Susannah Cahalan, a journalist for the New York Post, was afflicted with a very rare autoimmune disease called anti-NMDA-receptor autoimmune encephalitis. Where my type of autoimmune disease affects the nerve roots and autonomic nerves, this type of autoimmune disease affects the brain. Susannah ended up writing a novel documenting her experience called “Brain on Fire, My Month of Madness”. She writes on a few statistics that I thought were shocking:
“Although anti-NMDA-receptor autoimmune encephalitis is rare, it is one of the more than one hundred different kinds of autoimmune diseases that afflict an estimated 50 million people in the United States, a staggering figure that has more than tripled in the past three decades. An alarming majority of autoimmune diseases–around 75%– occur in women, affecting us more than all types of cancer combined. Autoimmune diseases are most likely the number one cause of disability in women of all ages. There are multiple theories about why women are so disproportionally affected, ranging from genetic, to environmental, to hormonal (most women are of childbearing age when they are diagnosed), to the fact that women’s immune systems are more complicated (they need to identify and safeguard fetuses, which are half-foreign entities, during pregnancy), and with everything more complex, malfunctions are all the more severe. For now, it’s just one more riddle in a series of question marks.”
I love this book. I feel a kindred spirit with Ms. Cahalan. Although we suffer from different types of autoimmune diseases, it is shocking to me how similar the experiences are. I feel like I could have written this novel. She so eloquently describes the pains, the frustrations, the sufferings, and the healings that I have desperately attempted to put in to words. In one chapter, she documents the frustration with gaining weight due to steroids and other medications necessary to keep her disease at bay:
“I know now that I focused on my body because I didn’t want to face the cognitive issues, which were much more complex and upsetting than mere numbers on a scale. When I worried about being fat forever, marred in the eyes of those closest to me, I was actually worried about who I was going to be: Will I be as slow, dour, unfunny, and stupid as I now felt for the rest of my life? Will I ever again regain that spark that defines who I am?”
Being chronically ill changes who you are. I have had to adjust to a life a little less glamorous than the one I once led. I’ve had to cope with laying in bed most of the day–everyday. I’ve had to come to terms with the fact that I may not ever live my life at 100% as I once did. I may not be able to return to nursing–the occupation that I knew I wanted to pursue since middle school. I may not be able to play the sports that once shaped who I was.
But through this experience, I’ve learned things that I may not have otherwise known. I’ve learned perseverance–how to press on when it seems the world (or at least the medical world) is against you. I’ve learned who is close to me and who will stick by my side even through the most boring, endless of circumstances. I’ve learned to knit. Learned to write–and hope to one day document my experience as Susannah Cahalan did so beautifully.
My doctors finally agreed to trial IVIG. It could start as soon as this week. Assuming my insurance approves it, I will be getting six infusions of 1 gram each of IVIG over three weeks–two infusions a week. This could end up costing as much as 120,000 dollars. Fingers crossed, I won’t run into any trouble with the insurance company who has proven to be anything but supportive throughout my illness.
Pray for me as these treatments will likely cause a host of side effects–aseptic meningitis (a nasty headache that they pre-treat with Demerol), and flulike symptoms, body aches, fever, general malaise. These shouldn’t be too hard to endure as I have dealt with similar things for a year and a half now. But there are worse side effects that are possible including blood clots, stroke, and anaphylaxis due to my IgA deficiency. (I need to remind the infusion nurse to bring an epi- pen.) I will keep you all posted on how the treatments progress. After a year of wanting the treatment, I’m finally getting it. Let’s hope it works.
I remember reaching downtown just as the sun began to set. San Francisco at dusk is a beautiful, bustling place. Brakes squeaking, fluorescent lights illuminating bars and restaurants. Riding road bikes through the streets sounds fantastic, but in the dusk, reality proves dangerous. Obstacles abound: trolley tracks that attempt to catch your thin wheels, taxi drivers whose patience is thin, not to mention the steep hills that define the city. As the orange light breaks through the small crevice between the grey cement sky scrapers, I give up. I get off my bike yelling to my companions, “I don’t have health insurance!” I thought I was cutting it close when we were descending the steep hill into Sausalito after the Golden Gate bridge. One patch of sand or small rock and us novice bikers could have met our Maker. After riding the ferry back to the city and attempting to ride fast to make it home to Nob Hill by dark, I had enough.
