Monday, February 23, 2009

Today- I cried

It is a busy morning. Running behind- a grabbed the next chart and headed for the exam room. The encounter sheet noted- new patient, "LYME." I entered the room and immediately noticed three people. There was a healthy appearing middle aged man, a healthy looking somewhat younger woman and a very elderly and sick appearing woman in a wheel chair.

"So who is the patient?" I inquired, secretly hoping it was not the lady in the wheel chair; but I knew otherwise.

I looked at my new patient. She looked ancient, weary and barely alive. She was incredibly thin, skin hanging on bones with a few strands of muscle. She couldn't speak or move. Her head fell to her chest; she was only able to keep her head in an upright position with help from the other woman, her caregiver. And yet, she managed a smile. I looked again at her virgin chart. She was 51 years only; the youthful gentleman was her husband.

"Well, I stammered, let me hear your story."
Her husband had only a thin folder with a few sheaves of paper. There was a clear history of tick bites. Her hair dresser once found a small tick embedded in her scalp. She had been an active outdoor person with dogs and horses.
And she had been perfectly well until some time in 2005.

They were country people. Her husband, a blue collar worker, was not the greatest historian; He told me what he could remember. It started with weakness in the right leg. A foot drop developed. The weakness spread to the other lower extremity associated with severe stiffness. Her condition gradually worsened, the weakness and stiffness spread and now affected her entire body. She had incapacitating anxiety and profound fatigue. Her speech became garbled, progressively, over the past year and one half. She was wheel chair bound for the last year and a half. Severe dysphagia- trouble swallowing, led to her dramatic weight loss. She was continent and maintained mental clarity, I was told.

In April 2006 she went to the Hershey Medical Center. She was told she had ALS. Nothing more was offered.

In July 2006, a friend suggested a Lyme test. It was positive. Her husband showed me an IgeneX IgM report, the IgG was missing. It showed positive bands in the 23,31,34,40 and 93 positions. It was noted to be CDC positive.

They live in rural Pennsylvania. No LLMDS there. They found a local doctor who tried to help. He ordered IV Rocephin for 3 months. She improved! Her strength steadily improved. Then, the Rocephin was stopped and she resumed the inexorable down hill slide. She was on oral Doxy and getting worse.

I examined her in her chair. I could not weigh her. She appeared moribund. She was unable to speak. I noted fasiculations in her arms. Her extremities were weak although she could move her arms some. The arms were loose with normal tone. Her legs were extremely stiff with lead pipe immobility. Her reflexes were normal. Sensation was hard to test.

ALS was high on the list of differential diagnoses. The textbooks list Lyme disease as a cause motor neuron disease- ALS; no one was paying any attention. why? If she got better with Rocephin why wasn't it continued? This seems like common sense. No?

Other diagnoses crossed my mind: Anxiety and stiffness.It could be an autoimmune disorder called Stiff Person's syndrome. Then I wondered if she had a primary muscle disorder. I ordered some tests and a consultation with a neurologists expert in muscle disorders. ALS was likely. But damn it- Lyme is known to cause it! And- she had started to respond to treatment; then the rug was yanked from under her.

The woman looked deathly ill. There was not much left to work with. I wished she had seen me or someone else a year ago.

I was afraid. I was afraid to treat her, but even more afraid to not treat her.
I ordered a PIC and Rocephin, with much trepidation, knowing that treatment can at times accelerate the progression of Lyme/ALS.

A profound wave of sadness came over me. I quietly shed a tear for this poor woman.

Then I became angry, as I considered the absurd politics of this disease.

But there were more patients to see; I went into the next exam room.

19 comments:

Lauren said...

I am very sorry to hear about your patient. I am sure you have seen your share of pain and suffering of this horrible disease. At the same time, it's noteworthy how empathetic you are with your patients- a wonderful quality.

I have been following your blog since I got dxd in September 08 and would love to speak with you for a second opinion. You seem so knowledagable and I really need to trust someone to help me after such a horrible ordeal- from being told I am nuts to Doctors just telling me it's psycosomatic.. If you would be willing to do a phone consult, I can pay you. I would really appreciate if you might email me your contact info- even if I have to travel to MD (I am in NJ), I will. laurenandjason@gmail.com

If I m treated properly, I think this can be beat. The longer this goes on, I am afraid that I will have this forever. Please help me. Thank you so much for your time and for your blog!

