Endocrinologist & Build a Bear

Friday we were lucky to get a cancellation apt. and get in to see the Endocrinologist. A miracle really. We reviewed past doctor visits and current bone age scans. A bone age is an X-ray of the hand that helps determine where the bones are at in development. Emma's first bone age was done at age 2 and her bones were only developed to a nine months old. I learned some bone ages are straight forward and you can tell the average age of a kid. Some are not. Emma's is one of those that is not. Some of the bones are 10 months delayed others are 1 1/2 years delayed. Typically a kid with hypopituitarism would be 2 years behind or more. Also we look at the growth curve of Emma's over her lifetime. While she is, and has been since she was 10 months old, been below the growth chart, she has always continues to grow at her own pace. A child with Hypopituitarism will have a sudden drop where they fall off the growth curve drastically. SO WHAT DOES IT ALL MEAN!

Emma will probably not be diagnosed with Hypopituitarism. She has to do blood test and another MRI with contrast of her Pituitary gland, to rule it out and to determine any other problems

What does that mean... The cases where growth hormone resolved a child's chiari and syrinx the child had hypopituitarism. So basically we are traveling on a road less, if ever it wasn't documented traveled.

I explained we understood that growth hormone treatment was a long shot. We have two neuro-surgeons, who agree it is reasonable to try, I figure if it resolved that would be amazing. But if it even just helps a little it might make it so brain matter doesn't have to be removed, or the surgery doesn't have to be as invasive.

She did mention we might have a hard time getting insurance to approve growth hormone. I told her i had talked to the drug companies and they will supply 6 months of treatment on a trial basis. So thats were we are at. We have another apt. in 2 months.

So since it was just Emma and I and it is rare we get to be out just us 2. We got lunch after the apt. and went to build a bear to spend her gift card. She was in heaven and the newest member of our family is Snuggles. She is well loved already.

Wow ~ We were suppose to be going to a 4 hour pre-op appointment tomorrow. Thanks to so many of you who fasted and prayed, put our names on prayer rolls, we will be enjoying Valentine's Day. I just have to say Thank You. Thank you for your faith, love & kindness. We are so blessed to have found the medical journal that had a case where a child condition was made better by growth hormones rather than surgery. We are so blessed to have found a doctor who is willing to try this approach. We realize that we are not out of the woods, yet. We still may need surgery. But for now, we are on a good path. The things i will be praying for now are that...

1. We get into an Endocrinologist

2. Insurance continues to approve treatment

3. Growth hormone is approved

4. The growth hormone resolves her condition

For if there be no faith among the

children of men God can do no miracle

among them; wherefore, he showed not himself

until after their faith

Quick update

I talked to Dr. Shafron at Phoenix Children's Hospital.

He was our Doctor who was taking it to a case study.

He brought up a third option. Of Course! They talked about doing a posterior decompression, but they also thought you would need to stabilize the spine, by doing a spinal fusion also. Then if more problems develop in the future we would have to go in and do the trans oral. I asked him about how many trans orals he does a year and he said none!

I also talked to him about trying the growth hormone as an approach, and he thought that was reasonable.

The good news is for now we won't worry about what surgery to do.

We will hopefully see improvement from the growth hormone and may be if we see enough. We may be able to avoid surgery all together. I realized it's a long shot. But one can HOPE!

Good News

So we met with Dr. Muhonen Friday. Then Sat. Nate and I sent him an e-mail telling him we wanted to see an endocrinologist and try Growth Hormone with Emma to treat her condition. There are some cases where it has been known to shrink the Chiari and Syrinx. There are also cases where it has had the opposite effect, and made things worse. We weren't sure how he would respond since he is the expert, and we are just worried parents : ), yet we were asking him if he would proceed a certain way. We had mentioned it in the appointment and he sounded open to it. We still kind of expected him to E-mail us back that we were crazy. Then just a few hours after, on a Sat. we got a response.

Yes on all your questions. I sent disc out yesterday.
For endocrine see Mark Daniels at Choc. No one compares for sure. I can help you get in. I need to check her every couple of months to make sure her nerves are still working.
Your plan is reasonable to try. Mm

Sent from my Verizon Wireless BlackBerry

I'm not going to lie we were really excited. Not only does he think it's reasonable to try, but he will help us get an apt. with a good Endo. We understand this is a long shot. But we are excited to find an amazing doctor, one of the best, who think our idea is good and worth trying. You may be wondering what this means, and what our plan is! So here goes. Start her on GH, then at 3-6 months get another MRI. If the syrinx is the same or shrunk then we will continue for another 3-6 months. If the syrinx is larger then we will stop treatment and proceed with surgery. As long as this works, she may be able to avoid surgery all together. I don't know. This will not fix the Basilar Invagination. It may create enough room where it is less of a concern, and we will be able to monitor her over the years. Who knows but we will deal with that when the time comes. For now we are happy to have found a doctor who is excited to work with us, that we trust.

Also good news for the week Emma has qualified for California Children's Service. It is kind of like an insurance but just for this condition.

Whew! Alot on our plate right now

First I have to say Thank You to all our friends and family who are praying for us, and who plan to fast for us today. I've heard people say that they could feel others' prayers, but I never understood exactly what they meant till now. Your prayers give me strength. I am overwhelmed with the decision we have to make for our Little Miss Emma Kate. I've seen people who have hard challenges in life and wonder how they do it. I'm beginning to understand how prayers to our Heavenly Father give us the strength to handle big challenges.

