“LuluBelle was born 06-20-2018. She was not diagnosed in utero, so 8 hours after she was born I was given the worst and most surreal news I have ever received.
The echo tech was so kind, his name was Terrence. At first he was so talkative, and then his silence began to feel the room. As the echo progressed on the minutes began to feel like hours and the sudden realization that bad news was coming began to sink in. My mom is a NICU nurse and before that day I had never really seen her truly scared about something medical. Dr. Reddy walked in moments later, and began to draw a picture of four chamber heart. I still remember feeling like I was watching myself get this news. The whole room was silent and his mouth was moving but it felt so foreign. He began to label her problems, most severe to least complex.
1. Severe aortic stenosis
2. Large posterior VSD
3. Severe coarctation of the Aorta
Within minutes, my biggest worries of will she breast feed, will we go home tomorrow, and I hope she sleeps well, became I hope she lives. She was shuttled off to the NICU to get her lines and medicine to keep her PDA open. I check myself out of the maternity ward and we transferred to CHLA.
When we got to CHLA we met with Dr. Hill. She explained that she was more of a electrician(electrophysiology) but she was covering for another doctor. She walked us through all of LuluBelle’s testing, blood work, new echo and she delivered the same horrible news again. Surgery was four days later June 25th. She had an incredible surgical team and Dr. Starnes was able to correct all three issues in one surgery. She came out of that surgery with some heavy bleeding and loss of electrical conduction between her atrium and ventricles. She had spontaneous conduction on and off for 14 days and then it went away. She was given a pacemaker and it turns out we did need Dr. Hill after all. She was discharged home 25 days after she arrived and only on aspirin and ibuprofen. July- October was fairly easy, follow up echos, cardiology visits and only great news. Flawless surgery, great outlook, be grateful.
The first week of October the unexpected happened, which is the worry with CHD kids. Stable can become unstable in days, hours or minutes. We though LuluBelle had caught a cold or RSV. Her breathing was so fast, when we got to the ER they ran tests for heart failure and LuluBelle’s number was in the thousands. They transferred us back to CHLA and there LuluBelle had an emergency cath. Her aorta had re-narrowed and to the degree her left heart function was severely declining. We met our current cardiologist and favorite anesthesiologist that stay. She came home a week later, but with many more meds. Sildenafil, Enlapril, Lasix, and We went to CHLA every two weeks for a long time and then every month. The watched her closely and I can happily say that today her left heart function is normal again.
Over the time from October to April LuluBelle was diagnosed with left pulmonary vein stenosis. Her left pulmonary vein combined so instead of having two she has two that turn into one. It is compressed by her aorta which makes it more difficult. She has right pulmonary artery stenosis and her pulmonary conduit from her original surgery was severely narrowed and leaking. She was scheduled for sure in July and at that time she had her new pulmonary conduit placed and some adhesions around her left pulmonary vein removed. She was able to come off of Lasix which is awesome news!
In September the pulmonary vein, despite the removal of adhesions had stenosis again. I reached out to Boston Children’s because they have a specific pulmonary vein stenosis program. They recommended we get her a lung scan to check the perfusion to her lungs, to see what the impact of the narrow pulmonary vein is. Her right lung is receiving 77% oxygen and it is slightly larger then her left. Her left lung is receiving 22% oxygen and is being affected by the stenosis in her vein. She is scheduled for a diagnostic cath in January. She had a rare condition in the pulmonary vein stenosis and right now she is sort of an enigma until we can get solid answers from her cath.
As a heart mom LuluBelle has changed my life. Her resilience, her happiness and her love are unbelievable. She breaks barriers everyday, conquering a new milestone, or a new skill. She had struggled with eating her whole life, she had a brief stint with a NG tube and high calorie formula is her jam. Her struggles they don’t define. They just strengthen her, they make her unique and amazing. They make people around her thankful for the small things in life that you took for granted. She is walking beacon of hope when it seems pretty hopeless. We are so grateful for the God we serve. He gave us LuluBelle and he wrote this story that while it’s so overwhelming, scary, stressful, hard and dark sometimes, is full of deep real love, extreme joy, unending gratefulness and lessons in faithfulness. Thank you for sharing our story and helping to raise awareness about congenital heart defects! These warriors are the most amazing kids and the world needs to hear their stories.” – written by her mother, Courtney. December 2019