What is Interstitial Cystitis (IC) or Painful Bladder Syndrome (PBS)?
Painful bladder syndrome (PBS) is a long term, painful condition of the bladder the exact cause of which is unknown, although there are many theories as to its cause.
Interstitial cystitis (IC) is a subtype of painful bladder syndrome where specific inflammatory changes in the bladder are present at cystoscopy (a telescope examination of the bladder) with biopsy findings that may include increased numbers of a certain inflammatory cell type (mast cells) in bladder biopsies.
The American Urological Association Guidelines define IC/ PBS as an unpleasant sensation (pain, pressure or discomfort) perceived to be related to the bladder, associated with lower urinary tract symptoms (such as frequency and urgency) present for at least 6 weeks in the absence of infection or other identifiable causes.
IC/ PBS can be associated with irritable bowel syndrome, fibromyalgia, chronic fatigue syndrome and other pain syndromes.
Who can be affected by IC / PBS?
90% of affected patients are women. Patients of all ages can be affected but these conditions occur most commonly in women aged in their 20’s to 40’s.
What are the symptoms of IC / PBS?
The key symptoms of IC/ PBS are pain, frequency of urination and urgency (the urgent desire to pass urine).
Pain in IC / PBS:
- Is felt classically as the bladder fills with urine. Urination usually relieves the pain.
- Is felt above the pubic bone but may also be felt in the vagina and urethra (tube draining urine from the bladder) in women.
- Is felt in the penis, testes, scrotum and perineum in men.
- May be experienced as a discomfort, tenderness, feeling of pressure on the bladder, burning sensation in the bladder or burning vaginal discomfort.
- May be felt in the lower abdominal area and can sometimes extend to the lower part of the back and/ or to the groin or thighs.
- May be constant or intermittent.
- May occur with sexual intercourse in both women and men.
Urgency is experienced as a constant and often intense need to pass urine.
Frequent urination usually occurs both day and night and in some patients may be very severe. It is not always related to a small bladder size and may be due to marked bladder hypersensitivity.
Particularly in the early stages of IC / PBS, some patients may have urgency and frequency without a true sensation of pain. What they may experience instead is a feeling of heaviness or pressure above the pubic bone.
Symptoms can begin gradually or suddenly and with no apparent reason.
In mild forms of IC / PBS or in the early stages of IC / PBS, symptoms may occur as transient attacks known as “flares” which may be mistaken for urinary tract infections. It is therefore important to have a urine culture to help distinguish these symptoms from a bacterial urine infection.
The symptoms of IC / PBS vary greatly from person to person and even in the same individual over time.
What is the cause of IC / PBS?
Unfortunately the exact cause of IC / PBS is unknown although there are many theories of causation and considerable research is ongoing into this condition.
It is becoming more accepted to consider IC / PBS as a condition that is probably due to many factors that result in the bladder wall becoming inflamed rather than due to a single cause.
Theories of IC / PBS causation include:
- An abnormality of leaky bladder lining- thought to be due to a defective inner bladder lining known as the glycosaminoglycan (GAG) layer of the bladder. This is thought to allow the urine to cause irritation of deeper bladder layers.
- Increase in mast cell activity -in the bladder wall could result in the release of excessive amounts of histamines (chemicals which result in inflammation).
- Autoimmunity – in which the patient’s own immune system attacks the bladder. IC / PBS is also more common in women with autoimmune conditions.
- Unidentified infection – due to an as yet undiscovered infective bacterium or virus.
- Toxic components in the urine – are theorized to cause inflammation of the bladder.
- Abnormal nerve activity may be a key factor in the chronic aspect of pain in IC / PBS.
A chemical has been identified in the urine of some patients with IC / PBS called antiproliferative factor (APF), which may be useful in the future diagnosis of patients but currently remains a research tool.
How is IC / PBS diagnosed?
There is unfortunately no simple single test that diagnoses IC / PBS. IC / PBS is essentially a diagnosis of exclusion – meaning that the patient must have:
- The characteristic features on history and examination of IC / PBS AND
- Other conditions that can be confused with IC / PBS are ruled out e.g. overactive bladder, urinary tract infection, other bladder pathologies such as cancer and bladder stones
Some of the tests that are performed to rule out other bladder conditions include:
- Urine samples – to test for bacterial and other atypical urinary tract infections.
- Cystoscopy – or telescopic examination of the bladder lining to exclude other bladder pathologies.
- Cystoscopy and bladder hydrodistension (performed under a general anaesthetic) – during which the bladder is distended with sterile fluid using a telescope. This can help with the diagnosis of interstitial cystitis if there are characteristic findings such as “glomerulations” (pinpoint haemorrhages which may be seen in patients with interstitial cystitis but unfortunately are not specific for this condition) and “Hunner’s ulcer” (focal areas of bladder wall ulceration). Bladder capacity can also be determined under anaesthetic and bladder biopsies taken as well as diathermy of any ulcers performed if necessary. Some patient’s symptoms will also improve after hydrodistension.
