Thursday, May 17, 2012

Stuck in Solitary

When I started this blog, I decided I didn't want it to be a blow-by-blow account of my journey through the medical world, a symptom diary, or a journal of the treatment regimens I've attempted. Instead, I've tried to emphasize the emotional and psychological challenges shared by patients forced to confront a chronic debilitating disease, and also make it a place to report on and attempt to interpret much of the MS research I find myself obsessively compelled to seek out. Occasionally, though, I've deviated from that path when events of enough medical interest crop up as I deal with my disease, and I recently experienced just such a development. 

Long time (and even some short time) readers of this blog probably know that I'm something of a mystery patient, as my diagnosis has been up in the air for quite some time. I was first diagnosed with "atypical Primary Progressive Multiple Sclerosis" back in 2004, about a year after my first symptom – a slight limp in my right leg – showed itself. My disease was considered atypical because my MRIs revealed only one significant lesion (a big juicy one at the base of my brainstem), lumbar punctures showed no O-bands or other CSF abnormalities, my medical history hinted at some kind of systemic autoimmune disorder, and I generally didn't conform to the any of the diagnostic criteria (click here) established for the different forms of MS. 

I ditched my first MS neurologist after a year because, despite his being a fine physician, he had the personality of a herring. My second MS neurologist immediately suspected that I might not have multiple sclerosis and had me tested for a host of different diseases that might account for my symptoms, including sarcoidosis, lupus, Hughes syndrome, Lyme disease, and others. When none of these tests came back positive, I was slapped with that "atypical PPMS" label again, and for several years that's where things stood. 

Never comfortable with my diagnosis, I pursued other opinions, my efforts including visits to the Johns Hopkins MS Center on two separate occasions. The doctors at Johns Hopkins finally told me that it was indeed quite likely that I did not suffer from MS, primarily because, along with all of the other strange elements of my presentation, my lesion had not changed at all in size or shape since it first been imaged several years earlier (it remains unchanged to this day), nor had it been joined by any others. These fine physicians couldn't quite put a finger on precisely what might be ailing me, though. They suggested I be tested for Sjogren's disease and also that I consult with a mitochondrial disease specialist. I did both and came up with nada. 

At about that time I was accepted into a research study being conducted by the National Institutes of Health, the US government's medical research arm, whose primary facility is located in Bethesda, Maryland. The study was designed specifically to identify clinically definite MS patients. During my four visits to the NIH, I was subjected to almost every conceivable diagnostic test, and had enough blood drawn to feed a vampire family of four for at least a month. After all the testing was complete, the NIH agreed with the doctors at Johns Hopkins, saying that although I definitely couldn't be classified as having clinically definite MS, they couldn't come up with a viable alternate diagnosis. So, for the last 18 months or so, I've been stuck in a kind of  never never land as far as my diagnosis goes. My local neurologist, no slouch in his own right, has by and large stuck with the "atypical PPMS" diagnosis, since MS is a diagnosis of exclusion, and we've excluded every other possibility we could think of. 

Recently, the NIH has decided that I could very likely be suffering from "Solitary Sclerosis", a condition that was recently described in a paper published by researchers at the Mayo Clinic (click here). The paper details seven patients who presented with progressive disability and only one visible lesion on their MRI images, all at the cervicomedullary junction, precisely where my lesion is located. Although my lesion (I've named it Adolph) has a number of very atypical features, and much of the other strangeness about my case hasn't been resolved, the NIH doctors feel that this newly described derivation of MS is the closest fit for my condition. I'm actually a little bit confused by this, because the folks at the NIH originally presented quite a long list of reasons why they didn't think I had MS, many of which now seem to have been minimized in order to make the Solitary Sclerosis diagnosis fit, but I will concede that this newly identified MS offshoot does cover some of the more glaring anomalies surrounding my condition. 

It may seem strange, but I've actually grown kind of comfortable not having a definite diagnosis, since having no diagnosis leaves open the possibility of any conceivable outcome, including total recovery. Solitary Sclerosis doesn't seem to be an especially cheerful diagnosis, as of the seven patients the researchers looked at, one is dead, two are quadriplegics and completely bedridden, and the other four are quite disabled and progressing rather rapidly. None of the SS patients responded positively to any of the standard MS disease modifying drugs, which is typical for patients suffering from the progressive forms of Multiple Sclerosis. 

I too haven't had much luck with the many MS therapies I've tried, although I did once get tremendous benefit from a large prolonged dose of IV steroids (which unfortunately later caused me to develop avascular necrosis (click here), an excruciating condition which I wouldn't wish on anybody), and I initially did surprisingly well on IVIG, which would be highly unusual for a patient suffering from a progressive form of MS. Overall, I'm still quite dubious of the SS designation, since I continue to have all kinds of weirdness associated with my disease, and as I've cut my swath through the medical community I've certainly left enough doctors scratching their heads in my wake. 

