Lyme in Atlanta

This patient is from Georgia. He used to a lot of outdoor work in his garden. He was also a frequent hiker on the Appalachian. He had a long history of tick bites which he thought were inconsequential. In 2007 he developed disabling pains of his arms and legs to the point that he was unable to walk.  He saw a neurologist who performed various tests. An EMG showed a severe polyneuropathy, motor and sensory. He experienced numbness and tingling. He experienced difficulty swallowing; episodes during which he was unable to speak and episodes of muscle paralysis. His muscles would suddenly go into acute spasms associated with myoclonic jerking and fasciculations. He went on to develop a host of other symptoms. Some of these included: emotional labiality, a loss of balance, unremitting tinnitus, extreme sensitivity to both light and sound. His skin became extremely sensitive to touch and he experienced a heightened sense of pain whenever his skin touched something. In fact, he has not had a repeat EMG because of intolerable pain.

He saw a variety of neurologist who ordered a multiplicity of other tests including MRI scans and EEGS which were of no benefit. After some time, he was not longer to walk progressing from cane to wheelchair.

Joint pain became prominent. He experienced pain and swelling in all his small joints, medium joints and large joints. He also developed pain in his cervical and lumbar spine areas.

On further questioning symptoms now include: fatigue, night sweats, air hunger, brain fog, trouble articulating words, forgetfulness, inattention, drooping of one side of his face among many others.

Doctors claim there is no Lyme in Georgia.

Let me take a step back. In 2009 his wife suspected he had Lyme disease. She was able to find a doctor who, over a course of years, treated him with antimicrobials including: Minocin, Zithromax, Flagyl and Malarone. The treatment was somewhat effective. The same symptoms were present but had lessened, maybe 30%.

Six months ago a new doctor, taking the place of the previous one refused to prescribe any more antibiotics. Over this period of time symptoms worsened dramatically.

When he saw me there was a desperation to find a doctor who would continue to prescribe medications.

The diagnosis of Lyme disease had been empiric. Lab tests had always been negative.

When I examined him he appeared chronically ill. Most notable, when I asked him to squeeze my hands to test his strength -- always a part of my exam --  his hands became locked in a spasm with every increasing pressure applied to my hands. My hands were stuck and beginning to hurt.  I did not know this is possible. I had to call my assistant to help pry open his fingers so I could remove mine.

He had never been treated with IV antibiotics and I felt this should be tried right out of the gate. So I ordered some lab tests.

A Lyme WB from Stony Brook found only a 64 IgM band and a 58 IgG band. This was frustrating. A WB sent to IgeneX found only a 58 IgM band and a 41 IgG band. I hit pay dirt with labs sent to Labcorp. The C6 peptide ELISA was 1.34 -- clearly positive for Lyme. In addition, serology for Rocky Mountain Spotted Fever was also positive.

My initial impressions included: Lyme, Babesia, Rickettsia organism. I ordered: Rocephin, Doxycycline, IVIG, Tindamax and Coartem.

I saw him at follow-up a month later. He had only taken the doxy, Tindamax and Coartem. The IV Rocephin and IVIG had not been approved. Nonetheless, he reported feeling significantly better. Surprisingly better.

Another test showed an immune deficiency. His total IgG, and subclasses 1 and 3 were low as was his IgM.

Additional comments:

I diagnosed CIDP clinically. He had pain, weakness, marked sensory/ motor abnormalities and absent deep tendon reflexes. The neurologists would not consider the diagnosis without another EMG which he is unable to have. Of note, he has a complete absence of reflexes: this is something neurologist love to see to make this diagnosis.

If his local doctors were not buying the CIDP diagnosis there was clear evidence of an immunoglobulin deficiency typical of Common Variable Immunodeficiency Syndrome. This diagnosis was also not made back home. Generally with this diagnosis it is possible to have subcutaneous Ig approved.

He has now had two sessions in my hyperbaric chamber. He is doing surprisingly well.

This case underscores the importance of treating clinical babesiosis. In this case I went straight for the big gun: Coartem. This seems to have paid off.

The positive test for RMSF might reflex some cross-reacting Rickettsia, less virulent than RMSF. He had never been prescribed doxycycline previously. The addition of this medicine may have been important. Don't forget to take doxy at some point during therapy.

I am hoping hyperbaric oxygen therapy will do for him what I thought IVIG would.

A key lesson in this case is: don't forget to order the C6 peptide. 

2013: A Brief update

Certain posts consistently have a lot of page views. This includes 2010: a brief update.  I don't agree with my 2010-self in many ways. Here is another update.

Update 2013

Testing:

The diagnosis of Lyme disease remains clinical.  The primary test for Lyme disease is the Western Blot, (WB).   An ELISA may be ordered (as a separate test) as long as it is not “reflexed” to a Western Blot.  The C6 peptide ELISA is always ordered, but not interpreted according to Immunetics criteria.   Western Blots are generally sent to one of several reference laboratories. I currently prefer Stony Brook because they take insurance and their assay includes many additional bands. Frequently, WBs are sent to two reference labs.

I do not order a lot of immunological tests like C4a because I have not found these tests to be clinically useful.

