Gerald R. Jerkins MD
Pediatric Urology Associates
of the MidSouth, P.C.
Memphis Tennessee

Spina Bifida

On this page:
Overview
Common Problems Associated with Spina Bifida
Treatment of Spina Bifida
JAMA Patient Page: Spina Bifida
Spina Bifida - Latex Allergies
Spina Bifida - Neurogenic Bladder
Helful Links

Overview


Spina bifida occurs when the spinal bones fail to close properly during early formation. Spina bifida occurs less often than Down syndrome, or in about one in 1,000 births. It is, however, the most common of the physically disabling congenital abnormalities. A parent who has one child with spina bifida has a greater chance (one out of 100) of having another. This increased frequency appears to be due to some combined effect of heredity and environment. There are now tests available to screen for spina bifida early in pregnancy.

A newborn with spina bifida appears at first glance to be normal, except for a small sac protruding from the spine. However, the sac contains spinal fluid and damaged nerves that lead to the lower body. Within the first few days, surgery must be performed to remove the sac and close the opening in the spine. Unfortunately, little can be done to repair the damaged nerves.

Common Problems Associated with Spina Bifida


Hydrocephalus
Approximately 70 of every 100 children with spina bifida eventually develop hydrocephalus, caused by an excessive increase in the fluid that normally cushions the brain from injury. The increase occurs because the spina bifida abnormality blocks the path through which the fluid ordinarily flows. This condition is serious and, if not treated, may lead to death.

The pediatrician should suspect hydrocephalus if the baby's head is growing more rapidly than expected. The condition is confirmed by a computerized X-ray of the head, called a CT scan or magnetic resonance imagery (MRI). If hydrocephalus is present, surgery will be necessary to relieve the fluid buildup.

Muscle Weakness or Paralysis
Because the nerves leading to the lower part of the body are damaged, the muscles in the legs may be very weak or even paralyzed in children with spina bifida. Their joints also tend to be very stiff, and many babies with this disorder are born with abnormalities of the hips, knees and feet. Surgery can be performed to correct some of these problems, and the muscle weakness can be treated with physical therapy and special equipment, such as braces and walkers. Many children with spina bifida eventually can stand and some walk, although the learning process is often long and extremely frustrating.

Bowel and Bladder Problems
Often, the nerves that control bowel and bladder function are damaged in children with spina bifida. As a result, these children are more likely to develop urinary tract infections and damage to the kidneys due to abnormal urine flow. Special techniques are available to develop urinary control and minimize infections.

Bowel control also is a problem but usually can be accomplished by children with this disorder. It may, however, take a great deal of time, patience, careful dietary management (to keep stool soft), and the occasional use of suppositories or other bowel stimulants.

Infection
Parents of children who have spina bifida and hydrocephalus or urinary tract problems must be ever alert for signs of infection. Fortunately, the types of infections that occur in these cases usually can be treated effectively with antibiotics.

Educational and Social Problems
Seven out of ten children with spina bifida have developmental and learning disabilities requiring some sort of special education. Many also need psychological counseling and tremendous emotional support in order to deal with their medical, educational and social problems.


Treatment of Spina Bifida


Parents of a child with spina bifida need more than one physician to manage their child's medical care. In addition to the basic care your pediatrician delivers, this disorder requires a team approach that involves neurosurgeons, orthopedic surgeons, urologists, rehabilitation experts, physical therapists, psychologists and social workers. Many medical centers run special spina bifida clinics offering the services of all these health professionals in one location. Having all members of the team together makes it easier for everyone to communicate and usually provides better access to information and assistance when parents need it.

Excerpted from Caring for Baby and Young Child: Birth to Age 5, Bantam 1999

© Copyright 2000 American Academy of Pediatrics

JAMA Patient Page: Spina Bifida


Prenatal care is an important step in preventing many birth defects. Besides regular prenatal visits to your doctor, good nutrition, including supplementing your diet with vitamins and minerals, helps reduce the risk of certain birth defects.

