Frequently Asked Questions about Spina Bifida
Click on the topics and questions below to see their corresponding answers.
As an overweight 21 year old with SB what concerns should I have?
: In adults, and recently in children, obesity has become a major health risk. In the general population, obesity has been linked to high blood pressure, diabetes, osteoarthritis, abnormal cholesterol metabolism, heart disease, sleep apnea, and psychological problems.
For people who have Spina Bifida, even more problems are associated with being seriously overweight. Approximately 50% of people with SB are obese. Weight problems make all activities of daily life more challenging, like maintaining mobility (or fitting well in your wheelchair) and good hygiene. This makes it harder to keep up in social and work situations. It adds to the amount of pressure on skin, thus increasing the already high risk of skin breakdown. Social rejection, which may already be a problem due to others' lack of sensitivity and understanding of disability, may be worsened.
Activities of daily living, particularly independence in dressing, continence management and hygiene (reaching those hard to get private areas) may be negatively affected by difficulties in moving a heavy body. This leads to a spiraling problem of decreased motivation, minimal energy use and weight gain. The good news is that health problems associated with obesity can be significantly decreased by adopting a healthy eating and exercise plan.
The SBA has more information on this topic in our Obesity information sheet.
I have heard that some flu vaccines contain latex. Should I avoid getting my flu shot if I am latex sensitive?
The risk of latex exposure is not a reason to avoid receiving the flu vaccine. There are vaccines available that do not contain latex. If you have had a severe (life threatening) reaction to a previous flu vaccine; or from latex exposure, you should speak with your physician prior to receiving vaccinations. Pain and redness at the site of the injection is not considered a severe reaction.
The FDA and the Latex allergy association have identified the vaccines this year which could pose risks for latex sensitive people; and those links are available here.
All people are encouraged to speak with their physician for individual advice, but in general, the medical office or pharmacy that is providing the vaccine, should be aware of any latex contamination in the product, and should share that information with you.
More information about latex allergy can be found at here.
The SBA also has a very informative latex allergy presentation from our 2010 national conference that can be viewed by registering with Spina Bifida University www.sbuniversity.org
Is it true that poinsettias can cause an allergic reaction to people who have latex allergy?
Poinsettias come from the same "family" of trees as the Hevea Brasiliensis (the natural rubber tree that is linked to latex allergy). Because they have similarities, poinsettias do have latex in their circulatory system, and research shows that up to 40% of people who are sensitive to latex may also have a reaction to poinsettias. However, there are some important differences. The risk of a serious reaction from exposure to poinsettias is very low. This is because the latex proteins are not airborne like they are with the Hevea Brasiliensis tree. Therefore, the person's airway and blood stream are not affected. This is where the important distinction is made between a mild reaction and potential life threatening one.
Although there is little research on the subject, a research team studied the effect of latex exposure via poinsettias in the 1990's and found that poinsettas cause a reaction from direct contact only. Therefore, exposure is only on the surface of the skin. In order to get even a drop of latex on the skin, the plant needs to be damaged by breaking the stalk or leaf. Even then, the small amount of latex that can be extracted from the plant could easily be wiped off the skin. For that reason, exposure to a poinsettia should not cause alarm; but in general, the rule of thumb is to avoid exposure to any natural latex. So, maybe this is the time to ring in the holidays with another lovely flower, and avoid the poinsettias.
Please see the latex allergy association newsletter for providing this helpful information or click here.
I read somewhere that drinking tea can affect absorption of folic acid in some women. Is there some truth to this?
There has been some research which suggests that consumption of some black and green teas inhibits absorption of folic acid/folate.
The abstract of one such study can be found by clicking here.
It is important to note that this is most likely preliminary research into the topic, and therefore more studies, similar to this one will be needed to gain a better understanding of the interaction between tea consumption and folic acid bioavailability. Furthermore, there are many factors to consider in any research study, so this study should be used for general informational purposes only, and interested people should retrieve and read the entire article to clearly understand the issue.
My sister has a baby with Spina Bifida, should I increase my folic acid intake?
This is an important question for all women who could potentially become pregnant. It is now an established fact that taking a vitamin with folic acid is an important step women can take to ensure a healthy baby. Well-conducted research has demonstrated up to a 70% reduction in births of babies with Spina Bifida when women take 400 micrograms (0.4mg) of folic acid daily. This applies to all women, not just those who have already had a baby with Spina Bifida.
Some women need more folic acid
If a woman has delivered a child with Spina Bifida, she should take 10 times the typical dose of folic acid - 4mg daily. Taking this dose of folic acid can reduces the recurrence of Spina Bifida in subsequent pregnancies.
Less is known about the likelihood of delivering a baby with Spina Bifida for women related to mothers of children with Spina Bifida. These women should take at least the regular daily dose of folic acid - 400 micrograms (0.4mg). Some would suggest that these relatives of children with Spina Bifida take the higher 4mg dose of folic acid for 3 months before attempting pregnancy and for at least 3-4 months into the pregnancy.
How to get more folic acid
The high dose of folic acid (4mg) should not come from taking extra multivitamins or prenatal vitamins because this could provide excessive amounts of the other vitamins. Instead, folic acid-only vitamins, which can be purchased at most pharmacies and health food stores, should be taken in a quantity that equals 4mg daily.
Although there is less science behind recommending 4mg folic acid for relatives of mothers having children with Spina Bifida, the risk to women taking this dose is minimal and the potential benefit is considerable. Therefore, I would suggest that the 6-8 months of high folic acid use is something to consider for women in this unique situation.
General SB Info
Can a person get Spina Bifida at the top of the spine?
: Yes. Spina bifida (open spine) can occur with any of the vertebrae. The condition occurs in up to 10% of the population and is more common in the lower spine. Usually it is found by accident when the spine is x-rayed for another reason. Spina Bifida is the non-union of the vertebrae during development, usually in the first month of pregnancy. This means that the bony part of the vertebra does not form a complete circle and there is an open part in the back – essentially a hole in the spine. While the spine has a defect in it, the spinal cord, which contains all of the nerves, may be unaffected.
When the spinal cord is affected there can be several different conditions from a tethered cord, to a fluid sac in the cord (syrinx), to an outpouching of the cord through the hole in the spine (myelomengingocele). These are usually diagnosed though an MRI.
If your Spina Bifida was found by accident when looking for another diagnosis, I do not think you need to do anything else. If you were having pain, weakness, or unusual sensations, particularly in your hands or arms, you may wish to have an orthopedist or neurosurgeon review your symptoms and test results.
Is Alopecia common in people with Spina Bifida, specifically Myelomeningocele?
Alopecia is hair loss and it has many potential causes. It can be due to genetics (i.e. male pattern baldness), medication side effects, hair products, hormonal imbalances, or other medical conditions such as scalp infections, autoimmune disorders, pregnancy, or mental health problems.
Alopecia is not directly related to myelomeningocele, but there can be hormonal irregularities as a result of hydrocephalus that may be related to hair loss. Therefore, it would be wise to look for changes in the level of hormones that come from the pituitary gland, as these may occasionally be different for someone with hydrocephalus.
It is also a good idea to ask your doctor or pharmacist if hair loss is a side effect of any medication you are currently taking. Also, think about any new soaps, shampoos, hair gels, perms or other chemicals your hair may have been exposed to.
Most often hair loss is a transient problem and is related to the stress of an illness or pregnancy. Usually the hair thickness returns. Of course, there is some hair loss/thinning commonly associated with getting older. If several family members are thin on top, it would not be surprising that you will follow the same course.
General medical /family doctors and dermatologists can be helpful in determining a cause and any potential treatments for alopecia.
I am an adult with Spina Bifida. How often should I see my physicians?
Adults with Spina Bifida should receive routine follow-up and surveillance of their shunts, kidney and bladder function, and mobility equipment.
The Spina Bifida Association recommends neurosurgical assessment of shunts at least every two years. There is a trend away from using frequent CT scans to monitor for subtle shunt malfunction, so the preferred way to look for increased brain pressures is by a dilated eye exam. Therefore, individuals with shunts are advised to see an ophthalmologist every year.
Individuals with neurogenic bladders should have a yearly assessment of their kidney function and see a urologist with expertise in bladder dysfunction. The Spina Bifida Association recommends a renal ultrasound every two years to look for hydronephrosis (swelling of the kidneys' collecting system) and hydroureter (swelling of the tubes between from the kidneys and bladder). If there is a change in dryness or an increase in urinary tract infections additional testing (i.e. urodynamics) is also advised.
Annual assessment of mobility aides, including braces, canes, crutches, walkers, wheelchairs, and cushions will protect skin, bones, and joints. The providers with the best training in evaluating mobility aides are physical therapists, orthotists, orthopedists, and physiatrists, so an annual visit with at least one of these specialists is recommended.
Finally, all adults need regular preventive services in primary medical care and dentistry so annual visits to the primary care doctor and dentist are important. The Health Guide for Adults Living with Spina Bifida includes a Personal Health Care Record that is helpful for keeping track of medical visits.
Does going through an early and irregular puberty really mean you will stop growing at an earlier age?
According to Adrian Sandler, M.D., in his (1997) book, Living with Spina Bifida
, many variables affect growth and ultimate height of a person with SB and short stature is common. Some of those factors include: level of lesion, other spinal deformities like scoliosis; and limb abnormalities.
Higher SB lesions often result in shorter stature. People with a lesion at L3 or higher typically reach less than the third percentile of height in comparison with their non-affected peers. This occurs in only about 40% of those with lower lesions.
Puberty also affects growth and height. Normal puberty begins around 11 years old. In precocious puberty, it occurs much earlier-before the age of 8 in girls; and before age 9 in boys. One major 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.
All children who experience precocious puberty should be assessed by an endocrinologist, especially if the bone age is advancing rapidly and there are concerns about restricted height. Some children receive hormone treatments to slow the progression of puberty. Other information on this topic, including the psychological impact of precocious puberty, can be found in Dr. Sandler's book.
