Spina Bifida and Sex

YES!!! People with Spina Bifida can have sex!!! Shocking revelation, I know!!!! :-P In fact, many of us (yes, I said US...I'm including myself in this one!) have GREAT sex lives in and outside of marriage and have families of our own. All people have a right to a full sexual life. Sex and reproduction are increasingly important for young people as they reach adulthood and beyond.

Sexual performance and spina bifida: The facts

• The nerve damage in spina bifida that affects urinary and bowel functions may also affect sexual functioning.
• Males may have normal sexual function, but this is commonly affected to some degree. Satisfactory erections are often possible, but there is no ejaculation. Other types of sexual dysfunction are also possible.
• Females are generally less affected in their sexual functioning.
• In males and females, altered genital sensation can affect - but does not prevent - arousal patterns and sexual functions.
• Orthopaedic problems, for example with lower limbs, can affect the ability to use some, but not all, sexual positions.
• Most males and females with spina bifida are fertile. Many of the issues surrounding conception in spina bifida are due to mechanical and anatomical difficulties in conceiving rather than a lack of fertility. There is access to assisted reproductive techniques. Consult your clinic about these.
• Since people with spina bifida often have latex allergies, you and your partner may need an alternative form of contraception to condoms. See your doctor for advice about forms of contraception.
• The major obstacles to a happy sex life are:

- lack of confidence and self esteem
- not meeting people
- no car
- not seeing ‘sex’ as being possible
- incontinence -- the same nerves that control urinary function also control sexual function. Therefore, if one has poor sensation, the other will also. It is very important to have an understanding partner and to communicate clearly about the possibility of urine or stool leakages during sex. Decrease the risk by emptying your bladder and bowels beforehand. This should take care of most of the urine and stool at risk for being accidentally released during sex. In case there is some leakage during sex, have a moist towel nearby to wipe it away. For more information, review SBA’s health information sheets on women’s and men’s health issues. 

- gaining independence from your parents.

Sex and spina bifida: Important questions and positive answers:

I don’t want to discuss sexuality in front of my parents. I’m too embarrassed.

This is a common concern.

A supportive environment is essential.

You should feel free to ask your doctor or continence nurse for any information about your own sexual functioning when consulting them for routine assessment.

Remember, these professionals have a lot of experience in dealing with sexual issues and will understand any concerns you may have.
The best people to speak to about sexuality, relationships and achieving a meaningful sex life are adults with spina bifida who have ‘been there and done that’.

When you first start to talk about sex it will be embarrassing, but after a while you will realize that it’s good to do so and that it will help you help yourself!

What about the way my body is ‘different’ from other people’s?

The issues of body image and self esteem that arise in spina bifida profoundly influence a person’s sexual expression.

Remember, people with profound disabilities are capable of active sex lives.

Be reassured. Stephen Hawking the famous physicist, has overcome a great physical disability. He has fathered children.

Could my baby have spina bifida?

Yes. If you or your partner have spina bifida your risk of having a child so affected is much greater. Did you know that folate (a safe vitamin) taken by women before and during pregnancy could significantly protect against spina bifida?

Genetic counselling is very important for both men and women who want to have a child. A genetic counselor will explain the risks and the ways of protecting your baby from getting spina bifida and what screening tests can be done during the pregnancy. Your doctor can arrange for you and your partner to see a genetic counselor before you get pregnant.

Sex and spina bifida: Important questions and positive answers for men

Can males with spina bifida father children?

Yes, of course. Many men with spina bifida are fathers.

Less is know about fertility in men with spina bifida than in affected women.

Men typically have problems with sexual function, which can be overcome.

Most men are fertile and can successfully father children.

What difficulties do men with spina bifida have during intercourse?

The major difficulty is achieving erections and ejaculation.

Difficulty in achieving erection is a common problem and is often easily treatable. Drugs, including Viagra, or injections often work. Physical techniques such as vacuum pumps or surgery are available. If you have difficulty with erections, you should not give up the idea of having sex until you have looked at treatment options.

Ejaculation is often impaired in men and achieving ejaculation is more difficult. Did you know that failure to ejaculate will not have any effect on the pleasure you give your partner?

There are safe methods of artificially collecting sperm which can be used to make your baby. This can be explained to you by a specialist.

Many young inexperienced guys worry that leg weakness will impair their sexual performance using the missionary position (on top). There are many other positions which can be used. Did you know that using these positions will not reduce the pleasure you give your partner?

Getting and Keeping an Erection 

This may be caused by your Spina Bifida or other conditions such as diabetes but can also 

be affected by whether you are feeling anxious, self-conscious or tired. Different 

treatments are available depending on the type of difficulty you’re having. Your doctor 

may want you to have a blood test to check your levels of testosterone, a hormone which 

can affect your body and feelings in different ways. The effects of some medication, 

nicotine and drug use can also be important. 

Treatments can include: 

Injections: 

Some treatments are given into the penis by injection. Prostaglandins, such as Alprostadil, 

have a high success rate with a quick response and few interactions with other 

medication. 

Papaverine has a lower success rate. Such treatments may be given at spinal injury units, 

urology clinics, or rehabilitation centres. You, or your partner, can learn to give these 

injections. There may be a risk of scarring, and the penis becoming misshapen (called 

priapism) or having a prolonged erection. There may be medical reasons why treatment is 

not suitable in your case. 

Alprostadil can also be given without injection, as a pellet in the urethra (Muse) but this has 

a much lower success rate and can also cause priapism, pain in the penis, and irritation to 

your partner’s vagina/mouth. 

