2016

Today is one of the hardest days since November 30th. On the precipice of 2016 and realizing Kate won’t be part of this coming year.

A good friend asked me if I would continue this blog. I imagine I will, but I am uncertain what it will look like and what I will write about. 2016 will be a year of change and finding a new balance in my life.

The past month has been full of sadness, pain, longing, regret, reflection, solitude, exhaustion…I think of Kate every moment of every day. I can feel her with me in everything that I do. Just as she and I lived our lives the past 8 years.

I am grateful for Jack. He is my anchor in all of the chaos of emotions right now. He needs his mom. That is a good thing for me. Yet another role for him that is too demanding – but he is wise beyond his years and he knows when I am sad and sidles up for a hug or to hold me (role reversal). Over the past 8 years, he has done so much, learned so much, and given up so much. And here he is – my little man – giving so much more of himself again while coping with the loss of his sister. Proud is not enough to describe how I feel about him. Awe maybe.

I am grateful to Brian. Stoic and kind. He gave me the gift of time with Kate after she passed. Respectfully allowing me alone to hold her and cuddle her and be with her over that night and into the next morning. He has let me grieve as I need to. Not questioning, not judging, nor hurrying me along.

Many of you wonder ‘how I am doing’. I am not doing well, but I am doing my best. I am moment to moment in my days. I don’t feel the need to explain why I can’t smile, or can’t find the energy to talk some days. I am grateful for the words of support from all of you. I am ok with this place of solitude combined with selfishness. I am doing what I need, when I need it. Nothing more. I am grateful to those who are holding vigil for me – who won’t let me be alone for too long.

The days are quiet and long. Kate took up so much physical, emotional, and intellectual presence every day. I hadn’t realized how much. I knew I was exhausted, but coming off of the constant lack of sleep, extreme vigilance, and adrenaline of caring for Kate has been physically and emotionally challenging.

Brian and I have a lot more time that we have ever had before. We’ve literally sat in the living room and stared at each other. We aren’t sure what words we should speak. What happened? Who are you? What do we do now? Someone should get up and do something, draw up a med, clean up vomit, change a diaper, do laundry, call/email a doctor…chase after Kate. I wonder how our relationship will be redefined over the coming months and years.

It feels like we are both waiting for her to come back – that we are simply having a little reprieve from the busy-ness of caring for Kate, like one of her Rogers House visit. We are are both waiting for her and to step into our caregiving roles again. We would both pick up that role again in heartbeat to be with her.  Then, like a heavy weight, the realization comes that she is not coming back. And the sadness hits again.

We have slowly emptied our house of medical supplies, medications and equipment. We’ve made gifts of treasured things to a few of her friends. Some things we still hold onto. Her room remains the same. Her favourite books, her puzzles, her Curious George stuffies and dolls, her clothes and sparkly shoes. I think that space will remain untouched for some time to come. It is a room I like to visit often – and sometimes I open the door in the morning just to say hello to Kate. I can see her there in her bed, hair tousled, face puffy from sleep, signing ‘Good Morning’. I can feel her presence in that room, and her ‘smell’ in her dresser drawers – and that is a good thing.

 

There are no words for the loss of a child. I’ve heard that said before by others. It is true. I was bold and confident in my belief that this would never happen to Kate. I believed in my heart that she would get through this. I believed she was different, that her strength and courage would pull her through. I believed if I was strong enough, determined enough and vigilant enough – I could pull her through. I was not naive in knowing this would be a very difficult thing for her, but I was shocked at her death. When she came home Saturday, November 28th, no one expected she would be dead 48 hours later. Not our strong and stoic Kate. I struggle with the worry that maybe more could have been done, and then remember the gift of getting her home, the shock of realizing she was dying, and supporting her in the last couple of days of her life.

Her death is a tragedy. It was not expected and it was not supposed to happen. I am still searching for the answers as to why.

She and I were a team. The last 8 years were an incredible roller coaster of love and determination. As much as I was her champion and supported her, she defined me, strengthened me, and helped me become the best version of myself. How do you lose and move on from a force like that in your life?

