Functional disability of children with spina bifida: It's impact on parents' psychological status and family functioning.

Ulus Y, Tander B, Akyol Y, Ulus A, Tander B, Bilgici A, Kuru O, Akbas S. (2012) Functional disability of children with spina bifida: It's impact on parents' psychological status and family functioning. Developmental Neurorehabilitation. 2012 Jun 19.

Department of Physical Medicine and Rehabilitation .

Abstract

Objective: To evaluate the impact of functional disability of Turkish children with spina bifida (SB) on parents' psychological status and family functioning. Methods: Fifty-four children with SB and parents were included. The Functional Measure for Children (WeeFIM), Beck Depression Inventory (BDI), and Family Assessment Device (FAD) were used. Results: Mothers' BDI scores were significantly higher than fathers' (p < 0.001). No significant effects of the knowledge of having children with SB before birth and the number of children in families on BDI scores and FAD sub-scores were found (p > 0.05). According to multiple regression analysis; significant correlations with fathers' BDI were problem-solving (p = 0.012) and general functioning (p = 0.037) and with mothers' BDI was roles (p = 0.018). Only childrens age was found to be an influential variable on WeeFIM scores (p < 0.001). Conclusion: Spina bifida healthcare should include psychological support to parents of these children and this support should be independent from disability level of children.

PMID:22712551

Improved Survival Among Children with Spina Bifida in the United States.

Shin M, Kucik JE, Siffel C, Lu C, Shaw GM, Canfield MA, Correa A. (2012) Improved Survival Among Children with Spina Bifida in the United States. Journal of Pediatrics. 2012 Jun 23. 

 

Division of Birth Defects and Developmental Disabilities, National Center on Birth Defects and, Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA; RTI International, Atlanta, GA.

Abstract

OBJECTIVE:

To evaluate trends in survival among children with spina bifida by race/ethnicity and possible prognostic factors in 10 regions of the United States.

STUDY DESIGN:

A retrospective cohort study was conducted of 5165 infants with spina bifida born during 1979-2003, identified by 10 birth defects registries in the United States. Survival probabilities and adjusted hazard ratios were estimated for race/ethnicity and other characteristics using the Cox proportional hazard model.

RESULTS:

During the study period, the 1-year survival probability among infants with spina bifida showed improvements for whites (from 88% to 96%), blacks (from 79% to 88%), and Hispanics (from 88% to 93%). The impact of race/ethnicity on survival varied by birth weight, which was the strongest predictor of survival through age 8. There was little racial/ethnic variation in survival among children born of very low birth weight. Among children born of low birth weight, the increased risk of mortality to Hispanics was approximately 4-6 times that of whites. The black-white disparity was greatest among children born of normal birth weight. Congenital heart defects did not affect the risk of mortality among very low birth weight children but increased the risk of mortality 4-fold among children born of normal birth weight.

CONCLUSIONS:

The survival of infants born with spina bifida has improved; however, improvements in survival varied by race/ethnicity, and blacks and Hispanics continued to have poorer survival than whites in the most recent birth cohort from 1998-2002. Further studies are warranted to elucidate possible reasons for the observed differences in survival.

PMID:22727874

 

Spatial navigational impairments in hydrocephalus.

Smith AD, Buckley MG. (2012)  Spatial navigational impairments in hydrocephalus. Cognitive Processing. 2012 Jul 18.

 

School of Psychology, University of Nottingham, University Park, Nottingham, NG7 2RD, UK, alastair.smith@nottingham.ac.uk.

Abstract

Whilst much is known about the neuropathological consequences of hydrocephalus, there have been comparatively few studies of the cognitive impairments associated with it. Studies using standardised tests of cognitive function have identified a general pattern of impairments, with patients exhibiting particular difficulty on tests of spatial memory and executive function. A strong prediction is that these deficits are likely to affect daily wayfinding behaviour, and we report a study of spatial and navigational abilities in a group of patients with hydrocephalus but without spina bifida. Participants completed a range of experimental tasks assessing spatial cueing behaviour, landmark memory and route-learning, and idiothetic path integration. This patient group was compared to a control sample matched on verbal, spatial, and intelligence measures, and hydrocephalus was found to be associated with relative impairments in each of the tasks. Patients exhibited reduced sensitivity to spatial cueing, less accurate route-learning, poorer memory for landmark objects, and less accurate spatial updating (with particular impairments in the calculation of heading). Overall, these data represent the first empirical demonstration of navigational impairments in hydrocephalus, and we suggest some of the cognitive, neural, and individual differences factors that may contribute to the pattern of performance reported.

PMID:22806680

 

Maternal occupational exposure to polycyclic aromatic hydrocarbons and risk of neural tube defect-affected pregnancies.

Langlois PH, Hoyt AT, Lupo PJ, Lawson CC, Waters MA, Desrosiers TA, Shaw GM, Romitti PA, Lammer EJ; and the National Birth Defects Prevention Study.
Maternal occupational exposure to polycyclic aromatic hydrocarbons and risk of neural tube defect-affected pregnancies. 

Birth Defects Res A Clin Mol Teratol. 2012 Jul 17. doi: 10.1002/bdra.23045. 

Texas Center for Birth Defects Research and Prevention, Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services, Austin, Texas. peter.langlois@dshs.state.tx.us.

Abstract

BACKGROUND:

This study evaluated whether there is an association between maternal occupational exposure to polycyclic aromatic hydrocarbons (PAHs) and neural tube defects (NTDs) in offspring. This is the first such study of which the authors are aware.

