In July of 2006, six weeks after our wedding and while on our honeymoon in the Bahamas, Dan and I found out that I was pregnant. We had a few days of excitement before the worry began. From the very beginning, the pregnancy was a difficult one. We had reason to think we had lost the baby a few different times. I had a constant feeling that something just wasn't quite right.
At my 20-week ultrasound, my fears were confirmed. Something was wrong with the baby's heart. The information that the doctor initially gave us wasn't entirely accurate, which led to a lot of uncertainty and emotional turmoil. The fear of the unknown, we learned, can be crippling. I had to get an amniocentesis procedure a couple different times and I had a few more ultrasounds before we learned the details about our baby’s problems. The time between learning something was wrong and finding out exactly what was wrong was a difficult time. Once we had some sort of idea about what to expect and prepare for, we felt significantly better about the situation and we felt ready to tackle it.
When I reached my 29th week of pregnancy, the contractions that I'd been having since week 25 became more frequent. I spent a few days in the hospital to monitor them and to attempt to slow them down. I tested positive for fetal fiber-nectin, which meant I had a very good chance of going into labor sometime within the following two to three weeks. By the grace of God, my body held out for another seven weeks.
I spent those seven weeks on strict bedrest and didn't lift a finger besides to shower, use the restroom and attend doctor's appointments. Dan was wonderful during that time. He did everything for me so I could rest and so that the baby would keep growing in my belly.
On February 19th, at 36 weeks pregnant, I had a particularly difficult doctor's visit. I came home feeling more overwhelmed, sad, frustrated and scared than ever. I was tired of the constant bad news that every doctor’s visit brought. The baby wasn't growing as much as he should have been, so there was continual worry about his small size and why he wasn’t growing much and whether or not he'd continue to thrive or even survive at all. I completely broke down. The stress had caught up to me. I laid in bed and cried. I had a chat with God that night. I asked him to finish this chapter, no matter what that meant. Six hours later, at 2:00 in the morning, my water broke. It was time. My prayer had been answered.
Labor progressed quickly (you can read the full birth story
here). I was in labor for seven hours. We attempted to deliver the baby but his heart rate kept dropping into a scary range, so the decision was made to do an emergency c-section. At 9:55 a.m. on February 20th, 2007, Elijah Daniel Porta was born! He was beautiful and perfect. He weighed 4 lbs. 14 oz. and was 18 inches long. Even though he was four weeks early, he didn't have a single prematurity-related complication. Thank you, God. His heart diagnosis was tetralogy of fallot with pulmonary atresia and MAPCAs. He had a few other anomalies that were found at birth: his right kidney had failed to develop so he had only one functioning kidney, his urethra didn't fully develop (hypospadias), and he had a sacral dimple. All genetic testing came back normal, so these anomalies were not related to a genetic disorder.
He has functioned just fine with his solitary kidney, and we don't foresee it causing any problems in the future. His hypospadias was surgically repaired in August of 2008. An ultrasound was done of his sacral dimple at birth, which revealed (incorrect) good results. At 28 months old and still not close to walking, we decided to look into it further. An MRI revealed that Elijah had a tethered spinal cord. He had the surgery to release the tether on June 18, 2009, and six months later, at almost three years old, Elijah started walking.
We had initially thought that an open heart surgery would need to be done within a week of Elijah's birth, but along with the MAPCAs portion of the diagnosis came extra collateral arteries that helped blood get where it needed to be, so immediate surgery was not necessary. We were told he would need two to three heart surgeries within the first few years of his life, the first being somewhere around six months of age.
Elijah was in the NICU for three weeks after birth, gaining enough strength to eat on his own. His heart condition made his little body work extra hard, so he tired very easily. We brought him home with a feeding tube, but pulled it out after just a few days because he started chugging down milk on his own.
He grew quickly and never had a problem eating once we got him home from the hospital. All of his doctors were amazed at his progress, considering the extra work his body was doing. At two months old, he had his second heart cath because his oxygen saturations were low (in the 60s). The cath revealed that surgery would need to be sooner than anticipated.
Three weeks later, on May 25th, 2007, Elijah had his first open heart surgery at three months old. Dr. Moga, his surgeon, placed a temporary conduit (shunt) that acted as his pulmonary valve into his heart and he also redirected some of his collaterals that were supplying duplicate bloodflow to his lungs.
Elijah unexpectedly thrived after his first surgery. His weight gain continued to surprise all of his doctors. He started out not being on the charts because he was so teeny and it didn't take him long to get to the 50th percentile for both height and weight. This is not typical for a lot of heart babies, and was a huge blessing.
