We learned that Amy was pregnant in late March 2006. Her pregnancy was going well until the end of September 2006, when she went to a routine midwife visit and found out she was in labor. She was only 29 weeks along, so they admitted her to the hospital for a few days to control the contractions, and she then went home on bedrest. Once she reached 36 weeks, her midwife let her get up and return to work. Two and a half weeks later…
Wilson Elliott Maifeld was born at 4:42 a.m. on Monday 4 December 2006 at our local hospital in Dickson, Tennessee. He was 18 ½ inches long and weighed 5 pounds 1 ounce. Immediately after his birth, his blood work showed he had a low platelet count (thrombocytopenia), and preparations were made to transfer him to Centennial Women's Hospital in Nashville. The transport staff began pulse-oximetry monitoring and discovered his oxygen saturation percentage was in the low 90s - and dropping. This reading should be 100% if the blood is being oxygenated properly. He arrived at the NICU and was immediately assessed for a congenital heart defect. Thirteen hours after his birth, we were informed of his diagnosis: Tetralogy of Fallot.
Three days after that, we found out why Wilson has a heart defect. He has a chromosome disorder officially called 22q11.2 Deletion Syndrome. This disorder has several other names depending on the whim and the background of the diagnosing physician, and our cardiologist and neonatologist called it DiGeorge Syndrome. It is sometimes also called Velo-Cardio-Facial Syndrome (VCFS) for the palate (velum), heart, and facial features exhibited. '22q' seems to be the most current, generic name. It is usually characterized by heart defects, feeding difficulties, immune deficiencies, palate troubles and development delays, but a person with 22q could exhibit any of over 180 characteristic problems (PDF Fact Sheet).
Wilson spent 10 days in the NICU as his condition was evaluated. Tetralogy of Fallot is a cyanotic heart defect, which means that his blood was not oxygenating properly. Many kids have to have surgery immediately to make a life-saving, temporary measure with what is called a BT shunt. Fortunately Wilson was doing well enough to let him come home and grow a bit before his big repair. His oxygen saturation ran in the high 80s - low 90s.
Within 5 days of arriving home, we knew we were in trouble. Wilson was extremely colicky. Our pediatrician suspected acid reflux and put Wilson on medication. This helped, but he would still scream inconsolably for multiple stretches of 3-6 hours nearly every day. Finally in early January 2007, he underwent a video fluoroscopic swallow study, and we learned he was aspirating his milk. Further testing revealed that he also had severe gastro-esophageal reflux disease (GERD). He was immediately admitted to the hospital and given an nasojejunal (NJ) feeding tube that ran down his nose, through his stomach, and into his small intestine.
Our doctors recommended a Nissen fundoplication ("fundo") and gastric feeding tube ("g-tube") placement. The fundo wraps the top of the stomach around the esophagus to keep stomach contents from coming up. The g-tube is a port through the abdominal wall into the stomach. After much research and consultation with other parents, we elected to refuse the surgery and to go home with the NJ feeding tube instead.
We had to re-tape Wilson's tube to his face at least every night and sometimes during the day if it became loose. Wilson developed an allergy to the adhesives in the Tegaderm™, and we experimented with countless other types of tape before discovering Hypafix™. Of course, we had to be very careful not to let him pull the tube out as an emergency room visit was required to replace the tube.
Wilson grew to weigh 13 pounds and was finally ready for his corrective heart surgery at Vanderbilt Children's Hospital in Nashville, Tennessee on 11 June 2007. A (DuPont) Dacron™ patch was sewn over his ventricular septal defect (VSD) and his pulmonary arteries were widened. It went well, and unlike most children with Tetralogy of Fallot, he is not expected to need further surgeries. He did not have pulmonary atresia (valve permanently closed) and his pulmonary valve was only slightly stenotic (narrowed). The stenotic valve could become problematic in the future and so it will be monitored closely by his cardiologist.
We finally weaned Wilson from his feeding tube in late September 2007. It took nearly two months of work to teach him to manage liquids and to swallow. During his ten months with a tube, he never once pulled it all the way out (although a few times he came close!). Each tube would last about 6 weeks before it would become restricted and need emergency replacement. Our experience greatly surprised our doctors, who predicted we would regret our decision and come back for the fundoplication within a week.
In September, he also had an Auditory Brainstem Response (ABR) test to confirm that he has hearing loss. He had failed his newborn hearing screening and we could tell that he couldn't hear very well. He has profound hearing loss in his right ear and moderate hearing loss in his left ear. He was fitted for hearing aids but as you might expect, he doesn't like to wear them.
Wilson had a CT scan of his ears at the end of October 2007. Such a scan is routine when hearing loss is diagnosed. They discovered that Wilson's middle and inner ears have some malformations. This causes what is called conductive hearing loss. Wilson could also have nerve-related, or sensorineural, hearing loss. Then they gave us the really bad news: Wilson has an unusual spot at the base of his brain that is 1 cm in diameter. A follow-up MRI helped a neurologist diagnose the spot as a hamartoma, which is an abnormal growth of disorganized brain tissue. He was almost certainly born with it. Hamartomas are usually benign it seems, but Wilson will need an MRI every six months or so to make sure it is not growing or changing.
Aside from his NICU stay, all of Wilson's care to this point had been through Vanderbilt Children's Hospital in Nashville. But in November 2007, Amy was transferred to Parkersburg, West Virginia. Now Wilson has a pediatrician in Marietta, Ohio and a visiting cardiologist in Parkersburg, but his other doctors are in Columbus, Ohio at The Ohio State University and Nationwide Children's Hospital. There he has an audiologist, otorhinolaryngologist (ear, nose, and throat doctor = ENT), and neurosurgeon. He receives speech therapy, developmental therapy, and physical therapy at our home and in Marietta through Ohio's birth-to-three program, Help Me Grow.
Wilson has been through a lot in his short life, but most people who meet him can't tell that there is anything wrong with him other than he is small for his age. He is a cheerful and social baby, curious about his world and learning more every day. He is a good sleeper and a good eater, and he loves to be tickled, splash in the tub, read books, and go on hikes (I wonder where he got that?). Fortunately, his immune system and calcium levels seem to be normal and he has stayed quite healthy. Most doctors are also surprised to find that Wilson doesn't have palate problems.
About town, people often tell us, "Oh, what a healthy, bouncing baby boy!" If they only knew our story…