Dec 31, 2010

New Year's Resolutions from a POTsie

Happy New Year to all of you POTsies out there.  I'm laying in bed, hoping I have enough energy to stay up until midnight, since I don't want to break my 32 year record of seeing the Times Square ball drop (several times in person).  This is the time of year when we should be grateful for what we have and hopeful for what the year ahead holds for us.

With that in mind, here are my New Year's Resolutions for 2011:

1. Eat super healthy, gain some muscle weight, don't forget to drink 3 liters of fluid per day and get more than 3,000 mg of sodium per day.

2. Try to get a little bit of exercise everyday, starting with exercising in bed and gradually increasing my tolerance levels to the point where I can walk 1 mile without passing out.

3. Keep researching for answers & solutions and don't give up!

4. Keep looking for the best doctors and don't waste energy being mad or upset about doctors who don't want to help or aren't able to help me.

5. Enjoy every minute of every day I get to spend with my loving family, my friends who stuck by me and my dog who never leaves my side (she even jumped onto the ambulance stretcher as they were rushing me away to the ER once).

6. Be thankful for the good things I do have in my life (see #5).

7. Smile and try to have fun at least once a day, even on the worst days.

8. Find my sense of humor that I seem to have lost.

9. Start that Beach Plum orchard I've always wanted to plant in my yard.

10. Share my experience with as many people as possible to help raise awareness about dysautonomia and POTS.

Dec 27, 2010

Dictionary of POTS Terms

Here's a plain English explanation of some of the fancy medical terms you may want to learn if you or someone you know has POTS:

Hypo - the term "hypo" at the beginning of a medical term means "under" or "low."  Examples:
hypotension - low blood pressure
hypovolemia - low blood volume (in simple terms: not enough blood)
hypoxia or hypoxic - low oxygen
hypochloremia - low blood chloride

Hyper - the opposite of hypo; used at the beginning of a medical term meaning "over," "high" or "too much" of something.  Examples:
hyperglycemia - high blood sugar
hyperkalemia - high blood potassium
hypernatremia - high blood sodium

Most of the "hypo" medical terms can be changed to the "hyper" terms.  Examples:
hypotension - low blood pressure
hypertension - high blood pressure

emia - used at the end of a medical term means "of the blood."  Examples:
hypoglycemia - low blood sugar
anemia - low red blood cell count

Syncope (pronounced sink-o-pea)
Partial or complete loss of consciousness with interruption of awareness of oneself and ones surroundings. (plain English - fainting, blacking out, passing out).

Some people get one or more warning signs from their body that they are about to faint.  For example, my legs start to feel heavy, and then as though they are weighed down with cement shoes, I get all sweaty from head to toe, I feel like the blood has flushed from my upper body downwards to my lower half, I lose my hearing and get a ringing sound (tinnitus) in my right ear, and I start to see only one color in tunnel vision - usually a grey blurry visual field, but sometimes red.  I'm also told by people who can see me that I tend to freeze in one place (probably because I'm trying not to fall over or bump into something, since I can't see) and I get a really spaced out look on my face.  These are called pre-syncope or pre-syncopal symptoms.  Not everyone with POTS has pre-syncope signs or symptoms.  Some people faint without any warning.

While many people use these terms interchangeably, it's important to be able to use them correctly when explaining symptoms to your doctor.  "I'm dizzy" isn't really that precise.  Try to describe your dizziness to your doctor in more precise terms:
Vertigo - a feeling of motion when one is stationary - you aren't moving but the room is spinning in circles around you (drunk college kids call this "the spins").  Sometimes you feel like the blood inside your head is spinning like a blender, but the room isn't spinning.
Lightheadedness - weakness or feeling as though your are about to faint (usually a pre-syncope symptom)
Disequilibrium - feeling or being off balance, most often characterized by frequent falls in a specific direction.

Blood Pressure (BP) -  BP is the pressure exerted by circulating blood upon the walls of your blood vessels. During each heartbeat, BP varies between a maximum pressure (known as systolic pressure) and a minimum pressure (known as diastolic pressure).  BP is usually expressed as systolic over diastolic. For example, a 'normal' adult blood pressure reading taken from your arm about 1 inch above your elbow would be 120/80 mmHg.  120 is the systolic pressure, 80 is the diastolic pressure and mmHg means millimeters of mercury - the measurement standard doctors use for blood pressure readings.

Orthostatic Hypotension - defined by the American Autonomic Society and the American Academy of Neurology as a systolic blood pressure decrease of at least 20 mm Hg or a diastolic blood pressure decrease of at least 10 mm Hg within three minutes of standing. 

Renin-Aldosterone Axis (RAA)
There is a very complex system of hormones, neurotransmitters and receptors in your body that regulate your kidneys and your blood volume.  Many people with POTS have an imbalance somewhere within the RAA system, which is also called the Renin Angiotensen System (RAS). Rather than explaining it here, just know that it's important for your doctor to check this system if you may have POTS.  For a more detailed explanation of the RAA/RAS, visit Wikipedia.

Arrhythmia - any abnormal heart rhythm caused by an abnormal electrical signal to the heart.

Sinus Arrhythmia (also known as Respiratory Sinus Arrhythmia [RSA]) - a naturally occurring variation in heart rate that occurs during a breathing cycle. Heart rate increases during inhalation and decreases during exhalation.  In some POTS patients, or patients with other forms of dysautonomia, the sinus arrhythmia can become exaggerated and may be considered abnormal.

Tachycardia - fast heart beat

Bradycardia - slow heart beat

Peripheral Nervous System vs. Central Nervous System - Your nervous system can be divided into two main parts: The Central Nervous System (CNS) includes your brain and the nerves in your spinal cord.  The CNS is like the supercomputer of your body - it processes all of the information received from other parts of your body and it helps one part of your body communicate with other parts to help all of our complicated body parts function smoothly as one body.  The peripheral nervous system includes all of the nerves in your body except the ones in your brain and spinal cord.  The main purpose of the peripheral nervous system it to connect your brain to all of you other organs (heart, kidneys, stomach, skin, etc.) and your limbs (arms, legs, fingers, etc.).  The peripheral nervous system can be divided into two parts: the autonomic nervous system and the somatic nervous system.

