The Journey Home – Part 1

Kate, November 2015

Kate, November 2015

On Saturday, November 28th, 2015 we brought Kate home from CHU Ste.Justine in Montreal. She was very sick from post bone marrow transplant complications. Her most responsible physician, Dr.Michel Duval, had determined nothing else could be done for her. He suspected an acute pancreatitis, possibly brought on by her graft versus host disease. Surgery was considered, but it was felt Kate might not be strong enough to endure the procedure. Another option was to bring Kate home, keep her hydrated and manage her pain with the hopes that she might recover.
Kate died at home November 30th. I have never told this part of her story, about her coming home. In memory of the anniversary of her death, I wanted to share it with you over the few next posts on this page.

 

PART 1: Monday November 23rd to Friday November 27th, 2015

Kate was admitted to CHU Ste.Justine from home on Tuesday, November 24th. She had been examined by Dr.Major on Monday in the MDU at CHEO. Kate was having pain, was not eating, her BMs (bowel movements) had changed again and her need for oxygen was unchanged. I was scared, confused, and had that gut feeling that something was terribly wrong.

Dr.Major was very caring, empathetic, but also had her serious face on. I had seen that before. She had taken on an enormous responsibility to try and navigate my little girl through the brutal post BMT complications over that fall. It was not her area of expertise, but she had stepped up in the absence of any others. She and I had a strong relationship over 5+ years. She knew Kate and she had been part of the team to recommend the bone marrow transplant. I think she felt incredibly responsible to her and to me.

Christine was not in clinic, Erin was in her place. I felt comfortable with Erin, but I longed for the ease I had with Christine. In those moments, I missed her calm presence and the history we shared over Kate. I was grateful for that small comfort over those few hours in MDU that day.  As I fought back tears and a sense of panic, I asked Dr.Major, “What should we do. I think this is GVHD”.  Dr. Major answered that she would consult with Dr.Duval by phone. She left and when she returned a little later she said, “Dr.Duval agrees, he feels the GVHD has returned”. It was a simple statement. It turned my world upside down and my heart sank.

The look that passed between us in the moment was doctor to mother, mother to mother, friend to friend perhaps. Devastation. Fear. Sadness.

Disbelief. How could this be happening?

I tried hard to control my tears. Fight them back. To stay strong and stoic. To be brave for Kate. It was a living nightmare and I felt panic and bile rise to my throat. “Don’t show it”, I thought to myself. “Stay in control. Think. Think. What is next, what can we do next”.  Kate was watching us closely and being silly as she mistook a young resident who was with Dr.Major for Christine. “Christine” Kate would say, waiting for her favourite nurse and friend to say “Hi Kate”. The resident was confused, and looked at me. “She thinks you’re Christine, her nurse”, strangely annoyed as I tried to explain. She looked at me with a blank look. She was young, the situation was clearly over her head, and she did not know our family. It was a familiar situation from the past 8 years. I directed my look to Dr.Major and she understood that the moment had come to ask this person who was not part of our circle to leave. She couldn’t understand or fathom the journey we’d been on, and the incredible crisis we now faced. I felt incredibly vulnerable and I did not want to break down in front of this stranger. Dr. Major understood. The resident left the room for something, she didn’t return.
I quickly began gathering our things in the room. Little room #7, the smallest possible exam room ever. The only one we had ever really known in our 5+ years in the CHEO MDU. Too small for Kate’s wheelchair or to fit more than 2-3 people comfortably, and yet, how many people had we squeezed in there at a time as they poked and prodded Kate? Maybe 6 or more sometimes? On that day we had at least 7, including Kate, as our palliative care team as visited and assessed Kate. And how many times did we have to rearrange the room so we could conduct a procedure in there with access to both sides of the tiny cot. “It is ridiculous how small this room is, and how much time we have spent in here”, I thought to myself as I gathered our things.

I kept my head down. Not wanting to look up and meet anyone’s gaze for fear that the tears would start to fall, that I would ‘lose it’. I didn’t want that to happen. Not in front of them, or Kate. “You have to stay strong, you have to keep it together, you have to figure out what to do next”, I told myself. In those moments, no one said a word. Maybe there was nothing to say, maybe there was too much to say. No one knew what would be appropriate. “Was this the end?”, I am sure they were all asking themselves.

Kate said goodbye to Erin and Dr.Major, she continued to wave goodbye to people we knew in MDU as we exited for the last time. Smiling and waving despite it all. As I neared the door to leave, the tears started to fall. We would never return to the CHEO MDU.

