A Definitive Diagnosis At Last? Is Sjorgen's Syndrome causing my POTS?

I got a call from Cleveland Clinic last week.  My lip biopsy taken the week prior tested positive for Sjorgen's Syndrome.  WOW!  I have had 1001 blood tests for autoimmune diseases like Sjorgen's and Lupus and all have been negative.  This is the first time a test has come back positive that may explain WHY I developed neuropathy and POTS.  I have been searching for the cause of my POTS since the first day I realized I had it, and this may be the answer I was looking for.  It's not a good answer, but it's better than no answer.  At least now I have a chance to try therapies that may improve my symptoms and help me get my life back.


My Cleveland Clinic neurologist wanted me to have the lip biopsy for Sjorgen's, even though my SS-A and SS-B antibody tests for Sjorgen's were negative.  I didn't think I had Sjorgen's which is commonly associated with dry eyes and dry mouth, but my doctor insisted that this lip biopsy was an important test to rule out Sjorgen's.  He said that 40-70% of the people who actually have Sjorgen's test negative on the SS-A and SS-B blood tests, and the lip biopsy is considered the gold standard.


Sjorgen's Syndrome (pronounced SHOW-grins) is an autoimmune disease in which your own immune system goes into overdrive and attacks your own healthy cells.  Sjorgen's antibodies commonly attack exocrine glands, which are the glands responsible for producing moisture and healthy mucus that lubricate your eyes, mouth, sinuses, gastrointestinal tract, lungs and other areas.  Sometimes Sjorgen's presents with "extraglandular" manifestations, meaning it harms something besides just exocrine glands.  In my case, it looks like Sjorgen's has damaged some of my autonomic and sensory nerves.


The Sjorgen's Syndrome Foundation has an informative chart that lists some of the impacts of Sjorgen's on the human body.  Sjorgen's is the most common autoimmune disease in the US.  There are at least 1 million people in the U.S. currently diagnosed with Sjorgen's, and experts estimate that there are another 3 million people with it in the US who have not been diagnosed yet.  The average Sjorgen's patient takes 6-7 years to get a proper diagnosis.  Due to the lack of knowledge about this syndrome and the difficulty in diagnosing it, there are probably many people living with Sjorgen's who are misdiagnosed.


There is currently no cure for Sjorgen's, but there are medicines used to help alleviate symptoms.  Many Sjorgen's patients use prescription eye drops, like Restasis or gels at night to alleviate dryness of the eye. Some even have silicone plugs inserted into their tear ducts to retain as many of their tears as possible.  There are also drugs used to increase tear and saliva production.  For patients with organ involvement, immunosupressive drugs, like Prednisone and Methotrexate are used.


I am looking for a Sjorgen's Syndrome expert, so if any of you can recommend one, I would really appreciate it!

Florinef, Oh Florinef, How I Love Thee...

I love my Florinef.  Normally I don't like taking pills, or any medicines.  I am the type of person who avoids even Advil and Tylenol, but I really love my Florinef.  It has been a big help in my overall POTS treatment.


If you have POTS, OI , hypotension (low blood pressure), or hypovolemia (low blood volume), you may want to talk to your doctor about trying Florinef.  Here is some information you may be interested in.


What Is Florinef?
Florinef is a synthetic form of a steroid that occurs naturally in our bodies.  Flourinef is in a groupd of steroids called corticosteroids.  The two primary corticosteroids in your body are cortisol and aldosterone.  Corticosteroids can be broken down into glucocorticois (like cortisol) and minderalcorticoids (like aldosterone).  Flourinef has properties closer to that of aldosterone.  These are not the type of steroids taken by gym rats and MLB players to get big muscles.


How Does Aldosterone Work In the Body?
Aldosterone works to control the levels of sodium, potassium and water in the body.  There are microscopic ion pumps in your kidneys that are responsible for maintaing the balance of fluid in your blood.  If you have an excess of fluid in your blood, which could cause edema, swelling and high blood pressure, healthy kidneys will release sodium through these pumps into your urine, so it will leave your body.  Generally speaking, wherever sodium goes in the body, water will follow. So when you lose sodium through urine, you lose water through urine as well.  However, small nerve receptors in your kidneys can detect if you have lost too much sodium and water, which can cause dehydration and low blood pressure. When that happens, your kidneys tell your adrenal glands, through nerve and hormone messages, to produce aldosterone.  The aldosterone works on your kidney to conserve sodium, and thus conserve water.  Instead of excreting sodium, aldosterone will swap out the sodium with a potassium ion, and you will excrete out potassium instead.  Since the sodium is retained in your blood, the water will follow the sodium and you will maintain the fluid part of your blood.


Of course, this is an oversimplified version of the complex processes that help maintain healthy blood volume, blood pressure and hydration of your body.  There are dozens of hormones, nerve receptors and pressure gradient detectors involved in regulating blood volume, but the explanation above is a basic summary of the role aldosterone plays in this complex process.  If you would like to learn more, you can research the "Renin-Aldosterone Axis" online.


How does Florinef work in the body?
Florinef acts like aldosterone in the body.  Florinef helps the kidney preserve sodium and water, and instead causes the kidney to excrete potassium into urine.  This helps increase blood volume and increase blood pressure.


