We met with CHEO specialists this week regarding Hillary’s progress battling this rare disorder. We were all pretty nervous, and I want to acknowledge the Social Workers at CHEO for supporting us on this journey.
Hillary had some bloodwork done, along with another two hour IV infusion of pentamidine to prevent pneumonia. But she remains transfusion independent. This is wonderful for her. Her blood production levels continue to show promise. In two weeks, her hemoglobin went from 89 to 91, platelets grew from 55 to 63, but her neutrophils fell from .6 to .4.
Her strange PNH clone remains the same at 3%, and her cyclosporine medication level has fallen below the therapeutic threshold, so we have to get that number back up as a matter of priority. Clearly, we are not out of the woods by any means, but this is promising at 3.5 months post ATG treatment, nevertheless. The transplant centre at SickKids in Toronto continues to monitor Hillary and is also pleased with the response thus far.
Since her neutrophils have not made the climb into the normal range (the white blood cells to fight off infection) we remain in isolation. We still get outside every single day for fresh air, and plan strategic outings (while wearing masks) to minimize germs. There’s nothing like being the very first one in the library doors. To balance it all out, we stay social using Google Hangouts and FaceTime. We think it’s a good strategy in this day in age, and it’s working beautifully.
We head back to CHEO back in three weeks, the longest stretch of time without measuring her blood levels. We now strive for normal levels at the six-month mark — February. We continue to be so grateful of the moderate presentation of this disease. The complications others are experiencing with this disorder are unbearable and too triggering to share.
Life continues to be busy and challenging for us, but it is for everyone else. Everyone experiences health challenges, time conflicts, and life changes. And some people are doing it completely alone, or often without the support of their family.
We firmly believe that the love that surrounds Hillary, from all over the world, helps lift and carry Hillary above our heads while she heals.
And so we press on and remain vigilant.
The rest can wait.
Thank you for your continued prayers.
We are happy to report that Hillary’s out-patient visit last week was short and uneventful since she did not require any transfusions! This is a big deal. Her hemoglobin rose from 80 to 89 on it’s own, keeping Hills above that critically low 70 threshold. Her platelets saw a small rise from 49 to 55, and her neutrophils remain low, moving from .5 to .6. Overall, this is a favourable response at Week 12.
During her visit, Hillary received her flu shot as recommended by CHEO and had no adverse reactions. We were not a flu shot family prior to this diagnosis. Everyone close to Hillary is now getting it to keep her as safe as possible.
Hillary is doing exceptionally well in all areas.
She says she thinks her immune system is finally coming back from its long vacation. We celebrated the three-month mark with our favourite home-cooked dinners (yes, four of them) and a cautious trip to the quiet Ottawa Art Gallery, which she had never visited. It was really good to get out, despite the risk.
Our discussion with the specialists at CHEO was delayed by two weeks, so we just keep hoping her numbers continue to climb while we work to ensure she remains infection-free. We are still waiting to see if her mysterious PNH clone has increased in size, and if her ferritin level is decreasing. Every day, we are presented with a strange new set of symptoms, but they are all minor compared to the struggles others are facing.
I’ve spent a considerable amount of time looking back…however useless it may be….going through every photo since her birth and charting/analyzing her health timeline. Since her condition remains idiopathic (or for unknown reason) there is always that lingering question: Is it genetic or auto-immune?
I think it all started on Day 5 of life -— a day I remember vividly. Or maybe it was 2016 when she caught that virus. Were her teeth problems caused by the anemia or did the anemia cause the teeth problem? When you map it out, it’s all so interesting and interconnected. Some of it is truly unbelievable…as in, I’m having a really hard time grasping reality.
I will share more, when I can, and will be sure to update you all before Christmas. We’re not sure what Christmas looks like for us yet, but we know that we just need to stick together and stay positive.
We are extremely grateful to have Hillary home and feeling well.
And so, we wait.
Life remains on hold.
Check out our sweet Hills in March 2017 at the young age of 3. She’s always loved YouTube.
We would like to share some good news with our family and friends following Hillary’s journey.
Hillary is showing a partial response to the immunosuppressive therapy after ten weeks. Her platelets grew on their own from 17 to 49 (normal is 130-380) and her neutrophils went up slightly to .5 (normal is 1.5 to 8.0). Her hemoglobin has yet to start increasing on its own (still at 80 and dropping), and another blood transfusion might put her into iron overload, so we are really hoping to see a rise in oxygen this week.
To celebrate, we fixed up her room with an air purifier, salt lamp, bed rails, and her keyboard and she’s back to sleeping in her own bed (with mommy snuggled in close). It gave her back some balance and much needed independence. She is feeling amazing….confident, secure, and happier than ever.
Next week marks the three month mark since immunotherapy began. This is when we discuss options moving forward. This is when we are supposed to decide whether these early signs of blood production are good enough to continue trying with the therapy, or if we should proceed to transplant.
This is when the search for a stem cell donor would actually begin by the hospital we ultimately choose for the procedure — Montreal or Toronto. If you’ve been told Hillary has a match, you’ve been misled. Hillary remains at the mercy of time and the selflessness of some other human being to see if a match will be there for her when she needs it.
Stem cell drives in Hillary’s name continue to be held in schools/universities across the globe — Edmonton, London, Toronto, France, Ireland — giving us hope and encouragement. I personally think it’s A LOT to ask someone to donate any part of their living body, so to see thousands of people mobilizing to hold these events is incredibly uplifting.
Are we headed to transplant? Will there be a match for her? Will they still be willing to donate? Will they pass the physical? Will they be a perfect HLA match? Will they be willing to donate again if the first transplant fails? Will they have antibodies that will complicate the procedure? Will Hillary survive a transplant? All unknown.
We do not have a match for Hillary, but we don’t want one. We want this therapy to work.
If we continue with the therapy, we should see Hillary’s levels continue to slowly increase. She would hopefully start attending school in the Spring when her neutrophils are in the normal range, and she would continue the medication for another year before being slowly weaned off.
If she is weaned off and her levels stay stable, she will achieve remission…possibly lifelong remission with no further complications or limitations. But if her blood levels start to drop from this point forward — over the course of this lifespan — we head to transplant. She may not need a transplant this month, but she may in a year, two years — maybe ten years.
