Posted by: searchingforsolidfooting | February 3, 2016

THIS.

I have been wanting to write. There are so many thoughts that come to mind, but the moment never seems right and the thoughts are too intimate.

It’s been 9 weeks and 2 days. I read this yesterday, it was written by Angela Miller:

 

Dear Newly Bereaved Parent

This will likely be the hardest thing you’ll ever do. Survive this. And eventually, maybe even thrive again.

At times it will feel virtually impossible. You’ll wonder how a human being can survive such pain. You’ll learn you know how to defy the impossible. You did it from the moment your child’s heart stopped, and yours kept beating. You do it with every breath and step you take. You’re doing it now. And now. And now.

Your fingernails will become bloodied from clawing your way from the depths of despair. Your spirit will grow weary from fighting to survive. Your eyes will cry more tears than you ever thought possible. Your arms will ache an ache for which there aren’t words. For a lifetime.

Your heart will break into a million tiny pieces. You’ll wonder how it will ever mend again.

But with every morsel of unspeakable pain, there is love. An abundance of love. A love so strong, so powerful, it will buoy you. You will not drown.

Others will say things that are intended to be helpful, but aren’t. Take what is, leave what isn’t.

Still, you’ll meet others along the journey who will get it without ever saying a word. Kind souls who will breathe you back to life again. Let them.

Years down the road you’ll tire of hearing the same advice and clichés, over and over again. Advice you don’t want or need. Everyone will try to tell you how to best “fix” your broken heart. The trouble is, you don’t need fixing.

There is no fix for this.

Eventually you’ll learn how to carry the weight of this pain. At times it will crush you. At other times you’ll learn how to shoulder the burden with newfound grit and grace. Either way, you’ll learn how to bend with the weight of it.

It will not break you. Not entirely.

And even if you don’t believe in hope– not even a little– hope will light the way for you. At times you won’t realize your path is lit. The darkness feels all consuming when you’re in it. But know the light is there. Surrounding you now. And now. And now.

Know you’re being guided, by all of us who have survived this impossible hell. You may not hear us, or see us, but we are with you. Beside you. Hand in hand, heart to heart. Always. Just like your child still is.

Above all else, know that no one can save you but yourself. You are the heroine/hero of this sad story. You are the one who gets to decide how, and if, you’ll survive this. You are the one who will figure out a way to survive the sleepless nights, and the endless days. You are the one who will decide if and when you’ll find a purpose again that means something to you. You are the one who will choose how you’ll live with the pain. You are the one who will decide what you’ll to cling to, what will make your life worth living again. You, and only you, get to decide how you’ll survive.

No one else can do this for you.

People will speak of “closure,” of “moving on,” of “getting over it,” of grief coming to an end. Smile kindly, and know, anyone who says these things hasn’t lived this thing called grief.

To lose a child is to lose the very heart and soul of you. It is overwhelmingly disorienting. It takes a long, long time to find yourself again. It takes a long time to grow new life around the chasm of such grave loss. It takes a long time to grow beauty from ashes.

There will always be a hole in your heart, the size and shape of your child. Your child is absolutely irreplaceable. Nothing will fill the void your child left. But your heart will grow bigger– beautifully bigger– around the empty space your child left behind.

The love and pain you carry for your precious child will be woven into every thread of your being. It will fuel you to do things you never dreamed you could do.

Eventually, you’ll figure out how to live for both of you. It will be beautiful, and it will be hard.

But, the love you two share will carry you through. You will spread this love everywhere you go.

Eventually, you’ll be able to see again. Eventually, you’ll find your way again. Eventually, you’ll realize– you survived.

 

Posted by: searchingforsolidfooting | December 31, 2015

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. 

 

Posted by: searchingforsolidfooting | December 14, 2015

Kate Drury – A Celebration of Life

 

In tribute to Kate – her Celebration of Life eulogy, December 12th, 2015.

 

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Thank you all for being here this afternoon to celebrate Kate. The love and support for our daughter over the past several years, and especially these past 2 weeks, has been incredible and it is a true testament to who she was, what she shared with her community, and the impact she has had on the world.

Brian, Jack and I are honoured that you are here to share Kate with us today.

Kate did not have an easy life. She suffered from an ultra-rare form of mitochondrial disease called SIFD – sideroblastic anemia, immune deficiency, periodic fever, and developmental delay. But we didn’t know this in the early days. When Kate was a baby – we simply knew she was unwell – and as her mom, I knew early on that something was wrong.