How long does it take to start feeling a benefit from Florinef?
When I started the drug, I was told it would take a few weeks before I noticed any difference, and that it could take up to 6 months to get the full benefit.  However, I started feeling better within 48 hours.  I had such low aldosterone when they started me on it, and was having such low BP at the time that I thought I was going to die.  I was 80/30 laying down flat with my feet elevated, and they couldn't even get a BP reading on me if I stood up - because I would either pass out or it would be so low the BP machine couldn't detect it.  Within 48 hours of starting the Flourinef, I felt like someone had plugged in my battery and given me some juice.  I contiuned to improve and feel better for about 3 or 4 months.  


What about weaning off/tapering down off Florinef?
After 4 months of taking .2mg in a split AM/PM dose, I was feeling so good, I thought I should try to lower the dose.  I am of the mindset that you should always take the least amount of drugs that you need to feel good.  I had a neurologist at the time who told me to cut the dose in half right away and check my BP a few times a day.  Whoa, was that a bad idea!  I later learned from other doctors who have more experience with POTS that, unless you are stopping it for a few days to have a test, you should lower the dose very slowly, especially if you had naturally low aldosterone levels before you started taking Florinef (which is why it's so important to test your aldosterone levels before you start taking Florinef!).  I was told that if you want to lower your Florinef dose, try cutting out half a pill (.05mg) once or twice a week, and then maybe 3 or 4 times the next week, and then see if you can go for 2 weeks at 1/2 a pill less.  This is a much slower taper than my first doctor told me about, and I was able to go down to .1mg in the AM and .5mg in the PM for a few months.  I am back up to .2 now, since I we now know I have hypovolemia, even on the Florinef - so I need as much sodium and fluid retention as I can get right now.


Why do so many POTS patients take Florinef?
POTS researchers have discovered that many POTS patients have low levels of aldosterone.  This can contribute to low blood pressure and low blood volume, which can cause lightheadedness, syncope and so many other symptoms common in POTS patients.  Since Florinef is the medicine that most closely mimics natural aldosterone, it is an ideal drug for patients with low aldosterone.


Researchers do not fully understand why so many POTS patients have low aldosterone, but they speculate that it may be due to problems with the autonomic nerves that help regulate the renin-aldosterone axis.  This is understandable, because so many POTS patients are also known to have autonomic neuropathy or overall dysregulation of their autonomic nerves.


There are other POTS patients who do not have low aldosterone, but some of them may benefit from Florinef as well.  Patients dealing with low blood pressure, lightheadedness, fainting and/or low blood volume may benefit from the increased fluid retention that Florinef causes.


How do I know if I have low aldosterone?
Your doctor can test your aldosterone levels with a blood test.  If your regular doctor is not familiar with this blood test, you may want to see an endocrinologist.  Ideally, your doctor should put you on a controlled sodium diet for a few days before the test, because a high salt diet can cause your aldosterone levels to decrease (which is normal).  Some doctors will test your aldosterone laying down, and then after a period of sitting up or standing, since your position can impact the test results.


If your aldosterone is low, doctors can do a few additional tests to determine the cause of your low aldosterone.  Some of the tests may include imaging, like ad MRI, of your adrenal glands to make sure they are normal in size and shape.  They may also do an ACTH stimulation test on you, in which they give you a small dose of a drug that should increase your cortisol levels.  Both coritsol and aldosterone are produced in your adrenal glands, and if one is deficient, it is important to find out if the other one is deficient too.  If the ACTH stimulation test does not stimulate your adrenals to produce cortisol, you may have Addison's Disease.  Fear not, because this disease can be managed through medication. President Kennedy had Addison's Disease, and he was one of the first patients in the world to regularly use Florinef as part of his treatment, because it was a very new drug at that time.


There are other causes of low aldosterone, which doctors call hypoaldosteronism.  You can read more about it here.


Keep in mind, that if you have autonomic neuropathy or dysautonomia, it is very possible that your low aldosterone levels have nothing to do with your adrenal glands being unhealthy, and it may be beacuse the messaging/receptor system between your kidneys and adrenals is not working properly, which researchers suspect to be the cause of hypoaldosteronism in many POTS patients.  As of right now, I am unaware of any way to test this.


What are the common side effects of Florinef?
Most patients tolerate Florinef well.  The most common side effect seems to be headaches, and that could mean that the Florinef is working too well and causing you some high blood pressure.  Always tell your doctor about any side effects you experience, but I would also recommend checking your BP if you have recently started Florinef and are getting bad headaches.  Some people say they got headaches for the first few weeks and then their bodies adjusted to it and the headaches went away.


Since Florinef works by excreting potassium and preserving sodium, it can leave you susceptible to low potassium.  Sometimes mild low potassium will not cause any symptoms, but it can be hard to tell because some of the symptoms of low potassium overlap with POTS symptoms: 
-weakness, tiredness, cramping of arm or leg muscles (in severe cases, temporary paralysis)
-tingling and numbness
-nasuea
-vomitting
-abdominal cramping
-bloating
-constipation
-heart palpitations (in severe cases, heart arrythymias that are dangerous)
-low blood pressure
-lightheadedness
-fainting
-passing large amounts of urine and feeling very thirsty
-confusion
-hallucinations
-depression
-psychosis
-delirium


Since it can be hard to tell the difference between your regular old POTS lightheadedness, palpitations and muscle cramps, I would also encourage everyone taking Florinef to make a plan with their doctor before they start the drug to regularly monitor their potassium levels.  Some doctors will order a blood test once a week for the first few weeks you are on it, or when they are increasing your dose.  If everything is going smoothly, they may only have you get the blood test every other week or once a month.


