The things us parents have to do!

This post is probably best not read whilst eating. Sorry!

Any parent would move the earth for their children. Having been in contact with many families of chronically ill children since Finlay was born we’ve seen the enormous lengths that parents will go to make sure their child gets the best care.

And I too hope that I would do anything in my power to care for any of my boys. But that doesn’t mean I’m always going to enjoy it…..

In addition to not having a pancreas and his heart defects, Finlay was also born without a gall bladder. Now amongst all things Finlay this has probably been the smallest issue. In the league table of important organs it languishes somewhere near the bottom just above little toes, appendices and men’s nipples.

But its absence does present problems. Finlay can’t store bile. His liver can produce it but it just flows straight into the gut rather than get stored in the gall bladder. So when Finlay has a meal there is no sudden rush of bile, just a slow trickle down from the liver.

This means he has difficulty breaking down fat. We can supplement the pancreatic enzymes he is missing but lack of bile makes it harder for them to work. And he needs that fat to grow and also to help absorb fat soluble vitamins, A, D, E and K.

So from time to time we need to monitor how well he is absorbing fat. The best way to do this is to look for how much fat hasn’t been absorbed by looking at a faecal sample under the microscope.

So our job as parents is to get the sample. Fortunately he is still in nappies (although we’re working on this) so it’s a little easier to collect but still not particularly enjoyable.

We’d rather not get the sample at the weekend as we’d have to keep it at home until Monday and I don’t really want to have a pot of poo in the fridge between the milk and the yoghurt.

So that leaves weekdays when he is at daycare. He tends to do his poos during the day when he is at daycare (that took a bit of training but well worth it!!) so that led to this morning:

“Er…a bit of a strange request but when Finlay does a poo could you just put it to one side, give me a call and I’ll pop down and collect it?”

Now we ask a lot of his teachers: blood glucose testing, bolusing, special drinks, medications, record keeping; but I thought maybe I was pushing it a bit far with this one. To their credit they didn’t bat an eyelid. What they said once I’d left the room is anyone’s guess.

So later the call came. “You’ve got some poo? I’ll be there straight away” And to the perplexed looks of my work colleagues I put the phone down, jumped on my bike and headed to daycare. They pointed me in the direction of a triple-bagged plastic pile containing one freshly soiled nappy and two rather disgusting minutes later I was back on the bike and pedalling to the hospital lab (very, very carefully. This is not the time to fall off and end up with a soiled backpack).

So now we just wait for the results and hope it’s a long time before we have to do that again.

I haven’t got any pictures for this post, for which I’m sure you’ll be glad!

Bubbleman

Finlay has a gastrostomy, a hole into his stomach through which we can feed him. Through his abdomen he has a 'Mickey' button, a short tube that goes into his stomach with a valve on the outside that sits against his tummy that we can attach a feeding tube whenever he needs feeding. On the stomach side of this button is a small balloon that is inflated with water to secure the tube and stop it falling out.

Tonight I was checking the water in the balloon by drawing it out with a syringe and replacing with some fresh water. Cameron, 8yo, was watching and starting asking what I was doing. Although he's seen the tube in daily use I can't remember if we've ever explained exactly how it works. So I explained about the ballon inside his stomach that I was inflating with the water.

"Cool!"

I suppose for an 8yo boy that would sound pretty cool. TBH it still quite amazes me.

Then Cameron turns to Finlay and says "Hey, Bubbleman...." I probably shouldn't approve but then Finlay found it funny and the boys started gently teasing him about being Bubbleman which had him in hysterics. Great to see all the boys getting on well and having fun with Finlay's condition.

Night night, Bubbleman!

Another not-so-normal day

Finlay's condition brings up scenarios that just don't happen to other parents. Sometimes funny, sometimes not. A couple of examples in the last few days.

I was dropping the brothers off at school before taking Finlay to preschool. I was getting Finlay out of the child seat on the back of my bike when all of a sudden he starts screaming. I look down and see the pump lying in the seat with the tubing and the site. Somehow his pump got caught in the seat and I've pulled the site out of his thigh as I've taken him out the seat.

I notice a bit of blood in the cannula of the site then look down at Finlay's leg and see a patch of red coming through his pants. We must have caught a little blood vessel as the blood is oozing down his leg. So there I am outside the school gates with a screaming toddler, pants round his ankles (remember it's winter in NZ now and it's freezing in the morning), a stream of blood down his leg and me desperately clawing through my bag for a tissue to stem the bleeding.

A couple of the mum's stop to ask if they can help. "Oh no. Thanks but we're fine." WHAT?? How is this fine? How is this normal? What am I saying??

