Milestones

June 19th is a pretty important day... at least in my opinion.

10 years ago today, Lennon's dad and I went on our first date...  <3  That's a great memory and a pretty big milestone.  We found each other at an unexpected time and built a life together.

June 19, 2016 is the day that Jacob (the first CARS2 kiddo) lost his battle to mitochondrial disease.  Lennon's journey was just beginning as Jacob's 10 years were ending.  10 years sounds like an amazing milestone for us to strive for with Lennon.  Hopefully it is just a starting point.


Last week we had our follow-up appointments with the specialists in Wichita in addition to checking the g-tube.  At both of the major appointments, we were faced with the question "When is enough enough?"  (Or something along those lines)

I was caught off guard.  I was not ready for that question.  I think indignant is a good word for it.

I found myself thinking, "how dare she ask me that?"  He's fighting, he's improving, he hasn't ever had an event that wasn't brought on by an infection... he hasn't had pneumonia... finding every reason in the world why we shouldn't be having this conversation.  But I think we all know that tomorrow could be our last day.  How many milestones do we have left?

Most people didn't realize that when we took our spring break trip, we were worried that it would be the last time our Texas family could see Lennon.  We didn't know how many more opportunities we would have... and when April came, that sense of mortality became even more prominent.  April was really scary.

Anyway, since last week's appointments, dad and I have been trying to deal with the big questions for what happens after.  How are we going to prepare for the days, weeks, months, and years after our sweet boy is no longer with us?  It suuuuucccckkkkssss to think about life insurance, funeral arrangements, coping.  It seems like an impossible task.  I have read about Maria's journey after Jacob, so I know that I'll get through it, but I also know that it won't be easy.

So on this June 19th, I celebrate the 10 years I've had with my love; and I celebrate the 10 years that Jacob had with his family.  I wish I could have met him...

Settling In

I guess I didn't realize how much time had passed since my last post.  We have been settling in to the new routine, new equipment, and new support. 

Lennon's nurse was able to start the day after school was out, which was a huge relief considering that grandma had to go back to the real world.  I love our nurse.  She "gets it."  Sure, she may never have had a case like Lennon before, but she understands the stress of hospital stays, the burdens placed on parents of kids with special needs, and the benefit of having an extra set of eyes and hands.  When we first talked about nursing care for the summer, I wasn't sure if I would want someone for the whole number of authorized hours.  Within the first 2 days, I knew that we would stick with the "regular schedule."  Unfortunately, she won't be "ours" forever. She has another placement throughout the school year.  Later this week, we will meet a new nurse who may take over as our year-round caregiver.

We had a post-hospitalization appointment last week. Lennon's weight is up to 28.4 lbs.  I think that is the highest weight EVER!  It's also at least 2 pounds more than when we left the hospital.  (Thanks for the ice-cream and tater tots, grandma!)  The g-tube makes it so easy to ensure that enough calories are going into him. It also makes it easier to give meds.  Lennon improves every day.  I was told that I would be surprised by his strength and perseverance, and I definitely have been.  A month ago, I didn't know if he would be able to swallow.  Now he's eating, rolling, making lots of noise, and regaining a lot of his strength.  Even tonight at our weekly dinner with friends, everyone commented about how good he looked.

Even though he is doing well, we are still very aware of the fact that we will have many more hospitalizations, doctor visits, travel expenses, and needs for medical and support equipment.  We were approached a few months ago about having some sort of fundraiser for this.  Sunday, we met to discuss our event All In for Lennon which will occur September 8th.  We would like to have a silent auction, raffle, and a Monte Carlo night in addition to a brisket dinner.  View the event page HERE.  More information will be posted as it comes available.

For now, I'm just happy.  I sleep better knowing that Lennon improves every day.  I don't feel confined to the house because I'm afraid of leaving.  I have been able to spend some legitimate quality time with the big kids and TEACH them some things that we've been missing.  Honestly, one of the best moments of my summer was being able to brush and braid Hannah's hair, knowing that someone else was taking care of Lennon's morning routine. I'm happy. I'm grateful. I'm proud of my family.  Here's to the beginning of a beautiful summer.

New Normal

This isn't the first time I've used the expression "new normal," and I'm sure it won't be the last. Each time Lennon has a routine appointment, ER visit, or extended PICU stay, it seems we have something new to implement, adjust, or research.

