'Every day is a bad day.' Sickle cell disease bill would educate public, track patients

Aubrey Wilsey of Mashpee is relieved that his son Jayden Wilsey, 13, was released from a month-long stay at a Boston hospital on Nov. 17. But Jayden's sickle cell anemia symptoms have persisted.

"He hasn't had a pain-free day in a long time," said Wilsey.

Mashpee Middle-High School officials have put together a plan where Jayden will attend school for half the day, five days a week.

In October, Aubrey Wilsey received a letter from the Cape and Islands District Attorney's Office requiring him to attend an Oct. 27 hearing about his son's school absences. School officials knew, prior to the hearing, about Jayden's diagnosis of sickle cell beta thalassemia, a rare form of the disease, Wilsey said.

Following the hearing, Wilsey and his family weren't penalized for Jayden's absences, Wilsey said. "We are hoping to get him back soon," said Wilsey.

Jacqueline Hailey, executive director for the Massachusetts Sickle Cell Association, said she's heard stories like Wilsey's "over and over again." Caregivers and those suffering from sickle cell disease face racial disparities within public health care systems, schools and in the workplace, she said. Black people are less likely to receive outreach, education, and most importantly routine preventative care, she said.

Sickle cell disease is a group of inherited red blood cell disorders. Signs of the disease can appear during the first year of life, usually around five months, according to the Centers for Disease Control and Prevention. Symptoms and complications are different for each person and can range from mild to severe.

Normal daily activities such as climbing stairs, running laps in elementary school gym class or roughhousing with friends can trigger a pain crisis because the sickled red blood cells can't carry oxygen efficiently."Let's just call it what it is. It's a Black disease. It's never been a priority," said Hailey during a phone call with the Times Thursday.

For Hailey, sickle cell disease is a public health crisis. Which is why the association partnered with state legislators like Rep. Bud L. Williams, D-Springfield, Sen. Liz Miranda, D-Boston, and Rep. David Rogers, D-Belmont, to support An Act to Improve Sickle Cell Care, which was heard by the Massachusetts Legislature's Joint Committee on Financial Services.

"We are hoping to fight on a higher level to decrease disparities, and address standardized care for those with sickle cell disease," said Hailey.

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Testimony moves the committee, says Williams

Sickle cell disease, said Williams, affects one in 12 people of African descent and is one of the most underfunded and overrepresented disorders in the nation," said Williams over the phone Wednesday.

Over 100 people testified at the Nov. 14 joint committee hearing for the bill. The show of support and testimony made Williams emotional, he said. For two hours, patients, doctors, lawyers, and health care specialists, shared their related struggles surrounding sickle cell disease.

"We file 8,000 bills a year. But the committee was moved by the testimony given for this bill," said Williams, who is also chair of the Massachusetts Black and Latino Legislative Caucus.

The joint committee will try to pass the bill in 2024.

U.S. Sen. Corey Booker, D-New Jersey, reintroduced the bipartisan, bicameral Sickle Cell Disease Comprehensive Care Act in March. The federal legislation would direct the Centers for Medicare and Medicaid Services to create a Medicaid demonstration program to improve access to comprehensive, high-quality, outpatient care — including clinical and support services — for individuals with sickle cell disease.

Education is main component of sickle cell legislation

Education is one of the most important aspects of the bill, said Williams. From schools to hospital emergency rooms, people who suffer from sickle cell disease are often misunderstood.

And that's because there are no public health initiatives surrounding sickle cell, said Hailey, of the state association. In hospitals, said Hailey, providers have a lack of knowledge about sickle cell disease. "Because of that, we have families in hospital emergency rooms who are being labeled as drug seekers," she said. The bill, said Williams, has the capacity to raise public awareness surrounding sickle cell. Education, he said, can improve standards of care across the state.

If passed, the state bill would establish a sickle cell steering committee within the state Department of Public Health, said Williams. The steering committee will make recommendations that can create better outreach and education. The group will also be charged with establishing partnerships with hospitals, colleges, and universities, and developing sickle cell educational material for the public and health-care providers. “This legislation is crucial in ensuring that our commonwealth continues to be an unprecedented leader in advancing health care, well-being, racial equity, and inclusion for all," said Williams.

Sickle cell disease registry

One of the biggest hurdles when supporting sickle cell patients, said Williams, is keeping track of who is suffering from the disease. The bill would also help establish a sickle cell disease registry; and create better methods to coordinate patients’ transition from pediatric to adult care. The steering committee will also investigate and report on standards of care for patients, and enhance access to patient services.

The lack of organization, and the absence of standards of care for state sickle cell patients, is frustrating, said Williams. Twenty-five years ago, Williams said sickle cell disease was talked about more often and was a chief concern within Black communities. "All of a sudden, there was radio silence on it," he said.

Sickle cell warriors bring families back into the fold

Sickle cell disease has been around for hundreds of years, said Hailey. But there have never been public health initiatives surrounding the disease.

"You see billboards for diseases like diabetes and cystic fibrosis all over the place," she said. "But not for sickle cell."

With additional factors such as language barriers, and limited access to health care, programs and services, families have become tired of advocating for themselves, said Hailey.

"They feel beaten down. They feel like no one is listening to them," she said. "It's exhausting."

In recent years, Hailey said the association has begun encouraging families to speak out about their struggles with sickle cell. When patients vocalize their difficulties, there is more interest in helping with the bill's initiatives.

"It's an uphill battle but we are still here fighting, together," she said. "There will be change."

Can the bill get over the finish line in 2024?

At the very least, Williams hopes the legislation will "get over the finish line in 2024." By passing the bill, the legislature would be sending a clear signal to the Black community that pushing the needle forward against the debilitating disease is a priority.

"For patients living through sickle cell, every day is a bad day. The pain is 24/7. It doesn’t go away," he said. " We want to make their days a little brighter."

Rachael Devaney writes about community and culture. Reach her at rdevaney@capecodonline.com. Follow her on Twitter: @RachaelDevaney.

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This article originally appeared on Cape Cod Times: Few sickle cell health initiatives prompts new bill, legislators say