Challenging Issues in SB Care in Adolescents and Adults : Urology

1. Challenging Issues in SB Care in Adolescents and Adults:Urology Eric S. Rovner, M.D. Department of Urology Medical University of South Carolina, Charleston, SC

3. Medical University of South CarolinaCharleston, South Carolina, USA Me

4. What do we know:Adult SB and Urology • Very little peer reviewed literature in adult SB • Absence of Level I evidenced based medicine for Urologic care in adult SB • General statements • Liptak: Evidenced Based Practice in Spina Bifida: Developing a Research Agenda, May 2003, Washington DC • Merkens et al: Guidelines for Spina Bifida Health Care Services Throughout Life (Draft June 2006) • Some strong opinions about what to do…..but not many!

5. The Adult Urology World for SB

6. Why transition to an “adult” provider? • Need for ongoing f/u of previously treated Urologic conditions • Monitoring of kidney function • Prior surgery, etc. • Continence (creation or preservation) • Need to address potential new Urologic issues • Sexual function • Fertility • Other (prostate, etc.) • Prevention and treatment of Urology problems likely to occur in SB with ageing • Stones, UTI’s, etc.

7. Transitioning to the Adult World Find the right provider…. despite the barriers Patient Economic Physical Compliance Other Provider Economic Medical Complexity Other

8. Key to Urological Health as an SB Adult Be PROACTIVE not REACTIVE

9. Importance of Continued Urologic Care • Renal • Renal function • “silent” problems • Hydronephrosis • Other • Urinary • Continence • Skin/Stoma problems • Post surgical reconstruction: • Rupture, cancer, metabolic, etc. • Infection • Stones • Sexual function • Fertility

10. Kidney: what does it do? • Goal of care is preservation of kidney function • Receives 20% of blood flow from the heart • Function • filters blood: products of metabolism, etc. • Makes urine • Maintains acid-base balance • Produces certain hormones: EPO, etc. • Regulates blood pressure • Regulates fluid balance • Creatinine • Is a rough surrogate for kidney function • Breakdown product from muscle • Normal creatinine for adult male ≈ 0.5-1.2 • But depends on: • age • muscle mass • Other factors (medications, etc.)

11. What can happen to the kidneys? • Infection (acute pyelonephritis) • Reflux nephropathy (chronic pyelonephritis) • Vesicoureteral reflux and infection • Hydronephrosis due to bladder/urethral problems • High pressure voiding/emptying • Obstruction, etc. • Non compliance with catheterization regimen • Hydronephrosis due to ureteral problems • Other: stones, etc. Renal failure → Dialysis → Transplantation

12. Why does this happen to the kidneys? • Lack of attention • Lack of compliance • Lack of follow-up

13. Kidneys and SB • At all ages: renal failure can be a cause of death from SB • Prior to CIC it was THE PRIMARY cause of death at all ages • Risk of renal failure is related to sensory level • Rarely seen with levels below L4 • More common with levels above T10 • Renal failure can occur with even minor neural tube defects such as SB occulta • In one study of SBO in 55 patients* • 43% had urological sx’s • All eventually became incontinent • 8 developed renal failure *Silveri, et al Ped Surg Intern, 1997

14. How to avoid Kidney problems? • Personal • Diet: ↓protein ??? • Fluid management • Hygiene: prevent infection • Adherence/compliance to medical regimen • Void or cath as directed • Take medications as directed • Regular monitoring • Annual check-ups • Renal function studies • X-rays as needed • +/- UDS • Aggressive interventions as needed • Treat infections quickly and completely

15. LEAKS Urinary Incontinence in SB Leakage of urine- lack of bladder control Condition defined by society/societal norms -Bothersome, affects social or occupational functioning Amount (volume/episodes) is irrelevant Incontinence as an adult is abnormal….. and can be treated

16. The Urinary Tract and Continence

17. Normal Voiding Cycle

18. Bladder Control System – Nerves, Spinal Cord and Brain

19. What are the types of UI in SB Adults? Any type of UI may be present: • Bladder: • Overactive (urge incontinence) • Poorly compliant • Underactive (overflow) • (Normal) • Outlet (urethra, sphincter) • Overactive • Fixed, non-relaxing (obstructing) • Dyssynergic (obstructing) • Underactive (stress) • Open bladder neck • (Normal)

20. Importance of urodynamics: • Diagnosis of UI for appropriate Rx • Identification of danger in LUT • Prognosis

21. What happens to the urinary tract with puberty and ageing? • Bladder function does not improve spontaneously • Bladder function may worsen • Incontinence • Renal failure • It may APPEAR to be better….but actually be worse !!!!!! • Prostate growth • ?continence improved in some • Worry about increased DLPP and renal failure • Difficulty cathing • Renal deterioration, when present, is largely SILENT • Must have regular monitoring • Renal function, imaging • Blood pressure monitoring

22. Management of Urinary Incontinence • Behavioral therapies1 • Pharmacologic (drug) Rx • Surgery Directed at the bladder, outlet or both 1. Mattiasson A. Urology. 2000;55(suppl 5a):12-13. 2. Mattiasson A. Neuro Urodyn. 2001;20:403-404. 3. Burgio et al. JAGS. 2000;48:370-374.

