Neil’s Batten Disease Journey
Hi Everyone!
I connected with an old friend a few days ago and she told me that she tries to stay away from facebook and instagram and thus, she wasn’t able to keep up with how Neil is doing.. Recognizing that not everyone has access to the Neil updates I post on Facebook and Instagram, I thought I might update here to let our friends and loved ones how Neil has been fairing living with Batten Disease..
He is 19 now and in September, will enter his final year in the multiple exceptionalities classroom at his high school.
He is well over 6ft tall and remains a burst of sunshine in our lives☀️
He has technically aged out of SickKids, but continues to receive his Brineura treatments there … mostly because his disease and the treatments are unique and specialized (and to be honest, the adult hospital that SickKids is steering us towards doesn’t seem to be very enthusiastic about taking over his care). To be honest, we love SickKids and are very happy to be there as long as they will keep him.
Unfortunately, he currently has a bacteria infection in his chest port that is proving to be difficult to treat with antibiotics.
Neil is currently the only person in Canada who has a chest port that connects to his cerebral spinal fluid in his brain. The chest port connects to a tube that runs up his neck (under his skin) and drains directly into a reservoir in his brain. As novel and easy as the chest port has made his treatments, it has also made treating his port infection more challenging. Unfortunately, it’s looking like the chest port and all the hardware will all need to be removed and replaced. This will be his 4th brain surgery. To be honest, we are dreading this. Every time he has had a brain surgery in the past, he has endured some decline. But it feels like we’re damned if we do, damned if we don’t though, because having a bacteria infection in his cerebral spinal fluid is very dangerous. It’s disheartening and absolutely terrifying.
Aside from that, he has been fairly stable, with just some gradual declines occurring. He can still stand and bear his weight, he also loves to walk in his gait trainer. He is fully gtube fed now because his ability to swallow food or liquid has been compromised… the positive outcome of that however, is that we can ensure he is getting all of his daily nutrition needs met.
He still has his sense of humour, despite his struggle to speak coherently.. when you take the time to really listen to what he says, you realize how hilarious he can be. He is showing some signs of dementia, but to be honest, it presents in an interesting way.. he often refers to his life in a fantasy world of his making, where he is a ‘professional hockey player’ (Montreal Canadiens, of course), and/or he plays for the Toronto Blue Jays.. he also owns a private jet and multiple mansions (he used to own mansions in the States, but he has decided he doesn’t like Trump, so he’s sold those and he has no plans of owning any more in the USA until Trump is no longer president! ).. I call him my ‘passionate little French man’ and he is! He feels passionately about that one! In truth, knowing dementia is a symptom of Batten Disease, if this is how it is showing up, we are happy to accept his magical thinking and magical worlds.
In many ways he remains very child-like in his gentleness and innocence. At times, Mike and I mourn the life he doesn’t get to experience… all the rights of passage: driving, post secondary school, a job, independence, falling in love.. all the parts that shape us.
Yet we also embrace what this disease does gift us with… a son who still wants a lot of hugs and wants to be with us / talk with us, still fully believes in Santa Clause, a child who we know is safe and sound in his bed every night.
It’s so hard to do this parenting thing isn’t it? The knowledge that if we do our job right, it means we have to let them go. It’s not so different for Mike and I.
The one thing we promised to each other when Neil was diagnosed, was that we would see him through this to the best of our abilities, regardless of what comes. And I can say with a clear conscience, that we do just that. Every night when I hug him good night, I know that there is a risk that he won’t wake up in the morning.
If this life has taught us anything, it is truly how to live one day at a time. We have to live like that, because it will bury us in fear if we try to guess at Neil’s and our future.
I know we are powerless over this disease. We are powerless over most things. I also know that nothing in this life is guaranteed..
He’s good today and that is enough.
Thank you again to everyone who has supported us and gifted us with your pure, unconditional love. It feeds our souls and truly helps us on this journey.
Update: May 5, 2023
It’s been some time since I shared where Neil is at in his medical journey. Batten disease is a relentless beast, yet somehow our boy continues to amaze us.
Our brave, brave boy teaches us how to be in the moment, because that is the only certainty any of us have. With everything he has to endure, he somehow perseveres through it all and remains himself.. his beautiful, funny, charismatic self.
He is not letting batten disease define him. I am working on not letting batten disease define me.. define my reason for being. I try really hard to be his mom first, and his caregiver second.
As I mentioned in prior posts, his treatment is not a cure, but it does slow the progression of the disease. So, yes, he is slowly regressing, but we are too close to him to see it.
I often ask family and friends to tell me when they see changes in him. I ask them to tell me, but it’s so damn hard to hear.
Mike and I tend to follow a bit of a pattern when coming to terms with his regressions.. It really is a process. We grieve every decline. It’s really hard to accept that pieces of him are fading away.
