auriculo condylar syndrome


The Day Bodee Was Born

Date of article 15.02.2004

The day Bodee was born was exciting and terrifying at the same time. Excitement at the fact that our baby boy was finally here and terrifying as we were being transferred to National Women's Hospital due to Bodee's small jaw. The thought of breathing and feeding problems was not something you really want to face when holding your baby for the first time. Bodee proved the doctors wrong for the first of many times that night by feeding with ease, he latched onto breast feeding like an old pro and never looked back, as for his breathing we were told he was breathing easily. He did not need a ventilator at all but we would be taking him home with an apnea monitor is case problems did occur. He was put in Special Care Baby Unit {SCBU} for the first five days where he thrived after getting over a small bout of jaundice. Day six arrived with him up on the ward with me and we could finally get to know each other a bit easier. Day seven and we were off home and starting life as a new family of three. Six years on and Bodee is an awesome kid doing all the things kids do, learning to swim, riding his bikes, going to school, playing rugby league and generally enjoying life as only kids can. The road to this point has definitely been an interesting one to say the least. In the last six years Bodee has become a brother to Zavier, a real little cutie who sadly died on the day he has born due to a placental abruption and to Kayla, his gorgeous sister who has the same syndrome as Bodee. The amazing thing about Bodee and Kayla is that their due date for being born was the same, being the 30th May. All my babies are May babies with Bodee being the 21st, Zavier the 26th and Kayla the 8th. To me Bodee and Kayla are like twins, only that they are six years apart!! Kayla's entrance into the world was pretty dramatic to say the least and our lives are going to be busy from now on. Bodee already has many operations under his belt, with many to come and Kayla will be the same. This thought can be overwhelming at times but with the support of our family, friends and the kids wonderful doctors I know we will all be fine. I know that we are up to the challenge that life brings us and I will always strive to bring Bodee and Kayla up as 'normal' {whatever that means!!} as possible. Now that Bodee no longer has a trachey the only limitations that he has due to this syndrome are when he is going through a jaw distraction process, otherwise it is all systems go at 100 miles an hour! With Kayla we have the extra challenge of feeding issues due to her micrognathia being more severe but over time this will be resolved and her path will be similar to Bodees'. At the moment with her trachey she will not be allowed to swim but as she grows she will be able to do most of the things other babies and toddlers do, she will just have to watched a little bit closer. At times you feel that you have absolutely no control over your life or the life of your children and the best I can suggest is to ask lots of questions and make sure you understand what you are being told. If you don't get it, ask again and again until it is clear. I have found that if you aren't afraid to question the professionals, they are more than willing to explain everything to you. Six years ago, when Bodee was born, the diagnosis was 'a variant of Treacher Collin's Syndrome', now that Kayla has arrived it has changed to 'Dysgnathia Complex', to me it doesn't really matter what it is called as long as your children are looked after correctly. This process should start when you are pregnant with the diagnosis of micrognathia from ultrasound scanning. If a profile cannot be seen at the 20 week scan then another scan or scans should be done until this is achieved. If Kayla's micrognathia had not been picked up on the scan we have been told that she would have either died at birth, or had severe respiratory distress causing brain damage! Luckily for us, due to Bodee having a wee jaw, the doctors demanded profile shots of Kayla in the womb, even though we had been told that we had a less that 2% chance of having another child with the same syndrome. Whilst in hospital celebrating the wonderful and safe arrival of Kayla I met a mother of a baby whose micrognathia wasn't picked up on scan. Her baby did suffer respiratory problems at birth and has some brain damage due to this. Unfortunately I have heard of quite a few other instances of this. The newest challenge has been getting used to feeding Kayla via, first her NG (nasal gastric feeding tube) and then a gastrostomy tube. It took a bit of getting used to not being able to feed her 'normally'. She is still, at eight months old, unable to eat or drink anything orally but we let her 'play' with food when she is having a g-feed so that she associates food with her tummy getting filled up. This is an important thing to do so that when she is able to swallow food properly it won't be a foreign idea to her. It is fun to watch her put baby food in her ears, up her nose and in her hair just like Bodee did at the same age!! November 2004 - Well time for an update, Kayla is now 2 and a half and Bodee 8 and a half and they are doing great! Kayla got her first lot of distractors on March this year and the whole process was quite different to Bodee due to her being so much harder to operate on due to her jaw being so much smaller than Bodee's. Kayla had to have a Red System distractor which was attached to her skull with 5 bolts on each side of her head, which was a really scary thought due to the fact that her bones are still pretty soft being so young. On the day of the operation we were all very nervous, including Bodee! It doesn't get any easier no matter how many times the kids get operated on. Even thou the Red System distractors seemed real scary at first they were really easy to deal with and Kayla coped amazingly with them. These distractors aren't bi-directional and only pulled her jaw out so in May the surgeons had to put on the normal distractors so that Kayla's could be pulled down as well. Because her jaw is so small instead of being able to go screw all the pins into the jaw bone the surgeon had to screw the top pins into her cheek bone and again Kayla coped like a star. All in all this whole process from start to finish was six months long and yes it was very tiring. Kayla fell over three times over that 6 months which caused damage to the equipment, this seemed to hurt the distractors more than herself and the 3rd time she feel she actually broke the top part of the Red system completely which lead to her going to theatre and getting everything removed. Luckily for us the whole distraction process was finished and her bone had hardened when this happened!! Going out to the shops with Kayla having all that hardware on could be quite frustrating at times as some people can be quite rude which upset Bodee, but on the other side of the coin we also had alot of lovely people who were caring and friendly. When Bodee had his distractors on I used to get really defensive but with Kayla I was alot more relaxed when I was out and about and I feel I coped heaps better when the rude ones. The change in Kayla's jaw is huge, she can stick her tongue out of her mouth, we can see into the back of her mouth and see her soft cleft palate. The surgeons are really pleased with the results so far and are now coming up with a plan for the next stage, whether that is more distracting or jaw hinge creation only time will tell. Kayla still does not eat and that is something we are now trying to work on. She is starting to show a real interest in food again so hopefully we will start having some success in getting her to swallow drinks & food so that we can one day get rid of her mickey button. It's fun seeing her reaction to the different tastes she is experiencing and so far she seems to be a savoury girl more than a sweet one!! Kayla also still isn't talking so we are carrying on with learning sign language and Kayla has a talking computer which we are trialing so that is another work in progress. She uses her talking valve all the time and makes a lot of cool sounds which sometimes are very simple words so only time will tell on how this all goes. She definitely gets across to everyone what she wants which is the main thing!! As for Bodee, he is growing up so fast it's scary. His jaw is starting to look pretty small again so he will no doubt be needing distracting again sooner rather than later. His main concern about that is missing Rugby league!! So we will have to have a chat to his surgeon about that one. We have only recently found out that Bodee has a sub-mucous cleft plate so that is getting investigated soon to so that will lead to another operation for him to get it repaired at some stage. Sometimes it certainly does seem never ending but I feel so lucky to have two healthy and fun kids and no ones life should never be too dull! I will sign off here, watch this space as our great kids grow and learn and teach us more than we ever thought possible. If you would like to add a picture of your child to this site please go the the Post a Pic page. We look forward to receiving any feedback, tips, questions and pictures you wish to share.

Cheers Deryn