A WALK IN THE SUNSHINE

This afternoon Sarah and I went for a walk around the hospital. Sarah was in her chatty mode. We talked about her school, she told me about a friend who is not always the nicest person to be around, she was telling me about how she was exchanging parts of her lunch with her friend, since she didn't like the new breakfast bar I had sent with her to school. From school we got to talk about her brother. I asked her how she was doing with her brother being in the hospital, since some stays are harder on us than others. She said she felt fine, since she can come and visit him when she likes. She doesn't like the bi-pap being on, since she can't cuddle as well with him. She wants to come back and see him this week, but not Friday because she has plans with her best friend. She asked if I remembered the time Jacob was in the hospital for 50 days, and she only got to see him three times? I said I will never forget that stay, since I wasn't sure I would be able to bring him home. She wondered why he was in that time. She didn't remember that he had been that sick, since she was so little back then. She talked about her classmate who lost her brother last year. She said her friend doesn't always talk about her brother anymore. She asked if I think Jacob will be in the hospital 50 days this time. We talked about how it is to loose a brother, and how we hope Jacob will be in our lives for as long as he possibly can. Sarah thought that he should stay with us until she turns 90. I said that would be fabulous, but I doubt his disease will let him. She agreed with me. I said I don't know how it is to be the sister of Jacob, and acknowledged it can't always be easy, but I also told her what a compassionate, strong woman she is turning into.

A twenty minutes walk with my girl was the absolute highlight of today with very little to no change in Jacob's health. I am amazed how far we have come, and how easy those very difficult discussions come today compared to only a few years ago. Sarah gets it all, and she can actually verbalize her and my biggest fears. We can talk about it, and I know she will be fine to go on with her day. As soon as we had finished our walk, she was ready to explore the gift shop and have dinner in Jacob's room. She sneaked in a shower in the PICU tonight, since she didn't want to miss a minute of the TV show the Voice when she got home. I asked her if she was comfortable to take a shower here, and she just told me it wasn't the first time. She did point out that the hospital towels are not great quality though. I simply love our girl to the moon and back. She brings light and laughter to any day.

Our Jacob is having a hard time getting a break. He desats on the nasal cannula, so truly needs the bi-pap around the clock right now. His stool is crazy due to him being on 2 IV antibiotics, and everyone is watching it closely for signs of colitis. He is getting all his stool replaced by IV fluids. He still has quite a cough. He has had a high heart rate for six days straight, and we're not sure what of all possible factors is causing it, but it is concerning to everyone.

I know this will be a longer stay. I have settled on "Jacob time", whatever it will take to get our boy back to his baseline once again. I brought my own cup in today, since I was tired of drinking out of paper cups. I have my egg crate mattress and own blanket for sleeping. I bought an extra bottle of contact lens solution, so I don't need to bring it back and forth every day in my overnight bag. I am moving in. We're on Jacob time now.

Love, Maria.

FULL FORCE

So from slow moving yesterday, we are now in full force. Jacob did not have a great evening and night. He was obstructive, kept desating, and had trouble breathing. This is the closest we have been to an intubation in years.

During rounds this morning, the team had completely changed their course of action. There was no more waiting and see, treatment was the word of the day. The x-ray now confirms a full blown pneumonia in addition to the aspiration we already knew about. They started Jacob on yet another IV antibiotics and also started him on steroids. IV fluids are on board again. We went up once again on his medication helping to reduce his secretions. Jacob was on bi-pap all day long. His heart is working so hard around the clock. He looked "septic" to the ICU team.

I can report Jacob is not worse tonight, so hopefully he is responding to all the different treatments we started today. I was up with Jacob most of the night once again. I am home tonight, and right now the only thing I can think of is catching up on some sleep while Joakim is taking the night shift in the ICU tonight.

Tomorrow is the start of a new week. We surely hope it will be a better one for our boy.

Thanks again for everyone cheering on our boy once again. It is very much appreciated. I hope I have some better news to report next time.

Good night, Maria.

SLOW MOVING

We are on day 4 in the PICU, and Jacob is not getting better. At times, we actually think he is looking worse. We know Jacob is going on week 3 of the rhinovirus and two consecutive hospital stays under his belt. Jacob is swimming in secretions. We deep suction Jacob as much in an hour as we typically do in a day...We need to give him Benadryl at night, which dries out his secretions, so he has a chance of getting some sleep. His heart is working extra hard around the clock. He has awful diarrhea.

I am unsettled. I know people are telling me that this strain of rhinovirus is really nasty, and has landed many kids in the PICU. But...is there anything more? Why isn't Jacob at least getting a little bit better each day? Are we missing something?

