Feeding Difficulties

Ben has been tube fed since birth, and ever since then, we have been determined that he will one day learn to take most of his feeds by mouth.

Ben, however, has other ideas.

Our dedicated occupational therapist has been helping us to work with Ben for the last 2 1/2 years to get him to make the transition to oral feeds.  Unfortunately, we go through the following cycle over and over again:

1. Ben does reasonably well for a few days,
2. Ben then regresses and absolutely refuses all attempts at oral feeds,
3. We back off for a few days to a few weeks.
4. Repeat.

When Ben was a year old, we took him to a feeding specialist at our favorite tertiary care center.  Of course, he did really well during that session.  Our goal was to get him to eat at least 30 cc by mouth so that we could do a swallow study to make sure he wasn’t aspirating.  Unfortunately, we haven’t been able to get him to that 30 cc goal a year and a half later.

Finally, we visited the feeding specialist again 2 days before Christmas 2008, a year and a half after our initial visit.  We probably shouldn’t have waited this long.  Ben did reasonably well during that session again, the specialist was great, didn’t scold us for not bringing Ben in sooner, and he offered a lot of good, practical advice.  He instructed us to keep feeding attempts short (10 minutes) and consistent, offer very limited interaction during the feed itself, until Ben cooperates by opening his mouth for the spoon.

At that point we are to praise him, and offer him a brief reward (e.g. getting to flip a few pages of one of the board books that he loves so much), remove the reward, and start again.

We came home very hopeful, since Ben had done so well that day.

Unfortunately, we have hit another bump in the road; another period of regression.  Ben has started to open his mouth less and less, and he gets more and more upset with each attempt.  We have gotten to the point where he won’t even take one spoonful in the 10 minutes; he clamps his jaws shut tight, and he shakes his head from side to side.  Our feeding specialist is awesome; he is accessible by email.  I told him the problems we’ve been having, and he suggested offering Ben a single spoonful, holding out until he cooperates, however long that takes, and then rewarding him with praise and removing him from his highchair and letting him play.

Sounds good in theory, but unfortunately we are afraid that Ben can outlast us.   He is a stubborn little guy when he wants to be, and we do have three other kids to attend to.  Worst of all, since we started all this Ben hasn’t been his usual happy giggly self, and that makes me sad.

So I’m not sure what we’re going to do at this point.  I think we’ll pay another visit to the feeding clinic soon and see what strategies they suggest.  If we’re not successful in getting Ben to transition to oral feeds with a home-based program, we may need to enroll him in an intensive feeding program at the feeding clinic which itself isn’t an easy option because it is located an hour away from our home.

Meanwhile, Nate has recently tasted his very first bites of baby cereal.  He made it look so effortless.  Ben was sitting just a few feet away in his highchair, and was completely unimpressed.

Who knew that such a basic thing as eating could be so difficult for some kids.

December

Thanksgiving weekend was REALLY busy, but fun.   Mark’s sister, brother-in-law and their two sons visited all the way from Pittsburgh.  We really enjoyed having them over.  Our boys were very excited to have their cousins here, and all the kids played well together.  It was nice to spend some time with family getting caught up.  We had our turkey with the usual fixings, and there are LOTS of turkey leftovers to enjoy this week.

On Friday, I did a (very) little Black Friday shopping, and went to work for a few hours.  Nate had his four month well baby check that afternoon.  He is growing well (17 lbs 9 oz) and is developmentally on target.  His eczema was TERRIBLE, though, and the pediatrician prescribed him some triamcinolone cream to use along with the moisturizers we’re already slathering all over him.  He only got 2 shots (Prevnar and Pentacel) as well as an oral vaccine (Rotarix).

On Saturday we made our annual trek out to the Christmas tree farm.  This proved to be a little challenging this year because we now have two babes in arms.  Initially we planned to just pick a pre-cut tree from the barn.  However, once we arrived at the farm, the big boys saw the tractor and really wanted to go on the hay ride.  In the end, we all rode on the tractor, and I waited with the babies at the drop-off point while Mark took Isaac and Jonny to get us a tree.

This worked out well since the weather was fairly warm (around 50 degrees).  Ben loves being outside when it’s not too cold and he was in a great mood, and he smiled and giggled, and even stood up nicely for awhile, leaning back on my legs for support.  And surprisingly, Nate was quiet.