Here is a quick rundown of symptoms: June 08- vertigo, neiro symtoms, anxiety, tingling and pure fear running through my veins, tremors, palps,blurry vision,joint swelling and pain..etc Have been on 100 mgs 2x per day Doxy since August and recently Jan upped it to 200, tried bactrim for Babesia for a mth- went off then went back on and now taking one pill per day. Now I feel hot all the time, along with chills and joint pain, and fluid in lungs.

Western blot positive for lyme and mycoplasma- clinical dx for babesia.

Please help me! I am a 29 yr old girl who NEVER felt so sick in my whole life!! I have been a trooper and struggle every day to go on but I want to kill these bugs!

Lyme report: Montgomery County, MD said...

No phone consults.
Blog is for informational purposes and my therapy- cheaper than a shrink.

I Take new patients referred through ILADS.

Seibertneurolyme said...

I like that description -- lead pipe immobility. I used to say hubby had transient quadriplegia for lack of a better description.

When hubby was in the neuro-intensive care unit at Univeristy Of Pittsburgh in Feb 2007 they didn't believe his Lyme and tickborne illness diagnosis. He was tested for stiff-man disease which was negative. At that time noise would trigger his seizure-like episodes. He could shake for 5 minutes or sometimes it might be 30 minutes or longer before he stiffened up like a board. He did have fasciculations also.

He was also unable to speak during these episodes or babbled nonsense.

IV Ativan or IV Dilaudid would resolve these episodes within 5 minutes. Without meds he would remain stiff for up to 8 hours -- at that point I threatened to administer my own meds and got some cooperation from the nursing staff.

The neuro we had consulted suggested hubby have the same brain surgery Michael J Fox had for his Parkinson's. He thought hubby's prior mercury toxicity might be the issue.

Hubby was discharged with no diagnosis and sicker than when he went into the hospital. I decided to try what had helped him the most in the past. As soon as we got back to the hotel I gave him oral quinine and clindamycin. With the first dose his tremors dramatically improved.

Within a week he was able to fly to a doc appointment in Missouri. The quinine and clindamycin at low dose (rotated with amoxicillin every 10 days) continued to work for 4 months. He just had some minor seizure-like spells once or twice a day but no more really severe episodes of muscle rigors. Sometime during that 4 months he even resumed walking for exercise.

We did do some LCD (lumbar cervical decompression) for his bulging discs found on the spinal MRI. That got him off the IV dilaudid.

Did a couple of rounds of chloroquine and primaquine and 6 weeks IV Primaxin (IV did virtually nothing) and then decided to go after the Bart found by Fry Labs on his bloodslide.

That's when eveything went south again and we were kind of back to square one.

The Babesia did disappear between the 2 bloodslides in March and September 2007, but I still wonder sometimes if the Bart or BLO or "mystery bug" was knocked down by the clindamycin and that is what really made the difference.

The 2nd go round took much longer to figure out (about 6 months) but hubby had the same dramatic response to Levaquin he had had to the Quinine and Clindamycin.

Currently lactoferrin and the herb Cryptolepis seem to be working miracles. Tremors and myoclonus are almost nonexistant -- seizure-like spells have decreased to once every 5 days or so -- the longest intervals between spells since they started in 2002 after his first treatment for Lyme (the herbal formula Borrelogen).

Hubby is not a sucess story yet, but I want others to know that there is hope even with severe unexplainable symptoms -- the usual response from neurologists for things they do not understand is go see a psychiatrist.

If someone tries 10 or 20 meds and suddenly something works I don't buy into the theory that it is psychosomatic.

My advice would be to not assume it is always Lyme, but to always do a trial of meds for Babesia and Bartonella if you aren't responding to traditional Lyme meds.

dogdoc said...

I'm sorry- so sorry for everyone involved. Why does it have to be this way? Why could the Rocephin have not been restarted when she started going downhill again? What did we have to lose? ALS is fatal. And treatment at the end can make it worse. But if you don't- well you have lost no matter what. Why the senseless handling of peoples lives this way?

Blackbird said...

How strong is the evidence that Lyme can cause ALS specifically?

I understood that the evidence was that Lyme can cause A motor neurone disease (i.e. it can cause motor neuropathy) but no evidence that it causes ALS specifically

The Martz case certainly does not have a typical onset for ALS - i.e. he had sudden onset whereas ALS has insidious onset.

