HOW TO AVOID A LIFETIME OF SPINAL COMPLICATIONS

Mothers at a health camp. All women who are hoping to conceive should get adequate amounts of folic acid. A deficiency can lead to neural tube birth defects, which affect the brain and spinal cord. Photo Dominic Bukenya.

What you need to know:

There are a number of conditions which can affect the spinal cord and lead to lifelong defects. Christine Katende and Beatrice Nakibuuka explore the risks and complications.

Divine Angel Atuhwere is 11 years old but she has never walked. Her mother Peace Kwikiriza, a single parent of two, says her child had a delay in development.
“I realised my child had an abnormality at birth. She had a swelling on her back and her forehead. Although she looked healthy, the baby produced a sound which scared me and I lost all the joy of having a newborn,” Kwikiriza narrates.
Dr Martin Kasendwa, a medical officer at Mildmay Hospital defines spina bifida as a neurological disorder related to malformations of the spinal cord.

This condition can be be detected during pregnancy through an ultrasound scan. “Much as the illness can be detected during pregnancy, it can also be identified at birth during the post-delivery examination of the baby. At this time, the opening can be closed immediately through surgery to reduce the risk of acquiring infections that may affect the brain and the spinal cord,” he says.

Causes of spina bifida
1. The most predominant cause of illness is the inadequate folic acid in the body mainly during the first 12 weeks of pregnancy, the time body organs such as the heart, lungs, spinal cord, and kidney develop.
“Folic acid is a vitamin that the body needs to grow and be healthy. It is found in many foods, but the manmade or synthetic form in tablets is actually better absorbed by our bodies,” explains the doctor.

“Folic acid deficiency has been clearly associated with open spinal dysraphism (myelomeningocele). Therefore, women should start taking folic acid as soon as they anticipate conception,” he adds.
2. Dr Kasendwa however, notes it is not only expectant mothers lacking folic acid who will end up with deformed babies but also diabetic mothers and those take peripatetic drugs.
3. Maternal diabetes has been associated with increased incidence of the neural tube defects. The incidence in the general population is 0.05 to 0.1 per thousand live births, according to the doctor.

4. Maternal exposure to certain medications such as valproate, carbamazepine, methotrexate has also been associated with spinal dysraphism in the foetus. The effects of these anticonvulsant drugs may be mediated through alterations in folic acid metabolism. It is therefore vital for an expectant mother to desist from self -medication and ensure that medication during pregnancy is prescribed by a doctor. “Open or closed spinal dysraphism occurs at a frequency of 0.5 to eight cases per 1000 live births. The incidence varies substantially across populations, in patterns that follow nutritional, geographic, and ethnic factors,” he notes.

Abnormalities in spina bifida
Neurologic deficits in myelomeningocele (also known as open spina bifida in which spinal canal does not close) are usually present at birth, but may progress if complications such as hydrocephalus or tethered cord develop. Careful evaluation and monitoring of the neurologic gaps and identification of their causes is important to detect and prevent further deterioration of function.

Spinal cord; the neurologic deficits usually depend on the level of the wound, and typically affect the trunk, legs, bladder, and bowel. The deficits are usually severe, resulting in complete paralysis and absence of sensation. The bladder and bowel are affected in 97 percent of patients, resulting in urinary and faecal incontinence.

Brainstem: The brainstem is the back part of the brain, adjoining and structurally continuous with the spinal cord. Most patients with open spina bifida have brainstem dysfunction, due to the Chiari malformation. Chiari malformation is a condition in which brain tissue extends into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward. This results into difficulties in swallowing, stridor (a harsh vibrating noise when breathing, caused by obstruction of the windpipe), and episodes where breathing stops.

Hydrocephalus: 90 percent of newborns with spina bifida will have enlarged ventricles, indicating some degree of hydrocephalus (accumulation of fluid in the head) which often leads to multiple hospitalisations for shunt revisions and the complications of this procedure is the major cause of death.

Management of the neonate (new born)
Immediately after birth, Dr Kasendwa says that the area where tissue damage has occurred should be briefly assessed to note its location, size, and whether it is leaking cerebrospinal fluid (CSF). “The defect should be covered with a sterile dressing soaked in a saline solution. And for large defects, they should also be covered by plastic wrap to prevent heat loss and it should only be done by a neurosurgeon,” he cautions, adding, “An infant should be placed in lateral (sideways) position to avoid pressure on the lesion”.

Newborns should also be evaluated for associated abnormalities in order to make appropriate decisions regarding treatment. The abnormalities include; clubfeet, problems with formation and joint movements in the hips, knees, and ankles as well as congenital abnormalities of the heart, airway, gastrointestinal tract, ribs, and kidneys.

Importance of folic acid in the diet

Folic acid is a critical vitamin that contributes to the proper development of a baby in the first 28 weeks of pregnancy where the spine (bone) come together to cover the delicate spinal cord and surrounding tissues.
Sidney Senteza, a nutritionist with Dama Medicinal Consultants says folic acid deficiency comes with high chances of the mother giving birth to a child with a neural tube defect known as spina bifida and other related conditions such as cleft palate.
Senteza further explains;
The vitamin helps in the development of the baby’s brain and skull.

It is important for red blood cell production
supports healthy homocysteine levels,
supports proper cell division, healthy bone formation and prevents depression.
“It is important for a woman expecting to get pregnant to take folic acid before the pregnancy occurs, to help support proper development of the baby’s spinal column.
After conception, an expectant mother should have a balanced diet that contains proteins, folic acid, carbohydrates, iron, vitamins C and B, plus calcium for proper development of the baby,” he notes.

Dealing with risk and defects associated with spinal disorders

Children born with spinal disorders are difficult to manage and they develop severe complications that affect them throughout their lifetime. They cannot walk or hold their bowels. Photo by Rachel Mabala.

According to Dr Martin Kasendwa, a child with spina bifida should be fed on foods such as fish, milk and green leafy vegetables since they contain iron and folic acid. The child should also be given plenty of water, fruits and vegetables.
A healthy diet prevents excess weight gain thus parents should avoid feeding patients with high fat and sugar foods such as potato chips, sweets, chocolate, ice-cream, fast foods and fizzy drinks should be limited.
As children with spina bifida get older, portion sizes should be monitored since those using wheelchairs will be less mobile than their peers and so will require far less energy.

Linear growth or height will also be slower than peers due to paralysis of lower limbs which also reduces calorie requirement. Adults too require low intake of calories to avoid obesity.
“A mix of all types of foods rich in fibre is best to help avoid constipation. However, with fibre-rich foods, inadequate fluid intake will make constipation worse as the fibre becomes hard and dry and immovable in the bowel. Many children and adults limit fluid intake to limit toileting or the need to self-catheterise but due to the nature of disability, the risk of kidney urinary tract and bladder infections is increased and adequate fluid intake helps prevent these difficulties,” he explains.
Sources of fibre include fruits and vegetables.

Additional reporting by Christine Katende