7.5 month old Nemaline myopathy - failure of NVS?

Please use this community for general discussions about Nemaline Myopathy.
User avatar
curiousgeorge
Level II Member
Level II Member
Posts: 66
Joined: Mon Nov 23, 2015 4:06 pm
City: Newark
State or Province: NJ
Country: USA

7.5 month old Nemaline myopathy - failure of NVS?

Postby curiousgeorge » Sun Nov 13, 2016 2:11 pm

We wanted to ask you for your advice about our son's situation. He is 7.5 months old and has Nemaline myopathy (neb-related).

He is now in the PICU because in the last 2 weeks, he was struggling with saturation drops while being asleep into the 80s, sometimes lower, and it took some minutes to recover by stimulating him. He has been 22 hours per day on the high flow with 15 liter per minute and was managing great until then.

Due to these new saturation drops, the physicians say that he needs noninvasive ventilation and gave him a nasal mask with pressure-support ventilation with now 17/7 and 40 frequency for the night and the times he's asleep in the day. The machine lets him choose whether he wants to trigger breaths or let the machine take over, and he lets it take over. (The physician didn't want to use bipap because then he still need to breathe by himself, but he should rather let the machine take over in order to be able to fully rest.)

The problem is, that he still -even with the mask- very often gets saturation drops and they say that it's because his throat muscles and tongue are so hypotonic and because his mouth is always open, only a tracheotomy would help. The pressure wouldn't get to the lungs, but get lost through the mouth. This happens always when he sleeps very deeply.

Now we are quite worried and shocked about the situation and wanted to ask your advice, whether the hypotonic throat/tongue and open mouth are an impediment for NVS? (The physicians don't want to use a nasal+mouth mask because of the fear of him throwing up and aspirating.)

Thank you very much in advance!

(Asked by a parent)

User avatar
bachjr
Medical Director
Medical Director
Posts: 135
Joined: Wed Nov 11, 2015 3:07 pm
City: Newark
State or Province: NJ
Country: USA

Re: 7.5 month old Nemaline myopathy - failure of NVS?

Postby bachjr » Sun Nov 13, 2016 2:13 pm

curiousgeorge wrote:We wanted to ask you for your advice about our son's situation. He is 7.5 months old and has Nemaline myopathy (neb-related).

He is now in the PICU because in the last 2 weeks, he was struggling with saturation drops while being asleep into the 80s, sometimes lower, and it took some minutes to recover by stimulating him. He has been 22 hours per day on the high flow with 15 liter per minute and was managing great until then.

Due to these new saturation drops, the physicians say that he needs noninvasive ventilation and gave him a nasal mask with pressure-support ventilation with now 17/7 and 40 frequency for the night and the times he's asleep in the day. The machine lets him choose whether he wants to trigger breaths or let the machine take over, and he lets it take over. (The physician didn't want to use bipap because then he still need to breathe by himself, but he should rather let the machine take over in order to be able to fully rest.)

The problem is, that he still -even with the mask- very often gets saturation drops and they say that it's because his throat muscles and tongue are so hypotonic and because his mouth is always open, only a tracheotomy would help. The pressure wouldn't get to the lungs, but get lost through the mouth. This happens always when he sleeps very deeply.

Now we are quite worried and shocked about the situation and wanted to ask your advice, whether the hypotonic throat/tongue and open mouth are an impediment for NVS? (The physicians don't want to use a nasal+mouth mask because of the fear of him throwing up and aspirating.)

Thank you very much in advance!

(Asked by a parent)

The settings and the machine are not good. He should be using an LTV or Trilogy but NOT on BiPAP setting. He should be on pressure A/C about 20 cm H2O with a similar back-up rate. The 17-7 is definitely not adequate. Also, the CoughAssist needs to be used appropriately. You should have the doctors call me after the learn by reading what to do. I have emailed you papers also. You need to read these.

JB
John R. Bach MD
Medical Director, VentilaMed BreatheNVS
Medical Director, Center for Ventilator Management Alternatives
Professor of Physical Medicine and Rehabilitation, Professor of Neurology, Rutgers New Jersey Medical School


Return to “Nemaline Myopathy”

Who is online

Users browsing this forum: No registered users and 1 guest