"I wish I had a new body"

What do you say to your child when he tells you that?

Sigh, its been a rough few weeks.

I'm SO behind on Jacks medical updates. Alot has been going on with our boy lately. Many Dr Appts, 2 new specialists, more testing and another IEP meeting at school... which I never seem to get through without a few tears. 

So here it goes....

September 19th Jack had the dreaded URODYNAMICS Study testing to see how his bladder is functioning. It was like everyone warned me about, bad, bad, bad.

Jeff was totally against it so we had some convincing talking to do before we could come to the decision to go ahead with it. I knew it would be an invasive and quite an uncomfortable test for our sensory boy. BUT in the end I couldn't see us moving forward without knowing more information on why he's lost some bladder and bowel function, which all started last October. Jeff did finally come around and felt better knowing we could stop the testing if he wasn't tolerating any of it...

Well, that sounded good anyway! It didn't quite go that way though. I should of known it would of been pretty difficult to give up once he was laying on the table and maybe so close to possible answers, even if he was screaming. You notice I have NO pictures for that day.

It was awful and the worse thing Ive ever had to witness him go through. Placing the catheter was the worst part, and the one in the other end wasn't any easier. They also used 3m Microfoam tape that he ended up having a severe reaction to. So when he was screaming in pain as we were trying to remove it, we couldn't understand why he was so upset. But we quickly found out when we saw his little bum when he got up. Both cheeks were bloody red that begun to turn into what looked like bruising. It was awful, they kept him for awhile to be sure he was OK, he had a lot of trouble sitting, poor kid. We made sure that was written in his records, never that tape again! Just praying we never have to repeat this test at all again.

Urodynamics RESULTS

abnormal.

He had what they called spasms where he shouldn't of. He also held a lot of urine for his little size, meaning he isn't likely feeling it much when he's getting full.

But to our surprise the Urologist said that he felt Jack has something called a TETHERED CORD.

"Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. Attachments may occur congenitally at the base of the spinal cord (conus medullaris) or they may develop near the site of an injury to the spinal cord. These attachments cause an abnormal stretching of the spinal cord. The course of the disorder is progressive."

Well that was a surprise.

Why would this Dr. think that?  he says because of Jacks clinical symptoms. For one, after he looked him over, he told me he had one leg longer than the other, a red flag for the tethered cord. I'm thinking this must be new because he was gone over very carefully with a thorough exam from a PT, at Children's 2 years ago. She measured all his limbs, feet, legs ect... and he was then examined by the Physiatrist afterwards because his foot was starting to turn in. I'm sure this would of been noted then.

Here's a few more:

Bladder AND Bowel incontinence (after over 2 years of being potty trained, now back in pull ups) increased pain in legs pins and needles sensation in feet, legs. His foot turning in more, he's been dealing with that for 2 years. (they told me it was a hip thing) and muscle weakness

BUT of course all these could be Mito issues too, the only 2 things that stand out more is the longer limb and foot turning in. He is still "twitching" which showed up on the overnight VEEG he had in June, but he didn't say if it had anything to do with this.

He then told me that many times the tethering doesn't show up on the MRI and if the child has enough of the symptoms then they sometimes go ahead with the surgery anyway. Of course I'm thinking, without evidence?  that's scary. There is always a risk with spinal surgery of any kind. It can be a 4-5hr surgery and lots of recovery time ect... The sad thing is he told me that not to expect the bladder/bowel stuff to improve after the surgery because that part normally doesn't change :( but the leg pain ect...can improve. AND of course preventing further damage.

He ordered an MRI of the spine, referred Jack to see a Neurosurgeon... and then I drove home in a daze.

On September 29th Jack had his MRI, unsedated.

He was scared. He's had a bit of a cough for a week now and I was desperate to get it calmed down that morning. Even if it meant pushing cough medicine in his GTUBE, as I'm pretty sure it doesn't work that way...but like I said, desperate! Just prayed he wouldn't have a cough attack and then have them tell me we would have to reschedule.

When he laid down the the nurse looked him in the face and said "we only allow you to blink and breathe" It was then I saw tiny tears flow from his eyes, and down his cheeks... I nearly lost it but pulled it together so he could remain strong without worrying about his emotional mother!

He was a champ and only got scared a couple times with all the loud pounding noises. I keep thinking they will someday figure out how to make these things quiet! seriously.

Jack needed his routine bloodwork, (every other month) This time for his GI who wanted to check his liver function (its been elevated) and to look for inflammation markers.

So we hung out by his favorite fish tank and his 2 favorite sea creatures. This hermit crab is HUGE and always fun to watch. Jacks favorite color is blue, so no surprise that he loves this little blue fish that always hides from us.

