Taking a Break

Friday was such a miserable day. Nolan wouldn't eat anything, though he seemed to be fine in every other way. His nose is congested, but it is always congested with the combination of reflux and the C-Pap machine. Friday, Nolan spent the entire second half of the day like this:


We decided to stop C-Pap therapy for the weekend to let him recover to his "normal" level of activity and eating. He ate a waffle on Saturday morning. Two bites of a grilled cheese sandwich at lunch. 3 bites of macaroni and cheese at dinner. We took him out for ice cream, hoping that would entice him to eat: no dice. He ate about 3 bites of ice cream before his stomach was "full." He did eat some kiwi fruit when we got home - he always prefers wet foods when the reflux/DGE gets really bad.

Apparently, his digestive system has decided to go on vacation.

He seems to be eating a bit more this morning. He has had dry cereal and wants some bread. Mornings are his best time for eating when he gets like this (his tummy has all night to empty), so we're trying to load him up with high calorie foods.

We haven't seen the delayed emptying really cause a problem since he was about 2 years old. For some reason, it seems to have made a return this week. Nolan seems a bit more perky this morning, so hopefully that is an indicator that things are speeding up in tummy-land.

We'll start C-Pap again tonight, to see if the C-Pap is causing things to get worse. If Nolan takes a plunge into the vomiting/congested world of not being able to eat, we'll have to take a longer hiatus from C-Pap therapy. I don't really see how the C-Pap could increase Nolan's motility issues, though - I really do think the flare of his issues is just coincidental with the start of C-Pap.

Now I'm off to go dump some DuoCal into a jar of yogurt. Hopefully he'll eat it!

Is This Week Over Yet?

Nolan has been having terrible flares in his reflux lately - last Friday he gagged and choked for two solid hours, and his eating really decreased. This week, we've had a lot of tummy pain and reduced appetite. Today, Nolan hasn't eaten anything.

Which is why I was surprised when he "baptized" the car with yesterday's lunch.

The car seat cover is now in the washing machine, Nolan has had a bath, and the car has been cleaned of the nastiness that was yesterday's lunch.

I'm so tired I can't even think straight. Nolan is fine, except for the whole not-eating tummy-hurting business.

This week can be over at any time.

There are a couple of bright spots.

1. I found a better mask for Nolan made by Philips Respironics: it is made for children in the 15 pound-40 pound range. Nolan is exactly 30 pounds, so he should fit into the mask. It also has a forehead strap to help keep the mask secured. It is also only $99, so I bought one. I emailed the respiratory therapist (who has no idea what to do with a child as young as Nolan), so she bought one, too. Hers will be here faster, so we'll use it until ours arrives. Then we'll have her get a different mask for us to try out. That way we'll have two masks to rotate.
2. Nolan's ear isn't leaking anymore. At least that is one thing we don't have to worry about this week!
   

Nolan is accepting the mask at bedtime, but he won't keep it on. There isn't much we can do about that, but wait patiently to see if he'll accept it over time. Arm restraints are another option, and we may have to resort to that if nothing else works - the "restraints" are also known as "no-no's," and are really just braces that keep kids from bending their arms at the elbows. This would keep him from forcibly removing his mask. I really don't want to have to do that, however. For one thing, he needs to be able to remove it if he is congested. Also, he vomits occasionally. So we can't have him throwing up into a C-Pap mask.

I am so, so glad we have our trip to the Cleveland Clinic scheduled for September 6. We really need some objective guidance on what to do with the little guy!

Exhausted

We started CPap therapy on Monday night. Nolan whimpered a few times while putting the mask and hose on, but was surprisingly accepting of the whole situation. We read our bedtime stories, then he continued to look at some books until he fell asleep. He was conked out by 9:00pm, and I was relieved that it went so easily.

He woke up at 11:00pm, crying with the blowing air shooting into his eyes (he had dislodged the mask). He was fussy about the whole set-up by that point, and we figured 2 hours for the first night was good enough. We took off the CPap and let him sleep.

Then came Tuesday.

Terrible, terrible Tuesday.

Horrific Tuesday.

A too-big mask hangs over Nolan's upper lip.

He wasn't in a great mood on Tuesday. Maybe because he had slept poorly the night before. Maybe because his stomach has been acting up more than usual. Maybe because he's three-almost-four, and he just felt like being cranky.

Anyhow, he was not going along with the CPap plan on Tuesday night. We put the mask on at 8:00pm. He screamed. He hollered. He cried.

We tried loosening the mask when he cried that it was hurting him.

We tried adjusting the straps when he cried that it was "too tight."

We tried tightening the mask when air leaked out of the top of the mask.

It didn't matter, he just continued to cry. This was a night that I was so, so grateful to have a caring spouse. We took shifts in the boys' room, trying to get Nolan to calm down and trying to get the perfect fit on the mask.

