Myasthenia Gravis Support Group
Myasthenia gravis (MG) is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. The hallmark of myasthenia gravis is muscle weakness that increases during periods of activity and improves after periods of rest. Although myasthenia gravis may affect any voluntary muscle, muscles that control eye and eyelid movement, facial expression, and...
I've been laboring to breathe for almost a month, and becoming increasingly sick on Mestinon, so higher doses would not help, nor was extra prednisone doing the job. My neuro has sent me to the ER twice. The first time was a disaster, with none of the caregivers understanding what to do. I was sent home still in very bad shape and very disheartened. The second time, this Thursday, I got luckier, with a competent ER doc who sent me straight to ICU where I am now.
Thankfully, they have kept me off a ventilator by using aggressive steroids, the Bipap, and IVIG, which started last night, and will continue, one per day until I've had 5. Hallelujah for IVIG!
What they measure here that gives the most info for them is the NIF (negative inspiratory force). I asked my respiratory therapist if that can be measured by any sort of tool at home, and she wasn't sure. Does anyone know? The gadget doesn't look that complex. Is that the same thing as an oximeter? Can I buy one somewhere?
I'm still learning how to cope with the med folks, so many of whom are pretty clueless, like the first ER doc who asked me why I wasn't trying an asthma inhaler to fix my breathing problems......Argggh! Or, way before I was diagnosed, when my breathing problems sent me to the ER, and they were labeled a potential heart attack or a panic attack....
I'm feel like it helps to be well armed with both data and info if/when this happens again
Tamara
Thankfully, they have kept me off a ventilator by using aggressive steroids, the Bipap, and IVIG, which started last night, and will continue, one per day until I've had 5. Hallelujah for IVIG!
What they measure here that gives the most info for them is the NIF (negative inspiratory force). I asked my respiratory therapist if that can be measured by any sort of tool at home, and she wasn't sure. Does anyone know? The gadget doesn't look that complex. Is that the same thing as an oximeter? Can I buy one somewhere?
I'm still learning how to cope with the med folks, so many of whom are pretty clueless, like the first ER doc who asked me why I wasn't trying an asthma inhaler to fix my breathing problems......Argggh! Or, way before I was diagnosed, when my breathing problems sent me to the ER, and they were labeled a potential heart attack or a panic attack....
I'm feel like it helps to be well armed with both data and info if/when this happens again
Tamara
http://www.mspinc.com/airway-supplies/nif-pressure-meters
The meter requires tubing and a mouth piece and you should have a nose clip in place as well when using one.
BIPAP is a beautiful thing when you can't breathe and it allows you to rest while it does most of the work...I am so glad you have that now Tamara.....and if you can get in writing what they need to check the next time you are in an ER so you get proper care quickly.
Hope you continue to improve....and keep in touch with us....speedy recovery......Hope you are home soon! Hugs, Marie
I use a regular inspiratory spirometer to gauge my ability to take a deep breath like the ones you get when in the hospital and they want you to deep breathe. I know on a really good day I can get 2500 ml. But on a bad day it can go well below 1000.
So glad you got competent help this time. I think we all need a write up by our neuros or pulmonologists as to what to check when we go into an ER. NIF and VC are much better indicators of how we are doing then other measurements. I was once kept waiting three hours because my oxygen saturation had not fallen as yet, often one of the last things to go, and the triage nurse did not understand this and put everyone else before me even tho I could not hold my head up or take a deep breathe..
I am so sorry that you have had a rough time and I know how difficult it can be to get someone who knows and understands this diagnoses in the medical field.
The one thing I know for sure is that an oximeter is not the samething as a negative inspiratory force which is I remember right measure the amount of force you need to take a breath I am probably wrong on this.
And yes it is great to be well prepared when going to the E.R.
Hope you get feeling better and the respiratory therapist should be able to explain it a lot better and then ask to speak to a social worker who can find out if you can use one in the home.
Hope this helps.
Chuck
I got a chance to talk to a different respiratory therapist last night. He suggested that I can get a scrip before leaving here for a spirometer. My pulmonologist also told me he'd get me in a sleep apnea study so I can get a BIPAP at home. I guess they're easier to get approved for apnea than for MG?.......go figure.
GreenMarie, after my last goofy round at ER, I took the good advice from folks here on DS and wrote up a whole list of proper protocols for first responders and caregivers, a list of meds I should not take, etc. So when I got here this time, I asked for a respiratory therapist and made sure she was measuring something other than oxygen sat...and I let her read my notes! You all have helped me so much to learn how to be a better advocate for myself. My husband is learning and researching too, so maybe this will all be worthwhile.
And, my neuro has already initiated sending me to UT southwestern med center, which is the teaching hospital, for follow up care when I get out of here. My neurologist is good, but they're the MG experts. So hopefully, I will FINALLY get the care I've needed for several years now, while I was rolling around out there in the ERs without a diagnosis!
Big hug to you both!
Tamara
Wellness,
I was at my family doctor this past week. When I told him that shortness of breath was one of my newer symptoms, that I was having difficulty climbing stairs and get very winded, he said he would prescribe me an asthma inhaler. I said for him to not bother (it's not that kind of short of breath). I'm sure he didn't understand at all.
