Spina Bifida and Pain

People with SB are at risk for chronic pain Pain can be felt in many ways. Sometimes (like if you smash your thumb), you know exactly where the pain started. That happens to everyone. Other times, it just hurts everywhere. As people with SB age, they often have pain associated with degenerative conditions that affect the spine, joints, or muscles. In SB, commonly seen conditions are: arthritis, osteoporosis, carpal tunnel syndrome, and tethered cord syndrome. Back pain is common in adults, and can be very debilitating. 

There are many causes of pain, and the each type determines how long it will last.

Pain is generally classified in two categories: acute or chronic. People with SB can have either type of pain, or even both at the same time.

What is Acute pain?
Acute pain is the kind that comes on very quickly, may be moderate or severe, and the cause or location of the pain can usually be identified. Acute pain can be controlled when the cause of the pain is identified. It can be treated, and it goes away within hours, days, or weeks.

What is chronic pain?
Chronic pain is a long term condition; and it lasts for weeks, months, or years. It comes on slowly over a period of time, the cause(s) may be difficult to pinpoint; and it can be very difficult to manage. Chronic pain affects every aspect of a person’s life; and the longer it persists, the more difficult it is to get under control. Therefore, it should not be ignored. Chronic pain is very serious; and should be treated as a disease in itself.

Can I prevent chronic pain?
Sometimes chronic pain can be prevented if the condition that causes pain is diagnosed and treated early. Acute pain occurs suddenly. It happens in relation to a specific tissue injury, and is often treatable. Acute pain can be severe, but doesn't last long. People who have this type of pain usually know why they hurt. That is very different from chronic pain, which is often ignored or not managed until it becomes a big problem.

How is it diagnosed?
Persistent pain-the kind that lasts a long time-for weeks or months, should be assessed by your doctor through both a physical assessment and a detailed discussion . Chronic pain affects all aspects of a person’s life. This is because the brain and the body are inseparable, so both are affected by pain, as well as the conditions that cause it. People who suffer from chronic pain often have fatigue and find it difficult to move around. Sometimes they are forgetful, have difficulty thinking clearly because they are distracted; or become pre-occupied by their discomfort, and find it challenging to focus on tasks at work or school. Many people with chronic pain find it difficult to exercise, eat properly, or sleep- that increases their risk for other health problems.

Chronic pain causes other conditions that make it difficult to cope- like anxiety and depression. Similarly, high stress or feelings of hopelessness increase levels of pain. So, it becomes a cycle that is difficult to break. This is why it is very important to treat the pain at 2 levels, the physical and the emotional level- and a team approach is the best way to do that.

Who should be on that team?
That depends on each person, The primary care doctor or physiatrist will help you determine which specialists you need. In general, the doctor who deals with the condition that is causing your pain, a psychologist, a pain medicine doctor, and a physical therapist or occupational therapist are important people to include in your care. If your pain affects your ability to work, then you might need a vocational specialist also.

How do you treat chronic pain?

Long term pain medications-If you choose to treat your pain with medications, you may need to take more than one medication. They can be in the form of pills, injections, creams, gels, or patches. Medications can be helpful in treating the pain, but have unwanted side effects, like nausea, sleepiness, and dry mouth or constipation. Some people need their dosage of medication increased or changed because they build up a tolerance to the drug, so they require higher doses to get the same relief as they once did on a smaller dose.

Pain management programs-are normally at “out-patient” hospital or clinic settings. Pain management programs are designed to help people who suffer from chronic pain. Pain management programs offer a variety of supports that treat the body and the emotions, like: physical therapy, occupational therapy,water therapy, physician care, psychology/counselling, relaxation, and bio-feedback. If a pain management program is not right for you, your doctor might suggest only one or two of these therapies.

Psychotherapy, often in the form of Cognitive Behavioral Therapy (CBT) is often helpful when treatments have not been successful. It is also helpful to receive CBT while undergoing other treatments. Counseling and CBT can help identify ways of coping with pain and its associated problems.

Intrathecal analgesia, or delivery of medication through a “pain pump” is often used for long term chronic pain, which cannot be managed through more conservative means. With intrathecal analgesia, pain medications and sometimes muscle relaxers can be given through a pump that is surgically implanted in the abdomen. The pump delivers medication directly into the spinal cord through a catheter. For some people, this method of pain management is successful for neck or back pain; and is advantageous because much smaller doses of medication can be used more effectively. However, it is not without risks, and your pain management doctor would be helpful in deciding if it is the right approach to manage your pain, when other treatments have not been successful.

