Agent Orange - is there a POTS connection?

Is there a connection between POTS and exposure to Agent Orange? Maybe. The answer is not that simple.

I have noticed several POTS patients posting comments on web forums about one or both of their parents being exposed to Agent Orange during the Vietnam War. My father was exposed to it as well in Vietnam, so I started to look into it.

What really peaked my interest is that several of the conditions linked to Agent Orange exposure are the same as or similar to illnesses that are linked to POTS. For example, about 1/3 of patients with POTS have some form of peripheral neuropathy, and peripheral neuropathy has been conclusively linked to Agent Orange exposure. Another rare disease, amyloidosis, is also linked to both Agent Orange and POTS.

What is Agent Orange?
Agent Orange is the code name the US Military gave to a herbicide is sprayed in Vietnam during the Vietnam War, between 1961 and 1971.  It was called Agent Orange because it was shipped in large metal drums with an orange strip on it.  They also used Agent Blue (another chemical with a blue stripe on the container) and other "agent" colors, but Agent Orange is the one that became infamous because of its devastating health and environmental consequences.  It was used to destroy and strip the foliage from Vietnamese forests to hinder the Viet Cong (for you youngsters-they were the ones the U.S. was fighting).  As if that wasn't bad enough, the U.S. military also sprayed agent Orange on rural farms, to force the Vietnamese peasants to flee the countryside and migrate to U.S. military controlled cities, which in theory was supposed to deprive the Viet Cong forces of supplies and food.  Whether it did that is debatable, but the bottom line is that if you mother or father was anywhere in Vietnam between 1961 and 1971, they were almost certainly exposed to Agent Orange, maybe even multiple times.

Agent Orange is a mixture of two chemicals:
2,4,5-Trichlorophenoxyacetic acid (called 2,4,5-T)  and  2,4-Dichlorophenoxyacetic acid
Towards the end of the Vietnam War, it was revealed to the public that the 2,4,5-T was contaminated with TCDD and that TCDD caused many of the adverse health effects correlated with Agent Orange exposure.  TCDD is a form of dioxin and has been described as "perhaps the most toxic molecule ever synthesized by man."  It was produced as a by-product of the Agent Orange production process by the Monsanto Corporation.  Monsanto knew that its 2,4,5-T was contaminated with TCDD and informed the US government in 1952, years before the Vietnam War.  Monsanto is one of the largest agricultural companies in the world - besides their Agent Orange poison, they are also infamous for introducing the world to DDT (another deadly pesticide) and genetically modified food.

The U.S. government, after years of denying any ill health effects caused by Agent Orange, finally admitted that "acute and subacute" peripheral neuropathy can be caused by Agent Orange, along with a long list of other serious health problems. There is now a program under which Vietnam Veterans can apply for health care and financial assistance if they can prove they were exposed to Agenct Orange in Vietnam, and that they developed peripheral neuropathy within a year after their exposure. So if you're a veteran and you were exposed to Agent Orange and you developed peripheral neuropathy 10 years after you left Vietnam, you're out of luck in terms of getting help from the Veterans Administration.  The VA asserts that neuropathy that is persistent and does not improve is "chronic" peripheral neuropathy, which they claim is not caused by Agent Orange exposure in Vietnam.

Personally, I think this is baloney.  It took decades for impacted vets and their families to convince the government to do some research to connect ANY illness to Agent Orange exposure in Vietnam. The only reason chronic peripheral neuropathy hasn't been "proven" to be connected to Agent Orange is probably because no one has done any comprehensive studies on the number of Vietnam vets who have "chronic" peripheral neuropathy. Who would fund these expensive, long-term studies anyway? Surely not the big chemical companies who make these toxic chemicals (like Monsanto). And not the government, because those same companies use lobbyists to pressure government regulators and elected officials NOT to spend taxpayer dollars researching the negative health effects of the toxic chemicals they make their money off of.  So yes, this sounds like a big conspiracy theory, but sadly that's the way Washington works sometimes.

Besides acute and subactue peripheral neuropathy, there are many other serious health problems that the federal government does acknowledge ia caused by exposure to Agent Orange. As of April 2011, that list includes:

AL Amyloidosis - A rare disease caused when an abnormal protein, amyloid, enters tissues or organs.  Amyloidosis can be an underlying cause of POTS in some patients.

Chloracne (or Similar Acneform Disease) - A skin condition that occurs soon after exposure to chemicals and looks like common forms of acne seen in teenagers. Under VA's rating regulations, chloracne (or other acneform disease similar to chloracne) must be at least 10% disabling within 1 year of exposure to herbicides.

