Pediatric Rheumatologist Appointment

Goodness gracious, it has been hot and humid lately. It was 95 degrees with 98% humidity the other day, and we were miserable in the sultry heat. Living in Western NY, we don't have central air conditioning, because summer typically lasts approximately 5 minutes and winter lasts for the rest of the year. Nolan sweats more than most kids, so he was literally soaked through in this weather.

Three days ago, a dear friend called to tell me that our beloved ENT was canceling all of her appointments. More specifically, her office was canceling the appointments on her behalf. There has been some sort of catastrophe and they do not know if she will be seeing patients again. I still have no idea what has happened, but I am worried for her and for her family. Of course, this leaves us in a bit of a lurch, as Matthew still has significant sleep apnea after his supraglottoplasty. Nolan is fairly complex as an ENT patient as well, so we need to find a replacement that can handle the boys' issues. I have a call into our ENT's nurse practitioner and we will see what she recommends.

Since Nolan had a pediatric rheumatology appointment, I thought I'd ask her opinion, too. His rheumatologist is fantastic, and always takes a good deal of time with our family.

We talked about our latest concerns, which include:

• Bradycardia (slow heart rate) when sleeping - his heart rate dips into the 30's on some occasions.
• Difficulty with sustained walking.
• Hearing loss and management.
• Periodic leg movements and waking at night.

Nolan's weight is hanging in there at 42 pounds, and he is 45.5 inches tall. His nighttime feeds are going well, so that part of his care is fine.

She wants Nolan to see a cardiologist, because his last echocardiogram and EKG were when he was 3 years old, before he had developed the heart murmur he has now. Adding in the bradycardia, it would be good to get a consultation. It is likely that everything will be reported as fine, but kids with connective tissue disorders have a higher risk of having cardiac issues. Better safe than sorry, in any case.

As far as his hearing and other ENT issues, she has a few recommendations. She is going to contact a few other families in the area to see what they are doing with regard to seeing an ENT. 

The periodic leg movements are not due to electrolyte deficiencies or to anemia (he had had blood work during the same time period as his sleep study), so it is likely there is a muscular or nervous system issue at work there. He has some minor central apnea as well as the gastroparesis, so there is something "going on" with his autonomic system. They can treat leg movements with amitriptyline, but this relaxes the palate and airways... which makes the medication a bad idea for a child with congenitally floppy airways. We'll just ignore the problem for now - there isn't much we can do about it. I am driving him to school, which lets him sleep a bit longer in the mornings.

Nolan's lack of endurance and fatigue is becoming a serious issue. Currently, we are carrying him when we are in a city or on vacation. As he grows, carrying him is becoming more difficult. He doesn't fit into a standard umbrella stroller anymore, so the time has come to look into a medical stroller. He won't need to be fitted for a pediatric wheelchair, as he doesn't need full-time use - he just needs a mode of transportation when his legs and body give out. 

We're going to look for one second-hand (Maclaren makes a medical stroller called the "Major," which isn't horrifically expensive - less than $1,000). With Nolan's complexity and mounting medical costs, she wants to investigate acquiring Medicaid as a back-up for covering medical costs. Most medically complex children have Medicaid, because private insurance does not cover DME - in the past, we have paid for Nolan's g-tubes, C-pap, and hearing aids out of pocket. Now we're adding a medical stroller. If he qualifies, we'd use our private insurance for most things, but the Medicaid for the surprising number of things private insurance doesn't cover. His medical costs can be staggering at times, so having a little help for things our insurance company won't cover.

It was a very productive appointment, and she gave us a few good ideas on where to look for a second-hand medical stroller.

We return to Buffalo on Saturday for Nolan's MRI (inner ear). 

Still Here, Just Incredibly Busy!

We have been so busy in the past 2 months. Some of the "busy" has come from medical appointments, but baseball/tee ball games, Piano Guild, and simply playing outside in the good weather has kept us hopping over the past few weeks.

On the "medical" side of life, Nolan's stridor has already returned post-surgery. Some nights he is quiet, and on other nights he is noisy. We have no idea if there is apnea associated with the "noise." Stridor is fine as long as he isn't dropping oxygen saturation numbers. Nolan will have a sleep study on June 23 to determine how he is faring.


Nolan had a vision appointment, and that went very well. His vision is now correcting to 20/20 in both eyes! When we first started treatment, his vision was 20/80 in the poorer eye (corrected vision - this means his vision was 20/80 when he was wearing glasses). Some people have asked if Nolan will continue to have to wear glasses, and the answer is "yes." He must wear them full time, or else the eyes may "separate" again (he could lose vision in the amblyopic eye). He is also nearsighted, and nearsightedness does not improve in children. The typical course for myopia in childhood is for it to worsen, as the eye lengthens as the child grows.