Alternative Therapies- Alternative therapies can provide temporary relief from pain, and can be used with other pain management plans:

• Massage Therapy
• Water (hydro) Therapy
• Acupuncture
• Meditation or Feldenkreis
• Music Therapy
• Pet Therapy

Surgery is sometimes an option if the source of the pain is known.
Support groups can be sources of information, understanding and encouragement.

***Information obtained from http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8277201/k.B2D5/Pain.htm

Spina Bifida and Expectant Parents.

An Expectant Parent's Guide to Spina Bifida:
If you are reading this, you have likely just been told that your pregnancy is affected by Spina Bifida (SB). Like most women receiving this unexpected diagnosis, you want to know what it is, and what to expect.

What is Spina Bifida?
Spina Bifida describes a group of neural tube defects (NTD's) that occur when the baby's developing spine (neural tube) fails to close properly. When most people speak of SB, they are referring to the most common and also most severe form, called myelomeningocele, which causes the spinal nerves to bulge through an opening in the back. Myelomeningocele usually occurs in the lowest part of the spine but can occur at any level. Spina Bifida is the most common permanently disabling birth defect that is compatible with living into adulthood.

What causes SB?
There is no single known cause for SB. It is due to a complex interaction of both genetic and environmental factors occurring very early in the pregnancy (by the 4th week). The genetic factors that can cause SB are not well known. Research has shown that supplementation of folic acid ( a "B" vitamin) reduces the risk of having a pregnancy affected by SB. However, many people who take folic acid still have babies with SB, and women who did not take folic acid still have healthy babies. No one knows why. It just happens. This means that there is still much to learn about the causes of SB, and it is nobody's fault.

How is SB diagnosed during pregnancy?

• Maternal Serum Alpha: Fetoprotein (MSAFP): also known as triple test or quad test, is a blood test performed between the 15th and 20th weeks of pregnancy to determine if there is a higher risk for NTD. MSAFP testing measures the level of a protein (AFP) made by the fetus and placenta. Small amounts of this protein normally cross the placenta into the maternal bloodstream, but when very high levels cross, it could be that the fetus has an NTD. However, MSAFP testing is not used as a diagnostic tool but rather as an indicator that further testing is needed, because the test is not specific for SB, and because high MSAFP may be present for a number of other reasons.
• Ultrasound: is the primary diagnostic tool for prenatal diagnosis of SB and other NTD's. It is a non-invasive technique that produces an image of the fetus. It can be used to identify many different problems in fetuses, but can also be used to identify important characteristic signs of SB in both the brain and spine. Cranial markers include the lemon sign (abnormal shape of fetal skull), the banana sign (crowding of the cerebellum in the back part of the head), and hydrocephalus (abnormally large ventricles or fluid spaces in the brain). The normal fetal spine resembles a string of pearls. An SB spine will appear as a string of pearls with some broken or missing pieces.
• Fetal MRI: May be recommended after the diagnosis is made through ultrasound to determine severity of SB. MRI is a powerful technology that uses magnetic imaging (not radiation) to give better pictures of the fetal spinal cord and spinal nerves. Through MRI, the doctors can see the size and extent of the SB. This information helps them explain the diagnosis and prognosis (expected long term condition) in greater detail. Fetal MRI is not necessary for all pregnancies affected by SB though, particularly when the ultrasound images are good.

What medical issues do people with SB encounter?
People with SB have a number of medical issues to deal with throughout their lives.

• Open SB lesions need to be surgically closed (usually within 72 hours after birth) to prevent infection and further damage to the spinal cord.
• Hydrocephalus is an excessive amount of cerebral spinal fluid (CSF) collecting in the ventricles of the brain. It occurs in approximately 80% of patients with SB. Sometimes it occurs before birth, but most often it occurs after the back has been closed. Hydrocephalus is usually treated by surgery to place a hollow tube called a ventriculoperitoneal (VP) shunt that transfers the fluid from the head to an empty space in the abdomen in order to prevent brain damage. (Please see the SBA's info sheet on hydrocephalus and shunts for more information).
• Neurogenic(dysfunctional) bowel and bladder is caused by damage to the nerves that control the function of the bladder and the bottom section of bowel and anus. In recent years, a number of treatments have been developed which allow individuals to achieve varying levels of functional continence. (Please see the SBA's information sheet on urologic care and management for more information).
• Many people with SB face mobility issues,which are dependent on the spinal level of the SB lesion. Most children are able to walk (though usually with the aid of orthopedic bracing and often with assistive devices such as crutches). Some are full-time walkers, but many walk short distances and choose a wheelchair for long distances. It is generally only those with thoracic or high lumbar spinal lesions who end up using the wheelchair as their only method of mobility.
• Although most individuals with SB have normal IQ scores, they may have learning difficulties; but these can often be addressed when parents and teachers understand the issues and work together.

