January 27, 2010

Cranio technologies DOC band

I keep forgetting to write about one thing we did do for Trey early on and that was to get him a DOC band (helmet looking thing) when he was 6 months old. Trey's head was misshapen from birth, I noticed it early on and was told many times by our pediatrician his head would be fine but it never changed. His head on his affected side seem to be larger and that was with looking down so it was not do to the microtia or HFM. I found Craniotechnologies here in Phoenix and requested a referral. This was something I didn't want to do and to be honest it was for selfish reasons. I didn't want people to think I was a bad parent thinking I let my kids head get flat though I know that is not what happens. But honestly it was the best thing we did. He only had a mild case but enough that our insurance company would pay for. It gave Trey more symmetry to his face by making his left eye appear smaller, it was always bigger and I swear he was never able to close his left eye all the way (lots of eye infections that miraculously stopped after the band). I did read in one article on HFM that many kids will have some plagiocephaly (medical term for misshappen skull), usually a flattening on the skull on the affected side above the eye. This was one of Trey's issues but the main reason his left eye looked larger. Though his plastic surgeon did not feel it was related, I am sure it is. I have seen pictures of to many kids with HFM whose head shape looked like Trey's. The band was so easy and the people at cranio technologies were wonderful. Of course the band had another side effect, it brought attention to him. I had to get use to people asking questions or making comments. I really don't mind, I am all about educating people so when I am asked questions I get that opportunity. My motto is if you have a question, just ask I rather give you my answer then you just coming up with your own right or most likely wrong. Trey was also diagnosed with torticollis, he holds his head a little to the side, we did physical therapy at home with instructions Craniotechnologies provided us. I am sure we didn't do it enough because I still see it today especially when I take pictures but so far I am not sure it is a problem or will lead to one, well lets hope not.

Did we know about microtia before Trey was born?

I have been asked this question before and have heard others wonder the same thing on the yahoo group message board. Did we know before Trey was born, sorta. We had a 3D ultrasound performed when I was 27 weeks pregnant at one of those private ultra sound places. The tech noticed the ear but was not able to tell us since it was for "entertainment" purposes only. Thankfully she informed my OB of what she had seen. I was not told of the possible ear issue until I saw my doctor 2 weeks after my ultrasound. I got my doctor to agree to further ultrasounds but by that time Trey was head down and laying on his left side, the ear in question could not be seen. I went through many emotions at the thought something was wrong and we wouldn't know for sure until the birth. I cried that first night. I looked at the 3D images and convinced myself they were wrong of course they look just like his ear. The interesting part is a week before my due date I saw a show on plastic surgery and it was about a boy who got a new ear with Dr. Reinisch. I then realized this was ours and Trey's fate but I at least knew there was something that can help and that gave me hope. Knowing ahead of time that something might be wrong was hard but a gift, I was able to deal with my emotions before hand and come to terms that we may have to face things we weren't planning on. I was not however prepared for the jaw issue and the words syndrome. But once we got a grip on what it was exactly we were dealing with I became so thankful it wasn't worse but sad Trey was going to have to deal with things you never want for your child.

January 24, 2010

Lets not forget Matthew

So Matthew is our baby of the family though he is hardly a baby anymore. But since he is not walking yet but working on it, he is still my baby. He just turned a year old last week. I can't believe how fast he is growing. He is starting to want to walk, cruises around all the time and I am sure he "thinks" about taking those first big steps but just not yet. I am trying to encourage the walking, I am not one for the crawling stage, I have always enjoyed it when they can finally walk. Now if I can only get a good picture of him. Between the falling, being knocked down by his big brother or being accidentally carried into a shelf his head is constantly bruised. But we are going to try and get his one year old pictures today. Lets hope the boys had a good nap and are happy for the pictures.