He has had to come home once from school, and was ill at school twice over the past month. This was not due to a virus, but caused by his gastroparesis. His stomach was bloated and he didn't tolerate his night feeds. This is a problem that we are monitoring, but he is growing and managing to do well overall. We take the bad days in stride and carry on.

We also had Nolan's IEP meeting, which will have its own post (because this one is getting too long)!

On the hearing front, things are looking good. He's only had one infection in the right ear since he had his fifth set of tubes placed in October 2012. His hearing levels were stable, with some improvement in the low frequencies (he had been testing at 75dB in the low frequencies, and he has returned to 60-65dB). We still cannot get aided testing performed, and have had no luck in finding an audiology location that will do real-ear measurements or aided testing. Nolan has some articulation errors due to this issue (he cannot discriminate "sh" from "ch," for example) - the articulation errors are minor and most people can understand him most of the time. He does quite well, despite being aided to only 40-45dB in the high frequencies (aided testing was performed at Buffalo Children's, but they cannot adjust his hearing aids).

 Nolan's right ear improved from 75dB at 500-1000 Hz to 60dB. His left ear, however, decreased from 45-50dB at 2,000-4,000 Hz to 55dB. His right ear drops to severe (70dB) at this level. His ears tend to "catch up" to each other over time. 

Nolan is able to detect spoken sound when it is at a volume of 50-55dB. He cannot comprehend words until they are between 85-96dB in volume. His aided hearing was not evaluated.

We have an appointment with the pediatric specialist in Buffalo on May 22. I am anxious to have the doctor take a good look at Nolan and determine what is causing his leg pain (and hopefully put some of the puzzle pieces together to figure out what is going on with our boys)!

Matthew is still having difficulty with his sleep apnea, and we will see the ENT for his tonsillectomy/adenoidectomy follow-up on June 3. He is on Prilosec and hopefully that will help with the inflammation in his esophagus/airway. Matthew still struggles with severe constipation and we saw a GI doctor in Rochester to try and sort the problem out. They believe he struggles with motility issues (in the same way Nolan does, but lower down in the GI tract). He is on an aggressive medication regime in the attempt to get the problem under control. There is some concern that he might have eosinophilic esophagitis (the concern is there with Nolan, too). This is an allergic condition where white blood cells called eosinophils attack the esophagus. This would explain Nolan's weird "exudate" during his last procedure, and we know that Nolan has a high eosinophil level from his biopsy. The high levels were blamed on reflux, but we may have to revisit the issue since Matthew also struggles with reflux and inflammation with motility problems. We won't deal with this potential issue until we get Matt's sleep apnea under control.

The appointments should slow down as summer approaches, and we are looking forward to having things quiet down for a few months! Matthew will require another sleep study, and there is a good likelihood that he will need a supraglottoplasty to trim away the floppy airway that prevents him from breathing well at night. Hopefully this will be the LAST procedure this year!

ENT Appointment: Ear Infection Damage Control

Heading into Space at the Museum of Science

Honestly, Nolan gets the worst ear infections I have ever seen. They appear whether or not he is on antibiotics, and they last for 2-3 months at a time. It is simply ridiculous. The last one he had was in October of 2012, which is when he had his tubes removed and a fifth set placed. The ENT had to place 2 tympanostomy tubes into his right ear to drain the infection.

This morning, he was draining infection from the right ear, and I called the ENT's office. "Can you be here by 11:30 am?" they asked. With a lot of rushing, we made it out the door by 9:30 am and were on our way to Buffalo. My mom is visiting from California, so at least the boys had some extra company in the car!

There was almost no one waiting at the ENT's office, which was a relief. We saw the nurse practitioner today, and she was fantastic. Nolan did have to take a trip to the procedure room to have his ear cultured and vacuumed out. He started screaming and thrashing the minute we started down the hall - he hates having his ears cleaned out.

The NP was very, very gentle and we got it done as quickly as possible, but Nolan was NOT amused. He only kicked her twice this time around, which is a slight improvement over last time. She went to discuss the situation with Nolan's ENT, since he has surgery planned on Tuesday. The ENT verified there was nothing else to be done, and also said, "He better not get sick!"

Ugh.

I will call and find out the culture results on Friday. If it is a fungal infection (again) he will need Lotrimin suspension for his middle ear. If it is a resistant bacteria, then we'll need a different antibiotic. At the moment, we're continuing with the oral Augmentin (he was already on this medication) and add in Ciprodex drops.

After the appointment, we took a trip to the Buffalo Museum of Science. Nolan was very impressed by the mummy exhibit, and told Dennis about the "dead mummies in boxes." Both boys loved the hands-on exhibits and it was nice to have some "fun time" with my mom.

September ENT Visit

I took Nolan in for his routine ENT visit. Of course, with Nolan, there is almost never anything "routine" since he has a plethora of ENT problems.

