Lucy Beth's Stuff

Lucy Beth   9/6/96 9 years old


 

My name is Lucy but my Dad has a few different names for me most of which suit me quite well. Most people call me Lucy Locket or Lucy Lastic. I answer to any name if I feel like it.
I tend to only do anything if I feel like it!!

I was born on 9th June 1996 at St Mary's hospital in Manchester.
I gave my mum a tough time, (& I still do hehe) she was in Labour for almost 20 hours with me. Well I was in no rush to make an appearance I was perfectly happy where I was thank you very much!
When I did finally arrive I was not happy & the midwife & doctor thought that I may be a bit poorly. I made a funny noise a bit like a person trying to breathe when they have a snotty nose. Well the staff tried to clear my nose but I still made that noise.
I was also pretty limp. I had what they called Low Muscle Tone. So they decided that I should get one of those cosy warm fish tank things in the Special Care Unit.

After about an hour my Dad came up to see me and the staff took some Polaroid's of us so he could show my Mum and everyone else.
Mum was still in delivery as she was not allowed to move yet.
I gave the staff in Special Care a bit of a headache. I behaved just like a premature baby but I was only 2 weeks early. I was by far the biggest kid in the unit. The staff all commented that they were not used to looking after anyone as big as me.

Nobody really had any idea what was actually wrong with me. The testing started. They tested me for just about everything. One of the test that they did pretty early on was a Chromosome test to see if there was a Genetic abnormality. They do this pretty much as a routine in Special Care. That came back abnormal when I was about 2 weeks old. They also took some blood from Mum and Dad to see if either of them had something similar. It turned out that my Chromosomes were a real puzzle and it took 2 months more to sort them out.

Sometimes people get some abnormalities with their Chromosomes like people with Downs Syndrome, they have an extra chromosome no. 21 so instead of having 2, one from Mum & One from Dad they have 3. Where did they get the other one? No one really knows.

Anyway I have some of no.9 missing and nos. 4 & 13 have some bits swapped.

While I was in Special Care I met a lot of special people who all looked after me but I met one really Special Doctor, Dr, Doug Sims. He was like another Granddad to me. He looked after me until he retired in 2003. My family and I would really like to thank him for all he did for us.

Anyway. In special Care they found out that I had a narrow wind pipe and that was what made it difficult for me to breathe without making a noise. I had what they said was a vascular clamp, some veins had grown in a circle around the wind pipe and restricted it I would probably grow out of it OK but if not it was easy to fix.

That was not all. I still had a blocked nose. & I mean blocked. I had a condition called Choanial Atresia. which basically means that where the nostril goes through the skull bone there should be a hole and I had one missing. A series of operations sorted this out. but I didn't start having these right away. Until I'd had a couple of these Dilation procedures I used to get a thick green discharge from my right nostril which my Mum & Dad had to suction away with a machine.

I spent 3 weeks in Special Care. I also had some problems with feeding as well, I didn't suck very well. Mum wanted to breast feed me but I had trouble getting it in my mouth, I only had a little mouth. So I had breast milk from a bottle.
I coughed quite a lot when I ate and for a while after I was also sick quite a few times too.
Mum and Dad were quite worried about this as I never seemed to be able to eat enough. The dr's started testing for other things connected with my feeding problems. I had a ph study done at Withington Hospital's Nesta Wells Unit which is a special unit just for us special kids.
The ph study showed that I refluxed very badly. Reflux is when Acid or food comes back up the throat from the stomach. They decided that I needed to get some help with this. I started to have my liquid feeds thickened with some special stuff that meant I could have a drink of milk or juice from a spoon. It made it almost like jelly. This helped a little but not much. So they decided that I needed an N.G. tube. That's a tube down my nose into my stomach. Yeah right like I was ever gonna let that stay put??? I became very handy at hooking the tube with my thumb and pulling it out, sometimes even before they finished taping it in place. I'm a little devil I am.

Well it became obvious pretty quick that I needed a more permanent solution, to make sure of this before I had a big Operation they did a video swallow study. That showed that I had a poor swallow reflex. I had had many chest infections and they were now pretty sure why. It turns out I had been aspirating when I ate. Basically I got food in my lungs and that causes pneumonia.

