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.
My son was diagnosed with a type 1 cleft Jan. 2008 and has since undergone a laser surgery to repair it. However, it was unsuccessful. I was wondering exactly what kind of surgery Jennifer had and if it was successful? My son has been on thickened liquids since he was 6 weeks old and still continues to aspirate. Any info would be greatly appreciated.
I just found your blog, my daughter 8 months old just had her laryngeal cleft repaired endoscopically by Doctor Rahbar 2 weeks ago! So far things are looking good. I’d love to keep in touch as it is hard to find someone with this rare problem!
I’m glad your daughter is doing well. I’d be happy to keep in touch.
Tracey
My daughter is 8 yrs old and has a laryngeal cleft grade 1.5 she has undergone 8 surgeries to date (Deflux injections) just to plug the cleft but they keep disolving and her cleft keeps re-opening she is going to undergo a laser surgery on June 16 here in Ottawa and your story has just given me hope. Take care and what a beautiful daughter you have. If you have any more info you could share about the surgery that would be great.
Claudia
My 4 month old son had a laryngoscopy and bronchoscopy done this morning and was diagnosed with a type 1 laryngeal cleft. He has had chronic wheezing, coughing, congestion, and after switching pediatricians and getting someone to finally delve into things we found out that he was aspirating…and this morning the confirmation of the type 1 cleft was made. I am sad to say that I have to quit breast feeding him and start thickening his feeds. When was your daughter diagnosed? What symptoms/complications have you encountered? The ENT that performed the procedure this a.m. said that typically conservative measures of thickening feeds is attempted and if surgery is still needed it is usually not done until around 1 yr of age. ?? Have you had continued success with the surgery? I would love ANY info. that you have!! Take care and thank you for sharing!!
Hi,
I was just rereading your post. I am wondering how your son’s surgery went and how the feeds have gone. I hope that all went well. Did you end up stopping breastfeeding?
My daughter is nine months old. She was diagnosed with a laryngeal cleft stage 1 when she was 2 1/2 weeks old after having a swallow study and a bronchoscopy. At the time of diagnosis, she had an ng feeding tube put in. I pumped around the clock for 4 months and gave her breast milk through the feeding tube. At each feed we gave her a pacifier so that she wouldn’t lose the sucking reflex. She had the corrective surgery right before she turned three months old and the feeding tube stayed in for another month until we had another swallow study. We were given the OK to nurse or bottle feed then. I have noticed lately that there is a difference between the sounds she makes when she is eating baby food and that of when she is drinking from a sippy cup or bottle. She makes coughing/choking noises from liquids and not with solids. I am wondering if thickening her liquids will help. She is scheduled for another swallow study next week to see what is happening.
The ENT who performed the surgery left the practice, so we have been seeing another doctor from the practice. He told me that he normally does not perform the surgery until the child is one year of age.
I hope that all is well with your son. I would love to hear from you or any of the other people on here who have dealt with thickening liquids and how successful the corrective surgery was.
Best Regards,
Chris
Hi,
I found your blog today in a random search…good for me;) I have a couple of questions for you about you little gal here (she’s a cuite). One is did you have surgery in Toronto? When and with whom? And what type of surgery? I could probably look back through your blog more but I admit I would probably get lost.
My daughter has had a Type 1/2 for 8 months after dagnosis(she is 15months), she has had restalin injections done that haven’t worked so I am now lookingat other options for her. We have an ENT here who is new to Cleft surgery and is a bit out of his league so I am looking for more info. We see him next week and I would like to see what else is out there for help/options
I’m babbling I know but any help you have would be great. I desperate. She has a tube feed now, is fighting aspriation pneumonia again her 11th time since her birth. I have read the post above and I have to say I think we are all in the same boat with this cleft..there is not enough info on them, not enough solutions beyond the thickeners, how do we keep our kids healthy?
wow! Glad I found this. My son was diagnosed with a type 1 laryngeal cleft in 2005 after a severe pneumonia at 5 months old and Mastoiditis (requiring an emergency mastoidectomy) at 7 months old. Dr. Gerber flew in to Children’s Hospital @ Scottish Rite to do his repair surgery in 2005. He was on Nectar and honey thickened liquids. I thank God for the service where I could buy the pre-thickened apple,orange, and cranberry juice boxes!! He was in PICU overnight and did wonderfully well. He had surgery on the Friday before Mother’s day of 05 and was home on Mother’s day! I weened him off of thickened liquids over a period of 9 months to a year. I didn’t rush him. He’s doing well now..just has a hoarse kinda voice. Hope all is well!
