Tag Archives: PCH

Introducing Benjamin

Introducing Ben to new people isn’t something we do as often as we used to; we have great family connections and have surrounded ourselves by the same people for most of the past nine years.

As I transitioned into a new job this year, it has been interesting and slightly funny (for me!) to try and explain Ben’s diagnosis without it sounding as dire as it maybe is.

Ben is an anomaly; he is one of a kind. There are still only a handful of people officially diagnosed with his type of PCH and when I create that visual for a new acquaintance, I don’t think they always know how to react.

I just want to clarify – it’s not sad for us. We are no longer sad or upset by his diagnosis.  We have sad moments and stressful times but we have mourned the son we thought we were going to have and now celebrate the amazing person Ben is.  That ship is so far gone for us; Ben is exactly who is meant to be and we’re okay with that.  So you can be too.

New people in my life have expressed sympathy over our “situation” – what situation? I appreciate your sympathy but really don’t need it (not meaning to be harsh, but this is our everyday… it’s just reality).  And I definitely don’t need your pity so don’t even go there.

What I do need is for you not to judge me when I come to work with a big mess of drool on my shoulder or if I wear the same thing for five days straight because my priorities don’t often include my wardrobe.

Explaining Ben to new people actually makes me feel bad because I know how it sounds.  It sounds tragic and terminal and unfortunate.  I have heard myself say several times “It’s really okay” or “It’s not as bad as it sounds or looks on paper.”

I do sincerely appreciate those who express curiosity or want to know more about him; I’m happy to answer your questions and share his life with anyone who wants to be a part of it. I can talk about Ben for hours if you want.

For some people, I believe that it is difficult to imagine that kids like him exist. But he does.  And there are other kids with rare conditions whose stories are similar; some of those stories are happy ones and some are sadder or shorter.  We are the lucky ones…

We are lucky to have a very happy, mostly easy going child, who considering the adversity and challenges he faces daily, soars through his life smiling and laughing and smiling and laughing… and sometimes biting.

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Concerns regarding FSCD funding process – a letter to MLA’s in the disability sector

Good morning!

I am writing you this morning as I am extremely frustrated by the approval and decision making process regarding services requested for my son.  We, as a family, absolutely do not ask for anything more than we need or our son requires and ensure that we do not abuse the system put in place to support children like ours but we have come to a standstill with our contract with Family Supports for Disabilities.

Our son Benjamin has a rare neurological disorder called Ponto Cerebellar Hypoplasia Type 3; I’ll spare you the details as I’m sure you will just Google it if you’re interested but what it has meant for us and more importantly him, are countless nights of not sleeping.  I’m not exaggerating when I state that our child does not ever sleep in a pattern or that he is up for hours at time each night.  In addition to that, he is supplemented food through a gastrointestinal tube which puts him at risk for aspiration and choking due to reflux and sensory issues.  For the past five years, my husband and I have tagged off and on to support Ben at night, taking turns sleeping the couch, watching Elmo with him for hours at a time or simply just playing with him but it’s come to a point where we are feeling burnt out.  I feel we have made a good run at trying to do it ourselves.  But the worst part of our exhaustion is how it relays to Ben; he definitely can feel the frustration when he’s been up  since one in the morning and we’re tired.  We try our best to be patient and accommodate him but for lack of better word – we’re pooped.  This is not a normal circumstance. We want to be the best parents for him, provide him the best quality of life and to ensure that he’s happy and to do that, we’ve applied for over night respite assistance and have even registered and qualified for the service from Nurse Next Door (a private company) to watch Ben through the week 10p.m. – 6 a.m.

However – FSCD will not approve the funding needed which I do not understand.  How is my child not considered as valuable as they funding requested?

I understand that FSCD may look into using the Supports Intensity Scale for future assessments and when that happens, Ben will without a doubt receive the financial supports he requires based on his medical and behavioral assessment alone but until then, I don’t believe that asking for overnight respite for him is much to ask; it will allow us to be our best for him during the day when we can engage him in his community, include him in social activities and allow him to have fuller days.  How is that not worth what we’re asking for?
Please point me in the right direction…. a direction that allows us to provide nothing less than the best support possible for our son.

Thank you for your time and consideration.

Regards,

Kristi Hall-Busque

Two years old and she knows…

Ben has not been feeling well lately and though is on what could be a permanent anti-biotic for his constant ear infections, guess what… he has a massive ear infection.  My poor boy just wants to curl up on his mom (or Nana when there) and spit, drool or flick his mouth (which is how he self stims/relaxes).  Last night, we’re curled on the couch with Poppy watching a Curious George Christmas special and she turns to me, asking if Ben is sick.  I told her yes, which is why he’s so quiet and curled onto Mommy.  She then tells me Ben is her big brother but that she is bigger than him.  Yes, that’s true Poppy.  A few minutes pass and she tells me that Ben is different – he’s not a baby but he’s small like a baby.  All her words and again, true.  “Mommy, Ben is our special boy.”  Yes, even more true.  Then she curls up along his back and plays with his hair and tells him she loves him.

 

She adores him, is his best friend, little sister and protector…

She’s two years old and she knows.