As always, Jack was thrilled to see HECTOR working that day :) The guy is amazing with those needles! Making our trips to Children's for labs so much easier (on the both of us!) He was definitely having a droopy day (I always can see it in his eyes) weak, weak, weak. But we were both exhausted.

LAB RESULTS

Liver looked much better!

BUT his SED rate and CRP were both elevated, indicating inflammation.

School IEP

So he falls asleep in the car and I still needed to get to that IEP meeting that just so happened to be scheduled on the same day as out MRI. We checked in at Children's at 11:30am but they didn't take him back until 1:05pm, then with terrible traffic through town, there was NO way I was going to be able to drop Jack off on the couch and then run to the school by 3pm....

I was naturally late.

out of breath, I ran in still with my Children's parent lanyard around my neck. There in a quiet room sat 6 woman at a table... waiting for me. Despite letting them know I was on my way, I felt awful.

The Principal, PT/OT, his Teacher, Formal Teacher, Special ED Teacher, Psychologist and Nurse.

The IEP meeting went well, besides me tearing up when I heard the phrase "as his disease progresses" I thought I was doing OK before that, but I probably would of lost it anyway. I think with the last few appts and the recent concern from his Drs, I was pretty fragile sitting there, and about ready to crack.

He is doing OK. Not great and in the low range of average in some areas and below average in others. His PT/OT said that his hand strength had declined quite a bit since she had tested him in June. Ive noticed it too but didn't think it would be that noticeable. Balance is still an issue, talked about his falls at school ect...

His handwriting is still very poor and he fatigues so quickly that she couldn't even finish some testing. She says after 3 mins of writing or drawing through mazes, he cant continue. This is why homework is such a challenge for him these days. So we talked about having him possibly use a keyboard at his desk. But first they need to see if using a keyboard is just as tiring! So more testing. AND also using a software like "Dragon", a dictation software.  

I just knew 3rd grade would be the year we would see a big change in his education, more accommodations will be needed as he falls more behind.

Since he hasn't made it to school much this year yet due to so many absences we also talked about having a modified school schedule. It was decided that he would start at 11am and stay until 3:40pm. They first suggested 3pm but it just doesn't make any sense to fight traffic to pick him up and then have to turn around and pick up Logan at 3:40pm. So we'll try this first. I'm not sure it will make any difference as he's never "all better" by 11am on those bad days. PLUS its not like Jack can sleep in, he still will need to get up and get in the car to take Logan to school at 9am... so not perfect. But we'll try.

Spine MRI RESULTS

On Oct. 4th, Jack had his appt. with the Neurosurgeon. Not sure I was thrilled with her, not exactly the warm fuzzy type, very get to the point and black and white.

{hey skinny boy!}

normal.

Jack was asleep throughout the visit, she tried to wake him to look him over, well he tried! But his Blood pressure was very low again (70/44) so I wasn't too surprised he was out cold. I just hate when he's like that. I got prepared to get the "does he walk or talk?" question.

I was pleased to hear it was normal but after what the Urologists told me---kids with Normal MRI's still having a tether and other Mito moms saying their childs MRI was also normal, but they went in and found tethering... it got me very worried.

I was expecting her to talk about the chances of it still being tethered, despite the normal MRI... but she didn't. She actually made me feel crazy for asking her about what the Urologist told me. Saying she didn't know why he wrote it in his notes and told me this. She just wouldn't say if she's seen or heard of kiddos with normal MRI's but later finding tethering. She just kept saying, "Hey, I love surgery but I don't know, because I don't operate on kids without any problem"  She told me twice I was welcome to get a second opinion.

Honestly, she made me feel like I wanted the MRI to be abnormal and surgery! I just wanted to be sure we were not missing anything. Obviously she went to Med school and has to know this happens occasionally. She finally said, "well, its really controversial".

So I don't know what to think. This is something Ill be definitely going over with Dr. S. later this month. 

The appt. didnt last more than 15 mins, I kept thinking, then why am I here meeting with you if theres nothing to discuss. Why didn't she just call me with the results if its so black and white.

BUT  before we left, She tells me that it would still be a good idea to have an upper spinal MRI... AH WHAT??!! You mean they didn't scan the entire spine??! (it looks like it above but this isn't) I guess it was stupid of me to assume that! "NO, they only ordered a lumber spine MRI" You would think they could of just scanned a little farther up his body, considering they weren't sure he had a tethered cord in the first place...ugh.

She shook my hand and said, "I'm sorry, but theres nothing I can do to help your son"  so that was that.

