Corinne’s visit to the University of California, San Francisco Neurology Center – June 2013

“What lies behind you and what lies before you pales insignificant when compared to what lies within you.”  ~Ralph Waldo Emerson

{My treatment regimen had remained the same since my last blog dated May 2013, except for the IVIG which I had been asked to stop before going to UCSF so as to not mask anything that may show up in the testing. My last dose was late April, 2013.

• Klonopin  (0.5 mg for sleep)
• IV normal saline (2-3 liters/wk)
•  IV amino acids (Aminosyn 8.5%, two times/month)
• Valtrex (500 mg BID)
• Procrit injection (4,000 units, bi-monthly)
• Aleve (as needed for head pain)
• Meclizine (as needed for dizziness)}

Corrine’s next stop on her search to treat the lesion on her spinal cord is a neurologist at UCSF – a top medical university.

I have always wanted to see San Francisco, but not under these circumstances.  Not long after returning from Visit #10 to Dr. Peterson, his nurse calls to say the plan is for me to see one of the neurologists at UCSF re: my spinal cord lesion.  When their Neurology Center contacts me to make an appointment, they have an opening within a week, but having only just returned from Lake Tahoe a couple of weeks earlier, I need more time to recover.  I chose instead a Monday in mid-June, giving myself more time to rest and work out the logistics of traveling – never an easy task.

Setting my mind into motion, I determine that flying alone is my only option.  However, I’ve never been to the Bay Area and need help navigating.  For instance, where is the doctor’s office in relation to the airport?  In what part of the city should I reserve a hotel room?  How will I travel between destination points?  A visit to Dr. P’s, because of its familiarity, is easy compared to this!  This is unknown territory and seems overwhelming.  Reaching out for help, I send an email to fellow Dr. P patients, explaining my dilemma, aware that many of them live in Northern California.  My life has been blessed with many angels disguised as friends and I am praying for an encore appearance.

Hereby enters Jeff, whom I first met in Dr. P’s infusion room a couple of years ago.  We have spoken on the phone only once or twice, but when I send out the call for help, he kindly offers his services.  Living a couple of hours away from San Francisco, Jeff loves the city and visits it often.  With his input, I am able to determine where to stay and most importantly, he offers to meet me there and transport me to my doctor’s appointment, and if I am feeling up to it, give me a mini-tour of what some say is one of the most unique and beautiful big cities in the world.

With so many uncertainties now put to rest, I prepare for the trip.  I have felt horrible this last week but I’m hoping to recover enough to consider this journey an adventure – a “vacation” that may include some medical help.  California here I come!

Day #1 Sunday

The flight to San Francisco is two hours, slightly longer than when I fly to Dr. P’s. This airport handles much more traffic than the one in Reno and it is crowded and over- stimulating.  Like always, I use a wheelchair and attendant, but finding food for lunch is difficult.  I’m informed there are no fast food chain restaurants in any of the terminals and the only place to get a quick “to go” meal has a line 20 people deep.  It’s important that I eat, since once I get to the hotel,  I’m there for the night.  My wheelchair attendant kindly offers to stand in line for me and after grabbing a sandwich and banana, we are soon on our way.

I’m wheeled across a street to the shuttle pick up area.  The sky is clear blue and the temperature is pleasant – warm for San Francisco I am told.  Sitting on a bench I watch a multitude of shuttles of all sizes and colors come and go.  Fortunately, I am sitting next to a family who happens to be going to the same hotel (I don’t believe in coincidences :)).

When our shuttle pulls up, one of the family members lifts my bag for me and hoists it into the van.  I’m amazed at the kindness of complete strangers.  When we arrive at the hotel, only minutes away, the same young girl carries my bag into the lobby – all without being asked.  I thank her profusely, not able to convey the gratitude I am feeling at this moment.  This trip, like all of them, will be difficult for me and any unexpected assistance is tremendously appreciated.

Checking in, I am told that it will be an hour or two before a room will be available.  Reclining on a comfy sofa in the lobby, desperately needing to elevate my legs, I finally get to meet Sheri, the hotel manager, and another critical angel in this adventure.  When I had called to make reservations, knowing that I was traveling for medical reasons, she went out of her way to make room for me during a very busy week.  She made it clear that she wanted to help.  Now, face to face, I rise to give her a much deserved hug.  She tells me that a room has opened – not one very close to the lobby, but perhaps I would prefer it to waiting….”Yes, yes –thank you, thank you!” I exclaim.

