JACOB DID GREAT!

Jacob had his big day today. We knew he was going to need a breathing tube during the endoscopy due to the anesthesia. The big worry was how easy it would be to take out the breathing tube again. Everyone was relieved that Jacob was breathing on his own right away after the procedure. Big sigh of relief. He has been on bi-pap all day to give him a little bit of a break, and he hasn't been too excited about that.

The anesthesia was carefully picked for Jacob, and a short-lived drug was used. He was already awake when we met him in the PICU, and has not taking a snoozer all day. This is the boy who once slept 16 (!) hours straight after anesthesia.

Jacob has been a little uncomfortable throughout the day. We had some seizures early on, but nothing his regularly scheduled meds throughout the day couldn't take care of. He has not peed on his own all day, which is usually a sign of pain for Jacob. He has also had more oral secretions than normal, but overall a pretty uneventful day in the PICU.

For the endoscopy, it did confirm that Jacob has ulcerative colitis. I can see the inflammation of his colon just looking at the pictures taken from the endoscopy. Jacob might have something going on with his small intestine too, but his GI doc didn't want to go there until we have the biopsy back tomorrow. Monday, we are sitting down with his GI doc to come up with a long-term plan of care.

So, the big question is if we will spend Thanksgiving at home or here? His GI doc said it is a 50/50 chance today. Jacob's attending on the floor will try to use his super powers to get us home in time for the holiday. He doesn't think any kid should spend two consecutive Thanksgivings in the hospital. As always we know our boy is in charge. Wherever he decides to be, we'll bring the turkey!

I wanted to end the post with one of our happy moments this week. Jacob got a visit from his best friend Selina. Jacob forgot about his colitis and being in the hospital for one evening as he got to do crafts and hang out with one of his favorite people in this world. Having Miss S visiting brought me back to this time last year when Jacob was fighting colitis for the first time. Miss S and her sister had no problem crawling up in the hospital bed for some craft time. It still gives me goose bumps thinking back on that special day A Special visitor. Jacob sure is very lucky to have Miss S in his life. She is a special soul.

 

I will sleep good tonight. I can't deny that I have been worried about how today would go. To see my boy peacefully sleep in the PICU tonight makes me incredibly thankful. That is honestly all I need for Thanksgiving.

Thanks for all your support always.

Love, Maria.

BIG DAY AHEAD OF JACOB

After a long busy day yesterday, I went to bed and the word that stuck with me was kindness. It was one of those days when Jacob and I started the day at 4 am, followed by breakfast with a friend, then off to work for a couple of hours, back to the hospital for the care conference, then back in traffic to go and pick up Sarah from her friend's house, squeezed in grocery shopping before dinner. The evening was spent cleaning up a little around the house, doing laundry, get Sarah to bed, answer some e-mails, and then some more work to top off the day. As I crawled in to bed, I realized that in the middle of this crazy busy life I am constantly surrounded by kindness. Friends checking in to see how we're doing all day long, Jacob receiving a new purple poop monster, breakfast, lunch, and dinner delivered to us. Sarah decorating two little Christmas trees at night, one for her and one for Jacob, all delivered in a big Christmas bag from another friend.

The kindness made me feel better about the fact that Jacob will need an endoscopy tomorrow or Friday. We had Jacob's team of GI, primary care, neurology, and metabolic at the table. The attending of the week is the hospital's Chief Quality Officer who also knows us well. Everyone was in favor of Jacob having an endoscopy despite the risk of anesthesia. Noone takes ulcerative colitis lightly. The fact that a full endoscopy has never been performed on Jacob despite multiple flare ups and now two involved hospital stays makes it hard to manage his disease. Jacob is now at a stage where he needs stronger drugs to manage his colitis, none of them come without nasty side effects. Jacob's GI doctor doesn't want to just throw these drugs at Jacob without knowing which one he actually needs and not. It was great to see how they all were thinking about everything from preparing Jacob for the procedure to who would be the best anesthesiologist, and a plan for how to safely do an endoscopy on Jacob. It gives me the chills to even think about the word anesthesia and Jacob in the same sentence. I loved that the CQO picked up on my fear, and he who lives and breathes patient safety at this hospital, felt that Jacob will be doing fine. I sure hope he is right...

We're still waiting to hear if the procedure can happen tomorrow or Friday. We are all hoping it will happen tomorrow, so we are one step closer to treating Jacob's colitis properly and hopefully also a step closer to coming home. Thanksgiving is exactly a week away. We would love to spend that holiday from home this year. If not, Sarah has one request this year. She wants to sleep in the hospital that night, if Jacob is here. She always gives me good perspective.

