Oliver Update

Monday, January 18, 2016

After my last post about Oliver, most people thought I should seek a second opinion from Boston.  Before I had braces put on I had three opinions.  For my brother in law's knee surgery he had three opinions.  My cousin almost had his foot amputated, but another surgeon said they didn't need to and were able to save it.   I had so much time before and didn't think to do this, but now I was strapped for time.   I was extremely stressed out about it though because there was a lot to consider.  I didn't know if we would even have enough time to hear back from Boston before his surgery, and should I cancel the surgery just to get the second opinion, even though I may still end up going with Primary Children's?  I had to have the CT scan, pictures, and the doctor's notes, all to Boston for a second opinion.  It was a lot for us to pray and ponder about in a short amount of time, but I was able to get everything to Boston and they got back to me quickly.

Dr. Meara and Dr. Proctor from Boston had a different timetable for the surgeries.  They said they would do it around 9-11 months when the bones were a little stronger.  They also said they would close the nasal cavity now, but Dr. Siddiqi told us not until he was 2 or 3 years old because they would use his cartilage from his ear and it needed to grow more.  When I told the secretary from Boston that (she was relaying the second opinion) she said maybe they would just close it for now and reconstruct it later on.  She also said they wouldn't work on the placement of his eyes until he was 7-9 years old because that would be the optimal time with the bones.  When I asked how they would do the surgery to repair the encephalocele she said they didn't say in the notes.  I explained to her how they would be doing it at Primary Children's hospital.  Dr. Siddiqi and Kestle discussed two ways of doing it, and one of them was waiting until he was 1 because the skull would be thicker to take from the skull and slice it kind of like an oreo cookie and use the part to patch the front, so maybe that's why they would wait a little longer at Boston because that was the method they would be using.  

Another thing to consider for Boston was insurance.  My insurance agent was able to get back with me to discuss my options for going out of state.  I would only have until the 31st of this month to get a new policy in place for a March 1st effective date, and it's expensive.  Not to mention going back and forth for surgery and follow up in the future.  Brendan and I prayed about it and we have felt good proceeding with the surgery here at Primary Children's hospital, but I'm glad I was able to hear from Boston.  

Oliver's surgery is scheduled for this Wednesday the 20th, but we won't know the time until the day before when they call us.  He has had a cold for a while now, and I took him in for his 6 month check up on Friday and the doctor said it looked like he had some fluid in his ears, so he might have an infection.  I'm not sure if they will cancel the surgery or not.  They will check him the day of the surgery and the anesthesiologist will decide if he is well enough or not.  Before the surgery the ophthalmologist will take a probe and try to unblock his tear duct if that is the problem with his goopy eye, but it could be something related with the cleft nose, and in that case it wouldn't help it.  They will also be drawing blood for his genetic test.  The surgeons will be making a zig zag cut from ear to ear and pulling back his scalp and part of his face off.  They will scrape off the tissue and fluid in the front of his skull and cut a hole in the side of his skull to use in reconstructing the front.  That hole in the skull is supposed to regenerate within 6-8 months.  They will then do some plastic surgery on the front of his face where his skin and hairline are stretched out.  His skin tags on his ear will also be removed.  He will lose quite a bit of blood, so he will have a blood transfusion.  I just called the other day to ask if we could donate our blood to him directly if our blood matched, and they said we could but they don't recommend it because it is too close of a match.

The surgery is supposed to be 4-5 hours long.  I've heard recovery is going to be very hard.  He will be very nauseous from the medication and probably not want to eat for 2 days.  The doctors told us his head is going to be so swollen the next day that we will be thinking we shouldn't have done the surgery.  They said to plan on 4 or 5 days at the hospital for recovery if all goes well.  Brendan's parents are going to have the other 3 kids while we are up at the hospital.  Brendan will be coming down a day or two for work, but I will be up there the whole time.  My sister Whitney lives only 15 minutes from the hospital, so I can go shower and rest if needed over there.  When Oliver returns home he will recover for another 2 weeks I think.  My friend's baby had surgery for a cleft lip and palate recently and she said it took a whole month for her to have her baby back.  She said he was so fussy and miserable.

