E Soskis

I often have difficulty taking deep breaths and at times become short of breath for no apparent reason. I understand that blood vessels become floppy with dysautonomia and blood pools in the mesenteric system of the abdomen - when it does this, there is not enough blood available to other body systems. The restriction of blood flow can be felt in the chest causing chest pain or trouble breathing - the muscles of respiration require adequate blood flow and when restricted, the chest cannot "breathe" very well. This can be particularly bad at night - especially if I lie flat - so, I sleep with my head up on a wedge pillow and several other pillows - this helps a lot.

Absolutely - stress reactions cause the release of many hormones that cause the nervous system to go haywire - I just have not figured out how to avoid stress - it seems to follow me wherever I go!

Never stick Q-tips in the ear canal - causes damage and can puncture the ear drum - the stimulation of the Q-tip in the ear canal can cause the tube behind the middle ear that drains into the back of the throat to spasm and result in coughing or a scratchy throat -

If you must clean out the ear, there are products over the counter that help soften ear wax for easy removal - just don't stick anything in the ear canal - I've worked in emergency department for over 30 years and have seen lots of ear problems caused by Q-tips.

does your skin taste salty? - kidney issues can cause skin itch and salts come up through the skin - liver dysfunction can also cause skin itch - routine blood tests would pick up abnormalities

Some hints related to plasmapheresis: hydrate, hydrate - drink plenty of fluids in the hours before the procedure; the procedure takes out blood sugar from the bloodstream so, eat something about 30 min-1 hour before the procedure and keep a bottle of gatorade, powerade, ensure, or something like that at bedside to sip on during the procedure; you are correct to pre-medicate with Zofran before getting started - might want to consider pre-medication with benadryl as it helps with nausea and tremors also. Be absolutely sure that the plasma team is not trying to keep calcium levels up with oral calcium tablets just before the procedure - it won't work and oral calcium takes hours to digest and get into the blood stream. IV calcium gluconate during the procedure will keep away the tremors and prevent the tetany of muscles from low calcium levels. If low blood pressure during and after the procedure is an issue, the MD can order IV fluids to be given during and after the procedure. I always receive at least one liter of Normal Saline during the procedure and sometimes, a second one after the procedure. If receiving IV calcium, your son must be on a cardiac monitor in an area where resuscitation is available. This is a national standard for the IV administration of calcium.

I have my procedure done in the PACU where there is always a monitor, resuscitation equipment, and experienced physicians and nurses. I have been receiving plasma exchanges for over 2 years now and I also started with 2 weeks in the hospital - a double exchange every other day. I am now down to once/month for exchanges - the plasma team can adjust how many exchanges your son can tolerate - I cannot tolerate a full double exchange as an out-patient but, I can make it to a 1.5 exchange before crashing with low blood pressure and nausea. Adjusting the pre-medications, and medications during and post-procedure makes a huge difference. I have IV benadryl, zofran, phenergan, and pepcid ordered on an "as needed" basis -

Hope this works for your son - it has made a big difference in the quality of my life

Lack of sleep and fatigue contribute to worsening symptoms of dysautonomia. Fatigue can put me down quicker than anything else. The brain fog gets especially bad - I lose my train of thought, can't put words together right, and have trouble concentrating - especially at work. I have had less than 4 hours of sleep every night for over a week now and I am almost a complete wreck. Stress is also a huge factor - so, combine stress and fatigue and we're doomed!

Well, theoretically, the "coat hanger" pain is due to the lack of blood flow to the area. I get this pain very regularly - it takes a few days to calm down and it seems to correlate with blood pressure issues as well. I sleep on a wedge pillow with two others on top of it - this promotes natural circulation and sometimes is the only thing I can do to relieve the pain. Anti-inflammatory medication (such as advil) does not work as it isn't so much a muscle inflammation problem. Lack of blood flow to any body part will cause fairly severe pain. I have never used muscle relaxants for this pain as they tend not to mix well with dysautonomia. I would speak with your MD about muscle relaxants and the side effects to expect if you decide to go that route - again, restoring blood flow to the area will be your best potential for relief.

The autonomic dysfunction keeps me either cold as ice or so hot, I feel like I'm going to melt. My skin feels like it has either been dumped in ice water or heated to a crispy crunch on a grill. All I know to do is keep a sweater in one hand and a fan in the other....

