Mast Cell Disease: When Your Immune System Becomes the Enemy

Crystal Goodwin

Three years ago, Crystal Goodwin, who just turned 30, lived a fairly normal life. She had a fulltime job and enjoyed hanging out with friends, working out at the gym and going on trips with her sister. A rare disorder called mast cell activation syndrome changed Crystal’s life almost overnight.

It all began in September, 2011. Out of the blue she had severe intestinal problems — pain, inflammation and bleeding. Soon after, about every three weeks, sometimes, twice a week, she had anaphylaxis, which is a severe, potentially life-threatening allergic reaction. When it happens, the immune system releases a flood of chemicals. The blood pressure drops suddenly and the airways narrow, making it hard to breathe.

Anaphylaxis symptoms

  • Skin Itching, flushing, hives, swelling
  • Eyes Itching, tearing, redness, swelling of the skin around the eyes
  • Nose and mouth Sneezing, runny nose, nasal congestion, swelling of the tongue, metallic taste
  •  Lungs and throat Difficulty getting air in or out, repeated coughing, chest tightness, wheezing or other sounds of labored breathing, increased mucus production, throat swelling or itching, hoarseness, change in voice, sensation of choking
  •  Heart and circulation Dizziness, weakness, fainting, rapid, slow, or irregular heart rate, low blood pressure
  •  Digestive system Nausea, vomiting, abdominal cramps, diarrhea
  •  Nervous system Anxiety, confusion, sense of impending doom

“I’d just walk into work and all of a sudden I’d have anaphylaxis,” Crystal told me. “I didn’t know what was happening. It felt like something was taking over my body. It did not feel right at all. I just knew there was something wrong.”

Anything and everything seemed to trigger the anaphylaxis. Crystal became afraid to eat or drink, to walk into a new environment, to smell or touch things, to leave home, to leave her bed. She could no longer work. Countless trips to the emergency room terrified and exhausted her. For a year, she was bedridden. For a year, she had no answers. In October 2012, she saw a specialist at Brigham and Women’s Hospital in Boston and was told she had mast cell disease.

Mast cells are a type of white blood cell found in the immune system. They contain histamine, which helps fight infection, but if too much is released or released inappropriately, it can wreak havoc. “When someone has a mast cell condition like Crystal’s, suddenly their mast cells just explosively release chemicals — out of the blue, sporadically and unpredictably, “explains Dr. Marguerite Pennoyer, the allergist Crystal now sees in the Portland area. “The cells line your gut, your skin, your respiratory tract and blood vessels, so you can have multi-system effects.

There is no cure for mast cell disease and the cause is usually unknown, but there are medications to control it — or at least, make the attempt.

Crystal Goodwin with medications

Today, Crystal is on 39 medications, which she must take four times a day. Most are for her disease, such as antihistamines and steroids, and the others are for their side effects — steroid induced osteoporosis, for instance. When she’s having an allergic reaction, she has to increase the dose of some of the meds. “For example,” she says, “Sunday night I broke out in hives, so I had to take extra benadryl and last night I had a moderate allergic reaction where I was really close to going to the ER, but thankfully, I got enough meds in me to calm it down. I’m constantly taking extra. I’m on tons of antihistamines, and I’m on more if I have symptoms.”

When she’s having an allergic reaction Crystal’s entire body usually turns red, she itches all over, gets bad intestinal pain and diarrhea, her lungs swell and she has trouble breathing, gets light-headed, and feels nauseous.

She’s not as terrified as she was in the beginning, but still gets scared and has nightmares. Her social life is basically non existent. No more going out with friends. Some are afraid to come near because even a faint smell of laundry detergent or hand soap could trigger an allergic reaction. “I’m always adding to the list,” she says. “Tape, certain plastics, paper dust — I had an allergic reaction to a paper shredder one time, just from the dust. It was one of my worst reactions, actually. Extreme cold, extreme heat, even the sun can cause a reaction.”

