The Next Round

It’s been a relatively quiet April on the health front, which I am so grateful for. I still had appointments almost every week but they were not necessarily related to diagnosis and treatment. I needed that break, and the universe gave me a win. I’m still filled with frustration about test results, the struggle to get a diagnosis, and the daily battle of managing a nebulous condition, but I’ve been able try to get to a better place this month, reorienting myself into a life that isn’t completely defined by being ill. Listening to the suffering and experience of others, trying to understand the historical civil unrest in my country, helping my family when I can, reading about prehistory, analyzing every detail of the results of the ancestry tests my husband and I did.  

But it's time to re-focus. I start the next round of appointments next week. I see my new local neurologist in two weeks, and I have an appointment Stanford on Tuesday and will finally see my neurologist there.

I’ve had that appointment for almost a year. I saw him last May, which feels like a lifetime ago. I had just finished my first year as a full time professor and it was the most brutal semester I’ve ever been through. I had to go on leave twice during the semester and my symptoms were worsening every day. I wasn’t using a wheelchair yet, but I needed to. I was struggling to drive and my independence was starting to slip away. 

I’m trying not to let the ghosts of that last appointment haunt this one. I left feeling like it was a great appointment but when I read his notes he mentioned that he discerned no walking difficulty and that I needed a “more positive outlook.”

I cried a little during that appointment. Life was pretty bleak at that moment. I didn’t get to say goodbye to some of my classes (again) because I was on leave, in my heart I knew that I was going to have to either radically change how I did my job or it was over, and the mysterious symptoms were only getting worse. Of course I was upset. I try to smile and keep a calm demeanor in appointments, otherwise you end up with notes about mental health and stress if your file, further invalidation. But I’m honestly getting to the point that I don’t care what they say. 

I spent a lot of years trying to justify and explain in appointments to get them to hear me. At this point, I don’t have the energy for that. Now I just try to present my symptoms and experiences and communicate effectively. These are my symptoms. Take it or leave it.

At that appointment a year ago, he explained that my mystery symptoms of weakness, difficulty walking, difficulty breathing, etc were not related to POTS. I had those symptoms for over a year at that point but I was still hoping they were related to POTS so I didn't have to get back on the hamster wheel of diagnosis. He gave me a list of tests he wanted me to do, and as I listened I knew those tests would take a very long time and that a diagnosis was at least a year off. At least. 

I started suddenly having mobility problems about two years ago and I told my primary doctor about it first. He took me seriously and said it was likely something else presenting itself. I refused to believe that at the time but I have told him since then that he was totally right. He figured that out before any other specialist. I really need to send him a fruit basket someday.

I saw him yesterday so that he could fill out my long term disability paperwork, and every time he sees me he asks, “did you finally get a diagnosis?” Nope. Together, we have been fine tuning innovative ways over the last few months to explain and label my symptoms for these forms without using a label that is unofficial, to the point that it becomes a humorous art. You have to laugh at the absurdity of it sometimes.

I don’t want to set myself up for disappointment, but I am hoping to finally get that label in these next appointments. Not just for the peace of mind but mainly because of the material benefits it offers: I could finally put it on my official paperwork, I can wave it in front of insurance companies who want to refuse treatment or deny benefits, and I can have access to other treatments options. If it is Myasthenia Gravis, I'll have a condition that sounds like a Harry Potter spell, so I'll feel pretty special.

I try to remember that a diagnosis is not the end game, a better quality of life is. But I can’t help wanting the diagnosis. It’s been the carrot I hold in front of myself as I’ve gone through all the torture testing for years. Yet, even with a diagnosis I know that my life wouldn’t change dramatically, but damn it would help. 

I’m doing my usual routine of carefully writing out symptoms, notes, and questions, and gathering test results to bring. Most of this has become second nature for me and my husband so it makes it easier to keep going, even when both of us are completely worn down by this process. We are staying in our favorite hotel again next week before my appointment. It’s pricey but I’m very much looking forward to that part. Sometimes it takes little things like this to make it easier, little rewards and treats along the way to make it a little more fun and enjoyable.

So this is what it looks like when you want to give up on this process but you continue to press on, trying to keep a “positive outlook,” keep digging deep for whatever pieces of hope you can find, and remember that you’ve come this far so you can keep beating on. It’s not easy, but it’s not impossible. That’s important to remember.

Whatever the results of the next appointments are, I’ll come home to my life with my cats, my husband, my music, my books, and it will be all good. I'm trying to remember that life will go on no matter what. I’ll keep working toward my goals, trying to create some art and music, write some words that have meaning, exercise as often as possible, and continue to feel grateful for this life and how lucky I really am. 

