EXPLORING

We're often wondering what is going on inside Jacob's head. What is he thinking? What does he feel? What does he want to tell us? Sarah loves when I am Jacob's voice. Our Jacob loves to be with his sister. He also loves to "boss her around". He always tells her what he wants her to do. There is also nothing that can stop our Jacob. He can do anything, and he is up for anything! That is our Jacob.

With the help of Jacob's speech therapist, we're exploring ways Jacob can better communicate with us. The goal is to give Jacob some sort of communication device that he can independently use to communicate with us. It is a big goal for Jacob, and it might take us some time to get there, but it is so worth it! We have for a long time used Jacob's eye gaze to make choices, but we're starting to think that a button might be better for Jacob to indicate what he wants. We have also found out that he likes to make choices with his foot rather than his hand! As we're working with Jacob's speech therapist, we will also consult with a speech augmentation specialist at Children's and through the school district. We're hoping they can guide us further!

Jacob loves his switch toys! He loves to be in control of his bunny and cat, and have them move when he wants them to move! It is so fun to see him smiling and being so proud!

Another big goal for Jacob has been to do some weight bearing again. Our boy has not put any weight on his legs for 3 whole years! That is how long it has been since Jacob was in a stander or walker. The thinking is that weight bearing together with Pamidronate treatments are the two best things to improve his osteoporosis. We received Jacob's supine stander months ago. The first time we put him in his stander, we thought he would break a leg! He simply didn't have enough support for his legs. We ordered new AFOs for Jacob, and then the long hospital stay and the holidays got in the way, but yesterday everything came together. Jacob got in his stander for the first time! This is quite the project! We're starting Jacob in the stander for 15 minutes each day. It doesn't sound like a big deal. Well, to get Jacob ready for the stander definitely takes some time. We also need to be two people to get Jacob in and out of the stander, since he needs to be supported both in his upper and lower body as we're strapping him in. I think that Gemma almost got more of an exercise than Jacob as we were embarking on Jacob's 15 minutes of standing today!

We have also started cranial sacral therapy for Jacob. With a soft touch his therapist releases restrictions in the soft tissues that surround the central nervous system. She is getting Jacob's spinal fluid to flow better. We have noticed that Jacob is more alert after each session, and it is also supposed to increase his immune system.

Jacob's colitis is in remission. To be able to write that sentence makes me so very happy and relieved. There were times before Christmas that I thought we would never ever get his colitis fully under control. Weighing each diaper and calculating fluid replacement at home. Accidents happening each day. Today, Jacob is in remission. The one thing we know in living with colitis is that the disease will flare up again. Noone knows when, and sometimes that just makes me even more thankful for where we are right now. We're still weaning Jacob off steroids, which will take another 1,5 months before we're fully off.

The same goes for Jacob's seizures. Jacob has no clinical seizures at this time. We are not using rescue medications daily or multiple times a day. It is a good feeling for us all. We do feel that Jacob is more obstructive right now, so we will discuss the bi-pap with his pulmonologist this coming week. It might be that we need to introduce the bi-pap at night again to make Jacob's overall breathing better. We have had a great 1,5 years without the bi-pap, but it might be time to give Jacob some support at night again.

Going into the new year and a flu season that is worse than in many years, we're hoping that Jacob can continue to stay at home healthy, so he can continue to explore!

Love,
Maria.

WINTER BREAK

Things have been quiet over here, no updates for a while. I decided to take a well deserved break from my laptop that often accompanies me at night in front of the TV. There is always something to do at work, emails to answer, or updates to read. So, this winter break I intentionally did less of that. Instead, I watched movies without banging the keyboard at the same time. We played board games with the kids. I finished a book. I organized and did things around the house. I practiced yoga. We had friends over for dinner. We were on a break.

Christmas already feels far away, even if my house is still breathing Christmas (and will for a bit longer)! After a filled Christmas Eve of Swedish food and friends, it was time to switch gear to American traditions. Unfortunately, Joakim got terribly sick with the stomach flu on Christmas Day, so we didn't see him all day! Thanks to Sarah, our Christmas Day breakfast and gift opening turned into a great morning and day.

After Christmas, it was my turn to get the nasty bug! Luckily, Jacob and Sarah were both spared this time.

After we finally got the bug out of the house, we started to enjoy the break again. We have had many lazy days around the house, we often didn't even get out of our PJs, watched movies, and played games. I love slowing down!

