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Spina Bifida is not one condition; it is a multitude of problems that affect the mind, the body, and the spirit. No two cases of Spina Bifida are ever the same. SBA offers support and essential information to those 70,000 Americans who live with Spina Bifida and their families.

Some of the challenges that potentially faces those with Spina Bifida are:

Latex Allergy
The first allergic reactions to natural latex rubber in people with Spina Bifida were reported in the late 1980's, one hundred years after latex was first used to make surgical rubber gloves. Since that time, research studies have shown that up to 73% of children and adolescents with Spina Bifida are sensitive to latex as measured by blood test or by a history of an allergic reaction. Synthetic latex (man made, such as latex paint) is not a threat because it is not made of natural rubber.

Although the cause of latex allergy in individuals with Spina Bifida is not known, it is theorized that sensitization has developed because of the early, intense, and constant exposure to rubber products through repeated surgeries, diagnostic tests and examinations, and bladder and bowel programs. People with Spina Bifida who have shunts for hydrocephalus, other allergies, and multiple surgeries may be at highest risk for latex reactions. Latex allergy is also a problem for people who don 't have Spina Bifida but who have had multiple latex exposures because of their occupation (health care workers) or their medical condition (asthma or other allergic disease, congenital bladder anomalies, frequent surgery or surgery in infancy).

Latex, a milky fluid harvested from the Hevea brasiliensis tree, a found in many common items, including medical and surgical gloves, urinary catheters, elastic bandages, bandaids, balloons, pacifiers, and condoms. Allergic reactions to latex proteins in these items can include watery and itchy eyes, sneezing and coughing, rash or hives, swelling of the windpipe, wheezing, difficulty breathing and the life-threatening collapse of circulation called anaphylactic shock.

Exposure to latex can occur when products containing rubber come in contact with a person's skin or mucous membranes such as the mouth, eyes, genitals, bladder or rectum. Serious reactions can also occur when latex enters the bloodstream, injected through the latex ports on intravenous tubing. The powder from balloon or gloves can absorb latex proteins and become airborne, causing reactions when breathed or touched by a latex-sensitive person. Food that has been handled by someone wearing latex gloves may also be contaminated by this glove powder. (People who have allergic reactions to latex may also be allergic to certain foods, including: bananas, tomatoes, potatoes, avocados and kiwi fruit.) The only way to prevent allergic reactions to latex is by avoiding contact with items containing latex and the latex-contaminated powder .

Latex is often a hidden ingredient in medical and consumer products, and it is difficult to know if a product does or does not contain the substance. For this reason, the Food and Drug Administration now requires labeling of natural latex rubber in all medical devices. Consumer products are not yet covered by a labeling mandate, however. Research is currently focused on identifying all the proteins causing the allergy, standardizing reagents for improved testing, developing rubber products that do not cause allergy, and possibly learning how to desensitize people with severe latex allergy.

Latex allergy is not fully understood at this time. Current knowledge and available evidence indicates that people that people with Spina Bifida are at significant risk of becoming allergic to natural latex rubber, with possible life-threatening reactions. Individuals with Spina Bifida and their families are urged to consider the following recommendations and to discuss them with members of their health care team.

1. All individuals with Spina Bifida should be considered at high risk for having an allergic reaction to natural rubber (not synthetic rubber) and should avoid contact with latex products in all settings from birth. Alternative products usually made of silicone, plastic, nitrile or vinyl can usually be safely substituted.

2. Individuals who have had an allergic reaction to latex should
* Wear a medic-alert bracelet or necklace
* Carry auto-injectable epinephrine
* Carry sterile non-latex gloves and other necessary non-latex equipment for emergency use

3. Latex allergy and latex avoidance should be discussed with all health care and community providers including school, day care, camp, and visitors bringing gifts to the child.

4. Consultation with healthcare providers familiar with the latex allergy is recommended before hospitalization or surgery to prevent inadvertent exposure and plan for latex-safe care.

The following commonly encountered items may contain latex and may pose a risk to the latex sensitive individual:

* Healthcare items which may contain latex: gloves, catheters, tourniquets, elastic bandages, ace bandages, IV tubing injection ports, medication vial stoppers, adhesive tape, dental dams, bandaids.

* Home/community item which may contain latex: balloons, pacifiers, rubber bands, elastic in clothing, beach toys, Koosh balls, baby bottle nipples, condoms, diaphragms, diapers, art supplies.

Hydrocephalus and Shunts in the Person with Spina Bifida
Hydrocephalus means there is a build-up of fluid around the brain. Like a bathtub with the water on and a partially clogged drain, this fluid on the brain can’t drain fast enough. This fluid is made by special brain cells to protect the brain and spinal cord. When there is too much fluid, it can be dangerous.

