Here is a series of articles and videos to guide respiratory decisions that must be made when the diagnosis is Duchenne muscular dystrophy. People need to be educated because not all pulmonologists will give ALL the options and you may have a tracheostomy placed prematurely. After reading or viewing the following educational links, and choosing a supportive pulmonologist, you will be forearmed and proactive so that a sudden, hasty decision will not have to be made under duress.
My son, Roy, with DMD is 32 and has used the night time v-pap with nasal mask and daytime volume vent with mouth interface (“sip-vent”) for over 10 years and has thus avoided a trach.
Derek getting his first mouthpiece vent.
Thank you Louis Boitano for giving so much to keep our Duchenne
children alive without needing trachs. RIP
Ways to setup a NIV system
There have been some questions on my sons NIV setup, We thought the answers should go in a more public area, so here is a description of our setup.
The gray hose used is a product used industrially called Snap-Loc http://www.cedarberg.com/snaploc.htm or Lock Line http://www.modularhose.com We have the Snap-Loc on this setup. It pops together like pop beads (get the pliers, they’re worth their weight in gold when beads break). It is available in ¼”, ½”, and ¾” inside diameter. There is a starter piece that has a threaded end and globe end, The threaded end is in standard pipe thread. we mount that to a custom built bracket that is clamped to the chair. Before it was on a flat piece of aluminum and wedged between the seat pan and frame of the wheel chair to bring the tubing up front. Some people fasten it to the head rest of the seat back, that is how we got it from our RT, mounting is all personal preference.
You don’t want to breathe directly through the Snap-Loc, it’s not food grade and hard to clean. A piece of food grade clear plastic tubing, found at any hardware store, with a vent fitting on 1 end through the Snap-Loc is best, it is replaceable and cheaper then new beads. A piece of wooden dowel shoved through the Snap-Loc makes it straight to better thread the food grade tubing through the beads. We use the ¾” with a ½” plastic tube inside. The Outside diameter of the ½ plastic tube is about 5/8″.
Here are the Snap-Loc pliers, extra beads, the clear tubing with fitting, and the custom mount.
We mounted the LTV on the back of his chair by bolting on a piece of 1/8″ x 1½” metal strap from the hardware store, and riveting the pole mount to it. That keeps it from swinging about and makes a nice neat package. We get power through a voltage reducer, they are available for about $125 to $150. It converts the chairs 25vdc to 13vdc and removes the burden of separate chargers and extra batteries. I just glued it to the bottom of the seat pan.
Here is a video explaining the self made system. The pre-built unit mentioned above is a nice solution if you can get it.
Feel free to share your setup or ask any questions you may have about our setup.