 

 

And we also ventured out in the cold weather once in a while!

 

The last day of the year, we celebrated with dear friends as always. It is always a great way to end the year.

 

 



Jacob also got to have some special time with his friend Selina this break.

Check out the hat that she and her sister hand made with the help of their grandma. Pretty cool! This is how tired our boy was after his play date!

Next week, we are back to our normal routine again of work, school, after school activities, therapies, and doctor's appointments. So before the wheels start spining again, my new year's resolution is to slow down this year. To not take on every project coming my way, and to simply have more time to "be" a little bit more. I am thinking this winter break got me off to a good start, now I just need to make it into my daily routine. I will let you know how it goes :-

I wish you all Happiness & Health in 2013!

Love,
Maria.

MERRY CHRISTMAS!

It's me. I just wanted to wish everyone a very Merry Christmas!

It has been busy around here. The holiday preparation has been going on, and I think my mom has broken a record in number of cookies and treats she has baked this year! I helped baking Swedish saffron buns, and chocolate dipped Marzipan candy yesterday. I love when our kitchen is in complete chaos, and I am right in the middle of everything!

On Christmas Eve, our family celebrates Swedish Christmas. We have invited two dear Swedish families to share in our Swedish julbord (dinner). Mom and dad are busy preparing Swedish Christmas dishes. They are telling me this has gotten so much easier with IKEA in town. So, tomorrow it is all about our family's Swedish traditions. On Christmas Day, we shift gear to our adopted American traditions. We will have a big Christmas breakfast and open gifts together. I love that we can do both!

Santa has delivered some gifts to me early this year! I have gotten two new bean bags! I especially like my very big bean bag! Three people can fit in it! Here I am snuggling with sis.

Apart from all Christmas fun, I think it is time for a tummy update! I got to see my GI doctor this week. He is now sure that I developed ulcerative colitis or IBD (Inflammatory Bowel Disesase) because of my mom's genes. It has nothing to do with my mitochondrial disease. This conclusion comes after we now know that both my great grand pa and my aunt have the exact disease I have. It is an auto immune disease that I have lived with without showing symptoms earlier. Most likely, I caught a virus in the hospital that "triggered" the symptoms of colitis/IBD. Everyone is extremely happy that my colitis is in remission. I have basically no symptoms right now. It makes for a very happy me! We will continue to wean me off the medications that I was started on in the hospital with the hope being that I only need one medication to stay in remission. My doc especially wants to get me off my steroids, since I already have such a weak immune system. I also got to find out that GI is very much like neurology. It will be trial and error to find out what medications will work for me, and we have no idea when my disease will flare up again. Maybe just knowing that it is likely it can happen again.

Also, mom and sis came down with a GI bug on Friday. They were both out of commission. Mom knew that if I got it, it would be a free ticket to Children's for me. Miraculously, I didn't get it despite mom and Sarah always being all over me! We're thinking that we might be starting to see positive effects from my new mitochondrial drug, since this is one of the things that has been reported by other families. We Mito kids just don't get as sick when we're on epi!

Lastly, seizures are at bay. My clinical seizures are controlled. We have not used rescue seizure medications since we came home. This is the best Christmas gift in our home.

Merry Christmas to you all!

Love,

Jacob.

FILLING IN THE BLANKS

Since September, we have had three involved hospital stays. All hospital stays involved Jacob staying in the PICU for part of the hospital stays.

Jacob definitely didn't look his normal happy self while being in the hospital this fall. Jacob could go in and out of seizures. Jacob was at times completely wiped out from high doses of seizure medications, and the only thing his body had energy for was some sleep. There were days when Jacob struggled with his undiagnosed colitis and the only two things he did was sleeping and seizing. There were days when Jacob's heart rate could raise through the roof from simply laying still in bed. Fevers coming and going each day. They are the moments when I am in despair. They are the moments when I honestly don't know how much longer Jacob and his body can continue fighting.

These are the times when I put on my big mama warrior hat. These are the times when I will be very clear and demanding with the medical team that something is very wrong with Jacob, and we need to figure out what it is in order to get him back to his normal self. These are the times when I tell them that I as Jacob's mom know something is really wrong with him.