Every individual with SB is affected differently, and it is impossible to fully predict a child's outcome before oratbirth.Outcomeshaveimprovedoverthepast50 years due to medical advancements. In addition, cultural attitudes toward individuals with disabilities have also changed, resulting in improved services. While some individuals with SB have significant disabilities, others are less severely affected. Many attain advanced education, and have careers and families of their own. They become doctors, teachers, artists, athletes, and parents. Spina Bifida is only one part of their lives; it does not define them.

What are the choices for women carrying fetuses with SB?
To learn the most about the prognosis for YOUR pregnancy, it is recommended that you meet with a pediatric neurosurgeon and/or SB clinic as soon as possible. You will also need to meet with a maternal- fetal specialist or perinatologist to closely follow your pregnancy. You may also meet other specialists who care for people with SB.Use these appointments to learn as much as you can. After you have gathered enough information to understand the immediate and long term implications of SB, consider the choices available to you—terminating the pregnancy, prenatal or postnatal surgery, or adoption—what matters is that you make an informed choice about what works best for you and your family. Prenatal or Postnatal surgery: For pregnancies diagnosed earlier than 25 weeks gestation, in-utero fetal surgery may be an option to close the baby's back before birth. There are potential risks and benefits of fetal surgery, and it is not appropriate for all women. For those who choose and qualify, an immediate referral must be made to a medical center where the operation is performed. Because fetal surgery is not standard practice at this time, most newborns with SB require surgery shortly after birth to prevent infection and further spinal cord damage.

Post-natal care:
Babies with SB should be delivered at a medical center that specializes in SB so they can receive specialty care during and after birth. This gives you and the specialists every chance to prepare for the best outcome. After surgery, the baby will be monitored in the neonatal intensive care unit. The average length of stay is 2 weeks, but this varies based on the child's needs. When the baby is discharged from the hospital, he or she will have periodic follow-up appointments with a pediatric neurosurgeon, orthopedist, urologist, and possibly other specialists. Appointments will be frequent in the first year, and usually less often as time passes.

Additional information
To get more information and lifelong support, please contact Spina Bifida Association at 800 621 3141, ext 35; or via the website: www.sbaa.org. Some of the following specific resources may be helpful through the website, www.sbaa.org: SBA Marketplace, SB University,Preparations,healthinformationsheets, E-communities-parents' listserv.

Spina Bifida & Agent Orange

Agent Orange or Herbicide Orange (HO) is one of the herbicides and defoliants used by the U.S. military as part of its herbicidal warfare program, Operation Ranch Hand,during the Vietnam War from 1961 to 1971. Vietnam estimates 400,000 people were killed or maimed, and 500,000 children born with birth defects as a result of the use of contaminated batches of the compound. The Red Cross of Vietnam estimates that up to 1 million people are disabled or have health problems due to Agent Orange. The United States government has challenged these figures as being unreliable and unrealistically high.

A 50:50 mixture of 2,4,5-T and 2,4-D, it was manufactured for the U.S. Department of Defense primarily by Monsanto Corporation and Dow Chemical. The 2,4,5-T used to produce Agent Orange was contaminated with 2,3,7,8-tetrachlorodibenzodioxin (TCDD), an extremely toxic dioxin compound. It was given its name from the color of the orange-striped 55 US gallon barrels in which it was shipped, and was by far the most widely used of the so-called "Rainbow Herbicides".

During the Vietnam War, between 1962 and 1971, the United States military sprayed nearly 20,000,000 US gallons (76,000,000 l) of material containing chemical herbicides and defoliants mixed with jet fuel in Vietnam, eastern Laos and parts of Cambodia, as part of Operation Ranch Hand. The program's goal was to defoliate forested and rural land, depriving guerrillas of cover; another goal was to induce forced draft urbanization, destroying the ability of peasants to support themselves in the countryside, and forcing them to flee to the U.S. dominated cities, thus depriving the guerrillas of their rural support and food supply.

The US began to target food crops in October 1962, primarily using Agent Blue. In 1965, 42 percent of all herbicide spraying was dedicated to food crops. Rural-to-urban migration rates dramatically increased in South Vietnam, as peasants escaped the war and famine in the countryside by fleeing to the U.S.-dominated cities. The urban population in South Vietnam nearly tripled: from 2.8 million people in 1958, to 8 million by 1971. The rapid flow of people led to a fast-paced and uncontrolled urbanization; an estimated 1.5 million people were living in Saigon slums.

