Posted by: searchingforsolidfooting | July 20, 2015

What Is Isolation All About?

As we go through the bone marrow transplant journey and are into the post 100+ days period (we are at Day 126), family and friends are wondering why they still cannot visit or come to our home for playdates when Kate does get home. I thought an explanation here might help:

To prepare Kate’s body for a bone marrow transplant, she was required to undergo ~9 days of intensive preparatory chemotherapy and immune suppression. From the moment we walked into her room on the 3rd floor of Ste.Justine’s hematology oncology building, we were masked, gowned, and became obsessive hand washers. Anything that came into Kate’s room had to be sanitized – toys, clothes, bedding from home, iPads, cell phones etc. ‘Visitors’ were limited to a finite list of 7 people that were very well briefed on protective isolation protocol. We ensured that list was comprised of people who could commit to helping us care for Kate while she was undergoing her bone marrow transplant, and we negotiated for her brother to be added as a #8 to that list. (He was amazing with adhering to the protocols – very conscientious).

We were required to maintain this ‘protocol’ from the moment Kate started her preparatory regimen until her neutrophils were back above 0.5

Neutrophil granulocytes (also known as neutrophils) are the most abundant (40% to 75%) type of white blood cells in mammals and form an essential part of the innate immune system. They are formed from stem cells in the bone marrow. They are the ‘first responders’ in case of a bacterial infection. (Interesting fact: they are the predominant cells in pus, accounting for the yellowish/whitish appearance.)

Once above 0.5 we were allowed to remove our gowns and masks, but hospital personal and anyone else entering her room had to keep masks on. Hand washing and sanitizing were still very important. With her neutrophil count above 0.5 Kate had limited protection against  bacteria, but still had no immunity against common viruses (e.g. the common cold), or fungus (fungal infections). Isolation was very important.

As Kate recovered her blood counts her ‘neutropenia’ (neutrophils below 1.0) continued to have ups and downs. Her body was weak and needed ongoing protection. She was also taking heavy doses of methylprednisone to control her graft versus host disease, and cyclosporine to suppress her new immune system (to prevent GVHD). When she exited her room she was required to wear a N95 mask to protect her from airborne viruses. She was also to stay away from other people as much as possible. Her room continued to be kept as a ‘clean space’ and her home was also meant to be a ‘clean space’ with limited visitors (at our discretion), because of her immune suppression.

As you all know, Kate wasn’t home long, she was back in hospital less than 72 hours after being discharged to home. When she returned to hospital, she was again confined to her room and protective isolation protocols were put in place again. Mask, hand washing, limited visitors (immediate caregivers and helpers only).

Now that we are back at Ste.Justine, on the post-BMT unit, this protective isolation protocol remains in place. We have GVHD, and the treatment of this is immune suppression which makes Kate more vulnerable to infection. Kate is allowed to leave her room her, but can only ‘play’ on the 3rd floor BMT unit. She can’t visit the playroom on her floor (post BMT recovery), or be near any other children or parents on the hematology oncology unit. Her room is limited to her nurses and to visitors that are ‘approved’. This means that as her parents, we can choose who comes into her room, but we have been educated to be cautious and limit how may people interact with Kate from the outside. It is for her protection as she continues to be immune suppressed.

We are so grateful for the offers of help and support. Many of you have been so willing to come and see us in Montreal, and it is sooo difficult to say no. But we have to. Believe me, we would love the help and assistance, as there is no doubt there is a lot fatigue among Brian and I and those who have been helping us. But to protect Kate, I have to say no.
We have limited our ‘caregiving team’ here to what is essential and to those that have been trained and understand the isolation protocols and we are grateful for their continued support.

Once Kate returns home, she will continue to be immune suppressed and we won’t be able to have visitors at our home. She will get to go outside and see people from a distance, but not much more. Crowded places are a risk for her. This protective isolation will end when her immune suppression ends and when her CD4 +T helper cells start to recover. This will be another important milestone for us as it will signal that she has better protection against viruses and fungal infections.

 CD4+ T helper cells are white blood cells that are an essential part of the human immune system. They are often referred to as CD4 cells, T-helper cells or T4 cells. They are called helper cells because one of their main roles is to send signals to other types of immune cells, including CD8 killer cells, which then destroy the infectious particle. If CD4 cells become depleted, for example in untreated HIV infection, or following immune suppression prior to a transplant, the body is left vulnerable to a wide range of infections that it would otherwise have been able to fight.

We are watching what is called the CD4 count in Kate’s bloodwork. A consistent count above 200 is the goal.  We anticipate that being sometime in October/November. At that point restrictions will be lifted, but we still need to be cautious as Kate’s new immune system will have not been exposed to viruses that are common and will have to be revaccinated against childhood disease. 

Julie

Posted by: searchingforsolidfooting | July 15, 2015

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

 

 

Posted by: searchingforsolidfooting | July 3, 2015

110 Days

It has been 110 days since Kate had a bone marrow transplant. 110 days since we jumped off that cliff with Kate in our arms.

