48 HOURS AND COUNTING

It is Sunday night, and we have been home for 144 hours or since Monday night. I was nervous taking my boy home this time. We had an involved care conference Monday afternoon in the hospital, and got detailed plans from GI, endocrinology, and neurology on how to manage Jacob's colitis, adrenaline insufficiency, and myoclonus from home. We were told we would do "hospital care" from home, and if we wanted to stay in the hospital, we could stay as long as we wanted to.

The thing is that we always want to come home as soon as we can, and we were willing to take on the challenge of stool replacement, measuring Jacob's daily input/output, doing labs from home, monitoring one of Jacob's seizure medication levels in his blood from home and "load him" if the blood level would drop too low. The care itself didn't scare us. It was the fact that last time we brought our boy home, he only stayed home for 48 hours. As we passed 48 hours this week, Jacob was still at home.

Jacob has now been home for six days, and it was the right decision to take him home. He is on a high dose of steroids, and it is controlling his colitis at the moment. Tomorrow, we are starting his wean plan once again. This time we're looking at 8 weeks of weaning steroids. It will be summer time before we have a chance to peel them off this time. The outstanding question is also if Jacob will always need to be on steroids for his adrenaline insufficiency or not. We can't test that until Jacob is back to baseline, so this is something we will work with endocrinology on in the coming months to come. As Jacob's colitis is better controlled, Jacob's myoclonus is also so much better. Myoclonus (extreme twitching to his face and body) always comes with pain and stress to his body. Dr. E said on Friday that Jacob looks pretty good. It means so much when the words come from her.

We unfortunately got some not so good news on Wednesday. Jacob is closely monitored by his ENT doctor due to the fact that he has cholesteatoma. This is a cyst in his ear eating away at his middle ear. If this is not controlled, Jacob will loose his hearing completely. You can control the growth of the cyst with ear tubes. Something Jacob had put in a year ago. Unfortunately he has lost one of his ear tubes. This means we are looking at another surgery, most likely in June. As any time Jacob is put under, there are always many who would like to have something done. We are looking at doing botox to Jacob's salivary gland to reduce his secretions. We are also checking with his GI doctor if he needs any further procedures, since he is suspecting Jacob has more than colitis going on in his tummy.

After having lived 3 weeks in the hospital, we are behind on life. This weekend was a catch up weekend. We finally got to do some yard work and clean out our garage. I had piles of paperwork to take care of, a house to clean, and Sarah was excited to plan play dates and sleepovers again. Today was the perfect day for a stay at home day. We all cuddled up on the couch to watch the movie Frozen. It was a great ending to a weekend at home.

To more days at home!

Love, Maria.

THIS IS WHAT I'VE LEARNED

So, I started this blog post Thursday morning...as the day took a turn for the worse with doctors not talking to each other I used the blog to get out my frustration...thanks for listening.

Let's try again.

The last two weeks gave us a reminder of how very sick Jacob can get due to his mitochondrial disease. Friday two weeks ago when Jacob was rushed in an ambulance with sirens on all the way to Children's, I didn't know if he was going to be able to fight through yet another crash to his body or if this was it. I knew Dr. E. felt the same way.

This snowy Thursday morning, I am once again sitting in the hospital chair with a cup of hospital coffee next to me. I do have my boy next to me in his wheel chair watching NBC morning show as big wet snow flakes are falling to the ground. Going on week three in the hospital, I am eternally grateful that Jacob was able to fight through sepsis and extreme withdrawal of stress steroids.

