Back to School and A New Pediatric Specialist

We have had a chaotic spring, with the boys' surgeries that took up our lives from late February through mid April. I am so grateful that everyone has recovered! Nolan went back to school today, and was happy to have his routine back.

I recently had a friend tell me about a pediatric specialist in our (relatively) local area who takes on difficult cases. My friend's children are also complex, and this specialist is keen on finding an accurate, global diagnosis for her kids. I am in the process of trying to get an appointment for Nolan, as he needs someone to review his entire case and look at the boy as a whole. Currently, his diagnoses are (organized by system):

ENT:

Chronic sinus infections
Chronic ear infections (5 sets of tympanostomy tubes)
Progressive, mixed hearing loss (currently moderately-severe to severe). Hearing aids.
Laryngomalacia
Obstructive and central sleep apnea - C-Pap user
Glossoptosis

GI:

Severe reflux/Nissen fundoplication with g-tube placement
Gastroparesis
Failure to thrive (supplemental feeds for ~ 800 calories/day)
Chronic diarrhea
Periodic vomiting attacks

Vision:

Astigmatism (both eyes)
Myopia (right eye)
Amblyopia (right eye) - treated with glasses

Urology:

Posterior urethral valves

Cardiology:

Innocent heart murmur in the mitral valve area (echo normal, sounds of regurgitation from mitral valve)

Other:

Hyperflexible joints
Mild hypotonia, primarily in the hands and arms
Fatigue with walking
Unexplained leg pain (severe & periodic)

In any case, I'd be glad to have his entire case reviewed, just to make sure someone hasn't missed something obvious along the way. In addition, I want to make sure that we're treating Nolan appropriately - I don't want to do any more surgeries until we're positive it is the right treatment for his system as a whole.

The pediatric specialist has two "sides" to her practice - a general pediatrics side and a rheumatology side. I am currently waiting to hear back from the office to see which "side" Nolan will be booked under - with the hyperflexibility and leg pain, they may book us under rheumatology rather than general pediatrics.

I just hope they can help us find some answers.

Doing Better!

New prescription sunglasses!

Nolan is almost completely recovered from his supraglottoplasty. He isn't on pain medication anymore, and we have stopped his steroids. I have to say, I am glad to be down to two medications again!

He is still choking on thin liquids, but that problem should resolve with time. He is able to eat most foods again, and we experienced one of the "good" side effects of steroids - they make people hungry. While Nolan still can't tolerate large volumes of food, he was eating constantly when on the steroid. I was very excited and thought that perhaps we could keep him on steroids to maintain the hunger - and then found out that steroids cause things like diabetes and liver issues and can't be continued at high doses for a long period of time. Still, it was great to see him hungry for the first time in...forever.

The little guy has been outside to play a bit, but does tire out easily. He rode his Green Machine for a few minutes yesterday, but couldn't pedal it on our driveway and started to scoot it around with his feet before heading inside for a rest. We also went to Matthew's first baseball practice last night, and Nolan did play on the playground for a few minutes.

It was nice to see an actual smile! He was completely wiped out by the time we got home, so he didn't eat dinner and had some serious bloating issues. He had trouble tolerating his feed last night, but seems fine again this morning. Even though he seems largely recovered, he doesn't quite have the stamina to do his normal activities.

Still, he will be ready to go back to school on Monday. We are all looking forward to the return of routine!

Revision Supraglottoplasty: Finally Home

Nolan's surgery went ahead as planned last Tuesday - this was a relief since the last one was cancelled due to strange "exudate" in his airways. Of course, the cancelled surgery still cost us more than $500 - just another bill to add to the growing pile!

In any case, we checked into Buffalo Children's at 6:00 am on April 9. Nolan was a bit wiped out, but was happy to play the wii in the pre-surgery waiting area.

In the middle of his game, he suddenly sat down and looked quite ill. He said he needed to throw up. We told the nurse and they brought us to a room with a gurney, and called his ENT.

Nolan's stomach is an up-and-down thing.. we've always wondered about hypoglycemia since he vomits more frequently on an empty stomach. They brought us down to the second floor pre-op area, and we waited in the chairs. As Nolan puked his guts up, the anesthesiologist visited with us. The nurse also came and took some blood for a blood glucose reading. His blood sugar was 60, which is at the lower limit of normal for a child. He doesn't have hypoglycemia, so the vomiting is due to some other factor.

In any case, the anesthesiologist saw Nolan's state and told me that I could come into the operating room to be with him while they put him to sleep. I have no idea why they didn't do this for the previous thirteen procedures, but boy howdy - it was so much easier. I walked him back into the OR and held his hand as he drifted off - there was no crying and no hysterics.

After 2 hours, we talked to the ENT for a post-op report. His epiglottis (flap that comes down when you swallow to protect your airway) was flopping over his voice box again, so they tacked that back up. This procedure (tacking the epiglottis to the base of the tongue) is called an epiglottopexy. She also lasered out the floppy tissue (called aryepiglottic folds) that flopped over his voice box. In addition to lasering, she sutured the area to (hopefully) prevent the tissue from collapsing again.

She also told us he has a condition called glossoptosis (gloss-op-toe-sis), which is when the jaw is too small to accommodate the tongue. His tongue falls to the back of his mouth and also obstructs his breathing. If this condition worsens over time, he may need a procedure to fix the problem. Hopefully he won't require help with this issue.

The PICU charge nurse came to retrieve us from the waiting room and brought us to Nolan's bedside. He was heavily sedated and on a ventilator.

