr. He said Jack will need 6 teeth pulled unless more fall out on their own in the mean time. So we are still waiting for insurance approval. This will be done at Children's Hospital under sedation. Maybe in January.

GI appointment

Jacks December GI appt. started much like this, had a tough time getting him dressed and in the car. He basically slept through the whole appt. except for when we forced him up out of his stroller to be weighed.

We talked a lot about his continuing belly pain and his GI decided to have him get that MRI of his belly (MRE) to see if there was any inflammation growing in his small bowels again, causing the continued pain. I told her I didn't want this one to be sedated despite him having a tough time in the machine awake. His last anesthesia MRI of his brain didn't go so well! I thought he could probably tolerate 20 minutes in there... I had hoped anyway.

We also talked about a GJ tube (adding the J would be another tube that would bypass the stomach altogether and would directly go into the jejunum, the 2nd part of the small intestines) but certainly we don't want to complicate him more and we should try continuous feeds with just the G first anyway to see if that helps with the belly pain after he's fed. I still haven't done it, I know this would be life changing for him, having him hooked up and have to wear his feeding pack all day and night. He would hate it, hates the feeling of being fed and has trouble carrying anything on his back with his weak hips...I'm still trying to work myself up to this conversation with him :(

We got him the MRE appt right away--we had to. His GI felt we needed to get these results in before he had his Pain Clinic Appt on the 22nd. We had to be sure what we were dealing with wasn't the inflammation, at least then they would know why he had stomach pain.

Abdomen MRI (MRE)

This whole appt. didn't go as I thought it would. We were told that Jack had to fast, so he couldn't have anything by mouth or tube after 8:30am. We were suppose to check in at 2:00pm, and start with the Barium (liquid that shows up on xray) at 2:30pm...As you can imagine, tell a kid they cant eat or drink and they all of a sudden are VERY thirsty!

He was getting very loopy by 3:00pm, still haven't been given the Barium! So then they tell me that they probably shouldn't of had me come in that early--WHAT?, nice. My poor boy. So lets get this Barium in him so maybe he wont be so hungry people!

Well then the tech brings out this huge bottle and says its all got to be in him in the next 40 mins, oh boy this wont be pretty. I had an extension but he said they didn't have a syringe to fit it...wow, they have to right? this is a children's hospital? He seemed very confused as to what to look for, I had a syringe but not the right one--anyway we figured it all out eventually...

So I slowly pushed it in him as he played Mario Cart :) thank goodness the game was open so he could keep his mind off it. He did feel a little sick through the whole thing but I got it all in in time.

So just as we were being led back to the MRI machine, she tells me, "oh Ill be doing his IV line" WHAT? an  IV? (for the dye) So why didn't anyone tell me this? She says the Radiologist always decides at the time of the appt if the child needs it....SO uh-oh, Jack immediately freaked when he heard her say that! started to cry and struggle in his chair. It took a long time, over 30 mins to plead with him to cooperate.

The nurses were starting to threaten him, saying they were going to have to have someone hold him down on a table. He finally agreed to sit in my lap, he even had some choice words for us all, it was not pretty. I told them he's not normally like this but heck the kid hadn't eaten for 8 hours by now! Give this poor boy a break!

I don't have any pics of the MRE, they tell me my cameras SD cards/cell phone data can be erased if I get too close to the machine so decided not to ask this time to do it from outside the room--besides by then Jack was pretty upset by then and he needed me to be his mom, not a photographer! of course my boy comes first :)

Jack had a tough time in there, it was about 30 mins long but for this one he needed to hold his breath for up to 20 secs several times. That's not easy for a little kid. Plus he felt like vomiting and we need to pull him out, thankfully he agreed to go back in for the rest of the testing, I felt so sorry for him. He was so BRAVE.

Well needless to say, we didn't get out of there until 7pm. Wow, we pulled into the parking lot at 1:30ish, it was a long day for both of us. The sucky thing was that we were suppose to get those results and page his GI Dr before she went out of town for the Holidays, well she was long gone and the office was closed of course.