This is what I've learned:

1. These really hard times make me appreciate my life so very much. There is really no time to sweat the small stuff when you are fighting with your boy for his life. That gives us a lot of happiness in our day to day life as weird as it might sound.
2. Sleep is not to be underrated. I can operate well on a few hours of sleep, but when you are loosing several nights of sleep mixed with worry, it doesn't make for a pretty me. My brain looses its' edge, and I snap. Yes, I snap for the little things, and have no patience for how absolutely disgusting the bathroom is in the PICU or how many times I woke up from a beeping medical pump.
3. The nurse of the day or night can make a huge difference to our day. If I get that brilliant nurse who knows her stuff and also really cares about my boy, I am in a good place. I can lower my guard, and let them take charge rather than me hovering over them making sure they are doing all the things they should.
4. I love my work. In the middle of crisis, I am so very happy that I can escape to the world of work that I know so very well. As I train people, I have to stay in the moment of the topic, and my mind can't wander off to places of respiratory distress, diarrhea, and myoclonus.
5. I can't stand the flight for life helicopters. Every time it lands on the roof of the hospital, it gives me the chills. I know it is a very sick child in that helicopter, and I always say a prayer for the child in the helicopter and all the other children in the hospital including my boy.
6. I need my girl. I love to be part of her middle school world and balance that with the hospital world. I missed her terribly when she was on spring break, and for the first time truly realized how important my time with her is when we spend weeks at Children's.
7. My husband is my hero. He never looses his calm. He is sharing in all parts of a hospital stay from sleeping on the hospital couch to taking work calls from the hospital room to advocating for Jacob at all hours of the day.
8. I need to surround myself with positive energy. In the middle of crisis, I need to find laughter and little pockets of joy. So many here at Children's are our friends now. They care for our whole family, we are especially close with the PICU staff. I love when the attending finds time in the afternoon to sit down with Jacob and me to chat about this or that. I love our community of support who never fails to get us going. It could be a bear hug from a close friend, an uplifting message, a meal on our front porch, and the list goes on and on.
9. Do something for myself. Five years ago when Jacob came down with sepsis, I only left Jacob's room to go to the bathroom and never showered once. I have no idea how many hospital stays we have had since then, but I have learned the day will be better if I do something for myself. I always shower. I always eat (and treat me to the things I really like). I go for a run around the hospital.
10. We're the lucky ones. It strikes me every time we're in the hospital. I never ever feel alone on this journey. Our family is far far away, but we have an amazing support system of friends, love, and strength. I love you all for that.

We're getting GI, endocrinology, neurology, and special care all in one room Monday afternoon. I hope it is then time to take our boy home. I've learned that there is nothing like having us all four under the same roof.

Love, Maria.

FRUSTRATED

Being back to being hospital mom, the worst thing is when you finally get discharged and then have to return right back to the hospital. You have barely settled at home or caught up on your life again, and then you are starting all over again. This is what we did last weekend.


The next worst thing is when you are told you are going to be discharged. You see the light at the end of the tunnel, and then the discharge plan changes. You are told you are not going home. This is where we are tonight. We have been told since Tuesday morning by our inpatient team that Jacob would go home Thursday or Friday. This has been repeated to us every morning on rounds. We were even told we would get an early discharge tomorrow, so we could get Jacob settled at home and get our nursing set up again.


This afternoon, I filled a red wagon full of bags with the things I have collected during this past week. I didn't leave any traces of myself in the hospital room. I wanted to make sure we had as little as possible to bring back home tomorrow morning.


Tonight, I didn't unpack my bags. I now know I have to bring them all back to the hospital tomorrow morning again. I am extremely frustrated with the medical team. For two days, we have been told we need to have a consult with endocrinology and GI before we go. They both needed to weigh in to our discharge plan. I knew it wasn't going to be easy to go home this time, but there was never a doubt of the medical team that it could possibly not happen tomorrow.


I so remember the hesitation of GI putting Jacob on steroids the first time he had colitis. They wanted to try every possible solution before they put him on steroids. That feels like a very long time ago. Since Jacob was diagnosed with colitis, he has been on and off steroids. Since Jacob's colitis flare up this past fall, we have not been able to get Jacob off steroids completely. When you get really sick, and you have been on steroids for a long time, you need a high dose of steroids for your body to respond. This is called your stress dose. When Jacob was septic two weeks ago, he was put on a stress dose of hydrocortisone for the first time. It is not great to be on such a high dose of steroids, therefore the reason to take him off so quickly last week. I don't think anybody could have foreseen Jacob's extreme withdrawal from hydrocortisone.


