MarlaJan Wexler wasn't exactly grateful to be diagnosed at age 27 with lupus, an inflammatory disease that makes her immune system attack the tissues and organs it's supposed to protect.
But it explained a lot. At her best, Wexler, now 36, felt worn out, even after a full night's sleep. At her worst, she couldn't get out of bed.
Now, a regimen of drugs — including an infusion once every four weeks of Benlysta, an immunosuppressive drug — keeps Wexler's symptoms in check.
Infused and injected biologic medications have been lifesavers for people who, like Wexler, have difficult-to-treat illnesses and few other options. But they come at an incredible personal and financial cost.
In addition to the medication's price — the Cherry Hill infusion center Wexler uses charges $8,300 — people who rely on biologics tether themselves to inflexible infusion appointments every several weeks and the imperative to have good health insurance.
"It's a lot of work to be sick," said Wexler, who lives in Marlton.
Specialty medications, including biologics, account for 1 percent of prescribed drugs, yet comprise about 32 percent of drug spending in the United States. As these sophisticated treatments become available to treat a broader range of illnesses, patients, physicians and insurers will need to reckon with their high price tags.
"Our big focus has been, we need new drugs — we only have a handful — but what we forget is once those drugs are out, can patients afford them?" said Susan Manzi, the medical director for the Lupus Foundation of America. "The global answer to that, often, is no."
Though they still account for a small minority of the pharmaceutical market, developers are investing heavily in biologics, a newer science that involves growing proteins and DNA structures out of live cells, and that holds promise for addressing illnesses that lack effective treatments. Biologics spending topped $120 billion in 2017, a 12 percent growth from the year before, outpacing overall growth in the $324 billion pharmaceutical industry, according to IQVIA, a market research firm.
The way they are made and administered — an infusion or injection — contributes to universally high prices around the world. A year's worth of biologic infusions runs tens of thousands of dollars in Canada and other countries where health care often costs less than in the U.S.
GlaxoSmithKline, which makes Benlysta, said it considers the research and development that went into the drug, as well as its value to patients, when pricing medications. Like many manufacturers, GSK has a financial-assistance program that offers rebates and free medication to some patients, based on financial need and type of insurance. The growing number of Americans with high-deductible health plans must pay a greater share of their health costs than in the past, helping to drive assistance plans.
"We do recognize that an increased drive toward more cost sharing that we are seeing in the U.S. health-care system may result in affordability concerns for some patients," said Anna Padula, a spokeswoman for the company, in an email.
Patients who rely on biologics often have few, if any, other options.
Remicade, for example, is one of the few treatments available for Crohn's disease, which causes such severe bowel inflammation that surgery to remove the affected bowel can become necessary.
Since October, Grace and Dan Jost have been taking their 10-year-old son, Aaron, for Remicade infusions at the Children's Hospital of Philadelphia about once every six weeks.
The hospital charges about $11,500 for each infusion, which comes down to $3,100 after their insurer's adjustments and payments.
A rebate program by Remicade maker Janssen knocks off about $2,800 per infusion, leaving the Voorhees family with an out-of-pocket cost of about $340 per infusion until they reach their health plan's out-of-pocket maximum.
As is the case for most patients with autoimmune diseases, the cost of the infusion is only one piece of the financial puzzle. The family expects to spend about $10,000 a year — the out-of-pocket maximum for their health plan — on medical expenses.
The cost is significant, but worth it, if it means Aaron can have a semi-normal childhood.
Dan doesn't like when Grace compares the diagnosis to a death in the family, but that's how she feels. "It's a death of the normal life you expect for your son," she said.
Grace, a retail pharmacist, and Dan, who owns an architecture firm, budget for the anticipated expense by cutting back in other areas, such as vacations.
They feel fortunate, knowing the cost could be crippling for some families.
"For us, it takes away $10,000 in savings. We plan for it and that's just the way it is," Dan said. "Someone making $50,000 a year who has to deal with this — it's a big deal."
Treatment regimens like Aaron's take a heavy personal toll, too.
In order for the medication to work properly, patients must be diligent about keeping their appointments, however frequent and inconvenient they may be. Weekend appointments are coveted and hard to get, which means patients must be able to take off from work or, in Aaron's case, school.
Grace cut back her demanding work schedule to 30 hours a week shortly before Aaron was diagnosed; now, returning to work full time isn't possible. She might consider a less-demanding job. But her family is insured through her employer, so a new job could mean a new insurer and disruptions in Aaron's treatment.
"There's no question in our society the cost of health care is driving a lot of decisions that people make about employment, where they work, how long they work," said the Lupus Foundation's Manzi, who also chairs Allegheny Health System's Medicine Institute outside Pittsburgh.
Lupus has changed Wexler's life trajectory.
A pediatric nurse, she took years off from work because she felt so sick. Now, she works from home 20 hours a week as a triage nurse, fielding phone calls and directing patients to the right department.
The job isn't what she envisioned for herself, but it comes with valuable health insurance.
Between the half-dozen medications she takes and medical visit cost-sharing, Wexler spends about $500 a month out of pocket on health care, in addition to insurance premiums.
It's a monthly expense she must budget for the rest of her life. Lupus is a chronic condition with no cure.
"As a nurse, I get it," Wexler said. "As a patient whose life has been changed exponentially, it's frustrating."
Though she can't do the kind of nursing she would like, Wexler has found another way to tap into her passion for helping people.
Wexler's blog, Luckfupus.com, has an international readership, and she speaks at health conferences around the nation as a patient advocate.
One of her proudest moments came during a pharmaceutical roundtable a few years ago, when she said she confronted a pharmaceutical executive about why the company wasn't investing more in drugs for lupus. She does not receive funding from the pharma industry.
"I can still make a difference — it's in a completely different capacity than I ever thought," she said. "But I'm going to do it."
Patients are optimistic that, as research advances, new medications will give them more options and bring down costs.
There has been a push to accelerate development of biosimilars — akin to, but not exactly like, a generic version of a brand-name drug — and spending in this area is on the rise, as patents on original biologics expire, said Michael Kleinrock, research director for IQVIA's Institute for Human Data Science.
But in health care, competition does not necessarily lower prices, in part because price is often hidden from consumers.
"It's a highly moveable feast as for what will happen," Kleinrock said.
What is a biologic? Biologics differ from traditional drugs in the way they are made and administered to patients.
Rather than combining different chemical compounds using formulas that are very exact, biologics are complex proteins and DNA structures grown out of live cells. More familiar examples include vaccines and blood products. But the new generation of biologics is developed to treat illnesses that have resisted traditional pharmaceuticals.