Thursday, November 3, 2011

Ears and Feet


A bit over a month ago, Maddy got new AFOs. She needs AFOs as her ankle joint is very unstable. When she stands, she stands on her ankle - and in the long term, she could do damage to her feet.

The AFOs that she has are not designed for walking. They are designed for infants, to immobilise a joint. The AFOs that are designed for walking at lighter, thinner and can fit inside a shoe.

A couple of weeks ago, we met with the orthopedic surgeon to discuss how the AFOs were working for Maddy. My main concerns were that they are quite heavy and they tend to slip off very easily. The problem is, though, that Maddy's legs are so short and fat that there is no "ideal" AFO for her. We are, however, going to try what is called a "Dennis Brown Walking Shoe". These are little leather boots which go over the ankle and are very supportive for little feet. We will go and have some fitted next week, and hopefully they will work better for Maddy :)


One of my current main concerns with Maddy is her hearing. I know that with cleft palate, and also with DD in general, hearing loss is not uncommon. Maddy has DD with a cleft and as such is more at risk for hearing loss. An article about DD that I read suggests getting hearing tests every six months or so during the first few years (from memory). I have been asking the doctors at the public hospital to test Maddy's hearing, and about 4-5 months ago, Maddy was apparently placed on the waiting list for a hearing test. After chasing them down, I learned that the machine was broken, but should be fixed soon. As I had a pediatric appointment coming up, I decided to wait and talk about it with them then.

Now, I know that Maddy can hear at least a bit - she does respond to noises (although sometimes she seems as though she may not hear, but I can't tell if she's just ignoring). She also responds to certain things that we say. BUT - she is now 15 months old and cannot say a single meaningful word, with the debatable exception of "mamamama" which she says when she is cranky. She also doesn't say a wide range of consonants, and hearing is so important to speech development at this age.

At Maddy's pediatric appointment, the doctor told me that it is not abnormal for the wait to be so long, and I shouldn't expect an appointment in the near future. The only way I could get a sooner appointment was to go to the private sector.

So, armed with the referral, I called a private ENT on Tuesday afternoon. He asked if I could come in the following day!! After all those months of waiting, I could get an appointment within 24 hours of calling a private doctor!!

Now, Maddy is at risk for hearing loss due to two different things - a) fluid in the middle ear due to cleft palate, and b) problems in the inner ear due to DD. The ENT first checked for fluid in the middle ear, and sure enough she does have fluid which could be causing some hearing loss. He would recommend that she has tubes put in her ear ASAP. He cannot accurately assess the inner ear until the tubes are placed and the fluid is drained. After this is done, she will need to have a special hearing test called an "ABR" which is performed under sedation and could indicate whether or not there is a need for hearing aids.

The complication, however, is that we cannot just rush Maddy into surgery. First of all, the private hospitals will not take her as it is necessary that there is an ICU just in case there are complications with the surgery. Secondly, they need a senior anesthetist for Maddy as she is difficult and even dangerous to intubate. So the ear tubes, logistically, cannot be placed until during Maddy's cleft-palate surgery. Last I heard, the doctors were planning to do this between 18-24 months of age, so still 3-9 months away.

The private ENT will be sending the public hospital his recommendation to do the surgery "as soon as it is safe to anesthetise her". Our next cleft appointment is a month away, and I will be urging them to consider surgery in the new year (I would ask for earlier, but we've already booked tickets back to Australia for Christmas!!). She will not grow significantly in a few months, so hopefully they will decide that it is "safe"...

We won't have any firm answers on Maddy's hearing until after the surgery is done!! "Wait and see" is the story of our life with Maddy these days ;)


  1. That is a terrible wait for hearing tests! I'm glad you will be able to get an appt soon. I had all the waiting on medical gets so frustrating!

  2. Could you have her "receptive language" (that is, her ability to understand words or sentences that she is not yet able to produce) assessed by a trained developmental linguist? A simple example: Can Maddy pick out a dog from an unfamiliar picture of a group of animals when you say "show me the dog"? If so, then she hears and understands the word "dog" whether she can say it or not. Tests like this can distinguish articulation problems (i.e., physical difficulties with speech) caused by her cleft palate from genuine language delay (i.e., delayed understanding of words and sentences) caused by impaired hearing.

    An optimistic note: Although hearing is essential for learning language (except sign languages native to the Deaf), very young children catch up on language learning remarkably quickly once their hearing issues are resolved.

  3. I forgot to add - she will be having a "developmental check up" in relation to speech/hearing and should start some speech therapy soon. So far, she hasn't had speech therapy but I think (hope) she is old enough now to benefit from it... I know that if it is caught early enough, hearing loss doesn't hinder development - but I get impatient knowing that there is a problem that at the moment, we can't really do anything about!!

  4. Lyla just got ear tubes back in August. She had fluid in her ears for months and the ENT told us she couldn't hear! The ear tubes made a huge difference- no more fluid....however she only says dada and nana. She is 13 months. Her doctors say not to worry about that yet. I guess it is a waiting game! Good luck with everything.

  5. I am 21 weeks pregnant and found out that our daughter (Piper) may have Diastrophic Dysplasia. She has short limbs, a cleft palate, and clubbed feet. This blog has been so interesting to read and is giving me hope for her future. THANK YOU for the inspiration!

  6. Dear "Anonymous", I'm so glad to hear that. Please if you read this, email me and join our DD facebook group!! It has been a huge support to me over the past 2 years. All the best with the rest of your pregnancy :) xox

  7. My name is Tara - I wrote in yesterday as Anonymous about my little peanut Piper. As I was reading your blog I was very touched when you wrote "I am holding onto hope. Hope that my baby girl will be happy. Hope that she will be able to live a long and fulfilling life. Hope that I won't have to lose her before I even have a chance to meet her. But most of all, hope that her life will make a difference somehow..." You were at around the same stage of pregnancy as I am now when you wrote this. You described exactly how I feel at this moment. Maddie's life is making such a difference to me now. I have hope now. Her middle name is now going to be "Piper Hope". I have three sweet daughters – a 3 year old and twin one year olds. Seeing your older daughter with Maddie made me feel so great about my daughters being a part of Piper’s life. THANKS AGAIN!!!

  8. Thanks for the best wishes for the New Year! I have posted before about my pregnancy. I am now 28 weeks pregnant.On Monday we saw our ultrasound Dr. and he told us that because Piper’s chest ratio is .72 that he felt that her dysplasia is a lethal form. We were crushed but after we left the office I remembered a post from your pregnancy about Maddy’s ratio being about the same and how that number is possible for life outside of the womb. So we sought a 2nd opinion 2 hours away and yesterday we saw an awesome Dr. who gave us a lot of hope. She is an expert on skeletal dysplasia and is writing a chapter to a book on it now. She thought that Piper’s case did not look lethal based on her chest size. THANKS FOR THE INFORMATION that is on your blog. I may not have even gotten a 2nd opinion if I did not remember the .7 ratio that you posted!!! Tara

  9. Hi Tara - I am SO glad to hear that. Maddy's chest ratio was always between 0.7 and 0.8. Many lethal forms have ratios of closer to 0.5. I can speak from experience when I say that a ratio of 0.7 is definitely not necessarily lethal!!

    I know many people with babies with skeletal dysplasia who went through pregnancy being told that their child had a lethal form and it was wrong. Most doctors have never experienced a case before, and even those that do can be wrong as well. I am so glad that you got a 2nd opinion!! I think of you and Piper often xox