Most of the time, it is easy for doctors to see that there is too much fluid on the brain. There are special fluid-filled pockets, or cavities, called ventricles, that get too big when there is too much liquid. In some cases, it isn’t as easy to see. There are cases where there may be a little more fluid than normal, and the person has little or no signs of a problem. In these rare cases, neurosurgeons might treat the problem where other types of doctors would not. Part of the reason why this might happen is because experts disagree on when the best time is to treat the problem.

The most common treatment for hydrocephalus is to insert a tube, called a shunt, to drain excess fluid from the head to another place where the body can remove it naturally. Shunts have valves that regulate both the direction and amount of fluid that is drained. All shunts have three parts: 1) a ventricular catheter to reach the area where there is too much fluid, 2) a valve to control flow (there are many types) and 3) tubing to carry the fluid from one place in the body to another.

Types of shunts
The most common type of shunt is the ventriculo-peritoneal (VP) shunt. This shunt drains fluid from the ventricle to the body’s abdomen. Other types that are less common are:
• Ventriculo-atrial (VA) shunts —VA shunts move the fluid to a vein, usually in the neck or under the collarbone;
• Ventriculo-pleural shunts — These shunts move fluid to the chest around the lungs; and
• Ventriculo-gall bladder shunts — These shunts move fluid to the gall bladder.

There are several types of shunt valves. All of them work by controlling the amount of fluid that is drained. Most are made to work automatically when fluid pressure in the head gets too high. Some valves also may have special devices to keep too much fluid from draining.

Experts have not yet learned which type of shunt is best for whom. So neurosurgeons usually pick ones that they think are best. Shunts can be put into one of these places in the head:

• The edge of the soft spot.
• Above and behind the ear.
• The back of the head.

Experts don’t know if one place is better than another. So where to put the shunt also is up to what the surgeon thinks is best.

About 80 percent of people with Spina Bifida have hydrocephalus that needs treatment. Almost all shunts are put in during the first days or weeks after birth. Sometimes the shunt will be inserted at the time of the initial back closure. A child who doesn’t need a shunt by the time they are five months old probably will never need one.

Signs of shunt problems
Signs of hydrocephalus (or of shunt malfunction) in infants may include:


• rapid head growth;
• full or tense soft spot (fontanelle);
• unusual irritability;
• repeated vomiting;
• crossed eyes;
• an inability to look up;
• periods in which the baby stops breathing (called apnea);
• difficulty swallowing;
• a hoarse or weak cry;
• difficulty in keeping the infant awake; and
• any worsening brain function.


A head ultrasound, Computed Tomography (CT) scan or a Magnetic Resonance Imaging (MRI) scan will show this fluid build-up, but a shunt still may not be working right even if it doesn’t show up on a CT or MRI scan. New, long-term treatments using small endoscopes may eliminate the need for a shunt. All patients with hydrocephalus should be seen by a neurosurgeon at least every one to two years.

Most people with Spina Bifida and shunted hydrocephalus will need the shunt for life. The most common problem with shunts is that they can get blocked up, break or come apart. About 40 percent of shunts will fail and need changing (or revision) within one year, 60 percent within five years and 80-85 percent within 10 years. About 20 percent of people with Spina Bifida will need more than one shunt revision.

The signs of shunt problems in people with Spina Bifida are different for each person. This can make it hard for families and health care providers to know what’s going on. The most common sign of a shunt problem is headache. Vomiting and nausea can happen, too, but not always.

Less common signs of a shunt problem include:
• seizures (either the onset of new seizures or an increase in the frequency of existing seizures);
• a significant change in intellect, school performance or personality;
• back pain at the Spina Bifida closure site;
• worsening arm or leg function (increasing weakness or loss of sensation, worsening coordination or balance and/or worsening orthopedic deformities);
• increasing scoliosis;
• worsening speech or swallowing difficulties; and
• changes in bowel or bladder function.

Shunt malfunction can look like any of the signs of a Chiari malformation or spinal cord tethering. In fact, when brain or spinal cord function gets worse and there is no other clear cause, health care providers should check to see if there are shunt problems.

To see if there is a problem with a shunt, health care providers will study images of the brain (usually a CT scan or, for children under one year, a head ultrasound). MRI scans can show shunt problems, but they usually are not necessary, are more expensive and may require sedation or anesthesia. When ventricles start to get too big, it is a strong sign that the shunt is not working right. It is important to know that some people (between 5 and 15 percent) with Spina Bifida may have very few signs or even no visible change in the size of the ventricles when the shunt is not working correctly.

