Tuesday, May 24, 2011

In Which the Third Time is Not the Charm

I was pretty sure Nolan had failed his sleep study, so I had Dennis come with me to the doctor's appointment. That turned out to be a good idea, since my instincts were correct: the supraglottoplasty has been entirely unsuccessful. His obstructive apnea remains, at about the same AHI (apnea-hypopnea index) as before the surgery.

In our quest to quell the apnea, nothing has worked. The tonsillectomy and adenoidectomy were useless - a procedure that cures obstructive apnea in over 90% of children with the condition. The supraglottoplasty was unsuccessful, which cures obstructive apnea in over 95% of children with laryngomalacia-induced airway problems.

The reason for the failure? Reflux. You have to say it like a dirty word to get the gist of how I feel about it - it has caused so many problems for Nolan, and nothing seems to work to counteract it.

We can hear the stridor returning. The apnea is obviously still there. Whether the reflux is causing the apnea because of a relapse of the laryngomalacia, or causing Nolan to obstruct on the reflux itself, is unclear. We do have evidence that he is failing his pH probes on the Zantac and Nexium, and the respiratory issues remain.

The ENT cannot do anything else for him, since the reflux will destroy any effort to surgically correct the apnea again. She recommended a procedure called a fundoplication, which I'll cover in another post. Trust me, there is no "fun" in fundoplication.

The surgery is irreversible and can be associated with gas bloat, retching, and a need to do more fundoplication procedures in the future. Most kids still have to take anti-reflux medication after the fundoplication is performed.

On the other hand, some children have great success with the procedure, and become totally free of apnea. Unfortunately, there is no way to tell if Nolan will be a success story or if he will have problems after the procedure is completed.

A C-Pap is not recommended for children with uncontrolled, severe reflux because the machine holds the airways open. Since Nolan's airway is likely contracting (laryngospasm) to prevent the reflux from entering his lungs, a machine that forces his airways open will simply provide a clear passageway for the acid to enter his lungs. Essentially, we'd be trading sleep apnea for aspiration pneumonia. I would like to get a second opinion on the matter, however, so I have made an appointment with the sleep neurologist for June 21.

We have decided to hold off for now. Nolan does have apnea, and the apnea will have to be addressed at some point in time: it can cause everything from learning issues, to CO2 retention, to cor pulmonale. For now, Nolan is not retaining CO2 and is growing, even if he is not sleeping well. We want to give him a few months on the high-dose Nexium (20mg, 2x per day) to see if it helps. If he is not clinically improved in September, then we will revisit the fundoplication idea and see a general pediatric surgeon.

In the meantime, we have requested another visit to genetics. Our ENT feels that Nolan does not have a syndrome. There are a lot of reasons for this: primary among them is that Nolan has normal cognition and no major problems with his heart or other metabolic processes. On the other hand, we are concerned that our not-quite-four year old child has had so many surgical procedures. We have a child with a congenital mixed hearing loss, urology issues, mild tone issues, laryngomalacia, severe extra-esophageal reflux disease, amblyopia/astigmatism, and chronic sinusitis/ear infections, and sleep apnea. Granted, many of these issues could be attributed to the reflux (the ear infections, sleep apnea, laryngomalacia, and sinusitis). The others, however, seem rather atypical. I don't know of many four year olds who wear hearing aids, are recommended for fundoplication, have airway issues, wear glasses, and have urology issues. Certainly, it is possible that Nolan got hit by lightning several times in a row - it just doesn't seem very likely.

I am going to make an appointment with Cleveland Clinic genetics to rule out any genetic or metabolic syndrome. Before we even consider a fundoplication, I need to make sure that there are going to be no more "surprises" that could complicate things.

In the meantime, our ENT wants another upper GI barium swallow test. I'm not quite sure why this is: when Nolan had his first upper GI barium swallow, the results were entirely normal. It isn't a very sensitive test for reflux (the gold standard is an impedance probe, or for LPR reflux, a pharyngeal probe). We know he has severe reflux, and I doubt his anatomy has changed during the past two years - though perhaps the test will show whether he is aspirating his reflux (barium would show up in the lungs in this instance).

I am waiting for the sleep study report to get mailed to us (the ENT's office printer was not functional, so I am still waiting for my child's medical records). For us to effectively advocate for Nolan (and to make the best medical decisions), we need access to the full report and the recommendations included by the sleep neurologist.

It looks like this summer is going to be rather busy...


Magic Ear Kids said...

How completely frustrating! Even reading this I wondered if perhaps you should *bleep* that dirty word, reflux.

Wishing you lots of strength this summer,


Julia said...

Yi-yi-yi. So, when do you get to the point where you look back on all this and laugh? Well, I guess you have to laugh a little even now, just to stay sane. I'm sorry about all the continuing bad news and frustration.

TheSweetOne said...

Have I said anything to you already about obstructive sleep apnea also being a cause of reflux? When the airways close, it creates a vacuum pulling acid further up the esophagus. Now I confess this was from another mom with similar issues to Lauren but she's a wise, well experienced woman and I trust her medical knowledge. Might be worth discussing with the ENT? Maybe changing the causal order might help solve some of the other issues? Hope the med stuff gets sorted out soon and the insurance people aren't %*#&^ about it!

leah said...

In Nolan's case, we're pretty sure the reflux is causing the obstructive apnea. We know the obstruction was caused by the laryngomalacia, which was corrected by the supraglottoplasty. He was scoped after the surgery and the obstruction was gone, but five months later it has come back. And his reflux pre-dates the obstruction. I guess it can be a chicken-or-the-egg thing, but at this point they have definitively proven that (in Nolan's case), the reflux is causing the airway collapse and obstructive apnea. The hypotonia probably doesn't help, either. We are going to see the sleep neuro to determine whether the C-Pap will help or not. We can't fix the obstruction without removing the reflux (if we do a fundo, we can move onto fixing the apnea permanently).

tammy said...

Deep breaths. Sending hugs and hoping that questions get answered so much sooner than later. If you do head to Cleveland, let me know and maybe we can meet for a little fun at the park or the zoo before your road trip back. Always thinking of you and your sweet boys. Sending prayers you find answers, I can't even begin to imagine your frustration.

PS - You truly deserve a PhD in all this. ; )

rouchi said...

I can just imagine the frustrations of a mother who needs to deal with things like this.I really pray for you and Nolan.Hope all good happens soon.Hope you get the solution to this. Wishes for you.

Herding Grasshoppers said...

Oh Leah, how discouraging! (Of all the times we wish that Mother's Instinct was wrong!)

Praying for you all - for wisdom in decision making (which you have in great measure), and for Nolan's HEALTH :D

He is SO BLESSED to have you for parents, looking out for his best interests.


'Zilla said...

I just found your blog while googling in regards to my son...I cannot wait to sit down and read the entire thing! See, my son has had horrible sleep problems since birth, reflux that went undiagnosed until he was 1.5yrs old and he just turned 2 end of May and is pretty much strictly breastfed, with very little amount of babyfood...he is going for a feeding evaluation on the 21st. I'm curious to see what you went through, your blog is making me feel so much better already, just knowing I'm not alone...

leah said...

Zilla, we really struggle with the reflux more than anything else. I wish you luck with your precious little one! Nolan was failure-to-thrive and was undiagnosed with severe reflux until he was 2 years old (we found the severe apnea at about 2 1/2 years of age). There is a wonderful parent forum at parent-2-parent which discusses reflux issues in depth. They have been immeasurably helpful to me! http://parent-2-parent.com/forums/forum.php