It’s been 16 weeks since my 10-year-old son bravely walked to the OR to give his bone marrow to his sister.

To Hope to curb the disease that we were told she would die from.

It’s been a little over 4 months in hospital for Kate…for our entire family. Watching the bravest, most stoic and loving child endure what would reduce most of us to tears. To watch her suffer. To watch her smile. To see the recognition in her eyes that she knows she has no choice. To know that who she is, the life she has had, the choices we’ve had to make for her, the challenges she has had to face are beyond anything anyone could ever have imagined or what many can understand.

How do I express my feelings after 4 months of this. Of watching this. Of knowing what I know. Of living what we have lived. Of feeling what I have felt and continue to feel.

Of interrupting her life and of being terrified I won’t be able to get her back to it.

My greatest fear was disrupting the life she had. And she did have a life. It wasn’t easy. Despite the smiles on our faces to mask the daily pain and challenges, it wasn’t easy. It has been 8 years of not easy. But it was her life and she was living it and making the best of it and we were (so far) keeping her safe and the monsters at bay.

But now…

…now there is uncertainty again. Where there was confidence in managing her disease, there is fear of her recovering from this. Where there was excitement, there is a sense of loss for her – time at school, time with friends, time with family, time in the water, time spent outdoors at parks – on bikes – running. Where there was a sense of Kate being happy, I’m now not sure.

The day-to-day fatigue dealing with chronic illness and developmental challenges has been replaced by profound exhaustion. Endless nights on the hospital room cot, sitting by her bedside crying over her small frail body and wondering “what have we done”, moments of hope for recovery that slide back into complications and fear for her life. Grief. Loss. Sadness. Fear. The emotions mix in a melting pot of pit of the stomach sickness.

You try to hold onto Hope. Because Hope is real and it is powerful. But you are the mom and the dad and you are responsible and Hope gets overshadowed by Fear. And it’s a dance between the two. And you want Hope to win and you want to understand Fear and accept it, to not let it take over and it is an exhausting day-to-day struggle. And you feel worn out. And you feel annoyed at constant reminders to “stay strong” “be positive”. You want her back. You want another chance at the decision to do this. And you can’t. There is nothing to do but go forward.

I try to remember who this is about. This is happening to Kate. It is her life so severely and terrifyingly interrupted. But as much as it is about Kate and has always been. It is about Jack and Brian and Julie and our family. Being scared for them, for us…for myself and how we will come through this.

It has been 7  years and 9 months of facing down the SIFD monster. A monster that had no name. A monster we fought to name. A monster we had come to understand and had learned to live with, but a monster we were told time and again had surprises for Kate. Nasty – life limiting – surprises.

I know she is strong and she is incredibly resilient, and I’m jealous of the kids who go home. Who suffer less. I am jealous of the kids playing in the park and crying over scraped knees.

I am amazed that she still smiles and giggles. That she still belly laughs at silly antics and strives to make friends with anyone she meets. That she loves her nurses and trusts her doctors. Where does that LIGHT come from? How can there be that much strength and will and love and FIGHT in such a tiny little girl?

 

This has been her life and we’ve brought it to some sort of crazy junction of Hope that she will get better and live an easier life, or Regret that we have chosen poorly and we have hurt her beyond repair.

Which is it?

I think she lives the Hope and I feel the waves of Regret.

The answer isn’t clear yet and it will be many more weeks and months in hospital and delicately stepping through this new existence of the post-BMT world before we know.

We are relearning her disease and what we have transformed it into by doing THIS. The doctors don’t even know. I think deep down we knew it would be up to us again to forge the path and guide them, lead them, help them navigate Kate. I don’t think they realized that Kate would have the lead. I bristle at the work I have to do to get them to listen, to engage, to discuss and debate and understand that WE, Kate and I, know this best. That we have the lead and they need to listen, support and follow. So I step back and start to build the relationships Kate needs to be safe. It’s not easy.

It’s not Fair. It’s hard. It’s exhausting.

It is what it is.

Put one foot in front of other. Take one more step forward. Ignore the pain and heartache. Wait for the good moments. Try to pain attention to them and enjoy them. Get through the bad. Try to recognize the Good and the Bad.

Try to live our best life. Despite.

Try to help her live her best life. And Jack.

How did we get here?

 

I just want her to be well.

 

 

 

 

Posted by: searchingforsolidfooting | March 8, 2015

Day – 3

IMG_2848

 

Time here is surreal. It crawls by at some moments, (e.g. when you are with an irritable Kate). And flies at others. I can’t believe a week is almost over. Today is Day -3.

Day 0 is the day of the transplant.

April 28th, the target date I have in my mind for going home, seems so far away.

Time is warped here.

Clocks move quickly and slowly at the same time. A moment can last forever and also pass so quickly. Kate can look good at one minute, and change the next. There is no solid footing to be had, and I have learned that the search for it is futile in this experience. I need to surrender to what is and let this happen and unfold, while being as vigilant as I can. I marvel at how we got her – how did this happen?

