Many spina bifida patients lead full lives thanks to their own and their parents’ heroic efforts

Ireland has one of the highest rates of spina bifida in the world. Occurring in one in 1,000 live births, it is estimated there are in the region of 500 children under the age of 18 living with the condition here. Spina bifida (Latin for “split spine”) is a multi-faceted condition, which, at its most severe, causes orthopaedic, neurological and urological issues for patients and their families.

As the name suggests, the spinal cord, instead of being enclosed within the bony spinal column, may be left uncovered. Myelomeningocele is a birth defect, involving both the spinal nerves and their covering, that occurs in the early weeks of foetal development. It is the most common and most serious type of spina bifida. In babies with myelomeningocele, parts of some of the bones of the back (vertebrae) do not develop properly and do not close completely around the spinal cord. As a result, portions of the spinal cord, nerves, spinal fluid, and surrounding tissues push through the incomplete backbone and are exposed. These tissues often form a sac that sticks out of a baby’s back.

Children born with a lesser form of spina bifida, called meningocele, in which just the lining of the spinal column protrudes and is contained in a sac under the skin, usually face less severe disabilities.

The level of myelomeningocele formation largely determines the severity of the disease. Babies typically lose nerve functioning below where the defect occurs in the spine. This causes a loss of sensation, bowel and bladder problems and muscle paralysis. The paralysis and resulting mismatch in muscle power can lead to spinal deformities as well as problems with the legs and feet.

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The development of a spinal problem, or curvature, is prevalent in the more severe cases of myelomeningocele. The curvature may be caused by: weak trunk muscles that allow the spine to collapse; abnormally formed vertebrae; or a combination of both.

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There are three common types of spinal deformity: scoliosis is a sideways curvature of the spine to the left or right. Scoliosis in children with myelomeningocele most often results from a lack of neuromuscular control causing weak trunk muscles that cannot support the spine. Kyphosis is an exaggerated rounded back that can develop from muscle imbalances in a child’s trunk that are associated with paralysis. Lordosis describes an exaggerated inward curve of the back that can occur when a child’s hip muscles are tight, causing the pelvis to push forward and an exaggerated curve to develop.

Unfortunately, these spinal changes are not static and so multiple orthopaedic treatments are often needed. As the child ages, their spine changes, while paralysis of the legs can lead to sitting imbalances that put too much pressure on certain areas resulting in skin problems. A kyphosis curve may also force a child to lean forward and rely on their hands for constant support. Without the full use of their hands, the child is not able to perform usual daily activities. In addition, severe curves can collapse space in the chest, which can potentially cause heart and lung problems.

So for some children and parents, spina bifida means recurrent attendances at hospital to have curves and other measurements continually monitored. Regular assessments by a multidisciplinary team will try to anticipate looming clinical need such as further surgical operations or the provision of aids and appliances to help with daily life. For some there is the additional burden of recurrent urinary tract infections due to a loss of bladder control leading to the possibility of a permanent insertion of a urinary catheter.

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The treatment of children with spina bifida is highly complex and individualised. It demands high-quality care from a range of experienced health professionals working collectively as a team.

Ultimately, however, many spina bifida patients lead full lives thanks to their own and their parents’ heroic efforts.

Their stories deserve to be heard.

muiris.houston@irishtimes.com