A normal urinary bladder should not cause pain. Pain or hypersensitivity in and around the bladder, with a frequent and often urgent need to urinate – whether sudden and acute or gradually developing and chronic - can have many different causes. It may be related to the urinary tract, the genital tract, the bowel, nervous system or muscles. It may be due, for example, to bacterial, viral or fungal infections, infestations, stones, benign or malignant tumours, endometriosis, systemic autoimmune disease, drugs or chemicals, and including more recently ketamine abuse. Table 1 on page 16 provides a summary of many possible causes of these symptoms. However, if a thorough investigation has revealed none of these disorders, there is another possibility. Interstitial cystitis/bladder pain syndrome (IC/BPS), also known as painful bladder syndrome (PBS) or particularly in East Asian countries hypersensitive bladder (HSB), and sometimes described as chronic pelvic pain (CPP), is a distressing, chronic bladder disorder of unknown cause, with persistent or recurrent symptoms of pain, pressure or discomfort related to the bladder and usually accompanied by a frequent and urgent need to urinate day and night. While the symptoms may resemble a urinary tract infection (cystitis), there is no infection to be seen in the urine and tests reveal no identifiable disorder that could account for the symptoms. What are the symptoms? The characteristic symptoms of IC are: Pain, pressure, discomfort or other unpleasant sensation related to the urinary bladder, a frequent need to urinate (frequency) and/or an urgent, overwhelming need to urinate (urgency) 2 International Painful Bladder Foundation 2014 The pain may be experienced as discomfort or tenderness or irritation or burning sensation in the bladder, in the form of spasms in or around the bladder, or stabbing or burning vaginal pain or may simply be a feeling of pressure on or in the bladder or a feeling of fullness even when there is only a very little urine in the bladder. In many patients, the pain is relieved temporarily by urination, while some patients may also feel strong pain following urination. The pain or discomfort may be constant or intermittent. It may also be felt throughout the pelvic floor, including the lower bowel system and rectum. In some patients the pain may be very severe and debilitating. Other patients, particularly in the early stages, may have milder frequency with/without urgency and without a sensation of actual pain. What they may experience, however, is a feeling of heaviness, fullness, discomfort or pressure or simply the feeling of having an irritated sensation in the bladder. Urinary frequency means that a person needs to urinate more frequently than normal during the daytime and at night. However, this will also partly depend on how much a patient drinks, on the climate where the patient lives, how much the person perspires and on medication the patient may be taking which could have a diuretic effect. In IC/BPS, frequency may sometimes be very severe with some patients needing to urinate 60 times a day or more, but frequency is generally seen as being anything over approximately 8 times a day. However, this figure of 8 voids a day should only be seen as an approximation since the number of voids per day depends on the individual’s way of life and environment. Frequency is by no means always related to bladder size. While some patients have a type of IC/BPS with a shrunken bladder with a scarred, stiff wall (fibrosis) and a small capacity under anaesthesia, other IC/BPS patients with a normal-sized bladder may nevertheless have severe frequency due to hypersensitivity on filling. A typical feature of IC/BPS is the need to empty the bladder several or multiple times during the night. The amount of urine passed may be small, even just a few drops. While a voiding diary can be useful to show frequency and the volume of urine passed, frequency can vary from day to day and week to week, depending on whether the patient’s symptoms are flaring or relatively calm. Urinary urgency in IC/BPS is an urgent or overwhelming need to empty the bladder due to increasing pain or discomfort or other unpleasant sensation that becomes impossible to tolerate any longer, and may in some patients be accompanied by a feeling of stress, malaise and/or nausea. Some patients find that having to postpone urination leads to retention or difficulty in getting the flow started. The nature and cause(s) of this urgency sensation in IC/BPS patients are still not fully understood. · Pain, irritation, pressure, discomfort or other unpleasant sensation that may worsen as the bladder fills; urinating often alleviates the pain and may give a temporary sense of relief; · Suprapubic pain or discomfort, pelvic pain (lower abdominal pain), sometimes extending to the lower part of the back, the groin and thighs; · In women there may be pain in the vagina and vulva; · In men, pain in the penis, testicles, scrotum and perineum; · Both men and women may have pain in the urethra and rectum; · Pain with sexual intercourse in both men and women (dyspareunia); pain on ejaculation in men; · Pain may worsen or be triggered by specific foods or drinks; · A frequent need to urinate (frequency), including at night (night-time frequency or nocturia); · An often urgent or overwhelming need to urinate (urgency). 