Somatic Nervous System - processes sensory information (smell, touch, taste, sight, sound) and controls almost all of the voluntary muscular systems in the body (moving your arms, legs, toes, etc.)

Autonomic Nervous System (ANS) - Your ANS controls all of the involuntary system of your body (e.g., the ones you don't have to think about for them to work.  For example, your ANS controls the speed in which your food travels through your digestive system, from your esophagus, to your stomach to your intestines, etc.).  The ANS is part of your peripheral nervous system and it plays a major role in controlling your heart rate, respiration rate, salivation, perspiration, temperature control, the diameter of your pupils, sexual arousal, digestion, bowel movements and urination.

Dysautonomia (autonomic dysfunction) is a broad term that describes any disease or malfunction of the autonomic nervous system.  This includes POTS, inappropriate sinus tachycardia (IST), vasovagal syncope, neurocardiogenic syncope (NCS), mitral valve prolapse dysautonomia, pure autonomic failure, neurally mediated hypotension (NMH), and a number of lesser-known disorders such as cerebral salt wasting syndrome. Dysautonomia is associated with Lyme disease, primary biliary cirrhosis, Chronic Fatigue Syndrome, Fibromyalgia, Shy-Drager Syndrome (multiple system atrophy), Marfan Syndrome, Ehlers-Danos Syndrome, hyerflexibility, and other rare genetic diseases.

Since I want this blog to be tween/teen friendly, I'm including a list of some of the different types of specialty doctors that POTS patients may utilize:

Neurologist - specializes in the nervous system (hopefully you can find a neurologist who has a sub-specialty in autonomic disorders)

Cardiologist - specializes in the heart and cardiovascular system

Pulmonologist - specializes in the lungs and breathing problems

Endocrinologist - specializes in metabolic issues like diabetes, and endocrine issues (adrenal, thyroid & pituitary glands, etc.)

Gastroenterologist - specializes in gastrointestinal issues (esophagus, stomach, intestines, colon problems).

Urologist - these doctors specialize in the function of the kidneys, ureter, bladder (and in men, the prostate) and all aspects of urination

Infectious Disease Specialist - some people with POTS may have or may have previously had an infection that caused or is causing their POTS symptoms.  An infectious disease specialist can help you diagnose and possibly treat infections.

Allergist/Immunologist - while these are two different specialities, doctors that practice one of these often work closely with doctors in the other specialty, or sometimes one doctor with hold degrees in both specialities.  An allergist looks for an helps treat allergies to foods, drugs, or environmental factors (like dust or pet dander).  An immunologist specializes in the diagnosis and treatment of immune system disorders, also called autoimmune disorders - like Lupus, Celiac, etc.

Psychologist - helps people learn to cope with the feelings, like anger, anxiety or depression, that can sometimes accompany serious medical problems like POTS.

Psychiatrist-similar to a psychologist, but a psychiatrist can prescribe medications to help deal with mental health issues in addition to providing counseling and various forms of therapy.

Physical Therapist - a P/T can help you design an exercise regimen suitable to your abilities that will make sure you're muscles and joints are strong and flexible.  This can help improve your symptoms over time.

Chiropractor - chiropractors help people maintain good posture and can help relieve back and joint pain.  They focus on proper alignment and support of the spine.

I try to define medical phrases in simple terms whenever I can throughout this blog.  If you can think of medical terms related to POTS that you would like to se listed here, please post a comment and I will add  them to the blog.

Dec 25, 2010

Surviving the Holidays with POTS

I hope all of you had a very Merry Christmas!

The holidays can be a stressful time of year for anyone, but especially for someone struggling with a serious illness.  You know you're supposed to be happy and cheery, but when you're in pain or having trouble breathing or some other disabling symptom, it's pretty hard to get into the holiday spirit.

This was my first Christmas season with POTS.  I was supposed to go to my in-laws house, about 30 minutes away, for Christmas Eve dinner.  Unfortunately, my breathing was so bad yesterday, on top of severe kidney pain (new diagnosis this week on that issue - see below) and a sore throat from a cold I caught from my mom, I was in no mood to be a passenger in the car - since riding in the car seems to make some of my symptoms worse (especially the vertigo).

My in-laws are wonderful people and they are very understanding.  They always throw a huge Italian feast on Christmas Eve.  I had prepped my own gluten free/lactose free high protein, low carb mostly organic dinner - it was a pretty awesome menu and it passed the "this is gluten free?" test for tastiness.  My husband and I skipped the Christmas Eve party and I ended up laying in bed crying every time a family member called to check on me - since I was so bummed that I couldn't be part of the festivities.

I felt like a dope crying over one missed dinner, but to me, it represents more than just one dinner.  It represents where I am at this point in my life - feeling sick and often unable to do what I want to do.  My husband did his best to cheer me up and we had some of the fancy food I had prepared the day earlier.  He put on the Charlie Brown Christmas album with all those Vince Guaraldi songs.  We are big Snoopy fans - our den is turned into 'the Snoopy room' for Christmas, decorated with 52 Christmas themed Snoopys and counting.  For some reason, this music made me extra sad and I started crying again.  I kept thinking, what the heck is wrong with me, why is a Peanuts song making me cry?  I think it just brought to mind all the happy Christmas memories I have from prior years, when I wasn't sick.  I was longing for my pre-POTS life, something I try not to do because it only makes me dwell on what's I missing out on, rather than the more positive "what CAN I do today?", which is essential if you're going to cope with the emotional impact of POTS. 

So to distract ourselves form the crappyness of the situation, we played some old school Nintendo on the Wii (the original Super Mario Brothers!), listened to some music that didn't make me think of Christmas (G. Love & Special Sauce always cheers me up) and went to sleep watching the Season 2 of the Golden Girls DVD we rented from the library (which always gives me happy memories of visiting my grandparents in Florida as a kid).  We also talked to our families as they were celebrating and everyone agreed to a "Round 2" of Christmas Eve festivities when I was feeling better.