Crystal Goodwin at the gym

A saving grace is daily workouts at Snap Fitness in South Portland where, so far,  she hasn’t had a reaction. Staff know what to do if she does — Call 911 and grab the epinephrine autoinjector. Personal trainer Darren Cook had never heard of mast cell disease before. Now, he’s well versed. He not only helps Crystal work out, he’s become a friend and strong advocate. Recently, he came up with the idea of holding a Push Ups for Crystal Challenge to raise money to help her with medical expenses. With the combined efforts of Snap Fitness and Back in Motion Physical Therapy, the challenge raised $7000.

Health insurance issues

If battling a horrendous disease wasn’t enough, Crystal has found herself caught in the middle of some health insurance battles as well. Because she can’t work, she collects Social Security disability benefits — about $20,000 a year.  She applied for Medicaid, but only qualified for a spend down program that required her to spend $8,000 out-of-pocket every six months before anything is covered.

Instead, she chose a Marketplace plan and qualified for an 87 percent subsidy.

In October, because of her disability status she became eligible for Medicare, but after researching all the plans, she discovered that many of her medications were either not covered or had high deductible. On top of that, the monthly premium would be $500. It added up to about $150,000 dollars a year, which she could not possibly afford.

She decided to stay with the Marketplace plan, but there was a catch. She would no longer qualify for the subsidy and will have to pay an additional $150 in premiums. Here’s what Medicare  says:

You can keep your Marketplace plan after your Medicare coverage starts. However, once your Part A coverage starts, any premium tax credits and reduced cost-sharing you get through the Marketplace will stop.

It’s a federal regulation and apparently, there are no exceptions to the rule.

When Crystal called the Marketplace to explain that she wanted to keep her current plan without the subsidy, she was disenrolled, then reinrolled in the plan. The consequence was that on October 1st she started at square one, with a $4500 deductible. The $1500 she had paid toward her original deductible was gone and no one seemed to be able to do anything about it.

She has spent countless hours on the phone, talking to one person after another, trying to see if she might qualify for a subsidy again and if the deductible could be rolled over. She has had no success. She decided to set up a gofundme account to raise money to help with her expenses.

Crystal’s Marketplace plan is through Anthem Blue Cross and Blue Shield of Maine. I contacted them and after she signed a HIPAA waiver giving permission to discuss her case, and asked about the issue with her deductible. This is the reply I received from Rory Sheehan, Director of Public Relations:

“It is important to note that insurers cannot make changes to plans purchased on the exchange without direction from the health insurance marketplace. Members with relevant life or eligibility changes must report these directly to the marketplace and this information is shared with an individual’s insurer. We are aware of Ms. Goodwin’s change in eligibility and had already begun the process to credit her deductible towards her new plan. We are working diligently to complete this as soon as possible and appreciate the opportunity to serve her and other members across the state of Maine.”

In other words, they are rolling over what she had paid toward her deductible. A customer service rep will call her when the process has been completed.

Crystal was thankful when I shared the news. She still plans to take on the federal government about the subsidy loss. Without the subsidy, not only is her premium higher, but her deductible went from the $1500 she had already paid to $4500 and she will have to come up with the difference.

Worrying about her health insurance coverage and how she’ll pay her medical bills has caused her a great deal of stress. Constant stress is bad for someone with mast cell disease. Really bad.

Could her disease ever go away? Sometimes it will simply burn itself out, says Dr. Pennoyer. It could take years though, and she’ll be left to cope with the aftermath, especially from all those medications.The specialist at Brigham and Women’s told Crystal he’d never seen someone this severe before. “Their hope is to be able to put me in remission, so that way I could go six months or a year without a reaction, but it’s been three years and the most they’ve gotten me to is every six to eight weeks and then I’m back in the ER with another reaction.”

Diane Atwood

About Diane Atwood

For more than 20 years, Diane was the health reporter on WCSH 6. Before that, a radiation therapist at Maine Medical Center and after, Manager of Marketing/PR at Mercy Hospital. Now she writes the award-winning blog Catching Health with Diane Atwood.