On the other hand, some people with shunted hydrocephalus can develop the slit (or stiff) ventricle syndrome. For these people, too much fluid drainage leads to very small (or slit) ventricles. In these cases, experts think that the walls of the ventricles temporarily block the shunt catheter. This leads to a series of temporary shunt malfunctions without any visible increase in the size of the ventricles. Families and health care providers must pay close attention to a person’s symptoms, especially if they are similar to those that were present with previous shunt problems.

Infections
Infection is a major problem that can happen with shunt operations. Between 5 and 10 percent of people will have this problem. Shunt infections are higher in babies than in older children and adults. Seventy percent of shunt infections happen within the first two months after a shunt operation. Eighty percent of these infections develop within the first six months. Skin bacteria (Staphylococcus epidermidis) are the most common causes of shunt infection. Half of people with shunt infections show signs of a shunt malfunction.

Additional signs of an infection include:

• fever;
• neck stiffness;
• pain;
• tenderness;
• redness;
• drainage from the shunt incisions or tract; and
• abdominal pain.


The diagnosis can be checked by putting a small needle into the valve or a chamber of the shunt and taking out fluid for study.

Infections are commonly treated with antibiotics and with removal and replacement of the shunt system. There are two ways of doing this. The first is to take out the shunt system and then put in a temporary external drainage tube at the same time that antibiotics are given. When the treatment is done, the tube is taken out and a new shunt is put back in. This almost always stops the infection, but it takes two operations. The second (assuming that the shunt is working) is to keep the infected shunt in until the end of the antibiotic treatment. Then the infected shunt is removed and replaced with a new one. The second way only takes one operation, but it does not get rid of the infection as often as the first.

Making decisions
The opinion of a health care provider is very important when working with someone with Spina Bifida and shunted hydrocephalus. When making decisions, here is some helpful advice to families and health care providers:

• Pay attention to a parent’s (and particularly mom’s) gut feeling about shunt problems — these feelings are usually right.
• Be aware that shunt problems can cause many symptoms that may not be obviously shunt-related.
• Be on the lookout for shunt problems, and make sure the shunt is working OK before performing other neurosurgical treatments.

Fact Sheet Contributor:
Mark S. Dias, MD, FAAP, Pediatric Neurosurgeon

Depression and Anxiety in Individuals with Spina Bifida
It’s normal to be sad or blue or anxious once in a while. Everyone feels this way. But when depression begins interfering with your life, and it doesn’t go away, you probably need help. How do you tell the difference between normal sadness or nervousness and depression or anxiety that needs treatment? Sometimes it’s not so easy. Let’s look at two teenagers and what’s going on in their lives.

Making a Diagnosis
Annie* is a 15-year-old who has lumbar-level Spina Bifida with shunted hydrocephalus. Her grades have gone down. She is having trouble sleeping and has headaches. Her parents say she’s been irritable lately. What else do we need to know? Does she have friends? Yes, and she still has good relationships with her friends. Her headaches occur mostly in the evenings. She is not vomiting and her vision has not changed. She does say she is not happy at school because some boys are teasing her. But Annie says she is not depressed. She looks a little worried, but she still smiles and jokes around occasionally. She does sound a little depressed, but a CT scan uncovered her real problem: Annie needs a shunt revision.

The first step in identifying depression is to rule out physical causes of behavior or mood change. Medical conditions, such as shunt malfunction, a severe infection, and some medications, may cause signs of depression. Once the underlying medical condition is remedied, the signs of depression disappear.

David* is an 18-year-old, and like Annie, has been having trouble in school. In fact, he quit college after failing a few courses. His appetite is poor and he has little energy. David spends most of his time watching TV, feeling guilty about failing school. He had friends in high school, but he didn’t know anyone at college. So now he has almost no friends at all. He is starting to have trouble taking care of himself. David isn’t taking care of his skin. He is not bathing. And he is not taking his medications regularly. He doesn’t enjoy things that once made him happy. He doesn’t keep good eye contact and doesn’t joke around the way he used to. These are all signs of depression. After three weeks on an antidepressant, David got better.

*Not their real names

To find out if a person is depressed, it is crucial to know his or her history, including health problems, drugs, family history, and current environment. Have relatives had depression? What’s happening at home? Has there been recent stress? Is the patient alone, or does he or she have lots of friends? Does he or she find pleasure in things that are normally enjoyable? Does the patient sleep well?

Doctors can also learn a lot from working with patients. Does the person look depressed? Does he or she make eye contact? If a health care provider suspects depression, two screening questions are asked:

1) During the past month, have you felt down, depressed or hopeless?
2) During the past month, have you been bothered by little interest or pleasure in life?

If the person answers “yes” to either or both questions, more screening is needed. Sometimes you can learn a lot by simply asking: Are you depressed? Few doctors ask this. If the answer is “yes,” even fewer doctors ask the patient whether they’ve thought about suicide. These are very important questions to ask.