Christmas Day was tough, but I fared much better.  My family went to my aunt's house in NJ, about 3 hours away, as they do every year.  I couldn't sit in the car that long having as many symptoms as I was these last few days, so my husband and I stayed home in NY.  I ended up on the phone with my sister as she was driving to NJ and I burst into tears when she said she'd miss me not being there.  It's like someone can look at me the wrong way this week and I start crying, and within seconds I'm mad at myself or being such a wimp for crying.  Not to mention, that crying seems to worsen my breathing and definitely burns my eyes more than normal - probably because all of the salt pills I have to take to keep my blood pressure up. 

My in-laws were supposed to go to another relatives house that they go to every year, but instead, they were kind enough to bring Christmas dinner to our house, along with my 2 sister-in-laws, our 2 nieces and our nephew.  It was such a delight to be able to spend time with all of them and not have to drag myself somewhere in the car, and also not to have to stress out over planning everything since it was a last minute decision for them to come over.  I'm sure if we had planned for everyone to come to my house in advance I would have worked myself to exhaustion trying to clean the house perfectly and cooking up a fancy dinner.  Instead, I decided to go with the flow.  I chilled on the floor and the couch most of the time, but we did manage to convince my mother-in-law to try the chicken flying game on Wii Fit - too funny!  I got a sore throat from talking too much, which happens to me these days every time I have a conversation with someone that lasts more than 2 or 3 minutes.  I ended up having one bad breathing attack towards the end of the evening (because I tried to play a Wii game standing up - my fault!).  

Overall, it was the most relaxed and enjoyable Christmas Day I've ever had.

Hopefully New Year's will go off without all the crying, but New Year's last year was the last time I went out and had fun with friends - my last hurrah before I got sick.   Unfortunately, January 2nd will be my one year anniversary of being sick, so I'm not going to get mad at myself if I cry that day.  We've rented Season 3 of the Golden Girls in anticipation of that day.

About the kidney pain if you're interested:
I've told at least 4 or 5 doctors about this horrible pain and soreness in my lower right flank, just below my ribs, but above my hip.  It's been happening on and off for the last few years, but much more often since I spent 2 months in the hospital this summer.  Some of them poked around (with my flinching every time they hit the 'sore' spot) and told me everything seemed normal.  I went to my local walk in clinic earlier this week because I thought I was getting an urinary tract infection and that is something you don't want to have over the holidays when the pharmacy and doctor's offices are closed.  It hurts like hell, and for people with delicate immune systems - like me and like many POTS patients - you have to take every possible infection seriously, because a little infection could turn into a big one if your immune system doesn't do it's job properly.  I am also extra cautious when it comes to kidney issues, because my dad lost his kidney to an infection when I was in 7th grade.  Luckily, he still has one kidney left that is healthy.  Sure enough, the doctor at the walk in clinic did a sonogram of my kidney and found a simple cyst about 1 cm in size (which he said was pretty common for someone my age and harmless) and he found that the pelvis of my kidney (the kidney pelvis is the part of the kidney that collects the urine just before it flows down to your bladder) was inflamed and that urine was backing up into my kidneys.  I am going to call a urologist first thing Monday morning, because this is exactly what caused my dad to lose his kidney to an infection.  It may be no big deal, but I still plan to have it checked out by a specialist, rather than rely on the walk-in clinic doctors assurance that "it's probably nothing."  Damage to your kidneys is not "nothing," and considering my family history and all the pain I've been having right where my kidney is, I have to get it checked out.  I  know that nerve damage related to POTS and other autonomic disorders can cause problems with the valves that control the flow of urine from your kidney to your bladder.  If that valve is damaged, it can cause urine to back flow potentially harming your kidneys.  I'm crossing my fingers that he's right and it is "no big deal."

Dec 16, 2010

Why is this happening to me?

Like many people with a chronic illness, I often found myself asking God (or my mom, who is a goddess in her own right), "Why is this happening to me?"  My mom is always able to come up with a nice thing to say, which usually makes me feel better.  God hasn't answered me directly yet, and I may never find out exactly why I came down with POTS.  I like to think that I was chosen to have this crummy illness because I am a natural advocate (an attorney by profession), and it sure seems like people with POTS need more advocates in this world, since the illness is not well known amongst the general public and even by the medical community.  Or I could just have really bad luck...

Whatever the reason was for me getting POTS, I may never know.  However, in a practical sense I have been able to identify some of the underlying physiological mechanisms that are causing my various symptoms.  I strongly believe that the more you know about your body and your illness, the better prepared you will be to fight it.  It's also important to know what's going on with your body, because not many doctors understand POTS.  Your regular family doctor may have never heard of it.  Even neurologists or other specialists you visit may have one or two other patients that have had it, but chances are they don't have a detailed knowledge of it.  It's a very challenging disorder that can effect almost every system in your body.  Here's what I have learned about what's probably causing my various symptoms.  I say "probably" because I want you to be clear that I am not a doctor, this is not medical advice, and what I say here may not explain what is causing you're symptoms.  Here goes...

Low Aldosterone/Low Blood Volume
Through specialized endocrine blood tests we discovered that I have very low levels of the aldosterone, an essential life sustaining hormone produced by your adrenal glands that controls the balance between sodium and potassium in your body, which allows your body to absorb and excrete the proper amount of water each day.  Without aldosterone, you can go into an adrenal crisis - your body will become so dehydrated, you can go into shock or, in extreme cases, you can die. Many people with POTS have low aldosterone, although we have not been able to identify a particular cause of my low aldosterone yet.  There are lots of different things that can cause low aldosterone (high sodium diets, laying down too much, autoimmune diseases, infections, etc.), but some people have low aldosterone for an unknown reason (called idiopathic hypoaldosteronism).  Low aldosterone causes your body to excrete too much water and sodium, and causes you to have high levels of potassium in your blood.  Without enough aldosterone, your body cannot retain the water and sodium from the foods and beverages you consume, which leads to dehydration.  Without enough water in your body, you develop low blood volume.  Many of the troubling symptoms of POTS are the result of low blood volume, which doctors call hypovolemia.  Studies have shown that some POTS patients are missing one third of the normal blood volume expected for someone of their size.

