Spina Bifida

Spina Bifida is one of the many birth defects neonates are at risk of. However, this particular defect is unique because it is characterized by problems in the central nervous system (CNS) and it has a low death rate. The causes of this medical condition are quite difficult to determine as they are subject to hereditary and environmental elements. Simply put, Spina Bifida refers to a situation where the spinal cord is not fully developed. In extreme cases, the spinal vertebrae could be so badly formed that the delicate spinal cord is left unprotected. In most cases, the spinal cord suffers damage due to this. The baby could suffer from reduced brain function and poor transmission of commands to affected organs. This slightly damaged link from the brain to the body tissues and organs leads to poorly developed body systems. There are other associated problems with this spinal defect even though it mostly affects the body's skeletal system. These problems are prevalent in people born with Spina Bifida (Fletcher & Brei, 2010).

Spina Bifida can manifest in three different forms.

Myelomeningocele

This is the worst form of Spina Bifida and it is characterized by an exposed spinal cord and protective meninges. Myelomeningocele has the highest rate of occurrence of the three forms and thus is often mistakenly referred to as Spina Bifida. In most cases, myelomeningocele causes mental, behavioral and societal problems due to its associated adverse brain effects which directly manifest in improper organ and nerve systems function (Dennis and Barnes, 2010).

Infants born with this condition experience lack of feeling in areas situated below where the disorder occurs. Excretory systems and organs like the anus and bladder are commonly affected. Furthermore, these infants have a high risk of suffering hydrocephalus whose prominent symptom is the increase in the volume of fluid in the head. This leads to a rise in the skull's pressure which causes it to become bigger than normal (International Federation for Spina Bifida, 2017).

Meningocele

In this form of Spina Bifida, the protective meninges and spinal fluid are left exposed from the spinal column which develops quite normally. Also, closed neural tube problems are common in this form and they are characterized by wrongly located developments of fat, bone or meninges which adversely affects the growth of the spinal cord. Meningocele is widely regarded to be a less acute condition when compared to myelomeningocele because the spinal cord is more protected. The occurrence of a protrusion on the sufferer's back repeats itself though it doesn't contain the spinal nerves in this case. These nerves are well protected from harm and thus do not suffer any lasting damage (Spina Bifida& Hydrocephalus Association of Canada, 2000).

Occulta

This is the least severe form of Spina Bifida. In this case, a few vertebrae are distorted but still have their protective layer of skin. Excretory systems and organs like the anus and bladder could also experience malfunctions as a result of Spina Bifida and many infants with this disorder live with hydrocephalus (abnormal buildup of spinal fluid in the head) (National Institute of Neurological Disorders, 2017).

The spinal nerves in this form are well developed and protected, thus cases of nerve disorders among occulta sufferers are rare. Occulta could be so mild that it has escaped the attention of medical personnel in some cases until it was discovered mistakenly much later. Noticeable symptoms of occulta are higher hair concentration or fat buildup around the spinal area, small body holes, birthmark and non-uniform skin color (Foster, 2016)

Depending on the severity of the condition, SB-affected people move using various methods. Some walk normally without needing any aids, others employ braces, crutches and wheelchairs to get around. Those whose disorders occurred very close to the brain might not be able to use their legs and employ wheelchairs. Those with this disorder occurring close to the waist could use crutches, braces or walkers for moving around and in some cases, could walk normally without any form of aids (Sandler, 2004)

The most severe Spina Bifida form 'Myelomeningocele' has a greater effect on motor skills. There is greater difficulty in moving to normal places like the bathroom, the patient could lose sensibility in the legs or might not be able to move them at all. For the other forms like 'meningocele', there is a less severe effect. In some cases, there are no effects at all while in other cases, acute leg frailty as well as bladder and anal problems could occur. In the case of 'occulta', there are usually no visible symptoms or disabilities thus motor skills are normal in most cases (Center for Disease Control and Prevention, 2016).

The adverse effects of Spina Bifida occupy a very wide range forms. They could be as severe as visible bodily distortions and improperly developed organs and as mild as bladder disorders that can be solved via surgery (Spina Bifida& Hydrocephalus Association of Canada, 2000).

The various types of effects that can be caused by Spina Bifida are classified into three wide categories. These include:

• Motor problems -- disorders in movement systems and organs

• Bladder and anal problems -- disorders in excretion of body fluids and operation of digestive systems

• Hydrocephalus (abnormal amount of spinal fluid in the head)

The seriousness of these defects varies depending on the part of the spine affected by Spina Bifida. When the affected area is close to the brain, paralysis of the legs and motor problems are prevalent compared to when the affected area is around the middle or the lower part of the back. Infants who suffer from hydrocephalus have a higher tendency to have mental issues (Greene & Copp, 2016).

