Spina Bifida and Adult Healthcare Issues

***The Following is from http://www.spinabifidasupport.com/adultsbhealthcare.htm***

The May 9-10, 2003, Evidence-Based Practice in Spina Bifida Conference was a milestone: the first time ever that researchers and medical providers from all over the country gathered to assess the current state of research in spina bifida and plan a much-needed, long-term research agenda. And not a moment too soon!
Although there are many more adults living with spina bifida than ever before, there’s very little high-quality research on spina bifida in general—and even less on how spina bifida affects people throughout the life cycle.
Our current system does not promote ongoing, coordinated health care for adults living with chronic conditions, which creates significant health management challenges for adults with spina bifida. Most physicians who treat adults were not trained in what were once considered pediatric disorders. According to Timothy Brei, MD, “We've grown beyond the boundaries of established medical research. We are the pioneers. Many adults with spina bifida know more about their bodies than the physicians they see.”
Mark Merkens, MD, suggests that adults who lack access to an adult spina bifida clinic might want to look for a rehabilitation physician for primary or specialty care.

Health Management and Aging With a Disability

As people age, they experience various physical manifestations of the aging process. Muscle strength, flexibility and endurance decrease, and the risk of osteoarthritis and osteoporosis rises. Aging affects the central nervous system, decreasing sensation and balance. The skin thins and becomes less elastic.
People with spina bifida experience the usual manifestations of age, but since spina bifida puts pressure on many body systems, age-related declines in affected areas may occur sooner or be more severe. 
"SB docs at national conferences always talk about aging "in SB years". That is to say, things that happen to people as they age, happen to people with SB faster. Because of how we're built, and/or the way we walk/roll in wheelchairs, we develop things like arthritis sooner. Rotator cuff issues. Hip problems. This also applies to internal organs. Kidney damage may occur sooner. Heart and lung issues from scoliosis some of us have. We just age faster. Nobody knows how long a lifespan those of us who are shunted can have!! I know of people with SB in their 50s and 60s and 70s.....but none with hydrocephalus. Had they been born with hydrocephalus in the 1930s, 40s and 50s, they probably would not have survived. Since 80% of people with SB also have hydrocephalus, we are the first generation to survive in large numbers, thanks to the advent of the modern day shunt in 1958."~~~ Jesus Arroyo 

Shunt Failure

Shunt failure happens to adults, even those whose shunts have not been working for years. The symptoms of shunt failure in adults are usually the same as those in children. Dr. Merkens describes the symptoms as follows: “The symptoms of shunt failure may creep on you. Your thinking may be slower, you may be less facile with words, more hesitant in general and a little clumsier on your feet. Neurosurgeons who treat adults may be skeptical of shunt failure in adults, especially if the shunt hasn't been working for a while. But what I tell my students is ‘If there’s a neurological problem, it’s the shunt, stupid.’ It’s the mantra in new generations of young neurosurgeons.”

Chiari Brain Stem Compression

Chiari brain stem compression is likely to cause sleep apnea or loss of respiratory drive. The symptoms may be subtle at first. Dr. Merkens says, “Maybe you've been doing fine for years, but now you've contracted pneumonia a couple of times and seem to have more trouble recovering.”
According to Dr. Merkens, it generally doesn't happen for the first time in adulthood. But neurosurgeons say that when you’re starting to think about chiari brain stem compression, it’s a good idea to also check the shunt. A shunt failure increases pressure in the brain, pushing the skull down into the cervical spine—exactly what chiari brain stem compression is. It could present with a weak voice, pneumonia, more difficulty swallowing, difficulty with vocal chords and sometimes even problems with the eyes.

Spinal Cord Tethering

Spinal cord tethering can occur for the first time at any age, although it’s more likely to occur if there has been a previous tethering and detethering, which may have created scarring. Tethering usually presents the same signs and symptoms in adults as in children: basically anything that changes below the level of the spina bifida—the level of the spine where the defect occurred. There may be urinary changes, such as infections or difficulty staying dry, or sudden changes in bowel patterns.
Skin sores may be another symptom of tethering. Orthopedic changes, such as a foot starting to turn out, or scoliosis that progresses fairly quickly may signal tethering, as can a change in sensory level. Dr. Merkens says, “If you used to be able to feel down to your shins but now can’t feel your knees, tethering may be responsible.”
However, before contemplating detethering surgery, the shunt should be checked. If a pediatric, orthopedic or urology exam shows changes that suggest tethering, the next step is to get an MRI of the entire neurological system, to check for tethering, spinal syrinx and shunt failure. If the results are inconclusive, a pressure check of the shunt may be necessary.

Urinary and Bowel Systems

The rate of renal failure has declined significantly since the introduction of CIC(intermittent catheterization), but we’re not home free. Medical providers know that CIC reduces urinary tract infections (UTIs) and prevents urine backups, but its long-term effects are still unknown.
Dr. Merkens mentioned two young men with artificial sphincters who ran into serious trouble by not following their programs and said, “We don’t do enough to identify good candidates for artificial sphincters. Good candidates must catheterize religiously and have a low incidence of UTIs. An artificial sphincter basically puts a cork in the bladder, and urine must be emptied completely several times a day, or else kidney damage may result.”
People who have had bladder augmentation need to be followed closely over their lives for any signs of cancer, problems with mucus or kidney stones. The long-term effects of augmentation are still unknown. Adults with illeal loops must be followed closely, because there’s a chance that over time the loops may allow urine to back up into the kidneys.
Most difficulties with bowel programs relate more to behavior than to the level of spina bifida. Success generally depends on a consistent diet, regular exercise and timed evacuation. Doctors are not yet sure about the effects of chronic enemas, rectal bulbs and similar practices, so affected areas need to be checked regularly. Dr. Merkens raised the issue of mega-colon  which refers to the bowel becoming larger from regularly holding large amounts of stool. Though it is certainly an excellent method of bowel management, we don’t yet know of potential complications of the Antegrade Colonic Enema (ACE) over time.
Dr. Merkens says, “We are learning to be more aggressive about bowel programs earlier in life to prevent later complications such as mega-colon  In Great Britain, for example, the goal is for children to be in a functional bowel program by the time they are 6-years-old.”

