A SPECIAL VISITOR

I am back! I have had a couple of rough months, but I am starting to feel better. Sincere thanks to all of you who have been cheering on me. I appreciate you all very much, and you help me through the toughest of times!

Yesterday I got the best surprise that anyone can imagine. My best friend Selina and her sister Leandra came to visit me! We had so much fun together. I got many drawings from both of them, and also a mobile with glass dolphins! It is nicely decorating my IV pole. I love this drawing of Selina and me riding a turtle!

We got some crafts from the play room, and started to play. We painted ceramics. I only wanted to do the same colors as Selina. We then made several drawings for each other. We had fun with the mermaid potato head, and we took turns putting the potato head together. The whole time, my EEG was running! I sure hope they had fun watching us play, since we were on camera the whole time! Selina and Leandra didn't care a bit that my head was wrapped in this white turban. I felt so good about playing, and simply see my friends again. I have already negotiated with sis that she will lend me her barbies and in return she get to use my IPad. I need them for my next play date in the hospital!

 

 

 

 

 

Today, I had a really good Thanksgiving with my family! They all brought Thanksgiving to me. Dad and I watched Macy's Thanksgiving Day Parade on TV while mom and Sarah cooked at home. They got the turkey in the oven when they woke up, and then made some side dishes to go with the turkey. The pumpkin pie they made last night. Mom loves that sis is getting into cooking, and she is good at it.

 

 

I always love when sis is visiting with me. Today was the first time in a month that I got to get out of bed! I loved being up in my wheel chair again. Sis and I had craft time together. We made a drawing of a turkey for the nurses! Sis then asked my nurse, if she could take me for a spin around the hospital. They said yes! This is a big deal, since I am always in isolation, which means I am not allowed to leave my room. The reason I am in isolation is not that I am contagious to other kids, but because my immune system is so low. It is better if I don't leave the room and that everyone entering my room is always gowning up. I sure don't need any extra germs! Mom and dad agreed that today was a good day for a ride around the hospital, since most kids have gone home for the holiday. The hospital is always deserted on holidays.

So, for the first time in 6,5 years I got to go to all the cool places!

 

 

 We also had some special visitors today. Teresa and Stacey came to see me. They gave me a huge giraffe! I named him Axel. Teresa and Matt spent Thanksgiving in the PICU with their sick baby Axel last year. Sadly, Axel passed away a few days after Thanksgiving from Mitochondrial Disease. His parents and aunt Stacey visited the PICU today, and gave out baskets and flowers to families spending this holiday in the PICU. What a neat thing to do in the memory of Axel. We also got a goody basket. Sarah loves surprises like that, and I love my giraffe Axel!

Mom says I also need to let you know how I am doing. They started me on steroids yesterday because my stool hadn't improved as much as they were hoping on my current medications. It seems to be helping. Cross your fingers & toes! I went 16 hours without needing to go, and today I have only gone 1 (!) time to the bathroom. If things continue to improve, I can hopefully start eating again in a couple of days. Please, please, send positive thoughts and vibes that the steroids will work, so I can get home in time for the holidays! I also had an EEG yesterday to see if my subclinical seizures had changed. I have now been on this experimental mitochondrial drug for a week, but unfortunately the EEG was unchanged. My brain is still buzzing away! Dr. C. says that we should not be disencouraged, since I have only been on the medication for a week, and we don't know how well I am absorbing the medication right now with all my stool output.

I am leaving you with a picture of mom & me. She is hanging out with me tonight. I have no intention of going to bed...I simply feel too good tonight for sleeping :-

Happy Thanksgiving everyone,

Jacob.

12 DAYS OF PICU

Since Christmas decorations are already all around us, and the decorations outside the hospital is 12 days of Christmas, what about 12 days of PICU?

It was a very sick boy coming to the PICU 12 days ago. He had stooled out 1,5 liter of stool in 1 hour, which was the final thing that made Jacob crash. He was dehydrated, malnutrioned, and had trouble breathing. His blood pressures were off, and he couldn't keep his oxygen saturation up. He needed the PICU. They stabilized him fairly quickly, and in 24 hours he was seen by more specialties than he has ever seen in his life, and had more tests than he would typically have in a year. Everyone was on top of making Jacob feel better, and understanding what was going on.

I typically dread going to the PICU. We always say we're in the fish bowl, since there is very little privacy with the big glass doors and the nurse sitting outside his door 24/7. But there are times when the PICU is the only place for Jacob. This was one of those times. I know it sounds like a cliche, but as always the PICU team is pretty awesome. I always feel that the chance of the PICU team missing something is low due to the number of tests and labs they are running at all times. I always feel as if Jacob is in very safe hands.