Chronic B - cell Leukemias-A type of cancer which affects white blood cells. VA's regulation recognizing all chronic B-cell leukemias as related to exposure to herbicides took effect on October 30, 2010.

Diabetes Mellitus (Type 2) - A disease characterized by high blood sugar levels resulting from the body’s inability to respond properly to the hormone insulin.  Note that diabetes can cause autonomic neuropathy, which can be an underlying cause of POTS.

Hodgkin’s Disease - A malignant lymphoma (cancer) characterized by progressive enlargement of the lymph nodes, liver, and spleen, and by progressive anemia.

Ischemic Heart Disease - A disease characterized by a reduced supply of blood to the heart, that leads to chest pain. VA's regulation recognizing ischemic heart disease as related to exposure to herbicides took effect on October 30, 2010.

Multiple Myeloma - A cancer of plasma cells, a type of white blood cell in bone marrow.

Non-Hodgkin’s Lymphoma - A group of cancers that affect the lymph glands and other lymphatic tissue.

Parkinson’s Disease - A progressive disorder of the nervous system that affects muscle movement. VA's regulation recognizing Parkinson's disease as related to exposure to herbicides took effect on October 30, 2010.  Interestingly, Parkinson's Disease has many overlapping symptoms with the various forms of autonomic neuropathy and dysautonomia.

Porphyria Cutanea Tarda - A disorder characterized by liver dysfunction and by thinning and blistering of the skin in sun-exposed areas. Under VA's regulations, it must be at least 10% disabling within 1 year of exposure to herbicides.

Prostate Cancer - Cancer of the prostate; one of the most common cancers among men.

Respiratory Cancers - Cancers of the lung, larynx, trachea, and bronchus.

Soft Tissue Sarcoma (other than Osteosarcoma, Chondrosarcoma, Kaposi’s sarcoma, or Mesothelioma) - A group of different types of cancers in body tissues such as muscle, fat, blood and lymph vessels, and connective tissues.

As you can see, several of these illnesses were only recognized by the VA to be connected to Agent Orange exposure in 2010 - that's more than 30 years after the Vietnam war ended! Heaven only knows how many brave men and women who served out country suffered and died from these terrible illnesses without getting any help from the government that intentionally and knowingly exposed them to these deadly poisons.

What's worse, is that it is clear that Agent Orange does not only impact the men and women exposed to it - it effects their children as well. This can be seen in the thousands upon thousands of Vietnamese children who have been born with all sort of horrible birth defects since the U.S. government sprayed over 20 million gallons of Agent Orange throughout the Vietnamese countryside.

While out government has been reluctant to admit there are any ill health effects attributable to Agent Orange, some progress has been made. In 1996 President Clinton launched an initiative to compensate the children of Vietnam veterans exposed to Agent Orange who were suffering from spina bifida, after research showed a strong correlation between exposure and this very rare and sometimes fatal disease. The law was finalized as of 2003.

The VA also recognized a long list of birth defects in children born to women who served in Vietnam and were exposed to Agent Orange, including but not limited to:
Spina bifida
Achondroplasia
Cleft lip and cleft palate
Congenital heart disease
Congenital talipes equinovarus (clubfoot)
Esophageal and intestinal atresia
Hallerman-Streiff syndrome
Hip dysplasia
Hirschprung's disease (congenital megacolon)
Hydrocephalus due to aqueductal stenosis
Hypospadias
Imperforate anus
Neural tube defects
Poland syndrome
Pyloric stenosis
Syndactyly (fused digits)
Tracheoesophageal fistula
Undescended testicle
Williams syndrome

As more research is done, and as veterans and their families continue to advocate for proper medical treatment and compensation of Agent Orange victims and their children, it is likely that more diseases and birth defects will be added to this list.  Maybe POTS will be on this list some day, or at least the form of POTS that can be attributed to autonomic neuropathy.

If you have a parent who served anywhere in Vietnam or may have otherwise been exposed to Agent Orange (some people were exposed in chemical factories, storage facilities or military bases in the U.S.), please contact me by posting a message below.  I will not publish your post unless you say it's OK. You may also want to register with the independent non-profit group that maintains a National Birth Defect Registry of Agent Orange connected birth defects. Basically, how it works is that you enter data about any health problems seen in your family in children whose parents were exposed to Agent Orange. Concerned scientists then use this data to advocate for further research into the more prevalent illnesses that show up in the survey and urge the government to admit responsibility if a link between a particular health problem and Agent Orange is established.  This is the groundwork needed to get the VA to acknowledge other diseases are caused by Agent Orange, which will lead to those suffering with Agent Orange illnesses to be able to get the medical care they need from the government.

