[AnnieB3]

Hi Annie

I was interested to see your comments on PI Max & PE max. I am 47 & have had mild lupus for many years. In 2012, the condition seemed different in many ways but most disablingly weakness in arms, legs, torso. Aspiration at night and then most recently respiratory problems & very slight intermittent ptosis. PI max is 18.4 cms and PE Max 23 cms. 6 months ago they were 32 cms & 51 cms h20. Sniff test indicates that I have a weak diaphragm. I am anti achr negative and my SFEMG was not supportive of MG although the RNS showed 'decrement which seems a bit artefactual' which I think means operator error. I don't feel convinced that I have MG but I'm pretty sure that there is more than normal fatigability in my chest/arm muscles.

I just don't know whether or not to bash my way through the uncertain doctors or sit it out a bit longer to see if things settle or worsen. My resp doc thinks there probably is a neurological cause but doesn't know what so is sitting on the fence. Your thoughts would be really helpful.

Many thanks, Clare

Clare, Welcome! I take it that no one has taken your situation seriously?

Your MIP is very bad. It is usually shown in a negative, such as -18.4. Do you live in the US? I'm only asking because ranges of tests can be different in other countries. Your MEP isn't great either.

In the US, if you have a MIP of -18.4, it's time to have a BiPap or be intubated! And since breathing is worse while sleeping, I have to wonder if you are getting enough oxygen!!! Has anyone even suggested an overnight oximetry for you?

I know a number of people who have both lupus and MG. It's not fun, but it happens. Would you mind sharing what drugs you're on for lupus? Some drugs for lupus aren't good for MG.

I'm frankly appalled at the lack of concern over your breathing stats. They should be doing that overnight oximetry, doing an arterial blood gas, redoing the breathing tests with a before without Mestinon and an after with Mestinon (or Tensilon).

They should redo the RNS AND do a SFEMG. It's pretty dangerous to assume that any decrement is "artifact!"

They should rerun the AChR antibody tests and send them to Mayo. They should do the MuSK test as well. AND they should check you out for LEMS. Antibody tests fluctuate and, depending upon what meds you are on for lupus, they might look negative when, in fact, they're not!

Also, has anyone done a simple chest x-ray to make sure that you don't have aspiration pneumonia?

Let me be blunt. If you have MG, and don't receive proper treatment, you can stop breathing and die. Do you think that would get a neurologist's attention? Good grief. I'm not saying that to scare you! But you need help right away. That level of poor breathing is NOT NORMAL and a cause needs to be determined right away.

And I'm not so sure you shouldn't be IN the hospital getting oxygen and being monitored! A lack of oxygen is very hard on the brain, heart, kidneys, and other tissues.

Have you seen a neurologist? If not, why not?! Your pulmonologist needs to be working with a neurologist to figure this out. A MG expert would be best.

Situations such as yours makes me wonder why doctors even went to school in the first place. Breathing well is essential to life, and to drop the ball like this is not only stupid, but cruel.

What else can we do to help?

If you can't breathe well, you need to dial 911. The same goes for not swallowing or moving well. A MG crisis is serious, and being in a hospital immediately is what you should do!

I hope you can find someone to help you!!!

Annie

[juliejayne]

Have you seen a neurologist? If not, why not?! Your pulmonologist needs to be working with a neurologist to figure this out. A MG expert would be best.

Situations such as yours makes me wonder why doctors even went to school in the first place. Breathing well is essential to life, and to drop the ball like this is not only stupid, but cruel.

Annie, I just wish Neurologists were all given a course of "listening to Annie", it would certainly help them.

I was just dismissed by my Neuro. At an earlier meeting she was concerned at my shallow breathing, so sent me to a Mensendiek therapist. Now at this last meeting my breathing was slightly improved as the weather was cooler, but not much. The therapist had said simply that the breathing issue was a symptom not the cause. And the final word of the Neuro... it is not in my field, go back and speak to your GP!

[Clareb]

Hi Annie, thank you so much for your detailed and emphatic reply. You've confirmed my concerns totally.

Firstly, I live in the UK. The benefits of the NHS are immense and we are very lucky but there are budget-related disadvantages as you can imagine with anything that is free. I take 10mgs prednisolone (reducing currently to see if this is contributing to weakness), Mycophenolate 1g daily, Hydroxychloroquine, AdCal D3, Omeprazole. My Lupus on its own is not too much of a problem and is an integral part of my life.

