Hear, Hear!

Listening to you, I get the music
Gazing at you, I get the heat
Following you, I climb the mountain
I get excitement at your feet

--The Who, See Me, Feel Me

Last Thursday, amongst two night shifts, two trips to the dentist and getting our ducts cleaned, we took Wyatt to the audiologist.  Like many preemie babies (with and without Down syndrome), he was referred for frequent monitoring of his hearing.  Those with DS are watched especially closely as there are a variety of hearing issues that can occur. He's had his hearing tested several times since birth , starting with our first trip when he was 4 months old (corrected).

Hearing issues are a common problem at birth (about 1-2% in the general population).  Many populations of infants are at high risk;  those amongst the highest include the premature, those with lower oxygen levels at birth, jaundice, irregularities of the head and face, infections, low Apgar scores and conditions such as Down syndrome.

Testing infants, as you can imagine, is a little trickier than testing a larger child or an adult that can easily indicate or describe what they are experiencing.  Also, there is the source of the potential hearing loss to consider;  is it a conduction issue or a sensorineural one?  Luckily, there are two different methods to test hearing in the little ones.  Both are automated, non-invasive and do not require the infant to react to anything.

ABR (or BAEP)

The Auditory Brainstem Response (ABR) or Brainstem Auditory Evoked Potential (BAEP) test monitors electrical impulses between the auditory nerve and key portions of the brain.  Electrodes are placed on the scalp and the activity is recorded as a series of clicks are delivered to the infants ears via small earphones placed in the ear.  Using this test, it can be determined what range of hearing the child has. 

OAE

The Otoacoustic emission test  (OAE) measures a sound bounced back from the inner ear and tests the functioning of the cochlea.  An earpiece containing both a microphone and a sensor is placed in the ear and a series of clicks and tones is measured once it has been processed by the inner ear.  Sounds that are required for understanding speech are tested and based on this criteria, the child either passes or fails. 


Wyatt has always passed with flying colours in his right ear, but his left was presenting with some odd results.  During the OAE, we were either detecting fluid in the inner ear or a small depression on the eardrum itself.  This time, as he is older and now able to be conditioned to a stimulus, we had a new test; he was taught that after he heard a sound, a mechanical toy would light up and play for a few seconds in a shadow box.  Tiny microphones, identical to the ones he wore in his ABR tests, were placed in his ears and he was given some toys to occupy his attention when he wasn't being tested.  He loved this test, was conditioned after two tries (!) and passed with flying colours.  So much so that he has been discharged from the high risk program at Erin Oak and will only have to return if we encounter problems (such as suspected damage from ear infections, etc). 

We were happy to get this news;  not only is it one less appointment to worry about, but knowing that his hearing is perfect and speech ready is a weight off our shoulders.  We kinda knew that anyway... That kid could hear a cookie wrapper opening in the next room with the radio on and his brother and sister noisily tearing about the place.  However, now we know his hearing will not hamper his language development.  Which is important, as I long for the day that he can finally say (using a word or sign) "Mama".

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Hyde, M., Newborn Hearing Screening Programs: Overview, The Journal of Otolaryngology, 34:2, August 2005.

Mersch, J., Kibby, J., Newborn Infant Hearing Screening, MedicineNet, 2012

Special Thanks to:

Infant Hearing Program, Mount Sinai Hospital, Toronto.

Erin Oak Centre for Treatment and Development, Ontario.
 

What a Long, Strange Trip it's Been

The last little while has been a complete blur of appointments and late nights.  Copious amounts of carbs have been consumed (and cheese, most often together).  Much sleep has been lost or interrupted.  Tempers have been a bit short on all accounts, including both of us, the kids and the people that we have had to interact with.  At various points, this past week has seemed to never want to end.

