Jennifer’s story

Jennifer is our little work in progress.  At 3 weeks old, Jennifer started having problems with eating.  This was about the same time that I had to stop breastfeeding and go to formula.  1, still to this day, wonder if things would have been better for her if I had only been able to continue breast feeding her.  During a feeding, she would arch her back, scream, turn purple, draw up her knees, and pull off the bottle frequently.  We tried different bottles, gas drops, thickened feedings, and every supposed remedy out there with no change in her.  Just after this started she was admitted to the hospital for a fever and sepsis work up.  The nurses noticed the feeding difficulties, but she was in the NICU of the hospital I work at as a favor to me and they weren’t totally used to a term newborn that old (almost a month old) so they weren’t totally sure why she would ask this way.


Once discharged, the feeding troubles continued along with frequent screaming unless she was being held and soothed.  On a Friday afternoon, I was at my whit’s end and was about to call to schedule an appointment for her when I noticed that her cheek was suddenly quite swollen.  Another hospitalization (this time at a Yale children’ hospital) and sepsis workup followed.  She was diagnosed after 3 days with paroditis.  During this hospitalization, the attending pediatrician also diagnosed her with silent reflux.  This started the process of her symptoms being minimized.  He didn’t want to treat the reflux at that time due to the difficulty they were having diagnosing the parotiditis and so that he wouldn’t “step on your pediatrician’s toes.”

The day after she was discharged, she saw her regular pediatrician who stated that she “could” have some reflux but that is was also likely colic.  He did start her on zantac though.  At first we saw a difference.  She slept better during the day (we were fortunately blessed with a wonderful nighttime sleeper, though she screamed all day) and initially we thought we had our answer.  We did some formula changes with minimal results.  Each subsequent visit was met by reluctance to increase her zantac dosage based on her new weights.  I was also told at each visit that she would “outgrow” this.  I do believer that at this time it was felt that I was overreacting to a crying baby and that I was overwhelmed at having a 2-year-old and fairly newborn twins.  In my heart, I knew it was so much more though.

At 4 1/2 months, I had finally had enough.  Seeing my baby being in  so much pain and having little time for my other two children, I took her for a second opinion to another pediatrician in my group.  She agreed that Jen had moderately-severe silent reflux and immediately changed her to prevacid.  I had myself a new baby after a few days!!!  We saw major improvement in feeding and her comfort level.  She did have a major flare at 6 months old, where she again became uncomfortable and refused to eat for 2 days.  I discovered the magic of Mylanta Supreme at that point and was able to ward off full food aversion.  All the while, she continued with gurgly breathing.  She was treated with 2 rounds of antibiotics to make sure this wasn’t a sinus infection.  No improvement was noted.

At 7 months old, we made our first visit to a pediatric GI doctor at Connecticut Children’s Medical Center.  I told him I was afraid she was having lung problems from the reflux and that I didn’t want her to end up with asthma.  At this point she had had several episodes of wheezing.  I was told by him that there is no cause-effect relationship between asthma and reflux.  He did a swallow study on her at 7 1/2 months old.  This showed microaspiration, meaning that formula was entering the airway but not fully going into her lungs.  It was recommended that we change bottle nipple types and continue thickened feedings. We were told she would outgrow both the reflux and the microaspiration.  At this point her reflux was pretty stable on the prevacid and besides a hospitalization for dehydration at 8 months old and the continued gurgly breathing, we were doing well.

At 13 months, we took her off her prevacid assuming she had outgrown reflux since she had been doing so well.  At this point I had noticed that she would cough every time she drank any liquids and asked for a repeat swallow study.  My request was not granted as I was told that she had previously been “cleared to eat”.  The first four weeks off prevacid went well.  The last two went not so well.  She would suddenly start to scream inconsolably.  It took me 1 1/2 weeks to figure out that this was reflux rearing it’s ugly head again.  Mylanta helped me figure out that it was reflux and not behavioral.  It would calm her instantly.  At this time she was put back on the prevacid and I again asked for a swallow study and the coughing had continued.  Again it was denied.

At 19 months, Jen was first seen by a pediatric pulmonologist also through Connecticut Children’s Medical Center for continued asthma symptoms that were viral, exercise, change in temp and emotionally induced.  She was diagnosed with infantile asthma, which came as crushing blow to me.  This was why I had first brought her to the GI doctor.  She was started on singulair, and continued on inhaled albuterol and pulmacort as needed.  Singluair was a wonder drug for her.  It basically took all the asthma symptoms away.  Upon follow up 3 months later with the pulmonologist, I mentioned the coughing with liquids to her and told her the GI hadn’t been concerned.  The pulmonologist ordered the swallow study right away.  Jen miserably failed it and had evidence of full aspiration with every swallow.  She was immediately put on nectar consistency thickened liquids and no longer coughed when she drank.  I really liked this pulmonologist but unfortunately she left soon after this follow up visit for another children’s hospital.

