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Guillain-Barré syndrome

Guillain-Barré syndrome (GBS), is an acquired immune-mediated inflammatory disorder of the peripheral nervous system (i.e. not the brain or spinal cord). It is also called acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio and Landry's ascending paralysis. more...

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Guillain-Barré syndrome

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Overview

The pathologic hallmark of the disease is loss of myelin in peripheral nerves due to an acute and progressive inflammation of unknown cause. It is suggested that it is an autoimmune disease, in which the sufferer's immune system is triggered into damaging the nerve covering. There is some support for this in that half of all cases occur soon after a microbial infection or respiratory or gastrointestinal viral infection. Many cases developed in people who received the 1976 swine flu vaccine.

Peripheral nerves originate in the spinal cord and proceed to their target tissues (mainly muscle, skin and all internal organs). Their most proximal parts emerging from the spinal cord are called nerve roots and the inflammation in most (but not all) typical Guillain-Barré syndrome cases starts in these roots. Therefore, this condition is also referred to as acute polyradiculoneuritis.

Recent studies on the disease have demonstrated that approximately 80% of the patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed axon loss. The cases indicating the demyelinating form (AIDP) are called "acute motor and sensory axonal neuropathy" (AMSAN); the cases showing only motor symptoms (diffuse weakness) are called "acute motor axonal neuropathy" (AMAN). In a different and infrequent variant called Miller Fisher syndrome, patients develop ataxia, loss of tendon reflexes, and difficulty moving eye muscles but not weakness or sensory loss. All variants of Guillain-Barré syndrome are now supposed to be an autoimmune disease caused by antibodies against a variety of gangliosides found in abundant amounts in the peripheral nerve tissue.

Prevalence

GBS is a rare disease affecting about 1 to 2 people in every 100,000 annually. It does not discriminate with regard to the age or sex of sufferers. When diagnosed in young teenagers, it generally does not recur for many years, although when it does, it often does so in the fourth or fifth decade of life, long after the patients may have forgotten the details of the original episode.

Cause

About one half of patients have a history of preceding viral infection within two to four weeks prior to exhibiting the onset of Guillain-Barré syndrome. Guillain-Barré syndrome may also be associated with immunizations, recent surgery or trauma, pregnancy, Hodgkin's disease, chemo-therapy, and connective tissue diseases. The most frequently associated viral agents are cytomegalovirus (CMV), HIV, measles and herpes simplex virus. A bacterium called Campylobacter jejuni has recently been shown to be closely related with certain subtypes of the disease.

Read more at Wikipedia.org

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Nosing into flu season
From Nursing, 9/1/04

HEALTHY PEOPLE between ages 5 and 49 now have the option to receive annual flu vaccinations intranasally. Prepackaged in unit-dose sprayers for intranasal administration, the live attenuated influenza vaccine (LAIV) is formulated each year to protect against the same virus strains as the inactivated vaccine that's administered by intramuscular injection.

Follow the manufacturer's guidelines for freezing and defrosting the vaccine to maintain vaccine potency. Before administering LAIV take a thorough patient history, and don't administer it in these situations:

* patient with known or suspected immunodeficiency disease

* current aspirin or aspirin-containing therapy in children or adolescents, which would increase the risk of Reye's syndrome

* patient history of Guillain-Barré syndrome (warning applies to the inactivated vaccine too)

* patient history of hypersensitivity to any components of LAIV or to eggs (warning also applies to the inactivated vaccine)

* patient history of reactive airway disease or chronic cardiopulmonary disease

* anyone with a chronic medical condition such as diabetes, renal dysfunction, or hemoglobinopathy

* a pregnant woman

* anyone in close contact with a severely immunosuppressed person, such as a bone marrow transplant recipient, while that person is in a protective environment.

Administering LAIV

Have your patient sit upright. Explain the potential risks and benefits of receiving the vaccine, potential adverse reactions, and what to do if they should occur. Have epinephrine injection (1:1,000) or a comparable treatment handy to treat a potential acute anaphylactic reaction.

Learn how to administer influenza vaccine intranasally in a few steps.

BY THE EDITORS OF NURSING2004

1. Thaw the vaccine by holding the sprayer in the palm of your hand, as shown, with your thumb supporting but not depressing the plunger. Don't roll the sprayer between your palms, which could dislodge the dose divider clip. Thawing may take up to 3 minutes.

Another option is to thaw the vaccine in the refrigerator at 36° F to 46° F (2° C to 8° C) for up to 24 hours.

2. Make sure the divider clip is securely attached to the sprayer plunger. This separates the dose into two portions, one for each nostril.

3. Remove the protector from the sprayer tip. Have your patient tilt her head backward and advise her to breathe normally. Place the sprayer tip just inside her nostril and depress the plunger to deliver the first dose, as shown.

4. Pinch and remove the divider clip from the plunger. Place the sprayer tip just inside her other nostril and depress the plunger to deliver the remaining vaccine. Follow standard procedures for biohazardous waste to dispose of the sprayer.

Teach your patient the potential adverse reactions, what to do if they occur, and what medications to avoid. Document the date, time, and route of administration; the drug name, dose, lot number, and expiration date; and any teaching you provided.

SELECTED REFERENCE

Supplemental Recommendations of the Advisory Committee on Immunization Practices (ACIP): "Using Live, Attenuated Influenza Vaccine for Prevention and Control of Influenza," MMWR, 52(RR13):1-8. September 26 2003.

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