Jibber jabber

We recently had Early Intervention here at the house to do a speech evaluation. When we had our cleft team visit at 16 months, Simon only had three spoken words (and eight sign language words) so the speech language pathologist on the team recommended a further evaluation. Of course almost as soon as we got home from that appointment he started learning new words every day.

I've been trying to get his words on video, but they are spontaneous and difficult to decipher if you are not around him all day, so no real luck yet. In the month between the cleft team visit and the Early Intervention visit, Simon learned at least 15 words. The one he uses the most, of course, is "NO!" The second most important is "Aya" which we just realized is the word he uses for his security towel.

So, long story short, he does not need speech therapy, which is great news. The evaluation measured him at a 15 month level (at the age of 17 months), and gaining quickly. We need to keep working on helping him learn to use a straw, which should help him keep his mouth shut and stop drooling. Although the drooling is already a lot better since we switched him from a bottle to a sippy cup.

Since I don't have any talking video, I'll just post an assortment of recent photos. We enjoyed a nice long visit with Teta, along with perfect weather, and a side trip to meet Simon's baby cousin Yasmine in Madison.

Cleft Team visit

a nice view from the Rush waiting room 

We have been working with a team of specialists for Simon's cleft from the beginning. Now that we are finished with the first big surgeries, we go see the team once a year for an overall well visit. We just had our first annual visit, which was a 2+ hour appointment at Rush where we saw the plastic surgeon, orthodontist, ENT, speech pathologist, and audiologist. Simon got a good report, his scars are healing well, his teeth look good so far, and his hearing is perfect. His ear tubes are still in place and working well, and he has not had any ear infections (knock on wood).

His speech development is on the slow side, so the speech pathologist recommended we have him evaluated by Early Intervention and see if he would benefit from speech therapy for a while. Of course, Murphy's Law, he has been working on a lot of new words in the week since we had our team visit. He now says "Mama" and "Dada" all day long, "done", "duh" (for duck) and "home". He is also making hilarious animal sounds, which we will try to get on video. On Friday he sucked water through a straw for the first time! That's an exciting milestone for a cleft palate baby who wasn't able to suck before.

The orthodontist said we have to get rid of his bottles, to keep the milk sugars from pooling around his teeth, since he is mainly using the bottle for comfort during the day. It's been a week since we took them away, and he is not impressed with the new sippy cups. He does pretty well with a regular cup, but he is still missing his security bottles for sure.

Surgery followup

We had our followup appointment with the surgeon last week, it's taken me a while to post. Simon is keeping me pretty busy. Anyway, everything is looking good, he is healing well. He has been still fussier and more sensitive than usual, but that's to be expected. We will have one more checkup in 6 weeks, then nothing until September when we will see the cleft team for the first of our regular yearly evaluations. He will see the surgeon, orthodontist, ENT and the speech therapist all in one afternoon to see how things are coming along.

Every day he has less cranky times. He has been pretty challenging to feed lately. He won't tolerate any utensils near his mouth, he wasn't a big fan of the spoon even before surgery. He likes to feed himself, but he hasn't been eating a whole lot, until the last couple of days it's gotten better. He is almost back to normal sleeping, just one wakeup at 4:30 a.m., and then back down until 6 a.m.

He loves his dishtowel more than ever, now he wants to carry it with him, usually in his mouth and trailing on the floor. Luckily we have 6 of them, so we can keep giving him a fresh one. He loves to hold his bottle while he's in the stroller or the carseat, but he thinks it's hilarious to turn it upside down and shake the milk out, which is why he has wet spots all over his sweatsuit above. He's also really interested in keys, wallets and phones, as well as cabinet doors and drawers, leading to some pinched fingers. He loves to shake containers to see what noises they make. This morning he was doing that and my vitamins ended up all over the bathroom floor.

Feeling better

Simon has been doing a lot of sleeping - not for longer than 2 hours at a time yet, but resting and healing. We got the go ahead from Dr. Smith to give him soft finger foods last night, and that helped a lot. He refuses to take any food by spoon or dropper or syringe, so it was pretty much just milk with a touch of rice cereal for the last couple of days, and the poor little guy was so hungry. So he's back to eating some of his favorites, avocado and squash and tofu.

He has several teary meltdowns a day still, he seems to just be a little edgy from lack of sleep and maybe some discomfort here and there. There are dissolvable stitches in the roof of his mouth and inside his nose, plus the unfamiliar feeling of having the roof of his mouth with no gap in it. He is also very clingy and snuggly, which is a change of pace for our little independent adventurer.

