During a routine ultrasound while pregnant, it was discovered that our baby’s kidneys were enlarged & covered in cysts. It was thought that our baby would have PKD – Polycystic Kidney disease, usually a genetic disease where fluid-filled cysts cover kidneys. PKD is rare in newborn babies, it usually discovered at a much older stage in life. We were told by doctors that due to very low levels of amniotic fluid during pregnancy that our baby may not survive her first 48-hrs. Amniotic fluid is needed for the baby to develop healthy lungs.
At 37 weeks, Olivia Grace was born. It was instant love. Our beautiful little girl had a mass of golden strawberry blond hair & big dark eyes. We were absolutely heartbroken that Olivia was immediately taken to the neo-intensive care unit & placed on breathing assistance. Olivia was in the beginning stages of kidney failure. The emotionally journey of having a chronically ill child had begun. At 1-month old while in NICU, Olivia’s blood pressure was skyrocketed, her electrolytes were all over the map & she was no longer clearing toxins from her body. Olivia’s kidneys had shut down. Olivia was transferred to the PICU where we were devastated to see our little innocent baby fighting for her life. We were terrified we were going to lose her. Continuous Run Therapy would run for 24 hours a day for over a week to help clear the toxins in her blood. Blood pressure would be monitored every few minutes. Eventually therapy would transition to daily hemodialysis. Step by step Olivia was building strength as fluids were clearing. Therapy was working,
Olivia would need to receive long-term dialysis until she grew big enough to have a kidney transplant.
Over a period of time Mom & Dad were trained by the nephrology nurse to learn peritoneal dialysis. Peritoneal dialysis could be administered at home until she reached the target weight & height for transplant. After 97 days in the hospital, Olivia was discharged. Olivia would be connected to the PD machine for 16 hours each night, eventually moving down to 12 hours each night as she grew bigger.
Each day after treatment, we try to keep Olivia as active as possible to help build up her strength with physiotherapy. Due to Olivia’s long illness & low muscle tone, Olivia is unable to crawl, stand or walk. All of Olivia’s nutrition is by g-tube as she has a severe oral aversion & reflux due to kidney condition.
Olivia's care is a constant balancing act & exhausting. Daily monitoring of blood pressure & weight is needed, along with frequent blood work to ensure electrolyte levels are stable. Olivia has been through several hospital stays, where she has endured countless surgeries, blood transfusions, infusions & serious peritonitis infections. There were times where we were so close to losing her.
Through genetic testing, it was discovered that Olivia has a rare disease caused by a mutation of the NPHP3 gene. The condition is called, ‘Renal Hepatic Pancreatic Dysplasia Syndrome’, (Ivemark’s Syndrome). The condition is so rare that only 8 cases are documented in medical books worldwide. Olivia is a mystery to the medical world. Unfortunately this disease has also affected Olivia’s liver. Olivia has liver cirrhosis, portal hypertension & is also cystic. Eventually Olivia’s liver will go into failure, needing to be transplanted.
Through these past 2 years, Olivia has shown us what an unbelievable fighter she is. Throughout each difficult procedure she is a happy little girl with a huge personality. She is gaining strength each day. Olivia has now reached the stage where medical work up is starting for transplant. At this time, we are unclear whether her transplant will be a dual liver & kidney transplant. Transplant is not a cure, but it can bring her a life of freedom from being connected to a machine for hours each night. She will be facing lifetime of challenges with having chronic kidney disease, other transplants will be needed in her lifetime. We are hopeful that Olivia will build enough strength so she can walk, attend regular school & simply have a simpler life.
Thank-you for following Olivia’s story, we thank everyone who has come into our lives, who have supported Olivia’s fight against this devastating disease. As our direction in life has changed, we bring our passion into bringing awareness to Kidney & Liver Disease and organ donation.
For updates on Olivia:
Information on Becoming A Kidney Donor;
To be tested to become Olivia’s Organ Donor, your bloodtype needs to be B+/- or O+/- & in healthy physical condition. Fill out this form & send it to Toronto General Hospital.