While one of the staff carries my bag, I slowly trudge to my room, exhausted.  My feet and legs are numb, my back stiff and aching, my brain unable to focus.  I can’t wait to sink into bed.  Looking out the window, I can’t believe I’m in San Francisco.  Seeing the layer of fog offshore, I’m reminded that I made it!  Tomorrow’s appointment is an early one, so I swallow my five different pills and off to dreamland I go.

Day #2 Monday

Jeff and I agree to leave by 7:15 for my 8:00 a.m. appointment.  We head downstairs with time to enjoy the spectacular breakfast the Embassy Suites provides. All goes well and we are on our way in time.  On these trips I am always concerned about getting enough bottled water in order to stay well-hydrated.  Thoughtfully, Jeff has brought a car trunk full for the both of us.

The feared San Francisco traffic is rather mild and we find the Neurology Center without a problem.  Borrowing a wheelchair from the Center, I chose to be wheeled in.  The less time I spend upright, the better for my legs, back and brain!  When the receptionist checks me in, she comments that her mother has the same first name as me – spelled and pronounced exactly the same – and, as it turns out, we are only months apart in age.  She then casually adds that her mother was named after a French actress.  OMG – so was I!!   This is the first time that I’ve ever heard anyone say this.  I stop to think – today is the day after my long-passed mother’s birthday.  I am in a strange place for an anxiety-ridden reason – a jaw-dropping spinal cord MRI.  Except for a very helpful friend, I am alone – no family or loved ones.  Is there any way that this can be a coincidence?  Not a chance.  Mom sends her love. 🙂

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During my previous visit to Dr. P, another patient informed me that he had never been anything but extremely pleased with any physician Dr. P has referred him to. Whether it was a gastroenterologist, dermatologist or orthopedist, they have all been excellent.  I’m hoping this will be the case for me.  The neurologist I was originally referred to would not be available for a month, so he in turn, referred me to his colleague, Dr. G. with Dr. P’s approval.

As we wait, Jeff and I joke to relieve the tension.  I am a bit apprehensive as my last visit with a neurologist near my home led me to the diagnosis of neuromyelitis optica (NMO) which is a disease that can result in blindness and paraplegia.  Although my NMO blood and spinal fluid tests came back negative, I’m still a bit afraid of what this neurologist will say.

Having had more than one negative experience with neurologists, when a doctor appearing very ‘old school’ with a bow tie, briefcase and gray hair, wearing a conservative, outdated suit, walks through the waiting room, we both kid that I’m in a boatload of trouble if that is Dr. G. J  Immediately after, a different man in a white coat with a stethoscope around his neck heads right over to me with an extended hand and a warm smile.  “Hi Corinne, I’m Dr. G.  Nice to meet you.”  He then introduces himself to Jeff and back to the exam room we go.

As I am wheeled down the hallway, I ask Dr. G if Jeff can join us.  He says it’s up to me.  Feeling that it is important to have two “partial” brains present rather than one, during what could be a long info-packed session, I make sure Jeff is following.  And honestly, if the news is bad, I’d rather not hear it alone.

Corinne gets a very thorough examination

As the appointment begins, it isn’t long before I realize that I will have to repeat all the many questions and exams neurologists require even though I had just did them in April.  Dr. G makes it clear that to draw his own conclusion, he has to start from scratch (why would I think otherwise?).

Dr. G is very thorough.  First, with the questions (history/timeline, medications, symptoms, family illnesses, etc.) including confirmation that I am using the wheelchair today primarily to save energy.  But one particular question catches me off guard…”How is your sexual function?” Dr.G casually asks.  Oh dear, I haven’t been asked this one before.  I hesitate, needing more clarification.  Squirming, I reply with a question of my own, “What do you mean?”  Silence follows.  Dr. G is facing me with a pleading look that suggests he’d prefer to not have to explain.  Thinking perhaps I may be misunderstanding something, I turn to Jeff.  The look on his poker face simply says, ‘sorry friend, I can’t help you with this one.’

Feeling uncomfortable with all eyes focused my way, it takes a few more seconds for it to register – this is not a ‘how is your love life’ question…it’s a neurological one. As I blush, I finally answer with a generic ‘fine’.  With that we quickly move on.

Next comes the physical/function exam – touching fingers to nose, fingers to fingers, toes to shins, etc., etc. (deep breath), and then the coup de grace: a timed “sprint” down the hallway!  This is a first for me.  I am asked to walk as fast as I can from line to line (perhaps 25 to 35 feet?).  The last time I’ve done anything similar was to “run” to answer a ringing phone.  Being an ex-athlete, I sense my competitive juices begin to flow and off I go.  As I cross the finish line, I hear “5.7 seconds” and I return to my seat feeling pretty good about myself.

