It has been seven weeks since our last update.
Hillary is in danger. We have to make impossible life decisions again. 

Three weeks ago, we asked for an in-person appointment at CHEO. We just didn’t feel right after the ER fever with no explanation. We felt showing up in person would put some positive pressure on her medical team. 

We brought forward a new concern about Hillary’s armpit. It’s been enlarged since her PICC line infection six years ago due to surgical error so we sought a physical examination to see if a diagnostic was required to rule our cancer (MDS). We also asked for another Paroxysmal Nocturnal Hemoglobinuria (PNH) test.

When we got to our early appointment, we could tell something was up. We were met with a team of people – a Hematologist, Case Manager, Social Worker, and Pharmacist. 

We were told that there has been a medical mistake.
That they didn’t notice until now.
That Hillary’s PNH Clone is dangerously high at 72%. 

That she is the very first and only patient at CHEO with PNH.
That she is presenting atypically. Rare.
That she may be in imminent danger.
That they don’t know what to do and they are referring us out. 

Hillary handled it well. Like a mature adult. There was no sugar-coating it. They re-did the PNH Flow Cytometry blood test right away and sent it to both The Ottawa Hospital (TOH) and Toronto in case there is an error in the Ottawa lab and sure enough — she’s in trouble. 

Her PNH clone reached 72% at our annual PNH test in May 2024 — 12 whole months ago. We were told it was 10% at that time. We’ve been living so carefree. The PNH clone in her neutrophils is now 62%, as of June 11. It came down but it stayed high all year. Treatment is recommended after 40%.

They say the error occurred since they’ve been looking in her red blood cells only — they didn’t know to report on her neutrophils — the ones that are mysteriously low. 

They advised that Hillary is in imminent danger of a blood clot in her leg or head or lung or a deadly infection from her suppressed neutrophils. That instead of being allergic to NSAIDS, we should now consider giving her a Baby Aspirin to avoid a clotting incident. If we want. 

That strenuous exercise may be dangerous because it induces hemolysis, and she can’t afford that. That this is why she can’t go on those long walks when she’s asked every single day. That this is why she has had so many headaches and so much stomach pain over the years. 

SickKids Hospital in Toronto chimed in and said she should immediately increase her water and iron intake to keep her blood stable. That she will need emergent care soon if the clone doesn’t go down on its own. 

Hillary is very concerned about her care. 

To treat PNH, Hillary will need weekly IV infusions of Eculimizab FOR LIFE, but the doctors don’t want her to take it. She’s too young to take this treatment path and the infusions bring about a great risk of bacterial Meningitis. 

CHEO has never administered PNH medication before, so they are trying to get it set up right now. There is no infusion centre in Ottawa. The infusions take four hours each week and there is no space for Hillary to get this done safely given her immunocompromised status. 

Solaris. Just like the movie about medical trauma and water. We just received pre-approval from our insurer to cover the $700,000 AstraZeneca complement inhibitor. She may need this tomorrow. 

In the meantime – until she has a life-altering incident in my arms at home — we’ve been given a slew of blood thinners and blood stoppers and are told it’s our decision whether to use them or not. The blood thinners bring with them a 2% risk of nosebleeds, among other bone marrow failure side effects. 

How am I supposed to make this decision on my own?
Steve keeps reminding me I’m not a doctor but I’ve been told to be one.
I have all these pills here and I’m supposed to decide what to give her.

No one wants to risk jeopardizing her good health.
Whatever happens next, it’s on me.

Hillary said she’s relieved it’s up to me. I share daily research with her and we feel very well informed about her situation. Still, we can’t seem to escape danger, no matter hard we try. She’s a kid. She fell in the pool yesterday and has major shin bruising and we literally don’t know if this will become a medical emergency for her or not. 

Navigating this is not for the weak.
HILLARY is a true warrior. Forcing the smile. Hearing my clear words.
We WILL get through this, just like so many before her. 

Referrals have been sent to SickKids in Toronto, St. Justine’s in Montreal, a PNH expert at The Ottawa Hospital, and if I want to take her to the National Institute of Health (NIH) in Boston – which I do, I think – I have to get that going myself and find money to pay for it. I don’t know how to navigate this. 

We are currently waiting to hear back from ANYONE to decide how to treat Hillary. Our next appointment at CHEO is on July 16. 

The doctors said I should be able to find some better experts in Canada to help Hillary since I’m a Director on the Board for the Canadian AA Foundation. 

I didn’t have the heart to tell them that I resigned shortly after joining. I’m too involved in both my daughters’ complex care right now to take on a volunteer marketing job in addition to my own full-time career that I’m barely clinging to. I also felt a serious conflict of interest approving research contracts for the same CHEO staff caring for Hillary. It wasn’t a good fit for me. I can’t jeopardize her care.

By the way, these medical dramas happen all the time in Hematology, all across the globe. Many patients switch doctors for they lack specialized knowledge in their particular blood disorder. 

