Declan’s Story – The First

... by his carer, Anne

The night Declan was born it was a shock to his parents and Medical Staff that he was born with Bladder Exstrophy, his bladder sitting outside his body and inside out. None of the Ultra Sounds had detected this and as his Mum had two good pregnancies (both girls) prior, there was no cause for alarm, B.E. only happens in 1 in 50,000 births, (mostly boys), perhaps it’s not surprising.

By the time he was 2 hours old he and his mum were on a Mercy Angel flight to Adelaide Women and Children’s Hospital for surgery. His closing surgery entailed attaching his bladder inside his body and closing, but then it was found he also had Penopubic Espispadias, the Urethra does not form properly, it was short and split and as a result was short of his penis, which was also quite flat. They made a Fistula (opening) at the front base of his penis because the Urethra could not be attached in his penis at that time, this meant that urine would leave the bladder and flow to the fistula to the outside.

I met Declan when he was a week old, just in time for him to go back into theatre for a pelvic bone graft. Babies born with B.E. and P.E., their pelvis does not meet and join together. Over the next 4 weeks he had 7 surgeries, a Bilateral Inguinal Hernias, more bone grafts and another Abdominal Wall closure when his wound ruptured at the original incision, it just seemed to go on and on.

When Declan was 5 weeks old I bought him home to Victoria and arranged an appointment with a Surgeon at Cabrini in Melbourne, his legs had to be strapped together for 6 weeks to stop him parting them until his pelvis grew together, so every time his nappy was changed or being dressed his legs had to be held together, a feat in itself. The 6 weeks were up between Christmas and New Year so I left the strapping off and next morning his bladder prolapsed through the fistula, his local Pediatrician was away, the Doctors on ER had no idea and had never heard of Bladder Exstrophy. I had an appointment at Cabrini on Jan 4th and went prepared for him to go into hospital, but a surgery date was arranged for Feb 14th 2008 at RCH to fix the prolapse and connect the Urethra into his penis so he would then wee out his penis, but it would be always uncontrolled as he did not have Sphincter muscles.

During the surgery it was noticed his pelvis had not joined and an Orthopaedic surgeon was called in, they met with me to discuss fixing his prolapse and then the Orthopaedic surgeon would take over and do a pelvic external fixation which comprised of pins inserted in the Iliac bones and connected together by Titanium clamps and bars which would stay in for 6 weeks. Poor little baby came out hours later, a 4 month old baby with so many drainage tubes and IV’s attached, there was even one in his head and of course his titanium handle. Over the next 2 weeks he made a good recovery and his Surgeon decided to leave the next operation, his words “making him a new willie” to later in the year as he would be back in 6 weeks for day surgery to remove the pins.

On Declan’s 1st birthday he was back in theatre to have his new “willie” and a belly button made and now urine would pass via his penis – the fistula would eventually heal and close itself, because his bladder was never full it couldn’t grow so was still only the size of a 20 cent piece and he was suffering kidney reflux. The downside being he would have to wear nappies and later pull-ups until he was old enough for Bladder Augmentation and Bladder Neck Closure.

Life went on and he started kinder and then school, the staff were aware of his condition and helped him change his pull-ups every 2 hours, he was also on a strict drinking regime and his teacher was very good at keeping him focused, as a result he never had any kidney or bladder infections and was a normal happy 5 year old, he learnt to ride his bike and a motorbike and started motocross.

His surgeon at Cabrini retired and referred us to a surgeon at RCH who believed he needed to have the surgery sooner rather than later, the kidney reflux was damaging his left kidney and his bladder was not growing. Just after his 7th birthday he was back in theatre for bladder augmentation, part of bowel sewn into his bladder to enlarge it, bladder neck closure and the neck of his bladder securely sewn off. His appendix was used as a channel from his bladder to a stoma for catherization called a Mitrofanoff. After 8 hours in theatre Declan was once again out with lots of drainage tubes attached to bags and IV’s, he was to leave them in for 3 weeks.

