One year ago

One year ago today, December 21st 2010 was the beginning of Jagger health problem, but at that time, we really did not know how bad everything was going to get throughout the year.
During a visit to the ped a few days earlier, we were told Jagger has a heart murmur but that is pretty common for babies, so not to worry about it, but we can get it check at the heart clinic if we want.
So just to be on the safe side, we made an appointment to the Sibley heart center. we went to the clinic  and they did an EKG, which show some abnormal results, so they decied to to an echocardiogram.
This lasted forever as Jagger was moving a lot.
After a few minutes wait, one of the doctor came by and told us something was wrong with Jagger heart. Of course it was a huge shock to us as he never had any symptoms (turning blue, sweating when eating, difficulty breathing, etc). We were told he has an enlarged heart and we should drive to the Special heart clinic that meets on Monday. Fortunately that clinic was closer to our home, however we were told to go there right away and not go home first. The urgency of the situation seemed a little strange so we only stop to grab a quick lunch and went to the clinic.
That is went the madness started. We were only in the clinic for 5 minutes when Dr. S. (Cardio) told us that Jagger has Hypertrophic Cardiomyopathy (HCM) which is an enlarged heart (left ventricular wall in his case), it is a very severe condition that affect his heart function and they admitted us to the cardiac unit at the Children Hospital.
Of course, we were shock beyond believe. 3 hours earlier we went in to check a "normal" heart murmur and now we were in the cardiac unit.
In the cardiac unit everythign has a sense of urgency and Jagger had cables hooked up all over him with doctors and nurses coming in and out every few minutes.
We saw several doctors and no one seems to know why that was happening, we talked to a genetic counselor that asked a ton of questions about our families to see if anyone has HCM since it is usually inherited. So we called our families in France and Germany to make sure but of course no one had it or died from a heart condition, so it was a total mystery.
The doctors were really worrying about Pompe disease, so they took a lot of bloods to do genetic testing. they said it usually takes 3 to 4 days to get Pompe results but they will put a rush on it and we should have the results before Christmas. We were also told not to look up Pompe on the internet, which of course we did and saw how devastating that disease was. I already knew some of it as a few weeks earlier I saw the movie" Extraordinary Measures" in a plane. So we were really hoping it was not Pompe, now we know it is actuality worst that Pompe but at that time we were just stressing out like crazy waiting on the results.
We also did not know if we would spend Jagger first Christmas in the hospital or not.
After one night in the cardiac unit, they saw that his heart function was not getting worst and he was not showing symptoms so they sent us home as they were nothing they could do until we get some of the genetic test results back. But we still did not have the Pompe results. We actually had to wait until the 24th in the morning to find out that Pompe test was negative.
So on the 25th, we sat at home, not really sure what had just hit us and just wonder if that would be Jagger first and only Christmas or not. Life has just change forever in a heartbeat.!

Almost Home (Hospital Stay day 20)

We had a chance to go home today but the hospice care is not going to be set up until tomorrow.
Yes, hospice care. We had several conversation with doctors and Palliative care team at the hospital and everyone seems to think it is the best option for Jagger. Now, hospice care for children works a little differently that with adults. For adults, it means that they have less than 6 months to live, for children, it just mean it is a terminal disease but there is no "timeline" for say.

The key is to try to avoid the hospital from now on (especially during flu season), since Jagger immunize system is so weak we need to try to stay home as much as possible. And Hospice care will help with that, as the nurse will be able to come every days if needed/ Since he has the port, the nurse will be able to start the IV very easily and Annett and I will be able to start the IV fluid as needed when his port is access.
We met the Hospice Atlanta nurse, Nurse D. She seems very nice and has a lot of experience with terminally ill kids and special needs kids.

M. (Genetic Nurse) also stopped by tonight, she is great, she stayed until 8.30 pm to make sure we had the answers to all our questions. We discussed the DNA sequencing and what will the results mean, what to expect with Jagger and a lot more. She is not able to give us an exact prognosis on Jagger since this disease affect each person so differently, depending on the exact gene mutation (this is why in part we doing DNA sequencing). However, over 95% of child with Leigh or Leigh like do not make it to their 5th birthday, so either way you look at it, it is not good:(
She also said that the diagnosis of Leigh like was pretty certain based on the MRI and the muscle biopsy finding, it is a certitude he has Leigh.
She warned us that our life is going to be a roller coaster with good and bad days but eventually the bad days we outnumbered the good days. She also said that the next few weeks will be critical, as it will take logner for Jagger to get back to "baseline" we need to make sure he does not get sick or he will have a very hard time get over it.
So we decided to cancel Annett's parents and sister (with 3 kids under 8) trip to Atlanta for the Holidays. It is better not to have 3 young child that just spend 10 hours in a plane come to visit us. we do not want to take any chances. We also cancel Jagger appointments to Ped, PT, OT, ST, etc. Hospice care will take care of his immediate needs, Baby can;t wait comes to the house once a week for PT and for the rest we will see how he is after flu season.
So we started packing and if all goes right tonight, we should be home tomorrow mid day, after 3 weeks!!

