Blog 46 - Op #2 for Jess - 5 years - Tonsils and Adenoids

Jess had a sleep study which showed slight sleep apnoea. It also showed that Jess was a very restless sleeper - which we already knew! She had been a restless sleeper from early on in her life. Jess would often move up and down her cot, then up and down her bed. When we first put her in her bed we found that she would fall out of it so we needed to put up a bed rail....and she would often leave her bed and be found on the floor or in Melissa's bed.

In the morning she would wake up very tired with tousled hair.

The Respiratory doctors suggested that she have her large tonsils and adenoids removed. It was hoped that this would help her to get more oxygen into her lungs, and help her to sleep better.....but as she would not be rated as a Category 1 it would be best for us to go privately, as the public waiting list was very long.

We took Jess to see the private ENT doctor. He agreed with Respiratory that she needed her tonsils and adenoids out - they were taking up half of her throat - so we made the operation appointment for the following Easter break holidays.

The time soon came and Jess went into hospital for her op.

Jess had to fast which I was really concerned about because Jess does not cope with change in routine. We didn't know how we would get her to understand that she could not eat.

But God came through and answered our prayer. We were rung by the anaesthetist who told us that she could have breakfast as it would be an afternoon operation. This solved the problem we had foreseen.

We went to the day waiting area and she sat in her bed for a while and played. We had brought her some comfort toys. She soon was bored and we went to the playroom which keep her entertained.

Back in the room we were still waiting. The other children in the room had been on IV infusions and were eating....so of course Jess wanted to eat! We had to physically remove her again from the day area so she could not see the food - I was not happy about the fact that they put her with children who were eating - how unfair on any child!

It came time for Jess to go in for her surgery and she went to sleep very quickly and calmly. We waited and it was only about half an hour or so before the doctor came out to tell us that everything had gone well. The tonsils and adenoids were bigger than what he had thought and were taking up about three-quarters of her throat. She could go home the next day. Too easy!

Jess woke up and took her pain medicine and antibiotics well.

She soon started doing her repetitive comfort behaviour - putting pegs in and out of a bucket over and over again. Then taking pen lids off and putting them on again over and over again.

The next morning Jess continued these comfort activities. She ate quite happily - Jess doesn't seem to feel pain at the same level that my other children do. And we went home.

Once we got home Jess continued to eat quite happily for a couple of days and then she decided that she did not want to eat - we think the pain finally sunk in. We tried to coax her with all her favourite foods but she refused. We kept the pain medicine up and forced her to drink water.... She would not drink anything else. Jess also decided that she would only listen to me and not Joe my husband....which meant that after I went back to school I had to ring home nad tell her over the phone to drink.... She was being very stubborn.

This week was a scary time as we didn't want her ending back up in hospital. And though well-meaning friends gave us lots of suggestions they had no idea what we were dealing with - we had a stubborn child with DS/intellectual impairment, who only eats a small range of foods and who will not eat anything else no matter what is offered....  This situation provides more challenges then for the regular child who will happily eat anything soft, cold and sweet. Not our Jess...

Again with her intellectual impairment we could not reason with her.

We had a paediatrician appointment and the doctor noticed that she did have an infection in the back of her throat which is probably the reason why she didn't want to eat....so Jess was put on more antibiotics. Soon after she went back to eating.

The operation was successful as she is a better sleeper and even though she still wakes up with tousled hair, she is not tired.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission

Blog 45 - Jess starts Kindy and then School

In 2011 it was time for Jess to start Kindy (also called Pre-Prep). She was with the same teacher that Melissa and Nick had and the teacher was enthusiastic about having her. With Federal funding Jess was able to have an aid to help her.

Jess enjoyed going to Kindy and being with the other children. At times she would show defiant behaviour and she would wander off. But she was able to follow the classroom routines and she was getting used to 'school' life.

Jess went to Kindy for a 5 day fortnight. She also went for two half days to the ECDU at the Special School. Jess enjoyed having the two teachers at the ECDU at her beck and call and had them wrapped around her little finger. There were things that we knew she could do that she wouldn't do at the ECDU unless she had help.