Now Jacob is diagnosed with adrenal insufficiency and his immune system is completely suppressed. They think he went into adrenal crisis on Saturday when I brought him into the ED. Yesterday, we started the wean of hydrocortisone once again. We are going 10 times slower than the first time. We know what signs to look for if Jacob possibly is having withdrawal symptoms. What we learned from endocrinology tonight is that if Jacob shows any signs of illness, he needs to be given his stress dose of steroids again. We need to be trained to give Jacob that dose as an injection at home. The message was that we should go to the ED this weekend for any sign of illness. It didn't sound like the best discharge plan.


Later on tonight, GI finally came by. With the start of Jacob's steroid wean, he is not getting enough steroids for his colitis. We are now seeing a flare up of his colitis, and no one is comfortable sending us home with his current stool output.


Nothing is easy when it comes to Jacob, I should know that by now. Tomorrow, GI and endocrinology need to put their heads together (finally) to decide on the exact dosing of each steroid. I don't think we can go home until we have a care conference with GI, endocrinology, and special care (who will manage the wean). Having been too many times in this situation, I can't see us go home until Monday or Tuesday now. It will also all depend on how Jacob is doing of course.


I am frustrated tonight. This whole steroid business with two different reasons (immune suppression and colitis) for being on steroids is definitely not straight-forward for anyone to treat. Thinking about how very sick Jacob has been these last two weeks, settling in at hotel Children's for a 3rd weekend in a row is the only way to go right now. I know that is true, it just sucks a little.


I am hoping we can get everyone talking tomorrow, so we will have more of a plan as we're going into the weekend.


Love, Maria.

SPRING BREAK IN THE PICU

Spring break 2014 is getting filed as probably the lousiest spring break we have ever had. We started spring break in the PICU, and we're ending spring break in the PICU. It might not matter that much if you don't have plans, but this year we did. Joakim's nephews came all the way from Sweden to spend a week up in Breckenridge with us. We had worked out a nursing schedule, so Joakim and I would spend three whole days together up in the mountains, something that is so very rare for us. Sarah was going to ski and be spoiled rotten by her cousins and having her parents to herself for a few days. Jacob was going to enjoy the spring break from home with nice spring weather, since his seizures don't agree with the high altitude of the mountains. Not much of our plan happened. There was spring in the air, but absolutely no break.

The good news is that we did get home on Thursday, and our boy looked really good! He was definitely weaker than normal, and we knew we were going to take it really easy, but we were home. Here Jacob got some cuddle time with his favorite baby Derek. I had a good feeling about being home again.

We had noticed that Jacob's myoclonus (twitching of his face, mouth, and hands) had started to act up in the hospital. It got a little worse every day. Since it is a movement disorder, and not causing Jacob harm, we typically let it run its' course. With each day, it got a little bit worse, and it started to look really uncomfortable for Jacob. It also makes it hard for Jacob to handle his secretions, since his face is moving constantly.

On Friday, Jacob's heart rate kept being elevated, he needed oxygen to breathe, he had extreme diarrhea, and his face was twitching constantly. Saturday was no better...I had escaped up to the mountains Friday afternoon to spend a little bit of time with Sarah. I missed her terribly during Jacob's hospital stay. She keeps me grounded when Jacob is really sick. This time around, there was no girl at home to keep me balanced. We had a great movie night together Friday night, and we had the best skiing day yesterday. My girl is skiing black runs, and she is faster than me! I knew it was going to happen, and yesterday was the day. I was so proud of her.

Sarah having lunch with her cousins.

As I was getting ready to drive back down to Denver, Gemma texted me to let me know that she thought Jacob had to go in again. He needed bi-pap to breathe, vitals were off, and lots of myoclonus and secretions. I thought if worse comes to worse, I'll drive straight to Children's in my ski clothes. I have come to the ED in wedding party clothes, so ski clothes should work too!

I did made it home, but as I was talking to Dr. C. for the third day in a row about his myoclonus, we decided it was time to bring him in again. She was concerned about how it impacted his breathing. Jacob didn't look great coming in to the ED, and they once again treated him as he had sepsis. He was put on the big gun IV antibiotics, and stress steroids once again.