Another weekend in the hospital (Hospital Stay Day 18)

We just spent another weekend in the hospital.
Nothing new is going on, we are slowly increasing Jagger feeds.
We had to go slow since he did not eat anything while he was in the ICU.
So he is on TPN (IV Nutrition) and half strength formula. so far so good, and we giving him a little more each day. The ultimate goal is to feed him 18 hours a day (Poor kid)

Dr. B came by and gave us an article on Leigh Disease, nothing really new that we did not know in it. A lot of incertitude about the course of the disease but the prognosis is certain and there is nothing we can do about it.
Since we have a more define diagnosis, all doctors attitude have change so much toward us, they pretty much agree with anything, any course of action we want to take.
The challenge the last few days is that the GI, and Neuro docs on call are not our doctors, and with the complexity of his condition it is very difficult to get a new doc up to date on all that happen.
However, we are lucky enough that Dr. K stop by to see how Jagger is doing even if he is not on call. he has been great with Jagger and helping us trying to figure this disease out and finding drugs that can help him with his movement disorder.
We were told that unless we have a setback, we should be going home this week!! we can't wait, since he has the port in place now, we could handle everything at home if we get nursing help.

Thanksgiving (Hospital Stay Day 14)

Today is Thanksgiving and we spent it in the hospital. Annett's parents had to cancel their trip as there is really no reason for them to come while we are still in the hospital.
Dr. B is out for a couple of days and Jagger is doing OK, he is on a ton of meds and they seem to start helping a little. They reduce his Methadone dose and he was very agitated so he is back on 3 times a day. I don;t like for him to be on so much strong meds but if that is what it takes to control the pain, then we don't have a choice.
We still had a Thanksgiving mean and it was great. Thank you to the volunteer that set up the meal for all the family that are in the hospital, it was very good and all the Thanksgiving favorite were available.
Jagger eyesight is starting to slowly come back it seems, we got a few smiles today, so that is good news, even if he still has weird eyes movement.

If you don't take this tube out I will ( Hospital Stay Day 6, ICU Day 3)

I was able to get some sleep in the sleep room last night, not great sleep like in a hotel or at home, but sleep never less.
Morning was pretty uneventful (which is good in the ICU), they talked about getting him off the ventilator but since we decided to have a port placed in him tomorrow (so we never have to look for an IV access ever again) they decided to keep him intubated until after the surgery. Annett went home in the afternoon, as I wanted to make sure she is there to received a package I had sent for her birthday. I ordered some Hawaiian flowers from the Big Island and they arrived this morning and were waiting on the front door for her.
While Annett was away and I was sitting in the room and I heard some strange "wind" like noise and I look up, Jagger was very agitated and all machines were beeping like crazy, so I went to the bed and saw the tube moving so I held it and the nurse came right away and told me he was just coughing, but unfortunately, he was more than coughing, while sedated on with hand restraints he somehow got the tube out of his mouth. So the nurse tool it out completely and gave him a little oxygen, but he was getting very agitated and he did like he did Monday, got very upset and stop breathing, so next thing I know, there are 10 people in our room and the Dr. Ka asked me to go wait in the waiting room, they are to re-intubate him as he was struggling to breath on his own.
Dr. Ka came get me abotu 30 minutes later and everything was back to "normal", Jagger was back on the ventilator and under heavy sedation.
Later, Dr. K (our Neuro) came by to discuss the result of the MRI, white matter was growing, which mean his brain was developing, however they found some "spot" in the basal ganglia which explained his movement disorder and the fact that Jagger does not control his hand/arm movement at all.
we will discuss the findings in more details tomorrow during our meeting.
The rest of the evening was pretty uneventful. Tonight, Annett will go to the sleep room and try to get rest.

