January 2, 2009

Our Miracle Eight-Year-Old

Our little guy, the one who was once given an 18% chance of survival, turned eight years old this month! We couldn't be prouder of our little man, William -- who is really not so little any more.

Star Wars lover that he is, William's birthday dinner out at Ella's Deli was followed by Star Wars cake and Star Wars -themed gifts. Check out William and Andrew sparring in their Star Wars gear.

Following the photos is a reprint of William's story—the story of his medical difficulties early in life. I wrote the story a few weeks after he came home from his long hospital stay, and I enjoy re-reading it every year around William's birthday.

You've come a long way, birthday boy!

Feb. 27, 2001

William’s Story

This is a story about our son, William. It is a story about his great struggle to live through a terrible ordeal. This is the story of Will’s second month of life.

William was a happy, healthy baby for the first 3-1/2 weeks of his life. Sometime during the second week of January, however, he managed to catch a cold that was running through the family. By January 15, he was sounding terrible, with labored coughing keeping him awake at night, so we took him to the clinic. Upon examination, our pediatrician, Dr. Yaffe, recommended that we take him to the hospital to have IV fluids started, merely as a precautionary measure. Although he wasn't yet dehydrated, William did have a fever, and the concern was that, at just 30 days old, he could go downhill quickly.

On the car ride from the west side Dean Clinic to St. Mary’s (a mere 20 minute drive), the unthinkable happened: William went into respiratory distress. By the time we reached the hospital Emergency Room, he was failing rapidly. Hospital personnel rushed him right into the Intensive Care Unit and intubated him. The whole "IV fluids as a precautionary measure" had gone right out the window. He was suddenly in very serious shape. Nurses and technicians began a number of lab tests, including a spinal tap to rule out meningitis. The tests revealed that he had RSV (a common virus, but one that is particularly dangerous to newborns) as well as pneumonia. He was going downhill by the minute, and the doctors and nurses seemed perplexed at the rate at which things were deterioriating. They took us aside and provided the shocking news that William might not make it. We couldn't believe how our son had gone from being a baby with what seemed like a nasty cold a half hour before, to a child in very, very critical shape. It was terribly frightening. Little did we know that things would only get worse the following day.

The second evening of William’s stay in ICU, he “coded.” As a mother, I will never forget hearing the “Code Blue” call, only to find that the code had been called for my son. It was 11:30 pm, and Joe had just left for home to spend the night with our daughters. I was alone, in PICU, watching with disbelief as the most surreal of scenes unfolded before my eyes. Gray on the table, William lay motionless as the doctors and nurses tried time and again to intubate him and provide him with much-needed oxygen. One intubation attempt after another failed. That was the night we almost lost him.

The Code Blue had been called because William’s tube, clogged with mucus, was suffocating him and had to be pulled. When his tube was pulled, the assumption was that he could be reintubated. That assumption proved frighteningly incorrect. Nurses manually bagged him while waiting for emergency room doctors to arrive on the floor. Two ER doctors ran to William’s aid at breakneck pace, attempting to help our little guy. However, despite a total of three attempts, neither ER doctor was successful at reintubating William. With each failed reintubation attempt, all Will’s heart rate and oxygen saturation levels would drop, prompting nurses to manually “bag” William with oxygen. He remained hopelessly gray on the table.

Fortunately, the pediatric ICU doctor was simultaneously racing from his home to the hospital. During that fifteen minute lag between the failed reintubations and Dr. Ponte’s arrival, nurses continued to take turns manually bagging William.

Pediatric ICU physician, Dr. Ponte, arrived and set to work immediately reintubating William. Success at last. It had taken three doctors four tries and nearly a half hour to successfully reintubate our little guy. Needless to say, it took nearly an hour and half to stabilize William after all the excitement. I was stunned and so grateful that our little guy had pulled through, but very fearful of the repercussions. William now stood in his most precarious position yet.

This is where the story takes an interesting turn, from a medical standpoint. During the course of the reintubation attempts, and confirmed by a bronchoscopy that followed, the doctors discovered that William had severe, 80% tracheomalacia, a congenital floppiness of the trachea. Now things were starting to make sense. We'd been wondering, together with Will’s doctors, how the RSV virus had gotten the best of a good-sized, full-term baby so dramatically and so quickly. Now the answer was clear. The doctors stated in certain hindsight that it was the tracheomalacia that caused William to go downhill so rapidly and the reason that he couldn't handle the virus on his own without intubation. As the virus produced mucus in his airways, the fact that his airways were already closing down 80%, meant that he was suffocating. That explained the unexpected onset of respiratory distress on the way to the hospital and helped crystallize why things happened so fast and snowballed so quickly. The tracheomalacia also explains why the ER doctors who responded to the Code, had so much trouble reintubating Will. Infant airways are already narrow; Will’s was intermittently 80% narrower than normal. The bad news: With severe trachemalacia, RSV, and pneumonia, little William’s chances of survival were placed at a bleak 18%.

