It was the summer of 2009 and Bella, a vivacious 10 year old, was vacationing at a national park in France with her family when she experienced a strange tingling in her lips. Without any apparent cause, her lips puffed up and her face swelled. In the absence of any other symptoms, Bella and her family laughed at Bella’s altered appearance.
Bella’s mother Lisa, of Seattle, described the moment as the calm before the storm.
“We had no idea at the time that it would be the beginning of a nightmare that would take years to diagnose,” says Lisa.
In the months that followed, Bella would experience similar swelling episodes. Her abdomen would suddenly become distended. Her feet and ankles would balloon. The symptoms would disappear as abruptly as they appeared, leaving her parents to assume Bella had suffered some sort of allergic reaction.
The mysterious symptoms started appearing more frequently and with more severity once Bella reached adolescence. Bella’s throat would inexplicably tighten, constricting her airway and sending her to the emergency room. Her painfully enlarged abdomen would swell even further when palpated by doctors. As the severity of Bella’s symptoms escalated, doctors explored possible causes, ranging from relatively benign — allergies, appendicitis, stomach flu — to drastically more serious — lupus, brain tumors, cancer. She missed seven months of her seventh grade year due to debilitating pain.
Six years after that fateful day in France, Bella finally received a definitive diagnosis. But that wasn’t the end of the family’s harrowing journey. The rarity of her disease, the scarcity of the life-saving medication, and the red tape that delayed appropriate and timely medical intervention would combine to push the family to new depths of hopelessness and despair. It wasn’t until Bella switched health insurance companies and a case manager reached out to offer help that the nightmare began to fade, and hope for Bella began to emerge.
Sudden and puzzling symptoms
Doctors finally diagnosed Bella with hereditary angioedema, or HAE, when she was 16. HAE is a rare, life-threatening blood disorder, characterized by leaky small blood vessels, which causes plasma to be pushed into surrounding soft tissue. Severe swelling follows. It can be triggered by dental work, estrogen, repetitive motions — and sometimes seemingly nothing at all. Because the disease is so uncommon, it often takes years to diagnose. Symptoms frequently present as acute appendicitis. Statistics show that as many as one third of HAE patients are missing at least one organ — the result of surgeries performed in an attempt to solve a medical mystery.
Bella was lucky that her diagnosis came before she lost any organs, but pinpointing the cause of Bella’s symptoms would not deliver the relief her family had been hoping for.
“Once we got the diagnosis, we felt like we’d won the lottery,” Lisa says. “We had a name, there were medicines. We thought we’d be OK.”
But Bella’s health was still complicated. Unfamiliar with the condition, ER teams would press on Bella’s swollen body, unintentionally making symptoms worse. The rarity of the disease meant Bella’s medical teams, often unfamiliar with treatment plans, presented conflicting protocols. Some suspected appendicitis; others assumed she must be experiencing an ectopic pregnancy. The IV-administered medication Bella needed to stop the swelling took 20 to 40 minutes to take effect, but, because it was needed so infrequently, it often wasn’t stocked at hospitals. Once it was delivered, it was often too late to do any good. Every time Bella went to the ER, it was like the first time: tests were repeated, delays were experienced, and her condition escalated.
“There just wasn’t a system in place,” says Lisa, recalling the stress and helplessness the family felt each time Bella was rushed to the hospital. “Every second that passed, we knew she could become a statistic, knowing that a third of HAE patients asphyxiate due to untreated laryngeal swelling.”
The family’s despondency peaked during an emergency room visit just one month after Bella’s diagnosis. Bella was awaiting her medication as her swelling worsened. Bella began to choke, and a nurse reached for a suctioning tool to clear her airway. Caregivers swarmed Bella’s bedside as Lisa locked eyes with her daughter who struggled to catch her breath.
“I remember thinking, ‘You might not make it through this one,’” Lisa says. “There were so many people in the room, and the medications exist. … I could not believe she was this sick.”
A game-changing phone call
About four months after the HAE diagnosis, as emergency room visits and school absences multiplied, Bella’s family switched insurance companies. Soon after, Lisa received a voicemail from a woman with Premera Blue Cross. She took her time calling back.
“I was prepared for a fight,” says Lisa, wary of starting a conversation that might mean facing additional hurdles related to the coverage Bella’s medication.
What Lisa didn’t know at the time was that Bella’s case had been spotted by the Premera case management team, which helps customers with complex conditions manage their care.
When Lisa finally called back, Michelle Magers, a social worker and certified clinical case manager, was on the other end of the line. Initially confused about who Magers represented, Lisa was comforted when Magers said, “I work for Bella.” Magers wasted no time creating a case management plan that would ultimately change the course of Bella’s recovery.
“One of the obvious problems I saw right off the bat was that every time Bella went to the ER, the hospital treated it as a brand-new incident,” Magers says. Repeated blood work and assessments delayed delivery of the time-sensitive medicine Bella needed, making her condition worse. “We really needed a plan in place so when she showed up at the emergency room, they knew exactly what was happening and exactly what to do.”
Magers connected with the pharmacies, ER staff and all Bella’s doctors to craft a plan of action. She had Bella’s diagnosing doctor review the plan, then presented it to the hospital. Magers made sure Bella’s name was flagged, so as soon as Bella showed up at the ER, the plan would kick in.
Perhaps most importantly, Magers asked Bella and her family for their input to the care plan.
“As a young teen, very few people thought to ask Bella for her opinion,” Lisa says. “Michelle did.”
Magers has also helped manage Bella’s medications. Methods of delivering the medication have become simpler, advancing from nurse-administered shots to self-injections. But along with each new drug comes paperwork and approval requirements.
“Michelle has been like Houdini releasing himself from ropes,” Lisa says. “She says, ‘Where are the knots, and how do we untie them?’ She’s enabled us to get out of the trunk and live instead of worrying about the bills and medicines.”
A model for change
The Premera case management program has been in place for 15 years, says Kathy Peters, senior manager of Premera clinical programs, and has been identified as the go-to case management model for other insurance companies. Customers who are dealing with multiple or complex issues are flagged so case managers like Michelle can reach out and offer help. In Bella’s situation, she was identified as a special case by the hospital because she had been admitted so many times.
“Bella is a great example of the type of patient our program can really help,” Peters says. “Our case managers, like Michelle, are trained to take a holistic look at tricky cases and figure out how to eliminate the barriers that cause frustration and get in the way of good medical care.”
In the three years since Magers joined Bella’s care team, Bella’s visits to the hospital have dropped to zero. Medication approvals have been streamlined and care plans formalized. Bella was able to return to school, go to concerts with her friends, take hikes with her family and get back to a normal life. Bella and her family give all the credit to Magers.
“She has a gentle voice, but I’ve begun to think of her as a bulldozer,” Lisa says. “She asks, ‘What’s the barrier?’ and it gets mowed down.”
This August, Bella packed her bags — including one suitcase just for medication, syringes and associated medical supplies — for a year abroad, the first part of a three-year global rotation program through her university. Her first stop: Seoul, Korea. Again, Magers stepped in with an exhaustive care plan that involved the translation of medical documents, ordering extra doses of medication and detailed coordination with Bella’s medical team in Seoul.
“I don’t have to worry about having access to my medication,” says Bella, now 20. “I never want to go back to that ‘survival phase.’”
Even though Bella’s condition is stable and her care plan for her semester in Korea is locked down, Magers does not rest. She’s currently working out the kinks to ensure Bella’s medications and authorizations are in place for her next adventure: four months in India in 2020.
Says Lisa: “We’re just hanging on for her ride.”