Now an update on where we are at. I'm never sure how much details to give. Sometimes this stuff can be overwhelming, and I worry about Emma reading too much info. If you don't know what a word is, don't worry I didn't know them either 3 months ago : ) I am amazed how informative wikipedia is. And google for that matter!

We saw a Neurosurgeon Friday. Dr. Michael Muhonen at CHOC. That brings our total to 3 neurosurgeons.. The one thing they all agree on is that she needs surgery. I have placed them in the order that we met with them. Here are their suggestions for surgery:

#1 Dr. Zouros at Loma Linda suggest doing just a Posterior Fossa Decompression Surgery. This surgery is fairly standard for Chiari and would consist of.... WARNING! scary details to come... a craniotomy (removal of a portion of the back of her skull), a laminectomy (removal of the back part of her first vertebra), a duraplasty (opening up the Dura, the membrane that hold our brain and spinal cord and the (CSF) fluid it floats in and they would add a patch to help relieve the pressure in this area.) They would also most likely remove the part of her cerebellum that has herniated into her spinal column. Then if all goes well and there are no leaks of CSF, or infections that develop she would be out of the hospital after 5-7 days home for 2 weeks, then when she feels ready she can return to school. They would have to shave a section of the back of her hair she would have a pretty good scar, but most of it will be covered when her hair grows back.

Pro's... The surgery is performed about 2 a month by most neuro surgeons. It has an 80% rate of stopping the progression of symptoms OR improvement.

Con... It does not address the odontoid putting pressure on her Medulla oblongata (Basilar Invagination & Platybasia) which is probably the problem that started all this. The long term side effects are not well known from not addressing this. But basically the bone rubbing on the brain stem is not good it can create a callus like thing and irreversible damage is possible.

#2 Dr. David Shafron at Phoenix Children's Hospital. (By the way I love Phoenix Children's Hospital all the staff really care about making positive experiences for the kids.) He told us Decompression surgery would be necessary and also thought more would be needed to be done to address the odontoid. He seemed to be leaning to a procedure called a trans-oral odontoidectomy. Which is where they would go in through her mouth, remove that bone, then may be a day or two latter flip her over and do a spinal fusion to stabilize the spine. Also the decompression surgery (what i explained above) would be done. Shafron took Emma's info to a case study, which has been reviewed, we are waiting to hear from him. Hopefully Monday.

Pros... Dr Shafron is a very kind doctor and he is in AZ. (Where we are moving to in the summer/fall. so we will be doing follow up with him in the future.) Also this procedure seems to address the area of concern directly, negating possible further surgeries in the future. Also this surgery has a higher liklehood of restoring CSF flow.

Con... we don't have AZ insurance, and that is a process! Also the surgery is very invasive. Emma would also have restriction of movement somewhat because of the fusion.

#3 Dr. We saw Michael Muhonen at CHOC (Children Hospital of Orange County). I liked the openness of this doctor. He said the reason Zouros wouldn't do an odontoitectomy is because he has never done one. So of course we asked if HE has! He said he does about one a year and has done 2 in the last 3 months. He has also trained under Dr Menezes who is the expert on this procedure. Muhonen performed about 2 a week while he was training.

Pro... of doing the Trans Oral odontoidectomy. The Problem is addressed, same as above.

Con... It is a much more invasive procedure and while Emma has symptoms, they are not severe. (yet!) It has a much higher risk for infection and problems (naturally it's more surgery.) Her hospital stay would be about 2 weeks, and I'm not sure how long after that till she would be able to return to real life! She would have spinal fusion which would restrict her neck movement.

So is anybody else overwhelmed? You should see me after an appointment with these doctors. My brain wires go all crazy and I start to ramble and make no sense, like in the movies when a robot gets overloaded!

There is also a less conventional approach we are pushing to try with Emma, and that is giving her growth hormone (GH). It is only successful in patience who have hypopituitarism. Emma has definite signs of GH deficiency. We have found a few cases where the chiari and syrinx were reduced with GH treatment. We have also found cases where it was made worse. It's a long shot. We get that. But it's an avenue we want to exhaust before we do surgery. We get the feel that Dr. Muhonen was a bit open to exploring this approach so we were happy about that.

After days like this, it help to take a step back and look at the positives. All three of the doctors are amazing doctors, who are capable to take great care of Emma. She has been taken really good care of by her school, she got 100% on her spelling test this week. Her friends have been great. Her Activity day leaders and girls are fabulous. This week she was the hostess for activity day and she was required to call all the girls and remind them. This is her second time, and she did awesome. The first time she was pretty nervous. This time She did it all by her self while I was getting the kids ready for dinner.

To end on a happy note

A really cute story... Last year Emma ran Marathon... where they run 3 miles a week for about 2 months, then when they have cumulatively run 26 miles, they run the last mile or so with the L.A. Marathon. Last year is when I first noticed Emma was having problems keeping up. BUT she did it. This year she wasn't going to do it, then she started doing P.E. with Kaylyn, a girl at school that is blind. Her and Emma are Kindred Spirits (for you Anne of Green Gable fans!) Emma has been learning how to be Kaylyn's eyes. So I talked to Kaylyn's mom and she is doing Marathon with Emma. So together they will run/walk their miles hand in hand. How cute is that? Squish squish it's brings tears to my eyes.

Thanks for your faith and prayers. have a great weekend