A child with spina bifida will need careful monitoring of predominantly four body systems: the neurologic, urologic, orthopaedic, and developmental/cognitive areas. It is important for routine evaluation of these systems in addition to well child care by either a pediatrician or a family practice provider throughout the lifespan of the child.

 

The most challenging and complex system to control is GU/GI. The neurogenic bladder in children with spina bifida ranges from incontinence issues to renal damage.

Bowel control/continence is essential to prevent urinary tract infections (UTIs) and social isolation, to name a few.

 

Typically the implementation of an intermittent straight catheterization (ISC) program, commenced in infancy, can prevent many disastrous kidney problems. Yet compliance and hostile bladder environments can wreak havoc on continence issues. Urinary tract infections, pyelonephritis, sepsis, and ureteral reflux are common manifestations of hostile environments that require careful medical attention and treatment. (See below for more information about these specific conditions.)

 

The stable urinary system is essential by maintenance of two urinary goals in children with spina bifida: good renal function and continence. Many urologic teams recommend diagnostic studies every six months to a year to monitor the bladder environment. Common tests include renal ultrasound (RUS), voiding cystourethrogram (VCUG), and urodynamic studies. (See below for more information on these common tests.)

 

“Spindel and colleagues noted that 37 percent of 79 SB infants experienced external sphincter changes during the first three years of life, with the greatest number of changes occurring during the first year,” according to a study by Mourzinos and Stoffel published in 2010. The authors further note: “Untreated urinary storage and emptying pathologies in children with myelomeningocele can affect long-term renal function.”

 

It is important, socially, for the child to either use ISC program from birth or to re-implement or begin at ages three to four to become ready for school-age programs. Many Head Start, preschool, or kindergarten classrooms will not admit an incontinent child or a child that requires diapers for continence. Therefore, preparation is the key to starting a successful continence program. Hence, to prevent further urologic complications it is essential to begin the bowel program at this stage as well.

 

Children with spina bifida, depending on their level of lesion, can be successful on a toileting program. Parents, healthcare professionals and school personnel need to be aware that the successful mastery of this program can be delayed over typical able-bodied peers. Other alternatives for bowel continence are oral medications, suppositories, enemas, and surgical procedures.

 

 

Orthopaedic concerns in children with spina bifida are mostly lower limb deformities or strength issues. Depending on the level of lesion, some common orthopaedic issues are clubfeet, torsional deformities, scoliosis, hip dysplasia or dislocation, knee deformities, feet deformities, insensate skin leading to breakdown even osteomyelitis, or ambulatory issues. Fractures can occur in children with spina bifida, especially femurs and tibias especially in more osteopenic cases like non-ambulators. (Brown, 2001)

 

Neurological concerns in children with spina bifida include chiari malformation, shunted hydrocephalus, and tethered cord. Common are shunt malfunctions/infections. Signs and symptoms of the above can be subtle and vague, mimicking each other.

 

Developmental/cognitive concerns in children with spina bifida include cognitive delays, especially in mastering sequencing, and multiple task completion, math and reading comprehension. Mild or severe cognitive delays can be associated with multiple shunt revisions or seizure disorders. “Neural and cognitive development in children with SB may be compromised not only by the disorder itself but also by hydrocephalus which contributes to variability of neuropsychological outcomes.” (Hampton et al, 2011)

 

Hypertension and obesity can be other medical concerns in children with spina bifida as well. (Mazur, 2011)

 

 

 

A 14-year-old boy presents with spina bifida, recent mellon antigrade continence anema (MACE) but had a chait tube that was changed to a Mickey tube. The family flushes him Tuesday, Thursday and Sunday with 1000 cc saline and capful of miralax. He has a lot of soiling in between after instillation. He sits for three hours on the toilet for this treatment.