Doctors aren’t all-knowing machines. Sick people know they are sick, and sometimes, they need to convince their doctors. Kids included. 

The doctors kept telling us to ignore it. Every three months. We pushed for Hypertension exams, MRIs, referrals to specialists…whatever we could get away with. They told us that some kids just have low neutrophils. That her headaches must run in the family. That her PNH Clone does not need to be tested again since she is not experiencing hemolysis —- blood in the urine. That the test is expensive. We complained in writing. It didn’t matter. 

Just like the movie Arrival about navigating rare disorders, this is pretty scary stuff. I can only describe it as swimming in the pitch black ocean. There is nothingness all around, just the black abyss – no answers, no lifelines – just the unknown. Reach out…… nothing’s there. Everyone’s left.  

I’m holding Hillary. She’s cold.
She’s asking me to save her. 

The only solution is to search within.
Hillary needs as much of The Fifth Element that we can give her right now to get through this. 

Hillary’s recent blood counts of June 11 revealed a hemoglobin of 128, platelets at 212, and neutrophils at a record high 2.5. She is stable and not currently in bone marrow failure. She is presenting atypically for someone with such a high PNH clone, but again, it’s hiding in her neutrophils instead of her hemoglobin. 

Hillary underwent an urgent MRI of the head last week since the first MRI did not look for any PNH factors, which can cause seizures. I am pleased to report that the MRI was totally clear. 

Hillary underwent an ultrasound of the armpit last week which revealed nodes double the size of the contralateral size, but without anything concerning. There are no signs of MDS and no need for a biopsy. 

Hillary proceeds to the dentist next week to rule out the possibility of infection. She had four severely infected molars pulled at diagnosis and I think her medical team might really be missing something here. This should have been checked in the ER when she spiked a fever. Hillary was set to get braces next week with Alyssa, but those have been cancelled. She can’t go to transplant with braces. 

She says she’s happy with her smile.

Hillary met with her Orthopedic Surgeon last week, Dr. Kevin Smit, the same doctor who saved Alyssa’s life six months ago. His eyes watered when she showed up in the T-shirt she made for him. He is helping Hillary with early intervention bracing to help her avoid surgery, and so far, it’s working. Her x-ray showed NO progression of scoliosis as a result. We discussed the bone marrow’s role in spine development, for both our girls and….I really value this doctor’s expertise a great deal. Hillary proceeds next month for a new orthopedic back brace.

Alyssa proceeds next week for PNH testing. 

Hillary underwent an ultrasound of her abdomen last week to check her liver, spleen, and stomach. Her organs appear normal in morphology but her LDH (lactate dehydrogenase) numbers in her liver have been more than double for the last several tests – a major indicator of PNH and requirement for treatment. I was granted an urgent appointment with a CHEO Hepatologist last Tuesday and she performed a more specialized scan of her liver, which again, was normal. 

The specialist admitted she had never heard of PNH and I begged for a referral to the Hepatologist at SickKids and she obliged. She ordered additional tests to check for cell destruction in other areas and those are all normal. She said I seemed very desperate. We really liked her and value her specialty. 

Cause this is where Hillary’s blood cells are dying — in her liver. 
At night. When she’s sleeping. 
Even though her liver is healthy. 

To try and explain further, Hillary has experienced a somatic mutation of the PIGA gene in her DNA. It didn’t show up in the genome mapping since there is nothing technically wrong with her DNA. CHEO doesn’t know how to test for this isolated gene marker so they are looking into it. We’ve confirmed that the small PNH Clone (.3%) was present prior to the start of her treatment, meaning, the PNH likely caused the AA – not the other way around. 

Hillary doesn’t yet have Classical PNH where she would need many blood transfusions – just a bunch of copies of it. Clones. Hiding. Waiting. 

She is missing a critical protein protecting the surface of her red blood cells. The missing protein is caused by a rogue antibody. They can’t find which antibody is causing the damage. They aren’t allowed to look further. 

The PNH will keep making copies of itself until it takes over her marrow and she goes into bone marrow failure — Aplastic Anemia (AA). The only way to get rid of the overzealous antibody is to replace the whole organ – the bone marrow. They say this is the best case scenario – provided she has a match. 

OR….this may all go away on its own. Spontaneous recovery. We so desperately want this. Watch the movie Miracles from Heaven for a glimpse into what we are always so desperately hoping for.

Hillary has adopted a full organic liver flush diet. It’s not far from the Mediterranean diet she was already on, but in just two weeks, we’ve pulled her LDH down quite a bit and we’re hoping the PNH clone follows. She gets tested again in three months. September. 

She’s also cut out the Tylenol suspension fluid, but I’m worried she will hemorrhage during a bad headache so we’re meeting with a Neurologist next week for another solution to help ease her suffering. 

Many other tests are going out and coming back every day.
Urinalysis, hormones, heavy metals, kidney, bile, vitamins, muscle function.
They are all coming back normal.
They said Hillary is different.
Very healthy with stable blood levels. 

