Falling Into Fall

I love the change of seasons in Canada.

I love the chill and coziness of the winter months. Cuddling under blankets, sipping warm drinks by the fire. Nesting inside our warm home while the snow flies. (I even love running in the snow).

I love spring. Shedding sweaters and turtlenecks for another year. Getting down and dirty in the garden. Loving the freshness and newness of the season and anticipating the summer months.

And summer – lazy days – a break from it all – a complete change in routine (both good and bad) – less time at CHEO – warm sunny days – cute little sundresses – flip-flops on my feet.

But fall is my favourite. Fall feels like the new year to me. It feels like the start of things – back to routine (yes, I like routine), cozy soups, cozy sweaters, the changing colours, a crispness in the air that requires just a little bit of bundling up, the warmth of a fall sun that takes the edge of a chilly fall day, getting back to activities for Jack and Kate…

 

Fall Leaves

 

Fall is a very significant season for life with Kate. Kate was born in October (2007). She was diagnosed with hearing loss in November (2008). She received her first hearing aids in November (2008). She was diagnosed as Deaf in October (2009). She received bilateral cochlear implants in November (2010). She was admitted for her first long stay at CHEO in November (2008). She visited the Mayo Clinic in October (2009). She received her eventual diagnosis of SIFD in November (2011). Kate started school for the first time in November (2012).

There are so many anniversaries to reflect on in the fall. It is an emotional and weighty season and it is a season of promise.

I wonder what changes, events, new milestones this fall with bring for us.

 

Julie

 

 

Kate is Deaf

I read this article a couple of days ago, and it got me to thinking and writing about what our journey with a Deaf child has been like. There is also some great advice in here about understanding the ‘capital D’ Deaf world and Deaf culture. Worth a read.

And then there was this article  today, written by a deafened woman (a hearing person who loses their hearing). Interesting take on cochlear implants and the idea of wanting to be ‘fixed’

It’s been a journey for me to have settled on whether or not to use the capital ‘D’ in describing my child as being Deaf. You see, using the word Deaf as opposed to ‘deaf’ (small ‘d’) denotes her inclusion as a person who is part of the Deaf culture. But I wasn’t sure that describes who Kate is, or was going to be, and then I realized it wasn’t for me to decide, it was for her to decide – or to show us who she is.

The small ‘d’ / big ‘D’ description of being a deaf/Deaf person is complex, and as a hearing person I feel reluctant to try and describe it here and really do it justice.  Big ‘D’ has to do with being culturally Deaf, born to Deaf parents and part of a very close Deaf community where aided hearing and use of your voice are not seen as something that are important to being part of the community (and sometimes discouraged). Big ‘D’ Deaf people could also be born to hearing parents but schooled in ASL, having attended Deaf school, and finding that their comfort lies more with the Deaf community than the hearing community.

Small ‘d’ deaf is using your voice, integrating into mainstream school, using hearing aids or cochlear implants and relying largely on your hearing to engage with the world. Often, small ‘d’ deaf people don’t identify with being deaf at all, and wouldn’t use the word ‘deaf’ in describing themselves. They are fully integrated into hearing culture.

And of course both of these, small ‘d’ and big ‘ D’ reside along a spectrum of choices, cultural priorities, language, interacting with the world, and decisions about aided hearing and use of voice.

 

Our Journey of Understanding

It wasn’t devastating for me when I found out Kate had moderate to profound hearing loss at 12 months old. I had suspected for some time and had been advocating for her to get hearing tested. It unfortunately took some convincing of her doctors. I didn’t realize the impact that being hard of hearing would have for her over the next few years. I thought with moderate to severe loss, with her cute little pink hearing aids, she would be able to hear us (‘normally’) and would learn to speak. I hadn’t had much exposure to persons who were hard of hearing or Deaf, only to my grandparents who wore hearing aids in old age (not the same). But over the next 6-12 months, I gradually came to understand that being hard of hearing was going to be a challenge for my child – and for us as a family. After her initial diagnosis, Kate continued to lose her hearing until at 20 months, she was diagnosed as profoundly deaf.

Deaf people don’t like to be described as having hearing loss, they don’t see it as a loss and prefer to be referred to as hard of hearing and/or Deaf. But you see, it is a loss in so many ways – and that doesn’t need to be construed as something negative in the long-term, but it is a loss.  It was a sadness for us, and we did grieve that loss for our child. I don’t see this as a slight against my Deaf and hard of hearing friends, in fact I know some of them would agree. I do understand their need to carve out their identify and separate it from the word ‘loss’, so in that context, I think the word ‘loss’ has be to understood to have two different meanings. When you are describing a Deaf or hard of hearing person, you don’t describe them as having ‘hearing loss’, it is offensive to them and an inappropriate way to refer to who they are as a person. But the grief and sense of loss for the family is real and has to be appreciated.