Keep our boy in your thoughts and prayers in the coming day or two!

Love, Maria.

DECISIONS, DECISIONS...

It is Monday night on the 9th floor. I am eating dinner from Panera Bread, and watching the Voice with Jacob.

Jacob's colitis is furious. I don't know any better word to describe it. The stool has its' own life and will, and Jacob's body has no other way than letting it flow. Colitis is not without pain. It hurts every time he has a bowel movement, and you can see on Jacob that overall he is not feeling great. I had a two hours window this afternoon where I didn't do anything but helping the nurse to change his diapers and at the same time giving him a bed bath in between diapers. The stress on Jacob's body is evident.

Jacob's myoclonus is also acting up. If you put your finger in Jacob's hand, you will feel his rhythmic myoclonus at all times. Right now, his myoclonus has taken over his face, especially his mouth, cheeks and eye lids. I can only imagine how it would feel to have your face rhythmically move at all times, and you have no control over it. For Jacob, it also makes it so much harder for him to handle his secretions when his face is constantly moving. After talking to Dr. C., we do think his myoclonus is acting up because he is just not feeling great. It could also be something we see due to his seizure medication Phenobarbital finally being at an all time low level for Jacob. We have drastically decreased that medication over the last week due to very high levels in his blood. We are tonight exploring different options to see how we can get his myoclonus under control.

Tomorrow afternoon, it is time for a care conference. The big discussion on the table is endoscopy or not. I had to find the post AT A CROSSROAD from last year when we were gearing up for a care conference discussing endoscopy, broviac and TPN. Care conferences are a great forum to get everyone on the same page. Care conferences are at the same time exhausting. You would not call all Jacob's core doctors into a room, if you didn't have a big topic to discuss. I do feel better today than a year ago about our discussion tomorrow, but I also know that there is no straight-forward decision. We are going to have to weigh risks and benefits, and all get on the same page. I know we have our two strong women Dr. E and Dr. C at the table with us as well as Jacob's GI doctor. It is such a difference that Jacob now has a GI doc who has followed him for a year vs. last year when we had to deal with a new GI attending every Monday morning.

So as we go into the care conference to make decisions tomorrow, we hope we can find clarity to do what is best for our boy once again.

Love from the 9th floor,

Jacob & Maria.

GEARING UP FOR YET ANOTHER HOSPITAL STAY

It is Saturday evening on the 9th floor of Children's Hospital. Jacob is getting his respiratory treatments for the night, and his nurse is preparing his evening medications. I have settled in the armchair with a cup of tea and pretzels. I am listening to Malena Ernman's new Christmas CD "I decembertid" on Spotify.

We landed in the ER mid day today due to Jacob's colitis acting up over the last couple of days. Yesterday was a long day of countless diapers, stool replacement, and dirty laundry. GI also started Jacob back on steroids last night. We were hoping with the steroids on board, we could make it at home until Monday when Jacob would see his GI doc. This morning we woke up to stool mixed with blood. By 9 am Jacob had already pooped out 0,5 liter of stool. We suspected Jacob's tummy needing a break from his formula. IV fluids is one thing we don't have at home.

This stay sure brings back memories from last year. Jacob spent the whole month of November last year with colitis in the hospital. We sure hope this will be a quicker stay. We are definitely ahead of the game knowing what we are dealing with, we have a good stool replacement plan in place, and steroids are on board. What we don't know is how long it is going to take for Jacob's gut to tolerate feeds again, and how furious his colitis will be this time. Patience is in my future.

This is Jacob's 4th hospital stay in 2,5 months time. It is not a good track record, so I am gearing up for another stay at Children's. This is what I have to remind myself of.

1. Stay calm and carry on. A hospital stay always means operating on little to no sleep and being an advocate for Jacob around the clock. There are so many little details to Jacob's care we have to relay to the medical team changing every 12 hours. Being "on" around the clock can be very tiring and sometimes frustrating especially when you have done it for days and weeks. I will have to blow off steam when my cup is full. Tonight during shift change, the new day nurse gave report and said that Jacob doesn't move and he doesn't do much. It hurt a little bit to see how she views Jacob, but then I had to remind myself that she is not in my house when we do craft days with his friends and she doesn't know that Jacob's plan for the day was to go for a bike ride with his friend. Stay calm and carry on. Don't take it personal.

