Mommy's Technical Notes re: ER visits

When we first started researching SMA, just before Oliver's "official" diagnosis, I remember reading different sites - university web sites, FSMA, various blogs - and wondering to myself what all these different terms and numbers all meant. What is a de-sat? Why is it important to have a pulse ox? For that matter, what is a pulse ox?

As we've walked along our SMA path, we've vowed to not only give Oliver the best care that we can but to also re-pay the SMA community: to "mentor" other families much like we were; to answer as many questions for our friends and family as we could; to raise awareness of the disease; and to raise awareness about carrier testing.

This post - the one you are currently reading - is meant to help answer some of those questions. We've told you about our ER trips and Ollie's fondness for riding in the ambulance. (He's a little boy, after all, and they like vehicles that go fast, drive on medians, and make loud "woo wooo wooo" sounds, right?) But how do we know when to suction or do CoughAssist? And when do we decide to call 911? And why does Ollie need to go to the ER so often? (Please feel free to ask us any other questions you may have. We're happy to answer them.)

So, we've been to the ER three times. On April 8th, the pediatrician's office called the ambulance for me. Our second visit was on May 4th after a routine visit to Duke to see the neurologist. And our most recent visit on May 29th. All three visits are likely due to mucus plugs - a fairly common malady for folks with SMA. Since SMA weakens the muscles used to breathe and to cough, small amounts of mucus - that unaffected people would just cough and clear - build up in SMA patients and clog a portion of the lung. In severe cases this can result in a partial collapse of the lung.

When we have these little adventures to the ER, they are usually precipitated by oxygen desaturations (often called de-sats). In Oliver's case, his baseline (or normal) oxygen saturations are between 97 and 100 percent at rest and between 95 and 98 percent when asleep. If he is awake and his sats drop below 95, we can usually bring them back up with some suctioning and CoughAssist. Dropping to 95 isn't usually a panic situation.

Everyone's sats vary at different times. When you sleep, your sats will decrease because all the muscles in your body relax - including the muscles that help you breathe. At night, when Oliver is asleep, he will often de-sat into the mid to upper 80s and his pulse ox will alarm. (The settings are adjustable on his pulse ox. We have it set to alarm if his oxygen drops below 90 or if his heart rate rises above 200 beats per minute.) This may happen only once a night or it may happen repeatedly. Usually, he will drop long enough for the alarm to sound once (or maybe twice) and then he'll bounce right back into the 90s. Sometimes it will sound more than once or twice and it takes turning his head or rubbing his leg to get him to take deeper breaths and bring his sats back up. Occasionally, we may even have to wake him to do CoughAssist or to suction.

If his sats drop and his heart rate rises at the same time, it's a pretty good indication that he's distressed. Each time we've gone to the ER, his sats have dropped into the 70s (or lower) and all of the CoughAssist and suctioning we've done haven't brought him back into the 90s. Right before the first ER visit, from the pediatrician's office, we were getting sats of mid to upper 60s when they called 911. By the time the ambulance arrived, the pediatrician had done quite a bit of suctioning and Oliver's sats were in the upper 70s.

The second trip by ambulance to the ER was right after we had gotten back from Duke to see Dr. Smith, Oliver's neurologist. Ollie had been sounding raspy while we were at the appointment. I'd done several rounds of suctioning. I checked his sats and suctioned a little - to be on the safe side - right before we got in the car to head home. Oliver slept the entire ride home.

When I got him out of the car, he was obviously distressed. His little face was covered in beads of sweat. He looked at me with concern in his eyes. I took him in the house and hooked up his pulse ox. I was expecting a reading of low 90s or maybe upper 80s. Nope. He was registering in the 70s. I did CoughAssist and suctioned repeatedly and only got him "stablized" into the mid-80s. My dear friend, Cathy, got to make the 911 call that time.

