EPI 743- TRIP 1 TO AKRON (October 25th-November 3rd)

This is long overdue, but here is the post about our first trip to Akron for EPI 743 drug trial.

First a quick background on the EPI med

There is still no cure for Mitochondrial diseases, but there is a drug trial for a med call EPI 743 (will be different commercial name when it comes out), it has shown to improve life of kids on it, and in a few instances even reverse some of the symptoms (however that was a very small study so those results should be taken with a grain of salt).

We have been trying to get Jagger in the trial since early March, we had it all set to go in June in Akron, OH (one of the very few sites in the country doing the trial and the closest to us) but then the Pharmaceutical company changed a few things in the protocol and it has to be approved by the hospital and the Institutional Review Board (IRB) and that took forever

In the meantime, the trial was moving from Phase 2A to 2B which required a known gene mutation and has a placebo element to it, meaning that half the participant will actually get a placebo for the first 6 months and not the drug itself.

So we could not get into Phase 2B since we do not have a gene mutation at this time, and we were not going to take our medically fragile/immune compromise child across the country to possibly get placebo.

So after months of waiting and sending medical records (almost 2000 pages), test results etc., we were finally admitted as the last patient in Phase 2A!!

 The trial consist of 3 visits to Akron Children’s hospital in the first 13 weeks, then a follow up every 3 months (which we can hopefully do in Atlanta, at least some of it) 

So we embarked on our big adventure.

For this first trip, we decided to drive, it gave us more flexibility and since and we were not 100% sure how long we needed to be there for the initial visit.

I am sure a lot of you drove long distance with kids, but with a special need child, it is a whole different ballgame! Annett was amazing at packing the car and despite being very very full with all the equipment we need for Jagger on a daily basis I was still able to see some of the road in my review mirror.

On October 25^th early afternoon we took off for Akron. The plan was to drive 6-7 hours and spend the night in Lexington, KY.

Jagger did very well in his car seat for the first 4 hour or so (and we found out during the trip that was his absolute limit), and we made it without much trouble (even if the last hour was very difficult for Jagger) to Lexington.

After a fairly agitated night (pain crisis that required morphine) we took off for Akron. The remaining 6 hours turned into almost 9 hours of absolute nightmare.

We got stuck in major traffic in Cincinnati first then in Columbus. It started to rain about 1 hour after we left Lexington on Friday morning and rained nonstop until Wednesday afternoon, thanks to Hurricane Sandy. Jagger was super agitated and we were only able to keep him in his car seat for 2 hours or so. About 1.5 hours away from Akron, Mister Jagger decided to poop, so I had to find somewhere to stop to change him, but we were in the middle of nowhere.

So I pull in this old country gas station and we started to change him by the gas pump (only spot that was covered). And here come the TMI, but it was the most explosive diarrhea he ever had. It took an entire packet of 75 wipes and a beach towel to get him clean, it was all over his back, up to his neck and of course he was screaming and moving the entire time and it’s really not easy to change a kid in the back seat of a car! And the poor kid was probably cold, as it was no more than 45 degrees outside!

But after all those emotions, we finally made it to our rental house by around 9 pm. Since we had to stay several days, we decided to rent a house, so we could use the washer and dryer and it was actually cheaper than a hotel.

We did not do much on Saturday and Sunday, and just relaxed, some grocery shopping and I drive to the hospital to make sure I knew where it was for the Monday appointment.

My good friends Jeremy and Cindy that live in the area came and had lunch with us on Sunday; it was so good seeing them. We all went to University of Indianapolis together back in the days and we really don’t get the chance to see each other very often anymore so it was a great visit.

I did get a call from Dr. C on Saturday night, so that is usually not a good sign, but he just wanted to move our 11 am appointment to 7 am as he had to try to get out of town before the hurricane hit full force.

So very early Monday morning October 29th (Jagger usually doesn’t fall asleep until 1 or 2 am and sleep until 10 am so this was tough on him) we drive to the hospital.

We met with Dr. C and his nurse Hilary, we had to answer a ton of question and fill many forms to get started on the trial.