For further kidney information & to help pediatric kidney patients in need, please donate to the Kidney Foundation of Canada in Olivia's honor, ‘Olivia Grace Pullen’;
Canadian Liver Foundation:
For more information on Polycystic Kidney Disease:
Take 2 minutes to register yourself to become an organ donor, you can save up to 8 lives: www.beadonor.ca
Help Build A New Nephrology Clinic at McMaster Children’s Hospital, ‘Caring For Little Kidneys Campaign’:
Information on the Kidney Transplant Program at The Hospital for Sick Children: http://www.sickkids.ca/TRMC/patients-families/Kidney-Transplant-Program/index.html
Let's be honest. I never expected to have a "complex child". Not for one minute while pregnant, did I consider that I may sit in an OR waiting room 14 times in two years praying for my son. You want to know the truth? Right before I went into labour, my worries were that I needed to apply waterproof mascara so that I would look beautiful holding my baby in our first photo and I was upset because I had yet to buy a crib skirt. I only held my Maksim a moment before he was whisked away without explanation - my mascara, although long-lasting slid down my cheeks and the crib skirt didn't matter anymore because it was almost three months before he came home.
Four hours after his birth I did not know my son's weight or length; instead I was told things like he may not survive, that they think he has a rare syndrome, that they have already had to save his life repeatedly and that he is being transferred to McMaster Children's hospital.
Maksim was tested and labeled with Pfeiffer's syndrome. It was a sporadic gene mutation of his FGFR2 growth receptor. Pfeiffer's affects the way the bones in the skull, hands and feet form which can lead to many complications. It is rare and it is frustrating because there are a lot of unknowns. Treatment in Maksim's case (and in most) is almost always surgical. As Maksim continues to grow additional surgeries will be required.
Nothing is what I expected it to be like. The bad parts are bad, I will admit that. I don't want Maksim's suffering to be in vain but you know what... the good parts are truly amazing! All three of us smile through the tears and keep trucking along. We deal with things as they arise and do our best to support each other and give our son the best and happiest life possible.
I am pretty sure I am not a good blogger. This entry is definitely full of missed opportunities to share because I am new at this idea of being open! A main goal of this website is to spread awareness so I guess if I am going to ask others to share, I have to as well. I think the reason I have been so guarded was because so much was happening so quickly and unexpectedly when Maksim was first born. Right or wrong, we felt it was our job to be strong for each other, our family, friends and most of all for our baby. At the time, sharing a lot of information we thought, would have solicited more panic, negativity and "poor Melissa, poor Daniel" The love and support we received is worthy of its own blog entry but unless one was there day in and day out, I think it would have been hard for one to understand the beauty that was also happening within those hospital walls. Despite all the crowds of doctors, wires, tests and surgeries, it was still our family. Daniel and I were new parents, bathing Maks, feeling his soft skin, holding him, adoring him and appreciating his life in ways that perhaps only parents that have experienced fear of loss could relate to. I still can't explain how the hardest, most stressful, painful time of my life, could be so beautiful.
Once finally home and trying to adjust to our new normal, it was and is challenging to go back into that hospital life for appointments and surgeries. We start to believe that it is behind us and that we can focus on regular day to day things and then BAM, another hospital stay. What gets us through? Where and how do we have the strength? Our Faith, our son, each other and everyone that loves Maksim. My son wakes up smiling and it is highly contagious. If you have met him he has probably stolen your heart therefore he and we have a tremendous support group of family, friends and medical staff constantly sending their encouraging words, prayers and love.
Don't get me wrong, I wish with all my heart that my son didn't know this suffering but I do believe that my husband and I have been given a gift. We are able to appreciate and cherish each moment and ability Maksim has; nothing is taken for granted. I am much more open with regards to my son now since I can see clearly that his story is one of resilience not sadness. The happiness and love just radiates off Maksim and it inspires and brings joy to all around him. For one moment, when he was first born, I will admit I thought, "Why me?" Now "Why me" is in the context of "What did I do to deserve the honour of being Maksim's Mama?" I am so proud of my son and I am ready to tell anyone that wants to listen.
Thank you for taking your time to read this.