Drugs: 

Such as Viagra and Cialis are available on prescription in limited amounts for people with 

Spina Bifida. A doctor who knows you will know whether there are any particular risks (such 

as a history of heart disease) for you in taking these and it is important for you to have a 

thorough physical examination in the area of your genitals to check there are no problems 

before starting to take them. They are available to buy privately on the net but you are 

advised not to do this – you may not always get the genuine article. There is some 

evidence that these have also boosted the sexual confidence of men with Spina Bifida 

using them. You need to take them at least 20-30 minutes before you hope to have 

intercourse and they will not work on their own: you need to have the usual foreplay to 

help you become aroused. If they don’t seem to work for you, try them out on three 

separate occasions before giving up and check with your doctor whether a different dose 

can be tried.

Vacuum devices: 

The penis is placed in a tube in which a vacuum is artificially created and blood is drawn 

into the penis, giving an erection. A constricting band is placed on the base of the penis to 

hold the blood in the penis and help the erection to last. They give a softer erection but 

enough for penetrative sex. They can be fiddly to fit and need practice and persistence. 

Newer models have an electric rather than a manual pump. If you have reduced 

sensation, you should not leave one on too long and should check your skin and pressure 

areas around the penis. Some vacuum devices are available on prescription from a GP. 

Some men, who can get a partial erection but want to maintain it, use just the ring part of 

the device and you can buy something similar in sex shops but need to be sure that you 

have the right size. 

Surgical implants: 

These are usually a last resort and you would need to be fit for surgery which would be 

carried out by a urologist. Older models involving a silastic (like flexible plastic) implant into 

the penis give you a permanent erection allowing penetration but not sensation or 

ejaculation. Newer models into the groin and penis allow you to press part of the implant in 

the groin to give an erection when needed and release it afterwards. Again they do not 

improve sensation or ejaculation. 

Premature Ejaculation 

This is ‘coming’ earlier than you’d like. When you’re masturbating, this may not matter so 

much to you but, if it is a problem for you or your partner, you may be helped by learning 

to understand your body signals or through some counselling or behavioral therapy. 

Medication may be considered as part of managing the problem. You may be able to find 

help with this through a local specialist clinic or Spinal Injuries Unit or through your urologist 

or urology nurse specialist. 

Slow Ejaculation 

When you’re unable to come to orgasm or it takes an age. Again, this may not happen 

when you’re masturbating and it might be caused by Spina Bifida or by how you’re feeling 

or a mixture of both. In some cases it may be caused because the nerve supply is 

damaged so that you don’t get the contractions that lead to orgasm or get the full 

sensation of orgasm. Again, remember that many people who have difficulty with 

penetrative sex can still have very good sex lives, finding pleasure in other areas of the 

body.

Retrograde Ejaculation 

This is when semen goes back into your bladder instead of out through your penis. It 

doesn't do any harm but can make your urine look cloudy when you next empty your 

bladder. It might be important for you if you hope to have children, and there are 

alternative ways of collecting your sperm. (See Fertility below). 

Fertility 

There are many proud fathers with Spina Bifida but, if you’re worried about this, you need 

to discuss it with your GP, urologist or other specialist who may run some tests to check that 

all is well with your sperm count. If it’s low or if you have difficulties with retrograde 

ejaculation (above) you may need to ask about referral to a specialist sexual health or 

fertility clinic or Spinal Injuries Unit. 

Golden rule:

Men with spina bifida can have great sex lives and can give their partners as much pleasure as guys without disability.

Sex and spina bifida: Important questions and positive answers for women:

Do I have a normal sexual response which is the same as for a woman without spina bifida?

Yes. Although women with spina bifida often report different vaginal sensation, this is not an obstacle to normal sexual responsiveness or your capacity to give pleasure to your partner.

Can women with spina bifida bear children?

Yes. Of course. Many women with spina bifida are mothers. Generally, fertility is not affected in women. In fact, it is important to use contraception to avoid an unplanned pregnancy.

Did you know that a study of women with spina bifida in Victoria found that although special intervention is sometimes needed, pregnancy and child birth is not tremendously difficult and does not do damage to, or worsen, mobility, continence, functioning or shunt function.

There are obstetricians (specialists who care for pregnant women and deliver babies) with experience in spina bifida who can advise and care for you. Your doctor can refer you and your partner to a specialist for advice before you become pregnant as well as for the pregnancy.

I am in a stable relationship and would like to have a child. How will spina bifida affect my pregnancy?

As described above, the course of pregnancy for you is similar to women without spina bifida except for:

• an increased risk of urinary tract infection
• the risk of pressure sores - from the extra weight you are carrying
• an increased risk rate of lower pelvic pain.

Should I have a vaginal or caesarean delivery?

The current recommendation is that you should be encouraged to deliver vaginally. It has been noted that women who have vaginal deliveries have fewer complications, faster recovery times and shorter hospital stays.

Women delivered by caesarean have a higher rate of complications, and surgery can be complicated if there is past history of urological surgery. Your specialist will recommend the best course for you to take.

Golden rule:

Women with spina bifida can have great sex lives and can give their partners as much pleasure as women without disability.

****************************Contraception*****************************************
If you are using condoms, remember to get latex-free ones – available from Durex or Trojan.
SAFE SEX is important for everyone, straight or gay, male or female, and whether you’re having 
full intercourse or oral sex. You are as likely as anyone else to pick up a sexually transmitted 
disease but, if you have reduced feeling, may not recognize it as quickly. Again 

remember to use latex-free condoms to avoid any possible allergic reaction.

There are natural skin condoms, however natural animal skin (i.e. lamb/sheep skin) condoms ONLY help prevent pregnancy as sperm cells are too big to pass through the skin membranes. Animal skin condoms have larger microscopic openings and may allow the organisms that cause STDs (i.e. HIV) to pass through which can infect a sexual partner.

****Do NOT use animal skin condoms to help prevent transmission of STD's or HIV.****

If you or your partner are allergic to latex or on latex precautions, the only full proof protection against pregnancy and/or STD transmission when using a non-latex condom is using one made out of polyurethane or polyisporene.

Durex makes a non latex condom called Avanti. Avanti is made from polyisoprene and Trojan has a polyurethane condom called Trojan Supra.

