The No-Win Scenarios of Illness

I’ve been thinking about no-win scenarios quite a bit over the last few years because illness is starting to feel like one. I’m starting to think that I am living in my very own Kobayashi Maru simulator.

*It’s going to get nerdy for a second* In the Star Trek universe, the Kobayashi Maru is a simulated test for Starfleet cadets. They are presented with a scenario that is rigged to cause destruction and loss of life no matter what choice is made, and the cadet's response to the test demonstrates their character and leadership skills. The conditions of the test ensure no can “win” it.

My last few appointments really solidified this for me.

Going to Stanford two weeks ago included a lovely stay at our favorite hotel in Palo Alto that was a serious splurge (but so worth it) and a very anti-climactic appointment. I had been waiting to see my neurologist for a year, and in that time I had to leave my dream job, stop driving, and I rarely leave my house. I went through more testing than I can remember in a year, on top of all the testing of the last 5 years. I had to start using a wheelchair, which I must say I’m finally getting pretty good at using. So much has changed. Appointments with him are usually about an hour, but I only got 20 minutes with him because he was running behind.

He explained that although the signs may point to Myasthenia Gravis, none of my test results can solidly confirm it, and he said we have to be absolutely sure because my treatment options going forward if that is the diagnosis “will not be good.” They can cause as much harm as they can help. Woo hoo! 

I have to do a CT scan of my thymus and then potentially have surgery to have it removed. Sometimes Myasthenia can be caused by an enlarged thymus or benign tumors on the thymus. A scan will show if it is causing trouble for me.

Other treatments for MG are immunosuppressants like Prednisone, which are known colloquially as “magic poison” for a reason, IVIG, or plasmapharesis. A constant dose of Prednisone can cause long-term damage and lead to other problems, but many people with autoimmune diseases rely on it. IVIG can also cause other problems. My new local neurologist who I saw last week explained all of these options are “multiple steps up from taking medication and dealing with side effects. We have to be sure.”

I already knew about these treatment options for some time. After I saw the note that said “serological Myasthenia Gravis” on my bloodwork a year ago, I have done a lot of research since then. I had never even heard of MG before that or even knew what a thymus was until a year ago. It’s amazing the things you learn through this process. 

I also learned that the medications I’ve been taking for MG and POTS contradict. I’ve been taking Mestinon for about 8 months, which is typically used to treat MG, and it has been a life saver.  It has made such a huge difference. However, it aggravates some of my POTS symptoms so I’ve had to take a minuscule dose and work my way up to a regular dose. The Florinef I’ve been on for 3 years is contraindicated for MG because it causes muscle weakness and is likely contributing to my breathing problems, which explains why I have never ever felt good on it. My attempts to get off of it have been unsuccessful but I have to keep trying.

I guess this is the thing with comorbidity: medicine is not an exact science and your conditions can conflict, each trying to prove their alpha status and dominance. So if I get the Myasthenia diagnosis I feel screwed with the treatment options. If I don’t, I continue to be exiled in the grey haze outside of the nirvana of diagnosable conditions, still waiting for a tangible label.

In the bigger picture, I’ve had to slowly accept that these conditions are likely lifelong and now it’s time to figure out what the way forward will look like.

It always feels like every ounce of progress that is made must come with some sort of setback or hardship, like the game is rigged for an unwinnable victory. Yet, I believe “defeat” and “success” are not absolute terms. They can be self-defined inside and outside of the parameters of this chronic illness game. I believe we are masters of our own destiny despite whatever external forces try to push and pull us from our trajectory. 

So if I really am in a Kobayashi Maru simulator, I need to figure out some cheat codes to either cope with these seemingly unwinnable scenarios or hope that someone accidentally steps over the plug and I go back to my normal life.

Stay with me on this metaphor. Recognizing that illness is like a no-win simulator is not a matter of resignation—it is a hail Mary, hard-fought, grit-filled realization that acceptance is the only way forward. It is the courage in realizing that you may not win the war but you won’t give up the battle, no matter how ugly it will get in the trenches. But it doesn’t have to be a no-win scenario. You have to find other ways to “win.”

You can change the game, change the rules, change perspective. Dig deep and find your inner James T. Kirk and change the conditions of the game and get away with it on pure charisma and bad-assery.