Low Blood Pressure Triggered By Low Blood Volume
Low blood volume causes low blood pressure.  Since there is not enough blood in your body to push against the walls of your blood vessels, veins and arteries, your overall blood pressure is lower than it should be.  120/80 is usually considered the "ideal" blood pressure for adults, but many people have healthy blood pressure that is slightly lower than this.  When I was at my worst with POTS, before any medication to replace my missing aldosterone, my laying down (supine) blood pressure was as low as 80/40 and I could not stand up without passing out, because standing up would have lowered my blood pressure even lower than that.

When a healthy person stands or sits up, the blood vessels in the lower parts of their body are sent messages from various parts of their nervous system telling the vessels to constrict, which pushes the blood upwards and counters the effect of gravity.  This ensures that blood will reach your upper body and brain even when you're standing up.  When someone with low blood volume stands up, even if the messages are sent to the blood vessels in their lower legs, and even if the vessels constrict, there may not be enough blood in their body to supply sufficient amounts of blood to their upper body and brain when they stand or sit up. 

Forgetfulness/Brain Fog/Headaches
The low blood pressure and low blood volume contribute to a lack of blood flow to the upper body and  brain - this causes many of the symptoms of POTS.  Sometimes doctors use the term cerebral hypoperfusion, which means not enough blood is getting to the brain.  For me, cerebral hypoperfusion seems to cause my headaches/stabbing/shooting head pains, difficulty concentrating/forgetfulness, brain fog, cognitive impairment and not being able to recall the word I am trying to say.  This is usually relieved after I lay down for awhile.

Tachycardia(Racing Heart Beat)
The blood flow in the human body is run by a very complex system of checks and balances, and I do not claim to fully understand it (yet!), but I do know that when receptors in your neck, called barorecptors, sense that there is not enough blood flowing to your brain, they send messages that result in your heart beating faster, which is your body's attempt to get more blood to your brain.  In a healthy person, your heart would be faster for a few moments when you stand up, your blood vessels in your lower body will constrict, pushing your blood upwards against the force of gravity, and you're baroreceptors in your neck would sense that you're brain is now getting sufficient blood supply, so that your heartbeat would return to normal.  But in someone with low blood volume, you heart will be faster, but there still won't be enough blood to get to your brain when you stand up, so you're baroreceptors tell your heart to be faster and faster, and you're heart will do that in an effort to make sure you're brain is protected from damage due to low oxygen levels - similar to an 'ischemic stroke.'

My heartbeat will be a pretty normal 60-80 bpm when I'm laying down, and by simply standing up, within seconds my heartbeat starts racing up and up to over 130.  The highest recorded measurement I had, before I was on medication, was 230 beats per minute, which is very dangerous, especially if it goes on for too long.  I collapsed to the floor shortly after this reading was taken, probably because my brain was trying to protect itself by making me pass out, which helps return proper blood flow to the brain. 

Passing Out/Almost Passing Out
When you're heart beating faster and harder isn't enough to compensate for the lack of blood flow to your brain, thankfully, you're brain is smart enough to protect itself from a lack of oxygen, so it makes you pass out, which usually results in you laying flat on the floor, in order to get that blood flowing back to your brain.  In that sense, passing out is almost a good thing - it's better to pass out for a few minutes than to suffer with a lifetime of brain injury that was caused by a lack of oxygen to your brain (an ischemic stroke)!  That's why it's so important for people with POTS to let friends and family around them know that if they feel very lightheaded or like they are about to pass out, the best thing to do is lay down with your feet slightly elevated.  Sometimes people try to help someone who is dizzy by holding them up or preventing them from falling out of a chair.  While it is certainly polite of them to help, it would be better to safely lay the person's head on the ground and slightly elevate their feet and legs.  Hitting your head on furniture or a hard floor is probably the most dangerous part of passing out with POTS, so try to pass out gracefully if you can.  :)

I'm "lucky" to get pre-syncope symptoms, that is, my body gives me warning signs a few seconds before I pass out.  I get lightheaded, dizzy, off balance feeling, my vision gets grayed out or blurry or tunnel vision, I feel like my blood is being flushed downwards to my feet, my legs feel heavy, like I'm wearing cement shoes, and I usually get a bit sweaty on my upper body, I feel shaky and I feel short of breath.  Just a few seconds of feeling like that is enough for me to know I have to lay down ASAP, to avoid passing out and hitting my head.  Unfortunately, some people with POTS and other syncope disorders do not get any warning signals, and they can be in the middle of doing something and all of a sudden they fall over like a marble statute.  If you see this happen, don't lift their head up.  Wait a few seconds to see if they "wake up" or call 911 - especially if they hit their head hard or they don't wake up quickly.  If you are "lucky" enough to get pre-syncope warning signals, there are a few counter maneuvers or body positions you can try to help increase blood pressure and blood flow to your brain.  I will be posting information on these counter maneuvers in a future posting called "Coping With POTS."

One of the chemical messengers your brain uses to tell you're heart to beat faster is adrenaline (also called epinephrine).  Adrenaline is very good at doing its job and when it is released into your bloodstream, you're heart will be faster and harder, which is you're bodies way of trying to get more blood to your brain, to protect your brain from injury due to low oxygen.  Adrenaline is the chemical that causes that rush you feel when you get really scared by something - it causes an instinctive "fight or flight" response.  Lots of adrenaline flowing through you body can make you feel shaky, anxious and very uncomfortable.  Doctors sometimes use the word tremulousness to refer to this shaky feeling.  Too much adrenaline can also cause you to get the chills or to get overheated very easily.  Adrenaline can also make your senses feel like they are on overdrive and can cause over-stimulation.

