Friday, March 21, 2008

Cranial Facial clinic - a shopping list of plastic surgery

31 March: Surgery scheduled. Pressure equalising tube surgery for ears (to correct fluid build up) and L&B Scope for Trach http://www.entusa.com/ear_tubes.htm

A much anticipated 6hr appointment with the Cranial Facial Clinic today. This is the team responsible for a multidisciplinary approach to evaluating and treating kids with a complicated diagnosis. "The implications of a craniofacial disorder on a child’s life are far-reaching. They encompass basic health and functioning, family and social relationships, quality of life, and adaptation in each stage of growth. "

Sometimes we bear the weight of water like a deep sea diver and the response is to be pulled ever deeper into an abyss, or like today, to laugh at the complexity. Throw anymore at us and the diving suit's already so heavy, it could hardly make a difference.

So nurse Christine and I do the rounds of the Cranial Facial panel: Plastic Surgery, Psychology, Genetics, Speech, Audiology, Otolaryngology (ENT), Dentistry.

Plastic Surgery
There's a little space that sits on the bridge of Reuben's nose, a slope that unites his beautiful eyes and which I declare is my favourite place to kiss, one of my favourite places on earth. And within minutes, Dr Hammoudeh designates it a CHARGE zone, a webbing that can easily be fixed with plastic surgery. Will I look at that same place again with such sentimental innocence? I know his delightful face so well and yet it's like a secret someone forget to mention before.

Medial canthus webbing between eyes - as above, will undergo a canthoplexy surgery to remove the widened skin which has the effect (apparently) of making his nose look wider than it is. We'll return in 3 months to put him on the surgical schedule. I think we need to look into this more and make sure it's the right thing to do although as Dr H said, it'll remove the "syndromatic look" Reuben has. Blissfully unaware mama.

Asian eye the crease between the ligament and muscle being amiss can be corrected through cosmetic surgery. Given the delicate nature of the skin at present, this surgery will be for a later date but will help in the long run not only aesthetically, but by removing the irritation of his lashes on his cornea. The surgery is called a blepharoplasty or eye lift! Now he really will look like a Los Angelino.

Microtia - surgery will be when Reuben is 4/5 years old to reshape his eyes by bilaterally restoring the heelix cartillage. Reuben has mild microtia. I remember when he was delivered thinking, Look at his little pixie's ear. Little did I know. The effect will be that from behind too, his ears will look symmetrical.

Nerve transplant - I am aghast at the prospect of a nerve transplant in the future, undertaken via a traditional jawline incision which could potentially restore Reuben's smile to the right side of his face. Rocket science indeed. Reuben has only mild facial paralysis to his 7th cranial nerve which means that he can still do a full pout or raspberry blow. This paralysis is one of the key features of CHARGE although not present in all kids and is not just aesthetic, but of course relates to speech, feeding and all oral motor development so is pretty significant.

Craniosynostosis - this is rather frightening. A child's brain undergoes rapid growth in the first year of life and to allow for this rapid expansion, the skull is made up of a number of bones rather than a single bone. They are separated by structures called sutures (expansion joints) that allow the brain to grow without being restricted. Craniosynostosis occurs when the sutures either fuse or are unable to expand. Dr H saw very mild temporal narrowing, not enough to warrant a CAT scan, but enough to put Reuben on the watchlist for what I would imagine would be an awful surgery.

Otolaryngology
ENT assess Reuben's ears and discover fluid which doesn't appear to be causing him distress but would certainly be painful at altitude or flying which is our intention in May. Apparently it was noted in August too. How did I miss that??? Reuben's booked for a ventilation/pressure equalising ear tube placement surgery on 31 March along with the much awaited L&B scope. That will determine the extent to which his throat, traumatised by 10 weeks of intubation and leading to the trach, has healed (we kept saying how do you know unless you look before the planned L&B scope in October although the reply is always Experience tells us and Knowledge isn't enough of a reason for anaesthesia). But given he'll need anaesthesia for the ear tube surgery anyway that gives us the opportunity to scope his throat too. Bingo. Communication impairment is my biggest gripe with the trach as the maintenance is pretty easy given Reuben coughs up most of his secretions in the day by himself although needs a helping hand at night when lying flat. Even our recent at first terrified sisters found this to be the case. There is some fibrous material around the trach stoma but no plans to laser as it's not affecting weekly trach placement. Must catch up with MK and other mums re their kiddos' tube placements, incidentally, the most common surgical procedure on kids.