Many family and friends have expressed a desire to fast and pray for us during his surgery, and we are so grateful.  We are not sure of the exact time of the surgery yet, but they say they usually do younger ones in the morning, so we are going to start fasting on Tuesday at 1pm.  We appreciate everyones love and prayers for Oliver and our family during this time.     

Hattie's Surgery

Sunday, January 17, 2016
Hattie's hernia surgery and recovery went well.   She was extremely excited before her surgery, because Brendan told her he would take her to get ice cream at the creamery.  I dropped Brendan and Hattie off at the hospital because there was a lot of stuff you had to do before the surgery, and I went to Whitney's house to feed Oliver and hang out for a little bit before the surgery.  I've been trying to keep Oliver out of all public places, so he can be well for his surgery, so I wanted to be able to feed him and not have to come back until Hattie was done.  Brendan said she got to pick out her pajamas to put on and they gave her a little doll that she got to draw it's face on.  The surgeon and anesthesiologist came and talked to us before and then Hattie got to pick a wagon or little tikes car to be wheeled off in.  She picked the car and the anesthesiologist pushed her away in it just fine.  She picked root beer for her smell to put her to sleep, and had no problems going down.  

While Hattie was in surgery, we went downstairs to get something to eat, and while we were eating we heard on the intercom, "Would the parents of Hattie Rowland please call...."  It kind of freaked us out for a minute wondering if something had gone wrong.  Luckily everything was OK, but the doctor put in a scope and found that the other side needed to be repaired also and we needed to give approval to fix both sides.

Hattie's surgery was only an hour and then we went to recovery where she had just woken up and was very grumpy.  She was crying and still in some pain.  She kept complaining she needed to burp, so we sat her up.  Luckily she didn't throw up.  Her next request was for her ice cream.  The hospital had a little ice cream cup, and she ate the whole thing.  We told her she could have her creamery ice cream later.  They gave her more medication, and Brendan turned on a show for her and she was fine. I think she was only in recovery for an hour and then she was released. 

 Before surgery

 After surgery

Hattie being released from the hospital.  They were all out of wagons, so she got a wheel chair.  She has her new doll she made in one arm and her throw up bag on the other.

Oliver is 6 months!

Thursday, January 7, 2016

I can't believe it's been 6 months already.  I feel it has gone by pretty fast.  Oliver is such a good baby and is doing so well.  He has been hitting all his milestones and is right on track for development.  He has been rolling over front to back and back to front for a couple months, he scoots backwards and in circles.  He has started rocking on his hands and knees and then gets up on his toes and bounces back to his knees.  He coos and tries to talk.  He is so smily and happy.  We just love him!

We didn't have any new information until yesterday when we were finally able to meet with the geneticist, Dr. Dugan, up at Primary Children's.   The genetic counselor first came in and went over all of our family history and extended family and asked lots of questions.  She left and came back with the geneticist.  We had brought up the idea of Oliver maybe having Goldenhar syndrome, but she told us that they don't really know what Goldenhar is.  There is no test and no explanation of why it happens and people are usually diagnosed when they have a lot of the symptoms and no other diagnosis.  She said one of the main features of Goldenhar is hemifacial microsomia where one side of your face like your jawbone is smaller, and Oliver doesn't have that.  She said I wouldn't give up so quickly and call it that.  She said that Janice (the genetic counselor) suggested it might be Frontonasal dysplasia, because of his features.  They mentioned some of the features and pointed them out in Oliver.  When I got home I looked it up and here is a description of it below from this website http://ghr.nlm.nih.gov/condition/frontonasal-dysplasia:

Frontonasal dysplasia is a condition that results from abnormal development of the head and face before birth. People with frontonasal dysplasia have at least two of the following features: widely spaced eyes (ocular hypertelorism); a broad nose; a slit (cleft) in one or both sides of the nose; no nasal tip; a central cleft involving the nose, upper lip, or roof of the mouth (palate); incomplete formation of the front of the skull with skin covering the head where bone should be (anterior cranium bifidum occultum); or a widow's peak hairline.
Other features of frontonasal dysplasia can include additional facial malformations, absence or malformation of the tissue that connects the left and right halves of the brain (the corpus callosum), and intellectual disability.