When I lay or sit still, I can almost always feeling my pulse in my neck, eyes, head, stomach and pretty much everywhere else.  When my heart is pounding very fast, I can also see my pulse flashing in my eyes.  These are annoying symptoms, probably caused by a mix of the tachycardia, over stimulation and oversensitivity of your nervous system, and the anxiety like symptoms caused by the adrenaline surges people with POTS get.

While most people with POTS experience these adrenaline induced symptoms that can look or feel like an almost permanent panic attack, not everyone with POTS has 'true' anxiety or panic disorder.  Studies have shown that about 97% patients with POTS were first misdiagnosed as having anxiety or panic disorder, when in fact they really had a disregulation of their autonomic nervous system.  That's not to say that people with POTS can't have 'true' anxiety or panic disorder.  After all, half a million people in the US have been diagnosed with POTS.  Certainly out of a population that large there are going to be a percentage of people that have 'true' anxiety or panic disorder in addition to their POTS diagnosis.  I was misdiagnosed with about 30 different conditions before we found out I had POTS, with several doctors telling me it was "all in my head" and I should just calm down and take a Valium.  Ironically, the only doctor who assured me that it was definitely not in my head and that I had a 'real' medical condition and definitely not anxiety or panic disorder was a psychiatrist!  I'm going to write about my misdiagnosis nightmare in another post, "POTS-Misdiagnosis Nightmare." 

If you think you have POTS and you're doctor is telling you it's all in your head - find a new doctor ASAP - and don't stop looking until you find a doctor who takes your seriously and will run the proper tests on you to diagnose or rule out POTS.  Check out my POTS Resource Links at the bottom of this page to see a list of doctors and medical centers that can diagnose and treat POTS.

Chest Pains/Tingling Extremities/Tinnitus/Visual Disturbances
The low blood flow to the upper part of your body doesn't just effect your brain and heart.  Low blood flow to my arms causes a cold, tingling and pins and needles feeling in your hands and arms.  Low blood flow to you chest, lungs and heart can cause shortness of breath (called dyspnea) and chest pains.  Low blood flow to your head can cause ringing in your ears (called tinnitus), and visual disturbances.

POTS is not just about low blood flow.  Many people with POTS have some dysfunction of their autonomic nervous system.  Your autonomic nervous system controls your breathing, your heart beat, your blood vessels, your blood pressure, digestion, urination, etc.  Basically, it controls all of those things your body does for you that your don't have to think about.  For example, when you eat a cheeseburger, you don't have to tell your stomach to digest that cheeseburger for you.  Your autonomic nervous system controls the various parts of your digestive tract and takes care of that cheeseburger while you get to do something more interesting. 

Dysautonomia can involve damage to the autonomic nerves themselves, or a problem with the chemicals that send messages between the nerves, called neurotransmitters.

Peripheral Small Fiber Neuropathy
My low blood pressure is also partially caused by the fact that the nerves that control the constriction of the blood vessels in the lower parts of my body are damaged.  This damage to these nerves is part of my dysautonomia.  I had a 3 mm punch hole skin biopsy (without anesthetic because I was allergic to the anesthetic shot the had available-OUCH!), when my doctors were looking for mastocytosis, another disease that can cause POTS like symptoms.  The lab results showed that I did not have mastocytosis, but that my small fiber nerves at the location of my biopsy (my left calf) were 80% deficient - 60% of the fibers that should have been there were completely missing (as a result of nerve death) and 20% of the fibers were still present, but showed signs of damage.  That leaves 20% of nerve fibers that were "normal."  Doctors have various terms for my dead and damaged nerve problem - Peripheral Small Fiber Neuropathy, Autonomic Neuropathy, Peripheral Autonomic Neuropathy.  Neuropathy is a term doctors use to describe when nerves have been damaged or have died off.  The name is less important that to understand which nerves are involved, and which of my body systems will be effected by this nerve death/damage. 

Roughly speaking, there are two areas of your nervous system: the central nervous system (CNS) which includes your brain, brain stem and your spinal cord, and the peripheral nervous system, which controls the nerves that flow from your spinal cord to the various organs and other parts of your body.  Then there are three important types of nerves that can be involved in "peripheral" neuropathy - sensory nerves (that control pain, touch, pressure, hot/cold sensations), motor nerves (that control your ability to relax/contract your muscles, movement, walking, etc.) and autonomic nerves (that control your involuntary body functions such as breathing, digestion, blood pressure, heart beat, etc.).  In your legs, if you lose autonomic nerves, it impairs the constriction of your blood vessels, and makes it more likely that blood will pool in your legs when you sit or stand upright, causing low blood pressure and all of the symptoms resulting from low blood pressure.

All three types of peripheral neuropathy (autonomic, motor and sensory) generally (but not always) start at the farthest points of the nervous system from the brain.  For example, people who have sensory peripheral neuropathy tend to notice numbness, tingling or pain in their toes first, and then if their peripheral neuropathy continues to spread, it can start to move up higher up their leg to their knees.  Sometimes it also starts in their fingers and spreads up their arms too.  While I've only had one skin biopsy, which is the best way to identify autonomic small fiber peripheral neuropathy, I suspect that my autonomic neuropathy has spread up to my mid-thigh region and that it is in my hands as well.  Signs indicating to me that my peripheral nerves are damaged in my legs and arms include tingling, pain, numbness, coldness, lack of sweating or too much sweating, blotchy purple skin color and swelling in my lower arms and legs when I stand up, the veins on the top of my hands tend to fill up with blood and get swollen when I put my hands below my heart level, and then they flatten out and my hands get almost immediately cold and clammy when I raise my hands above my heart level.

I am awaiting further testing to better delineate and describe the location and specific type of neuropathy I have, and I'm also trying find out what caused my neuropathy.  Hopefully it is something temporary (like an infection) that is not causing continuous damage to my nerves.  There is hope for people with peripheral neuropathy - under ideal conditions, your peripheral nerves can grow back healthy about 1mm per year.  Small fiber nerves, the kind I know are damaged in my legs, are very tiny, so 1mm of growth is a huge amount of healing to be happening in one year.  This is why it's so important to get a baseline level on how severe you're neuropathy is when it's first discovered - then you can go back a year or two later and see if you're nerves have grown back, stayed the same, or if they have gotten worse.