Over the years, we have gotten to know every attending doctor of the PICU. This time, we also got to spend more time with the Head of the PICU. She has always been close with us, and likes to come in and chat with us when she has a few minutes. This time she really advocated for Jacob, and made sure that noone rushed him out of the PICU before Jacob and we were ready. I was very pleased with how she led her whole team when it came to Jacob's care.

The PICU nurses are simply great. You have them at your finger tips at any given time of the day. We had two nurses that we really bonded with this time around, and that will be his primary nurses in the PICU moving forward. As some of you know, I always want to have things done yesterday :- One of the nurses had always taken care of my request before I even had time to verbalize it! That works well with me.

We also got the penthouse of the PICU this time. The PICU is under construction, and the new rooms are larger, has a better bed, and the storage area for personal belongings was a big hit with me! In the old rooms, it is basically no space for your own stuff, so they pile up on the floor and in the window within a few hours. We also had a fabulous Rocky Mountain view.

Since yesterday afternoon, Jacob is now up on the regular floor! This is a step closer to going home, so it is always a step in the right direction. Having a room where we can close the door, have our own bathroom, have a bigger space for our stuff - and still keeping the mountain view made me reflect back on the PICU.

When you spend 12 days down in the PICU, you start to scan their bathroom. The PICU has now upgraded to 2 (!) bathrooms for 34 rooms. It is not a lot, and with that comes the gross factor...It can be gross. Let me just leave it at that. I make sure to always get up early, so I have a chance of being the first shower user of the day. If not, I can't even start telling you about the stuff you can find in a hospital shower! The floor is always flooded by water and hair of all colors...I think you get the picture.

The PICU family fridge...gross....The smell by everyone's leftover is a fact. If you add that someone's soup is leaking out of the plastic bag, and someone has not put on the lid of their stew, you get the picture. I have had times where food disappears as well, but that didn't happen this time. We kept our food to ourselves this time! A great plus is now that you can eat in your PICU room. Before you couldn't. That is a huge improvement, since there are very few times you do feel comfortable to leave your child when they are PICU sick.

In the PICU there is something called shift change. It is between 645-745 am and pm. Since the nurses sit in the hallways giving report on all patients, they don't want families running through the hallways at that time. You have to either leave the PICU before the shift change and stay out of the PICU or stay in your room. I often use that hour as an excuse to leave the PICU room for an hour of the day, especially in the past, when you were not allowed to eat in the room. This time around, I used the morning shift hour for a morning run around the hospital. That really helped me to get the day off to the right start. But I have also forgotten about the shift change, and been in the room desperately needing a bath room or a drink, and realizing I can't leave....

Since the space is limited, I try to only bring things for the time I stay, and then bring more the next day again. What happens is that I always, always forget something! One day I don't have any more clean underwear, no bra or the last night, I had no PJ with me :- Jacob's school t-shirt is way too big for him, which became the PJ of the night for mama. One morning, I took out the last pair of underwear from the closet, and accidently dropped it into the sink! It has a sensor on it, so before I knew it, my last pair of underwear was drenched in water!

The PICU never settles for the night. It is up and running 24/7. This means that it is hard to go to bed. All lights are still on, and Jacob's care continues around the clock. This means that it is often midnight before I hit the sack. And then there is just a matter of number of times you will wake up throughout the night as the nurse or the respiratory therapist or doctor is in the room. I also have this tendency of having to use the bathroom at night. You should see me running through half the PICU to get to the bathroom when the only thing I want to do is sleep :-

By the end of the day, we're so thankful for having the PICU when we need it. There are times where it is the only place for Jacob to be, and the medical team is top notch. But for the little things in life, I feel as I just got the biggest upgrade by moving up to the 9th floor. It isn't Hilton or Four Seasons, but I can close the door when I go to bed, I can run to the bathroom in the middle of the night without having to greet anyone, and I have room to stock up on underwear!

Jacob has had a really great day today! His stool output had decreased yesterday, and today we're not even at half of that volume. If this continues, we might be talking feeds for Jacob sooner than anyone would have thought. This might also mean that we can avoid steroids, which would have been the next treatment option for Jacob. I am optimistic tonight.

And since we have a holiday around the corner,

Happy Thanksgiving to you all,

Maria.

NOVEMBER IS EPILEPSY AWARENESS MONTH

Today's blog post is on Miracles for Mito's website:

http://www.miraclesformito.org/default.aspx

If things go according to plan, Jacob will be moved up to the floor from the ICU today! Fingers and toes crossed!