I'm currently pursuing a few different diagnostic possibilities, which I'll report on if any turn out to be worthwhile enough to write about. But, for the time being, I have at least one foot (the gimpy one) planted in the MS camp, as a possible (probable?) Solitary Sclerosis patient. On the bright side, I'm quite likely the first ever Jewish member of the SS, quite fitting for a patient who long ago named his lesion Adolph. 

Yes, nothing like some horribly distasteful humor to finish up an otherwise serious blog post…

I have just had my CCSVI procedure 16th May 2012 and within hours I did not

need my glasses to read anymore. Also, my feet have warmth for the first time in years.

My blockages were as follows:

Right Jug... 50%

Left Jug...    70%

Azygos....50%

May Thurner - condition found.....95% blocked ......fixed using a  10/20mm stent

Just wanted to share my journey with CCSVI.

Goood luck to all those still seeking the procedure.

Mandy

It can be hard to hear of other people's improvements if you are one of the 1/3 that didn't see any changes after treatment, especially when standard therapies did not help either.  

There are many potential reasons for treatment to fail even if CCSVI is an important part of MS: maybe the interventional doctor missed something, or your particular kind of stenosis isn't well repaired with today's techniques, or the area may have been undertreated.

Aside from the issues of treatment failure due to technical difficulties is the possibility that blood flow issues are only a part of MS, or only significant in a subset pwMS. These things will be elucidated with time and research. 

But there is also the fact that decades of research on brain tissue from pwMS has shown permanent nerve damage that occurs even early in the disease.  Although logically a procedure that can restore blood flow offers the best chance for healing, many will need something more than just improved circulation to see any change in functional levels.

But I'd like to offer encouragement: we are at the cusp of big changes in MS treatments.  One day soon we'll have a better understanding of how much of what we think of as MS is caused by vascular problems, which procedures are effective, which pharmaceuticals can help maintain tissue health in the face of poor circulation (even when a particular person has blood flow problems that can't be fixed with a procedure) and which patients benefit most from a procedure.  

However the future for MS isn't only about CCSVI it is also about the rapidly growing field of regenerative medicine. 

As mentioned before in these pages, Dr Petrov is researching the use of autologous stem cell transplants along with CCSVI treatment.  In  his mind good blood flow accompanied by regenerative stem cells is the future of MS treatment.  But many other centers around the world are also successfully investigating stem cells as a stand alone MS treatment to regenerate brain cells and tissue.  

Dr Mark Freedman has been working on stem cell research in MS patients for many years.  His frustration that research funds were diverted to CCSVI has been mentioned by many CCSVI followers because the comments were so derisive of the vascular model, but his work on stem cells offers real hope for people who have already lost function.  Though much of Dr Freedman's research has been on stem cells after a strong chemotherapy drug kills the entire immune system (I talk about the problems with this type of therapy in my book), he is also researching stem cells as a regenerative strategy without the chemo.

There are also several companies making drugs to stimulate stem cells inside the body to repair damaged nerves after a stroke or other neurologic diseases.  One of these therapies uses a hormone and a red blood cell stimulator and it seems to help stroke victims recover more function.  They are already at stage II/III trials with stroke patients and expect their drug to be available soon.  

The regeneration of damaged nerves using these strategies is almost here.  

But if you have money and live in or near Texas, it is here--Texas passed a bill that allows private clinics to use stem cells for degenerative diseases even though the FDA hasn't approved the use of stem cells in these diseases yet.  

The reason the FDA has not approved stem cells for things like MS or Rheumatoid Arthritis (RA) is that although stem cells are commonly used in cancer therapy, there have not been an adequate number of stage III trials showing how effective they may be in slowing or stopping other diseases for the FDA to pass an approval.  See an article about Texas and their controversial law click here

The Texas law demands that any clinic doing these unproven stem cell transplants must have IRB approval to protect patients from false expectations or misleading advertising and it insists that the clinics keep data on treated people and effectiveness of therapy for the various diseases treated.  This will result in a huge amount of data regarding the usefulness of these therapies in short order.  Go Texas!

Another regenerative therapy is stimulating nerve growth through physical therapy; Dr Wahls is running a study to evaluate aggressive physical therapy and diet changes to help progressive MS. A progressive herself, she went from 4 years in a wheelchair to being able to bike to work and walk the halls of her hospital.  She details her program in the book "Minding My Mitochondria".  

