S.M.A – when the fact sheet becomes reality

Spinal muscular atrophy – three words that changed our lives forever. Three words that still feel like a massive, medical mouthful to say. Three words that seem far too serious and heavy to be associated with the fun-loving, light hearted little boy I know. Three words that still cause blank expressions, glazed eyes then shock and sadness when you explain the conundrum of what these three words actually mean.

Today is sma awareness day and it’s very easy to read a few fact sheets, check out some statistics and share a few neat graphics but what happens when you are the statistic, what happens when you’re living the fact sheet and what happens when the neat graphics depict your life? Well here’s my take on our family living the fact sheet for real:

The fact sheet:

  • Sma is a rare neuromuscular condition that causes progressive loss of movement & muscle weakness.
  • 1 in 40 of us carry the gene that causes sma.
  • There are three main types of sma – type 1 is most severe and children of this type will rarely see a second birthday.
  • Sma is a life limiting condition.
    There is currently no cure but there is now a treatment aimed at halting progression.

Living the fact sheet:

Sma causes progressive loss of movement – the reality of this statement is both terrifying and heart breaking to watch. When our little Zac was around 7 months old we noticed he stopped rolling onto his side to sleep, then he stopped rolling over all together. He never reached the milestone of sitting up and we watched him struggle for months with trying to keep himself up in his hi-chair and buggy before we finally managed to get a diagnosis. We saw his little legs lose all movement and we watched as he lost strength in his arms. It’s heartbreaking to say some of his first sentences were “I’m stuck”, “help me”. Worst of all we witnessed rapid deterioration of his respiratory muscles and we’ve helplessly sat by a hospital bed too many times while he’s battled with every fibre in his being to keep these muscles working. That was the reality for us but thankfully a new part was added to the fact sheet and a new treatment was discovered. We received a miracle and Zac started treatment – thankfully since then we’ve seen amazing progress and we’ve celebrated Zac getting stronger but it didn’t happen over night or by magic. We had to learn to be physio, nurse, carer as well as parents. We carry out daily stretches, exercises, breathing exercises, water exercises, positional changes – all designed to keep Zac’s muscles working and be as strong as they can. Then to best support all this work a multitude of equipment takes over your house; comfy chair, wheelchair, stander frame, bath seat, toilet chair, wizzybug, comfy mats & support cushions, breathing machines, feeding pumps & tubes. A cupboard needs set aside just for medicines & special feeds and pretty much the layout of your house needs designed around all of this stuff!! Daily life revolves around treatments, hospital appointments, home appointments, medicines and feeds as you fight daily to slow down this progressive loss of movement.

Next you deal with the fact that you are the statistic on the fact sheet – we are the 1 in 40 who are carriers of the gene that causes sma. This comes with its own journey of emotions (see my previous blog “learning to love your genes”). When you become the statistic and you become “sma parents” you devote your life to caring for your child. You stay up late researching medical terms, medical advances and all that surrounds life with sma. Your priorities in life change and you now focus on making it through the every day. Your financial situation changes, work situations change, your friendship circle becomes smaller and your life is somewhat ruled by this condition. But being this statistic has made us stronger – we’re a team; we’re both exhausted, both give everything we’ve got, we face the challenges together and celebrate the miracles together. Sma makes very different demands from both of us but each ones demands are equally essential to making our “different kind of normal” work.

Our whole life was thrown into a whirlwind that day we heard those three words – spinal muscular atrophy. We’ve become the fact sheet but the fact sheet can only give you words, the fact sheet cannot possibly convey the incredible human spirit that the faces behind the facts have. The fact sheet tells you that physical strength will deteriorate but sma warriors have the most super human strength of character, their determination is inspiring and they are far more than their condition.

So next time you see a fact sheet with those statistics & neat little graphics, remember there are real people living it and a whole lot more goes into daily life than just the sentences on the paper. We are a family where the fact sheet has become reality and now we’re living our “different kind of normal” to the best we can.

Advertisements

A crazy week with the Cams

Life with my two crazy boys is always full on & this week was no exception! Nothing out of the ordinary – just the usual appointments, phone calls, school runs & every day care that has become our “normal” but for some reason this week has felt crazier than ever so here’s the highs & lows of a week with two “different kind of normal” boys:

Monday: The week started slowly like most weeks – Monday morning is usually our time to catch up with everything after the chaos of the weekend. Zac has some weekly care that needs done for his tummy button so we make that a Monday morning job & we catch up with housework, look through the calendar & diaries and make sure we’re set up for the week ahead. Monday was fairly uneventful – just the usual daily physio, medicines, feeds and everyday routine that needs done.

Tuesday: School mornings are always a rush – Zac’s chest physio, meds, feeds then Caleb’s breakfast, uniform and we absolutely MUST leave the house at 8:40am. Leaving at the “wrong” time results in Caleb’s whole day being “wrong” so now at 8:39am we drop everything, there is no talking and we watch the clock until it turns 8:40am and we leave for school. School run done and time to prepare for hospital appointment. We head to Glasgow for Zac’s respiratory review and all goes well. We make it home for tea time and I’m just in the door when I have a phone call from psychology to discuss strategies surrounding food & sensory issues. There is a big pile of ironing sitting in a basket but I decide once the boys are in bed that “bake off” is a more worthy Tuesday night task.

Wednesday: This is the day I go to “real work” although I feel I’ve already done a full shift at home by the time I even get to “real work”. These days out of the house are supposed to keep me in the real world, keep me sane and give me another purpose – the reality is the only thing I can focus on on Wednesdays is making it through my “real day”, trying to do an okay job & I usually just feel exhausted after being on my feet all day. I get home, quickly have dinner then it’s time to start the bedtime routine for the boys.