3 International Painful Bladder Foundation 2014 Currently two main types At the present time, two main types can be distinguished: - the classic inflammatory type with Hunner lesion (formerly known as Hunner’s ulcer) - the non-lesion type Cystoscopic findings are different for these two types and the approach to treatment is also different. Further information is given under Chapter 4 Diagnosis and Chapter 5 Treatment. Current research into subtyping (phenotyping) may lead to the identification of more subtypes. Who gets IC/BPS? Men, women and children, of all ages, worldwide! As diagnosed at the present time, IC/BPS is mainly found in women (+ 80-90%). Approximately 10- 20% of IC/BPS patients are men who may in the past have been incorrectly diagnosed as having nonbacterial prostatitis (inflammation of the prostate gland) or prostatodynia (pain in the prostate gland). A complicating factor is that chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS), also known as prostate pain syndrome, is clinically very similar to IC/BPS and the two conditions have many overlapping symptoms. However, this possibility of misdiagnosis in men may mean that more men may in fact have IC/BPS than hitherto thought and the percentage of male patients with IC/BPS may therefore be higher. A diagnosis of IC/BPS should be considered in men who have pain perceived to be related to the bladder. However, both CP/CPPS and IC/BPS can occur together! IC/BPS is also found in children. However, since in the past the old NIDDK IC research criteria excluded children from studies, many doctors consequently thought that IC did not occur in children. There has therefore been relatively little research or scientific literature on IC/BPS in children in the past two decades and some doctors are still hesitant to give a diagnosis of IC/BPS in a child. It can, nevertheless, occur in children of any age. Many adults with IC/BPS report that they had urinary symptoms in childhood or adolescence. IC/BPS is found in all countries around the world and in all races. However, prevalence figures vary enormously from study to study and country to country and depend on what criteria and definitions have been used for diagnosis, what diagnostic methods have been used to reach the diagnosis and how big the population is in the study. The result is that nobody can say with any degree of certainty at the present time how many people may have IC, although a figure of 200-300 per 100,000 women seems to be a possibility, but it could be higher. Prevalence figures have tended to bundle all patients with a painful bladder together, without making any distinction between subtypes. At present the only distinction usually made is between the so-called Classic IC with Hunner lesions (formerly known as Hunner’s ulcer) and the Non-lesion type in the bladder. A relatively smaller percentage of patients (estimates vary from 10-50%) develop Hunner lesions (note: the old term was Hunner’s ulcer but this was a misnomer since these are not true ulcers. They are today commonly described as lesions). However, while this Hunner type interstitial cystitis used to be considered rare, it is now believed that it may be more common than originally thought. Many researchers now believe that the classic type with lesions and the nonlesion form may be two different diseases. Further information on this is provided in Chapter 4 on Diagnosis. How does IC/BPS start? The symptoms may begin for no apparent reason, or sometimes following surgery, particularly in the case of women following a hysterectomy or other gynaecological operation, after childbirth or following a bacterial infection of the bladder or repeated infections. Onset may be very slow, building 4 International Painful Bladder Foundation 2014 up over many years or it may be sudden and severe. Some patients recall having bladder problems in childhood or adolescence, needing to go to the toilet more frequently than others, long before they developed pain or hypersensitivity. In the very early stages of the disease or in a mild form of IC/BPS, the symptoms may only occur in attacks known as "flares". This leads many patients and their doctors to think that it may be an infection (bacterial cystitis). If the patient fails to respond to antibiotic treatment, it is important for a urine culture to be carried out (not just dipsticks) in order to be absolutely sure that bacterial infection can be excluded. However, the fact that a patient has IC/BPS does not mean that the patient never develops urinary tract infections (UTIs) in addition to their IC/BPS. An infection in a hypersensitive IC/BPS bladder can considerably exacerbate the IC/BPS symptoms, further irritating the already painful bladder. In this situation, following confirmation of an infection, the IC/BPS patient should indeed be treated with a suitable antibiotic to clear up the infection. But the symptoms of IC/BPS do not go away, they keep on returning. In some patients, the symptoms may gradually worsen, but this greatly varies from patient to patient and is not necessarily the case. The symptoms of some IC/BPS patients may increase very slowly over a period of many years or even remain stable and unchanged, while others (with Hunner lesion) may progress from an early stage to an advanced stage with a shrunken, scarred, stiff bladder wall (fibrotic bladder) and small bladder capacity in a shorter space of time. It should be emphasized, however, that many patients never progress further than a relatively mild form of IC/BPS and that many patients have a normal bladder capacity under anaesthesia and never develop a shrunken, scarred bladder and/or lesions. At the present time, there is no evidence to show that patients with non-classic bladder pain syndrome (without lesions) later go on to develop the lesion subtype. These appear to be two separate diseases of the bladder, although the symptoms may be similar. Exacerbation