We had done a lot of reading, and found out about a procedure where small chest tubes could be placed while still in the womb, and discussed this with our perinatologist—he felt it was "not worth the risk", believed Michael's lungs were hypoplastic and would not expand at all. Arguing basic hydraulics, we felt some expansion HAD to occur upon relieving pressure, but whether it would be 1% or 50% we had no idea. This discussion upset our perinatologist to the point where he yelled at us in frustration: "listen, this baby is gonna die no matter what you or I or anyone else tries to do, and frankly, I don't know how to deal with you people..."
Not ready to give up hope yet, on Saturday 4/11/98, we were trying to arrange a transfer to OSU University Hospital to see about getting the tubes placed to drain the fluid/pressure from around his lungs. We had talked with a doctor there that said he also thought chances were slim, but agreed some expansion could occur, and was willing to take a look at doing this if we could transfer to Columbus.
Easter Sunday 4/12/98, we found out God had a better plan. Paula's water broke, and we really knew that short of a miracle, Michael's options had run out. With every contraction, the pressure in Michael's chest was dramatically slowing his heart rate.
After 11 hours in labor, Michael's heart rate dropped to under 100 and our perinatologist decided to quit pushing, and performed an emergency C-section. Michael was born at 8:10 pm, with an Apgar score of 1 and a heart rate of 47. Dr. Okah from Children's was there at birth to place chest tubes, and when he did, they drained 120cc, and Michael's supposedly hypoplastic lungs suddenly were at 90% of normal! What a day for a miracle!
Michael was taken to Akron Children's where he was placed on an oscillating ventilator. Dr. Lin at Children's did an echo and suspected a heart defect. Michael was transferred to Rainbow Babies for a higher resolution test, and diagnosed with Total Anomalous Pulmonary Venous Return (TAPVR).
At nine days old, Michael had his first surgery—a pouch formed by his pulmonary veins was split open and sewn over a hole cut in the back of his heart, an anomalous vein was banded to limit flow, and his ASD was closed. A lung biopsy was performed at the same time to try to figure out why his chest tubes were leaking so much fluid. The day after his first surgery he lost 1800cc from his chest tubes, and six IVs were barely keeping up. The lung biopsy came back showing "pulmonary lymphangiectasia"—damaged/undeveloped lymphatic vessels causing massive leakage into the area around the lungs. Real bad news according to the hundred or so documented cases, but doctors were amazed at Michael's daily progress. Researching lymphangiectasia led us to a veterinarian at OSU that gave us some info on how they treat it in canines (sadly the only research being done anywhere). He told us about "pleuro-peritoneal shunts". Researching this we found a doctor in Indiana that had used this in very tiny infants for different diseases, but same lung leakage problems. Our doctors talked to the doctor in Indiana, but again, God had a better plan: one day one of Michael's chest tubes quit leaking, a couple days later the other one did too.
After three weeks of mostly pretty good days, when suctioning out his ventilator tubes, Michael got more upset than usual (he always hated this and tried to fight it). Michael's heart rate hit 240, alarms were going off... it had happened before, but not this bad. Right away Michael's pressures leveled off higher than before, and after an echo, it was determined that his ASD repair had torn loose. At 39 days, his second surgery was performed to fix the damage from his temper tantrum.
On 6/16/98 Michael came home! The next day, he seemed to be breathing slightly faster, and the next day, even more. We took him to Dr Ben-Shachar to get him checked. He said we needed to get Michael back to Rainbow—his pressures were back up.
At Rainbow, a Cath was performed that confirmed scar tissue was obstructing Michael's pulmonary inlet. Reconstruction would be needed very soon.
On 6/29/98, Michael's third surgery was performed- it was the longest of the three, but was a great success. His kidneys took longer than before to start working again, and he was quite bloated, but his pressures were greatly improved immediately. A second Cath was done to verify all was well. Success at last! I remember watching 4th of July fireworks on TV right after Michael was taken off the vent for the last time...
On 7/18/98 Michael came home "for keeps". We kept him in his room almost all the time, for the first month or so. We had a HEPA air filter in there, kept his room at 70 degrees, and always at 40-50% humidity. We also limited visitors. Michael was small but growing. The worst thing was he hated his NG tube—on September 8th, after pulling his NG tube for the third time in several hours, something had to change. Michael couldn't drink from a bottle, as he would get too much in his mouth, or forget how to breathe. I drilled a hole in his trusty pacifier, put in a fitting attached to a short length of tubing to a model airplane fuel tank check valve, and attached this to his feed pump bag. A tiny, tiny pinhole was poked in the pacifier nipple, and whenever he would bite down on it the check valve would make pressure, and a single drop or two would come out of the pacifier- the very first night he took 100cc while munching on his pacifier in his sleep! As they were hard to clean, we made up a lot of these, and Michael was eating 24 hours a day. One of us sat up watching over him around the clock, and every couple hours at the most it was time for meds. Within a week he was taking in more than his pump was supposed to give him. After about 4 months of this we switched him over to a bottle. It wasn't easy, but after a few weeks of mixing the pacifier feeder and the bottle (and the spitting up) he finally got the hang of it.
When nine months old, Michael's eyes crossed-over a period of just a couple days. We were worried about some neurological problem, but luckily, no. Michael had eye surgery at 16 months (after trying the glasses/eye exercise stuff) to straighten out his eyes. Our neighbor Dr. Swanson actually did his anesthesia, and afterwards came out and told us "heck- that kids lungs are fine". This was great to hear, as we had many worries about his lung condition and the anesthesia—apparently his lungs have grown normally, and the damaged part has been overpowered by his natural growth. At least this is our hope.
Michael had a little brother born one day before his first birthday, perfectly healthy, thank you. Paula started chemo on Michael's 2nd birthday, after 6 months chemo/radiation for non-Hodgkin's lymphoma, she is now also doing very well and is in remission.
Michael is not only a healthy three year-old now, but is also one of the most polite, brightest, happiest (but kind of shy) kids I've ever seen. He knows all his colors, half the alphabet, loves to sing, ride his bike, and run, run, run...We just wish his little brother could act more like him!
We truly live in a remarkable time—not so many years ago heart surgery was theory. Now medical miracles are performed every day. We cannot say enough to thank God for every day. Each day is a gift, and for our family. Although the past few years have been bumpy, we are still here and very thankful for them. Some lifetimes are measured in minutes or hours, and so often we seem to forget this. Hopefully anyone that might read this and is in a similar situation will remember to value every day. Even if you only get one day with your child, you are still more fortunate than many.
Sorry this "nutshell" rambled on for so long- so many things... Anyway, May God bless you and yours,