There is nothing more to do. One foot in front of the other, pace myself hard – but intelligently – know when I need to back off and know that I have another gear in me when it is needed most. See the race course as it’s been laid out. Push hard for that awesome outcome and be able to let go just a little of expectations when I need to

It is a Marathon.

A true physical and emotional marathon and I am grateful I have the experience as a distance runner to carry me through. This is not for the faint of heart. Everyone here is holding onto their values and courage with their nails dug in. Holding onto the things that give them strength. Foundations they have built over a lifetime. It is amazing to see how the human spirit can thrive in this type of environment – what people draw upon with this kind of suffering and stress. Raw fear and raw hope.

Kate is part of that for me. She has helped build me up to be the mom I need to be in this moment. We are a unit she and I. We make each other whole. Her laughter, her playfulness, her excitement at the simplest things, and even her tears.

I feel her strength and bravery and it helps me to fortify mine. That is an incredible gift that she has brought to my life.

 

Julie

Posted by: searchingforsolidfooting | February 17, 2015

Kate’s Bone Marrow Transplant

Yesterday, Monday, February 16th Kate was to be admitted to the Centre Universitaire Hospitalier Ste.Justine (Ste.Justine) in Montreal for conditioning therapy in preparation for an allogeneic stem cell transplant. A bone marrow transplant (BMT) on February 23rd with her 10 year old brother Jack as the donor.

Remember my last post about decision making. This is the decision that has been made. It is the biggest decision of our life and it has been incredibly difficult to make.

I am writing this, and we are not at Ste.Justine. Kate’s BMT has been delayed due to unforeseen medical complications. It might be a good thing as the Ste.Justine team gets better prepared for her and the medical complexities that surround her. BMT is never straight forward, but for Kate is it even more complex. The protocol ‘checklist’ has to be edited and designed to be specific to her medical complexity. We don’t have a new date yet, but this breathing room has allowed me to write this post and to share some information about this journey.

 

 

 

Where it began…

Many of you who follow this blog, or who have visited Kate’s CaringBridge site know the history of her diagnostic odyssey, medical journey, and her ‘status’ as the first child diagnosed in the world with a rare form of mitochondrial disease called SIFD.

Once the discovery of SIFD occurred a small group of children were identified who were diagnosed with the same recessive genetic disease – about 15. Many of those children were diagnosed posthumously. But one child who was alive was doing very well, and was not suffering from any of the complex medical conditions the other children with SIFD were suffering from. That child had had such acute presentation of SIFD at age 9 months (specifically the sideroblastic anemia and inflammatory cascades), that he had received a ‘hail mary’ bone marrow transplant. And it worked. He has not suffered any further episodes, and is the only known case with SIFD who has developed neuro-typically.

And so began the conversation with us two years ago about considering a bone marrow transplant for Kate.

 

Imperfect information…

So we don’t have perfect information about the impact of a bone marrow transplant for Kate. We do know that to date 4 children have been transplanted worlwide for SIFD. 2 have survived and are doing well – with no further episodes, and 2 have sadly passed away as a result of the procedure.

We know that the BMT would stop the episodes Kate is having, it would cure her sideroblastic anemia and it should prevent further damage from the disease. But this latter aspect of the BMT result is not clear. Kate’s brain, heart, kidney and liver could still be impacted further post-BMT.

We expect a better quality of life for Kate post-BMT, but getting there will require a significant amount of sacrifice and suffering on her part. A bone marrow transplant is not an easy thing to go through. It is considered one of the most invasive and difficult procedures in medical practice. We don’t know if she will get through it (5-10% chance of mortality), or if there will be unforeseen consequences because of the bone marrow transplant (5%).

We do know, based on the ever-changing information and insight we have into SIFD, that without intervention, these children tend to die before they reach adulthood. They suffer a ‘catastrophic event’ characterized by an acute onset cardiac condition (cardiomyopathy) or an acute episode of inflammatory cascade that presents like an idiopathic septicaemia.

SIFD is progressive and it is degenerative. There is no escaping that.

What we don’t have is the crystal ball to tell us when this might happen.

We have been given a choice – and our medical team feels that the BMT is the best course of action to pursue. Our other choice is to live with SIFD and take our chances on Kate dying from this disease.

 

What is a bone marrow transplant…

There are a ton of websites dedicated to BMT. The one I like best is www.bmtinfonet.org

 

Jack’s role…

In an allogeneic stem cell transplant, a donor is required. Typically the donor is sought out from within the persons immediate family as a relative can offer a better ‘match’ and the better/stronger the match the less risks there are with the transplant. Sibling matches are considered the best.
Neither Brian or I are a match.
Jack is a perfect match for his sister. A 6/6 sibling match.

We are grateful that Jack has agreed to take on this role for his sister. He understands that his body is strong and he has something he can share that might help Kate. His first reaction was that he might end up with a ‘clone’ sister (pretty funny). We’ve described the process to him. The surgery he will undergo. How he will feel, and the impact it will have on him and his activities. As is his nature, he has accepted this role with little drama.

He is not a hero, but he is very brave.

