Is There A Fundraising Walk For That?

My world is heavy these days and appears to be getting no lighter in the near future. This past week I was evaluated by a rheumutalogist *(that is probably spelled wrong but my spellcheck will not provide me with another spelling, so be it...), an ophthalmologist, an endocrinologist, one neurosurgeon, had a phone consult with a neurologist who specializes in seizures, had an emergency brain MRI, more labs that NIH probably handles in a busy month and capped off the week on Friday with the neurocognitive neurologist appointment. Herein out for the sake of typing efficiency, the double-neuro will be Dr. NeuroSquared. By the way, what is with my Friday afternoons these days? A far cry from pretending to stress about exams while enjoying dime beers at the Balcony. (I refuse to call it Top of the Stairs. End of Discussion.)

I have only about ten ounces of blood left in my body after the vampire nurses have had their way, and for someone who never adopted a street drug habit, am sporting some mighty impressive track marks. Anemia nonwithstanding, I managed to purchase an Easter basket for Ethan. And by Easter basket, I mean, of course, an Easter wicker hamper ... this thing is stuffed with enough toys and candy to keep a brood the size of the Jolie-Pitts busy for most of spring. Overcompensate with material things to make up for Sick Mommy Guilt Syndrome? Me? Never!

First, let me say, I have wonderful wonderful friends. Thank you so much for all the phone calls and emails and flowers (love the flowers!). Again, I am so sorry I am not getting back to anyone these days ... what with the bloodletting, the appointments, and all the sleeping in the between appointments and bloodletting ... well, there you go ... that has been the past couple weeks. I have not been getting back to anyone - not just you - I promise. Even though I have been a terrible correspondent in recent weeks, I so very much appreciate all of your kind words and thoughts and prayers. Please keep 'em coming.

I have a theory that there are three kinds of sick. There is Annoying Sick, this is the kind of illness otherwise healthy people get, the kind where you complain to friends on Facebook about how you have to take Sudafed and watch Oprah all day. There is Lifetime Movie Channel Sick, this is some sort of life-altering, even life-threatening, diagnosis that warrants enough drama to be interesting and heart-wrenching enough for a two-hour movie (maybe watched while one is Annoying Sick). LMCS requires the patient "fighting" and being "courageous" and having family and friends rally around them through treatment. There is usually some sort of walk for this type of thing. And then there is Scary Sick. Scary Sick is where you are actually jealous of LMCS people, which is, in a word, sick. I am sad to report I may be Scary Sick.

The good news is that the tumor appears stable! The bad news is ... read on ...

The dreaded appointment was to review the results of my neurocognitive assessment. I was referred to Dr. NeuroSquared, after the neurosurgeon at Hopkins noted my self-reported memory and cognition problems. The neurosurgeons' recommendation was to see this "specialized" neurologist to get a baseline cognitive function exam and see if she could shed light on why I was having reported cognitive difficulty (he did not think my brain tumor was causing those issues but could not tell me what else could be). The cognitive exams included neurological assessment and detailed cognitive function testing, which included, among other things, evaluation of memory, executive function, and fine motor skills.

I walked out of the testing feeling ill, knowing my results would be awful. I could not recall ANYTHING. I was shown a figure and then asked to draw it from memory and could not. I was given a list of fifteen words and asked to repeat them back and could not. I had to put pegs in a grooved pegboard with each hand and had the fine motor skills of a one-hundred-year old (testing showed me at the 2.3% percentile for this). I did not know the time or the date or who the President before Obama was (that one might be selective memory blockage - sorry Republicans.)

While I had been lamenting to Jeff for months and months that I cannot remember anything and was getting worse, he claimed that he did not notice. I knew, though, but chalked it up to the tumor in my brain. Most logical people would. Even if they were telling me I was having no impairment effects from the tumor, it seemed obvious to me that there were memory issues, and I underplayed how bad they were, thinking the thing in my brain explained them away, that since I had not walked out of the house wearing just underwear (*yet), I was probably still OK.

It seems insane to me, in retrospect, that Jeff did not notice I was such having substantial memory impairment. He sees me every day - but he claims not to have noticed. I think in his defense, I have always been more than a little attention impaired - LOOK SOMETHING SHINY! - Wait, what was I saying??