2. Don't sweat the small stuff. It is easy to get stressed about work and other things you are supposed to take care of when you are suddenly inpatient. With the number of times we are inpatient, we just can't put Sarah's and our lives on hold unfortunately. Work still needs to happen, homework and volleyball practices have to happen. I try to balance getting it done with giving myself just a little slack knowing my number one priority is to get our boy home again.

3. Accept a helping hand. It takes a village. It is so true, especially when we are living between the hospital and home. I have learned over the years to say yes to help. I know I won't have time to cook or go to the grocery store. If someone wants to do something fun with Sarah, it will be a great distraction for her while I am with Jacob. Coffee, chocolate, and dear friends can make all the difference in the world. They can turn a not so good day to a better day in no time.

4. Take care of yourself. I know what I need to do to stay "mentally strong" when Jacob is fighting. I know that an early morning run around the hospital will put me in the right mode for the day, I know taking a shower no matter how tired I am will make me feel better, drinking my morning coffee is absolutely crucial. Joakim and I always take turns sleeping in the hospital, and the night at home is sacred. There is no other time I appreciate my own bed as much as when I can trade it for the hospital couch. The night at home also gives me a chance to reconnect with Sarah, and that is probably the one thing that charges my batteries more than anything.

5. Take care of my boy. As the medical team is figuring out what is going on with our boy, and Jacob is fighting off another virus, seizures, or another flare up of colitis, my boy is my number one priority. I will lay with him in bed for hours, I will read to him, we will watch movies together or listen to music. Sometimes I just tell him stories. I will make sure to give him his bed bath, and make sure I give his stiff body a massage. I will get him up in his wheel chair as soon as he has the strength to sit up again no matter how many cords are attached to him. The PICU staff know that about me :- And for the ones who want to get to know Jacob outside the hospital walls just a little bit, I will tell them about Jacob's friends, Jacob already being deep into Christmas crafts, him actually being a pretty bossy boy as soon as he is in control of his talker, him loving a date at Starbuck's, and Jacob recently riding a bike for the first time. For the inpatient medical staff who always see Jacob at his worst, I think it is important for them to also see the other sides of his life. For them to understand why we want Jacob to keep fighting the good fight.

What do you do to gear up for a hospital stay?

Love, Maria.

WHAT'S UP WITH THE TURBO LIVER?

After the last scary hospital stay, we didn't really get a lot of answers from hematology about what is going on with Jacob's blood. His blood values stabilized after about a week, and we might never know for sure if it was caused by some of his medications or the infection he was fighting or both. We will work with Jacob's specialty doctors to decide what to do with the medications he is on which can cause issues in his white blood count. Jacob's hematologist is not worried about his bone marrow, which we are very happy about.

We also don't know why Jacob suddenly couldn't metabolize one of his seizure medications, which made him incredibly sedated. All his current and recent medications were cross checked with the seizure medication. Jacob's liver function has been closely monitored since we got home. We have also been checking the level of this particular seizure medication in his blood weekly.

We were doing well for two weeks. This week the seizure medication was at an incredibly high level in Jacob's blood once again. You and I would be in a coma. Jacob not so much. He was still breathing well, and going on with his day, but he was definitely more sleepy. He was in a stage between sleeping and awake for hours. I hate to see him that way.

Over the last couple of days we have started to drastically reduce the seizure medication. We need to find the balance between lowering the level in his blood as quickly as possible with keeping seizures and withdrawal at bay. With the help of our nurses and Dr. C. we have been able to manage this from home. It might mean a trip or two a day to Children's before the sun is up, and busy days at home and on the phone with doctors, but it is so much better than being inpatient.

The big question is why this is happening. The answer is we don't know. It could be a medication interfering with this seizure medication, it could be a medication taking away that was stimulating his liver. The problem is that we haven't really made any medication changes in a very long time. The bigger question is if there has been a change in his liver's ability to process this medication for some reason. Dr. C. has always called Jacob's liver the Turbo Liver. She has been amazed how his liver can spit out seizure medications like no other kid. When other kids are sleepy and sedated after high doses of seizure medications or strong rescue medications, it has never even faced Jacob. When kids need to be intubated being on strong continuous IV seizure medications in the PICU, Jacob is breathing on his own.

We are all worried if his liver function is now changing and what it means to Jacob and his overall mitochondrial disease. We are looking for answers where there are no obvious ones. In the meantime, we will continue to manage labs and medication changes on a daily basis, and hoping that it won't get out of control. I will say that I am slightly optimistic that we will have better labs in the morning, since Jacob had a pretty good day today, and is more alert again.

I wanted to end the post with Jacob showing off his new Mito hat made by another Mito mom. Jacob is ready for colder temperatures :-

I am hoping I can report some better news next time!