In the old days, Jacob was put on IV antibiotics any time he landed in the hospital, just in case. His colitis has had to change that treatment plan. Antibiotics are upsetting Jacob's gut, and can lead to a flare up of his colitis. Everyone is so much more cautious to start antibiotics, if it is not clear that bacteria is growing.

Tonight, the attending said it is time to start antibiotics after all. She thinks Jacob is fighting something more than the rhinovirus. His chest x-ray is unchanged, but his blood work is indicating he is fighting something more than rhinovirus, he spiked a fever last night, and his heart rate is very unsettling to everyone.

I am sincerely hoping the antibiotics will kick in, and make our boy better. No hospital stays are easy, but some are harder than others. This one is harder on me. I am exhausted. I think it is because we have had two back-to-back hospital stays. I just don't seem to be able to catch up on sleep, and there is a lot going on in our lives outside of the PICU as well. I am also an impatient soul. I want to see progress. It hurts my soul to see my kid sick in bed going on his 3rd week. With the constant deep suctioning, we honestly have to be at his bedside at all times, since it is even too much for an ICU nurse caring for two patients.

It is Saturday night in the PICU. Joakim and Sarah were here all day as well. We had thai food for dinner together. Sarah decorated Jacob's room with all his get well cards before she left. Now Jacob and I are cuddled up in bed. Jacob is watching Curious George, and I have told Jacob when he falls asleep I am going to escape to the world of "Breaking Bad". I just need a little break from the PICU!

Send positive thoughts and prayers the antibiotics will kick in, and make Jacob feel much better!

Love, Maria.

THE SNOWBALL EFFECT

I feel I am taking back years in time tonight, to the time when we truly never knew when Jacob would land in the hospital. If somebody would have told me this morning that Jacob would sleep in the PICU tonight, I would have said "no way". But that is where he is once again tonight.

He had his follow-up appointment from last week's hospital stay with Dr. E. this afternoon. I had noticed his heart rate was elevated and his work of breathing was a bit more today, but nothing alarming. I was blaming myself for not having given Jacob a dose of Tylenol before leaving home, since that decreases his heart rate. Instead I walk in to the Clinic with a child with a temperature of 101.1. I know that Dr. E. never ever takes a fever lightly in our boy. For her, fever is equal with an infection.

Suddenly everything happened at the same time. Jacob's diarrhea was out of control, secretions were non stop, his myoclonus was acting up. After suctioning, Jacob choked on his secretions, stopped breathing, and most likely aspirated IN FRONT OF DR. E! I am sure she has seen it all, but she is not comfortable with an emergency situation in her clinic. She wants it in the Emergency Room where there are resources. As I was sitting Jacob up, she grabbed for the suctioning, and we were able to stabilize him. After an episode like that, Jacob sounds if possible even worse. I know the only thing that will make his saturations go up is a good deep suctioning. Dr. E. didn't want to have any more suctioning happening in her clinic, and we got sent over to x-ray together with the respiratory therapist manager, and an oxygen tank with 3 liters of oxygen. He handed us over to another respiratory therapist, who knows Jacob very well. He was in agreement we had to deep suction right there in the waiting room of radiology. During suctioning, Jacob's oxygen dipped down to the 70s, and he required 6 liters of oxygen. Dr. E. called us to go straight to the ED after the x-ray. The snowball was going downhill.

We were greeted by a whole crew in the trauma unit. Dr. E. had rushed over to the ED with our red wagon we had left in the clinic. She knows my diet coke is close to my heart, so she grabbed my half full can and put it in the wagon, little did she know that it is an art to steer our wagon. We had a red wagon full of diet coke when she arrived :- Jacob got stable in the ED, but the snowball continued downhill. Everyone was pointing to his chest x-ray saying how "fluffy" the whole x-ray looked, and shortly IV antibiotics and fluids had been started. I just saw us being in for a very long hospital stay. Pneumonia had gotten worse....IV antibiotics on board....fever back....desating with suctioning....the snowball was still coming downhill.

So, tonight we ended our day in the PICU once again. It is the best place for Jacob to be when he is acutely ill. We have the same team as last week, so we definitely feel continuity in care. The PICU team doesn't agree with the ED's assessment of his x-ray. They don't think it looks any worse than last week, so they stopped antibiotics after one dose. They are testing him for respiratory viruses once again, since Jacob could have caught something last week in the hospital...I am sincerely hoping for a quiet night in the PICU, and that we tomorrow can get a better sense of what is going on with our boy. It just makes me so sad to see him this sick again. Early fall is typically our prime time. Jacob can go to school. Jacob can be outside with the cooler temperatures. We get to enjoy family time together before winter arrives.

