Pregnancy + Diagnosis During a routine ultrasound at 23 weeks, Josiah and Carly discovered that their precious baby girl had a severe congenital heart defect called Aortic Stenosis: a narrowing of the aorta. It is a fatal heart defect without intervention as it develops into a more severe condition called Hypoplastic Left Heart Syndrome (HLHS).
Boston + In Utero Surgery On April 13th, 2018, J&C traveled to Boston Children's Hospital and Joanna underwent an in-utero fetal cardiac intervention (FCI) operation called aortic valvuloplasty where the aortic valve is ballooned open to try and save the left ventricle from becoming hypoplastic (where it gets damaged and stops growing and developing and basically becomes non-functioning).
July 11, 2018 - Birth During the duration of the pregnancy it was unclear if the in-eutero procedure stopped the progression of Aortic Stenosis to HLHS. Joanna Marilyn McClure was born on July 11, 2018 at Washington Medstar Hospital in D.C. and immediately transferred to Children's National Medical Center next door. When Joanna was born her left ventricle was undersized and wasn't functioning normally, but she did have a left ventricle. Instead of treating Joanna as a straight forward HLHS patient and performing the 3 staged surgeries HLHS patients usually get (the Norwood, Glenn, and Fontan surgeries) Joanna's medical team decided to try a hybrid surgery approach instead.
2 Days Old - Hybrid Surgery (Surgery #1) At two days old Joanna underwent hybrid surgery where her ductus arteriosus was stented open and her pulmonary arteries were "banded" (basically crimped). This temporary circulation would allow Joanna to grow and get stronger in the hopes her left ventricle would also grow and "normalize." So for 7 months Joanna had weekly cardiology appointments in D.C. to monitor her circulation as well as her left ventricular function. Because this hybrid circulation is so unstable, Joanna was hospitalize on a monthly basis between August and February which is pretty much par for the course with.
February 2018 - Comprehensive Stage II Surgery (Surgery #2) As the months went on it was determined that Joanna's LV was not going to be able to do the job it needed to do. Joanna was a full-fledged member of the HLHS club. On February 8th, at 7 months old, Joanna underwent open heart surgery called "Comprehensive stage II." This is a combined surgery of the Norwood and Glenn surgeries. In a healthy heart, the LV sends blood out tot he body through the aorta and the RV sends blood out to the lungs. Since kids with HLHS only have one working ventricle, the RV, surgery re-plumbs the heart where the aortic valve is connected to the only working pump, the RV. So the RV becomes the "body pump" and the lungs now receive blood "passively." This just means since the lungs don't have blood being actively pumped through them, they simply allow the blood to flow through passively.
Joanna recovered quickly from that massive comprehensive stage II surgery and was discharged from the hospital on February 22, 2019, two weeks from the day of surgery.
Until recent weeks, Joanna has done well. She was finally able to gain weight and began hitting some of the normal baby milestones: two bottom teeth, began to eat solids, roll over, sit up on her own, etc. For the first time, the McClure family was finally able to resume some sense of normalcy as the family adjusted to a "new normal" with a child with a congenital heart defect.
June 6, 2019 - Poor Right Vent Function + Catheterization A few weeks ago at a routine cardiology appointment, Joanna's cardiologist noticed that Joanna had decreased right ventricular function. This simply means the squeeze of the RV wasn't right. Echo's didn't show any obvious reasons for the decreased function so Joanna was scheduled for a catheterization this past Thursday. The cath confirmed that everything from surgery looked great: her reconstructed aorta, pulmonary arteries, coronary arteries, etc all looked great. This would be good news if it weren't for the fact that there was no explanation for the decreased RV function. After Joanna came out fo the cath lab and was monitored overnight on Thursday, she came home Friday morning.
June 2019 - 18 Day Hospital Stay Between Friday and Sunday, Joanna grew increasingly uncomfortable and agitated to the point she was up ALL of Saturday night. So on Sunday afternoon around 1pm Josiah took Joanna back to Children's National. She was checked into the Emergency Department and then admitted into the Heart and Kidney Unit (HKU). The HKU began connecting IV's to give fluids as they began running tests as well as giving oxygen for breathing support. Tests came back positive for rhinovirus (common cold) and enterovirus. These could explain Joanna's low sats (blood oxygen saturation) as well as overall poor appearance (cold, clammy, sweaty, purple hands/feet/lips, etc). Through the night into early Monday morning Joanna required more and more breathing support to the point the HKU is not equipped to provide.