Information used for this article was obtained by:

http://www.sbhi.ie/images/Sex-for-Men.pdf

http://www.spinabifida-incontinence.info/sex.htm

http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8095349/k.F32A/Sexuality.htm

Spina Bifida and Lipomyelocele

***The following article is from http://www.spinabifidaassociation.org/site/c.evKRI7OXIoJ8H/b.8277191/k.86B0/Lipomyelocele.htm

What is Lipomyelocele?

A lipomyelocele is a type of lipoma that occurs with Spina Bifida. A lipoma is a fatty fibrous tissue mass in the spinal column that extends on the backside through a Spina Bifida defect (gap or opening in the malformed spine). It connects to the spinal cord against the skin. The defect is covered by skin, making it “hidden” (occult). This is termed, closed Spina Bifida (incomplete closure of the neural tube).

Lipomas are the most common type of occult (hidden) SB, although other types may be under-diagnosed or misdiagnosed. Lipomas often occur in the lowest part of the spine. The location of the lipomyelocele is important because any symptoms that occur, will present at that level of the body and below.

When does it occur? 

The defect occurs during the embryonic stage of development, often before a woman even knows she is pregnant.

What causes it?

A lipomyelocele is caused by premature separation (dysjunction) of the superficial ectoderm (which forms skin) from the neural ectoderm (nerve cells) which forms the spinal cord. The “not-yet closed” neural tube further develops into fat at that location. The lipoma is attached to subcutaneous fat and the back of the spinal cord; thereby “tethering” the spinal cord. The “tethered cord” can be symptomatic over time.

What symptoms occur?

As the baby develops, urinary and bowel incontinence can occur, poor or no sensation in lower part of the body may be identified, as well as limb weakness, difficulty with walking, and pain.

How do you treat it?

Treatment varies, and is based on multi-disciplinary clinical assessment, tests (such as urodynamics) and imaging (X-ray, MRI). Sometimes, the tethered spinal cord needs to be surgically untethered or released to prevent or minimize symptoms. De-tethering surgery may need to be repeated, but after each surgery, more scar tissue develops, which can cause re-tethering and other problems.

Every child is unique, so individual assessment will determine the best plan for treatment, and that can change. Generally though, ongoing specialist care will be necessary in the following areas:

• Neurosurgery
• Orthopedics
• Urology
• Gastroenterology

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

Spina Bifida and Nonverbal Learning Disorders

Nonverbal Learning Disorder Syndrome

  
    It is not uncommon for children with hydrocephalus to have learning disabilities. Nonverbal Learning Disorder Syndrome (NVLD) is a specific type of learning disability that affects children's academic progress as well as their social and emotional development. This specific type of learning disability has been identified in children with hydrocephalus.
    NVLD encompasses a combination of learning, academic, social and emotional issues. Most children with learning disabilities do NOT have significant problems with normal social and emotional development. Some children may have the academic difficulties associated with NVLD but do quite well socially and emotionally. A valid diagnosis of NVLD includes a combination of learning, academic, social and emotional issues as described in this article.Additionally, because the pattern of academic strengths and weaknesses may not show up early in life, and difficulties with social relations are not always apparent in the very young, it is often difficult to make a diagnosis of NVLD until a child is in middle to late elementary school.

ACADEMIC CHARACTERISTICS

    Children with NVLD have difficulty with mechanical arithmetic, particularly more complex math involving many columns (such as long division). They have problems keeping columns straight, often mixing up which column to put a number in when they carry over in addition. They can have difficulty with word problems or math reasoning, being unable to read a math problem and know what operation to perform. Higher math skills that rely on spatial abilities or seeing the relation between concepts (such as in geometry or algebra) are especially difficult for them to acquire.
   
    Children with NVLD often do quite well with word recognition, oral reading and spelling. While they might be slower in learning to recognize their letters, once they master early reading skills they show good phonetic skills (word pronunciation). However, reading comprehension is weak, especially for more abstract or novel subject matter. The child with NVLD may be able to read a paragraph quite fluently but then be unable to extract the main point or answer conceptual questions about what has just been read.
    Language abilities are also unique in a child with NVLD. Some may show an initial delay in early expressive speech but then rapidly show gains, progressing to become very talkative or even excessively verbal. This speech pattern (verbosity) has been termed "cocktail party speech" because although a great deal may be said, the content may have little of substance or value. Compared to their peers, children with NVLD tend to rely more heavily on language to engage and relate to people, to gather information and to relieve anxiety. For instance, when little, instead of picking up and manipulating an object that is new to them, they may instead question an adult about what it is, how it works, etc.
    Children with NVLD may develop a great deal of skill in talking their way out of challenging tasks or facing novel situations that provide anxiety. Their rote verbal capacities and rote verbal memory skills may be a personal strength but they show poor language pragmatics or the functional use of language.
    Nonverbal tasks may be quite difficult for children with NVLD. On tasks that require fine motor coordination these children often show early delay. Early paper/pencil tasks can be extremely frustrating for them and later handwriting may show poor quality. On formal tasks of cognitive functioning they do much better on verbal tasks compared to nonverbal tasks. Tasks that require interpreting or pulling together visual information can be hard, especially if it is not possible to explain the task verbally with step-by-step instruction. Additionally, verbal tasks that entail more complex problem solving or the integration of information from various sources are quite hard for children with NVLD. Children struggle with common academic tasks such as answering questions at the end of a chapter or performing on tests where the questions are worded differently from the study material.
    Humor or sarcasm can be hard for children with NVLD to appreciate. They often cannot understand jokes, or they interpret them in such a CONCRETE way that the humor is lost. Sarcasm, expressed by the mismatch between a spoken message and the facial expression or tone of voice, requires integration of information from different sensory modalities. Children with NVLD may interpret the message quite literally, missing altogether the information needed to recognize that is sarcasm.

