A female client with Guillain-Barré syndrome has paralysis affecting the respiratory muscles and requires mechanical ventilation. When the client asks the nurse about the paralysis, how should the nurse respond?
a. “You may have difficulty believing this, but the paralysis caused by this disease is temporary.”
b. “You’ll have to accept the fact that you’re permanently paralyzed. However, you won’t have any sensory loss.”
c. “It must be hard to accept the permanency of your paralysis.”
d. “You’ll first regain use of your legs and then your arms.”
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What is Guillain-Barré syndrome?
Guillain-Barré syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disease include varying degrees of weakness or tingling sensations in the legs. In many cases, weakness and abnormal sensations spread to the arms and torso. These symptoms may increase in intensity until the muscles cannot be used at all and the patient is almost completely paralyzed. In these cases, the disorder is life-threatening - potentially interfering with breathing and sometimes with blood pressure and heart rate - and is considered a medical emergency. The patient is often placed in a respirator to help him breathe and is closely watched for the appearance of problems such as abnormal heart rhythm, infections, blood clots and high or low blood pressure. Most patients recover, including the most severe cases of Guillain-Barré Syndrome, although some continue to have a certain degree of weakness.
Guillain-Barré syndrome can affect anyone. It can attack the person at any age and both sexes are equally prone to the disorder. The syndrome is rare and afflicts only one person in every 100,000. Generally, Guillain-Barré Syndrome occurs a few days or a week after the patient has had symptoms of a respiratory or gastrointestinal viral infection. Occasionally, surgery or a vaccine can trigger the syndrome. The disorder may appear within several hours or several days or may require up to 3 or 4 weeks. Most people reach the stage of greatest weakness within the first 2 weeks of the onset of symptoms and, by the third week of the disease, 90 percent of the patients are at their point of greatest weakness.
What causes Guillain-Barré Syndrome?
No one knows yet why Guillain-Barré Syndrome attacks some people and not others. Nor does anyone know what exactly triggers the disease.
What scientists do know is that the body's immune system begins to attack the body itself, which is known as an autoimmune disease. Commonly, immune system cells attack only foreign material and invading organisms. In Guillain-Barré Syndrome, however, the immune system begins to destroy the myelin sheath that surrounds the axons of many peripheral nerves, or even the axons themselves (axons are thin and long extensions of nerve cells that transmit the nerve signals). The myelin sheath that surrounds the axon accelerates the transmission of nerve signals and allows the transmission of signals over long distances.
In diseases in which the myelin coatings of the peripheral nerves are injured or affected, the nerves cannot transmit signals efficiently. This is why the muscles begin to lose their ability to respond to the mandates of the brain, mandates that must be transported through the nervous network. The brain also receives less sensory signals from the rest of the body, resulting in an inability to feel textures, heat, pain and other sensations. Alternatively, the brain may receive inappropriate signals that result in skin tingling or painful sensations. Because the signals that go to and from the arms and legs have to travel long distances, they are the most vulnerable to interruption. Therefore, muscle weaknesses and tingling sensations initially appear on the hands and feet and progress upwards.
When Guillain-Barré Syndrome is preceded by a viral infection, it is possible that the virus has changed the nature of the cells in the nervous system so the immune system treats them as foreign cells. It is also possible that the virus makes the immune system itself less discriminating about which cells it recognizes as its own, allowing some of the immune cells, such as certain kinds of lymphocytes, to attack myelin. Scientists are investigating these and other possibilities to determine why the immune system malfunctions or is disturbed in Guillain-Barré Syndrome and other immune diseases. The cause and trajectory of Guillain-Barré Syndrome is an active area of neurological research and incorporates the collaborative efforts of neurological, immunological and virologist scientists.
How is Guillain-Barré syndrome diagnosed?
Guillain-Barré syndrome is called a syndrome rather than a disease because it is not clear that a specific pathogen comes into play. A syndrome is a medical condition characterized by a cluster of symptoms (what the patient feels) and signs (what the doctor can observe or measure). The signs and symptoms of the syndrome can be quite varied, so doctors can, rarely, find it difficult to diagnose Guillain-Barré Syndrome in its early stages.