Initially Dr. Gremmels, Elijah's cardiologist, told us that his second open heart surgery (the "total repair") probably wouldn't need to happen until fall 2008, but with as fast as our boy grows everything comes sooner than expected. In February-March 2008 Elijah's oxygen saturations began dropping quickly and a heart cath revealed that surgery would need to happen soon.
The weeks leading up to his second surgery were tough. His health declined rapidly. His oxygen sats ran between 40% and 65%. He had blue lips and purple fingernails. He got sick often and his body was not able to fight anything off. He had a few scary blue spells because his body had little tolerance for anything out of the norm, such as taking a bath. He threw up often and had scary, high fevers. His body was READY to have a fixed heart!
On April 15th, 2008, Dr. Moga completed Elijah's total repair. He was able to do everything he set out to do. He replaced the conduit with a bovine valve, fixed the VSD (hole) and placed a stent in his right pulmonary artery. The surgery went flawlessly and the recovery had no unexpected bumps. We were told to expect a two-week stay in the hospital but we were home in eight days! He is a rock star!
Three years later, in February of 2011, another cath revealed that Elijah's valve needed to be replaced. Somehow this came as a surprise to us, and I was sent into a tailspin. The year following that news was the darkest of my life to date. Thankfully, God took care of us and I was able to accomplish the most important task during that time--taking care of our precious boys! Our little Sammy was now a part of our lives and was nearing his first birthday.
When I think back on that time, I picture myself shriveled up and in tears and I picture Elijah as happy and smiley as he always is. The stress did not affect him negatively, and I am constantly thankful for that. He absolutely kicked that third surgery's butt, which occurred on June 10, 2011. Dr. Moga replaced Elijah's very tired and enlarged valve (we saw pics...it was
very distorted and enlarged). He now has a cadaver valve since that turned out to be the best fit for his anatomy at the time.
Elijah was extubated the day of surgery and he experienced no post-intubation croup or complications, which was a miracle considering his history with croup (which began around age three, especially after being intubated). Everything about that recovery was smooth and miraculous. God took care of us. Our strong four-year-old came home after five days in the hospital! Seven days after being operated on, he was running around the zoo (gently). It was incredible.
He will need at least one more open heart surgery to replace his current valve, which he will eventually outgrow. The cath he had in April of 2013 showed no issues with pressures or function, so we are praying for a few more years without a heart surgery before it needs to happen again. Heart caths are the exception, as he has had and will need these yearly(ish) until he is done growing.
In the fall of 2012, we discovered that Elijah could not hear out of his left ear. Upon investigation, we found that he had yet another congenital anomaly that did not present itself until he was nearly six years old. The anomaly itself is extremely rare, but the fact that it didn't present itself until he was five was typical. A mass of tissue called a cholesteatoma had grown near and was pressing up against his eardrum, causing loss of hearing. The mass had grown so large that it had literally dissolved almost 100% of the bones inside of his ear. The surgeon removed the mass, but was unable to do reconstruction since the bones were absent. In October of 2013, Elijah will have a prosthetic device placed to hopefully restore hearing in that ear.
Academically, Elijah is on par with (if not slightly ahead of) his peers. He began reading at age four-and-a-half and his intelligence shines every day (i.e., he constantly outsmarts us). In almost every other area of development, he is delayed. Gross and fine motor skills continue to provide the biggest challenges for him. Climbing stairs are difficult and he prefers to have his feet firmly planted on the ground at all times, unless he is in a swimming pool (his favorite). He has a difficult time with buttons and zippers and anything requiring finger/hand strength. He is followed by special education services through his school for speech and physical therapy and he also receives outside services for occupational and physical therapies. His speech has come a long way, but he still has difficulty pronouncing a handful of sounds regularly (TH and L). With all of that said, he has come
so far. He has overcome some major obstacles in a short period of time. We are so very proud of him.
Elijah had an incredible kindergarten experience and he had the most special, caring team looking out for him. We feel blessed beyond words about the people God has put into his life, medically and now with his educational and developmental experiences, as well. We are looking forward to this new chapter called first grade!
We have dealt with behavioral issues off and on with Elijah since his last open heart surgery in June of 2011. He seems to need more sleep than his peers, so we do our best to ensure that happens. A rested Elijah is a happy, cooperative Elijah. A tired Elijah can be challenging. No matter what, we love this boy (and his sweet little bro, of course!) to pieces and I am constantly having to pinch myself because I cannot believe the obstacles he has overcome. And all with a big smile on his face. He is such a friendly, special little person and everyone who meets him falls totally in love with him.
We are blessed and grateful! We continue to walk out this path with our oldest boy and support him in any way that he needs. (It goes without saying that we feel the exact same about our incredible little Sammy, as well!)
To be continued! We never know what's next, but we know God has us cradled in His arms!
