Olivia Shorter was diagnosed with sickle cell disease when she was just seven days old and is continuing her fight as she looks forward to her eighth birthday on Sept. 20.
Just as she did to celebrate her 7th birthday, Olivia will host another sickle cell party blood drive for kids like herself and in recognition that September is National Sickle Cell Awareness Month.
The blood drive will be held with the American Red Cross on September 15 from noon to 5 p.m. at the Matteson Community Center, 20642 Matteson Ave. in Matteson, Ill. Walk-ins are welcome. Or you may register at redcross.org.
“Without regular blood donations, Olivia’s hemoglobin levels will drop to the point where she could die. My daughter is alive because someone took an hour of their time to donate blood,” Danielle Shorter, Olivia’s mom, said. “We, like other sickle cell families, receive transfusions regularly. I wanted to do my part to make sure there’s enough blood in the bank for all of us.”
The Shorter family hopes the blood drive and sickle cell party will become an annual event to help raise awareness and much needed funds for sickle cell patients and research.
Last year’s blood drive event drew more than 200 guests to the Matteson Community Center; about 75 visitors donated blood at the event. Olivia was named the 2018 Red Cross Blood Services Hero for her efforts.
“The grownups can give blood while the children play,” Shorter said, encouraging people to come out for the drive.
With good days and bad days due to the pain and the procedures she endures, it’s understandable that the little warrior was in no mood to talk to anyone after returning home from an MRI at Ann and Robert H. Lurie Children’s Hospital last Thursday.
The breathing tube that had to be inserted to administer the anesthesia needed for the MRI procedure left her with a sore throat.
“She’s in second grade now,” her mother said. “She started back to school with the other kids but had to go to the hospital for the MRI. We live in Matteson and her school is very understanding. This disease can affect a child’s education because she has to go for treatments and procedures and misses school when she’s sick.”
Fighting Sickle Cell
Affecting approximately 100,000 African-Americans, sickle cell disease is an inherited blood disorder, where the oxygen-carrying red blood cells take on a sickle-like appearance instead of the round disc shape they should be. The sickle shaped cells stick together and block off small blood vessels causing severe pain and organ damage.
To survive, Olivia has relied on blood transfusions since she was six months old. She also receives IV fluids and scheduled pain medications to help ease her pain, according to Lurie’s Children’s Hospital, where Olivia is a patient.
The best blood match for an African-American child with sickle cell disease usually comes from an African-American blood donor. Sickle Cell patients rely on 15 to 25 blood transfusions a year, according to information provided by Lurie Children’s Hospital.
“Livy is such a brave little girl, she doesn’t bat an eye when she gets her transfusions,” Shorter said.
Educating yourself as parents is one thing, but nothing prepares you for the horrible symptoms of sickle cell disease, Danielle said, recounting how at five months old, Olivia experienced something called, dactylitis (a form of inflammation in the fingers and/or toes that include swelling in one or more fingers or toes, and often in the entire hand or foot).
“It’s the worst experience in the world,” Shorter explained in a previous interview. “Dactylitis, plainly, is watching your child being tortured and there’s nothing you can do about it.”
Olivia in D.C.
As part of her fight against sickle cell disease, Olivia went to Washington, D.C. with her parents, Danielle and Adam, Sr. and her two brothers Adam Jr. 12, and Dylan, 10, over the summer to urge members of the U.S. Congress to help her and other children who have sickle cell disease.
“Olivia is a remarkable young woman,” said Kelly who represents Olivia’s district. “Olivia has become a powerful advocate for other children suffering from this disease. Her selflessness and willingness to educate her community is truly inspiring.”
The family’s trip to D.C. was part of Lurie Children’s Hospital’s Kids Family Advocacy Day.
“Being in Washington and seeing Olivia speaking to the lawmakers opened our eyes to her possibilities,” said Shorter. “Olivia has turned her pain into purpose.”
Though doctors at the University of Illinois Hospital say they’ve cured adult patients of sickle cell disease with stem cell transplants, there is no pediatric transplant program there. However other hospitals in Chicago reportedly offer the stem cell transplant treatment for children.
Shorter said she’s heard whispers about UI Health’s stem cell transplant treatment but has not spoken with anyone at the hospital about what the procedure entails.
“My fear is that Olivia may reject the stem cells,” Shorter said. “I’m waiting for them to come up with a proven cure where there will be no rejection before I allow Olivia to undergo a treatment like that.”
Dr. Damiano Rondelli, the Michael Reese Professor of Hematology and Director of the Blood and Marrow Transplant program at the University of Illinois Hospital, said during an interview with the that, “We have made great strides curing adults with sickle cell disease with stem cell transplants, but the unfortunate truth is that the majority of these patients have, until now, been unable to benefit from this treatment because there are no fully-matched HLA-compatible donors available in their family.”
With the new treatment, patients with aggressive sickle cell disease can receive stem cells from family members if only half of their human leukocyte antigen (HLA) markers match. Previously, donors had to be a family member with a full set of matching HLA markers, or a “fully-matched” donor.
Because HLA markers are inherited from parents, family members are the most likely to have matching proteins. In transplants, matching HLA markers between the patient and the donor help to limit the risk that the patient’s body will reject the donor cells, according to Rondelli.
“These are bone marrow stem cells,” Rondelli said. “The treatment involves sitting a few hours attached to a machine as if you’re giving blood, except only the white cells are collected from the donor for transplant to the patient.”