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Spina Bifida

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Care of Adults with Chronic Childhood Conditions

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Abstract

Spina bifida (SB) is a common birth defect associated with varying degrees of neurocognitive, functional, and organ system impairment and requiring lifelong condition management. In order to provide quality health care and promote quality of life for adults with SB, the adult primary care provider (PCP) should be familiar with: (1) basic pathophysiology and management of SB-specific comorbidities such as shunted hydrocephalus, neurogenic bowel and bladder, musculoskeletal impairments, and skin ulcers; (2) necessary adaptations needed due to physical and neurocognitive impairments associated with SB; and (3) condition-specific nuances to general adult care including weight management, sexual and reproductive health, and mental health. The PCP should also be familiar with the subspecialists and resources needed to provide comprehensive and coordinated care for this population.

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Author information

Authors and Affiliations

  1. Department of Internal Medicine and Pediatrics, Baylor College of Medicine, 6620 Main St Suite 1250, Houston, TX, 77030, USA

    Ellen Fremion

  2. Department of Pediatrics, Stony Brook University, HSC Level 11, Room 040, 101 Nicolls Road, Stony Brook, NY, 11794-8111, USA

    Jonathan Tolentino

  3. Department of Urology and Restorative Pelvic Medicine, Department of Internal Medicine, Houston Methodist Hospital, 6560 Fannin St. Suite 2100, Houston, TX, 77030, USA

    Rose Khavari & Julie N. Stewart

Authors
  1. Ellen Fremion
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  2. Jonathan Tolentino
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  3. Rose Khavari
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  4. Julie N. Stewart
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Corresponding author

Correspondence to Ellen Fremion .

Editor information

Editors and Affiliations

  1. School of Medicine, Hofstra North Shore-LIJ School of Medicine, LAKE SUCCESS, New York, USA

    Mariecel Pilapil

  2. Mount Sinai School of Medicine , NEW YORK, New York, USA

    David E. DeLaet

  3. University of California Los Angeles , SANTA MONICA, California, USA

    Alice A. Kuo

  4. Baylor Clinic , HOUSTON, Texas, USA

    Cynthia Peacock

  5. Brigham and Women's Hospital , BOSTON, Massachusetts, USA

    Niraj Sharma

Appendix

Appendix

Spina bifida (SB) condition fact sheet

Definition

• SB is the most common neural tube defect (NTD) and is due to failure of the spinal neural tube to close at 25–28 days postconception

• SB is classified by functional level: thoracic (flaccid lower extremities), high lumbar (hip flexion present), mid lumbar (knee extension present), low lumbar (dorsiflexion present), and sacral (foot plantar flexion present)

Epidemiology

• An estimated 1500–2000 babies with SB are born in the United States annually, with the highest incidence in the Latino population

• Approximately 166,000 people in the United States have SB, with the majority being adults

• Primary prevention for SB is 4 mcg folic acid for women without SB and no family history of SB, and 4 mg for women with SB or who have a family history of SB

Special considerations

Adults with SB may have the following comorbidities

• Shunted hydrocephalus

• Neurocognitive impairment

• Neurogenic bladder

• Pelvic organ prolapse

• Neurogenic bowel

• Skin ulcers

• Musculoskeletal concerns: scoliosis, varying levels of paralysis, contractures, foot deformities, pain

• Disuse Osteoporosis

• Latex allergy

 

Adults with SB have increased risk of

• Obesity

• Hypertension

• Anxiety/Depression

• Impairment of sexual function

Recommended screening and prevention

• Annual chemistry, blood urea nitrogen/creatinine, renal/bladder ultrasound

• At least annual urology visit

• Regular skin exams

• Evaluation of bowel and bladder regimens

• Evaluation of mobility impairment and adaptive equipment need

• Evaluation of adaptive supports for vocation and independent living

• If shunt, administration of pneumococcal vaccines at least 1 time (Pneumococcal 13-valent conjugate vaccine followed at least 8 weeks later by pneumococcal 23-valent polysaccharide vaccine)

Parent/Caregiver Resources

Suggested Reading List

  • Dicianno et al. [106]

  • Webb [130]

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Fremion, E., Tolentino, J., Khavari, R., Stewart, J.N. (2016). Spina Bifida. In: Pilapil, M., DeLaet, D., Kuo, A., Peacock, C., Sharma, N. (eds) Care of Adults with Chronic Childhood Conditions. Springer, Cham. https://doi.org/10.1007/978-3-319-43827-6_18

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