Ben’s Early Months

Benjamin was born at the end of April, 2006 at 36 weeks gestation.  My pregnancy was complicated only by high blood pressure toward the end.  Labor was induced, and Ben was born quickly – the doctor never made it in time to catch him and he plopped out on the bed! 

Newborn Ben

Right away we noticed that Ben was a little different.   His right arm wasn’t completely formed and he had only 1 finger on that hand.  He was very tiny (4 lbs 8 oz)  and his head was smaller than normal.  (None of this was noted on prenatal ultrasound.)  He was, however, pink and vigorous, and seemed to be doing OK after birth, that is, until we tried to feed him.  He stopped breathing each time and turned blue – very scary to see.   Our pediatrician decided then to transfer him out of our local hospital to the NICU of our nearest tertiary care center which was more than an hour’s drive away.  The transfer took place the day after Ben was born.  Fortunately, I was able to be discharged the same day.

Ben was seen by a geneticist at 2 days old and diagnosed with Cornelia de Lange syndrome (CdLS).  We were given a very poor overall prognosis for him, and I have to say it was one of the most difficult days of my life so far.  But Ben did well in the NICU – the immediate problem was feeding.  He was unable to eat by mouth without aspirating (breathing in his food) and so we had to find another way.  In the NICU he tolerated feeds via nasogastric (NG) tube, and we prepared to have a gastrostomy tube placed as a long-term solution.  However, the surgery was cancelled because the anesthesiologist was unable to intubate him.  They felt that it was safest to wait until he was a little older and bigger before trying to do the surgery again.

Ben NICU SnugliNICU learning to place NG tube

We brought Ben home from the hospital with an NG tube in place the day before Mother’s Day.  It was a very happy homecoming, and Ben’s brothers Isaac and Jonny were SO happy to finally have him home!  We were able to handle the NG tube feedings without too much difficulty, but Ben had continuous problems with reflux (vomiting) with every feed.  He was initially started on Zantac upon discharge from the NICU, but Reglan was added at about 1 month of age when he vomited so much that he was actually starting to lose weight.  On both medicines he did reasonably well with his feeds.  We used (and still use as of this writing) a feeding pump (a Zevex Enteralyte which we love) for all his tube feeds because he does not tolerate large volumes in his stomach.  His bolus feeds are run over a 45 minute period.

Right before G tube and Nissen surgery 

We kept him on NG tube feeds until he was 5 months old.  He finally had surgery in early October 2006 both to place a long-term G-tube and to have a Nissen fundoplication to prevent the constant reflux.  The surgery was done laparoscopically (only 4 tiny incisions) and involved less than a 3 day stay in the hospital.  Ben was sore afterwards for several days, but perked up nicely with doses of Tylenol with codeine!

Other than that, thankfully Ben has had no other major health issues that we have identified so far.  He does have some hearing loss (which can sometimes go along with CdLS) and wears hearing aids during the day.  He is developing very slowly, at his own pace, and we feel blessed to have him as part of our family, no matter what challenges we will face in the years ahead.

17 Responses to “Ben’s Early Months”

  1. kelly Says:

    I dont know if this post was here before day as I have never noticed it but I apologise if it was. It explains all the questions I asked you, I feel a bit guilty now for badgering you.

    Ben is precious. I can only imagine the shock you felt at his birth. Sonny was 2 days old when cdls was mentioned to me as a possibility. I had 2 days of relative normality before the carpet was pulled from under our feet.
    Your little one has been through so much so soon, he is incredible.
    I always say too, how lucky Sonny was to be born with the 3 siblings he has. Looks like I can certainly say the same for Ben. How cool to have 2 big brothers there to look out for him in the future.

  2. kfb1 Says:

    Yes, this page has been up since the beginning, but that’s OK – I definitely don’t feel “badgered”! You can email anytime! Yes, Ben has been through alot already. I hope he will never need any more surgery or stays in the hospital. You are right that he is lucky to have his 2 big brothers – they love him to pieces! Sonny is definitely lucky too – I love seeing photos of your kids together.
    – Karen.

  3. Dee Golden Says:

    We have been approached about adopting a two yr old with this condition…. any insights. We have been trying to adopt ofrever, it seems. Any advice would be prreciated.

  4. kfb1 Says:

    Dee,
    It’s difficult to say which issues you would face with any particular child with CdLS. Some kids are very mildly affected, and some (like Ben) have the more severe form. I would suggest contacting someone from the CdLS Foundation at http://www.cdlsusa.org for more specific information about the syndrome itself. You can also email me privately at benandhisbrothers@yahoo.com and I can try to answer any questions you might have. Some people on the CdLS online support group (the link for this site is higher up on the screen on the right sidebar) have adopted children with CdLS and can tell you what that has been like for them. Good luck with your adoption journey!
    – Karen.

  5. jason Says:

    my son has cdls as well. i see alot of the same things in johnathan as i read about your son. even his right hand looks about identical. the only thing i have read that j-bake doesn’t have in common is he has a trach. that is because he had the small lower jaw. ben is 2 months older than johnathan. we have our moments of having to go to the hospital for stays but as we look toward the future things look bright especially since he was born since our dr. thought he had trisomy 13 or 18 which not compatable with life. well good luck with all and i hope your family continues to do well -JASON BAKER

  6. Karen Says:

    Hi Jason! I remember emailing your wife back when our boys were still tiny babies. Glad to hear from you guys! I hope little Johnathan stays healthy through the winter and stays out of the hospital. Ben has a small lower jaw that makes it difficult for him to be intubated before surgery, but not small enough to require a trach.
    Like you, we are hopeful for the future.
    🙂
    All the best.