Fundoplication vs. C-Pap

We discussed putting off the fundoplication indefinitely, though I realize we may have to revisit the issue again in the future. Our ENT feels that Nolan's "awful August" was directly caused by the C-Pap - he started C-Pap therapy in August and became very, very ill with retching, choking, and exacerbated reflux symptoms. Apparently, they've seen this reaction in the other kiddos with severe acid reflux. On the other hand, Nolan is doing extremely well this month, and seems to be tolerating the full-face mask much better than the nasal masks. Either he has adjusted or we're just having a great month. Time will tell, but I have every finger and toe crossed that the full-face mask is simply negating some of the issues of the nasal mask (the nasal mask forced air directly into his nose, whereas the full-face mask simply pressurizes the air around his entire face).

Balance Issues 

Nolan was quite wibbly-wobbly in the office and fell a couple of times. We've seen this at home a lot, but it is the first time he's done it in a doctor's office. We've been attributing it to his sometimes wonky gait or to his amblyopia (both of which can cause "clumsiness"), but she thinks he has something vestibular going on. It would make sense since he has had a progressive hearing loss, but we'll see. She wants to do some sort of test in a spinning chair, but I don't really see the point. If it is vestibular, it isn't like we can do anything about it. She does want him to avoid contact sports that might result in a blow to the head because of the balance and hearing issues - which is strange, because he doesn't have enlarged vestibular aqueducts (these are the precautions usually given to kids with that "issue" who still have residual hearing). I'm letting it go - he's a little clumsy now and then, but it isn't the end of the world (and it isn't really a "fixable" thing, anyway).

Sinus Issues

His sinuses are horrible, of course, but they have been horrible for the past three years. With his form of acid reflux, the acid shoots past the esophagus and hits the nasal passages and airways. She ordered a CT scan of his sinus cavities to see how much damage has been done: cue episode 547 of "Reflux Sucks." Since he is a C-Pap user, he may require intervention with his sinus cavities, since they are chronically infected and he can no longer breathe through his nose. Another thing that I'm not too worried about, but we'll see what the CT scan shows.

The Dreaded Earwax

He had wax in his left ear, so we had to take a walk to the procedure room and use the Papoose Board of Doom. Since he has long-term tubes in his ears, we can't use DeBrox or any of the other over-the-counter wax  removal products. We're stuck with the ENT's metal earwax removal device, which Nolan loathes. Actually, loathes might be too kind of a word. He completely flipped out once we went into the procedure room - he remembers this room and hates it with an unparalleled passion. I can't say as I blame the kid.

A Reward for a Difficult Day at the Doctor's Office

After the wax removal, Nolan's ear hurt too much to put the hearing aid back in place. He was rather unhappy with all of us, so I tried to cheer him up with a trip to McDonald's. We don't go very often, but I looked up a McDonald's with a great indoor playground, which happens to be right next to the ENT's office. They had eating booths that looked like trains and a massive indoor playground. Nolan was happy by the time we left (thank goodness).

We will have the CT scan performed (outpatient, no sedation required) and return in early November to discuss the results with the ENT.

At least October is nearly doctor free!

Mr. Hungry

Whatever caused Nolan's problems in August seems to be gone, and I am SO very happy that his tummy seems to be working again. He has been absolutely ravenous lately, which is so wonderful I could literally do a happy dance.

The sleep neurologist said that one of the ways we could tell if the C-Pap was working was by Nolan's growth. He should experience a big growth spurt over the next few months. Judging by his appetite this week, I'd say we may be seeing one sooner rather than later. With the full-face mask, Nolan is actually starting to get some benefit from the C-Pap machine. We can still hear stridor (that comes with the laryngomalacia territory), but I think he is actually getting some quality sleep for the first time in his young life. Last night, he kept the mask on until 4:00am. This means he got 8 complete hours of sleep. For the first time, ever.

Yesterday, he ate breakfast, lunch, dinner, a string cheese, a pear, and 8 cookies.*

Grow, Nolan, grow!

In other news, still no word on the microarray results. This doesn't really mean anything one way or another - some cells just take longer to grow to obtain results. I did leave a message with genetics today to see if they'll call me back with some news (any news).

Nolan has an ENT appointment on Monday, and we are going to put off discussion of a fundoplication indefinitely. Despite our initial reservations and the horrible month of August, Nolan seems to be doing just fine on C-Pap and I can't see any reason to have a permanent, irreversible surgery performed on him when less invasive methods are working.

Here's to a happy, hungry kid!

*He wasn't exactly given permission to eat eight cookies. They were within reach and Nolan figured it was better to ask for forgiveness than ask for permission. I'm still in shock that he could down 8 of them!

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...