United States Air Force records show that at least 6,542 spraying missions took place over the course of Operation Ranch Hand. By 1971, 12 percent of the total area of South Vietnam had been sprayed with defoliating chemicals, at an average concentration of 13 times the recommended USDA application rate for domestic use. In South Vietnam alone, an estimated 25 million acres, 39,000 square miles of agricultural land was ultimately destroyed. In some areas, TCDD concentrations in soil and water were hundreds of times greater than the levels considered safe by the U.S. Environmental Protection Agency. Overall, more than 20% of South Vietnam's forests were sprayed at least once over a nine-year period. 

Health Effects in Vietnam

The Vietnam Red Cross reported as many as 3 million Vietnamese people have been affected by Agent Orange, including at least 150,000 children born with birth defects. According to Vietnamese Ministry of Foreign Affairs, 4.8 million Vietnamese people were exposed to Agent Orange, resulting in 400,000 people being killed or maimed, and 500,000 children born with birth defects. Women had higher rates of miscarriage and stillbirths, as did livestock such as cattle, water buffalo, and pigs.

Children in the areas where Agent Orange was used have been affected and have multiple health problems, including cleft palate, mental disabilities, hernias, and extra fingers and toes. In the 1970s, high levels of dioxin were found in the breast milk of South Vietnamese women, and in the blood of U.S. soldiers who had served in Vietnam. The most affected zones are the mountainous area along Truong Son (Long Mountains) and the border between Vietnam and Cambodia. The affected residents are living in substandard conditions with many genetic diseases.

About 28 of the former US military bases in Vietnam where the herbicides were stored and loaded onto airplanes may still have high level of dioxins in the soil, posing a health threat to the surrounding communities. Extensive testing for dioxin contamination has been conducted at the former US airbases in Da Nang, Phu Cat and Bien Hoa. Some of the soil and sediment on the bases have extremely high levels of dioxin requiring remediation. The Da Nang Airbase has dioxin contamination up to 350 times higher than international recommendations for action. The contaminated soil and sediment continue to affect the citizens of Vietnam, poisoning their food chain and causing illnesses, serious skin diseases and a variety of cancers in the lungs, larynx, and prostate.

Effects on U.S. veterans

Some studies showed that veterans who served in the South during the war have increased rates of cancer, and nerve, digestive, skin and respiratory disorders. Veterans from the south had higher rates of throat cancer, acute/chronic leukemia, Hodgkin's lymphoma and non-Hodgkin's lymphoma, prostate cancer, lung cancer, colon cancer, soft tissue sarcoma and liver cancer. With the exception of liver cancer, these are the same conditions the US Veterans Administration has determined may be associated with exposure to Agent Orange/dioxin, and are on the list of conditions eligible for compensation and treatment.

Military personnel who loaded airplanes and helicopters used in Ranch Hand probably sustained some of the heaviest exposures. Members of the Army Chemical Corps, who stored and mixed herbicides and defoliated the perimeters of military bases, and mechanics who worked on the helicopters and planes, are also thought to have had some of the heaviest exposures. However, this same group of individuals has not shown remarkably higher incidences of the associated diseases. Others with potentially heavy exposures included members of U.S. Army Special Forces units who defoliated remote campsites, and members of U.S. Navy river units who cleared base perimeters. Military members who served on Okinawa also claim to have been exposed to the chemical but there is no verifiable evidence to corroborate these claims.

While in Vietnam, the veterans were told not to worry, and were persuaded the chemical was harmless. After returning home, Vietnam veterans began to suspect their ill health or the instances of their wives having miscarriages or children born with birth defects might be related to Agent Orange and the other toxic herbicides to which they were exposed in Vietnam. Veterans began to file claims in 1977 to the Department of Veterans Affairs for disability payments for health care for conditions they believed were associated with exposure to Agent Orange, or more specifically, dioxin, but their claims were denied unless they could prove the condition began when they were in the service or within one year of their discharge.

By April 1993, the Department of Veterans Affairs had only compensated 486 victims, although it had received disability claims from 39,419 soldiers who had been exposed to Agent Orange while serving in Vietnam.

In 1991, the US Congress enacted the Agent Orange Act, giving the Department of Veterans Affairs the authority to declare certain conditions 'presumptive' to exposure to Agent Orange/dioxin, making these veterans who served in Vietnam eligible to receive treatment and compensation for these conditions. The same law required the National Academy of Sciences to periodically review the science on dioxin and herbicides used in Vietnam to inform the Secretary of Veterans Affairs about the strength of the scientific evidence showing association between exposure to Agent Orange/dioxin and certain conditions.