Several disorders have symptoms similar to those found in Guillain-Barré Syndrome, so doctors carefully examine and question patients before making a diagnosis. Collectively, the signs and symptoms form a certain pattern that helps doctors differentiate Guillain-Barré Syndrome from other disorders. For example, doctors will see if symptoms appear on both sides of the body (most common in Guillain-Barré syndrome) and the speed with which symptoms appear (in other disorders, muscle weakness can progress through months instead of days or weeks). In Guillain-Barré the reflexes, such as the reaction of the knee when hitting it, usually disappear. Because the signals that travel through the nerve are slower, a nerve conduction velocity test (NCV) can help the doctor in the diagnosis. In patients of Guillain-Barré syndrome, the cerebrospinal fluid that bathes the
spinal cord and the brain contains more protein than normal. Therefore, a doctor may decide to do a lumbar puncture, a procedure in which the doctor inserts a needle into the lower back of the patient to remove cerebrospinal fluid from the spinal column.
How is Guillain-Barré syndrome treated?
There is no known cure for Guillain-Barré syndrome. However, there are therapies that reduce the severity of the disease and accelerate recovery in most patients. There are also a number of ways to treat the complications of the disease.
Generally, plasmapheresis and high-dose immunoglobulin therapy are the remedies used. Both are equally effective, but immunoglobulin is easier to administer. Plasmapheresis is a method by which whole blood is drawn from the body and processed so that the white and red blood cells are separated from the plasma or the liquid portion of the blood. The blood cells are then returned to the patient without the plasma, which the body quickly replaces. Scientists do not yet 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 of the immune system and can be toxic to myelin.
In high-dose immunoglobulin therapy, doctors administer intravenous injections of protein that, in small amounts, the immune system naturally uses to attack invading organisms. Researchers have discovered that the administration of high doses of these immunoglobulins, derived from a set of thousands of normal donors, to Guillain-Barré patients can reduce the immune attack on the nervous system. Researchers do not know why or how this works, although several hypotheses have been proposed.
The use of steroid hormones has also been tested as a way to reduce the severity of Guillain-Barré, but controlled clinical studies have shown that this treatment is not only not effective, but may even have a detrimental effect on the disease.
The most critical part of the treatment of this syndrome is to keep the patient's body functioning during recovery of the nervous system. This may sometimes require placing the patient on a respirator, a heart rate monitor or other machines that help body function. The need for this complex machinery is one of the reasons why Guillain-Barré Syndrome patients are usually treated in hospitals, often in the intensive care ward. In the hospital, doctors can also try to detect and treat many problems that can arise in any paralyzed patient - complications such as pneumonia or injuries caused by prolonged bed prostration.
Often, even before recovery begins, people who care for these patients are instructed to manually move the limbs of patients to help keep the muscles flexible and strong. Subsequently, as the patient begins to regain control of the limbs, 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. These clinical trials begin with basic and clinical research, during which scientists work in collaboration with clinical professionals, identifying new approaches to treat patients with this condition.
What is the long-term perspective for those who have Guillain-Barré Syndrome?
Guillain-Barré Syndrome can be a devastating medical condition due to the rapidity and the unexpected appearance. In addition, recovery is not necessarily fast. As noted earlier, 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 can be as short as a few weeks or as long as a few years. Approximately 30 percent of those with Guillain-Barré still suffer a residual weakness after 3 years of illness. 3 percent may suffer a relapse of muscle weakness or tingling sensations many years after the initial attack.
Patients who develop Guillain-Barré Syndrome face not only physical difficulties but also emotionally painful periods. Often, it is 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 to the limitations of this condition.
What research is being done?
Scientists are concentrating on seeking new treatments and perfecting existing treatments. Scientists also examine the functioning of the immune system to determine which cells are responsible for initiating and undertaking the attack against the nervous system. The fact that so many cases of Guillain-Barré begin after a viral infection indicates that certain characteristics of these viruses may activate the immune system inappropriately. Researchers are examining those characteristics. As indicated above, neurological scientists, immunologists, virologists and pharmacologists are all working collaboratively to learn how to prevent this disorder and have better therapies available when it occurs.
