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ron mckenzie
10-11-2004, 05:39 PM
My daughter is 10 years old. She has a neuromuscular disorder called Angelman Syndrome. After going through a recent growth spurt, she also has an increasingly serious spinal curve of 83%. She has worn a brace since the curve began to develop, but it has been ineffective, as Doctors predicted. Her Orthopedic Doctor/Surgeon says surgery is now necessary, before the curve worsens.

My daughter can not speak and has no comprehension of her condition or the prospect of surgery. She communicates only through her frequent smiles and the sparkling of her eyes.

My wife and I are looking for recommendations for Doctors/Surgeons and Hospitals that will offer the best treatment for her. We live in Charlotte, North Carolina, but will take her where ever she will receive the best care.

Any recommendations would be greatly welcomed.

Thank you,
Ron

Alison
10-11-2004, 10:07 PM
Hi Ron

Sorry, my curiosity got the better of me (as it usually does) and I like to learn what different things are and if you don't ask, you never learn :-). I was wondering, what is Angleman Syndrome. It sounds familiar but I was just curious what it is. Hope you can help my curiosity and good luck with your daughter

Alison

ron mckenzie
10-11-2004, 10:54 PM
Take a look at this website. It does a much better job than I can to explain Angelman Syndrome: [URL=http://www.angelman.org]

Mary Lou
10-12-2004, 07:35 AM
Hi Ron...

I'm from Pennsylvania and my daughter has received excellent care from doctors at the Shriner's Hospital in Philadelphia and we just recently found an excellent doctor in Hershey PA. My first suggestion, is to ask your primary care giver for a recommendation. Never give up trying to find a doctor that is experienced in the type of surgery your daughter needs, is atleast familiar with your daughter's other health concerns and also is willing to listen to your concerns and answers your questions.

We went to five different doctors until we finally found the one who is right for us. It is always good to have atleast a second opinion before having surgery, but I think things go so much better if you are completely comfortable with your doctor.

Good luck,
Mary Lou

ron mckenzie
10-12-2004, 11:22 AM
Mary Lou,

Thank you very much, for your response and recommendation. We have heard good things about Shriner’s Hospital.
Her current Orthopedic Doctor has a good deal of experience and is well credentialed, however an acquaintance had a bad experience with him. Understandably, this makes us uncomfortable. As well, we would like to get a second opinion and be certain that our daughter gets the best available care, whether it is in NC or somewhere else. We are hoping that People with experience, like yourself, will make recommendations.

Ron

Carmell
10-12-2004, 12:42 PM
Hi Ron,

Shriners is always a good option. I've heard many good things from parents about the Shriners in Philly. There are two pediatric orthos in NC/SC on my list of experts, but I haven't heard any comments from parents about them, which makes me a little nervous. They are Dr. Jeffrey Shilt at Wake Forest and Dr. Fred Piehl in Columbia, SC.

Remember that each doc may have a different recommendation for your daughter. It's up to you (the parents) to make the right choice for her. Make sure the doc has experience treating children who are similar to your daughter. She is a unique case, but someone will know how to help her. It may not be local, but someone will know.

I wish you the best. Please let us know how things go and what recommendations you find.

ron mckenzie
10-12-2004, 02:21 PM
Carmell,

Thank you, very much for your comments and input. I very much appreciate them.

I have not heard of the doctors you mentioned but I will keep their names and ask around.

Thank you for taking time to respond.

Ron

lrmb
10-12-2004, 07:49 PM
Hi there,

I have also heard excellent things about Philly Shriners, particularly Linda d'Andrea (one of very few female scoliosis surgeons). Children's Hospital of Philadelphia is also one of the best hospitals in the country. I personally found Dr. Dormans there a bit blase about surgery in my case, but having said that, I know he has a great reputation as a surgeon.

In New York there are many good surgeons, among the best I have heard are Dr. Boachie-Adjei at Hospital for Special Surgery and Dr. Michael Neuwirth (not sure if he does pediatrics though).

Good luck Ron. Your daughter is so lucky to have you. ~Laura

ron mckenzie
10-13-2004, 10:32 AM
Thank you, Laura. Your comments and recommendations are very helpful. I have heard Dr. d'Andrea's name mentioned before.

Do you know, or have you heard of any doctor who is considered the preeminent pediatric authority/Surgeon on Scoliosis?
We are very reluctant to put our Daughter through this surgery, we want to make certain we are doing the right thing for her. Though she has Agelman Syndrome and Scoliosis, she is in very good health and is a very alert and happy girl. The last thing we want to do is impose any pain in her life.

Thanks again for your comments.

Ron

Carmell
10-13-2004, 12:26 PM
Hi again, Ron,

You asked about who the "preeminent pediatric authority/Surgeon on Scoliosis.." is. I don't think there is a right answer to this question. I know there are a handful (and the list is growing) of pediatric scoliosis specialists who have dedicated their lives to helping kids with unique and complicated cases. Scoliosis is a generic term. When you break down the causes for scoliosis, there are several areas where a specialist may come in handy. For example, my son was born with severe congenital scoliosis, but is otherwise a healthy child (i.e., no neuromuscular disease or anything else that would complicate his treatment for scoliosis). We searched for someone who had extensive experience in treating children who had similar issues as our son. Someone else may need a specialist who knows the extensive complications involved with other medical issues that could impact treatment for scoliosis. While yet another child may have infantile idiopathic scoliosis and needs to see someone who has extensive experience in early treatment (i.e., casting, etc.) for these kids. Its amazing how different each individual case is. No wonder there isn't ONE prominent and right surgeon.