Our Choice: Medpor

So after going to the Let Them Hear Foundation annual Microtia/Atresia Conference in October we have decided to go with ear reconstruction using medpor implant. We met with Dr. Brent, Dr. Reinisch and Dr. Lewin. All doctors are passionate about what they do. In the end my husband and I agree that we both felt medpor was the route we wanted to go and to go with Dr. Lewin since she accepts insurance. So the next question was when. I knew we can do it at age 3 but Aaron wanted to wait until 4, no real reason why but just thought we can wait. I spoke to Dr. Lewin on the phone for a half hour and asked lots of questions. She was so wonderful to talk to and assured me that 3 or 4 did not matter but that the earlier they do the surgery the kids just seem to bounce back faster and complain of less pain. She also mentioned that there are surgical advantages in doing it younger. Next thing we knew Trey's surgery was scheduled for March 23rd, just 2 weeks after his 3rd birthday. She was booked up until June and we wanted to try and get the second stage surgery in on the same calendar year and doing it in March versus June gives us that. Just saves us lots of money since our insurance is (I am hoping) going to cover it and one surgery will get us to our out of pocket maximum so the second surgery shouldn't cost us except for travel expenses.

So we are off to Cedars Sinai in March, we will have to stay in town for over 2 weeks due to follow up appointments. The surgery is expected to be 8-10 hours long but is an out patient surgery so he will come home that day. Pain should be minimal from what we are told, usually from the skin graft sites which will be behind the good ear and either from the scalp or groin area. Our hope is that it be a positive experience for Trey so he won't have anxiety over future surgeries for his jaw ( we expect 1-2 jaw distractions) So our plan is to go to Disneyland a week after the surgery. From what I have heard from others who have already done this journey is that it is possible. So that is the plan.

Right now I am doing my best not to think about the surgery and how Trey will look different the moment he is done. I hope he always knows that we feel he is perfect as he is and the surgery is not about "fixing" anything but we know that most likely one day having a small ear will probably bother him and that one day he will need glasses and living in AZ he needs to wear sunglasses. Straps don't work well for him to keep sunglasses on. Those are just a few of our many reasons to do the surgery and we right now can only hope for the best. Right now we have him talking about going to Disneyland after he gets his bigger ear. YEAH for Disneyland.

Microtia options

So in regards to his left outer ear, we have 4 options. The first one is of course do nothing. Second is prosthesis. A third option is rib graft surgery which is done at age 6 or 12 depending on the doctor and the technique used. The forth option is surgery with a medpor implant. All options are good options, not one option is better then another just different. Important factor is when deciding on surgery is to go with a doctor who is very experienced in this surgery. I asked each of our local plastic surgeons who they would go to if it were their child it helps to know who other surgeons think are the best. Rib graft is the surgery most commonly used, many surgeons are not familiar with medpor so they will not recommend it since it is relatively new like the past 15 plus years.

I bring up the part about medpor because that is our choice for Trey. For us medpor can be done at age 3, I personally like the medpor ears over rib graft. I also like that it is not as invasive as rib graft since they do not use the childs rib cartilage. We were opting for prosthesis but we didn't like that the color fads in time and it has to be replaced every 2-3 years. I know of families who have chosed each of the choices and they chose what is best for them and their child. Some wait until the child can make the choice and others make the choice for their child, it all depends on what factors are important to you and only you. That is actually what makes this all hard, there are no right or wrong choices, each has pros and cons. We have no crystal ball so we go with what we know at the time.

CT scan

So this is just a post to write what we learned about getting a ct scan. First try to do it without sedation. The scan takes no more then 5 minutes and if they can't lay down still, the nurses will burrito wrap your child. Trey was wrapped and screaming most of the time during the scan, but I would take that then having him sedated which is what we went through with the ABR. It might sound traumatizing to the child but really its not, he was calming down right before it ended but since the scan itself was so fast the crying didn't last long at all. I am sure some kids won't cry, no pain just laying down on a bed that moves a little. I was able to stand next to him the whole time. They also used toys to distract him while the scanning was going on but that is because we had it at a hospital where they have child life specialist whose job it is to help kids get through such medical tests. We also requested a copy of the scan and got a 3D image as part of the report on a cd. It was interesting to see how things are underdeveloped but sad at the same time.