Yes, attention: I have on more than one occasion neglected to put my car in park and it rolled into something (on the way to a graduation celebration, that "something" was a lake), I have left the house with two different shoes - one brown, one black, different heel heights - and headed to work (to the amusement of my then-secretary), I am terrible with names and if I say, "Hey YOU! It is so good to see YOU ... so good! ... YOU, there .... have you met my husband ... Jeff, this is that PERSON I was telling you about ... ?", it should be clear to you that in my mind you are Whats-Her-Face-That-Lady-With-The-Weird-Cat-That-Walks-On-A-Lease. Or whatever.

That has always been me. Spacey, yes. Dumb, no. Though, admittedly, sometimes it is hard to differentiate between the two, so I get how maybe he would not notice.

But, attention and cognitive skills involving memory and recall; the two are different animals. Despite the car-rolling footwear-ignorant name-forgetting, in the past, I have always had a virtually photographic visual memory. If I read it once or twice, it will be stored somewhere (and I read everything). School was always easy for me. Scratch that, school was always easy for me, except for law school, which is only easy for genetic freaks (Hello! to one-half of my Facebook friends and former Law Review Editors - I meant "genetic freak" in the nicest possible way!) or people disinterested enough not to care (Hello! to other half of those Facebook friends!). Point being, I always tested well, for what that was worth, and this fact was due largely to a better-than-average ability to recall.

My husband can attest that in the past, I had been blessed (?) with the ability to remember conversations verbatim and all corresponding details. This skill has proved useful in court, once or twice, but more often than not, serves no purpose in any sort of real-world context. Most marriage counselors would agree that no good will come of interrogating your husband about arguments you had a decade ago -- even if you KNOW you are right AND he still has not apologized (*just saying' J).

Poor Jeff has endured far too many conversations like this over the years:

You sir, are INCORRECT. What you actually said - seven years ago today - was that you thought Mary Sue Misckey was extremely ATTRACTIVE and you might date her if we were not dating, but we WERE dating, so you would not, but then you said if we had an open policy about dating, maybe you consider it. And then you ordered the Oysters Rockefeller and dropped your napkin on the floor by accident and then you asked Joe, our server, for another napkin, and then I said "Whatever!" and stomped off to the bathroom and the bathroom had sage-colored walls - no, no, more of a celery, really - and I looked in my Louis Vuitton (knock-off) bag and discovered I had left my Orchid Passion Madness lipgloss at home and so I returned to the table and we ordered entrees - filet for you, sea bass for me, I substituted the potato gratin for the roasted tomato - and your filet was undercooked, but tender. HOW DO YOU NOT REMEMBER THAT?!

I think if you marry a lawyer - especially a female one with tendencies toward the neurotic (all part of my charm) - you are on notice that such conversations will happen. That is my way of making a blanket apology for all such past conversations, without really apologizing. Hmm. So I M attempting humor because if I dwell too long on the real subject of this, I do not know how I will keep putting one foot in front of the other. Have you ever had an experience so bad that you do not dare let yourself start crying because you are afraid you will not be able to stop once you start? That is where I am right now. Jokes, no tears. Because the breakdown - once it comes - will be epic - and I just not ready for that. Not right now.

So, back to two Fridays ago ... I had the testing done and was told by the tech that the report would be ready for me on Monday. I was also told if this highly-specialized neurologist wanted to see me, she would call and bring me in (she only sees four patients a month). If not, then the report would be sent to my neurosurgeon and he could review the results with me.

Dr. NeuroSquared called the day after testing and I was on high alert. Red Flag Number One: She wants to see me. This meant I passed the threshold Objectively Screwed Up Enough To Be Medically Interesting cut. Red Flag Number Two: She refused to send me the report ahead of time, telling me "we had to review it in her office." Famous last words.

The testing proved mild cognitive impairment/dysfunction. What does this mean?

This is from NIH's publications website: Mild cognitive impairment. (from International Psychogeriatric Association Expert Conference on mild cognitive impairment). Mild cognitive impairment is a syndrome defined as cognitive decline greater than expected for an individual's age and education level but that does not interfere notably with activities of daily life. Prevalence in population-based epidemiological studies ranges from 3% to 19% in adults older than 65 years. Some people with mild cognitive impairment seem to remain stable or return to normal over time, but more than half progress to dementia within 5 years.

