We had a follow-up with the Genetics Lab at McMaster when Steven was a few weeks old. It was a different Genetics Counselor this time. He started off the conversation with “What were you people thinking?”. We were taken aback and defended ourselves as best we could. In the end, he apologized for the incorrect information we had been given by the previous Genetics Counselor. In some cultures, we might have chosen to take legal action, but we decided that it would not change Steven, and moved forward with as positive an attitude as we could muster.
Feeding an infant with a cleft palate is a tricky exercise, Steven could not suck liquid from a nipple. It took some experimentation, but eventually, Steven began to gain weight using a squeeze bottle with a very soft nipple. However, he was very vulnerable to pulmonary aspiration- inhaling liquid into his lungs. Within weeks Steven suffered from lung infections due to aspiration, and resulting fevers. Due to his unique cleft and sinus configuration, formula and saliva also made it's way into his inner ears through his eustachian tubes, resulting in severe ear and sinus infections. During his first 4 years, Steven was taking antibiotics on a constant basis.
Twice in Steven's early life, I had to restrain him so the Pediatrician could perform lumbar punctures to rule out meningitis. At one point Steven had mastoiditis, in which the skull around his ear became infected, and had to be surgically scraped out.
Steven also suffered low threshold seizures once his temperature reached 102F (38.9C).
The early years were difficult ones.
Eventually, we learned that Steven has a very weak immune system, and for nearly 25 years now, he has received monthly IV infusions of the blood product Immuno-Gammaglobulin. Thankfully the IVIG has made his life much better and with only rare infections.
Another major medical challenge for Steven has been Esophageal and Intestinal muscle spasms. The muscles that move food and waste downward in his system can go into extremely painful spasms, which can result in food and acid from his stomach moving upward into his esophagus.
The discomfort of the acid reflux is not nearly as intense as the pain of the spasms themselves. However, through medication and careful monitoring Steven's suffering has been kept under control.
Steven began walking independently at the age of 8. He wears rigid orthotics to support weak feet and ankles (to below his knees). Steven also wears a back brace for support due to severe scoliosis (lateral spinal curve) and kyphosis (forward spinal curve).
He has several congenitally malformed vertebrae at the mid-spine and in his neck. In the last three years, Steven has begun suffering seizures, originally one every 6 months, and now on a two to three-month cycle. He is now taking anti-seizure medication for his epilepsy.
Sound:
Aside from what I have written so far, I have to acknowledge that we had two other children in our home who took on the challenge of Steven as bravely and as lovingly as we did. Steven went to a lot of his brother's hockey and soccer games, scouting meetings and events, Air Cadets, band concerts and school functions. But there was a divide that occurred in that one parent was caring for Steven while the other gave attention to his brothers. It was rarely Mom and Dad together even from an early stage. We were fortunate that my Mother was able to assist us at short notice, especially in the early years of Steven's life.
When Steven was just over two months old, we noticed that he was not responding to sound while we attended a local parade. We tested this ourselves by banging pots while he was awake or asleep, and he didn't even flinch. Later we had his hearing tested and it was discovered that he had very little hearing – if any at all. Suddenly our practiced techniques of communication were of no benefit to our youngest son. Talking, singing, laughter, reading, records and tapes were not going to benefit our baby. Steven's deafness was a hurdle that seemed unfair and daunting.
Fortunately, we made connections with preschool services for the deaf through the Provincial School for the Deaf in Milton, Ontario. For nearly three years Steven had an in-home Teacher who would come bi-weekly to spend a morning with Steven. This Teacher was deaf too, and she insisted that we begin ASL sign language classes immediately and begin signing with Steven (and with her) immediately. In-home sign language classes were arranged by the School, and we slowly began to improve our vocabulary.
Steven's brothers also picked up sign language from the lessons we were taking. We all worked really hard to include signing in our home.