Dr. C did his evaluation of Jagger and they try to take blood. However that was not successful so we were sent to the lab on the other side of the hospital.

The lady in the lab was really good, but the orders were not put in the right way so after she was done and had Jagger blood, we realized we needed more blood, so we had to get him poke again:(

We then had to do a baseline EKG, and we were able to do it earlier than our appointment so we were back to the house by lunch time.

Jagger and Annett were very tired so they took a nap and I went to the Pro Football Hall of Fame. I have wanted to go there for a long time, so I was happy I finally made it there. It is a pretty cool place, and everyone that like football should check it out!

Tuesday morning we went back to the hospital to receive our first dose of EPI 743, and then we had to wait for 2 hours and do another EKG.

So in the mean time we went to the cafeteria and hanged out for a bit, as we were walking back to the heart center, we heard “Code Black” over the intercom and it turns out the hospital had a bomb thread! Their first ever, and of course when we were there! LOL

So the whole place was on shutdown and cops/maintenance people were going room to room checking for suspicious package.

We were still able to get the EKG done and then we went to the parking garage (attach to the annex and not to main hospital) so we were able to leave and head back to the house.

On Wednesday, we just took it easy, relaxed and started to pack.

While in Akron, we were able to see one of our new Mito Mom Friend Shawna and her adorable little boy Braylon. It is so nice to meet in person after talking all this time on facebook!

We also had a visit from my friend Chandra from University of Indianapolis. I have not seen her since we graduated (over 10 years ago) so that was great to catch up for a few minutes and she was able to meet Jagger, even if he was sleeping at the time of her visit!

We had the house rented until Friday am, but we decided to leave on Thursday morning and follow the same itinerary and spend the night in Lexington.

We still hit traffic around Columbus, but nothing near as bad as the way up, however Jagger was a lot more agitated and did not sleep at all (which is not good, as we can usually drive longer when he sleeps). Around Cincinnati, we had to stop to change him and Annett took over while I tried to calm him down in the back. Did not work very well, but we made it to Lexington around 10 pm.

The night was ok, and on Friday morning we left for home; however Jagger had another idea in mind, and was whining the entire time in the car, so we were not able to make it farther than Chattanooga and had to stop and spend the night at a hotel there. That was not plan, but if we did learn one thing with a special need child is that we MUST be flexible at all time, almost nothing goes according to plan!

Jagger was a bit better on Saturday morning and we finally made it home early afternoon on Saturday, it was great being home and I totally understand the saying “home sweet home”

We have to go back early December, and this time since we only have to be at the hospital for 3-4 hours, we will try flying.

That will also be a crazy adventure as we cannot take all his equipment with us in the plane, and it requires a TON of planning (especially with oxygen and meds).

But hopefully that will go well (and Jagger will not get sick with the all the germs in the place) and we can try to fly the next few times we have to go to Akron!!

 Annett and Jagger at the house in Akron

 Sleeping between Dr Visits at Akron's Children

 Ok, now I am awake! What are we doing here??

 Ready to go!!

 Hotel have big beds, I like that:)

1550 miles

This past week and a half we drove over 1550 miles. To put this into perspective

1550 miles= 2500 kilometers

This took us roughly 5 days. 2 days there and 2.5 days back as we had to stop several times for Jagger ( 3 hour car ride is his limit). Spent 3 nights on the road at different hotels and 6 nights in Ohio. I loaded and unloaded the car about 10 times.

We crossed 4 states.

When you drive 1550 miles/2500 kilometers in Europe you would have easily crossed three different countries, learned to say hello and ask for the toilet in three different languages.

In most of Africa, 1550 miles would have probably taken about 3-4 weeks including abandoning your vehicle and continuing by canoe, foot, motorcycle, mule etc. Or another way to look at it: 5 days of driving would have gotten you to the city limits or maybe the suburbs:). I may be exaggerating a little but it surely feels like it every time I am there. If you think traffic and roads are bad here in the U.S. you have seen nothing yet. ;)

In addition to the countless hours of driving I also spend an additional hour cleaning Jaggy's car seat due to major poop blowout and an afternoon and $50 at the car dealership where the nice mechanics took our car apart to look for my wedding bands and engagement ring which fell under the back seat underneath the carpet into the frame of the car. Thankfully there are no holes down there.
The poop blowout resulted in an additional stop in BFE at some ghetto gas station in freezing rain. 1 hour and 72 wipes later Jaggy was clean but Sebastien and I had poop on our pants shoes and jackets. Lovely.