The brain is actually very "plastic" meaning it can reorganize and rewire itself.  This is how aggressive physical therapy helps people reagain some lost function.  In an extreme example children with severe epilepsy have sometimes had one HALF of their brain removed to stop the seizures.  Such children recover nearly all of the lost functions if they have the surgery when young enough and train enough.  

The book "The Brain that Changes Itself" is a fantastic read about how aggressive physical therapy can cause the brain to rewire from damaged areas to intact ones so function is restored.  One thing mentioned is that a blind person's brain rewires itself so his ears also employ the eye's unused nerve cells making the ears far more sensitive and capable that ordinary sighted people.  

Some combination of these things may be helpful for progressive MS patients who did not see benefit from a CCSVI procedure, or people who got less than they hoped for: physical therapy, stem cells, and if blood flow was poor but not repairable possibly medication to enhance circulation or to make tissue resistant to low oxygen states. Altogether this may allow the brain to maintain function better. 

But the slow pace of investigtion can be frustrating especially if you have lost a lot of function even while on standard treatments.  It can feel like nothing may get here in time for you...I get emails from people who feel like the nursing home is only a few years away, and they want to know--If I don't get this now or if it didn't work when I tried it, can you share reason to hope? 

I'd like to share a bit of myself and an epiphany I had a couple years ago.

In August of 2008 after 17 years of MS, I fell and shattered the ball part of my arm bone in my shoulder joint. The break was so bad it could not be repaired; all they could do was tie my arm down and let the pieces heal into a blob in there to hopefully imitate the old ball.  Oh wow, did it hurt!  I couldn't walk with my cane, couldn't shower, couldn't drive; in short, I became completely dependent. I felt utterly helpless.  

On top of that dependency, I felt quite hopeless because suppressing my immune system had not prevented my MS from progressing to the point where I was heavily dependent on a cane or walker.  I was convinced the autoimmune model was missing something.  I could jog when I went on Copaxone--I never had a relapse or MRI-visible inflammation after starting it AND my arthritis was controlled.  When my MS progressed anyway they added methotrexate to try and suppress "invisible inflammation".  It didn't work -in fact it made me worse- and the slow but relentless slide continued.  I stayed with Copaxone because it works so well for my arthritis, but I've never thought it did much to stop MS progression.

I didn't think anything would change about MS in my lifetime; I cynically figured the day after I died there'd be an announcement about some mouse's immune cell and how the researcher who discovered it thought this would cure MS.  Little did I know as I despaired that something would force many researchers to look at MS with a completely different perspective in just a couple months.

I didn't know that Dr. Zamboni's work would come out in December.  I didn't know MS patient grassroots activism and advocacy would cause a complete uproar in the medical community.  I didn't know the Cleveland Clinic would advance a study that showed severe abnormalities in the veins of 7 MS patients in autopsy because of that uproar. I didn't know that in 3 years several  hundred studies would be out looking at this new idea and that other related ideas would be investigated like CPn as a vascular operator in MS or BNAC's work on cerebrospinal fluid flow.  I didn't know that all of these studies would lead to more studies in an ever widening net to capture and learn the truth.

I had no premonition of the promise so close... I didn't know there was so much reason to hope just beyond my sight.

While CCSVI treatment has not changed my MS much beyond a very welcome reduction in spasticity, I have great hope because there are suddenly many new directions of research.  CCSVI has REALLY shaken up the research community and resulted in many new ideas.  

I don't know what might happen next month or the end of this year, but I do know it is vastly more hopeful than it was 3 years ago when all we heard about was endless "breakthroughs" concerning some different immune cell that some dude somewhere "thinks will cure MS."

Please be hopeful and know that science can be very surprising; I'm ready for venous researchers to start talking about their breakthroughs but the cool thing is that even if the venous aspect of MS turns out to be only part of the story, there are really different avenues of nerve regeneration coming soon.  Things are really changing and this isn't the same old recycled ideas.  

Pump up the veins and pass the stem cells!