SOCIAL OR ADAPTIVE CHARACTERISTICS

    Novel situations can be particularly troublesome as they require generating responses that cannot be anticipated or practiced beforehand. Children with NVLD often rely on ROTE or PRACTICED behaviors that may not be appropriate for the context. For example, if they learn the right way to introduce themselves to an unfamiliar adult (by shaking hands and saying "pleased to meet you") they may attempt the same response in a group of children where it might be viewed as odd or "nerdy". When peers give them subtle feedback, such as raised eyebrows, they miss the information completely and cannot therefore modify their behavior next time. Peers may pull away or nonverbally signal the end of the conversation and children with NVLD may pursue the interaction, talking even as the peer turns his back. Children with NVLD wants friends, just like everyone else and they may intensify their efforts to reach out, despite repeated rejection. The recognition that they are being rejected may not come until they are older; their hurt and confusion grows because they are unable to understand the increasingly complex social rules of adolescence.
   

CAUSE OF NONVERBAL LEARNING DISORDER SYNDROME

    Although no cause for this disorder has been definitely identified, it is known that deficits in the functioning of the right hemisphere of the brain play a significant role. The brain is divided into two hemispheres, the right and the left, which typically compliment each other in functioning but are suited for different types of processing. The right hemisphere can integrate information from several sensory modalities at once (can interpret visual and spoken information at once, thereby clarifying how a facial expression can change the meaning of a verbal message) and is best for processing novel information. The left hemisphere processes information presented in a step-by-step fashion and is best at using information once it is well practiced or rote. Spoken language is processed by the left hemisphere, visual or nonverbal information is processed by the right hemisphere, and the right hemisphere additionally becomes involved in understanding anything novel or contradictory between the verbal and nonverbal messages.
    Deficits in the functioning of the right hemisphere, observed in children with NVLD, could emerge through various avenues. If there is any early interruption in the development of the central nervous system, the right hemisphere is more likely to be compromised than the left. Direct damage to the right hemisphere through trauma, tumors and/or seizures can cause compromise in right hemisphere functioning. Sometimes there is no known reason for observed weakness in right hemisphere functioning.

INTERVENTIONS

1. Children with NVLD do best with instruction that is verbal and descriptive in nature. Instead of showing them how to perform a math operation, for instance, they should be verbally instructed in a step-by-step manner.
2. Assess reading comprehension carefully because good oral reading can hide the extent of weak comprehension. Teach strategies to aide comprehension such as learning to identify the topic sentence and highlighting important information for later study or review. Tell them specific facts they will need to know for a test rather than asking them to determine, on their own, what important information within a text or lecture they should focus on.
3. Because language concepts can be weak, children with NVLD need to understand terms such as same versus different, part-to-whole relationships, how to classify or categorize objects and the difference between cause and effect. In expressive language instruction they should focus on staying on the topic, listening without interrupting, and recognizing when someone has signaled the end of a conversation.
4. Spatial concepts are difficult so children may need to learn verbal self-instruction for analyzing and reproducing designs. Certain tasks such as map reading or learning the location of all the capital cities should be avoided altogether. If telling time on a clock fact is very challenging, teach telling time with a digital clock instead.
5. Written work can be extremely frustrating due to the combination of mechanical problems related to fine motor delays and poor visual spatial skills. Decrease the quantity of writing expected and instead allow verbal expression of information. Additionally, teach early keyboard skills.
6. Involve the school counselor or social worker to foster social development at school. Friendship groups that involve a small number of selected peers are one intervention. Teachers can help identify which classmates would be most responsive to and supportive of your child. Specific and concrete instruction such as teaching the child how an and when to initiate peer interactions, how to wait one's turn or the appropriate moment to speak, how to make consistent eye contact and pleasant facial expressions can be very beneficial.
7. Create a supportive home environment in which your child feels secure and successful. Minimize demands that highlight your child's weaknesses by being clear and specific about what you expect. Observe your child carefully in novel or complex situations to gain an appreciation of strengths and weaknesses and set your expectations accordingly.

    Remember that watching you do something is not the best way for your child to learn. Instead, instruct them in a step-by-step manner. Also, reminder lists of even basic tasks such as daily hygiene and simple chores is very helpful. It is all right to point out to them what they may not yet have recognized about themselves, i.e.."You do much better when you know what's going to happen than when you get unexpected surprises." Constructive suggestions rather than criticism works best.
    And, as you've heard countless times before, you are the best advocate for your child. Collaborate with your child's school about proper inventions and work hard to develop and maintain a positive relationship with school personnel so that you can share what has worked for your child in the past and brainstorm with them about other interventions that might be helpful.

Nonverbal Learning Disorder Syndrome is not a widely recognized diagnosis and school personnel may be genuinely unsure about how to best serve your child. A comprehensive and thorough neuropsychological assessment by an experienced clinician, with regular follow ups, is critical to insure that appropriate strategies are put in place to assist your child in realizing his or her potential.

Nonverbal learning disorder syndrome is a specific type of learning disorder that has been identified with some children with hydrocephalus. The discovery of the NLD syndrome began in the early 1970's, however, even today many professionals in the field of education are uninformed about NLD and many children with this learning disability go undiagnosed.

What does nonverbal learning disorder look like?
    Children with nonverbal learning disorder are often very bright. Young children may even be targeted as gifted due to their mature vocabulary, rote memory skills and apparent reading ability. However, parents may notice early that their children have difficulty with things like interacting with other children, self-help skills or fine and gross motor activities. Parents may suspect that something is amiss early on, but they can't quite put a finger on it. Children with NLD may manage to go along through their early elementary years handling academic demands fairly well. But as they enter the upper elementary grades or begin middle school, they are left to handle more tasks on their own and things rapidly deteriorate. They get lost, forget to do homework, have difficulty following directions, struggle with math, can't write an essay and are continually misunderstood by both their peers and teachers. They are accused of being lazy, rude or uncooperative. They are not any of these things-they have NLD.