- Urodynamic tests – are not always essential and are sometimes used to investigate bladder function particularly if blockage/ retention problems or problems with the nerve supply to the bladder are suspected.
- Potassium sensitivity test – is a controversial test seldom used in Australia that is thought to assess the leakiness of the bladder lining. It is not considered to be sufficiently reliable for diagnostic purposes.
A patient may still have IC / PBS even if all these tests are normal, if they exhibit all the symptoms of interstitial cystitis / painful bladder syndrome.
What are the treatment options for IC / PBS?
There are many treatment options available for IC / PBS. Unfortunately there is no cure for this condition and no single treatment that works for all patients.
The choice of treatment will depend on the severity and type of the patient’s symptoms as well as patient and doctor preferences.
Different treatment options are tried until good symptom relief is achieved. Many patients need combined treatments. It is better to make changes in one treatment at a time to be able to assess its effectiveness.
It can sometimes be difficult to assess the effectiveness of treatments for IC/ PBS due to the natural history of the condition that is characterized by flare-ups and remissions even without any treatment.
Often “multimodal treatment” is recommended for patients with IC / PBS which encompasses:
- Dietary changes
- Alcohol, caffeine, spicy foods, citrus drinks and smoking can all potentially worsen symptoms of IC/ PBS.
- See below – “IC / PBS diet”
- Physical therapies including education in pelvic floor muscle relaxation
- Pelvic floor muscle abnormalities are very common in patients with IC /PBS and are a source of pain that can be managed with the assistance of an experienced pelvic floor physiotherapist. The emphasis is on reducing muscle spasm in the pelvic floor as well as abdominal muscles and hip muscles.
- Physiotherapy management also includes treatment of trigger points and hypersensitive areas.
- Cystoscopy and hydrodistension (bladder distension) – This procedure (performed under a general anaesthetic) can be both diagnostic and therapeutic in IC / PBS. While some patients can experience a flare in symptoms after hydrodistension, many patients can experience symptom relief for many months after hydrodistension. It is, however, common for symptoms to recur at a later date.
- Diathermy of inflamed areas (“Hunner’s ulcers”) can significantly reduce bladder pain.
- Cystoscopy and biopsy also excludes other bladder conditions that can be confused with IC/ PBS.
Normal bladder lining appearance at cystoscopy.
Inflamed bladder appearance after cystoscopy and hydrodilatation consistent with IC/ PBS.
Inflamed bladder appearance after cystoscopy and hydrodilatation consistent with IC/ PBS.
- Oral medications including:
- Amitriptyline (Endep®) – is a very effective first line oral medication in treating bladder pain and frequency (especially frequent night time urination).
- Anti- histamines - have been used for their ant-inflammatory effect.
- Elmiron® (pentosan polysulfate or PPS) – is one of the few drugs approved specifically for use in IC/ PBS. It is thought to act by helping to restore a deficient glycosaminoglycan (GAG) lining to the bladder over a period of several months.
- Bladder instillations – which involve the instillation of a chemical solution directly into the bladder using a catheter (temporary tube placed in the bladder via the urethra). This gives a high dose of medication into the bladder with minimal absorption of the treatment into the bloodstream to minimize potential side effects. Treatments used include:
- DMSO or dimethyl sulfoxide – a chemical solvent given as a once weekly instillation into the bladder over 6 weeks. It acts to reduce inflammation and bladder pain.
- DMSO “Cocktails” – where DMSO is mixed with other agents such as heparin (a blood thinning medication thought to help restore the bladder GAG lining) and steroids (which have an anti-inflammatory effect).
- Chlorpactin®- is a bladder instillation given as a single treatment under a general anaesthetic with the patient fully asleep as it is painful to instil.
- iAluRil®- is a bladder instillation administered over months containing chemicals which aim to help replace a potentially deficient GAG layer in the bladder.
- Pain management - which may include use of specific pain medications and consultation with a specialist pain management physician.
- Supportive therapy and stress management
- Although stress is not the cause of IC / PBS, the condition is a major cause of stress. Addressing stress, depression, anxiety and relationship difficulties that can occur in combination with IC /PBS is an important aspect of treatment.
- Relaxation techniques that can help improve overall coping with chronic medical conditions such as IC /PBS include meditation, exercise and muscle relaxation techniques.
Other treatments which are less commonly used for IC / PBS include:
- Botulinum toxin (Botox®) injections into the bladder – which can result in poor bladder emptying (urinary retention).
- Neuromodulation – in which electrical stimulation can be used to try and “recondition” nerves that control bladder function including:
- InterStim® - which is approved for usage in the urinary tract to treat only detrusor overactivity and urinary retention in Australia.