This is what 2016 will be for me. Learning how to move on, and if I can’t learn – then simply learning how to cope.

 

She was an incredible child. My own daughter.

8 years was not enough time with her. That is my biggest regret.

 

Julie

 

I invite you to watch Kate’s Celebration of Life Memorial from November 30th, 2015. 

 

A Month of Anniversaries

I wanted to share this blog post this past Saturday. It was the actual 4th anniversary of being told that Kate was diagnosed with SIFD. (Things have been a bit busy since Saturday).

November is also notable for Kate being diagnosed with hearing loss – and needing hearing aids, followed by her diagnosis 2 years later as being completely deaf. November carries a lot of emotional weight for me.

But this is about SIFD…

Kate suffered for over 4 years with an undiagnosed disease that caused multiple medical issues, and medical fragility. In the midst of an incredible diagnostic odyssey that led us to visit four different major hospitals in Canada and the US, and have Kate’s blood and tissue flown around the world, in the fall of 2011, our family (Jack included) did a simple blood test as part of FORGE (Finding of Rare Diseases in Canada), a genomics project led by Dr.Kim Boycott. The purpose of FORGE was to examine undiagnosed children suffering from rare diseases and see if they could identify the disease through national collaboration of physicians, scientists and researchers. In our case, our metabolics doctor, Pranesh Chakraborty, went one step further and collaborated internationally with a team from Boston to identify the TRNT1 gene that causes SIFD.

I’ve always said that I was waiting for the huge SIFD announcement. The national or international recognition of an ultra-rare – and devastating – disease to have been identified. But the sharing of the SIFD diagnosis for the first child ever, happened in a small consultation room at the Children’s Hospital of Eastern Ontario with two parents, their trusted physician, and his notes. It was a simple conversation, both Brian and I had ‘donated’ the same shitty gene to Kate and the result was SIFD. Our children had a 1 in 4 chance of inheriting this previously unknown disease.

Genetics is helping to identify these diseases more and more, and a new/novel disease discovery just isn’t news worthy any more. I can tell you that for the 7 families with children living with SIFD, and the 20+ others whose children have died from SIFD, it is huge and the anniversary of knowing is very significant, so we ‘celebrate’ the discovery of SIFD with quiet and personal reflection about that day and that conversation in the tiny consultation room with our metabolics doctor.

I remember the moment we found out about SIFD very well. It is one of those emotional memories that you can physically feel as you recall it. I thought I would feel so differently. I thought knowing what was ‘wrong’ with Kate would change everything. That I would be maybe elated or excited that we finally had an answer. Instead, I felt empty and numb – and came to the slow realization that there was monumental mountain of the unknown facing Kate and our family, and that we still really had no answers to help her. Nothing had really changed.

So here we are on the 4th anniversary of the discovery of SIFD.  CHEO released this little blurb a few weeks ago about it.

Teamwork solves the riddle of SIFD

If it takes a village to raise a child, in research, it takes team collaboration. Teamwork and new perspectives can rocket discoveries forward and help make incredible progress. At the CHEO, we see progress every day that directly benefits our patients.

Dr. Pranesh Chakraborty, a metabolic physician and Director of Newborn Screening Ontario, and his team partnered with clinicians and researchers at CHEO, to determine that mutations in a specific gene were likely responsible for causing SIFD (sideroblastic anemia, immunodeficiency, fever and developmental delay) in one of the young patients at CHEO.

Dr. Chakraborty’s lab, with the help of Dr. Martin Holcik’s Molecular Biomedicine lab, was able to rapidly kick-start the needed research – something neither could have done alone.

As one team, they were successful in their quest. And in 2014 they proved their hypothesis that the cause of SIFD is mutations in a specific gene. Their success came from teamwork not just across CHEO, but across borders. The CHEO team joined forces with researchers in Boston and clinicians around the world to make this discovery.

Like modern-day Sherlock Holmes, these researchers are medical detectives examining the clues in our genes to identify those which cause rare diseases. This kind of teamwork embodies CHEO’s values, and allows doctor and researchers to expand the field of medicine, and in particular rare disease research, at the pace they do.