Kate’s development was behind, she was not doing the things other babies her age should be doing. She couldn’t hear me and wasn’t responding to sound or even to my voice. And then she started getting sick. We came to call these periods of sudden acute illness as her “episodes” and in the past 8 years she had more than 40 of these episodes that were acute enough to require hospitalization or day visits for intervention at CHEO.
I became not just Kate’s mom, but her medical advocate, her medical coordinator, her nurse, her caregiver, her pharmacist, and her therapist. We were determined to find out what was wrong with her and help her, but Brian and I were also focussed on supporting her to be a little girl and to have a full life of all the things a little girl should be part of.

Kate and I spent far too much time in hospitals. Kate regularly visited more than 15 different outpatient clinics at CHEO, as well as diagnostic imaging, day surgery, and the bloodwork lab (of course).
She was a fixture there and everyone knew who she was. She was the happy little girl, who ran the hallways of CHEO. She loved heading to the cafeteria to buy her french fries and to visit her friend Linda, or to the snack shop to get her bag of chips – and pay the lady at the cash on her own like a big girl. Everyone knew Kate, and she had a smile and giggle for everyone.

She spent time on 4East, 4 West, 5East and 4 North as an inpatient. Basically every inpatient unit of the hospital.She was invited to participate in the CHEO Telethon as a featured patient story in 2012, and it was an honour for our family to share her story.

CHEO became a second home for us – and it felt like family there. We definitely had very difficult times, but in 2009 when the Complex Care Program pilot began – we finally found a safe haven for Kate amidst the chaos of so many medical specialists, emergency room visits and admissions. We are forever grateful to Kate’s complex care team, Dr.Natalie Major and our nurse Christine who took on the role of navigating Kate through her episodes and supporting her complex medical needs. They have been part of Kate’s life for close to 6 years and their devotion to Kate and willingness to listen and work in close collaboration with us saved Kate’s life more than once.

Although Kate’s life was very medically focussed, she didn’t live that way. Vanessa Waters, Itinerant Deaf Teacher, recently wrote this post on Facebook:

“Kate didn’t choose many things in her life. She certainly did not choose her disease and all that came with it. She did, however, choose how she would spend her life. She had a choice in that and she chose well.”

Kate chose to be happy and joyful every day. Even when she felt unwell it was sometimes a challenge to know how sick or in how much pain she really was. Kate didn’t feel sorry for herself, she wasn’t sad…she was brave, resilient and happy.

Many people would comment that “Kate didn’t look sick”, and only those who knew her really well could pick up on the subtle ‘tell tale’ signs that something wasn’t quite right that day. Kate wouldn’t talk about it, or let us know directly.

Many of her trusted therapists, teachers and caregivers spent years helping Kate to develop her language – as a deaf child, we taught Kate to use American Sign Language, and with her cochlear implants (or her “ears” as she called them), we also worked on teaching Kate to listen and to speak.

A skill we all worked on consistently was trying to give her the words she needed to tell us or her doctors ‘where it hurts’, but she wasn’t interested in learning that. Instead she learned to talk about the things she loved – like swimming, gymnastics, school, her friends Ani, Lucy, Myah, and Matthew, going for walks to the park, drives in her van, going to ‘the House’ (as she famously called Rogers House), her favourite songs, and her brother “Jackie”.

Kate had a rich life – last winter she learned to ski – which made me incredibly proud as I realized I am not cut out to be a dance mom. She loved to go tubing behind the boat with her friends – and was known to throw herself off of the tube on more than one occasion, bobbing in the lake while we turned the boat around to pick her up.

She spent a lot of time at the Ottawa Gymnastics Centre from a young age and was adored by her coaches and staff there. I’ll never forget watching her jump on the trampoline last fall, and sending her off to summer gymnastics camp, and thinking back to the days when we wondered if she would ever be able to walk.

Kate loved the water. We spent many afternoons at the JCC, our neighbourhood pool. Kate would walk straight to the edge and jump in – often into the deep end – fearless. She was so confident in herself that she would swim back to the surface – often she swam down instead of up. We never discouraged her, but instead followed her lead. I think she gave more than one lifeguard or swimming instructor a heart attack as she jumped in and sank, waiting for someone to pull her back to the surface by the straps of her bathing suit. Jumping off docks, jumping off diving boards, careening down waterslides…and even cliff jumping (or being thrown off the cliff). Kate embraced it all. If the other kids were doing it, she wanted to be doing it to. She just needed a little support to get there.

