Living
with GT
Learn to be safe and LIVE WELL
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Please
send in your stories and/or pictures of you living
your life to the fullest so that we can update your
story on our 'Stories'
page. My email address is Helen@Glanzmanns.Com.
If you don't already have a story on the page, I
would very much like to include you in this important
section of the website. The 'Stories'
page is one of the first places anyone new to this
site will visit and it's important, especially for
new GT parents to read of our successes in addition
to some of our more challenging times that are unique
to GT! |
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Thank
you for making this GT community a truly rare community
in more ways than the obvious one!
Helen
Smith
Glanzmann's Research Foundation
www.CureGT.com
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Dinner for a Cure Casino
Night
Sacred Heart, Cultural Arts Center
March 10, 2007 6:00 - 11:00
Was
a wonderful success!
David,
Gretchen, Tito, Randy, Jyll, Aidana, Chris, Julia, Emily, Dan,
KayTee, Bettie, Steve
To
our sponsors, our contributors and our volunteers, and to everyone
who was involved,
a warm and heart-felt thank you. We truly could not have done
it without you.
Pictures of the Friday night dinner
are here
And pictures of the Casino Night are
here
The
GT website has created the opportunity for people with GT from
all over the world to not only get vital information from other
GT patients but to actually FIND someone else with GT. The website
has inspired people to travel thousands of miles to Augusta just
so they can meet
other GT patients in person for the first time in their lives.
They also come to Augusta to meet the man who brings them hope.
Dr. David Wilcox
attends our event annually to keep us abreast on his research
progress. He demonstrates his accomplishments in the lab and reports
how he has applied our previous donations, and explains how he
plans to utilize future contributions in his pursuit of finding
a cure for Glanzmann's Thrombasthenia. Dr. Wilcox also enjoys
the opportunity to meet the very people his hard work and dedication
will affect.
An
Article from the Journal of Thrombosis
and Haemostasis
Prophylactic and therapeutic recombinant
factor VIIa
administration to patients with Glanzmann’s thrombasthenia:
results of an international survey
Antibodies
to glycoprotein (GP) IIb-IIIa and/or HLA may render platelet transfusions
ineffective to stop bleeding or to cover surgery in patients with
Glanzmann’s thrombasthenia (GT). Anecdotal reports suggest
recombinant factor (rF)VIIa might be a therapeutic alternative
in these situations. Objectives: An international survey was conducted
to evaluate further the efficacy and safety of rFVIIa in GT patients.
Patients: We analyzed the use of rFVIIa during 34 surgical/invasive
procedures and 108 bleeding episodes in 59 GT patients including
29 with current or previous antiplatelet antibodies, and 23 with
a history of refractoriness to platelet transfusion. (read
more...)
New
Products to Help
Stop Bleeding Quickly
By LAURA JOHANNES
July 3, 2007; Page D6
New advances in technologies that stem bleeding have been saving
lives in emergency rooms and the war in Iraq. Now similar advances
are being used in consumer products that treat bleeding, including
powders, bandages and nose plugs. The makers of these products
say they help stanch bleeding from cuts, nosebleeds and more-serious
wounds. Doctors say many of the products promote clotting, but
caution that deep cuts, or those where tendons are damaged, still
require medical attention. read
more...
Mom
starts nonprofit to fight disease
By Charmain Z. Brackett
Correspondent
Helen Smith
never thought she'd champion a cause until her daughter, Julia,
was born.
Julia, 7, has a rare blood disorder called Glanzmann's thrombasthenia.
Mrs. Smith, through the Glanzmann's Research Foundation, a nonprofit
organization she founded, helps raise money for research to find
a cure.
 |
"The
year she was born, there were only 200 reported cases"
of the disease, said Smith, whose organization will be the
beneficiary of a fundraising dinner at the National Science
Center's Fort Discovery in March. The event will feature
a silent auction with items such as a celebrity hunting
weekend.
Julia
was diagnosed with the disease when she was about 6 months
old. |
There were
some complications when Julia was born, and she spent the first
two weeks of her life in intensive care. more
Daily tomato juice eases diabetic symptoms
20/08/2004
- Drinking tomato juice everday reduced the blood’s
clotting ability in people with type 2 diabetes, report
Australian researchers this week, reports Dominique Patton.
