Quinn Michael Duncan was born on May 1st, 2006 in Sault Ste Marie Ontario Canada at 38 weeks gestation via planned cesarean section. Quinn is the youngest of three wonderful loving little boys.
At about 8 months gestation Quinn's fetal heart rate became very high. I was repeatedly put on a fetal heart rate monitor as the rate was well over 200-210 at times. Ultrasounds and blood tests were run, but no cause was found.
I had a number of ultrasounds done. We noted in each ultrasound that Quinn would not show the right side of his face. The technologists would repeatedly try to coax him to roll over but we never saw the right side.
There were no complications at birth and his apgar scores were 8 and 10. The only thing that was noted was that he appeared to have some bruising on his face surrounded by what appeared to be a white outline. (Known in the vascular anomaly world as a “halo”) Within the white outline there were many many tiny blood vessels which were very visible on the outside. A paediatrician was called to come and take a look. His right upper lip was also malformed, missing 25% of the tissue. I also noticed that he did not appear to have a rooting reflex on the right side of his face and did not respond to touch in that area.
The paediatrician thought it may be a birthmark and wanted to see him in a week to be sure. We thought nothing of this and brought our beautiful boy home 3 days later.
Three days later it became obvious that the "birthmark" was here to stay. I began researching birthmarks and I was introduced to an entire new world of vascular anomalies and associated syndromes. I was devastated to learn that my son might have more than just a birthmark. The term Port Wine Stain (A Vascular Birthmark) had been used so I was determined to learn all I could before his appointment. This is when I learned Quinn might have a syndrome called Sturge Weber. The odds of this were low and he hadn't had any seizures which are common with that disorder so I was cautiously optimistic.
The next day Quinn's doctor confirmed that it was in fact a vascular birthmark. She referred him to a Pediatric Dermatologist for an evaluation. At that moment our lives changed,but we could never have been prepared for just how much. She referred Quinn for a Cat Scan and an eye exam to look for disorders associated with Sturge Weber.
That night I noticed what appeared to be twitching of his hand and foot. I became concerned because Sturge Weber can include seizures. I wasn't sure if he was dreaming or if I was imagining it. During this time he developed what we thought was either a chapped upper lip, or a nursing blister, which just kept getting worse and more and more painful. It bled constantly.
Progressively over the next two weeks, my husband and I noticed this "twitching" Everyone kept telling us he was just dreaming. Then a few of them witnessed it and we decided to try to video tape the next one. The next one lasted almost half an hour and was much more pronounced. We were terrified.
We rushed Quinn to our local emergency room and he was admitted. He continued to have what were then confirmed to be seizures,despite medical intervention. Quinn and I were flown by air ambulance to The Hospital For Sick Children, in Toronto Ontario which is 8 hours from our home. Quinn's daddy drove down behind us, not knowing what he would find when he got there.
Quinn was promptly seen by the Neurology team and given a Cat Scan. I will never forget that day, though I wish I could.
The first thing the Neurologist said to us was that Quinn had,had a stroke just prior to birth and it was the damaged area that was causing the seizures. This was a very surreal moment for me. My younger brother had also had a pediatric stroke which happened during his birth. I could not believe that history was seemingly repeating itself.
As a result of the stroke we were told that Quinn had weakness on his right side and does not have full function of his right hand,thumb and shoulder. This was unexpected, but was only the beginning of what we would soon learn.
We were told that Quinn had PHACES Syndrome a very rare disorder that affects the brain,heart,eyes,skin and as we learned later, (In Quinn's case) much more. It was discovered by the CAT Scan that Quinn had a hemangioma, not a port wine stain. The hemangioma (a vascular tumour) was present not only on the surface of much of the right side of his face but also deep within his skin in the cheek area. He also has a number of internal ones as well in the neck and nasal areas.
It was then discovered that Quinn has a brain malformation. The right side of his cerebellum is significantly smaller than normal. (called a hypo-plastic right cerebellum) The cerebellum affects movement and co-ordination.
Quinn was started on Prednisone (an oral steroid) to help slow the growth of the Hemangioma. He continued to take high doses of the steroids for 6 months at which point he was weaned due to some thickening of his heart.
He also began taking aspirin every second day to help prevent another stroke as they were unsure of what the cause was. The cause of the stroke has been the subject of debate since the beginning but the current consensus is that it was caused by a blood clot in his heart.
At this point Quinn was also in severe pain from what we had thought to be a chapped lip originally. It turned out to be ulcerated and the lip was rapidly losing tissue. Plastic surgery was consulted and they stated that nothing could be done until the hemangioma involuted (regressed) Quinn lost all of his upper lip and tissue half way towards his nose. Fortunately much of it did grow back but he still faces a significant deformity with much scarring.
Quinn then had an echo cardiogram done to evaluate his heart, as a murmur was heard and he had had episodes of tachycardia even before he was born. The echo revealed that he had two small holes in his atrium called atrial septal defects.