Ten days later we came home and had to learn whole new techniques and care for our boy, he went back to school for 2 hours a day for the next week but then developed a bad infection so had to be flown back to RCH where we stayed 3 weeks, after numerous X-rays, Ultra Sounds, and blood test the infection was finally diagnosed and we were allowed home just in time for Christmas.

We spent our days now setting alarms, catherizing every 2 hours, flushing with saline and measuring all fluids that went in and came out and writing everything down. He was still on medication for bladder spasms and wore a leg bag at night.

When the new school year started Declan was in year 1 and was doing his own catheters under supervision, (I didn’t want any more infections). Three teachers at the school were trained in helping him and they set up a room for him with a toilet, basin and a hospital bed that lifted, at this stage he was still laying down and doing his catheters and I was happy to wait until he was ready to stand. One day I talked him into doing it in the toilet and he realised he empties better standing up,  was very happy to continue doing it that way. I bought a measure that fits in the top of the toilet bowl so I could still check on fluid going in and out. Boys have a habit of being very busy and not emptying properly which will lead to infections.

Going shopping, visiting or just out for the day became a whole new experience, from just packing pull-ups to remembering catheters, wipes and an empty pop top bottle, (it was the right size and easy to measure). Then I found pocket catheters to be a blessing as they had their own bag and was ready for him to use in the car, especially when most of the public toilets were filthy, twice  we were stuck in a traffic jam in Melbourne and he had to use them in the back seat.

In June, 7 months after surgery, Declan went back to RCH for a Urodynamic Cystography, which is a bladder pressure test done under X-ray and 6 pea size stones were located, so he was booked in for another operation to remove them in November, 1 year after his Augmentation, by the time that came around they had grown to 4 quite large pebbles and because of his fragile bladder surgery was the only option

We had spent 3 weeks in Greece in the September and did a cruise of the Greek Islands, before we left his Doctor gave us a course of Antibiotics in case of infection, he hadn’t had one through the year and he was not covered by Travel Insurance. The doctor also wrote out a letter explaining his medical condition and the reason we were carrying his supplies. Because his bowel is now part of his bladder and the bowel makes mucus, it continues to do so in the bladder and this then can block catheters especially at night when he is wearing a bag. As a result he needs Saline flushes, 200mls morning and night and 50mls at each catheter during the day, even drinking a lot of water does not help with him, so it was quite a feat working out catheters, night bags, 500ml bottles of Saline plus wipes, tapes etc and divided between 3 suitcases plus what had to be backed in our back packs. At the airport I put a leg bag on him and as we had a few hours stopover in Singapore, I could take it off give him a good flush and re-attach for the next leg to Athens. All went very well and he slept most of the flight.

After his bladder stone surgery he settled down to 3 hourly catheters and he was now using size14, he had started with size 8, to 10 to 12’s, I was concerned that in such a short time size 14 would hurt him, as they did to start with but he persevered and got used to it. We have since been to Italy and last year went on a cruise to PNG where we tried the Cliny catheter and once again took antibiotics just in case.

Declan has had 15 major surgeries in his short 10 years and the anesthetics are not good for developing brains in children, after his 8 hour surgery he had to learn everyday things again like holding a pen, his writing was scribble and he could not colour in the lines, all these he was good at previously, as a result it takes him longer to learn, but he keeps trying until it’s mastered, this condition is called Neurotoxicity and is a type of brain damage, it also affects concentration, coupled with the amount and types of drugs he has had  is it any wonder?

Being a Carer for Declan has been a challenge, firstly looking after a tiny baby through multiple surgeries and pain, he was originally only going to be with us for 12 months, has bonded us totally together, (I often joke how we are joined at the hip), organizing appointments, Ultra Sounds, X-rays, Urodynamics, holidays kinder and school, plus he took on swimming, motocross racing, cricket and table tennis. Rob and I didn’t think twice when we were asked to keep him full time, he was already calling me Mum, (his idea - I kept correcting him but he insisted) because of our age and we are retired he gets to go places and see things other children may never see and he takes it all in his stride, never skites or shows off, it’s given him a new perspective for learning and unless Medical Science takes on a drastic twist he has his “condition” for life and may even be able to father children, but that is still down the track and will be another story.

Written by Anne (August 2018)

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