Creutzfeldt-Jakob Disease and Patients
Deborah Schechter
Description
Collection
Title:
Creutzfeldt-Jakob Disease and Patients
Creator:
Deborah Schechter
Text:
You may have patients with Creutzfeldt-Jakob Disease. Creutzfeldt-Jakob
Disease (CJD) has greater public health consequences than the mere number of
reported case might lead one to believe because it is not a reportable disease
in most places, is often misdiagnosed, is infectious and is not killed by
normal sterilization. CJD is a horrendious infectious fatal brain-
deteriorating disease for which there is no treatment or cure. It is caused
by a prion, which is a protein. One strain of CJD (nvCJD, i.e. new variant
CJD) is linked to bovine spongiform encephalopathy (i.e. Mad Cow Disease) in
England. CJD is more common than reported. In one study of Alzheimer
patients 13% when autopsied were found to really have CJD.
In the United States and throughout the world people get CJD through 3 means:
familial (genetic), sporadic (don't know how) and iatrogenic (through a
medical procedure such as human pitutitary growth hormones and human
gonadotrophin fertility treatments received in 1985 and before, contaminated
surgical equipment and dura mater and cornea transplants.) CJD can take
decades after exposure for the patient to show symptoms. However, once a
person shows symptoms their decline is rapid and they die within a year of
first showing symptoms.
CJD should be considered whenever a patient develops a rapid dementia and
myoclonus. The initial symptoms are subtle and ambiguous and include insomnia,
depression, confusion, personality and behavioral changes, strange physical
sensations, and memory, coordination and visual problems. Rapidly
progressive dementia and usually myoclonus (involuntary, irregular jerking
movements) develop as CJD progresses. Also, language, sight, muscular
weakness, and coordination problems worsen. The patient may appear startled
and become rigid. In the final stage the patient loses all mental and physical
functions. The patient may lapse into a coma and usually dies from an
infection like pneumonia precipitated by the bedridden, unconscious state. The
duration of CJD from the onset of symptoms to death is usually one year of
less. A 14-3-3 spinal fluid test is over 95% effective in diagnosing CJD when
symptoms are present. (For information on the test contact Dr Clarence
Joseph Gibbs, Jr.; National Institutes of Health, (30l) 496-4821 or 6321.)
CJD patients often die at home and therefore have home health service and have
family members as caregivers.
Since early signs of CJD are often psychological, victims will often receive
psychological treatment as either an in-patient or an outpatient. In
addition, people, including school-age children of Creutzfeldt-Jakob Disease
victims who die in their 40s or younger, often require counseling to deal with
the death. And, then there's the recipients of medical treatments such as
human pituitary growth hormones and dura mater transplants who are at high
risk of CJD and the people with a genetic mutation for familial CJD who must
live with CJD hangiing over their heads. Another group includes people who
receive withdrawal notices that the blood they or, worst yet, their children,
received came from a pool which included a donor that died of CJD. While the
question of whether CJD is passed through blood products is yet to be
resolved, receiving this type of notice causes great
anxiety in people.
Also, since normal sterilization methods do no kill the CJD infectious agent
and, it can therefore be spread by surgical instruments, it is more of a
danger to public health than mere number of cases would suggest..
Whether CJD is spread by human blood is controversial,. Many people get
transfusions every year. Pooled blood products are withdrawn as a precaution
if it is found after the product has been released that a person in the donor
pool has died of CJD of is at higher risk of contracting CJD. However, by
this point it has often already been used in humans. Many people such as
hemophilliacs and Alpha !-Antitrypsin Deficency sufferers use blood products
constantly to maintain their health and therefore, receive many withdrawal
notices. In additon, many people get gammaglobulin shots. Also, while the
controversy remains as to whether CJD can be passed through blood products,
blood products continue to be used as an ingredient in vaccines such as the
measles-mumps-rubella, rabies and allergy shots; in InVitro Fertilization
(IVF) cultures; and in medical test fluids.
Even though people have recieved notices that the blood products they were
given have been withdrawn due to CJD risk they can still donate blood. Also,
England recently decided to not use its own people to get blood plasma any
more and to get blood plasma from other countries due to fears of nvCJD (the
CJD related to Mad Cow Disease) being transmitted through blood. Yet people
from England can donate blood in the United States and Canada. At the end of
this e-mail I have included the U. S. Congressional Mandate requesting the
Centers for Disease Control to conduct a study to assess whether the CJD
infectious agent is spread through blood products.
CJD Voice is an e-mail discussion group. Most members have lost a loved one
to CJD or currently have a loved one with CJD. Other members include people
who took human pituitary growth hormones as children and therefore are at
higher risk of getting CJD and people who received notices that the blood
products their children received were from a pool that included a person who
died from CJD. It provides support to these people as well as tries to
increase funding for CJD research so treatments and a cure can be found. The
CJD webpage has a message board, chat room, links to other websites with CJD
information and a list of CJD researchers accepting financial contributions.