Our most recent hospital stay was long and difficult.  It was our first real test of advocating for our son in a situation where the staff was unfamiliar with him. To be honest, I think dad and I both felt like jerks for questioning the nurse, but we know our son better than that nurse... and the resident on staff was going off the nurse's reports.  When our normal doctor came on that night, she assured us that we did the right thing, which helped.  I have always been leery of staff who don't know Lennon, but I try to be patient and give them the benefit of the doubt.  It isn't that I think they're not capable; it's that he's not like anyone they've ever seen before... and new nurses try to treat him like he is.

Over the last 14 months, we've had to add words like manifestation, baseline, presentation, myoclonus, desat, and episode into our vocabulary.  Doctors and nurses ask for a baseline each time we are in the PICU, but it is hard to answer, especially when the MRI shows 2 new affected areas in the brain.  Baseline going into the hospital in April was much different than baseline even now.  In our last stay, Lennon lost his voice and his swallow. His voice came back first, although he is still fairly quiet.  His swallow came back this week. We have been able to give him a few bottles, pureed fruit, and yogurt.  Now that all his medicine is given by tube, hopefully he can enjoy the full flavor of his foods.

Shortly after coming home, we were visited by representatives from home health and our insurance company. They are working to get us set up with the nursing and attendant care through the TA waiver.  As much as Lennon loved daycare, it's just not a great option for him. What gives most kids a runny nose and cough can, as dad says. "almost take him out." So for the next couple of weeks, grandma is staying home with Lennon so that dad and I can work.  Hopefully we can get her approved as an attendant so that she can be compensated, at least somewhat, for the month she is taking off work to be here.  Nursing care will probably start after school is out.  This is a new normal that I am looking forward to.  I am optimistic about finding a nurse that will stick with Lennon for the long haul.  Having nursing care will not only be reassuring, it will also allow me to spend more time with brothers, sister, and dad and in my classroom.

This new normal we are adjusting to includes more medical equipment, more monitoring, more prescriptions, more paperwork, more frequent doctor visits, more skilled care, and more unknowns.  This new normal also changes every day as Lennon regains his strength and as his brain heals.  We are hoping for another good summer. Last year we went from June-October without any hospitalizations.  For tonight, I'm just happy not to be spending Mother's Day in the PICU again.  Instead, we had a lazy day at home with all the kids and short visits from my aunt and mom.  Let's keep this our new normal.

10 Days and Counting

April 19th at noon, I left for Lennon’s pre-op appointment for his g-tube placement. We got home Sunday the 22nd around 5 pm. We left the house a little before 5 am Tuesday the 24th for the ER. April has been a chaotic month for us to say the least. In addition to our travels to Children’s Hospital Colorado on April 6th, we have also had 10 days of inpatient stays at Wesley Children’s Hospital.

The current stay has been a doozy. Although I had more than enough time to write about it, I simply couldn’t find the words to do it while sitting in the PICU.  To make a long story short, Lennon contracted three very common viruses. The kind that most people experience as an annoying runny nose and cough, but also the kind that sends his body into panic mode. He is recovering, but it is slower than we expected at first, especially since his seizures were not visible on the initial 30 minute EEG.  The MRI had much worse news though. There are two two new areas of the brain that show damage. We are seeing big changes in our little guy this time. He didn’t make a sound for several days. He is not biting or chewing. He did not seem to remember how to take a bottle either.

It is so strange and unsettling to be the parent at home with the big kids while dad stays and cares for Lennon. I didn’t have to prepare medicine when I got up this morning. I didn’t have to stop at daycare this morning before taking the boys to school. Sure, I got to work earlier, but it just didn’t feel right. I’m hoping that we are all back under the same roof soon.

Upcoming Surgery

At our appointment with the specialists at Children's Hospital Colorado, one of the things that was recommended was a g-tube.  This wasn't the first time we received the recommendation, and since we know that Lennon still isn't gaining the weight we would like to see, it makes sense to place one. So to make a long story short, we will go Thursday for a consultation and have surgery Friday.  There will be a 24 hour hold for observation, and hopefully, we will be able to come home Saturday or Sunday.

Children's Hospital Colorado

The wait for yesterday's appointment was both exciting and stressful.  We had no idea what to expect.  Would this be another appointment where we were in and out in 30-45 minutes with no new information or would it be longer and full of bad news?  I think dad and I were both pleased with the information and care we received.  

The Lobby

Children's Hospital of Colorado is HUGE!  It felt like being at an airport versus a hospital.  There were different areas of the lobby, each with a corresponding letter.  We were at "terminal F" as I called it, waiting for our flight to leave, haha!

The appointment started as they all do with vitals, weight, and height.  Of course, we couldn't get a blood pressure because of Lennon's movement disorder. At least they didn't make us try again.  His weight came in at about 11 kg or 24 lbs, so he's regained a little bit of the weight he lost.  The weight probably wasn't completely accurate since he's too long for the scale.