23. Despite attempts with conservative/medical tx: Elevated intravesical storage pressure (“Hostile” bladder) Leakage of urine between catheterizations Inability or unwillingness to catheterize urethra Obesity, contractures, mobility issues with ageing Need to divert urine (i.e., pressure ulcer) Other: Catheter complications Recurrent UTI’s (depending on the cause) +/- damaging reflux Urinary Tract Surgery in SB - Indications

24. Surgery of Urinary Tract in SB:Goals • Preserve kidney function • Preserve or create continence (if possible and/or desired) • Low pressure storage of urine • Periodic (or constant) emptying of urine • Awareness or treatment of reflux • Minimize UTI’s, stones, tumors, etc. • Minimize need for reoperation

25. Urinary Tract Surgery in SB Adults • Reconstruction of bladder or urethra or both • Broadly categorized as either: • Continent: • Store urinary safely until a periodic drainage can be effected (usually 4-5 x per day) • Incontinent: • Conduct urine immediately out of the body into an appliance While avoiding: UTI, renal deterioration, odor, skin and stoma problems While minimizing: short and long term costs, inconvenience, metabolic disturbances, stones, reoperation rate, and maintain body image

26. Types of Urinary Tract Surgery • Ureter: • Reimplantation, tapering, etc. • Bladder • Augmentation • Augmentation with catheterizable channel • Other: neurostimulator, Botox, etc. • Urethra • Closure • Sling • Artificial sphincter • BN reconstruction • Other: injectable bulking agent , etc. Individualized

27. Bladder augmentation

28. Artificial Sphincter

29. Artificial Sphincter

30. Long term (adult) postoperative problems OBESITY • Continent diversions: • perforation, difficulties with catherization • Incontinent diversions: • Stoma issues, skin issues, stenosis • Recurrent UTI • Stones • Metabolic issues • acidosis, bone density, etc. • Nutrition: Vitamin B12, folate,, etc. • GI dysfunction/incontinence: diarrhea, malabsorption • Malignancy (cancer) • Other: • Neuro/ortho problems: • Difficulty cathing due to obesity or progressive contractures • Tethered cord

31. What about artificial bladders? • Not yet…..but coming • Tissue engineering

32. CIC : Clean Intermittent Catheterization • Why: • Reduction of pressure by intermittent drainage of bladder • Continence • Avoidance of UTI, hydronephrosis • Clean…..not sterile (usually) • Difficult cases: • Coude tipped, Olive tipped, etc. • Single use closed systems • Hydrophilic catheters • Other issues: • Size of catheter (French) • Type of catheter (silicone, etc.) • Schedule of catheterization • Cleaning of catheters • Changing catheters Individualized: Listen to your provider and BE COMPLIANT

33. Other types of UI management: • Pads • Clamps • Plugs • Pessaries • Catheters: • Indwelling: Urethral or Suprapubic (abdominal) • External (Texas or condom catheters) • SKIN CARE !!!!!!!!!!!!!!!!!!!!

34. ED/Sexual dysfunction in SB • Growing into adulthood, many must address many similar issues to non-SB: • Concerns, fears, self-image, desires, etc • Independence, parental control and “over-protection”

35. Fertility in SB

36. Male infertility in SB • Age of puberty in SB males similar to non-SB males • Unlike females where early menarche is common • 17%-39% of SB males have procreated successfully • Difficulties tied to: • Erectile dysfunction • Ejaculatory dysfunction • Emission failure • Ejaculation failure (retrograde) • Types of failure depends on level • Above T10-L1: emission and ejaculation problems • Below L1: emission OK, RE likely • Decreased #’s of sperm or azoospermic in some • ??? Etiology ???

37. Male Infertility and SB • Retrograde ejaculation • Open bladder neck • Can “wash” out sperm and use • Assisted reproductive technologies • IUI, MESA, ICSI, etc.

38. Female Infertility and SB • Fertility in females less dependent on SB level • Primarily hormonally mediated • 70%-80% of SB females able to become pregnant • More common to have C-sections • Due to bony pelvic disproportion • More common to have UI, UTI during pregnancy • More common to have preterm delivery

39. Female Infertility and SB • OVERALL outcome of pregnancy is not adversely affected

40. Misc. Adult Urologic Issues • UTI • Upper vs. lower urinary tract • Colonization vs. infection • Stones • Hematuria (blood in urine) • Prostate: infection, enlargement, catheterization, cancer • Urologic cancer cases per year in the US: • Prostate (250,000) • Bladder (40,000): especially in reconstructions • Kidney (25,000)