Guillain-Barré Syndrome is something that has, through its affliction of my father, affected my life dramatically. Although it is too difficult to relay the entire "true-life" story of what my family has been through, hopefully you can understand from the descriptions below what this disease does to a person. Imagine that person being someone close to you, and then you can begin to understand what it has been like.
My father is home from the hospital now, after spending over 1 year there. He had varying diagnoses from "you will never regain movement of your lower body and will never progress more than you are now" to "we expect that you can make at least a 75% recovery". I am happy to say that he is walking aided, and is gaining strength day by day.
We are thankful he is home, and that the days on the respirator in I.C.U. are far behind us. Each day is a gift, and something that brings with it a little more strength, hope, and encouragement.
Kathleen
Guillain-Barré (ghee yan-bah ray) (GBS), Chronic Idiopathic Polyneuritis Demyelination (C.I.P.D.), Miller Fisher and other related syndromes are rare and unpredictable syndromes which can cause a person's life to go into utter chaos and turmoil. These syndromes, are disorders that consists of weakness and even paralysis of many of the body's muscles, along with abnormal sensations. The illness can be present in several ways, at times making the diagnosis difficult to establish in its early stages. The specific cause is not known. Research to date indicates that, the nerves of the person who has Guillain-Barré or related syndromes are attacked by the body's defense system against disease (antibodies and white blood cells). As a result of this attack, the nerve insulation (myelin) and sometimes even the covered conducting part of the nerve (axon) is damaged.
This causes delay or change of the nerve "messages", between the sender (usually the brain, cortex or spinal column) and receiver (usually a muscle). The abnormal sensations and weakness quickly follow.
Guillain-Barré (ghee-yan bah-ray) Syndrome is a disorder in
which the body's immune system attacks part of the nervous
system. The first symptoms of this disorder include varying
degrees of weakness or tingling sensations in the legs. In many
instances the weakness and abnormal sensations spread to the arms
and upper body. These symptoms can increase in intensity until
the muscles cannot be used at all and the patient is almost
totally paralyzed. In these cases the disorder is life
threatening -- potentially interfering with blood pressure, heart
rate, and breathing -- and is considered a medical emergency. The
patient is often put on a respirator to assist with breathing and
is watched closely for problems such as an abnormal heart beat,
infections, blood clots, and high or low blood pressure. Most
patients, however, recover from even the most severe cases of
Guillain-Barr??e syndrome, although some continue to have minor
problems. Guillain-Barré Syndrome can affect anybody. It can strike at
any age and both sexes are equally prone to the disorder. The
syndrome is rare, however, afflicting only about one person in
100,000. Usually Guillain-Barré occurs a few days or weeks after
the patient has had symptoms of a respiratory or gastrointestinal
viral infection. Occasionally pregnancy, surgery, or vaccinations
will trigger the syndrome. The disorder can develop over the
course of hours or days, or it may take up to 3 to 4 weeks. Most
people reach the stage of greatest weakness within the first 2
weeks after symptoms appear, and by the third week of the illness
90 percent of all patients are at their weakest. Return to Index No one yet knows why Guillain-Barré strikes some people and
not others. Nor does anyone know exactly what sets the disease in
motion. What scientists do know is that the body's immune system
begins to attack the body itself, causing what is known as an
autoimmune disease. Usually the cells of the immune system attack
only foreign material and invading organisms. In Guillain-Barré
syndrome, however, the immune system starts to destroy the myelin
sheath that surrounds the axons of many nerve cells, or even the
axons themselves (axons are long, thin extensions of the nerve
cells; they carry nerve signals). The myelin sheath surrounding
the axon speeds up the transmission of nerve signals and allows
the transmission of signals over long distances. In diseases in which the nerve cells' myelin sheaths are
injured or degraded, the nerves cannot transmit signals
efficiently. That is why the muscles begin to lose their ability
to respond to the brain's commands, commands that must be carried
through the nerve network. The brain also receives fewer sensory
signals from the rest of the body, resulting in an inability to
feel textures, heat, pain, and other sensations. Alternately, the
brain may receive inappropriate signals that result in tingling,
"crawling-skin," or painful sensations. Because the
signals to and from the arms and legs must travel the longest
distances they are most vulnerable to interruption. Therefore,
muscle weakness and tingling sensations usually first appear in
the hands and feet. When Guillain-Barré is preceded by a viral infection, it is
possible that the virus has changed the nature of cells in the
nervous system so that the immune system treats them as foreign
cells. It is also possible that the virus makes the immune system
itself less discriminating about what cells it attacks.