"I smell like roses"

We finally were able to give Jaggy boo a bath after the week long hospital stay. Bathing is limited at the hospital because his port is usually accessed.
He seemed to enjoy the bath. Was snoring most of the time:)

And now we have a squeaky clean baby who smells like roses as daddy always says.

Done with another hospital stay!

So it has been a while since we updated the blog, and we still have to write entries on the end of our Orlando trip and Sebastien Citizenship ceremony, we will try to do that shortly.

But for now, we just spend the last week in the hospital with another Pneumonia.

It has been nearly impossible to avoid those aspiration pneumonia, I believe this is our 4^th or 5^th already this year.

Last week Jagger was pretty agitated most of the week, we could tell something was not right. On Thursday I had a day trip to Chicago and he was not feeling well at all during the day, I was able to calm him down a bit and get him to sleep when I got home, but the next morning he was again having a lot of pain, agitation and labored breathing , so we took him to the ER.

And sure enough his white cell were pretty high and he had pneumonia, so for the 13^th time (I think), we were admitted to the hospital.

They put him on a different antibiotics than last time he had pneumonia since his stomach is still trying to recover from that one almost 2 months later.

On Sunday, they took more blood, the white cell were back down and Jagger was feeling better, so we were optimistic about going home on Tuesday.

But on Monday night, he started to have major breathing issues and was destating a lot, going down to the low 80’s, so we had to give him oxygen and we could only get him back to the mid 90’ on 50%.

So the doctor came back and we started packing our stuff to go to the ICU. But we were able to get his breathing under control around 6 am.

He had very thick secretions and since his muscle tone is very weak he could not cough them out so we had to do a lot of deep suctions. Jagger really hate that, but on the other hand who would like a huge tube going down your nose all the way to your throat!

We cut down one of his meds that can make secretions thicker and we were able to suction him thru the mouth (a lot better for him).

After that, he gradually got better and he is almost back to his normal self.

On Thursday afternoon, we were discharged and back home!!

We have a lot to do before  next Thursday, when we embark on our next big adventure, but we will post more about that this weekend.

Annett and Jagger taking a nap

" I m so ready to get out of here!!"

I pledge allegiance...

Sebastien successfully passed his citizenship interview and is now a full member of this society. He can vote and claim social security and can now join the peace corps. He was already able to join the army if he wanted to with his green card but not "fight" for peace. Go figure. He can also join the government except he will never be able to run for president.

Thankfully the branch of immigration services that does interviews and oath ceremonies isn't too far from our house. He had his interview on Thursday and was able to take the oath on Friday. I joined him for the oath. I had to be there to take a few pics at least. We usually do not leave the nanny alone with Jagger, this was an exception. In fact we never leave Jagger alone with anyone (last time we were both gone and Jagger was alone with the nanny he coughed really hard and tipped forward and fell off the bed onto the IV pole stand. Arghh. She learned her lesson and now builds a fortress around him if she has to step away for a moment. Of course Sebastien was far away at a mito meeting and I was at the dentist not really in a position to jump up and run home.)

The day of the ceremony we also had a nursing agency visit to do an assessment and intake paperwork. Jagger's routine was thrown out of wack and he was pretty restless and was breathing funny the entire day. Of course this was also the day my phone didn't work and although Sebastien and I were able to sit together while we waited for the ceremony to start we were soon separated as all family members were asked to remain in the back of the room.

I took his phone so I could keep texting with the nanny to get status updates on Jagger. I then communicated via thumbs up or down and head shaking or nodding to Sebastien. Although this place wasn't too far we still took two cars just in case I had to leave.

The ceremony was interesting. A little different than mine. As the MC read aloud (with much difficulty I may add) each country represented during the ceremony, that "former" citizen of that country had to stand up. There were over 40 nationalities present.