So far each day has been much better than the last, and although this surgery is more of a challenge for him physically than the last one, in some ways it has been easier. There is less medication to give him, and we don't have to deal with any apparatus like the nasal stents or the NAM. Plus we can distract him with toys and books, so most of the day he is happy as usual.

Home again

What a difference a day makes. Yesterday and last night, Simon was pretty out of it. We kept him comfortable with morphine until this morning, when we switched to Tylenol. As soon as he got the O.K. from Dr. Smith, he enthusiastically drank from his bottle. He was doing well enough by 1 p.m. that we were able to go home. As we expected, he was so happy to be out of the hospital and away from the IV and all the wires that he was cheerfully playing right away. He's still a little swollen and on a liquid diet for a while, but he's doing great and taking plenty of fluids.

Nancy surprised us by coming in at the last minute from Texas, and Simon enjoyed spending time with Teta as well as Mom-Mom and Grampy.

Surgery success

Everything went well, Simon did great. Today he is resting, and we'll try some food tomorrow.

Palate surgery tomorrow

A few pics from Simon's first passover seder last night. Yes, that is a cheerio on his face. No, it is not kosher for passover. His shirt says "Grandma's little matzoball".

We got the call from surgical scheduling this morning, we're on for 8:15 a.m. tomorrow morning. We will arrive at the hospital by 6:15 for registration. Simon usually wakes up between 5 and 5:30 a.m. anyway, so this actually fits right in with his normal schedule, except of course he won't be having breakfast.

We've had our pre-op meetings with the surgeon and the pediatrician, and Simon is good to go. He's completely healthy and all developmental milestones are right on track. He's just a few ounces shy of 20 lbs. He will probably lose some weight after surgery, since it will take him a couple of weeks to get back up to speed with eating. After that he will be on a high fat diet for a while to pack on the calories and pounds, he'll be having extra cheese and butter on everything, in addition to healthy fats from avocados.

The surgery itself will be around an hour and a half, not including prep time and getting him settled in his room afterward. I hope we'll be able to see him by 11 or so. Dr. Polley said we could expect recovery to be a bit longer process this time, starting with two nights in the hospital's Pediatric Intensive Care Unit. All lip and palate repair patients go to the PICU at Rush, so Dr. Polley can keep a close eye on them as they come out of anesthesia. We will be released when he is eating well on his own again.

Once again, thank you to everyone for keeping Michael's father in their thoughts and hearts.

The next surgery date

I took Simon to Rush yesterday for a checkup with Dr. Polley. The palate is looking good, the cleft is continuing to narrow as he grows, with the lip and soft palate being already brought together applying tension to the hard palate. We're going to give it another couple of months to close the gap as much as possible, every millimeter counts. Surgery will be April 20. I will post more information about the surgery closer to the date.

Cleft team appointment - 2 months post op

I took Simon to RUSH for his 2 month post-op checkup on Tuesday. He saw Dr. Figueroa (orthodontist, top) and Dr. Polley (surgeon, bottom) and they say everything looks great! His lip and nose are healing well, and the redness of the scar will continue to fade over time.

The best news is that his palate cleft is already closing quite a bit. Dr. Polley says that after he does the lip surgery and soft palate repair at the same time, the tension in the front and back of the mouth allow the hard palate to gradually grow closer together. This doesn't always happen, but it is working for Simon. He is very optimistic that we will be able to do the palate repair on the early side - he mentioned March dates, but I'm expecting more like April or May as we originally discussed.

We want to strike a balance between making sure the cleft is as small as we can get it before doing the repair, and doing it early so his speech development is not delayed. The other good news is that we don't have to go back until early February, when we will check the progress of the palate. It will be great to have a long break from doctors' offices!

September Has Been Craniofacial Acceptance Month

I have been meaning all along to have a page where we explain a bit more about facial clefting in general and Simon's cleft in particular, so I will squeak it in under the wire here right at the end of September. I will continue to add to this page as I find out more, and as Simon's journey progresses. Let's start at the beginning:

What is a cleft?

A cleft of the lip and/or palate occurs very early in pregnancy, between 7 and 11 weeks after conception. The face and head form from back to front, normally meeting in the middle and forming the two lines under the nose and the raised ridge running down the center of the palate as they fuse. If the timing is off for some reason, the lip and/or the palate fail to fuse completely, and the resulting gap is the cleft.