However with everything else going on with our young family and Steven's poor health being a constant strain- our efforts to be really great at sign language never really happened. For the most part, we were identifying things for him, letting him know what might be coming next (time for supper, time for a bath, want a drink etc). It didn't help that Steven never really responded to our signing efforts. Eventually, he was fitted with hearing aids of the highest available amplification. It was described to us that with these hearing aids at their maximum setting and Steven seated beside a jet engine at its loudest – he might detect some sound. We eventually learned that the tiny chain of bones in Steven's inner ears are abnormally formed and do not transmit sound at all, so we stopped using the hearing aids.
When Steven was two years old we enrolled him in a preschool class for deaf children run by our local public school board. That this program existed at all was a tribute to many local parents of deaf children, who had advocated very strongly for such a program to be in existence. Steven was not a typical two-year-old. He had poor muscle tone, could barely roll over, could not stand, and could only bear weight on his feet while in a standing frame. Although we were signing constantly with him and he was in a signing classroom environment, Steven only rarely would attempt to sign back to us. His poor muscle coordination impeded his ability to sign. There were concerns that he might be injured by the more rowdy two-year-olds in the class, but in fact, the other children were very gentle with him and accepting. The teacher located a two-person standing frame with a play surface in-between the two frames. Steven would be strapped in at one end his classmates took turns at the other end. This is an example of accommodations that were made for Steven, and that the other children accepted him.
Light:
By this time Steven was wearing prescription eyeglasses, both to strengthen a “lazy eye” and to properly focus visual input to the back of his eyes. The lens over the strong eye was patched to force the weaker one to work harder.
It was suggested to us by his Teacher of the deaf that Steven's overall vision might be more impaired than our Optometrist was capable of detecting.
An appointment at the University of Waterloo's Low Vision Clinic verified this. Steven was not only deaf but legally blind as well. The point at which Steven's optic nerves connect to his eyes has significant abnormalities – congenital optic nerve atrophy.
The immediate result of this dual diagnosis was that the support network from The School for the Deaf had to immediately terminate support both for our family and for Steven.
Not only did we feel overwhelmed (again), but also abandoned.
Steven's new support network was from the W. Ross MacDonald School for the Blind and Deaf-Blind in Brantford, Ontario (WRMS). We quickly learned that having a dual-sensory disability does not come with an increase of support.
The “Deaf Community” in Ontario is large in population and has a strong political lobby. There was a lot of Government money streaming towards an agenda that would recognize ASL as the official language of the deaf, and also towards “Defining Deaf People as a Distinct Cultural Entity”. One of the major achievements of this lobby has been the emergence of ASL interpreters on television etc.
To be deaf-blind in Ontario is another thing altogether. The deaf-blind community is very small in number. Many deaf-blind people also have additional disabilities including intellectual disabilities. The political lobby for deaf-blind people in Ontario simply cannot compete for Government or private financial support on the scale that the deaf community does.
That being said, WRMS did provide in-home teacher support on a monthly basis, to provide us with ideas for communication, and to observe Steven's response to visual stimulus. We were also put in touch with The Canadian Deaf-Blind Association(CDBA) – a parent-initiated organization supporting people who are deaf-blind. The CDBA is centred in Brantford, and many members have attended WRMS – and many of the board members over the years have included teachers and principals from the school as well as parents of deaf-blind children.
The CDBA has played a key role in Steven's life and in supporting our family.
The first time we felt that we truly “belonged” was at a CDBA parent retreat weekend, where we met many families with deaf-blind children of all ages. Many of the affected individuals are “deaf-blind-plus” - having additional disabilities beyond the obvious.
About this time Steven stopped wearing glasses, as the “lazy eye” was determined to be his attempt to focus, and because his visual intake was so impaired by the optic nerve issues. Steven's best field of vision is within 12” (30cm) of his face. He can detect movement up to 10 feet (3m). He also seems to be able to see things better by looking down and to the right or left. He is also able to distinguish colours.
School and beyond:
