rubytuesday

I was not diagnosed with POTS when pregnant but I weighed 6 lbs and was preemie when I was born. First child born 5 weeks early, weighed 6'1" (35 now and only recently found to have congenital coarctation of aorta and dilated ascending aorta--I have EDS). He went down to 5 lbs 4 oz and had to be fed by tube (couldn't suck) and had blood infection and was in incubator and bili lights. 2nd pregnancy ended in spontaneous abortion.

I get my IV gamma globulin at a cancer center's infusion center. It's a little depressing. A volunteer elderly cancer survivor visited me before the nurse started my 5 hour drip and chatted a few minutes and gave us a magazine--'What caused my Cancer'. It was cold in there and the recliners hard and slick--I didn't have enough upper body weight to keep the darn thing reclined. They were short on blankets and the lunch was surprisingly not only terrible but not healthy. I used my full length down coat for a blanket. And I will know to take snacks or send hubby to the cafe. If all keeps going well, I'll only have to go back for 2 more. I can do that--then we're going to have Home Care nurse show me how to give it myself in the comfort of my home. (I would be inserting big smiley face right here but sometimes when I try, the computer freezes up). Glad things went well. I was wondering how things were going to affect my dysautonomia but they used saline (only not like hydration rate--just before the globulin, to drip in between drips of globulin to keep the vein open and then to flush the vein after the globulin). Best wishes to you.

I had chicken pox like crazy (still have some little lumps/scars where I scratched too much). Got them when about 7. I was caring for little niece overnight when she broke out with them (and I was pregnant at the time). She was about 6. Then DS got them all over the place when he was 6 (I was room mother for his valentine's party at school that he had to miss). I am immunocompromised and now on gamma globulin. I cannot take shingles vaccine. I surely pray I don't get shingles as they can be extremely painful, debilitating and in some cases, very serious.

I see Dr. Charity Fox at James Cancer Center at (OSU--Ohio) and Dr. Richard Groger (Kettering, OH). The later doesn't switch you over to sub-q and infuses in 2 hours. I felt more comfortable with second opinion of the first mentioned who transfuses first 3, titrating rate slowly and if tolerated, switching over to home sub q.

So I have felt nothing more than a little sniffles later in week. And immunologist got approval to switch to home sub q Ig (after my third IV transfusion). Insurance denied at first but immunologist sent off appeal letter this morning and it was approved by this afternoon. I cannot help but wonder what life would have been like had this immunodeficiency been pursued/found years ago. But am thankful to have insight to take labs and seek second opinion so to get to do in home setting and health care coverage with pharmaceutical benefits.

