It's Been Awhile

I didn't realize it had been so long since my last post.  In some ways, the strain of the past year has caught up with us; but in other ways, we've just grown comfortable with our routines.

Since December, not much has changed.

Lennon did have a CPAP trial in January, which went well, but now we are working on getting a mask with a better fit.  We are still waiting on a sleep study to confirm his obstructive sleep apnea.  Children's Mercy called today and gave us their "first available appointment" on August 21st.  They've also added us to the cancellation list, so hopefully we get in sooner.  He has become completely reliant on supplemental oxygen wheras in December he still had some days on room air.

In February, Lennon got his wheelchair!  This is a game-changer not only for outside the house but also for just being at home.  When we're traveling, Lennon and his equipment are easy to transport.  He's well supported, and the tray under the chair can carry his suction machine and oximeter.  There's a frame for his oxygen tank and the handles adjust so that they're comfortable for both me and dad.  When we're at home, this helps Lennon to be able to sit with us and have a tray so that he can play while being supported.  It's a great alternative to his stander and his bed.

  

The greatest thing about February was not going to the hospital!  For the first time EVER, Lennon was home on Valentine's Day.  He did test positive for influenza A and strep throat though.  With the tremendous daily support of Nikki and the family, we were able to stay home and conquer the illness without hospitalization.  (Sorry PICU girlfriends... mom wouldn't let him keep your dates.)

In the last week, We've been to Children's Mercy in Kansas City for the sleep study consultation and to Wichita for routine follow-ups.  The Kansas City trip was long, but having the wheelchair definitely made it easier to manage.  They also had carts to take supplies from the parking garage into the hospital.  It's a little bit ridiculous how much stuff has to go with us, especially when we're trying to charge batteries for a long drive home.  Our Wichita appointments all went well, resulting in one minor adjustment in medication and plans to follow up in 3-6 months.

Health-wise, we've seen some definite improvments over the last few months.  Lennon is not vomiting nearly as often, even when he's sick.  He's having regular bowel movements now that we've figured out how often to provide support and which combination of methods will actually result in a stool.  Lennon's also lost a little bit of weight, which we think is helping his breathing.  To give some perspective, he went from the 14th percentile for weight in April 2018 to the 64h percentile in August to the 97th percentile in December.  His rapid weight gain was needed, but it was also hard on his respiratory system.  Since December, he's lost less than half a pound, which puts him in the 94th percentile for weight.

Today was our last home visit with Kim Peterson with Arrowhead West.  Since Lennon is turning 3 tomorrow, (Don't ask me how this happened!) he's being exited from the Tiny K program and will start to receive services through Bright Beginnings Early Childhood Center.  Kim has actually been with Lennon almost 2 years.  She came to us after his 3rd hospitalization when we were concerned about blindness.  She, like us, remembers him being a rough and tumble, knocking over trash cans, splashing in the toilet little boy.  She was with us through the genetic testing, the official diagnosis, and graciously rescheduled many home visits due to hospitalizations, illness, and simply sleep.  I've loved working with Kim as she brought Lennon new toys and introduced us to other providers who've given additional support for him.  I'm so glad that Kim will be helping with our transition to homebound services with the school district as well as his first IEP.

A few final thoughts:

• We are starting to have conversations about our next Mito awareness / fundraiser event. (Thanks to Chris Self for keeping us going in that front!)
• Some of you have asked how you can help:
• We NEED to find attendant care.  Vanessa moved and my mom will likely be going to Colorado to work for the summer again this year.
• If any of you are experts on "Tidying Up" we need your help!
• We use Amazon for many of Lennon's supplies. Check out his list here: http://a.co/bs7iDS9 
• Let us know if you're interested in helping out with the big kids when we have appointments or hospital stays.
• Lennon's Legacy Foundation is currently registered as a nonprofit organization in the state of Kansas. We are still working on our business plan and IRS tax exempt status.  If you're interested in being on the board, please let us know!

     

Seasons... a quick update

I'm sure you've noticed that we took a considerable break from the blog.

To every thing there is a season... and some are harder than others.  In the periods of waiting and watching, it is hard to provide updates.  We are well-settled into our routines now, but we still wait for answers from specialists.

Throwback Thursday

2016                                     2017                                     and           2018

Last month during our 2 day trip to Wichita, we saw the cardiologist, neurologist, pulmonologist, geneticist, and dietician.  We are hoping to have a sleep study scheduled soon and also got Lennon measured for a custom wheelchair.  These processes are slow-going.  There is a lot of waiting involved while insurance decides what and when they will cover treatments, tests, and equipment.  Meanwhile, we (parents, family, nurse, therapists etc) try to figure out how best to support him in the meantime.