What causes nonverbal learning disorder?
    Current evidence and theories suggest that the destruction, disorder or dysfunction of white matter in the right hemisphere of the brain could be the cause of nonverbal learning disorders. Researchers have associated NLD with patients who have neurological histories including:

• moderate to severe head injury
• repeated radiation treatments
• tumors
• seizures
• congenital absence of the corpus callosum (the band of neural fibers connecting the two cerebral hemispheres)
• hydrocephalus

    Each of these neurological conditions involves destruction of white matter connections in the right hemisphere of the brain.

Three categories of dysfunction present themselves in children with NLD:

• Motoric-a child with nonverbal learning disorders commonly appear awkward and is, in fact, inadequately coordinated in both fine and gross motor skills. He or she may have had extreme difficulty learning to ride a bike or kick a soccer ball. Fine motor skills, such as cutting with scissors or tying shoelaces, seem impossible for this child to master. A young child with NLD is less likely to explore his or her environment motorically.
• Visual-Spatial-Organizational-In the early years, a child may appear confused much of the time, despite a high intelligence and high scores on receptive and expressive language measures. It is difficult for this child to change from one activity to another or to move from one place to another. A child with NLD may use all of his or her concentration and attention to merely get through a room.
• Social-a child with NLD may lack the ability to comprehend nonverbal communication. He or she may have trouble perceiving subtle cues in social environment, such as when someone has gone far enough, the idea of "personal space," facial expressions of others and nonverbal signals that another person is registering pleasure or displeasure.

Common characteristics of nonverbal learning disorders

• performance IQ significantly lower than verbal IQ
• early speech and vocabulary development
• remarkable rote memory skills
• attention to detail
• early reading skills development, excellent spelling skills
• expresses him or her self eloquently
• lack of coordination
• severe balance problems
• difficulties with fine motor skills
• lack of image, poor visual recall
• faulty spatial perceptions
• difficulties with spatial relations
• lack of ability to comprehend nonverbal communication
• difficulties adjusting to transitions and new situations
• significant deficits in social judgment and interaction

Assessment

    The assessment of individuals suspected to have NLD should be conducted by an interdisciplinary team and focused on developing an appropriate intervention plan. There is no single test, cluster of tests or cut-off score on an individual test that will alone signal the presence of NLD. It is important for a child's parents to be involved at all levels of this process. The most crucial consideration when retaining a professional for an evaluation of your child is that he or she be knowledgeable of NLD and know how to distinguish it from other disorders with the same or similar symptoms.

A comprehensive assessment would include the following components:

• A thorough developmental history
• Neuropsychological testing
• A speech and language evaluation
• Educational assessments
• Occupational therapy evaluation

Some of the professionals who might be involved in the diagnostic process include the following:

• A developmental pediatrician
• A pediatric neurologist
• A neuropsychologist
• A speech and language pathologist
• An educational therapist

    Nonverbal learning disorders constitute a dysfunction in the basic cerebral processes and as such denote a disability that warrants specialized support and programs modifications and accommodations for a student with NLD. All of the identified supports, modifications and accommodations should be addressed in the student's IEP or 504 plan. Teachers and parents should work closely together in planning and accommodating each child's unique needs.

***It is important to note that not every child with Spina Bifida will develop nonverbal learning disorders.***

Spina Bifida and Chiari

Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. When the indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination. There are three primary types of CM. The most common is Type I, which may not cause symptoms and is often found by accident during an examination for another condition. Type II (also called Arnold-Chiari malformation) is usually accompanied by a myelomeningocele-a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back. This can cause partial or complete paralysis below the spinal opening. Type III is the most serious form of CM, and causes severe neurological defects. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia, and spinal curvature.

Medications may ease certain symptoms, such as pain. Surgery is the only treatment available to correct functional disturbances or halt the progression of damage to the central nervous system. More than one surgery may be needed to treat the condition. Many people with Type I CM are asymptomatic and do not know they have the condition. Many patients with the more severe types of CM and have surgery see a reduction in their symptoms and/or prolonged periods of relative stability, although paralysis is generally permanent.

The NINDS supports research on disorders of the brain and nervous system such as Chiari malformations. The goals of this research are to increase scientific understanding of these disorders and to find ways to prevent, treat, and, ultimately, cure them.  Current NINDS-funded research includes studies to better understand the genetic factors responsible for the malformation, and  factors that influence the development, progression, and relief of symptoms among patients with syringomyelia, including those with Chiari I malformations.

Symptomatic Chiari Malformation

Persons with Myelomeningocele have abnormalities that are not limited to their spinal cord. These include the development of hydrocephalus (in up to 85% of patients) and the development of symptoms related to the area where the brain and spine join (Chiari malformation).

What is a Chiari malformation?
Over a hundred years ago, Professor Chiari, an Austrian pathologist studied a group of patients who had a portion of the brain displaced into the neck. He graded these malformations in order of severity. Type I (the mildest form) occurs when the lowest part of the cerebellum, called the cerebellar tonsils, descends a short distance into the cervical spine.

What type occurs in SB? 
Chiari Type II or Arnold-Chiari malformation, is a more severe form in which the cerebellar vermis and some portion of the brain stem descend into the cervical spine. Many changes of the brain are associated with this abnormality. Although some degree of this malformation is present in the vast majority of persons with Spina Bifida, it appears to be symptomatic in about one of three individuals. This 33% may be a falsely low figure in that, as we follow patients for longer periods of time, adolescents and young adults appear to have some propensity to develop clinical progression. The development of symptoms related to the Chiari malformation is, in part, related to the severity of the malformation.