Thankfully, Will never had another Code, but he did have several more frightening episodes where his heart and oxygen rates dropped to alarming levels. The three weeks that followed were a series of highs and lows. Some days Will would seem so much better, albeit comatose. Other days, he’d have significant problems. I repeatedly asked medical personnel whether he was going to make it. The doctors and nurses were unable to provide much reassurance during the first week. They simply didn’t think his odds were good. He remained critical and extremely sedated in a drug-induced coma for ten days.

I stayed, round the clock, asking every imaginable question, watching monitors, and pumping breast milk for Will to receive through his NG feeding tube. Joe ran between home, hospital, and work, trying to keep everything together for our family of five. I had a “sleep room” across the hall from ICU, but I couldn’t sleep much, so I spent most nights in the recliner chair next to Will’s bed.

William underwent four blood transfusions and was administered unbelievable amounts of medication, including morphine, versed, and propophol. He retained water and swelled to the point that we didn’t recognize him. He had tubes coming from everywhere… his head, his groin, his arms, his legs, his nose, his mouth. Day after day, the machines did all the work for his little body.

Nevertheless, after the first ten days, William slowly but surely began improving, surprising one and all and pushing the bleak 18% survival statistic more and more assuredly out of our minds.

We watched with gratitude as the pneumonia and the RSV virus cleared up, but saw with dismay the beating his body was taking. With the pneumonia and the RSV behind William, there was still the issue of the tracheomalacia, the dependence on the ventilator, the very real addiction to the many drugs, and the difficulty with re-regulating all his bodily functions.

After about ten days in ICU, William’s physicians decided to decrease his medication and attempt an extubation. Joe and I watched the failed attempt with great sadness. Our little guy didn’t have sufficient strength or tracheal rigidity to last even a few minutes without a ventilator. A quick bronchoscopy attempt resulted in dramatic, pulsating bleeding from the mouth (a pretty horrific sight for even non-squeamish parents), and William was rapidly reintubated with what was likely now even greater damage to the already-compromised trachea. It would prove to be another two full weeks before another extubation attempt would be made.

Finally after a total of three weeks in ICU, a team of doctors and specialists prepared to extubate our son. Nurses and physicians warned us that a tracheostomy would likely follow, as William’s trachea would likely not be strong enough for him to breathe on his own. Although the mucus-producing RSV virus was now long-gone, William’s trachea had sustained great damage in the multiple emergency extubations/intubations and during the bronchoscopy. A hospital social worker arrived to explain William’s inevitable surgery and the 24-hour nursing care and other issues that would be involved after William returned home with a tracheostomy. After three weeks of bad news and complications, we were resigned to a tracheostomy. After all, it would help William return home to us, and that’s exactly what we wanted. It appeared William’s doctors felt the same way, and they told us simply to expect a tracheostomy. With that, Dr. Ponte and Dr. Bukstein performed the extubation, while Joe and I watched over their shoulders. The surgeon and his team were ready and waiting to take Will to the OR for his tracheostomy.

And then the miraculous happened. Wonder of wonders; this little baby who was not expected to have the strength or tracheal rigidity to breathe on his own, stunned doctors, nurses, and family alike when he started breathing independently immediately following the extubation. Instead of taking William directly into surgery for the tracheostomy, Will’s team of doctors and nurses began monitoring his breathing patterns and marking the number of seconds, then minutes, then hours, that he was able to independently breathe without the ventilator. They kept warning us that he would soon tucker out; his trachea was surely not yet firm enough. After all, it was only eleven days earlier that he’d had the failed extubation attempt and had endured the awful bronchoscopy. And yet, the hours kept ticking by, and William steadily breathed on his own.

After 24 hours, Will’s doctors made the incredible announcement that William was no longer a candidate for a tracheostomy. They expressed their utter amazement that William was breathing independently, stating that it was something they never would have expected. Amazingly, William’s trachea had firmed up enough in three weeks that he was able to move a sufficient amount of air. Although his tracheomalacia still existed, much of the excess swelling and inflammation caused by the virus, the multiple emergency intubations, and the bronchoscopies had subsided. Tired and overwhelmed and now dealing with such unexpectedly wonderful news, Joe and I shed tears of joy.

After extubation, things started to look up for William. Shortly after extubation, Will was moved out of ICU and onto the main floor, where he remained for a week with supplemental oxygen via nasal prongs. Despite major improvements, William did experience some setbacks, including persistent seizures. We also had to work on getting William off the feeding tube and onto a bottle. He'd lost his sucking reflex and his muscles had atrophied while being so sedated for so long, so he had to learn how to suck once again (using a Haberman feeder, a modified bottle with a special nipple, designed to feed babies with cleft palate).

