Spina Bifida: Fetal Surgery

a new study suggests children with spina bifida may do better, over time, if they have surgery before they are even born.

Researchers from nearly a dozen centers nationwide compared nearly 200 babies with spina bifida, half of whom underwent surgery in utero, while the other half underwent surgery after birth. They found that after one year, those who had surgery before birth were 30 percent less likely to need follow-up surgeries than infants who had surgery after birth.

Spina bifida is often detected 5 to 7 months after pregnancy begins. Surgeons can go inside a pregnant woman's uterus and gently stitch up the open spinal cord of the developing fetus. This stops the leaking of spinal fluid and spares a child brain and nerve damage.

"This is a big breakthrough. For the first time we can show a clear cut benefit, treating a non life threatening malformation by repairing it before birth," said Dr. Scott Adzick, chief of surgery at Children's Hospital of Philadelphia and first author of the study published Wednesday in the New England Journal of Medicine.

Seven out of 10,000 babies in the U.S. are born with spina bifida, and often require immediate surgery after birth to avoid complications, according to the Spina Bifida Association.

Prenatal Surgery Not Without Risks, Experts Said

While prenatal surgery is not a new procedure, many experts wondered whether it would subject both the fetus and the mother to unnecessary added risk. For years, in utero surgery for spina bifida has been done sparingly. And many experts still say that while it now seems like a brighter option, its risks keep it far from becoming a standard procedure.

"This is a highly technical accomplishment," said Dr. William Walsh, a pediatrician at Vanderbilt University Medical Center in Nashville, Tenn., one of the participating study centers. "The complications decreased with experience and expertise of the team."

Researchers reported that women who underwent prenatal surgery had a higher likelihood of premature delivery and uterine tearing at the surgery site during birth.

"The reason we did the trial over the last seven years was my concern that the babies were all being born premature, and the risks of prematurity would outweigh any benefits of the surgery," said Walsh.

Some experts say past studies have overstated the benefits of prenatal surgery.

"Previous reports have erroneously excluded fetuses that aborted or babies that delivered prematurely outside of the fetal surgery hospital, thus making fetal surgery appear as if it was beneficial," said Dr. Elaine St. John, associate professor of pediatrics at the University of Alabama division of neonatology, who was not involved in the study.

"The risk of prematurity is substantial and can't be ignored," said Dr. Noel Tulipan, director of pediatric neurosurgery at Vanderbilt, who has performed over 200 prenatal surgeries. "The challenge going forward is to devise ways to reduce that risks associated with the prenatal repair."

Still, Tulipan said mothers who qualify and who are informed of the risk should have the option since the benefits of prenatal surgery are becoming clearer.

Questions to consider when you choose a center for prenatal spina bifida surgery:

• What is the experience of the individual team members in performing the prenatal spina bifida surgery and in caring for both patients: mothers and babies?
• Does the team include the following members: fetal surgeon; neurosurgeon; maternal-fetal medicine specialist; maternal and fetal anesthesiologist; fetal cardiologist; neonatologist; advanced practice, operating room, labor and delivery and neonatal surgery nurses?
• Similarly, what is the experience of the team working together to perform this procedure and provide all care around it?
• Was the center a participant in the landmark Management of Myelomeningocele Study (MOMS)?
• Has the program published its basic and clinical spina bifida research findings in recognized scientific journals — thereby helping to advance the field’s knowledge?
• Is the pediatric hospital in which the fetal spina bifida surgery is performed ranked among the best in the world?
• Does the program have a dedicated birth unit within the pediatric hospital, staffed and fully equipped to manage the delicate delivery required after fetal surgery for spina bifida?
• Is the newborn intensive care unit experienced in caring for babies born after fetal surgery for spina bifida?
• Are all pediatric subspecialties that may be needed by the baby — such asorthopaedics, urology, physical therapy and radiology — immediately available?
• Does the hospital have a comprehensive program for long-term follow-up of spina bifida patients as they grow?
• ****Information for this entry was gathered from http://www.chop.edu/service/fetal-diagnosis-and-treatment/spina-bifida.html and http://abcnews.go.com/Health/w_ParentingResource/spina-bifida-babies-face-outlook-surgery-womb/story?id=12877182****
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Spina Bifida: Recurrence Prevention

Recurrence Prevention

All women who could become pregnant are at risk for having a pregnancy affected by Spina Bifida. Women who have: A child with Spina Bifida Spina Bifida themselves Already had a pregnancy affected by any neural tube defect are at greater risk of having a child affected by Spina Bifida or another neural tube defect (NTD). These women have an increased risk of having a future affected pregnancy or recurrence. If any of these apply, you should talk with your health care provider about your folic acid needs.Folic Acid Recommendations for ALL Women of Childbearing Age: All women of childbearing age need a daily dose of 400 micrograms (0.4 mg) of synthetic folic acid, in addition to a healthy diet. This amount is available through a multivitamin, available in stores. Additional Recommendations for Women with a Family History of NTDs: A higher prescription dose of 4000 micrograms (4.0 mg) beginning one to three months before pregnancy and in the first trimester of pregnancy, reduces the risk of having another affected pregnancy by up to 70 percent. This amount is available through prescription of four 1.0 mg tablets daily.

