Rare Global Advocacy Leadership Symposium 2019

"A rare disease is rare but the global rare disease population is the third biggest population after China and India," said Nicole Boise, the Founder, and CEO of Global Genes during her speech at the Rare Global Advocacy Leadership Symposium. She also added that rare diseases impact more people than Cancer and AIDS patients combined, therefore #rareisnotrare. 

Nicole Boise, Founder and CEO Global Genes

This was for the first time that the Patient Advocates in India came together for a meeting. The event was an eye-opener and a lot of knowledge sharing happened. The resolution was taken to request the Government to make changes in their existing programmes. Each disease group spoke about their unique challenges and finally, a common agenda was set to accommodate all diseases under a common umbrella.

Patient Advocates

Founder of Interstitial Cystitis India Balaka Basu was also present in the meeting and she raised the issue that a common portal should be created for patients where they can seek help for their conditions. She also added that MBBS syllabus should include a special paper on rare diseases so that a better awareness among the medical fraternity can be created. 

Arindam Moitra from Indian Alliance of Patients Group (IAPG)

Balaka Basu, Founder of Interstitial Cystitis India

All the patient groups agreed that research is required to find cure and treatments for the diseases. Clinical trials should be waived for a few groups of patients on compassionate ground. An action plan was also chalked out under the leadership of ORDI Founder Prasanna Shirol and Co-founder Harsha. Patient's groups have taken the responsibility to take specific action in the next quarter. 

Mr. Prasanna Shirol, Founder ORDI

Group Photo

Kegel or Reverse Kegel for Interstitial Cystitis Patients?

Kegel exercises are designed to tighten the pelvic floor muscles. It is frequently used to treat
 bladder incontinence. However, usually IC patients have a tight pelvic floor muscle and Kegel can actually cause more harm than good. Kegel’s can cause further muscle tension and muscle spasms. But, there are few patients who benefit from Kegel only if they do it under a trained supervisor. Especially patients whose only symptoms are frequency and urgency do benefit from Kegel.  

Ideally, patients of Interstitial Cystitis benefit more from Reverse Kegel and many physiotherapists recommend this to them. Reverse Kegels are the opposite of standard Kegels. Reverse Kegels focus on releasing and relaxing the pelvic floor muscles. Traditional Kegels focus on contracting and releasing the pelvic region. Therefore Reverse Kegel can help relieve pelvic pain and tension as well as increase flexibility. Both types together can help balance the pelvic floor. Reverse Kegels, in particular, may help make sex more enjoyable for women with dyspareunia (pain during sexual intercourse). 

People often clench their muscles tightly during tension. People normally notice this in their neck and shoulders but if noticed carefully then the pelvic floor muscle also gets tightened. Some people refer to this pain as “A Headache in the Pelvis.” This is a significant concern for people with underlying issues such as constipation, interstitial cystitis, and dyspareunia. Reverse Kegel helps in relaxing the muscle and therefore it is extremely beneficial for IC patients whose muscles are usually tight. David Wise in his book of the same name A Headache in the Pelvis: A New Understanding and Treatment for Prostatitis and Chronic Pelvic Pain Syndromes explained this beautifully.

10 Job Management Tips for Interstitial Cystitis Patients

Working on any job with chronic illness can be tough and have its own challenges. Here are some tips to manage your job if you have Interstitial Cystitis:-

1.  Make your employer aware of your health condition. Many of them have management policies and can be very understanding to help you with your needs.

2. Carry a card that lists your health condition and has the name of your doctor, emergency contact and medication that you are currently taking. It can be handy under emergency situations.

3. Emergency bathroom access letter: You can ask your urologist to sign a card/letter for washroom access. In this card, the urologist can mention that you need emergency bathroom access as your health condition doesn't allow you to hold for a long time. Not only in the office but it can also help while traveling or when you need to use public restrooms. Unlike West, these kinds of cards are an alien concept in India but you can always ask for a letter from your doctor. The letter should have the contact number, registration number and the seal of the urologist.

4. Keep yourself hydrated. It will help with your frequency and burning. The more concentrated urine is, the more painful it becomes. Also drinking enough fluids help to maintain focus, alertness and better health.

5. Take frequent breaks from your desk job. If you are doing a desk job, sometimes you have to sit in the same position for long hours. And this can worsen your pelvic floor dysfunction. So once in a while, get up from your seat, walk or stretch a bit and come back.

