My 35-year battle with a malformed urinary bladder

About six to eight times a day, depending on your fluid intake, diet, activity level, medications and health conditions, you feel the urge to go for a short call.

This natural process happens when your bladder fills up with urine. As it gets fuller, tiny sensors in the bladder wall send signals to your brain, letting you know it’s time to go to the bathroom.

At first, you can ignore the feeling, but as the bladder keeps filling, the urge becomes stronger and harder to ignore. When you finally go to the bathroom, muscles in your body relax to let the urine out. 

But as basic and almost mundane as it is, it's something Ann Gitau, 35, has never and will never experience.

 Born with bladder exstrophy — a rare condition that left her bladder unable to function—Ann has been navigating her whole life with catheters tethered to her body. A urinary catheter is a tube that is inserted into one’s bladder, allowing urine to drain freely.

When the Healthy Nation team meets Ann, a mother of two and the last born in a family of three, in Kiandege, Naivasha Sub-County, she is preparing to empty the drainage bag attached to her catheter. When she is done, she thoroughly washes her hands and settles for the interview.

She begins narrating how, immediately after birth, her parents realised she had an opening from her belly button to her vagina and needed an urgent surgery to close the opening.

"My parents told me that we stayed in the hospital for a month as I was being examined, where the doctors noted that my bladder had not fully formed, hence it could not store urine." 

Bladder exstrophy, seen in approximately one in 10,000 to 50,000 births, is defined by Johns Hopkins Medicine as an abnormality in the formation of the bladder and the bony pelvis. 

Instead of forming in its normal round shape, the bladder is flattened and exposed on the abdominal wall. Additionally, the pelvic bones are widely separated.

For this reason, Ann could not regulate her urine, and it flowed out continuously without her knowledge. "My parents kept on changing my clothes and when I was eight years old (in 1998), they took me back to hospital, where I had a surgically created opening (stoma) in the abdomen to allow urine to pass through to a suction catheter. Also, the doctors made a pouch-like structure, with my intestines, to act as a bladder to hold urine."

This, she says, made her join school when she was 10 years old. While in school, Ann had to be on her toes and ensure she emptied the suction catheter on time; failure to which urine would leak, forcing her to go home.

When she was in Class Seven, Ann's parents bought her a urine leg bag, which held the urine that drained from her catheter and fitted well under her clothes.

In high school, the challenges persisted as every time she took a cold shower, she would remove the catheter and upon returning it, she would bleed. This challenge made life harder for Ann, who chose to drop out, get married and start her family. 

Excited about this new chapter, the couple's sex life was strained as they were unable to have penetrative sex. This was because her vaginal opening was narrow as a consequence of the bladder exstrophy and the previous procedures performed to correct it.

She left her marital home and sought medical assistance from her doctor, who advised her to undergo another surgery to open up her vagina and allow for normal intercourse.

"After surgery and healing, I was supposed to be given a metal dilator (a device used to stretch or enlarge an opening) to ensure my vaginal opening does not close up, but I was not given.”Ann cites negligence, saying the doctors failed her at this point.

“A week later during my check-up, the doctors noticed that my vaginal opening had closed up, and advised me to go for another surgery, which was successful."

In 2019, Ann stopped using the suction catheter as she had outgrown the normal sizes and switched to a foley catheter - a soft, flexible tube that drains urine from the bladder into a collection bag. 

"As I grew up, so did the stoma, and I kept using a bigger suction catheter. When I got to size 22 of the catheter, I could not find a bigger size so I started size 22 of the foley catheter," she explains.

However, later that year and in 2020, she would feel as though the right side of her stomach was swollen although she was not in pain. Early 2022, the swelling started being painful and when the pain became unbearable, she decided to seek medical attention.

Upon tests, doctors could not find any issue, but accounting her history, they suggested a ureterosigmoidostomy surgery, where urine is redirected to flow from the kidneys to the sigmoid colon, the final portion of the large intestine.

"During the surgery, the doctors realised that if they tampered with the pouch that was initially created for storing urine, I could bleed to death. So, they aborted the plan," she recalls.

As months passed, she went back to hospital, where a test showed that she had kidney stones. She underwent laser lithotripsy, a minimally invasive procedure that uses a laser to break up kidney stones so they can be passed in urine or removed.

"When they were halfway done, the doctors informed me that my heart was failing, so they stopped the procedure and said it would be resumed after six months."

Back home, Ann continued experiencing severe pain. "I could not bend or work." 

She went to a different hospital for a second opinion, where a CT scan revealed a large and small kidney stone, with the large one blocking the flow of urine. As a result, the urine was backing up into the kidney, causing her right kidney to swell.

"In February 2023, the doctors put nephrostomy tubes - thin, flexible tube that drains urine from the kidney into a bag outside the body - on my back so that both kidneys do not fail."