What is Spina Bifida?

Spina Bifida is the most common permanently disabling birth defect in the United States.

What is Spina Bifida?
Spina Bifida literally means “split spine.” Spina Bifida happens when a baby is in the womb and the spinal column does not close all of the way. Every day, about eight babies born in the United States have Spina Bifida or a similar birth defect of the brain and spine. 

What causes Spina Bifida?
No one knows for sure. Scientists believe that genetic and environmental factors act together to cause the condition.

What are the different types of Spina Bifida?

Occult Spinal Dysraphism (OSD)
Infants with this have a dimple in their lower back. Because most babies with dimples do not have OSD, a doctor has to check using special tools and tests to be sure. Other signs are red marks, hyperpigmented patches on the back, tufts of hair or small lumps. In OSD, the spinal cord may not grow the right way and can cause serious problems as a child grows up. Infants who might have OSD should be seen by a doctor, who will recommend tests.

Spina Bifida Occulta
It is often called “hidden Spina Bifida” because about 15 percent of healthy people have it and do not know it. Spina Bifida Occulta usually does not cause harm, and has no visible signs. The spinal cord and nerves are usually fine. People find out they have it after having an X-ray of their back. It is considered an incidental finding because the X-Ray is normally done for other reasons. However, in a small group of people with SBO, pain and neurological symptoms may occur. Tethered cord can be an insidious complication that requires investigation by a neurosurgeon.

Meningocele
A meningocele causes part of the spinal cord to come through the spine like a sac that is pushed out. Nerve fluid is in the sac, and there is usually no nerve damage. Individuals with this condition may have minor disabilities.

Myelomeningocele (Meningomyelocele), also called Spina Bifida Cystica
This is the most severe form of Spina Bifida. It happens when parts of the spinal cord and nerves come through the open part of the spine. It causes nerve damage and other disabilities. Seventy to ninety percent of children with this condition also have too much fluid on their brains. This happens because fluid that protects the brain and spinal cord is unable to drain like it should. The fluid builds up, causing pressure and swelling. Without treatment, a person’s head grows too big, and may have brain damage. Children who do not have Spina Bifida can also have this problem, so parents need to check with a doctor.

How is Spina Bifida Treated?
A child with Meningomyelocele usually is operated on within two to three days of birth. This prevents infections and helps save the spinal cord from more damage.

A child with Meningocele usually has it treated with surgery, and more often than not, the child is not paralyzed. Most children with this condition grow up fine, but they should be checked by a doctor because they could have other serious problems, too.

A child with OSD should see a surgeon. Most experts think that surgery is needed early to keep nerves and the brain from becoming more damaged as the child grows.

Spina Bifida Occulta usually does not need to be treated. '

What can you do to prevent Spina Bifida?
Women who are old enough to have babies should take folic acid before and during the first three months of pregnancy. Because half of the pregnancies in the United States are unplanned, the Spina Bifida Association asks women to take a vitamin with 400 mcg (0.4 mg) of folic acid each day during the years of their lives when they are possibly able to have children.

Women who have a child or sibling with Spina Bifida, have had an affected pregnancy or have Spina Bifida themselves should take 4000 mcg (4.0 mg) of folic acid for one to three months before and during the first three months of pregnancy.

What is folic cid?
Folic acid is a vitamin that the body needs to grow and be healthy. It is found in many foods, but the man-made or synthetic form in pills is actually better absorbed by our bodies.

What conditions are associated with Spina Bifida?
Children and young adults with Spina Bifida can have mental and social problems. They also can have problems with walking and getting around or going to the bathroom, latex allergy, obesity, skin breakdown, gastrointestinal disorders, learning disabilities, depression, tendonitis and sexual issues.

What physical limitations exist?
People with Spina Bifida must learn how to get around on their own without help, by using things like crutches, braces or wheelchairs. With help, it also is possible for children to learn how to go to the bathroom on their own. Doctors, nurses, teachers and parents should know what a child can and cannot do so they can help the child (within the limits of safety and health) be independent, play with kids that are not disabled and to take care of him or herself.

Can Spina Bifida be detected before birth?
Yes. There are three tests*. 