Hillary’s doctors have trepidatiously recommended a surgical bone marrow biopsy and aspirate at this time. The last time we did this, Hillary tanked, requiring many blood and platelet transfusions. We don’t wish to proceed without input from an expert on PNH. They agreed.  

They said one of the first things we need to do is vaccinate Hillary for Meningitis. I almost blacked out. I cited Alexis Lorenze’s PNH vaccine injury scandal, and while different than Hillary, I want a full investigation into this recommendation. 

I need to talk with someone about this.
Hillary is due for her second dose of the meningitis vaccine now.
It was her 12th birthday last week.
Being fully up to date on vaccinations is a prerequisite for receiving the Solaris medication, but it accidentally maims people sometimes. Just take a look at Alexis, then please watch Netflix’s Take Care of Maya for me.

Hillary is presenting atypically. How can they know what’s going to happen to her? 

I am in for the fight of my life.
For her life.

All she wants to do is have a puppet show. 

Last night, Hillary cried in my arms. She said her stuffies are looking very dull. That when they’re sad, their colour fades. She said their colours get brighter the more you love them. That her unicorn can tell when she’s sick – that it’s reflected back to her like a mirror. I try and perk up their colour with kisses and love but she’s right. 

They are fading.

This all sounds very hard. I know.

I warned you years ago…this is a marathon, not a sprint.
I told you this wasn’t over.
I kept writing. Every three months.

I knew this day was coming. Most AA people do. 

We know that Hillary likely needs a bone marrow transplant to cure her PNH and AA. She requires a perfect 10/10 match and she needs to survive the secondary infections acquired in the hospital, in addition to any GVHD complications. She has been accepted into the transplant program at SickKids Hospital in Toronto and we meet with the team on July 7. 

It has begun. 

They are searching the global stem cell registry to see if she has a match. It’s been three whole weeks already, and we just don’t know how we are going to receive this news. She is asking why it’s taking so long. 

Maybe they forgot to look.  

There must not be a match there.
Of course there won’t be.
I didn’t do enough.
I wanted it way too badly. 

We have no idea if SickKids is going to deliver this news to us tomorrow morning. We won’t sleep tonight. 

We should tell Hillary on live TV. It might be time to let the world see this kid cry. She said she’s up for it if it means she gets to see Patricia Boal again. Canadian Blood Services is desperate for some good marketing to find a million new blood donors – well, here we are. If there is no match there, we are sure gonna need some creative help to find one before she dies on me. 

Cause that’s ultimately what we’re looking at here. I’m not allowed to have a saviour child like in My Sister’s Keeper to help ourselves. I have to beg on my hands and knees to anyone who will listen, yet, I can’t get the word out. I’m just Hillary’s “crazy” mother. 

Hillary is having a hard time being alone. I am not leaving her side. She said she just feels changed. The doctor made it feel like we are seconds away from an incident. We’ve cancelled pretty much everything and have two hospital appointments a week for the next eight weeks. She has gone very dark on me, with dark thoughts. She said she will never not be sad about this. 

She used to be the one holding the light. I am having to tell her again, like I did when she was fading at five years old….. to rage, rage against the dying of the light. Said by Thomas, but understood best in Children of Men, the best film ever made. 

I’m so grateful we held a bone marrow drive. Thank you to the beautiful people who supported Hillary for this day. Your time and effort are sincerely appreciated. She will not forget what you did for her. It will serve as a beautiful memory forever. She wants to be involved in helping herself and others.  

Hillary says it feels exactly like it did six years ago. 

It’s Mother’s Day. We’re scared. It’s her birthday. She’s in danger. Second and third opinions in Toronto and Montreal. A rare diagnosis with more questions than answers. The world on fire. 

But just like back then, we feel hopeful for the future.  

Because proceeding this way is unique. We accept this misfortune as a blessing. 

Typically, for AA/PNH patients, the hospital waits until the patient is in medical distress to initiate the transplant process – infected, receiving numerous blood transfusions, clinging to life. We are heading into the discussion with level heads and able bodies to help us advocate strongly. 

We still have Hillary with us. She is well.
She does not currently require treatment.
Medical professionals across Canada agree – she is different. 

We still believe in life-long remission from the treatment she received. There is still so much hope that Hillary may beat the PNH just like she beat the AA, without any intervention at all. Maybe Hillary will be one of the lucky AA patients who receives her ultimate gift from her own baby’s regenerative stem cells. Many AA patients go on to be healthy mothers – and blood donors. 

We believe in miracles. 

Please pray Hillary is healed through divine intervention. 

Please be there for us as our world keeps splitting apart. 

Please send strength to Hillary to get through whatever is next. 

Cause where we’re going, there are no roads.

We don’t wish to go gentle into that good night. 

Please don’t let Hillary disappear.

Help us fight for her while she’s still here. 

#StartWithHillary