And life? Well, it keeps happening. Alyssa was sent home from school on Halloween with a suspected broken arm and spent the day at CHEO and a couple days at home. The well has finally been repaired and the water has been retested, but two weeks without safe water was ….cosmically disturbing. Winter is coming…..and so is the flu. Please show your love for Hillary by getting the flu shot and staying away if you’re sick. She’ll be the first one to respectfully tell you to stay out of her bubble.
Most mornings, the height of the wall appears far too tall to even try and climb, for me anyways. But the kids….they jump out of bed as if every day was like a gift or something. They rub their eyes not to remove the evidence of sleep but to brighten their view for the fun day ahead. They don’t stop. They are resilient and fearless.
Each day is a celebration of life as we work at essentially surviving this rare illness they call “the beast.”
This is a big week coming up. Please pray for Hillary.
It has been five months since Hillary was first diagnosed with Idiopathic Acquired Severe Aplastic Anemia with PNH Clone, and we are now in Week 10 of isolation for her immunosuppressive therapy treatment.
She is doing very well. She is happy, active, eating well, and still singing.
She continues with her daily cyclosporine medication twice a day, along with anti-pneumonia medication (Pentamidine) intravenously every two weeks since her neutrophils (white cells) are almost non-existent at around .3. She remains highly vulnerable. Prolonged neutropenia is of great concern with this condition.
Hillary continues to present as rare and does NOT require blood pressure medication, magnesium by IV, anti-fungal prophylactics, or frequent blood and platelet transfusions like so many other patients do. In fact, her platelets grew on their own from 17 to 32 in the last two weeks, which is extremely rare and promising.
Still, her hemoglobin continues to drop, and she has been struggling these past couple days with fatigue, irritability, and a high heart rate. We are headed in to CHEO for a transfusion, just in time for Halloween.
We take zero risks.
Supportive care plays a large role in remaining infection free. We follow the numerous guidelines established by our medical team, but also do so much more in our desperate attempt to keep Hillary as healthy as possible. It means saying no to a lot. I hope we end up being proof that it might make a difference. She is just doing so well.
This past week has been particularly….challenging.
The line from the water well to the house broke (after 12 years) so I’ve been driving two hours a day to and from my parents’ house to give Hillary an epsom salt bath. Staying clean is an integral part of Hillary’s recovery since a common bacteria could prove deadly. Living with the possibility of groundwater contamination for a week with a severely immunocompromised child is like walking circles in Dante’s inferno. Quite the lesson in resiliency.
Thank God I’ve come to embrace some of the adversity experienced early on in this diagnosis. Some of the darkest times now seem so meaningful, so necessary — they happened to keep her safe and protected, and for that, my eyes are wide open. Blessings in disguise. Watch out for them.
I’ve also come to appreciate the power of social media. Since day one, I’ve been a member of a Facebook Aplastic Anemia support group which allows me to navigate this rare disease — in real time — along with 3,000 other “fighters” from all over the globe. It is powerful. Every test, medication, symptom, reaction, level, outcome….fear….I can talk to people all over the world before making an informed decision about our care for Hillary. I’d feel pretty alone without it, particularly since there is a significant lack of current research on this rare disease.
Sadly though, I’ve already witnessed four families lose their battle in a few short months — half of them because there was no stem cell match available in the global registry. One fell through at the last minute when the donor failed their physical exam.
After holding stem cells drives in Hillary’s honour at several universities last month (Carleton, Ottawa, Brock, and Guelph), there is another one coming up on November 12 at Algonquin College. This date marks week 12 of treatment…the week we hope to see a rise in all three levels. Another meaningful and moving event in support of Hillary and others in her situation.
Thank you for all of your words of encouragement over these past months. Just because we can’t come to the party doesn’t mean we don’t like being invited. Thanks to those inviting us to stay in the game of life, and for totally understanding when we say no. Hillary enjoys FaceTiming her friends and family and is making the best of her situation.
She met with the Make-A-Wish Foundation who gave her some awesome gifts and told her they are excited to make her wish come true! Wondering which wish gives her so much hope and encouragement.
We had a wonderful turkey with all the trimmings on Thanksgiving at home, just us.
We went and voted together with “McKibbin pizza and park” afterwards, our little family tradition.
The girls are going as creepy porcelain dolls for Halloween. We’re so proud.
Alyssa is good. We are all working very hard to keep balance for our sweet Alyssa, and it’s working. We won’t let up.
Every single night, I lay beside Hillary, holding her, waiting for her blood to grow. I watch her chest move up and down. I hear her dreamspeak. I pray on her. Today is fraught with complications and challenges, but at least we are here to conquer it together.
It’s all just strengthening our armour.
We will win this war.
We’re keepin’ her.
Two weeks ago, Hillary proudly walked out of the hospital and into her cozy home. The first thing she did was march straight up to her room to play her keyboard.
The first week back was challenging. Her sister Alyssa caught a small cold and had to stay home from school…and then Hills got sick. But, her doctors are encouraged that her body was able to fight off the minor cold on its own.
Hillary is now six weeks into her immunosuppressive therapy treatment. She received another hemoglobin transfusion last week, and her platelets have remained “stable enough to sustain life” without a transfusion. She was also given pentamidine, a medication given by inhalation which she will receive monthly to prevent bacterial pneumonia.
Her doctors gave us just a couple of other rules to follow.
No school. No large gatherings or crowds. No animals. No gardening. No mold. No sun. No contact sports. No rough play. No swimming. No physical exertion. No construction sites. No travel.
Don’t be around anyone who is sick (or has been sick recently), and if you must have people in the house, limit it to one or two at a time. Don’t go near daycares or hang around other kids. Enforce herd immunity and have everyone around her get the flu shot.
Be extremely careful with food preparation. Have her wear a mask when you go out. Put a bottle of hand sanitizer in every room, and use it often. Dust often, and keep everything very clean. First sign of fever, head directly to the emergency room.
She knows every single rule, and advocates for herself. She does not feel sorry for herself. She is a fighter. She is amazing.