In fact, you should start taking folic acid, one of the B vitamins, before you become pregnant. Getting the recommended daily amount of folic acid before you become pregnant and during your pregnancy reduces the risk of spina bifida (see below) and other neural tube defects (birth defects that affect the development of the brain and spinal cord).

An article in the June 20, 2001, issue of The Journal of the American Medical Association reports on the decrease in the number of neural tube defects in the United States that appears to be related to the addition of folic acid to all enriched grain products. The United States Food and Drug Administration mandated that all enriched grain products (breads, pastas, rice, flour and cereals that are enriched with supplemental vitamins) include folic acid supplementation as of January 1998.

What Is Folic Acid?
Folic acid (sometimes referred to as folate) is one of the B vitamins. It is recommended that any woman who could possibly become pregnant supplement her diet daily with 400 micrograms (0.4 milligrams) of folic acid. It is important that you get the recommended daily allowance of folic acid before you become pregnant because by the time pregnancy is confirmed, the baby's brain and spinal cord have already started to develop.

What Are Neural Tube Defects?
Neural tube defects are birth defects that occur in the early stages of fetal development when the cells for the brain and spinal cord form a tubelike structure known as the neural tube. The entire nervous system develops from this structure. Neural tube defects can involve the brain, spinal cord, meninges (covering membranes), skull and spine.

Spina bifida, a common neural tube defect, occurs when vertebrae (the bones of the spine that protect the spinal cord) do not close completely around the part of the spinal cord that they are meant to protect. This can cause the unprotected part of the spinal cord to protrude through the spinal defect. Although the spinal defect can sometimes be repaired through surgery, nerve damage that may have occurred because of this malformation may be permanent.

For More Information
National Center on Birth Defects and Developmental Disabilities
Centers for Disease Control and Prevention
(770) 488-7160
(888) 232-6789 (toll free)
E-mail: flo@cdc.gov
www.cdc.gov/ncbddd/folicacid

March of Dimes
(914) 997-4764 (TTY)
(888) 663-4637 (toll free)
E-mail: resourcecenter@modimes.org
www.modimes.org

Spina Bifida Association of America
(202) 944-3285
(800) 621-3141 (toll free)
E-mail: sbaa@sbaa.org
www.sbaa.org

Sources: National Center on Birth Defects and Developmental Disabilities (Centers for Disease Control and Prevention), National Institute of Neurological Disorders and Stroke, March of Dimes, Spina Bifida Association of America, AMA Complete Guide to Your Children's Health, AMA Home Medical Library

Inform Yourself
To find this and previous JAMA Patient Pages, go to the Patient Page Index on JAMA's Web site at www.jama.com. A JAMA Patient Page on prenatal care was published on May 27, 1998.

Brian Pace, MA, Writer
Cassio Lynm, MA, Illustrator
Richard M. Glass, MD, Editor

The JAMA Patient Page is a public service of JAMA. The information and recommendations appearing on this page are appropriate in most instances, but they are not a substitute for medical diagnosis. For specific information concerning your personal medical condition, JAMA and the AMA suggest that you consult your physician. This page may be reproduced noncommercially by physicians and other health care professionals to share with patients. Any other reproduction is subject to AMA approval. To purchase bulk reprints, call (718) 946-7424.

© Copyright 2001 American Medical Association. All rights reserved.


Spina Bifida - Latex Allergies


The incidence of serious allergic reactions to latex has increased in recent years. In rare cases, these reactions can be fatal. Health care workers and others who are frequently exposed to products containing latex should be aware of the potential for developing an allergic reaction. Individuals who exhibit symptoms of the allergy should be alerted to the need to limit future exposure to latex products.
What Is Natural Rubber Latex?

