Tuesday, December 27, 2011

Merry Christmas

Well it is Christmas today and we did celebrate it, well at least the best we could since Jagger was not feeling well, so it took us 2 days to open all the gifts and 4 tries, but we got thru all of it and everyone was very spoil this year as always!
For a while we were not sure if we were going to put up a tree and even celebrate, since things have been very busy and not so great for the last month, but again, we really don't know if this is going to be Jagger last Christmas or not, so we decided to put up a tree, decorations and celebrate the best we can.
Jagger was even OK in his chair so we actually were able to sit down on the 25th and have a nice meal, it is not often we can do that, as usually one of us has to hold him while the other eat, so sitting down all 3 of us as a family was nice!
The original plan was for Annett parents and her sister/husband and the 3 kids from Germany to drive from NYC and spend a few days with us, but per doctors order, Jagger cannot get sick this winter and he could be in real trouble if he catch a cold or the flu, as his body is still recovering from his last "Mito Crash". We cancelled most of our doctors/theraphy appointments for January and February to minimize the risk of Jagger catching a bug, so having 3 kids under 8 that spend 10 hours in a plane coming to visit was not a good idea and we cancelled their trip down to Atlanta.

To all of you that read this blog, Annett, Jagger and I wish you a Merry Christmas, enjoy the time with your family because you never know what life is going to bring next.

Thursday, December 22, 2011

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.!

Saturday, December 10, 2011

Allergic reaction to ????

So yesterday, Nurse D. came over and change the IV access for Jagger, as we cannot keep the needle in for more than a week.
It is quite an ordeal to change the access. It has to be sterile, so anyone helping or neat Jagger when it is change ahs to wear mask and gloves.
The area has to be clean with Chlorostep for 30 seconds then air dry before the needle can be inserted into the port. And if you know Jagger that is the hardest part has it is impossible to get him to stay still for more than 5 seconds so we have to hold his arms during this part of the procedure.
Then it has to be tape very well as you can not leave any opening for possible infection to get in his body.
Anyhow, we notice he was having a reaction this last night, btu it got worst overnight. He did it to a smaller extent at the hospital but this is really bad. We actually had to give him some morphine last night so he coudl sleep.

I am posting a pic to show how bad the reaction was. Nurse D. thinks it is the Chlorostep, but I am not sure if it is not a combination of that and the tape too.
Anyhow, Nurse D. came by and took the needle out and order a steroid cream to stop the swelling and redness. Since he has been getting nearly enough fluid thru the G tube we are going to try to hold off for a few days and not have to access the port until next week so his skin can heal

Wednesday, December 7, 2011

Settling at home

Been a few days sicne the last update, when everything goes well, I will probably only post about once a week or so.
You can sign up on top right to get an email when we have a new post up. I am not 100% sure it is working very well (so still check on here once in a while) but it should help everyone keep up with the updates.

Jagger seems a lot happier since we been home, he is totally a home buddy!! his eyesight is coming back slowly and he is one again smiling big for daddy each time he sees me! I so miss those smiles !
I can see why Dr. K. wanted us to go home as soon as we could when we were in hospital!
He has been on IV since we been back from the hospital (except for one night where we had an issue with the IV bag and could not get the air bubbles out, so we decided to skip the IV). He is eating more and  more and the vomiting is about the same as before the hospital stay. Because of his weak intestinal muscle, Jagger will always have vomiting issues, but those days he only vomit about 1 to 2 ounces a day. A few months back he vomited 3 to 4 ounces so we doing better in that category at least.

We have to give so many medications that I had to create a check list so we make sure he get all his meds everyday. Annett and I are mostly giving to him, but Tiffany is in the loop too in case we are not home for any reasons.
He is getting 6 meds around 8.30 to 9 am, 5 more at 2.30 pm, one med around 4 pm, 6 more meds at 9 pm and finally one more around 11pm. It is a big job keeping up with all the meds for sure.

Friday, December 2, 2011

Home sweet home (Hospital Stay Day 21)

After 21 days in the hospital, including one week in the ICU, we are going home!!
We left hospital early afternoon, it took all morning to make sure we have all papers needed to start hospice care, to have sample of the new formula ( we switching Jagger to a higher calorie formula, but it is not available at the store, it has to be special order at medical supply place).
Nurse D. came to the house and we filled paperwork for a few hours.
She will come at least once a week but more of course as needed. They will take care of all the drugs and suppliers, so all his medications will be deliver to our house directly.
She also called Walgreen infusion and they should come over tonight, deliver IV and show us how to start IV, as Jagger needs to be on IV for at least one week, while we try to increase his food intake thru the G tube.

OK, a few hours later and we are now all pro in starting IV fluid. It is not that difficult, the main thing is to make sure you keep everything very clean/sterile as his port line goes straight into his heart so you do not want to introduce infections.
Our house does look like an hospital now with all the meds and equipment, but at least we are home which is the most important.
Jagger eyesight seems to get a little better and according to Dr. K. he should start feeling better once he is back in his environment, so we will see.

Thursday, December 1, 2011

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!!