The Mighty Quinn
The Mighty Quinn was born on May 1st, 2006 in Ontario Canada.
Prior to birth there were no indications of any pre-existing medical issues.
Quinn, the youngest of three boys was born at term via planned C-Section. No medical conditions were noted with the exception of unusual bruising of unknown origin on the right side of his face. He was also born missing a significant portion of his right upper lip.
Within 24 hours the bruising quickly began to turn red and within 3 days of birth Quinn was seen by a paediatrician who thought Quinn might have a vascular birthmark and suspected it might be a port wine stain due to its flat appearance at the time.
One week later we took Quinn for a follow up with the doctor and she diagnosed him with a probable port wine stain and ordered many tests and referred him to specialists at a children’s hospital in Ontario, the closest one being an eight hour drive. We were told that Quinn would need to be screened for Sturge Weber to rule out any involvement with his brain.
At 2 weeks of age Quinn began to lose even more lip tissue which puzzled many local doctors and prompted many visits to the ER due to the extreme pain he was in. The only thing we were told was that it was chapped and that there was nothing they could do for the pain. At this point he stopped feeding and was screaming 24 hours a day in agony.
One day before he was due to see the paediatrician again Quinn had a very serious seizure which led to an admission at our local hospital and then an airlift to the children’s hospital 8 hours away when the seizures could not be stopped. Our son’s life was in great danger but we would come to know that it had not been nor would be the last time.
After an emergency Cat Scan and an evaluation by a large team of neurologists and dermatologists Quinn was diagnosed with PHACE Syndrome as opposed to Sturge Weber Syndrome (a very common misdiagnosis in the early days with a child who has PHACE) and we were told that he did not have a port wine stain but instead a hemangioma. It was a word I had never heard before but was about to become a part of our daily lives in a way we could never imagine.
After many tests we were shown the MRI images which showed that Quinn’s vascular tumour, or hemangioma was rapidly growing and consuming the better part of the right side of his face with much of it present under the skin. This rapid growth was causing his lip to ulcerate, breaking down the skin because it could not keep up with the growth of the vascular tumour. At this point he had lost all of his upper lip as well as half way towards his nose. A consultation with a plastic surgeon gave us hope that one day he would be able to have surgery to repair the disfigurement but at the time we were more concerned about keeping him alive which meant surviving the horrible seizures he was having and stopping the growth the hemangioma was having in order to lessen the strain on his little heart which already has two holes in it and had allowed a blood clot to travel to his brain before birth causing a serious stroke, which he somehow survived.
Quinn started a regimen of high dose oral steroids in hopes of at least slowing the growth and in an ideal world stopping it, but it was not meant to be. Steroids alone had no effect on Quinn’s hemangioma and within two weeks his face swelled dramatically and new ulcers were forming around his mouth. Upon seeing Quinn his medical team immediately suggested we put him on Vincristine a chemotherapy drug which had been shown to slow the growth in hemangiomas and we did not hesitate to give consent knowing Quinn’s situation was growing more serious by the day.
In July of 2006 at 2 months old Quinn began a yearlong treatment of Vincristine, 6 months of which he also was treated with high dose steroids. The treatment, mainly the combination of the two appeared to work for a few months as we held our breath because Quinn had many other health complications and scares during that time. In November of 2006 the decision to take him off of the steroids was made after an echocardiogram showed that they were contributing the thickening of the walls of his heart.
Within a month of being off of the steroids the segmental hemangioma began to rapidly grow again under the skin and on the outside with the thickening of many areas and continued to do so for a number of months while doctors struggled to find the right treatment for Quinn.
In the spring of 2007 we were advised to put Quinn on a drug called interferon which we were very against due to the high risk of neurological side effects. With numerous pre-existing neurological issues related to PHACE Syndrome we decided to get a second opinion and it was in July 2007 where we first met Dr. Milton Waner who not only gave us other options to consider but gave us hope that the severe disfigurements our son had suffered as a result of his hemangioma could be surgically addressed AND have a good outcome for him. He recommended we have Quinn taken off of the chemo as he had been on it for so long and did not think that it was wise to continue. He also recommended laser treatment something we had been unsuccessful at pursuing in Canada up until that point. Contrary to what we had been told by numerous doctors in Canada, Dr Waner stated that he felt Quinn would respond exceptionally well to laser treatment.
