Finlay the Athlete

Over the past five years our family has been helped by a huge range of individuals and groups. In time we hope to be able to give back to as many of these as possible. Recently an opportunity arose to help one of these charities. Here’s the story of how it went:

I like to run. I’m never going to break records but it keeps me fit, gets me out of the house and, since Finlay has been around has been a great source of stress-reliefrecords but it keeps me fit, gets me out of the house and, since Finlay has been around has been a great : my me time. So every year I run in the local marathon. And each year, while I’ve been pounding the local streets, my elder two sons have run in the 3K kids’ fun run. And every year Finlay has waited at the finish line to watch his brothers finish and get their medals.

But this year he turned 5 years old and it was finally his turn. He would get to line up on the start line alongside his brothers and hundreds of other local children.

Each year the marathon adopts a charity which runners can raise money for. By chance this year it was Ronald McDonald House. During Finlay’s short life he has spent many weeks in hospital far from home. While these have been difficult times for us they would have been so, so much harder without Ronald McDonald House. Having somewhere to stay right next to the hospital rather than in a cheap, ill-equipped motel a bus ride away has been invaluable. To have a place that is built and run solely for the purpose of helping families of hospitalised children just lifts a huge amount of stress from our shoulders at a time when we need it the most. We owe them a debt we can never repay, but this year’s marathon gave us the chance to give a little back.

I didn’t feel I could ask for sponsorship for running a marathon as I’ve run them before and, although each one is a huge challenge, I think to ask people to dip into their pockets I should be pushing beyond my limits to justify the sponsorship. However, although my efforts weren’t worthy of asking people to give, Finlay’s were. After all he has gone through in his short life and everything he endures on a daily basis just getting to the starting line was a victory of immense proportions.

So we set up a sponsorship page for Finlay and soon had donations stacking up from friends and family. Social media is great for this kind of endeavour as word spreads quickly around the world from friend to friend. But then things got bigger than we imagined.

Ronald McDonald House, with our permission, passed his story to a journalist from the local newspaper. Each year they have extensive coverage of the marathon the day after the race with results and reports. And they usually have a feel-good story to accompany it as there is usually a plethora of people raising money for countless great charities, running in memory of relatives or in support of friends. This year, out of all the fantastic personal stories they chose Finlay to be their main piece in the print version with a nice accompanying online version.

And there was an amazing response with complete strangers donating money and even a lady from a city in another part of the country tracking us down and phoning us to tell my wife how wonderful she was! Weeks later I am still getting people at work telling me they saw the story.

The race itself was great. We didn’t know quite how his sugar levels would react so we turned down the rate on his insulin pump before the race and gave him a much smaller bolus than usual with his feed beforehand. It turned out we over-compensated as he ran quite high throughout the race and afterwards. But rather high than low and we got him back in range later in the afternoon.

Finlay sprints toward the finishing line

He ran really well with Mum by his side, although he slowed a little half-way round as his heart struggled to get the oxygen round his body. Day to day it’s easy to forget that his heart still hasn’t been fixed properly and I dread to think what his oxygen saturation goes down to when he exerts himself – the cardiologists assure us he will stop long before his heart does and so far that’s been the case!

Thanks to the lady from the charity that offered to hold Vicky’s bag while she was running not realising that its contents were half pharmacy, half sweet shop as it contained any emergency provisions Finlay might have needed. I’m glad to report the bag was never opened!

Also thanks to the marathon runner that passed by who thought the backpack with his insulin pump was a miniature Camelbak! That’s the second time that’s happened although he was just finishing a 42K race so can probably rightly claim fatigue.

All in all it was a great day, he raised over $1200 and, thanks to the local paper, we have a great souvenir to go along with his first finisher’s medal.

The boys proudly display their medals

Progress

I can hardly believe it’s been over six months since I last posted here. I’m glad to report that the dearth of posting is mainly due to there being no major dramas recently. Finlay has just been getting on with life and doing what 4 year olds are supposed to do.

So this is just an overdue update for any of you that check in from time to time to see how Finlay is doing.

We’ve been struggling for a while with weight or, more precisely, lack of it. The different aspects of his disease seemed to be conspiring against us to stop him gaining the pounds: we find a solution to get round one issue just to find any positive effects are nullified by one of his other issues.

