Silent Sunday

On Sundays,  I plan (plan being the key word here) on posting pictures of River. No words, just pictures of my little man. For those of  you that have not seen his 1 year pictures taken by Jackie Willome Photography (who is amazing I must say), here are just a few of my faves:

Announcing the Arrival of River Scott Ross

As most of you know, River Scott Ross was born April 20 at 12:34 am. He weighed 6 lbs. 4oz. Since his birth he has been in the NICU due to low glucose levels which is caused by a problem in his pancreas. Our lives are now spent living at Dell Childrens Hospital and the Ronald McDonald House. River has surprised the doctors and is making a steady recovery. He's my little miracle! Once we get home, I will leave a more detailed blog about River's early arrival and life in the NICU. We are getting so close to bringing him home.

Here are some pictures of River in the NICU:

My Little Ray of Sunshine

I kept telling myself I would start blogging again, but never made the time to actually sit down and write. I have officially decided that it is important to start documenting the day to day challenges and joys of having a child with a genetic disorder. He is a very special little guy that brings so much joy (and stress too) to our lives. We have been through so much in 17 months! Things are starting to settle down now and we have become comfortable with our new normal. I hope this blog helps to raise awareness for others with Congenital Hyperinsulinism as well.

Here is an introduction to River's Story

When we found out we were pregnant with our first child, it was a huge surprise!  I immediately spent countless hours researching how to have the perfect pregnancy. I just wanted to make sure I was doing everything right for our baby. Little did we know that the pregnancy was the calm before the storm!

At 34 weeks pregnant, we had a quite a scare. I was at a chamber of commerce luncheon with my husband, and as we were leaving the luncheon, I took a tumble down 9 concrete stairs. I was wearing heels, which is not very smart when you are 34 weeks pregnant! I ended up with 2 broken arms and a fractured wrist. During the ordeal, they started to monitor the baby and realized I was also having contractions 10 minutes apart. We spent the night in the hospital hoping that my contractions would slow down. Luckily they did and we were able to go home. My husband quickly learned how to take care of a helpless baby! It was great practice for what was to come. I couldn’t take a shower, drive, feed myself, or even get dressed without the assistance of my amazing husband! He was a trooper for sure.

At 36 weeks pregnant I was diagnosed with pre-eclampsia and put on bed rest. A couple of days later, my blood pressure shot up and we headed to the ER. My doctor was at the hospital that night and decided to induce labor before my blood pressure got too high.

After days of being in labor, they finally decided to deliver via c-section. River was born 3.5 weeks early and weighed 6lbs 4oz. I heard him cry right away and my husband rushed over to snap the first picture of him on my cell phone. He came and showed me the picture while I was still on the operating table. It was love at first sight, I just thought he was perfect! River looked like a beautiful, healthy baby boy or so we thought.

Since he was born early, the hospital checked his blood sugar, even though it isn’t part of the normal newborn screen. They check blood sugar on premature babies because they often have problems with regulating blood sugar during their first couple of days of life. When River’s blood sugar was tested it was 9! The nurses thought it was a mistake. They immediately bottle fed him with formula and tested it again and it was 24. So they quickly hooked him up to a 12% dextrose IV infusion. He was maintaining his glucose levels in the 50's and 60's so they decided to let him out of the NICU and into our room. Knowing what I know now, I can’t believe he was let out of the NICU with sugars that low!  He was only with us until 3am that night when they came to check his blood sugar, he had dropped into the 30's so back into the NICU he went. A couple days later, after his blood sugar was still low, they decided to transfer him to Dell Children's Hospital in Austin. At this point, they still thought this was just a transient problem since he was born 3.5 weeks early. He was put on a 20% dextrose IV through a PICC line because a regular IV could not support the amount of dextrose he needed to maintain his sugars. A PICC line is a central line through an artery that goes straight into the heart. His poor little veins were not large enough to withstand the amount of dextrose he needed to keep his blood sugar stable. They did various blood tests and determined that his insulin levels were extremely high. This was the first sign we had that this may not just be "transient." They told us that we could have genetic testing done to determine if this was a genetic disorder so we agreed. We were also told about Childrens Hospital Of Philadelphia and how they might be able to help him out if they were unable to ween him off the dextrose IV. CHOP has a special unit for children born with Congenital Hyperinsulinism. His endocrinologist started him on Diazoxide and with the help of this medicine, he was able to come completely off of the dextrose IV infusion. It took several weeks to get there, but we were just so happy that he was free of the dextrose IV.

We did have to keep increasing the dose of the Diazoxide until we reached the maximum for his weight. His dosage had to be increased on a bi-weekly basis while in the NICU. River was also on a continuous feed through an NG tube and never really felt hungry. Needless to say, he never wanted to drink much from the bottle. After much deliberation and discussion we finally gave in and decided to have the surgery to put in a feeding tube (g-tube). A g-tube meant we could finally take him home!

After 8 weeks of anxiously waiting, we did get the results of the genetic testing back. River has defect of the ABCC8 gene and on the specific chromosome 3992g->a, which is a defect traced back to Germany and the Ashkanzi Jews. This defect causes diffuse hyperinsulinism. I still remember getting that news, it was a rough day. It broke my heart to know that this condition would never go away and that this would be a life-long battle for our son. This was news that no mom or day ever wants to hear. I was in complete shock and it took a while for "life-long" to really sink in. I kept asking "why us, why my baby?"

After a 2.5 month stay in the NICU, River was finally able to come home. The first week at home was very stressful keeping up with his feeding schedule and medications. Once we got into a routine, it became much easier. We were just so happy to have him home where he belonged! He is now on diazoxide and is fed over an hour and a half through the G-tube. He gets Pediasure mixed with polycose and Pro-phree to add extra calories. River can only go about 1.5 hours without food before his blood sugar drops below 70. As of now, we are able to keep his blood sugar above 70 after a fasting period, although he does on occasion drop into the 60's. Our goal is to keep up with the feeding regimen and diazoxide to prevent him from having a pancreatectomy. It has been a rough road, but we are willing to do what it takes to keep his blood sugar stable. We are looking forward to what the future holds for River as we hear it gets easier after the first couple of years!