* * * * *

As I write this story, William has now been home for a little over two weeks! It's incredible to have him home. He's now breastfeeding full-time, something the nurses said he'd never be able to do again! The Haberman Feeder is no longer part of our routine.

William’s neurologist ultimately determined that Will’s seizures were caused by his addiction to the morphine and other medications that kept him in his coma-like state in intensive care. As a result, William remains on numerous medications — three withdrawal medications (including methadone) to taper him down slowly, two anti-seizure medications, and two broncho-dialators to assist in his respiration. William will remain on the withdrawal and anti-seizure medications for at least six weeks, longer if needed.

William’s doctors expect him to outgrow the tracheomalacia within a year or two. Until then, any little cold or flu bug will be very dangerous for him. So he's quarantined this year until cold and flu season is over. We await May with great excitement.

William has had to return to the hospital only once… an overnight stay for observation last week, when his seizures began becoming more frequent. After more than an hour of seizure activity one day, he fell into a coma-like state. We were unable to wake him. Terrified, we had an ambulance meet us on our way to the emergency room. His overnight stay at the hospital proved uneventful. It appears that his deep sleep was merely seizure-induced exhaustion, and we are working out new combinations of medications that might help reduce the severity and frequency of the seizures.

Both the long-term intubation (a total of three weeks) and the RSV damaged William’s immature lungs. As a result, he has breathing difficulties (which the doctors call lung disease) which he may have to some degree for the rest of his life. We are told that his likelihood of developing asthma is high. However, the medications (including albuterol, administered through a nebulizer) help William dramatically, so it seems like something that is fairly easily controlled. William also has difficulty breathing when he cries, hiccups, or becomes agitated. That's specifically because of the tracheomalacia; apparently the trachea just closes down when he gets too much positive airflow going. But we’re told that's something that he'll outgrow. We just have to get through these winter months without him picking up another virus.

All-in-all, William is doing so much better than any of us could have imagined, just a few short weeks ago. He had an appointment yesterday with his Dr. Bukstein, his pulmonologist (who he’ll see regularly throughout childhood). Dr. Bukstein was very pleased with his progress. So are we!

William has an appointment with the neurologist next month for a follow-up on the seizure issue. The good news is that William underwent EEGs and CAT scans, and the neurologist detected nothing abnormal. We, of course, have been so worried about whether William received enough oxygen the night of the Code Blue, when he was so gray for so long. The neurologist assures us that any major anomalies would have shown up in the EEG and CAT scan. It's still possible that there could be slight problems that wouldn't have shown up in the tests, but major cognitive problems seem to be ruled out at this point. Notably, our little guy has a medical record at 2 months of age, longer than any of ours.

We have so much for which to be grateful. Friends and relatives offered prayers, supplied meals, and provided babysitting services for our daughters. I don’t know how we would have managed without their support. The doctors and nurses were superb and feel like a special part of our family.

We thank God every day that our little “Miracle Baby” is happy and healthy and with us today. We love being home, loving our sweet William, who grows stronger and healthier every day. What an incredibly sweet answer to many, many prayers.

I write this story today as a reminder of where William has been and how far he has come; as a special memento for William so that he understands the fragility and beauty of life; and as a remarkable family story to read and reread, so that we all might remember with gratitude the blessing bestowed upon us when we were given back our beloved Miracle Baby. I write this story with the utmost love for my sweet son, William.


KB said...

Elizabeth...what a beautiful and painful story. Thanks be to God for its very happy ending for you all.

Merry Christmas and many blessings to you and yours in 2009.

makingsevenheaven said...

Hey... always awesome to remember the miracle of William. Quick question... did the tracheomalacia ever resolve? We are dealing with this with Keeghan and just recently noticed that she seems to be sounding worse... she does have a cold. We haven't been to ent in a 14 months... she has seemed so much better. Just wondered what your journey looked like! Thx, hug to all the Leone's! C

Barb said...

Yay for miracle 8 year olds! Or miracle any year olds! Lookin' more and more like a young MAN!

Kris said...

Wow, that story bring back memories, and tears to my eyes! Happy Birthday William! Your birthdays will always be extra special for all those who love you! We sure are glad you are a miracle boy! The Delaneys

+JMJ+ said...

You have me in tears!

God bless you all and William!

Pamela said...

Happy 8th Birthday!!

Sandy said...

wow is he growing up, and thank you for sharing your beautiful story! i love miracles.

Kim said...

Thanks for sharing this amazing story!

Jeannie said...

Thank you so much for sharing this beautiful story of hope! What a wonderful blessing William is to this world!