If you scroll down a few pages in their Birth Defect Presentation, you can see that there are many more illnesses more prevalent in the children of Vietnam veterans compared to non-veterans, than those that are currently recogized by the US government.  Everything from dyslexia, to rare cancers to asthma - and this isn't surprising since dioxin, which is found in Agent Orange, is considered by scientists to be the most toxic chemical known to man.

My dad had significant exposure to Agent Orange when he served in the U.S. Military in Vietnam. Dad has had a long list of serious health problems since he returned from his tour of duty over 30 years ago: cancer, loss of a kidney, heart problems, strokes, asthma and peripheral neuropathy. My mom seems to be healthy as a horse, so we always joked that my health problems must have come from dad's bad genes.

Well, I'm not joking about it anymore. I was diagnosed with "profound autonomic neuropathy" nine months after the onset of my POTS symptoms. To date, no doctor has been able to come up with any explanation for what may have trigger my neuropathy. And the autonomic neuropaty I have seems to be worst in my lower legs and hands, which follows the typical pattern of "peripheral neuropathy." Peripheral neuropathy just refers to the fact that the damaged nerve fibers are peripheral to your central nervous system - that is, peripheral nerves are those other than the ones in your brain and spinal cord. Peipheral neuropathy typically develops at the further points of your nervous system first - your toes, then your ankles, then your calf, then you thigh, etc. At the same time, it can first occur in your fingers, then your palm, then your wrist, etc.

Peripheral neuropathy commonly impacts your sensory nerves, which control your sensation of hot/cold, pressure and pain. My dad has peripheral neuropathy that affects his sensory nerves. My sister and I noticed that our dad's feet were numb when we were little kids in the 1980s. We would tickle our dad's foot when he was relaxing on the couch watching a hockey game, and he wouldn't even feel it. Then, because we were curious rotten little kids, we would stick small sewing needles in his toes, and he still couldn't feel it. He was like a human science project to us.  We didn't know it at the time, but dad couldn't feel the needle in his toes because he had peripheral neuropathy of his sensory nerves.

Peripheral neuropathy can also impact your motor nerves. These are the nerves that control your physical movement and your muscles. These nerves help you walk and point your toes and grasp things with your hands, like pens and utensils. If your motor nerves are damaged, you can lose your ability to walk or use your other muscles. Since peripheral neuropathy usually starts at the furthest nerves form the brain, you are most likely to feel peripheral neuropathy of your motor nerves in your hands and feet. We are not sure if my dad's peripheral neuropathy is impacting his motor nerves at this point, but I suspect that is may be impacting him because he often trips when he is walking and sometimes his knees just give out on him and he trips or falls over.

Finally, peripheral neuropathy can also impact your autonomic nerves.  These are the nerves most often involved in POTS.  POTS is a form of dysautonomia, and dysautonomia means dysfunction of the autonomic nervous system.  As I mentioned above, they found that I had "profound autonomic neuropathy" after then examined a skin biopsy from my left leg.  I think my dad may have some autonomic neuropathy as well, because he has heart arrhythmia problems just like me, and breathing problems just like me. I'm hoping we can get a small tissue biopsy from his leg, like I had, so we can identify exactly what types of nerves are impacted by his neuropathy, and to what extent his nerves are damaged.

So we're not positive that Agent Orange can cause POTS, but there is significant evidence pointing in that direction.  If Agent Orange causes peripheral neuropathy, and peripheral neuropathy can be the underlying cause POTS, it is very possible.  Only time and more research will reveal the truth. 

Funding POTS Research and Organizing the POTS Community

Most of us POTSies know how hard it was to get diagnosed, because most doctors have never heard of POTS.  And once we were finally diagnosed, it was probably really hard to find a doctor who really had expertise in treating POTS.

We need to raise POTS to the same level of public awareness as other chronic illnesses like Multiple Sclerosis and Parkinson's Disease.  There are more people currently living with POTS people in the United States than Multiple Sclerosis and Parkinson's combined, yet hardly anyone, including medical professionals, has ever heard of POTS.

Estimated Prevalence of Diseases in the United States:
Multiple Sclerosis (M.S.) - 250,000 people
Parkinson's Disease - 500,000 people
Postural Orthostatic Tachycardia Syndrome - 1,000,000 people

Where are the research dollars going?
Check out this graph prepared by the National Institutes of Health (NIH), which summarizes the amount of NIH funding going towards specific diseases in 1996. The NIH is the source of almost all federal research dollars spent on medical research. Notice that POTS and other forms of Dysautonomia don't even make the list. Interestingly, ear infections (Otitis Media) get 10 million dollars a year in federal research dollars.