But the reality is we deal with all sorts of mishaps like this all the time. This is our normal. I knew that it was just a needle hole and it would stop bleeding in a minute. I knew that Finlay was just crying from the initial pain of the site being ripped out. He isn't fazed by blood. He sees his own many times a day. In a minute he would be happily running around after his brothers. To be honest, I was more concerned about the blood on his pants. More frustrated that I was going to have to go back home and get another site. Late for work again!

Are my priorities all wrong there? Probably. But that's our reality. Finlay was great. Recovered in a flash and didn't make a sound as I put the new site in. What a star for a three-year-old.

Then, on a lighter note....

At preschool we take in bottles of a special prescription drink for him to have at mealtimes, packed with goodies to try and beef him up a bit. And with every meal he is given replacement enzymes that his pancreas would have produced had he had one. So every so often the preschool remind us to bring some more in.

So a few days ago alongside the other messages, "more nappies for Johnny", "spare clothes for Olivia" is "Finlay - drink and drugs"!

I hope there were no inspectors from the education department around!

The Long Haul

I haven't posted for a while but with good reason. We took a 4 week holiday, the first time we've been overseas together for 6 years. Now Finlay has flown before but only hour long flights to and from Auckland for his heart op and when he was born.


The trouble with New Zealand is when you want to go to another country it's always a long journey. And with Finlay there's the diabetes to think about: the time zones, the feeding regime, the insulin.....


So rather than try a quick jump across to Australia or one of the South Pacific islands we go all out with 30+ hours, 12,000 miles, 4 flights and 12 time zones. NZ to UK. How would we manage this? Not even Dr Google could help out here. Plenty on single flights and a few time zones but nothing about multiple flights halfway round the globe.


So for anyone else thinking about it here's what we did.


Plenty of planning ahead: Insurance took a while but I'll save that for another post. Letter (+ copies) from the endo so we could get the pump, syringes, medication past security. Letters (+copies) from the dietitian so we could get his feeding pump and lots of bottles of Nutrini feed on with us and 6 cases of bottles in the hold. Organise to pick up an extra month's supplies from the hospital so we didn't run out half way through the holiday. Get extra prescriptions of pancreatic enzymes (the pharmacy had me sign a waiver as the computer flagged that I'd only just picked up a previous script. I have to say if I was going to OD on something I doubt I'd choose pancreatic enzymes and I don't think their street value would let me retire early).


Then there's packing his bag for the flight.First of all, a bag isn't going to do it. This was going to take a small suitcase. Enough bottles of Nutrini to get him through 4 flights, plus an extra couple in case of delays, then an extra couple in case of spillages. Feeding pump (too big for suitcase); bottle for feeding pump; 2 feeding sets for 2 days travel plus extra one just in case; gastrostomy tube; spare gastrostomy tube; spare gastrostomy button; lubricant for inserting button; infusion site; alcohol wipes; pimafucort tube for old insertion site; plaster for stopping pimafucort getting all over clothes; spare insulin vial; daytime pancreatic enzymes; nighttime pancreatic enzymes; apple puree for feeding the daytime enzymes; 10ml syringes for nighttime enzymes, 50ml syringes for feed; glucometer; spare battery for glucometer; spare battery for insulin pump; extra box of test strips; hypo gels; extra hypo gels; sod it, just throw a box of gels in!; glucagon, extra glucagon (unlikely to need glucagon at all but if we took one then needed it in the departure lounge in NZ then we'd have 30+ hours without any).


We just about managed to cram it into a small suitcase. Security were very different in different countries. NZ and Australia were pretty laid back about it, Dubai were fairly indifferent, Bangkok were quite strict and had a good rummage around and at London they made me open one of the bottles of Nutrini and drink some (horrible stuff!! I thought my grimacing face might lead to a full body search) then they swabbed his insulin pump for explosives. But all done in a friendly manner.


So what about the insulin regime? First, we disconnected the pump during each take off and landing. Our diabetes nurse sent us a powerpoint presentation about insulin pumps on flights and how the change in pressure can lead to extra insulin being given as the pump is a closed system where the pressure will remain high whereas the cabin pressure would drop leading to extra insulin being delivered because of this pressure difference. With Finlay being so small an unexpected dose like this could cause problems. This worked well and we only forgot to disconnect once. Oops!


Finlay and Ewan enjoying the flight

As for the rest of the time we didn't really know how he'd react to the time changes, the excitement of the trip etc. We had one long night on the way out as we flew west, then two short nights on the return as we flew east. We had no idea what that was going to do to his numbers. Vicky did a chart with all the flights on and planned feeding times then five rows each with the time at one airport, hour by hour. All very organised. We wanted to be fairly conservative with the insulin. So we dropped the basal rates to the lowest setting and gave larger boluses with meals.