I know how you feel, I do. I cannot say that DH is not supportive as far as doing grocery shopping (he'd rather go alone than have to try to shop and watch/worry about me going down). He sometimes is good to help as far as carrying laundry or helping put it away or 1/2 way making the bed (which I re-do). But other times if he hears the word laundry, he cuts out. Sometimes he'll fix light dinner (fry an egg or bologna or microwave or soup) and do dishes--other time, leaves dishes in sink. He takes me to the long trips to city specialists (hates to do that). When I had my first IV Ig transfusion, the doctor ordered it at a very slow rate, so it took 5 hours (plus 2 hours drive there and 2 hours back). He kept trying to get the nurse to speed up the rate since I was not having any reaction. We knew before we went it would be all day. He took me back to hemeoncologist at another campus site and told nurses and doctor yes it was my first trip there and it would be the last (construction--could not get to the building unless you could find parking in garage 2 city blocks away, walk in pouring rain to get into hospital only to be directed to the far end wing of hospital to get to hemeoncology unit). He even drove wrong way down 1 way street to drop me off at door while he parked in space he was not even sure was for that hospital building or not. When I bring up anything doctor said, he disagrees (I cannot believe he interprets things totally different, but then he is not in medical field). If I tell him about near syncope--he scolds me, tells me to leave things alone, that I shouldn't have tried doing it (but things don't get done if I don't do it). If I discuss health, he shuts down--gets sharp with me--'I feel bad too but I don't go on and on and on about it'. Which I don't but I'm usually alone all day with no one to talk to. If I remind him of appointment (and I try to do that a week ahead of time and intermittently) that he needs to take me to (and many times he will ask and I will tell him), still the night before he'll ask again 'where is it we're going. . .which one is that. . .what time is it. . .'. He doesn't want to look at directions on mapquest or the campus website, (so I write them down) but night before or on the way he'll ask me where it is. I my mind's eye (and I could be wrong), with these specialists and being told certain conditions (EDS, Delta Granule Storeage Pool Deficiency) are rare, and this new Vocal Cord Dysfunction (upper airway obstruction), and having to be tranfused with platelets before even having a tooth extracted, and being transfused with human antibodies for life, taking medications for osteomyelitis for life, I think it overwhelms him. In some way, I wonder if he is afraid of something happening to me (because I worry about something happening to him). So when he gets home at the end of the day, he's had the socialization that I haven't. He's tired from working and just wants to take the remote, lay back in recliner, snack and then maybe fall asleep. He suffers from depression and just weaned himself off his med because the co-pay with this year's insurance plan went up. He gets mean off antidepressant--I mean to waitresses, cashiere's, everybody (and he's a really good-hearted guy--good wit/humor) so I am trying to prepare myself psychologically to handle this til he takes the time to see his doctor and get on some generic brand. My mother 'tries' to be supportive but her life is literally consumed with other siblings, nieces/nephew paracites. Our DS seems to care less--rarely checks on me unless DH tells him to when DH travels. I have a sister 2 states away who lets me e-mail her about appointments (I save the sent e-mails so I can refer to what the doctor told me at the visit for the times/things I forget). She's super. And then of course all the people here are so very supportive and good to let us get it out when we need to. I tell DH (in front of docs/nurses) that he is my rock. I try to stroke his ego and give him support in that way. When we're both sick, we'll have a pity party and joke that 'they'll just find us dead in here some day'. I just try to make a conscious effort not to talk about how I feel. I think when I go from IV to the sub q Ig at home, by asking him to help me by sticking the needles (sites must be rotated) in places I cannot reach, for my infusion, he will feel like he is doing something that is helping me. Right now I think he feels helpless deep down inside. And men are supposed to be heroes.

Sending good vibes your way.

I have breathing issues and small pericardial effusion. PCP (internal med) and cardiologist say it is not concerning. I also was found to have Vocal Cord Dysfunction that makes it hard to sort out if my difficulty breathing and shortness of breath is stemming from exertion, vocal cord spasms/being shut when they should be opened, or from the dysautonomia. I also get pains in left chest (with myocardial events ruled out). You can get lightheaded/faint if you hyperventilate and you can hyperventilate without being aware (i.e. with feeling of anxiousness from difficulty breathing). I also have asthma. I try to focus on breathing slowly (sometimes deeply, sometimes shallow) in through nose and exhale slowly and gently through lips as if gently blowing out a candle. It is supposed to relax the vocal cords. You can also exhale using short shallow puffs and inhale the same way. Sometimes, I have to do the inhalations and exhalations in the same fashion through my mouth. When I cough, it is a dry cough. I keep water at bedside as meds and my membranes are dry. It helps with breathing/swallowing at night. I do not run low grade fever. Low grade fever/pain on inspiration could be symptomatic of anything from pulmonary embolism to respiratory infection/inflamation. Only a physician/testing would be able to sort that out.

I rode standard bike 10 miles every day (before I got hit by car, I would ride 50 miles, then when fibromyalgia hit, reduced activity to 3 miles on treadmil, 100 sit ups, wt. lifting, stationary bike or rowing and weight lifting regime every day). With the POTS I was reduced to recumbent stationary bike (and that was with working 55-70 hour work week).

