Laryngeal cleft message board up and running!!!

The new board is up and running!!  Please visit and join it here.  If your child has been diagnosed with a laryngeal cleft or is in the process of being worked up for one then this is the place for you!!  We also welcome parents of children with dysphagia and aspiration with other causes.

Thickener trashing party!!!!

Once we got the OK from the swallow study that Jennifer didn’t need thickened liquids anymore we decided to have a thickener trashing party!!  It is so expensive that I did have a hard time just throwing it away but at the same time after having to use it for about 1 1/2 years it felt good too!  Jennifer enjoyed herself too!!

This first pic is her showing the can of thickener:This one is harder to tell what is happening because of the way she did it but it is of her pouring the thickener into the trash:Unfortunately with the delay on the digital camera I didn’t get the next pic of her jumping up and down clapping her hands!!!  What a happy moment for us!!

Latest ENT/GI visits

Today was Jennifer’s next appointments with her doctors in Boston. She saw both ENT and GI. The visits went very well.

ENT: He is happy with how well she is doing. Said all looks great and she progressed quite fast after the surgery getting onto the thin liquids. The fact that she does still cough some when drinking is fine and that should slowly go away with more time as her body continues to learn how to swallow with her new anatomy. But no aspiration by swallow study is great news. He doesn’t want to see her for a year unless things change. If at a year things still look so great then he will officially discharge her from his service!!!

GI: We talked about how much Jen is still refluxing and how it’s actually hard to tell. Jen tells me at some point everyday that her tummy hurts. But she says that no matter what is actually bothering her. It could be her big toe but she’ll say it’s her ‘tummy’. And when she coughs (not while drinking) she almost always says it’s ‘on yuckys’ meaning that she refluxed. So we aren’t sure if she still refluxes or not. We are going to try cutting her prevacid to once daily now that the surgery site is all healed up though.

The mild fructose (sugar) intolerance seems to be getting better. Her theory is that she got it from some stomach bug and that she just needed time to heal her intestines. She’ll stay on the probiotic a bit longer until that is completely gone. No plans to retest currently.

Jen is handling soy lethicen and small amounts of soy oil currently. When I tried higher amounts of soy oil she was definitely refluxing so she wants me to stay where we are for 4 more weeks. Then try increasing the amounts of soy oil over 4-6 weeks. If she handles that fine then after that time period add in soy yogurt. If in 4 weeks she does ok on the soy yogurt then add in soy milk. By then it will be time to see her again. We are to NOT add in any dairy before seeing her again. She wants her tolerating soy well before even thinking about any form of dairy.

The only thing she is concerned about in Jennifer is her height. From age birth to 6 months she steadily increased in height to catch up from being born early. Then from 9 months until 18 months she was 50-75% in height on the growth charts. But all the sudden at age 2 she dropped to 15% and then at age 3 to 8%. All this time her weight has been steadily 50%ish. (Well not at birth!! Hee Hee!) But she said it is highly unusual for a child to drop percents in height only. Usually children will drop in weight only or wt and height together but rarely in height alone. For this reason she will probably have her evaluated by an endocrinologist to see if there is an underlying reason for this drop in height.

All in all that’s the visit. 1 year until ENT again and 4-5 mos until GI again. Mostly good news.

Surgery was a success……..she passed!!!!

Yesterday was Jennifer’s post-op swallow study. It has been almost 3 months since her surgery to fix the laryngeal cleft. Ready for the results………………………..

NO ASPIRATION!!!!!!!!!!!!!!!

It was perfect!! For those who would understand this part:  there was no penetration either!!! No silent aspiration, no nothing. 

She is fixed!! Dr. Rahbar is the BEST doctor in the whole world!!

After such a long journey I never thought we’d see this day. I had such doubts and trials. The decision to do the surgery was not an easy one and the second guessing was huge but in the end it turned out to be the right one after all. Thanks to the Lord!!

The update is coming today as yesterday was quite the adventure!! Her test was at 2pm. We got back there about 2:20 and it literally took about 5 minutes!! A usually swallow study test takes longer but they only needed to see if she was safe for the liquids. When we got done we had to go and see all the ’sights’ meaning all the things she loves at Boston Children’s. The bubbles, the lobster, the balls and especially the garden. We even brought bird food to feed the birds in the garden this time! We could have rushed out of there and gotten right home but the day before when I told her we were going she asked if there would be any boo boos that day. I told her there wouldn’t be but at the same time I wanted to make it as positive an experience as I could. She’s already quite weary about anyone doing anything around her mouth so I just wanted positive.

By the time we got done with the sights and getting a snack it was 4pm. Had some trouble getting out of the parking garage and I’m pretty sure I got charged twice. Should have realized that was a forbearing of the rest of the trip home! I went the wrong way into a toll booth and ended up on the wrong highway. That added 20 min to the trip. Then I missed my exit to get onto the last highway and this one wasn’t as easily corrected. We ended up having to eat dinner on the road and taking a back road to get to the highway we needed. This second mistake ended up adding 1 1/2 hours to the trip!!!! So a 2 hour trip home ended up taking nearly 4 hours!!!