Now don't get me wrong, I think all of these health issues deserve our serious attention and substantial government funding. I'm not saying take some money we are spending on ear infections and spend it on POTS instead. I'm saying, take some of the money we are wasting on corporate tax breaks for insanely profiting companies like General Electric and spend it on POTS. General Electric had $14.2 billion dollars in profits in 2010, and received a $3.2 billion dollar tax benefit - as if they needed the extra money with the billions in profits they made in just that one year. In fact, we don't even need the whole $3.2 billion from GE's corporate welfare... oops, I mean tax breaks, to study POTS. Maybe just 1% of that, which is $10 million dollars. And us POTSies have probably funded a few million dollars of GE's profits since they make most of the MRI machines in the world.

Why doesn't POTS/dysautonomia make the NIH list even though there are more people with POTS than some of the other well known chronic diseases? I had one doctor tell me "it's because those other disease are more serious." Really? I know people with M.S. who can run marathons (and god bless them for that!), but I don't know any POTS patients doing that. I know many people with diabetes who can get up everyday and go to work and live a totally normally life. I don't know many POTS patients who can go to work and live "normally." So I'm not saying there aren't people with M.S. and Diabetes who are seriously suffering (I know some of them too), but if we based federal research funding on suffering alone, then things like Familial Dysautonomia (Riley-Day Syndrome) should be at the top of the list, because those poor kids only have a 50% chance of reaching age 30, and it's not an easy 30 years for them if they do make it that long.

Federal medical research spending, like everything else in Washington, D.C., is based on who makes the most noise. You know the saying - "the squeeky wheel gets the grease." There are some very well greased wheels in the medical arena. Well known diseases like cancer and AIDS get tons of funding. While diseases the public is not talking about, like POTS, don't get much. Why is this? Well, you've all probably heard of the American Cancer Society and the AIDS Foundation, but how many of you, before you had POTS, ever heard of a non-profit working on POTS awareness? That's what I thought.

We POTSies haven't organized ourselves into a lobbying and educational force to be reckoned with like the AIDs groups, and the cancer groups and the M.S. groups... yet. There are some wonderful people who have started to get the ball rolling. The good folks at the Dysautonomia Information Network (DINET), the Dysautonomia Research Foundation and the Dysautonomia Foundation (which focuses on Familial Dysautonomia) have laid the groundwork by starting non-profits and putting information out into the world to try to raise awareness about POTS. But we clearly need a bigger better effort before everyone in America knows what POTS is, just like everyone in America knows what M.S. is.

Our POTS/Dysautonomia organizations aren't raising enough money for research. This is not a criticism, just an observation. I have spoken with the head of one of the larger POTS non-profits, and I won't say which one, but they only raise about $5000 per year. This is probably what the MS Society spends on postage every month. For example, in 1999 the National Multiple Sclerosis Society gave out $22.5 million dollars in research grants. Add this to additional funding sources, like the National Institute of Health and smaller private organizations and non-profits that work on MS issues. So how can a disease that only impacts 250,000 people raise so much money for research? Because the are WELL organized and WELL funded.

How do we get the POTS community to be well organized and well funded?
I think we should look at what other successful health based non-profits have done, and try to learn from them.

I also think we need to reach out to the pharmaceutical companies. There has to be someone making money on us, with so many of us taking Flourinef and Midorine. And those compression stocking companies probably make money off of POTS too.  Maybe a health insurance company would be interested in sponsoring us - if we find a cure for POTS, maybe they won't have to pay for so many ER visits for us anymore. In America, your cause is legitimized when you have a big national corporate sponsor. Sad, but true.

So out of the 1,000,000 POTS patients, someone has to have a relative or a friend working at some big pharma company, a health insurance company or one of those medical compression stocking companies.  And even if we don't have a direct connection, there is no reason we shouldn't ask for funding anyway.

Why isn't the POTS community organized yet?
Well, to be fair, POTS was only given a name in 1993, so it's not like patients could rally around a cause when the cause didn't even have an agreed upon name before that.

Also, about half of the 1,000,000 American's who are estimated to have POTS, don't even know it.  They are either undiagnosed or misdiagnosed with something else.  So that leaves 500,000 of us to get to work and organize.

I also think part of the reason it is hard to organize POTS patients is because so many POTSies are young teenagers and 20 somethings, and maybe they don't know how they can get involved. It's also hard to get involved when you are stuck laying in bed all day feeling like crud. But with the internet, now you can get involved even if you're stuck in bed. Teens are probably better than anyone at using social networking to organize for a cause, so get to work. Make a blog, start a Facebook group, start a mass mailing to your Senators asking for NIH funding.  And your parents, other relatives and friends can help us organize too.

