Rae Blaylark had nowhere to turn 22 years ago when her son was born with sickle cell disease and she was told that she was a carrier.
Sickle cell, an often-fatal blood disorder that mainly strikes people of African ancestry in the United States, was still rare in 1990s Minnesota. Getting answers to basic questions was nearly impossible. She vowed then no one here diagnosed with the disease would ever "feel like they have no support and feel like they're on an island of their own."
Two decades later, there's a comprehensive sickle cell clinic in place for children in Minnesota. Blaylark and others, however, warn that adult cases are climbing to crisis levels now as more West Africans and African-Americans move here and Minnesota has few care options for them.
Other Midwestern states have adult sickle cell clinics and hematologists who specialize in adult care. Minnesota has neither. The few hematologists here who do treat sickle cell adults are not specialists. Patients who age out of pediatrics at age 21 have no place to go for coordinated care.
That's led to a spike in deaths in Minnesota, but advocates say no one knows how many because Minnesota doesn't track adult cases. And because the disease damages organs, deaths end up attributed to specific organ failure and not sickle cell.
"I feel we've reached an emergency status," said Dr. Stephen Nelson, a pediatric hematologist who runs the sickle cell program at Children's Minnesota Hospital in Minneapolis. "We're now seeing the peak time when patients are dying is between 18 and 25."
Nelson and others in Minnesota believe race is the primary reason sickle cell receives so little attention and medical resources. It's the only disease where the life expectancy has fallen in the United States.
Minnesotans falling through the cracks
Sickle cell is characterized by misshapen red blood cells that can block blood vessels, cause pain and life-threatening conditions, like kidney failure and strokes.
Intense, unpredictable pain episodes, called crises, are the hallmark of the hereditary condition. Frequent emergency room visits, hospital stays and blood transfusions are the norm for many patients.
"One day you're fine, the next day you wake up in extreme pain. Cringeworthy pain," said Rae's son, Trey Blaylark, who struggles with symptoms in his arms and legs. "It's like your arm is dying because there's no blood flow to that part of your body and you can feel it dying."
Pediatric cases have grown from 40 to 400 the past 25 years.
If both parents carry the sickle trait, there's a 1 in 4 chance their child will have the disease.
Nelson's clinic handles most of the state's pediatric cases, including Trey's until a year ago. Genetic counselors, social workers and nurse case managers are part of the clinic's team that provides medical and psychosocial treatments. Rae Blaylark serves as the clinic's family health advocate.
The clinic's comprehensive approach over the years has improved outcomes for children, Nelson said. But when his patients age out of pediatrics after 21, some fall through the cracks due to the lack of coordinated care for adults, he added. "Some of my former patients have moved out of state to find providers, and some, unfortunately, have died," Nelson said.
Advocates say research and treatment for the disease are underfunded compared to similar conditions that largely affect whites. Sickle cell was discovered more than 100 years ago but until last year, only one drug had been approved by the U.S. Food and Drug Administration to treat it.
There isn't a national registry, so the Centers for Disease Control and Prevention doesn't know how many people are afflicted. The CDC estimates about 100,000 people — about 1 in 365 black babies and 1 in 16,300 Latino infants — are born with it.
In Minnesota, it's about 30 babies each year. Another 1,100 infants are born with the sickle cell trait. The state has no data for adult patients who moved here from other states and countries. Minnesota officials only began in 2015 to notify parents if their newborns are carriers of the trait.
Across the country, adult clinics can be found on university campuses, where a coordinated, multidisciplinary and centralized care model is the the standard in treating and managing the complex disease.
The University of Minnesota, however, scrapped its plans for such an adult clinic in 2014.
"Our care team is working to build a network within the care community that can provide greater access rather than driving patients to a single clinic," Naomi McDonald, the U's medical school's communications director, responded in an email.
The state's Eliminating Health Disparities Initiative, established by the Legislature in 2001 to improve health outcomes for people of color, hasn't done so with sickle cell, nor has the state's Center for Health Equity, created in 2013.
Two Minnesota Department of Health officials acknowledged the state's failure.
"A lot of it I think is historical and due to health equity issues," said Barb Dalbec, manager of the agency's program for children with special health needs.
"It's been a challenge because we don't have specific resources for sickle cell," said Joanne Bartkus, director of the state's public health laboratory. "So, how do you to reach this population that the state doesn't have any idea of how big it is?"
Nelson and Blaylark have pulled together an informal group on the matter, tapping the state Health Department, the U, health insurers, the Center for Health Equity, emergency room physicians and the governor's office. But it's still in its infancy.
The Health Department recently awarded Blaylark's Sickle Cell Foundation of Minnesota, the state's first community-based advocacy group for sickle cell patients, a small outreach grant. Children's Minnesota also received funding to prepare teens and young adults for their transition through education. That included an education/bowling party recently in Minneapolis.
But Bartkus said the department's response to sickle cell doesn't have the teeth to fully address the problem because it lacks official backing from the department, designated funding and strategy.