And it worked really well. His figures were generally in single figures or just a bit higher. Probably better than normal. We should fly more often! We only had one hypo episode when the plane was a little late arriving in Australia on the way back which messed our timings about a bit. I thought he'd be OK until we got into the terminal but I tested him as we were taxiing and he was LO. No symptoms!! But a quick tube of gel and a feed on the plane as the cleaners were trying to work around us and all was well. By the way, a great way to get through security quickly is to have your kid have a hypo when you land then they whisk you straight past the queues and rush you right through to your departure gate. I think they were trying to get rid of us before anything serious happened!


All in all it went much more smoothly than we had hoped. We were really worried that we'd be awake the entire journey testing him every half hour but it turned out really well. All three kids behaved themselves. No meltdowns on board. Not from our kids anyway! Didn't get much sleep but that would be asking too much.


It gives us real confidence about doing more in the future. A bit of planning and staying calm and all should be ok.


And the holiday was great!


Finlay inspecting the cannons at Edinburgh Castle

Hooray for the Wiggles!!

Before we first used the infusion sites we were told to go home and practice on each other. So we sat down after the boys were in bed and nervously prepared to insert a site. Vicky practised on me, and I practised on....er ....me! It was quite nerve-wracking, for the person on both ends of the needle! It's no little pin-prick: that's a pretty long needle she was wielding. Not a particularly nice experience.

So imagine what it must be like for a toddler or baby on the sharp end of the needle. Little or no knowledge of diabetes or why your parents, the two people you most trust in the world, are inflicting this pain on you.


In those early weeks and months, when the needle was just about the whole length of his thigh muscle, although it was hard for us to insert the site, feeling just so wrong to be doing that, he at least didn't put up a fight. He barely registered it sometimes. All his fight was on staying alive. His battle was bigger than a stupid needle.


But as he grew he started to make his feelings known. He learnt the routine. If both of us appeared at his change mat he knew what was in store for him. One to hold his leg bent to bunch enough muscle to aim the needle at (there was no fat on him, none) and one to wield the needle.


We tried to alter the routine to relax him but, though Finlay may be many things, he is not stupid. He always knew when it was time to change the site. And the stronger he got the harder it was getting for us.


But salvation came from the Wiggles. They are a four man children's entertainment phenomenon. I don't know how big they are in other parts of the world but if you're a preschooler in New Zealand or Australia these are the guys!


A year or two back we were having problems with recurrent skin infections at sites. The solution was to use some antibiotic cream after removing the site and stick a plaster over the cream to stop it going everywhere. It worked. No infection problem now. Recently we were scrambling around for a plaster after a site change and came across a Wiggles plaster. We popped it on and Finlay was delighted.


Now everytime we get ready for a site change, rather than the usual screams and burying his head in his teddy bear, he just looks at us sternly and enquires "Wiggles?". We show him the new Wiggles plaster and all is right with the world. All it takes is Dorothy the Dinosaur or Wags the dog to be slapped on to his thigh and site changes are a whole new experience.


Thank you Wiggles!

Wags and Dorothy keep happy Finlay infection-free

The Mistaken Professor

One of the common gripes amongst the diabetes community is the constant misconceptions. These range from the ignorant "you must have fed your child too much sugar" type of comment from the know-it-all mother at school to the well-meaning but exasperating "my friend's cousin's postman's gran's diabetes was cured by meditation and bathing in yak wee" type of comment.

It's frustrating when these come from friends, more so when they're from relatives, and disappointing when they come from ill-informed journalists in newspapers or on TV. And sadly sometimes even the professionals get it wrong:

While googling Finlay's condition I read a piece written by a professor of gastroenterology about diseases of the pancreas. It was a short, informative piece that covered conditions like cystic fibrosis and other inherited disorders. At the end he included this little gem, "In extremely unusual circumstances a child may be born without any pancreas.......This problem is not compatible with life"

Now Finlay clearly hasn't read this particular piece as, thanks to various medications and a lack of sleep on our part, he doesn't seem to be having any compatibility issues with life.

Now imagine you were a parent of a newborn baby that was diagnosed with an absent pancreas. The first thing you do (once you pick yourself off the floor and pull yourself together) is to hit the internet to find out anything you can. And with a rare disease like this there's not much out there. So it's not that hard to come across the article above. I did. I'd hate for anyone in the position we were in a few years ago to come across that kind of comment.

I think this is probably an old piece that just gets regurgitated from time to time but I think when experts give their names to articles on the internet there is a duty to ensure they are correct and up to date.

