Questions and Answers

I would like to address some of your questions that you have posed to me, so here goes. I will answer them to the best of my ability…

Q: Did you know before Spencer was born that he had this coronal synostosis?

A: No, we did not. Apparently this kind of condition cannot be seen in an ultrasound or in the blood work that is done during pregnancy.

Q: Do the doctor’s know when it happened?

A: No, they said it can happen early in the pregnancy or right before birth. There is no way of knowing.

Q: What causes right coronal synostosis?

A: So this is an involved question. Our understanding is all couples have a baseline risk of 3% to have a child with a birth defect that will require medical or surgical attention.
Non-syndromic cranio synostosis is typically a chance event that occurs as part of this background 3% risk.
Syndromic Cranio synostosis includes a group of conditions that can be inherited from a parent or start as a brand new condition in a child. These conditions involve fusion of multiple sutures and are associated with additional physical differences, commonly involving the face, hands, and feet.
Spencer has the non-syndromic one which we are thankful for and to quote the doctor “there is no rhyme or reason why this happens” it is a rare birth defect that occurs every so often and they still have yet to determine why.

Q: How long is the surgery and the recovery? 

A: We were told the surgery is about 4 to 5 hours long. There is a one night stay in the NICU to monitor the baby’s fluids and then usually a five day stay in the hospital to watch the healing and swelling. The baby’s eyes usually swell shut and remain so for about 4 days. Once the babies open their eyes they are sent home for the rest of the recovery. I’ve been told that the recovery at home is usually about another 5-6 days and then they return to their “normal” self. 

Q: Does unicoronal or right coronal synostosis affect the brain? 

A: Cranio synostosis is known to be a cause of increased intracranial pressure and children with one prematurely fused suture, such as unicoronal synostosis, demonstrate elevated intracranial pressure in 14% of cases. In Spencer’s case it does not affect his brain in any way. It is purely a cosmetic/aesthetic problem. Which we are also very thankful for!

Q: Is a blood transfusion necessary for this surgery? And can a parent or family member donate the blood?

A: Spencer will require 2 units of blood to be available for this surgery. In most cases they will use this blood. It has happened in the past that the baby did not require a transfusion but we are told this is very rare. Yes, we will have the opportunity to be a direct donor of blood if one of us meets the criteria. We have to be...

1. Compatible with Spencer’s blood

2. Healthy

3. Meet the criteria of the Canadian Blood Services

I thought that one of us would have to be a match for sure but apparently this is not the case. If we are not a match they will use blood previously stored by the Canadian Blood Service. They do not allow blood from family members only parents. Why this is I don’t know.  They said that is just the way it is. I am hoping that my husband is a match as I mentioned earlier in my blog, the Canadian Blood Service is not collecting or using female plasma so if my husband is a match Spencer will get all of his blood but if I’m a match he will only receive a part of it.

From Mother to Mother…

First and foremost I want to thank everyone for all the wonderful messages and comments that you have sent. The outpouring of love, support and best wishes has been overwhelming. I will address some of your questions later in my posts but for now I would like to share an experience that we recently had.

When I told my cousin about Spencer’s coronal synostosis she immediately told me that she knew someone whose daughter just had the same exact surgery and told me she would put us in touch. So the very next day I was given a phone number to someone named Deborah. At first I was really apprehensive about calling her as calling strangers is not something that I am comfortable with. But I quickly realized that I had to put aside my fears and pick up the phone.  It would probably be worth a phone call. Needless to say it ABSOLUTELY was. I could not believe how welcoming, supportive and forthcoming Deborah was. Our conversation flowed as if we have been friends for years. It turns out that her daughter, Chloe, has a different but similar condition. Her daughter had what they call Sagittal Suture Craniosynostosis (this is the condition where the suture running from front to back of the skull is fused) the surgery is not exactly the same however it is very very similar. Her daughter was only five months old when she underwent surgery. Deborah talked freely about the whole ordeal. She shared her fears, she shared information about the actual surgery and allowed me to go on and on with my questions, one after the other. It turns out that not only do both our children have similar conditions it turns out that Spencer will have the same surgeon as well, Dr. Phillips of Sick Kids Hospital. Deborah also shared many things that I wouldn’t have even thought about, like telling us that we should have clothes that don’t go over Spencer’s head for the first little while after surgery as it can be uncomfortable.  She told us that his sleeping habits (even though they may be great right now) will most likely change and that Chloe did not sleep well for about a month following surgery. She told us that I could rent a breast pump from Sick Kids and store the milk there for him. She told us that every single room has its own private bathroom, and bed for a parent to stay. The best thing Deborah did for me was send me a slide show of pictures she had taken of Chloe’s recovery.  I had some very scary pictures in my head of what Spencer might look like post surgery however looking at Chloe’s pictures was not so scary. She actually looked quite peaceful. Yes, she was very very swollen, and under heavy sedation but it wasn’t really quite as scary as I was thinking. I think she is a brave mother for taking and sharing these pictures and I hope I will have the strength to do the same.