METHODS:

Data were analyzed from 1997 to 2002 deliveries in the National Birth Defects Prevention Study, a large population-based case-control study in the United States. Maternal interviews yielded information on jobs held in the month before through 3 months after conception. Three industrial hygienists blinded to case or control status assessed occupational exposure to PAHs. Crude and adjusted odds ratios (ORs) with 95% confidence intervals (CIs) were estimated using unconditional logistic regression.

RESULTS:

Of the 520 mothers of children with NTDs, 5.0% were classified as exposed to occupational PAHs, as were 3.5% of the 2989 mothers of controls. The crude OR for PAH exposure was 1.43 (95% CI, 0.92-2.22) for any NTD and 1.71 (95% CI, 1.03-2.83) for spina bifida. Adjusted ORs were smaller in magnitude and not significant. Among women who were normal weight or underweight, the crude OR for spina bifida was 3.13 (95% CI, 1.63-6.03) and adjusted OR was 2.59 (95% CI, 1.32-5.07). Based on estimated cumulative exposure, a statistically significant dose-response trend was observed for spina bifida; however, it was attenuated and no longer significant after adjustment.

CONCLUSION:

Maternal occupational exposure to PAHs may be associated with increased risk of spina bifida in offspring among women who are normal weight or underweight. Other comparisons between PAHs and NTDs were consistent with no association. Birth Defects Research (Part A)

PMID:22807044

Sleep problems, chronotype, and diurnal preferences in children and adults with spina bifida.

Edelstein K, Cirino PT, Hasher L, Fletcher JM, Dennis M. (2012) Sleep problems, chronotype, and diurnal preferences in children and adults with spina bifida. J Biol Rhythms. 2012 Apr;27(2):172-5.

Spina bifida meningomyelocele (SBM) is a neural tube defect that involves dysraphism of the spinal cord and extensive reorganization of the brain. The authors assessed the relationship between chronotype, diurnal preferences, and sleep problems in individuals with SBM and healthy controls. Although individuals with SBM showed the characteristic decelerating quadratic relationship between age and chronotype, the curve was displaced, peaking at a younger age in controls compared with SBM (23.4 vs. 29.2 years). Groups did not differ in morningness-eveningness preferences. Individuals with SBM endorsed more sleep problems than controls. Further examination of the relationship between entrainment and sleep in SBM is warranted.

doi: 10.1177/0748730411435209

Use of intravesicular amikacin irrigations for the treatment and prophylaxis of urinary tract infections in a patient with spina bifida and neurogenic bladder: a case report

Huynh D, Morgan JA. (2011) Use of intravesicular amikacin irrigations for the treatment and prophylaxis of urinary tract infections in a patient with spina bifida and neurogenic bladder: a case report. J Pediatr Pharmacol Ther. 2011 Apr;16(2):102-7.

This case report describes the use of intravesicular amikacin irrigations to treat and prevent urinary tract infections (UTIs) in a pediatric patient with spina bifida and neurogenic bladder. A 15 year old Hispanic female was admitted for a UTI caused by Enterobacter cloacae and multiple-drug resistant Pseudomonas aeruginosa. A 7 day course of daily intravenous amikacin and ceftazidime was initiated along with twice daily intravesicular amikacin irrigations (15 mg/30 mL) with a dwell time of 2 hours. The patient improved and was discharged on prophylactic Bactrim SS (sulfamethoxazole/trimethoprim) 1 tablet daily and intravesicular amikacin irrigations (15 mg/30 mL) once every other day. Approximately 2 months after discharge, the patient developed another UTI from multidrug resistant Escherichia coli and was treated with a 14 day course of daily intravenous ciprofloxacin accompanied by daily intravesicular amikacin irrigations. Adjunctive therapy with either once daily or twice daily intravesicular amikacin irrigations successfully treated the patient's UTI. However, prophylactic treatment with intravesicular amikacin failed to prevent future UTIs in this patient.

PMID: 22477833 [PubMed - in process] PMCID: PMC3208437

Study finds in utero surgery preferable to surgery after birth for children with spina bifida

Study finds in utero surgery preferable to surgery after birth for children with spina bifida DALLAS (Feb. 9, 2012) — In a study to be presented today at the Society for Maternal-Fetal Medicine's annual meeting, The Pregnancy Meeting ™, in Dallas, Texas, researchers will report findings that show that, for children with spina bifida, surgery conducted while the fetus is still in utero as opposed to surgery on a newborn is more cost effective due to the costs associated with caring for a child with significant deficits.

 

This study, A Cost Effective Analysis of Prenatal Surgery for Myelomeningocele, sought to evaluate the costs and effects to the health care system of in utero surgery compared to surgery shortly after birth for children found to have spina bifida. Studies have already shown that in utero surgery results in a significantly higher quality of life for the child with spina bifida and places the mother at some health risks.

"Given the large costs associated with caring for a child with significant deficits, the improved outcomes with in utero surgery make it less costly over a lifetime than surgery after delivery," said Erika F. Werner, MD, with Johns Hopkins University, Gynecology and Obstetrics, Baltimore, Md., and one of the study's authors.

Werner and her colleagues built a decision model to evaluate the costs and effects to the health care system of the two surgery options. They found that in utero surgery is most often more cost effective compared to surgery after birth.

In addition to Werner, the study was conducted by Heather Lipkind, Joshua A. Copel, Mert O. Bahtiyar and Christina Han, Yale University, Ob/Gyn & Reproductive Science, New Haven, Conn.; and Stephen F. Thung, Ohio State University College of Medicine, Columbus, Ohio.

A copy of the abstract is available at http://www.smfmnewsroom.org/annual-meeting/2011-meeting-abstracts/.