Upon recommendation of the speech pathologist performing the swallow study, I took her to an ENT doctor at the hospital I work at next and had her vocal cords looked at.  They were terribly inflammed and swollen.  He said this was due to undertreated reflux and were causing the swallowing problems.  Once the GI got this news, he finally started taking us seriously and tripled her prevacid dosage.  About this time, she started having episodes at night where she would wake up coughing, gasping, choking, and screaming.  It was assumed that she was refluxing and waking up when she was aspirating and having difficulty breathing from the aspiration.  This would happen 4-6 nights per week and 1-3 times on those nights  Based on this it was elected to do an endoscopy and bronchoscopy.

The endoscopy showed no inflammation in either the stomach or esophagus, so we know she is on enough acid supression to protect them.  She did, however, have some inflammation in the small intestine and was tested for celiac disease.  Luckily this was negative.  The bronchoscopy showed snall amounts of both inflammation and cells evident of aspiration, but both were fairly mild.  

At 2 years old she had her vocal cords rescoped and there was no improvement so zantac was added at bedtime.  Jennifer’s issues were minimized again at this point by the GI doctor when I mentioned the continued vocal cord inflammation and the fact that she was now coughing at times on the nectar consistency liquids.  This was the last straw, as I knew there was so much more to Jennifer’s story.  Needing more answers and help for her I transferred her care to a multidisciplinary team (called the Center for Aerodigestive Diseases) at Children’s Hospital Boston.  They are wonderful, and at her first visit there in October 2007 they did more for her than had been done in all her previous visits combined.

The first thing the team there did was take her off milk and soy.  She had some symptoms with a formula change as an infant that led them to believe that she had MSPI.  With this strict diet, she is almost reflux free.  They also rescoped her vocal cords in the office, and with the added zantac they were finally healed!!  

The next visit in Feb 08 entailed a triple scope (this was delayed several months by a bout of pneumonia) involving an endoscopy (GI), bronchoscopy (lungs), and laryngoscopy (throat/upper airway) along with a 24-hour esophageal impedance study to quantify her actual number of daily reflux episodes.  During these scopes, it was found that the inflammation in the small intestine found on her last endoscopy  had healed on the milk/soy free diet.  It was also found that she has a rare anatomical abnormality called a type 1 laryngeal cleft.  This is what is causing all her dysphagia (swallowing difficulty) and aspiration and is the bigger picture that I kept saying all along was there!!!   Just after her scopes her swallow study was repeated to help them determine her next treatment plan.  She was found at that point to be aspirating the nectar consistency liquids too.  She was then immediately put on honey consistency liquids.  Due to the worsening dysphagia she is scheduled for surgery later this summer to repair the laryngeal cleft.
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8 responses to “Jennifer’s story

  • Fiona

    Hello!

    Wow – I have read your story of Jennifer and I could almost change this to reflect our problems with our son.

    Today, at a follow-up with the surgeon (13th specialist we have seen), a Laryngeal cleft was mentioned as a possible diagnosis. After doing some research and reading your story…I think we might finally be onto something.

    I would love to chat more with you via email if that would be okay.

    Thanks!
    Fiona

  • Natalie

    After reading your story, I could insert my 13 month old daughter’s name in the place of your daughters. We were just discharged after a 9 day stay for pneumonia. My daughter has been treated for reflux since 3 weeks of age, and couldn’t take thin liquids either. We had swollow studies done and they showed nothing, but I didn’t care what the results were, I didn’t give her thin liquids. During our hospitalization, they did the triple scope, and found damage to her vocal cords (discolored with polps and swelling), and her lungs were swollen and full of pus. The EGD looked great, but it should have since she is on twice as much Nexium as she should be on. She now has a NJ tube and is receiving feedings 20hrs/day. The only thing she can take by mouth is her meds which have to be given with lots of cereal. She has to have this for a month to let everything heal. They are planning to do another OPMS in a month and talked about another laryngoscopy because they said due to all of the swelling a cleft could have been hidden. I’m so glad I found your site.

  • jamie fernau

    I cant believe i found your story my 2 and half year old has been on honey thickened liquids for over a year now. she has had 2 bronchoscopies, tonsils and adenoids out, egd and ph probe and all were negative. she has had 3 ct scans of her lungs and it always shows a consolidated lung from aspirating. her last two swallow studies keep coming back worse than the last and i still have no answers she is on the verge of having a ng tube put in. im definately going to ask about the laryngeal cleft lip thank you for posting your story.

