Earlier this year, the International Continence Society released new terminology standards to aid in the diagnosis of ALL chronic pelvic pain disorders, including those that affect the bladder(1). This is a powerful document that will help physicians and patients identify and classify potential sources of their pelvic pain.
Consider the plight of a young patient with “bladder pain.” Their first stop might be their primary care provider who, usually, will suggest that a UTI is present. Women who seek care for their pelvic pain from their OB-GYN are often told that their reproductive organs are the cause of their distress. Men are labelled as “chronic prostatitis” and prescribed a variety of prostate medications that provide little, if any, relief for their bladder pain symptoms. From rectal distress to pain with orgasm, patients struggle to find comprehensive care, especially when their symptoms cross the lines of several medical specialties (i.e. urology, gynecology, gastroenterology, neurology, pelvic floor, etc.).
Nine Domains of Pelvic Pain
The ICS international committee has defined nine distinct domains which could be contributing to pelvic pain, thus should be evaluated. As you review this list below, ask yourself where do you fit? Which types of pain and/or discomfort do you have?
1. Lower Urinary Tract Domain
2. Female Genital Domain
- Vulva, vestibule and clitoris
- Intra-abdominal female genital pain
- Pelvic floor pain
- Female Sexual Pain
3. Male Genital Domain
- Sexual Pain
4. Gastro-Intestinal Domain
5. Musculoskeletal Domain
- Pelvic muscle pain
- Coccyx pain syndrome.
- Pelvic joint, ligament or bony pain
6. Neurological Domain
- Complex regional pain syndrome (CRPS)
- Somatic neuropathic pain
- Pain following mesh surgery
7. Psychological Domain
- Worry, anxiety and fear
- Depression and depressed mood
8. Sexual Domain
- Sexual desire disorder
- Sexual arousal disorder
- Orgasmic disorder
- Sexual paindisorder
- Chronic pain and fatigue syndromes
- Systemic autoimmune syndromes/disease
- Extraintestinal manifestations of inflammatory bowel disease
You may be asking why there is a psychological domain for chronic pelvic pain. The committee correctly acknowledges that worry, anxiety, frustration, helplessness, hopelessness are often seen in patients with chronic pain of any type. High levels of pain are interpreted as something seriously wrong with the body yet, without an explanation for that pain from their doctors, anxiety can occur. Similarly, depression occurs when patients lose activities that they value and/or if they struggle with finding help for their pain. Catastrophizing is also seen in IC and pain patients where the patient may worry and/or overestimate their reaction to an event (i.e. If I try to travel I’ll have a terrible IC flare.). Properly assessing these symptoms actually strengthens and supports and diagnosis of chronic pain. Treatment, of course, is also important.
Patients who struggle with multiple pain disorders (i.e. IBS, vulvodynia, prostatodynia, fibromyalgia) fall into a distinct subtype for IC/BPS known as functional somatic syndrome. Sometimes inherited or the result of traumatic injury, patients with FSS have unusually sensitive nervous systems. (see below)
Types of Pain
If you talk with a large group of IC patients you notice, very quickly, that they have very different ways of describing their pain and discomfort. Some can locate their pain (i.e. it’s on the right side of my bladder) while others can only vaguely point to the pelvis and/or say that “it burns.” Some experience consistent pain while others struggle with random, intermittent pain. Clearly, a “one size fits all” assumption about bladder and/or pelvic pain just doesn’t work.
A. Nociceptive Pain comes from actual damage to non-neural tissues.
B. Somatic pain comes from bone, joints, muscles, skin or connective tissue. It can cause throbbing, itchy sensations. It’s also easy to locate!
C. Visceral pain comes from hollow organs, such as the bladder or bowel. It can cause generalized cramping which can be difficult for the patient to locate. Visceral pain can come from: (1) direct injury to the organ (i.e. a surgical injury, chemotherapy, etc.), (2) Inflammation (i.e. infection, colitis, endometriosis) and (3) neuropathic/nerve dysfunction (i.e. neuritis following mesh placement).