We began to notice one main difference between the two settings. When Jess came home from Kindy she was communicating verbally with us....when she came home from the ECDU she wasn't - she would use her Makaton sign language. Upon querying the ECDU we found out that the other children were non-verbal...so that's what she was. Another behaviour she picked up from the ECDU was hitting as one of the other children was a hitter. We weren't happy about that.

Jess needed to be around verbal children and appropriate behaviour. She modelled her behaviour on what the other children were doing.

Towards the end of 2011 we had to decide what to do for Prep in 2012. This was a huge dilemma. Where was the best place or her? Should she go mainstream, should she go to the ECDU, should she do part-time in each, or should she repeat Kindy????

We deliberated for a long time. I spent a lot of time praying for wisdom. I even made a pro/con checklist for both settings. We wanted to make the right decision. This was really foreign for us because with Melissa and Nick there was no decision to be made - they were both intelligent so they were going mainstream. Our concern with them was 'Would they be teased and would they fit in because of how they looked?'

Jess on the other hand had suspected intellectual impairment so could go to either settings. It was up to us.

After consultation with the Kindy, the Guidance Officer at the SEDU, having a tour of the Prep rooms at the Special School, and then talking to the Special Education teachers and the Principal at my school where she would be going mainstream, we made our decision.

Our main concern was based on the Kindy's comment, that they didn't think she would cope in mainstream because of her defiant behaviour at transition time and her running off. They thought she should repeat Kindy. The Special School GO disagreed with this. The GO thought that it would be better to have a repeat year of Year 12 if necessary so she could have more job training. The GO believed that another year of Kindy would not achieve a lot.

Our decision was...that we would sit on the fence and put her part-time in mainsream Prep (3 days a week) and part-time in the ECDU at the Special School (2 days a week) and we would basically see what she told us by her behaviour where she would like to be. We didn't want to unenrol from the Special School in case Jess didn't cope in mainstream.

Well it didn't take long for Jess to tell us where she wanted to be!

In the first week of school she went to Prep for 3 days at my school, and then she went for one day at the ECDU, due to a holiday. The next week she happily went to Prep and then on the Thursday when it was time to go to the ECDU she didn't want to go! So we didn't make her - we enrolled her full-time in mainstream. She had told us where she wanted to be!

And I must say she looked very cute in her school uniform. My baby was growing up and in school. I had to teach on the first day of school, so I didn't get to take her to her room but I checked on her later on in the day and she was doing well.

Mainstream is the best place for Jess to be.

Here she is after receiving a Superstar Award on Assembly.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission

Blog 44 - Hit the Delete Negative Comments Button

Do you take to heart the negative comments of others? Do you believe the negative things that people have said to you? Have you not done things because people have told you that you couldn't or shouldn't? (Nothing life-threatening of course).


This morning I was reflecting on some instances in my adulthood when I was told by others to not do something. In my strong-will to prove the wrong I ignored their advice and good things came out of my perserverance.

 

You are not who people say you are.

 You are who God says you are.

Don't let people stop you from achieving your God given destiny.

 

 



• 'You are no good at teaching. Go and find another job.' My first principal told me this. She constantly criticised me and failed my probation. She destroyed my self-confidence and she then left the school. I decided that this woman was wrong because I thought teaching was what God wanted me to be doing... the children in my class loved me... so I set about proving her wrong. I worked hard on my behaviour management skills and self-confidence (I was very shy). The next principal who came along believed in me and nurtured me....here I am 24 years later still teaching, helping many children to learn and showing them the love of Jesus.

• 'Don't leave your husband....you may never find anyone else.' This was the advice by my   marriage counsellor when I was suicidal from the domestic abuse I was under. I again ignored this advice. I had made a very poor choice marrying him. I decided to make a better choice by leaving. And if that meant that I was to be alone for the rest of my life that that would be better then where I was. Again God came through for me and blessed me with a new wonderful husband and three gorgeous children.