Every week there is a new attending of the medical team. Every week, each specialty has a new attending. Every week it feels a little bit as starting over with Jacob's care in order to get the new team on board of what is going on with our sick boy. What we faced several times in the last couple of months is that the doctors actually think this is Jacob's baseline. They look at his chart with 40+ identified diagnoses. They take the medical information from his chart, look at Jacob in the hospital bed, and think - yes, this is how you would look like with this involved disease. They are sometimes a little too quick to assume that Jacob's mitochondrial disorder has progressed. They miss the basic question: what is Jacob's baseline?

When it comes to patient safety, this is an extremely important question. If you don't know your patient's baseline, how do you know what you should treat or not? I strongly believe that if the doctors would have taken Jacob's bloody stools more seriously and acted on them more proactively, we could have avoided a very involved PICU stay.

I have learned that one of my biggest roles as Jacob's advocate is to provide Jacob's baseline - even when the doctors are not asking for it. It helps a lot that I have an IPhone full of photos of Jacob. I very often have to pull out my phone and show photos of Jacob when he is "baseline healthy". I can see that many are surprised that Jacob goes to school, has friends, and is actively involved in all parts of his family's life.

This last hospital stay, Jacob's best friend Selina and her sister Leandra came to visit. They decorated Jacob's room and IV pole with pictures of Jacob and them playing together. They were riding on a big turtle together, they were playing outside together - in each picture Jacob is the boy in the wheel chair. He is easy to identify in each picture. Jacob also got many "get well" cards from all over the world that decorated his hospital door. The doctors who read Jacob's EEG also got to watch Jacob playing with Selina and Leandra on camera throughout the whole procedure.

Jacob had lots of visitors this time around, often several visits a day. The room was never empty. As Jacob's stay progressed, he had more and more stuffed animals in his bed to the point that they hardly fit in his bed at the end! Sarah came many times to see her brother. Every time, she crawls up in bed with him, kisses him, talks to him, and decides what they will do together. They might watch a movie together, they might read together, they might do a craft together, or Sarah might simply work on her homework as Jacob is watching her. No matter what, she is very comfortable interacting with her brother despite all medical equipment.

Jacob's home nurses came to visit multiple times. The nurses in the PICU and on the floor got to meet Jacob's home nurses, and shared insights and ideas.

All of these things are so very important for his medical team to understand and see. Don't take me wrong, I know that Jacob is very medically involved. It has been pointed out to me for over 6 years now. No medical decision around Jacob is easy or straight-forward. But if you also understand that Jacob is a very happy boy who is surrounded by love, family, and friends, you will also understand why it is so very important for us to get Jacob home again. You will understand why mom is not giving up to find an answer when her boy is sleeping all day, or pooping blood, or seizing all day long. It is not our boy when that is happening to him. Something is really wrong, and Jacob needs every member of the medical team to think outside the box as we're filling in the blanks for his doctors and nurses.

It can be trialing and tiring to be Jacob's advocate at times. It makes me sad when the doctors truly think that I want to keep Jacob to any price if the only thing he would be doing would be sleeping and seizing. It can be frustrating when assumptions are made that simply are not true. But it can also be very enlighting when the medical team "gets" Jacob. We had this wonderful PICU nurse for example. She got to meet all of Jacob's nurses and Sarah. At one point, she stopped me and said that she is amazed by the love surrounding Jacob. She said that being a PICU nurse she very seldom gets to interact with the paitent. Instead, she cares for the family members, since her patients are most of the time non-verbal due to sedation or a breathing tube. Her communication is mainly with the family members. She loved to see photos of Jacob, and she always stopped to admire the art work on his hospital walls. One Saturday afternoon, she told me that she thinks every person has a purpose in life. After having cared for Jacob and seeing all the people surrounding him, she was sure that Jacob is here for so many of us - giving us a bigger purpose. She teared up a little as she talked about our boy, who she had just met a few days earlier.

Yesterday, Jacob was such a show off! He did high fives for his new physical therapist over and over again. He also sat up with her, and had the best head control I have seen in a very long time. His head was so heavy, but he just kept pushing it up! He also decided to play the drums on his IPad, and you can all see how proud he is! I think I will keep this little video in case I have to show Jacob's baseline some time soon again :-

BUSY AND HAPPY!

Jacob has been home for a week! It was a little nerve wracking to bring Jacob home this time. Jacob has never been on these many medications in his entire life. Several medications can't be given with other medications, so we have had to work out quite the medication schedule. Today, we are giving medications at 14 (!) different times in 24 hours time span. One medication, we're giving at 4 am, since it is the only time we can fit it in. I am not a fan of those early mornings...