For parents, the most telling question may be: How is this affecting my child? Does she sleep well? How is his appetite? Is she interested in things that used to interest her? Is he able to go to classes, do his homework, study and concentrate?

Symptoms of Clinical Depression
Everyone has been sad or anxious, but clinical depression is more serious. Symptoms are more severe, last longer and generally don’t go away on their own. Clinical depression affects daily functioning. People who are clinically depressed don’t do what they used to do. Depression may affect daily activities, motor abilities, appetite and sleep. Most people with depression have less appetite, but some people, like teens, may eat more, especially sweet or high carbohydrate foods.

A common problem for people who are clinically depressed is insomnia. Insomnia is when people have trouble sleeping. It has different forms. Some people can’t get to sleep. Some people wake through the night. Others wake up too early and can’t get back to sleep. People who are depressed may pace, ring their hands, tug at their clothes and may seem restless all the time. Their speech and thinking may slow down, too, as if they were moving in slow motion. People with depression may also feel physical pain such as headaches or stomachaches.

Causes of Depression
We know that depression is linked to family history. If one person has depression, his family members are much more likely to have depression at some time. In identical twins this link is clear. If one twin is depressed, the chance of the other twin being depressed is 46 percent.

Most experts believe that depression is caused by problems with certain chemicals in the brain. These chemicals, called neurotransmitters, send signals from one nerve to the other. There are many of these chemicals, but researchers have found three that control our moods: 1) norepinephrine, 2) serotonin and 3) dopamine.

Depression is more common in women, in part due to hormonal differences. Some studies have shown more cases of depression in people with Spina Bifida. Young people with Spina Bifida are at a higher risk of depressed mood and lower self-worth, and are more likely to think about suicide. People with Attention Deficit Hyperactivity Disorder (ADHD) and/or learning disorders have higher rates of depression, too. This may be because of school failures, low self-esteem or a chemical imbalance in the brain. Depression also is more common among people in northern climates, especially in winter.

Stress, especially chronic stress, plays a role in depression. This stress could be from home, school or work, or from something else like a surgery. Depression is more common following a major personal loss, such as the death of a parent.

Low self-esteem is associated with depression, which is one reason why people with Spina Bifida are more likely to be depressed. Other risk factors include decreased social support and isolation. Learned helplessness — where people feel that there is nothing they can do to improve their situations — may contribute to depression. For example, imagine an 8th grader who tries hard to make friends, but is not able to. After a while, she gives up. And even after starting at a new school with all new kids, she doesn’t try because she has learned to be helpless.

Anxiety: Sister to Depression
If someone is anxious, they’re also much more likely to be depressed. The reverse also is true. Many drugs used to treat depression are used to treat anxiety. Like depression, anxiety runs in families. Signs of anxiety include feeling tense or afraid, a sense of dread, panic or terror. People who are anxious may worry a lot. Anxiety breaks up concentration and tends to result in a focus on oneself.

One type of anxiety disorder is obsessive-compulsive disorder (OCD). Signs include repeated, purposeful behaviors that try to reduce anxiety. Behaviors may include repeated hand washing, counting objects and needing objects to be arranged in a certain order. Severe OCD interferes with functioning and should be treated.

How to Treat Depression and Anxiety
There are many ways to treat depression and anxiety. Studies show that exercise can have a great effect. So one thing people can do is be more active. For people in wheelchairs, wheelchair-based sports are great way to do this. Sports provide a good workout and make it easy to meet people. Horseback riding is fun, too. It stretches the muscles and joints.

Drugs and counseling may be needed. Today’s drugs of choice are the SSRIs (selective serotonin reuptake inhibitors), which include Prozac, Paxil, Zoloft, Effexor and Serzone. Each drug has its own side effects that can be different in each person. Sometimes these side effects can be severe. It is important not to stop the drugs suddenly. Taper off instead. A sudden stop can cause dizziness, fatigue, headache, nausea and/or insomnia.

Counseling (including a type called cognitive-behavioral therapy) can be crucial, especially with anxiety. Counseling also helps if the person has low self-esteem or learned helplessness. Sometimes it’s very helpful for the entire family to receive counseling. Drugs alone won’t improve self-esteem or stop behaviors based on learned helplessness.

How long do you treat depression with drugs? It depends. For someone who has mild depression for the first time, treatment usually lasts for two to three months and then the drug is tapered off.

For people who have severe depression, troubles at home, low self-esteem or a hard time taking care of themselves, drugs and counseling are often necessary. Depression is very different for different people, but generally, doctors try not to give kids drugs for more than six months. Treatments can last much longer in cases where it is necessary.