As explained to me, despite the name, "mild" cognitive impairment for my age, level of education, and former occupation (I am not practicing right now and have handed off all my cases on an emergency basis), is not considered a "mild" finding. Given those factors - as explained to me - my memory and executive function are *way* below what should be expected. My test results might be acceptable in the context of a 90-year old with a GED, but as applied to me - age and education-wise - they are considered pathological.

Upon further review of the research, I discovered that the medical literature indicates clinically-defined mild cognitive impairment "should not interfere with daily activities of independent, unassisted living", such things as the ability to "purchase things at the grocery store" or "balance a checkbook." Because I went to law school to ensure I would still be able to pick out a cantaloupe on my own and, even, select the right bill out of my wallet to pay for it. In my early thirties. WITH NOBODY ELSE'S HELP.

So what does Dr. NeuroSquared think is the cause of this dementia lite at the age of 33? What about all the other stuff? All the abnormal labs, breast issues, visual problems, thyroid, debilitating fatigue? She has reviewed all of the test results, scans, labs, and consults with other specialists and has a working differential diagnosis:

Parneoplastic neurological syndrome

An exceedingly rare, irreversible and rapidly fatal progressive neurodegenerative disease. There is no treatment or cure for the disease.

Sweet. Mother. Of. God.

An Introduction to Paraneoplastic Neurological Disorders

http://www.paraneoplastic.org/

What are paraneoplastic neurological disorders? Paraneoplastic neurological disorders (PND's) are autoimmune diseases that occur in response to the presence of cancer somewhere in the body. The cancer cells in paraneoplastic patients express proteins which are normally only made in the brain. These proteins cause the body's immune system to produce antibodies in an attempt to suppress the cancer. Unfortunately, these same antibodies can trigger an autoimmune attack on the brain and the body's neurological systems. Doctors are not yet sure why the immune system reacts the way it does in some patients with cancer, but not in others.

What types of cancers are most commonly associated with paraneoplastic disorders? Breast, ovarian and lung tumors are the most common cancers to be associated with PND's. However, cases of paraneoplastic neurological disorders have also been found in patients with other forms of cancer, including lymphomas, testicular cancer, childhood brain tumors and cancer of the larynx. In about two-thirds of patients, the neurological symptoms of PNDs begin before cancer is found.

Is cancer always found in paraneoplastic patients? No. It is entirely possible to have the neurological symptoms and antibodies associated with a PND and not have any identifiable cancer. Some doctors theorize that this is because the proteins associated with PNDs act as a natural anti-tumor weapon, thus preventing the cancer from growing large enough to be found.

Are there different types of paraneoplastic neurological disorders? Yes. There are a number of different antibodies and autoimmune reactions that are classified as PND's. The most common PND's include Paraneoplastic Cerebellar Degeneration (PCD), Opsoclonus-Myoclonus Syndrome (OMS), Eaton-Lambert Syndrome, and Paraneoplastic Limbic Encephalitis (PLE). There are others as well. Symptoms and treatment options can vary depending on the type of PND and the underlying auto bodies involved.

What are the most common initial symptoms? Difficulty with walking, balance, or speech; hand tremors; memory and mood disturbances; lack of coordination; weakness. These symptoms may begin suddenly or may be gradual.

How are PNDs diagnosed? For the most common paraneoplastic syndromes, diagnosis is usually made by a blood test which looks for common antibodies associated with the syndromes. In more difficult cases, however, PET scans, spinal taps, and other diagnostic methods may be used.

What is the life expectancy for someone who has been diagnosed with a paraneoplastic disorder? It is impossible to give an average life expectancy for paraneoplastic patients. Many variables can affect how long a patient with PND will survive including their age, the type of underlying cancer, the type of PND, the success of cancer treatments, and other pre-existing health conditions. Several small scale research studies have indicated an average life expectancy of between two and three years. Although the International Paraneoplastic Association does not keep formal records, we have noted a wide variety in the life spans of the hundreds of paraneoplastic patients who have contacted us. Some patients deteriorate very quickly and pass away within months of the beginning of neurological symptoms. Others live for 3, 4, or 5 years. We have been in touch with paraneoplastic patients who survived for 10 years or more, and have at least one report of a paraneoplastic patient who lived for 35 years after neurological symptoms began.