Last year in July, Ann's nephrostomy tubes were replaced and the big kidney stone was removed. 

"They told me the small stone would dissolve on its own so I just stayed in the hospital for two days after the surgery then went home. Unfortunately, two days after discharge, the wound opened up and my waste was coming through it. I was taken back to hospital and the doctors told me it was fistula. I was hospitalised for a month until the wound recovered."

Was she told what caused the kidney stones? Healthy Nation asked her.

"Yes. They told me they were caused by urine residue and also infections from the catheters. So, I was advised to take a lot of water to clear the kidneys and change the catheters after two or three days."

Ann empties about three bags a day if she drinks enough water. If not, she empties the bag once. 

All her life, Ann is not supposed to wear trousers or tight clothes because of the tubes and bags. Also, she has to carefully sit down in a way that will not open the urine bags. In a week, Ann changes the catheter twice and urine bags three times.

"The catheter costs Sh80 and the urine bag Sh50.

“The urine bags are way cheaper than the leg bags - smaller drainage bags attached to the leg to collect urine during the day and it allows one to move around more easily - which cost Sh1,500.”

Ann has since stopped using the leg bags owing to their high cost.

“If I do not change the urine bag, it will emit a foul smell and the catheters can cause infections."

For Ann, her appeal to the government, especially the Ministry of Health, is to give her catheters for free as she will use them all her life. "Sometimes I do not have money to purchase them and other times they are not available. So, I'm always susceptible to infections. Previously, I have had to stay with one catheter for like a month and it really caused me problems."

She also appeals for financial support from well-wishers so she can buy catheters and urine bags.

Doctor's take

Dr Eric Irungu, a consultant paediatric surgeon at Aga Khan University Hospital, defines bladder exstrophy as a rare congenital condition that is part of a group of a spectrum of conditions called Exstrophy Epispadias complex.

For children who have bladder exstrophy, for one reason or the other which doctors across the world don't understand, the bladder does not form in the correct place (inside the pelvis within the tummy) and does not form a round structure.

So, when the baby is born, its parents may be able to see the abnormality of the external genitalia.

"They will have a red patch or a defect in the anterior abdominal wall or below the belly button, what we call the umbilicus. That red patch, which is now the open bladder, will be leaking urine continuously," he says.

While there is no particular cause, Dr Irungu says there are some associations with genetic factors and some environmental factors, but there's still no concrete evidence.

"Some of the risk factors include being born into a family where there is a history of bladder exstrophy. If a parent initially had bladder exstrophy and then they get children, the young ones have a higher chance of developing the condition."

The other category of people who may be at a slightly higher risk are those who have assisted pregnancies. Dr Irungu says these are people who had to undergo in vitro fertilisation (IVF) to get pregnant.

"For most babies who are born with this condition, especially within Africa and other low-and-middle-income countries, the diagnosis is often made at birth," he says.

“However, in some centres where there's good antenatal follow-up with skilled personnel, it can be picked up between 20 and 25 weeks on ultrasound, whereby doctors will try and see if the baby has a bladder and if it is filling and emptying as normal.”

Bladder exstrophy can only be corrected through surgery. One of the aims of the surgery is to reconstruct the urinary system and the genitalia. First, by making the flat bladder be a balloon-like structure so that it's able to collect urine.

"Second, to reconstruct the urethra - the urinary passage through which the urine will leave the bladder and go out. Next is to reconstruct the external genitalia. This is because the urethra normally passes through the penis in males and next to the clitoris in females, so surgery helps to make it look as much as possible as that of a normal person."

Dr Irungu says the other aim of surgery is to close the anterior abdomen. On top of that, the hip bones (that form the pelvis) are also opened up. “We also have to close the bones so that we recreate that pelvis. This helps the bladder to go back inside, after which we bring the muscles and the skin back together.”

Since there are very few patients with this condition, doctors all over the world are yet to fully agree on the best way to go about the surgeries.

Dr Irungu says: "There are some people who do all the operations in one sitting and that's in the early newborn period, mostly before three months of age. And there are some who stage the procedure. So, you do one stage immediately after birth within the first week or so and then you do the second stage after about six months to a year, and then you do the last surgery later in childhood, when a child is ready to start potty training."

Additionally, Dr Irungu says that currently there is no way of preventing bladder exstrophy because the cause is yet unknown. 

So, what are some of the complications that come with the condition? "One major problem is that children born with bladder exstrophy may have difficulties controlling their urine like other normal children, even after undergoing successful surgery. This is because the muscles that control urine did not also fully develop well."

Another challenge is having recurrent urinary tract infections, development of kidney and bladder stones and complications from surgery.

wkanuri@ke.nationmedia.com