The following day, Tuesday, November 24th, Kate and I arrived to Ste.Justine with Brian. Usually my dad drove us to Montreal for our weekly Tuesday appointment at the out-patient clinic. Today was different. As I packed the night before, I packed extra clothes and supplies for Kate. I packed the dry boxes and chargers for her cochlear implants, extra diapers, pyjamas, toys and puzzles. I also packed an overnight bag for Brian and I. I told my dad that I would not need him to drive us but would he please watch Jack. I didn’t know when we would be home.

I knew. 

The day was long on Tuesday. Our usual bloodwork was done, the dressing for Kate’s PICC was changed and the ‘caps’ to her PICC line also changed. As we entertained Kate in her isolation room, Dr.Duval came in and we discussed the plan. He wanted Kate to stay. Her bloodwork was showing unusual deviations in potassium and calcium, and she was clearly not feeling well. He felt we needed to get Kate back on TPN (total parenteral nutrition), where she was fed through her veins, being unable to digest anything directly from her gut. The GVHD was causing this. To start TPN, we had to be admitted.

I agreed, but was insistent that we get the TPN sorted out and then communicate quickly and directly with Ottawa to get the formulation compounded and delivered to our home. This had been an incredibly difficult thing to set up over the summer and early fall of 2015 in order to get Kate home. Since the plan had already been done once, I hoped it would be easier the second time. Dr.Duval agreed, but his demeanour was more of a ‘wait and see how things went’.

That day in clinic was long. I was spent emotionally and physically. I felt like I had nothing left and as I look back now, I think I knew how unwell my daughter was and that anything could happen. The past 9 months and the incredible stress reached a tipping point. I had been so resistant to this up until now, even when Kate was diagnosed with her acute onset hypertrophic cardiomyopathy in September, and we were told she would likely die from it, I did not believe it. Now I could feel the sense of panic, dread, fear, exhaustion set in. We had been through so much, and Kate had been through so much. I believed in her, but now I was scared.

I can describe in detail the two moments when I ‘lost myself’ during our 9 month BMT journey. This was the second moment as Brian was out of the room and I was alone with Kate’s nurse, Johanne. I cried. Hard. Big, snotty, hicuppy, heaving sobs and tears of grief. I was scared, “I can’t do this anymore. Kate can’t do this anymore. What have we done? Why is this happening? I am so scared”, I repeated over and over. The words poured out of me. Johanne listened and held me. I leaned into her.

Brian and I decided that I would go home and he would spend the night with Kate. I wanted to get back to Ottawa and try to coordinate things from that end in hope of getting Kate home sooner. I had learned from experience that I needed to be ‘on point’ with organizing discharge, coordinating home care, facilitating our TPN coordination, etc. We had learned through this process that though we had our complex care team championing us, I needed to work closely with them to make things happen. Kate did not have a the support of a post-BMT team at CHEO. Between Ste.Justine, complex care and myself, we had to fill that gap.

As we went upstairs to the hematology/oncology inpatient unit at Ste.Justine, we did not go to our usual room 2-12-25, but to a room closer to the nursing station. It felt foreign and turned around. Though we felt safe with the incredible nursing team at Ste.Justine, something did not feel right. I think Kate felt it too. It was late in the day and time for bath and bed. Kate was sad and kept signing and saying ‘Home, Home’. I can’t remember if she cried, but I knew she was sad. She wanted to go home, she did not want to stay. Was she scared? Did she know? My smart little girl had an incredible intuition for people and situations. Did she see the fear and sadness on my face?

It was incredibly hard to leave her that night, but I knew she was safe and with Brian. I wanted to get home in order to get her home. I had to make that happen.

Over the next 3 days there were phone calls between myself and CHEO palliative care, myself and CHEO complex care, myself and Ste.Justine (nursing, physicians, dietician, GI) trying to advocate for Kate and for her return home. I was met with reluctance by Ste.Justine as it was felt Kate was not stable enough and because of her fluctuating bloodwork they wanted ‘more time’ to sort out her TPN. I knew that if we did not get things sorted in the early part of the week, Kate would not get home for the weekend. CCAC (Community Care Access Centre) who would be in charge of Kate’s TPN at home, does not operate over the weekend (fyi: nothing in medicine happens on the weekend – it is a M-F 9to5 business).

I visited Kate over FaceTime. She would wave and giggle, signing and saying “Come, come”. I told her “Soon”, and she would sign back “Soon”. I made sure Brian was supported by Maimoona that week, one of the care providers we had hired privately to give us some respite during the long days in isolation. Kate was happy and joyful, despite the fatigue I could see in her face.