December 5th, Lennon flew to Wichita for possible pneumonia.  Either he had it and the antibiotics knocked it out right away or what they saw on the chest x-ray was residual scar tissue from prior aspirations. Either way, he was doing much better after a couple of days, so we came home.

Tomorrow, we travel to Wichita again.  This time we will meet with the gastroenterologist and hopefully figure out what is going on with the vomiting and lack of bowel movements.  (Sorry if that's TMI.)  Later in the day, we will see Dr. Beltran (geneticist) and Dr. Welch (neurologist) for follow-up appointments.

In happy news:

1. A miniature family reunion is in the works... :)

2. It's Winter Break! (No school for mom or the big kids until January 7th.)

3. Lennon was nominated for a TinySuperheroes cape and has received enough donations to rush his order.  https://tinysuperheroes.com/squad-super-lennon-k/ 

Saturday morning thoughts

Saturday mornings are great for thinking.  I brew a pot of coffee and enjoy the quiet while most everyone is still asleep.  To be honest, the only reason I got up so early (5 am) is because someone decided to kick his oximeter probe off his big toe. We listened to music for awhile, snuggled, and even did some dancing in the kitchen.  :)   

Of course that was before the vomit and the suctioning, which has become a pretty regular thing.  But we live for those tender moments between medical needs.  Since then it's been more suctioning, dressing, bathing, giving meds, venting the tube, monitoring vitals, and trying to figure out the best course of action.  Add in a splash of looking at old pictures and researching mitochondrial disease and treatments and you get the full picture. 

Have you ever felt like you're at the top of the rollercoaster just waiting for that last click before the plunge?  That's kind of what it feels like with our little guy.

No stool- click
Coughing- click
Elevated heart rate- click
Low O2 Sat- click
Vomiting or posttussis emeisis- click
Hiccups- click
Increased myoclonic movements- click
Fever- click
No nap / too sleepy- click

It is hard to live your life constantly on watch, on edge, and for Lennon to constantly be on a monitor or feeding tube.  October was hard for all of his caregivers.  Even the ones who are paid to do the aforementioned things.  We are not able to determine why he is having such difficulty lately.

Next week we have four appointments in two days.
     Seating clinic- fitting him for a wheelchair or stroller
     Neurologist- check levels and adjust medications
     Cardiologist- address elevated heart rate and the implications of seizure/stroke events on his heart
     Pulmonologist- address the retractions and concerns of aspiration with more frequent vomiting

This still leaves us with questions about his digestive system.

We could really use some good news or at least some definitive (or evidence based) answers.  It is increasingly difficult to hear "we don't know why" from our specialists, even though it is true.  Knowing what to anticipate with Lennon's health would make it much easier to address the bigger questions that have been weighing heavily upon us.

In April they asked us some really difficult questions, and now our providers are continuing to push us to make drastic changes.  We also feel the pull from family to consider a change of scenery.  Honestly, if we can't come up with a better plan for managing Lennon's health and keeping him well, we have to be closer to medical providers who can treat him quickly and effectively.

Yes, I think moving is a good idea. No, I do not want to go anywhere.  I am afraid of making the wrong decision, of putting undue stress on the Lennon and ending up in another hospital stay,  of uprooting our entire family, of giving us temporary relief with long-term negative consequences.  And then there's the consideration of time... because I'm pretty sure we are past the "if necessary" and to the point of "when are we doing this?"  But how long do we wait to see if he does better or to start applying for jobs and housing in a new place?  How do we even decide where, much less when?

7 hours of coffee, research, and care later... I still don't have the anwers.

October (Edited to add...)

October has proven to be a difficult month for Lennon each year.

His first October (2016), he had what seemed like constant viral infections.  His stomach seemed to be constantly upset and his diapers proved it. (I know, TMI.)

He was still very cute though!

This was when he was meeting all his milestones and before the diagnosis, but I remember thinking that something just wasn't right.

His second October (2017) we learned just how quickly a good day can turn into an extended hospital stay.  This ended up being his fourth stay at Wesley Children's Hospital.  We'd had a fantastic morning and afternoon.  He knocked over the trash can SEVERAL times, tried on his Halloween costume, and tried to carve pumkins with the big kids.  Then in the early evening, he spiked a fever and the seizures started.

We knew that we needed to be concerned.  We had already begun genetic testing and had actually just submitted our own blood samples for the genetic variance analysis, but I don't think we ever would have imagined the changes that we've seen.  It's crazy how long ago this seems.  It was one year ago today.

part 1

part 2

This is his third October.  Just a couple of weeks ago, we had our most recent hospital stay.  We've found that those viral infections are STILL a likely cause of his decline, even when we can't pinpoint what he had.

