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.
Tuesday, December 27, 2011
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.!
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
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.
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.
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!!
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!!
Monday, November 28, 2011
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.
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.
Thursday, November 24, 2011
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.
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.
Tuesday, November 22, 2011
Back to square one (Hospital Stay Day 12)
We are out of the ICU, after more than a week, and we are back on 5East, right across the hall from where it all started last week when Jagger went into respiratory failure.
It is nice to be back on regular floor, as there are no limitation on when you can come in and out and you can have food in the room. We had to take turn eating for the last week as you are not allowed to eat in the ICU.
On the other hand, now, we have to share a nurse with several rooms and it sometimes take forever to get what you need.
We meet the Attending that will look over Jagger, Dr. B is very nice and really take her time to answer all our questions.
Hopefully we will not be here too long, but Thanksgiving will be in the hospital this year.
Jagger seem to have calm down a bit even if he still arch his whole body like a cirque du Soleil performer and he still cannot see
It is nice to be back on regular floor, as there are no limitation on when you can come in and out and you can have food in the room. We had to take turn eating for the last week as you are not allowed to eat in the ICU.
On the other hand, now, we have to share a nurse with several rooms and it sometimes take forever to get what you need.
We meet the Attending that will look over Jagger, Dr. B is very nice and really take her time to answer all our questions.
Hopefully we will not be here too long, but Thanksgiving will be in the hospital this year.
Jagger seem to have calm down a bit even if he still arch his whole body like a cirque du Soleil performer and he still cannot see
Crazy weekend (Hospital Stay Day 11, ICU Day 8)
So we just had the craziest weekend ever. Jagger was literally "crazy" and no one could figure out why.
They tried so many different drugs this weekend, they thought maybe he has Benzo (the sedation he was on while on the ventilator) redraw, so they tried to give him small doses, but it did not help. From Friday 6 pm to Monday 9.30 am, he slept 7 hours, and only 30 minutes at the time max, he was exhausted and kept on moving in very strange ways almost like he was possess, and he lost his eyesight, he does not see anything and his eyes are mostly in the back of his head. So when you get close to him and talk to him he get scared because he can see you coming near him.
Dr. K said he might just need time to get the drugs out of his system and to feel better and said we need to try to get home as soon as possible because he will get better at home. But it seems we will be here for a few more days at least
It was so bad last night, that the fellow on call, decided to give him Cholorhydrate, he said that was the best drug to put kids to sleep and they used it all the time and he will sleep for hours. Well Jagger had some kind of reaction to the drug and his heart beat got elevated and he became all flush, and only slept for 20 minutes, the fellow was speechless, and said he never saw that.
Anyhow, after this crazy weekend, Jagger fall asleep at 9.30 this morning and slept most of the day, he was so exhausted.
Dr. Ka said they will try to get us back to the regular floor tomorrow because now that Jagger is stable he needs to get out of ICU so he does not get any virus or such.
They tried so many different drugs this weekend, they thought maybe he has Benzo (the sedation he was on while on the ventilator) redraw, so they tried to give him small doses, but it did not help. From Friday 6 pm to Monday 9.30 am, he slept 7 hours, and only 30 minutes at the time max, he was exhausted and kept on moving in very strange ways almost like he was possess, and he lost his eyesight, he does not see anything and his eyes are mostly in the back of his head. So when you get close to him and talk to him he get scared because he can see you coming near him.
Dr. K said he might just need time to get the drugs out of his system and to feel better and said we need to try to get home as soon as possible because he will get better at home. But it seems we will be here for a few more days at least
It was so bad last night, that the fellow on call, decided to give him Cholorhydrate, he said that was the best drug to put kids to sleep and they used it all the time and he will sleep for hours. Well Jagger had some kind of reaction to the drug and his heart beat got elevated and he became all flush, and only slept for 20 minutes, the fellow was speechless, and said he never saw that.
Anyhow, after this crazy weekend, Jagger fall asleep at 9.30 this morning and slept most of the day, he was so exhausted.
Dr. Ka said they will try to get us back to the regular floor tomorrow because now that Jagger is stable he needs to get out of ICU so he does not get any virus or such.
Saturday, November 19, 2011
Taking the tube out (Hospital stay Day 8, ICU day 5)
Jagger did good last night, nothing happen, which is good. This morning he had another blood transfusion.