Fortunately we were able to get a referral upon our return to Canada to an amazing surgeon who was sceptical but was willing to try laser on Quinn on a trial basis. 3 laser surgeries later Quinn’s hemangioma was virtually gone on the outside and the surgery seemed to kick start the regression of the tumour on the inside.
At that point we decided to schedule a follow up with the surgeon we were always led to believe would operate on our son. He then referred us to a colleague for some advice on the lip reconstruction and we were feeling encouraged.
For 6 months prior to that day we had watched as our son as we knew him slipped away, as he regressed developmentally into a world which we quickly learned was Autism. At this point Quinn had had almost 30 surgeries, countless complications and life threatening emergencies and 3 16 hour round trips a month to see specialists. We needed to hear good news that day but it was not to be. We instead left heartbroken and bewildered after being told that “there was nothing they were prepared to do for our son” that it was a blessing he had autism because it (the disfigurement) would never bother him and the kicker, that it could not be seen from far away so why were we concerned about it.
We were devastated, angry and confused because our son had had such amazing treatment in all other areas but when it came time for the plastic surgery he was promised, the rug was pulled out from under us in a way that left us heartbroken. We have never spoken publically about why Quinn was not able to get surgery in Canada until now.
Fortunately Quinn’s story was a familiar one to a number of people in the vascular anomaly world due in part to the severity and complexity of his case and his family’s desire to share his story with others in hopes of helping just one person, so word of the refusal to treat Quinn in Canada spread quickly and it was not long before word got to Dr Waner who told us he wanted to help Quinn and he gave his word that somehow he would make sure it happened. For the second time Dr Waner gave us hope, hope that we had lost.
Soon after Dr Waner pledged to help Quinn we were contacted by Sherri Foster the now director of the Waner Children’s Vascular Anomaly Foundation who explained the foundation she was setting up with Dr Waner and told me that they wanted to help Quinn through their new organization. We were blown away be the generosity and benevolence of an amazing group of people which grew to include the amazing Dr Aaron Fay who then set out on a mission to make this happen.
On April 16th 2009 Quinn walked into the OR in New York City an unresponsive 3 year old with Autism who had lost the twinkle in his eye and rarely smiled unless he was being tickled. He walked in a child who HATED to look in the mirror and who had been called a monster by some, a freak by others and was often the subject of many pitiful stares. Yes he was a child with Autism, but he was also a child with a facial disfigurement who was very aware of it.
Dr Fay saved Quinn’s eyesight that day by removing a large orbital hemangioma that was causing discomfort and pressure and often was the cause of him being unable to open it. Dr Waner did the first reconstruction on Quinn’s lip removing some of the hemangioma and damaged tissue and connecting it with healthy tissue giving him the first sign since birth of a normal appearing lip. We thanked him that day for helping to give Quinn back his smile but we were unaware of how powerful those words were.
The day after his surgery he was released and we brought Quinn to his hotel room to recover for the next week. That night in the room he walked up to a full length mirror and we held our breathe as we waited for his reaction because he looked pretty rough after the surgery, but to our amazement and what I like to call a miracle, he smiled as he touched his lip and then he turned to look at us (his parents) and flashed the biggest grin we had seen since the day his Autism regression began. Not only was he smiling suddenly but the twinkle in his eye we longed for was back. We began to take pictures and he would not let us stop, he was posing and we were in awe of the sudden and dramatic change in our child.
The surgery and time in New York led to a period of growth socially when we arrived back home and continued when we made our next visit to New York for surgery in July of 2009 where Quinn had liposuction of the regressed hemangioma from his face and fractional laser to help resurface some of his extensive scarring.
Fast forward one year later and our remarkable little boy is still smiling with the beautiful little mouth Dr Waner created for home and looking at us with two twinkling eyes thanks to Dr Fay.