Through this process, the list of 'presumptive' conditions has grown since 1991, and currently the U.S. Department of Veterans Affairs has listed prostate cancer, respiratory cancers, multiple myeloma, type II diabetes, Hodgkin's disease, non-Hodgkin's lymphoma, soft tissue sarcoma, chloracne, porphyria cutanea tarda, peripheral neuropathy, chronic lymphocytic leukemia, and spina bifida in children of veterans exposed to Agent Orange as conditions associated with exposure to the herbicide. This list now includes B cell leukemias, such as hairy cell leukemia, Parkinson's disease and ischemic heart disease, these last three having been added on August 31, 2010. Several highly placed individuals in government are voicing concerns about whether some of the diseases on the list should, in fact, actually have been included.

What is the Agent Orange Benefits Act?

The Agent Orange Benefits Act, Public Law 104-204, became law in 1996. The law established a benefits package for Vietnam veterans' children who were born with Spina Bifida, possibly as a result of exposure of one or both parents to herbicides during active service in the Republic of Vietnam during the Vietnam era.

The Act authorized the Department of Veterans Affairs to provide certain benefits effective October 1, 1997. These benefits include lifetime health care services for Spina Bifida and “any disability associated” with Spina Bifida, a monthly monetary allowance, and Veterans Affairs (VA) vocational training/rehabilitation services.

The VA defines Spina Bifida as all forms of Spina Bifida (except Spina Bifida Occulta). In establishing rules to determine the monthly monetary allowance, the VA indicated that “neurological deficit is the main determinant of disability for a person with Spina Bifida.” An estimated 10-20% of the population may have Spina Bifida Occulta; however, Occulta is not considered to have a neurological deficit according to the VA. The Women Vietnam Veterans' Children's Birth Defect Benefits Act is an act modeled after the Agent Orange Benefits Act; however, it only provides benefits to children of women Vietnam veterans whose children suffer from certain types of birth defects, with the exception of Spina Bifida.

Are any VA benefits available for children of Vietnam veterans born with Spina Bifida?

Children of Korean or Vietnam veterans born with Spina Bifida are eligible for a monthly disability allowance and for vocational training. A monthly allowance is set at three levels, depending upon the degree of disability suffered by the child. VA's Vocational Rehabilitation and Employment program administers a vocational training program to enable a qualified child to prepare for and attain suitable employment. Services may include counseling and rehabilitative services, education, training and employment services. VA pays for the costs of the services up to 24 months depending on need. For more information on Spina Bifida, please visit the VA's web site athttp://www.va.gov/hac/forbeneficiaries/spina/spina.asp or contact your local VA regional office at 1-800-827-1000.

What is the first step in establishing eligibility for VA Spina Bifida healthcare benefits?

Since VA Spina Bifida healthcare benefits are based upon eligibility determinations made by VA regional offices, prospective beneficiaries must first contact a regional office to obtain an Application for Spina Bifida Benefits (VA Form 21-0304). The local VA regional office can be contacted toll-free at 1-800-827-1000. Upon return of a completed application, the regional office will determine eligibility and notify the applicant of the determination.

Upon establishing eligibility through a VA regional office, what is the next step in obtaining VA Spina Bifida healthcare benefits?

Beneficiaries in receipt of a VA regional office Spina Bifida award should immediately contact VA's Health Administration Center (HAC) in Denver, CO, to register for health care benefits. The following information is required: name, address, and telephone number of both the beneficiary and veteran sponsor (and legal guardian when applicable); Social Security number of both the beneficiary and veteran sponsor; and, VA claim number of the veteran sponsor. Registration with HAC can be accomplished by telephone, fax, or mail

The telephone number for Spina Bifida Healthcare Benefits Information/Registration is 1-888-820-1756. The fax number is 1-303-331-7807. The address is VA Health Administration Center, Spina Bifida Healthcare Benefits, P O Box 65025, Denver, CO, 80206-9025

What other assistance is available?

The American Legion Service Officers (DSO) offer free advice and guidance for veterans who need to deal with the Department of Veterans Affairs (VA). This directory provides contact telephone numbers and addresses for the service officers in your area. Direct all correspondence to Department Service Officer. For the directory, please visit www.legion.org or call (202) 861-2700.

The National Veterans Legal Services Program specializes in legal issues and referrals for veterans. For more information, please visit www.nvlsp.org or call (202) 265-8305.