Sick Kids Hospital & Surgery

I have to say that I feel extremely fortunate that we have the health care system we do and the most amazing hospital to deal with. Sick Kids Hospital does this surgery at least once a week. Although Coronal Synostosis is not a common condition, (it occurs 1 in every 2000 births) it is a common condition at this hospital which makes it easier to cope with as well. The team who will be taking care of Spencer is made up of Neurosurgeons, Plastics, and Ophthalmologists. They do the surgery the traditional way and not the endoscopic way. When I first heard this I was ready to find somewhere that would do it endoscopicly. This way is a much less invasive surgery that involves the use of an endoscope. The endoscope is a small tube which the surgeon can look through and see immediately inside and outside the skull through very small incisions in the scalp. The surgeon opens the suture to enable the baby’s brain to grow normally. This type of surgery is followed by the use of a molding helmet. The more traditional surgery is an incision that is made in the infant’s scalp from ear to ear. The shape of the head is corrected by moving the area that is abnormally fused or prematurely fused and then reshaping the skull so it can take more of a round contour. Over the years a perfect mold has been made (so there is no more Art in it) and they use that mold to create the “perfect” head. Surgery can last up to five hours. The baby spends one night in the NICU plus an additional 5 days in the hospital for monitoring. Another major fear that I have is that there is likely a blood transfusion that will be necessary during this type of surgery. I confronted the doctors about why the endoscopic surgery is not done here and they basically explained that in their research it is not as effective as the traditional surgery and it usually requires a second surgery later whereas they have found doing it the traditional way is one surgery, no helmet and very little complications later on. I have to trust that they know what they are talking about so traditional surgery is the road we are heading down.
In regards to the blood transfusion there is one thing that is comforting and that is that my husband and I will be tested to see if we are a match. If we are than they will bank our blood and give that to him instead of having to go to the Canadian Blood Service. I am hoping that my husband is a match as they will be able to use all of his blood whereas if I am a match they can use parts of my blood but not my plasma. We were informed that the Canadian Blood Registry is not currently using female plasma in surgeries and I’m not exactly sure why. This is one thing I am going to look into and post soon. 
Our next two appointments have arrived in the mail and this is what they are. On March 30^th we will be seeing an ophthalmologist to make sure Spencer’s optic nerves are still healthy and that all is progressing as it should. We had an appointment a little while ago because we were concerned that he was not seeing but we know now for sure he is as he grabs for things, recognizes people’s faces and is overall meeting all of his other milestones. He is even trying very hard to crawl. On April 13^th we will have our next Plastics appointment, where we can ask more questions and inform them of our decision to go through with the surgery. We have pretty much decided now that we will be going ahead with it and it looks like it will take place in June.

 

 

Spencer at one month

Spencer at two months

Spencer at three months

Spencer at five months

Spencer’s Story – An Introduction

On August 17^th 2011 at 3:04 in the morning, Spencer Mason came into the world. He was 6 pounds exactly and I would like to say that he was perfect but he just wasn’t. His face was all smushed up. His nose was scrunched up, one eye barely opened and we sort of joked that he looked like he needed to put his thumb in his mouth and blow! But putting all jokes behind I knew deep down that something was just not quite right. I took Spencer for his 3 day check up and asked his pediatrician what he thought. His opinion was that he had seen this before, that it was probably “molding” from the delivery and after time he would just naturally fill out and everything would be fine. He said it could be from the difficult delivery that I had or the way I was carrying him inside and to just wait. I trust my pediatrician whole heartedly so I didn’t bring it up again for awhile. I went to see my OBGYN for my six week check up and casually mentioned to her my concerns about his looks and the fact that when we feel around his right eye socket the bone just doesn’t feel like it’s in the right place. She went on to say that maybe he had a compressed fracture from delivery and suggested we get an x-ray. I went right back to my pediatrician, who said he did not agree but said he would send me for an x-ray if I wanted and this is where our story really begins. What they would find shocked us all. The radiologist who looked at the x-ray felt he may have a prematurely fused suture in his head and recommended being referred to the Cranio-facial Clinic at Sick Kids Hospital in Toronto, Ontario.