 

Here is what I would add to this short article:

“And thank you to Kate Drury, a brave little girl, who donated her own genetic material so that this discovery could be made. A little girl carrying the weight of a genetic discovery on her shoulders. A little girl whose family never gave up to find a diagnosis for her. One of only 7 children alive with SIFD in the world today and the only Canadian alive with SIFD.”

 

Julie

“How To Be A Good Guest”

NOTE: We have been home for just over 2 weeks from a close to 7 month hospitalization. This blog post has been sitting in my ‘draft’ since late August. It’s interesting now to read it and reflect on our experience of such a short time ago. I want to say that our family is very grateful to the excellent medical professionals who have taken care of Kate. This is unedited and unrevised. A raw reflection of hospital living.

 

Living in the hospital with your sick child is not something I would wish on my worst enemy.  It is a sad and lonely existence.  It is stressful, exhausting and scary.

You lose all control over your life in hospital: personal space, likes/dislikes, routine, and decision making. You are reminded almost daily that you are not in control, that your autonomy as a parent is no longer. That discussions and decisions about your child, you, and your family will often happen without your presence or input.

And you have no choice. Your child is sick. You need to live this life, sleeping on a small cot, being woken throughout the night because of alarms or lights, physically and emotionally exhausted, separated from home – family and friends, eating what and when you can, limited from going outside or fresh air, living with incredible stress among strangers, all while dealing with incredible stress and heartache.

And you always need to remember to be a gracious guest. Smile. Say Thank You (a lot). Guard your emotions. Accommodate any interruptions of medical staff to your room. Repeat your child’s story endlessly and readily. Expect to meet an endless stream of medical personnel. Try to greet everyone by their title – despite the fact that for months on end they will simply call you ‘mom’. Expect no routine. Be agreeable with waiting all day for 5 minutes with a doctor. And be vigilant about keeping you child safe.

 

Be. A. Gracious. Guest. 

 

Check your emotions at the door – AT ALL TIMES. An almost impossible task given the circumstances you are living, but a clear expectation by those whose ‘house’ you are visiting.

If you are lucky, you’ll work with a medical team who are compassionate, patient, communicative, flexible and transparent. Doctors and nurses who understand your journey and the stress that comes along with it. Professionals who aren’t jaded by working with endless little patients and endless upset and frustrated parents.

Let me warn you. This type of environment and medical team cannot be an expectationWe have been lucky for the most part.

Hospital life is a constant delicate balance about living out a working relationship that includes respect and civility, but in an environment that is created and controlled solely by the medical professionals, and for the parent, under situations of extreme personal stress.

 

House Guest Rules:

  1.  Be Clear About How Long You Will Stay

I don’t want to be here. I would prefer not to be ‘visiting’. I’m sorry, but we have no idea how long we will be staying. I am not in control.

2. No Surprises Please

After living 8 years with a child who suffers from an ultra-rare disease, ‘no surprises’ has definitely been taken off the table. We will surprise you daily. 

3. Choose The Perfect Gift

I will bring you coffee, tea, Timbits, a fruit basket, cookies for the night shift. We are grateful for all that you do for us.

4. House Rules Rules

We will do our best to adjust to your ‘rules’, but we would appreciate some flexibility and compassion about the fact that we are reluctant guests and that some ‘rules’  are not to the benefit of our child. But again, I understand…I am not in control

5. Be Appropriate

I assure you, I am a calm, rationale and appropriate human being 99.9% of the time. I hope you understand that this is an incredibly challenging and difficult time for our family, and I am doing my best to be appropriate. If I am not at all times, I hope you are empathetic and able to be there to support me.

6. Help Out

I will do everything I can to help out. Changing beds, managing my child, helping with meds, holding her down for procedures, interpreting for you and for her etc.

7. Entertain Yourself

Done. An endless supply of stickers, play dough, colouring, puzzles, dolls, toys, crafts to play with. I’ve updated my Netflix subscription as well, and found a few magazines and books to flip through.

8. BYO

If only! An occasional glass of shiraz in a beautiful wine glass shared with a good friend would be so amazing right now.