Her doctors also told us to get plenty of fresh air, eat well, and be joyful. And, so that’s what we’re doing. We go for daily walks with our hats and umbrellas. We cook farm to table every day. We adoringly put Alyssa on and off the bus and make her feel special. We sing, dance, craft…snuggle. We are very busy decorating for Halloween. This year, we are going all out with a scary, not a cute Halloween. I think it’s safe to say we’re all pretty fearless now.
It will be 12 weeks on November 12. This is when we want to see her numbers go up — her hemoglobin, platelets, and neutrophils. These are the three blood components that are suppressed by the aplastic anemia. To give you an idea of where we are at, in simplified measurement terms:
- A normal range for hemoglobin (red cells/oxygen) is 120-150. Hills presented at 48, and sits between 70-100 now with regular transfusions. Her heart beats fast.
- A normal range for platelets to clot her blood is 250 to 450. Hills currently has 10. She can not bump her head.
- A normal range for neutrophils (white cells) to fight off infection is 4.3. Hills is around .2. She can’t get sick.
She basically has no immunity of her own. Interestingly, she will have to get all of her childhood immunizations over again. The current measles epidemic has me a little shaken.
Sorry that when you see her, she looks perfectly fine and approachable. She is not. Let me protect her.
Sorry to St. Leonard Catholic School that we could not come inside the school to see the 1,000 paper cranes that were made to pray for Hillary. Thank you Mme. Roy and Ms. Lalonde for organizing such a beautiful gesture of community. We were moved beyond words.
Sorry to all those coming out to join the stem cell registry in Hillary’s honour… that I can’t be there to meet you. That’s been hard. Thanks to Nicole Brooking for going and shaking hands on my behalf.
Sorry to all those who want to help, but feel helpless. I can’t have you over. I don’t know what to ask you for. All I can do is follow the rules to protect my kids. I can’t focus on anything else.
Sorry, sweet Alyssa. Sorry I had to cut your bangs in the CHEO bathroom the night before school. Sorry you have to bite your tongue when things are unfair. Sorry you’re scared. You’ve been the best big sister, and daughter, we could have ever hoped for.
I’m not strong enough to put it in writing.
Let’s beat this thing and then jump in a muddy puddle together.
Check out Hills on KISS FM 105.3 with her favourite radio host and advocate, Amy Volume, a person who really understands.
Hillary is going home tomorrow, after 27 days in isolation. We are ecstatic. We can’t wait to sit in the backyard. Cook. Walk up and down the stairs. The simple stuff.
Hillary received a hemoglobin transfusion yesterday and will receive a platelet transfusion tomorrow to prepare her for discharge. Thank you anonymous donors. Hillary’s neutropenia remains severe at .1 so we won’t be taking any chances with germs or infection. This is such a peculiar and rare condition — one that requires constant vigilance.
So for now, it’s just a waiting game. We continue the am/pm cyclosporine medication at home, and visit CHEO twice a week for blood tests. She will need several more transfusions, but one day, at around three months, that blood test will come back with the news that she is producing blood on her own. We’ve visualized it. Drawn the picture. Written it down as an affirmation and framed it.
Reflecting on our time here, with these emotional blog updates and our quest to increase the stem cell registry, we’ve come to believe that Hillary was chosen for this. Chosen just like the rest of the kids here at CHEO — to teach the rest of us a thing or two about life and how to live it.
The fight isn’t over. The game has just changed. We now have the home advantage.
This blog post would have been unfairly difficult to digest before the joyous school start-up earlier this week, so we’ve waited a little longer than usual to update you all. Since then, things have indeed improved, but our sweet Hillary still needs your prayers and positive energy.
We were expected to have been done with the first stage of her immunosuppressive therapy treatment after eight days, but it is now Day 19, and Hillary and I are still in isolation at CHEO, with another 14 days to go…maybe longer.
It’s difficult, but not a surprise. My connections with other strong mothers fighting this rare disease alongside their children provided advanced notice of the complications that would inevitably arise. They warned me this journey would be extremely long and arduous — almost impossible. I was ready.
The past two weeks have been very challenging for Hillary.
She has endured unimaginably painful headaches, violent screaming steroid rages, CT scans, ultrasounds, a heart echo, daily blood cultures, hourly IV medication infusions, cups full of pills, fevers, night sweats, swelling, and several platelet transfusions. Her blood pressure is all over the place and and her organs are working hard. She was in a morphine sleep for four days straight. She did not look or feel like herself.
Hillary’s headaches have finally become manageable after three adjustments to her medication doses. But on Hillary’s scheduled discharge date, she developed a staph infection in her blood. When the overnight attending physician woke me in the middle of the night and took me outside to talk about Hillary’s blood culture coming back positive for “gram positive cocci” — that was my darkest night thus far. I thought I was strong up until that point. Not so.
It’s quite common to develop an infection while your immune system has been essentially wiped out to nothing with this treatment. Some infections are worse than others, and some you can’t come back from. We are just relieved that the blood infection has been identified, and is being treated with the proper antibiotics. The infectious diseases team is involved daily and we strive to attain that coveted negative daily blood culture. It’s coming.
We are very fortunate to have CHEO’s diligent team of doctors, fellows, and nurses springing into action every single day for Hillary, because every single day brings with it a new challenge. A small fever is treated as an emergency, and every night is sleepless. Hillary’s room is a revolving door. She entertains around 15 different medical specialists each day.
We need to get a grip on the headaches, the infection, but most importantly, the aplastic anemia. Hard to explain to a normal six year old….. but not Hills. She gets that no matter how much it all sucks, we absolutely have to push through, and take our medicine even when we can’t open our eyes. She is astonishing. I’ve seen Hillary during her strongest fight response…. no filter. She is a hero and an inspiration — there is no doubt about it.
As of yesterday, Hillary has turned a corner and is now singing again.
You wouldn’t believe how resilient she is. She used the self-regulation exercises she learned in Kindergarten at St. Leonard School to breathe through the pain of her headaches. She is showing teenagers how to swallow pills. Thanks to some new Nike shoes from Baba, she gets up in the morning, and does a dance workout.