NEUROLOGY appt.

Oct. 5th 

Today Jack saw another Neuro, specializing in Neuropathy. Dr. S. referred us to him after I emailed his nurse about Jacks increasing leg pain/pins and needles sensation in his feet.

I LOVED THIS GUY!

Dr. R was really warm and easy to talk to. Don't you just always know if you're going to like a Dr. in the first couple minutes? He was great.

It was a productive appt., despite Jack looking like this in the waiting room! Another low BP today. Thankfully he woke up and was able to be examined and answer questions for him.

Dr. R. went over his whole body, testing his strength in his arms and legs. He quickly mentioned his droopy eyelids, asking me if this is normal for him. It really comes and goes, worse on his weaker days.

He saw some good stuff, Jack has pretty good leg/foot strength, his right is weaker than his left which surprised me. The right toe was very weak though, which could be a sign of Neuropathy starting... or NOT.

His reflexes are there, but dim. In fact he appears to have hyper reflexes now, which is even more surprising. He's always had NO reflexes in the knees but now that's changed.

He says he is definitely HYPERSENSITIVE to touch and pain. Even simple touching of his legs, feet and arms made him whimper. That also could be a sign of Neuropathy... or NOT.

WHATS THE PLAN

Dr. R. is going to review the EMG he had when he was 4 yrs old and go over what was seen then. He feels the symptoms he's having now does warrant another  

EMG/NERVE STUDY... boo.

"To find damage to the peripheral nervous system, which includes all the nerves that lead away from the brain and spinal cord and the smaller nerves that branch out from those nerves."

The EMG for Jack was quite traumatic for him to endure--probably because of his hypersensitivity, that he appears to of always had, even when he was a baby. The good news is he said he doesn't feel we need to do the muscle part, which includes needles. So that's a relief! 

We are still waiting to be seen at the PAIN CLINIC, but the wait is long, probably 3 months he says.

ALSO

At the end of our appt, he was actually able to talk to DR. S (mito Dr) and they both agreed Jack needs another Brain MRI with contrast. His last one was in 2009. His MRI's have been normal so far, but his MRS this last time wasn't.

It showed LACTATE PEAKS, Dr. S. said this just confirms Jacks Mito diagnoses. Lactate levels in the brain are normally very low or absent. This isn't an uncommon finding with Mito.

So since he needs another spinal MRI, we're going to schedule these together. This will be a long scan though :( I so wish this next time he could have some light sedation. An hour or so without moving might be a bit much for him to do without it. More to discuss.

GI UPDATE

So Jack still has a lot of tummy pain. Since his recent labs showed inflammation, his GI suggested we might want to order an MRI of his abdomen... that's right, ANOTHER MRI! But we talked today and she suggested we should do another KUB (xray) of his belly and see if he is constipated... grrrr..... OK, I know this kid is NOT constipated! But whatever, do another.

KUB RESULTS

abnormal: GAS!

BUT I wasn't surprised, this is what his belly scans always look like! NO POOP, just air. All the dark area is trapped gas. So what can cause gas to build in the bowels? The first thing they like to rule out is a HIGH FRUCTOSE Intolerance and or a LACTOSE Intolerance. Jack has tested negative for both, twice! 

We've used the lactaid and even removed his beloved coke and chocolate milk he loves so, but it hasn't made any difference in his pain. Dr. L feels this is likely due to his slow motility, he's just not able to move this air out on his own. He is not a gassy kid at all.

So the plan is to eliminate his blended food (which is only baby food) this means his school feeds and replace it with the ELECARE formula for all his feeds. Also start him on a med that he will take 4 X's a day, in hopes that it will help keep the gas under control. I'm praying this will make him feel much better. We will trial this for 2 weeks and see what happens. As far as the inflammation seen in his labs, we'll keep monitoring it.

WHAT NEXT?

Jack see's  Dr. S. for his 6 month Mito follow up this month and also see's the Children's Ped Dentist to talk about the 6 extractions he needs! Poor kid, he wont like that one. He will be sedated and it will be done at the hospital. His baby teeth aren't coming out on their own and causing extreme crowding. We need to make room for those big permanent teeth coming in. So basically he will only have 2 front teeth for awhile, can you imagine? I hope the kids at school will be kind :(

WHEW... Are you still with me?

I'm feeling bad that I haven't been spending more time here and checking up on our little Mito friends. I think I do more of that on FB these days, just easier for us Moms to update/comment there. Just know I'm thinking about you all, always continued prayers

for your littleones.



Thanks for stopping by and checking up on us. 

You can see why we call him our little HERO, I'm so proud of our boy!