Hi our 8 month old has just had his laryngeal cleft diagnosed after months of aspirating and chest infections. He is tube fed. The ENT said 3 months of conservative management and then if that fails to operate. What do they mean by conservative management? Any idea? Take his tube out? thick liquids? Anyone had the diagnosis of a narrow oesphagus with it?
Hi Nicola,
I think I emailed you personally but I know that some kids need esophageal dilations done if they have narrow esophaguses. Is this a consideration?
Usually conservative management means remaining on the thickener and watching what happens as some kids can learn to compensate for the cleft especially if theirs is mild. I’m not sure how this would work for a babe is that is tube fed though.
Good luck and please keep me updated.
Hi I never saw your reply and never got an e-mail. Thanks for answering. The ENT surgeon has just decided to operate. He is still tubefed as he aspirated thickened fluid immediately and got pneumonia. They are fixing the cleft and putting in a gastrostomy as they don’t feel confident he will stop aspirating. I am still hopeful he will prove them wrong and the gastrostomy tube can come out. They are still observing his narrowness so we will wait and see. Did your daughter have laryngomalacia too? They say he may need intubation for a bit in PICU as his throat is floppy. How scary was it being in PICU for you and her?? You are an amazing woman to support all these anxious parents!
My daughter is almost 8 months old. She was diagnosed as having a type 1 laryngeal cleft at 2 1/2 weeks old. She had the corrective surgery just before she turned 3 months. I am curious if the majority of the children who have had surgery had any related problems after the surgery- did any of them have any of the same symptoms or did any require an additional surgery? Over the last month and an half, my daughter seems to be having stridor episodes that are quite loud when she is sleeping, a few times of gagging, and some spitting up. (She does takes a med as a precaution for relux.) She had a cold during this time and we traveled to areas that had different climates than that of where we live, which did seem to bother her a bit. She is eating well, active, no fever, or any other symptoms. I have taken her into her pediatrician, who says that everything seems fine. I am taking her in to see the ENT in a few days. I am trying not to worry, but this has been very emotional for me seeing my baby go through so much and I am praying that she will not need to endure any more surgeries for the laryngeal cleft. I am hoping that it is nothing much, but I started reading things on the internet and I start to worry. If you or any of the readers have any words of wisdom, I would greatly appreciate it. Thank you!
Hi Chris,
I’m not sure if those symptoms could be related or not. I know that the surgery isn’t always sucessful. But if you aren’t seeing the same symptoms as before the surgery then hopefully it was. Did you already have the post op swallow study that showed no further aspiration? Does your daughter also have reflux or is the anti-reflux med just precautionary as you said? I ask because my daughter also has reflux and it gets worse when she has a cold. And her symptoms can be similar to what you are describing. I hope the ENT has some answers for you. If so, I’d love to hear from you again. Good luck!
Hi,
Thank you for the response. Prior to the surgery, my daughter aspirated frequently while nursing and made different sounds. We did have a swallow study about a month after the surgery and everything came back fine. Also, she had a ph probe study done before the surgery and it came back normal. The last few weeks she has exhibited a few symptoms that seem like reflux. So, she may have it now. We saw the ENT two weeks ago, he scoped her in the office and said that the scar tissue was red and swollen, which could be due to reflux. Additionally, one of her vocal chords is tight. The doctor prescribed Reglan in addition to Prevacid. My daughter showed no improvement with the use of Reglan and seemed to be making louder sounds. The GI doctor switched the Reglan to another med and we went back to the ENT. The ENT is requesting another swallow study and wants us to come back in four weeks. He says that if the swallow study comes back with a problem or if there is no improvement with the new med, he wants to do surgery soon and possibly will put in a trach. I am hoping and praying that we will not have to go this route. We have an appt. with the GI doc in one week. We are in a wait and see mode right now. I am having a difficult time not worrying and trying to not let my mind go crazy thinking about everything. I asked many questions of the ENT and was told that everyone is different and responds differently. Do you find that when your daughter has a cold that it takes longer for her to become well than other children? When she has a cold does she have louder breathing and stridor? Thanks for your support!!
I’m so glad I found your site. My son is 15 months and will be undergoing the surgery for a stage 1 cleft accompinied by tubes adnoids and tonsils.Every piece of information I can collect and finding other people who have even heard of this is very comforting. I will be praying that each of your children make a full recovery. Thanks so much and would love to talk to any of the Moms on this site. We are in Atlanta
Good luck with the surgery. I hope all goes well. I’m surprised they are willing to do the tonsils and adenoids at the same time. I asked about doing that as Jennifer has HUGE tonsils and sleep apnea but they said they never do them together here.
Please let me know how it goes.
I wanted to add I love the thickner trashing party and look forward to the day that we can have one too and just drink a glass of water not jello!