Strange, in your patient that reflexes were normal with that degree of lower limb altered tone and strange for possible ALS.

I agree that, even if there is doubt, if the patient agrees then treating as possible Lyme is, on balance, justifiable.

But the other issue is whether the patient and family are left thinking "if only Lyme had been picked up sooner, then it would never have got this bad"...when perhaps it is not and was not Lyme and she would have developed the condition anyway.

The Lyme serology could be incidental as it is not always symptomatic, or it may be contributing a small , antibiotic-responsive part to her overall apparently neurodegenerative presentation.

I wonder what her CSF showed.

Hope you post about how she does with the antibiotics.

Joanne said...

I too shed a tear when I read this.
Many of her symptoms were like mine 6 years ago but thankfully my GP considered lyme after a chance course of antibiotics improved my arthritis/muscle weakness.I gave her Burrascano guidelines and she continued to treat inspite of advice from our HPA in UK. Eventually I found an LLMD in UK and now after 3 1/2 years (2 years antibiotics)I can again walk upstairs and no longer have constant pain. Thankfully I was helped by two good doctors like yourself and so didn't develop as badly as your patient.

The real reason I shed a tear is because of a little boy aged 5 I know of currently being assessed for Lyme Disease who was normal until age 2 and whose symptoms developed very similarly to your patient. He is now in excellent hands with LLMD and also a LLMD's colleague a world top Neurologist. I hope it is Lyme, I hope they can help him for him, but also for the rest of us because until the Consultants like the few precious Doctors start to take this illness seriously more little children will suffer and that is what makes me praise you wonderfully courageous doctors for doing what you believe and not what you have been told to do.
Thank you.

Lyme report: Montgomery County, MD said...
This comment has been removed by the author.
lymie said...

Dr. Martz may have been a rather quick onset of Lyme produced "ALS," but many of the patients in this category have had a more gradual onset, with typical lyme symptoms and some with positive tests.

Furthermore, ALS is probably a group of symptoms that can be produced by more than one agent. Pathogens, chemical toxins, etc. So, the idea that a diagnosis of ALS tells you anything about the cause (and potential treatments) is false.

It has been known for over a hundred years that a quarter of ALS patients had a previous history of syphilis. So, why would it be surprising to find that another spirochete could also produce such symptoms? Do we learn nothing from medical history?

The problem with treating a woman in this condition is that she might not be able to take the full dose of antibiotic, might need to be ramped up, to avoid a damaging herx.

It is so enraging that this woman and many others have been failed utterly by the medical establishment, and doctors who then try to help basket cases are persecuted by state medical boards. This country is so corrupt.

Blackbird said...

To Lymie

Do you have a reference that says that 1/4 of people with ALS have had syphilis?

Thanks

Blackbird said...

To Lyme MD,

I read your comment before you removed it.

The following is about ALS:

http://www.wfnals.org/guidelines/1998elescorial/elescorial1998.htm

The following parts may be of interest:

Appendix 1 ALS-Plus and ALS-Mimic Syndromes

Appendix 2 ALS with Laboratory Abnormalities of Uncertain Significance (ALS-LAUS) Syndromes

Schema of El Escorial Revisited

As I am sure you are aware, it would be unusual for American Lyme to present with a purely motor form, with no sensory symptoms and no arthritis. It is usually a notably multi-system disease.

I would be glad to find that ALS can be caused by Lyme, as it would then be treatable, at least early on. If it triggers a cascade of events after infection, regardless of the ongoing presence of the infection, then that is less good news.

I am neither linked to IDSA or ILADS.

lymie said...

Blackbird, you sound like a lot of other people I have heard say that lyme cannot cause "ALS." And that kind of closed mind has sent people to the cemetery ahead of schedule.

The fact is that major medical institutions and very experienced ALS specialists have diagnosed ALS in people whose symptoms were actually caused by lyme. And these people have been told to get their affairs in order, had feeding tubes installed, etc. Subsequent treatment for lyme caused a reversal of these symptoms.

The point is that it is not so easy to determine when "ALS" is caused by lyme and when it is caused by something else. If the experts have made these mistakes, then it obviously does happen.

Lyme is not as predictable as you think it is. Perhaps cookbook medicine leads doctors to think everything is neat and simple. But the course of lyme disease and its presentations vary tremendously. As does response to meds. Perhaps it depends on what coinfections are present, or the germ load, or something else. But please do not rule out lyme when it doesn't follow the recipe.

