So, to Nissen or not to Nissen...

I finally found a good discussion on the Nissen fundoplication surgery - for children. Most of what I had read was relating to the surgery in adults.

So, we're dealing with several issues. The G-tube surgery has a relatively easy recovery as compared to the Nissen. The hospital stay would be much shorter. Less time in the hospital cuts our chances of getting an infection (staph, RSV, etc.). It also would be less time under anesthesia.

It would be ideal to do either or both surgeries while Oliver is still pretty healthy. Once his health declines or his breathing becomes more difficult, the whole anesthesia intubation/extubation monster rears its ugly head.

If we don't do the Nissen now, we pray that he doesn't have problems with reflux. Unfortunately, reflux is one of the major side effects of feeding via G-tube. Even if there have been no indications of reflux prior to the surgey. (Oliver's gastric emptying study only revealed one minor incidence of reflux just after starting the study. He has only spit-up 3 or 4 times - total - in his 6 months of life.)

If there is reflux, we could risk doing the Nissen surgery later or the tube into the stomach could be lengthened so that it empties into his intestines. Lengthening the tube means that his stomach would be bypassed and he would always feel hungry. Plus, if the tube goes into the intestines, you have to do continuous feeds. Bolus feeds are not an option. (Bolus feeds are feeds that are done 4 to 8 times a day for 15 to 30 minutes each time. This is more like a normal bottle-feeding schedule.)

As you can see, this is a complicated decision-making process.

We are leaning towards doing both the G-tube and the Nissen fundoplication. And doing it fairly soon. I would like to get all of his doctors together before the surgery to make sure that we are all in agreement on how to best handle Oliver's care. (I don't want to have to argue the pros or cons once we're in the hospital and have had the surgery.) I hope to do a little bit of educating with some of the info that I've found relating to these procedures in children with SMA. I'd love to get some of the standard of care guide information into his chart so that every doctor or nurse can easily reference it.

There is one other "fun" aspect of this process. One that we haven't really touched upon yet - the insurance company. I'm not looking forward to that battle.

How Long?

I paged the pulmonary doctor today to ask a few questions related to the G-tube surgery. The biggest question I had forgotten to ask was how long of a hospital stay would be involved. I wasn't prepared for the answer.

If we do both the G-tube and the Nissen, his prediction was around 2 weeks or more. That's assuming that things go well. If we just do the G-tube, it's probably 4 or 5 days. I had thought a couple of days for the G-tube and maybe a week for the combination.

But 2 weeks.

That's a long time.

"Breathing Basics: Respiratory Care for Children with Spinal Muscular Atrophy"

I just finished reading the new Breathing Basics brochure from FSMA. I highly recommend reading it. It outlines the respiratory challenges faced by a child with SMA. And it discusses the difficult decisions that parents must make in caring for their child. I plan on keeping a couple of copies handy to give to doctors that are unfamiliar with SMA - especially in emergencies.

And, after I read it, I pulled out the pulse ox and hooked Ollie up to it.

His heart rate is between 100 and 140. His oxygen saturations are between 96 and 100. In other words, he's breathing pretty well at the moment.

Our Visit with the Pediatric Surgeon

So we were hoping to meet with the surgeon and schedule a date to have all 3 procedures done - the G-tube, Nissen, and Botox injections. (See previous post.) The surgeon, however, is concerned about doing all 3 at once since that would mean Ollie would be under anesthesia for almost 2 hours: 30 minutes for the G-tube, 45 for the Nissen and another 30 for the Botox. The fear with SMA is that if you intubate (which is necessary while under anesthesia) you may not be able to extubate.

We're now trying to consult with an otolaryngologist about the Botox and whether it can be done without the anesthesia. Or whether the Nissen is a must-have with the G-tube. Basically, we're trying to pick 2 of the 3 procedures which means finding another way to do the 3rd choice or not doing the 3rd choice at all. More research and more questions for doctors and other SMA families.

On the way home from the consult with the pediatric surgeon, Ollie was sounding rather raspy and was unhappy about being in the car seat. As I was driving, I would be looking at traffic and then looking at Ollie in the mirror. Back and forth, back and forth. About half way back to the house, I decided to pull off the highway because he just wasn't getting any happier and the more he cries, the more gunk there is to suction out of his mouth with the blue bulb syringe. I had hoped to just suction his mouth, calm him and get back to driving. But there was soooo much stuff in his mouth. I ended up letting my friend drive us home while I sat in the back seat suctioning most of the way to the house. (Thank you, Cathy, for remembering how to drive a stick shift.)

I felt bad for the little guy but it was also a bit scary. Was it caused by a cold? Is it his SMA causing the extra saliva and mucus? Will he be able to travel in his regular car seat much longer? What do we get for the car if he can't? So many questions....

We made it home and I hurried him into the house and fired up the good ol' CoughAssist. Honestly, though, once he was lying flat on the floor he began to sound better. We did a couple rounds of the CoughAssist and I turned him on his side to let any extra saliva run out of his mouth. And then he smiled. A great big smile. As if to say thanks, Mama.

So since he's got the extra congestion in his nose, we're going with the cold. We had noticed a day or two ago that there was a little bit of color to his mucus. (Sorry, folks, this isn't a clean disease either. The words mucus and secretions are part of the daily vocabulary.) I called his pediatrician at every number I could find for her. She called me back and we talked about over the counter meds and antibiotics and humidifiers and saline drops. And she called a prescription in for some amoxicillin. (BTW, have I mentioned that we love Dr. Goodmon before?)