    • Tracey

      Hi Jamie!
      I’m glad you found it too then!! It’s so difficult when our kiddos go thru this stuff. Please do ask to see a pediatric ENT for a full evaluation for a laryngeal cleft. As that a rigid laryngoscopy be done as part of the evaluation. A flexible laryngoscopy in the office is NOT good enough as it will NOT show the cleft. Please let me know if you need more information.

  • Megan

    Hi and thank you for sharing Jennifer’s story. What a journey for you both – and good for you for not giving up. Mother’s instinct is so real and so intangible, and we are our babies best advocates. We are on a similar path with my son, who also has a type 1 laryngeal cleft.

    My son, Jack, was diagnosed at birth with Down Syndrome. He was really healthy until he was almost 3 months old, in June 2009. I had heard him making alot of wheezing noises – what we learned to be stridor. After lots of work to get him breastfeeding, we had a ton of trouble getting him to take the bottle when I had to go back to work. It took half a dozen nipples, but we eventually got him taking the bottle – he would dribble at least 1/3 of every bottle but he was taking them. And he had always been a “noisy” eater – with lots of gurgling etc. Jack did not spit up often, but when he did it was always the same – he would have a coughing attack, followed by projectile vomiting.

    The pediatric ENT diagnosed him quickly with laryngomalacia and recommended we work with speech therapists. We were lucky to get a swallow study that showed Jack was aspirating everything and he was admitted and put on an NG tube. All his feedings and meds were through the NG for months. We had a bronchoscopy in early August and learned he had significant swelling from reflux and also had a type 1 laryngeal cleft. Lucky him to be in such a small minority! The ENT did a supraglottoplasty to ease up his tight vocal chords – and we have not heard the stridor since!

    We now have him on honey thickened formula and cereal/babyfood and are working closely with speech therapy at Children’s Memorial in Chicago. We started on a slow flow nipple with the honey – you can imagine how long that took! (We are now up to a fast flow, but he only takes 3 oz or so at a time before pooping out.) It has been a very long 3 months with the NG tube, and we got to the point where his feedings were taking 1.5 hours! With a 3 year old in the house, that is unbearably long. This week we have our next swallow study and i am so hopeful that he will do well so we can at least move him to a nectar. He has some really minor heart problems, and poissible oxygen saturation issues, so he gets really tired eating and we are not sure he has the stamina to take enough calories to allow him to get off the tube.

    The ENT said that 40-50% of the time, kids with laryngeal clefts “grow out of it” so he is hopeful that Jack could avoid surgery. After reading your story, I am not as convinced. I just pray that we can get rid of the tube soon and take it from there. It makes it so hard to be mobile with him but we are working with his schedule to make life easier. Thank God for my unbelieveable nanny!

    I look forward to hearing how Jennifer is doing and watching her progress. I will let you know how Jack does…

    keep in touch!
    Megan

  • Pamela

    My son had his cleft repaired in 2005. I’m so sorry you went through so much with your daughter. I had an excellent Pediatrician who immediately knew something wasn’t right. my son had pneumonia at 5 months old and then got his first and only ear infection at 7 months old. That ear infection turned into Mastoiditis (even through 2 rounds of 2 different antibiotics). They did an emergency mastoidectomy and then sent us to specialists to find out why he got the mastoiditis and why it was a hospital grade infection (pic line meds the only ones that worked.). I finally mentioned to the specialist that my son coughed when drinking and he ordered a swallow study immediately. He failed miserably and was put on nectar thick liquid. he was still aspirating. We were sent to another ENT who did a laryngeoscopy and bronchioscopy and saw the cleft. She called in a friend of hers who is one of the foremost pediatric cleft repair surgeons in the country (OH HOW I LOVE THIS ENT & the surgeon she called in) and they did the repair in 2005. he goes in tomorrow for adenoid removal, ear tube removal and bronchioscopy. The things we do (gladly!) for our kids.!

  • Kristine

    Pam – Who was your ENT and surgeon?

  • tamika

    hey i know exactly how you feel im going throught the same with my son at the moment and have been for the last 2years.. everytime i took hm to the doctors and the hospital they would look at me like it was all in my head it wasnt until he went in for an operation to have his adnoids removed that the anetesis actually realised something was wrong with him.. after seeing way to many specialists and a number of tests we now know whats wrong and are finding out next month when this will be fixed.. ive been told it has a 50% chance it will fix but the other 50% it wont.. im praying that it does fix as im over seeing my baby go through so much and no one taking seriously.

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