Finally done with the physical part, Dr. G tells me I did really well.  Seated at his computer, it’s time to download the CD I brought with me and check out the scans of my brain and spinal cord.  I am anxious to finally see the lesion.  The doctor and I have both read the radiologist’s report, but neither of us have seen the images yet.

Dr. G brings up the scans of my brain first and immediately goes to the subcortical lesions (“unidentified bright objects or UBO’s), common in ME/CFS patients.  He gives me the same explanation all conventional physicians do – that they don’t know what causes them and that they are seen in a variety of people and are attributed to a variety of reasons.

As we stare at the image of my brain, I ask “is this where we would see encephalitis?”  He says yes and adds that I don’t show any.  Always amazed that my head could feel so bad and yet appear normal, I bluntly state that no matter what the picture shows, I know I have “brain inflammation.”  He mutters something to the effect that “MRI’s can’t show everything.”

A transverse as opposed to the longitudinal lesion found in Corinne’s spinal cord

Next he scrolls down to my cervical spine.  “There’s the lesion” he says as he points.  As a huge bright white cloud ascends the length of my neck up into the base of my brain, he explains “that’s the contrast (the injected dye) and it’s actually extending into some of the meninges that surrounds the brain.”  I can barely speak.  I turn to look at Jeff and his jaw is similarly dropped.  It looks much worse than I imagined and apparently, to Dr. G as well.

“I’m surprised you are doing as well as you are” he says while looking alternatively back and forth from my physical self, seated in the chair, to the image before him.  It’s almost as if he can’t believe they are the same person – the one who just sprinted down the hallway!

As we talk further, he clarifies that this is not transverse myelitis as the word transverse (crossing the diameter of the cord) would suggest bowel and bladder problems (aka, incontinence!).  Instead, he points out that the lesion is longitudinal (extending from C3-C7) staying on the outer, posterior part of the cord.  This is why my neurological symptoms are primarily sensory related rather than motor.  I immediately sense how fortunate I am.

When I ask Dr. G if he has ever seen anything like this, being a myelitis expert, he answers “all the time.”  When I ask if he ever sees them go away, he responds that he often does – “sometimes on their own, sometimes with treatment.”  As we discuss possible causes, like most doctors, Dr. G does not use the “C” word (CFS) throughout our appointment and carefully tiptoes around it by even asking me at one point if I, myself, am comfortable with the diagnosis.  I respond “I despise the name.  The name does not come close to describing the illness – but yes, if we are talking about a neuro-immune disease, I am comfortable with the diagnosis.”

As we discuss possible causes, infection, neuromyelitis optica, neuro-sarcoidosis (N-S), multiple sclerosis (MS), parasites and malignancy all come up.  But having seen my spinal tap results and now, looking at my MRI, he seems able to eliminate some.  Regarding MS, Dr. G tells me that the spinal cord lesions associated with that illness come and go, and are usually gone within a month so that diagnosis is unlikely for that as well as other reasons.  Regarding NMO, he says that the lesions with that illness appear different than this one.  This one looks like the ones he sees associated with neuro-sarcoidosis…however, other illnesses have to be considered.

Dr. G doesn’t think it is cancer or infection but only more tests can rule them out – yes, more tests!  But fortunately, he tells me I can do them at home.

“What’s really unusual is how little swelling there is” Dr. G.

As has so offered happened her and with other people with ME/CFS Corinne is told her presentation is ‘very unusual’.

While still staring at the MRI, Dr. G says “what’s really unusual is how little swelling there is.” Considering the inflammation shown by the contrast dye, there’s virtually no edema/swelling of the cord.  He goes on to say “and your spinal fluid (from the lumbar puncture) is so clean!”

Other than a couple of oligoclomal bands, which he says mean nothing, there are no inflammatory properties evident – no white blood cells, IgG, proteins, glucose etc. (I like to call it “a clean dipstick” J).  He suggests that it is perhaps due to the IVIG I had been on, but it was still very “unusual” – a word I have already heard multiple times from multiple doctors and radiologists.  I tell Dr. G that Dr. P calls me his “index patient.”  Still staring at the image of the lesion, he nods his head and says, “You sure are.”