Love, Maria.

THE TARGET ZERO FAMILY

In the spring, Children's launched a large training effort for all internal staff around preventable harm. The campaign is called Target Zero. The target being zero patient safety errors. It will take 18 months to train all staff. The Chief Quality Officer at Children's knows we would never leave Jacob completely in the care of the hospital staff. One of us will always stay with Jacob. He asked us if we were willing to share what patient safety means to us, and the fact that we never ever leave Jacob alone in the hospital. Children's interviewed us and created a video in the spring. It was not a hard topic for us to talk to considering how much time we have spent at the hospital.

As the training launched in the spring, we started to hear that staff loved the video. We talk about a medication error that happened to Jacob years back over Christmas, and the side effects Jacob endured. Jacob was sitting in between us throughout the whole video, so there was no question who the kid in question was.

By now, a majority of the staff at Children's has gone through the training and watched the video. Having spent four weeks at Children's in the last two months, we started to feel the impact of the video ourselves. We have become the Target Zero family.

"I saw you on the video. It was heartbreaking to hear what your family has gone through. I was ashamed of the harm that has happened to your son."

"Do you want to double-check Jacob's medications?" I know you have had medication errors happening to your son."

"I am going to give you the best care possible. I don't want anything to happen to your son again".

"I hope our team wasn't part of anything of the wrong doing".

We have become the face of patient safety to many. Due to Jacob being quite the frequent flyer, there is often somebody in the training room who actually knows us. They know that despite errors, we are highly committed to Children's. They know we always want to partner with Jacob's medical team, and make the hospital a better place for families.

During our last hospital stays, several nurses and doctors wanted to continue the safety discussion with us. We explained why we feel we as a family can add a layer of safety to Jacob by simply being in the hospital room at any given time. We are not the medical experts, but we are the experts on Jacob. We know what an elevated heart rate means for our boy, we know what can happen when Jacob spikes a fever, we know what it means when Jacob hasn't peed for a certain period of time, we know what is normal noisy breathing versus not, we know what is a seizure and what is not, and the list goes on and on. Jacob is often called a complex medical puzzle or a moving target by the attending docs. We feel if we can add our knowledge to the picture, Jacob will be safer. That is why we would never ever leave Jacob alone at Children's. It is not that we don't trust the staff, it is the fact that Jacob is too important to us to be the victim of a preventable error.

So as we continue to partner with the hospital, the goal is to make every family the Target Zero family.

Cheers! - Maria.

BATMAN AND BIKING

Jacob has been a busy boy, since he came home on Tuesday night. First of all, we are still home! Jacob looks pretty good, and his white blood count was finally normal on Thursday. It was such a relief. I guess I didn't realize how stressful this last hospital stay truly was until I could give out a big sigh of relief. These blood labs are stressing me out.

Jacob hasn't wasted any time, since he got home. He got his own talker this week. We call it his Batman talker, since it is slick and black and yellow. The talker helps Jacob to get out the super hero in him. We are truly amazed with how much Jacob is able to communicate with us. He is definitely a little bossy, but I'll take it any day! As he got his own talker, we were five (!) women in the room, and it got too loud for Jacob to hear his choices. He immediately told us "people, please be quiet". He then told us he wanted to hang with daddy, the only one not home at the time. We also love that Jacob is able to tell us how he feels. This has been our biggest struggle all these years to know how Jacob truly feels. We now know that he actually told us about his tummy and the ulcer on his butt before we even knew he was sick.

We have had a warm beautiful fall weekend, and Jacob didn't waste any time inside. He was working hard on painting his pumpkins this weekend.

Today turned into a very special day for all of us. Our dear friend Lorene brought her son's bike for Jacob to try. I think the video talks for itself. It is only 20 seconds, and well worth your time :-

It was a beautiful moment with Jacob, and we know what Santa will bring Jacob for Christmas. It was both wonderful and heartbreaking when Jacob kept telling us that he needed to go for a bike ride tonight. I had to keep telling him that the bike belongs to his buddy Robert. It was the first time Jacob has truly expressed a need for something, and I couldn't give it to him. It was both wonderful and bittersweet.

Tomorrow, we are back at Children's. It is time to meet with hematology, and come up with a plan for Jacob's blood issues. The reason Jacob has issues with his white blood cells is most likely due to long-term use of medications, so we are going to have to look at the most harmful medications for bone marrow issues and see if we can come up with a plan. We know it will involve both GI and neurology as it stands now.

Wish us luck, and hopefully Jacob can soon ride his own bike!

Love, Maria.