Many doctors also prescribe low dose potassium supplements to patients in Florinef.  This can prevent you from having the low potassium side effects.  My doctor has prescribed me 10 mEq (milliequivalents) a day of potassium chloride, which is the equivalent of about one banana.  I also eat a high potassium diet (white potatoes, dried apricots, coconut water, bananas, etc.).


Florinef can also make you more prone to infections and can mask the signs of an infection.  Florinef can make you more prone to oral yeast infections (thrush). If you have a yeast infection in your mouth, everytime you swallow you are introducing the infection into your GI tract.  To avoid getting thrush in the first place, I take probiotics (like Culturelle) daily, eat soy or coconut milk yogurt (because I a dairy free) and I also take a chewable acidolphilus after I brush my teeth right before bed, to prevent yeast overgrowth in my mouth.  I have been on Florinef for almost a year and have never had thrush or a yeast infection, so I guess my probiotics are working.  And probiotics are good for all those other GI symptoms POTS patients have too, so it can't hurt to add them to your routine.


Florinef is meant to cause fluid and sodium retention, and in many people, that leads to swelling in the legs, face or neck.  This is just water retention.  I get in my legs from the Florinef, but I have found that if I wear 20/30 mmHg compression stocking, it does not happen.  I think this moves the fluid from my legs elsewhere in my body, but I'm not sure where because I am not having swelling anywhere else (thankfully!).  I'd rather have fat legs and cankles than be passed out on the couch all day, but for some people, the swelling bothers them too much.  Not everyone on Flourinef has this.  Also, if you do get swelling that bothers you, you may just need a lower dose.


The other side effects of Florinef can be found here.  Be sure to read the prescription inserts so you know what to look out for.


What should I know about Florinef dosing?
My doctors told me the average patient takes between .1mg and .3mg of Flourinef per day, with .2mg being the most common dose I have heard of for adult POTS patients.  The pills come in .1mg tablets, and can usually be broken in half if you need a .05mg dosing.  Most people take all of their Florinef first thing in the morning, sometimes even before they get out of bed.  When I took .2mg in the early morning, I found that, even though Flourinef is supposed to stay in your body for over 24 hours, I had really, really low BP in the morning and it was too high at night.  So I split the dose, taking a .1mg pill in the early morning and another .1mg pill right before bed (around midnight for me).  That has helped me have more even BP throughout the day.  


No matter what dose you take, you have to increase your water and sodium intake, or the Florinef will not work.  I try to drink a large amount of fluids with it, to prevent stomach upset.  Other people take it with food to prevent stomach upset.


My experience with Florinef:
Overall, I think Florinef has helped with my POTS symptoms and I have not had any really bad side effects. I have had a little water retention in my legs, which is just cosmetic and can be prevented with compression stockings.  


I really know it is helping me, because I had to go off of it cold turkey for 48 hours for a test.  My doctor admitted me to the hospital for observation, since the sudden stopping of Florinef could be dangerous, especially for me because I had low aldosterone before I started taking it.  In the first 24 hours, my BP actually spiked, which is quite rare for me.  The nurse told me this was common when you went off Florinef. She said it's like your body is trying to compensate for the loss of this important hormone, but it overshoots.  The second 24 hours, I crashed as expected.  My BP was back to 80/30 again laying down.  My tachycardia was worse when I tried to stand up, my lightheadedness kept me in a fog all day.  Thankfully, I had the test on the third morning and was given IV saline and my Florinef right after the test.  Within 24 hours, I was back to my normal low BP and tachycardia levels.


I am not a doctor and none of this is meant to replace actual medical advice from your doctor.  I just thought you  may want to hear about my experience with Florinef.  If you have had a good or bad experience with Florinef, or you want to add some information to this post, please post a comment below.

How to Beat the Bedrest Blues

So many POTSies are stuck in bed most of the time, because it is so hard for them to sit up without having a massive "POTS attack" of messed up blood pressure, severe tachycardia and all of the vertigo, lightheadedness, fainting, etc. that goes with that.

So what's a POTS Grrl (or Guy) to do when stuck in bed?  Well, being that I spent over 9 months in bed or laying down on the couch in my den, I know a thing or two about beating the bedrest blues.

First we need to acknowledge that the bedrest blues are not just plain old laziness or depression.  If your body is so physically ill that you cannot get out of bed, it is not laziness. And if you can't get out of bed because you are physically ill, of course you are going to be depressed about it!  This is not some abnormal mental pattern  - the only person who wouldn't be depressed about being stuck in bed is someone who probably has no conscious awareness of the fact that he or she is stuck in bed - like someone who is 'brain dead.'  So, please don't think you have lost your mind just because you are really, really sad about being stuck in bed.  If you are having an emotion that %99.999 of all humans would experience in response to your current situation, you emotions are about as normal as can be.  It's OK to be sad about this.  BUT, no one wants to live their life being sad all the time, so you have to do something about the bedrest blues.

Some people turn to family, friends, counselors, psychologists, etc.  I did all of the above, and while I am eternally grateful for everyone who was kind enough to listen to me during this difficult time in my life, I don't think that talking to people was really enough to cheer me up.