They found
significant lowering of platelet aggregation
– the blood's ability to clot – after a daily dose
of juice for three weeks, according to the research letter in
this week's JAMA (Aug 18;292(7):805-6). (read
more)
The effectiveness of web-based translators varies from source to
source. A list of some of the more popular
language translators can be found here. For an example
of this page translated into Spanish, click
here.
Peripartum
management of a patient with Glanzmann’s thrombasthenia using
Thrombelastograph®
S.
Monte* and G. Lyons
Department of Obstetric Anaesthesia, St James’ University
Hospital, Beckett Street, Leeds LS9 7TF, UK*Corresponding author
Accepted for publication: January 7, 2002
We describe
the perioperative management of a 31-yr-old primipara with Glanzmann’s
thrombasthenia, who required urgent Caesarean delivery at 33 weeks’
gestation. Peripartum haemorrhage was inevitable and was pre-empted
by transfusion of multiple blood products, the effects of which
were monitored by Thrombelastograph®. The blood products given
are discussed. (read
more)
Sustained
Engraftment Post Bone Marrow Transplant Despite Anti-Platelet Antibodies
in Glanzmann Thrombasthenia
Background.
Patients with Glanzmann thrombasthenia (GT) have normal platelet
counts but abnormal platelet aggregation and carry the risk of
life-threatening bleeding. We report three patients who received
bone marrow transplantation... (read
more)
Ms. Helen
Smith
Glanzmann's Research Foundation
Augusta, GA 30907
Dear Ms. Smith:
On behalf
of the Department of Pediatrics and the children we serve, I want
to thank you and the Glanzmann's Research Foundation
for your generous donation... (read
more)
Peter
Zdziarski Read
Peter's story here. |
I
wanted to brag a little about Peter - he participated in his
first triathlon on Sunday in a relay team with his aunt and
uncle (Peter swam, uncle biked, aunt ran). He did a 1/2 mile
open swim in a lake and placed 14th out of 92 swimmers!
His time was 15 minutes and 45 seconds. He was actually the
2nd youngest swimmer in the water that day! His relay
team placed 7th out of 20 relay teams and 4th in their division.
I have a great picture of him getting out of the water I'm
hoping to post soon.
I know
some parents with young GT children focus on the sports
their children will not be able to particpate in. If you
focus on the amazing sports your kids can participate in
and encourage them - the sky is the limit!
Take
care -
Missy
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Monitoring
survival and function of transfused platelets in Glanzmann thrombasthenia
by flow cytometry and thrombelastography
C.
Male, D. Koren, B. Eichelberger, K. Kaufmann & S. Panzer
Department for Pediatrics, and Clinic for Blood Group Serology,
Medical University Vienna, Austria
Patients
with Glanzmann thrombasthenia (GT) may form isoantibodies which
induce
refractoriness or inhibition of function of transfused platelets.
We monitored the
survival and function of transfused platelets by flow cytometry
and thrombelastography
in a patient with GT. Gating on CD42a + allowed identification
of even a few transfused platelets. Only by gating on these CD41
+ CD42a + cells were we able to demonstrate their capability to
bind fibrinogen and PAC-1 upon activation. (read
more)
The
Seafood Bandage
A new powder made from shrimp stops serious bleeding—fast
Popular Science Magazine Staff Writer - Monica
Khemsurov | July 2006
Launch
the slideshow to learn how the seafood bandage works.
When it comes to war wounds, red is dead. Stop the bleeding, and
you save the soldier. It’s a simple idea that’s driving
a budding industry for fast-acting blood-clotting agents.
The latest
in the category: a pourable bandage called Celox.
Made by SAM Medical Products near Portland, Oregon, Celox is a
granular compound derived from shrimp shells. “It resembles
uncooked grits,” says Adrian Polliack, SAM’s head
of research and development. When poured directly into a bleeding
wound and subjected to pressure, the positively charged Celox
granules cross-link with negatively charged red blood cells, forming
a putty-like plug that blocks blood flow [see
slideshow].