1. A blood test during the 16th to 18th weeks of pregnancy. This is called the alpha-fetoprotein (AFP screening test). This test is higher in about 75–80 % of women who have a fetus with Spina Bifida.
2. An ultrasound of the fetus. This is also called a sonogram and can show signs of Spina Bifida such as the open spine.
3. A test where a small amount of the fluid from the womb is taken through a thin needle. This is called maternal amniocentesis and can be used to look at protein levels.

*Parents should know that no medical test is perfect, and these tests are not always right.

Can children with Spina Bifida grow up and live full lives?
Yes. With help, children with Spina Bifida can lead full lives. Most do well in school, and many play in sports. Because of today’s medicine, about 90 percent of babies born with Spina Bifida now live to be adults, about 80 percent have normal intelligence and about 75 percent play sports and do other fun activities.

How is Spina Bifida managed?
As type and level of severity differ among people with Spina Bifida, each person with the condition faces different challenges and may require different treatments.

The best way to manage Spina Bifida is with a team approach. Members of the team may include neurosurgeons, urologists, orthopedists, physical and occupational therapists, orthotists, psychologists and medical social workers.
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.

***The above article was Contributed by  Gregory S. Liptak, MD, MPH for http://www.spinabifidaassociation.org

Spina Bifida: Notable People Born With It

I wanted to show that Spina Bifida does not discriminate in any way. So here is a list of some of the most notable celebrities from the past and in the present that have been affected by Spina Bifida. 

• Tanni Grey-Thompson - Welsh Paralympic athlete, member of British House of Lords and television presenter. She is considered to be one of the most successful disabled athletes in the UK. She graduated from Loughborough University in 1991 with a BA (Hons) degree in Politics and Social Administration. Her autobiography Seize the Day was published by Hodder and Stoughton in 2001.

• Blaine Harrison - lead singer, keyboards, rhythm guitarist and former drummer of the British band Mystery Jets.  In 2009 He became a patron of the Attitude is Everything Charity, who work with live music venues in the UK to make gigs accessible for people with disabilities. Blaine was quoted in an interview with the BBC saying, "When we first got together as a band we decided that we wouldn't make my disability an issue, the same way we didn't make a big deal of my dad being in the band. We weren't interested in gimmicks... We wanted people to read our lyrics and listen to our music." Blaine has said playing live as someone with a physical impairment is sometimes hard work, seeing as many modern facilities still do not have the means to cater for disabled people. He has made it clear that Mystery Jets will only play in venues who have made an effort to cater to people with disabilities and refrain from playing in small, crowded pubs.

• Rene Kirby - US actor in films such as Shallow Hal and Stuck on You and the American Television series Carnivàle in a 2005 episode. 

• John Mellencamp -  American rock singer-songwriter, musician, painter and occasional actor known for his catchy, populist brand of heartland rock which emphasizes traditional instrumentation. He has sold over 40 million albums worldwide and has amassed 22 Top 40 hits in the United States. In addition, he holds the record for the most tracks by a solo artist to hit number-one on the Hot Mainstream Rock Tracks chart, with seven, and has been nominated for 13 Grammy Awards, winning one. His latest album, No Better Than This, was released on August 17, 2010 to widespread critical acclaim.

• Karin Muraszko - chair of Department of Neurosurgery at University of Michigan, first woman appointed to such a position in the US. Director of the American Board of Neurological Surgery.She is the medical director of "Project Shunt", the neurosurgery component of an annual medical mission by the Michigan, Ohio, chapter of the medical charity "Healing the Children" to Guatemala, which has one of the highest incidences of spina bifida in the world.In 2005 the Association of Women Surgeons awarded her the Nina Starr Braunwald Award "in recognition of outstanding contributions to the advancement of women in surgery".

• Jeffrey Tate - British conductor. as of the spring of 2008, appointed as the next chief conductor of the Hamburg Symphony Orchestra

• Hank Williams - (September 17, 1923 – January 1, 1953) US country music singer-songwriter.  Regarded as one of the most significant country music artists, Williams recorded 35 singles (five released posthumously) that would place in the Top 10 of the Billboard Country & Western Best Sellers chart, including 11 that ranked number one. he developed a serious problem with alcohol, morphine, and other painkillers prescribed for him to ease the severe back pain caused by his spina bifida. Father to Hank Williams, Jr who is also a US country music singer-songwriter. 

• Lucinda Williams -  American rock, folk, blues, country music singer-songwriter. Williams has received three awards from 15 nominations.

• Miller Williams - US poet, as well as a translator and editor. He has authored over 25 books and won several awards for his poetry. His accomplishments have been chronicled in Arkansas Biography. He is perhaps best known for reading a poem at President Clinton's 1997 inauguration. One of his best-known poems is "The Shrinking Lonesome Sestina."

