What is Spina Bifida?
While it primarily effects the brain and spinal cord, it also impacts related body systems, such as the bowel, bladder, swallowing and skin to name a few. How these changes affect one child can vary greatly.
Scottish Rite treats neural tube defects including:
- Caudal regression
- Tethered cord
Interdisciplinary Care for Children with Spina Bifida
- Spina Bifida Team
- Clinical Nutrition
- Therapy Services
- Transition to Adult Service
Spina Bifida Team
Our Spina Bifida Program was established in 1992. For the past 30 years, our Spina Bifida clinic has continued to provide coordinated care to help each family and child navigate a successful life.
The Spina Bifida Program at Scottish Rite for Children provides a multidisciplinary team of experts from Developmental Pediatrics, Neurosurgery, Urology, Orthopedics, Physical and Occupation Therapy, Orthotists, and nurses, speech therapists and medical social workers to help treat each child’s individual needs.
The goal is to eliminate hostile bladder dynamics through medical management, reduce the need for surgery, maximize renal outcome, limit the effects of urinary tract infections and prevent antibiotic overuse, and establish a care routine that allows for urologic independence.
For children with spina bifida, we regularly take a renal/bladder ultrasound, perform urodynamic testing and take a serum creatinine to measure the renal function.
Urodynamics is a pressure test of the bladder. “Uro” means urine, or related to the urinary system. “Dynamics” means moving against pressure.
The lower urinary system consists of the ureters, bladder, urethra and sphincter muscle. The bladder is a balloon-like organ with a muscular wall that expands and contracts. Urine received from your kidneys is stored in the bladder. The urine travels down tubes called ureters into your bladder. Urine is stored in the bladder until you decide to empty it. Emptying occurs by a contraction of the bladder muscle. A bladder contraction is similar to making a fist with your hand and squeezing it - the bladder contraction “squeezes” the bladder to empty the urine.
The sphincter [SFINK-ter] muscle sits right below the bladder. If you are not ready to empty your bladder, then the sphincter muscle tightens up, like a knot at the bottom of a water-filled balloon. When you decide that it is time to empty your bladder, the sphincter muscle opens up, like untying the balloon.
Urination begins as the bladder pushes the urine out down a tube called the urethra. The urethra opens to the outside of your body and urine is expelled from the body. Urination takes place only when the muscles and nerves are working correctly. Messages are constantly being exchanged between the bladder and the brain through a pathway of nerves in the spinal cord. Sometimes a head injury, stroke, or spina bifida will cause problems with a nerve pathway. Then, the bladder may not empty correctly. Persons sometimes have incontinence (leakage of urine) before or after having bladder or back surgeries. They need testing, called urodynamics, to determine how well the nerves and muscles are working.
The urodynamic test takes about one hour. Children may drink and eat a light breakfast or lunch before the test. If your child takes a bottle or pacifier, it is helpful to bring those with you. If your child takes medicine to help the bladder muscle (such as Ditropan®), this will need to be given two to three hours before the test. If your child has frequent problems with constipation, you should contact the nurse to receive bowel clean-out instructions. Children who have bladder control should not urinate for two to three hours before the test; they will be asked to empty their bladder into a special commode at the beginning of the test. This instruction does not apply to babies or children who have no bladder control.
One small tube, called a catheter, will be inserted into your child's bladder. It will be used to fill the bladder with water and measure the movement of the bladder muscle as it fills and empties. A very small tube will be inserted into the rectum. It will measure the pressure put on the bladder when straining or coughing. Two patches or wires, similar to an EKG test, will be placed at the rectum or at the opening of the urethra. These will monitor the sphincter muscle and its ability to hold urine in the bladder. Any discomfort felt during the test will go away within minutes following the test.
As the bladder is filled with water through the catheter, the patient may be asked to describe what the bladder feels like. The patient may be asked to strain or cough several times. When the bladder is full, the patient will empty it. Some tests are performed using X-ray dye instead of water, and X-ray pictures will be taken throughout the test. The entire test will be recorded on a computer, and the patient can watch on a screen as it is occurring. Everything will be explained by the health professional as the test is being performed.
The catheters will be removed when the test is over. The patient may have a little discomfort where the catheters were placed. This should last only a few hours. A warm tub bath will help to ease any discomfort. Drinking an adequate amount of fluid (usually eight to ten glasses) is important (especially water) within 24 hours after the test. This will help prevent a urinary tract infection. An antibiotic medication may also be given before or after the test.
The patient may continue their diet, medications and activities as normal, unless given other instructions by the doctor. After the test, the doctor will explain his or her findings and discuss treatment options.
Nielson-Ormeis, Betsy, “What is Urodynamics?”. Quality Care, Vol. 14, No. 3. pg.4, Summer 1996.
- Bruce Schlomer, M.D.
- Micah Jacobs, M.D.
- Yvonne Yuh-Ru Chan, M.D.
Our Neurosurgery team helps manage the child’s shunts and other nervous system impairments, such as Chiari II malformation, spinal cord syrinx and tethered cords.