Another Trip to Buffalo

Nolan, panning for "gold"

Nolan's post-sleep study ENT appointment is today, and I have been on pins and needles regarding the outcome of the study. I really, really wish the pediatrician had released the report to me, so that we could review the results prior to the appointment. On the one hand, if he has passed the sleep study, then we could have been relieved of stress over a week ago. If he has failed it, then we could prepare appropriate questions prior to considering our options. Doctors have a lovely tendency to spring the results on parents, then shove surgical papers at them before the information has been fully digested.

Anyhow, Dennis and I are both going to this appointment, just in case he did fail the test. If he has apnea, we will have to make a decision about doing a revision supraglottoplasty. In addition, if his laryngomalacia has returned, then we know his reflux isn't under control with the medication.

I'm trying not to think about it too much, since we simply don't know the results yet - if he passed the sleep study, then all is well and we can relax. In fact, if he passes this one, I think I will throw a party. Everyone is invited!

Matt is still on the DDAVP, without any success at the 0.2mg/day dosage. We increase the dosage to two pills/night (0.4mg/day) this week. I am hopeful that we will see some improvement, so that we can avoid a trip to the pediatric urologist. Buffalo is a great city, but I'd like to go there for a reason other than doctor appointments, thank you very much.

We did get some nice, sunny weather this weekend and the boys were invited to a birthday party in Dry Gulch - a pretend mining town in our area. They had a blast, digging up gems and seashells. Matt won an agate slice during a game, which made his day. I think we have two budding geologists on our hands!

ENT Appointment (Or, Finally: A Routine Appointment)

I am so thankful our trips to Buffalo have decreased this winter: spending less time in the car and more time doing our regular routine has been absolutely wonderful. We did have to pop in to the ENT for a quick (routine) check yesterday. For the first time ever, it was an entirely painless and procedure-free visit.

Despite having influenza recently, Nolan's nose and ears looked pretty good. No excessive wax, no infection, no congestion. Did I ever mention how much I love those "permanent" T-tubes? I love them. Love, love, love them. This is the longest he's ever gone without an ear infection, and these tubes are much less likely to fall out.

His breathing sounded pretty good to the doc, so there was no need to scope his upper airways to check on the laryngomalacia. We'll see what his sleep study on May 3 shows: if apnea is still present, we'll probably have to do another scope to see if the reflux is causing an inflammation of the tissue around his voice box. Until then, however, we're free to go about our daily business with no worries.

I'm not all that optimistic about the sleep study, primarily because I have heard some stridor and Nolan still doesn't sleep through the night. Not even close: he wakes multiple times to climb back into our bed. It is possible that is a habitual behavior, though, so we'll see what the sleep study shows.

She did increase his Nexium dosage to 20mg, twice per day. His reflux is barely controlled on the 10mg (2x per day) and Zantac, so we want to head off any increasing reflux before the sleep study. She faxed in the scripts for the medication, and we were free to leave. Nolan was over the moon that there were no "tubies" involved. So was I.

The one interesting thing I noticed while going through his medical binder was that his vision screening packet actually does include the data regarding his vision results. It is interesting for several reasons (nearsighted in one eye, farsighted in the other eye), but I'll write about that in a different post. His ophthalmologist appointment is only one week from Friday, so we'll find out if the screening machine was accurate.

GI vs. ENT: We Have a Winner!

I waited patiently throughout the day Tuesday, knowing that the turf war between the GI and ENT doctors was occurring. While I wasn't there to observe it, I assume the interaction looked something like this:

Before I could call the GI's office to find out which doctor was victorious in the epic Nolan Reflux Care battle, my phone rang. It was the ENT's office, proclaiming victory.

When we visit the ENT on November 15, we will discuss his laryngomalacia and the benefits/risks of the supraglottoplasty surgery. Then the ENT will place a pH probe in the office.

This will be quite a bit different from the last experience. Last time, Nolan was sedated for an endoscopy while they placed the pH probe, so he doesn't remember the traumatic experience. This time, he will be fully awake when they place the probe. It will be done in the doctor's office, and then we will take him home (as opposed to being in the hospital). This will be difficult, since three year old children are liable to pull the pH probe out- thus ruining the study. The ENT's office assured me they would set us up with arm restraints before we go home. Oh, goody.

On the plus side, Nolan won't have to undergo sedation (dangerous for children with central apnea). The ENT will be taking over most of his care, which will consolidate the number of doctors we see. There is the hope, however faint, that the ENT will come up with a comprehensive diagnosis for the little guy.

Unfortunately, November 15 is going to be a Very Sucky Day. Traumatic for Nolan, and stressful for us as we monitor him to keep him from pulling out the pH probe. I will drive back up to the ENT on November 16th to have the pH probe removed. We will receive the data a couple of weeks after that, and will know if his reflux is controlled enough to proceed with the supraglottoplasty.

Keep us in your thoughts- I'm not looking forward to this one!