Poor Oliver...he's gonna be seeing a lot more of his friends CoughAssist and bulb syringe for a while.

So what is Ollie's occupation anyway?

Oliver had his Occupational Therapy (OT) evaluation yesterday. Beth, the Occupational Therapist from the Raleigh CDSA, brought several switch-activated toys for Ollie to try. He loves the "Bouncing Butterfly" plays music and vibrates when he touches the super-sensitive plate switch. Each time he pushes on the switch it starts a new song - Farmer in the Dell, Home on the Range, Do You Know the Muffin Man, etc. He thinks it's sooo much fun! I love watching the smile on his face when he starts it. He's so proud of himself.

Beth was pleased with the "regular" toys that we have for him. We've tried to pick lots of lightweight and easy to activate toys like pinwheels, Oballs, Beanie Babies, helium-filled balloons, etc. She also liked that we had a bean bag for him. (Thank you's to Megan - our PT from Pediatric Therapy Associates - for the suggestion and to Santa for the delivery!)

So we have a few of the "adapted" toys on loan from the CDSA for the next month. Hopefully, we'll also have a feeder seat on loan in the next week or so. It may not work any better than the reclining booster seat we already have, but it's worth trying. Ollie loves his sweet potatoes!

PT Visit

Ollie has been getting physical therapy at home on Friday afternoons for a little while now. He loves it when Miss Megan comes to visit!

Today's session went very well. Ollie did a great job lifting his arms up over his body. He even got his elbows up off the quilt several times. He also surprised both Megan and me with rolling onto his left side. He can do it, but it usually requires a lot of help. Not today! He looked as if it was a breeze. Way to go, Ollie!

Well, gotta go - somebody is hungry!

We have an in-home Occupational Therapy appointment on Monday afternoon. I'll update you soon!

6 Month Check Up

We saw Ollie's pediatrician today. We love Dr. Goodmon. She has been fantastic through all of this. She even got us a couple of the blue bulb syringes from the hospital. You know, the ones that actually work. (We've purchased and thrown away four that were useless.)

Today, Ollie got 5 vaccinations:

• oral Rotavirus
• flu shot
• Synagis, a.k.a. the RSV vaccine
• Pentacel - diptheria, tetanus, pertussis, polio & Hib
  
• Prevnar - pneumococchal bacteria (like meningitis)
  

He weighed 14 pounds, 1.5 ounces - 3rd percentile.
He measured 26 inches long - 26th percentile.
His head circumference was 45.75 centimeters - 92nd percentile.
A "big" thank you to Daddy for the large noggin!
We'll go back in a month for another flu and Synagis shot. And then in 3 months for his 9 month check up! My how time flies...

Reality Check

A month is a long time in the life of an SMA baby. That point was driven home recently when Bekka and I suddenly realized how different life in February is from life in January.

On Janurary 11th, the cough assist machine was a suggestion. We would sometimes give him a treatment a couple of times a day just for practice and therapeutic purposes - but he didn't really need it. On February 11th, the cough assist is no longer an option, it's a way of life. We don't wonder if he needs a treatment, we wonder how often.

The blue bulb syringe - Ollie's great nemesis in life - used to be a once or twice a day annoyance. Sometimes during feeding, sometimes just because he was rattling a bit. Now it's a many times a day event. (Who's counting anymore?)

And now we have a nebulizer. Happily, it really seems to help him breathe a little easier and loosens congestion up, but we surely didn't need such a thing a month ago.

All the info on SMA and all the stories from other parents tell you that 6 months is often a turning point when life gets harder and the road gets rougher. When everything is going well, you say to yourself, "Hey, this isn't so bad. Little guy is doing great!". Then you find yourself preparing for a consult about a feeding tube and wondering whether a car ride is such a good idea because he might get choked up.

February 5 was six months.

Reality can be a harsh mistress

Pulmonary Visit - Feb. 6, 2009

Yes, we need to get this blog up and running! We have been sidetracked by Ollie's cuteness - at least that's our story. Perhaps we can get it up this week. Once it is functional we'll even be able to email updates from our phones.

Info from Ollie's Pulmonary Visit with Dr. Kravitz on February 6, 2009:

• Breathing function was decreased but still within normal limits.
• When they administered a breathing treatment, it caused a lot of mucus to be expelled. As a result, they've added a nebulizer to the machines we have for Ollie. Thankfully, the amount of stuff we are getting up is getting less after each treatment.
• We are to continue the CoughAssist twice a day (or more as needed) and the nebulizer at least once a day and no more than 4 times.
• We are to monitor his pulse and oxygen saturations with his pulse oximeter once or twice a day.
  
• After discussions with the neurologist and pulmonologist, we have scheduled a consultation with Dr. Henry Rice, a pediatric surgeon, (currently scheduled for next week on February 18) to schedule a gastronomy tube (G-Tube) placement with the Nissen fundoplication to minimize the risks of reflux and aspiration.
• In the meantime, we are also supposed to evaluate his secretion levels (they are already quite high) and they will consider performing Botox injections to slow them down. Apparently, this is beginning to prove more effective than drying agent drugs and doesn't have the side effect of thickening saliva and mucus, meaning a mucus plug in the throat or trachea is less likely to occur.
• We are scheduled to see the pulmonologist in 2 months (appointment is set for April 3rd), or sooner if anything changes dramatically in Ollie's condition.

So overall, he's doing pretty good. He's still eating well (both solids and formula) but solids are beginning to be a bit more of a challenge due to the excessive salivation.