Orthopedic and Skin Issues

According to a 10-year-old German study, over 50 percent of adults may have poorly fitting, poorly functioning braces. Premature arthritis is often a problem for adults with spina bifida, especially for those who use their shoulders to ambulate. Knees are also prone to arthritis, particularly in adults with a wobbly gait who don’t use crutches. Lots of back-and-forth and side-to-side motion is very hard on the knees.
Scoliosis can progress in adults if it wasn't fused in adolescence. Skin breakdowns can cause bone infections, especially at the heel or toes, which can be difficult to heal. Orthopedic problems can occur as a result of pressure and abrasion, poor circulation, neurological changes and edema. Dr. Merkens advises adults with spina bifida whose feet swell to elevate them several times a day. If your feet are purple or red, wearing pressure stocking is a good idea. Adults need to check their skin often. Wheelchair users need to use a mirror to check their rear daily and remember to shift positions every 15 minutes to avoid pressure sores.
Dr. Merkens recommends that adults visit a podiatrist annually, to ensure that their feet remain healthy.

Latex Allergy

Latex allergy is more likely to affect those born from the late 1980s to the mid-1990s. The rate of latex allergy in people with spina bifida born from 1987 to 1995 is over 50 percent, but the rate since then is much lower, almost certainly due to improvements in the latex purification process. Almost all large, teaching hospitals have latex-free operating rooms, but patients may have to specifically request a latex-free environment at smaller hospitals.
Before visiting the dentist, adults need to request a latex-free environment, which means that the patient in the previous appointment slot will need to receive latex free treatment, too. In fact, it’s a good idea to ask for the first appointment in the morning, when the room is cleanest and most latex-free.

Hypertension

Dr. Merkens reports seeing elevated blood pressure in many of his adult patients, at younger ages than usual. If blood pressure goes up, he suggests having a urologist evaluate the kidneys, because kidney problems can raise blood pressure. Elevated blood pressure—higher than 140/90—needs to be treated and followed closely.

Lung Health

There are two types of lung disease: obstructive and restricted. Obstructive is when something is blocking breathing, such as in sleep apnea. Restrictive lung disease is due to the inability of the lung to fully expand when taking a breath, which may be caused by scoliosis, Chiari, or even a weakness in the abdominal or chest muscles. Some of the signs are very subtle. But Dr. Brei advises adults who snore, feel tired or have to take naps during the day to see a physician for an evaluation.

Obesity and Exercise

Obesity is major health problem in the general US population, but it’s even more problematic in adults with spina bifida. According to Dr. Brei, “As we move into our teens, we have a tendency to become heavier, which contributes to high blood pressure, diabetes, heart disease and sleep apnea.”
Many adults with spina bifida have decreased mobility and spend more time in wheelchairs. Those who walk often tend to walk less as they age. Dr. Brei says, “Exercise is tremendously difficult for many of us, but it is so important. And vigorous, heart-pumping exercise is what we really need to maintain our cardiovascular health and manage our weight. Starting a program may be very difficult, but it’s essential to find a physical therapist, recreation therapist or fitness trainer you can work with.”
Regarding regular exercise, Dr. Brei says, “If I had the answer for this, I’d be rich. There are institutional barriers that are difficult to overcome, but it’s also true that some of us are not very motivated to exercise. I absolutely despise exercise. I've never felt those little endorphines that are supposed to make you happy while you work out. But I go to the gym as religiously as I can.”

Interpersonal Relationships

Interpersonal relationships are very important for everyone, including adults with spina bifida. Studies show that satisfying relationships improve both longevity and mental health. Dr. Brei says, “It’s not easy to go out and try to meet people. It’s often uncomfortable at first and it exposes us to hurt. But if we don’t seek out relationships, they’re probably not going to find us.”

Pregnancy

Spina bifida does not appear to affect a woman’s ability to become pregnant. Although the evidence is sketchy, it appears that spina bifida can complicate pregnancy, and although most difficulties may disappear when the pregnancy is over, there are some reports of women losing mobility and not regaining it after pregnancy. Women who want to become pregnant should work closely with an obstetrician specializing in high-risk pregnancies.
They should also talk to their doctor about taking prescription strength levels of folic acid prior to pregnancy since they are at higher risk of having a child with spina bifida.

Pain

Adults with spina bifida often experience considerably more pain as they age, which may be due to joint stress, muscle pain or arthritis. Dr. Brei advises adults in pain not to suffer in silence, but to tell their doctor how they feel. There are many pain management programs that can help.

Substance Abuse

Some research shows substance abuse as higher in adults with spina bifida than in the general population. If that’s true, it could be self-medication for pain, depression, isolation or any of a number of other issues. Dr. Brei says, “I worry because substance abuse hampers your ability to be independent, to succeed, to achieve goals. If you feel depressed or anxious, seek help. Counseling can help and several drugs out now are very effective in treating depression and anxiety.”