Some people take prescription drugs from their doctor to combat depression.  I am not a fan of taking any drugs unless you REALLY need it.  However, some studies have shown that SSRI drugs, commonly used to treat depression, have a stimulating effect on the nervous system that helps some patients with POTS even if they are not suffering from depression.  I tried one of these drugs for about 4 days, hoping it would help my POTS, but instead it made my face and neck numb and gave me these creepy crawly bug sensations all over my skin.  No thanks!  I'm not saying these drugs don't help some people, but before you start taking meds, I would encourage you to try non-pharmaceutical means to lift your spirits.

Some people turn to religion.  Some people speak with their priest, minister or rabbi.  I do not attend church, but have always considered myself to be a spiritual person who believed in God.  I have found praying to help me through the toughest days since I have been sick and sometimes I think it even helps me sort stuff out in my own head.  I think when you ask God for something in a prayer, you really have to ask for important stuff, at least for me, so praying can really help you think about what is important to you.  What really matters?  Hopefully you're not going to waste a prayer asking for something silly and frivolous, when there are things in your life and the greater world that are probably more deserving of God's attention, like healing your body and keeping your family healthy and happy and finding a cure for POTS.  Also, laying in bed bored is a really good time to read, and not many books are longer than the Bible.  Whatever your religion, or even if you are not religious at all, think of bedrest as an opportunity to get in touch with your spiritual side or maybe just educate yourself on all of the world's great religions.

All of these things are well and good, but I found that the best way for ME to beat the bedrest blues was to keep myself as busy as my physical condition would allow me to be.  I created a "CAN DO" list, where I literally listed everything I could probably do while stuck in bed, even if it was something I didn't want to do or couldn't do everyday.  When I was feeling really down because I was bored or felt trapped in bed, I would look at my CAN DO list and just start doing things on it.

Here's a sample of my "CAN DO" list from last year:
-talk to POTS friends on Facebook
-research POTS journal articles on laptop
-make a POTS blog
-call a friend I've been meaning to talk to but was too busy for before POTS
-10 leg lifts each leg (even if I only did one at a time and took all day to complete it)
-arm exercises with Poland Spring water bottles
-stretch often
-massage my legs an give myself a nice pedicure
-give my hair a good old fashioned mayo and eggs conditioner treatment
-give myself a bunch of little braids like I'm on vacation in the Caribbean
-give myself an avocado/cucumber facial
-take a loooooong bath (not with hot water so you don't lower your BP too much)
-play cards by myself
-learn card tricks from Internet
-play cards with a friend
-play board games
-invent board games
-study a foreign language from books on tape and/or videos
-listen to books on tape
-listen to music like I used to back in high school, and try to learn all of the lyrics to my favorite songs

-watch TV (again, depending on the dizziness)
-rent free movies from the library
-play silly video games
-figure out how to play Wii Sports games laying or sitting down
-watch movies and TV shows on Hulu.com for free
-look at stupid videos on YouTube
-pick a random subject and learn everything you can about it (example: butterflies of the northeast US or ancient Greek architecture)
-meditate/deep breathing
-some yoga positions
-pray
-write a love letter to your significant other
-make homemade greeting and holiday cards
-write a handwritten letter to your grandma or old Aunt Myrtle
-write poetry
-write a book
-keep a symptom journal
-keep a fun journal that has nothing to do with POTS
-figure out the top 10 things you want to do in life before you die; your "bucket list" and don't leave things off the list just because of your POTS - what would you do if POTS didn't limit you?
-draw
-paint
-learn to knit a scarf
-do more arts & craftsy stuff
-lanyard
-beading
-clip coupons
-plan your menu for the week, to minimize the amount of time you have to spend in the kitchen and to eat as healthy as you can (hint: leftovers for lunch!)
-read the newspaper everyday (dizzy dependent)
-put your family's budget in a spreadsheet so you can track what you are spending money on and see where you can pinch some pennies
-organize all of the photos I have piled in shoe boxes
-make an awesome photo album with funny sayings and stuff
-scrap booking
-sell some stuff on e-bay or Craig's List
-organize medical records into binders and make a list of ALL tests you ever had in date order (put ABNORMAL test names in CAPS)
-research family history on Ancestry.com and the Internet (again, only when I'm not dizzy)
-start seeds indoors in a sunny window
-paint some cheap terracotta flower pots
-plan the perfect party in your head that you will throw when you are feeling better and have given POTS a kick in the ass
-cuddle extra good with the dog

OK, if you could get up and walk around like a normal person, you probably wouldn't have time to do most of the stuff on this list.  But this list served three important purposes for me:
(1) It reminded me that my life was NOT over and that there were plenty of things I COULD do from bed, including things that I wouldn't have had time for if I had not been stuck in bed all day.

(2) It helped me not be so bored all the time, because when I was bored, I would just look at my Can Do list and pick something to do.

(3) As I started to get a little better, I began to add more physically challenging things to the list, which reminded me that I was making progress and getting better, even if it was much slower than I wanted to get better.

Just keep reminding yourself that your feeling like a wet noodle is only temporary.  Most POTS patients WILL FEEL BETTER over time.  It may take a few years, and t may be a very slow process with lots of ups and downs and setbacks, but just remember this is not permanent for most people.  Your life will get better, and you can't let yourself get stuck in the bedrest blues mode.  Your body needs time to heal, and the last thing you need is to get super depressed, which can slow down your healing.  In fact, your body can actually suffer real physical symptoms if you get really depressed, so do yourself a favor and consider "happy time" to be part of your overall treatment to help you overcome your POTS symptoms.  Do something that puts a smile on your face at least once a day, and even on those really awful days when you are in the ER passed out and not breathing and maybe your doctor doesn't know what to do - just take a second to think  - someday I am going to look back on this ordeal and say, "wow, that sucked!"  In the grand scheme of life, like 70-90 years, a few months or years in bed is not fun, but it is barely a blip on the screen when you look at the whole picture.

