It’s hard to believe that it has been over six months since my last update. Abbigail has fought so hard, to come so far, over the last four years, but she has made most of her leaps and bounds these past few months alone.
Just over six months ago Abbigail was once again facing huge life threatning battles that no child should have to. She was admitted to the hospital for a septic blood infection that was, without surprise, rare and not easily treated without strong antibacterial infusions. She spent three long weeks in hospital, mustering the strength to push out the monsters threatning her body and ultimately her life. Once the infections were under control, we were back to discussing the multiple and progressive nodules forming in her liver. Were they an anomaly or more cancer? No one could be sure because nothing with Abbigail has been text book up until that point and everything documented to date has not been able to discount further neoplasm (cancer). After numerous challenging and cautious fine-needle biopsies, we faced the dreaded consent form for an open liver biopsy and possible resection. Slicing and dicing a liver is never simple or clear cut and isn’t without immense risk for morbidity and even mortality. An other human being, possibly a father himself, had to ask us specifically if we understood that once we left our baby girl in his care, on that cold table, that she possibly may not wake up or that if she did, she would not be the same. Is this even part of real life? Often I find myself wondering these questions. That morning, I signed my name on the dotted line, giving consent to surgeons to open our daughter’s entire thoracic area and examine it for further cancer or at the very least remove a portion for lab analysis. Then I waited. I waited and stared at her empty bed on the fourth floor of CHEO, a place we’ve considered home for far too many years and I waited for the nurse to say she was out of surgery. When the desk clerk’s phone rang that time, I knew it was about Abbigail. I dashed to the elevator, flush, nauseated and petrified. What would he say as he removes his mask and sterile cap? What happened these last few hours in that cold sterile room? Where is my girl? Before he could speak I layed my eyes on her ragged body in recovery and sighed in relief. I sat at her bedside until her intubation tube was removed and she was awake enough to know I was there. The surgeon who has cut into our daughter more times than I can count, along with the radiologist who has invested numerous days and hours analyzing her multiple liver images and studying her nodules, smiled as they both confirmed that surgery was a success. They also confirmed the lab had enough liver sample to examine and diagnose these nodules without having to fully resect an entire lobe of her liver. After a long few days of epidural pain management, drug reactions and rehabilitation, we were finally transferred from the surgical and rehab unit back to our “home” unit on the oncology ward. Ten long days later, we received exhilarating news that it was not cancer but that further lab testing was required from SickKids hospital in Toronto before we could know exactly what the lesions are. Abbigail was feeling better, not eating much by mouth and still recouperating from an extensive thoracic surgery, but we still could not be discharged. Now, almost a month in hospital, barely home a day or two from our Florida trip, realizing we are never free from this world as we face our worst fears all over again. After much debate and review from many specialists, near and far, Abbigail was diagnosed with a rare disposition of extramedullary hematopoiesis. Abbigail received large doses of chemotherapy last year, and the theory is that her body has not been able to keep up with blood production post transplant and her bone marrow now is producing blood cells outside of her marrow (i.e. in her organs). This new diagnosis only added to her complicated medical care. Two incurable rare diseases battling inside of our five year old little girl’s body, that has yet to recover from years of cancer treatment and a life altering stem cell transplant that ultimately saved her life last fall. Her scars are gigantic – physical and emotional, but we finally went home after a long month of close calls in hospital. Despite being “out of the woods,” Abbigail now faced new hurdles and we didn’t know then just how big they truly were.
Abbigail had more than a hundred nodules confined to her liver. Continuously monitoring their activity with imaging and although they had not stopped multiplying, they had yet to spread to other organs. Also closely monitoring her liver health with regular blood work to ensure that these lesions did not affect the basic functions she desperately needed. Abbigail was tired, her entire body covered in cuts, bruises, catheters and scars. Smiling for photos with an NG tube still taped to her sensitive cheek, she didn’t let any of this stop her this summer. We spent every day possible, that we weren’t at CHEO, at our camp. Roasting marshmallows, wading in the waters, making forts and sand castles while chasing the boys. She even made a few new friends and won the hearts of every single other camper in the park. There wasn’t anyone who didn’t know who Abbigail was after her first week there. Memories were cemented in our hearts and despite the hurdles, the pain and sacrifices that don’t cease, we were blessed with an entire summer without a single night in hospital!
Once camp season came to a close and the leaves began to fall, Abbigail started school! Something I never allowed myself to think of again after last year because we were so focused on her treatment and survival. She has been surprising us and everyone else with her abilities and potential. She is speaking so many more words. She can now even understand French a bit. She has learned routine, made friends and been able to detach herself from me. Her learning is greatly affected due to the extensive damage to her brain from the OMS as well as the four years of dozens of repeated chemotherapies, but that does not stop her from always trying. She is part of a regular classroom with all of the support she requires to thrive. We are so proud!
Our spitfire never lets life settle down though, just as she adapted to the new routine, stopped crying when I dropped her off and began to have less OMS episodes at school we were facing difficult decisions regarding her overall health. Abbigail had started losing weight very quickly and without reason other than her appetite and eating habits. Post liver surgery, they inserted an NG tube for her nutrition because when a child has such a huge abdominal surgery, they are not able eat for days due to their “guts” being stunned and paralyzed, meaning unable to digest. So after a week of not eating, having huge stomach pain inside and out, her appetite that was finally returning post transplant had now taken a nose dive. Since June, she continued to lose weight and struggle with her appetite. In September, she had lost a few pounds and it was evident now in her face and it wasn’t long before doctors and specialists were considering a more permanent feeding solution. Abbigail was 50lbs in the summer and although it was an unhealthy weight for her height, we knew it was temporary and that it was caused by steroids. Presently Abbigail is holding at 35 pounds.