9. Leave No Trace

Keep our room neat and tidy. Check. We’ll likely leave a trace with you though. Kate is just too adorable to forget. 

10. Give Thanks

We are both grateful and we say thank you every single day.

 

As a patient-mom, I have lived more than my fair share of out-patient, in-patient, short and long term hospitalizations. I understand the medical system and I know how to navigate it – most of the time (it can be difficult and confusing still).

I am a good person, a good mom, and excellent patient advocate. My goal is to keep my daughter safe and well cared for, and I have clear expectations about how that is to be done. It doesn’t always fit with the ‘house rules’ of the medical professional I am working with. So I go back to my “etiquette” and do my best to get done what needs to be done in a collaborative and professional manner. Often times I am relied upon by these same professionals to help them understand my daughter better because of the complexity of her condition. But I am a mom. And I get tired and emotional and scared – and I am not always going to have the patience to say everything in my nice voice. I am not always the perfect “guest”, but I think I come pretty darn close. It comes with working within the imperfect environment of a hospital, and I think needs to be appreciated and understood by everyone involved in the care of a sick child.

 

Julie

 

 

 

 

A Few Thoughts About Patience

I am tired of being told to be patient.

I’m pretty sure if I hear the word one more time, being directed at me in a syrupy semi-supportive/patronizing tone with a hint of caution from a person who knows little/nothing about me/Kate/or our story, I might just lose it on the next person I hear it from.

It’s been 5 months, and no one can seem to figure out how to get this GVHD under control. We’ve dealt with an 8 week BMT, a very poorly orchestrated discharge post-BMT, missed diagnosis, extremely stressful medical situations where we went unheard, mistakes were made, and Kate suffered as a result, and now the yo-yo back and forth two steps forward 5 steps back (and three steps sideways) of trying to control for a complication that wasn’t supposed to happen with no end in sight and a changing roster of people in charge of finding the solution to fixing it.

I am tired of being told to be patient. 

I do not feel patient and yet I wake every morning and focus on what needs to get done that day. What do we need to do to get Kate one step closer to home. What craft game story video iPad game sticker activity can we do that day together where I will need to dig deep to be engaged and patient within the four walls of our isolation room. What questions should I be asking? What plan can we make to get Kate better?

I am patient. I have been patient. I am patient because I have no other choice. And neither does Kate.

 

Being told to be patient and that ‘these things can take time’ is like telling a marathon runner that you have moved the finish line. Actually, not moved it, but hidden it and there is no MapQuest, or GPS, or map or even a damn sextant to find the finish line. (You even begin to wonder if the damn finish line even exists). Just keep running until you find it. Oh…and be patient. And that exhaustion and despair and frustration you feel? Just be patient. We have been so close to that finish line too many times.

Who ever coined the phrase ‘good things come to those who wait’ should be taken to an isolation room with an unwell child and locked in…and told to be patient.

pa·tience
/ˈpāSHəns/
noun
1.  the capacity to accept or tolerate delay, trouble, or suffering without getting angry or upset.

 

As of today , it has been 5 months. Kate is up and down. Feeling well for a few days and a glimmer of hope starts to grow that maybe, just maybe our patience has finally borne some fruit – only to be dashed again by the latest set back of loose/liquid green stool, nausea and fatigue. (You know that marathon finish line I mentioned? Think about having it in your sights…finally…only to have someone steal it away and move it again.)

We’ve been patient through the post-BMT phase with trialing 4 different immune suppressive drugs. Five attempts at steroid wean. Endless imaging and tests. Different doctors with different plans. Different nurses with different approaches. And not much has changed.

Kate has been patient.

Everyday is a test of patience amidst our exhaustion and fear and stress and hope for a good outcome.

Patience is wearing thin for both of us.

 

Julie

 

The Best Of….