and remission Spontaneous flares and remission are a characteristic feature of IC/BPS in many patients. Many women find that their symptoms are exacerbated just before or during menstruation, during ovulation or if they are taking contraceptive pills. Women may also find that their symptoms temporarily increase while going through the menopause. Any kind of stress, whether physical or psychological, rushing around trying to do too much, can trigger a flare. Many patients also find that a flare can be triggered by certain foods and drinks and even certain medications, irritating the bladder. Cause Despite considerable research into many different aspects of IC/BPS, the cause is still unknown. The many theories include an increase in mast cell activity, an abnormality in the bladder lining causing leakage of toxic elements in the urine through to the underlying layers, neurological (C-fiber upregulation, central pain sensitisation), autoimmunity, allergy/hypersensitivity, occult infection (still being studied) and many more hypotheses. Some researchers have explored the possibility of heredity or genetics playing a role since IC/BPS may occur in more than one person in the same family (mother and daughter or two sisters). Another area of research concerns so-called antiproliferative factor (APF) in the urine of patients which may ultimately lead to a diagnostic marker. It is believed that APF may prevent cells from regenerating and lead to thinning of the epithelium. While once considered specifically to be a bladder disease, a more contemporary way of viewing IC/BPS is within the context of chronic (pelvic) pain syndromes. Research in recent years has 5 International Painful Bladder Foundation 2014 indicated that abnormal nerve activity may be a key factor in the chronic aspect of pain in IC/BPS and the way the pain appears to spread throughout the pelvic floor. At least a subset of patients suffer from one or more non-bladder pain syndromes in addition to IC/BPS and this is currently being looked at by researchers within the framework of the NIDDK Multidisciplinary Approach to the study of the Pelvic Pain disorders (MAPP project) in the United States. Current research is looking at chronic pain and central sensitisation. In summary, there are numerous different theories and much research has been carried out, but no real answers have so far been found. No-one yet knows what causes IC/BPS and whether it could indeed be a collection of different bladder disorders with similar symptoms. It is still an enigma! IC/BPS is not psychosomatic in origin, but can cause great stress and anxiety Although many patients may experience a worsening of their IC/BPS symptoms as a result of physical or psychological stress, it is particularly important to emphasize that stress as such is not believed to be a cause of IC/BPS and it is not a psychosomatic illness. The pain, frequency and urgency and consequent lack of proper sleep experienced by IC/BPS patients and the impact of the disease on every aspect of the patient’s life may themselves be a significant cause of not only total exhaustion, but also stress, anxiety, sleep disorders and depression. IMPACT ON LIFE The IC/BPS patient not only has to cope with the bladder disease itself and all its symptoms, but also the consequences of this disease on his/her life in the widest sense. IC/BPS can have a major impact on the social, psychological, occupational, domestic, physical and sexual life of the patient and affect a patient’s quality of life and the very structure of their life. Where am I going to find the next toilet? The frequent and urgent need to urinate can form an obstacle to work, travel, visiting friends, or simply going shopping. When outside the confines of their home, the IC/BPS patient’s life is dominated by the question “where am I going to find the next toilet?” Before every outing, the patient will carefully plan a network of toilets, known by patients as “toilet-mapping”. Many patients say: “If I don’t think I will be able to find a toilet, I simply don’t go out”. This kind of situation can make a patient uncertain and afraid to leave the safety of their home. Indeed, there are sadly many patients who tell us that they almost never go out. And let us not forget the patients in less developed countries where there may be no public toilet facilities at all. Social isolation The social consequences of IC/BPS should not be underestimated and may force a patient to adopt a completely different life-style. Through embarrassment that they have to use the toilet so frequently, patients may no longer visit even their family and friends. It’s difficult for them to go out to a cinema or theatre or even just for a walk in the park. Their social life may be non-existent and they may feel – and in fact be – totally isolated from the world around them. Some jobs are impossible with IC/BPS 6 International Painful Bladder Foundation 2014 The frequent need to urinate may make it difficult for some patients to carry on working or they may be forced to change to a different type of job that allows them the possibility of easy, frequent access to toilets. Work in some jobs becomes impossible when you have to keep running to the toilet, are suffering from fatigue or are drowsy from pain medication. The impact of IC/BPS on their work and career may mean missed workdays, unemployment and cause patients and their families considerable financial loss. This situation is far worse if the patient has no diagnosis and consequently no access to social or medical assistance. Physical and psychological impact of sleep deprivation and disruption In addition