Emily Rose

Emily Rose was born in Salt Lake City, Utah in 2001.  We couldn’t have been happier if she was born potty trained.  Let me explain…  I am in the Marines and was on an unaccompanied tour in Okinawa, Japan when I got the call from my wife (Patty) in January of 2001 that her 20-week routine ultra sound had shown no stomach bubble.  She was scheduled to go see a perinatologist and get a second opinion. The perinatologist confirmed the lack of a stomach bubble and informed Patty that Emily was also missing her left arm and there was some developmental problem with her right arm.  I returned from Okinawa and they referred us to a geneticist who told us that Emily had VACTERLS association.  The reason for the lack of a stomach bubble was likely due to her having tracheal esophageal fistula, a condition where the esophagus fails to connect to the stomach.  They also told us that we had a high possibility of Emily having an imperforate anus as well as other associated complications.  We were counseled that due to the extreme fetal anomalies we were eligible to medically terminate the pregnancy.  We debated over what kind of life Emily would have with all of these medical problems.   We decided to hope for the best.

When Emily was finally born, they gave her APGAR’s of 9 and 9 and told us that besides her lack of arms and mild hypoglycemia she was a healthy 8lb 9oz baby girl.  They totally dismissed the lack of a stomach bubble from the ultra sounds. Saying her chin could have been pushed in not allowing her to swallow.  Emily went home with mom 4 days later.

We had problems with feeding and Emily spitting up after just 1-2 oz. of formula.  We took her back to the doctor every day for the next ten days.  They seemed to be treating us as though we were just new parents and didn’t know how to feed a baby.  Finally we got a doctor to order an “Upper GI Air Contrast” on June 8th, which revealed a wide-open GE junction and severe gastro esophageal reflux.  Emily was admitted into Primary Children’s Hospital that night.  They told us that we were lucky that we didn’t aspirate her.  After 6 days in the hospital, they released Emily with a NJ tube and a pulse-ox machine, stating she had a hiatal hernia that was causing her small stomach and the acid reflux was causing her to stop breathing.  They did not want to do the surgery because I was pending orders again and we were moving as soon as Emily was stable.

Once we arrived in San Diego, Emily was scheduled to have her hernia corrected.  This is when we were informed that Emily did not have a hernia but had a condition known as microgastria. (A very rare congenital anomaly in which the stomach is small and tubular and fails to act as a normal storage organ) The NJ tube was removed and the surgeon placed a piece of surgical tube connected to her stomach for feeding.

This was later replaced with a low profile MicKeyâ fitting and then again with two Bardâ fittings one g-tube and one j-tube.  The j-tube got very inflamed and has since been removed and the hole surgically closed

We opted not to have Emily’s stomach enlarged using part of her intestine otherwise known as a Hunt-Lawrence Pouch due to the many complications.

Emily has cut back on her need for the feeding machine from all day to just at night while she sleeps.  Her last exam showed that her stomach is stretching on its own.

We just had her first mold for a prosthesis made last week. It will go on her left arm to give her something to oppose her right.  We are not sure that she will use it, we just want to give her the option.  She already does just about everything with her feet from eating to playing mommy with her dolls, so it will be interesting to see how she responds to a prosthesis.

As far as walking/running she was almost two years old before she started walking and has taken a few nasty falls on her face; however, we recently we got her a helmet.  It’s more for us than for her.  She loves climbing and running around with her big sister Katelynn (5), so we put her in it when she is playing on hard surfaces.  It lets us relax a little more not worrying about her knocking out her teeth.