Happy Monday! - Maria.

THE PLAN

It's Friday evening in the PICU. We're embarking on another weekend in here. If everything goes according to plan, we are going up to the floor on Monday. Jacob is definitely stable and more alert as he is now receiving adequate fluids and nutrition.

The good news is that we don't see any seizures. We're continuing with epi-743, and on Wednesday Jacob will have an EEG to see if his subclinical seizures have changed. I am a both nervous and excited to see the results from that.

This tummy monster that has taken over Jacob's life is no fun! For the first time since Jacob was an infant we're dealing with GI. It is never a clear plan, but more of a "trial and error" approach. I guess you could say that is how neurology works too, but we just have such a strong decision-maker of neurologist on our team, so I never feel that I am lacking a plan with her. The head of the PICU confirmed my frustration. This is often how it is to work with GI. There are so many reasons why you could have diarrhea, and each patient might respond differently. After further tests, GI is now not sure if we're dealing with a viral or chronic colitis or Inflammatory Bowel Disease. They are all on the table!

I never thought that the first question I would have in the morning is what the stool output has been over night or where we are with stool output throughout the day. My first thought in the morning is hoping for less poop! It has become an obsession of mine...So, I am glad to report that we have had LESS stool in the last 2 days! It could be a happy blip, so his stool output continues to be monitored around the clock. We're hoping that it means that the anti inflammatory drugs and antibiotics are working.

Jacob is today receiving all his nutrition via IV. We were told today that his stool needs to decrease 2/3 from where he is today for us to consider feeds again, so we have still an upward battle with the tummy monster!

We had a care conference this afternoon to discuss plan for Jacob, and how we can safely transition him from the PICU to the floor after he crashed on the floor last week. I am amazed with the medical team Jacob has. It was a great meeting, and I feel really good that we have the weekend in the PICU, and then if everything looks stable, he will be moved up to the floor on Monday. We're doing some changes to his stool replacement this weekend that should help his care on the floor to not be as intense as it is now. Jacob is one lucky boy to have Dr. E. & Dr C. They are such strong supporters of him, and they always set the tone for his care.

Tonight, Jacob and I are watching a Charlie Brown Thanksgiving in bed. Tomorrow, sis will come and visit with Jacob. In the middle of no clear answer of how much longer we will be here, I am tonight going to celebrate the progress we have seen over the last two days.

Have a great weekend everyone! Thanks for everyone's continuous support, food, goodies, messages & visits! We can't thank you all enough.

Much love,
Maria.

THE SNOWBALL

We're on week 3 in the hospital now, two stays in the PICU, and one stay on the floor. We have no idea on how long this stay will be at this point. One day at a time...

This hospital stays reminds me of that little snowball going down the hill and for each roll turning bigger and bigger and bigger. You put that snowball in motion, and there is simply no stop. Or as a friend of mine said yesterday "it is as if you bring in your used car to the shop, and suddenly there are all these things that need to be fixed that you had no idea about". I don't want to compare Jacob with a used car or a snowball, but I think you get the jist of what is going on.

The nasty seizures we originally came in for are now controlled. We have over the last couple of weeks done several medication increases, and we also tried IVIG. Something in that mix actually worked, and got Jacob to a much better place when it comes to his seizure control.

Jacob has been spared GI issues his whole life, if I disregard that little feeding button on his tummy. Not a little issue maybe, but something that we're so used to these days that I wouldn't even think twice about his g-tube. He tolerates his feeds great, and with the help of fiber and banana puree his tummy has been in good shape...until now...

The colon is very infected. There was talk about chronic colitis. Yesterday, they started talking about inflammatory bowel disease. We had so hoped that this would be a viral infection attacking the colon, but it seems like Jacob will add yet a diagnosis to his long list of medical complications. They have started him on anti-inflammatory drugs. We have to give them a couple of days. If they don't do the job, we might have to treat it with steroids. Jacob has had excessive diarrhea for 2 weeks now, and today was unfortunately no better. Since he is loosing so much stool, they had to bump up the stool replacement even further today. This is also what keeps us in the ICU. On the floor the nurse with multiple patients can't keep up with Jacob's stool output and replacing it with the right amount of fluids. Knowing what we do today, I can see that Jacob has probably been in quite a lot of pain over the last 2 weeks. It might even have triggered all the seizures we noticed without no clear explanation. His lethargy and sleepiness, fevers, and high heart rate can all be explained by IBD.

