Friday, April 24, 2009


You can't have a conversation about Spinal Muscular Atrophy without the topic of ventilation making it's way to the surface. Lately we have been questioning Kaitlyn's ventilation and whether or not she is being ventilated properly, or rather if there may be more things we could be doing to optimize her ventilation. Sometimes it can be hard to find a pulmonologist who supports, or even understands, non-invasive ventilation (NIV). As Kaitlyn's parents we are first in line to advocate for her every step of the way. Ventilation is no different.

The very first appointment we had with a pulmonologist we asked about cough-assist and bi-pap. We had read that they only way for children with SMA (specifically the weaker type 1) to survive and have any quality of life was to use these machines. The only other options would be to let nature take its course and our daughter would not have lived very long, or have an operation for invasive ventilation (a tracheotomy). We were told that NIV and cough-assist wasn't done on babies as young as Kaitlyn was at the time of her diagnosis (5 months). When in fact it is, and some babies use NIV from birth. We did eventually start an NIV protocol, but not until Kaitlyn was 10 months old!

We finally asked a bunch of questions during Kaitlyn's more recent hospital stay. (Goodness knows we were there long enough to ask several people LOTS of questions.) The result was a meeting with Kaitlyn's pediatrician, the RT's at our children's hospital, and the RT from our DME company to discuss Kaitlyn's ventilation. The result of this meeting is that Kaitlyn will now be properly followed for her ventilation needs.

We have had to increase Kaitlyn's pressures on her bi-pap again. She is now 20/3. The left side of her chest has taken a little dip over the past couple of months and she had started peridoxal breathing again. Peridoxal breathing means that as she breaths in her diaphragm expands and her chest falls, and on the exhale the chest rises. Typically this means that pressures need to be increased to help blow open the lungs, expanding the chest, so that Kaitlyn won't have to use her diaphragm to breath. The idea behind the bi-pap is that Kaitlyn allows it to do most of the work while she sleeps so that she can get some true rest and not have to work so hard to breathe.

This meeting allowed my husband and I to ask some questions and raise some concerns. Kaitlyn is dependant on Bi-pap, of this there is NO question. All the medical professionals involved would have to agree. She may only use it nocturnally (except when she has the common cold where she uses it 24/7) but without it she would get too tired to breathe after only a couple of days. She might not even make it through the first night as children with SMA have such low tone that they breathe too shallowly in deep sleep to ventilate their own lungs and they could even stop breathing! So our concern is at what point is she a candidate for a secondary vent? Our DME company told us that if something were to happen to Kaitlyn's bi-pap that they might not have the same unit to replace it with, and they may not have a machine capable of performing all of the things Kaitlyn's bi-pap does now. Then we would be without a bi-pap until ours got fixed!! This is really scary!

The next issue is if we are considering a secondary ventilation machine do we go with another bi-pap or do we switch Kaitlyn to a vent (LTV or some other similarly capable unit). A vent has far more options for ventilation than a bi-pap. It can handle higher pressures, it can be volume cycled rather than pressure, it has a lot more alarms, and can be used for invasive ventilation should the need ever arise! During illness the vent could be huge for Kaitlyn! It would allow her to remain home long after the bi-pap stopped working for her. The bi-pap only handles a maximum pressure of 30, of which Kaitlyn is using 20. When sick, if we use similar pressure increases as colds she has had just this past winter, then we would have to go as high as 26-27. This doesn't leave us a whole lot of pressure for emergencies!

It's unlikely that a vent will be approved for funding. Vents are much more expensive than bi-pap. We have also recently been approved for funding for a secondary cough-assist. I am ever hopeful that the cost of a vent would be weighed against the cost of a two week stay in PICU!! It's in everyone's best interest that we get the equipment Kaitlyn needs so that we can continue to manage her at home. So this is what we will be wishing for the next few months. (These things take time).

Otherwise, Kaitlyn has been fine. She has been making some advances in the way of speech. She is experimenting with a lot of new sounds. She uses a lot more words throughout the day now. Still no sentences or even close to it, but she is at least mixing some real words with the nonverbal communication that she has perfected!! We've NEVER had any trouble understanding exactly what she wants (her nonverbal communication is superb)...probably why she has gone so long without speech!! Yesterday she was a train..."CHOO-CHOO". Pretty funny ;o)

Today Kaitlyn "decided" that she was going to wear her new Dora PJ shirt all day!!! There was no way that shirt was coming off this morning without a WHOLE lot of grief! LOLOL We compromised on the pants. Doesn't she look proud of herself!?!

My Goofy boys acting all silly. They were blowing rude noises into my leather couch...which they thought was incredibly funny!!!

The weather has been so nice the past few days. Sunny and warmer! We have been going for walks after baths before bedtime. Just around the outskirts of our building, but it's better than nothing. This is Kaitlyn all clean and waiting to go outside.

Those of you that follow me on FB may have read that Alexander fell this week and split his chin wide open. He was racing up and down the hallway with Owen. They were each pushing a large truck, racing and making tons of noise! I was yelling at them to stop when I heard....CRASH! Then Alexander comes out of the hallway crying with a big split in his chin. Poor guy, it was too deep for dissolvable stitches, so he has six stitches that need to be taken out. More than likely he will be left with a permanent scar. Sorry for the picture quality, he wouldn't hold still!! (The stitches are blue).

1 comment:

Devon said...

Ow! I missed the fall! Poor guy...

Surely they will approve her for a vent...I cannot imagine them not doing so, especially since if her bi-pap goes out she will be without! AGH!

Hugs to you guys. How is everything else up in the frozen north? (LOL)