Born in spring 2010, 3lbs 12oz, 16.5 inches, born at 32 weeks (8 weeks early).
Surgical History: 3/31/10 Coarctation of the Aorta Repaired. 8/25/10 Open Heart Surgery VSD Repaired. 11/29/10 G-Tube placement. 8/30/11 Un-tethering of Spinal Cord surgery. 1/2013 eyelid ptosis surgery. Hospital History: 62 days NICU/ICN, 10 days for VSD repair, 3 days for GT surgery, 2 days for spinal surgery. 1/2013eyelid ptosis surgery.
River is the most amazing baby I’ve ever met. I might be biased, I am her mother. River has put us through the most difficult year of our lives. River is my hero.
I was 20 weeks pregnant with her when I found out that “it” was a girl! That was the same day that my other girl, my 7-year-old pit bull Kyan, died. That was also 3 days before Christmas. I was back home visiting, I cut my trip short and headed home. That was a very sad week. When I was 21 weeks pregnant we found out that our baby was too small, she also had SUA, Single Umbilical Artery, or otherwise known as a two-vessel cord. The implications for SUA are under-sized babies, also the possibilities of congenital defects or chromosomal disorders. Our lives had been officially flipped upside down, trampled on, and kicked in the gut(a few times). We were terrified and heading into unknown territory.
I was put on bedrest for the next ten weeks, or as I like to call it: torture. When I made it to 32 weeks, and had my weekly appointment with our Maternal-Fetal Specialist OB-GYN, that happened to fall on St. Patrick’s Day, and my mother’s birthday, the doctor saw something very worrying. He suspected a bowel obstruction to our already IUGR(Intra Uterine Growth Restriction) baby. Her growth had also again slowed down. She barely registered in the bottom of the regular growth scales for her gestational age. He ordered her delivered. Later that day, during my rush c-section, River was born. I had to be completely anesthetized for the operation since the spinal did not take very well. The anesthesiologist also had a very panicked and difficult patient on his table. River’s birth was scary, traumatizing and joyless. A sidebar is that also during the first week of her life, my son, husband, myself and friends where all infected with a horrible puking virus. 3 ended up in the ER with severe dehydration, including my husband and MIL. My friend taking my husband to the ER, and then me to the NICU in the same night pushed me over the edge. I about had a nervous breakdown.
Shortly after she was born we began to discover all of her many anomalies. Her heart was the big one. But the reason she was delivered was also interesting. It was not a bowel obstruction, but something called hydrocolpos, she had a imperforate hymen. A small, non-consequential defect that was easily repaired. Visiting her in the NICU and beginning my demanding pumping routine was what the next few months entailed. She grew very slowly, and at 2 weeks old they diagnosed her with Coarctation of the Aorta, which called for an immediate surgical repair. Her first surgery was a closed heart surgery, a left thoracotomy, and a repair of her aorta. They couldn’t fix her VSD at that time because she was still premature, too vulnerable to open heart surgery. She was only 3 and half pounds. The other scariest day of my life. She did recover though, she is a true fighter.
Her congenital defects are as follows: Large VSD, ASD, bicuspid aortic valve, anomalous origin of the left coronary, left superior vena cava, imperforate hymen. No chromosomal abnormalities. Yet-to-be-confirmed defect of her lower spine. She also has Horner’s Syndrome, causing ptosis to her left eyelid, a small amount of nerve damage that occurred during her first heart surgery.
Her heart failure as a neonate caused her to be a very poor feeder. She ended up leaving the hospital on an NG tube. I had to tube feed her at home, all the while pumping and attempting to nurse her. She nursed about 5 minutes a day. She began to have severe vomiting episodes. She has been diagnosed with GERD, but I’m skeptical. But none-the-less she had to be treated for her vomiting. As we went into her 4th month, her vomiting got worse and worse, I pushed for her VSD surgery. They were hoping to get her bigger and stronger before surgery, but she was getting sicker. She latched on one day and threw up on me. She never latched on again. Her gag reflex had become hypersensitive. She developed oral aversion to feeding.
