We've been on a rough ride lately. I've always felt so fortunate that Jack's surgeries have gone so well, having some minor complication, but Jack always battled on! In April we began to notice changes in Jack. His breathing was noisy. We took him to the doctors and were reassured when his lung sounded clear, stats were good and heart function was great. Seasonal allergies were the logical culprit. The weeks kept going and the noisy breathing continued past allergy season. Again more reassurance from doctors but with a plan. Four days of nebulizer treatments and if he wasn't better we would get a pulmonology referral. Days later Jack earned that referral. June 18 Jack had a cardiologist visit. He looked and sounded good, oxygen saturation was 92% as always. The following Monday I began to see marked changes in Jack's breathing and the way he was feeling. I called pulmonology to see if we could get in sooner on a cancellation And he was able to go in on Wednesday. In the office his oxygen saturation was only 86%. Thursday evening he began labored breathing and I knew we had something much bigger going on than noisy airway and a wet cough. I loaded him up and drove to MUSC. The ER was thourough and cardiology saw edema around his lungs on X-ray. He was admitted for further testing on Friday. Friday's X-ray and Echo showed large effusions on both sides of his lungs and the echo showed the culprit!
Jacks last open heart surgery was the Fontan. It takes the main vein from the leg and connects it to the pulmonary artery completing the bypass of the right side of his heart. Jack had a fenestrated Fontan where the surgeon left a "pop off valve" into the right heart to relieve pressure. Jack should always have passive blood flow through his body. Some Down's syndrome individuals (and many other single ventricle people) have higher blood/lung pressures which works against the Fontan flow causing a dangerous back up. The fenestration "pop off valve" allows that pressure to release and some blood to mix. Now that Ive explained this I can explain what's happened to sweet Jack.
Jack echo showed his fenestration had closed by his body "healing" it. We scheduled a heart cath for July 1 to reopen his fenestration and reduce the high pressures, so his chest could heal and stop creating the effusions. During the heart cath Jack had a dangerous build up of pressure and his heart sent un-oxygenated blood to all his organs. His blood pressure and oxygen dropped damagingly low. Thankfully a quick working team stopped him from getting to the point of needing compressions, got him to a stable point, placed chest tubes to relieve his fluid, and reopened and placed a stint in a new and larger fenestration. During the episode Jack's liver and kidneys were "bruised" the liver taking the biggest hit. His pulmonary bed in his lungs were shocked from the high pressure and large amounts of fluid around the lungs. His blood pressure was supported by maximum doses of three medications and was still bordering a dangerous low. His blood profusion to his hands, legs and feet was poor leaving them extremely cold. He was filled with fluid and running a fever that was not responding to Tylenol. The only good thing was his heart function.
Thursday the doctors began a method of weaning him off the blood pressure medications and ventilator. All was well until 7:30pm when Jack had a second episode like he did in the cath. It was a very traumatic experience for Jackson and I to witness and it seemed no one could answer our questions. The only thing we heard was he needed off the ventilator.
He had a third "mini" episode through the night. The staff shift changes occurred and a new cardiologist came on for the weekend- Dr. Zyblewski. In all our time here we have never had the privilege of her working with Jack. The team came to discuss Jack and she asked Jackson and I what we understood about the events and Jack's anatomy. She got paper and a pen and drew Jack's heart and explained the Fontan as I did above and then told us what has been happening. Jack has Fontan failure. That hurt. He has to have the fenestration or it causes the dangerous back ups and it could still happen with the fenestration as it did. She said if I had not brought Jack to MUSC the day I did he would likely not have made it the following day. She said many kids with Fontan failure will not make it to adulthood and most have liver issues because the liver is the first organ to hurt. Finally she explained that we had to get Jack off the ventilator. While the vent kept him alive it also worked against the Fontan increasing his blood/lung pressures putting him in a worse situation. He had a 50/50 chance that he would be able to come off the vent.
Honestly the team didn't expect he would be able to not be supported by the ventilator considering the reaction he had the day before. We composed ourselves and called the family to come be with us and see him. Dr. Zyblewski is incredibly smart, methodical and conservative. She told the team her plan and things she wanted to change and the order she wanted to do it all. Medications changed and then it was time to start weaning. The combinations she had created Jack responded to well. His blood pressure began to increase, his oxygen saturation increased and his fever broke. His hands warmed and by the evening his legs were warm again. It had taken all day but he had come a long way and was stable. Since the same team was going to be on in the morning and he was so stable she wanted to wait to pull him from the ventilator. Jackson and I finally had the ability to eat and went to the cafeteria with Hudson. When we arrived on the floor a team was surrounding Jack and he was fighting. He had woken up after lingering anesthesia wore off but Dr. Zyblewski said he woke appropriately. She again came up with her plan to go ahead and pull the vent since he ws ready.
After the pull he was agitated but was improving. Saturday she gave him tons of diuretics to pull fluid off of him from his cath trama. She added medications to try to dry up some of his chest drainage. Saturday he weaned off all blood pressure medications, nitric oxide and oxygen support. He had a restful day and night and today is looking much better than two days ago.
There are still concerns about his health. His liver is healing but slowly. His platelets are low and he may need donor platelets later today. Jack will be in the hospital for an extended period so he is going to get a PICC line tomorrow. We are EXTREMELY nervous about another round of sedation but it has to be done.
Please pray for a safe sedation and continued recovery.