I wouldn’t have expected anything different from Jack. He is an incredible boy and one of Kate’s greatest champions.

 

Kate…

I am lucky to have two incredible children. While Jack is brave, kind and empathetic, his sister is courageous, stoic, strong, loving and TOUGH. AS NAILS. She is a survivor. And she is one of the happiest people I know despite it all.

Kate will require 5-7 days of intensive ‘conditioning’ before receiving the transplant. To quote one of her doctors, they need to bring her to the brink of death and then bring her back again. Her intensive chemotherapy is needed to kill off all of her bone marrow to prepare her body to receive the transplant from Jack’s body.

The chemotherapy will be introduced through a central venous line (Broviac) that is inserted into a large vein above Kate’s heart. Because of the multiple blood draws, the infusion of chemo, medications, and the blood transfusions Kate will require, a central line is required to easily access Kate for these procedures.

Once Kate has finished the conditioning regimen (days -10 to 0), she will receive the transplant. This is a minor event I am told, but an emotional one, as the stem cells collected from Jack’s bone marrow are infused via an IV bag into Kate’s central line. The stem cells then find their way to the bone marrow space and start to set up shop.

Day 0 – Day 7 Are supposedly the most difficult. Kate will be very sick from the effects of the chemotherapy drugs and immunosuppression drugs. She will be on full isolation for close to 8 weeks. I can be with her, but will need to be gowned, masked and gloved at all times. Access to her and her room will be highly restricted.

Kate will lose her hair, she will have nausea, vomiting and diarrhoea, she will have sores in her mouth. The BMT team is ready to support her through all of it, and prepared to deal with any unforeseen issues that might come up.

Day 7 – Day 40 will be a period of waiting for engraftment – waiting for Jack’s bone marrow to take over and start to grow in place of Kate’s. Graft Versus Host Disease and rejection of the transplant are the risks during this time – as well as organ failure, bleeds, and infection. Kate will still be feeling unwell and recovering from the conditioning regimen and the transplant.

After 2 months – If all goes well and Kate is stable, she will be discharged to home. The first 6 months will be critical as wait for Kate’s T-cells to show themselves again. Until that time, Kate will have no functioning immune system and will need to be in isolation at home. Her nutrition and her hygiene and protection from any mold, disease, or illness are extremely important. A  common cold could be devastating. She will be required to wear a mask when she leaves the house and she will travel to Montreal and CHEO weekly for medications, assessment, medical management and follow up. We won’t be allowed any visitors into our home at that time.

Post 6 month – If all goes well, we expect Kate to be able to return to school and other activities. She will continue to be on several medications as her new immune system grows and gets stronger in her body, and she will be monitored closely. It will take at least 2 years for Kate to have a fully functioning immune system.

 

The Plan…

We are currently waiting for a new date for Kate’s BMT. Her bloodwork was poor last week and a surgery to install her Broviac could not go forward.

We are mobilizing friends and family to help us while Kate and I are in Montreal. We are grateful my parents have moved here for a few months to support us.

It is not clear what we need right now while in Montreal or when we are discharged to home, but we will be sure to ask as support will be welcome and much needed.

 

The path ahead…

It has not been an easy journey – these past 7 years. Having a child that is living with a long term chronic and acute illness that is ultra-rare has changed me / us / our family in ways many people could not even imagine.

Kate’s life, her journey, her diagnosis, have changed the lives of others.

That in and of itself is incredible. So much power and awesomeness that this little girl has brought to the world.

So the journey continues. And Kate will be awesome again. And she will help others to learn, and to live better lives and to not be devastated from the ravages of this disease.

That is not a gift I would be willing to give on Kate’s shoulders, but it is our reality. It is her reality.

 

I am grateful to the incredible medical team we have at CHEO and Rogers House, and for their guidance in this decision.

I am grateful to the team at Ste.Justine, and the international SIFD team for all the preparation they have done for Kate.

I am grateful to all of you who are following our story here, on CaringBridge, on Facebook and Twitter.

 

Life is hard – there is no doubt. And others have it harder – some easier. This is our place in life and we are challenged with making the best of it and living it the best we can and with as much ferocity and compassion as we can.

Go Kate Go! 

 

Julie

 

Posted by: searchingforsolidfooting | December 15, 2014

Santa Love

Something important – even magical – has happened for Kate. Like any young child, she loves and believes in Santa.

She has spent the past 7 years being terrified of the jolly old elf, but this year the tables are turned. Santa is a regular in our home. We look at picture books about him, we drag around Santa stuffies, we have Santa ornaments and we regularly stalk him at the local malls.

To be honest, we saw the Santa love starting to emerge last Christmas. She watched him from afar, from the safety of holding onto my hand or from her dad’s arms. She was curious of him, she liked to see him and talk about him, but there was no way she was getting close to him, let alone sitting on his knee.

Santa loved has lasted over the much of the past 12 months – in Kate’s world things don’t end just because the ‘season’ is over. We celebrate on an ongoing basis as long as Kate shows interest. We have easily watched Dora’s Christmas Carol, featuring Santa, more than 100 times. (Birthdays and talking about birthday parties lasts a LONG time around here too). But Santa love has gone into overdrive since he started appearing at malls, in holiday decor, at xmas tree farms, and in arts and crafts at school.