• 'Don't meet Joe. He might be an axe murderer.' I met my new husband on the internet in a chat room in the late 1990s. He was in the USA. At this time there was a lot of suspicions about chatroom romances. When he said he was coming out to Australia to meet me there were many people who were negative and told me not to meet him. But again God was already there working in the situation. I have been happily married to Joe now for 12 years.

• 'You have no right to have children when you know you have a 50/50 chance of passing on the Crouzon Syndrome gene. It is selfish of you to deliberately pass on a gene that probably means that the children will need painful operations.' Oh yes I have heard this, and seen it written under my youtube videos. I have asked my children and they have told me that they are happy that they were born.... They are not resentful of the fact that they have Crouzons...it is just a part of them... and they have seen many other children who have worse disabilities then they do. They are happy, intelligent, fun-loving, self-confident children...they are a blessing. God made these children the way they are meant to be.

• 'I would never put anything personal on the Internet. You never know who will read it!' In the early days of the internet I made a website about my life with Crouzon syndrome - it is still floating around in cyberspace. If I had listened to what other people said, then I would not have made that website, or the youtube videos, or the International Crouzon Syndrome Support Group on Facebook, or even this blog. If I had not done any of these then there would be a lot of people in the world who would be less educated and many people around the world feeling very isolated who are not isolated now because of me. I have been able to help so many people. Another one of God's purposes for my life.

• 'Oh your child has Down syndrome. I am so sorry.' This was engrained in my psyche. I believed that Down syndrome was a really terrible condition to be born with which is why I became so depressed when she was diagnosed. But you know what? It isn't! Yes Jess has her challenges, but what child doesn't? Jess has blessed our family and she has blessed so many other people already in her short life. I still remember when Melissa was at the doctor's waiting room and there was a depressed teenager there - her Mum said she never smiled. Jess went up to the girl and was entertaining her. The girl smiled! The mother was amazed. Jess is meant to be on earth and God has a purpose for her life.

My kids made up a quick dance before bedtime tonight - here it is. They are awesome.

http://www.youtube.com/watch?v=np6fdH5YeH0


Delete the negative comments out of your mind. Focus on the positive and press on to doing the things that God wants you to do.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission



Blog 43 - Nick has Coeliac Disease - 6 years

Nick was always a small baby and toddler. In both height and weight. The paediatrician had even mentioned in passing, growth hormones, in Nick's future if he did not grow enough.

We started to notice that he was complaining nightly of a sore tummy. We put this down to anxiety.

We also noticed that he was having daily diarrhoea. He did not complain about this so we think he thought it was normal. He has told us that it started in Prep when he was 5 years old.

We also noticed that Nick was getting a pot belly and his ribs were showing and even his vp shunt line stood out.

We were feeding him plenty of food and he never went hungry so we didn't understand what was happening.

Low and behold to our bewilderment Nick looked like this because he was malnourished. He had an allergy to gluten and wasn't absorbing any nutrients from food that contained gluten (wheat, rye, oats, barley). When we looked in our cupboards/fridge at the foods he was eating, and we read the ingredients on the packets, the majority contained gluten.

Nick had Coeliac Disease.

How Nick was Diagnosed

The children had their regular paediatrician appointment and when it was Nick's turn we explained  about his constant sore tummy complaints and his diarrhoea. The doctor looked at his exposed ribs (and vp shunt line) and his pot belly..and of course his lack of growth.

The first thought was a lactose intolerance and we were told to take him off dairy. This did not change any of his symptoms.

He also had to have a blood test which indicated that he may have Coeliac Disease. He was booked in to have a biopsy of his small intestine and the gastrologist would have a look to see what was going on.

The operation confirmed that yes Nick did have Coeliac Disease.  The villae were lying down and black. He would have to go on a strict gluten free diet straight away.

We saw the dietitian at the hospital and we contacted the Coeliac Society who supplied us with resources.

We had a friend a church whose son who was the same age as Nick, also had Coeliac, so they were a fantastic support and source of information.

This was another whole new world of learning. Scary at first. And also for Nick to come to terms with.