I have this problem and feeling kind of tight around my throat, like I can't get in good air. I'd mentioned it to my PCP in 2007. She ran pulmonary function tests at local hospital and pulmonologist interpretted it as my asthma being out of control. (Strange I'd thought as my asthma never behaved that way before). So when I was at a new referral to immunologist/allergist/pulmon., she ran another PFT with loop and showed me the results--Vocal Cord Dysfunction (I also have EDS). Had to go for scope--everything very very dry (which was why otolaryngologist thought explained the pills sticking and trouble swallowing). He started me on Evoxac (used for sjorgrens--strange as this fall I had to start restasis for dry eye and have the vaginal dryness). He explained some meds can make you very dry. The vocal cords were very red/inflamed/swollen. It is also referred to as upper airway obstruction. When I went back to this immunologist, I took a copy of the 2007 test/interpretation (as it sure looked similar to the one she ran to me). She said that that was VCD not asthma out of control (which was why the Rx's that pulmonologist kept adding made things worse--til I discharged him). Things that trigger asthma can trigger the vocal cords to go into spasms as well. When one breathes, the vocal cords should be open but in VCD they close (and mine close tight). I am doing speech therapy right now to teach me breathing exercises and rescue breathing. Immunologist and otolaryngologist tell me a lot of doctors (including ER) miss it and treat it as asthma. Speech therapist explained to me that with the swelling and spasms, things could sit atop the closed vocal cords when I try to swallow (I'd just aspirated a Midodrine and Fish Oil--thank God after many efforts and thinking this was it, leaning over/trying any air to cough the fish oil dislodged). I sure hadn't anticipated going to immunologist for immunodeficiencies to come out with that added diagnosis. The therapist seemed surprised at how quickly I picked up on the exercises but I think its from how I learned to help breathe from living with this all this time. GERD and/or post nasal drip are other things that cause irritation/spasms as well as things like cold air, exercise, cold/hot drinks, acidic fruits. Just thought I would mention this for consideration.

I am so happy for you. You are one tough cookie. I've run low BP (low normal) all my life so I don't know if I can get a fix on orthostatic hypotension (taking Midodrine and pushing fluids). Dr. is supposed to be adding something to get me hypertensive but I'm patiently waiting as since it is colder weather it hasn't been as much of a problem as when I venture outdoors to get some of that sunshine. I really, really miss my bike. The recumbent stationary bike is good to have but it is nothing like feeling the wind and sun. I have read that some research has shown IV Ig to improve dysautonomia (although I think it is considered 'experimental' by insurance payors). I am taking that now for PIDD/CViD. And I'm taking isosorb to boost calories/nutrition. You're truly an inspiration. Thank you for sharing and giving us hope.

I never fainted with 2 TTT but cardiac physiatrist was convinced it was POTS (guess by the VS) and started me on florinef (that did not stop my near syncope spells or BP plunges). I never started fainting until after 2 1/2 years of the near syncope. I have fainted 4 times last year--and I think what was going to be 5 but DH caught me going into a face dive into our concrete driveway that probably brought me to right away. Now I am staying more in OH and cardiac specialist in dysautonomia told me that with my ANS failing me that I will have fewer warning signs of faint.

So it is day 3 now. No adverse side effects, no need for tylenol or benadryl. Did 2 loads of laundry, changed sheets, did work on Council business coming up, updated spread sheet for expenses for taxes--a good day. Fizzled out by afternoon. So am jumping off here and spanking this dust, vacuuming and mopping floors while I have am still feeling good. This morning the vocal cord dysfunction does not seem as bothersome but things may change as I get things stirred up. Have another day at James tomorrow--seeing hemeoncologist for the Delta Granule Storage Pool Deficiency and then speech therapist for the VCD (says she's going to hook me up to heart monitor/pulse ox to check breathing on on stationary bike). I hope she uses recumbent stationary bike as that is what heart specialist told me to use with the dysautonomia. BP has still been hanging around low 90s/low 60s. Well, I fizzled out after all the dusting and vacuuming and cleaning bathrooms. I did have to stop once (stooping, bending, reaching sent by BP south). By around 1:15, I called it done. Hoping to tackle my floors on Fri. But I look at this as huge accomplishment (even though I didn't get it all done). Got sudden surge of fatigue and just had to sleep and rest.

