Betty Baxter's Miracle

Betty Baxter’s childhood medical records are no longer available. However, her detailed descriptions make her medical diagnoses discernable with reasonable certainty.
     Betty was born with a spinal curvature, congenital scoliosis. Physicians told her that her spinal bones were “matted together.” Spinal bones often do not separate normally during development in those with congenital scoliosis, resulting in vertebrae being fused or partially fused together. This is a developmental abnormality called segmentation failure of the vertebrae.(1) The bones being “matted together” is an understandable lay explanation for what physicians would have observed on X-ray. Scoliosis very often worsens as children reach adolescence and enter a growth spurt. This was true in Betty’s case.
     Multiple organ-system abnormalities can be associated with congenital scoliosis. Heart disease is the most common with reported incidence of 54%, septal defects being the most common abnormality and something that could have contributed to Betty’s dilated cardiomyopathy (enlarged heart).(2) Betty’s cardiac condition worsened as she progressed in her early teen years and was complicated by heart failure.
     At age eleven, she was diagnosed with St. Vitus Dance (Sydenham’s chorea) which is a neurological complication of acute rheumatic fever causing irregular or jerky involuntary movements. These symptoms usually resolve in a few weeks or months. It is possible that her cardiac condition could have worsened from acute rheumatic fever as well.(3)
     Betty described having painful kidney stones as a child, which is somewhat unusual in the pediatric population. However, kidney and urinary tract anomalies are common in those with congenital scoliosis, reported to occur in up to 18% of patients.(4) One potential cause of kidney stones in children is urinary outflow obstruction, an abnormality sometimes observed in children with congenital scoliosis.(5) Urinary tract abnormalities may have been the cause of Betty’s kidney stones.
     Spinal cord abnormalities are common with congenital scoliosis and can include: tethered cord syndrome (the spinal cord abnormally stuck to the lower spinal bones), Chiari malformation (the brain partially descended through the base of the skull) and syringomyelia (cyst in the spinal cord). Syringomyelia is common with congenital scoliosis and can occur as a complication of a tethered spinal cord or Chiari malformation.(6) Betty may have had all three of these spinal cord
abnormalities. It is clear that her spinal cord was affected. By the time she reached her early teens Betty had limited standing capability and her arms had become paralyzed.
     Betty hadn’t had a pair of shoes since she was a baby. Before she was healed, Betty’s mother tried to straighten out her foot on the palm of her hand to estimate Betty’s shoe size in order to buy shoes in anticipation of her healing. Foot deformities including clubfoot have been associated with congenital scoliosis.(7) This seems to have been the case with Betty.
     Betty described other medical conditions seemingly unrelated to her spinal deformity. She had a gastric motility disorder (gastroparesis) and was unable to digest solid food. She survived on liquids and intravenous fluids. She also described periods of transient blindness, deafness, and paralysis of her tongue, which are unusual neurological deficits to occur simultaneously, suggesting possible infection (encephalitis) or a demyelinating condition.(8)
     In the 1930s, there was nothing to offer in terms of medical treatment. Modern treatment options might include neurosurgical, orthopedic, and cardiac surgeries. Current tube-feeding practices could have potentially improved her nutritional status. Still, no treatments currently available could have made her pain free or given her normal health.

 

References:

1. Hedequist D, Emans J. Congenital scoliosis: a review and update. J Pediatr Orthop. 2007 Jan-Feb;27(1):106-16.
doi: 10.1097/BPO.0b013e31802b4993. PMID: 17195809.
2. Furdock R, Brouillet K, Luhmann SJ. Organ System Anomalies Associated With Congenital Scoliosis: A
Retrospective Study of 305 Patients. J Pediatr Orthop. 2019 Mar;39(3):e190-e194. doi:
10.1097/BPO.0000000000001279. PMID: 30376499.
3. Cunningham MW. Streptococcus and rheumatic fever. Curr Opin Rheumatol. 2012 Jul;24(4):408-16. doi:
10.1097/BOR.0b013e32835461d3. PMID: 22617826; PMCID: PMC3645882.
4. MacEwen GD, Winter RB, Hardy JH. Evaluation of kidney anomalies in congenital scoliosis. J Bone Joint Surg Am.
1972 Oct;54(7):1451-4. PMID: 4653629.
5. KEVAN STERNBERG, SAUL P. GREENFIELD,* PIERRE WILLIOT AND JULIAN WAN, Pediatric Stone Disease: An
Evolving Experience, the Journal of Urology, Vol. 174, 1711–1714, October 2005. DOI:
10.1097/01.ju.0000179537.36472.59
6. Wang, X., Yu, Y., Yang, N. et al. Incidence of intraspinal abnormalities in congenital scoliosis: a systematic review
and meta-analysis. J Orthop Surg Res 15, 485 (2020). https://doi.org/10.1186/s13018-020-02015-8 Özerdemoglu,
Remzi A. MD*; Denis, Francis MD†; Transfeldt, Ensor E. MD† Scoliosis Associated with Syringomyelia, Spine: July 1,
2003 - Volume 28 - Issue 13 - p 1410-1417
doi: 10.1097/01.BRS.0000067117.07325.86
7. WINTER, ROBERT B.; MOE, JOHN H.; EILERS, VINCENT E. Congenital Scoliosis A Study of 234 Patients Treated and
Untreated, The Journal of Bone & Joint Surgery: January 1968 - Volume 50 - Issue 1 - p 1-15
8. Keane JR. Multiple cranial nerve palsies: analysis of 979 cases. Arch Neurol. 2005 Nov;62(11):1714-7. doi:
10.1001/archneur.62.11.1714. PMID: 16286545.