Leilapalooza

In June 2014, after a long journey to conceive our 2nd child, we finally received the great news that we were pregnant. All was progressing perfectly, until, a routine pregnancy check up at 18 weeks, revealed that Leila's heart was shifted to the left and our doctor referred us to Boston Children's Hospital (BCH) for a fetal echocardiogram. After the test, we were told that Leila's heart looked normal and was functioning properly and we were sent home. We thought we were in the clear. Unfortunately that was not the case, and we received a second call and were asked to return to BCH for a fetal MRI. On 11/17/14, we were advised that Leila had a condition called Congenital Diaphragmatic Hernia, which is basically a hole in her diaphragm, which had caused all her abdominal organs, including her liver, to push up into her chest, which would hinder proper lung growth causing pulmonary hypoplasia. There are three types; right sided, left sided or bi-lateral. About 90% of the cases are left sided. Leila was diagnosed with right sided CDH, which is less common. We were also advised that there is a spectrum of CDH from a milder form to the most severe form, which is determined by the volume of lung tissue. The doctors indicated Leila was in the most severe category, with a 10-15% lung volume and advised that only some babies survive in this category. At that point, the doctors did not see any visible right lung and her left lung was very small. To say we were devastated was an understatement and heartbreaking, especially as we had to explain it to her big sister, Bella, who was beyond excited she was going to have a baby sister.

We met with the team of doctors several times and they came up with a plan to induce me at 39 weeks at Brigham and Women's Hospital and then transport Leila to BCH. The words the doctor said at our appointment I will always remember "prepare for the worst, hope for the best". 

I was induced on 02/18/15, and after 36 hours of labor, Leila was born at 8:01pm on 02/19/15. During labor they prepared us for all the possibilities and it was one of the most difficult things we've ever gone through. A day that should be a parent's happiest moments turned into the scariest ever. There were approximately 15 people in the delivery room prepared for Leila's medical interventions needed upon delivery. They did not want her crying and collapsing a lung even if she was able to. They prepared us that I wouldn't be able to hold, touch or possible even see her.

As they were cutting her cord, they placed her on my stomach for about 5-10 seconds and she looked up at me with her beautiful eyes and I am convinced she was saying, mom I've got this, what a gift! Leila was then immediately rushed by a team of doctors to be intubated to help her breathe. The team of doctors were amazing and within minutes she was intubated and she was rushed over to BCH. My husband, Jim, followed the team, while I was recovering from the birth.   

Immediately after her birth, after arriving at BCH, they placed her on (ECMO) Extracorporeal Membrane Oxygenation, a lung and heart by-pass machine that works by removing blood from her body and artificially removing carbon dioxide and oxygenating red blood cells. We were told this was only a temporary fix and several complications could occur, including bleeding in the brain.   

After several hours, I was wheeled over to BCH to meet our miracle girl. Although it was extremely tough seeing our baby hooked up to so many machines and not being able to hold her, we knew she was in great hands. Within 24 hours of being born, Leila went into surgery and they made an incision from her chest to her stomach to repair the hole in her diaphragm, placed a gore-tex patch to cover the hole in her diaphragm and all her organs were moved to their appropriate place. The surgery took several hours and was very dangerous given she was only a day old, but it was the only chance she had to give her lungs time to grow and heal while on ECMO, which was only a temporary fix of approximately 3-4 weeks and as long as she didn't incur any additional complications.  Amazingly,  she did great and even the doctors seemed surprised with how well she did.

The next couple weeks were very stressful and many nights were spent praying and hoping that she would make it through the night. She required several blood transfusions and several medical interventions. On day 16, they clamped her ECMO machine to see how she would tolerate it. She surprised her team of doctors when she was able to sustain breathing with just the ventilator and she was de-cannulated off of ECMO a couple days later, something they never thought was possible given her severe case of CDH.

 After 19 days, I was finally able to hold baby Leila for the first time. She was passed slowly onto a pillow that was on my lap by the nurse and the respiratory therapist. I was told to not move her much and holding her might stress her out, to be prepared that she might not tolerate it. I was so nervous she wasn't going to know I was her mother and that she wouldn't feel the mother/daughter bond with me. Leila just looked at me with those beautiful eyes and just melted in my arms. Words can't even describe how amazing that felt.

On 03/10/15, we were bombarded with the news that Leila had a pulmonary hypertension crisis and required Nitric Oxide and other interventions to be stabilized. They weren't sure what caused it, but once again prepared us for the worst. After a few tough days, she thankfully returned to her baseline and was stable again.