It started the day after the birthday party (Monday, April 23rd).  We had an OT visit where several things were looked at in detail.  One, how well Wyatt's mobility has come along in the last few weeks.  Since the last update, he can make his way across the floor, half dragging, half commando crawling.  We have not seen him sit up (by himself) since the party, however he has come very close several times.  It's probably like the rolling thing;  we won't actually see him doing it for a while.  We'll just look over and assume he just teleported, or in this case, was magically repositioned while we weren't looking.  We are trying to get him to understand what his legs and feet are for (other than additional hands);  we have a variety of sitting/kneeling/bent over positions for him to practice and to get used to putting some weight on his legs.  So far, not much, but we are trying.  Point of interest number two:  how far his eating skills have come in the same amount of time.  We talked about finding a good time for another speech-language assessment before the OT left.  We should have an appointment by our next visit.

The following day (April 24th) meant an appointment with the pediatrician for both Wyatt and Zoe.  I don't know about you, but a snotty receptionist can really set me on edge.  This one in particular seems to have majored in Passive-Aggressive with a minor in Bitch.  The day before, she had telephoned to confirm our appointment.  During that call, no actual appointment time was mentioned when she spoke to my husband; I assumed I had the correct time listed in my phone.  You will imagine my surprise as I blustered into the office, 15 minutes early (for a change!) only to be met with cold silence.  Once I had dug out our health cards and settled my daughter (who had just had what could only be a harrowing stranger experience in the elevator with a kindly old man who told us all about his twins), I turned to face the receptionist.  She barely made eye contact, quickly scooped up the cards and said quietly "I just called your house."  "Oh?"  I answered, confused.  "What's wrong?"  "You're half an hour LATE!" she informed me in clipped tones, barely containing her hostility.  I whipped out my phone to check the times and surprise, there was no match.  For some reason, our appointment had been moved to 9:45.   My husband (to the receptionist's surprise) walked in a minute later and reminded her that she did not mention a time on the phone and therefore, did not confirm the appointment properly. Mistakes had been made all around.  There was another period of awkward silence, where I prepared to get comfortable in the waiting room as I figured she would now make us wait.  A few minutes later, she approached the babies with artificial sweetness and told us that it was time to come in.  We maneuvered our beast of a stroller through the narrow hallways into the exam room and began to get them undressed for their weigh in.

Aside from that little bump, the rest of the appointment went well.  There was no one else in the office at that time, so the doctor was able to see us.  Zoe now weighs 9.14 kg (20.1 lbs) while her now little brother Wyatt weighs 7.95kg (17.49 lbs).  They are both small for their age, at 70cm (27.6 inches) and 67cm (26.3 inches), respectfully.  Their big brother Quinn was 25 inches at birth, just to offer up a little comparison.  Both rank somewhere in the 5th percentile for height on their respective charts (although Zoe has crept up a bit in the weight department).  Numbers aside, both are happy, healthy infants who are doing well in their own way.  Wyatt attempted to sit up while we were there, which made the doc happy.  Zoe wasn't too keen on walking barefoot on a cold floor, so she did not get to show off her running prowess.  The doc made sure that we got an actual appointment card this time and we took our leave.

Wednesday (April 25th) was the ENT.  Sean took Wyatt by himself, as it was an early morning appointment and it interfered with Quinn's schoolbus time.  I was expecting the boys home shortly after 10 but they didn't arrive until 11:30 or so.  I was a bit confused at first, but Sean explained that our bad doctor karma from the day before had spilled over into this appointment as well.  Although they were on time, the ENT was not.  After sitting for almost an hour, the doc wandered in the front door, ignored what was now a packed waiting room, went straight to the back and had the first patient sent in. It was apparent that he was in a shitty mood by the time Wyatt's name was called;  he then proceeded to take it out on Sean.  We haven't been exactly diligent with remembering to put drops of mineral oil in Wyatt's ears (to keep the wax loosened) and the doc let Sean know a little more brusquely than necessary that this wasn't cool.  In fact, his tone suggested negligence.  He even went so far as to accuse Sean of using Q tips on him which, if you don't know, are a no-no with ears as they are self cleaning (Q tips just impact the wax).  Sean tried to explain how we never use Q tips and clean only the external ear and when that was completely ignored, he half-desperately threw in a "you know my wife is a nurse, right?"  for good measure.  This all had very little effect on Dr. Grumpypants who continued by demanding to know when Wyatt had a specific hearing test done.  We do keep on top of all his hearing appointments, but lets face it, we have no idea what exact tests are taking place, just what the results are at the end.  This was not an acceptable answer either and prompted him to place an angry call to the audiologist's office. After extracting the gunk from Wyatt's ears (which, wasn't too bad at all), the boys were then sent back to the waiting room to wait.  During that stay, Sean overheard the secretary talking to the audiologist's office and learned that the specific test that the ENT was peaking about was not done at the last appointment... as it had been done at the one before.  This fact would have been apparent if he had bothered to read Wyatt's chart. A more sheepish ENT called them both back in to let them know the results of the hearing test and that based on that information and the examination that he had just performed, Wy's ears are just fine, thanks.  See you in 6 months.