I have always been a timid person. Yes, I realize I have white water rafted on the Nile, bungee jumped over gator infested waters, and swam in a rip tide that the next day killed six surfers with surf boards. That may not sound timid to you, but in my mind, I always knew these activities were dangerous. Like a little girl winces when the waves wash over her feet at the beach for the first time, not knowing whether or not the cold, salty water is something to be trusted and enjoyed. The difference between these activities in my past and this bike ride was simple yet substantial: health insurance.
I was 26 and needed to be responsible. If my wheel caught in the trolley tracks causing my bike to tip and my leg to scrape against the pavement, being dragged for 10 feet before coming to a painful stop, I would have to incur the ridiculous costs of the emergency room visit and subsequent treatments thereafter. Money I did not have.
Needless to say, I jumped off my bike and began pushing it up the sidewalk. My friends were laughing at me as they continued on bikeback all the way home. I didn’t care. I could not risk it knowing I didn’t have the means to clean up after my mistakes.
Knowing now what I didn’t know then, I would have continued on my bike ride. I would have finished the journey, finished the fun. Being sick and not being able to ride a bike makes me realize what I’m missing. I miss the laughter of my friends as we barely escape hitting a taxi. I miss the burn in my thighs as I push up California leaving the Financial District in my dust. More than anything, I miss the freedom I had, freedom from the burden of health insurance, financial stresses, and sickness.
And I miss the bike rides.
I promised myself I wouldn’t be Debbie Downer (wah, waaaah). I have neglected to write because I have nothing good and hopeful to say. But today, I changed my mind. I read:
“Maybe your [experience] was grim and horrible, but grim and horrible is okay if it is well done.” -Anne Lamott “Bird by Bird” (4).
My experience is real. And I will document it accordingly.
Sometimes I catch a glance of myself (or is it?) in the mirror. I barely recognize myself.
It has been a long 8 months since starting Prednisone, the steroid that saved my life and ruined it at the same time. What I mean by this is that it shocked my system and made it believe it was ok. It gave me the energy lacking that got me out of bed for the first time in months. I was able to walk down the hall to the bathroom without getting short of breath. My systolic blood pressure was now above 100–a large improvement from it hanging out around 80 causing dizziness, nausea, and lethargy. Not only that, the Prednisone helped the pain. On a scale of 0-10, my pain had been an 8. Now thankfully it was vastly decreased to a 6–note the sarcasm, but I will take it.
I would need pages to tell you the side effects of the Prednisone. This is the reason that it is typically avoided for long term use. I have gained 25 pounds in the last eight months. My face, though it has always been round, has increased in its puffiness. My face flushes randomly and consistently. I wake up in night sweats, drenched and wondering if I have a fever, but remembering Prednisone causes this. I have anxiety. I can not sleep more than a few hours at night–even with sleep aids. My heart races. I have heart burn and reflux. The part of the brain that regulates the production of certain hormones has actually atrophied (died) because of the Prednisone. My body has stopped producing regulating hormones because it is used to the synthetic version I take daily. This means it is almost impossible to wean off of the drug.
I have tried to wean off the steroids multiple times in the last eight months. Every time my blood pressure crashes, my whole body aches as if I have the flu, and I become short of breath when I stand.
How do I cherish the journey when that journey is so difficult? I am wearied with each passing hour. When, if ever, will I catch a glance of myself in the mirror again and actually recognize the face staring back at me?
I remember the first time. I looked up at the mirror and I flashed back to my days as a nurse working with transplant patients (though not long ago, it feels ages away). They take steroids and immunosuppressants daily to prevent the body from rejecting the new organ. My body decided to reject my organs–sans transplant. I remember thinking, “I look like my patients”. Pale, Cushingoid. When did I come (more like stumble) to this side of things?