When she mentioned these symptoms it did seem more likely than Goldenhar.  With Goldenhar it usually affects one side of your face, which we thought was the case with Oliver on his left side, but she pointed our that it wasn't just one side.  On his right ear there is a little raised part that looks like a skin tag was trying to form.  It isn't noticeable to others though if you weren't looking for it.  We thought that maybe the encephalocele was pushing down on his left eye socket causing it to spread, but both of his eyes droop down on the sides, which the craniofacial surgeon had pointed out before.  Also his nose does have the cleft on the left side, but the right side is kind of spread.  She also mentioned the corpus callosum and how the surgeons said his was smaller from looking at the MRI, so some of it was missing.  The MRI also showed that he had a lypoma (fatty tissue) in his brain, but I didn't realize that the lypoma was located in his corpus callosum, but she mentioned that's where it was and that lypoma in the corpus callosum was also linked with Frontonasal dysplasia.  She said it is possible that the lypoma is pushing aside the corpus callosum making it appear smaller, but it could all be there.  She said to take that with a grain of salt, because you really can't tell.  She said maybe delays or learning disabilities associated with that wouldn't be manifest until he was around 6 if any.

Frontonasal dysplasia happens when there is a gene mutation in at least one of 4 particular genes.  I probably won't explain this very well, but the website above that I linked to talks more about the gene mutation part.  There is a genetic test for it though.  We are going to have Oliver tested for it the same day he goes in for his big surgery on Jan. 20th.  We briefly talked about wether it ran in the family or not and the chance of us having another child with it.  She said we would start by having Oliver tested first and if he had it then we could discuss having us tested also.  It could run in the family, but it could be a new gene mutation only occurring in Oliver.  From what I read it sounds like it occurs more randomly and not as common running in families.  A couple websites also mentioned that it is rare with only 100 cases reported.  I think maybe that is for more extreme cases.  I'm not really sure.  As I was reading more about it online I came across these two articles where they did amazing surgeries for two babies at Boston's Children's hospital who had Frontonasal dysplasia, but theirs were a lot more severe.  They are two stories that will make you cry.  You can also see the zigzag scar across their heads that Oliver will have too.

Their surgeries were more severe, but there are similar risks.  In the first surgery they said it went well, but in the days following the baby Dominic had seizures, developed fungal meningitis, and suffered respiratory and feeding problems and was in intensive care for 3 weeks.  I've felt good about the surgeons we are using at Primary Children's but then when I read online about craniofacial surgeries they always talk about Boston, Dallas, and San Diego.  Then I start thinking should we be going there for these top surgeons?  Our neurosurgeon is Dr. Kestle and our cranifacial plastic surgeon is Dr. Siddiqi.  They will perform the surgery together and I've heard great things about them, and I've liked them when we've met together.  Just yesterday the geneticist counselor was saying that Dr. Siddiqi is a great craniofacial plastic surgeon and that's who she would want operating on her child.  I want Oliver to look and feel normal and sometimes I see these before and after pictures online and they don't look as good as I would want them to look, but people keep reassuring me that our technology is great and he will look normal.  Of course my main concern is not cosmetic, and more his health and brain, but I still worry.  I'm worried about the placement of his eyes and just not looking quite right.

Oliver's surgery is only 13 days away, and he has been sick with a bad cold.  I need him to be strong for such a procedure and so I think I'm going to have to be homebound for the next 2 weeks so he can get over his sickness and hopefully not catch anything else.  I know many people have expressed that they want to fast and pray with us, so I will post again right before his surgery when we will be fasting and other details.

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