Do paraneoplastic patients ever fully recover? Very rarely. Of the hundreds of patients who have been in touch with the IPA, we know of only two patients who fully recovered after successful cancer treatment. However, many patients find they stabilize with proper treatment and may even show some small but gradual improvement over a period of months. Early treatment before severe neurological damage occurs is key.

Do all paraneoplastic patients end up in nursing homes? No. While many patients will need some form of assisted care, many manage to remain at home with the assistance of loved ones.

So here is what I know: 1) The doctor suspects I have it, she is testing for it, but she needs to put all the pieces together before she can diagnose it. Blood, LP, many more tests in coming weeks. 2) I am almost 100% certain that if I DO have it, it is not due to testicular cancer.

Paraneoplastic assay labs tomorrow, done at Hopkins (7 am - nothing like a blood draw before sunrise). The blood will be sent to Mayo for analysis, then an EMG where they will try to stimulate a seizure (I have been instructed to stay up all night - apparently, tired brains seize better).

Later this week, another brain scan to look at blood vessels and a lumbar puncture. These doctors have *really* have wanted to puncture that lumbar of mine for awhile now, and I think there is no getting around this one. The LP will look for "inflammation and floating cancer cells in spinal fluid." I also need to pursue the breast biopsy, the thyroid biopsy, lymph node biopsy, thymus studies - maybe dermatology too - to try to determine if there is some occult cause (meaning, hidden, not witch doctor) of underlying cancer triggering the neuroimmune response.

I plan to do all of that at Hopkins because 1) It is close to where I live and 2) To do all tests together makes it easier to triage what needs to be done first, and for all the doctors to share results as they come in. As you would imagine, a lot of specialists are involved. (A tip for anyone reading because they are having their own medical problems: A one-stop major medical center makes it easier logistically).

I am really hoping she is wrong, a differential diagnosis is not definitive, obviously. But I cannot deny the fact that doctors do not think lightly about telling a patient that a horrifying and fatal disease is "of concern" to them. I am still with it enough to recognize, beyond the mere humanity aspect of such a thing, is the fact that they risk getting sued if they are wrong. (And I really hope she is wrong.)

I debated not posting this because I do not know if this is what I have and it seems too early to panic. I need to get the blood back and the LP test results before I will know ... but the blood turnaround time for these highly sensitive tests at Mayo is 21 BUSINESS DAYS. I realized I cannot keep my mouth shut about something of this proportion for that long. And when people ask how I am doing, the answer is somewhat more complex than "fine!"

PS To those very close to me, please please please stop telling me "not to worry." I know you are only trying to reassure me, but when the doctor with a 79-page CV who *only* does this type of crazy rare complex disorder tells you this is what she *is looking for* and orders 50,000 more scary, invasive tests and super-expensive tests to try to confirm it, worry is permitted. My Worry Is Hereby Noted, on the record. Big Worry. Lots of Worry. No matter what you say. Unless you somehow want to pony up a sample of your own spinal fluid for me to offer up to the lab, then I will worry less. Until then, my Big Worry will be ongoing until I get some test results to the contrary. Then I will go back to worrying about normal stuff, which I do well. For example, if things were "normal", I would be worrying about how the root part of my hair is now almost as long as the blonde part and how my eyebrows have not been waxed in so long that they look like they are eating my forehead.

PS (Last time - cross my heart, hope to ... no WAIT, not that) Reflecting back on the appointment, I asked the doctor about my driving. Can I drive again? Here was her response: "You can drive. It is in your best interest to stay as active and maintain your normal life, as much as you possibility can ... while you are still able to do so ..." The impact of that statement did not really hit me at the time, but now .... typing it ... ? Drs. do not say that to patients they think will be OK? More worry.

Alright, according to my research, there are only about ten doctors or medical centers that "do" cases of PNS - Hopkins being one - and Dr. NeuroSquared is the expert there. I am hoping she is wrong and was having an off day when I was in. I'll do all the tests and go from there. Right now I am just trying to keep breathing AND trying to avoid looking at these crazy eyebrows and black roots in the mirror.