The big boys came back with our tree, and we rode the tractor back.  They wrapped up our tree and we went home.  I’m glad we got the tree on Saturday because the weather has been colder and wetter since then.

Can you believe it’s only 23 days until Christmas???

Halloween and Fall Photos: “Where You Live”

We had a very fun Halloween.  I had the day off from work which is ALWAYS a good thing.  The big boys were so excited to get in their costumes.

Isaac had a grim reaper costume complete with a face mask that seemed to drip bloody fluid.  I can’t believe we let him get that!  He was under strict instructions not to drip any blood around little kids.  Not only was his mask scary, but he could barely see while wearing it!

Jonny was a racecar driver.  I got Ben a surgeon’s costume at the very last minute for half off at K-mart.  It claimed to be a size 3T-4T, but it was a very tight fit on Ben.  Even the hat was close to the right size for him!  Nate got to wear Ben’s penguin costume from last year.

We went trick or treating up and down our street.  Just a few houses after that, Jonny found a hornet crawling in his hair, and Isaac decided he’d had enough, and wanted to go home.  That was just fine with us!  We spent the rest of the evening handing out candy.

We live in rural central Pennsylvania.  On Sunday we decided to take some outdoor family photos.  Unfortunately, the weather was cold and overcast and didn’t make for great photos.  We should have gone out on Saturday when it was sunny!  Still, quite a few of them turned out well.  We went to a nearby national park; a truly beautiful place.

My dad was visiting and was able to come with us:

Central PA isn’t too bad to live in.  I just wish it was warm year round!

Memory Loss Meme

Whew!  I’ve had an extremely hectic couple of weeks.  I’ve had to work four late nights and both days last weekend, so that left very little time for blogging!  Hopefully things will be better next week.

I was tagged for this by Misty at A Day in My Life.  The instructions were pretty simple; open the 6th photo folder, then open and post the 6th photograph from that folder.  And hopefully you can remember what it is:

Since this is a relatively new computer, this was taken less than a year ago.  Jonathan got out ALL the plastic animals that he owned and spread them out on the playroom floor.  For some reason, they are migrating toward my computer chair!  I took the photo because they looked so purposeful.

I can also tell you that they weren’t very nice underfoot!

I’m supposed to tag 6 people.  I’m too tired to tag, so if you’d like to do this one, consider yourself tagged!

Goodbye Baby Carrier…

…hello big-boy carseat! After almost two long years, Ben has finally gotten too long for the baby carrier, although he still weighs well under 20 lbs.

Ahhh, much better!

To Test or Not To Test…

Finding out we were pregnant again was very stressful. We love Ben dearly, and he has brought our family so much joy, but all the same we worried about having another baby with CdLS. We understood that the recurrence rate for CdLS is fairly low, at 1-2% overall, but it was not zero. If anything, we were more likely to have a second child with CdLS than we were to have had Ben in the first place!

Most of the time, the mutation that causes CdLS is a sporadic event in the affected individual, and theoretically should not recur in a subsequent pregnancy. However, a few families do have more than one affected child. One possible explanation for how this happens is germline mosaicism in which one parent carries the mutation in the sperm or the egg but not in most of the other cells of the body. If germline mosaicism is present, then the recurrence rate for CdLS can be quite high; up to 50%.

It is a very personal decision whether or not to have special prenatal testing of a subsequent pregnancy to determine whether the new baby has CdLS. We opted to do this mainly for peace of mind, and to help us plan for the future if this new baby DID turn out to have CdLS, but we understand that many families may decide that the odds of recurrence are so low that special testing is unnecessary.

We had an ultrasound early in the second trimester, mainly to confirm my due date, and also to see if our new baby had any obvious limb differences. I had a routine quadruple screen blood test at about 16 weeks (which, incidentally, I never had with any of my previous pregnancies). I had a more detailed Level 2 ultrasound later in my second trimester. All of these tests turned out to be normal. If I had done my research earlier, I would have also requested a PAPP-A test as well. Low PAPP-A levels in the second trimester can be associated with CdLS.

The next big decision was whether or not to pursue more invasive genetic testing on the new baby. Genetic testing for CdLS is available through the University of Chicago Genetic Services Laboratory. We had already obtained a blood sample from Ben last summer, and his sample was sent to the University of Chicago for testing. A mutation was found in Ben’s blood sample in the NIPBL gene on chromosome 5, so it was possible to test the new baby for the same genetic mutation by obtaining fetal cells by amniocentesis. We opted to have this done (not an easy decision since amniocentesis has a 1 in 300 risk of miscarriage), and the amniotic fluid was sent to the University of Chicago along with blood samples from myself and my husband to be tested. None of the three samples had Ben’s mutation, so we are fairly certain that this new baby does not have CdLS.