So last year we made a couple of changes to his feeding and drug regimens and at last he is steadily gaining weight. Nothing earth-shattering but real, sustained growth. He has caught up with the bottom of the growth charts again, weighing in around the 3^rd percentile. Sitting here at 6’2” and just a few burgers short of 200 pounds it still amazes me that my son can be so small. But he’s never going to be a heavyweight and progress is progress. It’s really pleasing that our persistence is paying off.

So as I said in the first paragraph he is just doing what 4 year olds do. And that’s wonderful to be able to write! For the last six months or so, through the long, hot southern hemisphere summer, it has been possible to put everything about Finlay’s condition to the background. Never out of the picture completely (still multiple BG checks, quick calculations, boluses, medications: the never–ending grind of the chronic illness) but not the all-encompassing disease crisis management of the early years. We are now learning to view him as a growing, developing young boy. To look to his future with hope and excitement rather than trepidation.

And this year will see him take great strides towards that future. Our little boy is about to start school!!

 

The Finlay Clause

As parents we are the best advocates for our children. When a child has a rare disease that advocacy becomes all the more important. The nature of our children means that from time to time situations will arise where our children are not catered for.

This is what happened back in February when Pharmac, the government agency in charge of funding medicines and medical devices in New Zealand, announced plans to fund insulin pumps. This was a welcomed move as funding for insulin pumps until now has been on a piecemeal basis by different regional health boards  that has amounted to a bit of a lottery.

 However, the criteria to get funding presented a problem for us. Only type 1s would be funded and there were strict criteria based on maintaining HbA1c levels lower than pre-pump levels. As Finlay is not type 1 he would not qualify and as he was put on a pump only a few days after birth there is no pre-pump data to compare against.

So I found myself firing off a detailed email pointing out that their proposal would leave some diabetics without the care they need. Having never done anything like this before I had no idea what would happen. But earlier this month they published their revised policy and it seems they listened!

In what one of the diabetes nurses jokingly called the 'Finlay Clause' the revised decision now contains criteria for those with permanent neonatal diabetes to get insulin pumps and consumables. There is no requirement for these individuals to maintain specific HbA1c targets, only that a multidisciplinary team regards multiple daily injection regime as inappropriate and they consider the patient continues to benefit from the insulin pump. This is excellent news as it addresses my main concerns.

I’m sure their decision was not solely due to my correspondence, and I am aware that others had mentioned Finlay in their submissions, but I am delighted with my first foray into government policy consultation. In a separate document where they outline their responses to people’s submissions they specifically mention pancreatic agenesis in the section on neonatal diabetes, possibly the first mention of the disease in government policy documents anywhere in the world.

In my submission I mentioned three groups of patients: neonatal diabetics, those who have had their pancreases removed surgically, and Cystic Fibrosis patients who have developed diabetes as examples of non-type 1 diabetics that would be affected by their decision. I’m pleased to say that the first two groups are both now covered and CF patients may be included following a cost benefit analysis that is being carried out. In my mind it is a no-brainer. Hopefully Pharmac will agree.

So I am really very pleased with the outcome. Some of their other decisions around glucose test strips I don’t agree with and the strict criteria for type 1s to get insulin pumps may mean many will miss out but I am glad that with respect to my concerns they have listened.

The Amazing Pancreas

The pancreas is an amazing organ. It’s the unsung hero of the body. The body is rather like a team with each organ having its own specialised job to do: the heart pumps blood round the body, the lungs oxygenate the blood. But the pancreas is the utility player of the body, sitting quietly out of sight behind the big name organs, the stomach and liver. But there it carries out multiple functions key to the body’s survival. It’s not a one-trick-pony organ like some of its more illustrious teammates but a multi-skilled player deserving of far more credit than it receives.

The pancreas is responsible for supply and control of multiple hormones. Not just the all-important insulin but its partner in crime glucagon, as well as somatostatin and pancreatic polypeptide. On top of all this the pancreas produces many enzymes that help the body digest food: lipase, amylase, trypsinogen, chymotrypsinogen, elastase and carboxypeptidase. An impressive list for such an underestimated organ.

Of course all this makes living without a pancreas quite a challenge.

We have tried our best to replicate all the functions that nature perfected long ago but our attempts at being a pseudopancreas appear amateurish when compared to such an impressive organ.

The biochemists have given us the enzymes and drugs to help Finlay survive, modern medical technology has given us insulin pumps to finely tune our glucose control and the surgeons have aided us with 24-hour feeding access through a feeding tube. But it is one of the pancreases lesser-known functions that has left us struggling recently.

