FAQS about SB

A: You are now legally an adult and will be able to make your own decisions about your health care. In searching for adult-oriented doctors, there are some things to consider: Everyone should have a primary care physician to go to with common illnesses that anyone can get, as well as health maintenance care like immunizations, cholesterol screening, and blood pressure checks. For you, the best type of primary care adult medicine doctor is most likely a family medicine physician because they are familiar with Spina Bifida. You need a urologist who treats neurogenic bladders and, if you have a shunt, a neurosurgeon. As an adult, since you have stopped growing, you are less likely to need frequent care by an orthopedic surgeon or to have new bone issues requiring surgery. However, you would benefit from a physical medicine and rehabilitation specialist (also known as a physiatrist) who can help with musculoskeletal issues, as well as with bracing, wheelchair, and other equipment assessments. A physiatrist can also help with physical therapy and occupational therapy referrals, if needed. They can also help you find an orthopedist if a surgical issue arises. If you are a woman and are contemplating having children, it is best to discuss all of the issues of a potential pregnancy with an obstetrician who specializes in high-risk deliveries. You should meet with this sub-specialty obstetrician before you become pregnant.

A: Bladder augmentation is a major surgery that should not be undertaken lightly. However, this surgery protects the kidneys, preserves renal function, and makes it possible to stay dry between catheterizations in many children with Spina Bifida. Before resorting to surgical enlargement of the bladder though, clean intermittent catheterization and medication should be utilized. If those measures are not effective in reducing the bladder pressure, then bladder augmentation is a wise choice. However, once a bladder has been surgically enlarged (augmented), a strict catheterization program is necessary to prevent overfilling of the bladder; Overfilling of the bladder increases risk of leaking, bladder rupture, and bladder cancer. You should share specific questions and concerns with your child’s urologist. For more information, review SBA’s health information sheet on urologic care and management.

A: People with Spina Bifida who have any of the following risk factors are at higher risk for developing type 2 diabetes:

• Obesity: People who are overweight, especially those whose fat settles in the abdomen rather than the legs and hips, are at high risk for insulin resistance and diabetes. At least 50% of children and adults with Spina Bifida are obese, putting them at very high risk for diabetes in their life.
• Inactivity/immobility: Increased exercise helps to maintain a healthy weight. Furthermore, exercise burns glucose for energy, increasing the cells’ ability to use the insulin. There are many options for exercise for people with Spina Bifida, both those who can walk and for those who have difficulty with walking or use a wheelchair.
• Family history: Hispanics, American Indians, Asian Americans, and African Americans are the highest risk groups for type 2 diabetes. However, any family history of diabetes should be shared with your physician.
• Personal history of pre-diabetes or gestational diabetes (diabetes during pregnancy).
• Age: Risk increases with age. People over the age of 45 are considered high risk. However, insulin resistance and type 2 diabetes is increasing among children and adolescents. This increase is attributed to obesity, poor nutrition and lack of exercise. To decrease your risk, eat nutritious meals and maintain a healthy weight

For more information, review SBA’s health information sheets on obesity and diabetes.

A: Hydrocephalus can be associated with impairments in cognitive function, learning problems, and behavior challenges. Information about Spina Bifida, as well as the learning challenges and mental health issues that can occur with hydrocephalus, is available in SBA’s health information sheets.

A: Transitioning catheter care is a gradual process and depends on the child’s learning profile, hand-eye coordination, fine motor skills, and body habitus. Delays in cognitive or motor skills should be considered when teaching the child to cath, but should not prevent trying. Usually there are phases to learning how to cath. First is learning how to set up for the procedure. Your child can help get the materials together for cathing. Next, your child can help set up the materials for cathing. To get a feel for when your child can help with the actual cleaning, lubricating, insertion, and removal, he or she can practice with a Cheerio. Sometimes, an occupational therapist can assess fine motor skills and design a program for improving the motor skills needed for cathing and/or suggest positional or other adaptations to facilitate access to the urethral area.