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

Additional resources

http://www.publichealth.va.gov/exposures/agentorange/birth_defects.asp

American Legion
www.legion.org
Help with Claims and Benefits, Health Issues Line
202- 861-2700

National Veterans Legal Services Program
PO Box 65762
Washington, DC 20035
202-265-8305
Fax 202-328-0063
Email: info@nvlsp.org
Web: www.nvlsp.org

VA Health Administration Center
Spina Bifida Healthcare Benefits Information/Registration
P O Box 65025
Denver, CO, 80206-9025
1-888-820-1756
Email: spina.inq@med.va.gov
Web: www.va.gov/hac

Spina Bifida and Urinary Tract Infections

Urinary Tract Infections (UTI’s) are quite common in the Spina Bifida population. I, myself, have had numerous UTI's in my lifetime. There are 
two types of UTI’s: the bladder (cystitis) or the kidneys (pyelonephritis). These can present 
with no symptoms (asymptomatic) or with systems (symptomatic). I have always been taught by my doctors to never take antibiotics for an infection unless I was symptomatic. This would cut down on the chance of becoming immune to the prescriptions. However, you should speak to your own medical professional about what is best for your or your child. 

Signs of infection to look for are: 

• urine looks or smells different e.g. a 'fishy' smell 
• wet in between catheterization when usually dry 
• needing to go more often 
• burning or stinging sensation 
• blood in urine 
• temperature, headaches, vomiting 

When someone has cystitis, they generally need to pass urine more often than usual and if 
sensation is present they may have to hurry to the toilet (urgency). The urine looks cloudy 
instead of clear and may smell different from usual. Drink extra fluids and take your child to 
your GP or Paediatrician who may prescribe a course of antibiotics. Ensure that laboratory 
testing is carried out to confirm the presence of a UTI. 

A much more serious situation arises in pyelonephritis in which the infection affects the 
kidneys causing a high temperature, tummy ache, backache, and sickness and may 
cause scaring to the kidneys. 

Why UTIs are Common in Children with Spina Bifida? 

Children with Spina Bifida often lack feeling and control of their bladder and bowel in the 
same way as they may lack feeling and control of their feet and legs. This is because the 
nerves in the spine connecting the brain to the bladder (or bowel or legs) have been 
interrupted, or disconnected, by the Spina Bifida. The result is that they cannot usually feel 
when their bladder is full, nor can they empty it properly. The bladder may still contain urine 
(the residual urine) after they have tried to empty it. The stagnant pool or residual urine left 
in the bladder gets smelly and easily becomes infected and over a period of time stones 
may develop (urinary calculi) as well. Bladder Infections may affect the kidneys causing 
pyelonephritis and kidney damage, making the child ill. This happens because the bladder 
does not empty out the infected urine. It may also happen with ureteric reflux when the 
infected urine travels up towards the kidneys. 

Many children with Spina Bifida do not know when they have a urinary infection. Because 
they lack feeling they do not suffer from the scalding pain which other children feel when they get cystitis, nor do they know their bladder is not emptying properly. UTI’s should be 
confirmed with laboratory testing before treatment is administered. Treating children with 
antibiotics and extra fluids works more slowly if the bladder is not emptying. The best way to 
prevent urinary infections damaging the kidneys is to be sure that the bladder empties 
regularly and completely. One way of doing this is by clean intermittent catheterisation 
and by being vigilant with a hygiene program and following strict hygiene guidelines. (see 
“How to prevent UTI’s) 

How CIC Lessons the Chances of Urinary Infections 

Clean Intermittent Catheterization (CIC) means inserting a narrow tube (catheter) into the 
urinary passage (urethra) into the bladder. There are different length catheters available 
suitable for either boys or girls. The urine flows out of the bladder through the catheter into 
the lavatory, or into a container, until the bladder is completely empty and then the 
catheter is removed after complete emptying. This should be done 4 – 5 times a day. 
Regular drainage of the bladder will improve social continence and help to reduce UTIs. 
Catheterization should take place at least four times a day but for those who have a 
smaller bladder this may need to be increased to six times. With practice catheterization 
becomes quite easy and may take less than five minutes. Twenty-five years of experience 
of intermittent catheterization has shown that it reduces infections and prevents damage 
to the kidneys as well as improving those already damaged, if CIC and hygiene regimes 
are consistent. 

How to Prevent UTI’s 

• Maintain adequate fluid intake by encourage your child to drink at least 4 cups a day 
and carry a drink bottle with them at all times. (The aim is to increase this fluid intake to 2 
litres) 
• Ensure regular complete bladder emptying 
• Relieve constipation 
• Ensure the use of appropriate catheter size and type 
• Maintain a strict hygiene routine 

Bowels 

People with Spina Bifida are often constipated and pressure from an overfull bowel may 
add to the difficulty of emptying the bladder. They may also have poor control over their 
bowel in the same way as they have over the bladder. Since most urinary infections are 
caused by bacteria which normally live in the bowel, it is important to wash this part of the 
body carefully. Girls should be taught always to wipe from front to back and not from back 
to front. 

