Spina Bifida

INTRODUCTION

Peter is a 16-year-old boy with a history of spina bifida currently admitted for a Mitrofanoff procedure.

HISTORY

Peter was diagnosed to have spina bifida at birth. He is a premature baby at 8 months old and is delivered by Caesarean section. He was not able to recall his birth weight or any past admissions into the PICU.

Peter�s medical problems are as follows:

1. Paraplegia: He is unable to move his lower limbs or walk since young and has loss of sensation. He has been on wheel-chair since 4 years of age. A physiotherapist visits him once weekly to exercise his limbs. He had occupational therapy before to learn about coping with activities of daily living.

2. Orthopaedics: He developed scoliosis at the age of 5 which was corrected in 1996. Release of the left foot was done for bilateral contractures of the lower limbs in 1997.

3. Hydrocephalus: A VP shunt was inserted for him at 3 years of age and he has been on regular follow-ups with the hospital to check the shunt for infection or blockage and for maintenance and replacement. He complains of infrequent headaches, early morning vomiting and lethargy. However, these symptoms are not present now.

4. Neuropathic bladder: He is unable to empty his bladder and had been wearing pampers till now. Clean intermittent catheterization is performed 4 � 5 times daily by his parents. He is now undergoing the Mitrafanoff procedure to enable him to perform the catheterization himself.

5. Neuropathic bowel: He has absent rectal sensation and little or no bowel control. He has been using faecal softeners and suppositories to enable him to remove his bowel.

6. Skin: Pressure ulcers are common in his buttocks and back of the thighs and he needs to be hospitalized a few times a year for treatment.

He is on regular follow-ups with a team of doctors from NUH who are assessing his condition and possible complications.

Family

Peter was the 2nd child of the family. He has a 22-year-old and 12-year-old brother. There is no family history of spina bifida. His father is a construction foreman and his mother is a technician. They live on the ground level in a Bishan HDB flat. They do not have any financial problems in footing the medical expenses of Peter.

Daily living

Peter has been on the wheelchair since young. He is able to eat, comb and change his clothes by his own but unable to bathe himself. He needs to attend school in the morning. His father drives him to school daily and his brother will fetch him back in the afternoon. In school, his class is at the ground level and so it is convenient for him. He has a good friend who will wheel him out to the canteen during recess and lunch hours. His mother cooks dinner for him and the family everyday and she will buy meals from outside if she is not cooking.

Care-givers

He remembered that his grandmother has been his primary care-giver when he was young as both his parents are working. When he was six, his family moved to their current residence in Bishan and his father employed a full-time maid to take care of his daily needs. Right now, the family does not have a maid and the family members are taking turns to take care of him.

Social

Peter is currently attending secondary education at Pierce Secondary school near his home and will be taking N levels at the end of the year. He has a few good friends from his class who takes care of him but other than these, he does not have any friends from other classes or outside his school. Peter seldom goes out with his friends. He occasionally joined his family for an outing at Bishan park and go out for dinner during the weekends. He does not have a girlfriend now. The only other time he steps out of his home is the monthly visits to NUH. Occasionally, he needs to be hospitalized due to urinary tract infections and pressure ulcers or for operations.

Personal

Peter is used to his disabilities after living with it for so many years. He understood that his condition is a congenital defect and is able to accept it without any bitterness or grievance that he is unable to live normally as other teenagers his age. He has adapted well to his limitations and able to cope with daily living without any problems. He is very appreciative of his family for giving him so much encouragement and support. He wants to be more independent so as to lessen the burden on his family. He enjoyed a loving relationships with his parents, siblings, friends and doctors.

Peter is studying hard for his N levels in November. He wants to do his O levels next year and study in a polytechnic. After graduation, he plans to work to support his parents. He does want to get married and have a family but is worried that he may not be able to find a girlfriend.

EXAMINATION

Peter is well, comfortable at rest and responds to speech in full sentences. His vital signs are stable.

On general inspection, he is wearing pampers and is catheterized. Half his head is shaven and a VP shunt can be felt just beneath the skin and subcutaneous tissues. Pectus carinatum of the chest is observed. He appears to be stunted. His lower limbs are short and small with signs of muscle atrophy as compared to his upper limbs. There is a midline lump 5 x 7cm protruding out of his back in the lumbar region with a linear scar over it. The lump is hard, non-tender and is dull to percussion. The skin over it is erythematous. No pressure ulcers were seen on the back.

His lower limbs are in a state of contracture. Power is zero and no reflexes can be elicited. There is loss of all sensations starting from the waist now.

SUMMARY

Peter is a 16-year-old boy with a history of spina bifida presenting with a neuropathic bladder and bowel, short stature, paraplegia and bilateral lower limb contractures and asymptomatic hydrocephalus now admitted for a Mitranfanoff procedure. He has good family and social support and do not have any significant psychosocial difficulties or problems.

MANAGEMENT

Spina bifida is a life-long condition which is the patient has to live with. Management of Peter�s condition will be continued after the Mitranfanoff procedure with the focus on the medical and psychosocial aspects which will invariably gain in importance as Peter matures into an adult.

Medical

Disabilities and mobility: Physiotherapists play an essential role in reducing deformities and encouraging mobility. Peter should be encouraged to continue his weekly sessions with his physiotherapist. Recommendations can be made for other forms of beneficial therapies either home- or institution-based.

Self-catheterization: After the Mitranfanoff procedure, Peter will be able to perform self-catheterization. He should be educated on the proper way of doing it and reminded of the possible complications and problems that can arise.

Sexual function: Peter may encounter some sexual problems in the future such as impotence, retrograde ejaculation and infertility. He should be given appropriate sexual counselling in the near future.

Psychosocial:

Job placement: Arrangements should be made for Peter to obtain a job after he leaves school.

Emotional support: Peter and his family should be encouraged to join support groups of spina bifida.

Personal: His hopes and fears of the future must be adequately addressed. Members of the team must be alert to signs of distress and be ready to provide the necessary support.

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