You have to find your own cheat codes. Even if we can’t rid our bodies of illness, we do have the power to change our perspectives. These are some “cheat codes” I’ve come up with, things I think of or try to remember while fighting what seems like a no-win battle.
*You can find a printable version here

     1.   In the dark depths of a bad day or a bad week(s), try to remember that they serve to punctuate the sweetness of the ok days and even the once in a blue moon great days

     2.   Remember what you do have control over in terms of your illness and your life, whether it be your diet, your routine, your exercise efforts, your attitude, your will to keep going, your passions

     3.  The hard times and dark periods are like battle medals: you fought hard to win them. You survived this long, so you can survive whatever may come

     4.   You can live in denial that leads to endless frustration, or you can live with acceptance and find a path forward for the best life possible. This one took me a long time to really learn

     5.   The past and future barely matter. The present is really the only reality that matters. Live the fullest life you can in just this moment. 

     6.   You are the ultimate decider when it comes to how you handle and treat your condition. I really believe that we still have choices, even when it comes to medical treatment. Your intuition is an asset. Hold onto your voice because you are not powerless

     7.    Even if you have a tenuous relationship with hope and you and hope part ways for a brief time, hope will always be there waiting for you to return when you need it most

     8.   You have a story to tell that matters. Never surrender it

     9.   You play this game long enough, you get even better at it

    10.  Success and victory can be self-defined. The conditions of the game may constantly change and continue to seem insurmountable, but you can adapt and “win” each day on your own terms

Bonus: Chocolate is a great listener 

Keep up the good fight no matter what obstacles are placed in front of you. Cheat if you have to. We got this. 

Why Getting a Diagnosis Matters

I’ve been thinking a lot about why getting a diagnosis matters lately. Because many with Dysautonomia or other rarely diagnosed and invisible illnesses must go through months to years of appointments and testing to get a diagnosis, I have often wondered why we fight for one despite the constant obstacles and hurdles to obtain it. For many illnesses, especially rare and complicated ones, time to diagnosis is typically six years or more.

In 2011, I spent a year going through endless appointments and testing before I finally got diagnosed with POTS. I’ve spent most of the time since then going through endless appointments and testing to figure out what else is going wrong. Comorbities are common for Dysautonomia patients and they can spend years trying to obtain differential diagnoses. Never expect life to be easy, that's for sure!

I’ve mentioned before that about a year and a half ago I suddenly started having difficulty walking. I’ve gone from using a cane, to a walker, to now having to use my wheelchair if I leave house in that short amount of time. I am rarely able to leave the house at this point. Leaving the house is overrated anyway. Except, I miss it.

When I mentioned my walking issue to my doctor’s nurse practitioner when it started she basically told me I was imagining it. Invalidation all over again. So I tried to believe that for many months. But then I couldn’t get around and had to get a cane. I was continuing to lose my mobility rapidly. Hey, every 30 something goes through that difficult period of trying to figure out life while losing their mobility, right? Well, maybe not. I wasn't imagining this.

Having just one random illness no one has heard of is not a thing

I finally saw my neurologist this year. It had been two years between visits (the dude is popular and busy), and I told him what I was experiencing along with other disabling symptoms, he gave me a list of tests I would need to do. My heart sank. He was taking me more seriously but I knew all of those tests and appointments meant there would be no answers for at least another year. That was over seven months ago. I have only gotten progressively worse since then. My couch has a permanent Stefani shaped dent in it. 

While I fought to get the POTS diagnosis, I never dreamed of giving up. I'm in my fourth year of seeking a more complete diagnosis. The drive to give up has been surfacing from my subconscious, but that's not in my nature.

In one of the blood tests, I tested positive for an antibody for Myasthenia Gravis. Loss of mobility, weakness, difficulty breathing, and fatigue are signs of this autoimmune condition. In two weeks I do another test for MG. Maybe a diagnosis? My doctor had me start a medication last week that is used to treat this illness. But still, no official diagnosis. After all this time, will I finally know? But this led me to question why does a diagnosis matter? I am technically already being treated for this and have been learning how to cope with it, so why does the label matter?

Not many people want to be pigeon-holed and labeled. When you are desperately ill, that label is a like a trophy, engraved with the words "I Was Right!" Some never get a complete diagnosis and must suffer the costs of uncertainty long-term, but many of these patients still search for a label for their suffering. A label of an official diagnosis can matter for many reasons.

