Thank Your For
Visiting Help Baby Gracie.
We appreciate
your interest and would like to share
Gracie's story with you.
We would like to thank all those who have in
some way made a contribution to help Gracie.
The donations and benefits have been
wonderful and greatly appreciated. Please
click here for our acknowledgements page and
visit the sites of those who have reached
out to help Baby Gracie.
What is it?
Citrullinemia stems from a deficiency of
argininosuccinic acid synthetase
Inheritance:
It is suspected that the gene defect for
citrullinemia is an autosomal recessive
genetic trait and is unknowingly passed down
from generation to generation. This faulty
gene only emerges when two carriers have
children together and pass it to their
offspring.
Signs & Symptoms:
Symptoms are hyper ammonia accompanied by
lack of appetite, vomiting, listlessness,
seizures, and coma. Onset is usually at
birth, but symptoms may not be noticeable
for days or weeks.
Long term effects:
When left untreated, brain damage, coma and
death will occur.
Treatment:
Treatment may include high-caloric,
protein-restricted diet, arginine
supplementation, administration of sodium
benzoate and sodium phenylacetate. Dialysis
may be necessary in some affected
individuals.
There is no cure for this except for a liver
transplant, which we have decided to go for
Gracie, as this would greatly improve her
quality of life, and possibly give her a
longer lifespan. We ask that you help us by
praying for her, as that’s the only reason
she’s still with us and is doing as well as
she is. When Gracie was at her worst she had
fixed pupils so they were not responding to
light, and they had declared her brain dead!
Her ammonia level got as high as over 500
when normal is between 9-33. She was on
dialysis, she had a blood transfusions,
plasma, she was on life support, and had
lines going in and out of her little body
all over, but praise God answered our
prayers and brought our little girl through
it all!
We are pleased to
announce that Gracie received a liver
transplant on Feb. 8th, 2006 at the
Children’s Hospital of Philadelphia (CHOP).
Her transplant did away with her
disorder and now presents her with some new
challenges. This was the fourth time she had
been called up for a transplant. The
previous three times the potential risks
outweighed the potential benefit so we
returned home. Gracie is currently on two
anti-rejection medications as well as six
other medications. She had a rejection at 10
days post transplant that was treated
successfully with medication. Gracie was
able to return home on March 2nd and was
admitted to the East Tennessee Children’s
Hospital on March 6th. She was admitted due
to fever which among other things can be a
sign of rejection. While at the hospital she
was treated for infection with high dose
intravenous antibiotics. She also underwent
surgery to remove her mediport (a surgically
implanted intravenous access). Her fever
broke and she returned home on the 13th.
Gracie has a long recovery ahead with follow
up care at Vanderbilt Children’s Hospital
two to three times a month as well as
Children’s Hospital of Philadelphia once a
month. There are still many needs for
Gracie’s recovery and continuing medical
care. Gracie is still in need of your
prayers. She is currently doing well and
through it all she continues to smile. You
may keep updated by going to Gracie’s
website at
www.childrenofpromise.net/gracies.html
or
www.helpbabygrace.com.
Contributions can be made at any First
National Bank in Gracie’s name
First National Bank Locations:
200 East Broadway Lenoir City, TN 37771
865-986-3546
16239 Hwy. 70E. Lenoir City (Dixie Lee) TN
37772
865-988-2160
10514 Kingston Pike (West Knox) Knoxville,
TN 37922
865-988-2210
1708 West Broadway Maryville, TN 37802
865-982-9191