Have Your People Call My People

We had a great Halloween this year- perhaps a little too good. We have been so busy with trick-or-treating events that my kitchen floor has been a wee bit neglected. Still, October was a great month and we are looking forward to a more sedate November.

Spiderman had to go to the GI doctor yesterday, because his Nexium prescription is running out. Our visits to the GI doc generally involve brief discussions of Nolan's progress, weighing him on a scale, and a lollipop.

When the ENT did Nolan's bronchoscopy, we discussed his Nexium routine. Our morning routine was absolutely fine, but I found out that Nolan had been receiving his nighttime medication incorrectly. We were never really instructed on how to give the evening dose, so we were giving it to him just before bed. As it turns out, he should receive it 30 minutes prior to his evening meal.

I am WAY too scatterbrained to remember to give the child his medication at 4:30pm, so we set up a little alarm clock to go off at the appropriate time. It has been working like a charm, and we're readjusting to the new medication regime.


We know that the Nexium is doing a LOT of good for Nolan. He's a little older than 36 months, but I used the birth-36 month growth chart to show how much he's gained since starting the medication. It has literally been a Godsend for him.


His height is starting to drop a little (going from the 50th% to the 25th%), but this is likely due to the fact that he was a low weight for so long. His body has a lot of "catch up" growth to do.

The appointment was going swimmingly until I mentioned that our ENT wants to do a pH probe on Nolan to rule out active reflux before embarking on the supraglottoplasty for the laryngomalacia.

The GI doctor was a wee bit ruffled by this and didn't understand why the ENT would do the pH/impedance probe. Apparently, I entered a turf war I didn't know existed. The GI doc stated that if the ENT is going to handle the reflux, then she would discharge Nolan from her care and hand him over to the ENT for follow-up. She wouldn't write a prescription refill for the Nexium, because if the ENT is going to handle the reflux care, then the ENT should write the prescription for the medication refill.

The net result of the appointment is that I drove for two hours with two small boys, chatted about Nolan's wonderful progress on Nexium, then walked out of the appointment with a dozen phone calls to make. And without a Nexium prescription. Luckily I have enough to cover the rest of this month, and we see the ENT on November 15. To be honest, I would prefer for his ENT to handle his care entirely, because she is aware of how difficult Nolan is to diagnose and has been a champion for him from Day One.

The GI doctor is going to call the ENT doctor today, and then I am supposed to call the ENT and the GI docs on Wednesday to figure out the solution to the epic "GI vs. ENT Turf War." Hopefully we'll know who is taking charge of his reflux care by Wednesday.

ENT Visit

Wiped out from running

I am not quite sure how to fit the events of yesterday's ENT appointment into one post (it should really be three separate posts), but I am going to try.

The Never Ending Ear Infection

The "gunk" in Nolan's ear was not a sign of active infection. The ENT took a look and noted the material was definitely occluding the ear canal, but the eardrum and ear canal skin looked great. There did appear to be a large perforation behind the tube. She took him back to the Big Ear Sucker of Doom and removed the gunk. She also looked at the eardrum under the microscope, and noted the tympanostomy tube was actually lying on top of the eardrum. She removed it, and the "perforation" disappeared- it was just an optical illusion cast by the shadow of the displaced tube.

I was so greatly relieved by the news of the finally-resolved infection, that I didn't notice her grabbing the pneumatic otoscope. She made a "hmmph" sound and I looked up. She looked at me and said, "Fluid."

Rats.

So the never-ending-ear-infection is gone, but a new one is starting. The left tube is starting to extrude from the other ear as well. These were intermediate-length tubes, so they should have lasted for at least a year. They lasted for about 4 months.

Surgery is scheduled for October 12 to place a fourth set of PE tubes. This time, they'll be "long-term" tubes- I suspect they will be placing "T-tubes" into his ears on this occasion. The negative of T-tubes is that they must be surgically removed. The benefit is that they will not fall out in another 3 months, so we should be able to go for a longer period of time between surgeries.

The Random Hearing Test Results

Obviously, we need to figure out what Nolan's hearing levels truly are, and why his hearing levels fluctuate so much. We also need to determine the exact type of hearing loss for each frequency. Since we have three different bone conduction results from two different sites, we are going to have a full hearing test run at Buffalo Children's (WCHOB) sometime after his fourth set of tubes is placed. This test will be used as a "referee" for all of the other tests. Unless, of course, we get a fourth result. Anyone want to take some bets?

The Whole Apnea Thing

We're quite thrilled that the central apnea has all but disappeared. Unfortunately, the obstructive apnea has only gotten worse with time, and the ENT is not pleased. The neurologist labeled the obstructive apnea as "moderate," but the ENT heartily disagrees. It is quite severe, but she believes the neurologist downgraded her reading because she doesn't want to put a C-Pap mask on a child with a recessed mid-face (the pressure of the mask would only cause more mid-face retraction as his skull grew).