But in the end not much could burst my bubble! I was riding on cloud 9. I can do that trip in my sleep. I think I was just riding on a natural high and driving along thinking about how I never thought she’d get to this day and then ‘Oh there’s my exit’!!!!

Here is a pic of her getting ready to have the swallow study done:

And another of her out in the garden enjoying the birds:

Tomorrow is the day

Tomorrow we go back to Boston to find out if Jennifer’s surgery was a success.  She will have her post op swallow study.  It will have been almost 3 months since her surgery.  (I think it will end up being 2 months and 3 weeks to be exact).  

I’m nervously excited about it.  She’s been on thin liquids since this post and is doing well. She does still cough but more as a protection than choking like before the surgery.  And it is pretty infrequent.  I really do think the study will still show some penetration into her windpipe but no real aspiration.  I think when the penetration happens is when she coughs.  This is a huge step for her as it means that her body has started to recognize that fluids are coming there and triggers her cough reflex.  I *think* if this is all that’s happening then they will clear her to stay on thin liquids.  As long as there is no aspiration.

Another exciting thing about going tomorrow is that I will get to meet one of my online friends that has a kiddo with aspiration too!!  She found me through this blog and we have been emailing for a few months now.  Her daughter is today going for a G tube placement and to be scoped to see if she also has a laryngeal cleft.  Please say a prayer for them today.

A big day for us!!!!

Does the picture say it all?  Yesterday was a HUGE day for us!!!

We actually went out to run some quick errands and so I didn’t bring anything for snack or to drink.  Jennifer was just whining and whining that she was firsty!  So, I stopped and got a juice and put some in a cup with a straw for her and for Sam.  I figured I’d pinch the straw so that she would only get a little as that works ok for her.  But, then I decided to see what she would do with it on her own.  She did wonderful!  Drank 6 oz of juice and only coughed once.

Later when we got home we tried again with this juice box.  She was so excited!  It was for sure a Kodak moment!!!  No cough at all this time.  She then drank thin oat milk for dinner.  Again no cough.  

Hopefully there is no silent aspiration accompanying this as that has been her big MO in the past too.  We’ll watch close for the low grade fevers I blogged about a week or so ago and go from there.  Next swallow study is in about 3 weeks.

Yipee!!!!!

I think she may be aspirating again

After her surgery for the laryngeal cleft repair I were told to slowly start lessening the thickener in her liquids as that was the way to “reteach” her neuromuscular system how to swallow with her new anatomy. I was also told that it would be a long process that would be kind of like riding a bike where she would fall off multiple times but eventually get there.  I was also told NOT to be frustrated.

Fast forward about 7 weeks and I’m frustrated .  I know I’m not supposed to be and I’m really trying not to be but at first she was doing so well.  About every 4-5 days I was able to reduce the amount of thickener in her liquid and she was doing wonderfully.  No coughing and no signs of aspiration.  We got from honey thick to nectar thick and then just below it like a breeze.

Then we hit a road block.  She was 2 “steps” (meaning a step used in our thinning process) below nectar when she started out ok but then started coughing with drinking.  So I went back up a step as directed.  The coughing stopped but then I noticed that she would do this weird sounding swallow.  I thought with her it was behavioral (hard to tell these things on a 3 year old!!)  and so asked her why she was doing it.  I got various answers from “choking” to “it’s fun”.  So, I left the thickness as it was figuring it would be a good place for her body to work at.  We’ve been there for about 1 1/2 weeks though.

Fast forward to the last few days and she’s acting a lot like she did before we started thickening.  Cranky and chronically running a temp around 99.4-99.8.  Not a huge temp I know but this used to be what I thought her “normal” temp was.  It was always “oh Jen just runs a little warmer than the rest of us”.  Then we started thickening and she stopped aspirating and low and behold she actually is human and her temp usually is closer to 98.4-98.6.  So this really is a 1 degree rise for her.  Nothing acute for infection but more indicative of chronic inflammation in her lungs.  :(

I’m guessing this may mean she’s fallen off the bike and I need to get around to realizing it.  I’ll go a little thicker again and see where we go.

Down to nectar thick and glad to lend some support

We’ve been slowly decreasing the amount of thickness to Jennifer’s liquids since her surgery.  We are currently back down to nectar thick consistency.  This is quite nice progress since there is quite a difference between nectar thick and honey thick.  I think we’ll stay at this thickness a bit longer than we have at others though as she has been coughing here and there.  Only very small amounts but enough that we should probably give her a bit more time to get used to this thickness.

I also wanted to mention that I created 2 more pages for this blog.  One on MSPI and one on laryngeal clefts.  I also put up some new pics on the sidebars!  I intended for this blog to serve 2 purposes: 1) to get my thoughts and stress off my chest and 2) to lend support to others going through journeys similar to ours.  Both purposes have been happening nicely.  Since moving this blog from blogspot to wordpress it has really moved up in the search engines and I have been getting emails from other parents of kids with laryngeal clefts!!  I’m so happy about that!!  Keep them coming!!  Or click on the link for the support group/message board on the sidebar and join there.