Parents and children can use a hand-over-hand technique to begin learning the sequence of motor movements needed in catheterizing. Eventually, the parent can allow the child to cath under supervision. Some children are able to do this by the time they start school, but many are catheterized by the school nurse. Requesting catheterization training as part of the Individualized Education Program is another way to provide opportunities to learn the proper procedure. Some children, particularly girls, may have difficulty with cathing if there are mobility limitations (i.e. spina rods that inhibit bending) or body size issues. In these situations, or whenever catheterization of the urethra is difficult, the family can consider procedures such as the Mitrofanoff. For more information, review SBA’s health information sheets on toilet training and urologic care and management as well as Health Guide for Parents of Children Living with Spina Bifida.

A: Because Spina Bifida is such a complex medical condition affecting so many different parts of the body, there is a considerable amount of medical information that rapidly accumulates. Personal health records are now available in many formats.
The National Center for Medical Home Initiatives for Children with Special Health Care Needs has a section on their Web site for creating care notebooks. These notebooks are not Spina Bifida specific, but can be tailored to the family’s needs.

For more guidance about creating a personal health record and for a list of software programs and internet sites that offer free and for purchase personal health records, go the The American Health Information Management Association (www.myPHR.com) and select ” Tools and Resources ” followed by ” PHR Tools and Services “. More information on what to include can also be found in the Health Guide for Parents of Children Living with Spina Bifida.

A: You will want to first make sure you are healthy enough to exercise. Inform your physician that you are starting an exercise program. As you choose the right program for you, make sure the activities you choose are varied, fun, and rewarding. Exercise as often as you can, ideally performing strengthening exercises three to four days a week. The National Center on Physical Activity for People with Disabilities(NCPAD) advises that you be active throughout the day – just keep moving.

The Health Guide for Adults Living with Spina Bifida includes a section on physical activity and weight control that is helpful for adults concerned about their general health.  This publication is available at SBA’s Marketplace.

A: There are migraines that can mimic shunt malfunction by causing the same symptoms:

• Basilar-type migraine –Symptoms may include vertigo, double vision and vomiting.
• Sporadic hemiplegic migraine – Symptoms include transient symptoms of hemiparesis and hemisensory changes.

In both of these, the symptoms usually, but not always, precede the occurrence of the headache. Treatment for immediate intense pain associated with migraines is generally ibuprofen, Tylenol or prescription sumatriptan nasal spray. Chronic (long- term) treatment usually involves behavioral strategies (biofeedback, relaxation therapy, etc) and medications – the best studied of these are topiratmate (Topomax), valporic acid, levetiracetam (Keppra) and amitryptyline.
Because shunt malfunction is not always clear-cut, “no improvement” or “worsening” requires re-evaluation by the neurosurgeon and possibly further evaluation of the shunt.  In such cases, the patient needs to be in frequent contact with the primary physician or pediatrician and neurosurgeon. For more information, review SBA’s health information sheet on chronic pain.

A: The Social Security Administration has set some guidelines for different disabilities. It is important to view their Web site and discuss your options first with an SSA representative.

• http://www.ssa.gov/disability/ or 1-800-772-1213

Making sense of health care coverage can be confusing. These sites are very informative in making it all easier to understand and navigate:

• Learn how to get insurance. www.covertheuninsured.org/stateguides
• Robert Wood Johnson Foundation www.rwjf.org

These groups can help with obtaining prescription coverage:

• Partnership for Prescription Assistance: 888-4PPA NOW or 888-477-2669; www.pparx.org
• Together Rx Access: 800-250-2839; www.togetherrxaccess.com
• Hill Burton Facilities: 800-638-0742; www.hrsa.gov/hillburton

These sites can help with finding health insurance:

• High Risk Pools for Insurance: www.healthinsuranceinfo.net
• Insurance for Children: 877-543-7669; www.insurekidsnow.gov
• State Children’s Health Insurance Program (SCHIP): 877-543-7669

A: There are vaccines available that do not contain latex, so risk of latex exposure is not a reason to avoid receiving the flu vaccine. The provider of the vaccine should tell you about latex contamination in the product. For more information, review SBA’s health information sheet on latex allergy as well as SB University.