Quinn was released after two and a half weeks. He hadn't had a seizure in 10 days (He was put on Phenobarbital) and they were confident they were under control. We soon learned that with Quinn, expect the unexpected.
During his follow-up visit a month later it was discovered that his hemangioma was growing rapidly. The cavernous portion under his cheek was very big. His ear canal was closed off and he could barely open his eye on the hemangioma side. His right nasal passage had narrowed and he had a noisy stridor. He had also developed more ulcerations on his face and was in severe pain which was not being controlled with codeine. He was in agony and it was breaking our hearts.
Quinn was hospitalized and had surgery to put in a PICC line through which he received a chemotherapy drug called Vincristine. He also developed a Staph infection of his ulcerations and was on a morphine infusion to control the pain. Quinn began to have seizures again. It was determined that the steroids and the chemo drugs were affecting how the seizure meds worked. He was changed to two other drugs (Gabapentin and Clobazam) and was closely monitored in hospital as he had "break-through" seizures.
Quinn was on the chemotherapy for exactly one year. At the end of that year the hemangioma was still significant but we did not feel it was life threatening any longer so treatment was ended.
His surface hemangiomas have involuted considerably with the help of three pulse dyed laser treatments and natural regression. The deep hemangiomas have also begun to involute in his face. We are awaiting an MRI to see how things are looking on the inside.
At 6 months of age we learned that Quinn has 1 lesion ( tumors) inside the brain. It is called an intra cranial hemangioma as it is inside the area contained within the skull. It is on the right side in his Internal Auditory Canal. At age one this tumour was stable. We do not know if there has been any change since coming off of the chemo and are presently awaiting an MRI to investigate this.
Quinn's seizures are almost non-existent at this time. We only see occasional myoclonic jerks or short absence seizures. He is now completely off of all seizure meds.
Quinn had many issues with the PICC line (a type of central line) he had three different ones over the course of his treatment. One PICC line broke and part of it ended up in his heart and lungs, prompting an emergency surgery to retrieve it. Once again he was flown by air ambulance to Toronto. His last PICC line repeatedly became infected and had to be pulled. He had surgery to implant a port which we are currently waiting to have removed.
Physically, Quinn faces a number of challenges, in relation to the stroke, brain malformations and tumours. He is beginning to use his right hand much more often and is gaining strength on his right side. At 11 months of age he was able to sit unassisted and at 13 months he was able to completely roll over. At 20 months of age, 2 days before Christmas he stood on his own two feet giving us the best Christmas present ever. We are confident that one day he will walk.
Quinn faces many obstacles in the speech and language areas. This area was damaged by the stroke. His development in this area is very behind but he has a fantastic speech therapist who is working very hard with him weekly.
Quinn has some GI issues and a vitamin D deficiency and is being followed by a GI specialist.
Recently a number of obstacles have confronted Quinn and he will be going through a number of assessments to try and figure out what is going on so that we can give him the best possible start. He will be assessed soon for Autism which has become our latest concern. He also suffers from migraine, one of which saw him hospitalized this fall.
Quinn is followed by Neurology (Stroke), Neurology (Epilepsy), Neurosurgery, Hematology, Oncology (Toronto),Oncology (Sault Ste Marie), Dermatology, VAST, Plastic Surgery (Sick Kids), Plastic Surgery (Laser at an outside hospital) Cardiology, Opthalmology, Ocu-plastics, Developmental Pediatrics and GI. Quinn also has a local team consisting of an Occupational, Speech and Physical Therapist he sees weekly. We travel to Toronto (8 hours away) 1-3 times a month.
The process of reconstruction has begun. He has had two laser surgeries to date and will be having surgery to reconstruct his eyelid in the near future. He has ptosis (drooping of the eyelid), ingrown eyelashes and a lid that has been damaged by the hemangioma. It is interfering with his eyesight (he has astigmatism and ambloypia) and the lashes are causing great discomfort and there is a risk of scratching the cornea. Reconstruction of his lip and mouth area will be done at age 3 or 4 before he goes to school. He will also have resurfacing of the scarred areas at a later time.
Quinn is a wonderful little boy who just happens to have more than a few obstacles. They may be big ones, but that is not stopping him. Nothing has stopped Quinn and we have learned that he is the toughest little man out there. He has indeed earned his nickname, "THE MIGHTY QUINN"
Many people have been following Quinn's story and we feel incredibly blessed as a result. We are often told that his smile has endeared him into the hearts of many. We consider all of our new friends to be an extended part of our family. Thank-you. Thank you for supporting,praying for,loving, and for seeing what we have seen all along, not a boy who is defined by his "disorders" Though he is mighty in many ways, most importantly, he is Quinn.
Thank-you for taking the time to read Quinn's story. It is far from over so please stay tuned.
......to be continued.
QUINN'S STORY: THE FIRST YEAR