Kate was so proud to attend school and loved being with the other kids. We are so grateful for the support from her days at Westboro Preschool; her first day at school at Broadview where she strode into the school yard with her big brother Jack; and her short year in the Deaf and Hard of Hearing Program at Vincent Massey Public School.

School days were short for Kate, as her body was often exhausted. She would come home after her half day and often be asleep in the van when she was dropped off. Many times, I would be called to the school to pick Kate up – or I would call in the morning to let them know Kate wasn’t up for school that day.
While Kate found many skills difficult, she was keenly interested in learning and eager to be taught. She often surprised us with the skills she picked up – especially when she started to speak french while we were in Montreal these last 9 months, proudly signing ‘thank you’ to her doctors and nurses and then repeating ‘merci’ in french.

Kate loved many things: Dora, Curious George, princesses – especially Cinderella; her birthday, or “Happy Day” – which could have been everyday if it was up to Kate; singing – and sitting on her Dad’s lap in the office watching endless episodes of The Voice (…it’s a singing competition). Kate loved the grocery store, and helping to pick out tomatoes (I hate tomatoes). She loved going for rides with her dad in his truck or in her van. She loved to sit in our front window and watch the kids walk to school. She liked going to Jack’s hockey games, though she never watched and someone always had to chase her around the rink. She liked neighbourhood parties with our friends, and dancing – and she came to especially like Santa. He would have been a huge hit this year.

We often said that people underestimated Kate – what she understood, what she was trying to express, and what she was capable of – but Kate never underestimated herself.

Kate’s brother Jack was and still is an amazing big brother. Jack grew up with an unwell sister – he didn’t know anything different. There were many demands placed on him over the past 8 years, and the last 9 months have been very difficult. But he has never been frustrated or resentful of Kate. He has supported her in anything she has done, and has always been by her side. One of my favourite memories is of Jack hauling his sister on her toboggan around the field across the street. Encouraged by her giggles and requests of “more – more”. Pulling her behind his bike in her chariot – being gentle with the bumps. Cuddling with her on the couch, while they watched Dora’s Christmas – at anytime of the year. Teaching her fist pumps and gently ‘wrestling’ with her – and tolerating her hair pulling and biting. Jack has always been a kind and empathic boy. He said to us that after Kate passed “Kate will always be with us.”, and I know that is true – especially for him. She has left a huge imprint on his heart.

The last 10 months of Kate’s life have been incredibly challenging. She spent most of her time at the Centre Hospitalier Universitaire Ste.Justine, in Montreal in protective isolation following her bone marrow transplant. It sounds gloomy and lonely, but it really wasn’t for Kate. It was hard on our family, but Kate made her days fun. We spent endless hours making pasta out of play dough, playing ‘groceries’, endless puzzles, playing with stickers and cutting out hearts. When Kate could leave her room there were moments of playtime in the hallways in a little orange car from the playroom, or riding around on a ‘big wheel’ trike. Over the summer, we would leave the hospital for afternoon walks outdoors, exploring the surrounding neighbourhoods and parks.

Kate loved her doctors and nurses at Ste.Justine. She had name signs for her bone marrow transplant doctors and enjoyed teaching “Dr.D” and “Dr.B” and Dr.Teira and Dr.Cellot how to sign.
She was silly with her nurses and full of hugs and giggles. And they loved her back.
Kate would cheer and clap when a mini-wheelchair or gurney would arrive to pick her up for yet another x-ray, or ultra-sound…or a trip to install another PICC line. And she would wave at her ‘fans’ at the nursing station as we wheeled her past.
She matured and grew over those 9 months – and everyone remarked on how much little Kate had grown over the time she spent there. 
L’amour et le soutien de notre équipe de Ste.Justine avaient un rôle énorme dans ce que et nous serons éternellement reconnaissants pour le soin qu’ils ont pris de Kate.