Simon was born with a complete unilateral cleft lip, meaning the gap extended into his nostril, and a complete unilateral cleft palate, meaning the gap in the palate runs all the way from his gumline in front, through his hard and soft palate, to his uvula in the back.

What causes a cleft? Is it genetic or environmental, or just random?
The causes of clefting are only partially understood. In some cases it runs in families, and some ethnic groups have a higher incidence of clefting, but most of the time there is no family history and no connection to any other health problems. There are also some environmental factors which can increase the risk, such as maternal smoking, certain medications and high exposure to pesticides, but again most of the time it is just a random thing. We were completely surprised by the diagnosis, since we have no family history on either side, and I was extremely careful during the whole pregnancy, doing everything "right."

We didn't realize that clefts are the most common birth defect, affecting one in every 600 births in the U.S. We all know people affected by clefts, but most of the time don't realize it because of the wonderful success of repairs, which are performed so early most of us have never seen an unrepaired cleft lip.

Simon's timeline so far:

December 19, 2009:
19 week ultrasound reveals cleft lip and probable gumline involvement. We can't tell anything about the palate until birth.

February-March 2010:
Interview 3 cleft teams and select RUSH University Craniofacial Clinic

May 6, 2010:
Simon is born, and we see that he has a complete cleft lip on the right side, and a complete cleft palate, which means he will not be able to breastfeed, since he can't create a seal and get suction.

May 20, 2010:
Simon's first cleft team evaluation

May 25, 2010:
Remove a tooth that is erupting in the cleft site of the gumline

May 28, 2010:
Begin nasoalveolar molding (NAM) treatment

August 5, 2010:
Lip, nose and soft palate repair surgery, ear tube placement (age 13 weeks)

April 20, 2011:
Hard palate surgery and soft palate revision

TBD, 2017-2019:
Bone graft to complete gumline

More information to come...

Surgery followup

The roundest head since Charlie Brown

One finger escaped the swaddle when he fell asleep after a busy afternoon with Mom-Mom and Grampy.

We had our surgery followup appointment yesterday at Rush Craniofacial Center. Dr. Polley says everything looks great and is healing just the way it should. He will continue to wear the nasal stents for another month. The 6 weeks following surgery are the most critical time for healing, and we want to maintain the nice symmetry of the nostrils that we got from all that hard work with the NAM.

We are also putting a silicon gel on the scar several times a day to keep it soft, which is why it will always look like he has a runny nose for the next few weeks (although he also drools constantly, so probably his whole face will be kinda wet). Luckily he has developed an enthusiastic love for burp cloths and towels, which he grabs and stuffs in his mouth at every opportunity.

We'll be back for a checkup at the surgeon in 6 weeks. Tomorrow we have Simon's 4 month pediatrician visit, so we'll see just how big he's gotten and get him another fun round of shots.

ENT followup

Simon went to the Pediatric Ear, Nose and Throat doctor today to check on his ears post surgery. The ear tubes are in place and working well. He also passed his second hearing screening, so he's A-OK.

95% of babies with cleft palate need ear tubes to prevent chronic ear infection and/or temporary hearing loss due to an inability to properly drain fluid from the inner ear, so we decided to place tubes during his lip repair surgery.

Happy Friday!

Closeup with no tape

This morning the tape covering his lip finally fell off, and at the same time he sneezed out his nasal stents, so I got a few pics. The scar is kind of red right now, but it will fade over the next few months. The timing is different for everyone, it could take a couple of years to fully fade. I think it looks pretty great already, especially when he is sharing his sweet little smile with us.

Stitches are out

I took Simon back to the hospital this morning for his scheduled suture removal. We had to go through the whole Operating Room procedure again, fasting for 4 hours and arriving 2 hours before the procedure, but once we finally got there, it was easy. He was under general anesthesia again, so that's why all the precautions, but it was just through a mask for a few minutes, he was back with me and awake within 15 minutes.

We were looking forward to seeing his new lip without stitches, but we will have to wait a little longer, because they put suture tape over the scar to protect it. We have to leave it in place until it falls off on its own. Plus he's still wearing nasal stents for now, secured with more suture tape. Sigh. Can't wait to see his little face without all this medical stuff in the way.

He's feeling great and eating everything in sight. He even managed to gain half a pound this past week, in spite of all the difficult, slow feedings. He's a little chunker, and a charmer, smiling at all the nurses and doctors, even though he was so hungry.