Get Help
If you or someone you know seems to be depressed, talk to a doctor or nurse. Depression and anxiety are treatable. If you try to talk about depression or anxiety and your health care provider doesn’t react the way you think he or she should, find another provider who will listen more.


Fact Sheet Contributor:
Gregory S. Liptak, MD, MPH

Urologic Care and Management
The urologic care of children with Spina Bifida has undergone several important changes in the last decade. The emphasis is now on early catherization of the child's lower urinary tract function and preventive treatment to preserve both kidney and bladder function in an attempt to maximize the child's long-term functioning.

Newborn Assessment
As soon as the neurosurgical condition is stable, an assessment is made of the neonate's kidneys with a serum BUN, creatinine and renal ultrasound (ECHO); the bladder is evaluated with urodynamic studies. Children whose bladder will fill and empty with low pressure are managed expectantly. If the child's bladder pressure is elevated above 40 cm H20 during filling and/or the urethral sphincter is dyssynergic (not coordinated during a bladder contraction), then the child should begin intermittent catheterization (CIC) and possibly anticholinergic drugs to ensure complete emptying and low filling pressure. Antibiotics are usually prescribed for only a short time until the family is comfortable with the technique of catheterization.

If the kidneys are dilated (hydronephrotic) or the urodynamic studies reveal high bladder filling pressure or a dyssynergic sphincter with high voiding pressure, a voiding cystogram (VCUG) is performed. When reflux is present, most children will need to be started on CIC and antibiotics to achieve adequate drainage of the upper urinary tract. A high-pressure bladder in the presence of reflux and/or hydronephrosis warrants the additional use of anticholinergic medication. Children with lesser grades of reflux or mild hydronephrosis, whose bladders empty with low pressure, are managed without CIC because it is unlikely they will develop any damage to their kidneys. More importantly, however, the children are followed very carefully to see if their condition changes.

If the infant with impaired upper urinary tract drainage fails to respond to CIC and anticholinergic medication or the parents are unable to perform the procedure easily and routinely, a vesicostomy may be performed. This procedure consists of bringing the dome of the bladder to the skin as a stoma midway between the umbilicus and the pubic bone. The advantage of this temporary form of diversion is that it is easily reversible when the child is older and the family is amenable to CIC, and it does not alter the child's future continence mechanism.

Surveillance
Throughout infancy and early childhood the children are followed with routine urine cultures, evaluation of post-void residual urine volumes (in those not on CIC), and kidney and bladder ECHOs. Urodynamic studies are repeated on a regular basis in those children with normal or minimal denervation in the sphincter to see if their neurologic lesion has changed and/or spinal cord tethering has occurred and to determine if the children who are on expectant therapy, have altered their bladder and sphincter function such that prophylactic intervention with CIC and/or anticholinergic drugs is warranted.

When a child develops recurrent urinary infection, a VCUG or radionuclide cystogram (RNC) is performed (even if it has been done previously) to evaluate for reflux. Children not on catheterization are given appropriate antibiotics. In children on CIC but without reflux, only symptomatic infections are treated with short courses of antibiotics. Children on CIC with reflux are managed similar to those with normal bladder function, i.e., antibiotics as indicated, frequent urine culture, regular ECHOs and, if indicated, RNCs or VCUGs to monitor the progress of their reflux. If a child with mild reflux who is not on CIC develops symptomatic UTIs, he/she is begun on regular catheterizations.

The use of CIC, antibiotics and anticholinergics has resulted in a spontaneous resolution of reflux in more than 50% of children, with a reduction in its severity in another 30 to 40%. Antireflux surgery (required in 10 to 20%) is undertaken when children have repeated infection despite adequate antibiotics and a good bladder- emptying regimen. The success rate for surgery is almost as high as it is for children with normal bladder function, i.e., 96 to 98%. Recently, bulking agents (primarily Deflux) injected into the ureter adjacent to its orifice at cystoscopy has been promoted, with a success rate ranging as high as 80 – 90%, for the milder grades of reflux. It is imperative that the bladder needs to be optimally managed even in these cases of endoscopic treatment.

Continence-Medical Therapy
Because the emphasis has shifted to mainstreaming children with myelodysplasia into normal school and social situations, it is very important for bladder and bowel function to be controlled before the start of school. If the child is not on CIC in the months preceding this milestone, catheterization is started and urodynamic studies performed to determine how best to modulate the incontinence. Medications are given to a) lower bladder filling pressure and increase capacity, and b) raise urethral resistance to prevent leaking between each emptying. Success rates for achieving continence range as high as 65%. There are several alternatives within each category of drugs that may be given so that if one medication does not achieve the desired effect or produces side effects, another may be substituted. Even the method of administration has changed, for example, Oxybutynin (Ditropan) tablets may be dissolved in normal saline and instilled directly into the bladder through the catheter used for CIC.