to this, the pain and the frequent, urgent need to urinate make patients tense and exhausted from lack of sleep. Some severe patients need to urinate 40-60 times a day and may sleep no more than 20 minutes at a time at night. Sleep deprivation or disruption can have a detrimental physical and psychological impact on people. Without proper sleep, a person deteriorates both physically and psychologically. This too can make some types of work impossible and even dangerous. See Chapter 7 on Fatigue in IC/BPS patients. Emotional impact, depression and frustration From a patient perspective, the very fact that you have a disease for which there is no known cure makes you very depressed and frustrated. You may feel anger that it took so long to diagnose, that so many doctors may have told you that because they couldn’t find anything wrong, it must be all in the mind, stress, psychological… Although on the one hand, you as a patient know you have these symptoms, you may start to lose your confidence, question your own sanity and feel a sense of uncertainty, anxiety, helplessness, panic attacks, even agoraphobia, while depression can cause complete inertia, closing you off from the world, a situation that is often not helped by family and friends who say that if the doctor claims nothing is wrong, the doctor must be right. In this period of non-diagnosis, you may lose all faith in the medical system and doctors, and feel rejected by this system. The lack of proper sleep makes you continually tired. You may have problems tolerating treatment and may feel sedated and confused by pain medication. The fact that the disease you have affects the bladder and means that you keep looking for toilets makes you constantly embarrassed. Bladder problems are still taboo in today’s world and make you feel stigmatized and isolated from your friends. You long to be able to turn the clock back to when you were normal, and find it difficult to look ahead or make plans for the future. Impact on family life and relationships IC/BPS has an impact on the entire family from many points of view. It alters your relationships with your partner and children because your bladder condition means that you cannot act like a normal parent or a normal partner. As an IC/BPS patient you are tired and irritable from lack of proper sleep, from coping with the pain and from the constant trips to the bathroom. The inability to cope, to look after the family, to do normal things with your partner and children, may create a sense of guilt. Patients may be so anxious about when the next unpredictable flare is going to occur that they try to do too much at home for the family and thereby actually induce a flare. Other patients may suffer from persistent unrelenting pain that makes them so tired that they are continually exhausted. Members of the family don’t understand IC/BPS at all because they can’t see anything wrong on the outside. So unless they are very understanding, they may become resentful at the impact on their lives. If available, family counselling may help family members to understand the problems of the patient and to help the patient to solve the needs of the family in a low-stress way. Sexual relations IC/BPS can have a big impact on sexual relationships since sexual intercourse may be painful for both male and female patients. For some women it may be totally impossible because the urethra, 7 International Painful Bladder Foundation 2014 bladder and vagina are too painful. Anatomically, in women the bladder and vagina are close to each other and this can lead to pain or irritation during penetrative intercourse. In the case of men, ejaculation may cause them intense pain. This is an aspect of the impact of IC/BPS on a patient’s quality of life which is of very great importance. Sex is a normal part of the lives of human beings. If this form of intimacy is taken away, cracks may begin to appear in a relationship about which a patient may be very concerned and feel deeply guilty. Communication between the partners is essential. It is important for patients to be able to discuss this problem with their partner and for them to try to find solutions together, if necessary with the help of a sex therapist or relationship counselling with sex education. Patients themselves may find it difficult or impossible to raise this intimate and embarrassing subject with their doctor. It is therefore important for the care provider to raise this issue. Optimal pain treatment can also help the problem of painful sex in female patients. (However, it should be noted that use of painkillers such as NSAIDs may lead to erectile dysfunction in men!). Tips for sexual intercourse include: a warm bath to relax the pelvic muscles, urination before and after sex, thorough cleanliness by both partners to prevent infection, use of non-irritating lubricants, premedication such as painkillers 20 minutes before sex, engagement in foreplay to limit thrusting time, different positions to reduce pain and symptoms, and thinking of creative ways of sex without vaginal penetration (outercourse) so as to maintain some level of sexual intimacy. Patient support groups Patients and their families need to be well-informed about IC/BPS, its diagnosis, treatment and coping strategies. Patient support groups can play an important role not only in providing this kind of information but also in providing emotional support. Patient-to-patient counselling is invaluable since only another patient truly understands what IC symptoms are actually like and their impact on life. Contact with other patients can be a great relief and a big step forward in learning how to cope.