He is hands down one of the best markers of Kate’s development. Kate is entering the world of “magical thinking” and pretend play. Suddenly princesses have become important, and playing mommy to her dolls and stuffies, and Santa and loving him and are part of that. Kate’s ability to understand – at some level – the ‘pretend’ of Santa shows a change in her cognition. There is some research that shows understanding ‘pretend’ allows a child to develop greater social understanding, and the ability to understand someone’s else’s perspective and this leads to an increase in social interaction and social play.

For our family, it is the joy of seeing a little girl with so many challenges light up at the sight of a warm and friendly person in a bright red suit at Christmas time. It is the heart-bursting moment of seeing her ‘get it’, and to see her excitement and joy, and knowing that she is her own little person growing at her own pace, but that she can enjoy Santa just as others can.

There have been many Santa moments for us this year – all of them wonderful. The most recent highlight at the Rogers House Christmas Party, Kate asking Santa for what she wants most for Christmas – French Fries.

Kate and Santa 2014

 

Julie

 

 

Posted by: searchingforsolidfooting | December 1, 2014

The Art of Imperfect Decision Making

A Recipe for Making Decisions

How to make an impossible decision in 7 easy steps 

 

Ingredients:

Information and Research

Gathering of knowledge from ‘the experts and specialists’

Analysis

Questions

Discussion

Weighing of Opinion

Pros/Cons

Evaluation of information

Decisiveness

Gut Instinct

Support

 

Method:

1. Gather as much information as you can through research. Mix this with knowledge and expertise from many sources (e.g. experts, specialists, others who have made a similar decision), placing an emphasis on those who are leaders in the area/topic on which you are trying to make a decision. Be prepared to search this information out internationally.

2. Mix all of this information, knowledge and expertise together and conduct an analysis by comparing and contrasting everything you now know. Where is the information similar, where is it contradictory, what are the gaps in your information, what other sources of information and expertise might be available, and find out what you do not know that you need to know. After this step in the process you can move to formulating your questions and identifying what more you need to know or who else you might need to speak to about your decision.

3. Your next step will require several discussions about the decision you are trying to make. The structure of these discussions will vary, and your recipe for decision making could include: 1 to 1 discussion, informal conversations with trusted friends/family/professionals, formal multi-disciplinary team discussions, discussions with specialists in the field. You might conduct your discussions over the phone, face to face, or over video-conferencing. You may also need to consider travel to other destinations in order to conduct your discussion in person. Consider the following when organizing and preparing for discussions related to your decision-making:

– Bring a friend who can listen and take notes. It is difficult to take all the information in when you are in a very important discussion/conversation. Having another person to assist in the task of listening and note taking can be very helpful to your decision-making process

– Ask if you can tape record the conversation. This can be helpful so that you can hear the conversation again.

– Prepare in advance. Write down a list of questions and concerns related to your decision and send them to the meeting participants in advance so that they are prepared to address them. (Sometimes sharing this list with knowledgeable friends or professionals in advance can help you to refine your list of questions).

– Bring a notebook and paper.

– Ask another person (social worker, nurse, trusted professional) to facilitate the meeting so that you can focus on the discussion and not on managing the meeting.

4. Analysis and Weighing of Opinion.

You will need time after the gathering of information and knowledge, analysis and discussion to weigh the information you have been provided with. A helpful tool  in this step of the Decision Making Recipe is to develop a Pros vs. Cons list and then to add a value or weight to each of the Pros and Cons. An example of this can be found here, Ottawa Family Decision Guide.

It might also be helpful in this step of the process to engage a Decision Making Facilitator or Decision Making Services, a third party who guide you through this process. This is particularly important to ensure there is open communication and that all parties of the Decision Making Recipe are working together and communicating well. In intense medical decision-making, this can sometimes get lost – this is where facilitated sessions can help.

Professionals can help guide you through the weighing of opinions and information, and can help identify where your priorities lie with respect to the decision you are trying to make.

5.  You may have to repeat Steps 1-4 more than once in order to feel decisive about your decision.  It is important to know that important decisions cannot be rushed, and you should feel supported in taking your time to make a decision.

6. Gut Instinct.

Your gut instinct needs to factor into to your decision-making. If it feels wrong, it probably is. In my experience, my maternal instinct has been referred to by our medical team and decision-making team, and I have been told to listen to it and factor it into my decision-making.

But what if your gut instinct isn’t clear? What if it isn’t saying anything at all?

I call this the vortex of decision-making. Circling and circling because the information, expertise, knowledge that informs the decision you are trying to make is imperfect and incomplete. How do you make a decision with imperfect and incomplete information? You make the best decision you can with the information you have.