Dr. Sandler's book can be found at amazon.com.
Over-use (repetitive use) soft tissue injury
Is surgery usually the best treatment for carpal tunnel syndrome, or should a non-surgical intervention be considered?
Carpal tunnel syndrome (CTS) is a common complication in people who use wheelchairs for much of their mobility. CTS occurs when the area in the wrist, where a major nerve to the hand (the median nerve) traverses, becomes inflamed/irritated from repetitive motions such as pushing the wheels of the wheelchair to propel forward. This is called a repetitive motion injury. It can cause pain and numbness, initially from the wrist out towards the fingers. As the problem worsens, the pain and numbness can start to move up the arm.
There are several possible treatments for CTS, usually starting with the least invasive non-surgical options, and progressing to surgery if all else fails. Sometimes, if the severity of the condition is already advanced, surgery is the best option because the more conservative treatments may no longer work. Testing with EMG/NCS (electromyelography/nerve conduction studies) may help determine how severe the irritation of the nerves has become. These tests are often performed by neurologists or physiatrists (physical medicine and rehabilitation specialists).
Conservative treatments prior to surgery include:
- Wearing wrist splints that limit the bending of the wrist. These are most effective if worn as much of the day as possible. It is also important to wear them at night because most people bend their wrists when sleeping. Many wheelchair users find wearing wrist splints difficult when trying to propel the wheels.
- Making sure the wheelchair is fitted appropriately for the user, so as to minimize the repetitive movements at the wrist.
- Using alternative mobility such as power-assist chairs, full-power chairs, or scooters.
- Taking anti-inflammatory medications with the input of a physician.
- Injecting anti-inflammatory steroid medication into the carpal tunnel space.
The most important conservative, non-surgical approach is to rest the wrist and minimize the repetitive movements. None of the above treatments are curative if the repetitive injury to the wrist continues.
Surgery usually involves cutting the band-like tissue (fascia) and opening up the carpal tunnel space. This can be curative but will require a period of recovery where the wrist cannot be used. Even after this surgery, modifications will be needed to minimize repetitive movements at the wrist to prevent recurrence. In a small percentage of people some pain and numbness persists after the surgery.
There are several specialists who can provide valuable input into your decision. These include the previously mentioned neurologist or physiatrist. Also a wheelchair vendor can demonstrate mobility options with less stress on the wrist. A physical and/or occupational therapist can also demonstrate ways to minimize the workload on the wrist.
How can you distinguish between a neurological symptom of SB and anxiety?
This is a great and complex question. Anxiety can come from internal and external sources. External causes include stressful situations like public speaking, job interviews, meeting new people, school tests, etc. Internal causes of anxiety include medical conditions such as excessive thyroid hormone (hyperthyroidism), shortness of breath from asthma or smoker's lung (COPD), or chronic pain conditions. There are many other medical conditions that cause anxiety symptoms.
There are several internal psychiatric conditions of anxiety, including generalized anxiety disorder, adjustment disorders with anxiety, and panic attacks. Obsessive-compulsive disorder is also a type of anxiety condition. These technical classifications are ways to describe anxiety conditions which cause difficulties for people during everyday life. These conditions are neurologically-based, likely involving problems with communication between the nerves in the brain. Situations which promote these conditions include emotional trauma such as after an accident, following abuse, or during family strife like divorce or unexpected death.
It is very possible for someone living with Spina Bifida to experience anxiety for many of the reasons mentioned above. The structural changes in the brain associated with Spina Bifida could result in the nerve communication difficulties and brain chemistry problems leading to anxiety. Dealing with the multiple medical issues of Spina Bifida such as mobility challenges, bowel and bladder difficulties, chronic pain, and seizures can cause anxiety.
Another neurologically-based problem in Spina Bifida (usually when there is also Chiari malformation and hydrocephalus) is the social challenges that occur from non-verbal learning problems and visual-motor challenges. These brain-based impairments, which hinder social interactions with others, can lead to anxiety.
Finally, one must always consider the ventricular shunt in any neurologic symptom. While isolated feelings of anxiety without other symptoms is not a usual presentation of a shunt malfunction, if you are suddenly having anxiety symptoms without an identifiable trigger, the shunt should be evaluated. Anxiety-like symptoms including racing heart, sweating/flushing, nausea/vomiting, and dizziness are shunt symptoms that need attention.
So the bottom line is your anxiety is likely coming from many different causes both internally and externally. There isn't a good way to separate SB symptoms from primary anxiety symptoms. 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 what you can do to reduce your discomfort from it. Both counseling and medication can be beneficial. There is more information on anxiety in the "Living With Spina Bifida" section of our Web site.
Is there such a condition as neurogenic joint?
Neurogenic (aka neuropathic) joints are associated with conditions where sensation is diminished. These joints can be a result of absent or diminished sensation associated with Spina Bifida, particularly when there is also a Chiari malformation or syrinx (cyst) in the spinal cord. Muscle weakness around the joint also contributes by making the joint less stable and subject to frequent trauma which is what causes damage to the bones. Shoulders are often affected when an individual with Spina Bifida relies on the upper extremities to assist in propulsion (either with the wheelchair or from using forearm crutches). As the shoulder was not designed for such a work-load, it can be damaged from overuse and load stress.
Other joints that can be involved are the hips, knees, and ankles. The lower extremities are more at risk for someone who is ambulatory compared to someone who uses a wheelchair for mobility. Using properly fitting braces and crutches can decrease stress loads on the lower extremity joints. Using a wheelchair for longer distances can also help the lower joints, but will increase stress on the shoulders. Using propulsion assistance (power-assist or electric chairs or scooters) can alleviate some of the stress on the shoulders.
I recommend talking with your musculoskeletal team (orthopedist, physiatrist, physical therapist, and/or orthotist) about how to minimize the stress on your joints.
Spina Bifida Occulta (and Pain)
How can I get help for painful Occulta when my doctor says that it does not cause pain?
Spina Bifida Occulta (SBO) is a condition that often does not get much attention. Yet, it can significantly decrease quality of life for some people. SBO involves a minor deformity of 1 or 2 vertebrae, usually in the lower spine. Many people have it and never even know because it is not symptomatic in most cases. However, in some people, the spinal cord, which normally swings freely in the lower spine, becomes "tethered" or attached to the deformed bones, and becomes taut. As the person moves normally, the taut cord is pulled and stretched, causing chronic pain in the low back and leg(s), muscle weakness in leg(s), problems with walking, and sometimes, loss of urinary or bowel control. These symptoms are called, "tethered cord syndrome." In children with SB, a tethered cord is almost always released through surgery at a young age, often before any signs of a problem. In some cases though, as in SBO, there may be no symptoms until adulthood.
In adulthood, symptoms from a tethered cord can be very debilitating. They can be relieved through pain meds or alternative therapies, but both provide only temporary relief in the best case. There is only one recognized medical treatment for a tethered cord, which is tethered cord release surgery to "de-tether" the spinal cord from the vertebra(e) that it is attached to. The surgery has varying degrees of success for many people, and the outlook for adults is often different from that of children. To determine who will benefit from the surgery, the neurosurgeon will need to make a thorough assessment of the patient's symptoms, perform an MRI scan, and make recommendations based on that information.
If surgery is not recommended and pain medications alone are not effective, some people find alternative therapies to be helpful. Comprehensive pain management programs, water aerobics/therapy, physical therapy, passive stretching, acupuncture, massage, and heat or ice are most commonly used.
What type of treatments or nutritional supplements can I give my 17-year-old son who is an outstanding athlete in spite of his back problems from Occulta?
Congratulations on your excellent athlete. Spina Bifida Occulta (literally- hidden open spine) is a common finding occurring in 10% or more of the population. For most people, it is an incidental X-ray finding that is diagnosed when looking at the spine for other reasons, commonly during X-rays after a car accident. Usually Spina Bifida Occulta involves only the bony vertebrae. The defect is a non-union or hole in the vertebrae so they do not completely surround the spinal cord. Usually this is in the lower (lumbar) back or near the butt bone (sacrum) and involves 1-3 vertebrae.
Because your son is having back pain, it is important to consider an underlying problem with his spinal cord. This is best assessed by an MRI, which can look for developmental abnormalities of the spinal nerves, such as a tethering of the cord to the wall of the spinal canal, a thickening of the spinal cord, or splitting of the cord. These can all be seen on an MRI. If your specialist was a neurosurgeon or an orthopedist who has expertise in spine disorders, and this doctor reviewed the MRI, you can be comfortable that none of these findings were present.
As for back pain in young men with Spina Bifida Occulta and no spinal cord involvement, I would first attribute this to typical mechanical back pain like any high performance athlete can have. I would work with a physical therapist and trainer on back strengthening exercises, proper stretching, and warm up exercises. If the pain persists despite this, I would recommend a sports medicine specialist.
Aside from overall good nutrition, adequate calcium and vitamin-D intake, and good hydration, I do not know of any supplements helpful to the young athlete with Spina Bifida Occulta. I am not aware of any clinical trials in this area. I do not think participation in basketball is hurting his back. Remember 10% or more of the population also has this diagnosis, many who play sports.
As a 65 year old recently diagnosed with Spina Bifida, should I be more careful when I do things now or in the future?
The diagnosis of Spina Bifida is almost always by X-ray and/or surgical findings. The term Spina Bifida (open spine) means that the spine did not form correctly and there is an opening in the vertebrae. Because of this opening the spinal cord is not completely surrounded by a bony encasement and sometimes there is also disruption of the normal spinal cord development. All of this occurs very early in pregnancy and does not develop after birth. There is a blood test used in pregnant women to help screen for a developing fetus who may have spina bifida. The test, called alpha-fetoprotein is abnormally high in the mother's blood stream. This would lead her obstetricians to do follow-up tests – radiographic tests – to look for spina bifida in the baby.
After birth, Spina Bifida is evaluated most often by spinal X-ray and or MRI. Blood tests do not help in the diagnosis.