Medicines 

UTI’s should be confirmed with laboratory testing before treatment is administered. 
Antibiotics and other medicines are sometimes given in small doses for months or years to 
prevent urinary infections. They are mainly used for children who have reflux or for anyone 
with damaged kidneys to protect them from further harm. In spite of this, urinary infections 
may still break through and need a course of a different treatment. 

Other Remedies 

Many remedies have been used over the years to prevent or relieve urinary infections and 
two of these are worth mentioning: Vitamin C (ascorbic acid) and cranberry juice. Vitamin 
C acts by making the urine acidic and this discourages some of the bacteria which cause 
urinary infections. Cranberry juice helps to clear infections and debris after operations on 
the bladder (specifically e. Coli) 

Key points 

• In children with Spina Bifida urinary tract infections are mainly due to the bladder not 
emptying properly. 
• Poor drainage from the bladder encourages infections to spread to the kidneys where 
they may cause damage. 
• Good drainage is as important as fluid intake. 
• Intermittent catheterisation ensures good drainage and protects the kidneys. It may also 
encourage social continence. 

Spina Bifida and Natural Rubber Latex Allergy

Natural Rubber Latex Allergy in Spina Bifida

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

What is Natural Rubber Latex?
Natural rubber latex (NRL) is a milky substance tapped from the Hevea Brasiliensis (a tropical rubber tree). It can be heated and molded into hard rubber products like tires; or it can be dipped to make softer products like balloons or medical examination gloves.

What is latex allergy?
Latex allergy means that a person is allergic to proteins in the natural rubber latex. Although anyone can develop a latex allergy, it is thought to be caused by significant long term exposure to latex proteins that are released during processing of the rubber. The amount of latex exposure needed to produce sensitization or an allergic reaction is unknown, but softer rubber products that have been processed longer (like gloves and balloons) are seen as more allergenic; and frequent exposure to latex products increases the risk of developing a sensitivity.

People who have Spina Bifida and catheterize; or have several surgeries from very early in life, such as bladder surgery or shunt revisions, are at very high risk for allergy because of a “cumulative” effect over time. Symptoms of latex sensitivity can be minor, but without warning, may become life threatening. Many people are unaware that they are sensitized to latex because the symptoms can be vague and non-specific. Those people are at risk for a serious reaction.

What are the symptoms of latex allergy?

• Itching
• Skin redness, hives or rash
• Sneezing
• Runny nose
• Itchy, watery eyes
• Scratchy throat
• Cough
• Wheezing

The most serious allergic reaction to latex is anaphylaxis, a type of shock. An anaphylactic response to latex is a medical emergency.

Signs and symptoms include:

• Difficulty breathing caused by swelling of lips tongue or windpipe
• Severe wheezing
• Severe drop in blood pressure (hypotension)
• Dizziness
• Loss of consciousness
• Confusion
• Slurred speech
• Rapid or weak pulse
• Blue hue of the skin, including lips and nail beds
• Diarrhea
• Nausea and vomiting

Latex items

Because of its low cost, durability and versatility, natural latex has been widely used in the United States for over a century; and is used in the production of many common items. Although most medical products are labeled, household or recreational items which contain latex may not be labeled. For that reason, the Latex Allergy Association and the Spina Bifida Association work diligently to keep a current list of products that contain latex; and their “safe” (non-latex) alternatives.

What are cross reactions to latex allergy?
People allergic to latex may also be allergic to the proteins in some fruits and vegetables. Some of them include: banana, avocado, chestnut, kiwi, apple, carrot, celery, papaya, potato, tomato, melon, and avocado. Due to nutritional risks, people should not avoid eating these foods unless they have had a reaction to them and are advised by a dietary or medical professional to avoid them.

What steps should I take to prevent developing latex allergy?
The best way to prevent developing latex allergy is to avoid contact with latex or latex-contaminated powder. Contact occurs through contact with skin, inhaling latex spores, or internally through medical procedures or surgery, when latex touches the skin, mouth, eyes, genital areas or bladder. Severe reactions can occur if latex enters the bloodstream. Powder from latex balloons or gloves gets into the air. Therefore, people with Spina Bifida are at high risk for latex allergy and should avoid exposure to natural latex products from birth. Products made of silicone, plastic, nitrile or vinyl can be used instead.

Those who have had a serious reaction to latex should:

• Wear a medic-alert bracelet or necklace
• Carry auto-injectable epinephrine; and
• Carry sterile non-latex gloves and other non-latex medical items for emergencies.

Discuss latex allergy and avoidance with health care providers, schools, day care, camps, visitors and anyone else who is involved with the person who has Spina Bifida.