The reference you asked for can be found in the 1940s era syphilis textbook by Stokes, et al. This cites a study from 1900 or thereabouts which described 25% of ALS patients having a syphilis background. I doubt if present day ALS specialists know this though, and have probably not studied syphilis much or consulted older texts. This is why I asked whether we really learn anything from medical history, or just keep making some of the same mistakes over and over.

Blackbird said...

To Lymie,

I didn't rule out Lyme. I said that it could be justified to treat as Lyme, but doing so with an awareness that it might not be Lyme i.e. patient and her family knowing that it might not be Lyme.

It would be interesting to know why the first doctor stopped the Rocephin.

Lyme can cause an ALS-mimic - and an ALS-mimic can be misdiagnosed as ALS. Guidelines for diagnosing ALS include looking for evidence of infections including testing for syphilis and Lyme. Doctors are aware of the possibility of syphilis and Lyme as ALS mimics. I know there is controversey over interpretation of Lyme testing.

Surely you agree that it would be unusual for Lyme to present with only one symptom modality? It isn't cookbook medicine it is probability. But this lady is an individual and so her case is considered individually.

Thanks for the Stokes ref.

Please, do you have a reference about the cases of people told they have ALS and then being found to have Lyme and their symptoms improving with Lyme treatment?

Thanks.

lymie said...

[ALS-like sequelae in chronic neuroborreliosis]

Hänsel Y, Ackerl M, Stanek G.

Wien Med Wochenschr. 1995;145(7-8):186-8. German.

PMID: 7610670 [PubMed - indexed for MEDLINE]

---------------------------------

Immunologic reactivity against Borrelia burgdorferi in patients with motor neuron disease.

Halperin JJ, Kaplan GP, Brazinsky S, Tsai TF, Cheng T, Ironside A, Wu P, Delfiner J, Golightly M, Brown RH, et al.

Arch Neurol. 1990 May;47(5):586-94.

PMID: 2334308 [PubMed - indexed for MEDLINE]

---------------------------

Generalised motor neuron disease as an unusual manifestation of Borrelia burgdorferi infection.

Hemmer B, Glocker FX, Kaiser R, Lücking CH, Deuschl G.

J Neurol Neurosurg Psychiatry. 1997 Aug;63(2):257-8. No abstract available.

PMID: 9285472 [PubMed - indexed for MEDLINE]

Free article in PMC | at journal site
------------------------------

[Motor neuron syndrome and Lyme disease. Relation of causality or fortuitous association?]

Deibener J, Kaminsky P, Debouverie M, Aubrun P, Maurer P, Gérard A, Duc M.

Presse Med. 1997 Jul 12;26(24):1144. French. No abstract available.

PMID: 9255242 [PubMed - indexed for MEDLINE]

---------------------------


A predominantly motor polyradiculopathy of Lyme disease.

Scelsa SN, Herskovitz S, Berger AR.

Muscle Nerve. 1996 Jun;19(6):780-3. No abstract available.

PMID: 8609932 [PubMed - indexed for MEDLINE]

------------------------------

Here are a few, mostly case reports. They assume it is rare, but what is really rare is that anyone makes the connection. This kind of thing tends not to get published, as the major institutions who make mistakes do not publish about them, or in fact change their minds, regardless of what proof is shown to them.

Doctors who treat lyme have seen a number of such cases, but they have their articles rejected by medical journals, as this does not fit with the current thinking.

If you are a doctor with a particular interest in this subject, you can look up pubmed refs just like I can. If you really are genuinely interested, then ILADS can help you hook up with people who know a lot about this subject. However, they would not want to waste their time on a provocateur.

In fact, there is a doctor who would like to publish on a substantial number of such cases, but his only option at this point seems to be obscure foreign journals. Having seen how often this kind of thing happens, I know why medicine has not found solutions to so many problems. There are too many people committed to the status quo, regardless of its ill effects on patients.

As far as multisystem diseases are concerned, specialists tend to see only the symptom that is in their playbook, and ignore the others or call them nonspecific. And they assume lab testing for lyme disease is infallible, regardless of published evidence to the contrary. This really is just laziness on their part.

