The Glenn is the second in a series of three surgical procedures to mitigate the effects of hypoplastic right heart syndrome. In the most basic of terms, it involves ligating the BT shunt (which was placed during the first procedure), then connecting the superior vena cava to the right pulmonary artery. This allows blood to passively flow (by gravity) from the head and upper limbs into the pulmonary artery where it drains to the lungs for oxygenation. Once oxygenated, the blood is returned to the heart through the pulmonary vein where it flows through the left atrium and ventricle and is pumped through the aorta to the rest of the body. In Josephine's case, the operation also involved closing off the two large collaterals she had developed to insure that there would not be competing blood flow. The period between the Norwood (1st procedure) and the Glenn (2nd procedure) is considered the time of highest risk for an HRHS baby. Up to 1/3 of kids develop complications and die during "interstage". Making it to the Glenn is a huge accomplishment. Life after the Glenn, we were told, is considerably more stable. Like night and day. I wish this were true for us so far.
According to the surgeon, Josephine did very well during her surgery on Thursday morning. We arrived at the hospital at 5:30 a.m., were checked in by 6:30 and off to surgery by 7:30. At around 11:30, Michael and I were called to one of the consult rooms where Dr. Forbess soon arrived to let us know Josephine's status. Josephine was taken from the OR directly back to her room in the ICU where the nursing staff got everything situated. X-rays and ultrasound tests confirmed her lungs and heart were looking and functioning well. She was intubated and had two cardiac lines, one set of pacemaker wires, a foley catheter, three IVs (placed in both hands and her right foot), and an internal jugular line in her neck.
As it was explained to us, extubation generally occurs very quickly after the Glenn. This is due to the fact that the ventilator causes increased pressure in the lungs which makes it difficult for the Glenn circuit to operate fully. (Too much pressure means the blood cannot flow as easily from the head and upper limbs to the lungs...which means there is less oyygen making it into the blood circulating to the body. The overall result can be decreased oxygen saturation levels.) Most kids are extubated within the first day, and many are released to "the floor" by the second or third day post-op. By the end of the day on Thursday, Josephine had been extubated and was doing well, save a few areas of decreased function in her lungs. Michael and I were thrilled that things - for once it seemed - were going according to plan.
Overnight Thursday to Friday morning, Josephine worked harder and harder to breathe. Her tiny chest rose and fell rapidly, and tugged at her tummy and diaphragm which heaved in and out with every breath. A look at her x-ray and labs revealed increasing areas of collapse in her lungs. Talk of re-intubation began early in the day. After attempting to provide support via a small nasal cannula and a larger RAM cannula, the decision was made to put Josephine on CPAP. (In case you've never seen it, CPAP looks a bit like some sort of medieval torture device. Air is delivered through a small mask which seals around the nose...not too bad. But in order to keep it correctly positioned, a series of velcro straps must be positioned around and across the head, with another extending under the chin to keep the mouth closed. CPAP looks like the ugly second cousin of old fashioned orthodontic headgear. And Josephine hated it with a passion.) While we weren't terribly happy about the discomfort CPAP seemed to be causing, we were happy to have averted reintubation. By the end of the day, things were turning the corner. Josephine's lungs were starting to reinflate and her blood gas numbers improved.
On Saturday morning, Michael left the hospital at 6:20 a.m. to meet a delivery person at the house. Several weeks ago during a particularly brief but violent thunderstorm, a power surge caused the electrical striker on our gas stove to short out. Rather than pay half the cost of a new unit to repair the 20+ year old model that came with the house, we opted to purchase a new stovetop. We had hoped to have it delivered and installed before Josephine's surgery, but that didn't quite happen. Josephine and I (along with our cadre of medical staff members) started the morning optimistically. Although she was sleeping much of the time, I read her a couple stories and attempted my version of music time (which amounted to me singing along to music sputtering in on the hospital's much-too-slow internet connection.) Aside from the occasional squirming fit (which I assumed was brought on by discomfort in some capacity), Josephine was doing well. Her saturation levels were running in the high 80s and low 90s. She seemed to be breathing almost effortlessly, at a moderate pace with no abdominal pulling. Everything was good.