• Chandre Oram - Man who has a tail due to spina bifida. Chandre Oram is an Indian tea estate worker who lives in Alipurduar district of Jalpaiguri, West Bengal. He is famous for having a 13 inch long tail, which has made him an object of devotion to many, who believe him to be an incarnation of Hanuman, a Hindu deity that is a human of vanara clan whose emblem is monkey.

• George Schapell - US country music singer. Conjoined twin with Lori Schapell, Together they are the oldest confirmed conjoined twins. Lori was not born with Spina Bifida.  George has performed as a country singer. In 2007, George, who was at that time known as Reba Schappell, stated that although assigned female at birth, he identified as male and changed his name to George. George Schappell has designed support equipment for people with physical handicaps, including a specialized wheelchair and a mobility aid for dogs.

• Jean Driscoll - American wheelchair racer. She won the women's wheelchair division of the Boston Marathon eight times, more than any other female athlete in any division. Her wins in Boston included seven consecutive first place finishes from 1990 to 1996. Driscoll participated in four Summer Paralympic Games, winning a total of five gold, three silver, and four bronze medals in events ranging from 200 meters to the marathon.

• Aaron Fotheringham - American extreme wheelchair athlete who performs tricks adapted from skateboarding and BMX. Fotheringham calls his activity ‘WCMX’. He is the first person to successfully perform a backflip in a wheelchair at the age of 14, and a double backflip at the age of 18. He performs many other tricks in his wheelchair including 180 degree 'aerials', one-wheeled spins and rail grinds. He plans to fuse the back flip with the 180 aerial into what is known as a ‘flair'.
  In 2010 Aaron joined the Nitro Circus Live tour, an action sports road show that tours Australia, New Zealand, Europe and the United States. On the tour Aaron performed on a fifty-foot ramp-to-ramp jump doing backflips, double backflip attempts and on February 9th, 2011 in New Zealand, the world's first wheelchair frontflip.

• Robert M. Hensel - American Guinness World Record Holder for the longest non-stop wheelie in a wheelchair, covering a distance of just over 6 miles. Disabilities activist and poet. In 2000, realizing the need to focus more on one's abilities and less on their disabilities, Hensel sought to have a week designated that would bring to light the many talents and accomplishments being made by individuals with disabilities. Due to his efforts, Oswego County passed a motion that year recognizing Oct. 1-7 as Beyond Limitations week.

• CNN Co-anchor Judy Woodruff - has a son with spina bifida. She has been instrumental in the annual SBAA Celebrity Roast which raises funds for the Spina Bifida Association of America.

• Buddy Winnett - former horse jockey and trainer who is legally blind due to macular degeneration. Born in West Virginia to a large family, Winnett was diagnosed with spina bifida, and wasn't expected to live past the age of 6.

• Chicago Mayor Richard M. Daley - had a son named Kevin who had spina bifida. Kevin died in 1981.

• Quarterback Elvis Grbac - has a young son who has spina bifida. He was the quarterback of the National Football League's San Francisco 49ers.

• Poet Jay Bradford Fowler - 
   was an award-winning poet who was the author of 17 books of verse. He was born with spina bifida, and had been treated for degenerative arthritis throughout the 1990s. He had undergone at least 16 major operations over the years and had been bedridden the past eight years.
  The first of his 17 books of poetry, "Writing Down the Light," was published in 1987. A book of his collected poems, "Caged Angel," was published in 1997. His last book, "Outrageous Asylum, Sonnets from the Nursing Home," appeared in 1998. A final volume of his work is to be published this year.

• Author Paul Monette
   was the older of two boys. His brother, Robert, was born with spina bifida. Monette became Robert's protector and champion. He also decided he would need to live an exemplary life to compensate for his brother's illness.
  "Paul Monette: The Brink of Summer's End" is a poignant, illuminating documentary on Paul Monette, the National Book Award-winning author, who wrote with eloquence and fire about the devastation of homophobia and AIDS, the disease that left him twice a widower and ended his own life in 1995 at age 49.

• Sound Designer Jim LeBrecht - 
  born with spina bifida. He is also the co-author (with composer Deena Kaye) of Sound and Music for the Theatre: the Art and Technique of Design, and has been a visiting professor at Yale University. LeBrecht has won numerous awards, including the San Francisco Bay Area Drama Critics' Circle Award for Outstanding Sound Design, which might as well be renamed in his honor, since he's won it five times.

