Iris Wolf Updates on baby Iris

On 1/9/20, we had our mid-pregnancy anatomy ultrasound (a week or so late due to our travel at Christmas) and found out that our baby girl Iris, due on 5/10/20, has complex congenital heart defects including a hypoplastic mitral valve, smaller left ventricle and VSD (ventricular septal defect). We created this page to share updates with family and friends, especially when Iris is in the hospital. We decided to have Iris born in Cincinnati instead of Seattle in order to have the support of family to care for our older son, Henry, during her hospitalization.
Iris was born on 4/17/20. She spent 2 days in the CICU for monitoring of her heart, then was transferred to the NICU because her heart function was good but she had a cleft palate and needed to work on feeding. She spent 19 days in the hospital and came home with oxygen for obstructive sleep apnea and an NG tube to help with feeding. During her NICU stay, it was determined that she also had other heart defects including DORV (double outlet right ventricle) and an ASD (atrial septal defect).
On 5/26, Iris was diagnosed with a rare genetic syndrome called Kabuki Syndrome that explains many of her seemingly unrelated issues of left-sided heart defects, cleft palate, micrognathia/retrognathia (small recessed chin), obstructive sleep apnea, unusual ears, small size, sacral dimple, etc.
Iris was home for 25 days. On 5/30, she returned to the hospital due to decreased oxygen saturations. On 6/10, she had open heart surgery to place bands on her pulmonary arteries and open up the atrial septum of her heart, to keep too much blood from circulating to her lungs. She was in the hospital for 27 days after a complication from her intubation for surgery.
On 6/25, she came home. 2 days later she suddenly threw up and appeared to stop breathing. She was taken to the hospital by ambulance. The cause of the episode was never fully determined, but she had surgery on 7/8 for intestinal malrotation (Ladd’s procedure) and a Nissen fundoplication and g-tube placement for reflux (in hopes that this would prevent future episodes). She had many complications from this including wound dehiscence and infection/sepsis.
On 7/21, she suddenly went into respiratory distress/arrest on the cardiac stepdown floor and her heart rate dropped, requiring chest compressions. The cause of this was never determined; it may have been cardiac related, or septic shock. She was put on a ventilator and on medications to keep her blood pressure up. An echocardiogram showed that her left ventricle was becoming smaller and not filling with blood, so she would need a Norwood procedure to convert her heart into a single ventricle. This surgery was reschedule multiple times due to concerns about possible infection and her lungs looking cloudy on x-ray.
On 7/31, the attending CICU doctor sat down with us and told us that Iris would probably never be well enough to go home from the hospital - her pressures in the right side of her heart were too high, showing right ventricular diastolic dysfunction (the right side of her heart was not relaxing enough and had become thickened and stiff). She would be unlikely to survive the Norwood operation, which requires the right side of the heart to support circulation to the body. If she did survive the surgery, she would probably have to be on an ECMO machine (heart-lung machine) and would be unlikely to be able to come off of it, and even if she did make it off ECMO, she would probably not be a candidate for the next surgery she would need (the Glenn), and would not survive for long and would be very sick the whole time. We decided to start comfort care for Iris.
On 8/1, after a day of visits from family, we stopped the cardiac medicines that were supporting Iris’s blood pressure, expecting her to pass away. Her blood pressure dropped a little bit remained stable throughout the night. On 8/2, we had her removed from the ventilator and she was breathing fine on her own, and we were allowed to bring her outside for the first time since 6/27.
On 8/3, with the help of the palliative care doctors and Starshine Hospice, we brought her home for the last time. We spent a good week with her completing “bucket list” activities and spending time as a family at home. She passed away on 8/13 in her parents’ arms outside in Grammy’s backyard.
Iris spent a total of 38 days of her 118-day life outside the hospital. If you count the pregnancy, she was with us for exactly one year.