Welcome to our CaringBridge website. We are using it to keep family and friends updated in one place. We appreciate your support and words of hope and encouragement. Thank you for visiting.
Friends and Family,
You are all truly, sincerely awesome and the love and support we receive every day is amazing.
For anyone new to this conversation, Izzy was diagnosed 2 months shy of her 4th birthday with Neurofibromatosis Type 2 (NF2).
NF2 is very rare, affecting less than 4000 people (less than 400 children) in the US. Also, Izzy is very private and understands that some non-family adults know about her surgery so that they can pray for her, but would be truly devastated if any of her classmates knew she was having surgery. We have always promised to keep this on her terms, so please honor this wish of hers:)
50% of patients inherit it from their parents and 50% are spontaneous genetic mutations.
Most people are asymptomatic until their 20s.
NF2 is a disease in which you have a defect in your DNA that prevents you from making a gene that stops cell growth in nerve
cells (kind of like cancer).
This results in the development over time of multiple, BENIGN tumors throughout the body called schwannomas.
People with NF2 develop multiple other brain tumors called meningiomas and ependymomas.
The hallmark of the disease is the development of schwannomas on both of the vestibular nerves.
These nerves are associated with hearing and balance, so nearly 100% of people with NF2 become deaf, most in their 20s or 30s.
The natural progression of NF2 is that these tumors continue to grow and lead to complete hearing loss and facial paralysis.
Thankfully, we have known about her diagnosis at a point early enough to try to intervene - most people don't find out they have NF2 until it's too late and either they are already deaf or the tumor is too big to save hearing.
Her tumors remain very small (less than 1 cm on each side), but they have progressively gotten a tiny bit bigger every year.
No one knows the best time to resect them, but if they get bigger than 1.5 cm then the odds of saving hearing and preventing facial paralysis go down significantly.
If there is a chance to save Izzy's hearing, then we need to intervene on the tumors involving her hearing nerves before we miss our window to remove them safely.
We've been monitoring these tumors closely and unfortunately the time to try to resect them has arrived.
After 3 unsuccessful attempts to have the surgery done in LA (first Izzy developed pneumonia, then the hospital was bought out and the new owners decided not to allow pediatric surgery, and then rescheduled at UCLA children's only to have the surgeon's credentials lapse because they had been operating primarily at the old hospital) we decided that God was trying to tell us to stay home near family and friends.
The surgery will take place in at St. Louis Children's on Monday June 26 at 7:30am, and is for all intensive purposes, a "brain" surgery with all the additional risks involved with that. She will have a large incision over her ear and they will have to temporarily remove part of her skull.
The surgery typically lasts 5-6 hours and incudes a team of neurosurgeons and ENT surgeons.
If all goes well she will spend 2-3 days in the ICU and at least 3 days on the floor before being released from the hospital.
Right now her hearing is as perfect as it can be; however, there is a 50/50 risk of at least partial hearing loss and a 30% risk of complete deafness from a perfect surgery - odds which are hard to stomach.
Knowing that she could lose another day, or year, or years, of normal hearing just by intervening and trying to save hearing in the future is tough to swallow.
However, if we do nothing, her risk of deafness is 100% and she risks developing facial paralysis.
If Izzy wakes up without hearing in her ear, she will likely receive a cochlear implant at a later date.
If her hearing is ok after surgery, then she will need the same surgery on the other side at a later date.
For those of you wanting to read an article on NF2, we have attached an article written by her surgeon that may answer any questions.
Also there are a couple of decent websites -www.advocurenf2.org (
http://www.advocurenf2.org/), (
http://www.nf2crew.org/)www.nf2crew.org (
http://www.nf2crew.org/), (
http://www.ctf.org/)www.ctf.org (
http://www.ctf.org/).
A particularly nice blog called run4leah chronicles a teenage girl diagnosed with NF2 at 8 years of age and can be found at (
http://leahmanth.typepad.com/)leahmanth.typepad.com (
http://leahmanth.typepad.com/).
We are overwhelmed with the love and support we receive from each of you everyday.
You help give us the strength to battle this disease, the shoulders to cry on, the hugs to squeeze away the pain, beer and wine to get through the tough times, and the hope we need to help Izzy (and Claire and Jane) enjoy life, despite this devastating diagnosis.
Words will never be able to express how much you all mean to us.
Thank you from the bottom of our hearts for all of your love and support.