Due to the close link of neural tube problems and CNS disorders, people living with myelodysplasia will have brain problems which could lead to hydrocephalus. The prevalent brain disorder associated with Spina Bifida is the Arnold-Chiari malfunction (type II). It affects a large percentage of people with Spina Bifida and it is characterized by brain and cerebellar structural problems. These structural defects lead to a protrusion through the foramen magnum by the cerebellar tonsils as well as a movement of the medulla towards the cervical spinal canal, causing cranial nerves to be displaced. Over 25% of people with this brain condition suffer from brainstem deformities whose signs start as early as a few months. This condition could also lead to other Central Nervous System disorders (Botto, Moore, Khoury & Erickson, 1999).

Spina Bifida could cause acute deformation of the vertebrae leaving the spinal cord exposed. In most cases, the spinal cord suffers damage due to this and the baby could suffer from reduced brain function and poor transmission of commands to affected organs. This slightly damaged link from the brain to the body tissues and organs leads to poorly developed body systems (Spina Bifida& Hydrocephalus Association of Canada, 2000).

Spina Bifida doesn't have a clinical cure till to-date; however, improved therapy methods have successfully ensured that people suffering from this disorder can lived much longer and comfortably although continuous medical checks and tests throughout the life of the patient are needed. Over two-thirds of Spina Bifida cases can be avoided by daily doses of Folic Acid two months before and after pregnancy. This medication has proven very effective in increasing the number of births of babies without Spina Bifida. Nonetheless, intensive research and arrangement for instant treatment for babies born with the disorder is in progress (Mertens, 2012).

Despite this, Spina Bifida cannot be totally cured. The destroyed part of the neurological system cannot be amended or restored as well as the damaged nerves. The amount of therapy is a function of the seriousness or class of the defect. In most cases, infants born with occulta do not need any instant treatment, though there could be a need for surgery as the child develops. The important early steps in taking care of infants born with myelomeningocele include preventing the exposed body parts i.e. nerves and tissues, from infection and further damage. Normally, an infant born with the defect will be operated on within the first week to cover the exposed body parts and reduce the danger of infection or damage. This immediate operation could help the child enjoy normal body operation and put a stop to further nerve damage. A number of the patients could require more surgeries to help correct issues with the legs, waist or backbone. Those who develop hydrocephalus normally have to undergo a higher number of surgeries to change the malfunctioning fluid passage which could get blocked.

A number of Spina Bifida patients would need walking aids like braces, crutches or wheelchairs. The closeness of the affected part of the spine to the brain usually determines how much movement is possible by the patient. Those with the disorder occurring close to the brain could suffer lifelessness in the legs and need a wheelchair while those with the disorder occurring farther from the brain could function effectively with crutches, leg braces, walkers and bladder catheters.

Placing the child on specific exercise regimes for the legs and feet timely could be very helpful in getting the child prepared for the use of walking aids when the child gets older. Care for the bladder and digestive system disorders start immediately after birth and they take the form of bladder catheterizations as well as digestive system monitoring techniques (International Federation for Spina Bifida, 2017).

References

Botto, L., Moore, C., Khoury, M., & Erickson, J. (1999). Neural-Tube Defects. New England Journal of Medicine, 341(20), 1509-1519. doi:10.1056/nejm199911113412006

Center for Disease Control and Prevention (2016). Basics - Spina Bifida. Retrieved 22 February 2017, from https://www.cdc.gov/ncbddd/spinabifida/facts.html

Dennis, M. & Barnes, M. (2010). The cognitive phenotype of Spina Bifidameningomyelocele. Developmental Disabilities Research Reviews, 16(1), 31-39. doi:10.1002/ddrr.89

Fletcher, J. &Brei, T. (2010). Introduction: Spina Bifida-A multidisciplinary perspective. Developmental Disabilities Research Reviews, 16(1), 1-5. doi:10.1002/ddrr.101

Foster, M. (2016). Spina Bifida: Background, Pathophysiology, Etiology. Emedicine.medscape.com. Retrieved 22 February 2017, from http://emedicine.medscape.com/article/311113-overview

Greene, N. &Copp, A. (2016). Spina Bifida. Reference Module in Life Sciences. doi:10.1016/b978-0-12-809633-8.07194-6

International Federation for Spina Bifida and Hydrocephalus (2017).SPINA BIFIDA -- FACTSHEET Retrieved 22 February 2017, from https://www.ifglobal.org/images/documents/IF_SPINA BIFIDA_factsheet.pdf

Mertens, P. (2012, November). Spina Bifida and primary prevention. In Orphanet Journal of Rare Diseases (Vol. 7, No. S2, p. A18). BioMed Central.

Sandler, A. (2004). Living with Spina Bifida (1st ed.). Chapel Hill: University of North Carolina Press.

Spina Bifida& Hydrocephalus Association of Canada (2000) Spina Bifida. Retrieved 22 February 2017, from http://www.Spina Bifidahac.ca/pdf/Spina_Bifida.pdf