Navigating the Medical System

This is an extension of a recent post, but in this one I want to discuss some strategies I have learned to cope with the frustrating process of diagnosis, treatment, and management. I often think about what I wish I had known when this bizarre adventure started and if that information would've helped me navigate the medical system better. I hope this information is useful for the chronically ill and the healthy. This is also a collaborative post that includes the voices of some other POTSies who share their experiences as well.

I had no idea what I was getting into in 2011, but since then I have learned how to be a professional patient, how to talk to doctors, and how to try to get what I need from a broken medical system. Here are a few things I have learned over the last four years: 

 1. Be Prepared

Before I got ill, I lived in a world of make believe in terms of what I thought doctors and Western medicine could do. Shows like “Mystery Diagnosis” are great for raising awareness about rare conditions but they can make the process of diagnosis for complicated conditions seem so fast and complete. There’s always a happy ending. I know now that this is something of a fantasy. I was better off waiting for my entrance letter to Hogwart’s School of Magic than expecting diagnosis and treatment to be an easy process.

I have gone to maybe 200-300 or more appointments over the last four years and been on the endless hamster wheel of diagnosis and treatment that long also. The process is mind-numbing: see a doctor, wait a few weeks and do a test, wait a few weeks or months and see the doctor again, wait a few weeks and do a test, wait a few weeks or months and see the doctor again, get sent to another doctor, start the process over. Over and over.

When you see a doctor, you are usually one of maybe 30-50 patients that doctor is seeing that day. You have 15 minutes to communicate what a hot mess your life has become and then you are sent on your merry way. I have learned the hard way the Boy Scout’s mantra of BE PREPARED. Write down every symptom and every question that you have. Maximize that brief time. Start thinking about the next appointment, test, or referral and ask about your doctor about it to keep the momentum going because the system is prone to inertia.  Research the procedures and tests you will do to figure out what questions you have.

When my symptoms started, my primary doctor I had then didn’t believe me so I had to wait a long time before I got to see any specialists. So after the treatment he gave me made me worse and he ethically had no choice, I saw about 15-25 different specialists over two years and eventually realized I needed to go to a research hospital. It was one of the best decisions I ever made. This sped along the process because these doctors are better equipped to deal with complicated conditions.

Patients with complex conditions that affect multiple systems in the body have to suffer through fragmented care, meaning they see specialists who can treat one symptom but no one looks at the bigger picture. This is not your doctor's fault; it's unfortunately the way the system works. This delays diagnosis and treatment. This also means that the patient, who is suffering and ill, has to coordinate his/her own care. I spend a lot of time figuring out which test/appointment is next and being the line of communication between my doctors despite not having a medical degree, jumping through endless hoops while being ill. Did I mention being sick is a bitch?

2. Learn the language and educate yourself. Be your own advocate

The thing that makes me the most rage-full is that almost every person with dysautonomia has had to suffer through the “it’s all in your head” trap. Imagine suddenly dealing with disabling and even life threatening symptoms, being afraid, and coming to your doctor to ask for help only for him to tell you, basically, you are imagining it. This experience is almost universal for dysautonomiacs. I believe the fact that it affects mostly young women is part of the problem, but I’ll discuss that more another time. Even after all of these years and the definitive proof I have, I still deal with invalidation from doctors. I’m starting to think that it will never go away.

Therefore, educating yourself and presenting yourself as an informed patient is essential. This is daunting when you are very ill, but it is necessary. Learn the language related to your condition. When I talk to doctors I use words like “autonomic dysfunction,” “differential diagnosis,” “orthostatic intolerance.” Speak their language and more than anything else, advocate for yourself because no one else will do it for you. Refuse to accept the “it’s all in your head” trap and insist on being heard. Depressingly, fighting the medical system can sometimes be more difficult than battling your own illness.

Here are some great suggestions from other POTSies:

Jorden Chelsea describes the obstacles of invalidation from doctors:  “If I would've known then what I know now I would have made appts with endo rheumatology geneticists and autonomic specialists the exact day I got my diagnosis after my stress test. I wouldn't have ignored my symptoms for years and I wouldn't have accepted the misdiagnosis of anxiety from anyone, you know your body best but sometimes I've seen patients completely brush off a symptom they have if a doctor says it's nothing. Ask doctors who they know that can help with dysautonomia and don't be afraid to do your own research and present it to the physician you are seeing. Also talking to others who have the same issues within the area is helpful for finding qualified mds. Definitely compile a list of every single symptom you've experienced prior to your appt even if it seems unconnected. If I could go back I would not have stopped going to the doctors being persistent it took 6 years from the onset of symptoms to the slow progression for them to even order a stress test partially because I kept being told I was fine so I did not stay on top of appts.”

Christy Doe also offers some good advice: “Never go alone to an ER or doctor is my best advice. Then they have to treat you with respect and the dignity that you deserve. And there is a witness.”

Jessica Emerson reiterates this: “I've finally learned to stop going to appointments without a witness--some argue it makes you look funny..."Why does an adult need someone with them?" Because my memory and concentration are next to nothing by the time I've made it inside the exam room. Something healthy people can't understand.”