More than anything, I can’t decide which is worse: the symptoms of the disease or the side effects of the treatment.
“Radiculopathy: refers to a set of conditions in which one or more nerves are affected and do not work properly (a neuropathy). The emphasis is on the nerve root (radix = “root”). This can result in pain (radicular pain), weakness, numbness, or difficulty controlling specific muscles.” (Wikipedia)
Part of my autoimmune problem includes radiculopathy. I have had pain since the beginning (November 2011), and in fact, my neurologist related a problem I had 3 years ago with a radicular pain to this disease process. Recently, the pain has been getting progressively worse. Today, the worst is the L5 nerve root. It initiates in my lower back and travels down the front of my leg to my knee and most recently to the heel of my foot. This has caused weakness and immobility of my leg eliciting yet another ER visit and multiple followups.
I had another EMG done on my leg. This test tells us whether or not there is an active disease that is damaging the nerves–this is hard to explain. Previous EMGs have showed chronic changes in the L5 nerve root, but no acute damage. This means that the nerve was damaged at one point with disease, but there is nothing new going on. This test was no different.
Basically, the neurologist thinks the autoimmune process is progressing and causing the worsening of the pain. Then comes the long trial and error process to find a medication that helps the pain. I am already taking the maximum dose of an expensive nerve pain medication called Lyrica–so a neuropathic medication is not an option. I tried Sinemet , a drug typically given to people with Parkinson’s disease to reduce tremor. The problem with this medication is that it can affect the autonomic nervous system–which it did in my case, and caused worsening of my autonomic dysfunction. Next, I tried Baclofen, a muscle relaxer typically given to people with MS. This also worsened my autonomic dysfunction. Finally, just this past weekend, I was able to begin a medication called Nucynta–a pain medication that works through multiple pathways to reduce pain and is especially effective with nerve pain. This medication is also very expensive and was denied by my insurance company 4 months ago. My pain management specialist worked hard and got the medication approved. Luckily, I have experienced some relief from the pain with this medication (FINALLY).
What is frustrating is that my doctor prescribed this medication 4 months ago and I was unable to take it because someone behind the desk at my insurance company decided they would not cover it. Even though I am incredibly thankful that I am getting some relief, I can’t help but wonder what the last 4 months would have been like without the terrible pain.
Still waiting on the effects of the immunosuppressant and the decision for IVIG or not.
So I thought I would provide an update on my diagnosis/treatment journey.
About a month ago I came to the end of corticosteroid treatment that proved beneficial. I started to show signs of Cushing’s syndrome–moon face, weight gain, etc. (and no, I won’t post a picture). At this point, my cardiologist and I know that I need to begin weaning off the prednisone because the risks are now outweighing the benefits. Unfortunately, the doctor that is now in charge of my care, the researcher at EVMS, is not completely convinced that my autonomic dysfunction and multifocal radiculopathy are caused by an autoimmune process.
As his team is meeting and discussing what their plan for me is, I become sicker and sicker. About 3 weeks ago, I was at the point to where I could not get out of bed. Even making it to the bathroom was too much of a task. When I lifted my head, the room spun. I was short of breath and my heart rate was extremely unstable–reaching 160s-170s at rest. I knew I needed to be proactive and force the issue with the team.
I came asking for IVIG as my gut was telling me this is an autoimmune problem. The PA I saw was very attentive. He listened and I knew he heard the distress in my voice. I was at the end of my rope. I needed a treatment plan ASAP. Even though I tried to remain logical and clear, my emotions took over as I pleaded for the treatment. I tearfully asked him, “Matt, what would you do if you were me? What would you do if you had two physicians–your neurologist and cardiologist–who you trusted immensely, recommend this treatment of IVIG, and one physician who is the expert who wants to hold off?”
Matt took a deep breath as if he was really putting himself in my place. He hesitated as he answered, “Please don’t feel as if we are withholding treatment from you. That is the last thing we want to do. I will take your concerns to the head of the team and I will let them know your position. Call me on Friday (it was Monday) and I will let you know what they say.”