6. Relaxation exercise: In today's scenario when the work pressure is a lot, it's easy to get stressed up. That's why it is important to relax. Do a five-minute mindfulness exercise, deep breathing, doodles or anything that helps you to calm down.

7.  Carry your emergency stuff like icepack/ heating pad, antispasmodics, emergency medicines, essential oils, sodium bicarbonate/ baking soda with you. So if you have a risk of flaring, you are already prepared. The following heating pad is good for office use. You can put it on your chair and sit on it during a flare.




8. Wear comfortable clothing. Avoid tight fitted jeans and skirts. They can put lots of pressure on the pelvic area.  Use clothes that have soft fabric, allow air to pass and doesn't carry chemical fragrances such as detergent, perfumes, etc. So there are fewer chances of infections.

9. Carry your own food to avoid food induces flares. Outside food ingredients are not always known to us and may have trigger ingredients, therefore, it is best to carry food from home.

10. Lastly, always Prioritize your health. Don't hesitate and feel guilty while taking a Sick Leave. Remember you are trying the best and doing a great job. You and your health matters.

Article contributed by Arun Dahiya.

Instillations in the Bladder

Bladder Instillations come under the second line of treatment in Interstitial Cystitis. It is offered when the patient doesn't have relief from the first line of treatment like diet modification, lifestyle changes, stress management, and oral medications. 

In Bladder Instillations, a combination of drugs is delivered to the bladder to calm down the inflammation and protect the GAG layer temporarily.  The most common drug cocktail that is used is the combination of Lidocaine, Heparin, sodium bicarbonate, and Pentosan Polysulphate solution. This is administered to the bladder through a catheter and it takes around an hour to deliver it. After that, the patient is asked to hold it as long as possible. It is suggested that the patient should try at least half an hour to hold it before they pass urine. 

Apart from this, there are other kinds of combinations of drugs like DMSO or Dimethyl sulfoxide. However, many feel that DMSO is outdated and they prefer new drugs. There are doctors who use their special cocktails. For example, In the USA Dr. Christine Whitmore and Dr. Robert Moldwin use their own cocktails. In Hungary, Dr. Sandor Lovasz has invented a new device for bladder instillations where the patients can self catheterize the cocktails. In India Dr. Nagendra Mishra also has his own cocktail that he claims relieve patients for a long duration of time. You can read the interview with Dr. Mishra here.

The following video is on the self-catheterization invented by Dr. Lovasz.

Hunner's Lesion

Hunner's Lesion was previously called Hunner's Ulcer or Hunner's patches. Some call them a subtype of Interstitial Cystitis while other's call it a completely different disease. It is believed that 5-10% of IC/BPS patients suffer from Hunner's Lesion. Patients with this form of IC have more severe symptoms than patients with nonulcerative IC. 

Hunner's Lesions are distinctive areas of inflammation on the bladder wall and, like an ulcer, they can bleed, ooze, and may vary in size. Hunner's Lesion was first detected by Guy LeRoy Hunner in 1915.

The following definition by Magnus Fall was accepted by ESSIC: "Hunner lesion typically presents as a circumscript, reddened mucosal area with small vessels radiating towards a central scar, with a fibrin deposit or coagulum attached to this area. This site ruptures with increasing bladder distension, with petechial oozing of blood from the lesion and the mucosal margins in a waterfall manner. A rather typical, slightly bullous edema develops post-distension with varying peripheral extension". 

Hunner's Lesion can be detected with the help of Cystoscopy with Hydrodistension. However, many experts believe that lesions can be successfully seen without hydrodistension. 

As per ESSIC, In urology centers with the expert skills to detect Hunner lesions, Hunner lesions are detected in about 50% of the patients with BPS. The majority of BPS/IC patients with Hunner lesions, however, are probably not recognized in centers with less experience. This under-diagnosis is probably due to a combination of factors such as:

• The confusion caused by the name Hunner's ulcer while it is not an ulcer: the term Hunner's ulcer suggests that it can always be seen at cystoscopy without hydrodistension
• Hunner lesions can remain undetected at cystoscopy without hydrodistension
• Many urologists suppose that Hunner's lesions are rare; the fact that they rarely detect them is considered to be in line with this false impression
• Even when cystoscopy with hydrodistension is performed, Hunner lesions are likely to be detected mainly by experienced urologists. A biopsy may be necessary to prove that it is a Hunner lesion and/or to exclude a carcinoma in situ.

Source: ESSIC website.