Then the process of weaning off the sedation started so the doctors could take him off the ventilator.
As a parents this is real hard to watch, becuase as the sedation worn off, jagger was being more and more agitated and did not like that tube in his mouth. We had to try to keep him as calm as possible but it is not easy to do.
Finally the RT came over and took the tube off, the key was to keep him calm or it would get very upset and stop breathing again (he does that when he is very upset), so Annett took him in her arms right away and she was able to keep him calm enough that they did not have to put him back on the ventilator.
Jagger was of course exhausted so he took a little nap. When he woked up, he seemed pretty happy and even smiled at Annett for a few seconds, then took another nap.
Now, when he woke up from his second nap, he was a totally different child, he was very very agitated, did not recognize anyone and keep throwing his head back, and it was impossible to calm him.
The doctors started him a a couple of meds to see if he would help, but so far nothing seem to help at all.
Jagger is only taking short catnap but is not getting any restful sleep at all, I really wish they could find a drug that work SOON!
Then the process of weaning off the sedation started so the doctors could take him off the ventilator.
As a parents this is real hard to watch, becuase as the sedation worn off, jagger was being more and more agitated and did not like that tube in his mouth. We had to try to keep him as calm as possible but it is not easy to do.
Finally the RT came over and took the tube off, the key was to keep him calm or it would get very upset and stop breathing again (he does that when he is very upset), so Annett took him in her arms right away and she was able to keep him calm enough that they did not have to put him back on the ventilator.
Jagger was of course exhausted so he took a little nap. When he woked up, he seemed pretty happy and even smiled at Annett for a few seconds, then took another nap.
Now, when he woke up from his second nap, he was a totally different child, he was very very agitated, did not recognize anyone and keep throwing his head back, and it was impossible to calm him.
The doctors started him a a couple of meds to see if he would help, but so far nothing seem to help at all.
Jagger is only taking short catnap but is not getting any restful sleep at all, I really wish they could find a drug that work SOON!
Friday, November 18, 2011
Leigh Disease (Hospital stay day 7, ICU day 4)
After another crazy day yesterday and a second intubation, the night was pretty quiet. We met with a resident Surgeon this morning to discuss the Port surgery. Since the main reason we are in the ICU this time is because Jagger got over work, over upset when they could not get an IV going on Monday, and since unfortunately he will more than likely need quite a few more IV in his life, we decided to have a port implanted in his body. In a nutshell, is a device that is surgically place under his skin at the left front shoulder level that has a tube that goes directly into the heart. So we can deliver medication, hydration and get blood with only minimal "poking". You have to use a special needle that goes into a spongy reservoir right under his skin and if you use numbing cream he should barely feel the poke. Once the needle is in, it can stay for a week, so you technically only need to poke him once a week. The only drawback is that if we don't use it for a month we still have to access it and flush at least once a month so he does not develop a blot cloth.
The resident did not know much about the procedure, which made us a little worry but we made sure he was not the one doing the surgery. We also talked with the anesthesiologist as it is very tricky to use sedation on a Mito kid.
We were able to take Jagger all the way down to the surgery floor and give him a kiss before the surgery.
He was back in the room a few hours later and everything went well. They did not even used stitches and used some kind of special glue that should minimize the scar.
At 3pm, we had our meeting with the whole team, Dr. Ka (ICU attending), Dr. S (GI), Dr. K (Neuro), M. (Genetic Nurse) and a social worker were there.
The short term plan is to take Jagger off the ventilator tomorrow, to make sure all the anesthesia drugs are out of his body. He will also get another blood transfusion before then to give him extra strength. His Hemoglobin was back up to 9, but that is still a little low.
We then discussed about the Mitochondrial findings and some of the drugs we could use to try to minimize the symptoms, we have a few options mostly to reduce his movement disorder and to see if we can somehow get his cells to produce more energy. Of course, none of those drugs have been proven to work very well, but there is very little side effect, so the drugs might help him.
We also discussed his nutrition, we need to get him to eat more calories and take more volume in, but his gastric delay emptying make it very difficult. So we will have him on TPN for a few days, it is IV nutrition but you don't want to stay on it too long as it can damage the liver.
After everyone left, M. stayed a little longer and drop a bombshell on us, she said after reviewing the latest MRI and his tests results, Jagger has Leigh or Leigh like disease. It is the not only the rarer of Mitochondrial disease but also the most complex and least understood by anyone. And it has the shortest life expectancy.