{As it relates to clinical research, the definition of ‘index patient’ is as follows:  “The first medically identified patient in a family or other group with a particular condition, often an infection, which triggers a line of investigation.” As far as I’m concerned, this can be replaced with one word…”CORINNE”.}

Never really addressing my history of immune issues, he does say that he is not suggesting that whatever caused this is the same thing as what I’ve had for 23 years – or “my syndrome” as he refers to it.  Jeff, who can’t hold back any longer finally addresses the big white elephant in the room by emphatically asking, “But since Corinne has had documented immune dysfunction for many years, couldn’t this be what has led her to this point?”  Dr. G responds, “Maybe.”

Interested in treatment options, I direct the conversation in that direction.  Dr. G says that in his experience, these lesions need to be “knocked down hard” in order to prevent their recurrence.  He mentions a high dose of IV steroids as the first-line option but stresses that he would prefer to get a diagnosis so that he can use a more targeted treatment – something tolerable and more effective.  Until then, he would refrain from treatment. (It’s June. The lesion was found in February and I have yet to receive a diagnosis.  No diagnosis, no treatment.  Somehow I feel I won’t get one soon).

We then move on to my “to do” list:  1. A chest CT scan (including the lungs and thoracic lymph nodes); 2.  A PET-CT scan (head to tippy toes);  3.  Additional blood work (some repeats); 4. A complete, extensive eye exam;  5. A repeat three month C-spine MRI; and  6. A repeat lumbar puncture might be helpful now that I am off the IVIG, if the first five show nothing definitive. The reason for the scans, he explains, is to hopefully find somewhere else in my body, where the process that is occurring in my spinal cord may be happening.  Somewhere easier to biopsy than the meninges surrounding the cord – a much too risky procedure.

Corinne at the Golden Gate Bridge

Dr. G assures me there is no rush, that another month or two will not make a difference.  I can quickly calculate that getting the first five tests completed will take at least six weeks.  I will need recovery time from this trip and in between each test.  That means it will be at least August until there is any remote chance of treating my discomfort.

As I am wheeled out to the car, I glance at my watch.  We had been speaking for two hours!  All in all I found Dr. G thorough and compassionate although once again I leave another appointment without any answers.  Jeff and I are exhausted but I didn’t come all this way to not see the Golden Gate Bridge, so off we go!  I seem to be slowly slinking down further and further into the car seat as we cross the bridge, drive through world-renowned Chinatown, down crooked Lombard Street, then past several cable cars and notable Bay Area landmarks.

At one point, Jeff suggests I get out of the car so he can snap a photo of me and a gorgeous ocean view….but I am unable as I don’t have the strength to move.  Sadly, I am also too weak to stop and sample some of the great food San Francisco is known for.  The best I can do is agree to a take-out pizza.

Back at the hotel, we eat and talk…and talk…and talk, covering all angles of the appointment.  Things I forgot Jeff remembers, and things he forgot, I am able to remind him of.  It’s good teamwork – something us patients desperately need and are most appreciative when we get it.  Together, we are determined to figure this illness out!

Day #3 – Tuesday

After more discussion, Jeff is on his way home, burned out and barely functioning.  It’s difficult for me to find the words that express how grateful I am for his help.  Though he is happy to be able to assist me, I feel a bit guilty that he has to suffer as a result.

By 2:00 p.m. I am in bed – mentally and physically a wreck.  I sleep until 4:00 a.m. the next morning! 

Day #4 – Wednesday

The long sleep was obviously what I needed.  I feel up to handling the early flight and two hour ride home my husband thankfully provides.  I manage to get back into bed by a decent hour only half believing that I made it to San Francisco and back!  But the work is only just beginning.  Tomorrow I will have to continue my “full time job” of booking and getting to medical appointments.  I have Dr. G’s list of tests on the stand next to my bed.  First thing in the morning: schedule a chest CT!

Conclusion

It took me six weeks to complete the five tests required which would decide whether I needed another lumbar puncture.  Here are the results:

1. Blood work: which included angiotensin converting enzyme (ACE), C-reactive protein (CRP), paraneoplastic autoantibody panel, NMO-AQP-4 antibody IgG, RPR, and copper and sedimentation rate –  all normal/negative.
2. 

   Test results are mostly normal.

   Chest CT: (used to help diagnose sarcoidosis by showing the lungs, etc.) was normal  (no nodules, granulomas nor enlarged lymph nodes).