When these measures fail to lower bladder pressure and increase capacity, several alternatives exist. Transurethral electrical bladder stimulation (TEBS) has been conducted at a number of centers around the United States ; this consists of delivering an electrical stimulus to the bladder through a specially designed catheter for 90 minutes, 5 days a week for 5 weeks. In one study, this treatment has been shown to attain continence in 15 to 25% of children with another 25 to 30% requiring less medication to achieve a low-pressure large capacity bladder. In addition, a significant majority report improved bowel continence after completing one or two courses of treatment. Several research centers in North America have started to directly stimulate the nerves that control bladder and urethral sphincter function in an attempt to improve both storage capabilities and emptying efficiency of the bladder. Assessment of preliminary results from these centers has been encouraging. It is not known how long lasting the effects of these programs will be.

Injection Therapy
Over the last several years, advances have been made in the endoscopic management of incontinence. This has resulted in less invasive procedures being performed prior to major open reconstructive surgery. Botulinum toxin (Botox) injected directly into multiple sites within the bladder muscle can paralyze it leading to increased capacity and compliance, resulting in improved continence and kidney drainage. The botox may be effective for upwards of 6 – 9 months but repeated injections are needed to maintain its effect.

Dextranomer hyaluronic acid polymers (Deflux) injected directly into the bladder neck or continent stoma conduit (where it enters the bladder) is currently also being employed to enhance bladder outlet resistance and improve continence when drug therapy or prior surgery fails to achieve total continence. The effect seems to be stable but long-term studies are needed to judge it efficacy.

Continence-Surgical Treatment
When these measures fail to achieve continence, various formal surgical procedures have proven useful. Augmentation cystoplasty in which a segment of the gastrointestinal (GI) tract is isolated and added onto the bladder has been performed on numerous occasions. All portions of the GI tract have been utilized – stomach, intestines, colon and sigmoid. The bowel must be detubularized to prevent physiologic contractions that occur in the normal bowel segment from producing unwarranted incontinence. Mucus and urinary infection may be persistent problems, even years later. Perforation of the intestinal segment from overdistension has occurred, especially if the individual's bladder is not emptied routinely. A disturbing feature relating to these intestinal segments has been the slowly but steadily increasing incidence of tumor formation within the bladders of these individuals beginning 15 to 20 years after the augments were fashioned. Further investigative studies are under way to determine the true incidence and susceptibility to this potentially devastating complication. The need for long-term surveillance is paramount.

Autologous tissue engineered constructs of bladder tissue grown from an individual's own bladder biopsy material has produced favorable results in phase 1 trials. This may hold promise as a viable alternative to intestinal sources for augmentation, thus eliminating the potential complication noted above.

Procedures designed to increase bladder outlet resistance include bladder neck reconstruction, fascial sling or bladder neck suspension, artificial sphincter implantation and the Kropp procedure. The artificial sphincter consists of an occlusive cuff fitted around the bladder neck or bulbar urethra, a pressure regulating balloon reservoir and a deflate pump placed in the scrotum or labia. When the child wants to void, he or she squeezes the pump, opening the cuff and allowing the bladder to empty. The cuff automatically refills with fluid from the reservoir over the next five minutes occluding the bladder neck once again. If the child can empty the bladder spontaneously with straining before the sphincter is implanted, then he/she should be able to do so postoperatively. The long-term success rate for the artificial sphincter at least five years after implantation ranges from 65 – 80%.

All other operations designed to increase bladder outlet resistance require that the child catheterize him/herself afterwards to empty the bladder. In the fascial sling procedure, a strip of fascia (or fibrous tissue surrounding the rectus muscle) is isolated and wrapped around the bladder neck compressing and buttressing it against the undersurface of the pubic bone. The added resistance and the repositioning of the urethra prevent leakage between catheterizations in more than 80% of children. The Kropp procedure isolates a strip of anterior bladder wall, tubularizes and tunnels it below the surface of the posterior bladder wall in order to elongate the urethra. This creates a very effective continence mechanism (95%) but it requires reimplantation of both ureters in a slightly more lateral position to accommodate the neourethra, and augmentation cystoplasty because so much of the bladder is used in the reconstruction.

Continent & Incontinent Diversions
When the child cannot be catheterized transurethrally or requires a urinary diversion, most urologists are now performing an operation to create a continent catheterizable stoma. This conduit can be fashioned from ureter, appendix (preferred), fallopian tube (in girls), intestine or stomach—anything that can be easily catheterized. One end is brought to the skin and the opposite end implanted into a urinary storage reservoir if the bladder cannot be salvaged.