A: Anxiety can come from internal and external sources. Your neurology team should first make sure the shunt is functioning properly. Afterwards, a mental health specialist can help you identify the sources of your anxiety and help you reduce it, usually through counseling and medication. For more information, review SBA’s health information sheets on depression and anxiety.

A: Neurogenic (neuropathic) joints may occur when sensation is diminished, particularly when there is also a chiari malformation or syrinx (cyst). Muscle weakness around the joint also contributes by making the joint less stable and subjects bones to damage in the shoulders hips, knees, and ankles. Talk with your musculoskeletal team (orthopedist, physiatrist, physical therapist, and/or orthotist) about how to minimize stress on your joints. For more information, review SBA’s health information sheet on hip function.

A: Back pain should always be assessed to rule out a serious problem. This is best done by an MRI. If there is no spinal cord involvement, or treatable spinal problem, you should work with a physical therapist and trainer on back strengthening exercises, proper stretching, and warm up exercises. If the pain persists, consider visiting a sports medicine specialist. For more information, review SBA’s health information sheet on pain.

A: Although short stature is common in Spina Bifida for many reasons, one concern about precocious puberty is that in addition to premature sexual development, there is premature bone development which causes the growth plates of the bones to mature and fuse early; thereby limiting further growth. For more information, review SBA’s health information sheet on precocious puberty.

A: The M.A.C.E. (Malone antegrade colonic enema) procedure has been very useful in helping individuals with Spina Bifida become more continent of stool and have controlled bowel movements. Glycerine is used to help move the stool through the colon and is not absorbed into the body. Therefore, there is no risk to the developing baby. The salt and water will mostly be flushed through the colon with the stool. A small amount will be absorbed into the body, but salt and water are natural to the body and won’t cause any harm.

A: Spina Bifida can happen in any pregnancy, and 95% of the occurrences have no family history. The exact cause of Spina Bifida is unknown but likely involves a combination of genetics and environment. Factors that increase risk of a Spina Bifida-affected pregnancy include:

• Previous pregnancy affected by Spina Bifida
• First, second, or third degree relative living with Spina Bifida
• Taking valproic acid or carbamazepine (anti-seizure medications)
• Having diabetes before the beginning of pregnancy (not gestational diabetes)
• Inadequate folic acid intake
• Pre-pregnancy obesity
• Low vitamin B-12 levels in the body
• Having high temperatures early in pregnancy (which may include fever, or exposure to the heat of hot tubs, saunas, and tanning beds)

A: The two are inseparable because the same nerves that control urinary function also control sexual function. Therefore, if one has poor sensation, the other will also. It is very important to have an understanding partner and to communicate clearly about the possibility of urine or stool leakages during sex. Decrease the risk by emptying your bladder and bowels beforehand. This should take care of most of the urine and stool at risk for being accidentally released during sex. In case there is some leakage during sex, have a moist towel nearby to wipe it away. For more information, review SBA’s health information sheets on women’s and men’s health issues.

A: CTS is a common complication in people who use wheelchairs. There are several possible treatments for CTS, usually starting with non-surgical options like wrist splints and resting, anti-inflammatory medications, and then progressing to surgery if all else fails. CTS will not improve if the repetitive wrist movements do not decrease.

A: SBO is a minor deformity of one or two vertebrae that doesn’t get much attention, yet it can significantly decrease quality of life for some people who suffer from pain or neurological problems associated with it. In such cases, SBO is a more serious problem and requires a thorough neurosurgical assessment of the patient’s symptoms, and an MRI scan. Treatment recommendations can be made after all information is considered.

A: You should discuss this with your son’s neurosurgeon who knows the full picture. MRI studies almost always show re-tethering of the spinal cord after primary myelomeningocele repair and/or tethered cord release. The concern for symptomatic re-tethering occurs when there is back or leg pain; new or progressive weakness, tightness (spasticity) or spasms of the legs; or changes in the bowel or bladder function. If these problems are due to symptomatic tethered cord, the urodynamic study usually shows changes in bladder pressure, sphincter pressure, and/or muscle contractions. For more information, review the health information sheet on tethered cord.