My daughter is my hero and some thickener updates

The title says it all.  Jennifer is my hero in so many ways.  She has been through so much in her short life but she just rolls with the punches and moves on.  Now, don’t get me wrong, she isn’t all peaches and cream or anything.  She is a typical toddler/almost preschooler.  She’s stubborn and boy does she have a temper!!!  But despite all that, at the same time she just blows me away.

When we first found out at 2 that she was aspirating and that she had to go on nectar thick liquids she didn’t bat an eyelash.  I tried the stuff and man is it nasty.  Then a few months after she turned 2 we found out about the milk and soy intolerance and had to radically change her diet.  Her past favorites, like goldfish crackers for example, were no longer allowed.  She only half batted an eyelash at that one.  Then we had to go up to honey consistency on her liquids and she gave us trouble at first but then after a week got used to it and acted like it was no big deal.  Have you ever tried to drink something as thick as honey???  Can you even consider something as thick as honey still a liquid beverage???

We won’t even talk about her multiple hospitalizations, procedures and recent surgery here as they are in other posts except to say that she has always handled them like a champ.  Including going back to her recent surgery in my arms while singing to the anesthesiologist!!!

Her most recent intolerance is to fructose, AKA sugar, resulting in another diet change.  At first it was easily managed by just taking juice out of her diet (yup, you guessed it, didn’t bat an eyelash!!) but she then started having symptoms again.  So, onto another source of sugar out of her diet…….fruit snacks.  This is a kid who begs daily for some fruit snacks.  We actually used 1 fruit snack (not 1 package) for her for potty training too as she couldn’t have the mini m n m’s.  She’s been potty trained for about 8 months now but still begs for that fruit snack every time she pees!!  You can imagine how I thought it would go when I told her no more fruit snacks.  But the little trooper she is, I just told her that they were hurting her tummy now and that she shouldn’t have them anymore and she proudly announced to Daddy that she was having no more fruit snacks “because they hurt my tummy”!!!!  She really is my hero.  She’s just amazing.  No tears, no fuss, no nothing. 

And on the occasion that she does give me a hard time about something she isn’t allowed to have then I just ask her “what will happen if you eat that??” and she will tell me “it will hurt my tummy” so then I ask her “should you have it then?” and she’ll say “No” and move on.

On to the thickener updates…..I make her 24oz of VERY watered down juice at a time (20oz water to 4oz juice) and use 8 tbsps of thickener in it to make it honey consistency.  Just after her surgery we had to go up to 9 tbsps as she was coughing a bit due to the changes in her throat anatomy.  But after a week we were able to go back to the 8.  After unsuccessfully trying to go to 7 I went to 7 tbsp + 1/2 tsp and she did that successfully for 5 days.  Yesterday we moved down to just 7 tbsp and she did great with it. 

We are supposed to thin a bit more each 4-5 days until we get to either her next swallow study which is Oct 9 or until we get to regular thin liquids and then have the swallow study.  So far, so good.  Dr. Rahbar did say she’d have some trouble here and there (like we did at first try when I tried to thin too much) but the hope is to be on regular liquids by the swallow study.  70% of kids who have the cleft repair surgery are.  I’m praying we are in that 70%!!

Laryngeal cleft repair and post op visit

Jennifer had a type 1 laryngeal cleft that was diagnosed in Feb 2008 at Children’s Hospital Boston.  This is basically an anatomical abnormality in the larynx that allows food and/or liquids to enter the windpipe when they are swallowed.   For Jennifer the problem was with liquids.  After failing several swallow studies she was on honey thickened liquids.

Her surgery was done endoscopically (through her mouth) on July 14, 2008 by Dr. Reza Rahbar.  He is a wonderful man and will always have a special place in our hearts.  She stayed one night in the PICU to make sure that she didn’t have any airway problems immediately or in the first 24 hours post-op.  Here is a picture of her in the ICU just after she woke up:

and another picture of her PICU room:

She had no complications post-op and was eating several hours after surgery!!!  The following day she was moved to a regular medical floor for one more night.  She was so happy to get there as she was quite tired of being confined to the PICU bed.  On the floor she was allowed to go to the playroom to make crafts and walk around etc.  A couple more pics:

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Finally on going home day she was given a bit more freedom and was allowed to venture outside to the garden they have there.  That is one of her favorite places in that hospital along with the bubble area.  Here are some pics of those areas:

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 After being discharged she insisted on going back out to the garden to eat lunch.  There she got to have a visit with the clowns which she loved:

 

 

She has since had a post op visit where Dr Rahbar scoped her throat to look at the stitches and said her healing looked great.  From here starts the journey of weaning her thickened fluids.  For more of her day to day story during the actual surgery you can visit her caring bridge site too.