Lack of bowel movements has been a concern for quite some time, so we'd seen Dr. Moffitt for that earlier in the week.  In addition, for several days, we had noticed more movement, increased difficulty breathing, breakthrough seizures, and episodes of very high heart rate.  We hoped that by getting his bowels emptied, those symptoms would decrease.  Hello Miralax 4x daily...

Oxygen saturation 96% and Heart Rate 173 bpm

Thursday Oct 4, we went to the ER and had our first successful IV Argenine infusion in Dodge City.  His heart rate settled and his movements slowed, so we went home.  Friday, we went back to Dr. Moffitt to repeat tests since his heart rate was still too high.  They did rapid tests for influenza, RSV, and strep.  He came back positive for influenza A and B, so a flight to Wichita was arranged.

Upon arrival HR was still too high, but... the Miralax worked... really, really well.  If you have a weak stomach, skip the next sentence.  They said he eliminated a liter of poop in two stools.  How is that even possible??  Anyway, nurse Sally has forgiven Lennon, Dr. Moffitt, and me.

The EEG revealed that Lennon had continuous seizure activity on the right side of his brain.  We were able to follow the plan that was used in July for seizure control and we repeated his viral panel, only to find that influenza was not indicated at Wichita.  In fact, we didn't get a for sure diagnosis while we were there, so he was just treated with respiratory support and seizure control.  Medications were adjusted for weight gain.

Now after 2 weeks, it appears that the new levels are appropriate, but we had breakthrough seizures daily, especially when waking up for at least the first week after discharging from the hospital.  To help him during this viral season, we're adding a nebulizer and albuterol to his equipment.  We've also submitted requests for a percussion vest, and cough assist to insurance.  He also got fitted for custom AFOs since his feet are too big for the quick fits.  Those should be coming soon. The design is pretty awesome!

Although Lennon's health is our primary concern, we have also decided that our little guy is a living legend.  We want to make sure that his experience helps other kids.  I hope that when I create my next post it will be to give a full update on Lennon's Legacy Foundation.  We filed the Articles of Incorporation at the end of September, but there are a few more steps in the process.  As always, thank you for reading and for your support!



I originally posted this October 21, 2018, thinking that our October issues would be over.  I was sadly mistaken.

October 14th he flew back to Wichita after a seizure episode and was released the following day without further testing.

October 26th we flew back again and found continuous seizure activity, despite much improved outward presentation.  Several boluses of Keppra and Lacosamide later, he was mostly seizure free, although even yesterday (and today) he continues to have seizure discharges.  Most of his activity is in the right frontal lobe which presents as facial movements and nystagmus (eye twitching.) 

Considering there are only THREE days left in October, I am optimistic about being done at Wesley Children's this month.

Quarterly Check-up

Quarterly check-up with the geneticist, dietitian, and neurologist today in Wichita.  Things look pretty good although Lennon did have a breakthrough seizure as we were leaving the first appointment.  That just confirms the doctors' thinking that we need to increase medications due to his growth.

He is up to 34 lbs and 37 inches! Such a big change from April when he was only 25 lbs and 35.5 inches. He's gone from 18m clothes to 4T.

In addition to increasing one of the seizure meds, we are also going to decrease his feeds slightly. We need to be sure that his weight gain does not interfere with his breathing. We are taking away about 150 calories per day and will check his progress in all areas at our December appointment.

Other things that were discussed include getting either a wheelchair or medical grade stroller, a handicap placard, new AFOs, and swabs for the granulation tissue on the g-tube site.

*Pictures to be added later.*

Progression and Regression (tissues may be needed)

Lennon is almost 2 1/2 now.  He was 11 months when we had his first major hospitalization.

There are days that I honestly can't remember him... you know... the way he was.

NOTHING anyone says can prepare you to grieve for a living child.  

I miss my son.

He was a happy, healthy, little guy before THIS happened... our first PICU stay.

We came home thinking it was just a bacterial infection and dehydration that caused his seizures. We learned that we could treat him with an anticonvulsant and that he might be able to wean off of it in a couple of years.

but then THIS happened... PICU stays 2 and 3... when he couldn't see ( we still don't know if it is true blindness or cortical visual impairment), his lactic acid levels were too high, and the seizure activity was located in new areas of his brain... and the doctors started thinking there had to be something more, so they gave us the name MELAS and started the process to see IF insurance would allow this test and prepared to petition the state medical board.  

We sent Lennon's blood samples in June to test for MELAS.  We knew that the process would take 10 to 12 weeks.  That's a long and scary wait, but we had a wonderfully uneventful summer.

I miss my son and the way 

he could still crawl around, knock over trash cans, and keep going back for one more toss from Dad. (Spare us both any negative comments about that.)  