The third ER visit Neil has told you all about in his post. Will it be the last trip to the ER? Probably not. As his SMA progresses, all of Oliver's muscles will continue to get weaker....including the ones used to breath and to cough. In spite of all of our "drama", we consider ourselves very fortunate. The only hospitalization Ollie has had was for his g-tube and Nissen surgery. And, so far, he's not had a collapsed lung. Many families are not as lucky.

ER #3

Friday (5/29) proved to be an eventful day, but not in a good way. I arrived home a little early so that Bekka could head out to an appointment. I'd only been home a few minutes when Ollie's sats begin dropping. They very quickly descended into the 70s. Vigorous suction and cough assist managed to get him into the low 80s, but no higher. When he dropped into the 60s before bouncing back into the 80s and after we had been at this for several minutes, we made the call to 911.

By the time the paramedics were here, he was back to around 90, but still sounded horrible. They gave him a hit of supplemental oxygen and we headed out the door. Mom loaded up with Ollie into the ambulance and I followed a little bit later with some of the necessary bags.

By the time, we were all at the hospital and in an ER bay, he seemed to be doing really well. In fact, they were drawing up the discharge papers when his sats crashed again. We got his numbers back up pretty quickly, but his heart rate remained elevated. This is always a sure sign that he is still experiencing respiratory discomfort, even if his sats are in a more normal range.

We requested they send in a respiratory therapist to do deep suctioning. Several rounds of this brought up a large amount of very, very thick (but clear!) mucus. He began to improve slowly and Bekka and I were having discussions with the Doc about admission vs. going on home. The decision was not an easy one and they let us continue hanging out in the ER while we made the decision. Pretty soon, he began to perk up to normal Ollie mode and grinned at Daddy! We knew then we could head home.

In the meantime, members of the WakeMed branch of the Ollie fan club came by to see him. I don't think any parent wants to be a "regular" at their local ER, but it's a comforting feeling to know the nurses like your little fella so much they go out of their way to visit. We've been so impressed with our experiences at WakeMed and with Wake County EMS. We can't begin to offer enough praise for their professionalism AND their compassion.

As of now, we have backed off his Robinul dose (though not completely, as that is not a tenable situation) and are being even more vigilant.

We need a bigger house....

Don't get me wrong. I'm grateful for all of the supplies that are arriving on my doorstep - bottles of formula, feeding bags, tubing, etc. But it all arrives in bulk; one month's worth of supplies at a time.

Our house was "cozy" before Oliver arrived. For nearly nine months, we worked at cleaning out closets. We reorganized to make more room. We bought a small storage shed. And when Oliver arrived in August, our house became a little "cramped" with teeny clothes, play mats, swings, diapers and such.

Then, in December, the equipment began arriving. At first, just a CoughAssist and a pulse oximeter. Neither had much in the way of additional supplies (or "consumables" as the insurance company likes to call them). Then we got a couple good-sized boxes of SimplyThick to add to the breast milk. Then we switched to formula with the SimplyThick added.

In February, the newest addition to our equipment family arrived: a nebulizer. And several little boxes of albuterol to go with it. A mask or two. Some tubing. A makeshift adapter with a T-connection so Ollie could have his treatments while lying down. We'd gone from a little cramped to surrendering the coffee table to the equipment family.

We got home from the hospital on Tuesday with a couple of patient belonging bags with 5mL and 3mL syringes to flush the tubing for his feeds. Several Y-site extension tubes to make it easier to administer medicines via g-tube. A couple of 60mL syringes to vent his g-tube and give feeds via gravity. A few bags to hang feeds in case we got a pump.

Then Wednesday rolls around. The home medical equipment and supply company delivers a Kangaroo Joey feeding pump with small backpack. More feed bags. A pole to hang the feeds and mount the pump.

Today, a box of 30 feed bags arrives. Along with more Y-site extensions. Plus four cases of ready to feed formula. And one can of powdered formula. The tubing that runs from Oliver's Bard button to the Y-site extension tube hasn't even arrived yet.

"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.

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.