Hi there sounds like you've been thru the ringer and back. I can totally relate with you though. I've had lupus since I was 23 yrs old, I turned 50 this past February. It took my doctors 13 years before the "officially" said yep to lupus.
I have lupus in my lungs, heart, joints, skin, bowels stomach. I have RA, Fibro, Pulmonary Hypertension, T-cell LGL Leukemia, and recently added vasculitis to my list. I've got a totally obstructed SVC and it certainly doesn't help my breathing issues. I have fibrosis in the lungs and most likely the vasculitis is in the lungs. My breathing is so bad the pulmonary docs are unwilling to do a lung biopsy on me. I've been intubated numerous times due to pneumonia. I like you have NO immune system and when I say NO I mean NO. I have zero B-cells and my T4 count is only 4. That is lower than your average AIDS patients.
I've been on high dose steroids for almost 15 years and I've got osteoporosis so bad I had 15 rib fractures in 2014. I broke two ribs in February, just bending down to feed my cat. I also, fractured my pelvis and hip a few weeks later in February they think. I'm still having tons of pain as much as I did when they found the fractures. Oh, the steroids have affected my tendons and ligaments too. They tear and rupture all the time.
As for what do I do for care, I'm on oxygen 24/7, I'm on prednisone, plaquenil, MTX at very high doses, and I take an additional 33 medications daily. That doesn't include my pain meds for break thru pain, nor my nausea medications as those are PRN. I receive blood transfusions frequently and recently started IVIG infusions. Hoping that it will help the vasculitis. I sleep when fatigued and I do not travel anywhere to far from home. I'm always worried about catching something. I had a slight cough on Feb 28th and by 10pm on Mar 2nd, I was in the ICU, being put on a bipap machine, with a Doctor hovering nearby waiting to put me on a vent.
You asked what helps, well for me the best thing for me is HIGH dose prednisone. However, the prednisone is killing me with side effects. The increase in my oxygen has helped me. The Viagra that I take to treat my pulmonary hypertension has helped some. Going to pulmonary rehab helps and sleeping with my head elevated helps. Other than that nothing has worked for me. I've taken every single medication for the treatment of lupus/RA, except gold infusions. Nothing has stopped my progression, I believe that my Doctors taking so long to treat me has caused me to be so sick.
Now that I have the T-cell LGL Leukemia, I know more of what my fate holds. There is no cure for the type of leukemia I have. It's one that goes with autoimmune diseases. According to my oncologist most patients live 5-8 years, some 10 years. It's usually an infection that kills us and NOT the leukemia. At the present moment mine is quiet so we treat it with MTX except at high doses. The MTX is also used for the lupus/RA so it's like handling several things at once. I recently had a fecal transplant, due to the fact I couldn't clear the C-diff I seem to get all the time.
I turn purple when I do anything strenuous and found out that is related to my SVC being totally blocked. I was just told by the vascular surgeon that I will eventually be purple 24/7. I'm hoarse and that's another sign of the problem. I'm on blood thinners as I've got issues with blood clots, so I look like on huge walking bruise.
I have a great therapist who lets me rant and rave. I've got a wonderful support team here at HW. I joined in 2005 when I had my colon removed. This is a big family to me and everyone is supportive and helpful and I don't know what I'd do w/o them. Do I get discouraged, you bet I do, it's hard not to focus on that I'm going to get sicker and die. I have a beautiful daughter who will be graduating from college next Saturday the 30th. She's what keeps me going I guess. She has no memory of me ever being well, matter a fact she found me 4 days prior to her high school graduation unresponsive. I ended up on a vent and fighting for my life. Missed her graduation and was on the vent for 5 days. When she and my hubby arrived at the ER the Doctor told them I was very ill and he wasn't even sure I'd live thru the night. Guess I'm refusing to die on her if I can help it in anyway. My hubby says, "I'm not dying on his watch", yep he's prior military and he's like a drill instructor when it comes to my staying alive.
I had the nerve to mention to him last week that I was sick of all the appointments, pills, surgeries, admissions to the hospital. This missed holidays and important events in my life. That my quality of life sucked and that I was going to just stop taking my medications and just crawl into bed and wait till I died. Then I commented that I guess I couldn't do that because of him and my daughter. He replied to me "Oh, how selfish of us to want you around".
I fight to go on because I feel I owe it to my daughter and to the man who has been married to me almost 30 years. We will celebrate our 30th in June. I have many days where my pain levels are thru the roof, and I can't breath and I feel so ugly and unattractive. I have days when I think about taking my life and just bring this dark hole I live in to an end. What stops me I don't know but I have a very stubborn nature and I guess I refuse to let this darn disease win. Even though I spend almost every day at a solid 7 out 10 pain, I still push myself to continue to go on.
I'd suggest that if you're breathing is really bad that you discuss with your pulmonary folks. Ask the pulmonary doctor to do a six minute walk test with you. If you are destating while walking during the test they can prescribe oxygen for you, if they feel you require it. Once you start getting the oxygen you'll feel a lot better. Have you been checked for sleep apnea? If not, that would be my next suggestion to you. I have both obstructive and central sleep apnea. With the obstructive your body awakens you and you cough or whatever, with central sleep apnea your brain doesn't realize you've stopped breathing and unless someone is around to awaken you or something happens to make you wake up, you don't wake up and your CO2 levels begin to rise and you eventually become unresponsive.
As for you immune system I'd suggest you see an immunologist and see if they can do anything to help. I'm currently in a research study at NIH, due to the fact that I have no B-cells and my T4 count is so low. They became interested in me when they heard I had issues with warts on my hands and fingers actually I had/have them all over my body. The warts became so bad that I had to do radiation to get rid of them. I now suffer from radiation dermatitis all the time.
Anyway, the infectious disease folks have put me in a research study, because they believe I have an immune problem in addition to my autoimmune issue.
I'd suggest that you discuss a possible change in your mediation for your MCTD and see if something new may help. You already have a therapist and I'd say continue on seeing them. Come here and vent, ask questions, or whatever when you need help. We all get what you're going thru. I guess that's all I can offer to you for now. Don't give up, it sounds like you've handled things very well and that you're getting tired of this, we all do, but rolling over and giving up isn't the right thing either.
If you feel that you need a new set of eyes to look at you, then ask for that. There are several medical centers that treat lupus patients and have some of the best Rheumy's in the nation there. Remember, to just take each day as it comes and when you feel totally overwhelmed, then take things just a minute at a time.
If I can be of any help with any questions for you, please feel free to ask me. You can also contact me via email, mine is listed in my profile. I hope that things improve and that you find the support of the folks here helpful. Hang in there we all understand what you're going thru.
DX- RA, Lupus, Fibro, LGL Leukemia, Obstructive & Central Sleep Apnea, Pulmonary Hypertension, Bells Palsy, Gastroparsis, Blood Clots, Glaucoma, Chronic Pericarditis & Pleurisy, Severe Anemia. Way to many medications to list.