So, our beautiful boy is now 17! and he recently past his 150 infusion milestone. It feels a long way away from the boy who started treatment when he was 11.
Because of the disease, he has the gangly body of a 6ft, 17 year-old, but he maintains that innocence of the very young.
He still believes in Santa and the tooth fairy.
He loves his Marvel superheroes.
He’s still a giddy Montreal Canadiens and Blue Jays fan.
He wants to spend time with us and he still asks for hugs every day.
I know most 17 year-old boys aren’t asking their mom for a hug, so I cherish what we have.
When he was diagnosed, we were told he wouldn’t make it to his 16th birthday. It was so inspiring to beat that milestone.
We know there is no way to predict his future, so we really do try to live one day at a time and make the most of every moment with him.
The friends he played with when he was young, (before his diagnosis) are all about to graduate high school. They’re getting accepted into university and college. They’re getting ready for prom. I remember being that age. It was a time that felt kind of magical. It’s a time of best friends and first loves…first jobs, drivers licenses, midnight curfews, independence, nostalgia.. growth. It’s a right of passage for all of them, and for their proud parents.
Neil’s never going to get to experience any of this.
I knew this time was coming.
I thought I was prepared for it, but I’m struggling and it hurts.
Neil’s never going to leave this nest. Every facet of his life requires hands on support and help.
It’s more than just transfers and a wheelchair. It’s dressing, bathing, toileting, teeth brushing, putting on deodorant and shaving.
It’s gtube feeding, thickened liquids so he won’t choke and aspirate.
It’s a communication device because most people can’t understand what he’s saying.
When I mention declines, they happen in all of these areas, and more.
They happen in his speech - he will eventually lose his ability to speak altogether.
They happen in his fine/gross motor skills- he shakes and can’t hold his hands or his head still - eventually he will lose the ability to grip most things.
They happen with his vision - eventually, he will go blind.
They happen internally - we’ve been told that it’s a miracle he hasn’t had aspiration pneumonia yet, because every time he swallows liquid or saliva, a tiny bit drips into his lungs - eventually, he will most likely get it.
They happen mentally- this one’s tricky because most often, he seems engaging and aware - but he is getting more prone to magical thinking and believes he IS the characters of his imagination.
There are days he swears to me that he IS Thor’s son (from Marvel), and that he IS a Montreal Canadien player that just got a hat trick, and that he DOES own 3 mansions - eventually, he will slip further into dementia.
Not knowing ‘when’ declines will happen, and how severe they will be is challenging. Mike and I have had long discussions with medical team about what to expect for his future.. and what further degeneration will look like.
Eventually, his symptoms will become so pronounced that he will no longer qualify for treatment (he gets tested every 6 months.. if his ‘score’ slips, the Ontario government will no longer fund his infusions, which cost approx. $30K per/infusion).
If that happens, his disease will likely speed up and progress until it takes his life.
Some batten kids (even the ones on treatment) have contracted aspiration pneumonia, and that weakens their bodies and spirals them into a quick decline and untimely death.
Some batten kids, even the ‘healthy ones’, die in their sleep far earlier than was anticipated.
So amongst all the uncertainty that batten disease presents to us, we are literally forced to live. And we can only mentally survive it by taking it one day at a time. By loving him right now. By letting him know how precious he is to all of us.
Every single night, as I hug him and hold him extra long, I can’t stop that thought from running through my head, that this could be the last hug.
No one knows when their time is up, it’s difficult to live each day with that constant reminder that Neil has a life ending illness.
They say that batten disease is “the long goodbye.” Since he was 11 yrs old, we have not had a day where it wasn’t at the forefront of our minds. So if there is anything I have taken from this experience, it’s that it is SO important to do what you want with your life - and do it right now.
Take nothing for granted. Live each day as if it were your last and love your people with no limits… and tell them that you love them!
Right from the beginning, we have had so many people who have given us support, and we are deeply grateful.
Providing financially for Neil is expensive. Trying to do so while living on one income can be scary. The financial support literally keeps us afloat month to month.
The emotional support friends and family give us is monumental to our mental health.
This journey has taken us deep into the depths of sorrow, but it has also gifted us with the highest peaks of love and even joy.. joy that we had never fathomed existed before Neil’s diagnosis.
We have seen the absolute best of humanity, and we have been the recipient of so much love. THANK YOU… All of you, for supporting us and for continuing to follow along in Neil’s journey with CLN2 batten disease.
April 2022
I write this letter with a prayer in my heart that Neil would come through his upcoming surgery with good health and healing.
My name is Angela Wolfram. I’ve been Leah’s best friend since our sweet sixteen birthdays. She doesn’t know I’m writing this letter. Asking for help does not come easily or naturally to Leah or Mike.
Two important events are taking place right now with the Brochu’s:
1. Neil needs to have an emergency brain surgery first thing, Monday August 30th
2. Neil’s most recent decline has greatly altered his abilities. The Brochu’s urgently need their van to be fully converted to a wheelchair accessible vehicle
(at a cost between $15 - $30 thousand dollars.)