The rheumatologists only see the arthritis that is part of lyme, and ignore the neuro symptoms and the psych symptoms. The neurologists tend to get lyme wrong most of the time. What often happens is that doctors who can't figure out a multisystem disease send those patients to a psychiatrist! Patients have been told "no one has that many symptoms." Does this sound like doctors understand multisystem diseases?

This really is a very bad time to have lyme disease. I am so disillusioned with medicine.

Blackbird said...

Thanks Lymie for the refs. They were ones (case reports, as you say) of which I was aware but I was wondering if you had information that gave more of an idea of how often this is occuring.

Much of what you have presented is Lyme as an ALS-mimic. The one about the serology has been countered with one that says there is no link - so hard to make a fixed conclusion on that as yet.

I hope that the doctor you mention manages to get his work published and that it is compelling. As I say, it would be good news if treatable Lyme were found more often in people who initially appear to have a devastating diagnosis like ALS.

I agree with you that specialists can be deaf to issues outside their specialty and that is a real problem with undiagnosed Lyme. Family doctors seem in the best position to recognise the whole person.

And finally, not everyone who presents a challenging view is a provocateur. Opinions on Lyme have become a polarised and the truth may well be a hybrid of those positions.

lymie said...

Will the real ALS please stand up?

If a group of symptoms that has been given the ALS name is caused by more than one agent, how can you say which is a mimic?

Why is the medical profession, or at least some of it, resistant to the idea of multiple causes? They can imagine that pneumonia is caused by more than one pathogen.

The problem with ALS is that the medical literature basically documents failure: failure to find an effective treatment, failure to identify a cause. So, if you are looking for medical literature to back up a lyme causation for some cases, this will be hard to find. And cases submitted to journals will be rejected if they don't fit the current view.

And the other problem with ALS is that neurologists do their testing, try to find the right box to put these patients in, then tell them there is no treatment. That box is very soon put into the ground.

Are there any neuro diseases that neurologists can cure? Maybe they are so used to failure that it doesn't bother them anymore.

But if some of these cases are treatable, isn't it worth the effort to identify them?

Lyme report: Montgomery County, MD said...

All current medical texts list Lyme as a potential cause of ALS or motor neuron disease, one and the same.

There is no published research regarding treatment; so of course mainstream physicians do not treat even when the Lyme diagnosis is clear cut.

Unfortunately, Dr. Martz's studies, as alluded to, have failed to see the light of day.

LLMDS will of course give it their best shot.

If a patient is doomed to a horrible death, how can anyone with a drop of compassion argue against therapy?

Blackbird said...

To Lyme MD,

We agree - treat as Lyme if there is a suspicion of it. That is overwhelmingly better than leaving a potentially treatable condition undiagnosed and untreated.

I would be inclined to ensure that the uncertainty is very clearly communicated to encourage a realistic level of hope.

We have a different reading of current medical texts. I understand Lyme to be a differential diagnosis to be ruled out (as if that were readily possible) before a diagnosis of ALS can be given.

The link I gave before, for the World Federation of Neurolgists' criteria for the diagnosis of ALS doesn't work, here is a Tinyurl of it:

http://tinyurl.com/2c3dpo

Under the requirements for the diagnosis of ALS it excludes "...pathological evidence of other disease processes that might explain the signs of LMN and/or UMN degeneration,".

This site also talks of the ALS mimics.

And to Lymie,

the neurologists I know keep looking for Wilson's disease - just in case they may one day find it, as it is treatable. The ALS specialist looks for Lyme and syphilis because they are treatable.

Lyme report: Montgomery County, MD said...

Blackbird

I believe you are a well informed physician.

The CDC and IDSA are determined to undermine the treatment of patients like the one described here. Furthermore, they are determined to censure the medical licenses of physicians who think outside their narrow box.

If you are a neurologist I would appreciate your support. You can contact me through ILADS.

The notion that Lyme is an imitator is one that is worth full exploration. I have treated patients diagnosed with MS who have experienced a full remission when documented Lyme disease was treated. The guidelines for the diagnosis of MS also require that physicians rule out other causes of CNS demyelinating disease such as LD. Community physicians ignore their own specialty guidelines and mock physicians who diagnose Lyme disease. Unfortunately, many seem to ignore the clinical history and exam and make a diagnosis strictly based on MRI criteria.

Evelina said...

I'm really sorry to hear about your patient, I hope she's well.

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