Another thing to add to the list…

Last week, Ben was put into his standing frame in his AFO’s like always and lasted about thirty minutes before he had had enough.  When he was removed from his stand, the muscles in his ankle and calf on his right foot started spasming and continued to do so for about an hour and a half.  Scary stuff.

This morning we met with our Physical Medicine doctor, Dr. Watt (who is very cool!) and he informed us that this type of spasm is actually called Clonus.

Clonus as defined by Wikipedia is:  a series of involuntary, rhythmic, muscular contractions and relaxations. Clonus is a sign of certain neurological conditions, particularly associated with upper motor neuron lesions involving descending motor pathways, and in many cases is, accompanied by spasticity (another form of hyper-excitability). Unlike small, spontaneous twitches known as fasciculations (usually caused by lower motor neuron pathology), Clonus causes large motions that are usually initiated by a reflex. Studies have shown Clonus beat frequency to range from 3–8 Hz on average, and may last a few seconds to several minutes depending on the patient’s condition.

 

Sounds fun right?  Hardly. 

I am definitely starting to get the hang of this diagnosis stuff; I predicted that it was a neurological thing.  And in that of course means we now have to schedule a follow up appointment with Ben’s neurologist. 

It’s definitely that time of year – November through February is appointment time.

Still Affected

Ben had his foot surgery… months ago now but today when I was slathering him in lotion (mostly because he loves the tickle of it), I gave him a quick foot massage and he winced.

Where he had the pin and infection in his foot is a small little red indent.  It’s clearly sensitive.  And I am also clearly still sensitive because I burst into tears as I felt him pull his foot away.

I am still so angry that we let this happen to him and although there is no fault in it – shit happens… as his mother and protector I am furious.  And I am sad for him.  And I wish he knew how sorry I was that I let him hurt like that.

I’ve said it before, but will say it say it forever… never again.

Unbelievably Grateful

I’ve tried to write this post so many times but then I start to cry and can’t think of anything clever or properly thankful to write.

Francois and I are completely overwhelmed and appreciative of all the support we received over the past eleven days.  When we started fundraising for Ben’s equipment we figured nothing bad could come of it and maybe we could get a little money to help us out.

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YOU have no idea how much the smallest of contributions has made.  YOU who donated to us, have no idea how in complete awe we are of YOU.  We are completely grateful for YOU.

We cannot say it enough so here it is one last time.. we sincerely thank each of you – those who contributed, those who shared on social media, those who passed along Ben’s message, those who hold him, care about him and love him as much as we do.

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The Sickness Cycle

Ben, like many children facing similar challenges, has a sickness cycle.  He’s healthy for a month or two, then sick for a month.  Then healthy, then sick.

In February, we had our first unplanned Stollery stay and ended up there for a week.  Benjamin wasn’t tolerating feeds – orally nor bolus feeds and it quickly became a scary situation.  As everyone associated with him knows, weight loss/gain is our biggest obstacle and ensuring that he remains healthy within such great limitations is something we face daily.  I made an appointment with Dr. Chatur to get him checked and knew that the chances of being admitted were about a million percent (yes, that exists.  You can be that sure something is wrong with your child that is not fixable within your own means at home); I brought packed bag for the both of us.  By that afternoon, we were nestled into an emergency room bed.. then a few hours into the trach room (adorable little ones pulling out their tracheotomy tubes!) and then into our isolation room.  It was determined that Benjamin had caught some sort of viral thing and would need to be isolated to make certain he didn’t infect others.

The main concern while there was his hydration levels and for six days straight, he received a saline line into his foot – we’ve never seen him so puffy before!  We continually tried adding formula to his feeds but it took him up until day four to begin tolerating them and even then, at a slow slow slow pace.  Finally, an entire week later we were able to leave the hospital even though Bug still wasn’t quite back to normal.  Still, a few weeks later he was showing signs of a new virus and sure enough, he caught Bronchiolitis (infection in the lower lung).  We ended back at the Stollery on a Saturday night but fortunately the doctors did some testing and sent us home with the same plan we left with last time.  BUT with the exception of us having to follow up with Dr. McGonigle/Dr. Chatur during the week.

By far, those few weeks have been the most trying for us a family.  It’s a difficult situation to live in daily, unsure of exactly how Benjamin is being effected by these constant colds and need for medication.  We know he has troubles identifying pain so everything remains a guessing game.  Most children, when they get sick will “sleep it off” for a few days.  When Ben starts sleeping too much in a day, that’s a clear sign that we need to take him in.  He rarely holds a fever for than a few hours at a time because his body doesn’t regulate itself so even though the thermometer may not indicate it, he could be feeling awful.

Since the stay though, we have discovered one new, amazing trick.  In hopes of keeping him hydrated under any circumstance, we were only giving him water at night instead of his formula.  The water seems to be benefiting him in more than just that way – it’s created an increase hunger during the day and he’s now taking up to three bottles a day instead of fighting to get him through one.  Huh!  Who would have thought!

The above being said, we are extremely grateful for our family, friends, doctors and nurses who managed to keep us sane that week. You are all incredibly lovely and so much appreciated.