Dietary Considerations for Spina Bifida and Hydrocephalus

Category: Disability

Disability

What is Spina Bifida?

Spina Bifida (SB) is a condition that affects the spine and is usually apparent at birth. It is a type of neural tube defect and occurs when the spine fails to develop fully in the womb. A split in the vertebra or back bone can cause the spinal cord and nerves to be exposed. Where the split occurs and how severely, will determine how extensive the disability is. In Ireland, approximately 1 in 1,000 children are born with a neural tube defect.

How is it diagnosed?

It is usually detected in antenatal scans.

What effect does it have?

A cleft or lesion in the spine usually leads to some level of physical disability, depending on where on the spine the lesion was. In general, a paralysis is seen from the area of the lesion downwards. Some people can walk others may be wheelchair dependant.

Hydrocephalus

Up to 85% of those with SB will also have hydrocephalus. This refers to a build-up of fluid in the brain. As a child they may require insertion of a shunt to drain the excess fluid away. Hydrocephalus can influence visual perception and sensation in the hands which can make feeding difficult. Hydrocephalus has been shown to affect higher level thinking skills such as language and memory, in turn affecting decision making, logical thinking and organisational skills. Both comprehension and expression can be affected in subtle ways in this condition, affecting how well they can take on board recommendations either written or verbal, or express their own views effectively.

Spina Bifida and Diet

There are 3 main diet related issues for those with SB.

1. Obesity:

It is reported that in the USA, 50% of children with SB are obese. This level has also been seen in other studies. There has been no such survey in Ireland, though it is acknowledged that risk of overweight and obesity is high in this group. Generally the problem increases as a child gets older and moves into teenage years and adulthood.

It can be difficult to determine what the ideal weight for a child or adult with SB should be as it can be difficult to measure height accurately. In general, weight should be monitored regularly using a centile chart to ensure no rapid gains in weight.

Less activity, less lean body mass (calorie burning tissue) and a lower metabolic rate (lower rate of burning calories) have all been implicated as causes. Excess weight can have many negative implications:

Reduced mobility. Whether walking or in a wheelchair, excess weight can limit a person’s ability to move, change positions or transfer from chair to toilet or This can limit development of independence and self-care.

Increase pressure on For those in wheelchairs or using orthotics, increased weight will put increasing pressure on skin in contact with seats or tight straps. Sustained pressure on skin is a risk for pressure injuries (also known as pressure sores) which is a break in the skin that could become infected.

Breathing problems. For those in wheelchairs, the weight gain is usually concentrated on the chest area which will affect breathing and may make bone abnormalities such as scoliosis (a bend in the back) or kyphosis (a forward bend) worse which in turn impedes the lungs ability to expand properly.

Development of obesity related disease such as high blood pressure, diabetes and high

Social implications include decreased participation in physical activity and social

2. Bowel health / constipation:

Most people with SB will develop difficulties with constipation at some point. The main dietary causes are poor diets with low amounts of fibre and fluids. Reduced mobility also plays a role.

3. Pressure injuries:

Previously known as pressure ulcers, pressure sores or bedsores, pressure injuries can occur with sustained pressure on the skin. Reduced intakes of fluids and low intakes of protein and some vitamins and minerals can impair the healing of skin and increase the risk of these pressure injuries. Decreased sensation associated with SB can also increase the risk of developing a pressure injury, skin should be checked regularly for any sign of breakdown and contact your local health provider.

Nutrition Advice for those with Spina Bifida

Healthy eating to prevent excess weight gain

There is a wealth of advice to prove that offering a wide choice of food during weaning and beyond will increase the likelihood of children developing a varied and healthy diet into adulthood.

Use the Food Pyramid as a guide. Excess high fat, high sugar foods such as crisps, sweets, chocolate, ice-cream, fast foods and fizzy drinks should be limited to once or twice per week. As children with SB get older, portion sizes should be monitored as 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. Many adults require quite low intakes of calories to avoid obesity.

Some children especially those with hydrocephalus can appear to be ‘fussy eaters’. It is not known if this is related to the disease or a behavioural development. In general, unless there is a particular difficulty, a broad range of food should be offered and attention to fussiness or selectiveness avoided.

Include fibre daily

Sources of fibre include fruit, vegetables, legumes, wholemeal bread and wholemeal cereals. A mix of all types is best to help avoid constipation. However, inadequate fluid intake will make constipation worse as the fibre becomes hard and dry and immovable in the bowel. People with SB are at increased risk of kidney, urinary tract and bladder infections. Constipation can make infections worse as it increases fluid retention in the bladder. Adequate fluid intakes help prevent these difficulties.

Vitamins & Minerals

It has been shown that children and adults with SB have vitamin D levels lower than the healthy population. Vitamin D supplementation may reduce the risk of pathological fractures. Consult with your doctor or dietitian if you are considering giving doses above the normal recommendations.

Babies under 12 months - requirements are 5 micrograms of vitamin D as a supplement daily from birth if they are:

Breastfed
Have less than 300mls or 10 fluid oz (ounces) of infant formula a day

Children aged 1 to 4 years - requirements are 5 micrograms of vitamin D as a supplement daily from Halloween (October 31) to St Patrick’s Day (March 17).

Adults and children over 4 years old- requirement is 10 micrograms of vitamin D as a supplement daily from Halloween (October 31) to St Patrick’s Day (March 17).

Most people can get all the vitamin D they need through sunlight on their skin and from a balanced diet during the summer months.

People at risk of vitamin D deficiency (which could include those with SB)- recommendation is 10 micrograms of vitamin D as a supplement daily throughout the year. You may not get enough vitamin D if you have very little or no sunshine exposure. This may include the following groups of people.

If you are not often outdoors – for example, if your mobility is reduced
Resident in an institution like a care home
If you are at increased risk of sunburn due to decreased sensation

A balanced diet with fruit and vegetables should provide all the other nutrients needed. However, some children and adults whose calorie intake is low, should have their intake reviewed by a dietitian to ensure they are meeting their requirements. Those with wounds or pressure injuries may need supplementation and this should be discussed with your doctor or dietitian.