She also tell us what we should look for in the future and how the closest most promising research is at least 7 to 10 years away from a potential cure (and that is being optimistic). She wants us to start seeing a pulmnologist as most kids with Leigh have respiratory problems as the disease progress.
Unfortunately, Jagger also has a very severe form of the disease, and while it is impossible to know how fast it will progress, the progression is however unstoppable.
M also discussed the next step in testing, they will run next generation DNA sequencing on his blood and tissue, he will be one of the first one in the country to have that done. It should give us more details on the exact gene mutation that is causing the disease and should also tell us % of chance it will happen again if we ever decide to have another child.
So here we are now reflecting at another insane day, a week ago, we took our little boy to the ER for some blood in his stomach, and now he went to respiratory failure twice and we just found out his has a fatal, terminal, fast progressing disease, sometimes life just sucks!!
On a lighter note (well maybe not), I almost set the hospital on fire tonight! Annett went to the sleep room and her back was hurting, so I went to the family room and warmed up a heating pad in the microwave (as I did last night). After a few seconds I smell burn, and looked at the microwave, there is smoke coming out of it, so I stop it and open it, so not there is smoke all over the family room, I was just waiting for the smoke alert to come one, I started waiving my sweater to disperse the smoke. I also unplugged the microwave but smoke was still coming out, so Annett called engineering. They came ovfer and took teh micorwave down to their shop just in case he kept smoking.
About 30 minutes later, engineering came back to see me and told me he was sorry but could not save the heating pad! LOL, it was all charred and burnt.
The resident did not know much about the procedure, which made us a little worry but we made sure he was not the one doing the surgery. We also talked with the anesthesiologist as it is very tricky to use sedation on a Mito kid.
We were able to take Jagger all the way down to the surgery floor and give him a kiss before the surgery.
He was back in the room a few hours later and everything went well. They did not even used stitches and used some kind of special glue that should minimize the scar.
At 3pm, we had our meeting with the whole team, Dr. Ka (ICU attending), Dr. S (GI), Dr. K (Neuro), M. (Genetic Nurse) and a social worker were there.
The short term plan is to take Jagger off the ventilator tomorrow, to make sure all the anesthesia drugs are out of his body. He will also get another blood transfusion before then to give him extra strength. His Hemoglobin was back up to 9, but that is still a little low.
We then discussed about the Mitochondrial findings and some of the drugs we could use to try to minimize the symptoms, we have a few options mostly to reduce his movement disorder and to see if we can somehow get his cells to produce more energy. Of course, none of those drugs have been proven to work very well, but there is very little side effect, so the drugs might help him.
We also discussed his nutrition, we need to get him to eat more calories and take more volume in, but his gastric delay emptying make it very difficult. So we will have him on TPN for a few days, it is IV nutrition but you don't want to stay on it too long as it can damage the liver.
After everyone left, M. stayed a little longer and drop a bombshell on us, she said after reviewing the latest MRI and his tests results, Jagger has Leigh or Leigh like disease. It is the not only the rarer of Mitochondrial disease but also the most complex and least understood by anyone. And it has the shortest life expectancy.
She also tell us what we should look for in the future and how the closest most promising research is at least 7 to 10 years away from a potential cure (and that is being optimistic). She wants us to start seeing a pulmnologist as most kids with Leigh have respiratory problems as the disease progress.
Unfortunately, Jagger also has a very severe form of the disease, and while it is impossible to know how fast it will progress, the progression is however unstoppable.
M also discussed the next step in testing, they will run next generation DNA sequencing on his blood and tissue, he will be one of the first one in the country to have that done. It should give us more details on the exact gene mutation that is causing the disease and should also tell us % of chance it will happen again if we ever decide to have another child.
So here we are now reflecting at another insane day, a week ago, we took our little boy to the ER for some blood in his stomach, and now he went to respiratory failure twice and we just found out his has a fatal, terminal, fast progressing disease, sometimes life just sucks!!
On a lighter note (well maybe not), I almost set the hospital on fire tonight! Annett went to the sleep room and her back was hurting, so I went to the family room and warmed up a heating pad in the microwave (as I did last night). After a few seconds I smell burn, and looked at the microwave, there is smoke coming out of it, so I stop it and open it, so not there is smoke all over the family room, I was just waiting for the smoke alert to come one, I started waiving my sweater to disperse the smoke. I also unplugged the microwave but smoke was still coming out, so Annett called engineering. They came ovfer and took teh micorwave down to their shop just in case he kept smoking.