We also LOVED the thickner trashing party!! Anytime Jen sees that picture she gets soooo excited!! She will still talk about it and remembers it quite well surprisingly!
You will get there. I know it’s hard to have that faith but you will. And then you can have a thickener trashing party of your own!!
I first read the information you had posted on Jenny in June and felt as if I were reading about my own daughter. Thank you so much for sharing Jenny’story, as it may serve to help my daughter have a more normal life. Let me share her story:
Hailey is my adopted daughter who first came to live with me in August 2007 at the age of 13 1/2 months. She was thriving, but had a diagnosis of “baby asthma” and her breathing was terrible! She wheezed and would cough and sputter every time she drank milk or water from her sippy cup. I took her immediately to my family physician who helped to clear her lungs with albuterol and oral steroids. We then took her to a pulmonologist who ordered a swallow study. On October 6, 2007 Hailey failed this test completely, showing penetration and silent aspiration on everything from thin liquid to cracker.
That day, at age 15 months, she was taken off of ALL oral feeds and had an NG tube placed. Oh, how she missed food!! And oh, how I cried!! After two months of testing (endoscopy, MRI, testing for reflux) she had a G tube placed and also a reduction of a very large umbilical hernia. A Fundo was considered, but her reflux appeared minor so it was not performed. She then started feeding therapy which she adored!! Her desire was to eat! With hard work in therapy and a direct answer to her very first prayer, “Dear Jesus, eat. Amen” Hailey was able to resume eating just 4 months after her diagnosis, but only foods that were honey thick or thicker. In April 2008 Hailey had repair on her heart for Patent Ductus Arteriosis. Although Hailey had made rapid progress on her eating right after diagnosis, the last year and a half showed no progress with her primary swallow. I often asked for her to see an ENT but was told repeatedly that she would probably outgrow her condition. Then in June 2009 I found the site about Jenny while searchng for info on dysphagia. I literally got goosebumps as the similarities to Hailey were so striking! I had a chance to share what I found about laryngeal cleft to Hailey’s speech pathologist in June,2009 only to learn that she had been writing in her reports since October, 2008 that Hailey should see a pediatric ENT to have testing for a laryngeal cleft. (That same day I met with Hailey’s gastroenterologist who told me point blank “Hailey does not have laryngeal cleft.”) The speech pathologist worked on getting the proper referrals and in August 2009 we took Hailey (now 3 years old) to Dr. Mark Boseley, MD a Lt. Colonel in the Air Force who is stationed at McChord air force base here in Washington State. He practices on the side at Mary Bridge Children’s Hospital in Tacoma, WA because he believes in helping as many children with throat abnormalities as possible. He agreed that Hailey’s medical history, including aspiration pneumonias, and her swallow studies warranted larynoscopy testing. As Hailey also had abnormally large tonsils, it was agreed for him to remove them and her adenoids at the same time. Surgery took place on August 24, 2009 and indeed a type 1 laryngeal cleft was found. We are now awaiting a surgery date which will be within the next few weeks to repair the cleft.
We eagerly await this surgery knowing it will give Hailey the chance to finally eat “juicy” foods and no longer be so sad when her older sisters eat watermellon and other such delightful looking foods.
So, once again THANK YOU for sharing Jenny’s story!!! It gave me great information on a condition I had never heard of and the catalist to make sure she saw a qualified ENT that could diagnose her and make a surgical correction. (by the way, we learned today from Dr. Boseley that he did a residency there in Boston.)
I look forward to hearing from you, and will surely make another posting after Hailey’s surgery.
Sincerely,
Julie Evensen
Aberdeen, Washington
Hi Julie,
I’m glad that I was able to help in any way!! That is really why I started this blog. When Jennifer was being diagnosed there was NO info out there on it and I’m a researcher so I really needed more! For that reason I decided to put her story here to help even one other person out. I’m overwhelmed with happiness at the people that this blog has helped. It makes me feel so great that other kids can benefit from the heartache and misdiagnosis we went through.
I’m glad that Hailey is doing somewhat better since starting feeding therapy. That can do wonders and is enough for some kids with type 1 clefts. Just not enough for others. Jen was never even given the opportunity to try it. By the time we got her to Boston they said it was too late for her to make much benefit.
I wish you guys luck with her surgery. It was hard to have patience with waiting after it. But when that day of throwing the thickener away finally came we were so happy. (though she does still cough with liquids more than I’d like….)
In the meantime there are some ways she can have ice cream and popscicles like her sissies. I can share a recipe with you if you’d like. Just email me or comment again here. I probably should just do a post on it huh!
Tracey