My motto though my POTS ordeal has been "slow progress is better than no progress."  Don't get too bummed when you have had a good week, and then all of a sudden you have a bad one the next week.  Just think, "well hey, I will have another good week, or maybe even 2 good weeks in a row next month."

Keep your chin up!  Things will get easier and better for you!

How to Talk To Your Doctor About IV Saline for POTS

So maybe you've noticed that you feel really good after you get a liter or two of IV saline when you end up in the ER with your POTS symptoms.  And maybe you're thinking, hey, why can't I get this saline at home so I don't have to end up in the ER in the first place?  And maybe you've asked your doctors about this, and they roll their eyes at you and say "no way!"  This is a common scenario for POTS patients.

Most doctors don't want to be the first ones to try a novel therapy - they are worried you will sue them if it goes wrong, they are worried the insurance companies will give them a hard time if they prescribe non-traditional treatments, and they are worried about getting in trouble with medical licensing boards for prescribing non-traditional treatments.  So try to understand this from your doctors point of view.  If they prescribe you something out of the ordinary and not "proven" to work, they could be putting their career at risk.

That's why you have to you make it your mission to show your doctor that the therapy you want, IV saline, has been used before to treat POTS and its close cousin Orthostatic Intolerance, with excellent and safe results.  Prestigious researchers are currently studying the benefits of IV saline on POTS patients.  But any of us POTSies who've had saline while in the ER, don't need to wait for those studies to be published - we know saline makes us feel better and greatly improves the quality of our life. 

Here's a 10 Step Strategy you can use to encourage your doctor to let you try IV saline therapy.  I'll explain the details below.

1) Make sure your doctor understands that most POTS patients have low blood volume.
2) Make sure your doctor understands which POTS symptoms are probably caused or worsened by low blood volume.
3) If your doctor doesn't think that you have low blood volume, have him order a Daxor blood volume test to be sure.
4) Make sure your doctor knows you have tried less invasive ways of increasing your blood volume first.
5) Remind your doctor that you have felt so much better after getting saline in the ER (assuming that's true) and that you cannot afford to go to the ER a few times a week just to feel better.
6) Make sure your doctor understands that many other POTS patients have benefited from IV saline.
7) Make sure your doctor understands that top POTS researchers are doing studies on saline therapy for POTS, because they think it works.
8) Make sure your doctor knows that YOU understand the risks of having a PICC line or a chest port or regular IV infusions, and that you are willing to take the risk to see if you can get your quality of life back.
9) If your doctor is still reluctant, ask him to take your pulse, blood pressure and VO2 max measurements before a saline infusion, and then after you've had a liter or two of saline through a regular IV needle in your arm, to see if your numbers have objectively improved.  This may not work, but if it does, it's pretty much irrefutable evidence that saline will improve your tachycardia, BP and endurance, and thus your quality of life.
10) If all else fails, find a new doctor and repeat from Step 1.

STEP 1
Make sure your doctor understands that most POTS patients have low blood volume. It is important for your doctor to know that most POTS patients have low blood volume, which doctors call hypovolemia.  Vanderbilt University, one of the leading POTS research centers in the world, found that POTS patients had an average of a 12% deficiency in their plasma volume, but as high as a 27% deficiency. Plasma is the clear yellow part of your blood that carries nutrients and it's made up of about 80% water.  Additionally, the Vanderbilt study found that POTS patients had a 27% deficiency of red blood cell mass.  Red blood cells are the cells that transport oxygen to the rest of your body from your lungs.  With low plasma and low red blood cells, POTS patients were found to have an average deficit of about 460mL in total blood volume. I highly suggest printing out this Vanderbilt study and showing it to your doctor: Renin-Aldosterone Paradox and Perturbed Blood Volume Regulation Underlying Postural Tachycardia Syndrome.   This research study also discusses some of the possible causes of low blood volume in POTS patients, all of which seem to revolve around the kidney and autonomic neuropathy.

STEP 2
Make sure your doctor understands which POTS symptoms are probably caused or worsened by low blood volume.   Perhaps most interesting to POTS patients, the authors of the Vanderbilt study explain what happens to the body when blood volume is too low. The "reduction in effective circulating volume could trigger a cascade of perturbations associated with POTS. In the supine position, this hypovolemia may cause only modest or nonsignificant changes in heart rate and plasma norepinephrine. In the upright position, in the setting of gravitational blood pooling, the additional reduced volume could decrease the cardiac output and cause a reflex increase in sympathetic nerve activity. The result would be an increase in the upright plasma norepinephrine levels and an increase in standing heart rate, as seen in patients with POTS."

This is a fancy way of telling us POTSies what we already know from experience - when we are laying down, we feel kind of O.K. but when we stand up, all hell breaks loose. We get a rush of adrenaline, our heart rate skyrockets when we stand up and sometimes our BP drops. We get a flurry of symptoms that are caused by increased sympathetic nervous system activity and increased norepinephrine levels - like dizziness, shaking/tremulousness, and anxious feeling that isn't based on an emotional trigger, sweaty or flushed skin, dilated pupils, shortness of breath, etc., etc. All of this is releived quite a bit if we lay back down again.