I had a central tunneled catheter for about 3 years - I went weekly to a home health agency for flush and dressing change. Once a month I had to have heparin instilled and as time went by, before each plasma exchange, the plasma team would instill some Activase to be sure the catheter was fully open and functional. These tunneled catheters are not meant to last for long periods of time - they are supposed to be temporary until a more permanent graft or fistula can be established surgically in the arm or elsewhere. I think mine lasted an exceptionally long time before I became sick with sepsis and blood infection. Once a systemic blood infection occurs, the catheter must be removed. It is extremely important to recognize the symptoms of a blood infection and get medical attention right away - even if it is in the middle of the night. I knew I was infected because I felt different all over - sudden onset extreme weakness and fever. I could barely drag myself to bed. I told my family I had a sepsis and needed help right away and my son took me to the ER. After several months of battling sepsis that kept re-curing, I ended up with a "necklace graft" - I've had it for over a year now and have had no problems. Plasma exchanges have kept me up, working, and functional. I would hate to think what would happen if they quit working - good luck and take scrupulous care with the central line.

Yes - the more invasive the surgery, the more the disease flares. The surgery, anesthesia, and pain medications are detrimental to dysautonomia. I had a robot-assisted hysterectomy a few years ago and it took over 6 months for me to get back to "normal". Hang in there - do everything you know to do to get better and eventually, symptoms will calm down.

I have worked in the medical profession for over 30 years. I do not like the trend I see in medicine today. We are moving to medicine that provides "the greatest good to the greatest number" - the resources will be allocated to cover the most people and be withheld from the ones that cost "too much" - only the "haves" will be able to obtain expensive or experimental treatments - the "have nots" will only receive basic care and nothing else. Specialists will be few in number as there will be no incentives for physicians to specialize - in fact, there will be penalties for not providing watered-down care to mass numbers. Most healthcare will be provided by "extenders": PA's, NP's, and Med Techs. Few large centers of care will remain open as their funding will be decimated. On the other hand, drug companies, medical equipment suppliers, and the like will make huge profits. The only available health insurance source will contract with large companies to provide services and these companies will clean up - the profits will be astronomical. (Remember the cost of the Pentagon wrench?)

Some will think I'm merely cynical and jaded however, I have already seen the changes begin and it is not pretty. Look at the drug "shortages" that occur every week - the lists are on the FDA website. These drugs are no more in short supply than hot weather in the South! The supply is being controlled to drive up costs and increase profits of drug companies - at the expense of the patient and unfortunately, at the expense of saving lives.

Have they ruled out autoimmune causes?

My legs and feet commonly turn mottled if not outright purple from neuropathy. My hands also turn mottled and my fingers will turn white and lose feeling - especially in cold weather, in a cold store, or if I don't wear gloves when I handle frozen food items.

There are days I can walk around the block and there are days I cannot. I also have heaviness in my legs at times - I have neuropathy also - feels like bugs crawling on my feet and lower legs. I also have right hip pain - pretty much all the time. I didn't equate it with dysautonomia but, maybe it is.

I find the longer I go without walking (at least some), the worse the symptoms can become so, I try to move around more frequently. I also remember in college my Anatomy and Physiology teacher telling us "if you don't use it, you lose it".

I agree that midodrine is helpful. I suppose since the blood vessels are so floppy, midodrine gives them some tone and ability to constrict. I keep midodrine with me at all times - wouldn't be without it!

I know my BP is low when I begin to get extremely fatigued and feel weak all over. If I don't sit down or prop up then, I begin to get the symptoms you describe. Midodrine helps me a lot as well as staying hydrated - my doc once told me that my fingers should always be a little puffy - that would indicate I am holding on to fluid better. I can't always get them puffy but, I try to drink enough to keep my urine from being too concentrated. Perhaps you shouldn't get up from a seated position so fast - kind of gradually get up. I do this in the morning when I wake up - I sit up then dangle my legs then attempt to stand - it seems to work OK.

Wonderful! The IV port will continue to heal in and after a little longer, won't be painful anymore. The first few times it is accessed after insertion, it can be somewhat painful because it wiggles around a bit - this will get better and better. Just keep everything very sterile when accessing it - be sure and flush it well after the infusion using the heparin solution. Mine lasted a long, long time and the only reason it got infected was because I also had other central lines inserted near the port. I hope you keep feeling better and the saline continues to work well for you.