Audiology
Discuss Reuben's responses with his BAHA hearing aid and his increasing ability to determine the direction of sound. I still want to get to the bottom of his mixed loss, conductive (left) and sensorneural (right) and whether the tubes may make a difference at all to his hearing abilities. Making great hearing strides though though sadly no ABR hearing test today which I'd hoped for. Must wait 6m months.

Speech pathology
I'm disheartened to hear that even with the trach, Reuben should be making basic sounds like "p" and "d". We discuss the placement of a Passay Muir valve which will direct airflow from his trach to his mouth to allow him to vocalise. He'll need ENT clearance first so I just ask the pathologist to speak to Jiffy his trach nurse, and have to show an ability to manage his secretions. Reuben scores a borderline average on his cognitive abilities (fine with that as determined in his recent Occupational Therapy Assesments), but scores lower on his receptive (currently at 6 months) and expressive language skills (very low). I'm disheartened too to hear that although ASL (American Sign Language) will be a predominant mode of communication for him, being such an expressive language, how does that work when you can't express with one side of your face? How unbearably cruel hey? But I do show off Reuben's little tricks, like "Where's mama's nose?" which he points happily too although I'm encouraged by the pathologist to be a little more functional in my education! Reuben is recommended for Infant Stimulation to add to his current PT and OT and I walk away with a referral letter for the same. This is all about playing and having fun and will fine tune his fine motor skills, nesting, piling, knocking over etc. The ST did say that Reuben's communication difficulties are likely structural rather than neurological, ie the trach, the uncertainties of CHARGE and his 5m hospitalisation have held him back but he can still catch up. The trach of course interfers with so many other things too like taste and having the valve fitted should make a difference in terms of his secretions and restoration of some senses to his mouth.

Dentisty & Orthodontics
Reuben's 6 innocent teeth are examined and I'm counselled on the potential problems associated with low oral feeding and drinking, calculus leading to gum damage which normally only occurs in adults but which is also prevalent in tube-fed kids.

Genetics
I think the Dr is surprised I'm up to scratch on my CHD7s, mosaicism and all yet we have a long meeting about genetics, risk of reoccurence etc all the same.

Psychology
I'm interested in the behavioural aspects of CHARGE as relates to Reuben. His personality is one of immense patience (6hrs in clinic without fuss) and calm, yet he thoroughly enjoys the excitement of a swing or a good tickle. He's very tactile and enjoys eye contact which I'm hoping will rule out autism. Wait and see. He is a wonderfully kind and sweet boy. We often say, He's no trouble despite his medical complexity. I truly, sincerely, could not have dreamed of a sweeter natured son. Thanking God on Good Friday that he's so easy.

Six hours later, we leave the hospital with an imminent shopping list of surgeries for Reuben.
- ear tubes
- microtia reconstruction
- canthoplexy surgery
- nerve transplant
- blepharoplasty

to add to the already reviewed, descension of testes following ultrasound, procedures like the L&B and new gear like the passey muir speech valve! I'm surprised if anyone's reading this, but if you are, imagine living in it?

I'm physically and mentally exhausted when Jason picks us up. Writing this now I can't believe the complexity of CHARGE as relates to Reuben.

9 comments:

Kristi said...

My... you've been busy!
"UGH" about the Bard... can't get break in that area, can you?

Aren't the complexities of our kiddos astounding?
I remember our first trip to our Cranial Facial Clinic/Panel... it was exhausting!
Kudos to you for being able to gather and absorb so much info. AND... you are so lucky that Reuben was such a trooper!

And, yes... I read the whole thing! :)

hugs,
Kristi

Jacob's mum said...

Hi Catherine,

Definitely discuss some of the more experimental surgeries like the thing to the bridge of the nose and the facial nerve surgery with other parents on the Charge List.
I have not heard of any one here in the UK having the narrowing of the bridge of the nose to lose the "syndromatic look" is that purely cosmetic or are there any medical benefits at all?
I have met one family who had the nerve surgery, it was not a sucessful surgery and left the child (now a young adult) with much more noticable problems than the original facial nerve palsy. The mum was not happy with the outcome and regretted proceeding.
I understand that this was many years ago and things may have moved on - particularly as you are in USA rather than UK, however we were also advised that US consultants, where procedures are paid by insurance are much more "proactive" in recommending surgery than the UK (where as you can imagine the emphasis is to conserve NHS funds and avoid spending on unnessary surgeies where possible).
Anyway, just wanted to say you are right to talk to many other parents before you do anything you feel you may have reservations about......
Alexis

Jacob's mum said...