The vortex of decision-making, also known as “indecisiveness” can be debilitating. It can eat away at your soul. It leads to stress, anxiety, possibly even depression and should be avoided at all costs. You avoid it by asking more questions, asking repeated questions, clarifying as much as possible and eventually coming to a decision, even if you are making that decision with imperfect information. A recommendation for your Decision Making Recipe is to set a timeline for making your decision (if one has not already been set for you). Living with an ongoing decision is very limiting and difficult. Once it is made you will find yourself ‘unstuck’ and able to move forward.  The decision can be changed, but at some point you will have to commit one way or another.

7. Once you have made your decision, sit with it. Let it absorb, start to live it and get as comfortable with it as you can. Shift your mind-set toward the decision you have made. Then push the ‘Send’ button and let the professionals know the decision has been made.

8. Once you’ve made your decision you will slowly need to start sharing it. Make it clear to others that you expect to be supported in your decision. Those that are not providing support need to play a more minor role in your life. They cannot contribute to the vortex of indecisiveness. They need to be ‘on-board’. Finding those people and giving the important role of supporting your decision is a last and most important step in this process.

 

Other Things to Consider:

– Others cannot and will not make the decision for you, but you can still directly ask your trusted team “What would you do?”

– Many decisions are imperfect because there is not enough information available or there is no obvious choice between the decision(s).

– Decisions can be changed/reversed to a certain point. 

– You should never feel pressured about making a decision.

– Include others in supporting you once your decision is made.

 

Julie

Note: Our family has been going through the process of a very difficult medical decision for Kate for the past 2 years. The Decision Making Recipe is based on our experience. We have made our decision  and it is imperfect and lacks all the information we need, but we are moving forward. 

I wish you the best of luck with yours. 

Posted by: searchingforsolidfooting | November 16, 2014

Feeling Included.

Bloom is a fantastic special needs, medically fragile, medically complex parenting blog from Holland Bloorview and edited by my friend Louise Kinross. (Incidentally, if you like them please consider supporting them as they run solely on donations).

This is a recent article that was posted, and I wanted to share it with you here and let you know my thoughts about it.

The first time I read the Invisible Mom I didn’t think that was me. I understood it and felt strong empathy for Aaron’s mom, but I didn’t think I felt the same pain and angst of exclusion as she.

But then I started considering how Kate is ‘included’ and ‘excluded’, deliberately or not. I reflected on the moments where her differences stand out so starkly and where I hustle to make excuses for her…

…”she can’t hear you”

“yes, she’s 7…but she’s more like a 3-4-5 year old”

“she is signing or saying this that or the other thing”

“well, she could come to the party…but maybe I should come too…to help…and she’ll probably have to leave early”

She’s the kid who leaves early from school, is often sick, wears the funny helmet, has a tube in her nose, doesn’t speak, sometimes hits the other kids, is often in her wheelchair stroller because of fatigue, runs away and won’t come back, doesn’t understand when you ask what her favorite color is (but she can tell you her name and how she is!!). Some (few) make an effort to include Kate, but playdates, birthday party invites, get togethers with the girls is not really part of her life sadly.

She loses her peer group annually as others grow and mature and learn and she is left behind. Her reality is that adults are her friends – and the few children whose parents facilitate them staying engaged with Kate.

Do I feel left out? Sometimes, yes.

Am I sad?  Yes, I grieve ‘loss’.

More importantly, does Kate feel left out? No.  Is she sad? Nope.  She has her friends at school that will change year to year, but that she values nonetheless, she has her friends at Rogers House (Myah, Moon Pie, Buffa, Mat-teww), and her adult friends (Christine, Kat, Erin, Kara, Adrienne, Vanessa, Steffi, Tall Steve, and more) that she loves. She is developing her own friendships and through those connections, I am finding my peer group of moms and friends as well. A different peer group than what you would expect, but a very valuable one.

I don’t think I am invisible mom. I think people see me. If they don’t I usually make them see me and Kate.

I wish for so many things for her. I wish for playdates and friends and movies and outings and independence.

I do feel sad sometimes. But Kate isn’t. Not yet. For now she is happy. Like Aaron’s mom I hope she never has to understand or become aware of the pain of invisibility.

Julie

 

A

Posted by: searchingforsolidfooting | November 7, 2014

Anniversary Weekend – Part 1

Still waiting for our SIFD party

Still waiting for our SIFD party

 

Today is the anniversary of Kate’s diagnosis.

It’s hard to believe that three years ago today we sat in the office of Dr.C (you can read about Meeting Dr.C here) our metabolics and genetics doctor and learned that Kate has a new and ultra-rare recessive genetic disease that she inherited from Brian and I. It reminds me a lot of this article by Matthew Might, dad to a son who also has an ultra-rare disease and was also an N=1 (as I wrote about here).

To quote Matt Might:

If found my daughter’s killer.

It took over 4 years.

But we did it.

I should point out one thing. My daughter is still alive. 

Yet. My husband Brian and I have been found responsible for her death.

 

When we were told that they had diagnosed Kate, there was a moment for me – just a brief moment when I received the call to come in for a meeting with Dr.C to discuss our test results, that maybe there was something they could do for her. A moment of excitement (?)… No. That’s not the right word. HOPE.

