The Power of a Phone Call: Following Up on Sickle Cell Disease

April 18, 2014
By Kristina Grifantini

When a mother received a letter from the Illinois Department of Public Health stating that her newborn tested positive for a blood abnormality, she complied with the request to schedule a follow-up blood test with her provider. At the follow-up appointment, the hospital reported to her that her child did not test positive for sickle cell disease, a genetic blood disorder that affects tens of thousands of Americans.

When the mother continued to get mail from the state asking her to schedule another blood test for her newborn, she ignored it, figuring the hospital hadn’t reported her daughter’s results from the follow-up test yet. It wasn’t until she got a call from Anquineice Brown, an outreach coordinator at the Sickle Cell Disease Association of Illinois (SCDAI), that she found out her daughter wasn’t in the clear.

“The follow-up test results they gave the mother were incorrect,” says Brown. Because SCDAI receives notice from the state of any Illinois newborn screened as having a blood abnormality, Brown was notified that the mother’s infant still needed a screening. “The state was sending letters because she needed to go for another follow-up test. On the third test, her now 18-month-old child was confirmed to have sickle cell disease,” says Brown.

Brown referred the mom to a care coordination team where a hematologist and pediatrician work together to deliver care. “She told us she was so grateful we had called,” says Brown.

Because patients with sickle cell disease benefit from early treatment, newborn screening for the condition is mandatory in the US. Despite this, many get lost in the system: some families don’t get the results of screening; others don’t understand them or don’t follow up for a variety of reasons. Through a national effort called Working to Improve Sickle Cell Disease Healthcare (WISCH), NICHQ is helping centers around the country address this problem by improving follow-up procedures.

SCDAI, a participant in the WISCH program, gets anywhere from 0 to 60 notifications a day, says Brown (roughly 4,000 newborns are born with sickle cell trait and abnormal hemoglobin and roughly 90 are born with sickle cell disease in Illinois each year). Within 48 hours of the state’s notification, SCDAI sends a follow-up letter to the family encouraging the family to call the center. If families don’t make contact within two weeks, Brown or another coordinator calls them.

Follow-up procedures to newborn screening aren’t just about making sure the newborn is getting necessary care. During follow-up calls, the center—and others like it across the country—offer genetic counseling to the family. A person has sickle cell disease when he or she inherits two copies of the sickle cell gene, one from each parent. If a person inherits only one copy of the sickle cell gene and one normal gene, they have sickle cell trait and generally have no noticeable symptoms. Genetic testing lets parents of children with sickle cell disease know their status and risk of passing on the gene to future children. If both parents have the trait, there is a 25 percent chance with each pregnancy that any child of theirs may have sickle cell disease and a 50 percent chance with each pregnancy that any child of theirs would have sickle cell trait.

“Some parents think that because you have one child with sickle cell disease, the next three you have won’t have the disease,” says Brown. “We explain that every time you get pregnant, all the dice go back in the bag and roll out a different way.”

After talking with parents, the organization sends them educational materials in the mail. They also organize baby showers for newborns to introduce families to others with sickle cell disease and to consistently reinforce care messages to new parents. For families that are particularly anxious or request it, Brown will also do home visits to talk in greater depth about sickle cell disease. She and others at the center will also go, at a patient’s request, to a school, daycare or other location to give presentations about sickle cell disease. They also do public speaking and health fairs throughout the entire state. Through the partnership with NICHQ, the organization has been able to fine-tune its process, says Brown. “Helping us to set goals and measure outcomes has added value to our work,” she says.

Brown offers the following tips to other follow-up coordinators: provide as much information as possible during initial contact with the family, and “don’t give up trying to connect a family to care,” she says. “The family will always remember and appreciate your help.”

Language Barriers

In New York, the Bronx Lebanon Hospital Center follows up with families whose newborns have sickle cell trait or disease, says Mark Goodwin, program coordinator for the sickle cell department and participant in the WISCH program.

While the center has had success presenting at health fairs and doing on-site genetic testing around the community, it has struggled with a low retention rate for patients making follow-up appointments for genetic testing and counseling. Historically about 30 percent of parents made the appointments, rising to 50 percent last year with quality improvement changes and dipping back to about 40 percent this year, says Goodwin. He and the quality improvement team are analyzing past data to find out what caused the success and dip.

“We think it may have to do with the phone scripts,” says Goodwin. “The challenge is conveying how important the follow-up appointment is without alarming the family by saying it’s urgent.”

Other challenges are contacting the families. Many have cell phones with prepaid minutes and don’t get the follow-up team’s messages. Others are confused about the location of the genetic testing, which is off-site. And one of the biggest challenges is a language barrier. More than 50 of the parents served by the center are non-native English speakers.

While the center has made many improvements to address these challenges—such as creating educational materials in English, Spanish and French, and having a staff that speaks Spanish—it’s not enough, says Goodwin. “We have patients from Africa who speak many other languages. We have one father who speaks English but only reads Arabic. So the literacy issues in the community are large.”

Goodwin says the WISCH program has been helpful in making improvements. “We’re becoming more aware of the different methods other community organizations and hospitals use. By becoming aware of different options for reaching families, we hope to improve our rates even further.”