Thursday: I have a meeting with complex care & social work and despite me feeling very anxious about home care 6 months ago, I now find myself requesting additional hours of care & some weekend time. Part of me still feels guilty about this but the other part of me needs the time out – I guess this is a dilemma I will always face. Meeting over, schools out & my grandparents come to visit. Then the “hurricane” hits and I’m right in the middle of an aspergers melt down. It could be something different has happened in school, the excitement of family visiting, the snack wasn’t right, the noise level…….. there are endless possibilities but we’ve gone too far to try and reason it out and my job now is to try and calm the storm. We’re past the point of our normal cool down technique so now I just have to let him safely scream, punch & run it out. The window cleaner turns up at this point & I’m very aware I must look like an awful mum – one of my boys is in his pants screaming the place down while the other is wizzing around in his wheelchair & I’m standing somewhere in between the two waiting for this melt down to calm. The normality of the tea time routine finally brings the melt down to an end but by this time we’re running behind & the hubster returns from work to me saying “please let’s just get to 7pm with no hassle”. A glass of wine was poured at 8pm!

Friday: It’s a new day and all is okay with the world again. We have a morning physio session with Zac where we decide with the physio’s advice to scrap Zac’s stander and we have some new things to add to our daily routine instead. Zac is pretty tired after all the work out so he has a chilled afternoon with a few movies, I finally do the pile of ironing that has multiplied since Tuesday and me & Craig get an hour to ourselves when Zac’s home care arrives. My amazing parents then take Caleb for a sleepover so it’s a quiet night with the tv.

Saturday: With our big boy having the day with nana & popo we decide it will be easy to face the Saturday shops with just the wee one. Big mistake – it turns out none of my boys are great with shops and after setting up wheelchair & feeds we don’t get very far before there’s tantrums from Zac!! After a few hours of trying to pacify our three year old we leave the shops having only purchased a bat man toy car and a pumpkin spiced Frappuccino. Zac falls asleep on the drive home and me & Craig sit in the drive way for 10 mins – one boy with the grandparents & one asleep – winning at parenting!!

Sunday: Well we’ve made it to the end of the week and yep it’s been a crazy, exhausting week – all of this craziness goes on as well as all the normal stuff – washing, ironing, cooking, working, homework, house work, shopping…….. but this is our life & it wouldn’t be ours if it wasn’t crazy! Our life is filled with challenges & crazy happenings so I’m ready to face the next week & let’s see what our “different kind of normal” brings!!

Zaccy vs sleep studies: lessons learned from a 3 day sleepover.

Last week me & Zaccy spent 3 nights in hospital for sleep studies. Sleep studies are a part of Zac’s life with sma – as his muscles are weaker they can become more relaxed than normal during sleep so it’s important he gets his oxygen & carbon dioxide levels checked every so often to make sure he’s breathing efficiently during sleep. This time we were going to get a review of his night time breathing and we were aiming to set up ventilation called “bi-pap” – without being too technical this is a machine with a mask over the nose that delivers big breaths as you sleep keeping the airways open. The thinking behind it is that Zac’s chest shape would improve using it as he would be breathing more efficiently and should help him have more of a rest at night as the machine would help do the hard work! That was the plan, however, Zac never likes to follow a plan; after three nights of broken sleep we were discharged minus the bi-pap machine. So here’s the lessons I’ve learned from our little sleep study:

1) No-body sleeps much during a sleep study!! Your baby is hooked up to a few monitors, little stickers are dotted around their wee body and laser sensors are attached to ears, fingers & toes. It’s not much fun trying to sleep with all that gear and every time your child wants a change of position these clips & sensors need moved too. Then the monitors alarm and sing at you every wee while and the nurses pop in & out too so yeah not much sleep actually happens.

2) My child is mr chatterbox. On the third night we had the task of distracting Zac while we attempted to wear the mask for longer than 5 minutes. I found out that night that it’s possible to have a conversation about a few small stickers for a very long time – 2.5 hours to be exact! He definitely won the prize for “Chattiest in the ward” and won quite a few fans before finally giving in to sleep!!

3) Chocolate bars do not make a good breakfast. I found out that after around 4 hours of sleep a night it’s not a good idea to eat a caramel bar for breakfast – they do not give you the energy boost you desperately need and you feel like you could eat everything in sight for the rest of the day.

4) My little Caleb is amazing. Normally hospital stays are very difficult for Caleb – there’s too much change in routine and all the worry can be too much for him. Last week was different – my little Caleb took everything in his stride and was an absolute star! He coped with every arrangement and made me a super proud mama!! Although I was in hospital staying with Zac I learned a very important lesson about Caleb – he is learning to deal with his different kind of normal in his own way and I admire him so much for being the star he is!

5) I have a very lovely husband. As well as working his day job, taking meals out the freezer, recording my programs and bringing me chocolate, Craig also drove back and forth to Glasgow four times a day just so me & Zaccy didn’t have to stay in hospital all day. He very patiently listened to me as I went over every detail of the arrangements for each day & he stayed up to text me late at night when I clearly wasn’t getting any sleep at the hospital. I learned I really do have a top husband!

So after our 3 nights stay the doctor came to see us to let us know that Zac wouldn’t be taking the bi-pap machine home as they needed to look further into some of his results & machine settings. I looked & felt like a zombie & felt a bit like we had wasted 3 nights. That was a week ago & after catching up on some sleep I realised that the week hadn’t been wasted – I took more than just bags under my eyes home – I took home the fact that my little Zac had faced a challenge & coped yet again and that Caleb is adapting to cope so well and is a star! My boys made me so proud last week to be living our different kind of normal.