   3.  Learn the appointment, testing, and referral process

The medical system is slow, bogged down, and will work against you. It takes a lot of time, money, and energy to navigate it, which most sick people are in short supply of. Find out if you need to get a referral from your primary or if you can make the appointment. Because there can be a huge wait time for appointments, tests, and referrals, it is important to make them asap. You can also get on a waiting list and that may speed up the process. Anytime I have gotten a referral, almost inevitably I have not heard back about it. I always call to make sure the doctor’s office received it and try to set up an appointment. Sometimes a referral can get lost and eventually it expires. You could have to start the process again. Don’t be afraid to call and find out what the status of your referral is and if there is anything you can do to speed the process along.

When you finally get the referral and set up a test, you get to enjoy the fruits of your labor. I am not scuba diving here. I'm getting a VNG (videonystagmography). I really can't recommend this one.

4. Taking ownership of your own health: research, make connections, educate yourself  

Finally getting a diagnosis and getting off the hamster wheel is not the end unfortunately. Very few doctors will take the time or have the time to explain all of the details of your condition to you. You will have to gather information yourself. Connecting with other people online who have your condition is important as well, especially with rare conditions. Knowing there are people out there who deal with the same struggles is important for anyone. A word of caution: there has to be balance when researching and making connections. It is easy to make yourself crazy researching and sometimes I find that it is necessary to disconnect and take a break from thinking about being ill to focus on living.

     

      5.  Remember what you are fighting for and remind yourself of it from time to time

I have a had a few moments the last year of asking myself  “Why do I keep doing this? Why do I keep going through this?” I think we suffer through this process because we remember what it was like to have quality of life and to participate in that life. We remember what we have lost.  I don’t just suffer through this process for myself. I do it for my husband, my family, my friends, and all the things I still want to accomplish. You can write down for yourself who/what you fight for, and remind yourself from time to time that hopefully, in the end, it will be worth it. 

In the meantime, focus on what you can control and carve out a piece of happiness and joy where you can.

We can lament all day that the medical system is a for-profit system (in the US at least) that sometimes does the opposite of help patients and in which some who need care the most are denied access to it. We can cry foul that the undiagnosables with obscure illnesses must suffer because there is no profit in treating obscure illnesses, but this is not productive.

We have to work with and against the system as warriors. 

It even works against doctors. My primary doctor confessed to me the other day how much the system gets in the way of the quality of care he can provide patients. Luckily, we are living in an age when health records are digitally stored and more doctors are communicating with each other and their patients using technology. Technology has really helped the patient be involved in the process. The US is working on making health care more accessible and affordable. Patient advocacy and patient participation are becoming more prevalent in medicine . There is hope.

Your health is sacred. It deserves protection and cultivation. Don’t let anyone or anything intimidate you into thinking otherwise. And so we beat on, boats against the currents, keeping up the good fight.




 A patient with Crohn's Disease is working on a great project patients with chronic illnesses can use to keep track of their symptoms and treatment. You can find it here.

The Past, The Present, and What the Hell is Dysautonomia?

Last weekend, I was at my parent’s house not watching the super bowl, and my mom brought out a pile of pictures she was going through. She handed me a picture that gave me a punch in the gut. This is a picture from when my parents took my husband, my sister, and I to Hawai’i a few years ago:

I looked at this picture in shock and not recognizing this pre-sick version of myself. It made me sad. I showed it to my husband and it made him sad too. I don’t accept wallowing, but I do have moments of “whose reality am I living in because this isn’t mine.” Like stepping through the looking glass or that horrific awakening after being disconnected from the Matrix. This picture is like looking at memories from that sleek, spectacular world where everyone wears pleather and badass sunglasses while dodging bullets. Pre-sick is a stunning alternate universe. Looking at this picture was another “WTF happened to my life” moment. It does not do to dwell on the past, so I had that sad moment and then moved on. I laughed and told my husband “I looked so weird and fleshy then.” And damn, I miss that hair. 

What It Is:

This is the present. I was diagnosed with dysautonomia and POTS (Postural Orthostatic Tachycardia Syndrome) in May 2012 when I did my first Tilt Table Test. Dysautonomia and POTS are very rare conditions and are very rarely diagnosed.  It’s estimated that 500,000 people are diagnosed with POTS. There is no pamphlet or brochure for this condition and most of the doctors and specialists out there have never heard of it. There are no specific medications for dysautonomia. Many with the condition will hear from doctors that your symptoms are "all in your head." It's an almost universal experience. This is why it took me over a year to get diagnosed. It’s been almost 3 years and I’m still trying to get a more complete diagnosis. Once you get a diagnosis, a cardiologist and a neurologist are the first lines of defense in the battle.