Finally it was determined a ‘case conference’ meeting should be held. These are meetings with multiple clinicians and service providers involved in the case. They wanted to discuss next steps for Kate between the CHEO and Ste.Justine teams, and I wanted to discuss getting her home. The meeting was held on the morning of Friday, November 27th, 2015. It was a videoconference between CHEO and Ste.Justine. Kate’s most engaged teams were there; complex care, cardiology, palliative care from CHEO, Ste.Justine BMT, dietician, GI, and nursing. Also in the room were discharge planning from CHEO – supposedly to help with organizing home TPN.

The discussion was set around what to do as next steps and trying to plan getting Kate home. The news from Ste.Justine was incredibly somber and difficult to hear. They felt Kate’s GVHD was back, that they could not increase her steroids that they had been weaning because her body was no longer tolerating them. In order to control the GVHD steroids were needed, but the steroids were killing her. It was a unique situation, one they had not encountered before and they were at a loss. What was proposed to us was a choice; we could take Kate home and let her GVHD be managed minimally at a low dose steroid level she might tolerate. The teams could not predict what might happen. Kate might slowly fight the GVHD and recover, the GVHD could take over and cause other complications in other organs besides her gut, she could live weeks or years and would supported by palliative care. No one knew anymore than that. The other option was an experimental procedure involving ablating Kate’s new immune system and letting it ‘reboot’ (so to speak). The theory was that the new immune system received from her brother during the bone marrow transplant procedure was over-active and attacking Kate’s body, specifically her gut, as an invader. If we gave it a reboot, the killer cells would be eradicated and when they regrew would possibly not be as aggressive, thus ‘curing’ the GVHD.

It was a dangerous procedure. One the Ste.Justine had been discussing (unbeknownst to us). Kate would be at high risk for infection, and in her already fragile state there was no guarantee she would survive the procedure. We were given a 50/50 chance. It was extremely difficult process this option given the limited time of our meeting and the need to decide quickly.

Our option was to hope Kate could recover from GVHD on her own, and treat her palliatively with minimal intervention, or go the aggressive route and hope to ‘reboot’ her immune system in hopes that the immune system 2.0 might not have the same GVHD effect.

As we heard this news, tried to process it, Brian and I sat surrounded by her teams, but alone. He in Montreal and I in Ottawa. He with a team of 5-6 physicians and I surrounded by the same. All eyes on us. We were supported and most people were empathetic, but we were so alone in that moment. I watched my husband cry with despair and sadness over videoconference. He had been with Kate all week. He had nothing left. A bomb had been dropped in our lap, and we had no idea how to diffuse it and very little time to think about it.

I spoke up, finding my voice and stating what I had said all week long. “We want Kate home”, I said. “We need to think about this and we need the weekend as a family with Kate at home”, as tears fell down my cheeks.

The room was silent, because you know what? Nothing happens on the fucking weekend. TPN could not be arranged with CCAC. The discharge planning person from CHEO actually said, “we can’t do that over the weekend”. I looked at her incredulous and could not form any words. It was not a new situation with us, this obstinance, but it devastated me. I looked around the room for help, no one had anything to offer.

And then, our incredible Ste.Justine nurse, Karine, whispered in Dr.Duval’s ear that she would make it happen, and despite the head shakes around the table from GI that Kate was not stable enough, he also agreed to ‘make it happen’. Karine came up with a solution of sending Kate home with IV hydration. Over a 48 hour period she would be hydrated with key minerals added in to keep her stable. She would return to Ste.Justine on Monday for TPN, and that would give CCAC time to arrange TPN for home delivery on Tuesday.

I can never thank Karine enough or Dr.Duval enough. No one could have predicted what would happen next, but as things changed over the weekend, Karine had made it possible for Kate to be home. She cared enough about our little girl and our family to do what no one else would.

And so it was agreed, Kate would come home that afternoon, Friday, November 27th. I would drive to Montreal to fetch her and Brian, and we would make our decision at home as a family about what to do next, and return to Montreal on Monday, November 30th with a plan.

A Month of Anniversaries

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

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

But this is about SIFD…

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

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

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

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

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

Teamwork solves the riddle of SIFD

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

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

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

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

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

 

Here is what I would add to this short article:

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

 

Julie

“How To Be A Good Guest”

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

 

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

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

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

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

 

Be. A. Gracious. Guest. 

 

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

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

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

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

 

House Guest Rules:

  1.  Be Clear About How Long You Will Stay

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

2. No Surprises Please

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

3. Choose The Perfect Gift

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

4. House Rules Rules

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

5. Be Appropriate

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

6. Help Out

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

7. Entertain Yourself

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

8. BYO

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

9. Leave No Trace

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

10. Give Thanks

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

 

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

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

 

Julie

 

 

 

 

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

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