Natural rubber latex is a processed plant product derived almost exclusively from the tree Hevea brasiliensis found in Africa and Southeast Asia. Natural rubber latex should not be confused with butyl- or petroleum-based synthetic rubbers. Synthetic products, including latex house paints, have not been shown to pose any hazard to latex-sensitive individuals.


What Is Latex Allergy?

An allergy, or immediate hypersensitivity reaction, occurs when the body's immune system is sensitized to a foreign protein and reacts by forming a specific type of antibody, called an IgE antibody, specifically directed against this protein. This kind of antibody is responsible for a wide variety of allergic responses, which may range from hives to allergic rhinitis (hay fever), asthma, or rarely, life-threatening allergic attacks (anaphylaxis). These allergic reactions are provoked by exposure to common allergens including cat dander, ragweed pollen or antibiotics, such as penicillin. Latex allergic individuals make allergic antibodies directed against one or more proteins that are found in natural rubber latex.

What Triggers the Allergic Reaction to Latex?

When individuals allergic to latex come into direct contact with latex an allergic reaction may follow. Receiving medical or dental care from someone wearing a latex glove, blowing up a balloon, or breathing in glove donning powder are all common examples of circumstances where allergic reactions to latex have been triggered.

What Products Contain Natural Rubber Latex?

Latex is a common component of many medical supplies, including disposable gloves, airway and intravenous tubing, syringes, stethoscopes, catheters, dressings, and bandages. Many of these medical devices come into contact with mucous membranes, which enhances the absorption of latex proteins that can trigger an allergic reaction. Latex gloves also frequently are implicated in allergic reactions due to the repeated direct exposure of the wearer's hands to latex proteins or due to airborne latex proteins that are absorbed by powders used to lubricate some latex gloves.

While latex also is found in as many as 40,000 consumer products, including condoms, balloons, athletic shoe soles, tires, underwear leg- and waistbands, rubber toys, nipples, and pacifiers, these seem to only cause problems in the most sensitive patients.

What Are the Symptoms of Latex Allergy?

Allergy to latex proteins can cause symptoms similar to those seen in individuals who are allergic to bee venom or cat dander. Reactions on exposure to the allergen are generally acute and may mimic hay fever or asthma, with symptoms such as nasal congestion, hives or difficulty breathing. The most severe cases can result in anaphylaxis, a potentially fatal reaction that affects many parts of the body at once. Symptoms of anaphylaxis are usually immediate, progress rapidly and may include a dangerous drop in blood pressure, flushed skin, difficulty breathing, swelling of the throat, tongue and nose, and loss of consciousness. Emergency medical attention should be sought at the first sign of an anaphylactic reaction.

Skin problems resulting from the use of latex and non-latex gloves are frequently confused with latex allergy. Contact dermatitis is a frequent problem in glove wearers that can be caused by frequent hand-washing and drying with irritating soaps, skin abrasions from donning and removing gloves, and irritation of skin covered by an impermeable barrier. It can also be caused by contact allergy to one or several of the chemicals used in the production of rubber gloves. These are usually local skin problems but can involve larger areas. These local skin problems are not life-threatening, but may precede the development of latex allergy if latex exposure is continued.

How Do Symptoms Develop?

In most cases, latex allergy develops after repeated exposures to latex. It should be noted, however, that direct physical contact with latex-containing products is not needed to trigger the allergic reaction. Cases of anaphylaxis have resulted from inhaling latex proteins, which can be absorbed by the powder that is used to lubricate some latex gloves. When the gloves are snapped on and off, the proteins become airborne and can pose a risk to some individuals with latex hypersensitivity.

How Common Is Latex Allergy?

It is difficult to say how widespread the problem of latex allergy may be. More than 1,700 cases of latex allergic or anaphylactic reactions, including 17 deaths, have been reported to the U.S. Food and Drug Administration (FDA) since 1988. It is assumed that many other cases go unreported.

