? laryngeal cleft

[tillya]

From the moment I brought my daughter home from the hospital, I knew something just wasn’t right. She was a full term baby with normal vaginal delivery. The only complication of my pregnancy was bleeding during weeks 6-12. During that time I was on bed rest and prescribed progesterone. From week 13 on, I had a normal pregnancy. My daughter cried all the time. And by all the time, I mean there was nothing anyone could do to comfort her. She would not lay flat in her crib. I would either hold her, or when she did sleep, put her in her infant seat. After 2 weeks of this, she remained at her birth weight of 6lbs and 6oz. I went to a lactation speciality and realized I wasn’t producing enough breast milk. I would nurse, pump and bottle feed as a supplement. At 3 weeks she was gaining weight, but continued to cry. She had a barium swallow that was normal (showed decreased paralstalysis of the esophagus). She was being treated for reflux. We tried Zantac, Pepcid, and Prilosec, but nothing seemed to help. At 2 months, we added cereal to her bottles. She still remained miserable. At around 5 months, she went to a pediatric GI, and he prescribed Prevacid and changed her formula to Elecare and we continued with cereal in all her bottles. That seemed to help for awhile, she still would spit up, and have wet burps, but no longer cried every time she burped. I think this was about the time the breathing problems developed. She had a deep cough, and wheezing and rattling, but remained afrebrile. She was started her on Xopenex, Atrovent, and Pulmicort nebs, and was also given antibiotics and steroids. She would exhibit these respiratory symptoms for about 2 weeks, get better for a bit...days to 2 weeks max and then the cycle would start again. After a few cycles, her pediatrician referred us to an allergist and pediatric pulmonologist. Her allergy test reveal 2+ for cats, dogs, and feathers. The allergist was concerned about her cough, and had her tested for CF which came back negative. She had a saliva test done. We then went to the pulmonologist and she tried her on an inhaler (Flovent) instead of all of the nebs, but my daughter wouldn’t tolerate the mask. Her pediatrician continued her nebs-- Pulmicort bid, and Xopenex and Atrovent prn. The pulmonologist also ordered a modified barium swallow. My daughter coughs when taking thin liquids (pedialyte or juice, and even meds) and has always had to sit upright to get her bottle. The modified barium swallow was normal even though she did cough during the test. At 9 months, she started on Nexium. We continued with life, and dealing with the respiratory “cycles”. I’d even catch myself saying “ooh Sunny has been doing so good”, and it would have been less than a week. At 11 months she got a UTI, and was started on Bactrium. 10 days later she went to her follow up appointment and was doing well and looking great for her. The very next day, she had grunt breathing, temp spiked to 103, and she just didn’t look good. WBC was 20. She was admitted to the hospital and spent 6 nights there. Her pediatrican was stumped, diagnosis could be respiratory infection, urosepsis, or viral. She came home and was without symptoms for 2 weeks. Then respiratory problems started again. She had been wheezing, coughing and rattling for weeks. During this time she has seen her pulmonologist who started her on Singular and looked at her and said, “Sunny I don’t know what we’re are going to do with you”. She has also been to her pediatrician for her well baby visit, but was unable to get her immunizations because of her respiratory status and her temp was 99.3 He told me to keep doing what I was doing and hopefully she would outgrow this. Her current meds are Nexium 10mg q am and 5 mg q pm. Singular 4mg q pm. Pulmicort bid, Xopenex q 4 hrs w/a, and Atrovent tid. She has been on multiple antibiotic and taken 2-3 rounds of oral steroids. She has been treated for thrush 2 times. She has had 3 chest xrays, barium swallow, modified barium swallow, renal ultrasound, and vcug all of which were negative. She has tested neg for rsv 4 times.
For 3 weeks I listened my little girl wheeze and rattle and get short of breath just walking around trying to play. Deep down, I knew she wasn’t going to get better with time. I ran into a lady that recommended I call her son’s doctor in Atlanta. Her son has CF. The doctor was an allergist/immunologist, and since my daughter had already been tested for allergies, and was negative, I was afraid he couldn’t help. I debated for about a week and finally broke down a called. This is where divine intervention took place. Our appointment was scheduled for 4 days later! The night before the appointment, my daughter sat in her highchair and was gurgling so bad it sounded like she was drowning. I prayed that the Lord would look after her and keep her safe through the night. We made our 4 hr journey to Atlanta, and after a long history, the doctor said he wanted to admit her for some test. The very next day her temp spiked to 103. I was so thankful we were in the hospital. She had a sweat test for CF which was negative. Her chest xray was full of infiltrates. She also had another barium swallow that was normal. ENT, GI, and pulmonology were called in. She had a laryngoscopy, bronch, and EGD. The laryngoscopy revealed that her vocal cords were rigid and discolored. The ENT doctor said it looked like it was from chronic uncontrolled reflux. Her bronch showed that her lungs were swollen causing many of the passageways to be extremely narrow and her lungs were full of pus. The GI doctor said her EGD was normal and that she didn’t even have reflux. I was very confused by this, thinking that being on so much Nexium, there wouldn’t be any erosion and that her esophagus should look good. She also had an esophagram that was normal. And then a repeat modified barium swallow. She didn’t tolerate this very well, but it did show some microaspiration. The pulmonologist said it would take 3 weeks of antibiotics and 4-6 months for her lungs to heal. A different GI doctor came in to see us, and asked if anyone had talked to us about surgery for the reflux. I was puzzled by this because her partner stated my daughter didn’t have reflux. So after a 9 day stay at the hospital, we still don’t know what is causing my daughter to be so sick all the time. Is all her respiratory problems from aspirating when she eats or drinks, or is it her reflux causing the aspiration? That’s the million dollar question. To prevent further respiratory problems, the only thing she can take by mouth is her meds, and they have to be given with a lot of cereal. She has an NJ tube with Elecare at 48cc/hr for 20hrs/day. We are supposed to go back to Atlanta in a month to the Aerodigestive Clinic to see the pulmonologist and GI and to also repeat the modified barium swallow. They said she may have to have another laryngoscopy because she may have a laryngeal cleft that was hidden by all of the inflammation.
My daughter has been receiving tube feeding for almost 4 weeks now. She hasn't had any respiratory problems since the tube feeding were started. We are still waiting on follow up appointment to determine further treatment.
When they wanted to test my daughter for CF, I knew she didn’t have it. In my heart of hearts, I really think she has a laryngeal cleft, especially after reading your daughter’s story and others too. After reading things on the internet, it is my impression that LC is difficult to diagnosis because it’s rare and the ENT really has to know what they are looking for. I’m so glad I found your site...there is so little on the net about LC.

