Wednesday, December 21, 2011
But as he is growing up situations change. The old solutions become the new problems.
Braces are very practical and occasionally can be very stylish (especially ones with dinosaurs on!) but when it comes to toilet training they become a bit of a nuisance. Teaching a child the basics of toileting is always a challenge but throw in unclipping of braces, making sure the pump stays out of the toilet, watching where the tubing goes, it all becomes a bit fraught.
So thanks to a mother's suggestion on a forum we have taken delivery of this neat backpack with fetching fluorescent frog styling via the Diabetes Mall that will hold his pump and keep it away from any risky ablution-associated dangers.
Finlay sporting his frog covered backpack as he searches for pirates through his makeshift telescope
So far it seems to work pretty well. He is able to drop his pants at will..... hopefully only when necessary, but boys will be boys! We have managed to avoid having to fish the pump out of the toilet bowl, Finlay seems to like it and the other kids at preschool seem to just accept it.
Unfortunately with his infusion sites still in his thigh we've had a site ripped out following an over-eager pant-dropping but this is a learning process and he'll figure out the intricacies in his own time.
Looking pleased with his new gear
Wednesday, December 14, 2011
Wednesday, November 23, 2011
Recently a few diabetes bloggers I've been reading have posted pictures and videos of what their kids do despite diabetes, to show that these are just normal kids doing normal things. The pick of them was Reuben's chicken catching.
Now Finlay probably couldn't catch a chicken. Heck, I probably couldn't catch a chicken! But he can catch balls. This video was taken just before bedtime recently so excuse the pump weighing down his PJs!
In this blog I highlight many of the challenges that we have to deal with every day with our son's disease. It would be easy to be negative and focus on the downside all the time. Although there is nothing amazing about this video, the sight of Finlay laughing hysterically shows he is too busy enjoying life to let his condition get him down. So we shouldn't either!
Thursday, November 17, 2011
Another child at his preschool was being collected at the end of the day. He turned to his mummy and said,
" I want one of those" pointing to Finlay's pump.
"Ah, no. You can't get one of those" replied his mum.
"But... Finlay's got one!!...."
I hope their ride home wasn't too bad, with a 3yo tantrum from being denied the 'toy'.
Wednesday, November 9, 2011
So how can we give him enzymes during the night? The only drug available to us is the Creon that he takes orally during the day.
Option 1: Wake him up and give him some Creon capsules.
This worked the first night. We woke him up and he sleepily did as he was told, opened up and swallowed the enzymes mixed with some apple puree. The poor wee man didn’t know what was going on. By the next night he had worked out this was going to be a regular occurrence and he wasn’t happy. Tired, angry, crying and in no mood to start eating anything. A ten minute struggle to get him to eventually swallow his food was enough to convince us this was not a long term solution.
Thursday, October 20, 2011
Saturday, October 15, 2011
Wednesday, October 5, 2011
"How many are there in New Zealand?"
Pancreatic agenesis is clearly a very rare disease. How rare is difficult to say. Children with this condition have neonatal diabetes. The rate for neonatal diabetes is estimated at 1 in every 400,000 to 500,000 live births, and pancreatic agenesis is a rare cause of neonatal diabetes. So putting a figure on it is very hard; 1 in 10 million? 1 in 100 million?
In the US there is the Genetic and Rare Diseases Information Center (GARD) which has a searchable database of thousands of rare diseases. Run by the Office of Rare Disease Research (ORDR), a branch of the NIH, it is the most comprehensive resource for rare disease research. What information do they have on pancreatic agenesis? Nothing. There's a couple of conditions where bits of the pancreas aren't present but nothing for patients missing the entire organ. So there's the answer. My son's condition is too rare for the rare diseases database!!
I started this blog in an attempt to get some information on the web about this condition so that other parents would have better luck getting answers than we did when our son was diagnosed. The GARD database is another potential source of information that any future parents could come across. So, with that in mind, I thought I'd see if I could get pancreatic agenesis onto the database. Earlier today I sent an email to the people at the ORDR: (sorry, a bit heavy on the science!)
Dear ORDR people,
I am the father of a 3 year old son with a rare condition called pancreatic agenesis. While searching your database I was unable to find an entry for this disease. The closest ones appear to be pancreatic beta cell agenesis and agenesis of the dorsal pancreas. Complete agenesis of the pancreas results in a life-threatening phenotype due to the complete loss of both endocrine and exocrine function.
