National Patient Experience Week – An IgG4-RD Perspective

donna an igg4-rd patient

In the world of rare disease, there is nothing more important than the patient’s voice. From using your voice to help receive an accurate diagnosis and communicate symptoms with your care team, to advocating for yourself, there’s power in what you have to say.  

Donna, a post-anesthesia care unit (PACU) nurse who lives with IgG4-RD uses her voice this National Patient Experience Week to share her IgG4-RD diagnosis story, her care journey, and her critical patient perspective.   

Journey to Diagnosis 

I'm pretty lucky that I was diagnosed about eight months after the start of everything, but it was a tumultuous eight months of not knowing what I was going through.  

It started when I noticed I was having pain in my upper right abdomen after eating. I wondered if this could be a gallbladder issue, so I called my nurse practitioner, who ordered an ultrasound which revealed I had severe right hydronephrosis, (a lot of urine around my kidney because it wasn’t draining into the bladder.)   

We thought it was a kidney stone, so I had a CAT scan, which showed there was no stone. However, there was a mass in the bottom third of my ureter and it was suspicious for cancer. By this point it was October and I called to make an appointment with the urologist and was told I wouldn’t be able to see a doctor until February. I was thinking, “Someone told me they think I have cancer and now I have to wait three months.” 

Fortunately, I emailed a doctor from the hospital where I work and was able to be seen two days later. During that appointment, it was decided I’d receive a stent to drain the urine, and they’d also do a biopsy. They successfully inserted the stent; however, it took a second surgery to get a biopsy. That biopsy was inconclusive, so from there, I had a PET scan, which prompted my physician to question if I had retroperitoneal fibrosis.   

I didn’t know what retroperitoneal fibrosis was, so I did some research and learned I should go to Mass General for the best care. A couple more months went by, as did more tests, and though my urine was draining properly, the damage that had been done to my kidney was irreversible. My team still didn’t have a concrete answer to what was wrong with me, so with the consensus of my surgeon at the tumor board, it was decided to remove both my kidney and my ureter and biopsy anything that could be biopsied.   

When they did the operation, the surgeon was surprised to see fibrotic tissue in my retroperitoneal space. There was so much they couldn’t remove my entire ureter, so they removed my kidney and the top two-thirds of my ureter and sent that for biopsy.

It took two weeks for the biopsy results to come back. For those entire two weeks, I was convinced that I wasn’t going to make it to the next year.

I remember sitting on my couch, getting the notification that the results were in. I decided to rip the band-aid off and read the results. I scanned through them, and at the bottom, it read “no malignancy detected.” What an indescribable relief!

While my physician wasn’t originally convinced it was IgG4-RD, since my scans didn't show anything indicating IgG4-RD, he was after reading the operative report from my surgery. From there it was decided I’d begin treatment right away with both steroids and rituximab infusions.

The rituximab infusions weren’t considered successful based on my symptoms a couple of months later. The steroid treatment helped reduce the pain, but being on steroid treatment can have other negative effects. I started another medication, mycophenolate mofetil, and unfortunately, I’m allergic to it, so now, I’m still looking for the next treatment.

Donna’s IgG4-RD Patient Experience

As someone living with IgG4-RD, I think it’s important for healthcare providers to know that it’s a chronic disease. It's lifelong, and we're still working on ways to not only diagnose but to treat it and manage it. I've spoken with other people living with IgG4-RD and they’ve said when they go to the doctor, they don't wear makeup or dress up because if they look okay, their physicians might think, “You don't look sick. You must be fine. It can't be that bad.”
I’ve also realized in talking with others that I had more symptoms than I thought I did. I work the night shift so what I had originally thought was me just being tired was actually more than that
I used to be able to work three nights in a row and be fine. I can't do that anymore. I can't recover from that. And that's the thing that’s been noticeable for me.

Advice for Others

My best advice is to become your own expert. Nobody knows your body better than you, so don't be afraid to speak up. If you feel like you're not being heard, find somebody who will hear and understand you. Hopefully with all the education happening now around the disease, and the IgG4ward! Foundation, it will be easier to educate providers and this disease can be more top of mind for them. IgG4-RD affects so many organs, so neurologists, pulmonologists, immunologists, nephrologists, and more need to know about this disease so they can refer you to the right people. Also, connecting with other people with this disease is incredibly helpful too.

The Power of Exemplary Care

I get amazing care at Mass General with Dr. Wallace. I can message him, and he's always responsive and answers any questions I have. I’m also grateful to my urologist for thinking out loud. If it wasn't for my urologist mentioning retroperitoneal fibrosis, I wouldn’t have found the care that I have now. Speaking with my doctors and tackling this together is so helpful, and I hope others can have a similar experience.

Whether a physician, caregiver, or someone living with IgG4-RD, we invite you to join our Online Community.  

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