Marie

http://www.facebook.com/notes/ccsvi-in-multiple-sclerosis/when-treatment-doesnt-help-thoughts-from-marie/10150756949697211

On May 10, the Food and Drug Administration issued a medical alert noting that individuals with
multiple sclerosis should be aware of the risks of serious injuries and death associated with treatments for
chronic cerebrospinal venous insufficiency—and that benefits of those treatments and promotion as an MS
treatment “may lead people with the disease to make treatment decisions without being aware of the serious
risks involved.”
As always, the Society of Interventional Radiology strongly urges close communications between
doctor and patient. Those persons with MS are encouraged to talk to their interventional radiologists
and their other doctors about any concerns or questions. SIR members—interventional radiologists
who specialize in minimally invasive targeted treatments and who pioneered venous angioplasty and
stenting—endovascular techniques that may be central to novel treatments for MS—may note an increase
in calls from concerned individuals who have—or are seeking—treatment for CCSVI.
About 500,000 people in the United States have MS, and SIR understands the public’s desire to
advance treatment for MS, generally thought of as an autoimmune disease in which a person’s body attacks
its own cells. Currently, medicines may slow the disease and help control symptoms. The role of CCSVI (a
reported abnormality in blood drainage from the brain and spinal cord) in MS and its endovascular
treatment (through a catheter placed in a vein to widen) by an interventional radiologist via balloon
angioplasty and/or stents to open up veins could be transformative for patients and is being actively
investigated.

http://www.sirweb.org/news/newsPDF/SIR_FDA_CCSVI_51012.pdf
The FDA communication is directed toward individuals with MS, health care providers (including
interventional radiologists, neurosurgeons and vascular surgeons) and clinical investigators. Health care
providers were advised to inform patients that (1) there is conflicting evidence about CCSVI as a clinical
entity, (2) CCSVI’s relationship to MS is scientifically unproven and (3) consensus on the diagnostic
criteria of CCSVI has not been reached. The FDA also indicated that it has not cleared or approved any
angioplasty device or stents for CCSVI treatment and that the use of those devices in treating CCSVI is
considered off label. “While the FDA does not regulate the practice of medicine and health care
practitioners may choose to use a legally marketed device, based on their clinical assessment, for purposes
other than the cleared or approved use, the FDA believes the safety issues observed to date warrant a
communication on the subject,” stated the FDA announcement.
SIR supports and agrees with the FDA’s recommendations to encourage research on CCSVI and
the current knowledge regarding the safety and effectiveness of treatment procedures. SIR also
agrees that clinical research of CCSVI should be performed through well-designed clinical trials,
which should require approval through the FDA investigational device exemption (IDE) program.
In 2010, the SIR published the position statement “Interventional Endovascular Management of
Chronic Cerebrospinal Venous Insufficiency in Patients With Multiple Sclerosis: A Position
Statement by the Society of Interventional Radiology, Endorsed by the Canadian Interventional
Radiology Association,” which specifically notes
SIR recognizes the urgent need for more effective treatments for MS patients and the public’s interest
in rapidly making such therapies available to this patient group.
SIR recognizes that patients with MS constitute a particularly vulnerable population, whose safety
must be protected as new therapeutic approaches are evaluated.
At present, SIR considers the published literature to be inconclusive on whether CCSVI is a clinically
important factor in the development and/or progression of MS and on whether balloon angioplasty
and/or stent placement are clinically effective in patients with MS.
SIR strongly supports the urgent performance of high-quality clinical research to determine the safety
and efficacy of interventional MS therapies and is actively working to promote and expedite the
completion of needed studies.
SIR recognizes the challenge and the potential opportunity presented by promising early studies of an
interventional approach to the treatment of MS. SIR is pleased that public advocacy groups have
pushed the medical community forward to meet this challenge and is committed to assuming a national
leadership role in launching needed efforts.
Interventional radiology is a recognized medical specialty requiring dedicated training that
encompasses clinical patient evaluation and management, non-invasive venous imaging and the delivery of
targeted, image-guided minimally-invasive treatments to patients. Interventional radiologists perform
balloon angioplasty and stent placements on a daily basis in thousands of patients with diverse venous
conditions, including acute deep vein thrombosis, post-thrombotic syndrome, superior vena cava syndrome
and portal hypertension; they also perform procedures to maintain hemodialysis access. Interventional
radiologists perform many of the CCSVI procedures in the United States; they are highly qualified to
perform such treatments when appropriately indicated.
SIR will provide additional information for patients as it becomes available

In Finding Nemo, Dory the fish has a little song she sings, to keep Nemo from becoming too despondent...

When life gets you down, you know what you gotta do?

Just keep swimming, just keep swimming.

Here it is on video-- makes me smile.

link

I had an interesting conversation with Dr. John Cooke at the Hubbard Foundation conference.  It was good to catch up with him.  We hadn't seen him in two years, since Jeff was up to Stanford for his one year testing.  He asked how Jeff was doing, and to what did I credit his health after venoplasty.   I told him that Jeff was much more fit now, then he was at his MS diagnosis.  He was down 15 pounds, and his aerobic capabilities were great.  He out paces me up the hills when we hike and bike, and is much more active in his daily life.

"That's great!"  said Dr. Cooke.  "He's keeping the blood flowing."  