Hillary also started Grade 1 this week with M.F. McHugh Education Centre. Her daily in-room lessons from her teacher, Reg, are….moving, to say the least. I weep in secret each morning as Hillary packs her backpack and waits by the door for Reg. We even get visits from fellow fighter Nancy Kawaja, an educator with McHugh who is overcoming health challenges of her own. I’ve never seen education delivered this way — with pure patience, understanding, compassion, and joy… all from a hospital bed. How fortunate are we.
We would not have made it this far without my parents…Hillary’s Nana and Baba.
They’ve given up everything. They cook healthy food all day for both myself and Hillary (whatever she craves), do all of our laundry, buy us whatever we need (toys, games, supplies), provide relief for showers, take Alyssa for sleepovers … sometimes they just sit in the parking lot and wait. They are here every single day, and on call all night long. They are truly selfless, and we will forever be grateful. If you see them out and about in Orleans, know that whatever they are doing, they are doing it for Hillary. Give them a pat on the back.
I’m able to write this because I do envision a successful outcome with this treatment. One that offers complete life-long remission. We can be part of that 70% that does not have to proceed to transplant. I have a band of mothers at my fingertips stepping me through every single moment.
Erica in Saskatchewan has been through it with her son. It didn’t work, and they went to transplant for full remission. Their only match was a 60 year old woman from Germany.
Lori in Toronto did it, fought it, and had to settle for an unmatched donor for lack of an available one in the database. She is caucasian, like us. They are doing great.
Amanda in Nova Scotia is currently in it with her daughter, two months ahead of us, waiting for the immunosuppressive therapy to work. I get hourly updates.
What will our story be?
Well, I can tell you it won’t involve us not finding a stem cell match if we need one. Not on my watch. It was very difficult to miss the stem cell drive at my former high school, St. Matthew High School on August 29, facilitated by the Bruce Denniston Bone Marrow Society.
Natalie (Dixon) Spence, you went way above and beyond to support my family by organizing all aspects of the event. Not sure what we did to deserve you. Thank you. Kalum Figura, for repeatedly mobilizing the air waves to promote these stem cell drives…you play a large role in educating the public on this issue which is heavily misunderstood.
Amy Volume from KISS FM — we’re not sure what motivates you to go to the ends of the earth for Hillary, by getting Gabriel’s Pizza to donate pizza in exchange for a cheek swab…but thank you. Please keep loving her.
Nicole…thanks for shaking hands for me at the drive event, for reading Hillary books at bedtime via Google Hangout, and for bringing me chocolate. Susan, thanks for rushing to my side with your mom on our toughest day, for lighting prayer candles for Hills, and for bringing me chocolate. Victoria, thanks for bringing me chocolate, and a sweatshirt to hide it.
Corey, Hillary loves her necklace wears it every day. Glenna — just seeing your name on a card moved me. Molly Penny, your daily visits mean so much, and you never deflect from the core function of your role here at CHEO. These are only a few of the people who require our gratitude. But this is all I can offer right now from this room.
Life here is surreal. I’m pretty sure I have already died. Sorry.
I plug my daughter into a wall every night to sleep. I watch as they pull vials of blood from her bruised and pale arm every day while transfusing her with someone’s else’s blood into her other arm at the same time. I wear gloves to feed her medicine that would harm me if I got it on my skin. I no longer sleep in a cot on the floor. I sleep holding Hillary in her hospital bed.
It’s been dark. But, the light is beginning to shine through. And it’s brighter because we’ve witnessed true darkness with our heads held high.
It shines through our commitment to promoting Canadian Blood Services. It shines through the continued stem cells drives planned in Hillary’s honour, like the one coming up at Ottawa University on October 3. It shines through Twitter — where hundreds of people mobilized to help find single donor platelets for Hillary. It shines through the Grade 12 student who got swabbed for Hillary, and has already been contacted to be a potential match for someone in need. He said he will follow through.
It shines through Hillary.
She will forget. I will be re-born. We will heal.
Thank you for the thoughts and prayers that have encircled Hillary over the last several days. I can physically feel your angst as you await an update on her status.
The first six days of Hillary’s immunosuppressive therapy were not without incident, but they were pretty manageable in terms of what could potentially happen with this treatment — some hives, sore ankles, and mood changes. We were able to make crafts and read while lying in bed for her six hour daily infusions. She was a little disappointed when she swallowed a loose tooth, but she and Molly Penny figured it out with research and letters to the tooth Fairy. She was happy. Her doctors were very pleased. She was rockin’ it, as evidenced by her tweets.
But on Day 6 (Sunday), late in the evening, Hillary experienced a severe reaction. It was like a light switch went off, and Hillary felt as though her brain was melting. The long and extremely violent event (exacerbated by the steroids) sparked a spot call (emergency) in the hospital to bring experts to her aid as they tried to alleviate her symptoms. We called family to her bedside. It was unspeakably terrifying.
Thanks to the fast-actions of nurse Judy (whose paisley scrubs and kind eyes are forever ingrained in my memory), Hillary received several doses of morphine, lorazepam, some blood pressure medication, and a life-saving platelet transfusion. Words cannot express the relief felt when you hear that blood is available in your child’s blood type from the blood bank, ready to use. It gets me every time.
Hillary continued to be sedated throughout the night to manage her pain, and had a second headache episode in the morning. We were hoping the incident was isolated, but it continued and increased in frequency through Day 7…Day 8…Day 9….tomorrow is Day 10.
An ultrasound revealed healthy kidneys, liver, and spleen, but blood levels revealed elevated levels of cyclosporine. Her medications have been adjusted, and we will stay at CHEO until she is stable enough to return home. Hillary has been on 12 different medications, some have to be administered with gloves due to their toxicity. Balancing the doses of these mediations is like….an artistic science. Too little, and the therapy won’t work. Too strong, and her organs can be damaged. But one thing is clear — if we don’t treat, Hillary can’t live.
And so we push on, for her, and her sweet sister Alyssa.
Once discharged, we will return to CHEO three times a week for blood work and frequent transfusions. Hillary will need both hemoglobin and platelet transfusions on Friday. She may develop serum sickness. She could develop an infection. Emotions are high. Fear is real. Communication is hard. But Hillary is amazing, and everyone who comes into contact with her agrees. I can’t even begin to describe how strong she is.