When Kate’s profound deafness was confirmed, I started to move out of having my (incorrect) expectations that this journey wouldn’t be difficult. I knew then that Kate also had serious developmental delays, and an undiagnosed rare disease to contend with as well. I knew immediately that we needed to give her access to everything possible to aid her in communicating and interacting with her world.  At that time, I remained unaware of the deep-seated philosophies and cultural identity of the Deaf community. As with most beliefs, it lies across a spectrum. There are Deaf people who believe CIs are parallel to eugenics or an act of ‘genocide’ against the Deaf community by the slow elimination of their community and language. They feel the medical community sees Deafness as a sickness to be cured and hearing parents as trying to turn their deaf children into ‘normal’ hearing children.  They feel losing their culture, their language, and being seen as persons with a disability in need of medical intervention – they certainly do not see themselves this way and consider it highly offensive.

Other Deaf people are more moderate, and understanding that more than 90% of deaf and hard of hearing children are born into hearing families and communities. They understand that with infant hearing testing, we are identifying hearing loss at birth and with new medical technology we can facilitate the ability to support/regain this human sense. They understand the families desire to be able to communicate with their child and offer the best of both the hearing and Deaf world to their children. At a fundamental level, I believe that there are many in the Deaf community who understand that cochlear implants and other emerging technologies such as stem cell treatment and gene therapy (even more controversial) are here to stay. A huge choice is emerging for the Deaf community to embrace this community of deaf children and welcome them to the Deaf community. Families (like ours) want to use both our hands and voices. Not all deaf children will learn to speak, not all will have the option of CIs, and not all families will choose technology.

It is not an easy position to be put in – having a deaf or hard of hearing child. The choices are difficult and often influenced by others, as well as our own initial ignorance and biases. I feel fortunate that we kept out options open. Kate was not an easy cochlear implant candidate because there are risks associated with anesthetic for her. We had to very carefully weigh out all the considerations and scenarios for her. A very possible choice for us was to not risk the surgery for her and choose for her to be a Deaf person with no access to hearing. In the end, we felt that giving her access to hearing and all means of communication possible was her best option. I felt confident that we could support her auditory and verbal learning. I also felt confident that we could learn American Sign Language (ASL), and find support within the Deaf community.

Learning ASL has been a process. Like any language it takes time and commitment, and a lot of practice. In our family, I have taken the lead having studied for 3 years now at night school at our local community college. Others in my family and community haven’t been as interested, or aren’t around Kate as much, so it has come to educators and others in the Deaf community to provide that role modelling for Kate.

Support within the Deaf community has been more difficult. I had to advocate very hard to get a ‘Deaf education’ for Kate. There were many from the hearing community willing to support us; our hospital and our local public health infant hearing program, these were hearing people with skills in ASL, but the Deaf community was much more difficult to access. There was no mentorship from other families, other than to find online support groups of other hearing families with deaf children, and most of those were highly focussed on auditory and verbal. The Canadian Hearing Society had many resources to offer, if you were an adult, but they had nothing for children (and still don’t). The Deaf community was also a very difficult ‘resource’ to access. It took a lot of time to meet Deaf adults who were willing to work with us and support us, and even still, meeting Deaf families with Deaf children has not happened for us. I am very grateful for the Deaf teachers and friends I have, for their support and mentorship and understanding and support for Kate (Denise, Kat, Phillip, Les, Todd), but I feel that there is a lack of resources and outreach from the Deaf community. And this is my difficulty with the strong stance against hearing families and their deaf children who are choosing technology, cochlear implants and full integration into the hearing world/culture. Some members of the Deaf community have high expectations of us with respect to the Deaf culture. They should go to Deaf (residential) schools, they should play with other Deaf kids, they should learn ASL exclusively, they should not be ‘forced’ to learn to speak, we should not be giving them cochlear implants. It is a strong position to hold when there is not much support to the hearing families of deaf and hard of hearing children to access Deaf culture.

Having her CIs has not been easy for Kate either. Because she can ‘hear’, people make assumptions about her; that she can hear them from a distance, or above the noise of a crowded room. They don’t realize it takes a lot energy for her to focus on hearing, that her hearing is digital, and that they should speak close to her microphones, clearly and face her so that she can still see their lips. Though we are grateful she has them, CIs are aided hearing, they are not the same as natural hearing.

