For the past four years, Jamie Lanier has been fighting a foe that's a little too close to home – her own immune system.
The 37-year-old Mechanicsburg woman has lupus, a chronic inflammatory disease that occurs when the body's immune system attacks its own tissues and organs. For Lanier, it began wreaking havoc with her kidneys four years ago when she pregnant with her second son.
"I was huge. There was just so much water. My feet were swelled so badly that I could barely walk," she recalled.
The tipoff was abnormal levels of protein in her urine found when she was just eight weeks pregnant, but a biopsy was postponed until after she gave birth. Her baby came two months early and a biopsy seven weeks later led to a diagnosis of membranoproliferative glomerulonephritis, a long name for a type of inflammation of the kidney.
"I retained tons and tons of water and it was hard just to sit. All the fluid would pool in my legs and I felt like crap all the time," said Lanier, who worked a desk job as a collection agent. "I worked in a cubicle environment and I was sick all the time with every virus there was. I was in and out of work a lot."
At the time, she said her doctors told her they didn't know the cause. She was put on immunosuppressant drugs and steroids, but the regimen was never effective in stabilizing her kidney condition.
At Johns Hopkins Hospital in Baltimore, Lanier finally learned the cause of her kidney woes – lupus nephritis, which occurs when lupus causes inflammation in the kidneys, making them unable to properly remove waste from the blood. The kidneys leak protein into the urine, which can lead to scarring and even kidney failure.
Lupus, which affects 1 in 1,000 people, is nine times more likely to affect women than men because it is fueled more by estrogen than testosterone, said Dr. Lisabeth Scalzi, a rheumatologist at Penn State Milton S. Hershey Medical Center.
Its cause is thought to be partly genetic and also environmental, meaning something in the environment triggers onset in a predisposed person, she said. Most people are diagnosed between age 16 and early 40s.
Mimics other diseases
"Lupus can be very difficult to diagnose because there's not one test to diagnose it and it presents so differently,'' Scalzi said. "You can have 100 people with lupus and 100 different ways it looks."
Symptoms of lupus can be so general – things like fatigue and stiff joints – that people put off going to the doctor, she said. Some teenagers start having mild symptoms at puberty but don't get diagnosed until years later. Its most distinctive symptom, a butterfly-shaped rash across the cheeks, doesn't always appear.
To raise awareness, a public service ad campaign by the S.L.E Lupus Foundation and the U.S. Department of Health and Human Services featured women asking "Could I have lupus,'' and encouraged women to talk to their doctors if they were having many of the seemingly unrelated symptoms.
Lanier said a mix of feelings came with her lupus diagnosis.
"I was relieved because I finally knew what was happening to me, but it sounds scary to say you have lupus. I'm 37; this is ridiculous!" Lanier said. "If I think too far down the line, I get a little lump in my throat that makes me want to cry. I don't want to end up on dialysis. I think of my kids and it scares me that this could be my future."
A lupus diagnosis is made by clinical appearance and lab work. However, a positive result on the antinuclear antibody (ANA) test doesn't necessarily mean a person has lupus so it's important to have more specific autoantibody testing, doctors said.
"It's so important to educate the general public and primary care physicians about the symptoms because the sooner it's diagnosed and treated, the better the chances of managing it well," Scalzi said.
In the past, treatment often included daily, high doses of prednisone, a corticosteroid that suppresses the immune system. But Dr. Michelle Petri, a rheumatologist who runs the Johns Hopkins Lupus Center, said research showed that 80 percent of permanent organ damage was due to prednisone. A daily dose above six milligrams increases permanent organ damage by 50 percent, she said.
Petri said treatment guidelines now call for lower doses of prednisone and always giving hydroxychloroquine (brand name Plaquenil), which modulates the immune system without suppressing it. Hydroxychloroquine prevents half of lupus flares and can help prevent lupus kidney disease, brain lupus and blood clots, she said.
Promising research
Although Lanier knows of no lupus in her family, her mother, Sharon Fleming, has struggled with rheumatoid arthritis for 30 years. As it's turning out, she's a role model for Lanier as she now struggles with her own ongoing health challenges.
"My mom couldn't ride bikes with us or do a lot of things with us when we were growing up, but she showed me what it was like to live with something and stay positive about life," she said. "I have a really amazing husband and a very supportive family. They are what helps me get through this."
The diagnosis of lupus can be devastating because it can mean daily medications for the rest of life, but it's a condition that is much more manageable today than ever before as more active research is devoted to new therapies, Petri said.
"With treatment, 50 percent of patients can enter a kidney remission," she said – and the other half can find their kidney issues greatly reduced.
Doctors said promising work is being done with a class of drugs known as biologics, which are derived from a variety of natural sources.
Lanier takes an immunosuppressant drug called CellCept, the brand name for mycophenolate mofetil. This drug is much better tolerated than its predecessor, a chemotherapy drug that often caused nausea, hair loss and premature ovarian failure, Petri said.
Although Lanier's lupus affects mainly her kidneys, it is a systemic disease that can wreak havoc with joints, skin, kidneys, blood cells, brain, heart and lungs. Lanier is currently battling itchy, dinner plate-sized hives all over her body and aching joints, another hallmark symptom of systemic lupus. Her thyroid also seems to be affected and having type 2 diabetes doesn't help either.
She wishes she had been tested her for lupus earlier, which she thinks could have potentially prevented scar tissue on her kidneys.
"One day I can feel great and the next day I can wake up and have to force myself to get up," said Lanier, who is unable to work but has hopes of improving now that she has the correct diagnosis. Link @
www.pennlive.com/bodyandmind/index.ssf/2015/01/struggle_to_diagnose_treat_mec.html