My spirometry is not too bad, my airways are narrowed for some reason despite never having smoked, my PI Max at 32 was considered moderately weak and the PE Max was 'mild' at 51. My resp consultant merely stated the figures in my recent letter then signed out with 'Yours Sincerely'!! No follow up, nothing . .

The docs are quick to consider the cause to be my slightly low vitamin levels however, these levels have increased whilst my resp tests have deteriorated. I recently emailed my results to the British Lung Foundation Helpline for their comments and they rang straight back & were emphatic that I should push for referral to a regional centre for resp medicine so I realised they must be quite low. My resting O2 sat is good and I breathe ok at night so long as I lie in the recovery position!

You have given me plenty of fuel for my fire when I see my rheumatologist in a couple of weeks time. Do you have resp problems with your MG? D you find shivering wears your muscles out? I suffer badly with cold intolerance and shivering takes everything out of me, scarily so.

Once again, thank you so much. I have much more determination now.

Keep well. Clare

[Kitt]

Welcome Clareb.

[AnnieB3]

Clare, I wondered if you lived in the UK. Rach, who is on here as well, lives there, too.

I "get" the NHS. I do appreciate the constraints of socialized medicine. HOWEVER, I cannot and do NOT appreciate the a physician's CHOICE to not THINK and ACT when they have all of the power to do so, especially when someone's life is in imminent danger—as I believe your is. There are no constraints on thinking and caring.

I hope a neurologist will understand that any steroid will make an antibody test look normal when it might otherwise be positive. It's a steroid's job to make those antibodies disappear!

I would caution you about reducing your steroid! It can cause an adrenal crisis, where you have severe fatigue, sweats, and other symptoms such as cognitive dysfunction. And if you have MG, reducing it might bring on an MG crisis.

MIP and MEP don't reflect narrowed airways, but reflect a neuromuscular cause for poor breathing. How did they conclude that you had narrowed airways? Do you have asthma, too?

I've had low B12 and D. They did NOT cause my breathing to tank. Oy vey. B12 is necessary for the production of acetylcholine and might have a slight effect on muscular weakness. If you're deficient in B12, you can get Jarrow Forumula methylcobalamin on the Amazon UK site.

Unless someone is monitoring your O2 saturation while sleeping, there is no way to know if it is tanking. My resting O2 after three days during my MG crisis was 94%, however, my O2 while sleeping was 66%!!! Not good. You need that assessed right away.

Is the "recovery position" sitting up? It might help to sleep in a recliner, if you have one.

If you are shivering, you are probably hypoxic (not enough oxygen). Unless it's a blood sugar issue. Is that under control? And I wouldn't rule out an issue with drugs or an interaction of them.

You need more specialists: Pulmonology, neurology, and possibly endocrinology.

I'm really sorry you're going through so much. Lupus is hard enough to handle. Just work with your rheumy to get the care you need, but please don't rule out heading to the ER/hospital.


JJ, That's sweet of you, but tell that to any of the neurologists I've seen. I doubt they'd share that opinion.

Annie

[Clareb]

Hi Annie

Annie, thank you once again for your reply. Sometimes I think we lack the courage of our convictions to demand a pro-active approach from our Doctors. Also, you get used to functioning at a sub-optimal level after a while and become accustomed to new physical constraints. I have just been sent an appt to see my Rheumatologist on Saturday (?) and plan to take a list of points to discuss. I think I probably don't fit the text book picture of an MG sufferer hence the Dr's reticence but I also have Lupus and have had for years with no antibodies whatsoever!

The shivering is strange, no-one else seems to get this. It's not hypoxia nor low blood sugar etc, it starts with my cold intolerance and means that I start shivering at temperatures that wouldn't bother other people. If I can't warm myself up quickly ie if I am out watching one of my children playing rugby then my muscles will quickly exhaust causing my body to turn to jelly and make my already tired chest to be sooo much worse as the day goes on. My O2 sats are always good, don't know how I manage to keep them so high - probably because I was so very fit before things started to nose-dive.

The recovery position that I mentioned for sleep is a certain way the emergency services position you if they want to keep you flat whilst also keeping airways open etc. it's kind of on your side but tipped slightly forward. It is literally the only way for me to breathe by the time I am tired and in bed.

I love the NHS and have had 2 desperately ill babies in the past and they have had hundreds of thousands of pounds spent on them to keep them alive. However, for less immediate needs, the process can be very slow, inefficient driven by keeping costs down. I have tried private medicine with a resp consultant and that was worse,so much worse. What a waste of money.

I will let you know how I get on at my next appt on the 13th!

Thanks again Annie. Take care. Clare