Scientists are investigating these possibilities and others to
find why the immune system goes awry in Guillain-Barre syndrome
and other autoimmune diseases. The cause and course of
Guillain-Barré syndrome is an active area of neurological
investigation, incorporating the cooperative efforts of
neurological scientists, immunologists, and virologists. Return to Index Guillain-Barré is called a syndrome rather than a disease
because it is not clear that a specific disease-causing agent is
involved. A syndrome is a medical condition characterized by a
collection of symptoms (what the patient feels) and signs (what a
doctor can observe or measure). Because the signs and symptoms of
the syndrome can be quite varied, doctors may find it difficult
to diagnose Guillain-Barré in its earliest stages. Several disorders have symptoms similar to those found in
Guillain-Barré, so doctors examine and question patients
carefully before making a diagnosis. Collectively, the signs and
symptoms form a certain pattern that helps doctors differentiate
Guillain-Barré from other disorders. For example, physicians will
note whether the symptoms appear on both sides of the body (most
common in Guillain-Barré) and the quickness with which the
symptoms appear (in other disorders muscle weakness may progress
over months rather than days or weeks). In Guillain-Barré,
reflexes such as knee jerks are usually lost. Because the signals
traveling along the nerve are slower, a nerve conduction velocity
(NCV) test can give a doctor clues to aid the diagnosis. In
Guillain-Barré patients, the cerebrospinal fluid that bathes the
spinal cord and brain contains more protein than usual. Therefore
a physician may decide to perform a spinal tap, a procedure in
which the doctor inserts a needle into the patient's lower back
to draw cerebrospinal fluid from the spinal column. Laboratory
scientists, working with clinical neurologists, are conducting
research that may help provide physicians with more precise and
reliable diagnostic tests for this disorder. Return to Index There is no known cure for Guillain-Barré syndrome. However,
there are therapies that lessen the severity of the illness in
most patients, and there are a number of ways to treat the
complications of the disease. Currently, plasmapheresis and high-dose immunoglobulin therapy
are used in the more serious cases of Guillain-Barré syndrome.
Plasmapheresis is a method by which whole blood is removed from
the body and processed so that the red and white blood cells are
separated from the plasma, or liquid portion of the blood. The
blood cells are then returned to the patient without the plasma,
which the body quickly replaces. Scientists still don't know
exactly why plasmapheresis works, but the technique seems to
reduce the severity and duration of the Guillain-Barré episode.
This may be because the plasma portion of the blood contains
elements that the immune system needs to function. When these
elements are removed along with the plasma, the immune system is
not able to attack the nervous system as effectively. In high-dose immunoglobulin therapy, doctors give intravenous
injections of the proteins that the immune system uses to attack
invading organisms. Investigators have found that these
immunoglobulins, when given to Guillain-Barré patients, can
lessen the immune attack on the nervous system. Investigators
don't know why this is, but some suggest that the immunoglobulins
may overwhelm the immune system and keep it from attacking the
nerve cells and their myelin sheaths. The use of steroid hormones has also been tried as a way to
reduce the severity of Guillain-Barré, but controlled clinical
trials have not demonstrated that this treatment is effective. Much of the treatment for this syndrome consists of keeping
the patient's body functioning during recovery of the nervous
system. This can sometimes require placing the patient on a
respirator, a heart monitor, or other machines that assist body
function. The need for this sophisticated machinery is one reason
why Guillain-Barre syndrome patients are usually treated in
hospitals, often in an intensive care ward. In the hospital,
doctors can also look for and treat the many problems that can
afflict any paralyzed patient--complications such as pneumonia or
bed sores. Often, even before recovery begins, caregivers may be
instructed to manually move the patient's limbs to help keep the
muscles flexible and strong. Later, as the patient begins to
recover limb control, physical therapy begins. Carefully planned
clinical trials of new and experimental therapies are the key to
improving the treatment of patients with Guillain-Barré syndrome.
Such clinical trials begin with the research of basic and
clinical scientists who, working with clinicians, identify new
approaches to treating patients with the disease. Return to Index Guillain-Barré syndrome can be a devastating disorder because
of its sudden and unexpected onset. In addition, recovery is not
necessarily quick. As noted above, patients usually reach the
point of greatest weakness or paralysis days or weeks after the
first symptoms occur. Symptoms then stabilize at this level for a
period of days, weeks, or, sometimes, months. The recovery period
may be as little as a few weeks or as long as a few years. About
30 percent of those with Guillain-Barré still feel a residual
weakness after 3 years. About 3 to 5 percent may suffer a relapse
of muscle weakness and tingling sensations many years after the
initial attack. Guillain-Barré syndrome patients face not only physical
difficulties, but emotionally painful periods as well. It is
often extremely difficult for patients to adjust to sudden
paralysis and dependence on others for help with routine daily
activities. Patients sometimes need psychological counseling to
help them adapt. Return to Index Scientists are concentrating on finding new treatments and
refining existing ones. Scientists are also looking at the
workings of the immune system to find which cells are responsible
for beginning and carrying out the attack on the nervous system.
The fact that so many cases of Guillain-Barré begin after a viral
infection suggests that certain characteristics of these viruses
may activate the immune system inappropriately. Investigators are
searching for those characteristics. As noted previously,
neurological scientists, immunologists, virologists, and
pharmacologists are all working collaboratively to learn how to
prevent this disorder and to make better therapies available when
it strikes. Return to Index
Taken from Guillain-Barré Syndrome Fact Sheet, National Institutes of Health, Bethesda, Maryland.
What is Guillain-Barré Syndrome?
What causes Guillain-Barré syndrome?
How is Guillain-Barré Syndrome diagnosed?
How is Guillain-Barré treated?
What is the long-term outlook for
those with Guillain-Barré syndrome?
What research is being done?
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