Around 9:00 Josephine and I received Michael's text that he was driving back to the hospital. "All's good" I texted back, noting that the nurse and I had discovered a small amount of blistering and skin breakdown behind Josephine's ear. (Unfortunately, she has her momma's sensitive skin.) A short while later, Josephine woke up. It was clear to me that she was very uncomfortable. In addition to her normal squirming, she was arching her back and jolting her head back and forth. With each wiggle, she inched her CPAP mask further and further off of her nose, eventually moving it all the way to the side where it pressed into her face, hissing uselessly. I quickly repositioned the mask and continued to pat her back gently, hoping that she would settle and drift back to sleep. The absolute last thing I wanted was for her to be awake and in pain or scared. Although I desperately wanted to see her open her eyes and smile at me for the first time since surgery, sleep - painless and stress-free - seemed like a much better option in this situation. When she didn't respond to the normal repertoire of mommy-interventions (patting, shushing, sleepy sheep ocean sounds, placing her paci within distance), it occurred to me that she had not had any pain medication or anything for anxiety in several hours. I stepped out into the hallway and signaled the nurse, who had just stepped away for a moment to grab some breakfast. By the time the nurse arrived at Josephine's room 30 seconds later, Josephine's oxygen saturation levels had started to drop. All morning, she had been hanging out in the high 80s oxygen-wise. And now, she was at 75 and falling. The nurse called for a respiratory therapist, who happened to be close by. The respiratory therapist quickly pulled the ventilator flow tube attached to the mask and began to ventilate using the hand ventilation bag. Woosh...wooosh...woosh. Josephine was getting more and more agitated. Woosh..woosh...woosh. At that point, the respiratory therapist decided to suction Josephine's mouth to see if there were secretions making airflow more difficult. She stuck the suction tube into Josephine's mouth and I could hear what sounded like small amounts of saliva being pulled into the vacuum tube. Josephine looked horrified. Her eyes had snapped open and her face was slowly but steadily turning from a deep red color to purple. "Get me the deep suction tube," the respiratory therapist called calmly, but firmly. The energy level in the room began to rise. Grabbing another plastic-wrapped package out of the in-room supply drawers, the nurse opened the window to the adjoining room and said, "Get Dr. Moreland, please." Several nurses and an additional RT had entered the room and were moving around with purpose. Dr. Moreland then rushed in and began barking orders. By this time, Josephine's saturation level had dropped below 50. As they worked trying to re-establish her airway, I watched helplessly as she flailed around, oxygen saturation level finally landing at 19 as they pushed a paralytic through her IV and placed a new endotracheal tube. What had been our largest success in the past 36 hours had now entirely been undone in a matter of mere minutes. Josephine had been reintubated and was no longer breathing on her own. I could do little more than stand in the corner shaking and crying.
Over the past 24 hours, Josephine has been struggling a little with varying levels of lung collapse. As happened during our first stay, they are able to reinflate one side seemingly only at the expense of the other. They've sent off cultures of the secretions pulled from her mouth and throat, and so far the results have not indicated infection is present. Early this morning a nasal swab was taken to determine if she may have a viral lung infection. So far, nothing.
At this point - although intubated - she seems to be resting somewhat comfortably. Her oxygen saturation levels are in the low 90s, which is fantastic. The plan for today is to continue respiratory therapy in an attempt to get both lungs to reinflate. They expect that may happen by tomorrow. Best case scenario, the doctor says, is that if everything looks perfect we may be able to extubate by Tuesday or Wednesday. I realize it's only a matter of days, but it feels like a lifetime.
Having a child in the hospital is full of ups and downs, long-awaited triumphs and dismal disappointments. On one hand, a successful plication surgery and subsequent reinflation of a lung that has struggled for weeks to heal. On the other, a chylous effusion followed by a repeat lung collapse. Up then down. Up then down. I won't lie. Staying even somewhat positive is a constant challenge.
Although today is Thanksgiving and being in the hospital isn't exactly how we had originally planned to celebrate the day, we have much for which to be thankful:.jpg)
Turkey dinner? Who needs it.