While the neurologist wanted to try a few steroids to see if they would help with the apnea, the ENT was appalled at this idea. Those drugs are only used for very mild obstructive apnea- and they're only used for apnea caused by congestion. Nolan's apneas are completely unrelated to congestion (for that matter, so are his chronic ear infections).

To determine the best course of action, the ENT wants to find out why Nolan has such severe obstructive apnea. Since he's going to be sedated for the tubes on October 12 anyway, she is going to perform a flexible bronchoscopy while he's out. A scope will be passed through his airway and into his lungs to find the level of obstruction. We should find out the results on the day of the procedure, so we will have more guidance on how to treat the remaining obstructive apnea.

We are going to have a busy October, but at least we'll have a bit more information on Nolan's hearing and breathing issues!

Of Dinosaur Birthday Parties and the Hunt for Lotrimin

The Physician's Assistant told me I might get some odd looks from the pharmacy, since he was ordering a liquid suspension of Lotrimin that is typically used for Athlete's Foot for Nolan's ear. I went to the pharmacy on Friday afternoon, and the pharmacy technician said the order hadn't been filled yet. Sidenote: killing fifteen minutes in a Rite Aid with two small boys should earn any mother an award.

I returned to the counter and the tech had a big smile on her face. "Oh, no! this isn't a prescription product- it's over the counter!" Even better- I could get the stuff at low cost, and it didn't even need a prescription. I wondered why the PA had warned me about the difficulty of getting the Lotrimin suspension.

Then the tech handed me some Monistat.

"Erm... I know the main ingredient is the same, but we need this in a liquid suspension. It has to go in his ear."

I picked up the box and pointed to the applicator and cream mixture, so that she could see this was a tad different than what our ENT office had ordered. The tech looked flustered and said she needed to confer with the pharmacist.

Another fifteen minutes later, the boys had taken their blood pressure at the free stand about 50 times and the pharmacist came out from behind the counter to troll the aisles of Rite Aid with me. Apparently, the only over-the-counter formulations of Lotrimin are creams. After I convinced him that the creams will not penetrate Nolan's PE tubes to get to the source of the fungal infection in his middle ear, the pharmacist left to consult his Big Book of Medications.

He found the suspension the PA had ordered, and they have it in stock at their warehouse. This is the good news. The bad news? The suspension won't be in the store until Monday afternoon. Sigh...

Fortunately, the infection doesn't seem to be bothering Nolan very much. We had his dinosaur birthday party on Saturday, complete with Funfetti cupcakes, dinosaur pinata, and dinosaur adoption events. Everyone had a blast, and Nolan is finally convinced that he is no longer two years old. Happy third birthday, Nolan!

The dinosaur adoption center

Opening presents

Nolan vs. Tyrannosaurus

Recalcitrant Infection

When an infection lingers for six weeks, is resistant to amoxicillin and Ciprodex, and begins to threaten a child's residual hearing, something has to be done. The ENT's office stated we couldn't be seen until September 13, and I was reluctant to go back to our pediatric "random doctor of the day" clinic.

Instead, I called the ENT's office and plead our case: a child with pre-existing hearing loss, persistent infection, and antibiotics that aren't doing any good. While our ENT is out of town for an extended period of time, they could get me in today to visit with a physician's assistant (PA). I leaped at the opportunity. Could we be there by one o'clock? Sure!

I looked at my watch: 10:50am. We have to leave by 11:00 to make it by 1:00- I threw the kids in the car, added a few Capri Suns and a diaper bag, and headed out. Of course, this sudden journey meant that lunch was catch-as-catch-can. I'm pretty sure I won't be winning "Mother of the Year" for their lunch today (some breakfast bars, 'Nilla Wafers, and string cheese). Oh, well. We'll make up for it with an especially nutritious dinner.

The PA took a look at Nolan's ear, and decided to culture the nastiness within. Nolan was quite good and sat patiently while the PA looked for "Spiderman." Thank you, John Tracy: he never cringes from the otoscope because of the hope of a lurking Spiderman. That little trick has served us well over the past few weeks! The culture was quick and painless, and we should get the results from that test in a few days. It will be interesting to see if Nolan is infected with an antibiotic-resistant strain of bacteria.

In the meantime, the PA decided to suction out the gunk from Nolan's ear. The ear vacuum is Nolan's least favorite invention on the face of the planet Earth. We tried to do it without papoosing him, but we resorted to the full body restraint and a nurse assist. He proved to be tiny but mighty- but we got the job done. The little guy hates the Big Scary Ear Sucker, and I don't blame him. It's loud, it is poking into an infected and tender area, and he has to be held down against his will. It is also (unfortunately) necessary and the antibiotic ear drops will be able to reach his middle ear now.