So what does all of this mean?  Well, it means that if you have low blood volume and you figure out a way to correct it, maybe many of your POTS symptoms will be minimized or they may even totally disappear.  I'm not trying to give any of you false hope, because POTS is a chronic illness and it would be inaccurate to say you are going to wake up the day after a saline infusion and be cured, but if it makes you feel better, that's what matters.

STEP 3
If your doctor doesn't think you have low blood volume, because he's done a blood test and "everything looks normal," ask him to order a "Daxor blood volume test".  The basic blood test doctors usually use to check for low blood volume do not work on POTS patients, because those tests only look at the ratio of plasma to red blood cells.  As explained in the Vanderbilt study, the plasma to red blood cell mass ratio in POTS patients is normal because we are missing equal parts plasma and red blood cells (we are missing almost a third of our plasma and a third of our red blood cells).  The Daxor test is what they used in the Vanderbilt study.  It precisely measures the plasma volume, the red blood cell volume and the total blood volume.  The patient is given a very small injection of a radioactive isotope, Iodine-131 and the Daxor machine is used to analyze blood drawn at various intervals of time after the injection is given.  Most doctors offices will not have a Daxor machine.  You probably need to go to a large research hospital to get this test done.  The Daxor Corporation has an online list of medical centers that have purchased a Daxor blood volume measurement machine from them.

STEP 4
Make sure your doctor knows you have tried less invasive ways of increasing your blood volume first. Once your doctor understands that you have low blood volume, then he should be more willing to help you try to increase it.  Of course, the first lines of defense against low blood volume is increasing your salt and water intake and elevating the head of your bed.  The second line of defense is taking Flourinef, which helps you retain the water and salt you consume.  You should also make sure your vitamin levels are good, including your iron levels.  Iron deficiency anemia is very common in menstruating women, which also happens to be the same group of people most likely to have POTS.  If you are doing all of that, and it hasn't worked, or it's helping a little bit, but not enough, then it's time to talk to your doctor about trying IV saline therapy.

STEP 5
Remind your doctor that you have felt so much better after getting saline in the ER (assuming that's true) and that you cannot afford to go to the ER a few times a week just to feel better.

So many of us POTSies have been to the ER for various symptoms, maybe even before we were properly diagnosed with POTS, and it is very common for ER doctors to give us IV saline.

After 9 months of not getting an accurate diagnosis, I realized that every time I went to the ER and they gave me IV saline, I would feel amazing.  I literally was brought in by ambulance on a stretcher, unable to lift my head of a pillow, and after 2 or 3 liters of saline, I walked out of the ER feeling like a new woman. 

But visits to the ER just for saline are expensive and time consuming for the patient and a waste of hospital resources too.  And I doubt my insurance company was too happy about having to pay all those ER bills either.

If something makes you feel better, you should try to find the easiest, most affordable, most efficient way to do it.  Home IV saline therapy is definitely more convenient than running to the ER a few times a week, or even a few times per month.

STEP 6
Make sure your doctor knows that many other POTS patients have benefited from IV saline therapy. Besides me, I have found many other POTSies on the message boards and in chat rooms who have benefited from IV saline.  Some POTS patients just get IV saline once or twice a month, when their symptoms are really bad.  Other POTS patients, including me, have found that daily IV saline has really helped them.  I would be happy to speak with any doctor who wants to know about my saline therapy and how it has improved my POTS symptoms (objectively, measured by my doctor) and how much it has improved my quality of life (subjectively, measure by me).  There are also references listed below which confirm this.

I don't know of any statistics on how many POTSies are on saline therapy, and that's not surprising since the researchers barely know how many POTS patients exist.  Just because there is no hard data doesn't mean there aren't people out there doing it.  You just have to look for them.

STEP 7
Make sure your doctor understands that top POTS researchers are doing studies on saline therapy for POTS, because they think it works. There are ongoing research studies involving the treatment of POTS with IV saline, as well as past research studies that involved the use of IV saline in POTS patients.  None of the previously published studies says point blank "you should give your POTS patients 1 liter of IV saline everyday," but this should be enough material to give your doctor comfort in knowing he is not the first one to try IV saline for a POTS patient.  Your doctor can start IV saline therapy with you knowing that he will monitor you closely, and if there is no benefit seen in a few weeks, he may discontinue your therapy.  If there is a benefit seen, he should consider documenting and sharing it with other doctors so that information can be used for future POTS patients.

Here are the ongoing research studies:
1) Dr. Julian Stewart from the Center for Hypotension in Valhalla, New York (one of the top pediatric POTS experts) is recruiting patients for a new research study, and the recruitment ad says "[w]e believe that IV fluids and ORS [oral rehydration solution] can increase the blood volume to a similar extent and may reduce symptoms associated with OI and POTS." http://www.stars-us.org/news/news.html

2) Vanderbilt University, one of the best POTS research centers in the world, is currently recruiting POTS patients for a research trial on exercise tolerance after IV saline.

Here are some studies related to IV saline and POTS that have already been published:
Postural Orthostatic Tachycardia Syndrome: The Mayo Clinic Experience This Mayo Clinic study says, among other things,  "[w]e concluded from the results that midodrine and intravenous saline, when given in the short term, are effective in decreasing symptoms on tilt in patients with POTS."