Hugz to you, better dayz coming! :)
Connie
Although I am not a medical practitioner, I will share my experience with you
Yes, it is possible to measure your own NIF at home and no, it is not like an oximeter (which measures your pulse and the oxygen saturation in your blood). The NIF is short for negative inspiratory forceit measures the maximum strength of your inhalation against an occluded airway. In MG, you can have great breathing values in all areas but have terrible diaphragm strength and this is where it will show up. It is also called MIP (or maximal inspiratory pressure). There is a maximal expiratory pressure (MEP) too but they perform that in a special booth and I have never had that tested in an emergency setting, at least at my hospital. They always test my NIF and sometimes my vital capacity (VC).
I have been checking my own NIF for over 4 years. I have been hospitalized numerous times for MG relapse and the respiratory therapists who perform the breathing tests rarely have experience with MG. Also, they throw out the single-patient-use NIFometer after we use them, so I asked to take it home and they had no objection to it.
http://mercurymed.com/catalogs/RDR_NIF_NIFometer.pdf (see page 2. It uses a tiny plastic mouthpiece that attaches to this)
It is not recommended to check your NIF often, as it fatigues the diaphragm when you have MG. So, when Im aware of breathing trouble, I check 2-3 x that day, far apart, to make sure I am not deteriorating. If the measurement keeps dropping or if it does not improve with mestinon, I go to the ER. Also, if I am having other symptoms, such as difficulty swallowing or inability to walk, coupled with breathing trouble, I will go in, even if the reading is not particularly low.
The NIFometer only goes up to -40cm H2O but a normal result is actually above -60. However, I find that if I am reaching to check my NIF, I am already below -40 anyway. My neuro wants me at the hospital my NIF falls below -30.
I hope this helps.debra
Hugs
Annette
Art Chick, thanks for the good details. I'll ck those links you and Marie sent. So is there more than one measurement variable for NIF? All I know is that I was at about 10-12 when I came in, but today hovering just around 20, which they seemed to think is much better. I tried to suck hard enough to stay off the Bipap today, but just like you said, that in itself wore me out. A few deep inhales exhausted me for the next hour.
Thanks again to each of you who posted here. I feel so fortunate to have found such an encouraging and knowledgeable group of new friends.
Tamara
There are NIFometers now that go up to -60 now but mine only goes to -40.
http://www.oemeyer.com/Media/Default/hospital/specialityareas/ems/respiratory-disposables/mercurymedical-nif.pdf
For me, mid-low -20's is a big problem and I am winded walking and maybe talking and eating. I am choking and likely not able to walk, except possible to the bathroom with a walker. Below -20 is a MAJOR emergency. I am in the hospital before it is below that.
They kept asking me to use the spirometer to strengthen my respiratory muscles. NOT GOOD. We have MG and we fatigue. You need to do what you can to move but any repetitive exercise is exhausting and dangerous when you are in ICU. Also, at my hospital they use plastic spirometers too (that they have to throw out). Ask them if they have one that you can take home with you.
I am happy to hear you are improving quickly! IVIg was a miracle for me in the beginning. It started to correct my breathing within 24-30 hours of the first dose and got better from there. Sooo wonderful when meds take care of what is most important first!!
Debra
Thanks for the explanation about the levels. I asked the respiratory therapist tonight what the lowest number they recorded was after I came upstairs from ER to ICU and she said 15. I had a 12 but they averaged that with a higher number since they were taking at least two readings each time. I wasn't ever as low as 10. Sorry for the misinformation....I think that's my hazy brain from all the commotion these last few days.
What she did tell me though, is that she wouldn't extubate without at least an 18 or 19, and only then if the person wasn't having a lot of oral secretions.....so by that, I understood how perilous it was for me to be at 15, and how blessed I am to be sitting up in bed tonight, and not strung out on a respirator!
Thank goodness for IVIg and for the internist doc who bypassed the hospital neurologist in order to get me on it right away!
Big hug to each of you for your care and kindness!
Tamara
Although I'm grateful for whatever help this hospital team sends my way, I have thought of you guys often, reminded that we are the ones who understand our conditions far better than the professionals. The respiratory therapist told me I should be doing breathing exercises....the Physical therapist showed me muscle strengthening exercises he wanted me to be doing in bed. Arrrgh!
And now, it's 5am, the respiratory therapist just woke me up for her visit and I have no more strength to speak above a raspy whisper. The
NIF was -22, but each breathe is taking more effort than it did yesterday, when I was closer to -20, and I'm pretty sure it's because I expended too much energy just trying to talk to all the folks they sent to "help." Can you imagine how weary I'd be if I'd been trying to do their exercises too?
I'm so thankful for you all, and the way you've helped me navigate the minefield of professional care!
Hoping for a stronger day for us all!
Tamara
My husband was remembering that they had me do occupational, physical, and speech therapy on the same morning. I couldnt even change my gown or wash up after that because I was so exhausted that I was winded speaking again and sweating and the docs hadnt even rounded yet.
If you are fatiguing or regressing on your NIF, STOP. You are overdoing it. I would say skip the hundred and one good ideas and workouts they have set up for you. Take the opportunity to have your husband educate them on your condition. Let them know that when you have extra strength and energy stores, you will add their exercises into your day but now is not the time.
You sound good, Tamara. I think you have got this!
Debra