I have chosen both worlds for Kate, and as she grows and evolves, I see her ability to communicate emerging. We are now at a crossroads with her as she has had her CIs for over 3 years and still has little language. She hears us (well, we think), she understands us, she has some words, she has some signs – and we have been ‘educating’ her in both equally. But now I see that we may want to try even more immersion in Deaf culture and ASL in hopes that we can support her ability to communicate and develop language even more. This opportunity is available to us now because she is school-aged and can attend the one and only Deaf and hard of hearing program in our local school board.

It doesn’t matter to me if she becomes part of the hearing world, or Deaf world, or both. It matters that she is the best she can be and that she is happy. I think that is what we all want for our children.

I never thought I would hear Kate speak, say ‘mommy’ or tell me that she loves me. She can do both now, in ASL and in speech.

How do I describe Kate? She is Deaf and can hear and she is deserves both worlds.

 

Julie

A Week of Milestones

I’m going to have to really sit down a couple of evenings this week and write specific posts about the exciting week of milestones this second week of November means to our family.

Kate’s Confirmed Diagnosis

It will be one year ago today that Kate received a confirmed diagnosis of a rare mitochondrial disease. A highly specialized genetic sequencing had been done with Kate’s DNA in the late winter of 2011. After 8 months several genes were identified as suspicious.

Once Brian and I gave blood and the same recessive defect was found on our genetic code, the diagnosis was confirmed.  On this day, 12 months ago, we sat in a room with our metabolic doctor, Dr.C and talked about the fact that our daughter has a recessive genetic disease that she has inherited from both Brian and I. It was a disease that had been causing dysfunction of her mitochondrion – the energy producing part of every cell in her body – and had been wreaking havoc on her body, causing more than 10 identified medical conditions. There was very little understood about the dysfunction becausse on that particular day, at that particular moment, Kate was only known case in the world with this rare genetic disease. It had no name, it had no clinical description, there was nothing known about how the disease might progress, and there was no treament or cure.

This week both CTV News Ottawa and Global National will be running stories about Kate’s rare mitochondrial disease. The CTV story airs tonight (Wednesday, November 7th) at 6 p.m.  Stay tuned about Global Nationals story which will feature the specific genetic testing done to identify Kate’s disease and what this important diagnostic tool might mean for other children who are undiagnosed with chronic disease.

Kate’s Bilateral Cochlear Implant Surgery at Sick Kids

Tomorrow, November 8th is the second anniversary of Kate’s bilateral cochlear implant surgery at the Hospital for Sick Children in Toronto, Ontario.

This was the surgery was never to happen for Kate. We had known for more than a year that Kate had progressively lost all her hearing due to her disease, but because Kate was so chronically unwell and had suffered an acute life threatning event after a general anesthetic, it was recommended that she not undergo a CI surgery.

There will be detailed separate post on this story somday – it is a long and emotional one and I really want to tell it to you and do it justice.

What I want to say about today is that I am so grateful to two very important men in our lives. Dr. Blake Papsin, Kate’s CI surgeon in Toronto, who returned a phone call from a desperate mom after she left him a tearful voicemail asking for his help. A man with equal amounts of bluster and compassion. He is someone whose skill, expertise, and compassion for our child I will NEVER forget. He was able to give Kate a great gift, and his skill ensured a quick an efficient surgery that minimized the risk for Kate.

And Dr.Jamie Robertson, the anesthesiologist who made Kate his own personal case study. He made Kate’s safety and care his absolute responsibility and he did it with impressive elegance. He turned the study of medical anesthesia up side down ensuring that the drug cocktail and monitoring he used for Kate during her surgery would be the best possible and the safest possible. He even wrote us a summary letter describing his technique and offered to consult to CHEO or other medical centres on any further anesthesia issues for Kate.  I will never forget the closing line of that letter: “I truly hope Kate never has to have another procedure requiring anesthesia”.

Kate’s First Day of Kindergarten

What can I say. Struggle, decision, frustration, change, new direction, excitement, and joy – all words (and there are more) I would use to describe the process of registering Kate for, and starting her journey at public school with the Ottawa Carleton Disctrict School Board.

Would it happen this week? Kate was only in the hospital a few short days ago. Would we have and Educational Assistant, and Itinerant Teacher of the Deaf, and the supports needed in the classroom (amplification system, toileting, sound buffering).