Having said all that, there are a handful of surgeons in the country who I would trust my child's life with. Dr. Emans in Boston, Dr. Smith in SLC (our current ortho), Dr. Betz in Philly (Shriners), and several others. I think having a surgeon who is willing to help you search for answers is good. Someone who thinks they KNOW all the answers may not be "up" on the latest (but not necessarily the greatest) treatments. There is a lot to consider.

Good luck and let us know what you decide for your daughter.

ron mckenzie
10-13-2004, 12:59 PM
Carmell,

Thank you, once again, for your comments. They are very helpful. I appreciate you taking the time to help.

You have given me some new direction for thinking. Finding a doctor who has a great deal of experience with children with neuromuscular conditions, may be the best choice for our daughter.

There is so much information and so many doctors, I just feel that I don't have a good grasp on the situation.
We have a consultation with her current Doctor next week. We are preparing a list of questions. This session may also assist in our decision process.

Thank you also, for the names of the doctors. I will certainly add these to our reference list.

Hopefully, a clear course of action will present itself as we try piece together all our options and resources.

Ron

lrmb
10-13-2004, 11:55 PM
Hi Ron,

Just reading these posts and thinking how complex and intimidating it can seem. I wanted to echo something Mary Lou said in an earlier post: it can be useful (if frustrating) to see a few surgeons before making an ultimate decision about who gets to do the surgery. From my own experience, I saw three, and all were radically different, and I am still seeking a good match for my own various needs (trust, financial, etc). I know it can feel like such a long road, but it will be worth it in the end.

~Laura

ron mckenzie
10-14-2004, 10:50 AM
Laura,

Thank you again, for taking time to comment. We plan to speak with a number of surgeons, before making a decision.

If you don't mind me asking, it what ways were the doctors different?

Thanks,
Ron

Mary Lou
10-15-2004, 09:18 AM
Hi Ron...You didn't ask me how the doctors were different, but I'll give you our experiences anyway. Doctor #1-treated my then 12 y.o. daughter like she was two years old; never answered our questions; had no clue what CMT was (CMT is a genetic disease/condition which my daughter shows symptoms of and there are atleast four generations in our family with this condition);more concerned about my daugher's Kyphosis than Scoliosis; #2-not concerned at all with Kyphosis; told us it was nothing to worry about (79* at the time); didn't seem to suit our expectations; #3-wonderful doctor!; wasn't affiliated with a hospital that could do surgery; #4-Dr. Betz-Philly, PA-wonderful doctor; seemed knowledgable/concerned about possible CMT; six months-one year wait for surgery; (daughter doesn't want to wait that long); #5-Dr. Lee Segal-Hershey, PA-current doctor-surgery scheduled for Dec. 7th; wonderful personality; very concerned/knowledgable with CMT; calls me on the telephone whenever I have a problem/questions that I'm concerned about; told us more than anyone about the CMT/Kyphosis/Scoliosis connection; we trutst him 100%!

Hang in there, you will find the doctor that is right for your whole family.

Mary Lou

ron mckenzie
10-15-2004, 09:42 AM
Mary Lou,

Thank you for sharing your experience. I see what you mean.
We have our first consultation next week. I guess we will have to go from there. We have now, also secured a sponsor for Shriners Hospital, so we will see how that goes as well.

Thanks for taking time to respond.

Ron

lrmb
10-15-2004, 07:21 PM
Hi Ron,

Mary Lou pretty much made the point. Even though i had heard doctors would be different, I was surprised at just how much.

My first opinion told me that I should do surgery as soon as possible (I'm 28, with an upper curve of 46, slowly progressing), that it would take me 3 weeks to recover with no major side-effects. He was a bit blase for my taste--he called the surgery "easy"! Maybe I would have been an "easier" case than some he takes, but it was a bit of a warning flag that he wouldn't suit my personality.

The second opinion told me I was in great shape, that I might never need surgery if the curve didn't progress any more, and that it would be "no problem" to have surgery in my 40s or 50s. He seemed trustworthy, but certainly was not particularly interested in my individual case and was a bit abrupt with my questions.

My third opinion fell somewhere in the middle, recommending I delay surgery until my curve reached about 55-60, when progression would be inevitable. He took plenty of time in answering my questions. However, I really took a dislike to the attitude of some of his office staff, so that might influence my future treatment options. (I get the impression you have to deal with the office staff a lot during recovery and for insurance purposes.)