One of Finlay’s issues is his size. He is small. At 4 years old we can get him in 2 year old pants if we pull the elastic waistband really tight. Unfortunately fattening him up has not been easy. He grew reasonably well in the first couple of years but he has hardly put on any weight in the last year. And the problem seems to be an inability to digest and absorb his food. We can give him all the food and nutritional drink supplements we can shove in him. We can feed him through a tube when he’s asleep and we can give him lots of enzymes to replace the ones he can’t produce. But all this isn’t helping him grow. And it’s a clever little property of the pancreas that is to blame.

When food exits the stomach it is very acidic due to the gastric acid in the stomach. This creates a problem as the enzymes released by the pancreas to digest the food are destroyed by strong acid. So the pancreas releases bicarbonate (the same as baking soda) along with the enzymes to neutralise the acidic food and allow the enzymes to work.

So for our attempts at being a pseudopancreas we feed him some enzymes in tiny spheres that have a special coating that protects the enzymes through the acid stomach but which dissolve, releasing the enzymes, once they are in the less acidic duodenum. Unfortunately because he has no pancreas he has no bicarbonate released into his gut so the duodenum stays acidic. That means the little spheres carry on through the gut until eventually the environment is suitable for the coating to dissolve but by then most of the benefit has been lost and not enough food gets digested.

So what is the answer? With the rarity of Finlay’s condition there are no treatment guidelines, no established protocols backed by years of research and experience. We work on a combination of educated guesswork and…. well, complete guesswork. One strategy is to look at conditions with similar problems. In this case cystic fibrosis is a good model for what Finlay is dealing with. In CF the secretions from the pancreas can become blocked as the organs get covered in the thick gunk that typifies the disease. For many CF patients, reducing the stomach acid works well to reduce the acid environment in the duodenum and let the enzymes work.

So we have started a course of a proton pump inhibitor, a class of drug that reduces the amount of acid the stomach produces. Hopefully that will help reduce the acidity of the whole digestive tract and get more food being digested and absorbed.

Time will tell if this helps him put weight on but if it does it will be one more small victory in our efforts to replicate this amazing organ.

Living Up To His Name

When we named our children we just went with names we liked. Some people have names that run through the family. Others have strong opinions about what their children should be called. Some reflect the parent's aspirations, their religion or cultural background, others the parent's taste in music or TV programme. For us we just wanted names we liked that wouldn’t cause embarrassment as the children grew. There is a Celtic theme running through our boys’ names but that is coincidental rather than deliberate.

Fortunately there was plenty of middle ground between Vicky and myself that we didn’t spend months vetoing each others choices.  

One thing we didn’t really consider was the meaning of each name. But with hindsight it would have been hard to come up with a more fitting name than Finlay. Recently, following a discussion about names following the birth of a friend’s child, I found myself looking up names on the internet. Finlay, “fair-haired courageous one”.

I can’t think of a more apt name. This last week has seen our fair-headed warrior more than living up to his name. He has shrugged off surgery, a collapsed lung and an infection with a smile on his face and is now safely back home telling all his preschool friends about his adventure and the big planes he went on.

Every day he deals with the trials of his condition, the constant testing, medication, the literal highs and lows. But he remains one of the happiest children you could ever meet. Life is just one big exciting adventure for him and he’s going to rise to whatever challenges come his way.

Welcome home my courageous fair-haired boy.

Finlay does it the hard way...again!

Finlay underwent surgery this morning to insert a cardiac catheter so they could plan the next step in his care. A minor operation. According to the American College of Cardiology, “Cardiac catheterization is a common medical procedure that rarely causes serious complications.”

Sadly no-one told Finlay.

There is a bit of history here. When he was about 5 months old he was due to have a cardiac catheterisation. The day after arriving in hospital he developed an infection. This was at a time when he was a very sick child. His heart was failing and glucose control seemed a mythical concept so infections were not unusual (to be honest we’re finding glucose control to still be a bit of a myth but glucose management is a developing skill!). So it was a week of a screaming upset baby before the operation went ahead.

During the surgery the surgeons placed a small coil into his PDA, a small vessel between the pulmonary artery and aorta that should have closed after birth but didn’t. This is a fairly common heart defect and is easily rectified, the coil blocking the blood flow through the blood vessel and effectively shutting it off.

However, following Finlay’s op he started passing very dark red urine, the blood was passing round the coil and being shredded in a process called haemolysis. Once the surgeons saw this he was whisked into the operating room and the coil was taken out with another catheter.