Cardiac Shock At 1am Joanna was upgraded and transferred to the Cardiac Intensive Care Unit (CICU). In the CICU they are able to provide breathing support with oxygen on high-flow. Monday morning Joanna became increasingly agitated and the team gave her morphine to help her relax and rest. For a few hours Joanna did rest comfortably until the early afternoon when she began to ramp up and become inconsolable. Her vitals signs became troubling with low sats, high heart rate, fast breathing rate, etc. Instead of masking her symptoms with more pain meds, the team decided the best thing to do would be to sedate and intubate Joanna (place a breathing tube). The team prepped for sedation and intubation and everyone took their places. After administering gas, a breathing tube was placed and the doctor manually controlled Joanna's respiratory rate by hand squeezing a bag. The team waited for Joanna's vital signs to come back up but instead she took a hard crash as everything bottomed out. For the next 10-15 minutes the team scrambled to administer powerful drugs to kickstart Joanna's heart and raise her blood pressure. Slowly Joanna's vital signs did pick back up as she stabilized. This was a terrifying moment. After the fact, the doctor explained that Joanna's heart never stopped but she did have an incredibly weak pulse. If the team had waited any longer to intubate, she would have probably needed CPR and possibly would have ended up on ECMO (a heart and lung machine).
It's hard to comprehend that Joanna isn't in the hospital because of her heart. Her broken heart doesn't help matters, but it's a combination of viruses and septic shock/bacterial infection that has overwhelmed Joanna's weak systems. Colds and viruses that make a heart healthy kid feel sick, land "heart kids" in the hospital. The motor (their heart) that drives all of their body systems isn't able to power through sickness like normal healthy people. Joanna was discharged on June 27th after 18 days in the hospital.
October 5, 2019 - Stroke Saturday was one of the best family days the McClure's have had since Joanna was born. They went to the Joshua's Hands Fall Festival and enjoyed the beautiful fall weather, carnival games, food, puppet shows, live music, and barn yard animals. It was what any family would consider a "normal" family day. Once the McClure's got home around 2:30, Carly put Joanna down for a nap and as Carly turned around to leave, Joanna let out a whimper and as Carly turned around she noticed the right side of Joanna's face was drooping and her right arm seemed pinned underneath at an awkward angle. As Carly scooped Joanna up, Joanna was completely limp. Joanna was having a stroke. Carly rushed Joanna to the local Inova Loudoun ER while Josiah figured out childcare for Gabe, Auden, and Ellie before joining Carly. From the Lansdowne ER Joanna and Carly were airlifted to Children's National Medical Center in DC and Josiah went home to be with the older 3 children. At CNMC it was discovered from a CT scan that Joanna had a blood clot in her carotid artery which caused her stroke. Early Sunday morning, Joanna was airlifted to Johns Hopkins hospital in Baltimore, Maryland where they are known for advanced care in neurology. An MRI at JH's did confirm a stroke. There she underwent an unsuccessful thrombectomy procedure to try and remove the blood clot. After JH's was unable to successfully remove the clot, Joanna was transported by ambulance back to CNMC on Monday evening so Joanna could be back with her cardiac team that really knows her the best.
October 9, 2019 - Collapsed lung + Stressful Intubation Joanna was extubated on Tuesday evening and had a stable night into Wednesday. Throughout the day on Wednesday Joanna was pretty lethargic and not herself. At some point during the day, an X-Ray showed a partially collapsed right lung. The team hoped to be able to treat her collapsed lung with aggressive chest PT and high flow from the nasal cannula. But as the day progressed, she seemed to be going downhill to the point that her blood oxygen saturations levels or "sats" were dipping into the 50's (she's normally in the 70's-80's). The team decided to re-intubate Joanna to give her the extra respiratory support she needed. Everyone got prepared for intubation as they began to administer sedatives and paralytics. Once Joanna was fully sedated and paralyzed, the attending doctor placed the breathing tube and began bag breathing for Joanna. This is when things became VERY stressful. After placing the breathing tube and giving oxygen and nitric oxide, Joanna wasn't responding. In fact her sats dropped into the 20's. The attending doctor removed and replaced the breathing tube 2 more times thinking tube placement might be the issue. When this didn't work they called the anesthesiologist up to place the breathing tube. This 4th attempt didn't immediately raise her sats but as they gave Joanna time to respond, her sats slowly (and finally) began to pickup. During all of this ordeal, one of our favorite attending doctors was called in from home. He arrived shortly after the scariest moments and began to assess the situation. After making a few small changes, her sats immediately picked up into the 80's. The thinking is that all of these respiratory and sat issues were related to Joanna's collapsed lung, and not cardiac related. The ventilator will be able to apply air pressure to expand and inflate Joanna's collapsed lung.
After being intubated, Joanna had a quiet and stable night. She was stable all of Thursday and was taken for another MRI. The MRI does show slight change from the last MRI. It shows “progression” where outer edges of the affected stroke area have expanded. Thankfully there aren’t any new brain areas that show signs of stroke and he said infants who experience stroke can really bounce back to show almost no effects because of the neuroplasticity of their brains. Neurology won’t be able to do more of an assessment until Joanna comes out fo sedation and intubation, hopefully tomorrow. Then they can do an evaluation and give a projection of how they anticipate her recovery.
The goal now is to have a stable night and then beginning to ween ventilator settings and sedation medications tomorrow. If Joanna responds well then the team will move to extubate her tomorrow.
Prayer + Support Please keep Joanna in your prayers as she begins the slow road to recovery. As you pray for stability with her vital signs, pray that her right ventricle regains total and completely normal function.
In addition to your prayers, your financial support will greatly alleviate medical expenses above and beyond insurance coverage.