Basic Health Care Guidelines

Adults with spina bifida need to follow the guidelines for periodic health checks on all body systems: blood pressure, height/weight, cholesterol levels, colon health, tetanus shots, pap smears, breast exams, testicular and prostate exams, urinalysis, renal ultrasound, renal blood test, spine x-ray, eye exams and more. SBAA publishes guidelines on health maintenance for people with spina bifida, which Dr. Merkens is in the process of updating.
In summing up, Dr. Brei offers this advice: “Avoid smoking, drugs, and drinking too much. Find work you enjoy. If that’s a problem, volunteer your services. Develop social supports, such as finding others who enjoy the same hobbies you do, or join a group at church. If you’re working, get together with people at work. Meet your neighbors. Get lots of sleep. Find exercise you can do and stick with it.”
Adults living with spina bifida need to monitor their health regularly and seek out health care at the first signs of trouble. Health care is one of those areas where being proactive pays big dividends—and waiting too long to react can create serious problems.

+++This article was adapted from the 2003 SBAA Annual Conference session “Health Care Issues for Adults With Spina Bifida,” presented by Timothy Brei, MD, and Mark Merkens, MD.

***This information does not constitute medical advice for any individual.  As specific cases may vary from the general information presented here, Spina Bifida Family Support advises readers to consult a qualified medical or other professional on an individual basis.

Spina Bifida and Urologic Care

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

Newborn assessment

As soon as the neurosurgical condition is stable after birth, an assessment is made of the newborn’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.

Management of the neurogenic bladder

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 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. These children are followed very carefully though to see if their condition changes.

Surgical options

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%.

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.

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.

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.

Surgical treatments

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 diversion

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.

Incontinent diversion

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.

***This information does not constitute medical advice for any individual. As specific cases may vary from the general information presented here, SBA advises readers to consult a qualified medical or other professional on an individual basis.***

Spina Bifida and Precocious Puberty

Children with Spina Bifida and hydrocephalus may start puberty earlier than their peers

What is Puberty?
Puberty refers to normal body changes that lead to maturity and the ability to have children. Normal puberty begins between ages 8 and 12 in girls and between 9 and 14 in boys. Hormones made in the brain control the timing and sequence of puberty. These hormones stimulate other parts of the body to make sex hormones. The sex hormones, especially estrogen in girls and testosterone in boys, cause sexual maturation.

What are the stages of normal puberty in girls?
The physical changes seen in puberty are labeled by “Tanner staging.” Stage 1 is child-like (before puberty) and stage 5 is full maturity. The usual sequence in girls is:

• Breasts start to develop.
• Hips widen and a there is a growth spurt that usually lasts about four years.
• Pubic hair grows (three-to-six months after breasts develop).
• Other body hair grows.
• First period usually starts two to two-and-a-half years after breast development is noticed. Body odor may also change.

The average age that breasts start to develop in girls is 9 to 10. Puberty usually starts earlier in African-American girls than in Caucasian girls. Some experts found that breast development in African-American girls started a year before it did in Caucasian girls. Breasts typically take at least three years to mature fully.
If major breast development starts before age 8, it is considered early. (Sometimes girls will have some breast development, with no other signs of puberty. This isolated change is usually normal.)

What are the stages of normal puberty in boys?
The usual sequence in boys is:

• The testicles grow larger.
• There is a growth spurt.
• The penis grows larger.
• Pubic hair grows.
• Other body hair grows.

If boys show major development before age 9, it is considered early. Early puberty in girls or boys is called “Precocious Puberty.”

What is precocious puberty?
“Precocious puberty” means that these changes start much earlier than expected. Early puberty can cause problems. Once the growth spurt starts, bones become more adult-like. After that time, the bones stop growing and can’t grow any more. So, most children who start puberty too early will be shorter than they otherwise would have been.

Another major problem with early puberty is that body changes happen much earlier than in other children of the same age. This makes a child appear different. Children with early puberty might be teased. This is common for girls who develop breasts early. Girls might receive sexual advances for which they are not mentally and emotionally ready. Boys might become more aggressive than their peers.

Children with early puberty might feel alone and rejected. Adults might expect them to act older than they are. This can be hard for children because they can’t live up to how others think they should act. For children with Spina Bifida, early puberty and all of the problems that go with it can make daily life more difficult.

Why are children with Spina Bifida and hydrocephalus more likely to start puberty earlier than their peers?
No one knows for sure why children (especially girls) with Spina Bifida and hydrocephalus are more likely to have early puberty. Most think that hydrocephalus changes the time when the brain sends “puberty hormones” into the bloodstream.

What do I do if my child shows signs of precocious puberty?
Your child should be seen by a health care provider if he or she:

• Starts puberty early;
• Has an unusual sequence of the steps outlined above; or
• Has very fast sexual development.

Your child would be referred to an endocrinologist (a doctor who specializes in hormones). If no other problems are found, the most common treatment is to delay puberty. There are hormonal treatments that can stop puberty, usually after three to six months; and may reverse some changes.

LHRH analogs, synthetic hormones that block the body's production of the sex hormones that cause precocious puberty, are available under different names, but Lupron seems to be the most commonly used medication. Positive results are usually seen within a year of starting treatment with an LHRH analog, and the drugs are considered to be generally safe. However, all drugs potentially have side effects, and families should discuss this with their physicians.

