Dear Lawrence,
First, let me say that I?m sorry to learn of the difficult situation
you find yourself in and of the unfortunate events that have befallen
your son.
I am a surgical resident (MD), and can give you some information about
this situation, however this answer is no substitute for direct
medical advice from someone who can physically examine your son and
personally view his medical records.
Let me attempt to tackle your questions one-by-one:
___________________________________________
IF A "NORMAL IN GOOD HEALTH" PERSON#1 WERE USING AND ABUSING THE DRUG
"PREDNISONE""FOR A PERIOD OF 2 WEEKS, WHAT POTENTIAL HEALTH PROBLEMS
COULD OCCUR OR ARRISE FROM SUCH USE?
At the dose you mention (40mg), Prednisone (a glucocorticoid ? a type
of steroid), would affect the immune system and have effects on the
endocrine system. Prednisone is used to treat a wide variety of
diseases, including inflammatory disorders such as Multiple Sclerosis.
The dose for MS is typically 200mg once a day for a week, then 80mg
every other day for a month.
A few words about how cortisol acts normally in the body to put it in
context: In the normal adult, the body makes natural (or endogenous)
corticosteroids (cortisol is a principle steroid made by the body) in
a cyclical pattern (diurnal or circadian), following a day-night
rhythm. The hypothalamus (located in the brain) and pituitary gland
(also in the brain), and adrenal glands (two glands that sit on top of
each kidney) control the cycle as follows: ACTH (adrenocorticotropin
? a hormone) is lowest at about 10pm and highest at about 6am. High
levels of ACTH in the evening stimulate a rise in the cortisol level
in the blood between 2am and 8am. Rising cortisol causes a feed-back
on ACTH, resulting in decreasing ACTH levels in the blood.
Receiving corticosteroids exogenously (e.g., prednisone), causes a
suppression of ACTH and results in a suppression of cortisol
production by the outer portion of the adrenal glands. After
receiving prednisone for a two week period, your son?s adrenal glands
would have been suppressed, since he was receiving corticosteroids
from the outside and those made by the adrenals were perceived by his
body as ?not needed.? This is a problem when people are suddenly
taken off of prednisone, in that the adrenal glands are not making the
usual amount of corticosteroids and the person becomes more vulnerable
to a stressful situation (stressful here meaning primarily
physiological stresses ? illness, surgery, blood loss, trauma, etc.).
For this reason, patients taking prednisone for more than a very brief
period are usually ?tapered? off the drug, to allow their adrenal
glands to restart. A single dose of prednisone (10mg) can suppress
the adrenal glands for 1.25 to 1.5 days after the drug is
administered. Other corticosteroids can suppress the adrenals for 2-3
days after the drug is taken, so prednisone is considered one of the
?short acting? corticosteroids.
In terms of side-effects associated with a 2 week course of
prednisone, those mentioned above are the primary ones. Neurological
side-effects are quite uncommon, but can include increased
intracranial pressure with papilledema (high pressure in the brain)
usually after treatment, convulsions (seizures), vertigo (sensation of
room spinning), and headache.
From the point of view of infections, taking prednisone can mask
infections and cause impaired response to them, including any viral,
bacterial, protozoan, or helminthic (i.e. worms) infections. This
occurs because cellular immunity, humoral immunity, and neutrophil (a
type of white blood cell that fights infection) function can be
suppressed.
So-called steroid psychosis manifests as psychic derangements that may
appear when corticosteroids are used, ranging from euphoria, insomnia,
mood swings, personality changes, and severe depression, to frank
psychotic manifestations. Also, existing emotional instability or
psychotic tendencies may be aggravated by corticosteroids.
With regard to the next portion of your question ? What about your son
who was also inadvertently taken off of his phenobarbitol due to a
pharmacy error?
Phenobarbitol is a barbiturate that is used as an anti-seizure
medication, as you know.