We are to continue the Ciprodex, in addition to a new antibiotic ear drop (Tobramycin). Aminoglycosides always make me nervous because of the whole ototoxicity thing, but the persistent infection isn't great for his hearing, either. Technically, Tobramycin is an eye drop, but the germs in his ear don't know that (it hits gram-negative bacteria that are resistant to Ciprodex). Nolan hates ear drops, and now he gets two different kinds every day. If the infection doesn't clear up after this round of medication, we have to come back.

We still have our appointment on the 13th to discuss the whole shifting hearing levels thing, so any remaining infection can be dealt with by our trusty ENT. I really, really hope the infection is gone by then!

Bone Conduction Hearing Test

Nolan, with balloon-cat hat and kitty face.

For a quick recap, we have a hearing test that shows a rising slope audiogram via bone conduction (January 2010 at Buffalo-BHSC) and a hearing test that shows a traditional slope via bone conduction (July 2010 at John Tracy).

Since these two test results are the exact opposite of each other, we wanted to get a third test to clear up whatever conductive component might exist to Nolan's loss. The test yesterday showed (wait for it)... a third configuration! BHSC managed to get bone conduction with masking, which means they managed to get the bone conduction scores in each ear, individually.

While I can't remember the exact numbers, he is showing something that looks like a shallow cookie-bite configuration in both ears. His bone conduction score was 25/30dBdB at 500Hz and dropped to 50dB at 1000Hz, then came back up to 40dB by 4000Hz. He was extremely reliable, so now the question arises: does he have fluctuating bone conduction levels in addition to fluctuating sensorineural levels?

Our test in January showed a conductive component, but only in the high frequencies. The test in July showed a large conductive component from 250Hz-1000Hz, with normal cochlear function in that range. The test yesterday showed a conductive/mixed component at 500Hz, but sensorineural across the rest of the testing range.

*Insert hysterical laughter*

The only thing we can be sure of is that his loss is fluctuating across all frequencies. Our audiologist is suspicious that something was missed on his CT scan- we need to take another look at his vestibular aqueducts. This is the only thing that causes an air-bone gap in the low frequencies with fluctuating hearing thresholds (the fluctuations occur when there is no fluid or infection present, so these changes in hearing level have nothing to do with his ear infections- we omitted those results). The next step is to see the ENT, to review the data and determine what to do next. This might include a FOURTH hearing test at an independent location, where we're likely to get a fourth result.

We did not do air conduction thresholds yesterday, except for a quick Speech Reception Threshold (SRT). His SRT is 55dB in one ear and 65dB in the other (my guess is that his infected ear is the one with the worse air conduction threshold).

Speaking of the infected ear, it looks pretty bad. I have to call the ENT this morning to beg and plead to get in. The amoxicillin did nothing for it, he's had it since the middle of July, and is now at risk for complications from a long term ear infection (namely, mastoiditis: something we'd really like to avoid).

Once we get the infection cleared up and talk to the ENT about Nolan's fluctuating hearing loss and random air-bone gaps that seem to shift frequencies, we'll go back to Buffalo for the air conduction scores (these are what he really hears and are in the moderately severe range).

It's really hard to set hearing aids for constantly shifting targets- our only solution may be more frequent audiological testing.

Still Infected

Alas, the "pink medicine" is completely gone, and Nolan's right ear is still sticky. I took a peek in there with an otoscope, and it doesn't look good. Thankfully, I have oodles of Ciprodex. I am giving Nolan the Ciprodex twice per day in the hopes that it will stave off some of the infection! The pediatrician will be called to (hopefully) get in tomorrow for a different oral antibiotic. The amoxicillin didn't cut it this time! We can't get in to the ENT until September 13, so we're doing the best we can until that date rolls around on the calendar.

Nolan has his bone conduction hearing test* today, to try and replicate the results we obtained at the John Tracy Clinic. If we get the same results, then we know that Nolan's low and mid-frequency loss is largely conductive in nature. I have a feeling our September 13 appointment with the ENT will be a rather long one.

*For those who are worried about the ear infection affecting the results of the hearing test: we are running a bone conduction hearing test, which bypasses the middle ear and any possible infection or middle ear dysfunction. The infection won't have any effect on the hearing test results. It will have an effect on his air conduction scores in the right ear, of course!

Buffalo: Round 1 (ENT)

We headed up to the ENT this afternoon to determine if there was any fluid lurking behind the wax in Nolan's ears. With a flat tympanogram in the right ear, we thought some hidden fluid might be the culprit behind the latest loss of hearing.

The ENT looked in his ears, weighed him, and then took him back to clean out the wax. This is a very miserable process, involving a papoose board, a microscope, metal instruments, and some bleeding from Nolan's ears. I won't go into detail, but let's just say that it ranks pretty high on the "least favorite thing to do" list. His left PE tube had just fallen out of the eardrum, so she removed that, too.