It has taken him about 1 1/2 years to be truly comfortable with not being able to eat the food that most kids take for granted.


(After the diagnosis I found out from my mum that there were cousins on both sides of the family who had Coeliac Disease. It had come down the family line on either Mum or Dad's side)

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission

Blog 42 - Op #9 for Melissa - 10 years - Chiari Malformation Repair

Before Melissa had her mid-face advancement done she had had a CT scan of her skull and neck. A shadow showed on the top of her spine in the scan.

After the mid-face advancement was finished Melissa was sent for a MRI to investigate this shadow.

The results were that Melissa had a chiari malformation and a pocket of fluid in her spine called a syrinx. The chiari malformation is where the brain is growing down into the spinal column blocking off the flow of the cerebrospinal fluid.

Melissa was referred to a neurologist at the Mater to be assessed.

The neurologist examined Melissa and said that she had quick refluxes which meant the chiari malformation and syrinx was impacting on her functions. The neurologist decided that she would monitor Melissa and if her symptoms worsened then she would be referred to the neurosurgeon.

Over time Melissa began to notice more symptoms - she would get tingling in her hands and she would have 'drop attacks' where her legs would hurt and fall out from beaneath her, with her landing on the ground. She also began to have headaches.

As we were told would happen, the neurologist referred Melissa to her neurosurgeon.

The neurosurgeon requested a MRI with dye x 2 so he could see exactly what was happening.

Surgery was planned and a date set.

Initially the neurosurgeon told us that he would be performing two surgeries:

1) a revision of Melissa's vp shunt
2) the repair of the chiari malformation

I had heard of 'chiari malformation' on the yahoo email group I was on. I also googled it to learn more. What I googled was thankfully the same as what the neurosurgeon told us he was going to do.

He was going to shave off part of the inside of the top vertebrae and if need be apply a duraplasty to the brain in the gap. If need be he would also put in a shunt to drain the syrinx.

Melissa was admitted after having her MRI which we did not know was happening. I had to have a friend take Nick and I to the hospital after school. The plan was to have the shunt revision the next morning.

Melissa fasted from 2am. The next morning the at 9.45am the neurosurgeon arrived in the ward and told me that he would not be operating until the eye clinic had examined her eyes. They were supposed to have seen her earlier in the morning but no one was available. Melissa ended up having her eyes checked at 2pm - she was still fasting and crying from hunger.

It was after 3pm when we heard that the op had been cancelled because there was no pressure in her eyes. She could finally eat.

We then could not leave the hospital. We had to wait for the physio to come and assess her and for ENT to come and assess her. They arrived the next day.

The neurologist also came and documented Melissa's new symptoms.

The next day we were about to go home when we were told not to go home...that the neurosurgeon wanted to come and see us the next day.

So we waited and waited and waited. Eventually we were told that the neurosurgeon would not be coming to see us and to go home. I did find this frustrating but I've been coming to the hospital long enough to know that things happen and doctors can not always come when they say they are going to.

 

The neurosurgeon's registrar rang us to explain what had happened - the neurosurgeon had been caught up in theatre. Melissa would be booked in to have the chiari malformation repaired.

The operation went ahead. It took about 4 hours and she went to PICU afterwards.

The neurosurgeon took part of the inside of the top neck vertebrae and a 2cm x 3cm piece of the lower skullbone. He then had to patch the exposed spinal cord with a duraplasty. The decision was made to not put a shunt in the syrinx (pocket of fluid) - to let the body readjust on its own.

 

Melissa had a lot of pain with this surgery and a very stiff neck afterwards. We were told that the pain was mainly due to the muscles etc. being moved more than the removal of the bone.

She continued to have some symptoms post-op.

Another MRI was performed and it showed that the syrinx was still there but it was smaller. Time would tell whether more surgery would be needed.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission

Blog 41 - July 23, 2010 - Launch of Facebook International Crouzon Syndrome Support Page

I had been part of the Yahoo Email Support Group for a number of years. Finding that page was like finding my home. After all the years of feeling isolated and misunderstood I finally found a group where there were other people just like me!

