Thursday, September 8, 2016

Lyme - ALS and the practice of medicine


This 54-year-old female is happy. Rightfully so.  She has been pulled from the grave.
5 months ago she was handed a death sentence, ALS (Lou Gehrig’s disease), the worst kind at that.  She found she suddenly had trouble speaking, becoming hoarse then losing the ability to talk. Shortly thereafter she found it hard to swallow. She lost weight. She developed weakness of the right upper and left lower extremities. She went to a neurology clinic at a famous tertiary care center.  She was diagnosed with bulbar ALS, a most severe and deadly form. The expectation was that she would need a feeding tube followed by ventilator. The doctors at the clinic continued to follow her downhill course over a three-month period. An EMG test showed the expected motor neuron denervation.  The patient reported an ultrasound exam of superficial nerves showed nerve swelling rather than atrophy as expected.   Scratching collective heads, the neurology team decided to do a lumbar puncture. Lyme was considered. The endorsed DNA/PCR test was negative.  One of her doctors ordered a non-sanctioned test:  Lyme Western Blot antibodies in the CSF. The result was positive. A blood test for Lyme (ELISA first) was negative.  Nonetheless, she was referred to an ID doctor who was sufficiently impressed to prescribed IV Rocephin. After 2 weeks routine monitoring showed an elevation of the kidney function tests, BUN and creatinine and antibiotics were discontinued, despite the fact that she had already responded favorably to the 2-week course of treatment.  She told me her neurologists, expecting rapid deterioration, were recently surprised to see her walk into their office under her own power.
This was the point at which I met her.

When I first met her (6 weeks ago) she could barely eke out a few unintelligible vocalizations.    She coughed constantly (due to aspiration secondary to inability to swallow I suspected).  She had other limb weakness and joint pain and swelling.

She had lost 15 pounds and was clinically dehydrated, not in renal failure. Patients with neurological dysphagia (impaired swallowing) have trouble swallowing “thin” water.  Per my recommendation, with the addition of thickener to fluids, she was able to hydrate well and the kidney tests normalized.
After 6 weeks of IV antibiotics, our second visit, she spoke with me with a very hoarse, gravelly but also very intelligible voice. She was no longer coughing. She was getting stronger and becoming more active day by day.

During examinations she evinced typical neurological signs associated with ALS, including:  hyperreflexia, weakness and abnormal reflexes (Hoffman and Babinsky).

Her illness also encompassed my other typical Lyme features:   Brain fog, joint pain, night sweats and air hunger.  These symptoms were in various stages of getting better.
Lab results: Positive Lyme Western Blot IgG, 8/10 bands MDL, Giemsa blood smear positive for parasites (presumptive Babesia).

More of the story unraveled.  She lives in the country near Frederick Maryland.  She loves to garden.  She also is an avid camper in Southern Maryland.  She recalls finding a red patch on her right forearm 2 years which went away with the application of cortisone, given the diagnosis eczema.
She went back to the original neurology clinic and was told that she should only get 28 days of Rocephin based on CDC guidelines.  She also patient sought the opinion of another University based neurologist who said keep treating for Lyme.


Motor neuron disease or ALS is a known, albeit extremely rare manifestation of neurological Lyme disease.
Of course there are no guidelines for the management of Lyme induced ALS.  I suspect the tertiary care specialists (recommending antibiotics be stopped) were extrapolating from obsolete IDSA guidelines, predicated on the belief that Borrelia spirochetes do not persist after antibiotic therapy, a thesis that has been thoroughly discredited and longer the dogma of mainstream thinking.
This must stop.  Lives are at stake.

Health care professionals need to know what Lyme is and what the practice of medicine is.

Lyme is a new and emerging disease, an epidemic of epic proportion with the potential to kill and maim.  The clinical manifestations of the disease are protean.  The extent and severity of the disease has not been recognized by the medical profession and public health officials.  Progress towards a better understanding of the illness has been mired in war of ideas (driven by egos of certain individuals in the academic medical community). Very little research has been done and we sadly know very little about this new disease which reared its ugly head 40 years ago. In a general sense we do not know the optimal therapies for the management of the illness. We know even less about specific variations of therapy which may be optimally effective for the widely divergent clinical presentations of illness.

The practice of medicine is the application of the current iteration of the healing arts as they have evolved over hundreds of years. Today's doctors are better informed (much more is known) than their forbearers of years gone by. By the same token, doctors must be cognizant of the certainty that their future replacements will see them in the same light. The practice of medicine is based on a complex synthesis of science, fact, experience, educated guesses, empiricism, judgement and perhaps philosophy, prescribed by a thoughtful (hopefully intelligent) physician who is committed to his  creed and solemn responsibility. My dad (a surgeon) always said medicine is a calling, not a job. A bit preachy, but true.

Lyme patients frequently suffer a slow, smoldering sort of death with a quality of life comparable to that of terminal cancer patients.

Contemporary notions of medicine, created by institutions and corporations demand tangible metrics by which physicians be judged.  This may or may not work.  Currently the wrong metric is being applied, i.e. following guidelines.  This is bean counting.  What is important? the patients - of course. This may not be obvious to the institutions that oversee medicine.  The primary metric that should be followed is patient outcomes. This is the only useful measure, when the disease, as is the case with Lyme, is complex and poorly understood.

The "system" should learn from doctors who make their patients better, not condemn them, as is frequently the case.


6 comments:

Christian said...

PCR in blood is frequently negative. Tissue samples of affected tissue have higher positive rates. But how about pathogen concentrating methods? I'm talking about the FcMBL filter from Wyss institute. http://wyss.harvard.edu/viewpressrelease/215/harvards-wyss-institute-improves-its-sepsis-therapeutic-device
The filter could yield high concentration of pathogens, sufficient for a positive PCR. I spoke to Dr. Ingber about this and they were already explorning this possibility. Don't know about the progress though.

callingcolleen said...

My stepdad has ALS. I think there's a possibility it's Lyme. He has peripheral neuropathy which not a symptom associated with ALS, but is associated with Lyme. He also has had episodes of afib. We live in the PNW, but my understanding is Lyme is becoming increasingly common here as well. All that said, even if the chance of Lyme is slim, it would sure be great if we could save his life with doxycycline, right? So how can we get him a reliable test? Thanks in advance for your help.

T9im said...

Hi Callingcolleen: Contrary to the CDC and IDSA there is no reliable test but you can run an IgeneX Western Blot and see if there are any positive bands. We did this with our daughter. Note we have run 22 Western Blots (Igenex and Stony Brook), none positive via IDSA standards but have had various Lyme borrelia positive bands

lymie said...

Colleen, you should contact lymedisease.org for more information. They are located in California. Yes, lyme is on the west coast, carried by a different tick than on the east coast.

Lyme report: Montgomery County, MD said...

If patients with ALS and possibly Lyme have a lumbar puncture do every available test. Western Blots of CSF (MDL, CLongen, Stony Brook, IgeneX), C6 peptide antibody, PCR (recommend IgeneX. Run serum at the same time, 3 different labs and look at the bands on the strip. I just looked at a strip from MDL called "equivocal" with 4 CDC IgG WB bands and the fifth 58% if the control (60% a positive). Consider additional testing if unsure: Blood culture to ALS labs in Philadelphia. Nanotrap urine test (newer). Make sure. If you are sure go for it. In my experience Lyme/ALS patients who will respond to Rocephin will respond rapidly with the first 2-4 weeks.

Mdelia said...


"Health care professionals need to know what Lyme is and what the practice of medicine is. "

Thank you for this statement of courage, and please keep the info flowing.