Me again, Neil and I have been studing piccys of Jacob at birth and the wide brdge of the nose he has definitely looked more pronounced when he was a baby compared to how it looks now.
Neil has made you a little photo montage showing the changing looks of Jacob's face....! I'll email it over!
Alexis

Kristi said...

Hi again, Catherine...
I wanted to add to the comments made by Alexis regarding the facial nerve surgery... definitely explore/research the risks vs. benefits.
When this option was discussed with us regarding Gracie, we were told that there was about a 50% chance that the functioning nerve could be damaged... a risk we weren't willing to take. She must have a million different expressions and I know so much about her from each one of them... can't imagine losing that for her or us. And, my favorite place to smother her with kisses is on any of her several little dimples that form when she grins or smiles... wouldn't trade that for the world.
We were also told at the time that the surgery should be done before the age of five because much past that point the muscles in the face will not be as easily trained, thus losing some of the benefit of the surgery. We have not been told that advances have been made which lessen the risk of permanent damage... and she will be five next month... so our decision stands as is.
Regarding her nose... same with what Alexis said about Jacob... her nose is far less noticeable than when she was an infant/toddler. However, she was just prescribed glasses and it was a challenge to find a frame that fit her face and nose. She gets little marks on her bridge after just a few minutes - - so something to think about.
Also... thank you for all your detail in your post. We go back to our panel in July and I will be armed with a few questions for them thanks to your detail! :)

On a side-note about teeth... Miss Gracie LOVES the dentist and having her teeth brushed/flossed... all that oral stimulation and strong input in her jaw makes her very happy! My suggestion on this is to start brushing now... get Reuben used to it ASAP. We now use a Sonicare toothbrush and she will request her teeth to be brushed! We also go to the dentist (health permitting) every three-four months for a good "scraping" of the build up. I can only hope that Preston likes the dentist as much as she does! I won't hold my breath, though! :)

Kristi

Geraldine said...

Sounds like they are giving you the grand wish list of everything he could possibly need/want for the next few years. I think the cranial facial nerve surgey sounds like they're going for the perfect situation and by the sounds of it, it will fade within time. I'd wait and see for this one. The smile surgery is both cosmetic and important for eating/smiling functions so very important.

Take each day as it comes, focusing on what's important each day.

Wishing you all a happy Easter. Lots of love Geraldine and Mark
x

ellen charge said...

ok heres all my opinions as you asked for on the list i bascily wanted to read the blog first LOL well ear tubes all chargers need them at some point really good dranige and stuff scope for trach another good thing these toehr u\ops his face yes hes got parlysis lots do it is common but but if he is ok if these things dont stop him form doign things my opinion is y perform them many hugsz ellen

Mighty Max! said...

Thank you for your thorough post...reminded me of a few issues that I need to discuss with Max's team. In particular...the eye "lift". I talked with his doctors about that during his initial stay but obviously at the time, we had much more serious issues to address. But I just started noticing more and more recently that his lashes are dragging...especially with the lubrication to protect his eye. Putting a call into Cranio tomorrow. I have never heard of the nose bridge surgery. As far as the facial nerve...hmmm, that's a tough one. Again I talked with his team alot in the beginning about this (I was obsessed for awhile) and they said there was research being done but they wouldn't recommend it unless it was complete palsy on both sides. I will let you know what our team says when I ask again. I probably will not go for it with Max simply because I cannot risk him losing what he does have. And let me tell you, it is amazing how much he can convey with his face and sign. Don't worry about that at all my friend!

Again, thanks for the informative post...you are an incredible mother and advocate! YAY!

Ericap said...

Catherine, so much for you both to take in! However, at least you are getting good attention and fingers crossed for the oesophagus/trach scope on 31st March, tell hope his oesophagus is healing well.
Yes, a wish list from the Drs and I think it sounds that your are getting some great feedback/advise from the other mums of Charge children on their experiences of surgery, risk/benefits decisions to be made. You and Jason ultimately decide what you think is in the best interests of Reuben, not them, he is your baby.
With love
Erica

Pyro said...

Hi Cath,,
I read your blog and...your kiddo
Reuben undergo a lot of facial lists..so many..first time I encounter like this..But I know your kid is strong and can take on anything..We parents can do everything for our children..So..
Keep praying and I hope it'll be success..All is Well..Keep it always in your mind..

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