Tuesday, November 9, 2010

It Has A Name.....

So the verdict is in, well sort of.  :)   As is common with Claire and her situation, the scope answered some questions, yet the doctor still wants more information before he makes a definitive decision on whether to do surgery or not. 
The muscle above Claire's esophagus was contracted again (hence the above diagnosis) but not as contracted as previously.  The muscle was dilated again and the hope is that it will stay in place long enough for the swallow study to take place (and that Claire will cooperate for said swallow study) and he can make a decision on the surgery.  We have a swallow study and follow-up appointment scheduled for December, and he would like to see that information and move forward from there.  While I hate the waiting, this is okay as I don't think we would have been doing surgery in the near future anyway secondary to Baby Durham's approaching arrival.
The BIG news from this: we have a name for what Claire has:  Cricopharyngeal Achalasia.  She retains her diagnoses of dysphagia and developmental delay; however, this "new" diagnosis could explain her dysphagia and other recurrent symptoms.  It would be very nice if this was the case.  This condition is extremely rare in children and is often looked for only after everything else has been eliminated as causes or reasons for the swallowing problems.  It is apparently even more rare that surgery is needed, but it seems more likely than not we will be headed that way.  Again, hard to tell at this point.  The doctor here gave a very good explanation as to why the other doctors had not found this, which helped Jonathan and I to better understand the situation and how this is only now being considered as the "problem".  We are very thankful we have found a doctor who is able to help Claire and may finally put an end to her "suffering". 
The doctor also looked at Claire's airway.  The inflammation, redness, etc was still there.  As of now, Claire's airway is functional, which is a good thing.  However, the reason for the inflammation needs to be determined.  He still feels it is allergies and plans to send us to an allergist for further workup.  He referred to Claire as a "special situation/case" with the words "as you know".
That is the thing I take most from this situation.  We may not get answers quickly or as often as we want.  She is just too rare.  It is hard to imagine in this day and age of medicine, but we got the "rare" disease.  Guess someone has to have it.
Claire continues to be a happy and loving child.  She remains skiddish and sensitive but that may be her nature her whole life.  Kids in the medical system for long periods, especially at young ages, often develop those personality traits.  She is picking up more words every day and is more adventerous at therapy these days.  She actually looks forward to school and therapy and more often than not goes readily to her activities without crying for mom or dad. 
Not to be left out, Maggie continues to amaze us each day.  She loves school and dance and is very protective of her little sister.  She says she is looking forward to a little brother we shall see..... 
Thanks for all the thoughts and prayers and well wishes!  I will try to post if something exciting happens.  Otherwise, we will be getting ready for the arrival of baby Durham in early December and the swallow study/follow-up visit.

Monday, November 8, 2010

The Night Before a Procedure

Well, Claire will go to the hospital for another "scope" tomorrow.  It will be her 8th time under anethesia (don't think I forgot one...).  This should be a relatively fast procedure.  She should be in and out.  I imagine we spend more time waiting for the procedure then she does in the procedure.  I am not sure how long they will keep us afterward as Claire is usually very cranky and difficult after anesthesia so they may keep us a bit to ensure she calms down and doesn't need oxygen or anything.  However, I envision a relatively short day.  I am not sure how I feel about all this and what I want to "happen", honestly.  On one hand, finally finding something that is fixable would be nice, especially if it fixed everything.  However, that would mean a surgery that I am not sure I am ready for.  So, I will just wait and see what is found or not found.  The waiting is always so hard....that and not being able to feed a 2 year old any food until after the procedure.  :)
Thanks for all the thoughts!!!  I will update on the blog as soon as I can. 
Good night!!!

Wednesday, October 27, 2010

ENT Visit

So the ENT we see here in Arkansas has proven to be much more aggressive than the one we had in Denver.  I am not sure if it is the fact Claire is older now, a different approach, or because he is a swallowing expert (somewhat self-proclaimed because there really is not a true speciality), but he has really taken to Claire's situation.  He has many ideas and plans on how to "fix" Claire's swallowing situation. 

I do agree he knows alot about swallowing disorders, etc.  He writes articles for journals, does research and reaches out to other doctors across the nation to discuss swallowing and the kids they see with these disorders.  Conditions like Claire's are rare so it is often necessary to talk with doctors at other institutions for similar cases, etc.

Claire's tonsillectomy and adenoid removal were considered a success.  It also appeared the dilation of the muscle at the top of the swallowing pathyway made a huge difference in her eating ability.  After recovery she seemed to eat more, eat more easily and be happier all around.  However, within weeks of her recovery, the congestion that dogged us all last fall/winter/spring returned.  This is congestion that just sits in her throat.  It makes her breathing loud and sound "sick" at all times.  However, it is not in her lungs and does not result in pneumonia.  It appears to be all in her throat.  Then about every 4-6 weeks or so it "backs up" into her sinuses causing a runny nose,etc.  This then progresses to a cough, sinus infection or without tubes an ear infection.  So yes, Claire has had her first ear infection of the season.  I so wish they had put the tubes back in (they failed in May) when they had her under anethesia in Sept.  Oh well, there are worse things than ear infections....