His ears are clear, and there is no fluid. We're free to go to BHSC for the repeat hearing test on the 15th, confident that there is no cause for a conductive problem remaining in his ears. They did run a tympanogram, which showed a perforation in the left eardrum (from the recently removed PE tube- it is too small to see and should heal completely by the time of the hearing test). The right ear does show negative pressure, but they just "wait and watch" with negative pressure. Sometimes it means a fluid build-up is imminent, sometimes it just disappears on its own.

Of course, no ENT visit is complete without finding some new thing to be concerned about. Our ENT is concerned that Nolan's anti-reflux medication isn't quite handling his reflux. He's eating less, pulling at his neck more, screaming a lot. Also, he never sleeps (reflux parents, you know what I'm talking about). He's also snoring again and his tonsils are big.

The big tonsils and reflux/snoring issues worry our ENT a bit, because all of this can affect growth. We have managed to get his weight back to 24 pounds, 3 ounces with a lot of DuoCal, but we're not seeing an improvement of symptoms. Our ENT has ordered a sleep study to be done on Nolan, and if obstructive sleep apnea is found, a tonsillectomy and adenoidectomy will be recommended. I get to set up the "polysomnogram*" at my convenience, and we'll see the ENT again on March 1 for a follow-up. If his tonsils have decreased in size and his sleep study looks good, we can ditch the whole tonsillectomy idea. I really want to ditch the whole tonsillectomy idea.

She also sent a note to our GI doctor requesting a possible increase in anti-reflux medication. We see our GI doctor tomorrow, so this may happen fairly quickly. Now I'm preparing for Buffalo: Round 2 (GI). Time to charge that portable DVD player!

*I learned a new word today.

Fun With Doctors

We saw the GI doc today, which will necessitate a long update. I'm not quite in the mood for that, so I thought I'd share something I found on our ENT's website. They have a "kids corner," so I thought I'd check it out. How many words can YOU make out of "otolaryngologist?" There is also a coloring sheet. And a maze, which I hope is not part of a novice ENT's instruction manual:




I wonder if the GI doctor has a similar site. "Help the food find it's way through the intestines!" I'm not sure if these activity pages are weird or hilarious. I'm voting for funny.

ENT Visit

We headed up to Buffalo for a routine ENT check-up. Kids with hearing loss see an ENT on a regular basis to make sure the middle ear is as healthy as it can be, since fluid or ear infections can make a sensorineural hearing loss worse.

The left ear has a patent tube and looks great- no fluid, no wax, no infection.She took a look in Nolan's ear and confirmed the right PE tube was out, and that there was a lot of wax in that ear. She did a tympanogram on the right ear and the eardrum is a little stiff. After further investigation, the stiffness is not caused by fluid. I'm guessing the bad infection/perforation and two sets of PE tubes may have caused some scar tissue- hopefully the "stiffness" isn't enough to affect the hearing in that ear. Still, I'm thrilled because no fluid means no third tube and no adenoidectomy.

I did mention the possible PUV's and scheduled cystoscopy to the doctor and nurse, and that I was still concerned about his lack of appreciable weight gain. She entered the information into her computer and did the usual "mm-hmm" response.

Then she went to clean out his right ear (this involves a papoose board and all sorts of unpleasant metal funnels in the ear). After doing this, she checked his records again and saw that the ENT office hasn't taken an official weight since October 2007. She told the nurse to get a weight on him, then set us up for a 4 month appointment.

The nurse took his weight, furrowed her brows, then sent us back to the exam room. The ENT came back in, took one look at his growth chart, then proceeded to freak out. In a very professional, medical manner, of course. Apparently, seeing a drop from the 60th% to the 3rd% line makes more of an impact than the mom telling the doctor that he's only 21 pounds at 21 months of age.

A yummy cookie. If only he'd actually eat the whole thing!

After asking us if the pediatrician had started up a fail-to-thrive evaluation (um, nope!) and having me fill out another symptomology sheet, she set us up for a feeding evaluation at children's and a scintiscan to check for gastric reflux. Apparently, pulling food out of your mouth, refusing food after the first two bites, spitting up occasionally at the age of 21 months, and being very texture sensitive are indicative of a feeding disorder.

They're sort of treating it as an emergency and have us in for the feeding evaluation on Tuesday. There is a part of me that finds this rather ironic- he's been technically in the "failure to thrive" camp for EIGHT months. I have mentioned the weight issues like, um, Every. Single. Time.

The ENT is a tad concerned that this bizarre constellation of symptoms might be indicative of a syndrome. We've been there, done that before. It's more likely just a bizarre constellation of symptoms. .

For now, we have a bazillion appointments at Children's for the reflux evaluation, feeding evaluation, and cystoscopy. Hopefully it is just reflux and a little Zantac will help him put on the weight.