What is the benefit to delaying puberty in my child?
It is thought that delaying puberty helps children grow taller than they would have been otherwise. More important however, the delay in sexual maturation should allow children (and parents) to adjust to their growing bodies and feelings, and help them have a healthy sexual identity.

This information does not constitute medical advice for any individual. As specific cases may vary from the general information presented here, SBA advises readers to consult a qualified medical or other professional on an individual basis.

Spina Bifida: A Guide For Medical Professionals

***The Following is a conversation that Jesus Arroyo had with a doctor recently. Actually, it was a 3rd year medical student that has not had much, if any, experience with treating a patient that has Spina Bifida:

"I went to the doctor today for something. Third year Med student walked in first. Yep, I was annoyed. I understand people have to learn. But not on me. SB is so complex and complicated, the body systems it affects....at national conferences, specialists are constantly telling us SB is the most complex birth defect you could have that is still compatible with life....(hey there ya go, a real fact, not just a story. :-)) Anyway, so I was annoyed. And sure enough he was shocked....shocked I tell you!!....that he was witnessing a 37 year old with SB! And ambulatory! And of average intelligence even! So I schooled him. I was respectful. I was patient. I was nice. But I schooled him. "When you get to be on your own and you hear about a pregnancy with SB, don't go all 'worst case scenario, scare the shit out of parents, push abortion' on them. I understand it's your job to inform, to give possible outcomes, to prepare them. To be realistic. It would be medically irresponsible not to be. But don't go right to abortion; to 'no quality of life'. To 'he will be a vegetable'. A whole population of fantastic adults don't deserve that. Happy, successful adults. Patent lawyers and doctors, teachers and moms and students and husband's and counselors and stay at home dads and volunteers. We are a lot and we do a lot and we don't deserve to be outright dismissed from one ultrasound." There was more but that's the general idea. He was genuinely appreciative. Grateful even. Said I taught him something valuable today. Go me."***

It is never easy or pleasant to give expectant parents news that their pregnancy has a fetus affected by Spina Bifida (SB). In order to help you provide the best care possible for your patients, and to help expectant parents fully explore the options they have, the Spina Bifida Association (SBA) wants to ensure that you have the most current and accurate information about Spina Bifida.

What you may remember about SB does not necessarily reflect the advances in medical and psychosocial care over the last generation.

• It is currently the most common permanently disabling birth defect, that is compatible with an adult life expectancy.
• For prenatally diagnosed patients, motor function may be better if a baby with SB is delivered at 37 weeks by elective C-section before a trial of labor (Luthy et al, 1991). This practice is recommended by many SB clinics.
• Fewer than 5% of pregnancies affected by isolated neural tube defects will result in a stillborn child or preterm delivery.
• Because of new treatments, surgeries, and therapies, at least 75% of individuals with SB live into adulthood (Bowman & McClone, 2001).
• The majority of people living with SB are adults. Most of them have ventriculoperitoneal (VP) shunts, but their life expectancy is not yet known. While there are adults with SB living into their 60's and 70's, those individuals predate the development of the VP shunt.

What is Spina Bifida?
Spina Bifida is a general term used to describe a variety of different neural tube defects which include Spina Bifida Occulta (SBO), a closed neural tube defect, and Spina Bifida Cystica (open neural tube defect). The most common open neural tube defect is Myelomeningocele. When most people speak of Spina Bifida they are referring to Myelomeningocele.

What causes Spina Bifida?
Spina Bifida is caused by a complex interaction of both genetic and environmental influences that ultimately affect any given pregnancy. The genetic influences remain poorly understood. There are a number of environmental influences which have been identified, though the exact interactions of environmental factors with the genetic influences are also not well understood. The most common environmental factor studied has been folic acid (Hernandez-Diaz, Wetler, Walker & Mitchell, 2001). Other factors include: use of seizure medication during pregnancy (Hill, Wlodarczyk, Palacios,&Finnell,2010),maternalhyperthermiaduring early gestation (Suarez 2004), and maternal obesity (Stothard, Tennant & Bell, 2009). It is known however, that addressing all of these environmental factors before conception does not eliminate all possible causes of SB.

Current recommendation by the Centers for Disease Control and Prevention (CDC) state that all women of childbearing age should take a daily multivitamin with folicacid (400micrograms). Furthermore,women who have had a previous pregnancy affected by SB should take 4 milligrams of folic acid at least one month prior to conception, and through the first trimester (CDC 1991; CDC 1992). Unfortunately, even if all women did this, approximately 30% of cases of SB would still occur.

Medical Issues
Hydrocephalus- Almost all individuals with SB develop hydrocephalus due to the presence of the Arnold Chiarimal formation.For some individual sit appears that they develop some degree of hydrocephalus in utero.For many individuals, hydrocephalus develops post-natally, after surgical closure of the back. In the past, most individuals with SB required placement of a VP shunt to treat the hydrocephalus; but VP shunts are associated with potential complications. Pediatric neurosurgeons are now looking at new treatment options that might eliminate the problems associated with VP shunts; and are questioning through research, whether all individuals who previously got VP shunts actually needed them.