 On 03/21/15, Leila was very uncomfortable and they discovered she developed a blood clot in her right thigh and required weeks of injections and monitoring. The next several weeks had its ups and downs, (what they call the CDH cha cha dance), but things were progressing and the doctors were ready to try to extubate Leila off of the ventilator. Unfortunately, she failed extubation on 04/18/15 to room air after a short two hours and failed again on 04/22/15 to continuous positive airway pressure (CPAP) due to a rapid lung collapse and the team of doctors had to perform an emergency intubation. To watch your baby struggle and work so hard to do a simple thing as breathe is heartbreaking. Her amazing team worked so quickly and hard and stabilized her. One of the many things I love about her surgeon and CDH guru, Dr. Jay Wilson, was  he was always honest. He always told us what was happening and if he didn't know, he would tell us that too. This was one of those times. He didn't know why Leila was having such trouble with extubation, when her lungs were showing enough growth to sustain breathing without the ventilator.

On 04/30/15, they decided to perform a trial extubation, during a bronchoscopy to get more answers. Leila immediately desat, (decrease in oxygen saturation) and required immediate intubation and the test revealed she had granulation tissue of false cords, subglottic swelling and severe malacia of the left main bronchus. Given the findings she needed to have a tracheostomy and Gastrostomy-Jejunostomy (GJ) feeding tube. She had to be paralyzed for 7 days while she healed from the surgery. It was very difficult seeing her so sedated and unable to move or respond to touch.

After several more months of ups and downs, many tears of joy and fear, Leila, was finally scheduled to be discharged on 08/12/15, a week shy of her 6 month birthday. On the way to the hospital, we were advised that Leila had a desaturation of oxygen due to her trach slipping out, during morning doctor rounds and the team of doctors postponed her discharge. A disappointment for sure, however, we agreed it was best to make sure she was ready. Finally, on 08/13/15, discharge was a go and Leila was transported home. The day was filled with joy and anxiety and we were over the moon to finally have our baby girl home.

After Leila was finally home, we took advantage of every moment possible with both of our daughters trying to get into our new routines and back to some normalcy. We had the most amazing 27 days until the morning of 09/09/15. It was a typical Tuesday morning, I had just dropped my older daughter, Bella, off at school. She begged to stay home that day, but off we went, trying to get into our new flow of routines. After drop off, I wasn't planning on going straight home, but for some reason, I felt I needed to be home. The home nurse was giving Leila a bath and had taken Leila’s oximeter probe off her foot (which indicates her oxygen and heart rate).
Given Leila's frailty it was extremely important to monitor her at all times, we had medical orders for continuous oximeter monitoring and I did not give the nurse permission to remove it, nor did I realize that she had turned the oximeter machine off as well. If there is a bad oxygen read or if Leila's oxygen saturation drops below 95, it alarms, neither happened given the nurse negligently turned the oximeter machine off. I was sitting at our kitchen table, working on some paperwork, when I glanced up to see how everything was going. The nurse had just finished Leila's bath and was moving her onto the changing table. At the same time I looked up, I noticed that Leila was blue. I ran over to Leila and the nurse and yelled “she's blue” and a few seconds later Leila collapsed. The nurse did not drain the water from the ventilator tubing before she moved her, which was specifically discussed and when she moved Leila the water in her ventilation tubing drained down into her throat and essentially into her lungs, which caused her to aspirate, stop breathing and she went into cardiac arrest. I hollered at the nurse to start CPR and the nurse just froze and just kept repeating to Leila to "wake up". How does a medical professional not respond when their training is specialized for this kind of event? How does she not notice that her patient is blue? How can the one person who we needed the most to help save our child, not respond? We welcome this nurse into our home trusting that she would help our baby with the care she needed and she didn't even try. I know at this point the nurse isn't going to do anything to help save our helpless baby so I grab the ambu bag and oxygen tank and start bagging her. I try for a couple minutes and then called 911. I was on the phone long enough to tell them that Leila was not breathing or responding and to send help. Then I continued bagging her and I get her oximeter probe back on her foot. Her oximeter indicates that her oxygen was in the 40's and her heart rate was in the 50's. Meanwhile, the nurse still continues to do nothing except shout at Leila and say "come on Leila, wake up Leila".   