That 15 pound lose in a short period of 3 months had us remove the NG tube (in her nose) and have a surgically implanted g-tube directly into her stomach. After almost 5 months without a night in hospital, Abbigail and I were cuddling under nurse and doctor supervision, in the very familiar and oddly enough, comforting walls of her second home once again. December 7th she received her new “tubie” and again our trusted surgeon was successful in cutting through the layers of scar tissue in her abdomen to have the new device inserted. Only a few days to recover from yet again an other stunned tummy surgery and we were home to begin the holiday season with new hope for recovery from yet an other hurdle in the journey.
This Christmas should have been different though. For so many reasons. We never imagined still being in such a fight for our daughter and we never imagined experiencing the loss that we have. Being a part of a world where kids have cancer and it is normal, becomes scarier the longer you are in it. You form friendships, extend your family and fall in love with the strongest, bravest and most beautiful young souls that walk the earth. When things are good, in cancer world relative terms of course, we laugh, have private little room parties and make today count because we know how fragile it is. We often fall prey to thoughts of tomorrow and we allow ourselves to believe that it will all be ok one day…that is, until it’s not.
November 18th was not ok. Abbigail’s closest friend earned her angel wings far too early. Phoebe left this earth, forever 5 years old. She left her pain, suffering and sadness behind with her grieving mama, daddy, big sister and many family and friends. For Phoebe’s family, tomorrow is now too hard to face because today is already so painful without her here. My heart has been aching since that day. Abbigail understands Phoebe is gone, that we can no longer visit or play with her and that we can only see her in our photos and videos but she often asks “but mama where Phoebe?” It breaks my heart on so many levels. Losing Phoebe was harder than I could have ever imagined it would be. Christmas this year was bittersweet. We weren’t in intense treatment, there were no life-threatening emergencies or hospital sleep overs and for that we were blessed and grateful. But we also felt a huge sense of lose and guilt. Every twinkling light had new meaning, each gift unwrapped was a reminder that not everyone was so lucky. The kids were spoiled and had “the best Christmas ever” visiting family and making memories but I couldn’t help but carry the guilt with me. My friends also got to spend the holidays out of hospital for the time in five years too but for very different reasons. Phoebe’s passing hurt. It was painful. Unfair. Phoebe lives on though and will forever be our Christmas angel, reminding us to never lose faith and hope, just as she never did! As the years pass, treatments continue and Abbigail grows up, she will forever have Phoebe in her heart, watching over her…that I believe.
Today, Abbigail continues to receive immunosuppressive therapies both in hospital and at home. She goes to CHEO for infusion treatments every two weeks and continues with her daily oral treatments at home. Her medication list, for treatment and for symptoms caused by the treatments, is still very long and disheartening but in a world where there is very little known about her disease we do not have many options left. We are currently trying to slowly taper her off of the very aggressive steroid treatments she’s been on for four years in hopes that we don’t create the perfect storm in her body for an OMS relapse. If she regresses once the steroids exit her system completely we will have hit a wall in treatment options.
For those new to Abbigails journey or for anyone who is still confused about OMS, it is a rare (1 in 10 million) disease that presently is without a cure. Children who are doing well with OMS today are said to be in “neurological remission” however are prey to relapse or regression at any time and it is believed by the few specialists in the world, that these children will suffer several of these relapses in a lifetime. A regression could present itself as slight shakiness and imbalance, darting eyes, uncontrollable and aggravated behaviour, insomnia, muscle jerks and trunk or full body ataxia. These small symptoms can be triggered by any immune response; which are fatigue, stress (physical and mental), certain medications and sedatives or the more obvious, infections. Once the immune system is triggered, Abbigail’s brain is caught in a crossfire. Simply put, OMS is an immune disease characterized by her immune system being programmed to attack particular cells which were present in her cancerous tumours but that are also present in her brain. This means that part of her brain, the part that controls movement, stability, speech, some learning and development as well as behaviour and sleep, have the identical cells that her cancer has. When Abbigail’s immune system is activated, it begins to fight and destroy the cells in her brain, causing multiple cells to misfire and often creating the perfect environment for brain damage and that is when we begin to see the symptoms described above. If the damage caused is extensive enough, the regression in Abbigail’s abilities and functions could quickly become a relapse in the disease, requiring more than patience and antiviral or antibiotic treatment. During a relapse, OMS must be stopped as quickly as possible to minimize the permanent brain damage that is occurring. At diagnoses, the peak of her illness, and when she has relapsed in the past, She has completely lost the ability to speak, slurring her sounds, has also lost control of her trunk, losing the ability to even sit up on her own, she’s even lost her ability to walk and crawl during relapses and self feeding was impossible. This is when we would begin more chemotherapy, new experimental drugs and stronger immunosuppressive treatments to stop her immune system from functioning, putting her back in that vulnerable and dangerous state she knows all too well.
So this is where Abbigail finds herself in treatment today. Hopeful we can wean her off of the treatments that are affecting her growth and long term bone health but also fearful to remove the drugs that have kept her brain safe from relapses over the last two years. She has done each and every treatment protocol and trial available to children in Canada and the US to date and is still unable to wean from the destructive drugs keeping her immune system at bae.
I can’t thank you all enough for your support and prayers throughout this journey and for checking in over the last few months during my online absense. Abbigail and our family is continuously blessed by you all! Thank you!