Finding support and information on atresia options

The hardest part is finding out nothing can be done until Trey is older. As a parent I just wanted to do what I can and as soon as I can but I have had to learn patience which isin't the best thing for me. Thankfully I found a yahoo group support site for microtia and atresia. Since many kids who have microtia and atresia also have HFM this group site provided so much needed information on all levels that we needed.

We found out our choices in how we can address these issues. For the atresia we could do a BAHA (soft band or surgically implanted), canalplasty(creating new canal and fixing middle ear) if a good candidate or of course nothing. To begin this process you have to do an ABR test to make sure the affected ear has only conductive hearing loss, if the nerve is not functioning not much can be done. A ct scan can be done to determine if atresia repair surgery(canalplasty) is an option. Very complicated surgery and the best doctors will review ct scans for free. CT scan should not be done before age 2 1/2 or you risk having to do it all over again, child needs to be big enough for the inner and middle ear parts to show up. Unfortunately the ct scan showed Trey definitely is not a candidate. Not surprising to us, he had less then 20 percent chance to be a good candidate because he has HFM/Goldenhar (side note, we prefer HFM but officially he is diagnosed with Goldenhar, the geneticist who diagnosed him considers HFM to be the same as Goldenhar while others differentiate Goldenhar for when the eyes have dermoid cysts or skin tags, all of which Trey doesn't have)

We can do a BAHA (bone anchored hearing aid) that could be used to provide some hearing since his inner ear (the nerve) works. There are 2 options with that, one is a soft band type that goes on with the use of a band that sits on the skull, no surgery and it can work well, just that it is very expensive and our insurance will not pay for it. They would pay however if we had the same device surgically implanted.

We have elected to wait and see if it would be useful or needed before we do that surgery. One doctor in town would have done the BAHA surgery already, I kinda freaked out about the permanent of the surgery and that the doctor didn't seem to care if the posts goes to far into the skull and rests on the outer layer of the brain. Infections are not uncommon with the posts of the BAHA so I am just not ready to go for it. We tried a softband that was loaned to us by the audiologist but Trey would not keep it on for any longer then a few minutes, of course he was only 18 months old and he knew he had complete control over if he would wear it or not. Maybe in the future we could try it again.

The begining of Trey's story and ours.

When our son was born we began a new journey into parenthood that included the journey of dealing with Goldenhar/Hemifacial Microsomia with microtia and atresia. What all that means for our son in plan English is that one side of his face did not develop normally so his left side appears smaller and his external and middle ear did not develop on that side. We had no idea what i all meant except that he looked different so our journey began.

Starting at a week old we had medical tests to make sure his eyes, kidneys, spine and heart were fine and thankfully they were. So all we have to address is his lack of external ear, unilateral hearing loss and a smaller jaw. We see an ENT doctor 1-2 times a year to make sure his good ear is working fine. We had to go do and ABR hearing test to verify the hearing on both sides after he was a year old which meant he was sedated. If only we knew to do this test when he was an infant where they do the test while the baby sleeps so no meds to knock them out. We didn't like our first ENT who wasn't much help to us but love Dr. Mancuso our current ENT. We also see a craniofacial team that will address the ear and jaw issues. We know Trey will require jaw distraction surgery and lots of orthodontics work. So we also see a dentist who is part of the craniofacial team.

So far Trey is a happy 2 year old who has not shown any problems. His speech is normal and he is happy and very very active 2 year old. He is social but can be reserved especially in noisy environments which is not surprising since he can not locate sound well. Around age of 2 he started noticing the differences in his 2 ears but did not seem to care which I would also expect do to his age. Sometimes he doesn't hear us when we try to get his attention and other times I am sure he DOES hear me but ignores me, again age appropriate behavior.