Friday, December 26, 2008

Kenzie's Story

This post is a long one so be forewarned.

Kenzie Jade was born November 5, 2007. She was full term and able to go home normally with her parents (us), Amy and Jordan. From day 4 of life Kenzie had trouble digesting breast milk. She would vomit most of her feeds and by two weeks old she had gone from her birth weight of 6.2 lb to 5.8 lb. Kenzie was then admitted to the hospital for "failure to thrive". Between 2 - 3 weeks old, Kenzie's weight plummeted to 5.0 lb as she started to have massive diarrhea. At 2.5 weeks old the doctors at Schneider’s Childrens Hospital in Long Island put in Kenzie's first NG tube (a feeding tube that goes in through the nose), performed an endoscopy, and took Kenzie off breast milk and switched her to a specialized formula. Kenzie did well and was discharged from the hospital 5 days later with her NG tube in place. Over the next 2 weeks Kenzie really responded and her NG tube was pulled. At this point we thought the horrible stench of her new formula was the worst thing we'd have to deal with. She drank her formula by bottle and thrived.

At about 5 weeks old her health started to fail again and she was back below birth weight. The NG tube was put back in and a week later at 6 weeks old, Kenzie was readmitted to Schneider’s. On Dec 29, 2007, Kenzie underwent a surgery to place a broviac line into her vein. The broviac line is a special type of IV that’s able to provide TPN (total parenteral nutrition). Although TPN was able to keep Kenzie alive and provide her with the calories she needed to thrive, it carried many potential complications. We had her transferred to Columbia Children’s Hospital during this admission where she underwent one more surgery to insert a different broviac line. Recently, I've looked back at emails from this time and I realize that again, the way we got through this difficult time was that we really believed this would all be over within a few days. A month later we went home.

We managed Kenzie's TPN at home for 7 months. During this time Kenzie had a few colonoscopies, a surgery to insert a GTube (a more permanent tube to feed her straight in her belly) and she stopped eating by mouth. Right around her 3 month birthday Kenzie started refusing the bottle. She would force herself to gag and vomit when we put it near her mouth. We began intensive feeding therapy but Kenzie always got worse rather than better. Time passed and Kenzie was never able to tolerate more than 7 ounces a day of the formula they had her on. She was hooked up 24 hours a day to her GTube and 10 hours every night to her broviac line. We also noticed swelling in her face which became progressively worse. Kenzie was in and out of the hospital for rule out line infections (every time she had a fever of 100.4 or higher - which happened often... she was automatically admitted for 48 hours) from January thru June.

On June 6, Kenzie’s swelling was so severe her face was unrecognizable. In addition, she also had a fever of 103.8. We took her back to Columbia and she was diagnosed with SVC syndrome. SVC syndrome occurs when the main vein(superior vena cava) that leads to the heart is completely clotted off. This is extremely rare in infants and is a life threatening complication of the Broviac line. In other words, the very treatment we needed to keep Kenzie alive was killing her. Kenzie was transferred to the ICU and because she was so swollen she was kept intubated for over a week. She had 4 interventional radiology procedures in the interim, and on June 13, 2007 she had 2 stents placed to keep her veins open. While in the ICU, fluid accumulated around her lungs requiring a drain. Following her second procedure, Kenzie’s blood pressure and heart rate dropped. Blood pooled around her heart(requiring a heart drain) and while she was being wheeled back into the ICU she started to crash. Thanks to the great doctors at Columbia, they were able to save Kenzie's life yet again. Her swelling remarkably subsided and she went from a grand total of 11 tubes in her tiny body down to two. Kenzie spent 20 days in the hospital and again went home again on TPN. However, this time she was on a different formula that, for the first time in her life, she was able to tolerate! For the next 6 months we gave Kenzie shots of blood thinners twice daily to prevent her veins from clotting again. A month after the nightmare in the ICU, and exactly 7 months after her first broviac line was placed, Kenzie was taken off TPN. She was finally taking all of her calories by formula through her GTube! Kenzie went in for another surgical procedure July 27, 2008 to have her broviac removed. She continues to gain weight slowly and steadily from formula now with only 1 tube in her (her GTube)! We give her infusions once a week of immunoglobulins because her immune system is weak. Kenzie still has no diagnosis, despite several colonoscopies, endoscopies, neurological and cardiologic workups, immunologist visits, genetic tests, etc. We are currently looking into a genetic disorder but tests always come back normal for Kenzie.

For months we have tried to get Kenzie to eat by mouth, but to no avail. An unfortunate, yet common and sad side effect of GTube fed kids is that become afraid to eat. She fights us when we try to feed her anything. She screams, cries, gags, vomits, and pushes us away. Kennedy Krieger Institute has an intensive feeding therapy program designed for children like Kenzie. They have an amazing success rate at getting these kids to eat. We know it’s going to be a hard 8 weeks as we need to stay inpatient at John Hopkins Hospital in Baltimore. However, we feel it’s our only chance to give Kenzie a normal life. This oral aversion she suffers from is not something kids "just grow out of". When left untreated, the aversion gets worse and the behaviors amplify. We are hoping that since Kenzie is so young (their average age is 3 for children in the program) she has a better chance at success. Our personal goal is to get Kenzie eating enough pureed foods that she only needs GTube feeds at night. Anything less will be upsetting, anything more would be a dream. So in the next two weeks we will embark on this journey and as hard as it’s going to be, we have faced worse in the last 13 months.

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