Known risk groups include:

  • Health care workers and others who wear latex gloves
  • Individuals who have a history or who will be undergoing multiple surgical procedures, such as children with spina bifida
  • Individuals with a history of progressive allergic reactions to foods known to cross-react with natural rubber latex
Other risk factors are less defined but appear to include a history of:
  • Allergic rhinitis (hay fever) or any other allergy
  • Hand dermatitis, particularly if severe or if changing in severity in those who wears latex gloves

Outside of these recognized risk groups, latex allergy is very uncommon, with estimates of less than 1 percent prevalence in the general population.

Why Is Latex Allergy More Prevalent Now?

The introduction of Universal Precautions in health care settings to prevent the spread of AIDS and hepatitis B resulted in a dramatic increase in glove usage. According to the U.S. Food and Drug Administration, most of the gloves used are imported into the United States, and from 1991 to 1996 the number of such gloves increased by 247 percent to over 18 billion pairs of gloves. Latex gloves are the largest source of direct contact with latex products and the major source of latex aeroallergen in areas where powdered latex gloves are in use.

Who Is Most at Risk of Developing a Life-Threatening Anaphylactic Reaction to Latex?

The risk of anaphylaxis appears to be greatest in individuals with prior allergic reactions to latex-containing objects or prior, unexplained reactions or anaphylaxis during a medical or surgical procedure. Health care providers with a history of severe or worsening latex-glove-induced eczema, hives or work-related rhinitis or asthma-like symptoms should be especially cautious.

What Foods Are Known to Cross-React With Latex?

Some foods to which latex allergic patients frequently demonstrate sensitivity include avocado, banana, chestnut, kiwi, raw potato, tomato, stone fruits (such as peach, plum and cherry) hazelnut, melons, celery, carrot, apple, pear, papaya and almonds. Reactions are less common but have been reported to peanut, peppers, citrus fruits, coconut, pineapple, mango, fig, passion fruit, condurango bark and ugli fruit.

Reactions to many foods have been reported in latex allergic patients. In many cases, researchers have confirmed the presence of cross-reacting proteins with proteins found in latex. It is now thought that many of these allergenic proteins are plant defense proteins found widely in the botanical realm.

While food allergy is common in latex allergic individuals, neither the presence nor the distinct food allergies can be predicted for any patient. More severe latex reactions do appear to necessarily increase the risk of food reactions. Initial manifestations of food allergy can be severe and even anaphylactic. Latex allergic patients should have personal epinephrine syringes available at all times for this reason alone.

Patients with a history of food allergies to foods known to cross-react with latex rubber, particularly if expanding to new foods and progressive in severity, should be considered at risk for latex allergy as well.

How Can Latex Allergy Be Prevented?

All products and medical devices that come in contact with individuals at risk should be reviewed for possible latex content.

Respiratory exposure to latex proteins can take place in the absence of skin contact, since latex glove donning powders bind latex proteins in the gloves and carry them into the air. In recognition of this fact and that powdered latex gloves are the major source of latex aeroallergen, the American College of Allergy, Asthma and Immunology has issued a joint statement with the American Academy of Allergy, Asthma and Immunology calling for usage of only non-powdered low-allergen gloves. Synthetic and vinyl gloves may be acceptable alternatives.

How Can Health Care Workers Protect Themselves From Developing Latex Hypersensitivity?

Health care workers with a history of latex sensitivity who must wear gloves should stop wearing latex gloves, and their co-workers should not use powdered gloves. Care should be exercised in the choice of substitutes, since all synthetic or non-latex products are not equally impermeable to blood-borne pathogens.

Health care workers with a history of glove-associated skin irritations, or contact dermatitis, should use alternative gloves (which may include latex gloves) and topical treatments to relieve their symptoms. Petroleum-based products have been shown to compromise the barrier function of latex gloves, and care should be taken in the choice of treatments used to relieve contact dermatitis.

How Is a Suspected Latex Allergy Confirmed?