When Dennis was upstairs and I was downstairs, Nolan would scream, "Mommy, HELP ME! HELP ME! Mommy, come here!"

It was awful. I was sobbing.

Is CPap worth it? Will it even work? Will he even accept it? Is this causing more trauma and torture than a surgical fix for his apnea and reflux? Would the "surgical fix" even fix the problem? What are we going to do?

I pulled myself together and traded shifts with Dennis. I put on my Calm Tough Mom face and sat next to the hysterical Nolan.

"Nolan, crying isn't helping. I can't understand what you are saying. If you want Mommy to stay in your room, you have to stop crying. I will read you a book, but you have to stop crying."

I doubted that would work with a three-almost-four year old sent over the edge of sanity by a scary air blowing mask. Surprisingly, it worked.

Maybe because it was 11:00pm and he was really tired.
Maybe because he was tired of fighting.
Maybe because he is three-almost-four and trusts his Mommy.

His hysterics calmed and he fell asleep. He stayed asleep until 2:00am. When he woke up crying, we took the mask off so he could sleep through the rest of the night.

Last night was Wednesday. We put the mask on and there were no hysterics. We read Ferdinand the Bull and the Research Guide on Pirates (to the Magic Tree House series). Nolan fell asleep as I was finishing the biography of Captain Kidd.

The lack of tears and drama/trauma were a welcome relief. Unfortunately, he kept knocking off the mask - every 15 minutes or so, the mask would be hanging loose from his face. There is no alarm when he knocks it off, so I stayed up until midnight to continually replace it, then went to bed. He woke at 1:30am with the mask dangling around his neck, crying.

We got about 2 hours of CPap therapy last night.

The respiratory therapist said the company that makes the toddler-sized masks has discontinued the "small" mask. Nolan currently has a medium sized "mini me" mask and headgear (this fits most preschoolers). Fortunately, the head gear does come in a smaller size. We don't have many options with regard to the mask: we could go to a full-face mask, but the danger of Nolan throwing up in the night into a full-faced mask is very real. We wouldn't want him to suffocate if he got ill in the night: if we try a full face mask, we will need an apnea monitor and alarm. Another option is to try arm restraints. I'm not a fan of that last option.

We have a month to try different configurations, but there aren't a lot of different options available for a child as small as Nolan. The popular "kidsta" masks found online are for kids over the age of 7: if Nolan can't fit into the medium "toddler" mask, there is no way the masks for kids "over the age of 7" will fit.

Anyhow, tonight is Thursday. We will soldier on and try the therapy again tonight. Hopefully, there will be no tears. Hopefully, he will leave the mask on in his sleep.

Hopefully, this will work.

The Cleveland Clinic: A Cut Above

I was a little worried when I noticed that only about a quarter of Nolan's medical records had been faxed to the Cleveland Clinic. Knowing that we only get one shot at this, I was concerned that the partial records would make for an useless appointment.

I sent in medical records request letters to our ENT, Pediatric Ophthalmologist, and GI doctor, so that some of the missing records would find their way to the Clinic.

Yesterday, the phone rang. It was the genetic counselor at Cleveland Clinic - she noticed that some of the records were missing from the pediatrician's office.

"There is a record mentioning an echocardiogram, but there is no echo. There is a record mentioning an MRI scan, but there is no MRI scan."

"Are there any endoscopy biopsy results? Labwork? Ophthalmology reports?"

"No. There is no mention at all of his vision problems."

"OK, I'll get on that."

There were 54 pages sent, probably about 1/4 of Nolan's medical records. I'm working on getting the rest sent directly from the specialists. The records sent to the Clinic were missing his genetic testing work from 2008 (just Connexin 26, Connexin 30, and mito A1555G, which were all negative for mutations). The records were missing all mention of vision issues, reflux test results, biopsy results, labwork, etc.

The rest of the records should slowly start filtering in to the Clinic this week. I will fax the rest of the missing records (early ABR hearing tests, genetic test results, etc) tomorrow.

I am SO impressed that the Clinic noticed the missing records and called me about them. I honestly don't think anyone has ever sat down and reviewed all of Nolan's medical records before.

We may never have a comprehensive diagnosis, but I am impressed with the attention to detail and the professionalism at the Cleveland Clinic. I feel like I can trust their judgment - they have really looked at his entire case and have seen kids like him before.

I really can't wait for our appointment next month. We may not get answers, but maybe we'll get get pointed in the right direction with our care of Nolan's most pressing medical concerns. Sometimes, getting pointed in the right direction is the most important thing.

C-Pap for Kids: Not Much Fun

Mr. Bear tries out the C-Pap

I took a little jaunt down to the Home Healthcare Company today - the respiratory therapist there set us up with Nolan's C-Pap machine.