On 17-18 April 2018, ESSIC (European Society for the Study of Interstitial Cystitis) in collaboration with ICICJ ( Interstitial Cystitis Japan) and  SICJ (Society of Interstitial Cystitis of Japan) organized a conference on Interstitial Cystitis/Bladder Pain Syndrome with the theme as Hunner's Lesion in Kyoto, Japan. Dr. Rajesh Taneja and Dr. Nagendra Mishra from India were present at the conference. There it was discussed that with Narrow Band Imaging (NBI) bladder lesions are easily and clearly recognized. 

Treating Hunner’s ulcers can reduce symptoms significantly. The ulcers can be removed through fulguration (burned off with the use of electricity or a laser) or resection (cutting around the ulcer, removing both the ulcer and the surrounding inflamed tissue). Some ulcers may recur in the same location though. Cystoscopic injection of triamcinolone or Kenalog is also being researched.

Reference: IPBF, ICA, Wikipedia, An Overview of Diagnosis and Treatment, Jane Meijink, August 2018.

GAG Layer and Interstitial Cystitis- The Co-relationship

GAG is short for Glycosaminoglycans.  Normally, the inner wall of the bladder is coated with  GAGs that are secreted from cells of the bladder lining.  Like other organs, the bladder's inner surface is lined with epithelial cells. This mucus-like layer or GAG protects the epithelial cells, by preventing acids and toxins in the urine from coming in contact with this layer of tissue.

Source: Women's Health Matters

When a person has interstitial cystitis (IC), these epithelial cells become irritated and inflamed. Areas of pinpoint bleeding called glomerulations, or larger sores, called Hunner's ulcers, may appear in the lining of the bladder.

The cause of IC remains unknown; however, some researchers speculate that when the protective GAG layer of the bladder gets disrupted in some way, allowing substances in the urine to irritate the underlying epithelial cells it may cause burning and urgency. When the GAG layer is damaged, the bladder lining is no longer protected from urine and the waste products contained in it. The delicate epithelial cells are exposed to urinary toxins that may cause burning. The bladder would probably try to rid itself of the burning urine as quickly as possible through spasms.  As the epithelial cells of the bladder wall are damaged, they may become scarred and stiff. This makes the bladder less flexible and less able to expand. In some cases, the nerves in the bladder wall may also be irritated or damaged, prompting the urge to urinate more frequently.

The GAG layer gets damaged due to various reasons like:

• An unidentified infection, possibly a viral infection, may disrupt the GAG layer or damage the wall directly.

• Some substance in the urine may be causing the damage. Free radicals, environmental toxins, certain foods, bacterial toxins and certain drugs (e.g. antibiotics and radiation) may contribute to GAG and epithelium damage in the bladder.

• After initially responding to a bladder infection, the body's immune system may have begun to attack the bladder's tissues or the GAG layer.

• Stress may play a role, it can suppress the immune system, allowing pathogens to take hold and cause damage. Moreover, dietary absorption of nutrients may be reduced, potentially reducing the availability of raw materials for the GAG layer.

• Immune system cells, called mast cells, have been found in higher concentrations in the bladder walls of a patient with IC. These cells release histamines, which may be partly responsible for the inflammation of the bladder wall.

Tips to improve the GAG layer:

• GAG replenishment therapy has shown promise for interstitial cystitis relief in a few studies. 
• Intravesical (directly into the bladder) administered hyaluronic acid or a hyaluronic acid-chondroitin sulphate combination seem to be effective.
• Aloe vera or slippery elm may bring some relief and protection to the bladder lining in the absence of a functional GAG layer. However, they will not repair the GAG layer.
• Eating a nutritious diet can help supply the raw materials needed to make a strong GAG layer.
• Removing toxins and irritants may help to reduce further damage and irritation.

Source: Perth Urology Clinic

Diagnosis of Interstitial Cystitis

Diagnosing Interstitial Cystitis is not easy for the medical practitioner. For most doctors, it takes time to reach the final diagnosis of IC. As interstitial cystitis (IC) symptoms are similar to those of other disorders of the bladder and there is no definitive test to identify IC, doctors must rule out other treatable conditions before considering a diagnosis of IC. The most common of these diseases in both sexes are urinary tract cancer and bladder cancer.

Unlike a urinary tract infection, interstitial cystitis cannot be diagnosed with a simple urinalysis or urine culture. Rather, it’s a diagnosis of exclusion, which means that the urologist will first take a thorough history and then perform tests designed to rule out other diseases. These include infection, bladder stones, bladder cancer, kidney disease, multiple sclerosis, endometriosis, and sexually transmitted diseases. As IC has overlapping symptoms with other diseases the doctor would follow a process of elimination to establish IC.