Today, it doesn't look as if Jacob has a new infection and his labs are good. His vitals are stable as well, so sepsis has been removed from the table. We all think he is having withdrawal from his stress steroids. He was on a heavy dose of hydrocortisone three times per day, and we weaned him off it completely in 3 days time. As we weaned him, the myoclonus slowly increased and got completely out of control as he was off the steroid. His normal steroid for his colitis didn't do a thing to calm his body. We had been told that high heart rate, jittery, and irregular blood pressures were to be expected. The attending today said that we now have to add myoclonus as a possible withdrawal symptom.

The plan for today is to wait for cultures to come back, so we truly can rule out infection and start peeling off the three antibiotics he is on. Tomorrow, endocrinology and GI will be consulted for a very slow and safe steroid weaning plan. Dr. C. will be consulted for his myoclonus once again. We don't have a good sense of how long we will be in the hospital this time, so we are once again on Jacob time.

Goodbye spring break 2014!

Love, Maria.

PURPLE DAY

Today is Purple Day. People all around the world wear purple in support of epilepsy awareness on March 26th. Jacob and I decided to wear purple to make the hospital staff aware of Epilepsy. Jacob got out of his hospital gown and was sporting his Children's dark purple shirt. I must say I don't own much purple, but I did get out one of my few purple shirts.




Jacob has intractable seizures. This means that his seizures have failed treatment. Despite Jacob being on four different seizure medications daily, he has subclinical seizures around the clock. We have had to come to a point where we only chase clinical seizures or Jacob would be medicated at all hours of the day. It is not an ideal place to be, but it is something we have had to accept. And we have accepted it. Life is too short to not focus on the positive things in life. We know a seizure can take Jacob's life any day, but in the mean time, we enjoy the good things in life, like a seizure free day.




This hospital stay has given us a reminder of Jacob's clinical seizures, and how easily Jacob goes into status epilepticus. Status epilepticus occurs when a seizure lasts too long or when seizures occur close together and the person doesn't recover between seizures. It is a life-threatening condition in which the brain is in a state of persistent seizure. Some people say you are in status after 5 minutes, some say 30 minutes. Jacob's crew goes with 30 minutes.


In this last week, Jacob has been in status three times. It is not easy to break status epilepticus in Jacob, and often he needs multiple rescue medications to break his seizures. A lot of times, Jacob is breathing normally and his vitals are stable during his seizures, but on Friday he wasn't able to hold his oxygen and really struggled to breathe. His body endured septic shock. I was scared for Jacob's life.




Yesterday I got to hang out with the attending doctor in the PICU. He has taken care of Jacob from the time he was a resident to fellow to attending. We started chatting about Jacob, and all the different times he has taken care of our son. He reminded me of Jacob's uncontrollable seizures five years ago, and when we had to put Jacob in a medicine induced coma to stop his status epilepticus. The medicine induced coma entails shutting Jacob's brain off and restarting it to break his seizures. Jacob was in status epilepticus for 11 days or 15,840 minutes, since most seizures are counted in minutes. Unfortunately the medicine induced coma didn't fully stop Jacob's seizures, and Jacob has today lived with the diagnosis "intractable seizures" for five years.




The first time Jacob went into a seizure at age one, I was devastated. I felt as if my life had come to an end. It still aches my heart every time Jacob has a seizure. I am always afraid that we will not be able to stop his seizures. I always tell Sarah we don't use the word "hate" in our house, but for seizures I do use the word hate. Dislike is just not strong enough.


I am hoping one day that there will be that one magic medication for Jacob's seizures. In the mean time, we are just going to enjoy every seizure free moment we can.


And our little warrior is doing so much better today! It is even talk of going home tomorrow...cross your fingers.


Put on your purple PJ, and go to bed!


Love, Maria.

GOING ON DAY 3 OF SEPSIS

After our crazy Friday with Jacob coming down with sepsis, we have been going non-stop. The PICU is a busy place. Between nurses and doctors caring for Jacob, having family from Sweden in town, Sarah having a volleyball tournament, friends visiting Jacob, it has been a really busy weekend with little sleep or downtime.


