Pre-op appointments

Thursday, the day before the BIG DAY, was filled with appointments with different doctors and specialists.

First stop, the Pediatric Neurosurgeon, Dr. David Sacco.  We traveled by cab ($$$) to Dallas Children's Hospital, about 20 minutes away from our hotel and the actual hospital where Joey's surgery would be.  Let me just say, from the two I've seen, Dallas has amazing children's hospitals.  Every detail is designed w/ children in mind.  From the colors on the walls, to the twinkling stars in the ceiling, to the giant train room that is two stories tall and probably more square footage than our house!  Simply amazing!

Dr. Sacco actually called us back to his office himself, which is unusual.  He looked younger than I had seen in pictures.  How could he have so much experience?  We knew the Texans must have thought it was cold outside (it was 80 degrees that day!), b/c Dr. Sacco was wearing a wool cardigan and cords!  

The first thing he did when we sat down was he zeroed in on Joey.  He talked directly to him and tried to put him  at ease.  He took a few quick measurements and touched his head a couple times and rolled his chair back and smiled.  He knew that we were so scared and he put us at ease.  He went into the logistics of the surgery and exactly what to expect.  He was straightforward and it took him a while to crack a smile and a joke.  I didn't mind that he was serious... this was serious stuff!  But in the end, he really let us know, from his aspect, what his job was in the operating room, what he was responsible for, and assured us that he would be there from beginning to end.  That was very important to us.  So many other hospitals, the doctors come in and out of the room, only doing their job and then leaving again.  Dr. Sacco and Dr. Fearon are there, together, from incision to stitching.  He was confident, which led us to be confident in him.  Whew...

Next, we had media/photography.  Our friendly photographer Max, was a wonderfully, sweet man.  As soon as he shook our hands, he wanted us to know that we were in the best place possible, with the best team in the world.  You could just tell that he has the utmost respect for Drs. Fearon and Sacco.  Joey sat on my lap as Max had me move Joey in certain positions so that he had a lot of different angles to shoot from.  They blow the pictures up beyond lifesize so they have an idea of where they were before surgery and where they want to be.

Once we finished w/ Max, we moved across the hall to Dr. John Kolar, Anthropologist.  He was a newer addition to the team of doctors, having worked with them only a few years.  His role was to take precise measurements of Joey's head and face.  He had these metal instruments, that every time they got near Joey's nose, he opened his mouth to eat them!  The reason for the measurements was to track growth and development.  Another nice doctor who held Sacco & Fearon in the highest regards.

Next... the dreaded blood tests.  My poor baby.  The phlebotomist had a hard time finding a vein and of course, had to take 4 vials of blood.  This was the only part Joey had trouble with, as you can imagine.  He bawled his little eyes out, which made me cry too b/c I had to hold him down.  All I wanted to do was scoop him up and run out of the room.  It was the longest 2 minutes.  

Finally we were almost done.  Just one last person to meet.

Meeting the Bennett's

The thing we were looking forward to the most on that first day in Dallas was meeting the Bennett's.  I met Kari Bennett through a craniosynostosis support forum called CranioKids.  It has mom's (and dad's) from all over the world who can connect and share their stories and offer support and advice.  Kari's son Robby is 3 weeks younger than the boys.  He too suffers from metopic synostosis with trigonocephaly.  We formed an even closer friendship when we discovered that our sons' surgeries were scheduled with the same team on the same day.  Robby's surgery was in the morning and Joey's was in the afternoon.

We decided to stay at the same hotel so that we could be there for each other as much as possible.  It was such a relief!  

Off to Texas!

So, we were up and out of the house at 5am to catch the 7am flight to Dallas!  I can't believe the day is here.  We are going to another state to meet a doctor with whom we've only spoken to on the phone and via email.  I'm putting my son's life and happiness in this strangers' hands.  What are we thinking???

We were so lucky that the flights went really well.  The boys played on the hour flight to Philly.  They were so well-behaved!  I was able to feed them in Philly before we left for Dallas.  They slept on the flight to Dallas... the whole 2 1/2 hours!  What great little guys!!!!  I'm so proud of them!