Pelvic Exam: First the doctor would do a pelvic exam. During a pelvic exam, the doctor examines external genitals, vagina, and cervix and feels the abdomen to assess internal pelvic organs.

Urine Tests: The urologist would initially ask you to do a urine routine test and urine culture. This would help them to rule out Urinary Tract Infection.

USG of KUB: Ultrasonography (USG) of the KUB (Kidney, urethras, bladders) helps them to rule out any abnormality in the KUB. It would also help the doctor see the post void urine volume.

Cystoscopy with hydrodistension: The doctor may possibly do a procedure called cystoscopy with hydrodistension, which is performed under general anesthesia. In cystoscopy, the doctor inserts a thin tube with a tiny camera (cystoscope) through the urethra, which allows the doctor to see the lining of the bladder. Along with cystoscopy, your doctor may inject liquid into your bladder to measure your bladder capacity. Your doctor may perform this procedure, known as hydrodistention after you've been numbed with an anesthetic medication to make you more comfortable. Interestingly, distending the bladder can itself be therapeutic. About half of the patients get some relief for about three months after the procedure. The most common sign of interstitial cystitis is red pinpoint spots of blood (glomerulations) covering much of the bladder wall surface. Sometimes there are scars or lesions called Hunner’s ulcers, accompanied by low bladder capacity due to tissue stiffening (fibrosis).

Biopsy: During cystoscopy, the doctor may take a biopsy (tissue sample) of the bladder to rule out bladder cancer and look for evidence of the mast cells that indicate an allergic reaction or autoimmune response.

Urodynamic Studies: An urodynamic study is not essential to diagnose IC, however, it remains important for the confirmation of the clinical symptoms of IC. A urodynamic study may also be useful in selecting the therapeutic modalities for IC.

Potassium Chloride Sensitivity Test: Few doctors may also want to do a potassium chloride sensitivity test. In this test, your doctor places two solutions — water and potassium chloride — into your bladder, one at a time. You're asked to rate on a scale of 0 to 5 the pain and urgency you feel after each solution is instilled. If you feel noticeably more pain or urgency with the potassium solution than with the water, your doctor may diagnose interstitial cystitis. People with normal bladders can't tell the difference between the two solutions.

Cystoscopy- Frequently Asked Questions

We receive numerous questions from patients regarding Cystoscopy. Many patients are scared to undergo this particular procedure and often stop going to the doctor once Cystoscopy is advised. Therefore, let us learn a little more about Cystoscopy in this article.

What is Cystoscopy?

A cystoscopy is a procedure done by a urologist, a doctor specializing in the urinary system, to look at the inside of the bladder and the urethra. the urologist uses a scope to look at the inside of the bladder and also it's lining and in the urethra. The procedure may also be done to remove a bladder stone, take tissue for biopsy or to place a catheter inside.

When is Cystoscopy done?

Cystoscopy is done to check for any problems in the bladder and its lining. It is recommended for the following issues:

• Frequent urinary tract infections (UTIs)
• Hematuria, or blood in the urine
• Urinary frequency, or urinating more than 10 times a day
• Urinary urgency, or the sudden, strong urge to urinate
• Urinary retention, or when the bladder does not empty completely
• Urinary incontinence, or urine leakage
• Pain or burning before, during, or after urination
• Trouble starting the flow of urine, completing urination, or both
• Abnormal cells found in a urine sample

What is Cystoscopy with Hydrodistension?

Under general or regional anesthesia, this procedure uses a cystoscope to look inside the bladder after slowly stretching it with fluid (hydrodistention). This allows the physician to see changes inside the bladder that is typical of IC, including the presence of pinpoint-sized red marks on the bladder wall, also called glomerulations or petechial hemorrhages. This procedure also allows doctors to see Hunner’s lesion's(patches), which are present in a small number of people with IC.

Is Cystoscopy with Hydrodistention mandatory for the diagnosis of Interstitial Cystitis?

There is a huge dispute regarding this. A section of the medical practitioners believes that cystoscopy is not mandatory and IC should be diagnosed by following the process of elimination. Whereas another segment strongly believes that without Cystoscopy it is impossible to diagnose Interstitial Cystitis, especially it is required to check if the patient has Hunner's Lesion.