About 30 minutes later, engineering came back to see me and told me he was sorry but could not save the heating pad! LOL, it was all charred and burnt.
Thursday, November 17, 2011
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.
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.
Wednesday, November 16, 2011
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.
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.
Tuesday, November 15, 2011
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.
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.
Saturday, November 12, 2011
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.
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.
Monday, November 7, 2011
It's Mito
So this is going to be the first entry of this blog. Many things happened before today and you can read some of the background and issues that Jagger had at the top of the blog under the Jagger Tab.
So we had our appointment today with Nurse M (genetic nurse) to get the results of Jagger Muscle biopsy and Spinal Tab (ST) from August.
We went over many things but in summary Jagger has a very clear case of Mitochondrial disorder. At this time, it is not possible to classify it under a specific Mito disease, so she said to call is Jagger disease for now.
She explain a little about Mito disease in general and about the concept of baseline. For Mito kids, when something happen, such as an illness or something that make them very upset or change their routine dramatically, they can have what is call a "mito crash", when that happened, it can take them a very long time to get back to their initial baseline and depending on the severity of the "mito crash", they might never get back to baseline. So it is very important to try to do all we can to avoid any crashes for Jagger.
She also suggested we start him on a "mito cocktail" of drugs to see if that help him or not, it is not proven but it does help some mito patients. He will take Carnitor, to help carrying fatty acid into his Mitochondrial cells, Leuvocorin to help his brain function and Co Enzyme Q10 to help the energy move from within his Mitochondrial cells. Since they are consider supplements, some of those drugs are not reimbursed by the insurance and are very expensive. The Q10 for example is about $300 a month and very difficult to get as it is a special concentration that can only be bought on the internet.
We will try those drugs with Jagger for a few months and see if they improve his energy production or not. We have to get a blood test 6 weeks after we start the Q10 to see if his body absorb it or not.
So now all we can do is wait and see if the cocktail help him out, there is not cure for Mito and it is progressive, however, the range of the disease is very wide, it can vary between just having to take drugs and a special diet to very short life expectancy depending which organs are starve of energy
UPDATE 11/08: We received the summary notes from Dr. S (Neuro genetics Dr) this morning, and it said that Jagger Mito disease is "severe" based on clinical/test results. he also said he is not sure the cocktail will help him at all based on the severity of his condition. Hum, can this get any worst!
So we had our appointment today with Nurse M (genetic nurse) to get the results of Jagger Muscle biopsy and Spinal Tab (ST) from August.
We went over many things but in summary Jagger has a very clear case of Mitochondrial disorder. At this time, it is not possible to classify it under a specific Mito disease, so she said to call is Jagger disease for now.
She explain a little about Mito disease in general and about the concept of baseline. For Mito kids, when something happen, such as an illness or something that make them very upset or change their routine dramatically, they can have what is call a "mito crash", when that happened, it can take them a very long time to get back to their initial baseline and depending on the severity of the "mito crash", they might never get back to baseline. So it is very important to try to do all we can to avoid any crashes for Jagger.
She also suggested we start him on a "mito cocktail" of drugs to see if that help him or not, it is not proven but it does help some mito patients. He will take Carnitor, to help carrying fatty acid into his Mitochondrial cells, Leuvocorin to help his brain function and Co Enzyme Q10 to help the energy move from within his Mitochondrial cells. Since they are consider supplements, some of those drugs are not reimbursed by the insurance and are very expensive. The Q10 for example is about $300 a month and very difficult to get as it is a special concentration that can only be bought on the internet.
We will try those drugs with Jagger for a few months and see if they improve his energy production or not. We have to get a blood test 6 weeks after we start the Q10 to see if his body absorb it or not.
So now all we can do is wait and see if the cocktail help him out, there is not cure for Mito and it is progressive, however, the range of the disease is very wide, it can vary between just having to take drugs and a special diet to very short life expectancy depending which organs are starve of energy
UPDATE 11/08: We received the summary notes from Dr. S (Neuro genetics Dr) this morning, and it said that Jagger Mito disease is "severe" based on clinical/test results. he also said he is not sure the cocktail will help him at all based on the severity of his condition. Hum, can this get any worst!
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