"It's an ad hoc effort," she said. "We don't have specific resources other than the time that we're able to scrape together out of our schedules. "
Ultimately legislative changes might be the solution to bridge the gaps in care and increase awareness, said James Burroughs, the state's chief inclusion officer.
Like Blaylark, Burroughs and his ex-wife didn't know they were carriers of the sickle cell gene.
Their daughter, now 6, was born with the disease. One in every 12 African-Americans and one in every 200 Latinos are carriers.
Burroughs said trait testing needs to be expanded beyond newborn screenings because most adults learn they are carriers when their children are diagnosed with the disease.
'They think I'm faking'
Racial discrimination is a reality for patients. Nelson knows from personal experience.
Nelson, who is white, said nine years ago he refused to prescribe one of his patients Hydroxyurea, the only sickle cell drug and effective treatment on the market at the time.
"I decided he wouldn't pick up his prescription," Nelson recalled. "I decided he wouldn't take his medication; I decided he wouldn't come to clinic every month for his blood work because of what the narrative of an 18-year-old black boy from north Minneapolis, with little or no social support, was. And that's not OK."
Nelson never budged on his decision and he doesn't know what happened to the patient, who aged out of pediatrics a few years later.
Nelson said that regretful incident is an example of the biases, conscious or unconscious, and stereotypes that white physicians may harbor. Coupled with structural racism in the health care system, the result is poorer health outcomes for people of color, including longer wait times in the emergency rooms and less pain medication than their white counterparts.
"Minnesota is routinely at the top for health care outcomes but the subtext is for white Minnesotans because when you look at outcomes for Minnesotans of color, we are close to the bottom if not No. 50," he said.
Some sickle cell patients say they've endured medical care that lacked compassion, sensitivity and understanding.
Bathesheba Benson says the disease stole her childhood. She suffered two strokes, one at age 5 and then at 21. Now 34, she's struggled to find a competent doctor since leaving Children's.
"Some doctors here don't even know what sickle cell disease is," the Golden Valley resident said. "I'll find a doctor and then the doctor will transfer me to a different doctor to a different doctor to a different doctor. My health has been up and down."
She's in out of the hospital and said she lost a job when an employer thought she had AIDS.
Sickle cell patients regularly end up in the emergency room when pain crises hit, in need of potent opiate medication.
"We've gone many, many times to the emergency room," Burroughs said of his daughter, Teresa. "Those first few years were tough. She was given morphine, a really powerful drug, at 3. The pain episodes are that bad."
With the nation's opioid addiction crisis raging, adult sickle cell patients say they are increasingly dismissed as drug-seekers when they come in pain to hospital emergency rooms.
"Sometimes they think I'm faking," said 21-year-old Jasmine Toliver of St. Paul. "I actually had a physician who thought I was faking and she didn't want to help me. And she told me that I would be addicted to drugs and I could overdose. I was upset. I told my mom about it."
Benson said she recently "cried my eyes out, begging the ER doctor" before getting medication for a midnight pain crisis.
"You know how they have the ribbons for cancer and other conditions? I wish we had that for sickle cell disease," said Benson, who is originally from Liberia. "I wish people were more knowledgeable about it. I just wished there was more awareness."
'Different' if it were a white disease
Nelson began his career at Children's Minnesota's 25 years ago. At that time there were 40 kids in the program.
Today there are more than 300, and they are mostly the children of West African refugees and immigrants and Chicagoans who also migrated to the Twin Cities.
The clinic has grown from seven doctors to 15, along with nurse practitioners, a genetic counselor and nurse case managers who specialize in sickle cell disease.
Until a few decades ago, sickle cell was a considered a childhood disease because patients didn't live long enough to become adults. That's changed, Nelson said.
"We've done a good job in pediatrics, so there's an expectation that our patients are going to make it to adulthood," he said. However, care remains centered around children.
Meanwhile, adult centers have sprouted nationwide for conditions, like cystic fibrosis and hemophilia, which were also once deemed childhood illnesses, Nelson said.
"We'd be having a very different conversation if we were talking about cystic fibrosis," Nelson said of the hereditary disease that overwhelming affects white Americans. "There are robust adult treatment centers for cystic fibrosis."
Nelson hopes the fledgling effort by agencies, nonprofits and the medical professions will lead to the state's first adult clinic. He's on a mission now to find primary physicians to take his former patients.
That includes Dr. Hannah Lichtsinn, a Minneapolis internist and pediatrician, who heard Nelson speak to young doctors at the University of Minnesota on the urgency of treating adults sickle cell patients and signed up. Lichtsinn started treating people a year and a half ago. She now has 60 patients, including Toliver.
"The more I get involved in caring for people with sickle cell disease, the more ashamed I am about how our health care system has failed this group of people," Lichtsinn said.
Blaylark said she's not waiting for outside help. Her foundation, which began last year, recently raised $10,000 through a 5K walk at Bethune Park in Minneapolis.
"We have to start by helping ourselves," she said.
"It doesn't mean we don't expect more of them, but we're not going to not do anything because they're not," she said of the state's health systems. "We don't want to be silent; we don't want to be hidden anymore."