My port lasted 6 years and would have lasted much longer had I not developed an infection in a different central line that traveled to the port since they were in close proximity. I know people that have had them for over 10 years. It is individual - you can't predict. With good care and maintenance, they should last indefinitely. Your physician does not fully understand ports to be asking for a "time-line". It doesn't matter. If you have poor venous access and anticipate receiving regular IV fluids, you need a port. There is absolutely no need to fry all your peripheral veins attempting to stick in an IV. You need your peripheral veins for circulation and for simple blood draws. Look into the new generation ports: they are smaller and offer all types of IV access. You need to be speaking to a vascular surgeon - they are the ones that put in ports - or a good general surgeon. I work in trauma and our trauma surgeon placed my port - he did an excellent job!

Yeah - I worked in an ER for over 30 years and could stick any vessel anywhere anytime - I once had an addict tell me that if I took "my show" to the street, I would get 30-dollars a stick! What a hoot! Just be aware that as nice as a port is, it needs maintenance - it should be accessed at least once a month, flushed, then have heparin instilled. Most home health care companies that also perform IV sticks and meds will set up something to accommodate this. I may not have used the port for weeks to months at a time but, when I did use it, I used it a lot - it was well-worth it.

Let me say something about PICC lines: their use is exponentially increasing so consequently, so is the complication rate from them. PICC lines put you at risk for venous thrombosis, thrombophlebitis, and central line infections. I had a PICC for over a month in which I gave myself IV antibiotics twice a day - coincidentally for sepsis contracted through another central line. The PICC line did not get infected but, it left my arm with swollen veins and pain that is still present 7 months later. I have also been in a battle with cellulitis in the PICC arm just from now what appears to be a chronic phlebitis. Given the choice, I would never have another PICC placed - just too much trouble and too many problems.

I had a port placed for weekly IVIG infusions - it lasted over 6 years and I used it for IV fluids and other IV medications - it was wonderful and if I could, I'd have another one placed. I had to have a subclavian double lumen central line placed for plasmapheresis and it lasted 3 years then became infected which caused the port to get infected as well. I cried and cried when the MD told me I had to have the port removed as well as the central line. I now have a "necklace" graft that is used for the plasma exchanges but, it was major surgery and I have serious side-effects and consequences from it. The MD declined to place another port at the time he inserted the necklace graft due to potential cross-contamination and infection of both if one became infected. I now have no IV access as my left arm is messed up from failed graft and fistula attempts. My right arm is red, hot, and swollen all the time from the necklace graft. I have resorted to placing an IV in my foot in desperation when I became extremely dehydrated. The hospital staff are scared of the necklace graft and refuse to stick it for IV access - it is "against policy". If I really ever need and IV again, I will have to have a femoral line placed and so far, I have had 3 rounds of septicemia from femoral lines - so, if you can get a port placed, go for it! They are wonderful and relatively hassle-free.

Yes - to prevent this, talk to your physician about taking a bedtime/nighttime dose of phenobarbital

The whoosh sound in my ears is all about my BP - when the midodrine kicks in and my blood pressure rises, I hear the swoosh of the blood flow in my ear. When my pressure is very low, no whoosh!

Every late afternoon my eyes seem fuzzy and my glasses are definitely blurry. I think it has something to do with fatigue. I get up between 4-5am every day and by mid-afternoon, I am exhausted.

My blood pressure always dropped very low during the IVIG infusions - I would take a liter of Normal Saline after the infusion to help my pressure get up enough to drive home - I also had to take the IVIG at a much lower rate than most people due to the rapid BP drops - it was annoying to stay all day to get a simple infusion but, it was necessary and worked fairly well....

Yes - I had a hysterectomy some years ago and the hot I feel from the dysautonomia is distinctly different than the occasional "hot flash" from menopause. I can tell when it is about to hit - I feel weird all over then suddenly extremely hot - my skin is very hot to touch - I get weak and have to sit down for a moment or two - it only lasts a minute or so then, is gone. If anyone has suggestions as to how to control these episodes, I'm all ears!

I went gluten free for a summer and did not notice any difference in my GI symptoms. My physician said there was no need to test me since I didn't respond to the diet. I found it to be cumbersome, expensive, and was miserable the entire time I was on the diet.