I decided in 2010 to begin a support group on Facebook. I didn't want to compete with the Yahoo group, just to add another way of people to connect. I initially began advertising to people in Australia to form an Australian group but I was contacted by Tom Head in the UK who asked me to make it international which I did.

The group has grown and been able to connect people from all over the world.

People no longer feel alone. We currently have over 400 members.

I pray that the group will continue to grow and that we can encourage and support each other...and to also raise awareness that Crouzon syndrome exists.


http://www.facebook.com/#!/pages/International-Crouzon-Syndrome-Support-Group/146204398727264?fref=ts

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission

Blog 40 - Op #8 for Melissa - 9 years - Mid-face Advancement

When we went for Melissa's annual meeting with her team of specialists in January 2010 the plastic surgeon decided that it was time to do Melissa's mid-face advancement. The decision was based on the fact that the suction of the cpap mask was not allowing any growth of the mid-face area and Melissa's face was becoming more and more concave. The cpap machine had to go.

We were told that there were two options available. The mid-face advancement, called a Lefort III, could be done using the RED (Rigid External Distraction using a halo) or by Internal Distraction (using screws which show above the ears). We had seen a child at the hospital with the internal distractors, and we had seen photos on the internet of the RED.

The plastic surgeon decided on the RED because from his research the RED allowed more precise movement of the bones in more than one direction (the bone can only go forwards with the internal distractors).

Luckily I had been on an internet support group and had heard of this surgery and knew basically what was involved and what we needed to prepare.

We bought clothes with buttons, a blender for mashing up food, a very small toothbrush, lots of toys, puzzles, craft supplies, dvds etc. It ended up being a very long 4 month process.

It was hard to explain to Melissa what would be happening because all we had was the Internet photo. We were going into an unknown world (this is the major reason why I made the youtube video - to help others going through the same process).

On the morning of the op Melissa had to be at the hospital by 7 am and was fasting. It was a long 6 hour wait until it was her turn. We saw children come and children go. The afternoon patients were coming in when Melissa was finally called. We found out that they had been waiting on a PICU bed to be freed up before doing the operation.

The operation took 4 hours and we saw her afterwards in PICU. The next day she went back to the ward and the process began. I have made a youtube video to explain what happened. Click on the link below:


http://www.youtube.com/watch?v=GnP12pX1T9s


Melissa found the process very hard to deal with. She wanted the RED taken off, she cried from the pain associated with the stretching of the skin and muscles.

We became very worried about Melissa's state of mind as we could see her spiralling into severe depression.We took her to see the Child & Youth Mental Health Service at the hospital. Fortunately I had made contact with them before leaving hospital as she was very teary in hospital and because of her history of depression, I was concerned that it was beginning again. So they knew the situation and were very supportive.

I will always remember the doctor showing Melissa a deck of cards with pictures of teddies on them. Each of the teddybears had a face showing emotions. Melissa was asked to take out all the cards that showed how she felt. Every card she chose was sad...not one happy bear. My heart broke. The doctor worked with Melissa helping her to cope with her feelings and to get through the process.

Funnily enough she doesn't remember feeling depressed or seeing the doctor a CYMHS. Her mind seems to have blanked it out.

Once the RED came off we had a different girl living in our house! My husband and myself would catch ourselves staring at the 'new' Melissa. She too had to get used to her new face. The day she had the RED removed she told us that she wanted her old face back....she doesn't say that anymore.

 

In the months leading up to the operation Melissa was experiencing more social problems at school. She wanted to fit in and to be liked. As a result she made some poor choices and found herself on detention on a couple of occasions.

 

 

Soon after the operation a butterfly emerged. We noticed that Melissa started to hold her head up high, she pulled her hair back from her face, she become ambitious and self-confident. She would also stand up to her friends. Another plus was that she no longer needed her glasses or her cpap machine.

The surgery had been a major success. The plastic surgeon has since told us that Melissa has had one of his best results - thank you God.

© 2012 by Jenny Woolsey

No part of this blog may be reproduced without prior permission