Matt's Sleep Study: Post-Supraglottoplasty

I was happy to get a sleep study booked for Friday night - this meant that I would be able to sleep on Saturday during the day, since Dennis would be home to watch the boys. I drove Matthew up to Buffalo Children's and we were ushered into the waiting room just as the sun was starting to set.

The sleep study tech was not quite ready for us, so she sent us to the playroom. Matthew was happy to play with the toys. I, however, was not happy that someone had changed the channel on the waiting room television to play Bones. This television show is definitely not for children, and Matthew was rapt with attention as they showed a half-rotted corpse on the show. I finally found a power button on the side of the TV. Matt went back to playing with his pirates. I was not happy.

We were finally ushered into a room and Matt changed into his pajamas. Matt played on the Kindle Fire with the Bad Piggies game, using it to test out several rocket designs. Sleep studies are much easier at this age, as the kiddos can entertain themselves!

At 9:30, hook-up started. By 10:00, Matt was settled in bed and I was reading a chapter of Harry Potter and the Sorcerer's Stone. He wasn't happy about the nasal cannula, but left it alone. He's actually giggling in the picture below - I was worried it would take him a long time to fall asleep.

Fortunately, he fell asleep almost as soon as I turned the lights out. He stayed asleep until 2:30 am, when he woke briefly and said, "I think it is dusk outside," then immediately fell back to sleep. I managed to get about an hour of sleep, which is much better than getting no sleep at all. At least I would be somewhat coherent on my drive back to Jamestown.

The lights came on at 5:00 am, and we grabbed some Tim Horton's donuts (and a coffee for me). We were home by 7:30 am. I thought I would nap during the day, but I have a hard time sleeping during daytime hours. Matt and I both had early bedtimes that night, though!

I won't get the results from Matthew's sleep study until October 28, which is when we have our follow-up appointment with the ENT. Hopefully his sleep apnea is gone!

Nolan's Sleep Study Results

Nolan's sleep study was an exhausting, sleepless ordeal in late June. He woke frequently and cried. His legs bothered him a lot, and it was obvious from the sleep study report that he has difficulty with periodic leg movements and frequent arousals. He also has moments of bradycardia where his heart rate drops well below 40 beats per minute - this was observed in the PICU when he had his supraglottoplasty as well. He is fine during the day, so it is probably fine - but we will bring it up with his pediatric rheumatologist when we see her in September (along with the leg issues). Dysautonomia occurs with children who have Ehlers Danlos, so it is possible he isn't regulating his heart rate well. Better safe than sorry where the heart is concerned!

On the other hand, we received some fantastic, out-of-this-world news. His second supraglottoplasty/epiglottopexy actually worked. His apnea is now in the mild range for the pediatric age group. This means that we do not have to use C-Pap for the time being. He will have another sleep study next year, as he is high risk for the apnea to return with his connective tissue disorder. We are absolutely thrilled that we do not have to cart around a C-Pap machine and he is getting the oxygen he needs to grow.

So, goodbye C-Pap machine. We won't miss you.

Still Here - Playing Catch-Up and Matt's Supraglottoplasty

I have been absent from the bloggy world. I have a good excuse. We had four - count them, four - surgeries this past spring. Two for Matthew and two for Nolan.

First was Nolan's attempted supraglottoplasty in February, which couldn't be completed because he had funny, white exudate all throughout his airway. He had to be woken up from general anesthesia and we were sent home. The cultures were normal, so this mystery was never explained.

Second was Matt's tonsillectomy and adenoidectomy in March. An outpatient surgery with 8 long days of recovery.

Third was Nolan's supraglottoplasty and 3 day stay in the PICU in April. He was intubated on a ventilator for 2 days.

Fourth was Matthew's supraglottoplasty and overnight stay in the PICU for observation on June 25, 2013.

Honestly, I plead exhaustion. It was simply a ridiculously exhausting spring.

Matthew's surgery went very well. Nolan's genetic syndrome (Ehlers Danlos) wreaks havoc on his tiny body, and it is much more evident when comparing the boys' surgery recoveries. Nolan was in a lot of pain and had a lot of trouble swallowing and required ventilation assistance after surgery. Matthew cried for an hour after surgery, then demanded French toast and a cheeseburger.

Unfortunately, he was only allowed clear liquids for the rest of the day. The morning, however, brought French toast and an ecstatic Matt. "Mommy, this is the goodest breakfast ever!"

Matt's recovery was ridiculously easy. He didn't have to take any pain medication after the first day in the hospital. We went home 24 hours after the operation. 48 hours after his surgery, he was playing in his baseball team's championship game. They got second place.

Seriously. This is Matthew 48 hours after surgery. His recovery was insanely easy. It was nice to have things go so smoothly!

We have a sleep study on August 9, 2013 (this coming Friday) to determine if he still has sleep apnea. I certainly hope his apnea is gone!