Keep Jacob in your thoughts and prayers tonight.

Love, Maria.

TIME FOR AWARENESS

We’re ending Mitochondrial Awareness Week with our 3^rd annual Mito Awareness Day. Families from all parts of Colorado as well as out of State gathered at the Anchor Center for Blind Children. We had a beautiful fall Colorado day, so we could also utilize the beautiful garden of the Anchor Center. Many parents and kids came out in their Mito shirts and gladly decorated their arms with Mito tattoos. One of our Mito kids brought his new bike, and let the other kids try it out.

We put our kids to work today. We were creating a thank you video to all of our Courage Classic donors. The kids had fun coloring and making their signs for the video.  The gym and garden of the Anchor Center is a perfect place to run around. The kids explored the gym equipment, biked and ran around in the garden.

Dr. Abbie Collins talked about the Mitochondrial Diagnostic Process. She covered all the different steps in the process of finding a Mitochondrial diagnosis, and how very difficult it is to diagnose mitochondrial disease. She covered criteria for possible, probable, and definite diagnosis of a mitochondrial disease. She took time to answer questions from families wondering what would be future diagnostic tools, and what could be possible next steps for specific families. It left us parents with a deeper understanding of why it can be so very hard to get a definite diagnosis. Dr. Abbie Collins also talked about all the projects going on in the Mitochondrial Clinic at this time. Families got very excited about the upcoming Educational Symposium we are going to organize at Children’s Hospital together with the Mitochondrial Clinic. Stay tuned for more details!

 

Dr. Van Hove talked about newly diagnosed Mitochondrial Diseases in his lab, and the research papers he is ready to publish. He talked about how he selects the patients he decides to do full exome/genome sequencing on, and what criteria go into his thought process. He made us happy by letting us know that in each and every research paper he publishes, he thanks Miracles for Mito for our generous support. We are excited to get the word out about our non-profit.

It was a beautiful fall day in Colorado, the kids had a blast, and they never want to leave.  Parents hugged, and exchanged information on how their kids are doing.  In the middle of Mito, people once again found strength and hope in each other.

We are looking forward to seeing everyone early spring again. To a healthy winter for our Mito kiddos!

Love, Maria.

OUR MINI HOSPITAL

First thing first, Jacob is home! He is still pretty sick, but well enough for us to manage it from home. He has a nasty strain of the rhinovirus. The verdict is still out if Jacob has pneumonia or if the x-ray shows multiple mucus plugs. No matter what, he is treated with antibiotics, just in case.

If you follow us closely, you might have noticed that Jacob almost always ends up in the ICU (Intensive Care Unit) for a couple of days with each hospital stay.  The floor nurses and doctors get easily nervous when they realize the amount of care needed when Jacob is acutely ill. They also know how quickly Jacob can get worse. We are today very comfortable to be in the PICU. Over the years, the PICU has changed their philosophy of who is getting treated in the PICU. You don’t need to be intubated and completely sedated to get a PICU bed. Jacob is one of those kids who actually will recover quicker from a hospital stay, if he gets the 1:1 care he gets in the PICU. Ok, I am going to say something I never thought I would say, but I love the attention and care Jacob gets in the PICU when I don’t have to fear for his life (which has been the case a few times in the PICU). I know if Jacob gets dehydrated or he has a fever, it will be taken care of immediately. Not after we first have to wait for the doctor to be located, and then waiting for orders to be entered in the computer, and then for the pharmacy to actually bring up something as simple as a dose of Tylenol. On Sunday night when Jacob was spiking a fever of 104, it took the floor team 2 (!) hours to give him a dose of Tylenol. You can imagine Jacob’s overall status going downhill during 120 minutes of waiting time…I have come to love and admire the PICU staff very much. I feel safe when Jacob is in their care.

This morning it struck me that we have our own hospital at home. When we get up to the regular hospital floor, there is honestly nothing we can’t do at home to manage one of Jacob’s illnesses. All the respiratory equipment in Jacob’s hospital room from bi-pap, smart vest, cough assist, suction machine, pulse ox, and oxygen - we have it all in Jacob’s room.  He has his own mini hospital. By the time the pulmonary team rounded on Jacob, I was honestly ready to go home. I had performed his pulmonary toileting since the respiratory therapist wasn’t available, and I had given Jacob all his meds after they were scanned, I had changed his diaper multiple times, and I had started his morning feed. By the time the round came around, I told them that even if Jacob had been up on the floor for less than 24 hours, I was ready to take him home. I told them that at this point, I would rather do the care at home than in the hospital. They told me I had all the right arguments on my side, so we got discharged. Yeah!