It was decided that I needed a Gastrostomy and a Fundoplication. The Dr. that decided along with my Mum & Dad said she would refer her to a surgeon, but Mum & Dad said that they wanted a different surgeon to do the operation. The Dr. said oh he does not do simple things like that any more. Mum & Dad decided to ask him themselves. So the rang Mr. Adrian Bianchi who I had already been referred to by another Dr. for some other surgery that I would be needing in a few years time. Mr. Bianchi was very nice and said he would be more than happy to do my Gastrostomy operation. Mum & Dad told the Dr. that she was to refer me to Mr. Bianchi and he would do my op. We went along to see Mr. Bianchi who said that because I was such a Special Little Girl he would be happy to do any surgery that I needed and that Mum & Dad were to tell any other Dr. that I should be referred to him first in future. Mr. Bianchi has a very very good reputation and is considered one of the best Paediatric surgeons in the world.

Mr. Bianchi told Mum & Dad about an alternative to the Gastrostomy & Fundoplication which was a more permanent solution. He had done 12 other peoples operations like this already. What he suggested was that I should have a Gastro Oesophageal Dissociation. GOD for short. Basically he cuts off the food pipe (oesophagus) from the top of the stomach and cuts off the intestine from the bottom of the stomach and sews the two together so it bypasses the stomach. He then seals the top of the stomach and reconnects the bottom to the intestine about 40cm further down the intestine. He then makes a tube from the stomach and attaches this to the outside of my skin through a hole called a Stoma. Through the stoma you can then pass an NG tube through which you can pass liquid.
The theory is that if there is no connection from the top of the stomach then you can't reflux right? Well for most people yes but a few years later I proved it was possible to reflux from the intestine. Oops Mr. Bianchi had to contact all the other surgeons who were now doing this operation and tell them that they may need to modify the length of the reconnection bit.

The big difference with this operation is the NG tube and the fact that you can remove the tube and the stoma hole will not heal up.

I had that done when I was about 2 & 1/2 years old. It was a very big operation and took 7 1/2 hours Mr. Bianchi is very thourough. I needed a High Dependency bed for a few days after the op and I was in hospital for about 3 weeks.

I was started on feeds by a pump over 24 hours and they gradually increased the speed until my feed to about 12 hours and then I was fed overnight while I was asleep. Great yes? No not really. I love to play with tubes so much so that at the school I had started at by now (Rodney House Assessment Centre) they called me Miss Whiplash. If I could get hold of a tube I would whip it around and anyone that got in the way well that was just tough. Around this time I also needed Oxygen after a couple of boughts of pneumonia left me with a need for it. I had lower than normal Sat's (the level of Oxygen in the blood) but people started noticing that I was much better and more lively with the oxygen than without so Dr. Sims decided that I should stay on the Oxygen.
I was a devil for playing with the tubes at night and regularly gave my feed to the bed. I would pull out the tube and then proceed with my Miss Whiplash reputation with vigour. If everyone else was asleep I could play for hours if I was quiet enough.

After some time I started having trouble with my feeding again. I seemed to be in pain while I was beeing fed. This got very distressing for me & Mum & Dad. The Dr.'s were puzzled again. They could not find any reason for it. Everything looked fine. I spent many nights in hospital being observed and almost all staff saw what happened but had no idea why. Dr. Sims decided to try me on some medication for reflux which helped a little but not much. So he decided to do a Barium follow study. They gave me some liquid mixed with some radioactive stuff called Barium and watched what happened to it on an X-Ray machine. Everything looked great all fine then all of a sudden I refluxed, yep a perfect  recording of reflux. That was not supposed to be possible.

Over the next few months the Dr.'s tried to figure this out until Mr. Bianchi said that the only thing he could think of was to open me up again and see if he could see anything. He decided that he would lengthen the reconnection that he had done previously and hoped that it would work.

I went into St Mary's hospital this time instead of Pendlebury as I had had so many anaesthetics already and never had a problem it was decided that this would be OK & as it should be a short Op I would not need an intensive care bed or anything.
The op started and Mr. Bianchi did as he said he would. When he had finished he decided to have a look at the rest of my intestine to make sure there was no problem. He found that I had adhesions all over my intestine. That is what must have been causing me all the pain. He freed everything up and put me back together. However this made it a much longer op than they thought.
everything seemed OK and I went back to the ward. On the Friday they close the surgical ward at St Mary's so I was transferred up to Acorn Ward (medical) which was no problem as everyone on Acorn ward knows me very well.