In an effort to take this prolonged hospitalization day by day and moment by moment, I am working on reminding myself of the positives and good moments. Don’t get me wrong, the negatives and the dark times are not few and far between here, but for mental health purposes and trying to ‘live for the moment’ (ugh), here are the Ste.Justine, Block 12, highlights:

 

10. We have a big room. 

Totally important when you are living within four wall, in isolation, for a prolonged period of time and you have a child with special needs to keep happy and engaged. We have a room where the parent bed is ‘built in’, a move up from our fold out cot and DEFINITELY better than the chair beds we’ve experienced in the past. Kate has space to move around and play and be active. She has a large bathroom, with an appropriate tub and shower and enough room to fit two adults needed to help her shower, and her enormous IV pole. And windows. Big bright windows that she can look out (though the view is never guaranteed).

9. We are learning/improving our French.

Well, I was actually already bilingual. Level E (exempt from requiring further testing) as assessed by our federal government who are very keen to spend a LOT of $$ on making sure everyone in the Public Service is fluently bilingual and limiting the careers of those who are not. Wait…that’s another rant for another blog/site…sorry.
Ok, back to French Immersion Camp Ste.Justine. When we first arrived, I have to admit I was intimidated to use my french language skills. They were ‘rusty’ and I didn’t feel I could express myself the way I needed to, missing key medical vocabulary to communicate with the nurses and medical staff. The medical team was more than willing to work at functioning in English, but I soon realized that the nurses were more comfortable in French and I was going to get a lot more from them if I asked them to speak French to me. The information coming from them was more important than me worrying about my masculine/feminin pronouns etc.
I think I’ve gotten to a point where my French is now pretty seamless. A few members of the medical team have even complimented me on my French (merci!). There is really no ‘downtime’ from French here as most of the families and patients are French as well. If you want to talk to anyone and not go crazy from isolation – you need to parlez le francais. I’ve even participated as a ‘translator’ between French and English families in the parent kitchen as we all talked about our kids.
Kate has even started to pick up some French and is saying “merci” to her nurses and doctors. Yes, the developmentally challenged, Deaf child, is now trilingual. Ha.

8. The nurses here are amazing. 

They know bone marrow transplant, and they know post bone marrow transplant care and complications. They are helpful, insightful, willing to listen, willing to support, and they are above all sympathetic. I know that they know BMT well. They understand TPN and use it frequently. They know the complicated medications these kids are on and they know that when a parent is worried, there is a very good reason for that worry. They are intimate members of Kate’s care team and are briefed extensively on her history, complexity and the trajectory  of her BMT recovery to date. They often refer to her as “plein de surprises” (full of surprises) and because they are using the same language – I know it is a context about Kate that the BMT team is emphasizing so that they take nothing for granted with Kate and recognize that she is different and doesn’t follow the ‘typical’ clinical presentation.
It makes me feel safe. It makes me feel confident. It allows me to trust and to actually feel like I can close my eyes at night.

And that is huge.

The BMT team here at Ste.Justine has taken great care in caring for Kate. When nursing rotations are organized, they work hard to keep Kate with the same nurses to allow for consistency for Kate and facilitate communication. Kate has unique challenges with language, using a mix of ASL and spoken english to communicate, but the more you know her, the more you understand her. Ste.Justine has made that a priority in her care.

Again. I am grateful for that attention to detail to better support my child.

7. Patience.

Kate’s BMT team are a core of 4 physicians. We work most closely with two of them, Dr.B and Dr.D. This is actually how Kate refers to them because she can’t pronounce their full surnames. Dr.B she’s even reduced to simply calling “B”. (Yes, it’s adorable and he loves it).

They are wonderful with Kate. Patient, engaging, cautious, willing to learn and follow her lead. They don’t rush her with examinations, but flex to what she is willing to do. Nothing is forced, and they are deferential to her whenever possible.

Maybe it’s more than patience. It’s Respect.

It warms my heart to see them attempt ASL with Kate. Everyone is signing “see you later alligator” with Kate. It’s the common goodbye as they leave her room. Dr.D (with a smile on his face) even mentioned that he signed it to a colleague (who also knew Kate) at a recent medical conference here in Montreal.

It’s the small things, but it’s doctors who I can see are invested in my daughter. Who want the best for her and will be at her bedside at a moments notice to help her.

6. FaceTime

Technology has been a huge crutch for us here. iPad games are endless, and watching YouTube videos of Dora, The Voice, Curious George, ASL songs etc. are heavily relied upon by anyone who is taking care of Kate.