On  December 12  (Spencer was 4 months old) we met with a neurosurgeon who sat Spencer on my lap, felt his head for about a minute and confirmed what the radiologist had thought. He was diagnosed with Right Coronal Synostosis or Unilateral Synostosis.

The doctor told us that the coronal suture is the line that runs across the top of the skull, almost from ear to ear. On the side of the skull where the suture has closed, you can feel a raised ridge of bone. When you look at Spencer from above, you can see that his forehead on the affected side is recessed, or further back than the other side (which typically is further forward than normal in order to compensate for the brain's inability to grow on the affected side). In looking straight on his right eye seems bigger than his left and the tip of his nose is slightly deviated toward the side of the fused suture. He went on to explain that most parents say that their child looks worse when they see their child in a mirror. This was entirely true! The only comforting part about this whole ordeal was that we had a diagnosis now, and that it was purely an elective and cosmetic surgery to fix it. He was completely healthy otherwise.  However we were also told that he had a very mild case. We were informed that our next step would be meeting with the Plastic surgeon as they are the ones who are responsible for most of Spencer’s treatment and surgery. As a mother it is extremely scary to hear such things about your child but there is relief in this as well. There was a thousand things running through my mind at this time but I did not have the words or questions to ask so I was left guessing what this all entails.

I started my list of questions and a few days later we received our appointment for plastics which would take place on February 17^th 2011. Spencer would be 6 months old. I researched and researched online before our appointment so that I would really be educated and be able to ask all the questions this time without being caught off guard.  I should mention the day before we our plastics appointment Spencer had his 6 month check up with our regular doctor. His pediatrician was so pleased with how he looked. Spencer’s face was soooo much better. His eyes were almost even; his head had a slight ridge but overall looked and acted like a new baby. He was so smiley, he was rolling and was overall a gorgeous boy now which made all of this even harder. He even got my hopes up and recommended that we bring early pictures and show the progress he has made to the plastics as he believes maybe he won’t actually need surgery. He said he would maybe like to “wait and see what happens as he grows”. This made me incredibly happy. Maybe we weren’t going to have to go through this at all. His head was growing exactly the way it should be according to the plot graph that they use.

So with great anticipation February 17^th came and as nervous as I was I was also relieved that I could finally ask my questions and get the much needed answers I have been waiting for since his diagnosis.  Spencer’s doctor, a Mcdreamy sort of doctor. (If anyone watches Grey’s Anatomy this doctor was Mark in every way!)  He came in the room, put his feet up on the counter and with so much confidence began to tell my husband and I the procedure in which he would fix Spencer. He drew us a diagram of what his head looks like and what it would look like after surgery. I stopped him and then asked if he would look at the pictures we had so carefully chosen and I went on to tell him what my pediatrician said. He could tell that I was very anxious so he was gracious enough to look at all the pictures and information that I brought but then he said what I knew he would say. “He does look a whole lot better but he will not get better without surgery. He will never look entirely symmetrical without the surgery. In all the years of doing this specific surgery, I have never seen anyone get better and in fact he could get worse if we don’t do it.” There is a small window in which to perform this surgery (which is between the ages of 10 – 12 months) and it should be done within that time to get the most success out of it. Now we truly had a decision on our heads. Do we put Spencer through surgery at 10 months old or not?

If the doctors had said to me you NEED to do this surgery or Spencer’s head will not grow properly, or it will impede on his brain than surgery would be an easy decision but it’s not. It is cosmetic. It is so hard for me to decide to put my baby through such serious surgery for looks alone. I started discussing it with everyone who would listen and an overwhelming amount of people said “you need to do this for him” “when he is 16 you don’t want him to say to you why didn’t you fix me when you could have”. The world is a very cruel place and I’d like to believe that looks don’t matter but lets face it they do matter. I kept thinking about the physical scar that he will end up with and how bad it would look and then a very close friend said to me “a physical scar that will be covered with hair, is far far better  than an emotional one that will last a lifetime from the cruelty he may encounter” she was absolutely right! That pretty much sealed it for me. Surgery was the only option and I would have to deal with it for him.