Flash forward through a whirlwind of my 6 year old's actual birthday (April 26th), one twelve and one 16 hour night shift (April 28th and 29th).  It was now Tuesday of the next week (May 1st) and time for Wyatt's sleep study at Sick Kids.  Prior to this, we had to fill out a sleeping/eating log for 10 days;  it was only through some miracle that I remembered to yell at someone at the last minute to grab it as we were going out the door.  Even for overnight, I had to take a lot of stuff for me and one baby.  Jammies, diapers, wipes, cream, pillows, emergency bottle, Tylenol, toys, 'whoosh whoosh bear', the stroller to push all our stuff around in... plus I brought the laptop "just in case".  Although it turns out that there is a complementary TV in the room (and free WiFi!), I wanted the ability to play a movie or write/fool around on the internet if I couldn't sleep.  I figured that, much like sex in the champagne room, there was no sleep in the sleep study.  I had no idea how right I was going to be. 

It was a good thing that we arrived early as it gave me a little extra time to get lost.  It's a pretty straightforward hospital in most respects, but you have to trust me, getting lost is a specialty of mine.  Sean claims that I have a compass in my head like everyone else, but unlike everyone else, mine is broken and just spins.  I do make up for this with my map skills...  To prove this point, seconds after I discovered a wall map, I found the Atrium and the elevators that I needed.. 

Sick Kids is a fabulous place... I have mentioned this before.  The main elevators are a sunny yellow and the whole place looks like it was designed by a toymaker.  However, if you have vertigo like me, it's a little unnerving getting from the central elevator hall to one of the quads. 

You can see the yellow elevators on the left.  Photo courtesy of skate_simmo

We were greeted by our Tech who assured me that they do sleep studies on babies all the time. In fact, last week he had three babies there at the same time (the lab has four beds).  We were shown to our room and I set everything up while Wyatt got to play in his crib a bit and stretch out after the car ride.  I realized somewhere during my exploration of the room that psychiatry has ruined me:  the entire time I was setting up, all I could think of was how unsafe the room was.  Including the closet, which was just the right depth and had a bar in there that someone (small) could hang themselves with.

Our room.  My daybed is in the back there

My bed.  I've slept on a lot worse, trust me.  For my convenience, I'm right next to the hanging closet.

Baby logistics is another specialty of mine.  Knowing that I had to feed him for him to go to sleep and assuming that there was a tangle of wires in his future, I had to work out how we were going to do this.  Our Tech and I talked about it and he would come back to wire my son up when he started to look a little sleepy.  I tried to make Wy feel as comfortable as possible;  he had a few toys there, a few homey smells and I set up the laptop and streamed some music for him (they have a radio on all night for noise and company).  He started to look a little droopy and I called our dude in to (literally!) hook us up.

For those that don't know, during a sleep study you are monitored for quite a few things.  Your heart rate, your breathing and your oxygen/CO2 levels are important.  They also monitor brain waves so that they know how asleep you really are. Finally, they monitor movement, to see how restless you are.  Basically this translates to an ECG, an EEG and an oximeter.  There was also a camera in the room and an observation window if needed.

In total, there were about 20 'leads' or wires about his little person.  Two on each of his legs, four on his chest and the rest on his head.  There was also a O2 sat (oximeter) on his toe an a CO2 monitor on his chest (that was relocated to his back in the middle of the night).  He looked like a little Borg.