What should we do?
Here are some things I'd like to see happen in the POTS community:
1) a targeted effort to reach out to cardiologists, neurologists and family doctors on how to diagnose and treat POTS - since I think most POTS patients probably end up at one of these doctors when they first get sick and are searching for a diagnosis.

2) more media coverage of POTS - any of us can do this! call your local hometown newspaper, your school newspaper or your local TV station. Tell them you are trying to raise awareness about an unknown disease that affects 1 million people in the U.S. See if they will interview you. Show them the recent Wall Street Journal article on POTS and Dr. Levine's NASA research.

3) reaching out to medical schools and asking that POTS diagnostic criteria, appropriate tests and treatment be included in the curriculum

4) providing research funding for the existing POTS research centers like Mayo Clinic, Cleveland Clinic, Case Western, Vanderbilt and Dr. Levine's team in Texas

5) providing funding for medical students and residents to train in the field of autonomic disorder - we need more doctors working on this!

6) providing grants for educational conferences and training seminars regarding POTS and related forms of dysautonomia, to encourage existing doctors to learn more about the subject

7) providing funding to doctors willing to open up POTS speciality centers in regions that don't currently have one - so all of us don't have to drive half way across the country to see a POTS expert.  This funding could be used to train doctors willing to open up POTS/autonomic speciality centers in new regions of the country and possibly to help them purchase special testing equipment (such as Daxor blood volume testing machines, Thermoregulatory Sweat Testing Machines, QSART, hemodynamic test equipment, tilt tables, etc.)

That's just off the top of my head.  I'm sure if we got a bunch of POTS doctors and patients and caregivers in a room, we could think of more things that need funding and more ways to make it happen.

Perhaps we need a National Conference on POTS.  I know the doctors have medical conferences all the time, but I'd like to see one that involves patients as well.  This could be a group brainstorm on how to get POTS the attention it deserves.

Please send me your thought on this.  Would you be interested in participating?

POTS MENTIONED IN THE WALL STREET JOURNAL!

Dr. Levine's POTS exercise protocol, based in part on his work with NASA astronauts, has received press coverage in the Wall Street Journal!


This is good news for POTSies.  The more media coverage POTS receives, the easier it will be to get funding for desperately needed medical research into the underlying causes of POTS and viable treatments.


I have been waiting several weeks to hear back from Dr. Levine's team, to see if I can be accepted into their POTS exercise research program.  Stay tuned...

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?

2) 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."

3) 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.

4) 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.

5) 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.

Does EVERYONE have POTS?

My Aunt was just at the airport in New Jersey, and she met a young lady in a wheelchair while waiting for the plane.  She started talking to this young lady, and found out that she was on her way to Mayo Clinic to be seen for POTS.  My Aunt immediately called me to tell me about this young lady.  She was form Texas and had participated in Dr. Levine's exercise protocol.  Small world.  I am on the wait list for Mayo Clinic and I'm waiting to hear back from Dr. Levine's team about getting me enrolled in their second round wide-scale exercise study.

A few weeks ago, my mom's friend calls from her office, to tell me that her co-worker's niece was just diagnosed with POTS and doesn't know what to do.

My neighbor has a friend who's son was diagnosed with POTS.

My doctor think she had a patient a few years ago who had POTS, although the patient was never diagnosed with POTS because no one had ever heard of it.

This is so strange.  I never heard of anyone having POTS for the first 32 years of my life, and then I hear about 4 people within a few months of my diagnosis!  No to mention the hundreds of people I've "met" on the internet who have it.  Some from Long Island too.

I'm sure it's just a coincidence, and likely as more people are diagnosed, more doctors are going to learn about it, which means that even more people with get an accurate diagnosis.  I try my best to tell every doctor and nurse I meet about it, so that next time they see a patient with my weird symptoms, maybe they will think twice before sending them home without a diagnosis.

If every person diagnosed with POTS in the US (500,000 and counting) tells 10 people about POTS, and really takes the time to explain it to them, POTS can become a "well known" illness in no time.  

Saline Therapy for POTSies?

For the first nine months of my illness, I was popping in and out of the Emergency Room after fainting episodes with severe breathing problems, vertigo, tachycardia, tremors and a host of other symptoms.  We didn't know it was POTS, but we did know that whenever they gave me intravenous saline in the ER, I would feel somewhat better for about 24 hours.   IV saline seemed to lessen my tachycardia, increase my blood pressure, increase my energy levels, lessen my shakiness and tremors, lessen my chest and neck pain, and lessen my breathing difficulties - not completely, but just enough that I wanted more saline whenever my symptoms got really bad.