Later that day, in his presentation, Dr. Cooke discussed how the endothelium likes fast blood flow.  Shear stress, or the stress caused by fast moving blood through our body, is good for our blood vessels.  It keeps them open, and flowing.

You see, the body and brain respond to aerobic exercise in many ways that are being studied in the MS population.  I know I don't have to tell you this---the terrible tragedy in MS is that as the disease progresses, your ability to move is shut down.  It becomes a vicious cycle.  Your disabilities increase, your ability to move decreases, your blood flow slows down, your pain and fatigue increase, your MS progresses.

I want to encourage you, like Dory, to find ways to just keep swimming.  Find ways to move, everyday.  It may not be pretty, but your heart and circulatory system need this.  When Jeff was first diagnosed with MS, he had trouble walking.  He started with an elliptical machine.  He couldn't do very much, and his legs hurt...but he did it, as best he could.  After venoplasty, he was able to get back on his bike.  He started slowly, with street rides, and then progressed to mountain biking, as his balance and endurance increased.  Every single day, he gets his heart pumping.  And his veins are staying open, and his gray matter looks normal on MRI.

Here's some research to encourage you:

Highly fit multiple sclerosis patients perform significantly better on tests of cognitive function than similar less-fit patients, a new study shows.

In addition, MRI scans of the patients showed that the fitter MS patients showed less damage in parts of the brain that show deterioration as a result of MS, as well as a greater volume of vital gray matter.

"We found that aerobic fitness has a protective effect on parts of the brain that are most affected by multiple sclerosis," said Ruchika Shaurya Prakash, lead author of the study and assistant professor of psychology at Ohio State University.

link

Of course, with an MS diagnosis, there are many things to consider before beginning a new exercise program, and seeing your doctor is a must.

Since some people with multiple sclerosis notice an increase in symptoms when their body becomes overheated—a condition known as thermosensitivity—avoid exercising outside during the hottest time of the day. Instead, exercise before 10 a.m. or after 2 p.m., or in a cool room. Drink plenty of cool liquids and always listen to your body. Slow down or stop exercising if you notice any new symptoms or your symptoms worsening. “Pre-cooling” by immersing your lower body in a cool bath for 30 minutes before exercising may also reduce the risk of becoming overheated.

Always check with your doctor before starting an exercise program; physical therapists experienced with MS can help design and adapt exercise as your abilities and symptoms change.

Best Multiple Sclerosis Exercises  When choosing an exercise program consider your interests, fitness level, and expectations. To stay motivated, choose an exercise that you enjoy. Swimming and water aerobics provide overall fitness while keeping the body cool.  Gentle to moderate yoga that emphasizes stretching, breathing, and being aware of your body followed by relaxation is another good choice, as is Tai Chi, which offers slow gentle movements. Both yoga and Tai Chi can be done sitting in a chair.

link

If you have access to physical therapy, or exercise programs for people with disabilities, please take advantage of them.  Find ways to move every single day. 

If CCSVI and the cardiovascular factor is as big a part of MS progression as we're learning in new research---it is vitally important to stay in motion.  

Just keep swimming....

Joan

http://www.facebook.com/notes/ccsvi-in-multiple-sclerosis/just-keep-swimming/10150870778672211

Hello all, the head of our national IRB spoke at our conference and explained the implications of the FDA warning to Dr. Mehta. He has been told he must stop enrolling patients and must seek FDA approval (so called investigative device exemption) (IDE) with no certainty that the FDA will accept his efforts at "corrective action".

So David has been talking with the IRB, Dake, Siskin, Ponec, and others to plan the right thing to do. David will seek an IDE from the FDA so that the multi center Registry can continue and even grow but needs to work out the details.

With regard to our testing facility, AFI, we planned on shutting it down for a while now. We have completed our fMRI research, our clinical outcomes data has been accepted for publication and our perfusion pilot study is completed, as well....

Although our wonderful patients had to pay way too much personally for the testing and treatment here this did not come close to covering our expenses and the Hubbard family, not the Hubbard Foundation, made up the difference.

The Hubbard Foundation will now focus on education and trying to raise funds to research the vascular aspect of MS and other neurovascular disorders.

Arlene Pellar Hubbard

http://www.HubbardFoundation.org

Dr. Bill Code

"Nutrition for the MS Brain"

Fraser Valley MS Society Annual General Meeting

7 pm Thursday May 31st

St. Paul’s Presbyterian Church

8469 Cedar Street

Mission, BC

Registration not required.

http://www.facebook.com/notes/ncs-the-national-ccsvi-society/dr-bill-code-to-speak-in-mission-bc-may-31/414837811870421