I am no medical professional (though I would argue I may deserve an honorary medical degree at some point), but as far as I know a Tilt Table Test (TTT) is the only way to get a definitive diagnosis for POTS. The test sounds simple: you are strapped to a table while lying horizontally. Your blood pressure and heart rate are monitored continually and they lift you slowly up to vertical while still strapped to the table. My heart rate immediately increased almost 80 beats. 72 at horizontal to 145 at vertical. It was unpleasant. If your heart rate increases by 30 beats, you have a definitive diagnosis of POTS. I was earning extra credit with the 80 beats. POTS is subset of dysautonomia, which means a dysfunction of the Autonomic Nervous System. Not everyone with dysautonomia has POTS. There are a variety of subsets. Dysautonomia is is referred to as an "umbrella term." It is important to find out which subset you have to get more focused treatment.

The ANS controls all of the functions your body preforms automatically: breathing, heart rate, blood pressure, digestion, body temperature, sweating, etc. "Autonomic Dysfunction" means the body is no longer able to self-regulate these functions. Normally, when you stand your body is able to adapt to gravity and maintain blood flow to your essential organs. The words “postural orthostatic” and “tachycardia” mean that upon standing the blood pools in the legs, your blood pressure drops, and your body goes into overdrive to maintain blood flow to your brain. This is why fainting (syncope) is often associated with dysautonomia and POTS. This condition can be result of nerve damage so the nerves (your direct line of communication in the body)  are no longer working as the connection between the brain and the body. It’s like the body just suddenly forgot how to be a body (you had ONE job!). I explain to people that it’s like my “wi fi” was turned off and I swear I paid my internet bill. 

Symptoms typically related to dysautonomia include dizzinesss, excessive fatigue, GI problems (including gastroparesis and dumping syndrome), blood pooling, syncope, brain fog, difficulty sweating and maintaining body temperature regulation, tachycardia, bladder problems, hypovolemia, etc. Symptoms manifest uniquely for each individual, which makes diagnosis that much more challenging. 

Dysautonomia is not a disease; it is a symptom and a condition. The real struggle is not getting the diagnosis. It’s finding the cause. The Elusive Cause is more difficult to track down than Sasquatch and for most people The Cause is never confirmed. My doctor at Stanford believes The Cause for me is likely a conglomeration of genetic predisposition, multiple neck injuries (in gymnastics and a car accident), and other factors. The Cause is still elusive for me because my doctors couldn’t tell me why I had sudden onset of the condition. Most of the time people acquire dysautonomia after a major illness, often a viral illness. Recent research shows that dysautonomia also has an autoimmune component. It seems most dysautomoniacs (if not all) have a variety of other illnesses and conditions. As one of my doctors said to me with much appreciated empathy “Some people are just lucky.” Dysautonomia is incredibly unique for each individual and presents differently. It can be severely disabling to the point that some are unable to work. I was unable to work the first year and I work now but with varying levels of success.

There are very few physicians who have heard about this condition and only a handful in the world who are experts on the subject. If you get a diagnosis, I recommend seeking one of these individuals out at a specialty, research hospital. That was the best thing I ever did. I am now treated by an expert at Stanford University. There is autonomic testing which can help narrow a diagnosis but only a few places in the US that I am aware of have the facilities to perform these tests. Many of these tests were created at the Mayo Clinic. I’ve heard that’s one of the best places to go for autonomic dysfunction. 

There are no replacement surgeries for the nervous system. It can’t be grown on a rat and transplanted into your body. You’re stuck with that lemon. And here’s the kicker: there is no cure either. The only option at this point for dysautonomiacs is treatment of symptoms. But there is hope. Sometimes the condition improves on its own and sometimes it doesn’t. There is medication used off-label to treat dysautonomia/POTS and there are non-pharmaceutical options (my favorite kind) to manage the symptoms as well. Treatment for dysautonomia often involves medication that raises blood pressure such as Midodrine or Florinef, an increase in salt intake, exercise to build strength and avoid deconditioning, and other medications to manage the wide range of symptoms patients have related to the condition. I take a calcium channel blocker (Verapamil) to lower my heart rate, Florinef to help my body hold on to salt and raise my blood pressure, and salt pills. It seems that more people and medical professionals are becoming aware of this condition. 

There are a few great websites that explain POTS and dysautonomia that are indispensable to understanding the condition and finding treatment:

Dinet.org

The Wikipedia page on POTS

Dysautonomia International 

Mayo Clinic's page on autonomic neuropathy

The most important thing to remember is that there is hope and dysautonomia may be part your life but it cannot take away your humanity, your values, and your spirit. There are resources to manage the condition so that you can live the best life possible. 

Arming yourself with information is essential in the battle because you will often have to be the expert when talking to doctors, and taking ownership of your health is imperative. I stepped through the looking glass, but I came out the other side a warrior.