This professor has an impressive track record, particularly researching cystic fibrosis so I have huge respect for him but I do want him to correct the piece. So here's the letter I sent in March. It's a bit heavy on the science but I thought that might appeal to his academic nature. So far, no reply. I've removed the name and a few identifiers as it's perfectly possible that his PA deleted the mail and he's never read it so I don't want a witch hunt. But it would be nice to get a reply....

Dear Professor,

I recently came across a short piece you provided for the XXXXXXXXXXXX entitled "Inherited Pancreatic Disorders of Childhood". At the end of the article you state that being born without a pancreas is not compatible with life. I note that the article was posted on April 8th 2010. Coincidentally this was my son's 2nd birthday. He was born without a pancreas.

While your assertion was true at one point there are now a growing number of children surviving with this condition. The exocrine insufficiency can be treated with enzymes in the same way that you treat your CF patients while insulin therapy can overcome the lack of endocrine function. While treatment, particularly in the initial stages is far from easy, technological advances such as insulin pump therapy allow for better outcomes for these children.

The earliest record of long-term survival following pancreatic agenesis dates back 30 years (Howard et al, 1980, J. Pediatr. 97: 786-9) and while that case may be considered an outlier, the prospect for children born without a pancreas has improved greatly since the 1990s. I conservatively estimate that the number of surviving cases runs into the teens, and possibly higher. Personally I am in electronic contact with the parents of 4 others.

Currently, mutations in two genes have been shown to cause pancreatic agenesis. PDX1 was first linked to the condition in 1997 (Stoffers et al, 1997, Nature Genetics 15: 106-10). Homozygous, or compound heterozygous, mutation of this gene has been found in at least 3 cases, all of whom survived the neonatal period, and one of whom I know is continuing to thrive into his preschool years.

PTF1A homozygous mutations have been found in children from two distinct consanguineous families. Sadly these cases have also presented with cerebellar agenesis and they have all , indeed, proved incompatible with life (Hoveyda et al, 1999, J Med Genet 36: 700–704; Sellick et al, 2003, Diabetes 52: 2636-38; Sellick et al, 2004, Nature Genetics 36: 1301-5).

There is also a group, including my son, for which no causal mutation has been found. These children can have a range of associated conditions including gall bladder agenesis, cardiac defects, duodenal malrotation and others. This group includes a number of surviving children.

I raise this issue not as the pedantic ramblings of an irate father but out of concern for future parents of these children. When a child is born with a serious medical condition one of the first things most parents do nowadays is to enter the condition into an internet search engine. This is particularly true when the child has a very rare condition where the doctors treating their child may not have the answers they need. At this stage of their journey the parents are likely to be emotionally and physically drained and the last thing they need is to find a website with outdated or incorrect information telling them that their child is going to die.

I hope that for future articles you may write, and previous ones if you are able to edit, you could say that although serious, being born without a pancreas can, in most cases, now be treated with insulin therapy and enzyme supplements.

May I finally thank you for your research. Despite the reason for my email, as the parent of a child with pancreatic exocrine insufficiency who has benefited from research such as yours into diseases such as CF, I appreciate the work that you and your colleagues do.

With kind regards

Giving Back

Since Finlay came along we have been really lucky to have had help from lots of different individuals and charities. There's a list at the bottom of the blog - click away, they're all lovely people!

Since Finlay's first ultrasound diagnosis one charity, @Heart, has been with us all the way. They've been a huge support, emotionally and financially. They helped get me to Auckland (an hour's flight away on a different island) for the birth and later for his open heart surgery. They've been there to support us both at home and in hospital. From day one we always wanted to try to give something back. We just never imagined the chance would come so soon and in such a way....

One of our friends is a photographer, a really good one, take a look. When Finlay was 5 months old and we were about to fly him up to Auckland for his heart surgery we asked him to take some photos. Partly because we knew that would be the last chance to get photos without a scar, and partly because.....well, it might just be the last chance.

And the results were stunning:

A few months later, @Heart sent round an email about a photo competition being run by Canon (the people that make the cameras and printers). The winning picture would win $25,000 for their charity. So Vicky got on the phone to our friend and asked him to enter one of the photos. Being the great bloke that he is, he didn't hesitate in saying yes. He knew without asking which photo he should enter. Of all the fantastic pics there was one standout.

Over the next couple of months we tried to get as many friends, relatives and anyone else to vote for his picture on the Canon website. It didn't get the most votes but it got enough for the judges to take notice. Out of over 800, beautiful, stunning, inventive photos from around the country, all with heart-rending stories behind them, his was one of the final 5 to go before the judging panel, which included one of the country's top photographers and even the Prime Minister's wife.

An exciting, nervous few days were finally broken when our friend got the phonecall from Canon that he had won and our charity was getting $25,000. So many more families would be helped in the way we were thanks to our friend and Finlay. But for us the picture is worth a million dollars: