Monday, August 27, 2012

More than words, part 2

I said I would write more about our journey to give Ben a conventional voice. This is primarily a recounting of an enormous number of things that we "did" over many years, not really an examination of how it "felt" as a parent to be doing these things, which is probably more  enlightening. That will be for another day! Louise

I was visiting my family doctor. Ben was in his stroller, babbling exuberantly and charming the others in the waiting room.

The doctor came to get me and listened in. "I cannot WAIT to hear what he says when he speaks!" she said.

That was the hope that pushed me for the next eight years.

From the time Ben was two I pursued every known therapy and medical intervention that might bring us one step closer to speech. These are documented in stacks of binders of speech, hearing, swallowing and medical assessments and program plans.

I had big dreams and was capable of mounting large campaigns on my son's behalf.

At two years, two months, I had Ben privately assessed by a PROMPT therapist in Toronto (publicly-covered therapy didn't begin until age three in Ontario way back then). PROMPT was the "big" speech therapy here then, where therapists support a child's oral-motor movements by cuing the mouth and lips with their hands. The therapist said he had mild to moderate low tone of his oral/facial muscles that made it difficult for him to control phonation, jaw, lip and tongue movements. "Ben was observed to use up to three word utterances for a range of communicative functions," she wrote.

Over the next year we had many different therapists work with Ben. One PROMPT therapist saw him weekly. "I really like the new therapist," I wrote to a friend at the time. "I love the way she interacts with Ben. She always has some specific activity ready to work with him. They generally sit at a little table together. Ben usually loves whatever she has him doing, and really listens to her and does most of what she asks. He said two things at the last session -- 'oben' for open and 'go away.' He still has just about five to 10 words that we hear often. There are many sounds he is physically unable to make. The therapist says the problem is oral-motor low tone. She said everything is in place to help Ben now, and to give it a year and hopefully we will see some real progress." This therapist also suggested he had auditory processing problems, because he didn't respond to sound the way he should.

In fact, Ben had a permanent, moderate hearing loss in both ears -- which was magnified by constant middle-ear fluid due to structural problems with his eustachian tubes. But that hadn't been identified yet.

At 18 months Ben had his first set of ear drainage tubes put in to combat severe chronic ear infections and middle-ear fluid. While still under anesthetic he had an evoked potential hearing test and we were told he had moderate loss in both ears and needed hearing aids. I just looked up the hospital note (which I only purchased years later) and it says results showed a bilateral, moderately severe sensory neural hearing loss. However, the surgeon said he'd never drained such copious amounts of black fluid from a child's ears before and there was a question as to whether the hearing test was a true reading.

I thought about leaving that part that way.

But to be honest, the question about the accuracy of the test was raised by me. I called the audiologist and asked whether the reading could be affected by the huge amounts of liquid that had just been removed from his ears. Part of me couldn't believe that Ben's hearing was that bad because we had taken him religiously for hearing tests since he was a newborn. And part of me didn't want to put hearing aids -- and their associated stigma -- on my 18-month old. The audiologist said it was possible that the middle-ear problem had compromised the results. She suggested we hold off to see if we might get a closer to normal hearing test in the studio.

On sound-field tests -- where they pipe sounds into different sides of a room and if the child turns to the sound a box with animals dancing in it lights up -- he was testing in the borderline normal range. And following the evoked potential test, he continued to test in that range on what I think is a highly subjective measure. We now know those tests were wrong. Ben was visual. I believe his eyes were peeled for those dancing bears and his visual perception was mistaken for hearing. Despite frequent trips to audiologists, Ben wouldn't wear hearing aids regularly until age five. In an audiology report from our local audiologist at age five it says: "Ben has persistently demonstrated borderline [normal] hearing levels.”

It wouldn't be till he was nine that he had a videoscope (on a private consult we had in the U.S.) which showed he had abnormally small, compressed eustachian tubes which caused the fluid build up and malfunctioning of eight sets of drainage tubes. They blocked almost as soon as they were put in. We were forever squeezing ear drops (that never dislodged anything) in Ben's ears. But the drops caused him great pain. In anticipation of the dreaded drops, he quickly learned to cover his ears when I put him down at night.

For all those years I'd been convinced that Ben's ear infections were caused or exacerbated by my bottle-feeding him. I'd failed the cultural edict of "Breast is best." When I raised this question at the appointment at age nine, still consumed with guilt, the specialist laughed (but in a good way!). "His ears are structurally abnormal," he said, pointing to the image on the screen. "This has nothing to do with breastfeeding."

Sometimes I wonder what might have happened if I hadn't questioned the veracity of the evoked potential hearing test and been so persistent about it. And we had simply proceeded with hearing aids. That said, it was impossible to keep aids in his ears when they were infected.

I think we got his first pair at age four. The audiologist still wasn't convinced that he needed them. It was quickly apparent that the behind the ear aid wouldn't work because Ben's ears were so outstanding that the aids flopped forward and waved about. We got in-the-ear-aids, but because of his painful ear infections, he would not keep them in his ears. At one hearing test, when D'Arcy tried to put them in, Ben fought and cried until D'Arcy ended up in tears. The audiologist said she thought he was getting good sound input without them and we didn't need to persist. At the time Ben was in a junior kindergarten program for kids with physical disabilities but he didn't wear them there either: we were told that a staff person couldn't follow him around constantly to make sure he didn't pull them out and lose them. And at the back of my mind there was always the fear that if in fact his loss wasn't significant, the aids could cause damage.

In addition to speech therapy, we decorated the house with picture symbols and had a picture communication book. Ben was good at getting the book to show us what he wanted. When a therapist wasn't willing to prescribe a sophisticated voice device for Ben (because she wanted us to start with one with only four messages on it) I got a distributor to loan us one for free. I then videotaped Ben using it and went back and complained. We got the Dynamite funded under the assistive devices program. But though Ben became quite good at using it, it wasn't functional for two reasons: It was heavy and he couldn't carry it by himself and it wasn't organized in an intuitive, user-friendly way. It was based on pages and pages of embedded vocabulary, much like a dictionary. Can you imagine having to locate a word in a dictionary in order to use it? And then look up the next word? It took so long he lost incentive.

Because I was on American parent e-mail lists for kids with disabilities I began to hear about types of speech therapy we couldn't access in Toronto.

In addition to his low tone, Ben had picked up the diagnosis of apraxia -- a disorder where the child knows what to say but can't sequence the motor plans necessary to produce words.

And before he turned two he was diagnosed with an uncoordinated swallow, which had been the cause of many choking episodes when he started to eat real food. When Ben swallowed, not all the food went down. Some pooled at the back of his throat. So he needed to do extra swallows or he would choke.

"I thought I had accepted that he had a swallowing problem and that it was related to his low tone," I wrote at the time. "Yet when the OT came out after the feeding study and told me he did in fact have a problem, but that it was an uncoordinated swallow -- probably to do with his genetic condition rather than low muscle tone -- I was shocked and full of grief. My husband couldn't understand why I was so upset. It was then that I realized that somewhere in my heart that little flame of hope had been burning, the one that can lead me to believe things may be all right, even in the face of insurmountable evidence."

When Ben was 4 1/2, we took him to see Nancy Kaufman in Michigan. Nancy was the North American guru for treating apraxia by getting kids to do word approximations: boo boo (hurt finger); neigh neigh (horse); wuh wuh (dog); wa wa (water).

A few weeks before we left, I went back to church. I didn't know how to help Ben speak and my best rational attempts weren't working. I was desperate. During a snowstorm one Sunday only a handful of people turned up. Instead of a regular church service, the minister had us sit on the stage together for an informal gathering. We had an opportunity to ask for prayer and I asked that they pray for our visit to Michigan, and that it would help my son Ben, who was unable to speak. An older man turned to me and said: "Maybe God doesn't want your son to speak. Have you ever considered that?"

"No" I railed. "I don't believe in a god who won't allow my son to speak."

Nancy felt Ben’s speech problems were largely physical -- due to low tone, oral-structural deviations (small lower jaw and mouth; tight, then lips; receeding chin; velopharyngeal incompetence) and apraxia. She was thrilled when he scored well on the Peabody Picture Vocabulary, which tests single-word comprehension. She said he wasn't ready to begin her apraxia program because he first needed to improve his oral muscle strength and coordination. She also felt we needed a second opinion about whether surgery could fix some of his structural problems.

I was ecstatic that Nancy seemed to be drawing a more precise path to the treatment of Ben’s speech problems.

I was the one driving all of our therapy efforts and I had to convince D’Arcy of the merits of these expensive consults. I can see now that I had blinkers on. In order to research, arrange and afford these trips, persuade D’Arcy and make the long drives manageable for our two little kids, I had to be single-minded.

Five months later we drove to Westchester, New York to see Sara Johnson, the renowned expert in oral-motor therapy. Ben was four years and nine months. Sara diagnosed him with a severe oral-motor/feeding/speech disorder secondary to weak muscle strength in the muscles of the abdomen, velum, jaw, lips and tongue; motor planning deficits -- dyspraxia; hearing loss; and structural deficits.

We had to focus on strengthening his muscles through oral and feeding exercises to give him a base from which he could speak she said. Her recommendations were oral-motor/feeding/speech therapy two to three times a week. And 15-20 minutes of oral exercises daily.

We couldn't find a therapist locally who followed this program, so Ben's worker and I did our best with the daily exercises.

They included pre-feeding massage of his face; rubbing swatches of fuzzy, soft, smooth and rough fabrics on Ben's cheeks; rolling a vibrating toothette in Ben's lips in a variety of ways; and having him kiss a small ice cube.

Feeding exercises included using a particular curly straw and getting Ben to chew cubes of food at the side of his mouth.

Oral-motor exercises included blowing exercises with candles, bubbles and whistles. It was suggested I "construct a birthday cake out of styrofoam, tin foil and other decorations, place a candle in the cake, and provide jaw/lip support while Ben blows out the candle five times."

I'm not a crafty person, and I often felt defeated before I had even begun the set up for these activities.

Other oral-motor exercises included making ice straws and having Ben bite in a certain pattern on each side (this could also be done with veggie stix, which weren't yet available in Canada, so I purchased them in bulk in the US). Finally, we had a number of exercises related to Ben chewing gum on both sides of his mouth.

For the next year or so we would travel to Albany, New York (half the distance to New York City) to see one of Sara's associates every couple of months.

When Ben was five we got a second opinion on surgery to correct his oral differences at the New York University School of Medicine. Again, because I was on parent e-mail lists, I heard about monthly team meetings that were held at NYU bringing together multiple specialists to assess children with complex cases.

One mother wrote about a plastic surgeon there who had the highest rate of success in performing velopharyngeal flap surgery, which is used in kids whose palate doesn’t close properly during speech, causing air to escape through the nose and making consonants impossible.

It was thought that Ben had this problem, particularly since as a young child when he drank, liquids came out his nose.

This mom had contacted all of the surgeons across the U.S. to determine their surgical effectiveness – because a rare but scary complication of the procedure is that it can cause sleep apnea.

We drove the 10 hours to Manhattan and stayed for a nominal amount in a medical resident building right across from the hospital.

Ben was ‘presented’ in a large meeting room to a bunch of doctors and therapists and promptly crawled under a table.

He was seen by a plastic surgeon who said he definitely needed the pharyngeal flap surgery. But the catch-22 was that he couldn’t do the surgery until Ben had more speech (which would determine the type of closure needed).

We also saw an audiologist who insisted that Ben must wear hearing aids. He told us that even a mild hearing loss can turn into a major disability in a child with complex problems. Armed with this knowledge we returned home and succeeded in getting Ben to wear the aids (I don't remember what bribes and enticements were used).

We continued on with PROMPT therapy at home – at one point having four 30 minute sessions a week that cost about $700 a month. In addition, we were also able to get some blocks of publicly covered therapy.

Ben’s PROMPT therapist wrote: “Ben is a boy who demonstrates significant delays in body awareness, oral motor execution and sequencing skills, language skills and possibly auditory processing...Ben’s ability to plan some oral motor sequences independently is increasing, such as “no,” “yeah” “me” “on” “one, do/two, dee/three.”

When Ben was six we took him to see a developmental pediatrician at Holland Bloorview. I was frantic about his lack of progress over time. He always had a handful of word approximations but they never blossomed into anything more and sometimes he lost words. I'd never worked so hard at something in my life and had so little success.

"Things would have been different if Ben hadn't had severe constant ear infections for so long during such a critical period," the developmental pediatrician said. But he did, and the doctor felt his problems with speech were related to a central language output problem.

"It is my firm conviction that he has much more 'language' in his head than he is able to express with oral language," he wrote in his note. "I strongly encouraged Benjamin's parents to consider reinstitution of sign language."

We had introduced Ben and Lucy to sign earlier on, but Ben had only picked up a few signs and he couldn't manipulate his fingers into some signs. The doctor felt Ben would never make progress with spoken language but that sign held much greater promise.

He wrote to our schools for the deaf to ask about Ben receiving services or attending their school, but was told that his moderate hearing loss didn't meet the threshold for service. I feel that was a critical juncture where the system let us down.

A few weeks later we registered for a sign-language immersion camp for adults run at the Bob Rumball Centre for the Deaf. We rented a nearby cottage where a worker stayed with Ben and Lucy during the day, while D'Arcy and I and a SECOND worker went to sign-language immersion for adults. This was a costly endeavour but we felt it was valuable.


Your story is so familiar. I completely relate to your absolute determination that Ben develop his speech. I travelled the same road you did (just many years later), and visited some of the same specialists you took Ben to see. But to no avail.
Thank you for sharing this.
I look forward to reading Part 3 of your story.
(BTW, I love your title ;-))

Thank you for another great post and the beautiful photo. "Maybe God doesn't want your son to speak. Have you ever considered that?" Wow - that is something to ponder that we never consider. What gifts have come along the way as a result of this that we otherwise wouldn't have experienced? I also look forward to part 3!

Thanks Stacey! Ben has a submucous cleft palate (and Carter had a repaired cleft palate, right?)

I didn't realize you saw some of the same people. I guess there's a group of us moms out there who have been making the same rounds on our quests to find answers for our kids. Yes, I love the headline and I also love your blog and hope people check it out.

We actually made two more trips to the U.S. which will be covered in part 3. It seems almost silly to write about them. When did you stop speech therapy with your son?

I am really enjoying this series, Louise and look forward to the next part. What a journey -- and how you relate its complexities.

And that photo? Oh my goodness, he's adorable.

Louise, thanks for sharing your story! I saw the picture of Ben and realized it seemed familiar (my grandson Renzo has the same ears)... my grandson, whom I have helped raise for the past four years looks very similar. Then I read the post and found many more similarities ... regarding lack of speech in Renzo and your Ben and then again with the hearing issues. Renzo has a mild to moderate bilateral hearing loss. And, at five years old, we are on our 7th set of ear tubes! And, these ones don't seem to be working much better. While Renzo doesn't have a black fluid coming out, his ears when infected bleed. Sometimes it's a waxy type of bleeding, but lately, it's been just blood droplets. Anywho ... I was so intrigued with the similarites, that I read further into the blog and you, thinking that maybe Ben's diagnosis/syndrome could be an answer for Renzo (he had so many similarities ... the eyes, ears, curved fingers, etc). Renzo's 6th and 9th chromosomes are affected, and he is an anomoly, no other documented case reported ... we have no diagnosis, and sometimes that's one of the hardest things to live with. Renzo will be 5 in six days, he doesn't have any formal language, doesn't eat table food, and drinks only at night time by bottle. In fact, Renzo won't open his mouth to any food or drink unless it's his stage 2 baby food, or he's sleepy and wanting a few sips of a bottle (he doesn't sleep with it in his mouth, just a few sips and then he tosses it to the side). Anywho, while I don't wish this on anyone, I felt comfort in your post and it's similarities, so thank you. I do have a few questions though ... how did you know where to start? Who do you contact first? Renzo receives speech therapy at school and at home (though the home therapy is way above what I believe he is capable of), and we used to get feeding therapy, but those are hard to come by and we lost ours due to scheduling conflicts. Hearing aids, well with so many infections, he isn't able to wear them too often, and when he doesn't have any infection, they don't sit too great on his ears and/or he'll pull them off (more similarites). Guess I'm still looking for ways to help Renzo, I became a little complacent I guess in the past few years, and figured I'm just going to love him the way he is. But oh how I dream (and I do) to just hear him say "Nana" one time ...Any advice you could offer would be appreciated ...

Hi Bunny -- Thanks for your message. Ben's syndrome is on the long arm of chromosome 8.

We identified his abnormally shaped eustachian tubes when he had a videoscope (they put this down the nose) at age 9. This was never offered to us where we live here in Canada. He received it when we paid for a private consult in the US.

I would definitely ask for in the ear aids vs the ones that go behind the ear if Renzo's ears stick out (and they keep falling forward), Ben finally grew out of his ear infections at about age 5 or 6.

Does Renzo learn sign language? This is Ben's preferred mode of communication even though he has weak, uncoordinated hands and he can't do all of the signs. There are some amazing online sign language resources:

Communication is the most important thing and it doesn't matter if it's by speech or sign or pictures or a voice device or just in a child's own natural way of gesturing or using their eyes.

I look forward to hearing more about Renzo. If you send me your snail-mail address I can send you a copy of our BLOOM magazine

Awesome post. The lengths to which you went and will go to help Ben are amazing. It's a very long road and your determination is inspirational. When my son Deane was 3 or 4, I realized that his inability to walk and every other disability he had was secondary to him being able to communicate. We continue to struggle to find the way to unlock his thoughts.