As a side note, my own experience confirms this.  I had two tilt tests done, about 11 months apart, both times I was not on saline and I passed out almost instantly upon being tilted upright.  My heart rate skyrocketed and my BP dropped significantly as well.  During the first test, I was not on any drugs.  During the second test, I had been on increased oral fluids, salt pills and Flourinef, but that wasn't enough to prevent me from passing out.  I had a third tilt test after I was on the IV saline (1.8 liters per day) for about one month, still taking the same Flourinef dose.  I was tilted upright for 45 minutes and I did not pass out - not even close!  My BP dropped and my heart rate increased, but not that much.  I attribute the improvement in the BP and heart rate to the saline. 

Instead of just getting this benefit when you have a tilt test, why not get it everyday?

3) The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management IV saline for POTS is discussed under the "Non-Pharmacological Treatment of POTS" section of this article, to wit, "[a]cute blood volume expansion is effective at controlling the heart rate and acutely improving symptoms. Jacob et al. found that 1 liter of physiological saline infused intravenously over 1 hour decreased the orthostatic tachycardia from 33±5 bpm before the infusion to 15±3 bpm immediately following the infusion. The physiological saline was more effective at heart rate control than were treatments with either an alpha-1 agonist or an alpha-2 agonist. This treatment is not practical on a day to day basis as a medical setting is required to insert the intravenous catheter and infuse the saline. Recently, there have been reports of patients having regular saline infusions, typically 1 liter of normal saline every other day or every day. Many report an improvement in symptoms. However, there are not yet objective data to substantiate such benefit. Further, there is a risk of vascular access complications or infection. At this time, such therapy for patients with POTS should be considered cautiously."

4) Effects of Volume Loading and Pressor Agents in Idiopathic Orthostatic Tachycardia
Circulation.  1997; 96; 575-580
This is another Vanderbilt study, which compared the effect of IV saline, Clonodine, Midorine and a placebo on orthostatic heart rate changes and blood pressure changes on patients with Idiopathic Orthostatic Tachycardia (one of the older names for POTS).  The study found, "[s]aline infusion was most effective in blunting the orthostatic tachycardia in these patients (HR increase 32±5 before infusion and 14±2 bpm after infusion). Saline infusion also caused an orthostatic increase in SBP (-5±3 before infusion and 6±2 mm Hg after infusion, studies demonstrated that expansion of the intravascular volume by infusion of 1 L normal saline was an extremely effective (though very transient) means of improving the orthostatic tachycardia and orthostatic tolerance of our patient population. The likely mechanism is through the loading of baroreceptors, which would result in a reflexive lowering of sympathetic tone."  However, before you give this paper to your doctor, please note that it also says, without any analysis, that "administration of normal saline intravenously is impractical over the long term."  But keep in mind that this study was done by Vanderbilt in 1997 and now, in 2011, Vanderbilt is doing new studies on IV saline, so maybe they've realized that the benefits of saline therapy outweigh the practical difficulties.

5) Oral Fluid Therapy, A Promising Treatment for Vasodepressor Syncope
Arch Pediatr Adolesc Med.;1998;152:165-168.
This article is about using IV saline before a tilt test to predict whether oral fluid therapy would be a useful treatment for patients suffering from Vasodepressor Syncope (sometimes called vasovagal syncope, or as I like to call it, "POTS-lite").  It doesn't really offer than much insight into IV saline therapy, other than noting that fewer syncopal episodes occurred during tilt table test after patients had received IV saline, because the saline increased their intravascular volume.  And of course, this article is further evidence that all of us POTSies should try increasing our fluid and salt intake before looking into IV saline therapy.

6) Raised Cerebrovascular Resistance in Idiopathic Orthostatic Intolerance
This is another Vanderbilt study and the primary findings are, "in patients with IOI [idiopathic orthostatic intolerance-one of the older names for POTS], HUT [heads up tilt] causes a substantial decrease in cerebrovascular blood flow velocity. The decrease in blood flow velocity with HUT can be attenuated with interventions that improve systemic hemodynamics and therefore decrease reflex sympathetic activation."  In this study, the infused patients with saline (as the placebo) and two other drugs.  While the study is fairly complex, if you read it carefully you will notice that the saline performed almost as well as both of the drugs during the tilt test, without the bad side effects that one of the drugs caused.  Since the study was really trying to determine the cause of cerebral hypoperfusion, they don't talk about saline as a treatment or any other treatment, other than saying "the decrease in cerebral blood flow velocity can be attenuated with interventions that improve systemic hemodynamics."  As you can see from the results of the placebo in the study, those "interventions" include IV saline infusions.  Another finding of interest in this study, "[i]t is probable that in these patients, this sympathetic activation is reflexively mediated to compensate for excessive venous pooling with standing, compounded by hypovolemia."

Other Sources of Information Regarding IV Saline Therapy:
1) The Handbook of Clinic Neurology, Volume 74 - The Autonomic Nervous System, Part I
Here's a medical text book on the Autonomic Nervous System written by doctors saying that POTS and NCS patients can benefit from IV saline infusions (see the bottom left side of page 174).

2) Postural Tachycardia Syndrome (POTS): Clinical FeaturesThis is from Medscape, an online reference site for doctors, which says, "[o]rthostatic intolerance with low BP requiring repeat visits to the emergency room for intravenous saline infusions is uncommon but by no means rare."