The American Urological Association’s guidelines for IC consider cystoscopy with hydrodistention under anesthesia to be a third-line treatment for IC. This means that less invasive therapies should be attempted prior to this procedure. It may reduce pain and discomfort in some IC patients, which can last 3 to 6 months. However, not everyone benefits from this procedure, and it may take up to several weeks to notice any symptom improvement. 

How is Cystoscopy done?

The cystoscopy procedure usually takes about 30 minutes and is done on an outpatient basis. The urologist will recommend that the patient empty his or her bladder before the procedure begins. The step-by-step process may be similar to this:

1. The patient will be lying on an exam table. The urologist may place some gel or a local anesthetic in the patient’s urethra to aid in reducing any discomfort while the procedure is taking place.
2. The urologist will gently insert the cystoscope through the urethra into the bladder. The patient may feel discomfort or a pressure sensation. The cystoscope is a long, thin tube with a lens on one end that the urologist looks through or, on a camera-equipped scope, visualizes on a monitor. The other end of the cystoscope that is inserted into the urethra has a tiny lens with a light that allows the urologist to look inside the urethra and bladder. There are two types of cystoscopes. One has a flexible insertion tube, while the other is stiff.
3. Once the cystoscope is inserted, the urologist will need to instill some sterile water or a normal saline solution from the cystoscope into the bladder. The water/saline fills and stretches the bladder so the urologist can get a better view of the bladder wall.
4. As the liquid enters the bladder, the patient may again feel some discomfort as well as the urge to urinate. If necessary, the urologist can remove some of the liquid from the bladder during the procedure. Once the procedure is over, the urologist may drain the patient’s bladder, or ask the patient to use the bathroom to urinate before he or she leaves the office.

During the brief procedure, the urologist examines the lining of the urethra as the cystoscope passes through it and then into the bladder. Once the cystoscope reaches the bladder, the urologist examines the lining of the bladder. During the procedure, the urologist can remove a bladder stone or take a biopsy if needed.

How the patient may feel after the procedure?

Typically, a cystoscopy is done in the urologist’s office and afterward most patients go home the same day as the procedure. Sometimes after a cystoscopy procedure, the patient may:

• Feel a burning or soreness around the urethra.
• Feel slight burning while urinating.
• Notice small flecks of blood in the urine.
• Feel mild discomfort in the bladder area or kidney area when urinating.
• Need to urinate frequently or urgently.

These problems should not last more than a day after the procedure. If pain persists, bloody urine lasts longer than 48 hours, or the patient develops a fever, the patient should call the doctor. If necessary, the urologist may prescribe an antibiotic to take for a couple of days after the procedure to prevent an infection. If you have severe pain, chills, or fever (these could be signs of an infection), it is important to call the urologist’s office and explain the symptoms.

Basic Symptoms of Interstitial Cystitis

Basic Symptoms of Interstitial Cystitis are quite similar to Urinary Tract Infections (UTI).  No wonder most doctors get confused. Most IC patients are treated for UTI with antibiotics for a considerable period of time with minimal or no results. They undergo urine routine test, urine culture and Ultrasonography (USG) of the KUB (kidney, urethra, and bladder) and in most cases nothing significant is found in these reports leading to more confusion. Both the patient and doctor gets frustrated by this time. Some doctors eventually declare the symptoms as imaginary and refer the patient to a psychiatrist however compassionate doctors go ahead and do their bit of research, trial and error and diagnose the disease themselves or refer the patient to a specialist. 

The Basic symptoms are as follows:

• Frequent urination, often of small amounts, throughout the day and night (up to 60 times a day in severe cases)

• A persistent, urgent need to urinate

• Burning while passing urine

• Urethral burning

• Pain or discomfort while the bladder fills and relief after urinating

• Pain in the pelvis or between the vagina and anus in women

• Pain between the scrotum and anus in men (perineum)

• Pain in the lower abdomen

• Pain during sexual intercourse

Pain is a common factor in all patients and therefore IC is also known as Bladder Pain Syndrome (BPS). The above are the basic symptoms, however, the symptoms are often unique to each patient and they may have all the above or some additional symptoms like burning feet, backache, nerve pain, etc. Most IC patient has comorbidity like Irritable Bowel Syndrome (IBS), Fibromyalgia, Vulvodynia, etc along with IC and therefore they experience cumulative symptoms. 

If you are experiencing these basic symptoms for a longer duration of time then please go and visit your doctor to rule out IC or any other related disorder.