Hillary not singing right now, but she’s humming. And, she’s not the only child suffering in this hospital. I’m not the only mother sitting on an arm chair alone in the dark, afraid to sleep, eat, move.
Our story is not unique.
Please become a blood donor.
Please join the Canadian stem cell registry.
Please think bigger.
The fast track to treatment has been fast indeed, and tomorrow, Monday at 7:00 am, Hillary will be admitted to CHEO for the start of her immunosuppressive therapy treatment.
It will be an intense 8-10 days in isolation (with only mommy and a nurse) to bring down her immune system with hATG — to essentially stop her T-cells from attacking themselves. The more challenging part will be when we are sent home to administer the cyclopsprine medication ourselves for three months, without the CHEO army at our bedside.
Our doctor tells us this will be her most vulnerable time yet. She will have low neutrophils for the first month, which means she will have no chance of fighting off infection. Her lungs will be highly vulnerable. She will grow hair. A lot of it. She can’t be in the sun. She will gain weight. She will need frequent blood transfusions. She will not attend school for at least six months, and visitors will need to be carefully screened.
Our doctor told us this is also the most difficult part for families and relationships — not everyone understands the explicit nature of isolation. But this can work. It has to.
Acquired (not inherited) and idiopathic (meaning unknown).
Last week’s visit with SickKids Hospital in Toronto lent more insight into Hillary’s disease being auto-immune rather than genetic. Since all tests have come back negative for any underlying genetic cause (or stem cell defect), and since Hillary also developed an extremely rare PNH clone, her aplastic anemia is likely acquired.
Still idiopathic, meaning we will never know why, but acquired in that a virus or event likely happened along the way that made her body put up some defences and began attacking itself instead of the invader. This means this treatment path might work and bring with it life-long remission. We have hope.
While in Toronto, we met up with an aplastic anemia survivor. Naomi, we could not believe you were real. Naomi reached out to us very early in Hillary’s diagnosis by text. She is a 25 year old transplant recipient now in recovery. We have bonded for life. She gave us so much hope. We also met with a friend from high school.
Thank you, Colleen, for meeting us for cookies and a hug. Isolation can be pretty lonely. You made the world of difference with your friendship and laughter.
Meeting with Colleen made me realize….twenty years can’t interrupt the sense of community and belonging that the OCSB’s St. Matthew Catholic High School has afforded.
Like Natalie. Nat showed up at the last drive event, then went a step further and organized another bone marrow drive at our former high school in Hillary’s name. Join us on Thursday, August 29 from 3-6 pm at St. Matt’s in Orleans where the Bruce Denniston Bone Marrow Society and Canadian Blood Services will have 500 kits on hand. It takes 10 minutes to join the global registry.
#StartWithHillary and do it for Jeremy, who’s having a hard time finding a stem cell match since he is half-Chinese. Read more about #Match4Jeremy here, and please help give his family a chance at keeping their Daddy. More donor drive events have been confirmed for Caribbean Culture Days on September 28 and Ottawa University on October 3. A great deal of sharing is going on within Twitter, and Hillary will keep tweeting her savage tweets @StartWithHills to increase awareness on this critical issue. And if you have any doubts, read more here: Elmoazzen: Stem cell and cord blood donations can save countless lives (August 2, 2019).
Back to Ottawa and a sobering reality.
After our visit to Toronto, we rushed Hillary back home for two life-saving transfusions — hemoglobin and platelets. Then, the following day, dental surgery to pull two molars — a common procedure in order to prevent any possibility of infection. Then, some awful-tasting medicine and recovery.
Summer has always been a joyous time for us, filled with camping, boat-rides, food, and family. The days have been tougher this year. But, one thing about Hillary — she’s a winner.
She is to be highly credited for her current state of health — it’s quite rare to have remained infection free up until this point. Her precocious nature is a gift that has helped her make mature decisions about her health. She does not mind wearing a mask, and is now practicing swallowing mini Sweet Tarts in preparation for taking pills.
We work at getting through each day, and that’s it. Tomorrow is not promised — but it is planned. Please dream thoughts of wellness for Hillary as she works so very hard this coming week to beat this rare blood disorder, and come out the other side, singing.
Yesterday was Alyssa’s 9th birthday and another bittersweet day for the family. After a morning visit with CHEO, we were told Hillary is back in the “severe” range for aplastic anemia, particularly due to her low neutrophil levels. This puts us on the fast track to treatment. The risk of a deadly infection from germs, bacteria, and fungi are far too great to chance it. No more watching and waiting. It’s time to act.
This comes after a very eventful long holiday weekend, including an ambulance ride for a bonked and bloody nose. Thank you 911 Emergency Services, Ottawa Police Services, and Ottawa Paramedics Sam and Sebastien for your fast response, your calm competence, and your compassion for three, very scared girls. Alyssa’s coolness under pressure will never be forgotten. Hillary still thinks that she experienced “true magic” when she floated out of the back of the ambulance as the legs of the stretcher unfolded out of her field of vision. After some blood tests and a CT scan to check for a cranial haemorrhage, we were cleared to go home.
Our donation experience was moving.
On holiday Monday, we gave back in the best way possible by donating blood at the main office of Canadian Blood Services — Mommy’s 12th and Daddy’s first time ever. We saw several men donating platelets, which are interestingly yellow in colour. We watched as they sat for an hour and a half to filter the platelets from their plasma — a painless experience done as often as every two weeks. Women who have had children cannot donate platelets, and so these men…well, they are also life-givers, aren’t they. One of the men was making his 500th donation. Another, serendipitously, happened to be volunteer Stephane Goyer, who helped run our first bone marrow drive. And our phlebotomist, Christine, shared a story of love and loss with us. Hillary will need platelets this week. She warmly thanked several donors in person. It was a truly moving experience to say the least — not to mention, easy and fulfilling.
This is what the fast track includes.
Everything has been expedited. We’re off to Toronto on Sunday to visit with SickKids. When we get back, we head right into dental surgery, and will likely be admitted the following Monday to begin treatment, depending on how well she does.