The process takes about five minutes and features key advantages
over two kinds of high-tech bandages now in use in Iraq and Afghanistan.
Unlike HemCon, a bandage also made of shrimp shells that works
only on surface injuries, Celox can be poured into major wounds.
QuickClot, a clotting aid made of mineral powder, is similarly
applied but creates heat that can cause severe burns.
Celox could
hit war zones by year’s end, pending approval by the Food
and Drug Administration. After that, your first-aid kit.
Thromboembolic Adverse Events After Use of Recombinant Human Coagulation
Factor VIIa
Kathryn A. O’Connell, MD, PhD; Jennifer J.
Wood, PhD, MPH; Robert P. Wise, MD, MPH; Jay N. Lozier, MD, PhD;
M. Miles Braun, MD, MPH
JAMA. 2006;295:293-298.
Context The US Food and Drug
Administration (FDA) licensed recombinant human coagulation factor
VIIa (rFVIIa) on March 25, 1999, for bleeding
in patients with hemophilia A or B and inhibitors
to factors VIII or IX. Use in patients without hemophilia
has been increasing since licensure.
Objective To review serious thromboembolic
adverse events (AEs) reported to the FDA's Adverse Event Reporting
System (AERS). more
Blood, 15 October
2005, Vol. 106, No. 8, pp. 2596.
Viral
rescue of murine
Glanzmann thrombasthenia
Jerry Ware
UNIVERSITY OF ARKANSAS FOR MEDICAL SCIENCES
The platelet
integrin receptor is central to the platelet's role in hemostasis
and its absence results in the human bleeding disorder,
Glanzmann thrombasthenia (GT). In this issue
of Blood, Fang and colleagues describe ex vivo
viral transduction of mouse hematopoietic stem cells and determine
the in vivo efficiency required to rescue the GT phenotype in
a mouse model of the disorder. more
Preventing
Iron Deficiency Anemia
Iron deficiency
is still a big problem today. In fact, the World Health Organization
lists iron deficiency as one of the 'Top Ten Risk Factors Contributing
to Death'. more
Dow Jones
Business News
Novo Nordisk Says EU OKs NovoSeven For Two New
Treatments
Thursday February 19, 7:49 am ET
COPENHAGEN
- Danish pharmaceuticals company Novo Nordisk A/S said Thursday
the European Commission has approved NovoSeven for two new treatments.
NovoSeven (Eptacog alfa (activated)) was approved for the control
of bleeding in patients with factor VII deficiency
and Glanzmanns thrombasthenia refractory to platelet transfusions.
more
November
24, 2003
British Journal Recommends Immunization for Patients with Bleeding
Disorders
The British
journal Haemophilia outlined investigators' recommendations
appropriate for patients with bleeding disorders,
saying..." more
Glanzmanns Thrombasthenia
(GT) is caused by a deficiency of a protein on the surface of
the platelet, called Glycoprotein IIb/IIIa. As
a result, platelets fail to form a plug at the site of an injury.
more
Research Is being
done by various organizations. Because of the rarity of the disease,
funds for research are rare also. more
Stories about people
with GT:
At
first glance, Julia Smith's curly golden hair, huge
blue eyes and Cupid's bow lips bring to mind a life-size
porcelain doll. But the bruises that dot the 2-year-old's
body from head to toe tell a different story. Julia
was born with a rare, incurable bleeding disorder
called Glanzmann's thrombasthenia.
more stories
more
about Julia
The
Message Board is place to communicate and share ideas.
Please be sure to register and log in to the board. more
Fund Raisers
are put on by a variety of people. They have been very helpful
in raising money and gaining community awareness. more
Submit pictures,
stories & contacts to Helen Smith. See the contact
page.
| This
is a non profit website. The goal of this site is
to provide the general public, parents, loved ones,
and the medical community with accurate information.
All information is taken from sources believed to
be reliable. No treatment of GT should be undertaken
without medical supervision. Donations should be made
to the Glanzmann's
Research Foundation.
All donations are tax deductible. |