For an adult, an abnormal alpha-fetoprotein test result means something completely different and I would recommend follow-up tests looking into liver problems or other adult-onset conditions. There is more information on alpha-fetoprotein at MedlinePlus from the U.S. national Library of Medicine and the National Institutes of Health.
A diagnosis at this age – usually during a back X-ray for another reason – is an incidental finding. This means there is no health concern. Most people diagnosed with spina bifida at age 65 would be classified as having Spina Sifida occulta (literally - hidden open spine). This finding is the non-fusion/closing of the vertebrae mentioned above and is almost never associated with any neurologic problems. Approximately 10% (or more by some estimates) of the adult population has Spina Bifida occulta and never know about it. Again, this X-ray finding in adults is not associated with an abnormal alpha-fetoprotein. You should look for other causes of a high alpha-fetoprotein.
My 11 year old has Spina Bifida, a low grade, and her left kidney is in bad shape and an augmentation surgery (part of the colon will be removed and placed on her bladder for more urine storage) has been recommended.. Can anyone help me decide by way of more information about the surgery?
Bladder augmentation is a major surgery that should not be undertaken lightly, however, this surgery has proven to protect the kidneys, preserve renal function and make it possible to stay dry between catheterizations in many children with Spina Bifida over the past several decades. Before resorting to surgical enlargement of the bladder, clean intermittent catheterization and medication should be maximized. If a good catheterization program with medication is not effective in reducing the bladder pressure to a safe level, then bladder augmentation is a wise choice. It is true that there is the risk of leaking, rupture and bladder cancer but there are things to do that decrease those risks.
One very important thing that everyone in the family must understand is that once a bladder has been surgically enlarged (augmented) it is extremely important to be sure to stay on a good catheterization program. One of the things that cause the bladder to leak or rupture is overfilling. Because there can be a longer period of time between catheterizations without getting wet, some children and young adults often will not do their catheterizations as often. When this becomes a habit, the risk for leaking or rupturing increases. The person with an augmented bladder must be very responsible and take good care of their bladder. Tumors (cancer) in the bladder after having an augmentation have been reported. Although this is a rather small risk, the urologist will look into the bladder with a scope every year beginning around 10 years after the surgery to make sure there is no sign of any tumor.
If a person had an augmented bladder and develops a high fever, has abdominal pain or has signs of infection that do not have an obvious source (such or step throat or virus etc.) it is important to rule out leaking from the bladder. Testing can be done to check the bladder and determine if there is a problem.
If the bladder is not behaving and is putting pressure on the kidney and the kidney is deteriorating, the pressure has to be relieved or the kidney function will decrease. The kidney is an organ that does not "heal itself". Once kidney damage occurs it is permanent. When kidney function is lost, it is lost forever. Everyone needs good kidney function to live well. There are several ways to reduce the pressure in the bladder. A subrapubic catheter can be place through the abdominal wall and a leg bag can be worn to drain the urine. This is usually just a temporary measure and is not a good long term solution. The surgeon can make an opening into the bladder a let the urine just spill out onto a diaper. This is called a vesicostomy and is useful in infants and small children, but is not very satisfactory in older children or adults. Bladder augmentation is by far the best solution for a good life in a person who cannot control their bladder pressure with medicines and intermittent catheterization whose kidneys are deteriorating.
Cord Blood Banking
Please explain what umbilical cord blood could do for people with Spina Bifida. What hospitals do this?
Umbilical cord blood is one type of method for retrieving stem cells. Stem cell transplants are an important part of current and future treatment of some diseases which affect the blood and immune system, such as leukemia and some types of anemia. There is currently no indication of its use in the treatment of SB. This is because stem cells can only be transformed into blood cells at this time-not other tissues. However, the future holds many exciting possibilities that stem cells may be used to treat neurological disorders like Parkinson's Disease and spinal cord injuries. On that note, there is hope for Spina Bifida also as the technology becomes more advanced and widespread.
The March of Dimes website has a very informative link on the topic of umbilical cord blood and its relevance to stem cell transplants which may be of interest.
Furthermore, the National Marrow Donation Program has provided a list of hospitals that participate in the cord blood banking program.
Are people with Spina Bifida Occulta at an increased risk of osteoporosis?
We do know that both children and adults with repaired myelomeningocele have lower bone density in their lumbar spine and in their hips (greater trochanter) than the general population. This is true for both those who ambulate and those who use wheelchairs for the majority of their mobility. The decrease in bone density seems to be greater for those who do not ambulate, but may be due to other associated medical conditions rather than the lack of walking itself. The associated medical conditions that also impact bone density include kidney failure, use of anti-seizure medication, taking steroids for long periods of time, and associated genetic conditions such as Down syndrome.
There are no studies that I know of which show benefit from taking bone density medications in patients with repaired myelomeningocele. It is probably helpful to ensure adequate calcium and vitamin D intake. Adults should ingest 1500mg of calcium and 800 IU of vitamin D daily, particularly if they do not spend much time outdoors. If you are prone to kidney stones, you will want to talk with your health care provider before starting calcium or vitamin D supplements, as the risk of developing stones while on these supplements is not entirely agreed upon.
As for those with Spina Bifida occulta, there is less known. If there are no spinal cord issues, such as a tethered cord that interferes with the ability to ambulate, I don't think the bone density of the normally developed vertebrae should be decreased. The affected vertebrae that are not fused will be recorded as having a lower density because they are incompletely developed. Thus, I would suggest studying only to the fully fused vertebrae or the hip (greater trochanter) for the official measurement. As for those with myelomeningocele, calcium and vitamin D supplements should be helpful if there is insufficient intake in the diet.
Physical Fitness / Exercise
Do you have any exercise or safety tips for adults with SB who want to become active?
Make sure you are healthy enough to exercise. Inform your physician that you are starting an exercise program.
- Choose the right program for YOU and make sure to keep activities that you choose VARIED, FUN, and REWARDING.
- Exercise a minimum of five days a week, ideally performing strengthening exercises 3 to 4 days a week.
- Be active throughout the day - just keep moving
General Guidelines and Safety
Many people with Spina Bifida have latex allergy. If this is the case, always check beforehand to make sure that the exercise equipment is not made of latex. Equipment manufacturers such as Thera-band® offer latex-free versions of their products.
Breathing - Breathe normally while performing the exercises. Do not hold your breath at any time. To make sure that you are not overdoing the exercises, you should be able to maintain a normal conversation. If you are exercising alone, it is best to count out loud while doing the exercises. A useful technique is to slowly count 1-2 when performing the exercise action, and count 1-4 when going back returning to the starting position.
Posture - Sit up tall with both feet planted firmly on the floor.
Back Support - To make sure that your lower back is supported, roll up a small towel and place it behind your back.
Rest - Give your muscles a day's rest between strength training sessions. Your muscles may feel sore a day or two after you've started a new exercise. IF you are sore, wait until soreness has diminished before going back to strength training. Consult with your doctor if the muscle soreness lasts longer than two days. In addition, if muscle soreness last 2-3 hours after exercise, you know you have done too much. During the next exercise session, decrease the number of repetitions, sets, or weight until you find the right settings for you.
Am I at greater risk for a heart attack as a wheelchair user who doesn't do regular aerobic activity?
You ask an excellent and important question. There is no information specifically looking at individuals living with Spina Bifida, but our knowledge of the general adult population probably applies. Risk factors for heart attacks include the following:
- High cholesterol
- High blood pressure
- Family history of heart disease and early heart attacks
- Males over 50 years of age and post-menopausal females
Therefore, it is important to see your primary care provider regularly to address these risk factors. Smoking is probably the most important risk factor to try and avoid and there are newer treatments to discuss with your doctor if you are ready to quit. High cholesterol, high blood pressure, and diabetes can be treated and should be screened for regularly. Family history cannot be modified but your doctor should be aware of this risk factor.
Obesity is a difficult risk factor to control in the United States these days. We have an epidemic of obesity and people with Spina Bifida are included. The challenge for those with Spina Bifida is maintaining a healthy weight through appropriate diet and exercise. Both of these goals can be difficult if there are significant mobility and weakness issues.
Regular physical activity has been shown to protect against coronary heart disease. Any activity is better than none, but ideally at least 30 minutes of aerobic activity 3 or more days per week is best. Aerobic exercise for those with Spina Bifida can occur through adapted sports programs, gym memberships with assistance by those with therapeutic recreation or physical therapy backgrounds, or via a home program. The National Center on Physical Activity and Disability has information on exercise and fitness for individuals with disabilities, including some resources specifically for those living with Spina Bifida. Go to www.ncpad.org to learn more.
The other important part of fighting obesity is a healthy diet.Those with Spina Bifida likely burn fewer calories at rest due to a lower muscle mass. They also burn fewer calories if they do not have motor movement in the lower extremities (legs and feet). Therefore, many individuals with Spina Bifida need fewer calories in their daily diet. Many would maintain an optimal weight if their calorie intake were around 1000-1100 calories per day, which is significantly less than the typical American diet. Since each person is unique, it is best to discuss your individual daily calorie needs with a nutritionist who can also provide suggestions on how to shop for and prepare a balanced diet that would meet those calorie needs.
Although these exercise and nutrition goals are not easy, it is most important to attempt some physical activity and pay attention to how much you are eating everyday. Little things like using your manual wheelchair for shorter distances, substituting water for sugar drinks, lifting lights weights while watching television, or not going back for second helpings can all add up to help control your weight.
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.
Tethered Spinal Cord
My son was born with tethered cord and has not gained 100 % control over his bladder and rectum. I am not sure it is because of some medical problem, or if he is too lazy to go to the toilet. Kindly advise if we need to go for surgery or what next for us.
Discuss this with your son's neurosurgeon who knows all of the information, not just what is presented here. Having said that, from what you describe here, I do not think there is an indication for tethered cord releasing surgery. The most important features in your scenario are that there is no pain, change in neuromuscular function, or abnormalities on his urodynamic study.
