Treatment will depend on the specific features and symptoms experienced by the affected person but may include:
- Therapies for UTIs, which usually include antibiotics for the treatment and prevention of bacterial infections, and pain relievers (analgesics).
- Specific medications may be prescribed such as anti-cholinergic and alpha-1-adrenergic blockers, to help urine flow more easily.
- Some people may benefit from catheterisation (a procedure that uses a flexible tube, called a catheter, to drain the bladder and collect urine).
- Some people may need to undergo surgery to eliminate urinary tract obstruction and reconstruct certain parts of the urinary tract.
- Those with long-term kidney failure may require procedures that regularly remove excess waste products from the blood (dialysis). In rare, severe cases of kidney failure, a kidney transplant may be considered.
- A high-fibre diet or stool softeners and/or laxatives may be recommended to help relieve constipation. Some benefit from enemas when symptoms are particularly severe.
- An ophthalmologist may prescribe lubricant eye drops to use during the day and eye ointment at night for nocturnal lagophthalmos.
Surveillance
It is recommended that the following evaluations are carried out to monitor an individual’s existing UFS symptoms, how they respond to care and treatment, and whether any new symptoms emerge over time:
- Ultrasound imaging, to monitor for evidence of urinary tract dysfunction, should be carried out at least once a year throughout childhood.
- Kidney function should be monitored, as per the recommendations of a nephrologist and according to whether there is evidence of kidney disease and disease progression. On-going examinations by an ophthalmologist for nocturnal lagophthalmos.
- Assessment for constipation/encopresis at least once a year.
- Certain substances that cause damage to the kidneys (nephrotoxic substances), which includes certain drugs and antibiotics, should be avoided if possible.
Exploratory treatments
Some patients have reported benefits from Botox treatment of the bladder to reduce contractions. Other patients have reported benefits from neuromodulation, that involves surgical implementation of a device to stimulate nerves around the bladder, and is used to treat underactive and overactive bladder.