  7. Fiona O'Mahony Says:

    HI Karen,

    Love Bens story. Very similar to my 6 mth old Aaron. We’re still experiencing reflux but peg was put off for another 6 mths as he is having a ‘balloon’ inserted in his pulmonary artery at end of 2007. Aaron had been bottle feeding but contracted rotavirus in hospital and is on NGT since. He has not limb differences except for his baby finger. We’re very lucky in that respect. He is a happy baby when not sick and not in pain from trying to pass wind down. No constipation but an awful lot of uncomfortable wind. Maybe need to try a different formula.
    All the best.
    Fiona

  8. Karen Says:

    Hi Fiona! Thanks for writing about Aaron! I hope his balloon procedure goes well, and that you find the reason for all the gas. Ben is gassy sometimes, but not always – there doesn’t seem to be any rhyme or reason to it most of the time…

  9. kimmie Says:

    I am so glad you came to Jayden’s World,and even more wonderful is the fact I am talking to someone with the same challenges my family & I face everyday.Reading about your little fellow brings me back to Jayden’s stay at the hospital when she was born.What a trial those days were.I’m still learning….Thank you for subscribing to her blog.I look forward to talking more with you…How did you hear about the blog?I also have a website you can reach right off the blog if you are interested.Or you can go there directly http://www.jaydensworldcdls.com
    Thank you for writing,somehow I don’t feel so alone.There are none that I am in contact with here in Canada.Only one that I know of here in NB.But I haven’t been able to make contact.
    kimmie Bevans.

  10. Karen Says:

    Hi Kimmie! I do searches for CdLS blogs though http://www.technorati.com every now and then, and yours popped up! We don’t know anyone close by who has a child with CdLS, but I’ve met so many other families online. Check out my Blogroll! You might also be interested in joining the CdLS online support group through Yahoo groups – the link is on the sidebar to the right – I’ve found it to be really helpful to talk to other parents about some of Ben’s issues. It’s good to meet you!

  11. Liza Says:

    I just commented on another post, but had to pop up and say THE ZEVEX ENTERALITE RULES! LOL! Aside from some issues with the latch on the housing where you place the new lines that they solved midway through our usage of it, that thing is awesome!!! My little one now tolerates gravity feeds through a mic-key button so no more pump, but while we used it it was sooooo nice to be able to hang a bag, set the drip, and all sleep through the night…

  12. maricela valoiz Says:

    hi my my name is maricela valoiz well i was searching for pic of g-tubes and i found this page i want to tell you that i was surprise to read the diagnosis they gave your son by reading your story i can tell that you guys have been thru alot and you have been very strong.Well my daughter is a 1 month old her brain is not well developed (brain damage) and that syndrome is called dandy walker and she is still in the hospital since she was born and also she is not able to eat throu her moth so they told me that she needs a g tube but im scare and i want her to be fine and learn to eat throu her mouth we are practicing so she can learn how to do it , but her throat is very sensitive and floppy and when she eats some milk goes into her longs so that is why she is getting the tube.well i hope u send me and email and hope hope ur son is doing better now God bless you good luck i will be praying for ur son

  13. Fiona O'Mahony Says:

    Hi Karen,
    Congrats on your new baby boy. He looks lovely. I’m expecting my third baby, aaron being my 2nd who has cdls. We seem to be over the worst with Aaron and he’s improving daily. He is still not sitting and we’re hoping we’re
    nearly there as he’s almost 18 mths old now. I think I remember that Ben was sitting at 17th months. He needs a lot of therapies but thankfully the dr. visits are not as frequent. He’s on losec for acid reflux but never seems to show any signs of suffering from it. We’re reluctant to take him off though as he’s doing so well. He’s rolling onto his tummy and today for the first time he rolled back again. We’re thrilled with each new development. Another milestone reached. Take care.
    Fiona

  14. Narjis Says:

    Hey i just reed your blog while i was doing a assignment in my English class and i got to say i was amazed how you cared about your four kids and helped them and kept them on the encouraging track its wonderful reading about them and also hearing them achieve such amazing goals
    I would love to hear a reply from you and i was also wondering if i could interview you about my English assignment.

    Thanks

  15. Jennifer Says:

    awww baby boy!!! you are one amazing little prince.
    He is blessed to have such loving family and his family are blessed to have such an amazing little man!
    He really is a strong strong boy…
    kisses for him.
    xxx

  16. Nola Radford Says:

    What a wonderful website and thank you for sharing your family’s journey, I am writing a book about children with special needs learning to communicate. Would you be willing to share a picture–of your choosing—for me to use? Thank you for considering my request.

  17. Elizabeth Says:

    I was doing some reaserch on g tubes and I came across this beautiful boy and realized how much he looked like my baby boy Adam. The picture I saw took me directly to this site and I am shocked and happy to say that our stories are so much alike. I have 3 boys 7,3 and 2 the 2 year old is Adam he is my little angel I never really had the courage to speak to anyone about his syndrome but with everything that has happened I am ready to share. (I hope you understand what I am saying) well just wanted to say God Bless you and your family. Keep strong. (I know this post was posted a long time ago, but it is a very touching story, hope everthing is going well.)


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