But the next day we did get results: NORMAL

I was happy about that, but we also still don't know why he has the pain...and I put him through yet another test that didn't help him one bit. But we had to know for sure to move on.

Pain Clinic

So our long awaited Pain Clinic appt. arrives, finally after waiting a couple months to just to be put on the wait list and then another 3 months for our appt. Our Pain Appt is here and we can finally get some relief from Jacks leg pain, stomach and ect... and you guessed it, he's having a sleepy day again. We arrive at our 9am appt. to meet with a Psychologist first. Jack slept through most of the appt. So it ended up mainly just me asking the questions about his foot/leg pain, when its happens most, how many days a week, how does it affect his daily life, school ect...

Then our next appt was at 11:00am with 2 Pain Drs. After talking with me and asking me many questions, they finally had to wake him up to watch him walk a little bit and ask him a few questions about his pain which he mostly just grunted. He was clearly still out of it and didn't make a lot of sense.

Next we were sent for our 3rd appt of the day, to see the Physical Therapist

for a full eval. She measured and touched every part of his legs and feet.

She also had him go up and down stairs, timed him walking and running. Checked his gait and had him do a few exercises. She told me he has extremely weak hips and felt this might be the cause of his leg pain. She noted how he crosses his foot in front of the other as he walks, another sign of weak hips. She also noted his tight hamstrings. By then Jack was wide awake and performed quite well.

After all 3 appts. we had a lunch break. All the Dr's were then to go over the results and after lunch we were to meet again and they were to let me know their recommendations on how to treat his pain.

When we got back to the room to go over everything with one of the pain Docs,

Jack wrote this on the Dry Erase board for the Drs...how sweet is that? :)

Pain Clinic Recommendations...

Well now for the shocker...NONE AT THIS TIME.

Honestly I was so close to bawling my eyes out, she could see my disappointment. I couldn't figure out how they could come to this, nothing, nothing is all they can offer him?

"Well why do you say nothing Mrs. Pierce? We are offering you Physical therapy" Physical Therapy? You mean the Physical Therapy that we've had to discontinue years ago because we could no longer afford it? The therapy that will take months or who knows how long to strengthen his hips--as to relieve his pain, if that works or is the cause on his pain anyway?

I asked her about the pins and needles sensation he's having and she told me that because we didn't have enough of a pattern to it that she couldn't warrant recommending anything for the pain. That there just wasn't any good reason to if it wasn't happening like the "typical pattern" they see with neuropathy. So maybe we should of lied and said it was always in the evening? Jack had a tough time explaining his pain. He told them it didn't seem to matter what time a day it was, that it would come and go all day. I guess that wasn't the right answer. So frustrating.



I understand that they are really reluctant to prescribe pain meds to kiddos without real concrete answers to their pain, especially with a child that is often very sleepy anyway, and as she put it "a bit complicated" as there is too many variables and other issues he's dealing with that could be the cause to his pain. Like GI motility, weak hips, feeds and his sleeping pattern.

I could see she was getting annoyed with me as I shot down her PT advice. I'm sorry but unless you've been on this road, don't judge me. I am NOT a negative person, I'm willing to try anything to help my son.

I see now over the years Ive been passive with his care and don't raise enough hell when maybe I should have. I'm normally a shy person and have a tough time getting my voice heard...but this time I couldn't help myself to show my disappointment and emotions. She honestly thought she was doing her best with us but its like she never heard me or him.

This was the most disappointing appt. I guess I had hope that this was the appt that was finally going to help get relief from his ongoing pain. I'm not saying they didn't do their job or they clearly don't understand Mitochondrial Disease, as they see Dr. S's patients...but I was still crushed with the decision which I feel was mostly swayed by the PT.

To have to look at my son and tell him we did all these awful tests...2 long MRI's with horrible sedating affects, the MRI/MRE that made him want to vomit, the painful Urodynamics testing that made him scream and beg, the EMG that he could not tolerate and more...and tell him, I'm sorry but no one can help you. There isnt any Dr. that can make you feel better...is heartbreaking. I left with a paper that read 4 times, NO recommendations at this time.