• Journalist Gilbert M. Gaul - Gil is a 1979 Pulitzer Prize winner for Investigative Reporting and has written for the Philadelphia Inquirer and Washington Post. He has a son, Cary, who has spina bifida. Gil profiled his son's early years in the book "Giant Steps" which is currently out-of-print but can sometimes be found at used book stores and online.

• Actor Boris Kodjoe, whose own daughter suffers from spina bifida, is all smiles at the charity event. Austrian-born actor and former fashion model who works primarily in the United States. He is perhaps best known for his role as courier-turned-sports agent Damon Carter on the Showtime television drama series Soul Food and for his role as David Taylor in the film The Gospel. Additionally, he starred as Steven Bloom in the cancelled 2010 NBC action/drama series Undercovers, and as Luther West in the films Resident Evil: Afterlife and Resident Evil: Retribution.

Spina Bifida: Fetal Surgery

*** TO ENSURE ACCURACY ON THE SUBJECT, THE FOLLOWING INFORMATION WAS TAKEN DIRECTLY FROM WIKIPEDIA. ***

Fetal surgery research

1980 - Fetal surgical techniques using animal models were first developed at the University of California, San Francisco by Dr. Michael R. Harrison, Dr. N. Scott Adzick and research colleagues.

1994 - A surgical model that simulates the human disease is the fetal lamb model of myelomeningocele (MMC) introduced by Meuli and Adzick in 1994. The MMC-like defect was surgically created at 75 days of gestation (term 145 to 150 days) by a lumbo-sacral laminectomy. Approximately 3 weeks after creation of the defect a reversed latissimus dorsi flap was used to cover the exposed neural placode and the animals were delivered by cesarean section just prior term. Human MMC-like lesions with similar neurological deficit were found in the control newborn lambs. In contrast, animals that underwent closure had near-normal neurological function and well-preserved cytoarchitecture of the covered spinal cord on histopathological examination. Despite mild paraparesis, they were able to stand, walk, perform demanding motor test and demonstrated no signs of incontinence. Furthermore, sensory function of the hind limbs was present clinically and confirmed electrophysiologically. Further studies showed that this model, when combined with a lumbar spinal cord myelotomy leads to the hindbrain herniation characteristic of the Chiari II malformation and that in utero surgery restores normal hindbrain anatomy by stopping the leak of cerebrospinal fluid through the myelomeningocele lesion.

Surgeons at Vanderbilt University, led by Dr. Joseph Bruner, attempted to close spina bifida in 4 human fetuses using a skin graft from the mother using a laparoscope. Four cases were performed before stopping the procedure - two of the four fetuses died.

1998 - Dr. N. Scott Adzick and team at The Children's Hospital of Philadelphia performed open fetal surgery for spina bifida in an early gestation fetus (22 week gestation fetus) with a successful outcome.

Surgeons at Vanderbilt University, led by Dr. Noel Tulipan, made an incision in the mother's uterus to obtain better exposure to fetuses of 28 to 30 weeks' gestation. All 4 fetuses were born premature but with evidence of reversal of their Chiari II malformation. Only 2 of the 4 required ventricular shunts after birth. Fetal surgery after 25 weeks has not shown benefit in subsequent studies.

Subsequently, 4 medical centers conducted 253 open spina bifida repairs prior to the MOMs trial. The outcomes were mixed, and the only comparison groups were other children who had not undergone repair after birth in the past.

MOMS trial

Management of Myelomeningocele Study (MOMS) is a phase III clinical trial to evaluate the safety and efficacy of fetal surgery to close a myelomeningocele. This involves surgically opening the pregnant mother's abdomen anduterus to operate on the fetus. This route of access to the fetus is called open fetal surgery. The exposed fetal spinal cord is covered in layers with surrounding fetal tissue at mid-gestation (19–25 weeks) to protect it from further damage caused by prolonged exposure to amniotic fluid. The fetal surgery may decrease some of the damaging effects of the spina bifida, but at some risk to both the fetus and the pregnant woman.

The MOMS trial was closed for efficacy in December 2010 based on comparing outcomes after prenatal and postnatal repair in 183 patients - 77 patients were treated at The Children’s Hospital of Philadelphia, 54 at Vanderbilt University and 52 at The University of California San Francisco. Unfortunately the study failed to address the possibility that some of the benefit of surgery to central nervous system function in the intervention group may have been caused by early delivery from the intrauterine environment. This issue casts some doubt on the studies findings. A case controlled comparison of intervention vs conservative management would have been unethical because of the exposure of infants within a control group to the adversity of premature delivery.

The trial concluded that the outcomes after prenatal spina bifida treatment are improved to the degree that the benefits of the surgery outweigh the maternal risks. This conclusion requires a value judgment on the relative value of fetal and maternal outcomes on which opinion is still divided. Results were reported in the New England Journal of Medicine by Adzick et al.