Kate had many friends and champions. She had a smile and giggle for everyone and had a way of making everyone feel like they were the most important person in the world to her. She definitely had her favourites:

  • Tall-Steve who would be silly with her. Take her for impromptu walks. Let her drive the boat from his lap. And help her to ‘cliff-jump’ by hauling her up a rock face on the Big Rideau and throwing her off into the lake below.
  • Shawnee her gymnastics coach who tirelessly chased her around the gym and made Kate feel like the most accomplished gymnast by lifting her and supporting her on balance beams, bars, rings and trampolines.
  • Kara her caregiver who took Kate on endless shopping excursions and adventures – grabbing a box of french fries or chips along the way.
  • Sunita who helped her develop such a strong friendship with her daughter Anika. Who would be silly with her when her mom was tired. And who taught her how to do the ‘can-can’.
  • Stephanie who I credit with teaching Kate to say “Love You” – mostly because she wanted to hear Kate say it to her every time she left after one of her Montreal visits.
  • Her friend Kat from Rogers House who Kate adored. Kat made her feel special and loved and was ready to entertain Kate with endless crafts, salad making and big bubbles baths during her weekend stays at ‘the House’.
  • Kelly, her auditory verbal therapist at CHEO – who worked so patiently with Kate over 5 years and whom Kate adored. Acquiring language wasn’t easy for Kate. It was hard work disguised as play, and Kelly guided Kate into the world of sound, but also supported her in communicating with ASL.
  • And her ‘Grandy’ and ‘Bana’ who played the ultimate grandparent role in supporting Kate during her hospitalization, while being silly and fun and surprising her with endless “Happy Day” gifts.

And what I realize in beginning this list is that it is endless. Many of you have fond memories of Kate, and when you take a moment to reflect on them, I will bet they make you smile.

Kate loved everyone and she was loved back. Fiercely. That was her gift to others. She made people smile and brought them joy. She made everyone feel like they were her favourite person. Even in her most difficult moments, she would often be able to find a smile.

The decision to undertake a bone marrow transplant to treat Kate’s disease was not an easy one. Her brother, Jack was her perfect match, something that was rare – and Jack never hesitated to be his sisters bone marrow donor. He was brave and loved his sister unconditionally. It was an impossible decision for Brian and I, and as Brian said, the only perfect outcome would have been Kate surviving the bone marrow transplant and enjoying a healthier and longer life.

Kate’s BMT resulted in multiple complications, and though she was strong and she fought hard, in the end Kate’s body was tired. In the 6 1/2 months of her initial hospitalization in Montreal, Kate never asked for home. She accepted where she was and seemed as focused as we were on getting better. When she finally did come home in September, she amazed us all by rallying and improving – but something changed in the last few weeks. Our Kate was quieter and seemed tired. She continued to shine, but she had started to ask for home more and always wanted Brian and I close. “Come” she would say to us, even when we were standing right next to her.

We were faced with a very difficult decision when Kate’s graft versus host disease recurred again two weeks ago. We asked to bring Kate home while we tried to face yet another impossible choice – whether to undertake a very difficult treatment for the GVHD or whether to let Kate be supported palliatively. In the end, Kate decided. She took the burden of that decision from us. Her condition had quickly deteriorated and she made it clear that she wanted to come home. And we are so grateful that we could do that for her.

Kate spent her last few days supported by family and friends who loved her. She was never alone – someone always had her hand – there was someone with her to keep vigil at all times. Brian, Jack and I slept in her tiny room – crowded on the floor, and I in her bed. We nursed her, and comforted her, Brian slept with his hand clasped in hers.

And though she had incredible support at the end of her life – in the last moments it was just our perfect little family gathered together with Kate. Just the four of us – telling stories, sharing funny videos, laughing at Kate’s unforgettable belly laugh. We played “Fight Song” for her – and we cried – and we told her how much she was loved – that she would never be alone – that we would ALWAYS be with her. The terrible pain we felt in those moments was our wise Kate, taking a piece of our hearts with her. She knew we wanted to be with her always and our smart little girl took a part of us on her journey.
Kate’s life has had an enduring impact. She brought international communities of medical specialists and researchers together. The discovery of her rare disease and how it has been managed and treated has literally changed the lives for other children and families here at home and around the world.
What an incredible and beautiful legacy for a little girl.

I will miss her every day of my life. Her loss is an unimaginable pain.
I will miss her 2-arm squeezes, her “I lub you’s”, and her butterfly kisses on my cheek. I will miss her signing “good-morning”, her fine blonde hair, sparkly t-shirts, and her famous belly-laugh.