That week was agony. Not only did I feel terrible physically, but my mind was racing with what the team would come up with. Would they still not recommend the treatment? Would I have to return to the Mayo Clinic? Would I have to push the conversation and force the issue? All I want is to be a patient. I want to stop feeling like I need to drive the treatment process because all the doctors want to do the absolute least possible to get by and avoid liability. I just want to be the patient.
To my surprise, Matt called me himself on Friday morning. He informed me that they wanted to start a 6-8 week trial of Imuran–an immunosuppressant. This trial was to give us a better idea if the cause is immune related. If I begin to feel better, and objectively, my autonomic functions improve, we then will know if IVIG would be beneficial.
I couldn’t believe what I was hearing. He came up with a plan, provided a timeline, and even let me know that he had called my cardiologist and collaborated and agreed on a plan. Can you believe it?
I got a CBC drawn on Monday to make sure my WBCs were normal prior to the initiation of treatment. On Tuesday, I started the Imuran. I finally feel like I am comfortable with what is happening. I am in agreement with the plan and am hopeful this treatment will help guide us to a diagnosis. We will see if my gut was right and my disease is immune related. Fingers crossed I will have IVIG in 6-8 weeks.
And so we wait.
Do you want to know what makes me sick (no pun intended)?
The insurance company denied my Long Term Disability claim stating that there was not enough evidence to approve the claim. Therefore, I will effectively be terminated from my job this week–and lose my health insurance. The irony in the situation is that if I were to go apply for health insurance to cover me during my unemployment, they undoubtedly would deny me coverage based on the fact that there is evidence that I have a preexisting condition.
What in the world is wrong with this picture? The sick patient, who has already been thrown around the system–tossed from physician to physician, specialist to specialist, hospital to hospital–trying to get to the bottom of a complication health situation, has just been forced into the cracks– the cracks in the bottom of a broken system.
Basically, the marginalized patient is forced to:
- get medical care dictated by insurance companies not by the world renown specialists that deal with their conditions daily
- complete endless amounts of paperwork to claim, appeal, and fight for disability to cover medical and living expenses during their illness
- focus more on red tape than healing
We need to fix this problem.
I read an article recently that said the average length of time from onset of symptoms to diagnosis of autoimmune disease is 12 to 15 months.
That may not seem like a long time to you, but to me, it is a lifetime. Twelve months of vague, yet debilitating symptoms. 1 year of multiple specialist appointments a week. Countless blood draws, MRIs, EMGs, neurological exams. ER visits. Out of town specialists. 20 different medications. 40 different side effects to those medications.
In 8 days, I will reach that 12 month mark. Lets throw a party.
I do feel like I am close. I have an actual diagnosis of “autoimmune disease”–but they still don’t have the specific one. I want to name it: “Autoimmune disease that affects the autonomic nervous system and causes multifocal radiculopathies.” I guess that may be a bit long winded. Apparently I also have Schmidt’s syndrome- otherwise known as autoimmune polyendocrine syndrome type 2– and Hashimoto’s thyroiditis.
Autoimmune diseases are complex. Patients with these problems face pain, fatigue, changes in regulatory systems, and countless other issues. To doctors, patients who are seemingly healthy looking that complain of these symptoms often get misdiagnosed with things like depression, anxiety, stress, irritable bowel, and overuse injuries. At some point a blood test such as a positive ANA or an IgA deficiency will peak their interest and cause the physician to investigate further–or, in most cases, send the patient to a specialist who will investigate. What has really struck me a CRAZY is how sick I had to get before the physicians really took hold of my case. Now, I have 3 amazing doctors- a neurologist, a cardiologist, and a neuroendocrinologist–who are just around the corner from a larger diagnosis and treatment option. (Hopefully IVIG).
Take heart: As evasive as they may seem, autoimmune diseases can be managed. If you are going through this right now, all I can say is press on. Get to a doctor that will take the time to listen to you and really take in to account what your symptoms are. Don’t give up if the doctor tries to simply manage your symptoms. Do the best you can to eat right, drink fluids, and sleep well. Keep going. We will get there.