[sdsweet]

Hi, My daughter Amaya has a similar history and was finally diagnosed with a laryngeal cleft. She did have a scope at the age of 1, but we were told she was fine. This scope was done by an ENT at one of the best children's hospitals, but he didn't know what to look for. At 2 1/2, we went to a Dr who specialized in clefts. He said that to properly diagnose a cleft, you need to have a double scope (two scopes put in at the same time by an ENT and pulmo) to stretch the larynx. I hope you can find out what is going on with your daughter. Amaya was about to have a g-tube, when she was finally diagnosed correctly. Take care, Stacey

[tillya]

My sweet Sunny Bunny had her 3rd OPMS done today. She had silent aspiration on thin and nectar thick liquids. No aspiration of thick puree or honey thick liquids.
She will continue her NJ tube feedings for hydration and is able to eat food. The speech therapist wants me to give her 10cc of honey thick liquids a day with a cup so that she doesn't get delayed with drinking from a cup. (She wouldn't take a bottle or a cup during her test today.)
I spoke with her pediatrician this week about being referred to a pediatric ENT that specializes in airway problems. He agreed to that, but we are having the fight with the insurance company once again. Hopefully we will be getting that appointment soon. In the mean time, I am so thankful and so blessed that Sunny is doing well now.

[hailbysmom]

I'm glad to hear that Sunny was doing well at your last post! I hope everything is still going good. My son Michael, whom was diagnosed with his LC 1 1/2 years ago, began having problems respitory wise earlier this year. His pulmonologist diagnosed him with allergies to dogs, peanuts, and mold and prescribed Singulair along with Pulmicort and Albuterol via neb. This has helped, but he still struggles everyday with the many difficulties that having an LC and reflux and paralyzed vocal cords brings. It has taken us 3 years in total to find the many medical issues my son faces, but with a great team of specialists he lives a "so-called normal life." Believe it or not-children deal with things so much better than us parents do! He does have a modified diet and is banned from physical activity (long-term soccer and other such sports-due to a heart problem) but loves to play with his toys and be as boy like as possible. I just wanted you to know that it seems like there are never any answers and that days just get worse, but stay strong and very informed, even if that means you doing all of the research yourself, and one day there will be a breakthrough. I wish you the absolute BEST OF LUCK!