Jacob is still septic. The attending says his whole body is fighting really hard, and that is why we still see low blood pressures, Jacob not being able to keep his own body temperature up, and him not fully absorbing his IV fluids. 


The good news is that Jacob has not had any seizures today (knock on wood). He was extremely lethargic yesterday. He wasn't up for anything but resting. He is still not his normal self, but he is looking a little bit better today. This afternoon, he was watching Curious George on his IPad, and was pretty content.


The PICU team has figured out what happened on Friday. With Jacob having colitis, his intestines are very fragile. We noticed already on Thursday that Jacob had trouble going, and he had tons of air in his tummy. On Friday, we introduced protein into his regular formula based on a recommendation from his regular nutritionist. He is growing, and needed more protein. What was overlooked is that the protein powder includes an ingredient Jacob is allergic to. He had just the tiniest bit in his morning feed, but the docs think it was enough for the bacteria in his gut to make it into the bloodstream. It is officially called bacterial translocation of the gut. You typically won't get septic from it, but our little buddy unfortunately did.


Today, we started Jacob on tiny, tiny bits of his feeds again. The docs have warned me that this might be really hard on Jacob's gut, since his intestines are so inflamed. They are warning us that his colitis might flare up.


We know Jacob's body will need time to heal, and I have moved in to hotel Children's. We will stay as long as Jacob needs. I can't just yet wrap my head around what possible damage might have happened to Jacob's brain with being in status epilepticus several times this weekend as well as him not getting enough oxygen on Friday.


One step at a time. One day at a time.


Thanks again for everyone cheering for our boy once again. We feel your love, support and help always. I know I am behind on getting back to many of you, but do know that I cherish each message even if I don't get back to you right away.


Love, Maria.

YOU KNOW YOUR CHILD IS SERIOUSLY ILL WHEN...

• Your husband calls you in the car 5 minutes from home, and tells you to call 911. You should not wait to make that phone call until you get home.
• You can't get good pulses on your child.
• Your child is blue despite being on 5 liters of oxygen and bi-pap.
• Your child seizes for 70 minutes straight despite being loaded on more meds than you thought was possible.
• The ambulance crew wanting you to sit upfront with the driver rather than with your child (you still making sure you are not leaving your child's side).
• Sirens on the whole way from Broomfield to Aurora. Fastest trip to Children's ever.
• The ambulance crew starting IV fluids rather than waiting for the ED crew to access his medi-port to give fluids.
• Your child being surrounded by 15+ people in the trauma unit, and people wanting to do 10 different things at the same time.
• Dr. E rushing over from Clinic to make sure the ED crew is doing their job.
• Dr. C paging the Neurology attending from Las Vegas before ED has a chance to do it.
• Your child getting loaded with IV fluids and 3 different "big guns" IV antibiotics.
• Surgery seeing your child in the ED for possible emergency surgery.
• Beeping monitors that never stop.
• Low blood pressures scaring everyone and their brother.
• You repeating your story of what happened before you came in until you don't even know what happened at home versus in the hospital.
• You realizing that hours and hours have gone by, and you have not once had a chance to look at a watch.
• You realizing that you haven't had time to eat or drink or go to the bathroom for an insane amount of time.
• You being told by Dr. E. to get Joakim to the ED asap, and find somebody to stay with Sarah. This is the second time we have been told those words by Dr. E. It means it is serious.

You are told your child has sepsis, but no one knows why. Dr. E. thinks it could be gut bacterial translocation. PICU is not sure just yet what to believe.


I know Jacob is not yet out of the woods, but he is stable. He is sleeping and his vitals look ok. I will end with a few words from his nurse Gemma: Jacob amazes me every day. I can't believe how much strength is packed into that 67 pounds body. It's immense, and I can never wrap my head around it. He is an incredible human being and has been put here to teach everyone he meets what the true meaning of strength is.


I needed those words tonight. To strength.


Love, Maria.