Her VSD repair was at the end of August, she was 5 and half months old, weighing 11.7 pounds. She had already been a very calm baby, sweet and cuddly, beginning to smile at everyone, but sickly, she had been in heart failure. Her breathing was rapid, all the time. She sweated during the slightest activity. A few days after recovering from her open heart surgery she was a new baby. She was a victorious little champion. She was relaxed, happier, comfortable. She still had to be tube fed, but she was beginning to turn into the happy little angel that we know today. I gave up pumping while she was still in the hospital recovering. I couldn’t take the stress it caused me. I couldn’t get her to nurse anymore and it broke my heart. I was already losing my milk anyway. I barely had the time to pump enough. And then the doctors wanted her on a hypoallergenic formula, telling me that she might even be allergic to my milk. More mental mommy torture.
She did very well after her VSD was repaired. Like I said she was a new baby. We began lots of treatments after this. Developmental clinics, physical therapy, feeding therapy, GI clinics, nutritionists. She developed torticollis, a condition in which she prefers to hold her neck crooked. And now she is physically delayed in her motor skills. She is already premature, but on top of the many set backs with surgeries, growth, and living in a weakened state, she is very behind. She currently attends physical therapy and feeding therapy once a week. She is followed closely by her GI specialist and nutritionist. She received her G-Tube at the end of November, as she was continuing food refusal, and the NG tube is just a nightmare. I had to learn to place the NG myself, and had to do it constantly. It was uncomfortable and irritating to her, and was certainly not going to help her move forward with actually wanting to orally feed.
Her cardiologist has since only called for a visit every 6 months! She is off all of her heart meds and is basically experiencing a full functioning heart. She will continue to be monitored for the rest of her life. She will need another open heart surgery around the age of eight, to repair her coronary artery defect. She will most likely need her bicuspid valve repaired when she is an adult.
Update February 2011: We are continuing to work vigilantly to get her eat. She has recently showed us some dramatic improvement with feeding therapy! She is actually taking a few bites of yogurt and purees. She will teeth on biscuits and even swallow a little of the mush. We are very hopeful this is a trend we can continue! We have also, on our own, began a trial of a new formula, an organic formula with no corn syrup. We are seeing a lot of improvement with her GI problems!
Update October 2011: River had her Tethered Spinal Cord finally diagnosed as of about August 2011. We quickly moved towards surgery plans. Her neurosurgeon performed surgery on 8/30/11 and everything went very well and he was very pleased with the results. Even though he came to this diagnosis of spinal tethering per her April MRI, there is no other outward evidence that she had a tethered cord. Even now surgery is complete River is the same girl, with a new scar on her back. But this surgery will prevent (90%) her chances of developing the many adverse symptoms a tethered cord can affect.
Currently River is crawling and scooting, standing and cruising, and will walk assisted. Her motor skills are still strengthening and developing, and her gross motor delay is lessening. Scooting is here primary ability to move around, she prefers it. Her crawling skills have recently improved but she still doesn’t want to use it very much. Her oral skills have plateaued recently. She will eat a few mouthfuls a day of favorite foods, but still gags on certain things. Her vomiting has lessened, but is still a regular event if prompted. Recently her GI doc has laid out a plan for us to get her off of night feeds, continue with the blenderized diet, and see if we can increase her volume intake.
At home and with the supervision of her feeding/GI team we attempted a tube wean in December 2011. We cut out 50% of her diet from the tube and tubed her a lot of water. She successfully stopped vomiting almost immediately and within days was learning how to eat more effectively and with more volume.
She has had eyelid surgery on her Horner’s syndrome eye, and it has beautifully corrected her ptosis. Today she still eats about 60% of her daily needs, we blenderize our own diet for her that is tube-fed, about 10-15 oz. a day. She’s been diagnosed with early onset astigmatism, especially in her Horner’s syndrome eye(left). She had gross motor delays up until about three years old. She now enjoys trampolines, tumbling, ballet class, tricycles, and can run her little legs off! We have currently ceased all therapies, her speech came a long way and continues to progress daily, she has been labeled as developmental speech delay.