A ventriculoperitoneal (VP) shunt is a tube with a valve that relieves pressure in the brain cause by hydrocephalus. It moves CSF away from the brain and into the abdomen.
Below are common signs of hydrocephalus or shunt problems in patients with spina bifida.
If these symptoms occur, call your doctor or the Spina Bifida Clinic nurse coordinator.
|Bulging fontanelle (soft spot)||Headaches||Headaches||Headaches|
|Swelling or redness along shunt tract||Swelling or redness along shunt tract||Swelling or redness along shunt tract||Swelling or redness along shunt tract|
|Change in appetite||Decreased school performance|
A programmable shunt is a specific type of shunt that can be adjusted from outside the body. These valves are designed to be adjusted by a strong magnetic field found in the external adjustment tool. Some of these valves may be susceptible to adjustment by strong environmental magnetic fields and care must be taken to keep toys with magnets and other sources of magnetic fields away from the implanted device.
If you child has a programmable shunt and receives an MRI, they need a Neurosurgeon present to check their setting after the procedure is complete.
The term “tethered cord” means the spinal cord can no longer move freely as the child bends and moves. All children with spina bifida are at risk for tethered cord. Some develop symptoms and problems with this.
• loss or change in how the legs work
• change in bowel or bladder pattern
• back pain
• unusual or severe pain in the legs and feet
• scoliosis that gets worse very quickly
• change in arch of feet or running in of ankles, curling of toes
These symptoms usually occur slowly over time and are progressive.
The eight pairs of cervical nerves control movement and feeling in the shoulders, arms and hands.
Cranial Nerves (Brain Stem)
The 12 pairs of cranial nerves control movement feeling in the head, face, neck and arms.
The five pairs of lumbar nerves control movement of the hips, legs and feet.
The 12 pairs of thoracic nerves control movement and feeling in your chest.
The six pairs of sacral nerves control the bladder, sexual organs and the colon. The first pair also helps control movement and feeling in the feet.
AFP is a protein made by a developing fetus. The level of AFP may identify a possible neural tube defect.
Anencephaly is a severe neural tube defect that occurs early in the development of an unborn baby. Anencephaly occurs when the upper portion of the neural tube fails to close. A large part of the brain and skull does not form. These babies do not survive.
Cerebrospinal fluid (CSF)
CSF is a clear liquid made in the brain that flows around the brain and spinal cord and then is reabsorbed.
Chiari II malformation
Chiari II malformation is a condition in which the cerebellum portion of the brain protrudes into the spinal canal. It is seen in most people with spina bifida.
Lipomas are benign, slow-growing tumors that come from fat cells. They are not cancer. A lipoma in the spinal canal can cause spinal cord compression.
Meninges are membranes that cover the brain and spinal cord.
Meningocele is a form of spina bifida in which the covering of the spinal cord pushes through an opening in the spine forming a fluid-filled sac. Typically, there is no nerve damage. Surgery is usually needed to remove the sac.
Myelomenigocele is from the Latin words for “spine” and “swelling.” This is usually referred to as spina bifida. A baby is born with a sac protruding from an opening in the spine. This sac contains nerves and part of the spinal cord.
Neural tube defect
The neural tube is a narrow channel that folds and closes between the third and fourth weeks of pregnancy to form the brain and spinal cord of the embryo. A neural tube defect occurs when the neural tube does not close. Examples of neural tube defects are anencephaly, MMC and meningocele.
A pseudocyst refers to a collection of fluid around the bottom opening of the VP shunt. It can create abdominal pain and/or cause the VP shunt not to work as well.
Syrinx refers to a fluid-filled pocket in the spinal cord. It can press on the spinal cord and cause damage.
- Brad Weprin, M.D.
- Dale Swift, M.D
- Brett Whittemoore, M.D.
- Angela Price, M.D.
- Bruno Braga, M.D.
The Scottish Rite for Children staff (doctors, nurses, dietitians, therapists, pharmacists, psychologists and child life specialists) have developed an informational program for you and your child. The bowel program we use at Scottish Rite is like a set of building blocks. It begins with your child’s daily routines and habits and builds to include fluids, fiber, exercise, toileting and medication.
Bowel management continues throughout life. As your child gets older and things change, we will help you and your child make adjustments to the bowel program. The more consistent your child is with a bowel program, the more successful it is!
Maintaining good nutrition is an important aspect of any child’s health. A balanced diet will help assure that a child receives all the calories, protein, vitamins and minerals that he/she requires. In turn, this will promote growth and brain development.
The unique challenges associated with spina bifida mean nutrition plays a critical role in assuring each child develops appropriately and has an amazing quality of life. Because of this, a registered dietitian will meet with you regularly during your clinic visits. The dietitian will be able to educate you and your child from infancy to the late teen years on the role food, fluid and nutrients play in maintaining his/her health.
Drinking adequate fluids is an essential part of feeling well. Water is essential to digestion in preventing constipation, promoting urinary health and delivering essential nutrients throughout the body.
- Add fruits, herbs or spices to water such as lemon, lime or apples and cinnamon.