MRI studies almost always show re-tethering of the spinal cord after primary repair or myelomeningocele and/or tethered cord release. One can almost consider this as an expected part of the post-operative healing and scaring process. The concern for symptomatic re-tethering is raised 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.
Your son's bowel and bladder control seems quite good. Having a scheduled bathroom routine for bowel and bladder, as well as a contingency plan at school for the occasional accident, is an excellent idea. As constipation is a big problem in Spina Bifida and can explain your son's occasional symptoms, I would discuss ruling out occult constipation with his Spina Bifida team. An abdominal x-ray can assess his stool load.
It was confirmed by an MRI a couple of months ago that I have another tethered cord. What should I do?
From your question I am uncertain if you have previously had a tethered cord release which resulted in the colostomy and decreased mobility or if you have not ever had surgery.
If you already had a tethered cord release, it is very common to have MRI evidence of retethering. MRI findings of tethered cord may or may not be associated with tethered cord symptoms such as you described (bowel/bladder symptoms, pain, decreased mobility). Thus, just because you have recurrent tethered cord findings on your MRI does not mean that surgery will improve your symptoms.
If you have not yet had a release, your symptoms may be improved with surgery by a neurosurgeon with experience in tethered cords.
From a surgical standpoint, the number of previous tether cord releases makes a difference on the outcome of each successive surgery. There seems to be diminishing benefits from each subsequent tethered cord release, particularly for adults. Therefore, it is best to discuss what you hope to achieve from the surgery with the neurosurgeon to determine if these goals are likely to be achieved. From anecdotal reports of neurosurgeons affiliated with the SBA, it seems that pain can be improved but not necessarily completely resolved. Unfortunately, retethering after any surgery is likely and whether the pain returns or further loss of function occurs cannot be predicted.
Please see the May 2009 Ask the Doctor question and response in the archives for more information on tethered cord.
Is tethered cord release surgery going to cure me of tethering cord or will it eventually tether again?
Tethered cord is a difficult topic because almost everyone who had a myelomenigocele surgery develops a tethered cord. The spinal cord can become tethered to an immobile part of the lower spinal area. This happens for many reasons, one of which is the healing and scar tissue that forms following a tethered cord release. So, it is very common for a released cord to become tethered again. The tricky part is determining if a tethered cord (usually found on MRI of the spine) is causing problems or symptoms, such as back pain.
Adults versus children
In children, the most common reason for tethered cords to become symptomatic is during a growth spurt when the cord would normally ascend up the spinal canal in response to the increase in height. Because it is fixed (tethered) in place, it cannot move upward and causes tension. This is a good reason to untether and most children have less pain.
For adults, who have stopped growing, the more common reason for a tethered cord to become symptomatic is trauma. This can be from a fall, a result of heavy lifting, due to a jolt to the back, or during the pushing phase of childbirth. Since these traumas can also cause mechanical back pain not directly involved with a tethered cord, it is helpful if there are additional findings that indicate the spina nerves are affected. These objective findings include:
- a change in gait,
- inward turning of the feet,
- falling, and
- change in bladder function.
Symptomatic tethered cord
A symptomatic tethered cord can be the case if there is a change in urodynamics or a change in findings on manual muscle testing. Because these tests provide more information when they are compared to results obtained from before the symptoms of tethered cord developed, it is important to have regular follow-up with the providers who perform these tests so that recent "baselines" are in your medical records.
Tethered cord release surgery has risks, including permanent neurological changes such as:
- loss of sensation and further lower extremity weakness,
- change in gait, and
- occasionally irreversible loss of the ability to ambulate.
Therefore, most neurosurgeons try conservative treatment first. This includes rest, anti-inflammatory medications, and rehabilitation therapy. If the neurosurgeon believes this is a symptomatic tethered cord, it is also important to evaluate the shunt before considering an untethering procedure, because tethered cord symptoms can result from shunt problems.
Because the backs of adults are thicker than children, the surgery is somewhat more involved in adults. The recovery is similar in that there is a period of immobility where the patient must remain flat, followed by a gradual increase in activity. The exact timing of the recovery is patient and surgeon dependent. The individual undergoing surgery should also be aware that during the healing process the cord will likely tether again, which may or may not result in a return of symptoms.
Have you come across shoulder dimples before and what could it mean?
This is an excellent and common question. Let me answer the easy part first. The dimples in the shoulder areas are not related to Spina Bifida. They are innocent and do not indicate any problem.
Now for the issue of the dimple at the base of the spine, also known as a sacral dimple or the fancier term, "coccygeal pit" — these are also almost always innocent and not related to Spina Bifida providing ALL of the following are true:
- The dimple or pit is located within the crease between the two buttocks (also called the natal cleft or gluteal cleft).
- The dimple is within 1 inch of the anus and is not bigger than ¼ inch in size.
- The base of the dimple can be seen.
- There is no hair, skin color change, blood vessels (telangiectasias), or blood vessel tumors (hemangiomas) around the spine.
- There is no fatty mass felt under the skin.
- The buttocks crease (gluteal cleft) is straight.
- There are no abnormal neurologic findings on exam (i.e. weak legs, abnormal reflexes)
If ALL of these statements are true, then the baby can be followed. Quite rarely, the dimple itself can grow a hair or become infected, which would need further evaluation. Otherwise, nothing needs to be done, and problems are so infrequent that a preventive surgery is not advised.
Now if ANY of the above statements are not true or there are dimples higher up on the back (above the buttocks crease), further testing is advised.
The first step is to get an ultrasound of the area of the back where the dimple, hair tuft, skin color change, blood vessels, or fatty mass is located. If this is abnormal, then an MRI is usually requested to get a more detailed look. Since the ultrasound can be done without needing anesthesia, it is the first choice.
One last comment about skin color changes — a high percentage of non-white babies have a blue-gray discoloration in the buttocks area called Mongolian spots. Ninety percent of Native Americans, 80% of Asians, and 70% of Hispanics have Mongolian spots. Less than 10% of whites have Mongolian spots. By themselves, these color changes are also not related to Spina Bifida. They are innocent and generally resolve as the baby gets older. No additional testing is necessary as long as there are no other findings.
Genetics of SB
Is there a technique used at birth to lessen Spina Bifida?
Spina bifida is not caused by anything that occurs during the birthing process. Spina Bifida is a fetal development problem that occurs very early in pregnancy – within the first month. There are several risk factors for Spina Bifida (which are reviewed in the June 2007 "Ask the Doctor" in the archives), but the exact cause or causes are not known. However, it is safe to say that the fetus will have already developed Spina Bifida before the delivery process.
If your developing baby has Spina Bifida, there are things that the delivery team can do to lessen complications during the birthing process. The optimal delivery team is a high-risk pregnancy specialist with experience managing a spina bifida pregnancy. Babies with open Spina Bifida (Spina Bifida aperta), where part of the spinal cord is outside the skin, are usually delivered by C-section to decrease the trauma to the exposed nerve tissue. Once the baby is delivered, she is wrapped in a protective covering and often started on antibiotics to decrease the chance for infection. The baby then goes to surgery to get the exposed nerve tissue covered by a skin layer. These babies are best cared for in intensive care nurseries in hospitals with on-staff neurosurgeons, urologists, and orthopedic doctors who have experience treating newborns with Spina Bifida. If you know you are carrying a baby with Spina Bifida, discuss with your obstetrician your options for meeting with these specialists before your baby is born.
To find the hospitals that have these teams, try contacting your local Spina Bifida Association or your nearest children's hospital. Best wishes on your pregnancy.
What would be the risk of passing Spina Bifida and Hydrocephalus to my child through artificial insemination?
It is unlikely that the donor sperm was the cause of the offspring with spina bifida and hydrocephalus, particularly since the donor has no personal or family history of spina bifida. There are many possibly reasons for that pregnancy to have resulted in a neural tube defect, including the known risks of maternal obesity, diabetes, and certain anti-seizure medication. Whether any of these risks were part of that preganacy is not mentioned and probably not known from the profile.
It is good that you are taking folate supplements in preparation for your planned pregnancy. Because you have a medical history of spina bifida occulta, it is best that you take a higher dose of folate, 4mg instead of 0.4mg, beginning at least 1 month before starting your insemination program and through the first trimester of your pregnancy.
For more information on folic acid click here.
What are the factors that can increase my chances of having a pregnancy with Spina Bifida?
Spina Bifida can happen in any pregnancy. Spina Bifida happens in about 7 of every 10,000 pregnancies, with over 95% of the pregnancies affected by Spina Bifida happen in families with no 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 pregnancy
Factors known to increase the chance of having a pregnancy affected by Spina Bifida include the following:
- A previous pregnancy affected by Spina Bifida
- A 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
Factors which may increase the likelihood of having a pregnancy affected by Spina Bifida include:
- 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)
Recommendations to reduce the risk of a Spina Bifida pregnancy
While there is no guaranteed method to prevent Spina Bifida, recommendations which may decrease the chances of having a pregnancy affected by Spina Bifida include:
- Taking folic acid 400 micrograms (0.4mg) daily for all sexually active women of childbearing age. Since more than 50% of pregnancies are not planned, the daily intake of a multivitamin with folic acid will benefit all pregnancies.
- For those who have already had a pregnancy affected by Spina Bifida, taking 4mg of folic acid daily for at least 3 months before attempting pregnancy and for the first 3-4 months of the pregnancy.
- For those with relatives affected by Spina Bifida, take at least 400 micrograms of folic acid daily and discuss taking 4mg folic acid for the 3 months prior to pregnancy and the first 3-4 months of pregnancy.
- Women with diabetes should maximize the control of their diabetes and strive for good glucose control.
- For those women on anti-seizure medications (particularly valproic acid or carbamazepine), discuss with your doctor which medication is best for both seizure control and for your planned pregnancy.
- Maintain a healthy diet with a balance of all food groups, including meats and other sources of vitamin B12.
- Work with your physician on maintaining a healthy weight.