After nine months of bouncing around to dozens of specialists and emergency rooms, we figured out I had POTS.  I spent about 2 months in the hospital, on IV saline for most of that time.  When I finally went home, I really missed the "alive" feeling I got from the saline, so I started to ask my doctors to let me try it at home.  Most said no, because it wasn't "proven" to improve POTS symptoms.  I argued with the nay saying doctors that we didn't need a fancy research study to know that it improved MY symptoms, especially since they could objectively measure my improved heart rate, blood pressure and respiration rate.  There are also a handful of medical journals that mention IV saline being used as therapy for POTS patients, in particular hypovolemic (low blood volume) POTSies, which my doctors think is the type of POTS I have.  My rational argument did not get me anywhere.

I ended up in the hospital again a few months later, this time because my gastrointestinal symptoms were so bad that I couldn't eat or drink anything without choking, puking and having terrible breathing problems.  After losing 15 pounds in about 2 weeks, I finally ended up back in the ER and my doctor admitted me to the hospital to give me IV saline and to try to figure out why my GI system was going haywire.  She saw how much the saline improved my symptoms and allowed me to walk around a little bit more, instead of laying in bed 24/7.

We also noticed that regular saline (which is just medical grade purified water and sodium chloride) seemed to trigger hyperventilation attacks in me.  We don't know why this was happening, but I suspect it was some sort of acid-base imbalance in my blood due to the Flourinef I was on, which severely alters your electrolyte levels, my chronic dehydration since I couldn't drink enough fluids and had lots of diarrhea due to the GI problems.  When my doctor switched me to 5% dextrose + .9% sodium chloride, the hyperventilation pretty much stopped happening right away.  A one liter bag of 5% dextrose + .9% sodium chloride saline provides you with a whooping 9000 mg of sodium chloride per day.  This is way too much salt for normal people, who are supposed to consume 3,000 mg of salt per day or less, but this is great for most POTSies, because POTS doctors say we should try to get 5,000 mg to 10,000 mg of salt (sodium chloride) per day [Note - if you have POTS with high blood pressure, do not increase your salt intake without consulting with your doctor first].

When I was in the hospital, after awhile, the veins in my hands and arms started to collapse from all the IV catheters, which only last a few days and then have to be replaced in a different spot.  The would put an IV line in the top of my hand, and within the hour my vein would collapse and the IV fluid would start filling up my hand.  This hurts like hell, doesn't get you the hydration you need, and can cause tissue damage or infections if the IV line is not removed quickly.  That's why they won't let you leave the hospital with a regular IV line in your hand or arm.

Since I didn't want to live in the hospital forever and I didn't have any good veins left to get saline or to give blood for tests, my doctor suggested that I try a PICC line (Peripherally Inserted Central Catheter) in my arm, so I could receive saline infusions at home and have a painless and easy way to draw blood for all the lab tests I was getting every week.

A PICC line is a very narrow flexible tube (called a lumen) that is inserted in a large vein in your arm and it extends up your arm through the vein towards your heart.  The end of the lumen rests in a very large vein just at the top of your heart.  The other end of the lumen comes out of a tiny hole in your arm and has an IV like cap at the end of it that can be used to draw blood out of the line (without any needle touching you) or to infuse saline and other medicines if needed (also without any needles touching you). The length of the lumen depends on how large of a person you are.  Mine was 40 cm long.

Despite the risks, it seemed like a good idea because it would allow me to (hopefully!) start to function again, but I was scared to get a PICC line.  I had the unfortunate circumstances of hearing a hospital roommate get one inserted 'bedside' by an IV nurse, without any Novocaine to numb her arm or any pain meds.  She made the most awful blood curdling scream you've ever heard and she cried for about 3 hours afterwards.  And because of nerve damage associated with my POTS, I am more sensitive to pain than I used to be, so even a tiny needle stick feels like a huge knife in my arm.  I was dreading what a larger, long tube, would feel like getting inserted towards my heart.

Not only could it really hurt to get it inserted, I also knew there were serious risks associated with PICC lines.  You can get serious blood infections or blood clots, both of which are very serious medical problems and both of which could be fatal.  About 20% of people who get blood infections die.  PICC lines can also attach themselves to your vein and cause venous spasms or a tear in your vein, which could lead to internal bleeding.  Your heart could get irritated if the line is not placed properly or if it is too long or if it becomes dislodged.  The lumen could break apart in your arm and get whooshed by your fast moving  blood right into your heart, which could kill you almost instantly.  All of these scenarios are pretty rare, but these are serious risks not to be taken lightly.