An allergy skin test may be done to test for latex allergy, but there are currently no reagents commercially available for the test in the United States. Recent research has demonstrated that latex skin testing with the material that is currently commercially available in Canada and Europe has better diagnostic sensitivity than the current available FDA approved blood tests. Because of the potential for a life-threatening anaphylactic reaction to the test itself, skin prick tests for latex allergy should be performed only under the close supervision of an allergy specialist. An allergist-immunologist also can order an IgE allergy blood test to confirm the presence of IgE anti-latex antibodies. Skin patch tests are used to evaluate the cause of skin irritations, or contact dermatitis, caused by chemicals inherent in rubber gloves.

How Are Latex Allergies Treated?

The best treatment for latex allergy is avoidance. Allergy specialists can provide latex allergic patients with information which will help them identify situations that place them at risk; strategies for avoiding an allergic reaction; and information about sources of natural rubber latex exposure and ways to avoid skin and mucosal contact.

Patients with latex allergy are at risk of asthma on exposure to latex-containing aerosols and should try to avoid areas where powdered latex gloves or their products are used.

Personal measures including warning bracelets and adrenaline syringes, like those commonly used for bee sting allergic patients, should be worn or carried at all times. Unexpected exposures to latex during dental, medical or surgical procedures may be prevented by warning health care providers of latex allergy prior to any scheduled visit and at the time of emergency visits.

Allergic reactions to a wide variety of fresh fruits, vegetables, legumes and nuts may complicate the course of latex allergy. Most commonly, banana, avocado, kiwi, hazelnut, raw potato, tomato, papaya, citrus, celery or stone fruits are involved.

Updated March 2000


© Copyright American College of Allergy, Asthma and Immunology




Spina Bifida - Neurogenic Bladder


The normal function of the bladder is to store and expel urine in a coordinated, controlled fashion. This coordinated activity is regulated by the central and peripheral nervous systems. But what happens when the bladder malfunctions as a result of dysfunction, trauma, disease or injury? The information below should help you recognize this problem before it causes serious damage.

What happens under normal conditions

The urinary bladder, a spherical organ, has a soft inner lining (similar to the inner cheek) and an outer muscle layer. In addition to the bladder, the bladder neck (funnel-like outlet of the bladder which leads to the urethra), the urethra (tube-like structure which serves as a channel to carry urine from the bladder to the external surface) and the external urethral sphincter muscle (group of muscles which surround the urinary passage below the bladder neck) complete the lower urinary tract.


The muscles and nerves of the urinary system must function in a coordinated fashion with the bladder in order to perform its two major functions of storage and elimination of urine. Nerves carry messages from the bladder to the brain and then from the brain to the muscles of the bladder telling them to either tighten or release, allowing the bladder to empty during urination.

Most babies with spina bifida develop further problems later on, including:

What is neurogenic bladder?

Neurogenic bladder is the loss of normal bladder function caused by damage to part of the nervous system. The damage can cause the bladder to be underactive, in which it is unable to contract and unable to empty completely, or it can be overactive, in which it contracts too quickly or frequently.

What are some risk factors for neurogenic bladder?

Risk factors for neurogenic bladder include various birth defects, which adversely affect the spinal cord and function of the bladder, including spina bifida and other spinal cord abnormalities. Tumors within the spinal cord or pelvis may also disrupt normal nervous tissue function and place an individual at risk. Traumatic spinal cord injury is also a major risk factor for development of neurogenic bladder.

What are the symptoms of neurogenic bladder?

Inability to control urination, also known as urinary incontinence, is perhaps the most common symptom associated with the neurogenic bladder. This may be caused by abnormalities in bladder capacity or malfunction of control mechanisms such as the bladder neck and/or external urethral sphincter muscle that are important for the bladder's storage function.