Care and Feeding

First of all, thanks to everyone for the well wishes. We are at day 3 post-op, and it's a job of work keeping this little guy fed and clean and medicated. In some ways it's like going back to the newborn days, sleeping 2-3 hours at a time, and eating veeeeerryyyy sloooooowwwly, while spitting up a lot. He HATES his antibiotic liquid and the ear drops, but the worst is the nasal stents, which go very far up his nose, and he's got stitches inside his right nostril so that's no fun. It means he continues to have a tape moustache for a couple more months.

Every day is a little better than the day before, wheezing and screaming less and laughing a little more. We go back to the operating room on Wednesday to remove the sutures from the outside of his lip, but since he'll only be under anesthesia for a few minutes, hopefully the recovery will be a lot easier.

Simon 2.0

We were pleasantly surprised that we were discharged from the hospital today around 1 p.m. It was a tough night for me and Michael, with all the wires and monitors, and Simon pretty out of it from the morphine. We didn't get much sleep. In the morning, we let it wear off so he could eat, and that helped him turn the corner. He is eating about half his normal amount, but that is good enough for now. He is on Tylenol with codeine, and we hope to be using only infant Tylenol later today.

Here are some before and after pictures from our day and a half at the hospital.

Surgery Day

Simon is out of surgery, and he did great! No problems, his nose and lip were repaired, his soft palate and uvula were also repaired, and ear tubes were placed. His hard palate will be repaired later, probably next spring or summer. He will be evaluated every few months to see if the lip/soft palate repair is bringing his hard palate closer together.

He looks great, a little swollen in the upper lip, and he has stitches and nasal stents to maintain the new shape of the nostrils. He is wearing little velcro arm casts to prevent him from putting his hands in his mouth, and he has 2 IVs, for saline and morphine. He is still sleeping under the morphine, the nurses will try him on Tylenol with codeine later on and dial back the morphine.

If he eats well from the bottle we'll go home tomorrow. We're spending the night in the pediatric Intensive Care Unit tonight. Thanks to everyone for the well wishes and support this week, and especially to the grandparents (all 4!) for coming to wait with us today. Also, of course a big thank you to Dr. Polley and his team here at RUSH and Dr. Walner, for their great work helping Simon.

We will post pictures later on. We've been up since 2 a.m., so we're going to follow Simon's lead and take a little nap.

Prepped and Ready

Well, we are gearing up for Simon's surgery next week. He had his last pre-op appointment on Wednesday, and as lovely as Dr. Figueroa (the orthodontist, shown above) is to see, we will not miss these weekly adjustments. We asked a ton of questions, and I think we are now as ready as we'll ever be to send our little baby off to the operating room.

By chance I happened to take Simon to see a nurse practitioner at the pediatrician's office who used to work in the Pediatric ICU at Rush. She has taken care of many of Dr. Polley's lip repair patients and she had wonderful things to say about the nursing staff and about Dr. Polley. It means a lot to us that he has fostered this good relationship with the nurses in the PICU. Dr. Figueroa says that their arrangement there is the best kept secret in Chicago, because the two children's hospitals in town tend to be so busy, whereas you maybe get a little more staff time at the Rush PICU, as well as private rooms where we can sleep in a fold out chair and be with our little guy 24 hours.

Surgery will be next Thursday morning. We will get the exact time the afternoon before. We'll post updates here when he's done.

We have a date

First let me say that those tools do not go in Simon's mouth, thank goodness. They are used to adjust the NAM device that he is wearing to reshape his nose and bring the cleft palate/gumline closer together. We went in for our regular adjustment, and after another infuriating 45 minute wait in the exam room, we finally got the orthodontist, plastic surgeon and nurse coordinator in the same room at the same time!

We have a date for Simon's first surgery: Thursday, August 5. Three weeks from now, when he will be 13 weeks old. Everything is going very smoothly and exactly according to the schedule outline we were told from the beginning, but it is just agonizing waiting for it all to come together.

In spite of my complaining about the waiting and the difficulty of dealing with the NAM, the craniofacial/cleft team at Rush has been great to us. Very encouraging and helpful, and we have had a chance to see families at other stages of the process while we are in the offices, all the way up through 16 years old.

This first surgery will be to close his lip and build a nasal floor on the right side, using all existing tissue that will be repositioned. He will also probably have the soft palate closed at the back of his mouth, and the ENT will examine him and place ear tubes if necessary (to relieve buildup of fluid in the eardrum). His palate will not be repaired now. That is the second surgery, which will probably be around 1 year old.

Here is a video produced by another hospital, which explains a bit about the process. There are a few shots from the surgery itself, which can be a little hard to watch, but it is just very brief.