Due to this severity, pancreatic agenesis was until fairly recently a fatal disorder, with patients succumbing during the neonatal period. The first report of long term survival appeared in 1980 but this remained the only case until the early 90s. Since the mid-90s survival has increased, however the total number of cases is very low (I suspect <100). A good review, although a few years old now, was published by Baumeister and colleagues.
There are two known genetic causes of pancreatic agenesis, homozygous (or compound heterozygous in one case) mutations in PDX1, which results in loss of the pancreas with no apparent associated anomalies, and homozygous mutations in PTF1A, which result in loss of pancreas and cerebellar agenesis which is sadly a fatal condition.
A third group exists, which includes my son, where pancreatic agenesis occurs with cardiac defects, for which the genetic etiology has not yet been published. This includes the proband from the Baumeister article referenced above. This group can also have a number of other associated anomalies including gall bladder agenesis, intestinal malrotation and developmental delay although the occurrence of these varies within the group.
With the rarity of this condition I found it very hard to get information following my son's diagnosis. An entry on your database may help future parents in their search for answers.
Many thanks for the work that you do.
It would be nice to get this condition recognised more widely. It may help parents trying to understand the condition but also might help medical staff diagnose a condition they are unlikely to have encountered previously and may never have heard of. As Finlay's neonatologist said when giving us the diagnosis, "Finlay is not on page 1 of the textbooks". I'll be interested to see if I get a reply.
Friday, September 23, 2011
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.
Sunday, August 28, 2011
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.
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!
Saturday, August 13, 2011
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!
Tuesday, August 2, 2011
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
Saturday, July 2, 2011
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
Wednesday, June 15, 2011
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....
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
Friday, May 27, 2011
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:
Wednesday, May 18, 2011
Everyone I work with is used to the calls: "he's high", "he's low", "he's pulled his site out", "another kid pulled his site out".
And they understand that it doesn't get turned off, ever. Meetings, lunches, visiting dignitaries; if preschool calls it gets answered. At least I have a fairly unobtrusive conventional ringtone. It's not like I shatter the peace of the workplace with 8 bars of the latest hip hop hit, or tinny Beethoven, or some irritating 80s song that keeps going round in your head all day until you work out who sang the original.
I try not to be too disruptive and if needed a quick explanation is accepted without question, but I realise it can be annoying.
So yesterday the phone rings and I see it's preschool. But this time there's no numbers, no infusion set crisis. For once it was just a normal call. Something they could have been asking any parent. It was great. No quick-fire calculations needed, no apology as I slipped out for an hour to inject my son.
End the call and back to the meeting, "Sorry, wasn't important after all" Oops!
Sunday, May 15, 2011
So I need to introduce you to his big brothers.
It would be easy to exclude them from the blog but they have lived with diabetes as long as we have. At an age when most kids associate hearts with the lovehearts on a greetings card, they can recognise a cardiology diagram from a medical textbook. They have seen their brother's seizures, watched the scars heal from his surgeries. They know how to use a glucometer, where the glucose gels are kept, that when I tell them to get Mummy quickly they need to do it quickly. That they do this without complaint makes me so proud.
As the eldest, Cameron is the one the others look up to. Recently, our city has been struck by a series of earthquakes, one in February killing 181 people. With each big aftershock that shakes the house the younger ones look to him. His coolness and calmness in the face of nature's wrath is incredible in an 8yo. The other two see him looking OK and they stop crying, or get a hug from him to calm them down. At when it comes to Finlay he is just as good. Encouragement to drink his milk, a cuddle to ease the pain of a site injection. Now when Finlay is upset at a site change or at the hospital he cries for his brother.
Ewan is the livewire of the family. Despite Finlay's trials Ewan is the one that's going to cause me to lose my hair as he grows up. He and Finlay are often to be found plotting. With all Finlay's problems we were worried that there would be jealousy between the brothers but not a bit. They are the best of friends. They still argue, they're brothers! But we are so pleased with how they get on.
And this is where the guilt sets in.
They way they have dealt with Finlay and everything associated with him has been incredible. They deserve so much from life. And we constantly fail them. Diabetes and feeding regimes take up the majority of our time. Time we should be devoting to his brothers. We should play with them more, read to them more, teach them more, listen to them more. But too often we run out of time, or if we get the time, are just too damn tired. It's not how it should be, but sadly how it is. All we can do is try to give them as much time as we can. They are good boys and are growing up well. In spite of life's trials they will do well.