Acid Reflux/Vomiting/Diarrhea/Stomach Pains
When something is wrong with your autonomic nervous system, it's as if there is a short circuit in one or more places in your body and things that were supposed to happen on their own start to not work as they should.  I developed acid reflux for the first time in my life when I developed POTS.  This is likely because the peripheral nerves that control my lower esophageal sphincter - the valve at the bottom of your esophagus that keeps stomach acid down in your stomach - isn't working properly.  Sometimes my valve stays open, probably because the nerves that once told it to stay shut are damaged.  This valve is probably also "leaky" because my diaphragm muscle, the muscle under your lungs that control your breathing, is also weakened - possibly due to autonomic nerve damage or due to a problem with the muscle strength itself (I don't know yet-more tests I need to have).  We do know, as a result of pulmonary function tests that my ability to breathe in and out really hard and fast is about 70% weaker than it should be. 

There are also nerves that control how quickly something passes from your stomach to your intestines, and how quickly something travels through your intestines to... well you know.. the toilet.  OK... if you don't want to hear about this, skip to the next paragraph.  While I don't want to gross out anyone reading this, especially those of you who know me, I think it's important to share this information with other POTS patients so they know they are not alone in suffering, and so that maybe someone else suffering with these symptoms will go to their doctor with this info and get an accurate diagnosis.  So grow up... everyone poops!  I had horrible diarrhea when I first came down with POTS - several times a day for several months, usually starting when I woke up in the morning and lasting until about 3 pm.  At the same time this was happening, I was puking once or twice a day for about the first three months.  Of course that caused me to lose about 50 pounds within the first few months I was sick.  I became malnourished and dehydrated, even though I was eating a very healthy, but high calorie diet, and I was drinking 2 liters of water and electrolyte fluids (PediaLyte and Coconut Water) a day.  Thank goodness I was a little chunky when I first got sick.  I went from 180 lbs. to 130 lbs. in three months.  I'm 6 ft tall and I was very athletic, so even though 180 sounds like a lot, I didn't really look that heavy at 180 (but that was the heaviest I ever was in my life), and I looked emaciated at 130.  The last time I weighed 130 lbs. was junior year of high school and I don't think I was as tall back then.

Thankfully my bowels are doing a little better now, but my GI symptoms still act up one in a while, and we're not really sure what triggers it, but I have some educated guesses (more on that later).  I'm back to a healthier looking 148 lbs. and I really have to work at it to keep my weight up (mostly I'm worried that losing weight means I'm not absorbing the vitamins and minerals I need to get healthy).  I get a bloated/full feeling even from eating small meals, I get stomach aches if I drink too much water at once, I vomit about once a week - usually right after drinking fluids - we're not certain, but it is probably because the autonomic nerves that control the speed of my digestion or my stomach valves are damaged.  This is unpleasant, but it could have been worse - some people with autonomic nerve damage cannot control their bladder or their bowels at all.  I consider myself lucky that those parts are still working for me.

Chills/Goosebumps/Overheating/Night Sweats
Your autonomic nervous system also plays a big role in regulating your body's temperature.  It's likely that the chills, goosebumps, overheating and night sweats I get are because of damage to some of the nerves in my autonomic nervous system, although these body temperature related problems can be exacerbated by too much adrenaline, which is discussed above.
Shortness of Breath/Hyperventilation/Sore Neck and Shoulders
Many people with POTS, myself included, have persistent shortness of breath.  In my case, it seems to be worse when my other symptoms are worse, it's worse in the morning, in the cold and it also seems to be worse when I stand or sit up.  Many POTS related problems can contribute to shortness of breath, and in my case I honestly don't know what's causing it, but I suspect it has something to do with the low blood flow to the upper part of my body.  If my heart or lungs aren't getting enough blood, I end up gasping for air, feeling oxygen starved.  It could also be caused by damage that may have occurred to my peripheral autonomic nerves in my controlling my heart/lungs/diaphragm - my doctors are looking into that, but figuring out your POTS symptoms is a long process.

When I feel short of breath, sometimes I start to hyperventilate.  Many people associate hyperventilation with anxiety, and just think "if you calm down, you won't hyperventilate."  In some cases, I'm sure that's true, but my hyperventilation only happens when my other symptoms are bad or when I've been standing upright too long and my pulse starts to race, so I think it is more of a biological trigger than an emotional one.  Let me be clear - it is pretty terrifying when you feel like you can't breathe and your heart starts racing to 130+.  Even the calmest person in the world would get a little rattled if that was happening to them several times a day without explanation!  I'm sure the surges of adrenaline (explained above) and the racing/pounding heart beat don't help the breathing situation either.  But personally, I don't think my hyperventilation has much to do with emotional or anxiety triggers.  I was on vacation in VT snowboarding and having the time of my life (totally relaxed!) when the first hyperventilation attack happened to me, and happened about an hour after I had my first horrendous attack of diarrhea and vomiting (at a ski lodge of all places).

Hyperventilation can also be a sign of an acid-base imbalance.  When you hyperventilate, you blow out too much carbon dioxide, which your body needs to maintain the pH balance of your blood.  When you hyperventilate, your blood may be fully oxygenated, but the abnormal pH of your blood makes it difficult for that oxygen to reach the tissues that need it - essentially causing a low oxygen condition even though your blood is filled wit oxygen.  Many different medical conditions and medications can cause a problem with the pH balance in your body, including low aldosterone and some drugs used to treat low aldosterone.  I'm awaiting tests from my doctors to see an acid-base disorder this may be triggering my hyperventilation.

