E O
AST
145th Year, No. 137
REGONIAN
SATURDAY, SEPTEMBER 4, 2021
$1.50
WINNER OF THE 2020 ONPA GENERAL EXCELLENCE AWARD
GET OUT THE BOOTS AND COWBOY HAT,
THE PENDLETON ROUND-UP IS BACK
INSIDE
SAVING
PROVIDENCE
Hermiston family races
to raise $400K to treat
daughter suff ering
from genetic disorder
By WIL PHINNEY
For the East Oregonian
H
ERMISTON — The
hazel eyes of Provi-
dence Grace Rector
dart back and forth
behind thick lenses in round
red frames. Her face constantly
twitches, but otherwise it is with-
out expression. Her arms are
constantly moving, unintention-
ally. Her little legs are strapped at
the ankle to the footrests on her
wheelchair.
Providence means “protective
care of God,” which is refl ected
in the strong faith of her parents,
Miranda and Jason Rector, but
they know a contribution from
science will be needed to fi nd the
medical miracle their daughter
needs.
And now, two-and-half years
after their daughter was diag-
nosed with Early Infantile Epilep-
tic Encephalopathy-37 (EIEE-37),
the Rectors and other parents like
them are hopeful — and optimis-
tic — that a potential study of gene
replacement therapy could one
day lead to a better quality of life
for their children.
In August, with the help of
Rare Village Foundation, a fund-
raising platform for families of
children with rare diseases, three
mothers — one from Croatia, one
living in the United Kingdom
and one in Colorado — estab-
lished a nonprofi t called “Finding
Hope for FRRS1L” (pronounced
“frizzle”). In less than 24 hours
more than $100,000 was raised,
and within fi ve days the total had
doubled.
Miranda, who is enrolled
Chippewa from the Turtle Moun-
tain Band of Chippewa Indians
of North Dakota and also Puerto
Rican. People in Puerto Rico,
where several children with EIEE-
37 live, have given more than
$50,000.
What FRRS1L is
FRRS1L is the name of a
protein-coding gene contained in
all cells of the brain. It helps send
messages between cells. The gene
disorder, which results in loss of
function for that gene, is a muta-
tion triggered by the combination
of each parent’s damaged DNA.
The disorder produces epilepsy,
progressive dyskinesis (abnormal
Audrey Bailey/Contributed Photo
The Rector family of Hermiston, in a photo taken in the fall of 2019, includes Miranda, Jason, Providence and Joshua. Providence had recently
turned 2 years old. “It was a big celebration for the family, as it is every additional year we get with her,” Miranda said.
movements), developmental delay,
poor muscle tone, brain volume
loss, and gradual loss of respon-
siveness to the environment.
Children with this gene disor-
der develop, albeit with delays,
until about ages 1-2 when they
begin having seizures that cause
regression. Within months, or
even weeks, children lose their
ability to move, speak, eat orally,
and control their head and their
body. Babies with the FRRS1L
gene disorder grow up to be
wheelchair bound, non-verbal,
tube-fed and in need of constant
care.
For Providence, it took just
three months to go from a crawl-
ing baby to one that is trapped in
a body overtaken by seizures and
involuntary movements.
helped create the fundraising page
www.FRRS1L.ORG and mobi-
lized other families. The exact
number of children in the world
living with FRRS1L gene disor-
der is unknown, but in a Face-
book support group, parents of
14 FRRS1L children have started
to raise funds. Miranda Rector is
helping the group with public rela-
tions.
The initial goal was $100,000
in the first month, the amount
needed by Dr. Berge Minassian, a
gene therapist at the University of
Texas-Southwestern, to compose
a team of researchers.
Maniassian told parents he
would investigate possible treat-
ments, but he advised them that
it would require at least $400,000
to fund the project up to clinical
trials.
Fundraising promising
In August, the three women
See Providence, Page A8
Miranda Rector/Contributed Photo
Providence rests from extreme exhaustion due to respiratory distress,
para-infl uenza, and uncontrolled seizures during her June 2020 hos-
pital stay at Dorenbecher Children’s Hospital at Oregon Health and
Science University in Portland. She is pictured with a gastrostomy-jeju-
nostomy tube that prevents the risk of aspiration during coughing fi ts.
A G-J tube is a single tube that passes through the abdominal surface,
into the stomach and down into the second part of the small intestine.
Where are the workers?
Workers in short supply amid
year of explosive job growth
By SUZANNE ROIG
The Bulletin
BEND — With record job growth
in Oregon, Whitney Keatman never
imagined she would have problems
fi nding workers for her Scott Street
location of Sparrow Bakery.
And she didn’t. It was getting them
to stay that has plagued her long time
Bend business.
In the past year Keatman, a
co-owner, made 117 job off ers and
most were accepted. But after just
three months, only about 42 work-
ers remained. The bakery needed a
minimum of 20 workers at the Scott
Street location and by the end of this
summer there were six.
So last week, she made a hard
decision: She will close the bakery
at the Old Iron Works Arts District.
EDITOR’S NOTE
Today starts part one of a fi ve-part
series by EO Media Group to publish
over the next fi ve weekends, looking
at the issue of the lack of workers for
jobs in Central and Eastern Oregon;
why workers are not returning to
previous-held jobs; and how busi-
nesses are pivoting to function with-
out being fully staff ed.
Dean Guernsey/The Bulletin
Co-owners of Sparrow Bakery in Bend, Whitney, left, and Jessica Keatman
with their sons, Brooks and Abel are at the Scott Street bakery location,
which is closing due to a worker shortage.
“We’ve been struggling for seven
years with moments of success,”
Keatman said. “Over the years
we’ve had on again and off again
good managers. When you don’t
have the right management, you
have a hard time retaining workers.
“It’s common for people to come
and begin training and then get
another job off er that competes with
wages,” Keatman said. “There’s no
allegiance. It’s a competitive market
for employers.”
See Workers, Page A8