Sleep Study Number Six

Nolan had his follow-up sleep study from his revision supraglottoplasty last night. I am absolutely exhausted, so this post may be a complete jumble of incoherent mumbling. Fair warning.

We arrived at Buffalo Children's for the hook-up at 8:00 pm. The children's hospital is much better than the alternate sites because the tech is familiar with children and all of the equipment is properly sized. They also perform CO2 monitoring at the children's hospital and do not perform this test at the alternate sites.

Nolan was a champ with the hook-up. He's so used to the process that he didn't blink an eye.

The only part of the process that he hates is the nasal cannula. He couldn't remember what it was called and kept telling us that he "hated that mustache!" He turned the volume up to maximum on the TV speaker and watched some Sesame Street.

The same episode plays in a loop, so our wonderful respiratory technician brought in a DVD player with Toy Story. Nolan was quite happy with the arrangement, particularly when she rearranged his head wrap so he could put his hearing aids back in.

He fussed a little when the movie was over, and asked us to take his "mustache" off several times. He finally fell asleep, but not for long, He cried out frequently, but the tech came in and told me it was during stage 3 sleep. His legs moved all the time, waking him from sleep. I heard a few apneas, but I am not sure if they were central (some centrals are normal) or if they were obstructive apneas.

With the constant thrashing and crying out, I did not sleep at all. At 5:00 am, the lights came on.

We woke Nolan up. Well, we sort of woke him up. He was in a zombie-state for quite some time.

We left the hospital and drove 2 hours to our house. Nolan fell asleep on the way home, so he got a little more sleep in the car. I am beyond exhausted.

Tomorrow, we get up at 4:00 am to take Matt to the hospital for his supraglottoplasty.

At least we're never bored.

Ehlers Danlos Support Groups and Resources

This post is mostly for my own benefit, as I have been searching for EDS support groups online. I just want to link to them here, so I don't lose them!

I found one group for parents of children with Ehlers-Danlos Syndrome:

Ehlers-Danlos Syndrome: Parent Group

There is another Facebook Group for the Ehlers-Danlos National Foundation (EDNF), and the main website for the organization.

I also found an interesting article on the genetics behind Ehlers-Danlos Syndrome - The European Journal of Human Genetics has the article here.

I still can't find much in the way of severe hearing loss and its relation to EDS. It isn't a common issue to have with the syndrome - it is possible, but very rare.

I have been trying to find cases of EDS similar to Nolan, and have found a few cases that have his gastrointestinal problems. The gastroparesis and cyclic vomiting are Nolan's biggest hurdles in the GI world at the moment, though he is gaining weight and growing with his night feeds.

We will have to make some decisions about Nolan's participation in certain sports as he gets older. For now, we are letting him continue with soccer since he 1) loves it and 2) the sport is gentle in the younger leagues. In the U-6 league, there is a lot of chasing the ball and not a lot of contact between players. He will start swimming lessons on July 8, and hopefully he will take to it like he did last year. Swimming is a good activity for kids with EDS.

With that said, we are heading out in the disgusting drizzle for Nolan's soccer practice. We are not sure where summer is, but we'd like the sun to return to Western NY!

New Ear Molds

Nolan was in desperate need of new ear molds. We picked them up last week - he went for the green-and-blue look this time.

Next week is the last week of school, and we are eagerly counting down the days! Both boys have their class field trips on Friday (Nolan is going to a local playground with his class, and Matthew is going to the Erie Zoo).

I have to admit, I am looking forward to summer. It will be nice to get a break from the early morning routine!

Matt's Surgery Scheduled

We have been so busy that I haven't had time to write. Baseball, soccer, doctor appointments, school events and projects, and piano recitals have kept us overwhelmed with activities! June is a crazy month.

Matt had his ENT follow-up for his tonsillectomy and adenoidectomy (T&A) surgery. He still has very obvious signs of sleep apnea. Since our ENT did the scope at the time of his T&A and found the laryngomalacia, we know that the obstruction is not going to go away without help.

Matt received a once-over and he is not hyperflexible like Nolan is, so he probably does not have Ehlers Danlos Syndrome. His reflux, laryngomalacia, and constipation issues are likely coincidental. This is good news, as he should heal well and not have a recurrence of the laryngomalacia once surgery is complete. He will remain on anti-reflux medication indefinitely.

Matt's supraglottoplasty has been scheduled for June 25. They will laser out the floppy arytenoid folds so that he can breathe freely at night (if you have no idea what a supraglottoplasty or laryngomalacia are, then read more about it here and here).

Fortunately, my mom is flying out to care for Nolan while we are in the hospital with Matt. We need someone who can take care of his g-tube and give him Zofran (if needed) while we aren't at home. I am grateful for the help, as our only other option is to have one parent at the hospital and one at home with Nolan.

Nolan also has new earmolds, but I'll share those in another post.