Sometime overnight I aspirated from into my lungs and my O2 Sats dropped to about 45%, really bad as normal is over 90%. They got me on a ventilator and managed to get my sats up to 60% but that was still no good. They called my Dad at home as my Mum was staying at the hospital with me. They told my Dad that he better get there quick as it did not look like I would make it. Dad quickly called my Nana to come mind Chris my big brother. He raced down to the hospital to find that I was waiting for the team from intensive care at Pendlebury and my only hope was that they could do something to help me. They got me to intensive care and they changed me over onto an oscillating ventilator which pumps up the lungs in short sharp shocks to keep the lungs moving. That helps to clear the gunk off the lungs. Over the nest 24 hours my sats gradually came back up until by midnight I got to 100%. In total I was on the ventilator for 18 days. A very long time. I had some problems when they tried to reduce the sedation to let me breathe on my own. I have Epilepsy and as I had not had any of my normal medication because of my operation I started having some severe fits, One of the medication that they use for sedation in intensive care is also used as an anti epilepsy drug so they hoped that that would stop any fits but when they reduced it I started.

Anyway I got through all of that and was eventually transferred back to St Mary's. Up till now I had been on Oxygen all the time so they continued to reduce the amount of O2 I had every day until eventually someone noticed that it was actually switched off. The hospital could monitor my sats properly so they could do this safely. Mum & Dad had no way to check it out. So not only did I get through the most scary time of my and my Mum & Dad's life I got off the Oxygen too.

Since then I have been better as far as my chest is concerned.

I once again had problems feeding having pain during feeds. I spent a lot of time in Booth Hall hospital under the care of Dr. Thomas's team. They really could not find anything wrong, with it looking more and more like I was having the same problem with my intestines it was again not looking good. I may have needed another operation. In a last ditch attempt they decided to change the feeding method. I am now fed by Gastrostomy still but now I have 5 bolus feeds per day. A bolus feed is when someone holds a syringe filled with my special milk and allows it to drain into my stomach by the power of gravity. It took a long long time to build up to the amount of feed I need to keep me healthy. This was the only thing that had not been tried. It worked thankfully. However just after coming home from hospital I spiked a high temperature. I used to do that a lot. Mum & Dad took me back to Booth Hall where the Dr.'s had some trouble finding out why. It turned out I had a urinary tract infection. I had had a temperature of around 40`C for around 3 days when they figured it out.  They needed to get me on IV antibiotics and pretty quick too. They could not get a line into me at all. No one could find a vein anywhere. 2 nurses tried 2 Doctors tried an anaestatist tried then a consultant aneastatist. They all had a go at sticking me with their needle but I won no one got a line into me. That meant trouble. They told Mum & Dad that I would have to have a central line. This meant an operation. Mum & Dad were really scared as Mr. Bianchi had told them after my last op that it would not be a good idea to do any surgery unless it was really needed. Luckily it was Mr. Bianchi that was called in at 9pm to insert my central line.

The infection was that bad it took 14 days of IV antibiotics through my line to get me nearly right again. I went home with my central line still in place which scared my Mum & Dad. luckily there was a nurse at school who used to work at Christies Hospital, a cancer hospital so she was used to central lines. My line needed to be flushed twice a week even though I was not using it. The Children's Community Nurses used to do this either at school or at home. About 4 weeks after I came home a nurse came out to my Nanas on a Saturday to flush my line, but it was blocked. I had to go straight up to the ward at Booth Hall where they would put some special stuff in the line to unblock it. They did & I and my dad had to wait for 4 hours for it to work. During that time I spiked a high temp again. Dad went and got the nurse who took my temp and decided we needed to call the Dr. While we were waiting for him Dad remembered that I'd had my line flushed at school on Monday and when I got home I had a temp. When he told the Dr. they both realised what had happened. I had an infection in the line itself. Whenever the line was flushed the bacteria was flushed into my blood stream and I spiked a temperature. So More IV antibiotics through the central line & then the line had to be removed.

A couple of weeks after this I got another Urinary Tract infection. This time they got it in time and managed to get a line in. So more IV antibiotics and that meant that I would need some more tests after I was better to make sure my kidneys were still OK. A young Registrar decided to give me a prophylactic dose of antibiotics , just one dose every day until I had these special scans done on my kidneys.

The results were amazing. Since starting the regular dose of antibiotics I have only had 2 days off school in over 18 months, & that was because the school nurse said I had to stay off because the Dr. had given me some different antibiotics for a slight chest infection. I have not looked back. I have improved in all areas of my life.

I still have plenty of trails in my life but I am happy. I have developmental delays in all areas. I can not walk, I can not talk, I can not stand, I can not take care of myself, I can not feed myself, I have Epilepsy, I am fed through a tube. etc there is lots I can not do for myself.
However I can: sit on the floor, play with toys, find just about anything funny, play with my brother & family. Basically my life is one big playground. I have fun fun and more fun.

Wow what a lot of words Dad & I am not finished yet! Do you think anyone is still reading?

Shall I tell them about my trip to see Mickey Mouse in 2004?

Why not?

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