A Veteran’s Fight Against Retroperitoneal Fibrosis

Dick Cee, a veteran and former medical professional, has shared the reality of living with Retroperitoneal Fibrosis, from painful treatment to its lasting emotional and physical toll. We hope his story not only answers questions readers may have, but also highlights the strength required to face RPF. Thank you, Dick Cee, for sharing your experience.

Diagnosis:

How did you first become aware that something might be wrong?

Had vague discomfort in lower left intestines, which I thought could be an ulcer, never having had one. Also testicular tenderness and different urination pattern, which I figured was just old age (70 at the time).

What symptoms led you to seek medical attention?

 Bad kidney pain which I thought might be a kidney stone, never having had one. Went to ER where they took images but did not diagnose RPF.

Time from first symptoms to diagnosis? And what tests/procedures confirmed your diagnosis?”

The above ER visit, which included imaging, did not diagnose RPF. While waiting for an appointment with a urologist nearly two months out, my left leg swelled up horribly, forcing a return to a different ER where new images identified a growing mass which was causing the iliac vein to be blocked enough to impact my leg. This time RPF was diagnosed. It was nearly five months from initial symptoms described above. I was hospitalized, and while medical people discussed who should perform a biopsy to see if the type of RPF mass was idiopathic or malignant, I insisted they start treatment for idiopathic RPF, since if it was malignant, I would surely die soon anyway. The location of the mass in the vicinity of the abdominal aorta caused the various experts to want to pass the buck.

How did you feel when you received your diagnosis?

Since the initial physician identifying the mass said, "If I were you, I'd start getting my affairs in order," I knew it was no minor condition with a guarantee or likelihood of getting on with my life. Thus, I expected the disease to kill me. Having survived the Vietnam War as an infantryman and numerous other traumas, I just thought I'd been lucky to live as long as I had and my time had surely come this time. I felt sad to have to abandon my wife. I've always been OK with the fact I'm going to die, even though I have fought for my survival during trauma.

Treatment

What treatment options were presented to you?

 Due to location and nature, surgery was out of the question. Thus, the only option was Prednisone, which was immediately augmented with azathioprine to reduce the amount of Prednisone necessary to achieve regression of the mass.

Which treatment(s) did you choose and why?

 It was pretty much take the combo I describe or do nothing.

How did you feel about the information and support provided by your healthcare team regarding your treatment options?

The rheumatologist to whom I was assigned had no previous experience with RPF. I felt like she had attempted to conduct serious research into current treatment protocol, but I wasn't particularly confident in my treatment. The rheumatologist I describe had horrible patient interaction and clearly should have chosen a different career. I worked in the medical field. I know a crummy physician when I see one.

Any side effects or complications from the treatment? If so, how were they managed? How did they impact you?

I had horrible side effects from treatment with Prednisone, and the azathioprine was probably instrumental in causing me to have skin cancer in multiple locations. The Prednisone made me constantly want to cry or kill as though I was possessed; I got myopathy to the point I could hardly move; permanently damaged eyesight; and the myopathy was so severe that continually a turd could slip out without my knowing it other than the after-effect. I have a fat ring on my abdomen that was never there before and bags under my eyes, both of which affect my self-image and self-esteem. While actively taking Prednisone I always felt HORRIBLE!

How effective was the treatment in managing your symptoms?

With all of the above said, the drug regimen worked perfectly, removing the disease and allowing me to live. The problem was wanting to live during the treatment and in the aftermath.

Outcomes

How has your condition changed since starting treatment?

Being afflicted with classic old age in the midst of two bouts of RPF and two bouts of Polymyalgia Rheumatica (PMR) has left me permanently compromised, to say the least.

Have you experienced any relapses or improvements?

 Relapse as indicated above. I have improved regarding putting the RPF into regression and remission, but it has taken its toll with the side effects of the treatment combined with aging. I have experienced degenerative spinal disease, which I believe was exacerbated by all the sitting I had to do while the treatment was taking place.

How often do you need to follow up with your healthcare provider?

I see a rheumatologist every six months. My primary care provider knows little to nothing about the disease.

What has been the most challenging aspect of managing your condition?

If I don't take an autoimmune suppressant, it is clear the disease, PMR, or both will return, and the autoimmune suppressants of any type have undesirable side effects.

Quality of Life

How has Retroperitoneal Fibrosis affected your daily life and activities? and how has your condition impacted your mental and emotional well-being?

Very compromised physically and emotionally from multiple treatments, but I am aware that the age of onset of the disease and the number of years under siege while simultaneously entering serious old age, the cause and effect is not so clear.

What support systems (family, friends, support groups) have been helpful to you?

Great support from my spouse who has continually told me I am actively bringing something to her life, but has given me permission to call it quits if the ongoing fatigue of old age and dealing with the disease is more than I am interested in engaging.

Are there any lifestyle changes you have made to better manage your condition?

I have given up many things, to include vigorous exercise, which means I won't get hurt falling, but there is not a second that all of it does not cause depression.

What advice would you give to someone newly diagnosed with Retroperitoneal Fibrosis?

Good luck.

General Thoughts

How has your condition impacted your caregivers or family, and what challenges have they faced?

My wife has had to endure periods of my being quite compromised physically, knowing the physical disability made (makes) me suicidal even without the Prednisone. RPF really took me down overall.

What do you think could be improved in the diagnosis and treatment process for Retroperitoneal Fibrosis?

 I will say that before a competent physician figured out what my problem was, one bozo physician wanted to put stents in my ureters without addressing the cause of the restriction, and another physician said my spleen, which I still have, should be removed because it was damaged. So I guess my advice would be move cautiously with a second opinion.

Is there anything else you would like to share about your experience?

It has clearly been life-transforming at multiple levels.