When the bladder remains as the urinary reservoir its neck might need to be obliterated, especially if the outlet resistance is very low, in order to eliminate the possibility of urinary leakage from the urethra. The continent stoma is small and inconspicuous and is easily covered with a band-aid or small gauze pad. Continence is achievable in over 90% of children. The children with this anatomic arrangement have done well on a reasonable long-term basis but more time is needed to be certain about late follow-up. The only consistent yet easily manageable complication has been stenosis at the skin stoma site. Most families report the child's quality of life and care giving are vastly improved, with many children becoming more independent in managing their urinary tract than previously achievable.

Urinary diversion, commonly performed in the 1960's to the mid 1980's, consists of isolating a segment of small intestine, attaching one end of it to the ureters after they have been detached from the bladder, and brining the opposite end to the abdominal wall skin as a wet stoma. The urine passes through this system continuously to a bag placed over the abdominal wall stoma. This operation is rarely performed anymore unless it is deemed difficult or impossible to get the child or adolescent to catheterize their urinary reservoir on a regular basis. Individuals who currently have these incontinent stomas are not necessarily candidates for either an undiversion or a continent stomas operation, especially if they have not had any adverse reactions from their original conduit surgery. The development of complications, e.g., pyelonephritis (kidney infection), urinary tract stones, obstruction in the urinary conduit, or stomal stenosis requiring a revision, may warrant conversion of the conduit to a continent stoma.

Obesity
Obesity is the most common nutritional problem in North America. Obesity may be a cosmetic concern for affected individuals. More importantly, however, obesity is a major health threat. In adults, obesity has been linked to high blood pressure, diabetes, osteoarthritis, abnormal cholesterol metabolism, heart disease, sleep apnea, and psychological problems. Similar health consequences occur for children and adolescents who are obese. Psychological problems are of special concern for developing children, who may be negatively stigmatized by others if they are obese and thus develop poor self-esteem, greater risk for isolation from peers, and depression.

Obesity in children can be determined in several ways. Body Mass Index, or BMI, is a number calculated from a person's weight and height. It can provide a reliable indicator of body fatness for most people and is used to screen for weight categories that may lead to health problems. However, it may be difficult to get an accurate measure of height in a person with spina bifida. Arm-span may be substituted for height in some individuals. Another method is to use calipers to measure skin-folds and plotting the results against standardized charts. The National Center for Health Statistics / Centers for Disease Control (NCHS/CDC) has several growth charts and calculators that may be helpful. [http://www.cdc.gov/nccdphp/dnpa/obesity/index.htm] Children whose weight for height exceeds the 95th percentile are overweight, and those who fall between the 85th and 95th percentiles are of concern.

How Obesity Develops
All energy intake (measured in Kilocalories (Calories)) comes from food that is eaten and then used by the body to meet its needs. Obesity results when a person's intake of calories exceeds his or her energy needs for bodily functions (metabolism, physical activity, the thermal effect of food, and growth). Similarly, weight loss results when the body uses more calories than are taken in through eating. On average, most (about 60%) of our energy is used for basic metabolism (which is controlled in part by inheritance); physical activity uses about 25%, the thermal effect of food about 10%, and growth about 5%. Our bodies do a good job of balancing short-term excesses in both energy intake and use. Continued excessive intake, however, will lead to storage of energy in the form of fat (weight gain), and continued insufficient intake will force the body to use stored energy (fat and other tissue like muscle) for the calories it needs to function (weight loss).

Special Concerns for Individuals Who Have Spina Bifida
Obesity is an even greater health problem for people who have spina bifida. Obesity further limits mobility and self-help skills, leading to a spiraling problem of decreased energy use and weight gain, making it harder for the individual to keep up with peers in social and work situations. It adds to the amount of pressure on skin, thus increasing the already high risk of skin breakdown, particularly in areas that are insensate or become wet. Social rejection, which may already be a problem due to others' lack of sensitivity and understanding of disability, may be worsened. And activities of daily living, particularly independence in dressing, continence management and hygiene, may be negatively affected by difficulties in moving a large, heavy body and decreased ability to reach private areas of the body.

Very young children who have spina bifida usually grow at about the same rate as their non-disabled peers and are quite physically active, so they usually do not become obese. As they grow older, however, children who have spina bifida, especially those who also have hydrocephalus, are at very high risk for developing obesity. Beyond age six at least 50% of children who have spina bifida are overweight, and in adolescence and adulthood over 50% are obese.

