Heart Surgeries for Nora Rae
Nora Rae Pfannenstiel – our perfect little peanut – was born February 25, 2019 with a condition called Hypoplastic Left Heart Syndrome, or HLHS. October 31st, 2018 is when Mom and Dad received the devastating news that their pregnancy was going to be atypical, because a heart defect had clearly been seen during a sonogram that day at KU Med. Our stomachs dropped and our hearts broke as the world stopped spinning and put all its weight onto our shoulders. We felt alone crying in a room holding hands after having been asked if we would like to continue with the pregnancy at 20 weeks. Fast forward past 11 echocardiograms/sonograms at KU Med between October 31st and January 15th, when the cardiology team had determined that immediate steps would need to be taken following Nora's birth, or death would likely occur. So, we were referred to Children's Mercy Hospital, where we call our "second" home.
The weeks following up to delivery were hard to handle because no clear diagnosis could be made during Nora's fetal echocardiograms, and it felt as if every appointment brought worse news than the last. We had hope at the time that a two-ventricle repair would be possible and would have likely be successful, but as the appointments went by, the reality sets in that Nora will likely need a single-ventricle repair. Time has allowed us to become accepting of that fact. We did all we could do. We tackled each day as they came.
Knowing now the most likely course of action after delivery would be a sequence of three staged heart surgeries rerouting the path of the returning blood flow to bypass the heart directly to the lungs: Children's Mercy's palliative care team met with us to discuss the post-surgical complications as well as the lifelong limitations associated with "the Failing Fontan". We had the difficult discussion of which of the many post-surgical complications would cause us to re-evaluate the quality of life for Nora, because Nora is our primary focus and we want what is best. We remember telling the palliative care team how it felt as if choosing the surgical route was a selfish decision forcing Nora to endure so much so early in life. It is a hard decision that many families, they said, have chosen both paths.
Nora came to us the morning of February 25, 2019 in true Nora fashion - on her own terms: Frank breach position and an "urgent" cesarean section. There is a difference between urgent and emergency C-section, but that is a story for another day. We spent our time getting to know each other in the NICU and PICU at Children's Mercy Hospital in Kansas City in the days leading up to her first heart surgery. She had her Hybrid Stage-1 Palliation on March 6. It's hard to imagine handing Nora over to the surgical nurse to be whisked away for surgery, but Mama wouldn't stop holding her baby that whole morning.
The three of us had ups and downs during our stay in the hospital. Days passed, tubes were removed, and things generally progressed in a good direction. We even started feeding Nora breast milk from the bottle. But Nora, like so many of these heart different babies couldn't keep up on her feeds. It takes a lot of work for a baby drink milk from a breast or bottle. So, a G-tube was placed in late March to ensure that we could feed Nora at home even when she just couldn't handle the bottle. The day arrived on April 2 that we were discharged from the hospital and got to go home.
We rushed home to keep on her 3-hour feeding schedule, and then laid down on our bed with little Nora. We looked at each other and thought, "How in the Hell are we going to do this?" The first weeks (is still) were hectic! It felt as if we were running our own little science experiment: feedings every three hours, vitals twice a day, weight and video of Nora daily, as well as logging every wet and dirty diaper. We were discharged from the hospital but added into the CHAMP program. CHAMP, Cardiac High Acuity Monitoring Program, is a way for parents to relay important information daily back to the cardiology team. They look at the values and trends and are alerted with data outside a target range. This video, although is an ad for Microsoft, shows baby Winston and his family working with Lori Erickson, who runs CHAMP and whom we know well. Please watch the video, because the benefits of having this team on-call I cannot express.
Other than our cardiology/occupational therapy appointments every two weeks and sporadic physical therapy things have calmed down to a manageable schedule. Jake has returned full-time, but because of Nora's condition daycare was not advised due to sudden onset of fatal symptoms associated with HLHS Jessie has only returned to work part-time. Jessie watches Nora during the morning, and Jake comes home for lunch to take Nora to grandpa Pfannenstiel's house where Pat watches Nora until Jake gets off work to pick her up around 4pm. Rinse and repeat, five days a week. It has been a roller coaster of good times and stressful situations at home. Below are links to photos and updates of Nora's development, if you care to peruse:
Nora Rae’s Heart Journey.
Nora's next surgery, the Comprehensive Stage-2 Palliation has been scheduled for October 3rd. (Nora's second surgery will consist of the Norwood and Glenn procedures together.) Nora will come back from the all-day surgery with an open chest to keep fluid from building up pressure on her heart. She will likely be on a heart-lung bypass machine for a few days (called ECMO) to give her heart a period of rest to recover. The recovery time in the hospital has been estimated at 2-3 weeks given there are no complications.
We would like to thank all of those who have donated handmade blankets and clothing, toys and books, and your thoughts and prayers. Thanks to those who brought us food when it was difficult to find our bearings at home. Thanks to Lawrence Lawn Care for relieving the stress of finding time for yard work by mowing pro-bono for the time being. Especially thanks to our father who has graciously taken on the role of "nanny" by going part-time at work. It is a difficult time for us, but it has made our bond stronger and it helps knowing we're not alone. We had previously decided that throwing a proper baby shower wasn't what we would like to do at the time because Nora's outcome hadn't yet been determined and it seemed presumptuous that we would even have a child. As a young family, we are becoming financially stretched thin and are asking for your help. The monthly payments to both Children's Mercy and KU Med are being charged to credit cards and cannot be sustained through another year with a new deductible and a new max out-of-pocket. Anything helps. If you want to donate, if you can donate, Nora thanks you. The Pfannenstiels thank you!
We will try to keep people posted, through GoFundMe and Jessie and family's Facebook pages.