- Avoid unnecessary exposure to heat sources such as hot tubs, saunas, and tanning beds.
Are there specific precautions to having a VP shunt such as avoid rolling or hanging upside down or putting pressure on the location of the shunt?
For a specific answer to this question, it would be important to know more information about your hydrocephalus, type of shunt, and your neurosurgeon's preferences. Thus, the best answer to this question should occur between a patient and his/her neurosurgeon.
In general terms, over 90% of neurosurgeons who were recently surveyed about sports participation and ventricular shunts felt non-contact physical activity was safe. A review of the literature found that less than 1% of all shunt problems were attributed to sport-related incidents. The Medical Advisory Board and the Board of Directors of the Hydrocephalus Association have issued a consensus statement encouraging participation in "regular activities including school and after school physical education programs and recreational sporting activities." This would include usual amounts of tumbling, rolling, and hanging upside down. This would also include non-sustained pressure over the shunt and tubing.
There is a difference of opinion about participation in contact sports, with equal thirds of neurosurgeons recommending:
- no participation in any contact sports,
- participation in some contact sports,
- full participation in any contact sport
This is another specific question for your own neurosurgeon.
Can you please demystify the rumor as to whether or not those with SB and shunts have heightened hearing sensitivity, meaning they are more sensitive to loud noises than those who do not have Spina Bifida?
You are not alone in feeling that you have noise sensitivity. This is a problem that others with hydrocephalus and shunts also complain about. We do not know exactly how common this is. We do know that it does not affect everyone, but it certainly bothers some of those with shunts.
We also do not have studies of treatments for this problem of hyperacusis (oversensitivity to certain frequencies of noise) associated with shunts and hydrocephalus. There are treatments for hyperacusis in the general population. There are audiologists and ENT physicians who work in this area. They use a desensitization technique also used in tinnitus (a persistent ringing in the ear) called Tinnitus Retraining Therapy. In this therapy, patients listen to a broadband noise at soft levels for a defined period of time each day in order to increase their tolerances to sound.
Aside from Tinnitus Retraining Therapy, you can use noise reduction head phones, ear muffs, and ear plugs to decrease the intensity of environmental noise, particularly when you will be in situations where loud noise is common.
As for your concern about deafness, you should have a hearing evaluation performed by an audiologist. Deafness has many causes and is not typical of Spina Bifida, hydrocephalus, or shunts. Therefore, you should have this formally assessed.
I am an adult with Spina Bifida that would like to have eye surgery to correct my near-sightedness. Is it safe for me? I have a VP shunt.
Corrective eye surgery is usually an outpatient procedure performed without general anesthesia. Since there is no general anesthesia and the surgery is limited to the cornea of the eye, this surgery is safe for someone with a ventriculoperitoneal (VP) shunt.
It is important for everyone to visit an eye doctor on a regular basis. The Health Guide for Adults Living with Spina Bifida includes a section on making doctor's appointments that is helpful for adults concerned about their general health. This publication is available at SBA's Marketplace
Can I go scuba diving with a ventricular shunt?
Your question raises many issues. While you asked specifically about diving with a ventricular shunt, there are additional considerations for someone with Spina Bifida.
The quick answer to your question is that the limited evidence suggests that someone with a ventricular shunt that does not drain into the blood stream can dive safely up to four atmospheres of pressure. This means that those who do not have a shunt that goes to the atrium (a ventriculoatrial or VA shunt) is most likely safe to dive down to 100 feet. This is based on research using decompression tanks (hyperbaric chambers) which showed that the shunts worked normally up to this pressure. The specialists believe that the shunt will drain normally because the brain/ventricles and the abdomen will be equally affected by the increased environmental pressure of the deeper water.
Now for more of the special considerations:
- Some diving agencies allow for shunts only as long as the rest of the neurologic exam is normal. Others recognize the lifelong changes of conditions such as Spina Bifida and allow divers to be certified.
- Shunts that are old or are no longer believed to be working may not equally respond to the pressure changes and are not known to be safe for diving.
- Shunts draining a syrinx (spinal cord cyst) have not been tested. The spinal cord is not as capable of releasing nitrogen as the brain and may be more susceptible to decompression effects on the spinal blood vessels.
- Shunts to the atrium are affected by the blood stream which may get nitrogen bubbles when surfacing during a dive. These may adversely affect the shunt and are not allowed by most diving certification agencies.
- Individuals with seizure disorders are not allowed to dive. Some certifying agencies allow for divers who have been seizure free without medication for five years.
In addition to the shunt, potential divers with Spina Bifida also need to consider:
- Thermal regulation issues – Diving in cold water or at deeper levels of warm water will promote vasoconstriction of the bodies blood vessels, meaning blood will be diverted from the skin to the body's core organs. This can adversely affect the limbs that do not have the sensation to recognize the cold temperatures. Thus, warm water diving and using wet suits are advised.
- Musculoskeletal strength, mass, and sensation – Low muscle mass in the paralyzed limbs can affect buoyancy and may require weighted belts to help keep the diver submerged. Low bone density in the affected limbs may result in fractures if diving off the side of boats without accessible platforms. Limbs are subject to scrapes, cuts, and burns from underwater coral or plants. Wetsuits can protect the skin from these noxious agents. Another consideration is wetsuits that fit improperly as a result of decreased limb bulk may not adequately protect against the cold water.
- Effects of bowel and bladder conduits and reconstruction – Bowel or bladder conduits, including Mitrofanoff and cecostomy continence ostomies, will allow water to back-flow into the bowel or bladder at depths as little as 5 feet below the surface. Therefore, the bowel or bladder can fill quickly, a particular concern for an augmented bladder which may rupture. Therefore, those with conduits are not advised to dive but may safely snorkel at the surface.
- Latex precautions – Some diving equipment may contain latex, including the snorkel you breathe through. Check with the equipment companies first.
- Fitness issues – Most SCUBA divers propel themselves with their arms and legs, with most of the force generated by finned lower extremities. Propelling by arms alone generally requires more energy and oxygen use, which can affect how fast the diver uses up the air in the SCUBA tanks and how quickly the diver becomes fatigued.
As always, individuals living with Spina Bifida are encouraged to participate in any and all activities that interest them. Because diving has unique safety issues due to the need for a specialized breathing apparatus and the special considerations of the increased atmospheric pressure from swimming underwater, one needs to be aware of all of the issues to remain safe for themselves and their diving partners.
There is a certification process available from the Handicapped SCUBA Association. For more information, please visit www.hsascuba.com/. The Health Guide for Adults Living with Spina Bifida is a good overview of the medical issues surrounding Spina Bifida.
References used include:
Cheng JF, Diamond M. SCUBA diving for individuals with disabilities. Am J Phys Med Rehabil. 2005;84:369-375.
Can Spina Bifida be related to the cognitive and behavior modification?
Because this young man has been diagnosed with Spina Bifida at the age of 16, I would guess that he has Spina bifida Occulta. This means that there are no external protrusions of spinal cord tissue above the surface of the skin. This diagnosis means that the vertebrae did not fuse and that there is an opening in the spine. Usually the spinal cord is not affected. Occasionally (and infrequently) there is involvement of the spinal cord, such as a tethered cord or split cord. This can be determined by MRI. However, none of these findings are usually associated with impairments in cognitive function or behavior problems.
If there is hydrocephalus, most often associated with Spina Bifida aperta (open Spina Bifida) and visible spinal cord tissue above the skin layer, then learning and behavior is sometimes affected. Hydrocephalus can be associated with impairments in cognitive function, learning problems, and behavior challenges. Information about Spina Bifida Occulta, as well as the learning challenges and mental health issues that occur in Spina Bifida with hydrocephalus, is available in the Fact Sheets section.
I am an adult with Spina Bifida. What can I do to manage my back pain?
For an adult living with Spina Bifida, back pain can result from many different causes. Important factors of the back pain include its location; when during the day it starts; whether it is present only when you are standing up, sitting, lying down, or all of the above; whether the pain travels up or down your back, into your neck or into your legs; whether you have a shunt, a Chairi II, or spinal rods; whether you ambulate, with or without crutches, or use a wheelchair for most of your mobility.
Upper Back Pain
Back pain is an issue with lots of considerations! It is probably best to break it down by region:
Upper back pain. Pain in the:
- neck area,
- shoulders, or
- upper back
may be a sign of a shunt or Chiari II problem (a possibly something called a syrinx). If there is associated upper area weakness or abnormal sensations (numbness, tingling), these should be evaluated fairly quickly with the person managing your shunt. Other problems in the upper back can come from spina rods that are broken or starting to poke through the skin. If you have rods, it would be good to have your back looked at and X-rayed.
Mechanical back pain in upper back
Pain in the upper back not caused by rods, shunts, Chairi problems, or a syrinx, can be mechanical. Mechanical back pain can result from:
- excess weight, or
- chronic pulling on the spine.
It may be helpful to evaluate your gait and orthotics if you are ambulatory, or evaluate your seating and wheelchair if you use this for mobility.
For women, another consideration is how much their chest is causing tension on their back. Some women have undergone breast reduction surgery to relieve this tension.
Lower Back Pain
Lower back. When the lower back is involved, the first thing most people ask is whether they have a symptomatic tethered cord. While almost all individuals with a repaired myelomenigocele have a tethered cord, most do not cause problems. A symptomatic tethered cord usually causes neurologic changes in addition to pain. The neurologic changes include a change in:
- more tripping and falling,
- bladder changes, and/or
- pain shooting down into the legs.
Helpful testing in determining if a tethered cord is problematic includes formal urodynamics and manual muscle testing. Not all institutions routinely perform these tests; however, it is most helpful to have baseline tests from which to compare. Thus, for those who have access to urodynamics and/or manual muscle testing, it is best to undergo periodic screening when you well so that there are baselines to compare when you do have problems.
Mechanical back pain in lower back
More commonly, adults with Spina Bifida develop lower back pain as a result of:
- abnormal mechanics involved in ambulating with weak lower extremity muscles
- using older, poorly fitting orthotics.