All of this gasping for breath usually results in me breathing shallow breaths from my upper chest area, rather than using my diaphragm to breathe deeply.  This may be the way I breathe because my diaphragm is damaged, as explained above, but it's also natural to breathe quick shallow breaths when you hyperventilate or have other breathing difficulties.  Repeated shallow upper chest breathing can put a strain on chest, neck and shoulder muscles, and that is probably why they get so sore sometimes.  Also, since people with POTS tend to lay down so much, they may be developing bad posture or may be weak from inactivity.  I am definitely guilty of this.  When I was in the hospital for two months, I could barely hold up the weight of my own head because my neck and back muscles had become so weak.  That started because when I was first admitted to the hospital, they wouldn't let me out of bed AT ALL because they didn't know why my heart was racing so high every time I stood up.  It was really degrading - they made me use a bedpan to go to the bathroom.  I was not happy about that and I didn't think it was necessary.  Sometimes if the nurse wasn't around, I sneak over to use the bathroom, holding on to my IV pole for balance - within seconds the nurses would come running in to my room screaming at me because my heart monitor alarm would start beeping  because my heart was racing off the charts.  Of course, all of that forced bed rest only made all of my symptoms worse.  I eventually convinced them to give me one of those little portable potties, so I could retain a tiny fraction of my dignity!
I am extremely tired all the time since I developed POTS - just washing dishes for a few seconds or walking from my bed to the bathroom makes me exhausted.  When you heart beats so fast just from sitting or standing up, naturally - you get tired really quickly.  It feels like I've just completed a hour long cardio work out, without that energizing feel good sensation you get after a real workout.  Also, lots of people with POTS report feeling very tired after a passing out episode.  I don't notice this, but I think it's because I'm just tired all the time anyway.

It can be hard for me to sleep.  It's pretty difficult to fall asleep with your pulse racing and your head spinning with dizziness and your pulse flashing in your eyes, and thumping in your neck and temples, and don't forget the stomach aches and acid reflux creeping up into your throat.  I have to fall asleep with the TV on and the volume turned up pretty loud.  That way I can't hear my pulse and I can't see it in my eyes because the ambient light and flashing from the TV masks it. 

Speaking of fatigue, I don't have the energy to type anymore right now.  I'll be back with another post when I'm feeling a little better...

What is POTS?

POTS (Postural Orthostatic Tachycardia Syndrome) is a disorder of the autonomic nervous system.  The autonomic nervous system controls the involuntary functions of your body, like breathing, blood pressure, heart beat, digestion, etc.  These are the things that keep you alive, that you normally don't have to think about.  When you eat a cheeseburger, you don't tell your stomach " O.K. stomach, let's get to work and digest that cheeseburger."  Your stomach and the rest of your gastrointestional tract just do it for you, because they are controlled by your autonomic nervous system.

People with POTS have a problem with one or more parts of their autonomic nervous system, and since the autonomic nervous system is so important to good health, people with POTS can suffer from a long list of symptoms, although not every person with POTS has every symptom.  The "S" in POTS stands for "Syndrome" because doctors don't really know what exactly causes POTS.  The medical community calls a list of symptoms that are related and commonly seen together a "syndrome" to help with diagnosis, but there is no definitive answer at this time on what causes POTS in each patient.  POTS was only recognized as a syndrome and given a name by researchers at the Mayo Clinic in 1993, however other researchers had described its symptoms under various terms since the 1940s. 

Some POTS patients can find an underlying cause that has triggered their POTS - like a recent viral infection.  I have found two underlying causes for my POTS, autonomic small fiber neuropathy (death and damage to the nerves that control my blood vessels, amongst other things) in my legs and low aldosterone, the hormone produced by your adrenal glands that controls your sodium/potassium/water balance, and thus your blood volume and blood pressure.  However, my doctors have not been able to figure out what is damaging and killing my small fiber nerves, and what has caused my adrenal glands to stop producing aldosterone.  I am trying to find a doctor who is willing and able to run tests on my to help figure this out.  Like every well informed POTS patient, I am looking for an underlying problem that is hopefully treatable and reversible!

Here's a list POTS symptoms I've had, and for the record, I did not have any of these problems before I got really sick in January 2010.  Before all of this I was a healthy, active, athletic person. Thankfully I don't get every symptom at the same time, but these are the ones I've had often enough to remember:

-rapid heart beat (tachycardia), especially when standing up

-heart beat increasing 30 beats per minute (bpm) or more upon standing - I am usually 80 bpm when laying down and then 130 to 160 bpm as soon as I stand up, and it keeps going up the longer I stand up

-low blood pressure, made worse when I stand up


-vertigo (this is different that dizziness - for you older POTS patients, vertigo is that spinning/swirling feeling you get when lay down after you've had too much alcohol - in college we called it "the spins")

-fainting (also called syncope)

-almost fainting (pre-syncope)

-severe shortness of breath

-rapid breathing/hyperventilation that is not due to an emotional trigger

-sharp and dull chest pain in various places all over the front of my chest cavity

-sharp sudden stabbing pains and also dull aching/pressure pain all over my back, especially near the back of my armpits, between my shoulder blades and along the bottom of my rib cage in the back

-aching/sore neck and shoulders

-tightness in my chest and throat that makes it hard to breathe

-horrible night sweats

-heart palpitations

-thumping feeling of my heartbeat in my stomach, chest, neck, ears, eyes, thighs and fingers

-an very noticeable sinus arrhythmia (a sinus arrhythmia is when your heart beats faster as you breathe in, and slower as you breathe out - it is considered normal in healthy people, but mine has become more noticeable and more exaggerated since I developed POTS)

-seeing your pulse flash in your eyes, even when tey are closed and you are in a dark room

-extreme fatigue (as in, laying in bed for a week straight and not being able to go downstairs to get a glass of water)

-despite being tired, you can't sleep (try sleeping when you're heart is beating 130 bpm and your pulse is flashing in your eyes!)