The ENT doctor feels that Claire has this congestion in her throat because the muscle above the swallowing pathway (esophagus) is contracting again.  This means it did not stay dilated after the surgery (T&A) as he had hoped.  He feels because of this her food, drink and secretions are pooling in her throat and have no where to go.  This is a very reasonable theory and actually he feels very likely.  So, in two weeks Claire will go back to the hospital for yet another round of anethesia where they will put a scope down her throat and see what the muscle is doing.  If it is contracted, he will dilate it again and then the hard decisions will come.  Claire will then undergo a swallow study approximately four weeks after this scope to see where we are there.  If all goes as planned I will be out of the hospital from my C-section and able to participate.  Should be interesting with a days old baby, but hopefully a mom, dad and nana can handle it all.  We shall see...  I will provide more information after the scope and as we know more.  The swallow study will not be until December.

Surgery will be necessary if they find this muscle is contracting and the "cause" of the problems.  This is a very delicate procedure and quite frankly the more I read the more nervous it makes me.  We will have alot to discuss should this be found to be the problem.  This surgery involves cutting into Claire's neck and cutting the muscle to keep it from contracting.  While stroke victims and others often have this procedure, it is relatively rare in children.  So, we will have decisions to make should that be the road that is followed.  However, the first step is to determine if this is even the reason for it all.  :)

As mentioned earlier then ENT doctor also discussed her allergy situation and started an antihistamine and nasal steroid.  Poor Claire, so many medicines!!!  I just want her to not have to be so sick with congestion all the time.  As she gets older it definitely bothers her more.  But she is a hard one to keep down!! 

Less than six weeks until we meet the new addition to our household!!  However, the focus here remains on Halloween and luckily the costume plans have not changed in a few days so maybe we are set.  The Candy Fairy is even prepared and ready to go. 
Thanks for keeping up with us!!

Milk and Soy Free Diet Update

About a month ago, the GI doctor asked us to try a milk and soy free diet for four weeks and see how Claire did.  Both he and the ENT doctor are convinced Claire has a food allergy.  Her blood test has come up negative, but the GI doctor was not at all surprised by that and says that means "nothing" in regards to whether she is indeed allergic.

So after removing milk and soy for a month, there has been little to no change.  Her skin still has the "rashes" and her congestion is still there.  So, neither Jonathan nor I feel milk/soy free is the answer.  We will see what the doctor has to say when his nurse returns with the doctor's thoughts (not thinking in the near future with how that office works and how busy it is).

I would probably agree that Claire has an allergy to a food; however, the process of finding it is cumbersome and there are most likely other reasons for Claire's congestion.  So until we get those "fixed" not sure about the allergy situation.  The doctor was also concerned that we were getting into allergy season, so I am not sure if we are going to do any more searching for a while. 

Claire is now on a nasal steroid and an antihistamine per the ENT so that should help with the situation as well.  Her sister Maggie has moderate eczema and struggles with rashes at her joint areas nearly year round.  So, it is possible we are just an allergy family; I know I certainly have alot, unfortunately.

I will keep you posted!  For now, Claire is delighted to have cheese back in her life.  :)

Who We Are and Why a Blog

Hi!  I am starting this blog to see if it will help family and friends keep updated on Claire.  We have had so many questions and requests for updates that we thought a blog might help.  Then you can choose when and how much you want to know or be updated.  I hope you like the idea.  With the new doctors here in Arkansas seemingly more aggressive and with Claire growing older, it seems there are more changes, more often.  I hope you find it helpful!!!  We are so thankful for all of the love and support you have shown to us through this journey!

As most of you know, Claire has a number of health problems which have resulted in numerous "diagnosis codes" being used to describe her condition(s).  They even ran out of lines on the military paperwork when it came to diagnoses.  However, the simpliest way to describe Claire's diagnosis today is Severe Dysphasia of Unknown Origin with Developmental Delay.  This does not begin to cover all of the little details the doctors continue to find every time we go, but overall this covers her. 
She currently has a "Mic-Key Button" feeding tube that was placed at 5 months old.  She was fed only through tube feeds from 5-9 months when pureed foods were introduced.  Also at that time, honey-thickened liquids were introduced.  Presently, she still remains on honey-thickened liquids, which she drinks through a straw (she has very little ability to drink any other way); however, her "food" diet is not restricted.  She struggles at times with chewing and with weak muscle structure; however, she undergoes therapy to work with that.  She has progressed to nearly eating everything a normal two year old would eat, as long as it is not liquid (ie soups, popsicles, etc or they must be thickened).

Claire undergoes 5 sessions of therapy weekly to work on various issues, mostly as a result of her developmental delay.  She has two sessions of physical therapy a week to work on strengthening her muscles nearly all over her body.  Her abdominal region is particularly weak, and it is likely that one hip/leg is stronger than the other.  The physical therapist is confident she will catch up quickly.
She has occupational therapy once a week to work on her sensory integration disorder.  This is a somewhat common disorder that encompasses alot of different characteristics.  Our pediatrician attributes Claire's issues to the fact she has been in the medical system since 3 weeks of age with all kinds of people prodding and all kinds of tests.  She is making great strides and hopefully we will see her become less skiddish over time.
If Claire has a biggest need for assistance it is in speech and feeding.  At evaluation, one of her speech percentiles was at less than 1% of children her age (which means 99% of children her age were farther along than she was).  She goes twice a week and works on both speech and feeding during her sessions.  The feeding is coming along nicely as is the speech.  She has added many words just in the last month, and we are so excited to see that she seems to understand that she needs to speak our language for us to understand.  She had very little issue with receptive language and that continues to be a strong point for Claire.

Overall, Claire is a happy and loving child.  We are very blessed to have her and realize her health issues are not as severe or limiting as some.  For that we are thankful!!!  
I will try to update at least once or twice a month depending on the going-ons of the month.  I will certainly update whenever we find out something new or things change.
Thanks for all the support and love!  We are blessed!!!!