I have PIDD/CVID, my IgG total is ok but the subclasses are low IgG 2, IgG3, IgA, T cell count is a little low, T helper cell count is low, and I do not respond by making antibodies to pnuemonia vaccine challenge (and I'd had 2 pneumonia vaccines within 2 1/2 years). These results have been consistent over the course of at least 3 studies in the Ig's and subsets and compliments and challenge tests. I have an Infectious Disease doctor who treats me for osteomyelitis (one organism is usually only seen in severly immunocompromised people--i.e. HIV, TB, chemo patients, cancers) which led him to explore deeper into my immune system. He told me I needed Ig but since I had been referred by a hemeoncologist I had to see to his collegue (immunologist/allergist/pulmon), she reviewed tests, said same thing, clearly I needed this (ran more tests with more of same results). And she set things in motion. My ID doc had got it set up after I'd seen this immunologist but I didn't want to use him even tho he's an hour's drive as opposed to 2 hours to university's infusion center, because he doesn't switch patients over to home sub q (maybe he thinks it's too risky, I don't know). But my immunologist plans to switch after 3 mos of IV Ig. I at least want to try it at home as I could do it the time of day/evening that worked best for me and work around appointments I may have. I do produce antibodies, but just not enough to protect me from infection. Unfortunately, they do not have anything they can administer to give me IgA. Insurance carriers use different criteria for eligibility. In my case, my immunologist talked to my insurance while I was there (I also had called and documented--and had the representative document our conversation). My plan did not require pre-cert for the IV Ig at the infusion center (in-network) and was covered under my major medical. I still have to pay deductibles and co-pays up to my max out of pocket. I am not sure how the home sub-q is considered, but I have it documented that the insurance told me only the Home Health needed pre-certed.

Day 2 after my infusion--still knocking wood--have felt pretty good (surprisingly so quickly) in the morning, with more energy. Yesterday I did 2 loads of laundry and changed sheets and was going to go with DH + DGD to get her a doll she'd been wanting (but something came up). As day drew on, became more and more fatigued and throat sore a bit. But get this (and I apologize as gross)...in Dec. when immuno looked into one ear--waxy. I use q tip very gently as if I go the slightest too deep I get ear aches. I also use low blow dryer after showering on the ears after towel drying. Last night 2 inch long bloody gunk surfaced from the ear that I could literally pull out in a thick wide piece. I did not notice any change as far as hearing but I'd never in my life had anything like that. It's usually my other ear that hurts, but this was mixed with blood. I haven't had to take any tylenol or anything else. I'm going to tackle dusting, vacuuming and mopping floor this morning before the surge drops. I am still pushing fluids like crazy and am hoping this is helping. The thing is what they gave me (nurse said was Hizentra but must have been the privagen my immuno mentioned earlier) was 10% concentration. This Hizentra is much thicker and more concentrated, 20% for when I get to switch to home subq. That may be where I could run into higher chance of side effects. They waited 1 full hour after my pre-treat and would infuse very slowly over 5 hours. That also probably helped to reduce risks of side effects. But you never know as each vial you get has different people's antibodies. Thanks for the support. I hope yours go better. I was wondering if I should take tylenol or benadryl the next day (received absolutely no 'discharge instructions' per se, rather than the sheet that tells you what to watch for/call doc for/go to ER for). Sure hope others will go as well. I imagine they will up the rate a little with my next infusion.

Jen, Many of those issues with the blood counts and kidney function , headache, herpes, hives, lips swelling, difficulty breathing (had you not had those prior to) could definitely be from the Ig product (according to packaging insert). So sorry you experienced that. You never know with each vial given since each vial contains components from different individuals. I hope they figure things out for you.

Well, Jen. What I have learned, what they gave me IV is only a 10% concentration of antibodies. The plan is to switch me after 3 IV Ig monthy doses to subq Hizentra, a 20% concentration, at home. I suspect this may be where I'll run into the most problem with but am keeping my fingers crossed. Since the Hizentra is more concentrated, you're getting a lot more antibodies and the solution is much thicker which is more traumatizing to the tissue. They infused my IV Ig over 5 hours at a very slow rate so to try to minimize/avoid reactions/side effects (waiting 1 hour after pre-treating me before starting the drip). I wish you all the best.