To be specific, the study found that prenatal repair resulted in:

• Reversal of the hindbrain herniation component of the Chiari II malformation
• Reduced need for ventricular shunting (a procedure in which a thin tube is introduced into the brain’s ventricles to drain fluid and relieve hydrocephalus)
• Reduced incidence or severity of potentially devastating neurologic effects caused by the spine’s exposure to amniotic fluid, such as impaired motor function

In Europe, open fetal surgery for spina bifida was introduced in 2003 by the Polish pediatric surgeon Janusz Bohosiewicz in Katowice. Through the end of 2011, more than 40 fetuses with spina bifida were operated at this center.

Endoscopic fetal surgery

In contrast to the open fetal operative approach performed in the MOMS trial, a minimally-invasive fetoscopic approach has been developed by the German pediatrician Thomas Kohl of the German Center for Fetal Surgery & Minimally-Invasive Therapy at the University of Giessen, Germany.

This approach under general materno-fetal anesthesia uses three trocars (small tubes) with an external diameter of 5 mm that are directly placed through the maternal abdominal wall into the uterine cavity under ultrasound guidance. Following intrauterine access, part of the amniotic fluid is removed and the uterus is insufflated with carbon dioxide (this technique provides superior visualization of fetoscopic spina bifida closure, is called PACI (partial amniotic fluid insufflation), and has been safe for mothers and fetuses alike in over 70 procedures on human fetuses). After fetal posturing, the neural cord is freed from pathological adhesions and covered with patch material. Watertight closure is demonstrated by intraoperative bulging of the patch. Accordingly, reversal of hindbrain herniation can be documented within days after most procedures.

The observations in mothers and their fetuses that were operated over the past two and a half years by the matured minimally-invasive approach showed the following results: Compared to the open fetal surgery technique, fetoscopic repair of myelomeningocele results in far less surgical trauma to the mother, as large incisions of her abdomen and uterus are not required. In contrast, the initial punctures have a diameter of 1.2 mm only. As a result, thinning of the uterine wall or dehisscence which have been among the most worrisome and criticized complications after the open operative approach do not occur following minimally-invasive fetoscopic closure of spina bifida aperta. The risks of maternal chorioamniotis or fetal death as a result of the fetoscopic procedure run below 5%. Operated women are discharged home from hospital one week after the procedure. There is no need for chronic administration oftocolytic agents since postoperative uterine contractions are barely ever observed. The current cost of the entire fetoscopic procedure including hospital stay, drugs, perioperative clinical, ECG, ultrasound and MRI-examinations is approximately €6,000.

In a cohort of 20 infants that underwent fetoscopic surgery on the lesion between July 2010 and December 2011 and were studied during the first six months of life, reversal of hindbrain herniation was observed in 18 (90%) and shunt insertion was required in only eight (40%). Normal to near normal leg function was observed in about two thirds of the infants. An abnormal foot position at birth was observed in only two. The fetuses that were operated at a mean of 24 weeks of gestation were born at a mean gestational age at delivery of about 33 weeks of gestation. In contrast to open fetal surgery, leukomalacia has not been observed in neonates following the fetoscopic approach. Moreover, following the fetoscopic approach, postnatal spina bifida surgery can now be avoided in most patients.

In 2012, these encouraging results of the fetoscopic approach were presented at various national and international meetings, among them at the 1st European Symposium “Fetal Surgery for Spina bifida“ in April 2012 in Giessen, at the 15th Congress of the German Society for Prenatal Medicine and Obstetrics in May 2012 in Bonn, at the World Congress of the Fetal Medicine Foundation in June 2012 and at the World Congress of the International Society of Obstetrics and Gynecology (ISUOG) in Copenhagen in September 2012, and published in abstract form. In contrast to the low maternal and fetal complication rates that can be achieved by the current fetoscopic approach, its clinical introduction was affected by technical difficulties and a number of adverse fetal outcomes: Three of the first 19 procedures could not be completed, three fetuses died, and the mean gestational age at delivery was 29 weeks of gestation. As a result, the approach was heavily criticized by the independent authors of a controlled study about this cohort and deemed unethical by others. Yet, even in these earliest cases statistically significant better motor and sensory function of the lower extremities as well as a statistically significantly lower shunt rate could be demonstrated in contrast to the control patients that underwent standard postnatal procedures.