What has been very significant for Kate is being able to stay in touch with family and friends over FaceTime. She can say good-night to her dad and brother, catch up with her aunts, uncles and cousins from Newfoundland, Halifax, Saskatchewan and Ontario. She can dance the can-can with anyone who will take on the challenge, sing Happy Birthday endlessly with anyone willing, do the hokey-pokey with her buddy “big Jimmy”, or sneak some FaceTime with friends who rent out boardrooms at work for that specific purpose.

One of my favourite FaceTime moments is when she gets to chat with her friends at Rogers House. Rogers House has been a special place for Kate and a huge support to our family. The kids in respite there are Kate’s friends and the nurses, social workers, play therapist and doctors are more than professionals working to support her – they are genuine in their enthusiasm and excitement when they get to chat with Kate.

5. Netflix

Despite loving books and being a voracious reader….OMG….whoever invented Netflix…thank you! I have consumed more shows than I care to name and average 2-3 movies a week. It’s what you do when you are in an isolation room and can’t leave your child who goes to bed at 7 p.m.

It’s also what you do when you have energy for nothing else.

4. The other families here

When we first arrived here much of our stress was quite elevated. Everything was so new and felt so complicated, not to mention the many unknowns of our child going through a bone marrow transplant.

The third floor of the hematology oncology wing of the hospital is kept for a very specific group of families. There are a maximum of 6 families at a time and most times there are only 3-4 of us. We eat, sleep, ‘lounge’, and do everything else in close quarters with one another, and so we talk.

We share stories about our kids…”what does your kid have”
We share what we have learned, how to understand tests, bloodwork, complications and what questions to ask the doctors and nurses.
We share our worries and concerns.

We come from varied backgrounds and speak many different languages. Being with these families is one of the few times where I felt truly understand and where the rarity of Kate’s disease did not stand out.

3. Kate’s BMT was successful

Despite the many complications we have had post BMT, for all intents and purposes Kate’s BMT is a success so far. Her blood counts are stable and almost normal. Her lymphocytes are recovering. Her chimerism tests are solid. She has not rejected the new marrow. And she is still here.

2. Our evening walks

Several weeks ago, after we had arrived back to Ste.Justine having been transferred from CHEO, Kate was still in isolation in her room and only allowed to take brief walks on the third floor. Kate was still very unwell with lots of ups and downs and I was worried about her ability to cope and keep up her incredible stamina long term. I insisted that Kate be allowed outside for daily walks. Her team agreed and were willing to make this happen – not an easy thing to do because it means disconnecting her from her TPN feeds, IV hydration, IV medication etc. and flushing/heparinizing her lines and installing all new lines when we returned. But it is 2 hours of incredible freedom for Kate. And it is beautiful.

The first time we went for one of these walks, Kate hugged a tree. She climb onto someone’s lawn, sat for a moment and looked at the surrounding garden, then got up and walked over to a lone ornamental tree and hugged it.

Sometimes your instincts as a mom are bang-on.

Now our walk is a ritual. Sometimes an opportunity for Kate to have a  much needed nap, other times she simply watches the people and cars while humming a little tune to herself.  We’ve passed by quiet playgrounds and took a chance to swing or go down a slide, and we walk through lovely parks enjoying the shade and the view.

Huge. Huge for this little girl who hasn’t had the chance at a any of this for 4 long months.

1. Happy Incredible Amazing Kate

And then there is Kate.

What can I say? She is Incredible.

I work hard at keeping up beat, but my energy is waning and I want to go home. I am positive and I am hopeful, but I don’t feel much like laughing or talking or being silly most days.

But Kate does. So I do it for her.

She loves everyone she meets and wants to be their friend. She develops relationships and bonds with her nurses and doctors on her own – with little guidance from us. She patrols the elevator and has made the short hallways of the third floor her play space. Her eyes shine bright with love and trust and a willingness to endure.

Though I know she longs for it as much as I do, with tears in her eyes she has only asked for home twice.

She is Incredible.

 

 

Julie