While prednisone can rarely cause seizures, your son, who has a known
seizure disorder secondary to a streptococcal meningitis /
hydrocephalus as a child and who now have a developmental delay, the
withdrawal of phenobarbitol is a very likely cause for his grand mal
seizure. Also, phenobarbitol increases the rate of metabolism of
prednisone, so initially at least, the prednisone that he was
inadvertently taking was being removed from his system more quickly
than one might expect, until the phenobarbitol level in his blood
dropped.
A temperature of 108 is technically termed hyperpyrexia and can cause
further brain damage by causing death of neurons. You don?t state how
long your son?s seizure lasted, but a prolonged seizure (more than 30
minutes) can indicate a condition known as ?status epilepticus.?
Status epilepticus can also cause brain damage through a mechanism of
hyperexcitability of neurons ? the neurons are so overstimulated that
they die. In an unresponsive person, this can usually only be
diagnosed by performing an EEG (electroencephalogram) to look at the
electrical activity of the brain.
The ?temperature control center? of the brain is the hypothalamus.
Having a severe seizure can cause damage to the hypothalamus and
damage the body?s ability to control it?s temperature, possibly
resulting in your son?s temperature of 108.
Your son?s ulnar deviation is likely a manifestation of this damage to
tissues in the brain. Because the left hand deviates, the damage
likely occurred on the right side of his brain, in the area that is
involved in control of motor functions. Unfortunately, such damage is
rarely reversible, although the brain has remarkable plasticity,
meaning that neurons can sometimes be remodeled to perform new jobs so
that some level of function can be restored. This is often the goal
of physical therapy, occupational therapy, and rehabilitation.
Improvement can take months to years, however.
You also mention that your son was found to have possible Crohn?s,
IBS, Ulcerative Colitis, and/or Clostridium Difficile. Let me try to
sort this out ? IBS (Inflammatory Bowel Disease) is a broad category
of disease of the bowel where there is increased inflammation. The
two main types of IBS are Crohn?s disease and Ulcerative Colitis (UC).
These are both bad diseases. Crohn?s patients can have ?fistulas?
and lesions of the bowel anywhere from the mouth to the anus. The
fistulas appear as improper connections between the bowel and either
other organs or the skin surface ? for example, they can have
?enterocutaneous fistulae,? which would be a connection from the bowel
to the skin out of which stool could be expressed. Another
possibility may be enterocystic fistulae, where a connection forms
between the bowel and the bladder, resulting in repeated bladder
infections. Ulcerative colitis is usually limited to the end portion
of the large bowel. IBS usually results in alternating bouts of
diarrhea and constipation in addition to the above problems, and can
sometimes cause bowel obstructions. Although surgeons delay as long
as possible, patients with ulcerative colitis usually eventually
require a colectomy to remove the diseased portion of the large
intestine. The way to determine which type of disease your son has is
to perform a colonoscopy (placing a long tube with a camera into the
rectum) to directly visualize any lesions and possibly take some small
tissue samples (biopsies) to look at under a microscope. There are
treatment options available for IBS, depending on the type and extent.
Patients with IBS can live full lives (a friend of mine is a medical
student with Crohn?s ? he has earned a Ph.D., and will soon earn his
M.D.), however they have additional challenges.
Clostridium difficile (C. diff) is a microorganism that can infect the
bowel and cause diarrhea. It is most common in hospitalized patients
and those on antibiotics. The stool can be tested for the toxin that
this organism produces. If your son tested negative, this may be why
they are exploring the IBS possibility.
This finding that your son may have IBS of one type or another is not
likely to be related to the errors made in his medications or his
seizure, and was likely found incidentally while he was in the
hospital.