Honestly, I'm tired...and was feeling a bit hopeless as I wheeled my boy to our car.

As soon as I got settled in the car, I got a call from the Sleep Center asking to see Jack. The Pain Dr must of felt bad enough to refer him right away somewhere. I'm not sure why we're seeing the sleep center but maybe for meds to help him get down earlier as he is often is too hyper to get to sleep at a normal time. This might help us get him in a better sleeping pattern. Melatonin has never helped much before at home. So we have that appt

late Jan.

So whats next you ask.

Some good news is that I found out that Jack will get some financial help with the Physical Therapy if it is billed through Children's. Seattle Children's doesn't offer outpatient therapy unless its to a child recovering from any injury or surgery ect... But there is a Children's PT office just minutes south of us, in the same office as his GI Dr.

His appt is tomorrow (praying he's up to it) to go over any exercise routines we can try at home and I'm hopeful we can possibly get weekly therapy started there too. I still need to ask my insurance what they will cover first...so its still up in the air whether we can do this.  We'll see.

So whats been going on with Jack this week?

On my news years post, I already mentioned him peeing blood last week while we were up at my parents, he awoke crying and lots of blood and clots in the toilet. It kinda scared me, assuming this was only a bad UTI though. He also complained of left side pain. I took him in right away and had a urine sample done. They told me that they did find blood and clumps of white blood cells, and his urine was "cloudy" with lots of debris in it. So we sent it out to see if it would grow something to be sure this was a UTI. I asked him about a possible Kidney stone but he felt he would be in more pain.

Two days later and still nothing had grown. Jack was put on antibiotics anyway due to what they saw in the urine. The PED (which I really like and isn't our normal Dr., darn) said he had talked to his Mito Dr (Dr. S) and Dr. S. got a Nephrologist involved because they were concerned that nothing had grown and he was having that left flank pain.

The Nephrologist pulled up his Kidney U/S done in September and noted that they were ABNORMAL--HUH? no one told me this! That they showed dilation on the left kidney and lots of debris in the Ureter. Now whether this is alarming or not I don't know but Jack is still having that lower left side pain.

So Dr. S. said it was urgent that we get him to have some labs and another urine taken to check his Kidney function. Poor kid wasn't too happy about another stick :( But you can see a cherry Pepsi helped make it all better afterwards :)

The PED called me Friday night and said that the kidney function looked OK :) but they were still waiting on a couple more and the Metabolic panel which wont come back until next week (tomorrow probably now) After all this was a Friday and a Holiday weekend, isn't that the way it always is? Jack likes to get sick on weekends when no Drs are around.

Pam, Dr/ S's nurse also emailed me to let me know if Jack developed a fever, had more bleeding or more pain that I was to take him to the ER immediately.

His fever has really only reached a high of 100.8, but it has gone up and down, staying at 99.6 tonight now. It really hasn't done anything and I don't see any blood visibly anymore, just little brown flecks and its still cloudy with a lot of stuff in there but he feels ok besides the left side pain, diarrhea and his poor little feet have been in more pain this weekend, but I'm sure that's not related at all.  I'm hoping that the tests coming back tomorrow will tell us more of whats going on. The PED thinks they'll want to repeat his Kidney Ultrasound again to be sure nothing has worsened.

So that's where we're at.

Until I get a call from a Dr. on what to do next, I'm watching our dear boy closely, and praying whatever this is the antibiotics are currently kicking its butt!

Thanks for getting this far, if you even got this far! I should never wait so long to update because its just too overwhelming to read!...so I'm sorry for that. This was probably more for me to get it all down and out of the way ;p

In the mean time, nothing can compare to

what other littleones are going through

as I type this tonight.

KEEP PRAYING for all our mito kiddos...

but put in an extra one for these

amazing little boys...

BRENT, KYLE & NOAH

They're all struggling for their life tonight.

They are WARRIORS in every sense of the word.

Please pray for strength, peace and healing for their families.