-temperature sensitivity (getting REALLY cold and shivering when it's not even that cold out, getting REALLY hot and sweating when it's not that hot, taking along time to recover from being too cold or too hot)

-abdominal pain of every kind

-bloating, even after eating small, healthy meals

-feeling dizzy/lightheaded after eating (doctors call this post-prandial hypotension-it's because the blood is pooling in your belly to help digest your food)

-getting the chills after eating, and no amount of blankets or hot tea will make it go away

-acid reflux/GERD

-vomiting (almost every day for 3 months, but less often now)

-diarrhea (I'm not going to be shy about this - this is too important a symptom to leave out because it made me really sick.  I was getting is several times a day for months and months and I started to get malnourised, which can create very serious complications for someone who is already sick.)

-sudden weight loss (I lost 50 lbs., but I've gain about 10 back now.)

-random shooting/stabbing pains in my head, face, chest, and legs that actually make me scream out loud and drop to the floor, whatever I'm doing

-oversensitivity to pain and touch (when this symptom was really bad, I didn't even like getting hugged because it hurt and it seemed to overstimulate me - needles for the hundreds of blood tests I had were torture, even though I never had any problems donating blood or getting shots in the past)

-oversensitivity to noise (I'd jump out of my chair if someone knocked on the door or if my dog barked, and I'd get headaches very easily from quietly watching TV or listening to my I-Pod)

-heightened senses (I'm the first one to smell something weird, or to hear a strange noise in the distance, or to taste that milk has started to spoil)

-hyperreflexia (your reflexes are on overdrive and you literally jump at the slightest noise or unexpected touch)

-very dilated pupils, even in bright light (dinner plate pupils)

-light sensitivity (I get burning eyes, dizziness and headaches in bright light)

-irregular menstrual cycles and a worsening of most of the other POTS symptoms before and during menstruation

-the skin on my legs turns blotchy and purple if I stand up for a few minutes, and every time I take a bath (I can't stand in the shower most days)

-flushing (my arms, upper chest and face turn purplish red, seemingly for no reason)

-worsened allergies (I always had mild allergies since I was a kid - normal stuff like dust mites and dog dander, but never needed medication for them and never had any food allergies - now I'm on allergy meds everyday and I have to carry an Epi-Pen around with me)

-lactose intolerance for the first time in my life (and I'm a cheese addict!)

-suspected gluten intolerance (still waiting for more testing)

-chemical/medicine sensitivities (if the recommended adult dose for a medicine is 10 mg, I probably only need 1 mg even though I'm a 6 ft tall adult)

-pins and needles/tingling/creepy crawly skin sensations (especially when I'm given medications that effect the nervous system)

-dry mouth, eyes and skin (despite the fact that I'm drinking 100 oz. of water and juice per day)

-frequent infections/weakened immune system (I've had a constant sore throat for 11 months.  I've also had several unusual small cysts on my skin, which my dermatologist said were infected with a common and normal skin bacteria.)

-terrible fatigue, especially in the morning.  All of my symptoms tend to be worse in the morning, until about 3PM when I start to come alive a little bit.

-forgetfulness/spaced out feeling

-easily distracted/hard to focus

-not being able to find the word you're trying to say (doctors would call this recall or cognitive impairment)

Gosh, I'm getting tired just typing all of these symptoms up.  I've had other symptoms, but frankly, I'm having a brain fog moment and I can't think of them.  There are many more symptoms associated with POTS, and each POTS patient seems to present in their own unique way.  For more information on POTS symptoms, you can visit the Dysautonomia Information Network or the Wikipedia page on POTS.

Next time I will explain why some of these symptoms occur.

Dec 15, 2010

Welcome to POTSgrrl!!!

Who am I?  Why am I starting a blog?

Well, I've been laying in bed sick, in and out of the hospital, for the last 11 months, but before that, I was an athletic, outgoing, energetic, environmental attorney and loving wife.  I'm still a loving wife, but most of those other descriptors don't fit me that well anymore.  After 9 months of medical hell, I was finally diagnosed (through countless hours of my own online research) with Postural Orthostatic Tachycardia Syndrome (POTS), a type of dysautonomia, which is just a fancy way of saying my autonomic nervous system is really messed up

Over the past few months, I e-mailed updates to friends and family from my hospital bed, and many of them have told me that my way of explaining this dire medical problem is kind of funny, and that my story of getting a proper diagnosis is so outrageous, that people need to hear about it.  They keep telling me to write a book about this crazy roller coaster ride I've been on.  I don't know if I'll ever get around to writing a book, as it is difficult even for me to concentrate long enough to type this blog post.  I'm more interested in writing about this disease to help raise awareness, amongst the general public, but especially amongst the medical community.  It should not take nine months to get a diagnosis for any disease, but especially one so severe.  I live in the suburbs of New York City, and we are supposed to have the best doctors in the world here in NY, and I even though I went to the #1 rated hospital in NYC and #6 in the country, they were not able to give me a proper diagnosis.  It wasn't until I read something on the internet about POTS that made me think - "Hey, my symptoms completely match this POTS thing!"  I asked my doctors if I could have POTS - they raised their eyebrows and said they'd look into it.  Sure enough, after consulting with some other experts, they agreed I probably had POTS.  The internet may have saved my life.

I'm writing this blog in simple terms to help explain what I've learned about POTS to young people.  Many POTS patients are tweens and teens.  I'm having a hard enough time understanding and coping with POTS at 32 with 7 years of higher education under my belt, and I can't imagine how much harder all of this would be if I was still a kid.  I think everyone with POTS, no matter what their age, should try to learn everything they can about their symptoms.  The more accurate information you have and the more you understand your symptoms, the better your chances to find solutions that work for your POTS symptoms.

I also want other POTS patients to know they are not alone in their battle.  There are at least 500,000 POTS patients in the U.S. alone!  Since it is a difficult disorder to diagnose, and so many of us are undiagnosed or misdiagnosed for so long, my guess is that there are probably another 200,000 people who have it and don't even know it.  So remember, you are not alone.  We are all looking for answers to what causes this illness, and how the heck we can get rid of it and get back to our "normal" lives.

So read all of my blog posts and please feel free to post about your POTS experience.  I'd love to hear what state you're from, when you were diagnosed, what doctors were helpful to you, and what tips and tricks you have figured out that help you feel better.