• Mobility -This issue is dependent on the spinal level of the SB lesion. Most children are able to walk (though usually with the aid of orthopedic bracing and assistive devices such as crutches). Some are full-time walkers, but many walk short distances and choose a wheelchair or electric cart for long distances. It is generally only those with thoracic or high lumbar spinal lesions who end up using the wheelchair as their only method of mobility.
• Neurogenic Bowel and Bladder -This issue is common in SB because the nerves that control the bladder, anus and a small segment of the sigmoid colon are controlled by sacral nerve roots (S2-S4). For the majority, bowel and bladder continence is achieved by medical and/ or surgical management. In recent years, a number of treatments have been developed which allow individuals to achieve varying levels of functional continence. The value of functional continence is that it promotes skin health and facilitates inclusion of people with SB to a greater degree of societal participation.
• Intelligence and Learning Issues -Most people with SB have normal IQ scores but may have learning difficulties. These can often be addressed when parents and teachers understand the issues and work together. Despite having normal intelligence, many adults with SB still struggle to live independently. Research
  suggests that there are likely multiple reasons for this, including the occurrence and type(s) of learning issues present, family and individual beliefs and expectations, and potential lack of exposure to peer and societal experiences compared to their non- disabled peers.

Quality of Life in Adulthood

Research over the last decade has demonstrated that self-perceived quality of life for persons with SB does not appear to be influenced primarily by medical issues (though medical issues may influence quality of life at times of medical crisis or stress). Rather, studies have shown that for most individuals with SB, quality of life is more strongly associated with the individual's beliefs and experiences, and that the family also influences the individual's beliefs in his or her capabilities. (Sawin& Bellin, 2010). Thus, lack of independence in persons with SB today appears to be more strongly influenced by learning issues that are inadequately addressed, and by negative attitudes and societal limits.

When Delivering the Diagnosis:

• Acknowledge your level of knowledge about SB. If you don't know, tell the patient you will find the information, or refer them to someone who does know. Choose your words carefully. This will likely be remembered as the worst day of their lives and your compassionate words and approach will echo in their memories.
• Avoid giving a definitive prognosis.There is a broad range of outcomes, and even with the most detailed information available, an accurate prognosis is difficult to provide. Giving parents the potential best and worst case scenarios will help the family begin to adjust to the shock of the situation, and allow them to process the information, so they can make the decision that is best for them.
• Be careful not to infer blame it is no one's fault. While we know a number of factors that might be associated with SB, we really don't understand all the genetic and environmental factors and interactions that cause SB.

Families will be weighing all of their options: Termination or continuing the pregnancy. For some people, adoption may also be an option. For those who wish to continue the pregnancy, the option of fetal surgery exists, although in-utero repair is not a cure for SB.

Fetal Surgery
Fetal surgery as a treatment option began in the late1990's and showed some initial promise related to some of the medical issues seen in SB, particularly the incidence of Arnold-Chiari malformation and hydrocephalus.The NIH-funded Management of Myelomeningocele Study (MoMS) began in 2003 and ended December 2010. The first results of that study were published in 2011 (Adzick & Spong, 2011). Outcomes were evaluated for 158 subjects at 12 months of follow-up.Findings indicated a reduced rate of VP shunt placement in the treatment group compared to the control group (40% versus 82%) ;and a decreased rate of hind brain herniation (64% versus 96%). In addition, some improvement in motor outcomes was noted at 30 months (Adzick & Spong, 2011). However, there were noted potential complications, and the original article did not address all issues affecting individuals with SB, mothers, nor any longitudinal data on health outcomes. While the study shows promise for fetal surgery as a treatment option, some cautions remain, including the concern that fetal surgery may pose risks for subsequent pregnancies due to uterine damage. This is currently being studied. SBA's Professional Advisory Council provided an additional response to this publication on SBA's Web site under their Published Research section. In light of the complexity of an SB diagnosis, it is important that families have access to people who can provide accurate information about what to expect over the long-term in order to make an informed decision.

Referrals

• As soon as possible, refer the patient to a pediatric neurosurgeon, and/or to a SB Clinic to get information on longitudinal care issues.
• Ensure that the people you refer to have the most current diagnostic information (fetal ultrasounds or MRI scans). That will ensure that the consultants are able to give the most accurate information about what to expect in the future should families decide to continue with the pregnancy.
• Families would also benefit from referral to a geneticist or genetic counselor.
• Pregnant women should be referred quickly to a Perinatologistor Maternal- Fetal Medicine specialist. Families who wish to consider abortion will need to discuss that option fairly quickly and should discuss medical issues related to the procedure with their health providers.
• Women who wish to consider the option of fetal surgery will need immediate referral to a center experienced in this intervention. Fetal surgery is done prior to 25 weeks gestation. It is important to note that not all women with an affected fetus are candidates for the procedure.
• Let families know about Spina Bifida Association (SBA), which has many resources that are useful and informative to families. There is an information sheet available to expectant couples on the SBA website. The expectant mother or family can also contact the SBA National Resource Center with any questions. SBA can also help link families to a local chapter that can also provide resources and support.

For More Information
SBA offers a wide variety of in-depth health information sheets which cover all aspects of Spina Bifida care.

Interdependent But Autonomous (how to Raise Your Kids, hee hee O:) {Guest Post}

So here we go….

Moosey is gonna tell you how to raise your kids.

Hee Hee.  Just kidding. 

Well sorta.

I’m just going to impart some knowledge on what I know about growing up as a child with Spina Bifida.  So I guess this entry is geared toward those mothers and fathers of children with special needs, but hopefully all parents can take even a little something from anything I write, regardless of whether or not your child was born with a disability.

So…..parents of children with special needs……I want to share with you something important. Stuff I always tell new parents of young ones with SB.  Stuff I believe they need to hear.  Ready?  Here goes: Your attitude, especially early on, will determine your child’s attitude. Children are likely to live up to what you as parents think of them. If you think your child is not capable of doing anything, neither will they. If you think they can’t succeed, or that they will do nothing with their lives, so will they.

