Jun 9, 2009

"BIG" Appointments

No surprise. The otolaryngologist who in March wanted to wait 3 more months to see if there was any more improvement before going forward with surgery completely changed his tune. I don't know if he forgot that he said "the ball was in his court" to figure out a plan or what happened. Truly I'm stunned by what was revealed today.

Essentially the reason that we are not hearing much sound from Ainsley while she's wearing her PMV is that her vocal cords are not moving well. She has a lot of scarring. That we knew but had been told they could remove scarring with surgery. Well now he is saying that she has severe scarring (i.e. damage/fixation - medical term ankylosis) to the arytenoids and that is why we aren't hearing much sound and this condition is irreversible. He said he is surprised that her voice is as effected as it is by this scarring and on the other hand surprised that she can tolerate the PMV and cap as well as she does. It must just be borderline that there is just enough room for her to breath but not enough vocal cord movement to talk (with the PMV).

So essentially it seems as though there is no way to give her a voice. The surgical options of a cricoid split or partial cordectomy could leave her airway open and vulnerable to infection especially since she micro-aspirates. We could try that route and potentially get the trach out but it could be at the expense of her lungs or maybe her voice.

So we left the appointment feeling very deflated. Especially because this damage is due to the intubation immediately after her birth and we can't help but feel perhaps it could have been prevented. It sounded as if she may never speak and always need the trach.

Ainsley's speech therapist came with us to this appointment to try to get answers about Ainsley's voice. She brought with her a voice output device that we'll be renting. We tried it out while we waited for her second appointment. She loved it and thought it was a ton of fun. I'd brought her PECS book and she was able to identify every picture card when asked to point to it. Cognitively she's starting to understand a lot more. And just as if to prove him wrong she started vocalizing with some modulation of tone. It's not speech but perhaps her vocal cords are not as fixed as he thinks. Here is some video I took:

And look how happy she is here to have a voice.

So I guess our plan is to work on sign language and use an alternative communication device and most likely keep the trach indefinitely. Unless she decides to prove him wrong. We'll see him again in the fall. We'll keep using the PMV since she seems to be able to tolerate it and it keeps her airway used to being exposed to air. There is no need to push the cap anymore since it's unlikely she'll be able to be decannulated.

The eye appointment was confusing. The ophthalmologist was against the idea of a tarsectomy and tarsal switch surgery to correct Ainsely's ptosis until the end when I showed him a picture of her eyes before and after her last cranial reconstruction surgery. Then he said yes a frontalis sling made sense for her before but not now (pointing to the photo). But she's right there and I pointed to Ainsley. Why the pictures changed his mind at the end I don't know but after 20 minutes of discussion he said that is the way to go 30 seconds after seeing the picture. He did assure us that though the tarsal switch would be irreversible it wouldn't cause damage to her vision although she may sleep with her eyes open. He thinks it would be best to wait until she is at least 4 years of age when that area is mostly full grown. But at least he agreed that is the way to go. He admitted he is conservative. He doesn't even believe in lasik eye surgery so that says a lot. So now we can make the follow-up appointment with the other surgeon and see if he wants to do it this year or push it out.

In late July we should find out if Ainsley needs the hip reconstruction surgery and if so when.

Since there is the potential for her to have 2 surgeries this year it is probably good to leave her airway alone anyway so we don't risk damage from intubation. Then perhaps in the fall we may reevaluate if we are desperate enough for a chance to remove the trach that we'll try questionable airway surgeries. We know we can do the trach thing forever if we need to, but we are disappointed. At least we know Ainsley will be okay no matter what. She is a happy girl and will remain so even if she can never speak and requires a trach to breathe.


  1. Oh Susan. I can feel from your writing how disappointing this day was. I wish there was another answer. I can't help though, going back to the picture of Ainsley with the communication device. She is so delighted. That makes all of this just a little bit ok! Hang in there. Suzanne

  2. Susan,

    I am sorry to hear this news, and I am sure no matter what I write will ease your disappointment.

    The future though is unsure, and our little ones have a way of proving many wrong in the medical field.

    Ainsley is happy and well loved by her family and very lucky to have such a strong role model as yourself Susan.


  3. Susan, that is an incredible amount of information to take in, in one day. I'm not sure how you had the energy to blog it for us. You know, it's the old adage ... one day at a time.

    And, can I just add ... it really makes me mad when doctors flip flop on their answers. Fact is, you probably know more about what is the best course for Ainsley than they do. You have a great attitude, but I too can sense your disappointment. You are an amazing advocate for Ainsley. Hang tough, but cut yourself some slack every once in a while. You are one of the BEST moms I know.


  4. I just started following your blog and I'm so sorry that you are going through this. How incredibly disappointing! I just don't know what to think about these Doctors/ENT's. We have been through roller coaster of emotions too with the trach and our journey also began with an intubation. Your girl is so cute and happy and seems like she's making great strides!

  5. I can see from your blog how disappointed you are. I cannot imagine fighting as hard as you have for Ainsley just to hear she may keep the trach. Don't give up. She is a beautiful, beautiful girl and brings me such joy every time I read about another accomplishment she's made. I love the video of her voice. She's going to surprise ALL of us!

  6. Susan I don't even know what to say, I am sad for you. I can tell how disappointed you are with both Drs. I am sorry that the ENT gave you false hope only 3 months ago to completely dash it all away. My hope is that Ainsley defies the odds and proved them wrong. Look how far she has come recently. She is a little miracle worker, it may just take her some time to make this one happen.


  7. She is beautiful. I have not made it over to look at your blog/story before, but you guys are obviously awesome parents. It is always a difficult time when the state of play changes.
    All the best.
    from cherienz (tracheostomy.com)

  8. I've only commented once before, but I read your blog often.

    My daughter is just about 2.5 years old, has a trach, g-tube, fundo, and she is also a patient of Dr. Inglis. From my understanding, he is pretty conservative too. My daughter is trached because of upper airway obstruction due to severe dysphagia. Standard treatments for swallowing disorders are not recommended, or done at all in Washington, and Dr. Inglis told us he thought they were a waste of our time. The same thing applies to us, time will tell, and if we don't see improvement in the next 2-3 years then we will go out of state.

    Ainely is such a beauty! I love her laugh and smile! The last few months have been difficult here on my end, so I haven't commented but I did want to let you know that we go through a lot of the same kind of stuff with tolerating/not tolerating PMV's and caps and food. Is it 1 step forward and 2 steps back, or 2 steps forward and 1 step back? I don't know, but we spin wheels a lot.

    I do think that it is very hopeful that she does have range in her tone, and some vocalization. Keep that in mind and run with it! I love her videos by the way!

    Take care!
    Jennifer (from Gig Harbor).

  9. Oopps! I accidently left out a letter in Ainsley's name.

  10. I know how hard this must be for you. Just hang in there. Take it one day at a time. You have many that are praying for ya'll. Ainsley is blessed beyond measure to call you Mom.