CLEVELAND — In 2020 Sierra Butler finally learned what was causing her rashes, fatigue and extreme joint pain. After numerous visits to various doctors, she was finally diagnosed with Lupus, a rare autoimmune disorder affecting about 300,000 Americans, 90% of whom are women.
But Sierra’s case was more complicated than most.
“Unfortunately, I have a specific type of lupus that's pretty stubborn, I have drug resistant lupus,” she said.
And it didn’t take long for it to become much worse.
“I was pretty much on my deathbed in and out of the hospital every week, I was in kidney failure, which was leading to severe cardiac distress,” Sierra said.
Bloodwork pointed to systemic lupus erythematosus, or lupus in the blood which can affect any of the internal organs. She was also dealing with lupus nephritis, which is kidney inflammation due to lupus.
Sierra’s poor kidney function caused her to retain fluid, and she was in and out of the hospital almost every week and was so extremely weak she could barely stand on her own.
In addition to all this, she developed cardiac tamponade, which is when the fibrous sac around the heart fills with blood or other fluid and puts pressure on the heart – preventing the heart’s chambers from filling properly. The condition is life-threatening if not treated quickly.
Sierra’s doctor suggested she seek care at Cleveland Clinic.
“The biggest change. was having a team versus individual doctors. and when I first came to the Cleveland Clinic, I was in a wheelchair. I was not able to walk. I was bald because I lost my hair from previous treatments. I was really, ill,” Sierra said.
That’s where she met rheumatologist, Emily Littlejohn, D.O. She and her team helped stabilize Sierra’s lupus using a medication regimen of oral immunosuppressive therapy and IV infusion chemotherapy.
Then they went a step further and crafted a plan to help Sierra and her husband, Jer, have a baby. They switched her to pregnancy safe medications and little Mea arrived in November 2023 with no problems.
However, shortly after, Sierra’s lupus started to get out of control again.
Sierra had already tried all the available medications, so Dr. Littlejohn suggested chimeric antigen receptor (CAR) T-cell therapy. Cleveland Clinic is a site for the first clinical trial in the U.S. for this treatment for systemic lupus.
“CAR T-cell therapy involves filtering out the T-cells, which are white blood cells in your immune system, and sending them to be genetically engineered to have a special receptor inserted into them. We then reinfuse the new T-cells into the patient, so these specifically engineered cells can find lupus-causing cells and kill them,” says Dr. Littlejohn.
Sierra spent two weeks in the hospital getting chemotherapy to prime her immune system to essentially rebuild it. Then the modified T-cells were re-infused and went to work finding the lupus cells and killing them.
“I have been relatively pain free, which has been a first for me in a really long time. And then the other big thing that I've noticed is my energy levels have really increased, which fatigue was a big issue for me,” Sierra said.
Sierra knows the deformity the lupus caused to her hands is likely permanent, but she says this treatment is the first glimmer of hope she’s had since her diagnosis. She wants to be the mom who teaches her daughter to ride a bike and play soccer, not sit on the sidelines. It also gives her hope for the research and for the future of other autoimmune diseases.
“The big milestone for these patients would be that they stay in disease free remission for six months to one year. That would be my hope. because in this space, If they can go two or 3 or 4 years, that would be life changing. And to me, that would mean that it's likely that the disease is really put into remission, and remission is not a term that we typically use for lupus,” Dr. Littlejohn said.