It came together and though it is still a process in the making. Kate managed to get to school in time for the 2012-2013 picture retake day. She wore her fuschia sweater with the bow, her black tutu, and her ‘fancy’ shoes. She walked into her classroom with her pink backpack and went straight to her cubby, turing to me to wave goodbye. She is so big and she is still so little. I felt tears of joy well up, but none were spilled. I think they simply dried up because I was so happy for HER.

I feel like the second week of November needs a celebration night in our house. Cupcakes and sparklers might be in order for Friday night family pizza night.

Julie

Precious Ears

Cochlear implants are tricky devices. They are an incredibly complicated and delicate technology that have produced amazing results for deaf children, providing them access to hearing. The doctors and specialists who work with these devices are highly specialized in their field. The idea of threading a electrode array through a tiny, pediatric cochlea, is unbelievable in and of itself – let alone the surgery required to get the device into a child’s head. But I digress…

For parents, the mystery of the cochlear implant, of having a bionic child, is not lost, but it can be often forgotten and made to seem just part of ‘normal’ life. You get used to the routine of putting on your child’s ‘ears’ in the morning. You grow used to adjusting maps and volume to gently ‘wake up’ you child’s hearing so that they are not shocked by the usual sounds of the morning routine coming out of profound silence. You smile when your young child asks for her ears by pointing to them as a request.

For Kate, receiving bilateral cochlear implants (CIs) was a medical feat. Over the first year, it was a challenge to get Kate used to her devices. Over the second year, Kate was learning to listen and starting to respond to sound. In the past 6 months, she is clearly showing she understands what we are saying to her and responding with either ASL (American Sign Language), or in recent weeks very early verbal responses. The give and take of hearing, understanding, responding – the reciprocity of language based on hearing – can be taken for granted. In our home, it is absolutely celebrated on a daily basis. It brings tears of joy and moments of pain and fear. But mostly tears of joy. 🙂

There is also a vigilance needed when you have a child with cochlear implants. There is technology to be aware of, devices to maintain, and ensuring your child’s magnet sites are healthy. A breakdown in any one of these means no ‘ears’ and that is not a good thing.

Physically one thing to watch for is breakdown of the skin at the magnet site. The magnet that holds the processor to the skull sits just under the skin of the skull. The thin scalp skin lies between the two magnets and provides a very poor barrier between them. Young thin skin is susceptible to being irritated and ‘breaking down’. Parents play with magnet strength and try different means of balancing healthy skin with a processor that will stay stuck to the head without constantly falling off.

I watch Kate’s skin closely at her magnet sites. We had a serious infection at the magnet site 18 months ago and I am not keen on repeating that. Kate woke one morning with a magnet site so swollen it was the size of a plum and her team had thought it possible that her magnet may have actually moved. After xrays and a brief hospital admission, in the end, a strong antibiotic and keeping her device off for 6+ weeks was what was needed to heal the site.

This Friday, I knew that Kate was in trouble again and would need to have her magnet site seen by a doctor. Redness, inflammation, localized swelling, yellow discharge, crusting, (am I being too gross?), were all symptoms of a bacterial infection.  Staphylococcus aureus (staph aureus) is a common skin ‘flora’ (i.e. it lives on our skin), but it should remain on the surface of the skin and not enter the body – when it does, a nasty infection occurs. Cochlear implant site infections are particularly concerning because the bacteria can adhere to the metal of the magnet and travel along the electrode wire that threads through the cochlea ending at the auditory nerve microscopically close to the brain. Staph – or any bacteria – close to the brain is a serious meningitis waiting to happen.

Staph aureus – kind of pretty in a bacterial sort of way

 

Kate was started on Cephalex Friday.  48 hours later, her infection at the magnet site looks the same – no better and no worse – so a new antibiotic as been prescribed, Septra.

The sad thing – and joyful – is that Kate has been ‘asking’ for her ears by signing for them. She is not happy about wearing just the right side and keeps taking the device off and throwing it at me. This morning, she went and got her devices from her drying box and asked for them again. I think with perseverance and consistency we can get her to wear the right side on its own. I guess the feeling of wearing one device and hearing from one side only is not comfortable for her. I try to imagine what it is like by covering one of my own ears.

I’m hoping all of this is resolved quickly and passes easily. Having a child with an immune deficiency who is battling a staph infection can make one nervous.

We have also made so many gains in the past several weeks – words coming fast and furious – that I just want to get back to that as soon as we can.

Fingers crossed that we get her ears back soon.

 

Julie