Dr. Larson arrived very shortly after we entered the exam room. He asked what we were hoping to get out of the appointment and we didn't know how to answer.  As with most of Lennon's visits, we didn't know what to expect.  

Dr. Larson examining Lennon

While we were there, we discussed a number of things that we've already discussed with all of our Kansas doctors, but it was good to hear another perspective.  Dr. Larson spent a lot of time letting us ask questions that ranged from diet to life expectency.  After several hours, dad went to get lunch for Lennon.  During that time, Dr. Van Hove stopped in and thanked us for coming.  I wish dad had been there to meet him too because you could really sense the devotion Dr. Van Hove has to studying and finding solutions for mitochrondrial patients.

The highlights of our visit are as follows:

1. We discussed the genetic testing results again.

2. We signed consent forms for registry into the NAMDC database as well as Dr. Van Hove's study.

3. Lennon had a skin biopsy.

4. A new supplement has been recommended.

5. We got information for www.mitoaction.org, www.miraclesformito.org, and Living Well with Mitochondrial Disease: A Handbook for Patients, parents, and Families

Vocabulary 
From a clinical standpoint, Lennon still has MELAS (mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes), the cause of this is most likely from the defect in his CARS2 gene.  Typically MELAS occurs because of a defect in the mitochondrial DNA itself, but in Lennon's case, his mitochondrial DNA did not show pathogenic mutations.  CARS2 is a gene that impacts the mt-tRNA synthetases, so according to Dr. Larson, even though CARS2 has not been linked to MELAS before, it makes sense that the two could be linked. If Lennon's biopsy completely confirms the CARS2 mutation, he would likely be the first to have CARS2 be the cause of his MELAS.

So basically:

clinically- he has MELAS  //  genetically, he has a mutation of the CARS2 gene.

Rare

One of dad's main questions was- "Is this rare?"  Dr. Larson said he considers him a rare disease specialist, so yeah. :)

One in 5,000 individuals has a mitochondrial disease, but as you keep moving through the subgroups, the condition becomes less and less common.

Ok, as I'm trying to reread this, it seems like a bunch of mumbo-jumbo. This is why I'm not a doctor.

In addition to the things I already wrote about, we also discussed placing a G-tube. More to come on that later.

If you made it this far, thanks for bearing with me.  It was a LONG drive to and from Denver. We thank Lenea for keeping the big kids so that they didn't have to travel.  We are glad that the weather wasn't too severe, although we did see our fair share of snow and slick roads.  But for now, it's back to "normal" as we know it. :)

Oh!  Shout out to Maria who is hosting the Miracles for Mito event tonight!  Check out the organization at www.miraclesformito.org.  She has been a tremendous help to me and many other mito patients.

Many hats... but no cape

I didn't have to work today... at least not at school.  (I do, however, need to go in to print things for the upcoming week.)  But it's been a busy day on the phone.

As some of you know, insurance was dropped for all the kids.  With 8 days notice, we have had to scramble to secure new insurance so that there wasn't a lapse.  In theory, we are covered.  I'm just waiting and hoping not to see the words DENIED or PRE-EXISTING CONDITION when it comes to Lennon.  Even though we are all on dad's insurance through work, I'm still working and trying to deal with the state to continue coverage for Lennon, especially since he's been determined to meet Social Security disability requirements.

The life lesson of the day is that parents of chronically ill children wear many hats.  I think most moms feel like they've got too many jobs, but I truly wasn't prepared for the extra responsibilties that come with Lennon's diagnosis.  I am a teacher, and I'm a mom... so some of this is familiar but:

I am not a nurse.  I never imagined trying to manage 7 prescriptions in 13 doses each day. 

I am not a dietician. I didn't think I'd spend hours each week trying to locate recipes that are both nutrient dense and calorie dense.  (Almost everything you find on the internet is geared toward weight loss instead of weight gain.)

I am not a personal assistant.  I used to be the administrative assistant to an executive director within our school district. Even his filing, correspondence, and scheduling needs were less demanding than those of my son.

I am not an occupational, physical, or speech therapist.  Thank goodness I have support from Arrowhead West in this area, but I research toys, exercises, and therapies that might benefit my boy.

I am not a lobbyist.  I keep finding myself called to advocate for patient and family rights, especially concerning insurance and safe access to alternative medicine.

With all these things that I am not, I suppose I have to accept that in fact, I am.  So today, I sat here in my Wonder Woman shirt, given to me this Christmas by my fantastic sister-in-law, and hope that some of her super-powers will pass into me.