If Hillary had access to a matched sibling donor, we would be going directly to a bone marrow transplant. Since we don’t, immunosuppressive therapy is considered the safer, less invasive, first-line treatment. The treatment begins with intensive administration of hATG medication for eight days in hospital followed by three months of daily cyclosporine medication at home, in strict isolation. It will not be easy. There are complications and visible side-effects. If it works, we continue for a year, maybe longer. It offers a 75% chance for remission. You can try the therapy first then go to transplant, but you can’t try it the other way around. We have hope that it will work.
The gift of life must be under that Christmas tree.
If it doesn’t work after three months (around Christmas-time) then we will go to transplant. We are hopeful there will be a 10/10 unrelated donor match waiting for her in the global registry. But hope alone is just not enough for me as a mother. Not when there is something I can actually do about it. I am in contact with too many people who were told there was a match, only to have it fall through. I cannot take that chance. I will not. I would want someone to fight for me. All it takes is to raise awareness. That’s it. The rest is up to you.
Even if Hillary doesn’t need a transplant now, she may need it in a year, in five years, in ten years. And what about the other 800 people in Canada waiting for a match? The other 18,000 around the world? More than half of the people waiting will not find one. Like this 9-year-old who is desperately searching for a stem cell match for aplastic anemia, just like Hills. It takes 10 minutes to #GetSwabbed to see if you can save a life. There are several upcoming opportunities in Ottawa to join the stem cell registry. Stay tuned for more information, and look for Hillary in Maclean’s magazine in September. She’s being featured as part of a national campaign by MediaPlanet for her efforts in raising awareness on this critical issue.
Unbelievably, the hardest is yet to come.
We’re told it’s a marathon, not a race. And while it’s being done in isolation, it’s not being done alone. Thank you to my parents for being there for every single appointment and procedure since day one, no matter how difficult or inconvenient it may be. Thank you to Anna for providing the only respite us three girls had this summer while Steve was at school. Driving to the east end of the city and swimming in your ozone pool every day with no one around was pretty much the best gift Hillary could have been given, especially because it came with your complete understanding. Thank you to the OCSB for letting me feel useful at work, for the very little I can do right now. You truly walk the walk when it comes to how to #BeWell, #BeCommunity, and #BeInnovative in the workplace. Thank you everyone who came out to celebrate Alyssa’s birthday on such short notice. She really deserved a stress-free day. She said it was “the best birthday ever” so that’s a win.
Thank you Nicole for rushing stuffed animals to the emergency room in the middle of the night. Thank you Stephanie for mobilizing an entire army of lacrosse players to spread awareness. Thank you Uncle Billy for installing a hot water tap outside for Hills to swim safely. Thank you Nadine for understanding. Thank you Dani and Kristi for rescuing me over and over. Thank you Mme. Brownlee for being Alyssa’s favourite birthday guest. And, if any of you fellow golfers out there really want to help us thank the Bruce Denniston Bone Marrow Society for saying yes to every bone marrow drive, please register for their upcoming September 20 golf tournament, their only fundraiser of the year. Click here: http://www.dennistonsocietyottawa.org/rcmp-denniston-bone-marrow-golf-classic-2019/
And Hillary. How is Hillary doing?
She is absolutely amazing. She is happy and has no problem making good choices, which is why she is doing so remarkably well. She’s very smart. She craves power-walking along the canal and loves playing her piano. She is not in pain and does not feel cheated. She’s fighting. She sees the big picture. And she never, ever stops singing.
This is the scariest place we’ve ever been. Please continue to send your light and love in Hillary’s direction.
“The little reed, bending to the force of the wind, stood up again when the storm had passed.”
“A rare presentation of a rare disease” is how the senior transplant specialist categorized Hillary’s condition during our visit to Chu Sainte-Justine in Montréal last week. In all of their years treating patients with aplastic anemia, they’ve never come across a patient quite like Hillary. That’s her in the photo (right) in the awe-inspiring Sainte-Justine hospital atrium.
Hillary continues in the watch and wait phase. She is floating in the “grade 2 non-severe” category right now, but can dip into life-threatening “severe” at any moment. She might need to go to transplant as an emergency, she could coast at the moderate stage for years on immunosuppressive therapy drugs, or she could still snap out of it with a spontaneous recovery.
After taking Hillary’s rare presentation into consideration, Montréal suggested a more conservative alternative before going to transplant, and CHEO concurs. It has a chance of working, but they’ve never tried it. After another 1-2 transfusions, instead of treating Hillary with hATG and cyclosporine, we are going to try the less invasive cyclosporine alone, without the hATG. If it doesn’t work after three months, we will go to transplant.
Does Hillary have a stem cell match?
We have been told that she does have a common HLA type, so that is promising. But, securing her transplant match can only be done when she needs it, as an emergency measure. Understandably so. They can’t secure a match now when someone in a more dire situation may need that match sooner. Makes perfect sense. Guess they can’t keep it in a cooler on ice for us.
This means that even if there is a match in the global registry for her right now, there might not be one in three months when we need it. What’s more concerning is that even if a match is found, getting in touch with that donor, having them pass their screening test, and making sure they follow through also has to happen. This takes weeks. We know someone right now who only has one match available in the entire world to survive, but the donor’s phone number is out of service. That’s right folks.
This is why we can’t stop.
Donor clinics continue to be held all over the country in Hillary’s honour this summer. We are planning another large drive event in Orléans at the end of August (date and location to be determined).
Until then, our calendar is full. Hillary needs to have two teeth pulled, to prevent a potential, dangerous infection. Alyssa and Mommy are taking a tour of the cord blood facility at Canadian Blood Services to learn more about that process (even though sweet Hills requires a bone marrow transplant). We are headed to SicKids in Toronto in the end of August to meet the foremost aplastic anemia specialist in the country — the only doctor conducting expansive research on this disease.
That’s why we have to #StartWithHillary and never stop. Alyssa said it best last night before drifting off to sleep. She said it’s like playing the impossible level of a video game.
I asked her how we should beat the level. I said fight. She said pray. We’ll try both.
It’s been several weeks since our last official update, and so much has happened. Thank you for patiently waiting and understanding how difficult it has been. We’re actually not quite sure how we are functioning as a family, but we wake up each morning and start again.