I have gotten so used to Jacob’s room having equipment under his bed, next to his bed, in his bathroom, and in most rooms of our house. Today, I realized we have copied the equipment of a regular hospital room in our own home. It doesn’t make me sad, not any longer. It is what it is. And if anything, it will mean we can get our boy home sooner. And Jacob has not yet had a chance to celebrate Sarah’s 11^th birthday. I am hearing we are doing a movie night tomorrow evening with both kids on Jacob’s big bean bag, and that I can’t find in a hospital room at Children's!

Love, Maria.

Nope Jacob - we are NOT all done yet

In light of Mitochondrial Disease Awareness week, we are asking key people surrounding our Mito kids what they have learned about Mito. Jacob's speech therapist Brittany wrote the following blog entry, and it made me both cry and laugh this morning. Enjoy!

Hi everyone – my name is Brittany and I am Jacob’s speech-language therapist who works with him 1-2x a week helping him become more proficient with his talker.

When Maria first asked if anyone working with Jacob wanted to write on the Miracles for Mito blog I was a little hesitant. What would I say? I’ve only known Jacob for a few months and there is still so much about mitochondrial disease that I don’t know about or understand. However, after a few minutes of thinking, so many thoughts came to mind. Here is a little glimpse of my relationship with Jacob and what I do know about mitochondrial disease.   

I started working with Jacob in the beginning of June. I am a new graduate who specialized in school at working with individuals with more complex communication needs who required AAC (talkers) to communicate. I never in a million years thought I would get the opportunity to work with so many COOL kids who use talkers. Jacob, along with many of my other clients, is one of those amazing, brilliant, and extremely cool kids. When I first met Jacob I had no idea what to expect from him – how will he communicate? What can I do to help him? Will he be able to use this talker? Boy was I surprised. I quickly learned that he was not only able to use the talker proficiently but he was using it in so many FUNNY ways! By the second session he had told me he loved me. Now usually I will run away from a boy who says those words after only 2 meetings, but with Jacob I welcomed those words with open arms. I frequently get an “I love you” each session. Another phrase Jacob loves to use with me is “All done”. The first time he said it I was only at his house for about 10 min (I usually stay for an hour). I, along with his nurse, cracked up laughing saying, “Jacob, I am not leaving yet! I just got here.” I told him I appreciated the fact that he was advocating for himself but I was staying another 50 min whether he liked it or not. It’s become a ritual now for him to say “All done” about halfway through the session – I think this is more as his little joke with me rather than he really wanting me to leave. At least I hope it is. When I can tell he’s becoming a little tired with the session I pull out one of his many iPad games that he enjoys playing. He’s really good at them, especially bumper cars, and usually beats me even when I am trying my hardest. Boy when he wants to he can be pretty accurate with hitting that switch. 

 

In August I did a race with Jacob. It was an early Saturday morning and I was so excited to be able to run with him. ‘He’s going to love this’ I thought. He always wants to go outside so I thought this would be fun for him. The race day wasn’t too hot and although I was pushing him in the stroller it wasn’t too much of a struggle to finish. I couldn’t wait to ask him what he thought about the race when I saw him next. Well to my surprise he said “Bad” “No” when I asked him how he felt about it. Maria, his Mom, still thinks he enjoyed it so maybe he was just giving me guff like he frequently does J

 

 

That’s just a small glimpse of my relationship with Jacob. Working with him and some of my other kids has taught me several things about a disease I knew nothing about prior to June of this year. I now know that this is a disease that affects people very differently. For example, some people with mitochondrial disease are able to move their bodies and show expression via vocalizations, body movements, and even speech. With Jacob, however, his body doesn’t let him express himself too much in these ways. Once you give him his talker, though, he DEFINITELY lets you know how he feels.  I also know that although mitochondrial disease can cause a person’s body to fail them at times, it doesn’t take away the best parts about them. Jacob’s spirit, brilliance, wittiness, and kindness are very much present inside of him. Working with individuals affected by this disease has taught me to NEVER look at a person and assume their brain is as affected as their body. The greatest things about them are still very present and when given the opportunities they have the ability to show that.  

I look up to the individuals, families, caregivers, and friends who have been affected by and are helping to increase awareness of mitochondrial disease. I feel extremely blessed to work with these individuals and get to see inside their world for a few hours each week.

Brittany

 

P.S. Jacob didn't get out of the PICU today...he started having excessive diarrhea, and everyone agreed that the floor is not as good about making sure that Jacob's fluid intake is sufficient when his stool is out of control.  We are hoping he can leave the PICU tomorrow. Unfortunately, Jacob will miss Gemma's wedding tomorrow...