We arrived in Dallas at noon, EST, one CST.  Of course, we had to find a van/taxi big enough for 5 adults, two infants and the luggage.  Our hotel was 30 minutes from the airport.  Dallas is such a big city!!!  And we got a bad taste in our mouths right away when the cab driver wanted to charge us $200 cash for the ride.  We couldn't believe it!!!  Luckily, Joe's negotiation skills were able to knock it down to $100.  Still way too much!  And to top it all off, we knew it was going right in his pocket.  He didn't even have a meter running or a paper of his rates.  LOVE being ripped off.  

October 8th is the day!

I remember what I felt when we got that phone call... October 8th in Dallas, TX.  My heart dropped to my toes and then up into my throat.  Ok... this is real.  I wanted to start packing already!  But with this, came a whole new bevy of issues.  Do we bring Dom?  Who will stay with him at the hotel?  What is this going to cost?  Do we fly or drive?  And so many more...

A bonus to knowing our date... our friends that we met online ended up being scheduled the same date, the same hospital with the same team of surgeons!  What an amazing miracle to have someone to go through this with... step by step... day by day.  Absolutely unbelievable!

Feels good to get some answers

We got the answers we were looking for!  Dr. Fearon called us and we spoke for over an hour on the diagnosis, the procedure, risks, expectations, etc.  He is a very soft-spoken, gentle-sounding man, with a vast knowledge about the until-now "underground" world of craniosynostosis.  He immediately put us at ease.  "No, it's not cosmetic surgery," he said.  "It's reconstructive surgery."  

See, we had previously been told that it was "necessary cosmetic surgery."  When you attach the words "cosmetic surgery" to a procedure for infants, it takes the seriousness out of the situation and makes it seem elective.  We were told leaving it alone wouldn't affect his brain, wouldn't put him at risk for headaches,  behavioral issues, or even teasing about his abnormal head shape.  However, trusting in our parental instincts, we just didn't believe that was all true.

In speaking with Dr. Fearon, he assured us that we were doing the right thing.  What was also nice to hear, was that he offered to write a letter of referral to any doctor we wanted to see.  He said we could go anywhere we wanted to; no hard feelings.  We felt so much better after speaking with him.  We knew that we would do anything we could to get to Dallas.

As we researched trigonocephaly further and other possible methods of treatment,  we stumbled across a forum called craniokids.org.  It's a place where parents from all over the globe, from the US, to Scotland, to Australia, all give each other advice, support, and a friendly conversational atmosphere.  As it turns out, we came in contact with a mother in Virginia who has a son, one month younger than the boys, and he has the same condition.  We began a virtual friendship, and it was nice to chat with someone who was going through the exact same thing at the exact same time.  She too, was trying to get an appointment with Dr. Fearon in Dallas.  We ended up having so much in common that now we have a very special bond.

The answer to our prayers!

So, we were left with a daunting decision about whether to subject our first-born son to this seriously dangerous surgery.  We felt as though we were looking through a tunnel and couldn't see any light.
One night, we decided to Google "trigonocephaly treatments" and we came across the CranioFacial Center in Dallas, TX.  Instantly, we knew it was a long-shot, because there is no way our insurance will approve us to go all the way to Dallas for treatment when it's available here in Buffalo.  We decided to investigate anyway.
As we read about the center, we discovered that the surgeon is dedicated to only treat craniofacial conditions.  Unlike the Plastic Surgeon up here who would've been partnered w/ the Pediatric Neurosurgeon, and she does several boob jobs a year, Dr. Jeffrey Fearon only performs craniofacial surgeries.  Dr. Fearon does around 100 surgeries a year, compared to 5 a year here in Buffalo.
The following is from Dr. Fearon's website: www.thecraniofacialcenter.org, and is a detailed description of the surgery to correct Joey's condition.  Caution:  the contents may sound graphic to some

The Dallas Remodeling Procedure.

