Naomi was named after Naomi from the Book of Ruth. Her name means “my joy,” “my bliss,” and “pleasantness of Jehovah” and she truly is my joy. We named her this after we found out about a birth defect that she has called an omphalocele. An omphalocele is a rare abdominal wall defect in which the intestines, liver, and occasionally other organs remain outside of the abdomen in a sac because of a defect in the development of the muscles of the abdominal wall (exomphalos). We first learned about this defect when I was just 18 weeks pregnant and we knew that it would hold some challenges for her but like Naomi in the Bible we had faith that God would restore her. My doctor was convinced that this defect was the result of a fatal chromosomal defect called Trisomy 18. If Naomi were to have this, she would not survive more than a few hours past her birth. My husband and I didn’t want to be caught off guard, so at the recommendation of our doctor, we scheduled an amniocentesis to find out if she had this defect. After several days of waiting we received good news: Naomi did not have Trisomy 18!! What she did have was an extra piece of a chromosome which will cause her to have smaller extremities (arms, hands, legs and feet) and possibly have some difficulty with speech. She and I have this in common.
After her birth, the doctors at the NICU were not convinced that the amnio was correct so they reordered the tests using her blood and added another test to check for Beckwith Wiedemann (an overgrowth disorder usually present at birth, characterized by an increased risk of childhood cancer and certain congenital features. Beckwith syndrome can also cause child behavior problems.) We got a consult from a geneticist who advised us that children with omphaloceles either have Trisomy 18, or they have Beckwith Wiedemann, it’s rarely, if ever just a random defect. When the repeat panel came back, it confirmed what the amnio had already uncovered. The Beckwith test took longer, but last week we got the results back and they were negative! Despite the claims of the medical staff, Naomi’s omphalocele is benign with no identifiable cause. Her surgeons are treating it with medication to help skin grow around the sack. At some point in the future they will begin to wrap it in such a way that it begins to be compressed back into her abdomen to establish domain in the area her organs should be located. She will need at least one surgery to completely resolve the defect but other than some possible long term issues with digestion she should have no long term issues from it.
Currently, eating has been a challenge for her. Once she was able to begin starting feeds, the surgeons were pushing her to increase her continuous drip by .5mls per hour each day. She was not able to tolerate very much before she would begin vomiting and we would have to stop her feeds completely for several days so that she could recover. The first time she had issues I suggested that we give her a couple of days between increases but it took three more instances of vomiting before the doctors and surgeons were convinced. The last instance of vomiting was so severe it seems to have caused her to aspirate into her lungs which has further complicated her existing lung disease and caused pnemonia which she is currently fighting off. She has also had 3 separate infections of staph and gram negative rods in her trachea. The doctors suspect that she is “colonized” with these bacteria which means she will always have them, and sometimes they will make her sick.
Because she was born so prematurely her lungs were not developed enough to handle breathing on her own. She has been on a ventilator for most of her life. The medical staff has extubated her three times, the first time she was not able to breathe without the ventilator for any longer than 10 minutes. The second time she only lasted an hour. Most recently was the third attempt and she was on a RAM cannula (CPAP) for a week before getting too tired to maintain her oxygen levels without a great deal of assistance. Prior to this last attempt the doctors were preparing us for her to undergo surgery for a tracheostomy (an opening in the windpipe which would allow direct access to the breathing tube and make breathing easier for her). This would be a better course to her staying on the ventilator with an endotracheal tube which will cause more and more problems for her the longer she is on it. It is unclear to us at this point as to whether this is still our next step or if the fact that she did so well before being reintubated has changed things, especially since she contracted an infection around the same time. We are unsure whether she became sick because she was tired or if she was tired because she was getting sick. The doctors have also been concerned because of her lung disease, that she will develop pulmonary hypertension, however, at this point all the tests have been negative.
We continue to pray for complete healing and restoration of both her lungs and her omphalocele. Although God has not yet answered those prayers in the way we would like, His grace has protected her from several other serious complications that she could potentially face with everything else she is going through. We are trusting God to continue to work in her little body and pour out His favor on her and to show us how He wants to use these experiences to bring glory to Him. I can say this for certain, even if no one else is impacted by her, knowing Naomi has already changed my life for the better. She has taught me more about perseverance and strength over the past few months than I had learned in my previous 34 years. I can’t wait to learn more from this beautiful daughter of mine, her testimony is going to change the world for the better.