Symptoms including a dribbling urinary stream, straining during urination or inability to urinate may also be associated with neurogenic bladder. Urinary retention may result either from loss of bladder muscle contracting performance or loss of appropriate coordination between the bladder muscle and the external urethral sphincter muscle.

Irritating symptoms, such as urinary frequency and urgency, may be evidence of bladder hyperactivity. Other irritating symptoms may include painful urination (dysuria ), which may be a result of a urinary tract infection (UTI) caused by urine being held too long in the bladder. UTI with fever is a sign of potential severe kidney infection (pyelonephritis ) and is a more worrisome situation as it may result in permanent damage of the kidney(s).

Stones may also form in the urinary tract of individuals with a neurogenic bladder caused by the stoppage of urine flow and/or infection.

Abnormal backup of urine from the bladder to the kidney(s), also known as vesicoureteral reflux (VUR ), may develop as a means of releasing high pressure within the bladder. A UTI is of particular concern as VUR may place the patient at significant risk for a severe kidney infection by transporting infected bladder urine directly to the kidney(s).

How is neurogenic bladder diagnosed?

When neurogenic bladder is suspected, both the nervous system (including the brain) and the bladder itself are tested. In addition to complete medical history and physical examination, diagnostic procedures may include: ¤ X-rays of the skull and spine ¤ an electroencephalogram (EEG) to identify brain dysfunction ¤ imaging tests of the bladder and ureters ¤ function tests that involve filling the bladder to see how much it can hold and if it empties completely

How is neurogenic bladder treated?

Medication for treatment of overactive bladder may improve or relieve irritating symptoms and/or incontinence. Antibiotics are important for treatment and prevention of urinary tract infections (UTIs), especially in patients with vesicoureteral reflux. Other medications may improve bladder control by increasing outlet resistance at the bladder neck.

Clean intermittent catheterization (CIC) was developed by Dr. Jack Lapides in the early 1970s as a means of emptying the bladder in the case of a bladder muscle that is unable to contract or in patients with loss of appropriate coordination between the bladder muscle and the external urethral sphincter muscle.

Surgical cutting of the external urethral sphincter with the use of an endoscope passed through the urethra may allow free flow of urine into an appropriate receptacle and eliminate the need for CIC in order to empty the bladder. Also, endoscopic injection of paralytic agents directly into the external urethral sphincter muscle is another technique that provides temporary relief.

Permanent stents can also be used in the bladder neck for effective urinary transportation.

At times, however, damage to bladder anatomy and function is so severe that capacity needs to be improved with bladder augmentation (increasing bladder size with various tissues), vesicoureteral reflux needs to be corrected and/or an alternate tube for emptying the bladder may need to be surgically constructed.

What can be expected after treatment for neurogenic bladder?

A person can expect extensive follow-up evaluation of bladder and kidney function. This may involve repeated X-rays, ultrasound, blood tests and bladder function tests.

Although some characteristics of the neurogenic bladder may improve or resolve, most issues typically require constant attention and reevaluation.

Proactive management of patients with a neurogenic bladder may decrease the risk of damage to the bladder and kidney(s) and, therefore, potentially limit the need for future intervention. This has been shown in management of neurogenic bladder patients with spina bifida.

Frequently asked questions:

What is the likelihood that my child with spina bifida will develop neurogenic bladder?

The risk of neurogenic bladder is significant in this population and, therefore, careful and frequent evaluation of bladder function is recommended.

What are expected limitations for a patient with neurogenic bladder?

Limits are typically a function of the cause of neurogenic bladder (such as spinal cord injury, for example), rather than the neurogenic bladder itself.

Can the effects of a neurogenic bladder be prevented?

Some effects of neurogenic bladder are preventable with aggressive management with medication and at times appropriate surgical reconstruction.



Helpful Links


http://www.healthychildren.org/English/health-issues/conditions/developmental-disabilities/Pages/Spina-Bifida.aspx
http://www.ninds.nih.gov/disorders/spina_bifida/spina_bifida.htm