In my practice, the surgery to correct a single suture craniosynostosis is always performed with both a craniofacial surgeon and a pediatric neurosurgeon, both of whom are present for the entire operation.  I believe that having two experienced surgeons always with the child, improves the speed and safety of the operation.  In addition, only pediatric anesthesiologist’s with extensive craniofacial surgical experience are typically selected.  During the operation, the family is given hourly updates as to their child’s condition.  The average correction takes just under four hours from start to finish, although the actual surgical time is much shorter, averaging about an hour and 45 minutes (about half of the total time in the operating room is spent preparing the child for surgery, and then slowly waking the child up).  We do not shave any hair for surgery, although many centers still prefer to do so.  The surgery is performed though an incision that is made from ear to ear across the very top of the head.  Many years ago, I developed a wavy, “zigzag” incision to replace the standard straight-line incision, so that when children get their hair wet it won’t part on a straight-line scar and make the scar obvious (see Publications #10).  Recently, I have shortened the length of this incision (starting it well above the ears) so that the scar will be even less noticeable.

The goal of the operation is to remove the areas of skull that were affected by the fused suture and rebuild the skull into a normal shape (which is slightly over-corrected to compensate for the abnormal growth that that will occur).  In order to rebuild the skull, it is necessary to somehow hold the bones together, so that they can heal.  Most surgeons use plates and screws to set the bones, however, I prefer to use dissolving stitches for a number of reasons.  To begin with, after operating on children who had initially undergone surgery at other centers, we discovered that when other surgeons used metal plates and screws to hold the skull bones together, with the subsequent growth of the skull these plates will end up on the inside of the skull with the screws poking into the brain (see Publications #12).  Studies elsewhere have noted the same thing happens with some of the dissolving plates and screws: they can also end up inside the skull against the brain before they dissolve. While I am not aware of any cases in which this has caused a problem I have decided to use dissolving stitches to avoid any potential problems (see Publications #20).  I have also found that when plates and screws do dissolve, they can end up leaving a weak space inside of the skull bones that can make additional operations (if needed) much more technically difficult.  For this reason, I feel fairly strongly that they should be avoided in children with syndromes (who are most likely to need additional operations) such as Apert, Crouzon and Pfeiffer, etc.  Finally, in a small percentage of cases, dissolving plates and screws will melt into a liquid that collects in a pocket under the skin, before it opens a hole on the face or scalp and drains out.  Given all these potential problems, I am convinced that while it is technically more difficult to use dissolving sutures to rebuild a skull instead of using plates and screws, it is what I would want for my own child.

At the end of the operation, we close the scalp with dissolving stitches.  We never use metal staples or non-dissolving sutures, as these can hurt when they are removed.  We also do not put any bandages on the child, and do not use any drainage tubes (I believe that these tubes make the recovery actually slightly more complicated).  Instead, the child is given a shampoo before we leave the room and the hair is combed over the incision.  Many surgeons do like to use head wrap bandages; this is not “wrong” in my opinion, but these head wraps are unnecessary.

The child usually will spend one night in the pediatric intensive care unit and are transferred to the floor the following day.  While most of my patients might be ready to leave the hospital in about 24 hours, I recommend all stay for at least two nights total in the hospital.  The risks of the surgery are very small at the most experienced centers.  In a published report (see Publications #15) looking at a combined experience with our center and one other center, we found that no infections occurred in infants undergoing operations for craniosynostosis (although this is certainly possible).

Nevertheless, we have a policy of playing soft music in the child’s room after surgery because a study showed that soft music relaxes children, and reduces the amount of pain that a child will report feeling after the operation  (it also reduces the amount of pain medicine needed).

At our center, I routinely give infants a drug called erythropoietin before surgery (see Procrit Information), which typically raises a child’s blood levels to help prevent the need for blood transfusions, hopefully improving the overall safety of the operation (see Publications #19 and #25).  I also use a “cell-saver” that sucks up most all of the blood lost during the operation, filters it, and lets us give the baby back it’s own blood. These two different techniques (and many other smaller things that are also done) when combined together make blood transfusions fairly uncommon in my practice.  Some research suggests that patients who get blood transfusions are more likely to get infections after surgery.