3. Extensive eye exam:  (which included dialation and optic nerve scan) was normal except for some dry eye – normal for my age and not enough to fit the criteria for Sjogren’s. (Having been concerned about NMO and its associated blindness, I was very happy to see a healthy optic nerve!).
4. PET-CT scan:  (head to toe).  In this test, radioactive glucose is injected into the blood stream after fasting.  The cells that are the most metabolically active (like cancer cells) will take up the most glucose and “light up.”  My scan showed very mild hypermetabolic lymph nodes in my chest (along the trachea – mediastinal).  All else was normal.
5. C-Spine MRI:  The three month repeat C-spine MRI revealed the longitudinally extensive lesion was stable in size and continues to enhance with contrast (though slightly decreased), indicating inflammation.

After I completed the tests, Dr. G phoned from UCSF.  He had not received any results and wondered if I had completed any of them. (?!).  Even though he was the ordering physician and each order contained his contact information, he had not received a thing!

Thankfully, I have learned to always request copies of everything generated from each appointment (including CD’s of scans), so I was able to read him each report over the phone, in addition to faxing copies and mailing the CD. I would advise everyone to do the same.

Discussions with Dr.G by phone, centered on the “slightly hypermetabolic” thoracic lymph nodes seen in the PET scan.  Though they were “non-specific” and not at all enlarged, they were the sole “abnormality” observed.  Since evidence of sarcoidosis can show up in these nodes, Dr. G felt like he could narrow his focus to neuro-sarcoidosis (N-S).  He suggested a biopsy (called a mediastinoscopy) would help in the diagnosis and thus a targeted treatment – for instance, Remicade and methotrexate which he mentioned as a well-tolerated and effective option he has had success with.

I, in turn, was skeptical.  What little I had read about sarcoid revealed that it is an autoimmune disease which usually attacks the lungs (leaving nodules and/or granulomas) and that the percentage of sarcoid patients who develop N-S (brain/spinal cord) is very low, perhaps one to five percent (?).  I have seen a distinct connection with my ME/CFS and my neurological symptoms (for example, mental exertion leading to increased leg numbness and pain).  After 23 years, I wasn’t convinced that my “old” illness had morphed into something “new” – or that I now had two separate maladies, both neurological, one on top of the other!  So I asked Dr. G how his sarcoid patients function…

“Well, if they have nodules in their lungs they may have difficulty”…

“Wait, wait”, I interject, a bit fired up,  “No, the ones like me – no nodules anywhere, just a spinal cord lesion – how do they feel?  Can they go out to lunch without paying dearly for days?  Can they go shopping?  Are they homebound, unable to sustain activity?”.

“I see what you are saying” he answers.  “No, they function fine.” (silence).

Dr. G then went on to say that I wouldn’t be the first person who had two things suggesting this could be neuro-sarcoid in addition to “your syndrome.”  He attempted to encourage me by emphasizing that N-S is treatable/curable and that perhaps healing the lesion would also improve my other symptoms – never suggesting however that it would cure everything.  He stressed again the importance of a diagnosis rather than simply hitting me with a wide-spectrum immunosuppressor such as steroids.

With no answers forthcoming from this visit, the future is unclear

We ended the conversation with me expressing my doubts of actually being able to pinpoint a definitive diagnosis and him responding in a compassionate, encouraging manner, leaving me with some difficult decisions to make.  A mediastinoscopy would be more invasive than any test I had done so far and I wasn’t sure I wanted to put myself through that.

At one point I asked if there was a chance that I could wake up one morning paralyzed.  Dr.G reassured me that that wasn’t the way these types of lesions behave.  Not sure that anyone really knows what type of lesion this actually is, that uncertainty remained in the back of my mind, not totally resolved.

Dr. G had assessed that another lumbar puncture was not necessary because it would only show the inflammatory properties that did not appear in the last one (possibly due to the IVIG I had been on)….but, I had other ideas.  Contacting Dr. P, we decided I should return to Lake Tahoe to have another one done.  Since I had been off the IVIG for four months, we were hoping that my spinal fluid would now present something significant to solving this mystery and besides, it could be beneficial to send samples to Dr. Lipkin for his and other future studies.

Having not had difficulty with my last two lumbar punctures (third time’s a charm?), I made an appointment for September (2013).  Now, with the added discomfort of periodic, unbearable worsening of symptoms, including numbness so bad my shoes would fall of my feet without me knowing, periodic buzzing down to my toes hinting to “faulty wiring,” increased hypersensitivity with stabbing pains in my legs along with a brain that felt like it was “rotting,” I was back to planning a travel agenda, yet again – highlighted by that now familiar refrain… “Would someone like to help me get there?”

(Next blog…my September lumbar puncture.)

Til next time…Feel Good!

• Check out Corinne’s Blogs on her experiences with Dr. Peterson