When brain tissue is below the level of the fourth cervical vertebrae (C4), the likelihood of serious symptoms occurring from this abnormality is high. Conversely, minor degrees of descent of the brain well above the level of C3, are less likely to have clearly recognized progressive symptoms. Symptoms tend to come from one of three areas of the central nervous system: cerebellum, lower brain stem and spinal cord. The symptom complex is quite different with each of these and appears to be predictable to some degree, based on the patient's age when symptoms are first noticed.

What are the symptoms?
Newborns and young infants with symptoms tend to present with difficulty swallowing that leads to: poor feeding, weak or poor cry, inspiratory stridor (noise on breathing in), frequently exacerbated by crying, arching of the head, and possibly facial weakness, among others. When severe, the symptoms may result in insufficient breathing to maintain life. The symptomatic Chiari malformation is currently the leading cause of death in the Spina Bifida population, particularly in early life.

A child or adolescent may develop stiffness or spasticity of the arms or hands. This stiffness may be so severe that the upper extremities will not work smoothly. If these symptoms are progressive, they may make the upper extremities useless to the patient. Associated with the stiffness may be loss of feeling or sensation in the hands or arms. This loss may not be to all types of sensation, but may be restricted to certain types, such as the loss of pain or loss of temperature sensation.

The third area that can be affected is the cerebellum. This portion of the brain, located in the back of the head, controls balance and coordination. These symptoms are the least likely to occur and may be the most difficult to pinpoint- especially in the younger child.

What tests are done for Arnold Chiari malformation?
There is variability in how medical centers perform testing of brainstem function. With the advent of magnetic resonance imaging (MRI), the evaluation of structural abnormalities of the cervical spine has become greatly simplified. Although other methods are available, MRI provides extremely high-resolution images of the area of concern with little to no risk to the patient. Not only can the position of the brain be obtained, but even cysts or other problems within the spinal cord can be assessed. The MRI scan can also be used to evaluate the flow of cerebrospinal fluid (CSF) around the back part of the brain.

Additional examinations, which test the integrity of the functional aspects of the brain stem and spine, are sometimes helpful. These include a CO 2 ­breathing test that reflects the function of the brain's breathing center, somatosensory evoked response (timing of electrical signals that connect the arms and legs with the brain), brain stem evoked response (ability of the brain to process sounds) and others.

What is the “shunt first” approach to care?
Of primary importance in patients suspected of being symptomatic from their Chiari malformation is to ensure normal intracranial pressure prior to any consideration of intervention for the Chiari malformation. This means that the patient should have her/his shunt evaluated first. The “shunt first” approach cannot be over emphasized. If there is any question concerning shunt function, the system should be surgically inspected. Once it is determined that the intracranial pressure is normal, and that the shunt is working properly, the next question to be answered is whether the patient's symptoms are life threatening and/or progressive. Most patients with radiographic evidence of a Type II Chiari malformation do not need surgery and appear not to be progressive. If serious symptoms are present, consideration is given to surgical intervention.

What about surgery? 
The purpose of the operation is to unroof the cervical spine over the abnormally low brain and to reestablish normal movement of the CSF.

The operation is relatively safe in experienced hands but should be performed by a surgeon especially trained and experienced in operations on children's brain malformations. The technical excellence necessary to perform this operation is high and there is no substitute for significant experience.

The alternative strategy of further conservative care for patients with symptomatic Chiari malformation demands special comment. It is currently believed that the majority of Spina Bifida patients do not demonstrate rapid clinical progression. Opinions vary as to whether an individual patient should have surgery or simply be clinically monitored. Generally, the more the patient's progression is felt to be secondary to their Chiari malformation, the more universal is the acceptance of operative intervention. Serial examinations of the individual are essential in the evaluation of this problem and again, fix the shunt first!

The following are diagrams depicting Chiari II

Information provided by: http://www.spinabifidaassociation.org

Spina Bifida and Hydrocephalus

Hydrocephalus and Shunts in the Person with Spina Bifida

Hydrocephalus means there is a build-up of fluid around the brain. Like a bathtub with the water on and a partially clogged drain, this fluid on the brain can’t drain fast enough. This fluid is made by special brain cells to protect the brain and spinal cord. When there is too much fluid, it can be dangerous.

Most of the time, it is easy for doctors to see that there is too much fluid on the brain. There are special fluid-filled pockets, or cavities, called ventricles, that get too big when there is too much liquid. In some cases, it isn’t as easy to see. There are cases where there may be a little more fluid than normal, and the person has little or no signs of a problem. In these rare cases, neurosurgeons might treat the problem where other types of doctors would not. Part of the reason why this might happen is because experts disagree on when the best time is to treat the problem.

The most common treatment for hydrocephalus is to insert a tube, called a shunt, to drain excess fluid from the head to another place where the body can remove it naturally. Shunts have valves that regulate both the direction and amount of fluid that is drained. All shunts have three parts: 1) a ventricular catheter to reach the area where there is too much fluid, 2) a valve to control flow (there are many types) and 3) tubing to carry the fluid from one place in the body to another.

Types of shunts

The most common type of shunt is the ventriculo-peritoneal (VP) shunt. This shunt drains fluid from the ventricle to the body’s abdomen. Other types that are less common are:

• Ventriculo-atrial (VA) shunts —VA shunts move the fluid to a vein, usually in the neck or under the collarbone;
• Ventriculo-pleural shunts — These shunts move fluid to the chest around the lungs; and
• Ventriculo-gall bladder shunts — These shunts move fluid to the gall bladder.

There are several types of shunt valves. All of them work by controlling the amount of fluid that is drained. Most are made to work automatically when fluid pressure in the head gets too high. Some valves also may have special devices to keep too much fluid from draining.

Experts have not yet learned which type of shunt is best for whom. So neurosurgeons usually pick ones that they think are best. Shunts can be put into one of these places in the head:

• The edge of the soft spot
• Above and behind the ear
• The back of the head

Experts don’t know if one place is better than another. So where to put the shunt also is up to what the surgeon thinks is best.