Jacob is despite this extreme diarrhea in good spirits. I think he finally feels better, since they are so on top of his nutrional intake now. I am sure he was dehydrated and malnutrioned last week, especially when we saw the long list of nutrional values being low in Jacob.

We are glad that we today have an answer to what is going on in Jacob's gut, but very sad to add this diagnosis to Jacob's already too long list of medical diagnoses. Nutrional intake is going to be a high priority for Jacob moving forward. We might have to change formula and add quite a bit of supplements to his diet. There is also a suspicion that he has a fat malabsorption after his Vitamin K deficiency on Friday. If that is the case, he will be put on a specific vitamin supplement for that as well. Dr. C. is also not sure if this is related to his mitochondrial disease or not. There is not a direct link between IBD and mitochodnrial disease, but a few cases reported.

Dr. E is back in town again. We're happy to have her involved, since she is going to be our key player for helping us manage this together with GI. But before we can even go there, diarrhea has to stop. It is really disencouraging to not see any improvements, if anything today was worse.

On a postive note, we're starting epi tomorrow morning! We are excited to finally get the ball rolling on that one.

With Thanksgiving only a week away, we're pretty sure we will spend this year's holiday in the hospital. It is not what we want, but we'll take Thanksgiving to where Jacob will be :-

Much love,
Maria.

A QUIETER DAY

Today has been a quieter day. Quiet is good in the PICU. Quiet means a break from all the acute illness and unanswered questions we have dealt with in the last week or so. As I woke up yesterday and found out about Jacob's blood coagulation issue, I got an enormous knot of worry in my whole tummy. It felt as the knot just grew bigger and bigger, and it took over my whole body. It was literally hard to breathe. I had decided to go for a run during the shift change, and the nurse encouraged me to still do it, since they were waiting for his blood plasma and Vitamin K to come up.

As I was running, I felt as I was running to survive. I knew that maybe this was it for Jacob. Would Jacob make it through the day? As I was running, the knot didn't necessarily go away, but it didn't grew bigger. At least not after I had been running stairs for 10 minutes after my run. My mind got a little break because I had to focus on my own breathing. That can sometimes be the absolute best medicine in the middle of a crisis.

As we were waiting for rounds, the knot grew bigger again. I was supposed to run the Family Advisory Council meeting at the hospital, and Joakim would be with Jacob. The nurse told me that I shouldn't leave Jacob, since there was simply too much going on. It was a relief that someone told me that I had to let one thing go, and completely focus on Jacob. I have such a hard time letting things go, so it was important for somebody else to tell me.

Yesterday, Jacob received blood plasma and Vitamin K due to his blood not coagulating. After hematology reviewed Jacob's labs, they do think that they have found that Jacob has a Vitamin K deficiency. They told us today that we should be very happy that it was discovered, since he could have bleed out...The cause was his excessive diarrhea, but he will from now on always take Vitamin K daily. They also want to do further testing on how he absorb fat, and if he has issues with that, more vitamins in addition to K will be added to his diet. There is a specific vitamin cocktail that cystic fibrosis patients are on that might be in Jacob's future.

As Jacob's blood coagulation stabilized, the GI doctor wanted to get the colonoscopy out of the way. Something we couldn't do as long as Jacob had issues with his coagulation. The scope did confirm colitis. From the scope, it looks like the colitis is either viral (something he picked up in the hospital) or chronic. If it is chronic, Jacob needs to be treated with steroids. Metabolics needs to be involved, so they want to watch Jacob over the weekend so they can get all the test results back before we go down that road. Jacob's stools have definitely improved today, but he is still spiking fevers. We were also told that when you have colitis you can be better for a day, and then be back in the old pattern of excessive diarrhea again. We're of course crossing fingers and toes that this is a viral colitis. If that is the case, it should go away on it's own.

I have to once again say that I am super impressed with the PICU. There was nothing they didn't cover yesterday. I felt that they did everything possible to find out why Jacob was so terribly sick. They checked Jacob's liver, heart, and brain yesterday to make sure that nothing had changed, which is always a possibility with mitochondrial disease. After blood tests, an echo, and a brain CT scan, we could give out a big sigh of relief. Everything was unchanged.

That tooth that got stuck in Jacob's tummy is still a little mystery to Infectious Disease, so they ordered an ultrasound of his tummy. We don't have a final read of the ultrasound yet. On the CT scan of his tummy, they also saw a cyst! The ultrasound did confirm that this cyst is benign, and that Jacob has had it since birth. Whew!