D. Neuropathic pain can come from an injury to the nervous system and often causes a burning pain. Patients may develop chronic regional pain syndrome where even small stimuli can trigger intense discomfort.
E. Hypersensitivity occurs when nerves become more active even from the smallest stimulation. We see this in patients who have functional somatic syndrome, characterized by very sensitive skin, food sensitivity, chemical sensitivity, drug sensitivity and even smell sensitivity.
F. Central sensitization occurs when the nervous system gets “wound up” and becomes highly reactive. This results in changes in both the peripheral and central nervous system. In many cases, simple ordinary touch or pressure which should not cause pain, does cause pain.
IC/BPS classified as a syndrome, not as a disease
The ICS considers IC/BPS one of the chronic pelvic pain syndromes rather than a disease. They define a syndrome as a “complex of symptoms and signs that collectively indicate a disease, disorder or dysfunction in the absence of obvious pathology.” Dr. Christopher Payne (Vista Urology) recently said “Most patients diagnosed with IC/BPS actually have Bladder Pain Syndrome. The key word is SYNDROME. There is an underlying assumption that this is a disorder of the bladder and is due to chronic inflammation. Both assumptions are generally false and bladder-centric treatments have poor response…. it is abundantly clear BPS (without Hunner’s lesion) is most commonly a complex phenotype of neuromuscular-psychosocial disorder. Other BPS phenotypes that may be explored include neuropathies (pudendal and other), allergy driven BPS, and systemic pain.” (2)
“There is an underlying assumption that this is a disorder of the bladder and is due to chronic inflammation. Both assumptions are generally false and bladder-centric treatments have poor response.” – C. Payne
Hunner’s Lesions May Soon Be Considered Separate Disease
The exception could be the 10% of patients who struggle with Hunner’s lesions which have unique and definitive biopsy results. Biopsies of lesion tissue show deep, profound inflammation that is NOT found in the majority of patients with glomerulations. Dr. Payne recently said “It is clear that ulcerative interstitial cystitis (UIC) is a distinct disorder that is linked to BPS only through common symptoms. In fact, they have little more in common than do meningitis and tension headaches as causes of head pain.” He also said that there is not a single case report of non-ulcer patients progressing to ulcer disease.
It’s worth noting that researchers in Europe discovered that patients with Hunner’s lesions often have the polyoma BK virus in their urine.(3) This virus affects most people during childhood where it establishes a lifelong infection in the renourinary tract.(4) The infection is completely asymptomatic except in patients who are severely immunocompromised (i.e. transplant recipients) where it can cause severe hemorrhagic cystitis.
The Patient Perspective
I’ve had the privilege of working with thousands of patients in the past twenty years through the IC Network thus it comes as no surprise that IC should be considered a “syndrome.” The vast majority of patients report more than bladder symptoms, such as IBS, vulvodynia, pelvic floor dysfunction and the like. I’m one of them. It’s crystal clear that in this group of patients, probably the majority of patients, this “syndrome” involves muscles, nerves, and other related conditions. When bladder therapies are ineffective, it’s time to look beyond the bladder, right?
What makes this terminology guide so powerful is that it gives patients a tool that they can use with their clinicians to say “I have this AND this AND this.” And, I hope, it will spur clinicians who are often absorbed within their own specialty to look outside of the box.
I’m reminded of the dozens of IC patients who had completely inappropriate hysterectomies only to discover, after the fact, that their pain involved their bladder and/or their muscles. Consider as well the millions of women who develop bladder problems after childbirth, often the result of muscle or nerve injuries.(5) I’m also thinking of the IronMan athlete who called last year struggling with severe urinary symptoms. After a year of bladder and/or prostate therapies, he had experienced no relief and reached out to the ICN for help. His symptoms weren’t consistent with bladder wall damage. I suggested that he have a pelvic floor assessment where they discovered that he had extremely tight pelvic floor muscles. Many cyclists also struggle with pudendal nerve entrapment.(6)
What we need are more doctors like Chris Payne, Jeannette Potts, Robert Echenberg, Ken Peters and Robert Evans who are willing to study the entire pelvis, not just the bladder. Each has worked hard to educate other medical care providers about the complexitie