About 80 percent of people with Spina Bifida have hydrocephalus that needs treatment. Almost all shunts are put in during the first days or weeks after birth. Sometimes the shunt will be inserted at the time of the initial back closure. A child who doesn’t need a shunt by the time they are five months old probably will never need one.

Signs of shunt problems

Signs of hydrocephalus (or of shunt malfunction) in infants may include:

• Rapid head growth;
• Full or tense soft spot (fontanelle);
• Unusual irritability;
• Repeated vomiting;
• Crossed eyes;
• An inability to look up;
• Periods in which the baby stops breathing (called apnea);
• Difficulty swallowing;
• A hoarse or weak cry;
• Difficulty in keeping the infant awake; and
• Any worsening brain function.

A head ultrasound, Computed Tomography (CT) scan or a Magnetic Resonance Imaging (MRI) scan will show this fluid build-up, but a shunt still may not be working right even if it doesn’t show up on a CT or MRI scan. New, long-term treatments using small endoscopes may eliminate the need for a shunt. All patients with hydrocephalus should be seen by a neurosurgeon at least every one to two years.

Most people with Spina Bifida and shunted hydrocephalus will need the shunt for life. The most common problem with shunts is that they can get blocked up, break or come apart. About 40 percent of shunts will fail and need changing (or revision) within one year, 60 percent within five years and 80-85 percent within 10 years. About 20 percent of people with Spina Bifida will need more than one shunt revision.

The signs of shunt problems in people with Spina Bifida are different for each person. This can make it hard for families and health care providers to know what’s going on. The most common sign of a shunt problem is headache. Vomiting and nausea can happen, too, but not always.

Less common signs of a shunt problem include:

• Seizures (either the onset of new seizures or an increase in the frequency of existing seizures);
• A significant change in intellect, school performance or personality;
• Back pain at the Spina Bifida closure site;
• Worsening arm or leg function (increasing weakness or loss of sensation, worsening coordination or balance and/or worsening orthopedic deformities);
• Increasing scoliosis;
• Worsening speech or swallowing difficulties; and
• Changes in bowel or bladder function.

Shunt malfunction can look like any of the signs of a Chiari malformation or spinal cord tethering. In fact, when brain or spinal cord function gets worse and there is no other clear cause, health care providers should check to see if there are shunt problems.

To see if there is a problem with a shunt, health care providers will study images of the brain (usually a CT scan or, for children under one year, a head ultrasound). MRI scans can show shunt problems, but they usually are not necessary, are more expensive and may require sedation or anesthesia. When ventricles start to get too big, it is a strong sign that the shunt is not working right. It is important to know that some people (between 5 and 15 percent) with Spina Bifida may have very few signs or even no visible change in the size of the ventricles when the shunt is not working correctly.

On the other hand, some people with shunted hydrocephalus can develop the slit (or stiff) ventricle syndrome. For these people, too much fluid drainage leads to very small (or slit) ventricles. In these cases, experts think that the walls of the ventricles temporarily block the shunt catheter. This leads to a series of temporary shunt malfunctions without any visible increase in the size of the ventricles. Families and health care providers must pay close attention to a person’s symptoms, especially if they are similar to those that were present with previous shunt problems.

Infections

Infection is a major problem that can happen with shunt operations. Between 5 and 10 percent of people will have this problem. Shunt infections are higher in babies than in older children and adults. Seventy percent of shunt infections happen within the first two months after a shunt operation. Eighty percent of these infections develop within the first six months. Skin bacteria (Staphylococcus epidermidis) are the most common causes of shunt infection. Half of people with shunt infections show signs of a shunt malfunction.

Additional signs of an infection include:

• Fever;
• Neck stiffness;
• Pain;
• Tenderness;
• Redness;
• Drainage from the shunt incisions or tract; and
• Abdominal pain

The diagnosis can be checked by putting a small needle into the valve or a chamber of the shunt and taking out fluid for study.

Infections are commonly treated with antibiotics and with removal and replacement of the shunt system. There are two ways of doing this. The first is to take out the shunt system and then put in a temporary external drainage tube at the same time that antibiotics are given. When the treatment is done, the tube is taken out and a new shunt is put back in. This almost always stops the infection, but it takes two operations. The second (assuming that the shunt is working) is to keep the infected shunt in until the end of the antibiotic treatment. Then the infected shunt is removed and replaced with a new one. The second way only takes one operation, but it does not get rid of the infection as often as the first.

Making decisions

The opinion of a health care provider is very important when working with someone with Spina Bifida and shunted hydrocephalus. When making decisions, here is some helpful advice to families and health care providers:

• Pay attention to gut feelings about shunt problems — these feelings are usually right.
• Be aware that shunt problems can cause many symptoms that may not be obviously shunt-related.
• Be on the lookout for shunt problems, and make sure the shunt is working OK before performing other neurosurgical treatments.

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.

This article is from http://www.spinabifidaassociation.org 

Contributing Editor
Mark S. Dias, MD, FAAP, Pediatric Neurosurgeon

Spina Bifida: It Doesn't Just Affect Humans

Spina bifida is a birth defect involving abnormal prenatal development of one or more of a dog’s vertebrae, which are the bones of the spine. What causes spina bifida isn't well-understood, although there probably is a strong genetic component. Malnutrition and ingestion of toxins during pregnancy may play a role. The vertebrae of dogs with this condition aren't properly fused, which interferes with the function of the spine and the shock-absorbing intervertebral disks. This defect allows the spinal cord to squeeze out through gaps in the spine, causing a variety of symptoms. Owners usually notice signs when affected puppies try to walk, between one and two weeks of age. They show hind-end weakness, poor muscle tone, incontinence, incoordination and abnormal use of their tail. There may be a draining mass or skin dimpling on the dog’s back, over the site of the spinal defect. Fortunately, spina bifida doesn't worsen with time. 