A person like Kate needs to be remembered. She has literally changed the lives of others for the better. She was the embodiment of what is right with this world and what we should all strive for; courage, strength, joy and a love for life and others before anything else.

That will be part of her legacy, and I plan to live it for her as best I can.

I am so honoured to be Kate’s mom.

 

“Goodbye my dolly”

Posted by: searchingforsolidfooting | December 7, 2015

Kate’ Celebration of Life Memorial

We are planning a memorial for our little girl. It will be a celebration of her life and we hope anyone who feels touched by Kate and her story will attend. 

 

Katherine (Kate) Drury
Celebration of Life Memorial
Sunday, December 13th at 4:00 p.m.
Ukrainian Hall, 1000 Byron Avenue, Ottawa, K2A 0J3

Please come and celebrate Kate’s life with us on Sunday, December 13th. In memory of Kate, we encourage you to wear something pink or purple.
Kate loved cutting out and pasting hearts. We would love it if you would bring a heart with you that you can leave behind after the memorial service.

Thank you,
Brian, Julie and Jack

 

Posted by: searchingforsolidfooting | December 5, 2015

Kate

I am sitting here this evening reflecting on an incredible outpouring of love, support and inspiration over the past 4 days from friends, family, neighbours, physicians, nurses, volunteers, teachers, therapists, coordinators who were a part of Kate’s life.

Kate died November 30th. She was 8 years old.

There are no words to express how I feel right now. Just raw emotion and a gut wrenching pain that no mom should ever experience. My daughter did not deserve this end. She deserved to live. She was a fighter, but she was also pure joy and light. Something this world desperately needs.

In the days to come you will learn more about Kate, because I will continue to write about her and celebrate her. We will have a public memorial on December 13th to celebrate her life and we are welcoming all to attend. (Details to come).

A person like Kate needs to be remembered. She literally changed the lives of others for the better. She was the embodiment of what is right with this world and what we should all strive for. That will be part of her legacy, and I plan to live it for her as best I can.

 

Kate’s Mom

Posted by: searchingforsolidfooting | November 30, 2015

Kate

Kate came home Saturday from Ste.Justine. She had been battling a recurrence of her graft versus host disease (GVHD) and we had a case conference on Friday with CHEO and Montreal to discuss possible next steps.

On Friday afternoon, Kate had a significant deterioration. She was kept comfortable by her Montreal team, and on Saturday morning it was determined that Kate would come home and be cared for her by palliative care team from Rogers House.

Kate was awake and alert when we arrived home. She saw her Christmas tree, she had time with her grandparents and with her brother. Her pain was quickly managed and upon getting that relief, Kate went into a deep sleep. She has not woken since Saturday evening and friends and family are with us holding vigil and helping us to say goodbye to her.

We love her and we are doing all we can to support her and let her know how much all of you love her. Thank you for all the words of encouragement, support, and prayers over the past few months.
Julie, Brian, Jack and Kate

 

 

Posted by: searchingforsolidfooting | November 9, 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

Posted by: searchingforsolidfooting | October 20, 2015

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I haven’t written a post specific to Kate in quite some time. I think the last one was when we had it hit day 110 and had already experienced many serious complications post bone marrow transplant. As I reflect back on that post, I can feel the raw emotion of those days. The exhaustion, fear, and anxiety. The crushing guilt of having made the wrong choice for Kate. Not knowing what the next week, or month, or her future would hold. Not knowing if she would survive, or come out of this entire mess intact.

And here we are, 7 months post bone marrow transplant, 219 days. I’m not sure much has changed. Except…

Kate is home.

She is not well, in fact she is still very sick. I think many people understand that when you are home from hospital you must be ‘well’ and ‘recovered’. Kate is neither. She is home because we fought hard to get her here. We saw her wasting away in a hospital bed day after day, and we saw no significant changes in her care or treatment, only unending complications. Any child in that environment would not thrive or survive. We could see the ‘shift’ happening in Kate – quieter, less enthusiastic, disinterest in getting out of bed, sadness and tears when her dad or brother visited and then left for home again. She had become accustomed to her little world of four walls and seemed resigned if not accepting that this was her world. She still had her smile and he chuckles, but a shift had happened.