In pregnancy there are no guarantees, and we still don’t know what special challenges our new baby may have. I am not sure how many parents of kids who have CdLS choose to have all of this prenatal testing for subsequent pregnancies. For us, having the tests helped to decrease our stress level considerably, and I am glad they were available, and that we had them done.

Grasping

Even though he hasn’t been feeling well for over 2 weeks, Ben is definitely showing some signs of progress with his gross motor skills. He first showed signs of grasping toys and other objects months ago, however he quit altogether for some reason shortly after that. He became very resistant to having his left hand touched for any reason, and he made little effort to grasp anything (although he would happily bat at objects and spin them).

We mentioned all this to his physical and occupational therapists, and they thought that maybe he was having some sensory integration issues with his hand. So we started some exercises to de-sensitize his hands and arms. We have three plastic containers filled with different things; one contains rice, one has black beans, and one has lima beans. We started having him immerse his hand in each of these. At first he hated all of them, but he has since come to enjoy the rice and the black beans. For some reason he still hates the lima beans! Then we placed small rings into the beans and rice (almost submerged but still visible) for him to pull out, and he’s been doing great.

Since we’ve started those exercises a few months ago, we’ve seen definite signs of improvement. He now readily reaches for and grasps toys and rattles, and gleefully shakes them. He even puts his toothette (that we use for oral motor skills) near (but not in) his mouth:

One of his favorite toys these days is this drum.  It’s made of real goatskin and makes a very satisfying sound:

He has done the 360 degree roll a few more times – we will have to make sure he doesn’t get too tangled in his feeding tube:

And he is still working on his sitting:

A few days ago we had dinner at our favorite Mexican restaurant.  We put Ben in a big-boy highchair for the first time (and wedged him in well with his coat).  He played happily with his toy for a few minutes, then decided he’d had enough!

We are SO pleased with his progress so far!  Hooray for Early Intervention!

Winter’s Here!

Yesterday evening and this morning brought our first snow of the season.  Isaac and Jonny were THRILLED, although it wasn’t enough to warrant a snow day.  Thankfully it was all melted by midday, but I’m not sure how since the temperature hovered in the 20’s all day.

So, is anyone in my Trini family up for a visit to frigid Central Pennsylvania? 

Hiatus

Mark’s stepfather passed away unexpectedly in an accident last week, so we spent another weekend in the Pittsburgh area for his funeral.  We were all very sad, and the boys will miss their Pappy.  We have just returned home this evening, and I probably won’t update the blog for a few more days until things settle down.   

On Vacation at Last!

My, what a busy week this has been!  But it’s over now, and I’m on vacation until the 25th, thank goodness.  We’re not going anywhere, or doing anything fun, but it is so nice not to have to go to work for awhile.  I’ve not really had a vacation since Ben was born, so this is an especially welcome break.   Isaac has been off school now for a week, and so far has been having a good time, even though he is already complaining about missing his school friends!

One of Jonny’s birthday presents was a brand new bike.  He and Isaac have been very enthusiastic about riding their bikes this week.  Since we don’t really have anywhere safe to ride close to home, Mark has been driving them to the local park to practice.  I went with them on Wednesday before work.  It was a beautiful morning, and Ben slept peacefully in the shade.  Hopefully we’ll be able to go often enough that Isaac will be out of training wheels soon.

Ben seems to be having a little bit of an off-week.  He hasn’t been too interested in oral feeds at all.  His occupational therapist suggested backing off somewhat, and going back to oral stimulation without food for now.  It’s a little discouraging, but we know that teaching him to eat by mouth will take time and patience.

I am cat-sitting for the next few days!  I love cats, but Mark and Isaac are both terribly allergic to cats, so we can’t have one in our home.  Our neighbor asked us to look in on their kitty while they were gone on a trip, and I gladly accepted.   Jonny has been helping me take care of her, and I’ve been getting my fill of cat-kisses every day.

Here are Isaac and Jonny early this morning fighting over who gets to hold Ben first.  As usual, Ben was very tolerant of their pulling and tugging…