I found out from the awesome girl who was in the hospital bed next to me, who had a dual lumen PICC line in her arm for IV antibiotic therapy, that you could get a doctor to give you a shot of Novocaine in your arm before they inserted the lumen, to help minimize the pain.  Apparently the nurses who do it at the bedside are not allowed to give you a Novocaine injection or any numbing agent (at least in the two hospitals I was in).

So I decided to wait for a radiologist to be available to do the procedure under fluoroscopy, with the Novocaine shot to help numb my arm.  In hospital lingo "under fluoroscopy" means that the radiologist or surgeon uses an x-ray machine that takes a continuous x-ray image and displays it on a big TV screen, so the he can see where the lumen is going as he inserts it into your vein.  I figured this was probably a little safer than letting a nurse do it at my bedside, since the nurses only use a sonogram machine AFTER they have inserted the PICC lumen, to see if it is in the right place above your heart.  The radiologist can also add a few stitches to hold the lumen in place, to minimize the risk that it will get puled out or pushed further in towards your heart, and I don't think nurses do that bedside.   So basically, I would advise anyone who is getting a PICC line to have it inserted under fluoroscopy by a radiologist or surgeon who can give you a Novocaine shot before hand.  They usually only do the stitches if you are expecting to have the PICC line for more than a few weeks.

Well, even with the Novocaine I screamed bloody murder the whole time they were inserting it, but that's probably just because of my increased pain sensitivity due to my neuropathy.  I felt all 40 cm of that lumen getting pushed through my vein in my arm and chest - YUCK!  The radiologist told me he'd only had one other patient feel it like out of the hundreds of PICC lines he inserted over the years.  Making matters worse, he inserted it once, and decided after looking and the x-ray image that the lumen was too long and since he didn't want it to irritate my heart, he pulled it all the way out, shortened it and put it back in a second time.  I let the f-bomb fly out of my mouth more than once during that procedure, but the radiologist was cool and told me to just let it out.

After it the PICC was fully inserted, my armpit was sore for about a week, and I kept feeling the lumen in the middle of my chest - possibly because he had bumped my vein above my heart pretty hard the first when he inserted the PICC line that was to long, or possibly because my body is just way too sensitive to any sensation, so this little tube was irritating my veins.  The soreness in my armpit was barely noticeable by the second week.  I made a point of using the arm just as much as my other arm, because I didn't want it to get weak just because it had a PICC line in it.

My doctor ordered a home care company to send a nurse to my house once a week to change the dressing around the PICC line entry site (the tiny hole in my arm).  The first week, the home care nurse also taught me and my husband how to flush the line to prevent blood clots and how to do the saline infusion on my own.  It was pretty easy once I did it a few times on my own.

I had the PICC line for about about 3 and a half months, getting 1-2 liters of 5% dextrose + .9% sodium chloride saline every day.  It allowed me to feel good enough to start doing exercises, which I needed to do to gain back my strength after laying in bed on most days for about a year.  It also allowed me to sit up and stand up for longer periods of time.  I managed a few hours in my greenhouse on one good day, and a few hours in my garden on another good day.  I made slow progress, but after 3 months I was able to go into a store without my wheelchair and oxygen (left those in the car just in case) and was able to walk around, albeit a bit shaky and short of breath, for almost an hour.  This was a monumental achievement, since I hadn't been to a store without a wheelchair in over a year and I hadn't stood up and walked for a hour since the first day I got sick 15 months prior.

Just as I thought I was really making progress, the day after my glorious one hour shopping adventure, the arm I had my PICC line in started to turn grayish purple, like it was swollen with blood that didn't have much oxygen in it.  I called my doctor and my home care nurse came to check it out.  They sent me to the emergency room to check for a blood clot or an infection, and to get the PICC line removed.  I was pretty scared, because I knew how serious it was to have a clot or an infection in a large vein that leads directly to your heart.  We all suspected a blood clot, including the ER doctor, but they did an MRA (an MRI that looks at your blood vessels and heart) but they didn't find a clot.  The ER doctor was a jerk; she refused to remove the problematic PICC line and tried to send me home with my Barney colored purple arm.  Thankfully we got a hold of my family doctor at some ungodly hour of the night and she admitted me to the hospital for further evaluation and to get the PICC line removed ASAP - the next morning.  A doctor removed the PICC line from me as I laid in my hospital bed.  It can be dangerous to remove the PICC line if there is a clot, but it had to come out.  He removed it gently, but I still felt an uncomfortable, but not painful, sensation in my armpit - like my vein was attached to the PICC line lumen and when he pulled it out, he tugged on my vein too.  The doctor said that usually doesn't happen, which is of course why it happened to me.

