Maddie, born 2008I never thought I'd know so much about the human heart, especially what could go wrong with it, but when our third child was prenatally diagnosed at 26 weeks with a CHD, a new world opened up for us. Our baby's heart may have been broken but our hearts were shattered that day. We learned that without intervention, she would not survive and we were given three options: termination of the pregnancy, a three-staged surgery, or the comfort care method which was to take our baby home and let her pass away. We were lucky to have the surgical option and chose the three-staged operation route. Our "heart journey" quickly began.
Madison Grace, "Maddie" decided to unexpectedly join our family on March 13, 2008, arriving a month early by an emergency, crash c-section at the University of Washington Medical Center. Her birth was a very scary experience and we all feared the worst with her early arrival, but we quickly learned that our 5 pound baby girl was tough and would overcome any obstacle that came her way! After stabilizing Maddie, she was transferred to Seattle Children's Hospital CICU Unit where her diagnosis was confirmed: Pulmonary Atresia with Intact Ventricular Septum, Ebstein's Anomaly, Atrial Septum Defect, Patent Ductus Arteriosus and Hypoplastic Right Ventricle. In short, the right side of her heart was severely underdeveloped and simply non-functional.
On day 6, Maddie underwent her first open-heart surgery, the BT Shunt which basically created a bypass to get the blood to her lungs for oxygenation since her own anatomy did not suffice. She did fairly well post-op and after spending her first 19 days of life in the hospital, she was discharged and finally able to come home! It was a bitter sweet moment for us. Maddie needed a feeding tube the first few weeks after coming home but finally regained enough strength to orally bottle feed. But we knew that our stays at Children's were not over as in 4-6 months, she would outgrow the shunt and need another surgery in order to survive.
At exactly 4 months of age, Maddie underwent her second open-heart surgery, the Bi-Directional Glenn. She cruised through her recovery and was home just 5 days after another big heart surgery! During this stay, we also learned that Maddie's kidneys were not growing symmetrically and our appointments with Nephrology began. She had a VCUG and this showed grade 3 and grade 1 kidney reflux. Luckily, she hasn't had any UTI or kidney infections and we've been told this is something she should outgrow. For the meantime, she is on an antibiotic for UTI prophylaxis.
Since her second open-heart surgery, Maddie has been growing and thriving. She is developmentally on track despite her early birth, hospitalizations and procedures she's endured. By looking at her, you would never know she has such a special heart. She is cyanotic since her oxygen saturations hang around 74-78. She is now on only 2 medications and we visit her cardiologist every 6 months. She's had one cardiac catheterization since her Glenn to coil off some veno-venous collateral vessels that were causing a steady drop in her 02 sats. We are now waiting for the Fontan. Upon completion of this stage, her heart will function as a single ventricle, the left side of her heart doing all of the work while blood just passively flows to her lungs.
Even though we are not "out of the woods" yet and coping with a heart defect is not easy, Maddie has shown us a strength that we can all admire and strive for. She is our little miracle and brings such HOPE to our family!
UPDATE February 2011
In May 2011, Maddie underwent the final stage of her palliative repair, the Fontan. Prior to this surgery, I was more worried about the toll it would take on her mentally than I was with the physical aspect of healing. But in typical "Maddie style", she had a fairly smooth recovery and handled it all very well. She was readmitted with pleural effusions after being home for a week, but our total time spent in the hospital after was just 2 weeks. She was such a trooper through it all. Her heart now functions as a single-ventricle, the left side of her heart doing all of the work while blood just passively flows to her lungs. She will be a single-ventricle or half-heart for her lifetime.
We have seen so many changes in her since her surgery. She had a growing spurt, her personality really blossomed and she's so outgoing! Prior to surgery, she was very tired, sluggish and just didn't feel well so this change in her has been so incredible for us to see. She's a spitfire somedays...and we welcome that energy around here. Her heart agrees with the Fontan and we couldn't be more grateful. We are thankful to her surgeon, Dr. Mike McMullan at Seattle Children's as well as her cardiac team, for giving Maddie every opportunity to live a happy & healthy life!
Story by Maddie's mom, Katie - Washington
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