Pacer Update and Fun Stuff

Josh Medical Stuff / Pacers & Vent Update

When we saw Joshua's pulmonologist last time, when we started the pacers in October, he told us Joshua could use them as tolerated. He suggested maybe like 15 minutes to an hour long three or four times a day to start. So, those were the orders he wrote (the 15 min-1Hour 3 or 4 x a day). But he failed to write that could increase as tolerated in the orders, so school and home nurses weren't able to let Joshua use them as much as he'd like.

At first, yes, Josh needed more breaks from the pacers because when you get shocked in a nerve causing muscles to contract really big when you've never had that happen before is going to cause you to fatigue quickly. Josh also had to get used to trying to eat, drink and talk around the times the breaths came on suddenly. And we had to watch and get used to increasing and decreasing the number of breaths the pacers gave per minute.

But as Joshua got stronger and more used to the big breaths and his muscle doing more work, he could tolerate the pacers longer and longer. On weekends, when no nurse was on duty, he would put on the pacers about mid-morning and leave them on all day - until a nurse would come at night and he had to get ready for bed. And he did great with them.

So, we were due for another doctor visit December 1st (with the pulmonologist). When we last saw a doctor (endocrinologist Oct 16th) Joshua weighed 86 kilos (about 189 pounds). December 1st, though, he dropped to 83.2 kilos (about 183 pounds). He lost 6 pounds - and that's even over the Thanksgiving holiday when we all ate more than we usually do! And Josh has grown a little and is just over 5 ft 1 inch tall now.

I really think the pacers are worth their weight in gold! Not only is Josh getting a workout by taking this big breaths all day, but he is also able to move about a whole lot more (not being connected to a ventilator all the time). And when Josh is on the ventilator, his nurses are noting that his tidal volumes (how big his breaths are) are much larger than they used to be. They are amazed.

Josh did come down with a cold though over the weekend prior to going to the doctor this time. His nose was pretty plugged up and lots of drainage. So, we let him stay home after his doctor's appointment and get some rest. And the doctor recommended Sudafed (or the generic kind). We picked that up and also gave him some Afrin Sinus No Drip nosespray. They have really helped and he's feeling much better today (December 3rd).

Dr. Smith (pulmo doc) also wrote orders this time saying:

"amplitude to right 4.5 and left 5.5. If needs adjusting, call Dr. Smith for adjustment values. Rate 7-14 breaths per minute. Hourly checks for CO2 levels while awake. Keep CO2 in 30-45 range. If CO2 <29>

Josh is to use his ventilator when not on the pacers (unless his CO2 is less than 29 and he's needing that to increase).

Here are his ventilator settings:

Ventilator: LTV 950
PEEP set at average of 5
Mode: SimV/CPAP, Pressure Control 24, backup volume 425
Breath Rate ladder for night 12-24. Pressure support at night 14 cmH20,
ETCO2 30-50. Inflate cuff at night.
Rate ladder for daytime set @ 12-18 cmH20 (Do rate ladder last),
Pressure Support ladder daytime 12-25 cmH20 (do Pressure Support first). Deflate cuff while awake.

And these are Joshua's medications:

Bacroban ointment 2%, apply to trach at night
Cortef 5 mg, 1 tablet in the morning
Cortef 5 mg, extra tablet if running fever >99.8
DDAVP Nasal 10 Mcg/inh, 2 sprays each nostril twice daily
Norditropin Cartridge 5 Mg/1.5 Ml, 3 mg subq 6 days a week
Synthroid 0.75 Mg, 1 every morning
Calcium 333 mg, Magnesium 133 mg, zinc 5 mg, 1 each morning
B Complex, 1 each morning
Acetyl L-Carnitine 400 mg, Alpha Lipoic Acid 200 mg, 1 each morning
Flovent 44 mcg 2 puffs twice daily
Pataday allergy eye drops, 1 drop each eye every morning
Ibuprofin 400 mg daily as needed for pain or fevers
Albuterol HFA, 2 puffs inhaled as needed (THIS ONE IS NEW and due to school nurse request - has not been used yet)

I wrote all these specifics that may be hard to understand for the normal person who isn't taking all these medicines or on a ventilator, etc. because I wanted anyone out there who might have a child with this disorder to have it. Since Joshua has been pretty stable with these settings and medicines/vitamins, maybe it can help someone else too.

If anyone reading this is interested in what we use to help keep Joshua's pacemaker antennas in place, we have found this works best for us so far:

Gold's Gym Waist Trimmer Belt from Walmart. It costs $5.00 and made of soft rubber foam material. It is designed to make you sweat off pounds. But we turn it inside out and I've added a little extra velcro to help hold the end of the strap in place. But you can tighten it as much as necessary to hold the antennas in place and it doesn't roll up or bunch up as badly as Ace bandages, etc. Josh simply can't use the tape or bandaids recommended to hold each antenna. The adhesive breaks down his skin too badly.

Oh, and instead of using the belt that came with the pacer box (look at the picture up top and you'll see the boy wearing the box at his waist) we have purchased a small black backpack and placed the pacer transmitter box in it. It keeps the wires from the antennas up high so they don't get in the way when Josh needs to go to the bathroom, etc. Josh likes the backpack MUCH better.