There are many reasons for this. Neurological impairments that lead to even slight mobility problems make it harder for individuals who have spina bifida to be physically active. Due to the requirements of school and work for sedentary activity, and due to the increasing difficulty of moving a larger body that has a mobility impairment, school age children who have spina bifida typically become less active as they grown older. Small children grow rapidly and so they require a large number of calories for growth. Older children and adults have slower growth, however, and on average those who have spina bifida will not become as tall as their non-disabled peers. So, those with spina bifida have fewer requirements for growth, as well. Also, studies have shown that people with spina bifida have less lean body mass than their peers, and even when other factors like physical activity are equal, have a lower basic metabolic rate (fat cells have slower metabolic rates than other cells like muscle cells). In some families, food is used to try to compensate for the child's disabilities. (“At least he or she can enjoy eating.”)

Preventing Obesity−−a Family Affair
Prevention of obesity is the only sure method of promoting optimum nutritional health. Preventing obesity for individuals who have spina bifida is a family affair that must begin early in a child's life and continue indefinitely. Fortunately, if healthy eating and exercise habits are begun early, they can become part of a very enjoyable lifestyle, rather than one that is perceived as a life of deprivation.

Most eating behaviors and food likes and dislikes are learned in the context of home and family. Thus, by modeling and teaching healthful eating habits, family members have a chance to improve the child's health. This may be difficult, especially for families where eating patterns and genetic factors have led to obesity being commonplace. When a child has spina bifida and family members are somewhat frustrated by their inability to correct their child's underlying disabilities; making this contribution to health and well-being can be very rewarding.

Strategies for Success
The most helpful strategy for preventing obesity is to help the child view food as a necessity for growth and activity rather than as a reward for managing the difficulties of daily living. The goal is to condition the child to perceive food not as an emotional, but rather a physical, necessity.

Children can learn about good nutrition as they help plan family meals and shop for ingredients. Remember, children cannot consume food that is not available!! Caregivers need to purchase nutritionally sound, healthy foods using the "Food Pyramid" [http://www.mypyramid.gov/] as a guide. Most food servings should come from fruits, vegetables, bread and cereals, fewer from dairy products and meats, and only a small amount from foods high in fats, processed sugars and other carbohydrates. Remember that fat has 9 Calories per gram while carbohydrates and protein have only 4 Calories per gram. Therefore, decreasing fats can have the greatest impact on weight loss.

Food should be eaten at regular times during meals that are pleasant and that take enough time for individuals to eat slowly and realize when their hunger has been satisfied. Treats and snacks should be limited to times when a little extra energy is really needed and should be both nutritionally sound and enjoyable. Food and visual reminders of food should be removed from the environment and other cues for increasing enjoyment of life, such as posters about exercise or hobbies, should be substituted. Entertainment should rarely center on food or meals. Children should receive only non-food rewards for positive behaviors. Children need to learn to distinguish between boredom and hunger, and also to enjoy foods other than those with high fat or high sugar content. Studies reveal that if such foods are strictly limited from our diets, we lose our taste for them and crave them less and less.

The second important strategy is to help children who have spina bifida enjoy exercise. Physical activity has two benefits; it burns calories and can decrease hunger by resetting the body's "thermostat." Most physical activities that other children enjoy can be adapted for children who have mobility impairments. Horseback riding, tennis, swimming, and wheelchair sports like basketball and track are life-long activities that most children love and families can enjoy together. Community organizations such as the YMCA should be encouraged to create such opportunities for individuals who have physical disabilities. Small children can be even more active as they learn to complete some helpful tasks around the house. Such activities not only burn calories, but also help children feel good about themselves as capable people who can help others. Watching television or sitting in front of a computer are activities that demand few calories, and should be limited.

Weight Reduction
Once people become obese, losing weight is hard to do. Most can only lose about 10% to 15% of their body weight, and even these individuals usually regain this weight after their strict program of diet, exercise and behavior modifications is withdrawn.

Once people with spina bifida have become obese, it is even harder for them to lose weight than it is for others. It is not impossible, however! If an individual is motivated to lose weight and limits caloric intake while also increasing exercise, weight can be reduced. The assistance of a nutrition consultant may help in such cases, since a very low calorie diet—1,000 calories per day or even less--may be needed. It can be difficult to create an interesting, enjoyable and nutritionally sound diet using so few calories. Weight reduction strategies should be started one by one, so the individual and family can become used to new patterns of living. Trying to do too much at once is often overwhelming and self-defeating. Most importantly, the individual should recognize that any weight reduction contributes to good health, and that losing weight should be don e in small measures and will take a long time. Small weight losses should be celebrated, (but not with food!) because they really are important!

In summary, individuals who have spina bifida are very likely to become obese unless they, with the help of their families and friends, prevent gaining too much weight during their childhood and adolescence. Obesity has many negative consequences, so it needs to be prevented or managed to achieve optimum health and quality of life. The benefits of healthy eating and sufficient exercise for individuals who have spina bifida are numerous and important, and last throughout life. Perhaps no other single intervention will make such a positive contribution to long-term good health and quality of life.