They then took the oath, pledge allegiance to the flag, a video played of president Obama welcoming the new citizen to this great nation, another video played showing scenes and landmarks of the US, economic wealth and employment and pictures of other oath ceremonies across this country. The videos were accompanied by patriotic soundtracks such as "America the beautiful" and "God bless America" by Lee Greenwood. The ceremony ended with the star spangled banner of course. Then everyone went to the front and received their certificate of naturalization. This whole thing lasted an hour although we spend 1.5 hours prior waiting for the ceremony to start.

Let's hope he was naturalized in time to vote in this upcoming election.

I'm in candy apple heaven

While Jaggy got a super sized Mickey, I chose candy apples as my souvenir from Disney. I know they don't last but the memory of their deliciousness will remain engraved in my taste buds forever:). These by far are the best candy apples I have ever had. Each apple dipped in caramel then milk chocolate, white chocolate and dark chocolate. Really, it can't get any better! With every bite I commented to Sebastien that I was in candy apple heaven. I just wish Jaggy could have enjoyed them as well.

Happy Birthday Boo-Boo (a look back at the last 2 years)

So Jagger turned 2 last Friday, that on its own is an amazing accomplishment.

What a big boy he has become and he even broke 25 lbs yesterday, that is amazing considering that he was stuck at 15 lbs for almost 7 months

He has one of the worst/most severe type of Mitochondrial disease, and I know several families with the same disease that lost their children before 2 years old, so we are bless to still have him with us.

But if you told me in January that he will see his second Birthday, I would have not believe you.

His pain was so bad and not controllable that we were adviced to get our parents to town to see him one last time, so on a short notice, we got my parent and brother to fly from France and Annett parents to drive from New York.

And by time my parents got here, he was doing better and was discharged a day later, so that was great and unexpected but we did dodge a bullet.

We still struggle with his pain despite the incredible amount of medication he is taking, and I am not sure if we will ever able to avoid pain crisis, but at least we can try to make them shorter and more manageable.

So let’s take a look back (in numbers) at the last 2 years with our little boo:

11  trips to the ER

10   hospital admission/stay (including 6  In ICU)

60  days in the hospital (including 30 days in ICU), roughly 11.5% of his life has been spend in hospital L

5   days on a ventilator

2   Surgeries (and that number is still low probably because he can’t have any more anesthesia without a 50-50 risk of not making it)

85  days with a NG tube (feeding tube in his nose. We switch to a G tube directly in his stomach on August 17^th 2011)

63  doctor and Therapist visit outside of our home (and that stopped on December 1^st, 2011 when he was put on hospice care, now we only go see Mito specialist a few times a year)

2 weekly therapy visits in our home (PT and OT)

11  daily medications  (plus some as needed for pain

31   doses of those medications he has to takes each day (plus some as needed meds for pain)

18-20 numbers of hours he sleeps a day (and the remaining 4-6 hours he is usually in pain for at least half of those)

And for the crazy number of the day, in his first 2 years of  life, Jagger medical bills (as of September 1^st) are: $510,270, no this is not a Typo, over HALF a MILLION DOLLARS. Now of course, that is not the amount we paid since we have insurance and Deeming Waiver, but we still had to pay a portion, it is not free even with good insurance!! I’m still shock at the number, this is absolutely INSANE that healthcare would be so expensive!!

And on the bright side:

2 visits to Disney (never to the park as he only has enough energy to be up 1 hour at the time during the day, but he still got to meet Mickey)

                                                                                                                                 

So as you can see, it is a daily struggle (and a lot of work) just to keep him alive, but it’s also a honor to do all we can to make his short time on his earth as happy and fun as possible.

I honestly did not think it was possible to love someone as much as I love my Jaggy, and his perseverance, positive attitude and happiness taught me (and everyone around him) so much. For a non verbal, non mobile kid, he definitely  know about to brighten a room and make everyone around him happy and smiley.

We really don’t know how many more birthdays (if any) we will have with him, so we just take it one day at the time, and make the most of each moment he is awake and we spend with him (as cliché as it may sound! LOL)

Such a big boy!!