Those using wheelchairs for mobility may be sitting poorly, and using suboptimal cushions or chair designs. An evaluation by a clinician with expertise in rehabilitation, orthotics, and physical therapy can be helpful. Therapy that strengthens and stabilizes the truncal and gluteal muscles can improve gait mechanics and decrease strain on the lower back. The use of additional gait stabilizers, such as newer or different braces, canes, crutches, or walkers can redistribute balance and pressure off of overused joints. Weight loss can also decrease the strain on the lower back. For those in wheelchairs, changing the height of the back of the chair, cushion, foot rests, and/or arm rests may alleviate pressure on the lower back. It may be time for an evaluation with a wheelchair vendor that can assess these issues rather than order a newer version of the same chair you have always ordered.
I am a parent of a child with Spina Bifida that would like to use holistic modalities that complement modern medicine. How safe is chiropractic care and medical massage?
After performing a literature search, we found that chiropractic manipulation and medical massage has been used not uncommonly by families who have children with Spina Bifida. One study found that 38% of the 37 children with Spina Bifida surveyed had tried this. There was nothing in the study about the success or complications of these therapies.
Further searching did not reveal any papers about complications arising from medical massage or chiropractic manipulation. We could not find a study comparing these therapies to traditional care or using standard randomized or blinded study designs. Thus, we cannot comment with conviction on the benefits of these therapies.
We were able to find case reports by individual practitioners reporting benefits with pain and bladder function. As always, individual cases without comparisons need to be considered with caution.
As for general cautions about spinal manipulation and massage, we would advise you to make sure that the treating clinician is aware of the following issues associated with Spina Bifida:
- If there is a shunt, make sure to be careful with the shunt tubing. Tubing can become calcified and brittle after several years and may be prone to breaking if the head is quickly turned.
The Chiari II malformation means that the cerebellum is down into the upper part of the cervical spine and may also be sensitive to rapid head twists.
- The lower spine is not completely fused and therefore not as strong as an intact spine.
The area under the myelomeningocele repair site may be pressure sensitive and contain a pseudomeningocele (fluid filled sac) which should not be pressed on with significant force.
The bones in the lower extremities may be thinned (osteopenia).
- The hips may be displaced (particularly if the Spina Bifida is high lumber or thoracic level).
Finally, please let your other Spina Bifida providers know about these therapies so they can monitor for any complications.
Can you please provide me with some information regarding poor bladder control and sex?
Concern for your girlfriend is commendable. I hope you will use this answer and this website to help her feel more comfortable in your relationship. Good communication, understanding each others' feelings and concerns, and respecting each others' beliefs and choices are the most important part of a relationship. If a couple is mutually ready to explore their sexuality together, his or her Spina Bifida can be accommodated.
Almost all women with myelomeningocele have changes in the sensory and motor nerves involving the bladder and pelvic area. Many need assistance with bladder drainage and some with bowel emptying. Most of the time emptying the bladder with catheterization and emptying the bowel with and enema or suppository will allow for "accident-free" intercourse. Knowing that a little leakage of urine is harmless to both partners, you can show your girlfriend that a small accident is no big deal. Bowel leakage can be quickly cleaned away with a moist towel so it does not get into the vagina. If bowel contents accidentally get into the vagina, this can be cleaned out using a douche; however, douching is not routinely needed before or after sex. After sexual intercourse, the woman should again empty her bladder to decrease the risk of a urinary tract infection.
For those women who do not catheterize, they should also urinate before and after sex. If they still have significant urinary leakage during sex or frequent urinary tract infections, they should talk to a urologist about the possibility of urinary retention. Significant urinary retention is not good for the kidneys and a clean intermittent catheterization program may be warranted.
In Spina Bifida, the vagina and sex organs can also be involved. For women with Spina Bifida, sexual arousal may not always induce vaginal lubrication. Thus, it is important to have a lubricant available for the man and the woman to use. Some women with Spina Bifida can experience orgasms, more commonly if they have lower myelomeningocele levels (lumbar level or lower).
Like for any sexually active couple, safer sex is important for women with Spina Bifida. Barrier protection is necessary to prevent sexually transmitted diseases; however, latex barriers should be avoided. Non-latex condoms and other barrier contraceptives are available.
Spina Bifida may also affect the bones, joints, and muscles which may affect how the woman can move her hips and legs. Sometimes tightness of the muscles or contractures of the joints can affect the woman's comfort in certain positions. Patience, understanding, and communication can help the couple find the most comfortable position for both partners.
It is important to remember that sexually active women with Spina Bifida can become pregnant. Because 50% of all pregnancies are unplanned, any woman with Spina Bifida who is having sex should take 4mg of folate daily. This is 10 times the recommended dose for women without Spina Bifida and should not be taken in the form of 10 prenatal vitamins, which would provide too much of the other vitamins. Instead, folate-only vitamins are recommended.
Finally, while I answered questions about sex and Spina Bifida, the bigger picture of sexuality was not addressed. Women with Spina Bifida can have happy, healthy sexual relationships, with or without sexual intercourse. More information on both sexuality and sexual function is available in the Health Guide for Adults Living with Spina Bifida available from the Spina Bifida Association Marketplace Publications webpage.
I want to develop a sexual relationship with my partner but I am afraid of losing control of my bowel and bladder. What do I do?
The most important thing you can do is talk to your partner about your concerns. If your relationship is intimate enough to consider becoming lovers, you are also intimate enough to make sure you are both secure and comfortable with being sexual together.
Prepare for sex by emptying both 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. There is no harm if some urine or stool is temporarily on either your or your partner's skin. Any stool that gets into the vagina should be cleansed away with a douche; however, douching is not routinely nor needed nor advised after all sex.
Most important for both of you is to discuss how you will handle the possibility of this leakage occurring before you start. Communication is key to any relationship and talking about this will allow for the physical intimacy to come naturally.
Can a person with Spina Bifida father a child?
The quick answer to this question is yes. The longer answer is - how a man with Spina Bifida fathers a child depends on many issues.
In order to conceive a child a man needs to be able to form and maintain a penile erection firm enough to penetrate his partner's vagina. He must also be able ejaculate enough healthy semen to fertilize his partner's egg.
Men can form erections either through psychogenic (mental arousal) or reflexive (physical stimulation) mechanisms. The lower the level of the myelomenigocele lesion, the more likely a man can form and maintain an erection. In the few studies that have been published on this subject, about 70% of the men with Spina Bifida reported having erections, most as a result of direct stimulation. Whether these erections could be sustained for sexual intercourse was not reported. For those who have difficulty, there are medications, devices, and internal prosthetics which can help. Most adult urologists can assist the man in choosing the most appropriate method.
The other requirement for fertilizing a partner's egg is the ejaculation of sperm. Some men with Spina Bifida can have erections and orgasms, but the sperm does not take the normal course through the urethra out of the penis. In these men, the sperm travels backwards into the bladder (called retrograde ejaculation). While there is no known harm to the man when this happens, the likelihood of impregnating his partner is low. In these circumstances, a fertility expert can help the couple using special techniques to obtain viable sperm from the man.
Finally, men with Spina Bifida can become loving, supportive fathers to children through a host of other ways. For example, couples can consider surrogate sperm donation or adoption as other ways to start a family together. In the end, we all know "Any man can be a father. It takes someone special to be a dad."
I have problems maintaining my erection because of lack of sensation in my genitals. Is that common for men with Spina Bifida (SB)? If so, what can I do to remedy that problem?
Males with SB below L2 or with Spina Bifida Occulta may have normal sexual function but could have some difficulties. Partial erections may occur, making it difficult to have penetrative sex or to ejaculate at the right time during sex. Men with lesions above L2 commonly have difficulty with attaining or maintaining an erection and experiencing ejaculation (called erectile dysfunction or ED). Satisfactory erections are often possible, but there is no ejaculation in most people who have myelomeningocele.
(There is an excellent and very informative presentation on SB University (www.sbuniversity.org) that addresses these issues titled Sexual Function and Health in Men with Spina Bifida.)
Can ED be treated?
Although ED is common in men with SB (as it is with men in general), most still have normal sexual desire, can achieve erections, and have a satisfying sex life. The good news is that erectile dysfunction in men with Spina Bifida is a treatable condition.
Because the desire for sex is strong in most people, and many men have ED, it is normal to seek out medical advice for ED at some point in life. Almost all men with myelomeningocele will need medical help in order to have penetrative intercourse. Some medical options for ED include:
- Oral medications like Viagra, Cialis, Levitra, or others which regulate blood flow to the penis, but still require some form of sexual stimulation to work effectively.*
- Penile device like vacuum constriction pump (external)
- Penile prosthesis (internal)
- Injection therapy
- Transdermal patches
More than 90 percent of people with myelomeningocele have neurogenic bladder and bowels. Management of continence is very important because continence (or incontinence) affects the likelihood of getting urinary tract infections (UTIs) which could affect sexual function, and of having a partner who is interested in having sexual contact with you. Bladder and bowel continence in SB is inseparable from sexual function in that nerve damage that affects urinary and bowel function may also affect sexual function and a full bladder may interfere with sexual activity. Thus, continence is inseparable from intercourse, and a urologist should be a key member of the care team.
Do you know what type of technology is available that condenses all of my child's medical history so we do not have to pay for films and carry huge files?
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. Most individuals with Spina Bifida see multiple providers, have numerous radiographic procedures and surgeries, and use equipment for bracing and mobility. Thus, it is important for families to keep track of significant information whether they move frequently, vacation out of town, or simply try to communicate and coordinate services among multiple medical providers.
The type and amount of information to keep and carry with you can vary from the basics to vary detailed, comprehensive records. The format used can be paper, electronic hard storage (i.e. CD-ROM or Thumbdrive) or internet-based.