- Use sugar-free flavoring liquids or packets.
- Carry and refill a water bottle throughout the day.
What is fiber?
Fiber is the part of food that is not completely broken down by the digestive system, therefore it is not absorbed by the blood stream. Fiber stays in the intestines and acts like a “broom” to help move waste through the digestive system.
Types of fiber:
• Helps to slow down the movement of food through the digestive system, makes the “fullness” feeling last.
• When bowel movements are loose, soluble fiber soaks up the water to make stool firmer and slower to pass.
• Helps speed food through the digestive system.
• Insoluble fiber mixes with other waste materials in the intestines, adding bulk. This makes larger and softer bowel movements.
Most whole foods contain both types of fiber which help promote regular bowel movements. However, not enough fluid combined with a high fiber diet can make constipation worse.
Ways to increase fiber
- Eat foods that are naturally higher in fiber - fruits, vegetables, grains, beans or nuts.
- Choose “whole grains” versus processed grain - 100% whole wheat bread or brown rice.
- Ellen Dillard, R.D.
- Josiah Baker, R.D.
Our OTs can perform catheterization screenings to assess appropriate underlying skills needed for self-catheterization, help coordinate equipment concerns if not able to ameliorate in clinic and check-in periodically for any needs. Should your child need any ongoing services, our OTs can either refer to outpatient services at Scottish Rite for Children or another appropriate provider depending on needs presented.
The undeveloped or damaged nerves cause differences in the sensation or feeling in an individual's skin. This often leads to wounds and pressure sores.
Pressure ulcers occur when there is constant pressure on the skin, especially over bony areas such as the tailbone, hip, spine, knee, ankle, heel, shoulder blade or back of the head. The skin gets squeezed between the bony area and something hard (like a mattress, chair, brace, cast, swimming pool ledge or foot plate of wheelchair). Skin breakdown can also happen in skin folds.
- Shear — tissue rubbing against tissue, causes deep wounds
- Friction — rubbing a body part against a surface
- Pressure — skin pressed between bone and a hard surface Heat exposure — sunburn, hot water, hot cement etc.
- Moisture— increases shear/friction effects (urine, stool, sweat, and wound drainage)
What are the risk factors for pressure ulcers or skin problems?
- Body parts with little or no feeling—usually buttocks, legs and/or feet
- Movement — using a wheelchair, “bunny hopping” or commando crawling
- Incontinence — urine and bowel movements can cause skin irritation
- Use of braces — especially in the first month or after six or seven months of wear
- Being underweight or overweight
- Summer — more wounds occur during this time due to outdoor activities or heat
- Check the skin each morning and each evening for red or open areas. Teach your child to use a skin inspection mirror to view hard-to-see areas.
- Use backpacks and cup holders on wheelchairs—do not use the wheelchair seat as a storage area.
- Check the wheelchair cushion each day to make sure it’s in good working order. Contact the wheelchair vendor or therapist if the cushion needs to be repaired.
- Remind your child to do weight shifts every 15 minutes with all activities—while in the wheelchair, biking, horseback riding, etc.
- Use custom gel pads over bicycle seats and horse saddles to reduce pressure to the buttocks.
- Prevent burns by using sunscreen when outdoors. Hot sand, hot concrete, hot wood, hot plates or hot metal can burn skin, too. Know where your child’s feeling starts and stops.
- Leave wheelchairs in shady areas when not in use to prevent burns from skin contact with the metal parts of the chair. Feel the wheelchair cushion for hot areas.
- Wear swim shoes for all pool activities to prevent scrapes on the feet.
- Check the braces often to see if adjustments need to be made for growth.
- Use barrier cream for bowel cleanouts and for incontinent episodes. Keep your child on a bowel program.
- Wear shoes and socks always! Use long pants for crawling.
- Do extra skin checks after periods of high activity.
- Keep your child at a healthy weight.
- Talk with medical or nursing staff about your child’s wound risk factors prior to any surgery.
- Share these ideas with teachers and school nurses so care can be taken.
II. Psychotropic Medication use in Children and Youth with Spina Bifida
II. Psychotropic Medication use in Children and Youth with Spina Bifida
Transition to Adult Service
- From infancy to toddlerhood, and from young child to teenager
- From preschool to high school, to job training or college and beyond
- From living at home, to becoming a member of the community
The goal of transition at Scottish Rite is the gradual developmental of self-directed care responsibilities from the adult to the young adult.
Through this process, your Spina Bifida team at Scottish Rite plans to give optimal support to the youth/young adult and the family. This will be based on individual need within the context of the medical health system, community, and further education or workforce or other activities appropriate to the individual.
This process will involve multiple steps, and that is why it is important to address transition at every clinic visit. Formal planning for transition in the program begins at about age 14 – or sooner if the situation demands.
It will be important to set aside time with our social worker during your annual visits. Our social worker is our key resource to helping families navigate resources including legal needs, referrals, insurance and medical providers.
Transition is a process. Our goal is to successfully bridge the gap between pediatric and adult health care and ensure that your family is well supported while crossing that bridge.