Friday, May 6, 2011
The week before Christmas we packed the tents and headed off to Abel Tasman, at the northern tip of the South Island of New Zealand, for a week in the sun. We piled the boys in the cars and headed north stopping in Kaikoura for lunch. Now Finlay doesn't travel light. There are a few extras we need to pack for him. Quite a few. What with his insulin pump, feeding pump, infusion sites, feeding sets, insulin, daytime digestive enzymes, night-time digestive enzymes, gastrostomy tube, glucose meter, test strips, spare test strips, alcohol wipes, glucagon, spare glucagon, syringes (various), plasters, antibiotic cream, spare cartridges, meter batteries, pump batteries, pump power cord, spare gastrostomy tube, lancets, back-up gastrostomy button, bottles, mixing pots, hypogels, spare hypogels, back-up hypogels, emergency back-up hypogels...it's a wonder we ever manage to leave the house.
So perhaps we shouldn't have been too surprised to get to lunch and discover we hadn't brought any of his milk! For a diabetic child with a highly regimented feeding plan requiring a high-carb prescription-only milk formula five times a day to delicately balance his blood sugars between damaging highs and potentially lethal, seizure-inducing lows this was possibly the worst thing, other than Finlay himself, that we could have left behind.
So we hastily booked a campsite in Kaikoura, and while Vicky, her sister and the boys enjoyed the delights of the spa pool, I bought the most sickly, sugary milk drink I could find, made sure I had plenty of hypogels (just as well we packed the emergency back-up ones!) and turned the car around heading back for Christchurch.
What should have been a lovely spot for lunch!
But it was worth it in the end!!
Thursday, May 5, 2011
Recently F has had a few lows in the evening. Something has changed. He's growing, or not growing or doing something different. He is normally fed through his tube at 9pm so we like his sugars to be at the low end of his range by then. But recently he has started dipping just before we check him. Twice recently we've had to get the dreaded orange box out the fridge. Sugars down in the low 1s (about 20-30 mg/dl) accompanied by seizures.
In the last week we've had a CGM put on him for a few days so we can see what is going on but that's for another post.
Anyway, fair to say we've been a bit on edge in the evenings recently.
So a few nights ago when F wakes up crying we're straight into panic mode. Vicky runs in and grabs him. He's screaming, hands shaking, not yet in seizure. Vicky grabs the meter and fires the lancet into his toe. He is still screaming, looking straight at me, almost through me with that fixed stare that just says "help me". Definitely hypo, no doubt.
OK, glucose gel or run for the box in the fridge? There's a gel on the table...grab it.... tear it open...try to get F to take it....refuses, more screaming.... Of course he refused it. He's hypo, can't think straight. Have to get it down him somehow....or run to the fridge? That's it....glucagon before he seizures.....
Vicky stops me. "Er, 8.4" (about 150)
"What??? Let me test him" 9.3
Oh....F calms down in mum's arms. Just a bad dream. And when we think about it, it's exactly how his brother's were when they were his age and had a nightmare. Shaking, staring eyes, screaming.
So we settle him down and turn out the light. I still head for the fridge, but for the chilled green bottle rather than the orange box!! A far more pleasant medicine!
Friday, April 29, 2011
Although he has just turned 3 we weren't expecting him to be in 3 year old clothes. He is still small for his age. So we picked the 24 months jeans and gave them a try. But no, so onto the 12-18 month ones. OK for length but still ended up down round his knees when he stood up. Thanks for adjustable waistbands! Pulling the elastic tighter and tighter we managed to get them looking pretty respectable.
We had a clinic at the hospital today and the news is all good. His weight is increasing well. It would be easy to get frustrated that he can only fit into clothes for children half his age but that would be to forget where he has come from.
And for all his lack of size, he gives as good as he gets when it comes to his brothers. He's got all the spirit (and more than enough volume!) to get his way. He's going to be alright.
Tuesday, April 12, 2011
Modelling the teddy bear range
Now the main problem is keeping his pants up. All those that are long enough for him seem to be made for kids twice as fat. Then we hook his pump onto the belt and five minutes later he's wearing them round his ankles. I realise that the fashion of today might be to wear your pants ridiculously low, and maybe the local teenagers think he's the coolest 3yo in the neighbourhood but it's just plain wrong.
So thanks to one of the parents at his brothers' school we now have a cool set of braces...with dinosaurs on!! He seems to like them. Not sure what he'll think when he looks back in a few years but it's saving our blushes for just now.
How many dinosaurs does it take to hold up an insulin pump?