3) CFIDS, a non-profit chronic fatigue group, states on it's website website that IV saline helps orthostatic intolerance.

4) Here is a quote from a doctor on a natural medicine website I thought was interesting. I believe it is a book excerpt:
"He has given intravenous saline to patients with postural orthostatic tachycardia syndrome (POTS) and reports that they feel substantially better after the volume load. This probably happens because the saline counteracts an overall reduction in blood volume in patients; essentially, they feel better because their veins and arteries are carrying the amount of fluid they were designed to carry (I will discuss this and other research on POTS in the next chapter). But Dr. Bell shares my view that saline alone might do as much as saline loaded with vitamins and minerals."  - Benjamin H. Natelson, M.D.

Those are the saline related references I have found so far. If you know of any, or if you personally have had success with saline therapy, please let me know by posting a comment to my blog.

STEP 8
Make sure your doctor knows that YOU understand the risks of having a PICC line or a chest port or regular IV infusions, and that you are willing to take the risk to see if you can get your quality of life back.  While millions of people have had PICC lines and chest ports over the years, there are serious risks involved with them.  You should become familiar with those risks before talking to your doctor about getting IV saline therapy, and then you should discuss those risks with your doctor too.  You and your doctor will have to weigh those risks against the possible benefit of IV saline therapy for you - and because there isn't tons of empiric data, you won't really know the benefit to you until you've tried it.  I guess you can note the benefit if you've had IV saline through a regular IV needle in your arm, like you often get in the ER.  If that made you feel awesome, then imagine how you'll feel having that everyday or several times a week.  If that didn't make a difference for you, then daily IV saline might not be worth the risks of having a PICC line or a chest port.

In deciding whether to go with a chest port or a PICC line, keep in mind that PICC lines are meant to be more temporary (a few months to a year at most), while chest ports can last a year or more (some people have them for years) depending on how often you stick a needle into it.  In order to preserve my chest port for as long as possible and to protect the skin that sits above the port from being damaged too much, I get my needle put in by a nurse once a week.  That is better than being stabbed 7 days a week with a needle and it will minimize wear and tear on the chest port itself, which can only be stabbed with a needle a finite number of times before you need a new one.  Putting in a new one means another surgical procedure, so that's why it's important to me to try to make it last.

Another benefit of having a PICC or chest port is that you no longer have to get stabbed with needles all the time for blood tests.  You can simply have the blood drawn out of the PICC or port tube, without any needles involved.  However, not all labs and nurses are qualified to draw from a PICC or a port, so you may have to go to make special arrangements for that.  My home care nurses, who come once a week to change my bandage, are qualified to draw blood from PICCs and chest ports, so I have my blood drawn by them rather than going to the lab like I used to.  The nurses at my hematologist's office are also qualified to draw blood from PICCs and ports, because they deal with blood tests and infusions on patients with ports and PICCs all the time. 

STEP 9
If your doctor is still reluctant to let your try saline therapy as part of your overall POTS treatment, ask them if they will let you have one or two liters of saline on just one day though a regular IV needle, and ask them if they will check your heart rate and blood pressure when you are standing up for 5 to 10 minutes, before and an hour after the saline.  If possible, ask if they can also check your V02 max (a way of measuring your oxygen consumption levels that helps measure your endurance or your overall cardiovascular fitness).  This usually requires a treadmill and a machine that measures your breath.

The point of this little science experiment is to show your doctor measurable results of improved heart rate and blood pressure when you are standing, after a saline infusion.  This is similar to the tilt tests in all of those research studies in which patients fainted less often and had improved heart rate and blood pressure during tilt testing after they received saline.

Before you do this, ask your doctor if the results are favorable, would he change his mind and allow you to have a trial of home saline therapy?  If he still says no, then don't bother with the experiment.  Find a new doctor who is more open minded and more willing to help you find treatments that improve your quality of life.

STEP 10
If you've gone through all of this, and your doctor still says no, I would ask the doctor to give you a clear explanation as to why he thinks saline therapy is not appropriate for you.  Is he just concerned about the risks of having a PICC or chest port?  Do you have some sort of other medical condition that could be worsened by saline therapy (kidney failure, high blood pressure, an allergy to something in the saline, a high risk of infections from an underlying medical condition, etc.).  Maybe he suspects your problem isn't low blood volume, and if so, you should ask him to explain why he thinks that.

When all else fails, if you truly believe that your POTS symptoms are being caused by or worsened by low blood volume, and if you've tried increasing your fluids and salt and you've tried Flourinef and probably a hundred other things, and you know you feel great when you get saline in the ER, then you may want to consider getting a second opinion or finding a new doctor.

It's very hard for us POTSies to find a doctor who know what POTS is, and even harder to find one willing to work with us to devise a treatment plan.  But you can never give up.  Just keep pushing until you get what you need to feel better.  You will get there someday!

As a final thought, I have been told by my doctors that I can't have saline therapy forever.  I understand that, but I'm hoping the saline will give my body the jumpstart it needs to get me out of bed (it has!) and more active (it has!).  I am in the process of signing up for Dr. Benjamin Levine's famed POTS exercise protocol, and I think the saline will help me be better able to tolerate the intense exercise regimen that awaits me.  Believe me, no one wants this chest port out of my body more than I do!  I look forward to the day when I can get it taken out and walk about without a needle hanging off my chest.  I also look forward to a day without POTS, or at least a day without any really bad symptoms.