On the other hand, if you believe that your child has potential, that your child has a future, that your child can do whatever he or she wants to do, even if it means they may need some extra assistance or need to find new and different ways to do things, then they will believe it, too. They will grow up confident young men and women and they won’t let anything stand in their way.

Sure, it may take them awhile longer to accomplish things; it may be that they will reach a goal in a different way than other boys and girls. But if you believe in them, they will believe in themselves. Your attitude early on is the single most important factor in determining what they think of themselves and what they will believe they are capable of doing.

And your attitude……needs to allow them to fail.

I have no doubt that all the parents reading this love their children. I have no doubt that you all want nothing but the best for your kids. You all want to protect your kids from pain, from sadness, from disappointment. Just like every other loving parent out there, you don’t want anything bad to happen to your kids.

However, I’ve seen too many parents, particularly in the disability community, “over” protecting their kids. It’s a hard thing for any parent of any child, with a disability or not. You question, “When do I let go?”  “How much should I let go?”  “What if my child fails?”  “What if my child tries something and finds disappointment?”

It’s hard, but I’m here to tell you that teaching your child how to be self-sufficient and as independent as possible is the best thing you can do. Obviously, as with every other kid on the planet, kids with disabilities also are different from one another. And, physically, some of our kids may not be able to do as much as others. That’s okay. Allow them to do as much as they can do. It will not only help their self-esteem when they can do things on their own, but it teaches them responsibility.

For example, I don’t know, let them help you clean the kitchen when they are younger. Maybe they can set up the dinner table. Show them how to clean their rooms, then after a while, let them do it themselves. You will be giving them skills for life. Later on when they reach adulthood, they will be better able to take care of themselves.

I understand that for some of our kids, they will always need some kind of care. That’s okay. Some of our kids will do just fine as adults and on their own.  But some will need more help.  Into adulthood, some may always need assistance of some kind. That’s fine.

That’s when I think of a phrase I first heard in 2003 from a doctor (with SB I might add) in Texas at the first SB national conference I went to…….he said……”interdependent but autonomous”.  And I loved it.

I believe that no one is truly “independent”.  Nobody.  Able bodied or not.  No one is an island and everyone needs somebody for something.  We are all “inter”dependent.  For example when you go and buy a car, you ask for advice, you check with friends, you do your research.  You depend on others for help.  You go and buy a house.  God I hope you ask advice and help on that, or it’s gonna suck to be you!  Or when you have a doctor’s appointment and you need someone to cover your shift at work, you ask for help.  We are all connected.  Nobody is truly “independent”.  And neither should our kids be.  If they need help of any kind, that’s fine, I believe that’s just the human condition.

However, “autonomous” means that even though we may depend on others for some stuff, at the end of the day we need to be responsible for our own actions and our own lives.  We can have others help us, but ultimately whatever happens at the end of the day needs to be one’s own choice and one’s own decision.

Or as Judith E. Heumannm, one of the leading disability right’s advocates in the country, so beautifully put it:  “Independent Living is not doing things yourself; it is being in control of how things are done.”

So….some of our kids, when they become adults, may need things like a home health care someone or other, coming in helping them with grocery buying or whatever.  That’s fine.  They can still be autonomous.  They are still in control of how things get done.

Parents, I know it’s scary. I know that you don’t want them to get hurt; you don’t want them to suffer. As they get older, you may be afraid of other things as well, but there are always options. When they choose to move out, they may be just fine.  All on their own.  Just fine.  But if they need help, there are always programs that teach them life skills. Voc Rehab and whatnot.  And there are hand controls for cars.  There are also home health care workers who can come to their homes once a week to help clean up, to help cook, to help buy groceries, or to help assist in whatever medical needs they might require. There are always options.

And for those that don’t leave right at 18 like you’re “supposed to” (I guess), they can still be “interdependent but autonomous”.  For some, it means they make their own appointments, do their own cooking, and provide for their own transportation. Some have cars and drive themselves wherever they need to be. They still have independence. They have friends. They do things. Some are taking college courses online. Some do volunteer work that really makes them happy and makes them feel good about themselves.

And anyway, “Independence” doesn't mean they have to move out at 18 and live alone.  But…..damn….it does mean…..jeez…..at the very least it means that if they are physically and mentally able to cath on their own (if the doctor deemed it necessary for them to do so in the first place) then oh my god they should!!

OH MY FUCKING GAWD, there is simply no reason a 14-year-old should not have some kind of outside activities or social life, simply because they don’t know how to cath. Fucking pet peeve of mine to see mom doing stuff like that.  Pisses me off and makes me sad like you wouldn't believe!!

OK Moose, calm down…..OK um, where was I….oh yeah…….so don’t misunderstand me. When I say, “let your kids be independent,” I don’t mean “Parents, leave them alone”. Unfortunately, I see it happen where a child with disabilities who is becoming an adult wants to be more independent, wants to learn to do more on their own, wants more freedom. The parents are hurt and confused. They spent their life protecting their kids, loving their kids, trying to shield them from pain and disappointment, and now the child is saying, “I want to do things on my own.”  But for you, the parent, its scary.  Maybe even offensive to hear that.  Yes, offensive.  “I am so and so’s caretaker, their protector.  That’s who I am.  That’s my sole purpose in life.  They need me.”  And I think parents fixate on that identity.  So the parents may react with hurt and confusion and say, “Okay, you want to do things on your own?  Fine. Do things on your own.”  And then they abandon their kids.