Resistince is futile.  (He's not crying BTW, just mid-babble)

He got a little Teletubby hat made out of some Surgifix (stretchy tube mesh that they put over IV's and lines and things) to keep the wires on his head.  The Tech put a towel over the lead box on the bed hoping that Wyatt would forget it was there and I was given the OK to feed him.  I set up shop beside the bed, gingerly pulling on the wires all the while hoping there was enough slack.

He wouldn't sleep for love nor money.

Usually he falls asleep during his bedtime "snack".  No dice.  I whipped out a bottle of homogenized and heated it up for him.  Nope.  At one point I  turned on the end of the hockey game, hoping he'd get tired (he watches a lot of hockey with Daddy if he can't sleep at night).  We called Daddy, we sang songs, we rocked, we burped, we got Tylenol.  Finally, about 10:30 he went to sleep in my arms.  I carefully tucked the towel underneath him so that he would not immediately roll on the wires (or catch them in his sleep), put up the side rail and tiptoed to my cot.  I didn't realize how tired I really was until my eyes closed while I was typing on the laptop.  I shut everything down, pulled the blanket over me and drifted off. 

At 2 am I woke to him stirring and was at his side a minute later when he started to cry.  Once he is down, Wyatt does not wake up in the middle of the night unless he is sick.  What I found when I got there was pretty much heart stopping:  all the wires were wrapped around his neck.  I sat my crying boy up and started to gently un-garrote him;  he looked like a kitten tangled in a ball of yarn.  At one point I had to go get the Tech for help as I was afraid of dislodging everything.  It took a bit, but we were able to free him from his wire-y prison. 

I am sooo tired!  I also HATE this hat!

For his trouble, he also ended up with a nasal cannula, which he also hated.

This thing is 'teh suck'.

You can bet that he was wide awake after that, so I put the radio on over the phone and held and rocked him.  He kept trying to pull out the cannula and that one purple wire in the photo above, so the only thing I could do was to put him on my left shoulder, tuck his one hand into my armpit, hold him (under the wires) under his bum and hold his other arm down with my right arm as it held him over the wires.  And rock.  Which I did...  For a solid hour.  I thought he was asleep at one point and tried to put him down but it didn't work as he was crying and rolling and threatening to get tangled up all over again.  Finally, he was a sleeping rag doll again around 3:15 and I tiptoed back to my cot.  I had a hard time settling as well;  I think I got back to sleep around 4, although I woke up several times to unfamiliar hospital sounds.

My eyes opened again at 10 minutes to 6, so with a sigh, I started quietly packing up the room.  The test ended at 6 and our Tech was there promptly to unhook Wyatt from his torment.  We turned all the lights on and found my little lamb sound asleep.  I hated to wake him up but it was time to go.

Wakey, wakey Wyatt.

The conductive jelly that was in his hair, despite the reassurance that it was water soluble, would NOT come out with warm water and a cloth.  I got him dressed, packed up the room, stripped the beds and was out by 6:30.  I managed to get lost a completely new way before (gratefully) finding the Whaaambulance and my family waiting for me outside the Elm St. entrance.  We rocketed home in the fog and by afternoon, all 5 of us went down for a nap.  After a nice bath, Wyatt also had 95% of the tape and goo out of his hair.

My week did not end there as I had a 12 hour night shift the next night (May 2nd).  I also start a new 4 day tour tomorrow (May 5th) but the kids have settled back into their routine.  I won't know the results of the study for a while.  In fact, I'm not sure how I will find out exactly;  we will probably hear from one of his doctors and/or get a phone call or a letter.  I do know that while I worked the night away, everyone slept very well at home, in their own bed. 

It's been a busy, crazy, cranky and tiring spell for Team Logan, but we have come out the other side of it.  I know I will be happy to see Wednesday morning (May 9th), when I start my next run of 5 days off. One of the good things about blitzing through a bunch of appointments is getting them all over with... which we have, until the next go around in a few months time.  In the meantime, we won't be idle waiting for results.  Instead, life will go on as it does for my raucous household.  Full of laughter, life and love.

Now that's worth the price of a few crappy nights sleep, isn't it?

Speak to Me

Speech can be very difficult for those with Down syndrome.  Due to delays in memory and aural learning, as well as co-ordination issues due to lack of muscle tone and control, speech  develops very late compared to "typically developing" peers.  Most actual speech therapies start after the first year (about 18 months of age), which begs the question, what can be done in the mean time?