Jacob also got a PICC line yesterday. They needed more access than the medi-port, so he needed another central line. It was really hard to put in the PICC line, and the nurse who did it unfortunately made the recommendation to never do a PICC line on Jacob again. His veins are as small as an infant. This is not great news for us, since Jacob has needed a PICC before when he has been really sick. There are other alternatives, but a PICC would be preferred. The nurse was afraid that Jacob had more blood clots as well, so an ultrasound was ordered to check for that today too. Luckily, no more blood clots.

It sure is an emotional roller coaster we're on. In 24 hours, Jacob was suddenly the attention of the ICU Team, Infectious Disease, neurology, cardiology, the liver team, hematology, GI, metabolics, special care - and possibly having a cyst in his tummy...as we ran into Jacob's pulmonologist, he was asking if he needed to be involved, and we thanked him, but said no :-

Today, Jacob has been more awake. He is not needing the bi-pap during the day any longer. He only took one nap all day long. We have been reading books and cuddled together. I don't think he is completely out of the woods just yet. There are just so many things going on, but I do feel that we are in a much better place tonight than 48 hours ago and 24 hours ago.

Jacob is such a resilient kid. Everything his body has gone through, and he gave me a half smile tonight. He is my own hero. The nurse today also commented on Sarah, which made me such a proud mama. She said that she very seldom sees siblings so comfortable around their sick siblings. I don't even think about it any longer. When Sarah is in the room, the bed is hers. She'll find herself a spot next to Jacob, and she knows what she can and can't mess with. She will cuddle and talk to him, and it is the most natural thing to her in the whole world. That is why she is here, to see her brother.

Tonight, I am hoping that we can continue to see improvements with Jacob's colitis, so we can go back to why we originally came here. Seizures are much better controlled today than when we got in here two weeks ago, but we still want to start on epi, and see if that can help Jacob's overall seizure control.

And to end the night, I today found out why my mom's family moved to Sweden. My grand pa had colitis in Finland during the second world war. He could not be treated for it in Finland, and therefore moved to Sweden with his family where he could get treatment and get well again! That is something I never knew about until today. I told Jacob the story as he was falling asleep.

Thanks to all of you who continue to check in on Jacob at all times. We never feel alone.

Much love,
Maria.

A VERY SICK BOY

Yesterday they found out that Jacob has colitis. He has a very nasty inflammation of his colon. That would explain the blood in his stool. What noone could find out is the cause for his colitis....Everybody was scratching their heads...Everything continues to come back negative.

At the same time, Jacob started to have even more excessive diarrhea. He was loosing more fluids than he was taking in. He was pale, mottled, and his breathing got labored. His heart rate was high, and his blood pressures were creeping up. He was desating as I was suctioning him. Many signs that Jacob wasn't doing well. The attending doctor said that "we were falling behind". Before we knew it Rapid Response Team was called. This is like an internal 911 call in the hospital. A complete medical team comes and does a full assessment. This has happened to Jacob before, so I know how it is when you suddenly have a room full of people all trying to figure out what to do with your child. It was decided that it was time to move Jacob down to the PICU once again.

My friend Karen was visiting with me all this time. As I was watching over Jacob, she literally packed up our hospital room of 2 weeks in minutes! She got everything packed and ready to go, and I could stay with our boy.

As we came down to the PICU, Jacob was put on bi-pap to help him to breathe. He was also receiving fluids, albumien, put on two antibiotics again, and I am probably forgetting something.

Dr. C. came by last night, and we talked about how to move forward with epi, and that we might have to wait a little bit for Jacob to stabilize with his colitis and also make sure that his fluid intake stabilizes. One of the things that you have to test before starting on this medication is your coagulation.

This morning as I woke up, I was told that his blood coagulation was out of whack, and that he needed blood plasma and Vitamin K. Yet another step in the wrong direction...We now have the PICU Team, Neurology, Metabolics, GI, Infectious Disease, and Hemoc involved to try to figure out what is going on with Jacob. Noone knows. That in itself is my biggest worry. If they don't know what it is, they can't treat it. He is also getting a PICC line today, since they need more access than the medi-port.

We have been here before with Jacob. Not exactly the same issues, but that place of complete unknown. We simply don't know what it is that is making Jacob so very sick right now. That thought is with me all the time. It doesn't even leave me when it is time to sleep. Seizures have moved down on my worry list as we're now trying to figure out why and where he is bleeding from, and why he is so sick.

Thanks again for everyone's enormous outpour of support. It is amazing. We never feel alone on this journey. If I don't get back to you all right away, know that we're busy watching over our boy.

Much love,

Maria.