As you can see, a lot of my "issues" with the surgeons were personal impressions about how I would be treated along the way if I trusted them with the surgery, as well as their medical recommendations. From what I've read, knowing and trusting your surgeon can really help make the experience somewhat less stressful. One thing I learned from Dave Wolpert's book, which didn't apply to me as I went the rounds this time, but which seems very important, is to have the doctors specify which vertebral levels they would fuse in surgery. Even one vertebral level can make a difference to future mobility and risks. (Have you red the Dave Wolpert book? It's really great. You can get it from this website, if you call they might rush it to you as well.)

All the best with your appointments. We'll be hoping you find somebody right for you and your daughter.
~Laura

ron mckenzie
10-18-2004, 09:56 AM
Laura,

Thank you, for sharing your experience. I appreciate hearing about what you have learned.

We feel pressured to have this done quickly, because of the severity of her curve, but we want to make sure we are doing what's best for our daughter.

No, I have not read that book. I will look for it on the website. I very much appreciate your comments on the vertebral levels. I feel there is so much I do not know.

Ron

jc3
10-19-2004, 12:37 PM
hi laura
i was curious as to which three drs. you went to. If you don't want to say their names on the boards could you send me a private message or an e-mail my e-mail is
jc3palm@aol

thanks. My daughter's surgery is in 2 weeks, she has a 52 degree curve and she is also having some ribs removed.
good luck
jennifer

ron mckenzie
10-19-2004, 01:38 PM
Laura,

Can you please tell me how to get Dave Wolpert's book and it'sd title?

Thanks,
Ron

cowprintrabbit
10-19-2004, 02:09 PM
You can order it from http://www.curvedspine.com/

I just ordered the download version, and have not gotten very far yet, but it seems really good!

I just finished reading "The Scoliosis Sourcebook" by Dr. Michael Neuwirth (reccomended to me on here) and it was really informative, too.

Christine

ron mckenzie
10-19-2004, 02:56 PM
Christine,

Thank you very much!!
Ron

LindaRacine
10-19-2004, 04:08 PM
You can also order the Scoliosis Surgery book on this website at:

http://www.scoliosis.org/store/books.php

--Linda

ron mckenzie
10-19-2004, 04:10 PM
Thank you.

Ron

dare2care2005
10-26-2004, 08:48 PM
hi ron,

i have a cousin who has Angelman Syndrome. she's 17 years old and a sweetheart. she always smiles when she sees me. like your daughter, she can't walk or talk and she's tubefed (don't know if you're girl needs that). anyway, i wish you and your angel luck



Ginny

ron mckenzie
10-27-2004, 10:40 AM
Ginny,

Thank you for your wishes. No, she is not tube fed. She actually has a healthy appetite and can use utensils with assistance. She can't walk, but she trains everyday on a partial weight bearing treadmill. We hope she will walk with assistance one day. She is initiating steps on her own, after a year of training.

We had our first meeting with her Orthopedic doctor/surgeon, last Wednesday. He is recommending fusion from T-3 to the sacrum. This sounds very radical to me. He says she may loose some or all of her ability to take steps. I was wondering if you or anyone would like to comment on that?

Thanks again for your kind response.

Ron

dare2care2005
10-27-2004, 03:17 PM
That is likely (but not definetaly) that she will lose her mobility, but it would be alot better than to have the scoliosis get worse and cause trouble for her lungs and heart. she could die from her scoliosis if it does get bad enough (i'm not trying to scare you) but it would have to get into the 100 degree category for that to happen

ron mckenzie
10-27-2004, 03:37 PM
Thnaks, for your input. We will be seeing other doctors. I just want to know that this extent of fusion is necessary. I want to be certain there is no alternative.

Ron

Carmell
10-27-2004, 04:51 PM
Hi again, Ron,

The fusion recommendation you mentioned sounds very radical to me. That is a HUGE amount of the spine to fuse. Fusing to the sacrum restricts more mobility than fusing the entire thoracic spine. I'm glad you are getting more opinions. Hopefully the other opinions will be by a surgeon who has experience with patients your daughter's age and with similar medical backgrounds. Good luck and keep us posted.

ron mckenzie
10-27-2004, 05:21 PM
Carmell,

The doctor who suggested this has a great deal of experience with these surgeries and with children with similar conditions. I asked him if there were any alternatives to such a radical procedure and he said "No". In fact, I rephrased the question and asked what options there were and he said "none".

We have an appointment with a doctor at Duke Medical Center, in a few weeks. We have copies of my daughter's x-rays, which we will be taking along. We are also waiting for an appointment with Shriners Children’s Hospital, as well as continuing to explore other hospitals and doctors.

Thanks, for your comments.

Ron

Alexandra Jones
10-28-2004, 12:11 AM
Hey
My name is alexandra and I am 25 years old, I I was you I would try Shriners they have already helped me so much and I am looking foward to My surgery on November the 3rd in portland oregon. Shriners helps kids enfant to 18 all you have to do is find the nearest shriners club type place or waht ever they call it and get an application from them.

hope this helps
Alexandra

ron mckenzie
10-29-2004, 01:58 PM
Alexandra,

Thank you very much, for your comment. I wish you great success with your surgery. I'm sure you do fantastic.

Yes, we have entered an application to be seen at Shriner's, we are waiting for a response from them now.

Thanks, again. I'll remember you on Nov. 3rd.

Ron