Conor's story as told by his mother, Miranda McManus:
You can also follow Conor's story on Facebook at www.facebook.com/CaringforConor.
Look on the right of the screen and scroll down to the bottom of the photos to find a video that is four years old, so a little outdated, but tells the beginning of Conor's story quite well. It is an audio montage based on a one-hour interview, created and set to music by a local radio station for their yearly radiothon. This YouTube video sets the radio montage to pictures of Conor through those four years.
Conor was born a happy, healthy baby boy on July 14, 2005. He is the youngest of our crew with 3 older brothers and a sister. When Conor was getting ready to go for his 4-month well baby check, I started noticing that one of his eyes looked a little larger than the other. When we saw the pediatrician, she also took note of a large number of “birthmarks” that had popped up all over his body and remembered a disorder from medical school that concerned her. She immediately made an appointment with a neurologist that Friday (3 days later) and had a brain MRI scheduled for the same day. In my initial shock I said that we couldn’t make that appointment because we were flying out to go see my husband’s parents so we needed to reschedule. As I was driving home, I was like–what am I thinking!–I canceled the flights and got the appointment rescheduled back to its original time.
That Friday we found ourselves surrounded by a room full of doctors and med students with a diagnosis of Neurofibromatosis Type I….and it all began…
We were told that NF could be very mild. As a scientist, I went digging and knew all of the percentages, all of the possibilities. The MRI came back clear, and we were thankful, but not really, because our world had been rocked and of course we were imagining all of the things that could go wrong. We met other doctors, including a geneticist, and basically learned that Conor would be monitored closely, having another MRI in 4 or 5 months, and that we couldn’t worry about anything until we were faced with it.
When the next MRI came around, we were faced with it. Tumors had appeared in Conor’s right orbit, extending back into his brain, and we were immediately sent to a neurosurgeon who informed us that Conor needed to have a craniotomy. He also told us they would not be able to get all of the tumor because these tumors get all wrapped up in the nerves and the area of the brain in which the tumors extend is just too dangerous to operate. Not even 10 days later, they were cutting into my baby’s skull–straight across his head, from ear to ear–and we were in the ICU and hospital for the next week. Amazingly, he bounced right back after surgery; I witnessed for the first time the strength and resilience of my little boy.
Over the next few months Conor started visibly changing. His right eye started to bulge out and actually lower in level to a point that I emailed pictures to the neurosurgeon while we were on vacation. He immediately scheduled another MRI, which showed extensive growth of the tumors and that the eye was bulging out past the socket, so it was hanging over. In a week, he was back in for another craniotomy, this time with the help of a craniofacial surgeon for some pretty extensive facial and orbital reconstruction…not even 14 months old and he was in the hospital another week for another craniotomy. Another quick recovery from an amazing kid.
Three months later, the next MRI showed extensive tumor growth. We were told that he needed another operation, but that without someone who could operate around the optic nerve, there was nothing that the neurosurgeon could do. We visited an adult ocuplastic surgeon who was not really interested in getting involved with Conor’s case and, after meeting him, we didn’t feel comfortable letting him touch our child.
We were referred to Duke after speaking with other physicians where we met with a craniofacial surgeon, neurosurgeon, and an oculoplastic surgeon. A month later, I had still not heard anything and was told by one of the doctors after contacting all of them by email that they were tossing around Conor’s case like a hot potato–that his manifestations were rare, and that I needed to see someone who saw a little more of this type of thing. We were referred to the two best specialists in the country for pediatric eye tumors–luckily, both were in Philadelphia, so we were able to see them both in one trip. When we saw the second doctor, Dr. Katowitz at the Children’s Hospital of Philadelphia, we knew we were in the right place. He immediately walked us up to the neuro-oncology department to meet Dr. Belasco, and our treks to Philadelphia began.
They decided it would be best to remove Conor’s right eye. A little over a month before his 2nd birthday, we traveled up for Conor’s 1st surgery in Philadelphia; we were back six weeks later for a prosthetic eye fitting, and have been there at least every 3 months since. He has had numerous tumor debulking surgeries in Philadelphia and has been treated wonderfully by a very cooperative team of doctors. We had no idea at the time what a wonderful thing it was to remove Conor’s eye, but developmentally, he took off after it was removed. He was in so much pain, but could not communicate how much; we realized it when the pain was gone.
The summer of 2009, we spent most of our time in Philadelphia because an MRI showed significant tumor growth. They decided it was best to debulk the tumor, wait three weeks and start a yearlong IV chemotherapy protocol under a clinical trial. There is not any chemotherapy effective for shrinking these tumors, and some chemo and all radiation can actually cause the tumors to turn into an extremely aggressive type of tumor, so it is considered a success for the tumors to stop growing while on trial. He completed the year in September 2010. Following chemo, they did another debulking surgery in January 2011 to try to clear some tumor out and do some cosmetic fixes while the tumors were stable and less vascular due to the chemo.
