Amanda Erickson’s disease is so rare it made an episode of “House.”
She has Churg-Strauss syndrome, a disorder where inflammation restricts blood flow to vital organs and tissues, sometimes permanently damaging them. Not only is it rare, it’s hard to diagnose. There’s no cure. And, it can only be controlled with steroids and other immunosuppressant drugs.
It first threatened Erickson’s life, then her chance at ever having a child naturally. If the best things in life don’t come easily, the 37-year-old was in for a fight.
“Having come through something so terrifying and real, my entire outlook on life changed. It definitely made coping with all the chaos and horrible that would come later much easier,” she said. “But the prospect of never being able to have kids naturally really shook me to the core.”
The diagnosis
In her 20s, Erickson struggled with allergies, asthma and a never-ending sinus infection.
The allergies were “wicked,” Erickson said. “It felt like I was allergic to the world,” she said.
But, asthma was a bigger problem. In a matter of months, she went from having no symptoms to needing an Advair inhaler.
“No one could explain why, at 27, I suddenly developed such intense and threatening asthma. It would be almost two years before we were able to tie the asthma to the underlying disease,” she said.
She lost her sense of taste and smell for almost two years, resulting in sinus surgery in 2004.
Then, in 2005, like a domino effect, Erickson suffered one major attack on her body after another.
She was hospitalized for pericardial effusion, where fluid develops around the heart and suffered cardiac tamponade. A few months later, she suffered pleural effusion, where excess fluid builds around the lungs. She then developed neuropathy in her hands, arms, feet and legs, and massive clots in her left leg and lungs.
After the pleural effusion, a blood test showed Erickson had a higher than normal count of a type of white blood cells called eosinophils. Eosinophiis normally make up 1 to 3 percent of white blood cells. A count higher than 10 percent is considered abnormally high and indicates Churg-Strauss syndrome.
Upon diagnosis, she began a high dose of steroids to help manage the disease.
“You swell up like Violet Beauregarde in ‘Willy Wonka,’” she said about her reaction to the steroids.
Through swelling up like a blueberry and all, her then boyfriend, Brian Forrest, stuck by her. They were soon married.
Heartbreak
In 2007, Erickson and Forrest saw Minnesota Perinatal Physicians to see if she could have children in spite of her disease. The mortality rate for Erickson was too high. She was initially told there was a 5 percent chance of mortality. The current U.S. maternal mortality rate is a fraction of a percent. She and her husband were advised not to have children naturally.
“That was crushing, especially because around that time, all of my friends were starting to have children,” Erickson said, who added that she began avoiding her friends – and the pain.
She and her husband left the office thinking they would never have kids naturally. For the next three years, they considered adopting, finding a surrogate or being an awesome couple, without kids.
During that same time, she went into a chemical remission. With the exception of upper respiratory infections, her disease stayed manageable.
A medical milestone
Fast forward to 2011. Erickson was “getting to the age where it was do or die,” she said.
For the second time, she and Forrest found themselves at Minnesota Perinatal Physicians. Before they walked through the door, Donald Wothe, MD, had done plenty of research. As much as he could, that is.
There were “only a few case reports” of women with Churg-Strauss syndrome trying to get pregnant, Wothe said. There’s currently no good data on how many women with Erickson’s disease have tried to get pregnant and have been successful. That’s because, on average, people don’t develop the disorder until their mid to late 40s.
“It’s very unusual,” he said.
Women struggling with the ability to control their symptoms are at higher risk for attempting to get pregnant, Wothe said. When women with a rheumatological condition get pregnant, a third get better, a third get worse and a third stay about the same.
Wothe couldn’t tell Erickson into which camp she would fall, or whether her body would reject a baby. But, when he saw Erickson, her symptoms had been under control for a few years – and that was promising.
“He said, ‘let’s give it a shot,’” Erickson said.
When Erickson and her husband left the building, she cried. They called their parents, and they cried with them.
“It was both incredibly exciting and terrifying,” Erickson said.
She had battled so much. If she got pregnant, only to lose the baby, she would be devastated.
Breakthrough
The couple tried anyway, and it took longer than they would have liked.
“The minute you decide to have a baby, it never works,” she said.
Then, last fall, Erickson was a week late when she “felt pregnant.” She told Forrest that if nothing happened by the next morning, she’d take a home pregnancy test. She got up early to take the test. The lines were faint, but she took the test into her bedroom, jumped on the bed and told her husband, “Your boys can swim!”
But, he was skeptical. The lines were so faint. About an hour later, Erickson took a digital pregnancy test. She got the same result. She was pregnant.
Her husband was shocked, excited and terrified – for her and the very real threat they faced.
For the past eight months, Erickson has administered daily shots to her stomach to prevent blood clots. She’s experienced more “scrutiny” than a non high-risk woman would, like seeing an obstetrician about twice as usual. She’s had more blood work and trips to her rheumatologist, hematologist and general practitioner.
“Now, we’re almost done. It’s just astounding to me,” she said.
Erickson is due on Aug. 16 with a boy. Her husband and her mom, a retired RN who will act as a doula, will be by her side at The Mother Baby Center.
She faces significant risk for recurrence after she delivers her son, Wothe said. Fortunately, she’ll be able to take medications that were limited during the pregnancy. Close monitoring and the ability to intervene in more ways will help make the postpartum period safer.
“I’ve been waiting for the other shoe to drop,” she said. “There’s so much that could have gone wrong and didn’t.”