One of the areas that you can work on with your infant is developing the face and mouth.  These are the same exercises that one would use to strengthen the facial/mouth muscles and increase sensation in preparation for eating.  These techniques are courtesy of ICDSP:

Facial massage:  Using two fingers or your thumbs, stroke the baby's face from the upper cheek close to the ears down to the corners of the mouth.  Then, stroke down from under the nostrils to the top lip.  (Do this 3 or 4 times a day before eating)

Palatal massage:  Insert a clean finger into the baby's mouth and stroke the roof of the mouth (the palate) from the middle to the side, stopping at the gum line.  Return to middle and continue to other side; repeat 3-4 times prior to every feed

Gum massage:  Trace along the gums with firm pressure from the front to the back on each side, top and bottom.  Do this 2 to 3 times, twice a day.

Chewing/Toy Mouthing:  introduce and encourage the child to use a variety of teething rings, soft toys and feeding utensils;  introduce horizontally and to the side to encourage biting of the toy.  Do this 3 to 4 times, each side.

Along with these techniques is the single most effective tool to strengthen the oral muscles:  breastfeeding.  As it provides more resistance than bottle feeding, it encourages the development of the muscles of the lips, cheek and tongue and enhances the coordination of these with breathing.  Soother use is also encouraged for this reason.

Another area that can be worked on is sound recognition.  As children with Down syndrome are primarily visual learners, the goal is to help the infant link sound with facial expression.  When the child is alert and relaxed, find a comfortable position that places both of you at eye level.  Remove any background distractions such as a radio or the TV and make eye contact with your child as you make sounds.  As you make each sound, monitor your baby for any reaction.  Encourage repetition by repeating any sounds that he or she makes.  You can make non-speech sounds (which include clicks, pops, "raspberries", etc) or speech sounds (which are repeated consonants or vowels or combination).    Make exaggerated examples slowly and clearly to help the baby hear and react to the sound.¹

Talking, singing and playing with your baby face to face will also encourage the little one to pay attention to sound. Ensuring optimum hearing through frequent assessment will also help them acquire language. 

Although statistically, many children with Down syndrome do not learn to talk until much later than their typical peers, it is possible to help them prepare for this eventuality.  By encouraging strong facial muscles along with memory, speech and language skills, the child will be able to speak more clearly and have an increased vocabulary.  Which, is extremely important for those very first spoken words. 

I won't say "Mama" for a while yet, but it will be phenomenal when I do.

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1.  Courtesy of R. Grey, Speech-Language Pathologist at Trillium Health Centre:

Down Syndrome and the Developing Child

One of the most common questions I get asked is "how is Wyatt developing?"  When I don't have an easy, static, predictable answer (such as "he's working at a ____ month level", for example), I get a lot of confused looks.  With that in mind, this Teaching Tuesday is dedicated to development in the child with Down Syndrome, from birth to 5 years of age.

Each child with Down Syndrome is different.  Although all with Trisomy 21 share a third copy of the 21st gene (for example), how that gene expresses itself varies from child to child and is unique to him or her.  This also applies for both Translocated and Mosaic Down Syndrome.  As the physical aspects vary, so do the developmental delays that each child faces.

It is also important to understand how children develop.  Most children acquire and hone new skills and achieve 'milestones' that are not a result of direct teaching (with a few exceptions, such as counting, reading and toilet training).  Instead, children learn in the family setting, by being encouraged and modeling behaviour in a social environment.  Parents shape appropriate behaviour, language and actions by being sensitive and responsive to needs and rewarding where this has been achieved.  A wide range of learning and social opportunities are explored in the context of the family, as well as through books, toys, outings and social interactions. (Buckley, Sacks, 2001) By comparison, children with learning disabilities may have to be actively taught certain skills due to their individual deficits.

