The IVIG shortage and CJD: Putting patients at ease
The IVIG shortage and CJD: Putting patients at ease
By Nina Elledge CRNI
President, Access Professionals
Castro Valley, CA
The nationwide shortage of plasma-derived intravenous immune globulin (IVIG) recently has received attention in the news media, on the Internet, and in professional magazines. Most of us who work in the hospital or home care have noticed a problem procuring the drug for patients who, along with their families, are increasingly asking us to address their questions regarding Creutzfeldt-Jakob disease (CJD) and the nation's blood supply as well as manufactured components. The following brief summary of the causes of the IVIG shortage and the pathophysiology of CJD should prove helpful in answering these questions.
The Department of Health and Human Services has asked the six companies that currently make IVIG in the United States to address the causes for the drug's shortage. Here are some facts about IVIG and its U.S. availability:
· It takes 200 days to create IVIG from plasma.
· By 1997, 7% of the nation's supply of IVIG had been recalled or withdrawn, and four companies manufacturing the drug had increased their exports. It remains unclear whether this exporting practice was fueled by increased profit margins overseas.
· When a manufacturer of another plasma product drug was found to be in significant violation of manufacturing standards (the facility had never been inspected in its 50 years of operation), the government's inspection process and scrutiny lead to new procedure requirements that slowed IVIG production.
· Doctors are prescribing IVIG in increasing quantities to treat many diseases, but there are inadequate studies showing the benefit of IVIG for outcomes for some of these diseases.
· Many more disease states or conditions have been identified as treatable by IVIG therapy. Technological advancements fuel the need to treat these conditions. Many more patients are living longer with chronic conditions, and IVIG is a life-saving therapy for them.
· There is concern that CJD, an incurable disease, can come from plasma or plasma-derived drugs. In 1995, the FDA recommended withdrawal of products containing plasma from donors who were subsequently diagnosed with CJD.
· CJD was first identified in 1921, and since then there have been no proven cases of CJD transmission via plasma or plasma-derived pharmaceutical products.
Creutzfeldt-Jakob disease is primarily identified in individuals 50-75 years of age and occurs in about one out of one million people each year. A definitive diagnosis can only be made on autopsy, and there is no known cure.
It can take up to 30 years before the onset of symptoms, which makes linking them to the disease extremely difficult. Signs and symptoms of the disease include:
· insomnia, depression, confusion, and personality and behavioral changes;
· later, a rapid progression into dementia;
· myoclonus;
· loss of all physical and mental functions with lapse into coma and death, usually of opportunistic infections.
Some 90% of cases occur spontaneously, and are thought to be related to direct contact with infected neural tissue, inoculation with contaminated human growth hormone, corneal transplants, or contact with contaminated surgical instruments. Documented cases of CJD transmission during medical procedures have always involved direct exposure to or contact with body tissue.
CJD is not caused by a virus or another known infectious agent, but rather by an unconventional agent consisting of protein called a "prion." Because of this, it is not considered contagious in the traditional sense.
It is theorized that nearly 10% of the population inherits CJD through a mutated gene coding for the prion protein. These prions are believed to be responsible for other fatal brain diseases in humans and animals, including kuru, Gerstmann-Straussler-Scheinker disease, feline spongiform encephalopathy, scrapie, and bovine spongiform encephalopathy (BSE), or "mad cow disease."
Mad cow disease was identified in 1986 in Britain, and was thought to have resulted from feeding cattle with contaminated ruminant products. In 1996, the British government concluded that there may be a link between CJD and BSE, probably caused by the consumption of contaminated beef prior to 1989. BSE has not been detected in the United States since the institution of a surveillance system in 1990. No British cattle or ruminant-based feed have been imported into the United States since 1989. There is no direct evidence that BSE can be spread to humans.
Experts agree that the risk of getting CJD from a plasma-derived medication or the nation's blood supply is extremely low, and there has never been a proven case of CJD transmission to a recipient of blood products or plasma-derived medication. Infection from blood transfusion remains theoretical.
Also, synthetic production of human growth hormone began in 1985, which has reduced risk factors in this population. As we know, potential donors of blood and blood products are screened for many risk factors (including those mentioned above), and the donation of blood in this country is also voluntary, which further decreases risk of disease transmission. The Red Cross has in the past recalled blood products and medications containing plasma of a donor known to have been diagnosed with CJD because of FDA recommendations. The FDA was acting on fears of a repeat scenario of previous years in which hemophiliacs became infected with HIV after being treated with contaminated factor.
All of the above factors affect the availability of IVIG in the United States. Recent press has fueled the surrounding controversy. Will the problem be resolved soon? The answer lies with the manufacturers themselves, and the answer is a difficult one: They are acting on many different influences from many different sources in their decision to make this drug available.
How do we get this drug for our patients? By working closely with the patient's prescribing doctor to develop documentation of necessity and benefits, the physicians can then take this information to the manufacturers to get the patient's needs met.
The IVIG shortage also necessitates a close look at these infusions and answering the ethical question of life-sustaining need vs. research and unproven use. The issues are difficult and time-consuming. For nurses, the bottom line is to provide proactive nursing and patient advocacy to help patients get their medical needs for IVIG met.
(Editor's note: Information for the above article was compiled from the following sources: CJD Voice & Webring at http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm, and the American Association of Blood Banks at http://www.aabb.org.)
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