In conclusion, both the open and minimally-invasive fetoscopic fetal surgical procedures offer the chance to improve the postnatal prognosis and quality of life of patients affected by spina bifida. Fetuses that benefit the most seem those with higher lesions, normally appearing leg movements and foot position, and only a mild dilation of the lateral ventricles despite signs of hindbrain herniation. Vice versa, it seems unlikely that leg, bladder or bowel functions that were lost prior to the procedure can be regained by either approach Furthermore, fetuses that already exhibit a moderate degree of hydrocephalus at the time of fetal surgery will require postnatal cerebrospinal fluid shunting anyway. Further studies are required in order to assess the value of fetal surgery on postnatal bladder-, bowel- and sexual function.

As in patients who undergo standard postnatal spina bifida closure, deterioration of neurological function from tethered cord, surgical re-interventions, complications of hydrocephalus and Chiari II-treatment must be expected in some patients after open and minimally-invasive fetal surgery, regardless of the quality of their neurological status in the first years of life.

More on Spina Bifida. October is Spina Bifida Awareness Month

I understand the importance of October being Breast Cancer Awareness Month and advocating for breast self exams, mammograms and such. I really do. So, please don't mistake that. But I do wish that more people were aware that October is also Spina Bifida Awareness Month. There is no treatment or cure to get rid of it. Once you're born with it, you have it for the rest of your life. And it affects your life and that of your loved ones in a way that you can't imagine unless you do have it yourself. Its a lifetime financial cost of $532,000-$1,000,000....much of which hasn't always been paid by insurances because of the "pre-existing condition clause". It's urinary and fecal incontinence for most. It's pressure sores and shunts. It's deadly infections. It's a lifetime of surgeries, specialists and doctors' visits. It's deadly allergic reactions to latex for most. It's kids suffering ostracism from their schoolmates for "being different". Its mental and social issues for some. Its a minimum of 166,000 living with this disability in the US alone. Its abortions because moms cant handle dealing with these issues. In fact, according to a recent Wayne State University study of several countries, the study found that 64% of the children diagnosed in utero with Spina Bifida were aborted. 64%!!! There is no KNOWN cause. There are only hypothetical guesses as to why it occurs. It's an average of 8 babies per day in the US born with Spina Bifida or a similar defect of the brain or spine. Its that Hispanic mothers are 1-1/2 times more likely to give birth to a child with Spina Bifida than non-Hispanic Caucasians. It's the most common permanently disabling birth defect in the US--its more common than Muscular Dystrophy, Cystic Fibrosis and Cerebral Palsy(which are all well known and advocated for disabilities) combined. Currently, there are about 3.5 MILLION people with Spina Bifida in the world. Folic acid MIGHT HELP prevent it by as much as 75%.

So----what is "it"????? Spina Bifida is a neural tube defect that occurs when the spinal structure fails to form and close properly. This occurs in utero during the first 2-4 weeks after conception, before most women even know they’re pregnant. There are 3 main types of Spina Bifida: Occulta, Meningocele(pronounced like "my ninja seal"), and Myelomeningocele(pronounced like "Milo my ninja seal"). Occulta and Meningocele are a less severe form of Spina Bifida. People with these types of Spina Bifida may have minor disabilities or no problems at all. Myelomeningocele is the most common and severe form of Spina Bifida. Most people that have this form are almost always in a wheelchair. It is also the form of SB I was born with. This occurs when the meninges of the spine and spinal nerves actually come through the opening in the spine. This form of Spina Bifida can cause the most nerve damage resulting in disabilities. Spina Bifida isn't a birth defect of the poor, the malnourished, or the underprivileged. It knows no geographical or socioeconomic boundaries. The country with the highest rate of SB occurring is Ireland.

PLEASE EDUCATE YOURSELVES!! If you'd like, you can ask me anything you'd like to know since the internet's info can be a bit overwhelming--and even incorrect. So when you're "thinking pink" for Breast Cancer Awareness, don't forget to "think teal" or "think yellow" for Spina Bifida Awareness too. The next kid born with Spina Bifida could be your own.

The Treatment of Spina Bifida by a New Method

Spina Bifida Awareness Month Begins Today

October is Spina Bifida Awareness Month. So I wanted to share my story again. Read on: 

I was born with myelomeningocele (pronounced "Milo My Ninja Seal") Spina Bifida at the L1, L2 region of my spine. You can read all about the different forms of SB here. This means that the Spina Bifida lesion on my spine was in the lumbar region at the 1st and 2nd vertebrae of the lumbar region. I am paralyzed from my knees down. I use to walk with leg braces and crutches until they started to cause pressure sores, or blisters, on my feet. So I decided at the age of 12-13 to just use my wheelchair permanently. I don't regret that at all. For me, it is what it is. Braces or wheelchair---for me, it doesn't matter how I get around as long as I do. And, truthfully, the wheelchair gives me a certain independence and speed that leg braces just could not do. 