When traveling from one city to the next, either permanently or for vacation, the most basic information to take is:
The names, telephone numbers, and addresses of:
- All medical providers
- Indicate their specialty (i.e. neurosurgery, urology, orthopedics, OT/PT)
- All hospitals where surgeries occurred
- New physicians can contact the medical record departments for reports
- All locations (hospital and private facilities) where radiographic studies were performed
- Orthotic, mobility, and DME (catheters, diapers) providers
The names, telephone numbers, and addresses of:
A list of medication, latex, and other allergies and the reactions
While this basic list can facilitate transferring records to another provider, there are many inefficiencies that often result in incomplete information for the new medical team. Maintaining a personal health record can help during this transition of care.
Personal health records are now available in paper, digital, and virtual/internet formats. For those who prefer paper format, two popular choices are:
Health Guide for Parents of Children Living with Spina Bifida
Available from the SBA Marketplace, this guide has a sample personal health record which guides the user to document the important information. In addition, there is useful information for parents and for providers who may be less familiar with Spina Bifida.
The National Center for Medical Home Initiatives for Children with Special Health Care Needs has a section on their website for creating Care Notebooks. These notebooks are not Spina Bifida specific, but can be tailored to the family's needs. The website for these Care Notebooks is:
For those who wish to use electronic format, the paper forms above can be scanned onto a CD-ROM or thumbdrive which decreases the bulk of the files. It is important to clearly label each scanned file for ease of retrieval. The file format should be one that is universally used, such as .pdf.
There are software programs, either free or for purchase, that create personal health records. Some have the ability to upload images. Most allow for multiple records so there is access for the whole family. None is specifically designed for Spina Bifida, so it may be useful to use the sample health record from the Health Guide for Parents of Children Living with Spina Bifida as a guide of what information to save.
Many institutions now have CD-ROM discs available for radiographic studies. The actual images in addition to the reports can be scanned onto the disc. This is ideal for the receiving surgical specialists to be able to visually compare future studies to the previous ones.
I strongly recommend all of my families to have a scanned disc of the last stable brain scan (CT or MRI) with them whenever they travel in case of the need to "rule out shunt malfunction."
For the truly mobile, there are virtual personal health records that can be stored on the internet. Different companies offer additional services, such as medical alert bracelets which contain the identification number, a useful feature if there is an emergency when the primary caregiver is not present.
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 " or click on this link:
Finding Clinics & Doctors
Can I go to my University's health clinic for care or should I find an off-campus physician?
Your question raises several issues:
- Where should you go for health care as an adult with Spina Bifida?
What information should you have with you when you are away from your home medical team?
Most University Health Services and Clinics offer basic preventive and illness care equal to a primary care practice. They are often staffed by adult trained nurse practitioners, family medicine or general internal medicine physicians, or pediatricians with expertise in adolescent health. The providers are well-trained for common acute and chronic medical conditions seen in college students (such as strep throat, sinus infections, asthma, diabetes, thyroid problems, etc.). They should seek assistance from specialists familiar with the unique needs of an individual with Spina Bifida, just as your family physician back home would do.
Therefore, it is fine to go to the University Health Clinic for the same illnesses you would see your primary care doctor. For care related to your shunt, neurogenic bladder, neurogenic bowel, skin breakdown, or mobility equipment you should see the related sub-specialist you see back in Indiana. One way to find those specialists is to contact either the local Spina Bifida clinic or SBA chapter near your school. They can recommend the correct sub-specialist. You can find the contact information for local Spina Bifida clinics and SBA chapters on the SBA website at: www.spinabifidaassociation.org
If you use the University's student health insurance as your primary coverage while you are at school, you may need to get referrals from the Student Health Clinic before seeing outside specialists. Ask about this when registering for school.
Also remember that one area where University Student Health Clinics are very helpful is assessing and treating stress related conditions. College is stressful for many students regardless of their other medical conditions, and Student Health Clinics have processes in place to help. If you occasionally feel overwhelmed at school, you are not alone.
Because you are living away from your usual Spina Bifida medical providers, you should have your important information with you. The minimal information you should always have with you is:
The names, telephone numbers, and addresses of:
- All medical providers
- Indicate their specialty (i.e. neurosurgery, urology, orthopedics, OT/PT)
- All hospitals where surgeries occurred
- New physicians can contact the medical record departments for reports
- All locations (hospital and private facilities) where radiographic studies were performed
- Orthotic, mobility, and DME (catheters, diapers) providers
A list of medication, latex, and other allergies and the reactions
- A list of medication names, strengths, and dosing recommendations and who prescribes each of them
- A list of medication, latex, and other allergies and the reactions
- Your insurance information and a copy of your insurance card(s)
- A digital or film copy of your most recent brain scan (CT or MRI) if you have a shunt
One way to have all the relevant information with you is to obtain a copy of the SBA publication Health Guide for Adults Living with Spina Bifida . There is a personal health record section which is specifically tailored for someone with Spina Bifida. In addition, the guide is a good overview of the medical issues surrounding Spina Bifida, which can be helpful to you and any medical provider less familiar with Spina Bifida care.
I am 18 years old and need to find a doctor. What do I do?
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:
What type of doctor or doctors do you need?
- 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 and are likely to have some experience with issues that can occur in Spina Bifida.
- Adult primary care doctors are not expected to replace your specialists, like your urologist or neurosurgeon. It is ideal to have ongoing care with both of these specialists as an adult. You can get a recommendation from your pediatric urologist and neurosurgeon. You will want a urologist who treats neurogenic bladders and a neurosurgeon who treats ventricular shunts (if you have a shunt).
- As an adult, you are less likely to need frequent care by an orthopedic surgeon. Since you have stopped growing, you are less likely 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, wh eelchair, and other equipment assessments. This doctor 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.
What doctors are on your insurance?
- Not all doctors are on your insurance plan. Thus, make sure the doctors recommended by your pediatric team are listed in your insurance company's provider manual. Take the manual with you to your pediatric doctor and ask who they know and who they would recommend from the list.
- At age 18, many young adults remain on their parents' insurance plan for a period of time. This is an opportunity to get in the door at the adult doctor's office. If you are planning to apply for Supplemental Security Insurance (SSI) and take advantage of getting Medicaid insurance in the future, ask the new doctors if they keep their existing patients when their private insurance changes to Medicaid (some do).
Is the new doctor's office accessible to you (including being latex-free if you need to avoid latex)?
- Call ahead of time (before your first office visit) and ask about accessible parking, elevators, stairs, and the distances you will need to travel to:
- get from your care to the office,
- get blood drawn
- get radiographic tests done
- How is latex handled in the doctor's office?
Get old records sent to your doctor ahead of time.
- Ask your pediatric doctors to send at least their last office visit note, last operative report, and, if possible, a summary letter to the new doctor.
- Ask the radiology department if they can create a CD-ROM of your more recent tests (many can do this now). Bring this to your new doctors office.
- If the radiology department does not have this capability, ask them how you can get your most recent radiology reports. You will want at least:
- CT of the head and shunt series (if you have a shunt)
- Urologic studies (if you have a neurogenic bladder)
- MRI of the brain and or spine (if you have a Chairi malformation or had a tethered cord release)
- Xrays of any spinal rods, prosthetic joints, internal braces
- Make a list of all of your medications (and pharmacies), allergies (including latex), and other doctors and have a copy ready to give to your new doctor(s) for them to keep. If the doctor needs to order equipment or supplies for you, put the contact information for the company on the list.
- Go to the new doctor when you are feeling well. Spina Bifida is complicated and the new doctor may need to spend most of the time reviewing your history. S/he may ask you to come back for a second visit to finish the initial evaluation. S/he may also ask for new baseline tests. Please understand this helps her/him understand your medical issues better and will improve the care provided to you.
More information on health care for adults is available in the Health Guide for Adults Living with Spina Bifida available from the Spina Bifida Association Marketplace Publications webpage.
My girlfriend is now pregnant and is using M.A.C.E to help her go to the bathroom. She uses glycerin and salt and water. Can this affect the baby?
Congratulations on your girlfriend's pregnancy. First, I hope she is on 4 mg of folic acid every day to help decrease the risk of myelomeningocele in your child. For more information on folic acid supplementation, please see the June 2007 Ask the Doctor question in the archives.
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.
What is my likelihood of being able to get pregnant? As a woman with Spina Bifida?
Congratulations on your decision to become a parent! I am happy to report that many women living with Spina Bifida are able to become pregnant, maintain a safe pregnancy, and deliver a healthy baby.
Folic Acid every day
If you have had regular menstrual periods then you are most likely able to become pregnant. Not all women are aware of this fact and some assume that because of their Spina Bifida and/or hydrocephalus, they are not capable of becoming pregnant. There is no evidence that women with Spina Bifida have lesser fertility than women without Spina Bifida.
For this reason, it is very important that all sexually active women who are of child-bearing age take a multivitamin with folic acid every day. The recommended dose of 400mcg (0.4mg) is in most daily multivitamins. If you are planning to become pregnant and you have Spina Bifida, the Spina Bifida Association recommends you take 10 times the regular recommended folic acid dose. This equals 4mg of folic acid daily.
You can get folic acid over the counter at most pharmacies and health food stores. If you take 4mg daily you should not get this dose from taking extra multivitamins or extra prenatal vitamins. You would get too much of the other vitamins. Therefore, you should buy folic acid-only vitamins and take as many pills as needed to get 4mg. Start taking the 4mg dose at least 3 months before attempting to become pregnant and at least 3-4 months into the pregnancy.
Ability to become pregnant and maintain pregnancy
Although the ability to become pregnant is not significantly changed by having Spina Bifida, the ability to maintain the pregnancy to term can be impacted by the level of lesion, amount of previous abdominal and pelvic surgery, and structure of the genital tracts.
Because some women with Spina Bifida have structural changes in their uterus (womb), it is helpful to have an examination by a obstetrician/gynecologist (possibly with ultrasound) to make sure the uterus can support a pregnancy. During this evaluation the:
- pelvic size,
- lower body flexibility, and
- shunt tubing
can also be evaluated. Because pregnancy in Spina Bifida is not routine, seeking care form an obstetrician with expertise in high-risk pregnancies is advisable. Notify this obstetrician's staff about any latex allergies and negotiate a latex-free delivery environment.