We also had to invest in a food scale and white board to start weighing Jacob's diapers and recording his stool output. We do that, so we know when we have to do stool replacements. If Jacob poops more than a certain amount in one diaper or in 24 hours, we have specific instructions on how to replace the stool.

We also came home on continuous feeds, which means Jacob is hooked up to his feeding pump around the clock. Over the last week we have worked on getting Jacob some breaks from his feeding pump a few times a day, and at the same time slowly, slowly progressing his feeds. It is going to take weeks for us to get back to his old feeding schedule.

Lastly, Jacob's already low immune system is now even more suppressed due to him being on steroids. We have started moving all his therapies to the home, and unfortunately Jacob is not allowed to go back to school until May! It makes my heart a little bit heavy, but it does make sense. We are meeting with the school district next week to see what we can do in terms of home schooling. We're going to have to rely on Sarah and Jacob's sweet friend Selina for socialization this winter! I can't think of two better people to keep him company. We're also going to have to make sure that Jacob is not exposed to any children with cold symptoms. If Jacob is exposed to the flu, he will start taking Tamiflu prophylaxis.

We are also keeping ourselves busy with several doctor's appointments and tests each week. They are monitoring Jacob's overall health very closely. We have multiple labs, which need to be run each week. We're lucky to have Gemma who can simply draw labs in the home!

So, a lot going on for sure! I am considering myself quite the time manager, but Friday I just didn't seem to be able to keep up with all of Jacob's stuff. I was so hungry from not being able to eat all day, so had to get Joakim to take a break from his work to make me a sandwich :- I didn't even make it down to the kitchen!

In the middle of our busyness, we're SO happy! There were times in the hospital when I didn't know if we ever would be able to take our boy home again. I don't think anyone of us could have imagined Jacob looking this good after a week at home. He is pretty much back to his old self - just quite a bit stiffer from laying in bed for over a month. He interacts with us and he is smiling! He is busy making his Christmas gifts for his teachers and therapists together with Gemma and sis. He is hanging out with Libby at night. Mysteriously, the stool has slowed down quite a bit, and seizures are well controlled. Jacob is happy to be home, and so are we! I am catching myself smiling and feeling warm in my whole body from simply looking at my boy.

I am ending this post with a photo of Jacob. Sarah had "hat day" at school today, so Sarah decided Jacob got to join in the fun!

Thanks again to all of you for following and supporting us during the last couple of trying months. We're hoping we can continue to report from home!

Much love,

Maria.

A STEP CLOSER TO HOME

This afternoon it was time for the dreaded care conference. I had a long list of questions for the doctors, and was ready for difficult decisions. Dr. E., Dr. C. and Jacob's pulmonologist had all come to support the right decisions for Jacob. It was great to see our core team around the table.

Each Monday, the attending for each specialty team changes. This means that every Monday we do get to see a new GI doctor. To our surprise, the attending of the week did not agree at all with doing a full endoscopy of Jacob! With Jacob advancing to his regular feeds in 24 hours time, and the stool slowly tampering down over the last 2 days, he felt that something was working, and we should not put Jacob through a procedure that could have life-threatening consequences. You could see a sigh of relief around the room. At the end of the conference, Dr. E. said that it had been the most productive care conference in a long time!

So when the big obstacle was out of the way, the next big question was "when can we go home?". Friday is the day we're shooting for. Friday! That is only 3 days away! I can't belive it...They still want to monitor Jacob over a few more days, and there is a lot of paperwork that needs to be put in place from new medications, to Jacob's experimental drug from the research pharmacy, to a whole GI plan to go home on, appointments to be scheduled across specialties, and figuring out a schedule for Jacob's all home medications and feeds, weaning plan for Jacob's steroids and other GI meds. We will also get Jacob's Pamidronate treatment (for his osteoporosis) done in the hospital this week, so we don't have to come back for that on Monday.

Jacob will stay on steroids for a bit longer. GI thinks that it might actually help him. They will slowly wean him off them, but it could mean that Jacob will be on steroids for about 2 more months. This is not great for him due to the immune suppressing side effects, but if it means we get to go home, we'll take it.

Sarah is planning Jacob's home coming party. Pizza is on the menu and she is still figuring out the movie of the night. Polar Express is high on the list. We can't wait to have Gemma and Libby back on Team Jacob. We can't wait to all sleep under the same roof again.