Occasionally, he would wake and try to rip the tube from his throat. He was quite wakeful despite heavy sedation - he ended up requiring Fentanyl, Ativan, Versed, and Precedex to maintain a restful state. He did have a fever for the first two days in the PICU, despite the Tylenol they were giving him.

It was a long two days, though at least Nolan was resting comfortably by the second day. His PICU nurse was with him for three days and it was wonderful - his nurse was really amazing and took great care of our little boy. Finally, Thursday arrived and we were able to extubate him.

It took quite some time for the Versed and Remi-Fentanyl to wear off, and our first attempt to take the tube out failed (his O2 sats kept dropping so they left it in for a while). Finally, he woke up enough for the PICU staff to remove the tube. Parents who have gone through this know how heartbreaking it is. Nolan was awake and thrashing, terrified and not really understanding why he couldn't speak. Tears poured silently down his face... the doctors needed Nolan to respond to commands before they could pull the tube. Nolan couldn't do this. There were just silent tears and terror. Finally he mouthed the words, "TAKE THE TUBE OUT." His silent "scream" let the team know he was conscious enough to breathe on his own. They took the tube out.

Once the tube was out, he immediately went back to sleep. His oxygen saturation was great, and we would monitor him for another 24 hours in the PICU. If he could stay awake, eat, and drink, we would be discharged the following day.

Nolan slept all day and did not eat or drink anything. I did not want to stay over the weekend in the hospital. The medical staff wanted to be sure he could drink without choking before sending us home. Fortunately, we talked with the residents and Nolan's ENT - since Nolan has a g-tube, they would allow us to go home despite the lack of eating or drinking.

The following night was really rough. Nolan was very wakeful, but his voice hadn't come back. He would sit up and mouth words, trying to communicate. He couldn't swallow his own saliva, and needed to spit into a towel frequently. Since he was only there for monitoring, his PICU nurse was moved to another patient across the ward and I was the one "on duty" throughout the night.

Friday morning, Nolan woke and was in a great deal of pain - his legs were really bothering him. He gets a strange leg pain every once in a while - not exactly cramps, but he will yell at us to crush his toes, pull on his leg, and pull his feet off. I have no idea what causes this, but I was glad that other medical personnel saw this! Nolan felt very poorly, and we were discharged at 11:00 to go home. I was beyond tired, and grateful to get home.

The leg pain continued for the rest of that day, and was actually more difficult than anything else. He writhed on the floor yelling, "PULL MY TOES OFF!!!" There was really nothing I could do to help him - fortunately the episode passed after a few hours and he was fine later that evening.

Nolan can't swallow liquids well, so he coughs and splutters frequently with drinks. We are putting liquids through his tube. Fortunately, he can eat some foods. Cheese puffs, pancakes, and other dissolvable foods work well. He tried a bite of pizza, but ended up spitting it out. He can't do any "hard" foods like apples or chewy bread. He's still spitting out his saliva - but this should get better over the next few days.

His low-grade fever has made a bit of a comeback, but each day is better than the last. We have even seen a smile or two over the past day or so. I'm not sure when he can return to school. He is far too ill at the moment to do anything, and the choking problem has to be resolved before he can return safely to his classroom. It will probably be another couple of weeks before he's well enough to return to regular activities.

A small smile.

Ready for Surgery Tomorrow

Our second attempt at Nolan's revision supraglottoplasty is tomorrow. His ear infection seems to be under control (still on the Ciprodex and antibiotics), though his stomach has been acting up. Lots of fluke vomiting attacks and hurting tummy moments - he's had a day or two of "good eating," but is otherwise not doing well on that front. Hopefully his stomach will be in good spirits tomorrow morning.

We have the dog at the kennel, Matthew is with our friends for a couple of days, and we're heading to bed so we can be up at 3:30 am tomorrow. Hopefully his airway will be clear and the surgery will proceed without a hitch.

Sweat Test

Since Nolan has both digestive problems and respiratory issues (along with chronic sinus and ear infections), a sweat test was ordered to determine if he has cystic fibrosis. We went up to Buffalo Children's on Tuesday and checked into the lung center.

The technician cleaned his arms and placed the electrodes on his right arm. She added the Pilocarpine and turned on the electricity - the electricity generator looked like a giant car battery.

Nolan was very brave and sat still for the test. The technician said it feels like the pins and needles when your leg falls asleep - a very strong tingling sensation.

Nolan did say, "It hurts, Mommy," a few times, but he sat still for the test.

After five minutes of stimulation on his right arm, the area was wrapped and the same process was repeated for the left arm.

Once both arms were finished and wrapped, we went off to wait for about 30 minutes. Nolan's sweat was collecting into the filter paper as we waited in the small waiting room in the CF center.

After the thirty minutes had passed for each arm, the gauze was unwrapped and the filter papers were taken by the technician to be weighed and analyzed for the concentration of chloride in his sweat. We left and went to Chuck E. Cheese as a reward for being so very brave.

I called the pediatrician's office today to find out what the results to the test were. Sweat chlorides over 60 mmol/L are positive for cystic fibrosis, chloride levels between 40 - 60 mmol/L are equivocal (might have CF), and levels below 40 mmol/L are considered negative.

Nolan's sweat chloride level was 9 mmol/L - very NEGATIVE for cystic fibrosis.

I am glad we have ruled it out, as our treatment strategy would change if he had CF.

I am extremely relieved that his lungs are healthy and we won't ever need to worry about this again.

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.