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

Additional resources
www.aaaai.org
www.asbah.org
www.latexallergyresources.org
www.latexallergylinks.org
www.osha.gov/SLTC/latexallergy

This following link is to a list that provides a guide to some of the most common objects containing latex and offers some alternatives. It is not meant to be a comprehensive listing and was updated in the Fall of 2012. It is required by law that manufacturers must label any medical items that contain natural t is required by law that manufacturers must label any medical items that contain natural rubber latex. ALWAYS CHECK THE PRODUCTS PACKAGING. If in doubt regarding the safety of an item, call the manufacturer.

http://www.spinabifidaassociation.org/atf/cf/%7B85f88192-26e1-421e-9e30-4c0ea744a7f0%7D/SBA-LATEXLIST-2012-ENGLISH.PDF

Spina Bifida Stats

Factors that increase risk of Spina Bifida include:

*Previous pregnancy affected by Spina Bifida

*First, second, or third generation relative living with Spina Bifida 

*Taking valproic acid or carbamazepine (anti-seizure medications)

*Having diabetes before the beginning of pregnancy (not gestational diabetes)

*Inadequate folic acid intake

*Pre-pregnancy obesity

*Low vitamin B-12 levels in the body

*Having high temperatures early in pregnancy (which may include fever, or exposure to the heat of hot tubs, saunas, and tanning beds)

*Ireland has the highest rate of SB in the world. Even in America, the highest rate of Spina Bifida occurs amongst those of Celtic descent. Followed by Guatemala. And among ethnic groups, Hispanics have a higher rate than other ethnic groups. Cause is unknown but it may be due in part to an inability of Hispanic women to absorb as much folic acid as non Hispanics. There are genetic and environmental and as yet undiscovered causes for SB, but this rise in incidence rate is likely due to Hispanics inability to absorb folic acid as efficiently as other ethnic groups.

*Worldwide the incidence of Spina Bifida is about 7 per 10,000 live births. Since there is 6,700,000 (6.7 billion) there is approximately 4.7 million living with Spina Bifida in the world. To put that into perpective that is MORE than the population of the Dallas Texas Metroplex.

*The abortion rate for Spina Bifida pregnancies is 64% worldwide!!! This explains why there arent even more births of babies with spina bifida. 

Spina Bifida and Tethered Cord

The following information comes from: http://www.ninds.nih.gov/disorders/tethered_cord/tethered_cord.htm

What is Tethered Spinal Cord Syndrome?

Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.  Attachments may occur congenitally at the base of the spinal cord (conus medullaris) or they may develop near the site of an injury to the spinal cord.  These attachments cause an abnormal stretching of the spinal cord.  The course of the disorder is progressive.  In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence.  This type of tethered spinal cord syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida.  Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and loss of bowel and bladder control emerge.  This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time and may be exacerbated during sports or pregnancy, or may be due to narrowing of the spinal column (stenosis) with age. Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord.  Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia.  This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms.

Is there any treatment?

MRI imaging is often used to evaluate individuals with these symptoms, and can be used to diagnose the location of the tethering, lower than normal position of the conus medullaris, or presence of a tumor or fatty mass (lipoma).  In children, early surgery is recommended to prevent further neurological deterioration. Regular follow-up is important: retethering may occur in some individuals during periods of rapid growth and may be seen between five to nine years of age.  If surgery is not advisable, spinal cord nerve roots may be cut to relieve pain.  In adults, surgery to free (detether) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms.  Other treatment is symptomatic and supportive.

What is the prognosis?

With treatment, individuals with tethered spinal cord syndrome have a normal life expectancy.  However, some neurological and motor impairments may not be fully correctable.  Surgery soon after symptoms emerge appears to improve chances for recovery and can prevent further functional decline.

What research is being done?

The NINDS (National Institute of Neurological Disorders and Stroke) conducts and supports research on disorders of the spinal cord.  The goals of this research are to find ways to prevent, treat, and cure these disorders.

NIH (National Institutes of Health) Patient Recruitment for Tethered Spinal Cord Syndrome Clinical Trials

• At NIH Clinical Center
• Throughout the U.S. and Worldwide
• NINDS Clinical Trials

Organizations

National Organization for Rare Disorders (NORD) 55 Kenosia Avenue Danbury, CT   06810 orphan@rarediseases.org http://www.rarediseases.org  Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291	Spina Bifida Association 4590 MacArthur Blvd. NW Suite 250 Washington, DC   20007-4266 sbaa@sbaa.org http://www.spinabifidaassociation.org  Tel: 202-944-3285 800-621-3141 Fax: 202-944-3295
American Syringomyelia & Chiari Alliance Project (ASAP) P.O. Box 1586 Longview, TX   75606-1586 info@asap.org http://www.asap.org  Tel: 903-236-7079 800-ASAP-282 (272-7282) Fax: 903-757-7456