Wednesday, February 16, 2011

CHD Spotlight 3

This weeks spotlight is a true miracle. She is a little girl who defied all odds and surprised every last doctor. We spent most of our sons life together in the hospital with this sweetie and will forever be close to her family. Her story is written by her mom.

Elaina Jean Gomez-Lott

Elaina was born 5 weeks early, on January 11, 2008 by emergency c-section. At birth it was apparent that something was drastically wrong. She was transported to PCMC where she was diagnosed with Tetralogy of Fallot with pulmonary atresia, a VSD and coronary fistulas/sinusoids. She was later diagnosed with DiGeorge Syndrome.
Due to the unique anatomy with her heart, it was decided to forgo a traditional TET repair and instead do the 3 stage surgery similar to that done for HRHS/HLHS. She had a BT shunt when she was 14 days old and weighed 4lb 6oz. She did well after the surgery and was able to come home, but 2 weeks later was Life Flighted back to PCMC with RSV where she spent 3 weeks in the PICU. Finally she was able to come home again!
Two and a half weeks later she aspirated on a feeding and stopped breathing. She was once again Life Flighted to PCMC and spent 2 more weeks in the PICU. This time she was sent home with an NG tube for feedings and hopes that she would stay home until August when she would be having her Glenn. She also started having seizures caused by swelling in her brain from oxygen deprivation when she stopped breathing. Her time at home was once again brief. She was able to be with Mom on Mothers Day, and then she started having fevers and her pediatrician admitted her to PCMC for observation. Because of the respiratory problems she’d had over the winter months, we all thought that her increased oxygen needs were due to her sick lungs. She continued to have episodes of reflux aspiration, so her NG tube was moved to an NJ tube and she was placed on a continuous feed. She continued to decline and so an echo was performed and it was decided that her BT shunt had stenosed and she was in heart failure. She took a trip to the Cath Lab where they tried to balloon her VSD in order to allow additional blood flow, and buy her some time to get better before having her Glenn. This didn’t work and on her 5 month birthday she crashed and landed back in the PICU.
They had no choice but to do her Glenn the next day, even though she was sick and weak from all that she had been through. They also did a ventricular septostomy, creating one large left ventricle. The surgery didn’t go well, and over 8 hours later she came out of the OR with ECMO on standby.
She was on full ventilator settings, was hooked up to an oscillator and nitric oxide. She struggled for the next 6 days, and when Dr Hawkins went to close her chest, he didn’t like what he saw and she went back to the OR to have her Glenn redone. The surgeon also added a shunt from her aorta to her pulmonary arteries hoping to get more blood flow. They attempted to do an axial fistula procedure, but the veins and arteries in her arms were to small to allow the procedure to be successful. Once again she struggled for more that 8 hours in the OR and I wasn’t given much hope that she would pull through. The surgeon sat me down and told me that he had done everything he could, but that he didn’t know if she would even survive the night. Once again, ECMO was on standby. Four days later she was finally able to have her chest closed. She was still struggling, and was put on full ventilator settings along with nitric oxide. Her saturations did not improve much and after a very detailed echo, she had to go back to the Cath Lab for a coil occlusion to cut off blood flow to a collateral that had grown and was diverting blood from her heart and lungs. I was told that it was a long shot, and that if it was going to work, I would see the results within 24 hours. 72 hours later it seemed that this had failed, her saturations were in the 50’s and low 60’s on full support. The CICU/PICU doctors talked to me about removing her from all support and letting her go because there were no further interventions available for her. I just couldn’t do it, I felt that Elaina was still fighting and I would fight with her.
Very slowly over the next few days her saturations began to improve. Within 2 weeks she was able to be extubated. She stayed on c-pap for almost a week before being weaned to high flow oxygen. We stayed in the PICU for another 6 weeks. She slowly began to improve but I still wasn’t given much hope for her future. At that point she was on 3 liters of oxygen and her oxygen saturation levels were about 70-75%. I was told that she had severe pulmonary hypertension, caused in part by the long periods of time that she was on the heart/lung bypass machine during her 2 Glenn operations. The general concensus at that time, was that as her body continued to grow, her pulmonary arteries would not. I was told that she would get more and more cyanotic, until she was no longer able to support organ functions and she would pass away. The doctors thought that this would happen within no more than 3 months.
I was then given the option of taking her home on Hospice with a full DNR or sending her to South Davis Community Hospital with a partial DNR in the hopes that she would continue to improve. In October of 2008, after 143 days at PCMC, she was transferred to South Davis. Elaina is a fighter, and she has proved stronger than anyone could have thought possible.
She began to improve significantly, by Christmas she was down to one liter of oxygen with saturations of 75-80%. In February 2009, it was decided that she was strong enough to undergo surgery and she was able to have a g-tube placed along with a nissen fundoplication. She has continued to get stronger, and after 18 months at South Davis, Elaina was able to come HOME! She’s been home since April of 2010, and I’m loving it! She’s on oxygen (with sats of 85-90%!!!!), she has a feeding pump, but she’s not on Hospice! She still has her struggles, and Cardiology still doesn’t know what, if any, surgical options are available to her. When we saw Cardiology last summer, they told me that her heart function was in the normal range for her stage of repair, and that as long as she didn’t have an increased oxygen need, we could come back in 1 year!
It’s been 2 1/2 years now since her Glenn. I’ve gotten to celebrate 3 Christmas’s with her. I’ve been able to celebrate 3 birthdays. These are things I was told that I wouldn’t have. I am so grateful for Elaina. She is so strong and amazing. Because of her long hospitalizations, she has significant developmental delays, but she is a happy girl and is full of love. She still doesn’t eat anything orally, but she likes to drink water. She’s crawling and into everything, and I’m hoping that one day she’ll be able to walk.
She turned 3 in January and just started pre-school. Due to her immune issues, she is home schooled for the time being. She should be able to go to the classroom later this spring. She loves to watch Disney movies, her favorites are Cinderella, Monster’s Inc., Finding Nemo, Little Mermaid and Beauty and the Beast. She likes other movies as well, but those are her favorites and are guaranteed to make her giggle. She also loves to play with her toys and with her big brothers. She is almost always happy and smiling. She is a blessing to our family!


1 comment:

By the Bluegrass said...

Oooooh, what a little beauty! I hung onto every word of her story written by her mom! What a strong will she has. I will always think about her and her family and will pray that she will continue to get stronger and stronger! Thank you for sharing.