Our appointment with the Hospital for SickKids on Hillary’s birthday was difficult. Their transplant team went over the various treatment options, risks, and possible outcomes. We also received some news that Hillary had developed a peculiar Paroxysmal Nocturnal Hemoglobinuria (PNH) clone, which now makes her condition even more rare. It’s not “classical” PNH, but a clone. As her PNH clone changes (in either direction) — and her Aplastic Anemia fluctuates between severe and moderate — different treatment options come into play. Her condition remains idiopathic (meaning for an unknown reason) with different signs leading both towards and against it as a immune-related response. More tests are out all over the world. Our doctor said it best — we are in no man’s land. We now know it’s okay to be emotional, unbalanced, and exhausted.
The experts at CHEO and SickKids have been incredible, and they fully support our decision to welcome another opinion from the transplant centre at Chu Sainte-Justine Hospital in Montréal, Québec. They want to see Hillary. And so, we are off to Montréal this week to help us make the most educated, informed decision we can regarding the survival of our daughter.
Here are our possibilities as they exist at this moment.
Watch and wait. This is where we have been for the last two months. This means frequent blood tests to check her hemoglobin, platelets, and neutrophils, and giving her blood transfusions when needed. Hillary remains neutropenic, meaning she can’t fight off infection, so we are staying at home in isolation. It’s tough. But if anyone gets it, Hillary does. She’s not sad. Short term pain for long term gain. We can handle that, if it means we get to keep her. But, each blood transfusion adds new antibodies to her system which can change her chance of success for a bone marrow transplant, and transfusions over the long term can cause organ damage. So, we watch and wait, but are planning for imminent treatment.
Immunosuppressive therapy. This treatment involves bringing down her immune system with anti-thymocyte globulin (ATG) for five days in hospital, and then trying to kick start her bone marrow with strong drugs for a year at home (cyclosporine). But, it only works half of the time, and many go on to a bone marrow transplant anyways. While it is less risky than a transplant, she can still develop a deadly infection during treatment. And, the disease may come back later in life, in a more serious form. Calculating the odds.
Going to transplant. If we had access to a 10/10 sibling stem cell match, we would have gone immediately to transplant. If Hillary dips into the “very severe” category, we go right to transplant. Hence, the desperate quest to find a 10/10 unrelated donor match. But, the dangers of proceeding with a bone marrow transplant are serious and life-threatening, particularly with an unrelated donor. First, is finding that perfect match. While we still remain confident that her match is out there due to her common HLA type, and that a potential match will follow through when called upon to donate (God, please), it remains unconfirmed at the moment. A transplant will mean relocating to either Toronto or Montreal for at least 100 days. Praying for her survival will be the most time-consuming part.
Clinical trials. Half-match (haploid) transplant. Immunosuppressive therapy variations. And of course, there is still the chance of a miracle….a full unexplained recovery. Still.
We will continue to encourage the world over to join the global stem cell registry to increase Hillary’s chances of having a match available when she needs it. We are in contact with so many families who have found their “one” match in the world, so many people waiting for it to happen, and so many whose children have died waiting. We simply can not fall in the latter category, and we will forever advocate for everyone to have an equal chance at survival. In the words of Hillary’s favourite Princess Jasmine in the new Aladdin, we will not go speechless.
We have always been very cautious parents when it comes to caring for our children. Making this decision will be the single hardest thing we ever do. Your compassion and support is all that is needed. The rest has no meaning.
Hillary received a welcome hemoglobin transfusion today to give her some energy for the weekend. It turned her cheeks a rosy pink which we have missed seeing. This is her in the parking lot at CHEO right after her transfusion, feeling great.
During some fun in the backyard at home, Hillary took a tumble walking up the stairs and it was back to the emergency room for the McKibbin family. Hills was examined for a leg bruise and sent home relatively quickly after some late night ice cream. It was a long day but everyone was happy, so we didn’t count the hours.
Thank you to the anonymous donor from Canadian Blood Services who helped keep Hillary singing today. Hillary thinks that each pack of blood should have an avatar/emoji on it showing the type of person who donated, and we think that’s a great idea.
Please know that Hillary is happy, active, funny, and feels very loved and secure. Oh, and for those who know her best, she has not stopped singing.
#GetSwabbed in #Ottawa for @CanadasLifeline RELOADED. Please come back to St Francis Xavier High School on July 4 from 3-6 pm to join the global stem cell registry. There will be more staff and 1,200 kits available. #StartWithHillary and remember why.
Well, Ottawa. You did it again. After an unprecedented 630 of you joined the global registry on June 24, another 251 of you showed up to our second bone marrow drive. Another 600 of residents in the Ottawa region applied online for a swabbing kit since the beginning of our campaign on June 17, as evidenced by metrics provided by Canadian Blood Services. Almost 1,500 in this city alone. That is simply incredible.
We have so many people to thank.
Thank you to Steve from the Bruce Denniston Bone Marrow Society and his volunteers who have given countless hours to this campaign, with competence and sincerity. To Paul, the RCMP Mountie who stood beside us as we shook every hand. To the group of PetroCanada employees who came in after work to get swabbed together. To Moncion’s Your Independent Grocer for providing cookies, crackers, and water for both events. To Gabriel’s Pizza in Riverside South for attaching our drive poster to every pizza delivery box. To Sheldon of Best Green Hedges for filling in the ditch Hillary dug on Mother’s Day…you made it into a garden instead of a graveyard, and we will keep it healthy forever. To the Ottawa Catholic School Board for lending their school facility, setting up each event, sharing our call through your vast online network, and most of all, keeping our family in your prayers… we are forever grateful, but not surprised.
To Tom for making it happen, to Kristi for taking care of sweet Alyssa, to Kalum for no holds barred, to Nicole for being everywhere, to Anna & Vicky for getting me through, to Tommy for making Hills’ video, to Amy for speaking volumes… to Greg, Doug, Stu, Carla, Jon, Victoria, Patricia, Dani, Mary, Susan, the Ostapyks, Thomas, Kay, Erica, Molly Penny…to Mayor Jim Watson, to CHEO President Alex Munter, to Dr. Leibman, to both of our workplaces (the OCSB & OCDSB), to our family who was there for us, who understood our pain, to Nana and Baba for taking care of Hillary, just the way we needed it…
Please God, get us through this, so we can spend the rest of our lives thanking those who have helped us. Thank you, Ottawa. Thank you.