The moment did not last. As Dr.C talked about genes and exome-genetic sequencing and recessive genetic this and that. I listened. But I didn’t at the same time. I knew that if they could do something for Kate. If this was something ‘common’ or least ‘known’. If the medical team could help her – they would start with that.

They didn’t.

We looked at graphs. We looked at stains of Kate’s actual genetic coding for the TRNT1 gene. We talked about genetic condons of GCT –  GAT – ATC and any other combination of those 64 triplets of nucleotides that make up our genetic code.

And then, Dr.C took a ‘picture’ of Kate’s TRNT1 gene profile and overlaid mine and the Brian’s. They matched. My heart fell into my stomach. I was trying to understand. Trying to nod my head in comprehension. Trying to be brave. Trying.

Kate has inherited the exact same mutation from Brian and I on the gene known as TRNT1.

It had never been seen before.

Our children had a 1 in 4 chance of inheriting this disease. A silent killer. Completely unknown.

Jack isn’t affected, but could be a carrier. We haven’t had him tested.

The mutation has caused a deficiency in the protein needed for this gene to do it’s job. That deficiency (variable) has caused an incredibly shocking cascade effect through Kate’s body – resulting in the multiple medical conditions and ongoing acute illnesses she suffers today.

To quote Dr.H (PhD), a researcher working on Kate’s disease. “It is hard to believe a deficiency on one gene could wreak so much havoc on the body”.

 

What We Know

Here we are, three years after our ‘diagnosis’ and here is what we know.

There are approximately 18 known cases of SIFD worldwide. After Kate was diagnosed, our brave Dr.C took a leap of faith and share the genetic findings with a physician he had been working with at Boston Children’s Hospital. Their collaboration led to the first cases being confirmed, and to the establishment of an international medical and research team that continues to work on SIFD to this day.
Their efforts have resulted in a paper that describes the condition of SIFD. You can read about it here:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3761334/

A description of the TRNT1 gene deficiency that causes SIFD was recently published in the Journal Blood (September 2014).

http://www.bloodjournal.org/content/124/18/2763.full.print?sso-checked=true

Here is a picture of how the mitochondria are affected by TRNT1 deficiency – making SIFD a mitochondria disease. (I’m still trying to understand it too. Sigh.)

 

F1.medium

Many of the diagnosis of SIFD in the patients referenced in the article were done post-mortem. These children tend to die in early childhood – under the age of 4. They were identified and tested based on the cluster of symptoms they had presented with.

To my knowledge, there are 5 children alive today who are known to have SIFD. Four in the UK, and 1 in Canada. Kate.

I have recently been told of 2 more cases in the US and Brazil, but am not sure if these children are still alive.

The children have a variable severity of disease. Ranging from severe to mild. Kate is considered moderate. The children are treated with blood transfusions if their sideroblastic anemia is severe enough, immunogloblulin therapy to treat the b-cell immungloblulin deficiency, many have tried Kineret and prednisone to ‘treat’ or control the episodes of fever or inflammatory cascades (with differing degrees of efficacy), all are hospitalized regularly and monitored for cardiac myopathy – a severe and deadly effect of the disease, and inflammatory cascades (or Kate’s Episodes as we call them), which can also be life-threatning.

To date, 4 children have received a bone marrow transplant (BMT) to treat the disease and curb it’s course.

The first child done was a ‘hail mary’ as his sideroblastic anemia and periodic episodes were so severe his doctors had no other recourse. His BMT was done before SIFD was even discovered. He is doing very well. No effects from the disease.

The second child died during the BMT procedure. She was also very unwell.

The third child is also doing well and had less severe form of the disease. He is off of any intervening treatments and not experiencing any further episodes.

The fourth child was transplanted just recently (October 2014) and there is no information about his/her status.

BMT is an option for Kate. A terrible, awful, terrifying option – but an option nonetheless. It has been offered to us.

The CHEO Research Institute, Boston Children’s, and the Manchester, UK Children’s Hospital are all working on different features of the disease.

I call them regularly asking them if they are any closer to a cure. They are not. They are understanding the protein deficiency better – and all the mice they give it to die. They know what needs to be fixed, they just aren’t sure how to do it. I’ve offered to bring Kate for a visit – to create enthusiasm among the research team – to introduce them to the  little girl behind all of these incredible efforts – to expose them to the genuine JOY that is Kate.

There is nothing more motivating than wanting to help her.

 

My hope is that we find more children like Kate and the others and are able to learn from one another to help facilitate better management of the disease – share our stories – support our medical teams in finding a treatment or a cure.

I am looking for ways to do that.

 

Julie

 

Posted by: searchingforsolidfooting | October 31, 2014

Turtle Turtle

Turtle 1

 

Turtle.

It’s my word for when I retreat into myself.
It’s when I know that I am struggling and need to shut out the ‘noise’ of life.

I go silent. I get quiet. I don’t return email or calls or texts. I don’t go out. I sleep in. I cancel things – or I don’t show up.