- Experimental treatments – Many have been tried with variable results in patients with refractory IC / PBS who have failed all the above treatments prior to consideration of major surgical options. Such treatments include
- Immunosuppressant drugs such as Cyclosporine A and prednisolone.
- Hyperbaric oxygen therapy
- Major bladder surgery – is rarely required in IC / PBS. It is considered only in the very small minority of extremely severe cases of IC / PBS where the patient’s bladder is small and scarred resulting in low bladder capacity. Surgical options vary from complete bladder removal (cystectomy) with a urinary stoma / bag or bladder reconstruction surgery.
What is the IC / PBS diet?
Many patients find that the following foods and drinks can worsen their symptoms of IC / PBS:
- Carbonated drinks
- Alcoholic drinks
- Very spicy foods
- Citrus fruit
- Acidic foods and drinks e.g. Vitamin C tablets
These foods and drinks do NOT, however, affect all patients with IC / PBS and an item that may cause a flare of symptoms in one patient, may have absolutely no effect on another.
The only way to test a person’s sensitivity to various foods and drinks is to exclude them from their diet for a period and then slowly introduce them individually and monitor the response. It is a matter of trying to see what diet suits an individual best without becoming paranoid about everything that is eaten and drunk.
What is the relationship between IC / PBS and Stress?
IC / PBS is not caused by stress, but emotional and physical stresses can certainly cause flare-ups of the condition.
IC / PBS is in itself a potential severe cause of stress to any patient due to the pain, urinary urgency and frequency as well as lack of sleep that the condition can cause. The fact that the precise cause of the condition is unknown, and the treatment is often variable and unpredictable can cause any patient anxiety and concern about their future.
Management of stress is vitally important in improving how an individual manages their IC / PBS and copes with the potential problems that it can cause.
What is the relationship between IC / PBS and Urinary Tract Infections (UTIs)?
IC / PBS flares can be mistaken for a urinary tract infection (UTI), hence the need for urine culture before treating symptoms with antibiotics.
IC / PBS patients can also experience UTIs that can result in flares of the condition. An infection in the already sensitive IC / PBS bladder can result in marked pain and exacerbation of symptoms. It is imperative in these situations to perform a urine culture and treat any UTI appropriately with antibiotics.
While some patients can develop the onset of IC / PBS symptoms after a severe bacterial infection of the bladder, most patients with IC do not show a clear relationship between UTIs and the cause of this condition.
What is the outlook for a patient with IC / PBS?
The course of IC / PBS is extremely variable.
- Many patients never progress further than a relatively mild form of IC / PBS and maintain a normal bladder capacity. Symptoms may, however, recur and then go into remission over the course of years.
- The symptoms of some IC / PBS patients may increase very slowly over a period of many years
- It is rare for patients to progress from an early stage to an advanced stage of IC / PBS (with a small capacity, scarred and stiff bladder) in a short period of time.
- It is not inevitable that a patient affected by IC / PBS will develop a small, scarred bladder.
What causes flares and remissions of IC / PBS?
The spontaneous flares and remissions of IC / PBS characterize the condition in many patients. In some women changes in sex hormones seem to affect the bladder and this can result in exacerbations of symptoms:
- Before or during menstruation
- During ovulation
- While taking the oral contraceptive pill
- Temporarily during the menopausal years.
Other common potential triggers for flares include stress, dietary changes, change in medications and sexual intercourse.
When a patient experiences an acute flare of symptoms, it is useful to have a urine test performed to exclude a urinary tract infection as the cause of worsened symptoms.
What can be done to help at the time of a flare of symptoms?
- If a severe flare of symptoms occurs, it is important to exclude a urine infection by having a urine specimen taken with the local doctor and start antibiotics if needed.
- Simple analgesics – Over the counter medicines such as paracetamol +/- codeine are very effective painkillers.
- Anti-inflammatory medications e.g. over the counter Naprosyn®, Voltaren®
- Amitriptyline (Endep®) can be used for symptom flare e.g. for period of a week if the patient has used this medication before.
- Hot baths
- Cold or hot packs
It is important to remember that flares will eventually settle.
Other sources of information about IC / PBS
- International Painful Bladder Foundation
- Conquering IC
- Pelvic Pain Association of Australia
- Interstitial Cystitis Association
These websites have contact details for support groups for IC / PBS. Many patients with IC / PBS find it very useful and supportive to make contact with fellow IC / PBS patients who can give both practical and emotional support.
What can patients do to help themselves?
It is important to be positive, patient and proactive in approaching IC / PBS. While no single treatment works for everyone, with time and trying different strategies all patients can be helped.
It is important for patients to be involved in their treatment and try and define which symptoms are the most bothersome, so that various strategies can be tried to target those symptoms.
The aim is not to let the bladder and IC / PBS symptoms control an individual’s life. A positive and flexible attitude as well as learning techniques to cope with the stress caused by IC / PBS will help improve coping with this condition.