The address for the CJD Voice Webpage is
<URL Redacted>
Please feel free to visit the website and to refer other people to it.
Feel free to distribute this information to whomever you choose.
<Email Address Redacted>
The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob
Disease Program
A Congressional Mandate
In response to concerns that CJD may be transmitted through blood or
blood products, the U.S. Congress requested that the
CDC conduct a study to assess whether CJD is a threat to the safety of
the nation's blood supply. Researchers believe that
the risk of transmission to humans through blood products is very
small since there have been no known cases of CJD
contracted by humans in this manner. However, precautions are being
taken to assure that the blood supply is safe from this
infectious agent. Current blood safety policy requires that any blood
products made from blood donated by a person who
later develops CJD, or is found to have risk factors for CJD, must be
withdrawn. Until the question of transmissibility is
resolved, the availability and the price of blood products will
continue to be adversely impacted by shortages caused by
recalls and the destruction of blood products which, to date, has cost
over $100 million.
The Program: How You Can Help
Because the signs and symptoms of CJD may not develop for up to 30
years, a person could be infected and not show any
symptoms during his or her lifetime. Furthermore, there is no
screening test available for CJD and the only sure way to test
for CJD is by analyzing brain tissue after death. The CJD program asks
families of individuals who have received blood
products to donate brain tissue after their death. The brain tissue
will be shipped to Stephen J. Armond, M.D., Ph.D.,
Professor of Neuropathology, University of California, San Francisco
to test for evidence of CJD. Other brain tissue will be
stored at CDC in Atlanta for analysis in the future when more is known
about the cause of CJD.
Participation in the CJD program is voluntary. The CDC is aware that
the death of a family member is a difficult time to make
important decisions. Therefore, the CDC is working through your
regional coordinator, physician, and treatment center staff to
provide support to you and your family as you discuss brain tissue
donation. To help with making an informed and rational
decision, the CDC has developed information packets which answer
commonly asked questions about the program including:
how to authorize the donation; how confidentiality will be assured;
the ability to retrieve the brain tissue without disfigurement
and without affecting funeral arrangements; and the time frame for
receiving test results.
Disease (CJD) has greater public health consequences than the mere number of
reported case might lead one to believe because it is not a reportable disease
in most places, is often misdiagnosed, is infectious and is not killed by
normal sterilization. CJD is a horrendious infectious fatal brain-
deteriorating disease for which there is no treatment or cure. It is caused
by a prion, which is a protein. One strain of CJD (nvCJD, i.e. new variant
CJD) is linked to bovine spongiform encephalopathy (i.e. Mad Cow Disease) in
England. CJD is more common than reported. In one study of Alzheimer
patients 13% when autopsied were found to really have CJD.
In the United States and throughout the world people get CJD through 3 means:
familial (genetic), sporadic (don't know how) and iatrogenic (through a
medical procedure such as human pitutitary growth hormones and human
gonadotrophin fertility treatments received in 1985 and before, contaminated
surgical equipment and dura mater and cornea transplants.) CJD can take
decades after exposure for the patient to show symptoms. However, once a
person shows symptoms their decline is rapid and they die within a year of
first showing symptoms.
CJD should be considered whenever a patient develops a rapid dementia and
myoclonus. The initial symptoms are subtle and ambiguous and include insomnia,
depression, confusion, personality and behavioral changes, strange physical
sensations, and memory, coordination and visual problems. Rapidly
progressive dementia and usually myoclonus (involuntary, irregular jerking
movements) develop as CJD progresses. Also, language, sight, muscular
weakness, and coordination problems worsen. The patient may appear startled
and become rigid. In the final stage the patient loses all mental and physical
functions. The patient may lapse into a coma and usually dies from an
infection like pneumonia precipitated by the bedridden, unconscious state. The
duration of CJD from the onset of symptoms to death is usually one year of
less. A 14-3-3 spinal fluid test is over 95% effective in diagnosing CJD when
symptoms are present. (For information on the test contact Dr Clarence
Joseph Gibbs, Jr.; National Institutes of Health, (30l) 496-4821 or 6321.)
CJD patients often die at home and therefore have home health service and have
family members as caregivers.