The morning after in the ICI (Hospital Day 5, ICI day 2)

 I was able to get one of the sleep room and get a little rest while Annett stay in the ICU room. We will take turns so at least one of us get some sleep. however it did not work as plan, early this morning (at 3 am), Annett texted me to come back to the room.The resident in charge of night shift was worrying about Jagger low Hemoglobin level (around 7, while normal is 10.5 to 13.5), and wanted to do a blood transfusion. He seems to think it will help him get stronger and fight any possible infections. At this point, we really wonder what else can possibly happen, and what he will need next, this seems to be going downhill every days. We decided to call my Dad for some advice and he seems to think it would be a good idea based on the situation. So we agreed to do the transfusion. However Dr. Ka (Attending ICU) decided we should wait until the morning as it will be easier to deal with any possible side effect when the day team is there.


Annett texted me at 8 am (you cannot get in or out of the ICU between 7 and 8 am) that M. (our genetic nurse) was here to check on him. We had a good conversation with her and we have a meeting set up for Thursday with the entire team to discuss/come up with a plan for his care. She also agreed that the blood transfusion was a good idea and that we need to get his hemoglobin level as high as possible before we take him off the ventilator.
Dr. Ka  explained to us that Jagger was actually doing most of the breathing on his own and the ventilator was only helping a little bit. They have him under heavy sedation but that does not seem to work so well, he does open his eyes a lot and move quite a lot, so they keep having to give him extra boluses because of his disease, his body metabolize the sedation very fast.
Since, he is already under sedation, I have been calling all his doctors to see if they want/need any test. A few needed some, so he had a Endoscopy and a MRI. The endoscopy was perfect not even showing any inflammation, so it is still a mystery where the blood in his stomach came from (hte one reason why we came to the ER in the first place last week).
Dr K (our Neurologist) will take a look at the MRI later today and will come talk to us tomorrow. He has a MRI 6 months ago, so we will be able to compare them and see how the disease progress and if his brain is still growing as it should.
Hopefully we get good news tomorrow. Looks like I am getting the sleep room again tonight, Annett wants to stay in the room with Jagger.
Today is also Annett's birthday but since we are stuck in the ICU she decided she did not want to celebrate her Birthday this year. Hopefully we can still do something special all 3 of us once we get out of this place.

Craziest Day Ever (Hospital stay day 4, ICU day 1)

Jagger did pretty well this weekend, and I was really pushing to go home yesterday but they wanted to keep him one more night to make sure he did OK with his feeds and was hydrated enough.
However, Jagger woke up this morning crying and has been in pain all day. Since he lost the IV they started in the ER on Friday, they had to try to put another IV in. Our nurse tried a couple of times and could not get it, so she call the flight team. They are the life support helicopter pilot and they do IV in the field in critical patients so they are very skill at it. Unfortunately, they could not get an IV going on Jagger either.
So Dr. S. (our GI Dr) decided to give him some morphine thru his G tube since he has been crying all day, but that did not calm him down at all. they also decided to stop Reglan ( his med for delay gastric emptying) since he was starting to show some side effects. to counter those, they gave him Benadryl and that put him to sleep for about 20 minutes.
I had to go home to make a few calls and posponed my business trip for this week, and things went donwhill fast while I was gone.
Jagger was still not calming down and getting very dehydrated so they really had to get an IV in him. they call the PICU team (that have a sonogram machine) but they were still struggling and could not get the IV going.
they did find a good vein in his neck and tried that, but it did not work. That however put Jagger over the top and he was so upset and exhausted from crying the entire day that he was having trouble breathing and went into respiratory failure. They had to intube it him right there in the room (no time to go to PICU).

When I got Annett texts I came right back to the hospital right away and never drove that fast my entire life (less than 10 minutes for a 20 minutes trip).
By time I got here he was intubated and ready for transport to the PICU and under heavy sedation.
It took a while to get everything set up in ICU but he was out of danger for now. They also decided to do a femoral central line so they will have a good IV access, since he was sedated he hopefully did not feel any pain at that point.
So now we standing here in the PICU looking at our son on a ventilator and he almost died, while the plan was to go home today.
It is crazy how life can take a turn for the worst so fast.
They are starting him on a lot of different meds and we will talk to the doctors in the morning to see the plan of action.

Going to the ER (Hospital Stay day 1)

Jagger has been very agitated for a few weeks now (since he got his last round of shots) and while I was in Chicago for business, Annett found some blood in his stomach when she vented his G tube, so here we go to the ER again.
After over 6 hours wait, Jagger was admitted to the GI service. We need to find a way not to have to wait in the ER forever and get his admission speed up, since we always end up admitted anyhow!
Tiffany was able to stay with Annett until I got back from Chicago.
I went straight from the airport to home to get clothes, blankets, pillows, etc and I was at the hospital shortly after midnight
Let's hope this is a short stay , but with Jagger you never know.