So, to summarize: It appears that your son failed to receive his
Phenobarbital, suffered a seizure. Either the severity of the seizure
(i.e., prolonged muscle contractions) or damage to the hypothalamus
caused hyperpyrexia, which may have caused further damage to the
brain. His ulnar deviation and diminished mental capacity are likely
a result of this brain damage. He may have had further compromise due
to sudden withdrawal of prednisone and resulting adrenal
insufficiency, which can cause a drop in blood pressure. His possible
IBS is likely an incidental finding and will require further workup,
likely by a gastroenterologist (a doctor specializing in the
intestines). His motor and mental abilities will possibly improve
with physical therapy, occupational therapy, and rehabilitation.
Vitamins are not likely to improve his condition, but probably won?t
hurt him either. I would run any vitamins or other alternative
therapies by his primary physician to insure that there are no
interactions between the medications.
Here are some references where you can find more information:
RxList.com has a large database of drug information. I use it in the
hospital all the time. They also list a ?patient information?
section. Here are links for prednisone and phenobarbitol:
http://www.rxlist.com/cgi/generic/pred.htm
http://www.rxlist.com/cgi/generic3/phenbarb.htm
Here is a list of support groups for IBS patients and professional
organizations ? they have a wealth of information on support,
treatment options, diets, etc.:
http://www.ibsgroup.org/
http://www.helpforibs.com/supportgroups/ubbthreads/ubbthreads.php
http://www.ehow.com/how_11374_find-support-irritable.html
http://www.ibsassociation.org/
http://www.iffgd.org/
There are also the following usenet newsgroups:
Alt.support.ibs
Alt.support.crohns-colitis
I hope this answer was helpful. Please feel free to ask for any clarification.
-welte-ga |
Clarification of Answer by
welte-ga
on
29 Nov 2004 18:01 PST
I understand the point made by some medical professionals regarding
steroids allowing bacteria to grow more unchecked than they may
otherwise. This is a problem when people have bacteria in their
blood, for example, and are on steroids such as prednisone, due to
it's immunosuppressive effects. In that situation, the bacteria may
grow more quickly and spread more widely than they might if the body's
immune system were not affected by the corticosteroids.
Regardless of this effect, ulcerative colitis and Crohn's disease are
not caused by an overgrowth of bacteria. Their cause is unknown, but
may be an autoimmune phenomenon. That your son was found to have IBS
at the same time as his seizure is very likely a coincidence - the
disease may have been discovered at a later time if he hadn't had the
seizure. Because he was in the hospital in the ICU, he would have had
very close monitoring by the medical staff, who would have been
looking at every system in his body for any type of disease or
malfunction that could have compromised his well-being. I dont' have
the details of his medical record, but if he had diarrhea in the
hospital and a positive test for blood in his stool, then the next
test would be for c. difficile bacteria. If this were negative, then
and he had a significant blood loss, then a colonoscopy would likely
be performed to make sure he didn't have a bleed from somewhere in his
intestines. During such a colonoscopy, IBS can be diagnosed. I had a
patient in a similar situation just last week, who had no history of
anything like this, but was found to have Crohn's disease when a
colonoscopy was performed.
People with internal hemorrhoids and constipation can certainly also
have IBS (either UC or Crohn's). IBS is often characterized by
intermittent bouts of constipation and diarrhea, and contipation can
lead to or worsen hemorrhoids (both internal and external). None of
these conditions is really related to problems with bile - that is
usually something more like gall bladder disease or, sometimes, liver
disease.
Phenobarbitol causes an induction of liver enzymes that metabolize
prednisone. This means that as long as there was some phenobarbitol
in your son's sytem, the liver enzymes were operating at a faster rate
and would have cleared out the prednisone for the first few days more
quickly than normal. The size of this effect is variable between
people.
The biggest problem here was clearly that your son did not receive his
phenobarbitol, likely resulting in his seizure. Many people take
prednisone for many reasons and do not, to my knowledge, have any
higher incidence of IBS (either UC or Crohn's disease), so this
exposure to prednisone for 2 weeks was unlikely to be the cause of his
disease.
-welte-ga
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