 

Mom does note that while standing she can feel a lot of stool in his lower rectum/anus while getting into the shower. He just keeps leaking. Mom tried for the last few weeks to do alternating full capful and half capful of miralax but mom noted he was getting too constipated. He has been dealing with a decubitus ulcer on his sacrum due to this long sitting program. He will be in high school next year. He is smart enough to go to college if he desires and is an ambulator. His shunt was externalized at a recent hospitalization for a shunt infection. He has missed a lot of school due to medical issues and surgeries. He has a mitrofanoff and performs an intermittent straight catheterization program (ISC) every four hours while awake. He has a history of attention deficit disorder and mild Asperger’s syndrome.

 

 

 

 

The team chose to increase his regimen to 1,000 ml of saline plus three caps of miralax plus one teaspoon of salt. Prior to using this we added a bisacodyl enema through the tube. They also increased him to four times per week – Tuesday, Thursday, Saturday and Sunday. The plan is to try to get him down to 30-40 minutes on the toilet, maximum.

 

The KUB test identifies how much is in the bowel and where in the colon/rectum.
The “corn” or sitz test tells you how long it is taking for bowel movements to move through the colon/rectum.
 
3. Is the bowel program of three hours too long to sit? What other options for bowel training could be implemented?
 
Yes, any increased pressure – meaning added weight or sitting in one position for too long – in an insensate child can cause pressure areas, especially sacral. You may want to teach wheelchair push-ups, change positions, use special cushions or other material to decrease pressure to the area. Bowel programs should only take 15-30 minutes at the most for any child or young adult. 
 

4. What preparation strategies for a child with urinary and bowel continence issues to become more independent can be implemented now?
 
Occupational therapists, physical therapists and rehabilitation specialists can assist with proper tools and techniques for dressing, bathing, accessing supplies and equipment for accomplishing tasks like this so the child can, over time, feel confident and master such skills for the rest of their lives instead of constant assistance from parents or caregivers.

Be sure the child/young adult can perform such personal hygiene tasks like opening packages, inserting catheters, inserting suppositories or enemas as well as access to facilities to wash hands, perform task and dispose of biological materials safely.

Once ready for transitions, the team may need to investigate options in the disability office at the apartment or college for dry runs in maneuvering and accessing required facilities to navigate their new environment.
 
5. What tests or knowledge does the child or young adult need to know to troubleshoot shunt malfunction now and once in independent living situation?

Typically most providers recommend yearly radiological testing like shunt series and CT scans to be sure shunt and tubing are functioning and not getting kinked, displaced or disconnected.

Any atypical signs for the patient include: nausea, vomiting (out of proportion with viral illness presence), lethargy, severe headache not resolved with minimal pain reliever, mental status change, slurring words, or acting confused or mixing up statements that should be logical.
 
6. What educational materials could be introduced?

Well-reputed websites like www.sbaa.org and www.uromed.com are good resources. You may consider booklets from respected companies like SBAA, Uromed, Mentor, and Hollister. You can also check local libraries, colleges, hospitals, and Offices of Disability. And you can start a medical notebook or zip drive that contains all pertinent health records and histories to help navigate issue when they occur.

 

 

 

Brown, J. 2001. Orthopaedic Care of Children with Spina Bifida: You’ve come a long way, baby! Orthopaedic Nursing, 20 (4), pages 51-58.

 

Dunleavy, M. J. 2007. The Role of the Nurse Coordinator in Spina Bifida Clinics. The Scientific World Journal; 7, pages 1884-1889.

 

Hampton, et al. 2011. Hydrocephalus status in spina bifida: an evaluation of variations in neuropsychological outcomes. J Neurosurgery Pediatrics; 8, pages 289-298.

 

Mazur, L. 2011, The prevalence of hypertension in children with spina bifida. Acta Paediatrica; 100, pages 80-83.

 

Mourtzinos, A. and Stoffel, J. 2010. Management Goals for the spina bifida Neurogenic Bladder: A Review from Infancy to Adulthood. Urol Clin N Am; 37, pages 527-535.