Since early signs of CJD are often psychological, victims will often receive
psychological treatment as either an in-patient or an outpatient. In
addition, people, including school-age children of Creutzfeldt-Jakob Disease
victims who die in their 40s or younger, often require counseling to deal with
the death. And, then there's the recipients of medical treatments such as
human pituitary growth hormones and dura mater transplants who are at high
risk of CJD and the people with a genetic mutation for familial CJD who must
live with CJD hangiing over their heads. Another group includes people who
receive withdrawal notices that the blood they or, worst yet, their children,
received came from a pool which included a donor that died of CJD. While the
question of whether CJD is passed through blood products is yet to be
resolved, receiving this type of notice causes great
anxiety in people.
Also, since normal sterilization methods do no kill the CJD infectious agent
and, it can therefore be spread by surgical instruments, it is more of a
danger to public health than mere number of cases would suggest..
Whether CJD is spread by human blood is controversial,. Many people get
transfusions every year. Pooled blood products are withdrawn as a precaution
if it is found after the product has been released that a person in the donor
pool has died of CJD of is at higher risk of contracting CJD. However, by
this point it has often already been used in humans. Many people such as
hemophilliacs and Alpha !-Antitrypsin Deficency sufferers use blood products
constantly to maintain their health and therefore, receive many withdrawal
notices. In additon, many people get gammaglobulin shots. Also, while the
controversy remains as to whether CJD can be passed through blood products,
blood products continue to be used as an ingredient in vaccines such as the
measles-mumps-rubella, rabies and allergy shots; in InVitro Fertilization
(IVF) cultures; and in medical test fluids.
Even though people have recieved notices that the blood products they were
given have been withdrawn due to CJD risk they can still donate blood. Also,
England recently decided to not use its own people to get blood plasma any
more and to get blood plasma from other countries due to fears of nvCJD (the
CJD related to Mad Cow Disease) being transmitted through blood. Yet people
from England can donate blood in the United States and Canada. At the end of
this e-mail I have included the U. S. Congressional Mandate requesting the
Centers for Disease Control to conduct a study to assess whether the CJD
infectious agent is spread through blood products.
CJD Voice is an e-mail discussion group. Most members have lost a loved one
to CJD or currently have a loved one with CJD. Other members include people
who took human pituitary growth hormones as children and therefore are at
higher risk of getting CJD and people who received notices that the blood
products their children received were from a pool that included a person who
died from CJD. It provides support to these people as well as tries to
increase funding for CJD research so treatments and a cure can be found. The
CJD webpage has a message board, chat room, links to other websites with CJD
information and a list of CJD researchers accepting financial contributions.
The address for the CJD Voice Webpage is
<URL Redacted>
Please feel free to visit the website and to refer other people to it.
Feel free to distribute this information to whomever you choose.
<Email Address Redacted>
The Centers for Disease Control and Prevention (CDC) Creutzfeldt-Jakob
Disease Program
A Congressional Mandate
In response to concerns that CJD may be transmitted through blood or
blood products, the U.S. Congress requested that the
CDC conduct a study to assess whether CJD is a threat to the safety of
the nation's blood supply. Researchers believe that
the risk of transmission to humans through blood products is very
small since there have been no known cases of CJD
contracted by humans in this manner. However, precautions are being
taken to assure that the blood supply is safe from this
infectious agent. Current blood safety policy requires that any blood
products made from blood donated by a person who
later develops CJD, or is found to have risk factors for CJD, must be
withdrawn. Until the question of transmissibility is
resolved, the availability and the price of blood products will
continue to be adversely impacted by shortages caused by
recalls and the destruction of blood products which, to date, has cost
over $100 million.
The Program: How You Can Help
Because the signs and symptoms of CJD may not develop for up to 30
years, a person could be infected and not show any
symptoms during his or her lifetime. Furthermore, there is no
screening test available for CJD and the only sure way to test
for CJD is by analyzing brain tissue after death. The CJD program asks
families of individuals who have received blood
products to donate brain tissue after their death. The brain tissue
will be shipped to Stephen J. Armond, M.D., Ph.D.,
Professor of Neuropathology, University of California, San Francisco
to test for evidence of CJD. Other brain tissue will be
stored at CDC in Atlanta for analysis in the future when more is known
about the cause of CJD.
Participation in the CJD program is voluntary. The CDC is aware that
the death of a family member is a difficult time to make
important decisions. Therefore, the CDC is working through your
regional coordinator, physician, and treatment center staff to
provide support to you and your family as you discuss brain tissue
donation. To help with making an informed and rational
decision, the CDC has developed information packets which answer
commonly asked questions about the program including:
how to authorize the donation; how confidentiality will be assured;
the ability to retrieve the brain tissue without disfigurement
and without affecting funeral arrangements; and the time frame for
receiving test results.
Citation
Deborah Schechter, “Creutzfeldt-Jakob Disease and Patients,” Digital Resource Foundation for Orthotics and Prosthetics, accessed November 5, 2024, https://library.drfop.org/items/show/210605.