A typical coinfection panel includes serology for: Ehrlichia, Anaplasma, Bartonella, Babesia microti, Babesia duncani (still frequently called WA1). I may include Rocky Mountain Spotted Fever as well as a few others.  Positive results are very helpful but negative results are neutral and do not exclude the presence of disease. A thin stained blood smear should be considered in the workup because Bartonella-like and Babesia-like organisms may be seen. More advanced microscopic tests may be performed but are not standard.  Immuno-florescent microscopy. e.g. FISH) may be helpful in some cases.  Blood cultures may be very useful.  The test is costly and positive result requires a PCR for confirmation.  PCR testing may be done with cord blood to exclude placental transmission to a new born baby.  PCR should be used with non-blood body fluids such as joint and spinal fluids. Antibodies should also be measured, i.e. WB, C6 peptide.

Antimicrobials:
 

For the treatment of resistant Babesia, Coartem should be used rather than Larium as the next step. I generally avoid quinolones such as Levaquin and Factive because of tendon toxicity. Bartonella species may become rapidly resistant to quinolones: Biaxin/doxycycline/Bactrim/Cleocin in combination with Rifampin is a better approach. Rifampin should never be used alone because of the rapid onset of resistance. I left Zithromax off the list, which I still frequently prescribe, because it may have greater cardio-toxicity.

A wide spectrum of intravenous antibiotics may be used.  Rocephin and Flagyl remain very important.

Biofilms formation is an effective strategy for long term survival of microbes. 

This segues into hyperbaric oxygen therapy which disperse biofilms. I have written about its effectiveness and usefulness elsewhere.  I try to incorporate Hyperbaric oxygen therapy, HBOT, when feasible, especially in tough cases.

 

IVIG may be incredibly helpful but is usually only be approved for certain types of neuropathy.  A skin biopsy sent to Therapath, looking for small fiber neuropathy is now an important part of my practice.

An evaluation of immunoglobulins, including IgG subclasses has become part of the typical workup. Deficiencies are very common.

Triple Whammy

Sometimes a patient doesn't see it when it is so obvious to others.  That's the way it can be with slow and steady progress. 

I have known this patient for 7 years.  When I first met her she was on death's door.  Her prior doctor had given up and told her there was simply nothing else he could do. I treated her for a time. There were some ups and downs with one life-threatening event. I certainly hadn't given up --  but one day she was gone. Like many other critically ill patients, who never give up and whose disease is relentless, she had made the rounds. She had seen three other well known doctors who had been unable to help her. Then she completed the circle and made another appointment in my office. She walked through my office doors about 4 months ago for the first time in as many years. She was in a wheelchair:  unable to walk because of weakness. Communications were difficult in part because of strange, what I call, chorea-form like, jerking movements about her face, head and neck. Like many other Lyme patients she exhibited strange, uncontrolled, body movements outside the realm of standard medical diagnostic groups. Other physicians invariably diagnose a psychological basis for the movements. 

But that was not the only reason communication was difficult. Her speech was severely slurred (dysarthric); she lacked adequate muscle control of muscles of her tongue and mouth making it impossible to enunciate clearly. To make matters worse, she was severely cognitively impaired. She lacked the ability to put words together in proper sentences. This is what we call aphasia. Her thoughts were scattered and very difficult to make heads or tails of. She looked sicker than I remember the last time I saw her.

When I see patients like her, now, I go down the short list of aggressive therapies which might help. First there is IV antibiotics. She had been on numerous drugs for many months without much improvement. Then I think immunotherapy. Getting it approved is the trick.

Lyme is a multisystem disease. It is also a multisystem, within a system disease. When it affects the nervous system it generally effects multiple components. The nervous system is extraordinarily complex, but following the KISS, principle, for the patient and myself, (Keep It Simple Stupid) I break down the nervous system into a few basic components. The central nervous system consisting of the brain and spinal cord. The cranial nerves, a dozen nerves branching  directly from the brain. And the peripheral nerves: everything that branches off from the spinal cord, including motor, sensory and autonomic parts.

The brain being the preeminent piece of the central nervous system (CNS) can be assessed with MRIs, PETs, SPECTs, other imaging modalities and paper and pencil tests. The autonomic nervous system is best assessed with a tilt table test looking for POTS. The peripheral nervous system has traditionally been evaluated by electrical testing, (NCV/EMG). But now I have another modality.

Her EMG test was normal but I still thought she had a problem with her peripheral nerves. I performed a skin, sweat biopsy test which looks for something called small fiber neuropathy.

The lab sent us a report saying it was essentially the worse looking SFN they had seen. Bingo. Her insurance company approved her for IViG, the best immunotherapy available.

Insurance companies will only approve the $10,000.00 dollar every three week treatment if you can convince them you have one of a limited number of progressive peripheral nerve disorders. This is not an easy feat.

The third item on my short list was HBOT, Hyperbaric oxygen therapy. I have written some about HBOT already and so far I am very impressed.

Now her treatment consists of 1) antibiotics, 2) IViG and 3) HBOT.

Today she walked into my office under her own power. Her speech was clear and easy to understand. Her eyes were clear and her thoughts were completely cogent.

This is not to say she does not have some other, very serious problems which we are working on, and expect to continue working on for a long time to come.

Today, she really didn't get it. She had no clue how far she has come.

Her transformation is beyond words, so far.

Incidentally, we doctors are all used to patients trying other physicians. I would if I were you. We expect this. You should never feel embarrassed going back to a previous doctor. The doctor is (usually) happy to see you back. We doctors do things differently. Sometimes one will have more success than another.