Could this be?  Could he be the answer to our prayers?  We sent him an email detailing our journey thus far, along w/ pictures of Joey's head at different angles so that he could see what we were dealing with.  And we waited for a phone call back.

 Notice the sharp point of the forehead

 Notice the triangular shape of the head

Metopic Craniosynostosis? Trigonocephaly?

What do these big words mean?

Craniosynostosis is a condition where the early (premature) fusion of the sutures of the bones in the skull occurs. This premature fusing of the sutures restricts and distorts the growth of the skull. The resulting cranial growth restrictions can lead to increased cranial pressure growth which can lead to vision problems and impede intellectual development. Also the abnormal head shape is associated with low self-esteem and possible behavioral problems. It is possible to correct these deformities in some cases which can relieve cranial pressure and in the long-run, improve the self-esteem of the child.  (www.craniochat.org)







                                         Trigonocephaly (Metopic synostosis):

Trigonocephaly involves fusion of the metopic suture. The metopic suture runs from the top of the head, at the fontanel or soft spot, down the center of the forehead to the nose. It is different from the other sutures of the skull because it is the only one that normally begins closing in infancy. A ridge can easily be seen running down the center of the forehead and the fontanel is usually absent, or closed. In looking straight on at your child, in addition to seeing a ridge running down the center of the forehead, the forehead will look narrow, and the child's eyes are usually spaced closer together than is normal. When viewed from above the forehead has a triangular shape, like the bow of a boat. The incidence of trigonocephaly is somewhere between one in 2,500 to one in 3,500 births. The reason for this range is that it is sometimes difficult to determine if a child has trigonocephaly or not.  (www.thecraniofacialcenter.org)

So these are the words that were given to us to explain why Joey's head looked the way it did.  Such big words for such a little baby!  Now, we were left with a decision to make.  Leave his head alone, and let nature take it's course.  Or, have surgery to correct it.  The pediatric neurosurgeon was on the fence with Joey's case, calling it mild/moderate.  He said that if it was his kid, he didn't know if he would do the surgery.
Great!  That doesn't really help us at all!  It's OUR kid!


The surgery was presented to us as a 6-8 hour surgery, where Joey's head would be cut from ear to ear, his forehead would be removed (and possibly his brow bone too), it would be cut apart and reshaped with the  pieces.  What skull was left over would be discarded and the holes would be patched with metal and dissolving plates and screws.  So, to elect "cosmetic surgery" to fix the pointy forehead would leave Joey with a scar on top of his head from ear to ear, and plates in his head.  We were also told that the screws may always stick out a little bit, so you would feel little nobs on the head, but may or may not see them.
So, wait a minute... I'm going to fix one problem and give him two more?  We were also advised that he would be restricted from contact sports.  Just being a boy is a contact sport!  


Joe and I looked at each other and said, there's got to be a better way.




A few pictures of the point...

What to do next?

Joey was about 9 months old when we decided to ask a neurologist about the shape of his head.  The neurologist said, very nonchalantly, "What I see, when I look at him, is a cute kid!  I don't see anything wrong with him."  That was so frustrating because we knew that this head shape wasn't normal.  We felt like we were crazy for pursuing this.  However, we asked the doctor about a cranial remolding helmet., This would essentially put pressure on the parts of the head we didn't want to grow so that the only places the head could grow was where the helmet made room for.  The NS said we could look into that if we wanted, but he suggested a CT scan first, to see if the sutures in the skull were still open.  If they were not, the helmet would do more harm than good.  So we made the appointment.

The happiest day of our lives

Joey's journey began on October 22, 2009. Our first-born son came into the world at 5:30pm, two minutes earlier than his little brother Dominic. When he came out, the doctor's exclaimed, "Oohh.. he has red hair!" I began sobbing, knowing that this little man that had been inside me this whole time, was a real, live human being and he was mine. To add to my enjoyment, Dominic arrived healthy and strong and my life was never the same.

From day one, we noticed that Joey's head was pointy in the forehead. We were told that it would round out soon enough, and we accepted that. Weeks went by, and his head never rounded out. But we were so in love with him that it never bothered us much.

 Happy Birthday!

 Joey- 1 day old