Parents, please don’t abandon your kids.

It’s understandable that you would always want to protect them.  They were born with these special needs and from the day they were born, you were all they had to protect them from the big mean scary world.  Good job, go you, you did a beautiful thing. <3

But they grow up.  And you are not doing them any favors by hanging on to that identity of “protector”.  Maybe without that label of “protector and caretaker”, you don’t know who the hell you are….well figure it out!  Because they won’t need the same amount of protecting and care that you gave them when they were 2.  Let go.  But don’t completely abandon them.  That’s just mean.  Don’t be mean.  Find that balance.  I don’t know where that balance is, but find it.

You’re their parents, and they love you, and they will always need you. They are just asking for the same thing most young men and women want: more freedom to fly, more freedom to succeed, and yes, the freedom to fail. When they do succeed or when they do fail, don’t abandon them. Be there, cheering them on, encouraging them, helping them.

I feel I should put a disclaimer here. I didn't grow up being all that independent. I didn't have a mom who, from day one, let me do as much as I could by myself. I had an overprotective mother.

On the one hand, she would have me talk to the doctors and always make my own appointments. Yes, that was very empowering; yes, that taught me some responsibility.

“Tell them what’s wrong.  You talk to them.  It’s your body” she’d say.

“Tell them it hurts. A lot,” she would always advise.

So yeah, very independent with my medical care.  That was all me basically from the time I was 9.

But, on the other hand, she was afraid of my having new experiences. She was always afraid I would get hurt, always afraid of her little boy experiencing pain or rejection or suffering.

There came a point when I finally let my mom know that I wanted more freedom and more independence. She took a long hard look at me and said, “OK. This is new and scary for me, but you’re right. You can do this.”

Whether it was, “You can go to college,” or “You can meet girls, you can date, you can marry,” (“And you can get divorced.” -2011) (“And you can get remarried.” -2013) she was afraid for me, but she let it happen. AND she was still there for me. She did not go from one extreme to another. She did not go from being overprotective of everything I did, to completely abandoning me. She did it right.  Ultimately, she did it right.  She was there when needed, and when I seemed to be doing OK on my own, she left me alone.

To this day, my mom is still like that. I see her almost every day (as every good Mexican son should do  ) and she is still there for me, listening, caring, interested in my life. But the most she ever “does” for me is buy some milk if we run out and she happens to be at the grocery store!  She knows that she has given me so much by letting me have my independence.

Some practical stuff my mom taught me was to clean the kitchen, and she taught me to do laundry; my dad taught me how to manage money. They gave me many practical life skills that I needed for the future. I will always need them, I will always love my mom for everything she is, but I am so thankful for the chance to succeed, and yes, to fail, on my own.

The point is, parents, don’t underestimate your kids. Don’t completely abandon them.  But don’t underestimate them.  Remember that independence and self-sufficiency are two of the greatest gifts you can give your kids. Those skills will help them long after your physical bodies are no longer here to watch over them. Teach them independence and self-sufficiency and you have truly given them the greatest gifts you could ever give.