And for tonight, I think I might sleep ok on the hospital couch for the first time in a very long time. Jacob's GI issues are not fully under control yet. It might be something that he will battle for a long time, but it is such a difference to be able to manage it from home.

A sincere thanks from all of us to you all for supporting us throughout this challenging hospital stay. We now just need smooth sailing between now and Friday...

Much love,

Maria.

AT A CROSSROAD

It's another Sunday night at Children's Hospital. Tonight marks 4 weeks at Children's Hospital for Jacob. It has been a month with ups and downs. From uncontrolled seizures to tummy problems that I never could imagine. Stool output that is out of this world. I can today analyze stool color, consistency, and smell like never before. I have a pretty good guess, if we're looking at a diaper under 50 ml or if we're dealing with a 200+ ml diaper. I always cross my fingers that the diaper will be smaller than my guessestimate, always making sure that the nurse is not keeping any wipes in the diaper that might make it a few mls heavier :- I can by now declare that I am a stool control freak when it comes to my son!

Thanksgiving truly gave us hope. We had a wonderful holiday all four of us, and there was hope that Jacob finally had turned a corner. He had had two good days in a row, and mysteriously the poop had faded away. The steroids were doing their job! As much as Jacob is not a great candidate for steroids, we had found something that had put the output to a stop.

Or so we thought...after Jacob had a great visit with our friend Camilla and her kids on Friday, the stool came back...not one diaper, multiple ones...Yesterday was the same...with every diaper change I felt my hope sank lower and lower. The steroids were probably not working after all...Today has been a little bit better, but we're nowhere close to where we were on Thanksgiving.

On a positive note, Jacob seems to be feeling pretty well. He is up in his wheel chair multiple times each day. We read, listen to music, and do crafts. We work on his range of motion and stretches every day. He is so stiff! His physical therapist would not be happy, if she saw his hamstring stretches these days...You can loose so much flexibility from simply laying in bed for a month...Jacob is completely weaned off his bi-pap at night, and we're very close to have weaned Jacob off his oxygen during the day. If it wouldn't be for his tummy, Jacob should be home by now!

I know we're at a crossroad now. We have tried all the things GI told us we could do. We have tried multiple medications, and we have tried steroids. I am sure they will stop steroids tomorrow. If they don't see drastic results with the steroids, they can't justify keeping them on board due to the side effects.

They have started Jacob on pedialyte tonight, and we will see if he tolerates it or not. If he tolerates it, they will slowly, slowly start feeds again. The big question is still what is truly going on with Jacob's tummy? GI has wanted to do an upper gastrointestinal endoscopy for a week now. Dr. E. put her foot down. She thinks it is not safe for Jacob to be under anasthesia. When we all gather together for yet another care conference on Tuesday afternoon, we do know that the big questions will be - now what? Will it be safe to do a full endoscopy on Jacob? What are the risks? Can he safely go through that without risking his life? And if he is put under, should we put in a broviac in case he will need TPN long-term? We know the medi-port is not ideal for TPN long-term. And our big question is: how do we get Jacob home?

We are all very tired. It is such an emotional rollercoaster to go from hope to disappointment so many times in the last month. The ugly face of mitochondrial disease has popped up a few too many times. Every decision with Jacob can't just be made as with any other child. There are always multiple considerations that has to be weighed with every decision. The team has been great to involve the right people throughout this hospital stay, so that has been working great. The choices are just very limited when it comes to Jacob. I can see that even clearer as Jacob is growing up. There are simply less things to try.

And sis needs her brother back home. Joakim and I have both commented on how well Sarah seems to have taken this hospital stay despite it being the longest one we have done in three years time. She is older, more mature, which is helping her a lot in understanding what is going on. At the same time, that also makes it easier for her to put two and two together. She knows her brother hasn't been this sick in a very long time. Her first question about Jacob is if he has pooped. Yesterday evening, she simply had it. All sadness, frustration, worries came out in one go. In the middle of being exhausted myself, it is good to be able to share those feelings together.

And for Thanksgiving, both kids got Christmas PJs. Sarah was very adamant that both of them are wearing them tonight despite sleeping in two different places.

 

 

We're at a crossroad tonight with hard decisions ahead of us. I hope we make the right ones. Our biggest motivation is to bring our kiddo back home again.

Good night,

Maria.