 

The Shift

We received very difficult news about Kate in late August. Terrifying news. And we made a decision at that time that Kate would come home. As a child who required total parental nutrition (TPN), this was complicated. One of us had to be trained to manage her PICC (peripherally inserted central catheter), and the obvious choice was me because I was in Montreal with Kate, and most at ease with her medical equipment. Getting trained was another matter – we had been asking about ‘home TPN’ since July. We had been told that ‘yes, this was possible’, however, jurisdictional issues came into play as Kate was an Ontario patient in a Quebec hospital. It was not clear who would deliver the training I required and how they would address the issue of different equipment and set up. So we were delayed – again and again. Eventually it was our bone marrow transplant team at Ste.Justine who stepped up and provided ad hoc training during breaks in our daily hospital life. The home care team in charge of TPN training at Ste.Justine refused to assist us, or train me because of the jurisdictional issues, and Ontario would not let Kate come home unless I was trained. Our home hospital in Ontario does not do TPN training and we were given the option of transferring Kate to Sick Kids (who co-ordinate all TPN for children in Ontario) where we would have 2 weeks of intensive TPN training. I obviously said no to this as transferring Kate to another hospital was clearly not a safe option.
So, bedside training it was…sneaking away to a room to learn about pumps…reviewing heparinizing her PICC…antiseptic protocols…accessing the TPN to add some specific vitamins for Kate etc. It was a crash course and it was perfect (Thank you Karine and Martine!).

With TPN training ‘done’, now we had to sort out all of Kate’s complications, determine which medical team and specialist was the lead for what, outline an emergency care plan, outline a general care coordination plan, and have a comprehensive discharge planning meeting to discuss concerns or questions. Brian and I split that discharge planning meeting between us. He was in Montreal with our team of doctors and specialist there, while I attended the meeting in Ottawa with our team there. It was a typical multi-D (multi-disciplinary meeting) about Kate that we have done many may  times before, with about 20 people in attendance. After that meeting, a plan was beginning to come together.

 

Coming Home Would Be Complicated…

Kate’s bone marrow transplant was supposed to require 6-8 weeks in hospital. This would be followed by  a 6 month recovery in protective isolation at home, while her immune suppressive drugs were slowly weaned as her new immune system started to take form and recover. She had a perfect sibling match and was therefore at very low risk for unforeseen complications. She would be back to her life better and ‘healthier’ in 8 months to a year.

This was not the case for Kate.

Kate developed a serious condition post-bone marrow transplant called GVHD – graft versus host disease. Her GVHD recurred 5 times between April and August, and became dependant on steroids. This means even with careful reduction of her steroids, at a certain threshold of dosing – the GVHD would flare again. We also tried several types of immune suppressive drugs over the summer – hoping that we might find one that would be better at controlling the GVHD. Eventually, in August, the team at Ste.Justine took a very aggressive approach to the GVHD that was attacking and destroying Kate’s gastrointestinal system. By this time, Kate had not eaten orally with any kind of regularity, since mid-March. We had attempted several times to kick start her intestines with ‘trophic’ feeding through her NG tube of supplemental nutrition, and we would get to a certain point only to slip back into diarrhoea, nausea and vomiting. The symptoms of GVHD. We had at least 3 different discharge dates over the summer, that we would reach within days, only to slip back into the GVHD cycle. Then throw in a couple of courses of antibiotics for suspected infections, and a true blood borne infection that ‘stuck’ itself to her PICC line – requiring intensive antibiotics and the removal of her life sustaining PICC for 10 hours.

We were stuck and we felt like we would never get Kate well enough to leave the hospital.

Physically we could see Kate deteriorating further because of the prolonged time confined to a hospital room where she spent most of her day in bed. Kate wasn’t allowed to play in the hallways with the other kids, or go to the playroom, because of protective isolation – keeping her safe from others because of her immune suppressed state. She was allowed to roam the hallway on the third floor transplant unit – alone, but she tired of that trip pretty quickly.
Kate had also developed a tremor which we believed was from her medication. The tremor affects everything Kate tries to do independently – drink, dress, walk, play. The only time she gets a reprieve from it is when she is asleep and her body finally relaxes and does not shake. Muscle biopsies and scans did not show a specific cause for the tremor, but did now significant atrophy of her muscles and poor mitochondrial functioning (poor energy supply to her muscles). Combined with her significant weight gain from steroids, Kate was walking less and less.