I stayed in the hospital waiting for further evaluation for a possible blood clot, the results of the blood tests they did in the ER looking for an infection (which take 48 hours to get back) and to get a chest port inserted since I still needed the IV saline every day to function.  If I learned anything from my PICC line experience, it is that you should have an emergency plan set up with your doctor on what you will do if you have a complication - how will you get the PICC removed if needed and how will you get the hydration of meds you need everyday when the PICC is removed?  Better to have a game plan in advance that to have to figure it all out at 3 am on a Sunday night when the hospital has a bare bones staff and you can't find your doctor.

I had the chest port inserted yesterday and I should be out of the hospital in 2-3 days.  Many doctors have told me that a chest port is better than a PICC line if you are expecting to need it more than a month or two.  Lots of people have chest ports that last a few years, while PICC lines are usually used for one year or less (and some doctors say 3 months or less).

A chest port insertion is more serious and complicated than a PICC line insertion.  A chest port insertion procedure usually involves the surgeon inserting a port, about the size of a large soda bottle cap, under the skin of the upper chest area, with a flexible silicone tube that connects the port to a large vein nearby, commonly the jugular vein the neck.  There is usually one incision to insert the bottle cap sized port and a second incision a few inches above that to connect the port tube into the a large vein near the neck.  Some ports are placed lower on the chest, and some women prefer the port to be placed along the bra line under under the breast, to hide the scar better.  I asked for this, because I like to wear bikinis and tanktops in the summer, but my surgeon said the upper chest area is less likely to cause complications since there is more tubing involved in the lower chest port placement and there is more likely to be irritation of the skin covering the port if it us under your bra or often rubbed against when you lay on your side to sleep.  He also said the surgery is more invasive and the recovery takes longer from the lower port insertion, so I sucked it up, and traded my vanity for a safer, less painful option.  Hopefully the port will allow me to feel good enough to hang out at the beach in my tiny bikini, and once the incisions have fully healed, you can shower and swim normally with a port - which you can't do with a PICC line.

The chest port sits under the skin, and a nurse or doctor can then get blood or give fluids or liquid medication into the port without significant pain for the patient.  If you get daily infusions, like I do, they can stick a small extension tube through the skin that covers the port and change that once a week, rather than piercing the skin that covers the port everyday.  Plus, you can be taught how to do the infusions yourself if you have the extension tube.  When you are not using the port, they inject a small about of heparin into it, and it can stay like that for several weeks.  If you weren't using it longer than that, you can just flush it with heparin every few weeks.

In my situation, they put me under "twilight" sedation to install the chest port.  Patients under twilight sedation can respond to simple directions the surgeon gives them, but they are relaxed and usually don't have any memory of the surgery.  Apparently as soon as the surgeon began to inject me with Novocaine, to numb the area where he was going to cut me open to insert the port, I freaked out, ripped off the safety straps that tied me down to the operating table, and I grabbed the surgeons arm so hard he almost stabbed himself with the Novocaine needle.  I am not a violent person, and I have no memory of doing this, thank goodness!  The doctors said it took three guys to hold me down.  They think I did it because I am so pain sensitive and the Novocaine needle hurt me so bad that I fought back out of pure instinct t protect myself.  Luckily, no one was hurt besides me.   I felt so bad about this when the doctor told me about it, and I profusely apologized, but he just laughed it off as 'all part of the job.'  He even complimented me on my physical strength, which I guess isn't so bad after a year in bed, since it took 3 guys to hold me down.  I am strangely proud of that.


After I went bonkers, they increased the anesthesia they gave me from just "twilight" sedation to fully knocked out anesthesia, which required the emergency insertion of breathing tube to keep my lungs functioning properly.  When you are deeply sedated under anesthesia, your lungs can stop working properly, so that is why they put a tube into your lungs, to pump air in and out.  The breathing tube is big and it gives you one hell of a sore, scratchy throat when you wake up from the surgery.  Since I started a brawl in the operating room, and since they had to have three men physically hold me down as the increased anesthesia set in, I ended up with a killer sore throat, terrible whiplash in my neck, and some odd bumps and bruises you don't normally get from a chest port procedure.  It should all go away in a few days, and then I can back to recovering from POTS, instead of recovering from an operating room brawl.  Don't let my experience deter you from considering a port.  The surgeon told me that this rarely happens, which, as I said earlier, is why it happened to me.  24 hours after the surgery, the surgeon removed most of the bandages and the neck incision sealed up well.  The chest port bandage should come off tomorrow or the next day, so we can see if I am ready to go home from the hospital.

So now that I've scared the crap out of you about all of this, I thought I might share with you some info you can give to your doctors if they are reluctant to let you try to IV saline.  Check out my In Support of Home IV Saline Therapy page for medical journals and research studies that may help you convince your doctor to let you try out IV saline at home.