The Lehnhof Family

VACTERL is an acronym for a combination of birth defects. A child with three or more of these birth defects may fall under the VACTERL association. Since Nathan was born with VACTERL, people ask a lot of questions. Here is the rundown:

Vertebral abnormalities: Nathan was not born with typical vertebral abnormalities. Nate was born with a longer than normal spinal cord, but it was not tethered as is common with VACTERL.

Anal atresia: Nathan was born with an imperforate anus because his anus was connected to his prostate. Nate had a colostomy: his intestines were pulled to the surface of his abdomen to dispose of waste into an artificial bag. At one year of age Nate underwent a reconstruction of his anus. Six months later he had a surgery to take down the colostomy and now through a bowel routine will be able to be potty trained as typical, though we have been warned it might be a long process.

Cardiac defects: Nate was born with several holes in his heart, ASD and VSD. They were repaired when he was 8 months old durng open heart bypass surgery. Due to the complications of a weak heart during his first months, Nate developed pulmonary hypertension, high blood pressure in the lungs. Nate wore oxygen full time for almost a year, and now wears oxygen at night.

Tracheoesophageal fistula: Nate was born with a connection, or fistula, between his windpipe and esophagus. Nate has this repaired the morning after he was born.

Esophageal atresia: Kids with the fistula typcially also have esophageal atresia, which creates reflux and other eating problems. As Nate matures he will be able to learn how to manage these issues through dilation of his esophagus, feeding therapy, and medication.

Renal abnormalities: For Natha, this is the most serious of his association. Nate was born with a single displastic kidney. Nate's kidney is weak and unable to pump fluid quickly enough to flush itself well. Over time this created a large ureter, where bacteria was able to grow and urine became slow moving, like a dam in a river, and flushed urine back into his kidney. This is called kidney reflux. Over time the reflux created infections and damaged his kidney. Last year Nate had surgery to taper his ureter and place an internal stent. Together these help create a faster flow of urine to his bladder, decreasing his kidney infections. Since this time Nate's kidney fuction has not worsened. It is anticipated that at some point Nathan will need a kidney transplant. We can do our best to prevent this and increase the fuction of his kidney by feeding Nathan large volumes of fluid and keeping his electrolytes balanced properly. Typically Nathan's kidney functions between 15-30%. Kidney disease creates other abnormalities we have to watch closely with Nathan: poor growth, dental problems, poor development of fine motor skills in the mouth due to lack of appetite and oral stimulation, etc.

Limb abnormalities: Nate was born with pronation and weakness of his feet and ankles that will improve over time through physical therapy and aids such as braces for his feet. Nate's muscles are weak, but are able to be strengthened and simply require extra work.

I wish that was everything, but that is just a brief overview of VACTERL association and Nate's health history. Nate is a tough kid and we love him and are proud of his strength!