A trip to Philadelphia in October 2011 showed no appreciable growth of the tumors in his brain, orbit, face, and neck, but that he has started having little isolated tumors pop up in other places. Luckily, these tumors are not as dangerous as the ones in his head, because they tend to stay more isolated, but seeing anything new show up is scary for us nonetheless. This trip also brought a surprise CT scan in addition to the normal MRI, and brought the surgeon to consult with a craniofacial surgeon to plan another surgery to debulk the tumor and do some pretty major facial reconstruction as well.
Conor had this surgery in March 2012. He was again cut from ear to ear--for the third time. They did extensive tumor debulking, but only in the orbital area, and drastic facial reconstruction involving cutting out three sides of his right orbit and grafting in bone taken from another part of his skull. He was in the hospital for the better part of a week, much of it on a morphine pump for the pain. The day after we were able to leave the hospital and go stay in a hotel, Conor took himself off of all pain medicine, including Tylenol. We remained at the hotel for a week before we were allowed to return home. Two days later, Conor insisted on going to baseball practice. A week later, he was leaving on a four-day field trip with his class to Williamsburg, VA.
A follow-up MRI in Philadelphia six weeks after the surgery brought some surprises. For the first time, the MRI showed an irregular finding on his left optic nerve--straight out of our nightmares...his good eye! His left optic nerve is tortuous, which is a precursor to the development of a tumor called an optic glioma. Another trip to Philadelphia over the summer brought us a new specialist that will need to follow Conor to detect any changes in Conor's vision. At this point, any change in the left eye on either his scans or in his vision buys him chemotherapy again.
We visited Philadelphia again in October 2012. This visit brought a three-and-a-half-hour MRI, this time one of the knee as well as the normal brain, neck, and orbits to search for a potential tumor. Thankfully, the scan did not show a tumor, and while that does not mean that one is not there, if it is, it is a superficial tumor that should not cause him any long term problems. The scan did rule out any bone tumors or plexiform neurofibromas, which is what the oncologist was concerned about. The scan of his brain did not show any appreciable growth of the existing tumors, and most importantly, no change in the left optic nerve. However, it did show some new signal intensity in the left basal ganglia in his brain that the radiologists read as a low-grade glioma...another tumor. We are thankful that Conor will not have to undergo chemotherapy at this time. Rather, we will wait and watch and see, but it is tough to know as we look at Conor that he has these ticking time bombs in his brain.
Conor's most recent surgery was Jan. 3rd, 2013, to further debulk the tumor in his orbit and upper part of his face. Dr. Katowitz walked out of surgery, handed me Conor's eye in the waiting room, and said that we were going to need to make an appointment to have another made before we came back home because that one would no longer fit. Luckily we were able to get in quick and only had to spend one extra day in Philadelphia.
Our visit to Philadephia in April 2013 was a whirlwind. Conor had an MRI and appointments with the ocuplastic surgeon, the neuro-ophthalmologist (who is monitoring his vision), the neuro-oncologist, and the craniofacial surgeon, who also sent him to see a neurosurgeon. He also saw the ocularist, the craftsman who makes his prosthetic eyes, who had planned to do some modifications on Conor's eye, but decided not to. The MRI showed "new" tumor growing along nerves involving his upper spine, the C5, C6, C7, and T1 vertebrae. The good news is, it is not currently in the "soft spine" (spinal cord), and it is not really new; the tumor was on the previous scan as well, but was apparently missed with all of the focus on everything else, and is unchanged from that previous scan--so if it is growing, it is not growing fast!
The craniofacial surgeon wants to perform another surgery. The access would be by craniotomy, which would require another zipper incision across his head from ear to ear, but this time his skull will be cut into and his brain lifted to access the back of the orbit from above. The point of this surgery is to place titanium mesh to mimic his dysplastic sphenoid bone (he was born without it). The hope is that this mesh will slow further progression of tumor into brain, and at the very least, stabilize the bone grafts from the last reconstructive surgery. We knew this was coming because he had discussed it previously, and because this is a major surgery and Conor's other physicians do not have enough of an understanding of the procedure to offer an opinion about whether or not this should be done, I consulted with the other best craniofacial surgeon that deals with NF (not easy to find!). He is affiliated with Seattle Children's Hospital and graciously agreed to look over Conor's case and offer an opinion without us having to take a trip to Seattle! He agrees that this is the best thing to do, and this surgery is planned for Nov. 25th, the Monday before Thanksgiving. He will be in the hospital about a week, probably still in the ICU on Thanksgiving, and will have to stay in Philadelphia for a total of about two weeks.
Thankfully, his MRI in July brought no surprises that would interfere with his surgery! Conor is excited about the surgery and is ready to get it done. How can you be excited about someone sawing into your skull?! I hope this lasts as he gets older. He's amazing!
This has been such a journey, and we have one amazing kid! It never ceases to amaze me how well he handles all of this–his strength is inspirational. He lives as normal a life as possible–plays sports, goes to school–and has a personality and smile that wins everyone over.