In November of 2010, I was called to the Emergency Department
to evaluate a woman who had been sent in by her rehabilitation facility because
she was not acting herself. Ms. Jackson
(as I will refer to her here in order to keep her identity confidential) was a
51 year-old woman who had recently been diagnosed with the Human
Immunodeficiency Virus (HIV) infection. She
had been sent to the rehabilitation facility after presenting to a local
hospital with weakness and was found to be anemic. Further testing revealed that she had some
inflammation of her stomach lining, so this was treated and she seemed to be improving.
But in the ensuing days, her ability to
perform higher-level cognitive skills began to decline over the span of two
weeks. Her daughter noticed it first,
and then medical personnel began to pick up on overt signs of “encephalopathy”
– a generalized disturbance of brain function. So they brought her to the hospital, and this
is where I met her for the first time.
Ms. Jackson was not the typical case of encephalopathy that
came through our emergency room doors. Most
of the time, in the barrage of blood tests and imaging that most patients with
this symptomatology are subjected to by the first physician who treats them, a
hint is usually uncovered which oftentimes leads to the answer. And then I start appropriate treatment as they
continue being cared for on the medical ward, and the patient sometimes gets
better. Things were different for Ms. Jackson.
Her blood tests and imaging were of no
help. I even drew fluid from around her
brain and spine – the infamous “spinal tap”. Even this was not helpful.
So I reset my thoughts and started over with her. There were a few aspects to her physical exam
that were a bit unusual. For one, she
was extremely cachectic. This pointed to
the chronicity of her disease, and was most likely due to the HIV infection
itself. She was also “catatonic” – her facial
expressions were largely frozen in place, and it seemed as if she wanted to
speak but did not have the energy to even move her mouth. The most curious part of her exam was her
apparent loss of the usual spinal reflexes in her arms and legs. We asked specialists in neurology to assist in
the diagnosis, and their recommendation was to send her blood to be tested for
the presence of certain antibodies that can attack nerve cells – specifically,
one that is named GQ1b. The problem with the test for this antibody is
that the answer is unavailable for up to two weeks. In the meantime, we tried every antibiotic and
steroid available to us to treat Ms. Jackson, hoping that one of them would spark
her recovery. But her illness prevailed,
and eventually she began to have seizures and difficulty breathing. After many lengthy discussions with the
family, we decided it would be best to pursue comfort-care for Ms. Jackson so
that she would no longer suffer. After two
weeks of merciless testing, imaging, and experimenting, Ms. Jackson left our
ward to die at home in peace.
The next day, her GQ1b
antibody test resulted – much to our surprise, she was found to have a
small amount of this rare antibody circulating in her blood! Now what? Over the last week of her hospitalization, her
family had begun the coping process that had ultimately culminated in them
accepting her imminent death. Was it
ethical for me to call her family and tell them to bring her back to the hospital?
What does the presence of this antibody
even mean?
I went to the medical literature for guidance. It turns out that the GQ1b antibody is not very well understood. What we know is that the nerve cells in our
brain and spinal cord are made up of a substance called “ganglioside” that can
sometimes be the victim of an antibody that our own body can spontaneously produce
as a result of an intestinal infection or other abnormality of the bone marrow.
It is rarely observed, and because of
this, much of what we know about the diseases caused by this antibody is from
individual case reports. This antibody
has been connected to the Miller-Fisher syndrome and Bickerstaff brainstem
encephalitis, both of which are very rare conditions – both of which shared
features that were present in Ms. Jackson. Albeit the quantity of antibodies present in
Ms. Jackson’s blood were less than the usual patient with these potential
syndromes, the laboratory result in front of us brought up the question of the
next step. In a patient still currently
hospitalized, there would be a discussion of whether trying a procedure called “plasma
exchange” would be beneficial. It would
involve plugging a very large tube into one of the patient’s large central
veins, removing blood from the patient’s body, filtering it through a
dialysis-like machine, and then returning the cleaned blood to the patient, free
of any harmful antibodies. But
laboratories sometimes make errors, and what if the low level of antibody to GQ1b present in Ms. Jackson’s blood was a
mistake and didn’t mean anything at all?
With Ms. Jackson, there remained the bigger question of
whether interrupting a coping family’s mourning of a soon-to-be-departed family
member was justified in the midst of a potential neurologic diagnosis whose
outcomes after treatment are not predictable or guaranteed by any means. And what about a single laboratory test whose
significance is not fully understood and, frankly, may not mean anything at
all? As a comparison, some patients have
detectable levels of rheumatoid arthritis antibodies circulating in their blood
without ever developing symptoms of arthritis.
For these uncertainties, we decided not to interfere with the dying
process. This was not a blocked coronary
artery that would lead to certain death if left untreated. With Ms. Jackson, there were too many “ifs”. So she passed peacefully with family at her side,
and we were left to wonder whether pursuing aggressive therapies in this dying
young woman would have changed anything.
With regards to our current practice of medicine, what we do
now is based simply on what we know. And
what we know is heavily dependent on our past experiences of human disease. Yet we know that there is a plethora of
knowledge to be discovered, and hundreds of diseases still to be named. So why should anything ever be deemed impossible? One hundred years ago, we thought that heart
attacks could only be treated with bedrest – now we have stents and surgery
that have allowed people to take back their lives. Thirty years ago, we believed all stomach
ulcers were caused by stress – now we know a large portion of them are caused
by bacteria that can be successfully treated with antibiotics. In fifty years, I may look back at Ms. Jackson’s
case and see a clear diagnostic or therapeutic option that we had never tried. But such are the shortcomings and beauties of
medicine. Practicing medicine in the 21st
century where our imaging and laboratory testing have sometimes accelerated
past our knowledge of new human disease, it has never been more important to
uphold the ethical principle of non-maleficence (i.e. “First do no harm”) to help
guide us in making these tough decisions for our patients and their families.
Doc Veritas
Doc Veritas