Before pregnancy begins, it is important to review chronic medications, particularly those for bladder management, hypertension, and seizures. There are medications considered safer in pregnancy which can be used. It is also important of the high risk obstetrician to know about previous surgeries, especially abdominal and pelvic procedures and complications.
During pregnancy, women with Spina Bifida can have unique medical concerns. Many women with Spina Bifida develop frequent urinary tract infections, and may be encouraged to monitor their urine at home using urine dipsticks to look for infection cells and bacteria.
As the baby develops, it can press down on the bladder, out on the spine and spinal cord, up on the lungs, and out on the ureters, urinary diversion tracts, and bowel and cecostomy tracts. There have been reports of:
- worsening scoliosis (curvature of the spine),
- decreased bladder function,
- urine trapping and reflux into the kidneys,
- difficulty catherizing Mitrofanoffs and other continent diversion tracts, and
- prolapse (outpouching) of tracts though stomas.
Pressure on the peritoneal shunt tubing can affect drainage of hydrocephalus. The increased weight of the baby can cause pressure sores, balance and mobility issues. All of these issues can be managed with the assistance of your high-risk obstetrician and adult Spina Bifida specialists.
Despite these many considerations, many women with Spina Bifida have healthy pregnancies and safe deliveries resulting in beautiful children. More information is available in the Spina Bifida Association's Health Guide for Adults Living with Spina Bifida.
As a mother-to-be with Spina Bifida what type of delivery should I expect – natural or C-section?
Continue taking 4mg of folic acid daily through the first 3-4 months of your pregnancy to maximize the health of your baby.
Type of Delivery
The type of delivery depends on multiple physical factors, including:
- sensation level,
- ability to push with pelvic muscles,
- size of the pelvis, and the
- flexibility around the hips and knees.
The more conducive these factors are to the birthing process, the more likely a vaginal delivery can be performed. If it appears the baby will not be able to be pushed safely through the pelvic area and down the birth canal, a Cesarean section will be recommended. If an elective Cesarean section is chosen, the urinary tracts should be evaluated (particularly if there has been re-implantations, diversions, or conduits) to make sure they are avoided during the delivery incision.
Women with Spina Bifida have successfully and safely received epidural anesthesia for their delivery. Because the anatomy of the spine is different in Spina Bifida, the epidural may need to be placed using ultrasound guidance. There are reports of successful epidurals for most myelomeningocele lesion levels, with or without scoliosis. As with all pregnancy issues in spina bifida, monitoring the effects of the epidural is trickier than in usual pregnancies. Therefore, it is best to deliver with an obstetrician and hospital experienced in high-risk pregnancies.
Effects after pregnancy
Long-term effects after pregnancy in Spina Bifida have been reported and include:
- changes in mobility,
- bladder function, and
- lower body sensation.
The exact risk numbers are not known, but reports are usually isolated cases rather than common experiences. The birthing process, with deep pushing and drawing back of the legs, has occasionally caused tethered cord symptoms. Most resolve with rest and anti-inflammatory medication, but rarely women have eventually undergone a tethered cord release as a result of the birthing process.
Plan before you try to get pregnant
For women with Spina Bifida who are not yet pregnant, discussing your medical and surgical history with your Spina Bifida team and high-risk obstetrician before conceiving can maximize the preparation for a healthy delivery.
When do I let my child with Spina Bifida catheterize?
For those who use it, catheterization is an important part of bladder and kidney care. Transitioning care to your son or daughter is also important in the path to maximal autonomy. However, there is no standard timeline that every family must follow.
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, making sure to remain clean or sterile (depending on which technique you use).
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. The urology nurse can also provide guidance.
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 which 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, which allows for easier visualization of the catherization channel.
More information is available on this Web site in the Fact Sheet section and in:
Children With Spina Bifida: A Parent's Guide (The Special Needs Collection) Edited by Marlene Lutkenhoff
Health Guide for Parents of Children Living with Spina Bifida Edited by the SBA Editorial Review Board
Both books are available in the Marketplace section of this website.
My daughter has had symptoms of possible shunt malfunction: vomiting, visual difficulties, muscle numbness and weakness, and back pain. After having MRI and other tests, it seems that the shunt is working properly. In the absence of other evidence, the doctor thinks it may be migraines. Can you provide any insights to help me understand this issue better, including treatment for migraines?
There are a couple of types of migraines that can mimic shunt malfunction by causing the same symptoms:
- Basilar-type migraine – typically will have vertigo, double vision and vomiting
- Sporadic hemiplegic migraine where one can get transient symptoms of hemiparesis and hemisensory changes.
In both of these types 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 sumatrptan 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.
Do people with Spina Bifida have a similar sex drive to their peers with
normal nerve function below the waist? If so, do they have difficulty
finding a partner?
The answer to your first question is "Yes." Most women (and men)
with SB think about sex and would like to have a sexual partner. Some
have sex partners, but others don't achieve that point in their life.
This is often due to:
- Difficulties managing urinary and bowel incontinence
- Delay in receiving sexual education
- Lack of social interaction/opportunity
- Low self-esteem
- Lower overall neurologic function, which may be due to hydrocephalus
- Lack of independence from parents or caregivers
- Difficulty with positioning due to problems with bones, joints and muscles that affect movement of hips and legs, muscle tightness/contractures.
Regardless of whether you have a sexual encounter in your life though, it is possible for anyone to have a loving, caring, intimate relationship with another person.
For more on this topic, please watch for the SBA's new information sheets on men's and women's health.
Are people with Spina Bifida at higher risk for diabetes?
Diabetes is a serious disease that affects the cells, muscles and fatty tissue of the body and can eventually affect the organs (kidneys, eyes, heart). There are two types of diabetes.
Type 1 diabetes, also known as "juvenile diabetes" or "insulin dependent diabete," is less common (occurs in only 5% of all diabetes) and occurs when the body does not produce insulin (a hormone that regulates movement of glucose into the cells) to convert sugar and starch into energy. Type 1 diabetes is diagnosed early in life, and there are no known risk factors or preventive measures. More commonly, discussion about risk factors refers to type 2 diabetes, which accounts for 95% of all diabetes.
Type 2 diabetes, also called "adult onset" or "non-insulin dependent diabetes," is a chronic condition that affects how the body uses insulin to metabolize glucose (sugar), which is the body's main source of fuel.
People who have type 2 diabetes either do not produce enough insulin or the body resists the effects of the insulin. This often comes on slowly, and may initially appear as "pre-diabetes"- a condition where blood sugars are elevated but not high enough on several measures to be diagnosed as diabetic. Pre-diabetes is a warning sign that there is a probable risk for diabetes to develop.
Although experts still aren't sure what causes type 2 diabetes, there are clearly identified lifestyle risk factors as well as action steps that can delay or prevent the development of the disease. People with SB 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 SB are obese, putting them at very high risk for diabetes in their life.
Inactivity/immobility- Increased exercise helps to maintain a health weight. Furthermore, exercises burns glucose for energy, increasing the cells' ability to use the insulin. There are many options for exercise for people with SB 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 group 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 II diabetes is increasing among children and adolescents. This increase is attributed to obesity, poor nutrition and lack of exercise.
Eat nutritious meals and maintain a healthy weight (see SBA's info sheet on obesity )
Have regular health check ups
Are people with Spina Bifida at higher risk for diabetes?
According to the National Sleep Foundation (www.sleepfoundation.org), 15% of young and middle aged adults, and more than 50% of older adults experience chronic pain (see SBA's info sheet on pain) which prevents them from sleeping well at night. This probably comes as no surprise to people with SB because the types of pain that cause the most sleep disturbance include: headaches and back pain (remember to check the shunt first), jaw and ear pain, and musculoskeletal pain (arthritis and fibromyalgia). Chronic pain, coughing and difficulty breathing (whether you are aware of it or not) can awaken a person from sleep from one to several times each night, and may be difficult to get back to sleep. To worsen the problem, medications are often required to treat pain, and they may cause disturbed sleep also. The Sleep Foundation reports that this is a vicious cycle which disrupts all aspects of a person's life. When you have difficulty sleeping or feel fatigued during the time, it is time to discuss both the pain and the sleep problems with your doctor, who will probably refer you to a sleep specialist. Yes, there are doctors who focus only on sleep disorders, and there are many disorders, so they need to be diagnosed and treated by a specialist.
Along with medical advice, there are things you can do to help improve your sleep, such as: don't stay up too late or go to bed too early (7-8 hours of sleep is recommended for adults), don't eat food late at night, eat balanced, healthy meals and avoid gassy or spicy foods. Avoid caffeine at night and don't drink too much alcohol. Similarly, if you are able to do vigorous exercise or activity (exercise is important for good sleep), don't do it late in the evening. Rather incorporate your exercise routine into your earlier activities of the day, and keep your evenings free for relaxing activities that help you get ready for sleep. This means that you should not keep your television or radio on during the night either. Sleep in a comfortable, clean, quiet place so you can rest.
Health Care / Health Insurance
How can you get health insurance and help paying for medications if you don't qualify for health care coverage any other way?
The Social Security Administration has set some guidelines for different disabilities. It is important to view their website and discuss your options first with an SSA representative.
Making sense of health care coverage can be confusing. These sites are very informative in making it all easier to understand and navigate:
- Partnership for Prescription Assistance
- 1-888-4PPA NOW or 1-888-477-2669
- Together Rx Access 1 800 250 2839
- Hill Burton Facilities
- 1-800-638-0742 or www.hrsa.gov/hillburton
Insurance For Adults or Families
Insurance for Children:
- www.insurekidsnow.gov or 1-877-543-7669
- State Children's Health Insurance Program (SCHIP)
- 1-877-543-7669-search by state
* 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.
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