In August, when we received very difficult news. I was home for a short weekend with Jack, maybe my fourth of the summer. Brief breaks where I could sleep in my own bed, change out my clothes from Montreal, enjoy the quiet of my home, and spend some time with Jack.
On that weekend, I received a call Friday morning from Brian and Kate’s doctors. I was asked to come back as soon as possible. I took Jack with me.

Kate had developed a very serious and unforeseen condition. We left our children with the nurses, and went to a meeting room with her lead BMT doctor and Kate’s nurse. We were given the details about what they had found after some diagnostic testing. We were told the condition Kate had developed was serious, and that given what they knew about her disease, she would likely die from it. We tried to be smart, to ask questions, to try and find the loop-hole or the error they had made in coming to this conclusion. Our doctor has kind eyes, and they are always full of hope. When I looked at him that day, I saw sadness – profound sadness for us and for Kate. In that moment I felt bad for him – that he had to take on this task. I asked him how many times did he have this conversation with parents?

He said, “too many times”.
I asked what we should do? What do we tell Kate? Jack?
“Kate is already showing you what you need to do” – he said. “You just need to follow her lead, she is going to show you the way”. “Just love her and follow her lead.”

Such powerful words. And true words.

And that was when we told the team we were taking Kate home. And they agreed.

 

And Now…

Obviously the epilogue to that moment is that Kate is doing much better. The condition she had developed stabilized to everyone’s surprise. It has not been ‘fixed’, but she is living with it and it is monitored regularly. We spent that weekend with our kids, loving them and playing with them. Taking them on picnics and taking pictures. We laughed and in the next moment we cried. Our hearts ached constantly, and we felt sick with fear – but we were focussed on ‘following Kate’s lead’ and she is a joyful and happy child, even when she is struggling.

On September 17th, we packed our van and left Kate’s room at Ste.Justine. I had joked with our nurses that when it was finally our turn to leave the hospital – as we had seen so many others do before us – I wanted a parade and balloons. Our entire team made that happen for us – cheering Kate on as she left room 2-12-23 for what we hope is the last time. (Since Kate has been gone, 4 weeks later, they still refer to room 23 as “Kate’s room” had haven’t had another patient in there yet.)

Home has been amazing – and exhausting. It has taken a lot of time and way too much energy to settle in. Issues with our TPN pumps, trying to set up a workable schedule with home care nurses and personal support workers, fighting to get Kate the rehabilitative therapy that she needs in place, trying to get back into a routine as a family after having lived apart for 7 months, and incorporating all of my new nursing skills and duties have been some of challenges we’ve had to meet.

I can honestly say that now, at week 4, despite being tired, we are finally feeling settled. Kate is doing well. She is happy and wakes every morning with a smile. She loves being home with her brother and we have seen a big change in Jack. He has carried an incredible weight for an 11 year old boy – not to mention the fact that he feels responsible for Kate has her bone marrow donor. I am happy to see them together again.
Brian and I are doing our best to keep medications organized and on time, prepare food for Kate (yes, she is eating again…slowly) according to antiseptic prep protocols, and keep our home sanitized and a clean environment for Kate. There is endless laundry, cleaning, and still sleepless nights from getting up with Kate every 2 hours – but we go for walks, we sit down to dinner as a family, Kate is living with us in her home and she is still here.

 

The moment I heard the news about Kate’s post-BMT complication in August.  I was in my car. Brian had asked me to pull over and he and the doctor talked to me together. On the phone I was calm, rationale, trying to pull the pieces together and find the way to push through. It is my natural state – find a way through for Kate. But when I hung up the phone and the news really hit me, I looked up. I looked up and I said, “I’m sorry!”.

“I’m sorry. I made the wrong choice. Please – PLEASE – don’t take her.”

“I’m sorry.”

I still feel that way. I may always feel that way. I am not sure we made the best decision for Kate. We have dug her into a incredible hole. But she is incredible and she may just have enough JOY and strength to climb out. She is that incredible.

Maybe in a year I will feel differently. Or feel profound relief and gratitude that she was not taken from me. That I got to keep her.

We are happy to be home. Joyful to be home.

 

Julie

 

Posted by: searchingforsolidfooting | October 4, 2015

“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

 

 

 

 

Posted by: searchingforsolidfooting | August 4, 2015

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

 

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