In 1975, they didn't really have the technology they do today with ultrasounds and such. So no one knew that I would be born with SB until I was actually born. I still moved around inside mom's belly just like my brothers did before me. They don't have SB, by the way. At birth, the lesion in my spine was repaired as much as possible and my back was sewn shut. I developed hydrocephalus when I was 10 days old and the VP Shunt was put in place on the 11th day. It was revised again before I was a yr old and hasn't been revised or used since. I still have the same shunt today and it's still not in use. In all, I have had over 50 operations in my life. Literally from head(the shunt) to toe(to straighten my big toe) and everywhere in between. However, I don't have that many scars. Some of the surgeries were redone. Because of so many hospital stays and doctor visits, I am severely allergic to ALL latex products. 

I went to school just like everyone else and had someone assist me in getting to my classes with my books until my senior year when I chose to go at it alone. I never had any of the comprehension or learning issues that are sometimes common with SB. In fact, I was an A & B honor roll student with the occasional C in math. No one ever ostracized me or teased me because of my disability(that I know of, anyway!) and I always had a ton of friends...still do to this day. Socializing is something that has always come very easy for me. 

Now for all the yucky personal stuff related to Spina Bifida. YES!!! I CAN have sex. I have been told that women with SB have multiple orgasms easier and more frequently than women without SB. I don't know if it's true. I only know of my experiences. All my life, I have dealt with the urinary and fecal incontinence that is so common with SB. Urinary incontinence is most often controlled by using a urinary catheter and inserting it into the bladder to empty it of the urine. I do this every 4 or 5 hours. Fecal incontinence...Ugh.....no one likes talking about shit, right???? Oh well, Its a must in this case. Yes, I suffer through it as well. People with SB often lack the ability of being able to "hold it" when they have a bowel movement. So "accidents" do occur. Yes, it can be embarrassing and it can fuck up your entire day....but Shit Happens...You deal with it and move on. I am, however, very careful about what I eat when I'm around friends and family that have not dealt with this issue to spare them of it. I do the same if I'm out somewhere and they don't have wheelchair accessible bathrooms. 

Now for all the important information and statistics to tell you exactly what SB is now that you know how it relates to me. There is no treatment or cure to get rid of it. Once you're born with it, you have it for the rest of your life. And it affects your life and that of your loved ones in a way that you can't imagine unless you do have it yourself. Its a lifetime financial cost of $532,000-$1,000,000....much of which hasn't always been paid by insurances because of the "pre-existing condition clause" that use to be in effect before Obamacare. It's urinary and fecal incontinence for most. It's pressure sores and shunts. It's deadly infections. It's a lifetime of surgeries, specialists and doctors' visits. It's deadly allergic reactions to latex for most. It's kids suffering ostracism from their schoolmates for "being different". Its mental and social issues for some. Its a minimum of 166,000 living with this disability in the US alone. Its abortions because moms cant handle dealing with these issues. In fact, according to a recent Wayne State University study of several countries, the study found that 64% of the children diagnosed in utero with Spina Bifida were aborted. 64%!!! There is no KNOWN cause. There are only hypothetical guesses as to why it occurs. It's an average of 8 babies per day in the US born with Spina Bifida or a similar defect of the brain or spine. Its that Hispanic mothers are 1-1/2 times more likely to give birth to a child with Spina Bifida than non-Hispanic Caucasians. It's the most common permanently disabling birth defect in the US--its more common than Muscular Dystrophy, Cystic Fibrosis and Cerebral Palsy(which are all well known and advocated for disabilities) combined. Currently, there are about 3.5 MILLION people with Spina Bifida in the world. Folic acid MIGHT HELP prevent it by as much as 75%. But there is no guarantee. 

So----what is "it"????? Spina Bifida is a neural tube defect that occurs when the spinal structure fails to form and close properly. This occurs in utero during the first 2-4 weeks after conception, before most women even know they’re pregnant. There are 3 main types of Spina Bifida: Occulta, Meningocele(pronounced like "my ninja seal"), and Myelomeningocele(pronounced like "Milo my ninja seal"). Occulta and Meningocele are a less severe form of Spina Bifida. People with these types of Spina Bifida may have minor disabilities or no problems at all. Myelomeningocele is the most common and severe form of Spina Bifida. Most people that have this form are almost always in a wheelchair. It is also the form of SB I was born with. This occurs when the meninges of the spine and spinal nerves actually come through the opening in the spine. This form of Spina Bifida can cause the most nerve damage resulting in disabilities. Spina Bifida isn't a birth defect of the poor, the malnourished, or the underprivileged. It knows no geographical or socioeconomic boundaries. The country with the highest rate of SB occurring is Ireland. Anyone with SB has the chance to live long lives, just like anyone else.