Three is the Magic Number

 

Beep, beep, beep, beep. I roll over blurry eyed and stretch out an arm to snooze my morning alarm before work. After some feeling around in the dark I realise it's not my iphone waking me up and it's only 3am. I turn over & Kayli isn't in the bed beside me. A lighter sleeper than myself, she's already in Oli's room turning off the alarm. This time around he's managed to pull the face mask off his ventilator that he sleeps with through night. It's a CPAP machine that helps regulate his breathing while he sleeps. Other nights it can be a problem with his feeding machine; he's knocked it over, there's a kink in the tube or it's out of battery. We give a machine feed which runs constantly & slowly through the night. Pumping a liquid based feed into his gastrostomy tube while he sleeps.

Seeing Oliver hooked up to several machines through the night was unnerving to begin with. It leaves you on edge knowing that you're relying on everything working as it should, but it's near enough all we've ever known for Oli and has become commonplace. The same can be said for the majority of Oli's daily routine. 

The morning starts with his breathing routine to help clear his airways. A couple ml of saline drops in his nebuliser. A mask that converts the liquid into a mist, which Oli inhales. This helps to clear his lungs. We follow it up by two puffs of a blue inhaler via a face mask. Next we use a cough assist machine, which pushes and pulls air into the lungs to clear out any mucus. This is particularly needed at this time of year when common colds and flus begin to appear. Afterwards he has two puffs of his brown inhaler; a preventative steroid to help keep lungs strong. We then brush his teeth and follow this up by using his suction machine to suck any excess saliva and toothpaste from around his mouth, which he can't swallow on his own. Twenty minutes later and he's good to go.

Whilst Ava's eating her bowl of cereal her brother is starting his own breakfast routine. One which involves eight syringes. None of which he receives orally. They are all given through his feeding tube, which goes directly into his belly.

First we give him his 'flush':

- 7.5ml clobozam to manage seizure activity

- 3.5ml keppra also for epilepsy

- 10ml lanzoprazole to limit reflux

- 10 ml digestive enzyme

- 5ml elderberry syrup

- 60ml water, coq10 and a laxative to help his bowel movements

We wait half an hour to ensure the medicines have been dissolved properly and then Oliver has 2 more syringes full of feed. He eats a lot of the same foods we do (although allergic to eggs & peanuts); we just combine a mixture of different foods up together in the blender. 

It's a lot to stay on top of. All before 9am.

We'll repeat this medicine/feed process four times through the day.

Add into the mix the time for preparing medicines, warming feeds, cleaning and assembling equipment, carrying him between various rooms of the house, getting him strapped into his stander, giving him repeated chest percussion physiotherapy and suctioning his constant reflux (excess saliva & mucus) and it's very much a full time job. One at which Kayli excels through the week. Working from home of late has opened my eyes yet further to what an incredible job she does.

There has been a relative calm over the past year. As the blog title says we have been 'learning to live with Leigh's' as a family. I haven't updated my blog during this time as we've gotten into a smooth routine of every day life, managing Oli's illness as best we can. We're fortunate to have not had any serious hospitalisations, only routine check ups (the picture below shows Oli going for a recent brain scan) and, for the most part, Oli has been in a constant state of good health, as good as can be for having mitochondrial disease.

We celebrated Oli’s 3rd birthday last week. THREE!
It was a bittersweet day, one of happiness and pride, but the sickening reality is we don't know how many birthdays we'll get to share with Oliver. 
Sadly, many little boys and girls similar to Oli will never get to see three candles on their birthday cake. Life expectancy for a child with Leigh Syndrome is 2-3 years and presently there is no cure.
The earlier the onset, the shorter the lifespan.
I had approached the day with intrepidation. Three was always a significant milestone for me in Oli's life. For the most part Kayli & I have stayed positive and ignored the 'facts' of what he will & won't accomplish and in what timeframes. But with the 3rd birthday approaching I couldn't help but remember some of my early readings into the disease from when Oli was first diagnosed. 
'Life expectancy generally 2-3 years'
At times when he was regressing or we were told he wouldn't have long to live, three was always the marker. I've found myself in a blurred state of living for each day tinged with living for the next milestone. Although I don't say as much, in the back of my mind I'm thinking 'come on Oli, make it through to another Christmas with us'. Or 'I hope he's still well enough to be able to do this'. I hate when those thoughts creep in. It's unfortunately the harsh reality that every mito parent faces. Living day by day.

There was a moment of sadness first thing in the morning on his birthday. The three of us all went into Oli's room to wake him up.

After several minutes in his bedroom, a sombre looking Ava skulked out of the room looking dejected. 'What's the matter, Ava, are you ok?' 'No. Oli's 3. I thought he was going to be able to walk & talk.'
She was recalling a conversation from a nearly a year earlier in which she had asked us when her brother was going to be able to walk & talk, to which we gave a hopeful response of 'I'm not sure Ava, maybe when he's 3.'
Without knowing it at the time, it was a significant piece of hope that Oli's big sister clung to. Ava took the literal meaning and had hoped the moment he turned 3 she would have her brother walking beside her. It's in those moments you realise what an impact Oli's condition has on those within his life.

COVID-19 has added an extra layer of complexity to Oliver's illness.

Parents of mito kids dread the winter months. Mitochondrial disease is degenerative and any sign of flu can wreak havoc in the little bodies of those children that have it. 

As a quick refresher on the science side; Mitochondria are the part of our cells responsible for creating more than 90% of the energy needed by the body to sustain life and support organ function. When a person has mitochondrial disease, their mitochondria are unable to completely burn food and oxygen to generate energy, which is essential for normal cell function. Over time, without this energy, organs begin to fail. The parts of the body, such as the heart, brain, muscles and lungs, requiring the greatest amounts of energy are the most affected.

This has left Oliver, and countless others like him, particularly vulnerable this year more than ever. The coronavirus outbreak ensured we were mostly housebound, bar exercise, for around 4-5 months this year, heeding the doctors warnings to keep Oli 'shielded'.

Not all storms come to disrupt your life though, some come to clear your path. Whilst a COVID enforced lock down has left us feeling isolated, it has brought with it countless blessings in disguise. None more important than the dedicated and focussed time we've spent with just Oli and Ava. No distractions.

When at times in the past we haven't been able to see the forest for the trees, 2020 has at least allowed us to slow things down and appreciate the smaller moments.

That being said, the more dedicated time we spend with Oli, combined with the progression of his disease this year, only serves as a reminder of the complexity of his situation. His disabilities become more apparent as he grows and every day tasks become more difficult.

His biggest recent regression, which we're hopeful we'll see improvements in soon, has been with his reflux, linked to picking up a cold. 

Oli's been having a hard time clearing his secretions. A pool of saliva and mucus forms in the back of the throat, and at times Oli won't have the strength to clear his throat or swallow. We've had several hairy moments just in the past few weeks where he will begin to choke on his own saliva and his face will start turning blue. We have to be on hand to help clear his airways by patting him on his back and chest or by constantly using his suction machine which pulls out the spit to help him breathe.

It's painful as hell to watch our little boy go through this turmoil, but he battles it with uncompromising strength and an unwavering happiness. He's had some tough challenges to face in his three short years of life, but he’s done it all with a constant smile on his face. All we can do is protect him and show him love, cherish every day.

Life has an unexpected richness to it now. It has opened our eyes to what is important and what we hold dear.