   Official Recognition and Documentation

Insurance companies and the medical system seem to operate in a very black and white world although there are some of us who live permanently in the grey area in between. There is nothing cut and dry about rare, complex conditions. Yet, a label and diagnosis fits into that tiny square in many medical forms titled “medical conditions" (good luck fitting more than one condition in that square). Having to list a grab bag of symptoms doesn’t seem to hold much weight in their world. Sometimes a grab bag of symptoms leads doctors or medical professionals to instead give a patient a psychiatric label such as “depressed” or “anxiety.” It’s easier than admitting they don’t know sometimes or investigating further. The irony is that depression and anxiety often are an effect of disabling symptoms met with invalidation, not the cause.

This is unfortunately incredibly common for many Dysautonomia patients. There can be serious ramifications from this invalidation. A lack of diagnosis could lead to patients not getting approved for a needed test or treatment option. It could mean a patient cannot get needed accommodations at work. It could impede accessibility to essential services for the chronically ill and disabled.

For those who are so disabled that they are unable to go to work or attend school, an official diagnosis could be a life line. This could help them qualify for disability or other types of assistance. For many, a diagnosis is essential for these practical concerns.

Often when you are chronically ill, you will encounter many doctors who do not know your medical history, you will have to fill out stacks of paperwork, and you will need to communicate to the medically uninitiated (such as employers, family members, etc) your needs. A diagnosis provides a concise explanation. 

Truth really is stranger than fiction. You can't make this stuff up. There’s nothing quite like having an entire Wikipedia page dedicated to your rare condition that you can direct them to when you have a diagnosis. The label leads to vital information and resources. It provides you with a prognosis and information to understand your body. A grab bag of symptoms rarely does. That leads to fruitless internet searches and typing into Google "am I dying?" The label can matter.

 

     Validation (Hey, I’m not just crazy!)

Finally leaving the inhospitable world of uncertainty behind for the more ordered, life-affirming nirvana of diagnosis gives patients validation, especially if they have been told they are wrong by many doctors. It lets patients at least take a break from the endless hamster wheel of diagnosis. This has practical financial, legal, and bureaucratic concerns as I mentioned above, but it also assuages the psyche of those who are desperately ill but have been denied proper care and treatment under the label of mental illness.

When I was finally diagnosed with POTS, I wanted to sing in the streets. Then I wanted to send the primary doctor I had who told me over and over “you’re just stressed” and ended up making me worse a flaming bag of dog poop. Ok, I won’t do that but I still think about it.

After being told by so many doctors that I was wrong, I was finally right. When you get a diagnosis, it's like reading a prequel to your life. The plot holes, disjointed timelines, and weird flashbacks of your story start to make sense.  I have learned that sometimes you will still encounter invalidation and continue to have to “prove” you are ill, yet you can wield a clinical diagnosis like a weapon against these forces. You aren’t wrong. You have proof. The label can matter. 

     Treatment

Perhaps most importantly, a diagnosis can lead to treatment options where there were none before. As with most rare conditions, no specific drug has been created to treat Dysautonomia and POTS as a whole, but there are drugs that treat symptoms such as Midodrine. Drugs are typically used off-label for Dysautonomia and POTS, such as Florinef (which was created for Addison’s Disease). The FDA did recently approve Droxidopa, an Orthostatic Hypotension drug.

It is difficult to treat a patient without a specific diagnosis, so finally getting that label gives a patient some options as opposed to few or none. Even with a probable diagnosis, you can have treatment options. For example, since my doctor suspects Myasthenia Gravis, I have started taking Mestonin and started physical therapy. Proper treatment can provide hope and can lead to a better quality of life. The label can matter.

 

 ***

HuffingtonPost posted an article listing other, innovative avenues for patients with complex conditions to pursue when you are trapped on the hamster wheel of diagnosis. POTS is even mentioned in the article, how about that! Depending on what happens over the next few months, I may pursue #3. 

I have been telling myself over the past year that perhaps a diagnosis doesn’t matter, but that’s the drive to give up talking. It does matter. The process to get there is painstaking to say the least but I have written before that there are ways to navigate it, which is here. 

Even if I also become one of those who must suffer a label-less existence, I know I'm not wrong, and that's what matters the most.

My last trip to Stanford Hospital. Keeping up the good fight

Why does getting a diagnosis matter or not matter to you?

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.