With Neil’s decline he is unable to independently transfer from his wheelchair to the van seat. Leah has been supporting Neil’s weight, lifting him to his seat despite needing regular back epidurals herself. This is an accident waiting to happen.
Like you, I’ve watched in awe as the Brochu family navigates obstacles with Neil’s declining health with grace and courage. Despite the stress this brings, Mike and Leah are forever nurturing parents with unconditional love and unlimited patience.
They don’t ask for much although their need is great.
I know we can work together to help the Brochus’ get a Van lift for Neil.
Thank you so much
Angela Wolfram
It’s been an action packed few months for this kid!
In October he got his new port placed in his chest (this is the port that accesses his cerebral spinal fluid via a tube from his brain.. it’s accessed on infusion day)... then last Wednesday, almost 3 months to the day, he got his feeding tube (G-tube) placed!
Recovery from the port placement was a little tough on him, but his recovery from the G-tube has been phenomenal! He’s been tolerating the feedings perfectly, with very little pain from the surgery.
Neil can still take food by mouth - it’s just that he has been having more and more choking incidents (another symptom of Batten Disease, is the slow deterioration of muscle tone in the mouth, jaw and throat).
He has been struggling and having choking incidents with food for awhile, but just within the last couple of months, he started choking on liquids. That was scary - he had an x-Ray video swallow study done, that showed that every time he took a drink, drops of liquid we’re getting into his lungs. We were told by the specialist at the hospital that he “should have pneumonia” with the amount of liquid getting into his lungs - but his angels were with him, and thank God, he didn’t.
We are now using a thickener to help him avoid that when drinking, going forward.
So, as a result of his swallowing issues, he was losing weight. Every time he put anything in his mouth, he feared choking on it.
He had been on the priority g-tube surgery list at SickKids for many months (the backlog was due to Covid), so his turn couldn’t have come at a better time!
He never ceases to amaze me - no matter what this disease puts him through, his sunny personality continues to shine.
Through it all, his humour has remained at top form, and even in the hospital, just after his surgery, he had his nurses and doctors all laughing regularly.
It was a bit of a juggle at SickKids due to Covid. They’ve tightened safety measures even more than before .. currently, they allow only one parent in the building at a time (even for kids who are admitted into a single room) .. it was pretty funny seeing all the parents (us included) handing off kid-duty in the freezing cold outside the building, with big burly security guards on the look out, like bouncers!
The learning curve on the g-tube is pretty steep
for Mike and I, but so far, so good!
We opted to have me attend the g-tube parent class (again, only one parent permitted)..
I was completely overwhelmed that day - feeling WAY out of my comfort zone, but as long as none of the horrible things they had to show/tell me about happen, I’ll be fine (knock on wood!!).
And even if they do happen, they gave me all kinds of resources to reach out to.
Outside of his medical excitement, Neil’s been enjoying attending school from his big comfy chair at home over the last many weeks. And I have been close by just in case he needs help while on his computer.. I’ve been so close that I have heard everything that goes on in a day at school - and I must say, the next time Neil complains about going to school, I will have quite a laugh, because holy cow, school was NEVER this fun when I was his age.
His phenomenal teachers and EA’s make SUCH a fuss over him - that kid is SHOWERED in love from them. They do so much to acknowledge him, and make him feel valued and loved. They do the same for all the kids in his class. We are so grateful to them.
We are also forever grateful for his bi-weekly enzyme replacement therapy treatments that provide us with precious time with our boy.
2020 was hard on him, and it was heartbreaking to witness, because he did experienced some significant declines. We do truly believe however, that the g-tube will help get him to a place where his body is healthier and stronger - and hopefully, will help him maintain his current health and lifestyle.
We know that Neil leads an untraditional life - and that his future is unknown.
We do also know that he is a rare and precious gift and that we have been honoured with the privilege of safeguarding and nurturing him for as long as God sees fit to let us have him.
I have to believe it like that.
Because otherwise, I will go crazy trying to change the unchangeable.
Keeping that at the forefront of our minds allows us to keep the mindset of ‘one day at a time’.... Besides, anyone can handle whatever their “it” is, when they break it down to just one day at a time.
Thank you so much for your continued love and support of Neil, Mike and I.
I don’t know if updating through this gofundme page is the best way of sharing with everyone.. I just know that so many people who donated to us over the past 3 years have asked to be kept up to date on how he is doing - and I know I don’t have everyone here in my Facebook friends list .. I should probably do this via blog or website - ugh, I’m just not that organized yet!
Regardless- I know it’s been a while, and I thought this would be a great time to share now that we’re home from the hospital ❤️
Thanks again to you all.
Stay safe and healthy!
Leah, Mike and Neil