For perspective, the video with Dad is from less than a year ago.

  

 

In mid-September of last year, we were beginning the process of submitting Mom and Dad's DNA to perform a variance analysis since the typical MELAS genes were labeled

Variables of Unknown Significance.

In October, we had been nearly 5 months seizure free.

Then came PICU stay number 4... and we were STILL WAITING for conclusive genetic test results.  

Fall of 2017 was difficult, watching him get thinner and weaker, but not really knowing why or how to help.  

After what seemed like an eternity, we received his test results not long before Christmas.  

CARS2

Not even the specialists in Wichita could tell us what we might expect since only 3 children were documented as having the genetic mutation prior to Lennon.

And then waking up 364 days after his first hospitalization with more seizures... PICU stay 5

     

Choosing to take a trip to Texas in March to celebrate his birthday, partly because we weren't sure if we would get to celebrate another one...

  

Driving to Denver to see the specialists who worked with the first child diagnosed with CARS2 and following their recommendation to have a g-tube placement surgery to fight his failure to thrive (AKA him not gaining weight for nearly a year) only to end up in the PICU for real after 2 days at home.  PICU stay 6/7 depending on whether you count his post-surgery stay.

                           

Coming home and wondering if we were ever going to hear his voice again, if he would ever swallow again, what it was going to be like now that he needed 'round the clock care including skilled nursing.

Not taking ANY videos on my phone for over a month and VERY FEW pictures.

Until I could finally accept our new normal.

***

Being blessed by time with family at the end of July.

       

Only to be hospitalized again the day we were supposed to come home from our short vacation in Wichita... PICU 7/8

But here is our champ, our beloved, showing everyone what he's made of!

He is the light of our lives and "the cutest baby ever" according to his siblings.

   

I may never actually post this... it may stay a draft forever.

Usually when you hear words like progression or progressive, you think of moving forward in a positive way, but progressive movement disorder in itself is not a positive experience. 

With the progress of the disease comes the regression of its host.  

As the mitochondrial disease moved forward, Lennon's abilities moved backward.  

Yet he continues to impress us all. 

It just means that our celebrations are different now because he is different now.

I Love Lennon!

I miss his cry.

I miss his laugh.

I miss the messes he made.

I miss hearing him say my name...

and Christian, and Bubba, and DAD!, and Ha-na Ha-na Ha-na, duga duga duga, and COOKIE!

I miss watching him enjoy his food, smacking his lips and shoving it into his mouth with both hands.

I miss him constantly being underfoot.

I miss him crawling into my lap.

I miss the TV or computer going off and having to plug the router back in because he'd crawled behind the couch and disconnected the WiFi.

I miss him speeding down the hallway to the toilet before I could even walk to the kitchen and hearing him spashing in the water... or dropping toothbrushes in. (YUCK!)

I miss the sound of the trash can falling over and dishes being thrown from the diswasher.

I miss his hugs and kisses when he had better control of his body.

I love that he still giggles.
I love the way he yawns. (That hasn't changed a bit!)
I love sharing new (old) music with him.
I love the snuggles.
I love that he loves his family.
I love the way he still plays with everyone.
I love how hard he works.

But I (not so) secretly long for the little boy who was... even though now he is so much more.  

My baby, my fighter, my beloved, 

Lennon.

Going Green

Mitochondrial Disease Awareness Week is September 16th -22nd.  I'm hoping that you'll all support Lennon by "Going Green" for Mito. 

The problem I've been facing recently is that I've been going green with envy.  It's embarrassing to admit this.  How can I be so overjoyed for others and so envious of them at the same time.  My heart is overflowing with things to celebrate and yet is broken by... Lennon's circumstances.  It's just not fair.

That green monster is kicking my butt... Whispering in my ear.  Every time I celebrate, I get that whisper telling me another thing that makes him misunderstood or another milestone that he won't meet or a journey that seemed so much longer or harder for him.  Someone keeps trying to steal my joy!

The envious voice keeps wanting to lash out, to paint a vivid picture of the suffering that Mito has caused not just Lennon but our entire family.  While his story deserves to be told, it shouldn't come from that source. 

My baby is a warrior, a charmer, a blessing! He will continue to fight and surprise us all.  Saying that it isn't easy is an enormous understatement!  There is constant emotional conflict... and a part of my story that I'm not quite ready to write.

I hope to keep getting good news like friends' pregnancies, new treatments, quick test results, and kiddos learning and growing. I'll be celebrating along with each of you... and I'll keep seeing things to celebrate with and for Lennon.  That green monster will not get the best of me.

If you're interested in Going Green, please message me or leave a comment here on the blog! I'm thinking Thursday, September 20th would be a good day for it.

As always, thank you for your support and encouragement!