Turtling comes from a place of being overwhelmed, which can be an easy thing to happen when you have an extremely unpredictable life.

It’s my code word, and those close to me know what it means. I’ve even thrown it out there on FB before “Turtle” will be my post, and my code word is understood and respected. Some friends will respond with “hugs” or words of encouragement, others with a “question mark”. It depends on how well you know me, and how well you understand the life I am leading.

My turtling metaphor indicates retreating. Pulling into my shell and self-isolating in a hope for self-preservation. It is instinctive and unplanned. It just – happens, and it is absolutely necessary. No one can keep this pace indefinitely. It is unplanned, but it happens when it is needed and when there is space and time for it. (It’s hard to turtle when you have to “on” and have to be a medical/school/therapy/treatment/acute illness hardcore warrior mama). It’s like getting a bad cold  or flu when you are on holidays…like your body finally realizes you are on a break and “have time” to get sick.

And Turtling isn’t depression. It’s blue and can be sad, but it’s different every time. It’s sort of an unplanned shut down. You haven’t plugged your power cord in for awhile and you’ve been ignoring those pop-up warning messages, so you go into forced shut down. It doesn’t feel good to go into unplanned shut down, and you aren’t sure how long it will take to power up again.

Why Turtle?

The shell. I pull into it. It’s sound proof and there are no windows or doors to peak in to see what I am doing. It’s dark and muffled and the space is small enough that I don’t have to invite anyone else in. But…if I need to poke my head out for something, I can.

And it’s hard – the shell – it protects me. I like that.

Am I Turtling now? You are probably wondering as I write this. I’d have to answer yes. Having a hard time with wanting to be out and about. Overwhelmed with decision making and intense responsibility that are beyond what you can imagine – or anyone should have to go through. (Seriously beyond. Like 1 in 7 billion beyond). Taxed.

How long will the Turtle last? It’s hard to tell. This one is a doozy. I feel like it will be awhile.

Do I still smile while I turtle? Yes. I do. And people ask how I am and I say ‘fine’. What do you say? Just smile and nod. Smile an nod. (And I find cute images of turtles to share).

Turtle 2

Those mom friends of mine out there who are reading this, and have special kids like Kate understand what I am writing here. I’m sure you call it something different. I know you have experienced it – and will again. My words of wisdom/advice, embrace it. Let it wash over you and don’t fight it. Let the Turtling happen. Every now and then poke your head out to see what is going on, to remember there is a life you have outside the shell that is important, be sure to get the basics done, and make at least one plan for the day (today I will make a meal, today I will run one errand, today I will make one phone call), and slowly ramp up those ambitions to draw yourself out of the shell – but don’t rush it. Decide what ‘noise’ is the most important and pay attention to that first.

Be social?  Well, you can. But it will exhaust you and people won’t understand. Phone calls can be good, if you have the energy. You may want to warn the person on the other line that you will be random, you may cry, you may rant, may be angry, or just sullen and moody. Try not to be too angry at every other living person. I walk around in a fog of feeling like I am on parallel universe to others who can’t relate at all to my life. (Warning: hanging around really happy people may make you want to slap them.)

You may also want to avoid driving. (Those red things at intersections are stop signs).

Exercise? Again, you can. But sleep and pyjamas feel more like the right thing to do. Then again, going for a good walk is probably well advised. (And I am a RUNNER).

Eat Well? It is advised. Throw in some chocolate for good measure. Be wary of too much sugar and caffeine though. You may not realize it, but Turtling likely means you are running on fumes and adrenaline. Adding a load of caffeine and sugar to that mix can be harsh. Wine is also a part of this. I’ve been told that more than 9 (6 ounce) glasses a week can be problematic. (Really??!!)

Sleep? Yup. At first you won’t. The lead up for me is my mind racing, staying up too late, not able to settle into sleep (and Kate doesn’t help that). Then the crash that precedes the Turtling happens and post crash all you will want to do is sleep. Hopefully you can set yourself up to get some. Respite from your child will also help.

(The above are the four most basic prescriptions of the ‘experts’ the doctors, counsellors, and advisors that we speak to. They do work, but when you are in the middle of Turtling you sort of want to thumb your nose at them. It’s hard to get the momentum to do any of these well. Self-care isn’t the first step when you are Turtling.)

Just remember you can’t stay in the cozy shell forever. Life is still happening and moving along. For the life we lead, Turtling too long can be disastrous. And you can probably count on your unpredictable kiddo to yank you out of Turtling PDQ (pretty darn quick), if they decide to get acutely unwell while you are holed up in your shell. “No more of that mom, I need attention now! Off to CHEO we go!”

And so the cycle goes. Recognize it for what it is.We can’t live this life of constant stress, worry, fear, anxiety, sadness, grief, joy, happiness, relief, frustration, anger, vigilance without turtling every now and then. I have realized it is physically impossible. I/we need moments where we shut down. It is a basic survivability necessity.

Turtle Turtle, welcome to your shell.

Julie

(Written as I sit here in my pyjamas and contemplate my ambition for the day. A shower perhaps?)

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