Wednesday, March 30, 2011
But that ceased following the birth. Initially it simply wasn't something we thought about. We were so busy coming to terms with our new reality, looking after Finlay, caring for our other boys, working and, if we had the time, looking after ourselves that any idea of socialising was a non-starter.
But as time passed and Finlay became more stable we yearned for a return to some sort of normality. At first we would go out one at a time: a night with the girls for Vicky, a work night out for me. But how could we leave Finlay so we could both go out? How could we get a babysitter able to do BGL checks, set up tube feeds, mix and dose enzymes, treat hypos and, eek, use glucagon???
Luckily we know a few current or former nurses who were glad to let us out for the evening. I'm so glad we had these people around as they gave us the confidence to leave Finlay in someone else's hands for a few hours. Without someone with some medical knowledge to be our first babysitters I doubt we'd ever have had the courage to progress to other friends.
We're also lucky to have had a sick child in the age of cellphones. Halfway through dinner we'll get a message telling us what his BGL was, then we can relax. I don't know how parents of diabetics ever went out before cellphones!
Now we have non-medically trained friends babysitting. It's a lot for them to take on and we would only ask people who were relaxed about it. But it's such a huge help. The cellphone sits on the table. I still get a bit nervous until the text comes through. But that'll ease eventually...probably.
Sunday, March 27, 2011
When you have a child born with a rare disorder, or with symptoms that the doctors just can't fathom, one of the overwhelming feelings is loneliness: a desperate need to know what lies ahead, what the doctors can't tell you.
Finding someone who has been through it all is a massive step forward, the lifting of a huge burden. For us, the doctor in charge of Finlay's case giving us a paper about a German girl that had survived gave us the hope we so desperately needed.
Later, through the internet our story was able to do the same for another family. This article appeared in an Australian magazine about a year and a half ago.
If, in the future, a desperate parent sticks 'pancreatic agenesis' or 'born without a pancreas' into Google, hopefully they'll come across this site and will get some of the answers they need.
So, if you're reading this thanks for coming to the site. The more hits, the greater the chance we can be found through the search engines.
Saturday, February 19, 2011
All smiles from the big brothers
Over the next week Finlay struggled along, each day seeming to bring a new problem. His blood sugars swung wildly from hypos to the 20s (360 to 500+). In such a tiny baby a small bit of insulin makes a huge difference, getting the balance right was near impossible. He was growing, but only very slowly, his stools were fatty and colourless, suggesting he wasn't absorbing his food and his liver might not be working properly.
At six days old he had an ultrasound of his abdomen to see if his liver was ok. The good news was that his biliary tree (the plumbing that drains bile from the liver to the intestine) looked to be in working order but they told us he was missing his gall bladder! This hit us hard as we had gone from a child with a heart condition to one with diabetes as well and now we were being told there was a third issue. How much could one child take?
And there was still no real explanation for his inability to digest his food properly.
On day eight we were given a reason. The pancreas is responsible for producing insulin. It is also responsible for enzymes that digest food in the intestine. The fact that he was diabetic and wasn't digesting his food suggested that perhaps is pancreas was missing too!!
This was my lowest point. Our son had multiple heart defects and we were being told he was now missing two other organs. How could he survive? All I could think was the next meeting we would be sat down and told to prepare to say goodbye. We went back to where we were staying with our other two boys, trying to be as normal for them as we could. I can't remember much of that night but I doubt I slept much.
The next day we were ushered into a room with the neonatologist that had been looking after Finlay. "This was it" I thought. He sat us down and explained that he'd searched the medical literature. In his understated words Finlay "isn't on the first page of the textbooks". He then gave us a paper he had found in an obscure German journal. It detailed the story of a German girl born with an almost identical diagnosis to our son. No pancreas, no gall bladder, serious heart defects. At the time the paper was written this girl was 2.5yo. Still alive!!!
All of a sudden we had gone from desperation to hope. This little girl had given us more than we had dared hope for.
Sunday, February 13, 2011
Initially Finlay seemed to be doing well. Each two weeks we got to see the little guy developing and hear his heart pumping away. Despite the underlying problems all seemed well. We started to look forward to these extra ultrasounds. A silver lining to the cloud of his heart condition.
It was about the beginning of the third trimester at the end of another seemingly successful appointment, the sonographer was making all the measurements for Finlay's notes: head circumference, abdomen size, leg length, etc when she noticed that he was a bit on the small side. Nothing to worry about, just a one-off measurement. It would probably be back to normal at the next scan.
