Rare Diseases

Illness, 4 walls closing in on Arkansas woman; rare disease complicates insurance

Becky Malin, 27, had her first reaction from mast cell activation syndrome, a rare disease, when she was 2 months old. She has been confined to her apartment for eight months because she no longer has Medicaid coverage for the expensive treatment to help her fight the syndrome.

Creases on her torso show the imprints from her intestines pushing at the skin on her stomach. She has started to retain water, bloating from malnutrition. Her hair, which used to hang down to her shoulders, is cropped close to her head and falling out in dark-brown clumps.

Becky, 27, has been shut in her apartment for the past eight months, hiding from the irritants that make her immune system react — changes in the weather, almost all antibiotics, perfume, pepper, even laughing too hard can cause her to go into anaphylactic shock.

Because of her disease, Becky’s body has trouble absorbing most foods, leading to malnutrition.

She has been diagnosed with mast cell activation syndrome, a rare disorder that affects almost every part of the body. Statistics on the number of people who have a mast cell disease vary because it is difficult to diagnose.

Mast cells are a type of white blood cell and part of the human immune system. They exist in almost all parts of the body and help maintain equilibrium. When someone has a mast cell disease, aberrant cells overreact.

What might make an unaffected person sneeze can cause Becky’s throat to close.

“It takes my body over,” Becky said. “Mast cells are in every tissue. Every organ. Every bone.”

Treatment for this syndrome is expensive and is complicated by the variety of ways it manifests.

Many insurance companies do not cover treatment for it, said Lisa Klimas, a Massachusetts-based advocate for those with mast cell disease.

Klimas, who has a version of the disease, has been studying it since 2013. She has a master’s degree in biology, and her graduate research focused on microbiology and infectious bacteria. Her website, MastAttack, gets close to 4,000 views on an average day.

“It [treatment] should not be very expensive, but it is,” Klimas said. “Insurance companies don’t like to pay for it. … It’s something that’s a huge struggle for most of us.”

Becky, who had her first reaction when she was 2 months old, received at least four denial notices from Medicaid in March alone. Among the reasons cited for not paying for X-rays and needed shots: “Benefits exceed maximum of $500 per state fiscal year” and “Injection requires a specific diagnosis code.”

Becky provided the Arkansas Democrat-Gazette with screenshots of emails that document her most recent complaint to Medicaid, submitted online Sept. 25. She has not received a response, she said.

The ICD-10, Medicaid’s list of covered illnesses, contains several forms of mast cell disease, but Klimas said Arkansas’ limits on coverage are among the most restrictive in the country.

Becky said a contributor to her low level of care may be a lack of mast cell specialists in the state.

“It’s not just Arkansas, but Arkansas is a good example of a state that is doing a massive disservice to their patients with mast cell,” Klimas said.

Klimas works with mast cell patients across the country and said they often are denied care because a proposed treatment isn’t approved or recommended by the U.S. Food and Drug Administration.

“This means that patients are told their diagnosis is covered, only to discover this is meaningless when [Medicaid] will not cover any therapies the patient actually needs,” she said via email.

Online searches of mast cell support groups and forums reveal patients’ complaints of denials and requests for advice. While some reported success after appeals to insurance companies, others said they could not find care or get insurance coverage.

A spokesman for the state agency that oversees Medicaid in Arkansas said that denials like those Becky has received may be a result of a billing error or treatments not meeting the guidelines outlined by the program.

“We don’t just blanket not cover illnesses,” said Amy Webb, a spokesman for the Arkansas Department of Human Services.

Valerie Slee, vice chairman of the Mastocytosis Society, a nonprofit that researches and advocates for people with mast cell disorders, said mast cell diseases were added to the ICD-10 in October 2016 after nearly three years of work.

Despite coverage from Medicare and a supplemental health plan, Slee, who has mastocytosis, said she had a medical bill of just over $777,000 last year.

Because mast cell disorders are inherited, family members often take to trading medications when they can’t afford to fill prescriptions, waiting for insurance coverage to come through, Slee said.

Klimas said medical professionals often disagree on treatment methods, partly because many doctors are unfamiliar with the disease, which may also complicate getting payments from insurance providers.

“Whenever you have a disease that’s complex, there’s always going to be treatment options,” said Slee, who has been in advocacy work since 2002. “I don’t think that should be a decision that’s managed by…