Just an update that I had my first IV Ig (Privagen I think although the nurse said Hizentra, and Hizentra comes in subq not IV solution) for the PIDD/CVID today. With the drive, total time was 9 hours. Transfused the Ig very slow rate after pre-med with benadryl and acetamenophen. A little after about 1/2 way thru I felt a little light headed/chest pain (but that's not atypical for me). BP was 92/62 before we left and got as high as 102/44. The systolic mostly hung in low 50s and diastolic in low 90s. So far so good for me. Hope the next 2 go off as well (and hoping don't start feeling fluish/migrainish tomorrow). Am planning to lay low tomorrow. I was having some shortness of breath/difficulty breathing/tight throat but was thinking it was irritation with the Vocal Cord Dysfunction from the cold room and the terrible spicey lunch they served (also high sodium and caffinated drink--when you need to overhydrate with the Ig).

Floor nurse in hospital, moving up ladder with masters in Health Service administration in managerial level of hospital 25 years. (Fibro, hypersonomia and CFS dx'd around 1998). Dysautonomia, POTS, Orthostatic hypotension dx'd in 2011.

I wasn't a 'sweater' unless vigorous exercise til the POTS. Walked block and a half w/ DGS this summer one morning to yard sale. A little old lady, a teenage boy, his mom, DGS (7), all others cool as cucumber and I suddenly and dropping pools of sweat all over the lady's table as I was paying her and she bagging things up--of course when sweat like that--includes my nose running like a faucet. I was so embarrassed--hair was soaked, arms were wet and sticky (DGS didn't want to hold my hand) and I had tissue in pocket but that was useless. Dr. G says it's just dysautonomia thing. That's why we have to drink, drink, drink to replace the insensible loss of fluid.

I am underweight and feel better when I weigh more (but still have syncope) but if I gain more than what my 'typical weight' had been, I start to feel struggles with that--as if body is working harder lugging around excess baggage it is not accustomed to--and the fluid retention gets worse.

I've never gone in for IVFs--just drink, drink, drink. I'm still very dry (had to be started on Evoxac). My lytes, hemoglobin, hematocrit are always ok. When I do get IVs in ER for other reasons or surgeries and such, I retain the fluid and it takes forever to get it off me (which is harder on my breathing and probably the heart). Now with doing the Ig transfusions, I'm going to have to try to at least triple my norm today and during tomorrows transfusion. With gastric issues and filling up with fluids, you feel waterlogged and it takes away your appetite (that I suppose I could take in as more fluids like jello but no nutrition in that). I'm starting Rx strength nutritional sustanance when it gets in to pharmacy Tues after my all day transfusion tomorrow (so can add that to my fluid list). I don't want veins traumatized any more than they absolutely have to be--get too many blood draws and on IV Ig--the least invasive way to get the fluids is always the least risky.

No matter about the BP, HR of 40 is pacer range. You need to check this out with your cardiologist.

When I was working we had FMLA (family medical leave act) that also applied to individuals. Can probably download form from government website, but I got from HRC (they probably are mandated to carry). To make things simplist for my PCP, I would fill it out and just have her review it and if concurred, sighned and dated it. The FMLA was good for 1 year but had to be re-initiated each year. That being said, if you did not have enough sick time banked, you would only receive payment for those hours in your sick bank. You could still be on FMLA but you wouldn't receive any pay if you had no sick time. In my case, even tho I had tons of sick days accumulated (working 25 years), I could not keep up with 50-60 hours week in high stress/physically demanding on-call 24/7 job after significant trauma when I was hit by a car. Since my employer could not enable be to go to part time under FMLA--as the salaried job had to have a full time abled body, I was forced into early disability retirement (that my employer supported so it went through quickly and smoothly).

You need to get in to your cardiologist. 40 is too low--pacemaker material. When I was having more OH and less POTS, while the BP plunged, rather than going tachy, it now hangs in 60s--sometimes 52. My cardiac specialist told me my autonomic nervous system was failing, that I would have fewer warnings of faint and that I would probably need a pacer on down the road.