But it wasn't. Although his heart seemed to be beating away happily he had hardly grown. They couldn't find any reason, everything else seemed to be fine. At each of the now more frequent scans his heart took backstage to the new problem of his lack of growth. Early intervention was mentioned. As long as his heart seemed strong they thought he'd be ok but his size was the real concern.
In New Zealand all the paediatric cardiologists are in Auckland, an hours flight away on another island. With his heart problems Finlay needed to be born there in case he needed urgent surgery. After 36 weeks the decision was made to get Vicky on the plane.
During the last few years we've seen the best in people. The world can seem a harsh place with the daily dose of war and crime on the news but we've experienced the kindness and willingness to help those in need that makes us human. In Auckland, Vicky was confined to the maternity ward while I was staying in a motel about a mile away down the hill in Newmarket with our other two sons, then 2 and 5. Dragging two bored children who just wanted to stay with mummy back and forth from the motel to the hospital just added to the stress around the birth. But then a lady working in the Auckland office of Vicky's employers turned up with a spare car for us to use, a family we had never met but was related to Vicky's work colleague offered to take the boys when we needed, two friends living in Auckland gave up their time to help out where they could. To all these and many others we will be forever grateful.
After a week in the hospital the doctors decided it was time to get the little man out so Vicky was induced and in the early hours of the next morning Finlay made his appearance. With all the uncertainty about his heart and doctors unable to explain why he had stopped growing we had no idea whether he would come out alive, whether he would be whisked straight off for surgery or what the next few days would hold for us.
When he came out and gave a full-blooded cry it was amazing. The anxiety evaporated as he showed he was meaning to stick around for a while. He was small, but he was alive. After a quick cuddle with mum, he and I were whisked away to the neonatal unit for the start of what would be a long stay. His heart seemed to be stable and all signs were good. His initial blood glucose was low, 1.9 mmol/l (~34mg/dl) so he was given IV dextrose. Those numbers meant nothing at the time and blended in with the dozens of other figures and measurements that were thrown around. How that was going to change in the coming days and weeks!! But I'll save that for part 3.
In the palm of my hand: Finlay a day or two old
Monday, February 7, 2011
Our story is a little different. I'll need to break it into bits as it wasn't a single diagnosis, it was an evolving one over months.
Finlay is our third child. By the time Vicky was pregnant we were old hands at all this baby stuff. The first scan at about 10 weeks was fine: head, body, two arms, two legs. Everything in the right place. So when we went back for our 19 week scan we were as relaxed as could be. After all, it was a planned pregnancy so Vicky had been on the folic acid and vitamins, eaten well, looked after herself; I'd performed my bit with consummate skill; we were in our thirties but short of a risky age. Our other children were healthy. What could go wrong???
The young sonographer was very good. After she'd shown us the basics she started doing all the measurements while we grinned like Cheshire cats and started thinking of more boy's names. She started looking at the Doppler showing the blood flowing through the heart. Even with the third child I still thought it was great watching all the red and blue colours swishing through my son's body. It was all looking so good that the sonographer's calm, unflustered comment that she couldn't quite see something properly and was going to get her supervisor didn't raise a flicker of worry for us. Even after the supervisor said she would book us in for another scan at the hospital with a specialist the next day we weren't all that fazed. After all, she said that it might not be anything, the specialist would get a clearer picture.
I think the first realisation that things were far from good was the next day when we parked at the hospital and went into the radiology reception. Just as we were telling the receptionist our names someone appeared at our shoulder and whisked us straight down the corridor past the waiting patients. "ok, that's not normal" we thought as the penny dropped for both of us.
After much scanning and measurements and more Doppler images that seemed to go on for an age, the specialist sat down and told us the news that no expectant parents want to hear. Our son's heart appeared to have a number of problems. There were definitely some holes, and some other problems that she couldn't quite see properly. "Will he live?" I can't remember the answer but it wasn't a definite yes.
It was then that the enormity hit us. A few tears from Vicky while I did the staunch husband thing, comforting and giving empty assurances that all would be well. My stiff upper lip act lasted as far as the car....
Vicky plays the piano, and is damn good at it. When she's stressed she sits down and gives the ivory a workout. That morning the music soared. I stood in the kitchen listening, and completely lost any composure I had left. A minute or two later, the kettle finished boiling, I picked myself off the floor and got on with our new life. I don't think she noticed.
Wednesday, February 2, 2011
Finlay at 5 months modelling his pump
Recovering well a couple of days after his op