Children with Down Syndrome do have a set of general strengths and weaknesses;  these will impact on the child's ability to learn and grow.  The following developmental profile for Down Syndrome is courtesy of "An Overview of development of infants with Down Syndrome (0-5 years)" (Buckley, Sacks, 2001)

Social Understanding and Interactive Skills

• Relatively strong and less delayed (compared to speech and language)
• Can make eye contact, smile, coo and babble from early months
• Socially sensitive and understand non-verbal cues (facial expression, tone of voice, body postures) in first year

Motor Skills

• Most (such as reaching, sitting and walking) delayed
• Main milestones are steadily achieved
• Most children become mobile and independent in self-help skills (ie: feeding, dressing)

Speech and Language Skills

• More delayed than non-verbal understanding and reasoning
• Most understand more language than they are able to express (due to speech production difficulties)
• Signing helps majority of children to communicate (and reduces frustration)
• Hearing problems are common and contribute to difficulties in this area
• Speech is most serious delay as it impacts all areas of mental and cognitive development

Working Memory Development

• Impairments with verbal short-term memory
• Impacts on child's ability to process information

Visual Memory and Visual processing

• Relative strengths
• Auditory processing and memory more impaired
• Visual learners;  use visual aids for all teaching

Reading Ability

• Often a strength (as early as age 2)
• Builds on visual memory skills
• Reading activities can be used to teach spoken language 

Numbers

• More difficult
• Delayed when compared to reading

Social Behaviour

• A strength
• Less likely to develop difficult behaviours than other children with cognitive delays
• More likely to develop difficult behaviours than typical children of their age

Not surprisingly, there are different developmental guidelines that have been established for children with Down Syndrome.   Among those are Milestones for Children with Down Syndrome (Buckley, Sacks, 2001) and a series of growth charts that are Down Syndrome specific (Courtesy of the American Academy of Pediatrics).  Utilization of both are important as it allows for a child with Down Syndrome to be assessed more fairly;  not in comparison to his or her typical peers, but to peers with Down Syndrome and most importantly, to the child themselves.

Hearing Complications with Down Syndrome

Not only does hearing allow us to experience the sounds around us, but it plays a major role in the development of speech. Children with Down syndrome can have a variety of hearing issues, both congenital and acquired, which if not treated can lead to greater problems.

Congenital disorders of the ear generally involve a sensorineural issue (whereby the sensory information is not transmitted or received by the auditory nerve properly). Often this involves the higher tones and is therefore not discovered until later on (as the child would continue to hear many of the sounds around them) and would therefore impact on their development (especially with speech). Routine screening allows for the discovery of congenital ear disorders. Down syndrome may affect any or all of the structures in the middle and inner ear.

Acquired hearing disorders can be divided into two categories: conductive hearing loss and sensorineural hearing loss. These can occur separately or together; many children with Down syndrome have a mixture of both.

A build up of cerumen (ear wax) can easily interfere with the functioning of the eardrum. If mobility of the eardrum is decreased, the sounds transmitted to the middle ear will be decreased as well. This is easily treated by a variety of measures including drops and suction removal by a qualified professional.

Those with Down syndrome can be more susceptible to more infections than the general population and as a result have a higher incidence of ear infections. Over time and especially if left untreated, these infections can cause damage. Fluid buildup in the middle ear, a ruptured tympanic membrane (eardrum) and difficulties with the ossicles can all result from infection.

As well, a condition commonly referred to as "glue ear" can develop. Sticky mucus can accumulate in the middle ear which can literally encase the ossicles and prevent them from vibrating (and therefore, transmitting sound). People with DS can produce more or stickier "glue" which frequently becomes infected as it does not drain away. Further hindering drainage is overall smaller Eustachian tubes in those with Down syndrome as well as the tendency for these tubes to be sharply curved.

With both of these conditions, antibiotics are most often prescribed. If the fluid or glue buildup is persistent, the child may have to undergo a tympanostomy and have "tubes" or grommets placed in the eardrum to allow the fluid to drain away.

As with any potential problem, early intervention is the key. Have your child assessed early by a trained audiologist and your medical team to prevent and minimize the chances of long term hearing loss.

"Anatomy of the Human Ear" shared with permission by Perception Space-The Final Frontier, A PLoS Biology Vol. 3, No. 4, e137 doi:10.1371/journal.pbio.0030137. Creative Commons.