This morning, we met with the transplant team at Sick Kids Hospital in Toronto via telemedicine. It has really sunk in, now. So many of you messaged birthday wishes to Hillary. So many have sent personal messages of hope. So many have asked for an update, and we have so many people to thank. But, today was simply the hardest day of our entire lives thus far… juxtaposed with the pure joy and happiness of our now six-year-old child. It’s been very hard keeping her bright light contained while we try to keep her safe and infection free, but we are going to continue to fight for Hillary’s safety.
Please give us some time to provide an update. We need to spend time together as a family. We need to rest for a minute. We need to regroup.
Once we’re ready, we will come back fighting — stronger and more unified than ever. We will be ready for the second July 4 stem cell registry event, and the one after that. We vowed to leave no stone unturned for our daughter, and that’s exactly what we’re doing. There is no feeling like it in the world. Steve and I will be there on Thursday to thank everyone personally for such selflessness.
Thank you for your patience, support, and understanding. Please continue to have hope in your hearts for Hillary as she battles this bizarre medical mystery. Positivity is the most important gift we can give her… and her sweet sister, Alyssa.
Wow Ottawa, you smashed it last night. You turned up in droves to join the global stem cell registry. After an unprecedented 630 registrants, we ran out of kits, and had to turn another 500 away.
To the mom and her son from Montreal who was turned away, please message us. We will drive a kit out to you personally. To the family and friends who sat at the back, feverishly assembling more kits for Canadian Blood Services, thank you. To the handful of people that came to join, but fell outside the 17-35 demographic, thank you for coming. Please continue to support this cause, and keep donating blood. To the friends and family that we have not seen in decades who came for just a hug…you’ve moved us with your kindness and love.
To the young men and women of all backgrounds who came out, shook our hands, and said “no problem” … you really demonstrated that you got it. You heard the call, understood this complex issue, knew how easy it was, and did what you could. You just needed a reason. We are organizing another event within the week. If you were willing to come out last night, please be willing to come back for another try.
We’re sorry we ran out of kits, but we’re not sorry you heard Hillary. She knew if she spoke loud enough, you’d hear her. This is Hills, and big sis, sweet Alyssa, on their way to CHEO yesterday morning at 7:00 am, before the successful drive event. You might want to turn down your volume.
Please join us on Monday, June 24, 2019 at St. Francis Xavier High School, 3740 Spratt Road from 3:00 to 7:00 pm to join the bone marrow registry in person. It takes ten minutes to see if you can save a life. Young, male donors ages 17-35 offer the best patient outcomes, but females can donate, too. Please print the poster… post it, pin it, share it.
Please shake our hands, every single one of you. The impact you are having on generations of thinkers by joining the registry — or simply sharing and spreading awareness — is truly profound. Be fiercely proud.
We sent out a cry for help, and you heard it. That’s what makes us human… alive. Our DNA is not ours alone. It belongs to the evolution of mankind. There is still so much work to do here, particularly for the underrepresented. We will #StartWithHillary and never, ever stop.
This is Hillary. She is six. She has big dreams of being a rockstar. She is always singing. On May 12, 2019 (Mother’s Day), after a blood test, we were called in the middle of the night to bring her to the nearest emergency room immediately.
Hillary underwent two urgent life-saving blood transfusions. She was admitted to the Children’s Hospital of Eastern Ontario (CHEO) and further tests ensued. A chest x-ray and surgical bone marrow biopsy revealed that she did not have cancer. Samples of her blood were sent out all over the world — London, New Zealand, Vancouver, and California. We found ourselves tumbling down the rabbit hole as doctors systematically ruled out over twenty genetic causes and viruses in a race against time to determine the best treatment.
Hillary is sick.
She has been diagnosed with an extremely rare and fatal disease if left untreated, Idiopathic Aplastic Anemia. Affecting approximately one per million people a year, this disease causes Hillary’s bone marrow to stop producing enough red cells, white cells, and platelets. She continues to receive blood transfusions to keep her alive. We knew Hillary was one in a million; we just didn’t know it would be in more ways than one.
Her low red cell count means she is pale and constantly exhausted. Her low white cell count means she is highly prone to infection. Her low platelets have resulted in full-body bruising and uncontrolled bleeding. She cannot attend school and requires constant care. Time has stopped for all of us. All we can do is watch and wait.
Hillary needs a life-saving bone marrow transplant.
Her big sister, Alyssa (8 years old) was immediately tested as a potential donor. She is only a partial match. Hillary requires a 10/10 match. This has been extremely difficult for Alyssa in so many unfair ways.
A transplant from an unrelated donor provides an estimated 85% chance of survival from this disease. If we are lucky enough to find a match, we will then need to relocate to a hospital with a transplant centre (Toronto or Montreal). We must then pray that her body does not reject the transplant, and make sure she remains infection free. Hillary’s recovery will be critical. She has a long journey ahead of her towards a healthy life.
We’ve organized numerous blood donor clinics in the city of Ottawa on behalf of Canadian Blood Services — only to now be in desperate need of blood. Our daughters have donated their hair twice to the Canadian Cancer Society — only to have to watch Hillary lose all of her hair for her upcoming treatment. We’ve given our hearts and time to causes like United Way Ottawa as a recognized Community Builder to help those in need — only to now be a position of need ourselves.
Not everyone will find a match.
This call for help is not just for Hillary. It’s for every human whose life can be saved by the selflessness of another. As you’re reading this, there are 18,000 people around the world waiting for a match — 800 in Canada alone.
Wherever you are in the world, join us in our quest to increase the World Marrow Donor Association registry. Hear this call for a cause bigger than yourself. Willingly offer something that replenishes within yourself effortlessly, and let young children like Hillary grow old, sing…shine.
Reality has been blurred with nightmare. We just can’t take a passive stance. We have to fight. We will leave no stone unturned. Your participation is appreciated beyond measure, as are your prayers for our sweet, sweet Hillary. God help us.