JENNA'S DIAGNOSIS
VACTERL ASSOCIATION - Each letter of this name stands for the anatomy affected
V=vertebral dysgenesis - irregular
A=anal atresia &/or fistula - no abnormalties
C=cardiac anomalies - ASD & VSD (2 holes in the heart)
T=trachea - tracheal fistula(connection) to the stomach & tracheal narrowing (stenosis)
E=esophageal atresia - disconnected from stomach - ended in a blind pouch
R= renal(kidneys) anomalies - no abnormalities
L=limb anomalies such as radial dysplasia, thumb or radial hypoplasia, preaxial polydactyly, syndactyly - short forearms,missing radius & thumbs. Hands flexed, missing fingers
Vater or Vacterl Association is defined as an association characterized by the sporadic, nonrandom association of specific abnormailities. The incidence is rare at only 250 known cases worldwide. It is not a syndrome and its not considered to be linked to heredity.
Babies born with these problems normally have mothers who received good prenatal care & were in healthy condition prior to and during their pregnancy.
It is thought that some sporadic event during early pregnancy (4-6 weeks gestation) occurs affecting a specific mesodermal area of development in the baby which may produce simultaneous anomalies of the hindgut, lower vertebral column, lower urinary tract, and developing kidney. About 2/3 rd's of all malformations in Vacterl are in the lower body segment while 1/3 rd have been located in the upper body segment, as in Jenna's case. There are an average of 7-8 abnormalities per patient.
As of late, the cause is unknown. Parents with Vacterl children are no more likely to have subsequent children with this association in the future than any other person with only healthy children.
The prognosis of these babies is often very good, but dozens of surgeries are often a byproduct of this condition, and children spend much time in and out of hospitals, delaying their development often times.
Jenna's case was on the severe end of the spectrum and had it not been for the tracheal stenosis which was extremely difficult to repair in such a little baby, she may have gone on to overcome every other physical obstacle in the future, with God's help alone.
Fantasya
