Kim Skelly’s IgG4-RD Story

Living with a rare disease can be lonely and overwhelming, but this Rare Disease Week, we want to build strength within the IgG4-RD community by breaking down the walls of isolation. While you may feel alone and like you're the only person living with IgG4-RD, you're not.

In fact, there are hundreds of thousands of people worldwide who share your diagnosis, like Kim. Kim, a flight nurse based in the Pacific Northwest, experienced an illness that came on fast and left medical professionals leaning toward a cancer diagnosis. In her own words, Kim details her experience and where she stands today:

After taking a nap at work (yes, I was napping at work: I'm a flight nurse), I woke up with an acute onset of abdominal cramping, nausea, and diarrhea. Over the next few hours, this turned into excruciating abdominal pain and severe vomiting. I got home from work and tried all of the home remedies for nausea and vomiting, but nothing was working, and the pain was getting worse. I knew something wasn't right.

My wife took me to the local ER. A CT scan revealed a bowel obstruction caused by a large 5x7 cm mass in my omentum as well as many smaller masses throughout my abdomen and pelvis. The immediate reaction of the ER physician was great concern for ovarian cancer.

A larger teaching hospital, Oregon Health and Science University (OHSU), in Portland, was consulted due to the concern of metastatic ovarian cancer. OHSU recommended a CT-guided biopsy of the mass. I was admitted to the hospital, had an NG tube placed, and was on high-dose pain and nausea meds.

About a day and a half later, I was taken to radiology for my CT scan. While doing preliminary scans before the biopsy, it was found that I had developed a bowel perforation. I was rushed to the OR in septic shock, where the mass was removed to biopsy, and I had 12 inches of my small intestine removed.

After waking up in the ICU from my procedure, the surgeon described my abdomen and pelvis as looking "very bad," even more concerning for cancer. The mass that was removed during my surgery was sent to pathology for a biopsy, and I was referred to OHSU's gynecology oncologists for follow-up.

My initial biopsy came back non-malignant. However, at my first gyn-onc appointment, the doctor told me this was most likely ovarian cancer and ordered a second biopsy. The second biopsy was non-malignant as well and showed similar sclerotic tissue to the first. The pathologist reviewed the initial biopsy, comparing it with the second, and made the diagnosis of IgG4 related disease. I was referred to Dr Ghetie, a rheumatologist at OHSU and began treatment.

In June 2023, I started a prednisone taper for over three months and soon after, began Aziathioprine as well. I followed up with my rheumatologist in November 2023 and found an increased IgG4 level after four months of oral treatment.

At this time, my rheumatologist decided to start me on Rituxin infusions. I have completed my first round of infusions with significant improvement in my symptoms (my symptoms mainly include joint pain in my lower extremities, fatigue, and brain fog). However, lab work two months after the infusion has shown an even more elevated IgG4 level than November. Currently I am continuing to take the Aziathioprine and have my second round of infusions scheduled for May/June 2024.

I was out of work recovering from my major surgery for five months. In mid-September 2023, I returned to work full time and have had no limitations. Although I am still not in remission, I am confident in the treatment I am receiving and am so fortunate to have been diagnosed fairly early!

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Responses

  1. Kim, I’m so sorry about the nightmare you had to go through. Unfortunately, that kind of thing seems all too common with this disease. Hopefully, by getting the medical practitioners, patients, and caregivers all communicating and pulling together, IgG4ward! will help make progress so fewer patients and their families have the sort of experience you had. On the very positive side, however, it sounds like you are making good progress yourself. That is wonderful!

  2. WOW Kim, I felt every word in your story. Such a scary time for you. I am happy to know that you recognized “something is wrong!”
    Question:
    Sounds like you received a diagnosis and treatment fairly quickly. What was your time to diagnosis?