Spina Bifida (Myelodysplasia)

             Spina bifida (Myelodysplasia) is a birth defect that affects the lower back and, sometimes, the spinal cord. Spina bifida malformations fall into three categories: spina bifida occulta, spina bifida cystica with meningocele, and spina bifida cystica with myelomeningocele. The most common location of the malformations is the lumbar and sacral areas. Myelomeningocele is the most significant and common form, and this leads to disability in most affected individuals. The terms spina bifida and myelomeningocele are usually used interchangeably.

Classification
1. Spina bifida occulta
In this mildest form, there are usually no symptoms. Affected individuals have a small defect or gap in one or more of the vertebrae of the spine. A few have a dimple, hairy patch, dark spot or swelling over the affected area. The spinal cord and nerves usually are normal, and most affected individuals need no treatment.

2. Meningocele
In this rarest form, a cyst or fluid-filled sac pokes through the open part of the spine. The sac contains the membranes that protect the spinal cord, but not the spinal nerves. The cyst is removed by surgery, usually allowing for normal development.

3. Myelomeningocele
In this most severe form, the cyst holds both the membranes and nerve roots of the spinal cord and, often, the cord itself. Or there may be a fully exposed section of the spinal cord and nerves without a cyst. Affected babies are at high risk of infection until the back is closed surgically, although antibiotic treatment may offer temporary protection. In spite of surgery, affected babies have some degree of leg paralysis and bladder- and bowel-control problems. In general, the higher the cyst on the back, the more severe the paralysis.

Causes
- The causes of spina bifida are not completely understood.
- Scientists believe that both genetic and environmental factors act together to cause this and other NTDs.
- However, 95 percent of babies with spina bifida and other NTDs are born to parents with no family history of these disorders.

Signs and Symptoms
1. Trouble swallowing, which can lead to choking
2. Hoarseness
3. Seizure disorders
4. Leg weakness and paralysis
5. Orthopedic abnormalities (i.e., club foot, hip dislocation, scoliosis)
6. Bladder and bowel control problems
7. Urinary tract infections
8. Poor renal function
9. Pressure sores and skin irritations
10. Abnormal eye movement

Many children who have severe spina bifida develop an allergy to latex, a natural rubber product that is used to make objects such as toys and health care supplies. Doctors do not know why many children with spina bifida develop this allergy. Take extra care to keep your child who has spina bifida away from products that contain latex.

Treatment
1. Surgery to repair the spine :
Surgery to repair the spine usually takes place within 48 hours of birth.
During surgery, the surgeon will place the spinal cord and any exposed tissues or nerves back into your baby’s body. Any gap in the vertebrae (the bones that make up the spine) is closed and the spinal cord is sealed with muscle and skin.
2. Treating hydrocephalus :
If your child has hydrocephalus (a build up of cerebrospinal fluid (CSF) on the brain), it will need to be treated with surgery. The surgeon will implant a thin tube called a shunt to drain away excess fluid to another part of the body, usually the abdomen (tummy).
3. Physiotherapy :
Physiotherapy is one of the most important ways of helping your child manage their condition so they're as independent as possible. For spina bifida, the main aim of physiotherapy is to promote movement and independent mobility to prevent the leg muscles from weakening.
4. Occupational therapy :
The aim of occupational therapy is to allow your child to develop skills in everyday activities. An occupational therapist can identify problem areas in everyday life, such as getting dressed, and will help work out practical solutions. This can be by encouraging certain movements or providing equipment, such as handrails, to make the activity easier.
5. Assistive technology :
Assistive technology can help children with spina bifida gain more independence and control over their symptoms.
6. Treating urinary incontinence :
It is important your child's bladder function is assessed by a urologist (a specialist in treating conditions affecting the urinary system) in the early weeks following birth.
- Clean intermittent catheterisation
- Antimuscarinics
7. Treating bowel incontinence :
Children with urinary incontinence often have bowel incontinence as well. Some cases of bowel incontinence can be treated using a combination of dietary changes and toilet training techniques.
- Anal irrigation
- Antegrade continence enema (ACE)
- Colostomy and ileostomy

Prevention
1. A B-vitamin called folic acid can help prevent spina bifida and other NTDs. Studies show that if all women in the United States took the recommended amount of folic acid before and during early pregnancy, up to 70 percent of NTDs could be prevented. It is important for a woman to have enough folic acid in her system before pregnancy and during the early weeks of pregnancy, before the neural tube closes.
2. The March of Dimes recommends that all women of childbearing age take a multivitamin with 400 micrograms of folic acid every day before pregnancy and during early pregnancy. However, a woman should not take more than 1,000 micrograms (or 1 milligram) without her provider’s advice.
3. Healthy eating includes eating foods that are fortified with folic acid and foods that contain folate, the natural form of folic acid that is found in foods. Many grain products in the United States are fortified with folic acid. This means that a synthetic (manufactured) form of folic acid is added to them. Enriched flour, rice, pasta, bread and cereals are examples of fortified grain products. (A woman can check the label to see if a product is enriched.) Folate-rich foods include leafy green vegetables, beans and orange juice.
4. Women who already have had a baby with spina bifida or another NTD, as well as women who have spina bifida, diabetes or seizure disorders, should consult their health care provider before another pregnancy about the amount of folic acid to take. Studies have shown that taking a ten-fold larger dose of folic acid daily (4 milligrams), beginning at least 1 month before pregnancy and in the first trimester of pregnancy, reduces the risk of having another affected pregnancy by about 70 percent.