By definition, spina bifida is a congenital disorder, meaning that it is present at birth. Spina bifida is not progressive and does not worsen with time. Signs of this condition usually are first noticed when affected puppies try to walk, between one and two weeks of age. Sometimes, dogs with spina bifida never develop observable symptoms.

As mentioned above, some dogs born with spina bifida never show signs of their condition. Owners of dogs that do develop clinical signs may notice one or more of the following signs:

• Rear limb paresis (weakness)
• Slight to moderate incomplete paralysis of one or both hind legs
• Decreased muscle tone (hind end)
• Muscle atrophy (hind end)
• Ataxia (lack of coordination)
• Fecal incontinence
• Urinary incontinence
• Loss of sensation in the perineal area (the region between the tail and the genitalia)
• Decreased anal sphincter tone
• Decreased ability to use the tail

In rare cases, there may be a draining mass on the dog’s back, towards its tail. Other possible signs are dimpling of the skin and/or abnormal direction of hair growth over the affected area of the animal’s spine.

Dogs at Increased Risk

Spina bifida can occur in any dog of any breed or mixed breed and of either sex. However, it is most commonly seen in English Bulldogs (and in Manx cats), which is why a hereditary component to this condition is suspected.

Diagnostic Procedures

If the spinal cord is unaffected by the defective bone fusion that is called spina bifida, the dog probably will never have symptoms of neurological abnormalities, and the condition actually may never be diagnosed. However, when the spinal cord pushes out through the gap in the abnormal vertebrae, the dog’s rear-end ambulation and control of its urinary and rectal function typically are adversely affected.

A veterinarian should be consulted when a young puppy is not able to walk normally as compared with the abilities of its littermates. The veterinarian will take a history of the puppy’s environment and development and will perform thorough physical and neurological examinations. She may or may not be able to localize which areas of the spine are affected based upon the results of those examinations. Additionally, a miminum data base of blood work (a complete blood count and serum biochemistry profile) and a urinalysis are almost always advised to assess the puppy’s overall health. This is especially important before heavy sedation or general anesthesia is administered to facilitate a more in-depth neurological examination. An electrocardiogram (ECG) to evaluate heart health, and a thyroid panel to evaluate thyroid gland function, may also be recommended.

Spina bifida normally can be definitively diagnosed with simple radiographs (X-rays). Often, it is discovered incidentally when thoracic (chest), abdominal or hip X-rays are taken for some unrelated reason. Radiographs usually will reveal the anatomical defect in the upper parts of affected vertebrae. Computed tomography (CT scan) and magnetic resonance imaging (MRI) are available in some specialty clinics and at veterinary teaching hospitals; these can be helpful to pinpoint the precise location and the extent of bony and soft tissue abnormalities in dogs with spina bifida. A number of other advanced laboratory and electrodiagnostic tests, such as myelography and cerebrospinal fluid analysis, are available if the veterinarian thinks that they may be helpful in a given case.

Special Notes

It is important for owners to remember that spina bifida may or may not cause any discomfort or noticeable signs in affected animals. If the spinal cord does not protrude from the opening in the unfused vertebrae, the disorder may never be diagnosed and may never need any sort of treatment or medical management.

Treatment Options

The management of spine and spinal cord disorders depends upon the nature and severity of the particular abnormality. Treatment is rarely attempted for dogs with spina bifida, mainly because there is no reliable way to “treat” - or fuse - the defect in the abnormal vertebral bones. If a dog has clinical symptoms as a result of a spinal malformation, it means that the spinal cord has already been damaged. At that point, treatment is almost never available or effective.

There is no current treatment for spina bifida in domestic dogs. Attempts to correct the vertebral defects surgically have not been successful, and management with drugs, supplements or dietary modification have no meaningful impact on the condition. Puppies with spina bifida are born with one or more vertebrae that did not develop normally, leaving a gap at the uppermost side of the bone that exposes the spinal cord. The abnormal opening makes the spinal cord vulnerable to trauma and provides a pathway for it to “pop” or squeeze out of its normally protected canal. When this happens, it usually leads to significant neurological damage, because the edges of the gaps in the vertebral bones are rarely smooth and because the spinal cord is extremely fragile. Once the spinal cord is damaged, the adverse consequences are almost always irreversible. Euthanasia is often the most humane alternative for puppies that are severely affected by spina bifida.

However, if the spinal cord of an affected dog does not bulge out through the gap in the upper side of the vertebrae, and if the spinal cord and associated nerves otherwise remain undamaged, the dog may never have noticeable symptoms associated with its condition. In those cases, the animal probably will live a full, happy and pain-free life – at least, without complications of spina bifida - even without any medical treatment for the disorder.

Prognosis

The prognosis and life-expectancy for dogs born with this anatomical spine defect is quite variable. If a dog has significant spinal cord damage caused by the congenital failure of one or more of its vertebrae to fuse, and if it shows severe signs of neurological deficits, its outlook is guarded to grim. These dogs – usually young puppies at the time of diagnosis – often are in a great deal of pain and discomfort. Their inability to walk normally, combined with both fecal and urinary incontinence, probably will prevent them from living an acceptably long or good quality of life. Many caring owners opt for euthanasia when their puppies are so disabled from the disorder. On the other hand, the prognosis for dogs that have no observable symptoms of spina bifida is quite good. They probably should be restricted from engaging in vigorous athletic activities, be well-trained and be encouraged to be calm, in order to enhance their chances of avoiding future spinal cord damage if at all possible.

While spina bifida has been reported in a wide variety of dogs and, there is a high incidence of this condition in young English Bulldogs and Manx Cats. Spina Bifida has also been reported in the German Shepherd, Weimeraner, Pekingese, Dalmatian, Chinese Crested and Swedish Vallhund.

The following links are of two of my favorite animals with Spina Bifida. 

https://www.facebook.com/mickeythebulldog

https://www.facebook.com/dot.the.kitten