***Jesus Arroyo is 37 year old man with SB myelo, shunted, ambulatory with braces, Chiari II. But that's not the most important thing he wants you to know about him. The most important thing he wants you to know, and in keeping with the theme of his blog entry, is that life happens to everyone. Typical things and SB things. And we have to be prepared for it all. Mentally and emotionally capable to handle whatever life throws at us. Married for more than 10 years, divorced, and recently remarried, he points out that his divorce is the most difficult thing he's ever gone through. Harder than 20 surgeries, more emotionally taxing than the 2 shunt revisions he had this summer in the time span of 6 weeks, after having no problems with his shunt for 17 years. Hundreds of trips to the ER. But something "typical", something that everyone can go through, a divorce was more painful than anything SB related. Something "the normals" go through. So how do you handle that? Or surgeries? Or anything else life throws at you? With emotional strength, maturity, and the experience you got during the times you failed in life. With family, friends, loved ones and support, but also from learning from your failures. And that's what this blog is about. The power to fail.***

~~~You can follow Mr Arroyo's blog at: http://mooseysramblings.wordpress.com/ For more entries just like this one, be sure and click on his Disability Advocacy link on the right. Leave Jesus some love and tell him Dawn sent you!!! ;-)

Spina Bifida Occulta

What is Spina Bifida Occulta?

Spina Bifida Occulta (SBO) is a group of conditions affecting the spinal column. The spinal column is made of bones, called “vertebrae.” They support the body and protect a large group of nerves, called the “spinal cord.” The spinal cord carries nerve signals from the body to the brain.
SBO is common; 10 to 20 percent of healthy people have it. Normally it is safe and people often find out they have it through an X-ray. Spina Bifida Occulta usually doesn’t cause nervous system problems.

Forms of Occulta
There are forms of Spina Bifida Occulta that do cause problems though. They are:

• Lipomyelomeningocele and lipomeningocele — this is like a tethered spinal cord, except it is attached to a benign fatty tumor;
• Thickened filum terminale — the end of the spinal cord is too thick;
• Fatty filum terminale — there is a fatty lump at the inside end of the spinal cord;
• Diastematomyelia (split spinal cord) and diplomyelia — the spinal cord is split in two, usually by a piece of bone or cartilage; and
• Dermal sinus tract (with involvement of the spinal cord) — the spinal canal and the skin of the back are connected by what looks like a band of tissue.

Signs of Occulta
People can have these forms of Spina Bifida Occulta even if there is nothing wrong with the spine. However, there can be neurological complications associated with SBO. The most frequently occuring complication is a tethered spinal cord. A tethered cord occurs when the spinal cord (usually at the lower end) is not attached correctly to the rest of the body. The cord gets stretched and damaged.

The signs and symptoms of a possible neurological complication like a tethered spinal cord include:

• Pain in the back or legs;
• Weakness in the legs;
• numbness or other changes in feeling in the legs or back;
• Deformed legs, feet and back; and
• Change in bladder or bowel function.

People who could have a spinal cord problem should see a health care provider right away.

What about people with no signs of a problem?
Eighty percent of those with a spinal cord problem will have skin over the defect with:

• a hairy patch;
• a fatty lump;
• a hemangioma — a red or purple spot made up of blood vessels;
• a dark spot or a birth mark — these are red and don’t include blue-black marks, called “Mongolian spots”;
• a skin tract (tunnel) or sinus — this can look like a deep dimple, especially if it’s too high (higher than the top of the buttocks crease), or if its bottom can’t be seen; and
• a hypopigmented spot — an area with less skin color.

No matter the age, people who have these signs should see a health care provider who treats the spinal cord. Not every person with a skin defect of the lower back has Spina Bifida Occulta however, but that can only be determined through physical examination and diagnostic tests. This is especially true for those with sacral dimple. An ultrasound (only for newborns) or magnetic resonance imaging (MRI) may be used to confirm a problem.

This information does not constitute medical advice for any individual. As specific cases may vary from the general information presented here, SBA advises readers to consult a qualified medical or other professional on an individual basis.

Article obtained from: http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8277205/k.5ED4/Spina_Bifida_Occulta.htm

Spina Bifida and Obesity

Obesity among Persons with Spina Bifida

Obesity is the most common nutritional problem in North America, and it is a serious health threat.

How does obesity develop?
All energy intake comes from food that is eaten (measured in calories) 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 which causes 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 resulting in weight loss.

How is obesity determined?
Obesity can be determined a number of ways. Body Mass Index (BMI) is a number calculated from a person's weight and height. BMI can provide a generally reliable indicator most people; and is used to screen for weight categories. The categories that may lead to health problems are overweight and obese. Anyone with a BMI over 25 is classified as overweight; and anyone with a BMI over 30 is classified as obese. BMI is not a direct measure of body fat percentage- it is calculated from an individual's height and weight which includes both muscle and fat.

It may be difficult to get an accurate measure of height in a person with Spina Bifida, so arm-span may be substituted for height in some individuals. Another method is to use calipers to measure skin-folds; and plot 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.

What are the health concerns for obese people?
Obese adults are at risk for: hypertension, dyslipidemia (high LDL cholesterol, low HDL cholesterol, or high levels of triglycerides), type 2 diabetes, coronary artery disease, stroke, gallbladder disease, osteoarthritis, sleep apnea, respiratory problems, some cancers (endometrial, breast, and colon), and psychological problems.

Similar health consequences occur in children and adolescents who are obese. Psychological problems are of special concern for children, who may be negatively stigmatized by others and could develop poor self-esteem, greater risk for isolation from peers, and depression. In some families, food is used to try to compensate for the child's disabilities.

Very young children who have Spina Bifida usually grow at about the same rate as their peers that don’t have Spina Bifida and are quite physically active, so they usually are not obese. As they grow older, and especially if they also have hydrocephalus, they have a very high risk of becoming obese. After age six, at least 50% of children who have Spina Bifida are overweight; and in adolescence and adulthood, over 50% are obese.

Special concerns for individuals who have Spina Bifida
Obesity causes greater health problems for people who have Spina Bifida. Obesity further limits mobility and the ability to manage activities of daily living. This leads to a spiraling problem of decreased energy use and weight gain, making it harder to keep up in social and work situations. Obesity puts more pressure on skin, thereby increasing the already high risk of skin breakdown, particularly in areas that hold moisture.

Social rejection, which may already be a problem due to others' lack of sensitivity and understanding of disabilities, may be worsened. Activities of daily living, particularly independence in dressing, continence management and hygiene, may be negatively affected by difficulties in moving a large, heavy body as well as decreased ability to reach private areas of the body.
Neurological impairments that lead to mobility problems make it harder for individuals who have Spina Bifida to be physically active. Due to the requirements of school and work; 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, so they require a large number of calories for growth. Older children and adults have slower growth, and on average will not become as tall as their peers who do not have Spina Bifida. This means that people with Spina Bifida have fewer nutritional requirements for growth. 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).

Preventing obesity−−a family affair
Prevention of obesity is the only sure method of promoting optimal 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 begin 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.

What are some 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. Most food servings should come from fruits, vegetables, bread and cereals, fewer from dairy products and meats, and only a small amount from fats, processed sugars and other carbohydrates. 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 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.

Help children who have Spina Bifida enjoy exercise. Physical activity has two benefits; it burns calories and decreases hunger by re-setting 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.

I’m obese. How do I lose weight?
Once people become obese, losing weight can be challenging. 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. 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.

Any weight reduction contributes to good health and should be celebrated (but not with food)! Losing weight should be done in small measures, so may take a long time. 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.

This information does not constitute medical advice for any individual. As specific cases may vary from the general information presented here, SBA advises readers to consult a qualified medical or other professional on an individual basis.

Additional resources

www.choosemyplate.gov/
www.ncpad.org/

The above entry is from: http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8277197/k.109E/Obesity_among_Persons_with_Spina_Bifida.htm