His PDA was going to have to be closed surgically. In the end they decided that if they were going in they might as well fix a few other things at the same time. So he ended up having open-heart surgery and a quick two-day visit to hospital turned into a month-long stay.

Fast forward three and a half years and another cardiac catheterisation. This time the heart side of it seemingly went very well. The right side of his heart has grown well meaning that the major replumbing work that we thought he’d need might not have to happen. He may get away with just closing up the remaining hole in his heart and heading off to live a long healthy life. But not quite yet. They are going to leave it for a couple of years and then go back in and see if that’s the right decision.

So a really positive outcome. The proposed fix is the best option we could have hoped for and should leave his heart as close to normal as possible giving him the best chance of a long happy life.

But Finlay being Finlay he wasn’t going to let that be the end of it. He seems to have a penchant for the dramatic. So while under the anaesthetic his lung collapsed and he is developing a chest infection. He seems to be in a bit of discomfort after the op but has managed to eat something and is on the mend. However his oxygen saturation is down and he is being given oxygen to help. He was supposed to be coming home tomorrow but the doctors are concerned about him flying following his collapsed lung so it may be after the weekend before he makes it home.

Looking just a little sorry for himself

Tomorrow I’ll have to sit down with his brothers and explain that Mummy and Finlay won’t be here when they get home from school. I’ll try to make it clear that he is going to be OK and just needs a bit more time to get better but it’s more difficult with them this time. For his first heart surgery the boys were much younger, more accepting of whatever we told them. Now they are more questioning, querying anything they don’t understand. Hopefully I can provide soothing answers to whatever questions they have.

Knowing my boys probably their first question will be ”Can we have pizza then?” And, guilt-wracked, I expect I’ll concede. Maybe comfort food is what we’ll need.

Hopefully I’ll give them all the answers they need to be positive about the situation. Because, despite everything, I am positive. His heart is doing well and the rest he can deal with. He’s come through tough times before and he will again. He’s a tough kid. He may have one dodgy gene but the other 23,000 are bloody good!

Thanks for understanding Boss

Hey Boss,

Over the last few years you’ve been really great when it comes to my son’s condition. You’ve taken a genuine interest in his wellbeing, you’ve indulged my obsession with all things pancreas and heart related and you’ve readily accepted that sometimes family has to come first. While I have tried not to take liberties, the nature of his disease has meant that sometimes I haven’t been as productive as I would have liked. Knowing that you are not going to be analysing my every move has allowed me to concentrate on doing what needs to be done. Perhaps I haven’t always articulated how much I appreciate this freedom.

I tell you this now because I may not be working at my best over the next few days. This morning I dropped my wife and son at the airport for a flight that took them to the children’s hospital in Auckland. They are now safely in the heart ward and tomorrow morning my son will undergo cardiac surgery.

As cardiac surgery goes it is fairly minor, an exploratory op for more serious intervention later on, but any procedure under general anaesthetic where surgeons are poking and prodding around in his heart carries some obvious risks. In his case the added complication of maintaining blood sugars at optimal levels while being nil-by-mouth brings its own challenges.

After a long hug from me at the airport he ran off grinning, pulling his little suitcase behind him, wide-eyed with anticipation. For a week he’s been telling anyone and everyone that he is going on the big plane. He knows he’s going to see some doctors but how much he comprehends is hard to say. Perhaps the less the better.

And the hardest part for me is not being able to do anything, a feeling of complete and utter uselessness. In reality it doesn’t matter if I am ten feet away or a thousand miles. I can’t help him in those crucial hours. But given the choice I’d rather be there. To calm him before the op, to comfort him after. But I am not the only consideration and neither is he. His brothers need to get on with their schooling. They need to have their lives carry on as normally as can be, some constants they can rely on: school, friends, routine. The money still has to be earned: the wolves at the door know no compassion. And hauling the whole family up and down the length of the country is costly – costs we can put off until his big op later in the year.

So tomorrow I may appear less focused. I may be quieter than usual. I expect I’ll be checking my cellphone frequently. If I take 10 minutes out of the office at any point don’t worry, it’s going to be one of those days. Some have suggested taking some time off but sitting at home waiting for the phone to ring seems like the worst possible option.

So I’ll be at my desk as usual. I won’t be starting any ground-breaking new projects. I think I’ll just catch up on some of the mindless paperwork, nothing I can’t drop halfway through. I’ll give as much as I can but it won’t be 100%. My body will be here, my mind and my heart will be in another city.

Knowing you’ll understand eases my burden greatly.

Thanks Boss