FUN STUFF

Okay, now on to some fun (well some of it's fun). I work at a daycare that requires me to be fingerprinted. I had an appointment to go do that yesterday (Wednesday). The place I went to for it is right in front of a Big Lots store and a Family Dollar in New Braunfels. While I waited to be fingerprinted, I was in the waiting room looking out the window at those stores. They weren't too busy at that time of the morning, so I thought I'd sneak in there after I was done and see what I could find for the kids. I can't really afford to be doing this (since we are struggling so and really need to come up with our tax money for our house very soon plus I had to shell out 45 bucks for the fingerprinting!) but I just couldn't resist doing a little something for the kids.

This is what I found:

They are supposed to be ornaments for a Christmas tree - little stockings of different kinds. They are so cute. And I found glitter glue stick pens so I could write the kids' names on them. I also found some cheap plastic ball ornaments that were very bright and shiny the kids would love. So I got those to hang from the ceiling in the classroom.

The kids' eyes lit up (not to mention some of the other workers' eyes) when I got to work with my goodies. I'm still working on getting them all put up. I'll do some more of that today when I go in to work.

After work last night, my sister insisted she treat us to the movie, A Christmas Carol in 3D. She even invited my daughter, Steffany (who is about 6 weeks from her due date). We left for the movie after getting my hubby off to work for the night. We dropped by and picked up Steff at her apartment and headed for the IMAX theater on the NorthWest side of San Antonio.

The special effects were great - it is in 3D. But the story itself has been told and retold so many times, it was a bit old to me. Josh really enjoyed it though. He recognized the story from other shows. He was afraid he wouldn't like the 3D part - because he didn't particularly like water or air being blown at him, etc. during past 4D movie experiences. But once we assured him nothing like that would happen, he calmed down and enjoyed the show. Here's a clip from the movie:

And pictures of Josh and Steff from the movie theater:

One more fun thing I'd like to share is a video my sister showed me last night, then we heard mentioned on the Craig Ferguson show, too. It is called Surprised Kitty

I hope you all enjoy it as much as we do!

Pacers are ON!

FINALLY we went to the doctor yesterday, October 6th, and got to turn on Joshua's diaphragmatic (breathing) pacemakers.

What exactly does that mean? What all is involved?

Well, check out this diagram again:


It shows an electrode implanted near the neck that connects to the phrenic nerve. The phrenic nerve causes the diaphragm to contract. Then the diaphragm contracting makes air suck inside the lung. When it releases, the lung pushes the air back out.

In the diagram above, just below the electrode is a receiver. It receives impulses and sends them to the electrode, causing the stimulation for the diaphragm to contract.

Both the electrode and receiver were surgically implanted into Josh's chest back in June. His incisions and placement of the devices were a bit different than in the diagram above. Here is a picture of Josh's incisions from back in June and July.





He has two over the nipple area and two underneath. The two above are very deep where the doctor went in and placed the electrode around the phrenic nerve. The two below are where the doctor made much shallower cuts to make pockets to hold the receivers. The receivers are about the size of quarters. The electrodes and receivers look like this:



Over the receivers, on the outside of the skin, when you are going to turn on the pacers, you wear antennas. They look like this:



The antennas plug into a small box, called a Mark IV Transmitter. It looks like this:


The box sets how strong the "shocks" are to make the diaphgram contract and it controls how often it sends the shocks. There are controls for strength for both the right and left lung. And you can turn on or off each side. This box is worn on a strap that can be connected around the waist like a belt.

Well, yesterday, we went to see Dr. Kelly Smith, Joshua's lung doctor (pulmonologist) in San Antonio. He placed the antennas on Josh's chest with the circles around the receivers. We held them in place with a wide Ace bandage we bought. He then plugged in the antennas in the Mark IV transmitter box. And he turned on just the left side, to set the strength. And for the first time, we saw how it looked when the shock made Josh's chest move and take in a breath. I hope you can see this video I took showing the left side pacer working:



If not, here are some still pictures from the doctor's appointment:

The doctor gave Josh a break after setting the left side. Josh got back on his ventilator and the doctor went to see another patient. Then, he came back and set the right side. Then, he turned on both sides at the same time. He adjusted and readjusted many times the strength on both sides. And the doctor changed the breath rate several times.

All the while, we kept Joshua's trach hooked to his End Tidal CO2 monitor (a tube that connects the trach to a box that checks how much CO2 is in his breaths). And we kept his pulsox hooked up to his finger. That checks the amount of oxygen in his blood. It also tracks how fast his heart is beating.

When Josh was off his ventilator and we started the first side of the pacers, making adjustments to strength and rate, his CO2 went up to the low 50's which is a bit too high. And his oxygen saturations were down in the low 90's to high 80's. But by the time the doctor got everything set just right, the CO2 went down to the low 30's and his oxygen saturation went up to near 100 and stayed there. Both those were perfect! And his heart rate went from being over 100 beats a minute to down in the low 80's - much better.

We were very pleased with the results. But we have to keep in mind that using the pacers will make his diaphgram muscle pretty tired out and sore, since he's not used to it working so hard like this. So, he is only using his pacers for short times and then taking breaks. He will stay on longer and longer as we go, as he tolerates it. Josh gets to decide and that is great.

The only problem we have seen so far, and it concerns me a little, is a little blistering around the top outside part of the antennas on Josh's chest. I noticed them this morning as we went to put the pacers back on for today. Josh didn't complain about them, but I could see it was a bit worse on the right side than the left. The right side is set a bit stronger than the left. I'm not sure if that is the reason for them or not. But, I put ointment on them and will check on them again tonight. If they get worse, I will have to call the doctor and see what he recommends.

Well, I've gotta run now. I've got to head to work soon for my third day working in a daycare center!