She was rather flummoxed when she saw how tiny Nolan is: he is almost four, but he has that blasted flat nasal bridge and is on the small side for his age. There is a mask for toddlers, but the nasal mask is a bit too big. She is going to do some research online to see if she can find anything smaller (she can't - I've already looked). She did mention using a high-flow nasal cannula: this is what they do for premature infants, since they are too small for the typical C-Pap masks. We're not sure if this would work with an almost-four year old child's breathing method, though (infants breath entirely through their nose - a four year old is more likely to open his mouth and lose the pressure splinting open the airway).

Insurance will cover 50% of the machine. The rest will be paid by us, on a monthly basis. C-Pap machines are sold on a rent-to-own platform. Our first bill will be the highest, since that includes the disposable items like the hose, mask, filters, etc. Insurance will cover the rest. Maybe.

The "maybe" comes in the form of a little blue chip that sits inside the machine.

This chip is our police officer. It records exactly how long Nolan uses the machine each night. If he doesn't use it for a minimum of 4 hours each night, the insurance company will refuse to pay for the machine. Then we'll get stuck with the entire bill.

The monitoring chip was the brainchild of insurance companies, who got tired of adult patients requesting a C-Pap and then not using it because it is uncomfortable. Of course, children are a different scenario entirely. Due to other health concerns in young children, frequent congestion or colds may make wearing the C-Pap difficult at times. Let's face it: it is pretty rare for a 4 year old to require C-Pap, and most kids who do require therapy have complex medical issues.

So if Nolan gets sick on the C-Pap and can't wear it for a few nights, insurance won't cover any of the bill. Mr. Blue Chip says so.

He starts using the C-Pap tonight. We'll see how he does. He's been refluxing like crazy since Friday, and has a rattling cough. He may get too sick on C-Pap to make the entire enterprise feasible. In which case we can just stop the rent-to-own deal and we'll only be out $500.

Of course, if C-Pap doesn't work, we're left with a fundoplication and revision supraglottoplasty as our only options.

Despite the hassle and money involved, I really hope this works. We need our little one to grow!

Getting Things Ready for Cleveland Clinic

Our pediatrician has faxed off the necessary medical records to Cleveland Clinic - it is a great relief to have this done. I picked up my copy of the faxed documents, and realized several records were missing. They sent:

A letter from his GI noting adequate control of the reflux (this was sent a year ago).
A letter from his ENT showing poor control of his reflux, and showing the diagnosis of laryngomalacia.
One audiogram.
A report from Nolan's posterior urethral valve surgery.
One sleep study report.
His normal EEG findings from the staring spells a long time ago.

They are missing all the labwork (showing the slightly elevated AST levels), his endoscopy biopsy results (showing ectopic gastric mucosa and flattened villi), his MRI's and CT scans, all ophthamology reports, and all of his pH probe results. Not to mention the four sets of tubes from chronic middle ear infections, the history of being failure-to-thrive (especially since he is dropping on the charts again), and the surgical failures to control apnea (tonsillectomy and supraglottoplasty). And then there is the mixed hearing loss which is slowly progressing over time.

I made a few phone calls to specialists this morning: I called the GI to get his biopsy record, lab work results, and pH probe results faxed over. I called the ENT and the audiologist to have the same thing done. I still need to call the ophthalmologist.

I have several release forms to fill out and send back to the specialists - it takes quite a lot of work to get everything organized. I wouldn't be so worried about the whole thing, but our insurance company has approved only ONE visit with the geneticist, and won't cover any lab work or follow-up visits. Because of this, we need to have all the ducks in a row. We only get one shot at this - we need to make it count.

Hopefully all of the records will be into the Cleveland Clinic in short order.

In Which I am Very Stealthy

Matt and Nolan at Library Story Hour.

This blasted ear infection is driving me crazy. It isn't exactly clearing up, and the ear drops are such torture for Nolan. I discovered a way to get the ear drops into him without any upset, though. I wait until he's deeply asleep, then I sneak into his room, put the drops near his ear canal, tip his pillow, and then hold my breath for 2 minutes. He fusses for just a second, then goes right back to sleep.

I have excellent ninja skills.

This tactic does not work in the early morning, however. When I try giving him ear drops at 6:00am, I end up with a howling, angry boy. Apparently, my ninja skills do not work during daytime hours.

Anyhow, his ear is still leaking some fluid and looks kind of nasty through the otoscope. I'm not a doctor, but I'm pretty sure "oozing" means "still infected." I'll give it until Friday (one week), then I'm either calling the pediatrician back or calling the ENT's office. I don't want a repeat of last year's 8 week long epic ear infection.

Ugh.