The first to arrive was a police officer and I asked him to continue to ambu bag Leila while I get a new trach to change it out, in case, it was a mucus plug (a buildup of mucus at the end of the trach tube that blocks breathing) that was causing her to not breathe. I changed out her trach and a few minutes later I noticed her trach had slipped out. I try to insert her trach back in, however, was unable to, so I get another trach and  complete another trach change. Meanwhile, the EMT's arrived and the police officer passes the ambu bag to the nurse and she finally snaps into it and starts to ambu bag Leila while the EMT's get updated on the situation and start performing CPR.   

Leila is still lifeless at this point and after several moments they find a faint pulse. I help the EMT's get her onto the stretcher and into the ambulance. It takes three attempts but the EMT's finally are able to administer the epinephrine into her thigh bone. And then finally, she has a good heartbeat and oxygen level...thank goodness! I make the dreaded call to my husband, Jim, that we are on our way to the hospital and to meet us there. We arrive at the hospital and they started running tests.  

At this point, Leila is not opening her eyes but is responding to pain. I had no idea the events that were about to transpire. The next hour or so is just a blur and after a couple of hours Leila is transported to Boston Children's Hospital. They had a very difficult time regulating Leila’s body temperature, which is common with oxygen hypoxia (lack of oxygen to the brain). They covered her body in ice packs and hooked her up to an EEG to monitor brain activity and seizures. Once again, I'm unable to hold or comfort my baby. Over the next 24-36 hours her symptoms worsen and they decide to do an MRI. On 09/11/15, we met in a conference room with an entire team of doctors, nurses and social worker. Within minutes they inform us that Leila had suffered severe global brain damage, on all parts of her brain. They told us that she will never walk, talk or recognize us again, and they want to discuss our options. This can't be happening, not to our baby girl. Not after all she's been through. 

The next several weeks and months were heartbreaking to watch. Leila stared blankly at nothing, had brutal autonomic storming episodes (rapid breathing, sweating, agitation, abnormal posturing and body stiffening, which is very painful) in which we couldn’t even comfort her. There is nothing harder when a parent can't comfort their child. The team of doctors continued to adjust her medications to get her comfortable. As if that wasn't painful enough, we are constantly being advised of our "options" to turn Leila's ventilator off or to look at a long-term facility and how difficult this life would be. We are so frustrated that we request a meeting with the ethics committee and advise them of our wishes to take Leila home and that we don't want one more person discussing "our options".

Things progressed slowly and it was very difficult to watch Leila go through the storming episodes and see her body in such distress. We continued to comfort her as best as we can. Finally after weeks of trying to get some positive answers, we met with Dr. Tasker, one of the top neurologists at BCH, who reviewed Leila's images and examined her. He indicated that 95% of patients with Leila's global brain damage typically are in a vegetative state, however, that Leila is in the other 5% category, which is unknown, she can be anywhere from moderate to severe and the next couple of years will determine what Leila is capable of and if other parts of her brain will compensate for the dying sections. This was the most encouraging news we had heard and finally had some hope. After an additional 70 days, on 11/17/15, Leila was discharged again, which ironically was the same date the year before that we found out Leila had CDH. We were so grateful, but also nervous. The first several weeks back home we worked on getting into our new routine again, with new therapies, doctors’ appointments and nursing.   

On 12/03/15, we found Leila unresponsive physically and her heart rate kept dropping into the 60's. Leila was transported to BCH by med flight and they indicated she was in neurological distress and it was presumed that Leila was overmedicated by the home nurse. Luckily it was not significant and she was released again on 12/05/15. Thankfully the next couple weeks were uneventful and we spend her first Christmas with her at home. 

The next several months we continue to work closely with her therapists and doctors to help her adjust at home. Leila is on a ventilator 24 hours a day, continuous oxygen, fed through a feeding tube 20 hours a day, takes 15 different medications 12 times a day, requires daily nursing help to assist with her daily needs, has several therapies and doctor’s appointments weekly and requires constant monitoring.  And most recently it was discovered she has a large hole in her heart (Atrial Septal Defect), which will require surgery in the near future. 

Our amazing baby girl has endured so much in her short life already and we are beyond thankful for our little miracle who continues to fight so hard everyday! She has a spirit and determination that is so inspiring and everyone who meets her, falls in love with her! And although she still has many mountains to climb she is making amazing progress everyday!!!
 
In an effort to assist with purchasing a medical vehicle, which will make transporting Leila to and from doctor appointments with all of her medical equipment easier and to help with the ongoing medical/financial expenses to keep her home thriving and healing, we are asking for your support!

We truly appreciate all the love, prayers and support we have recieved these past 18 months and couldn't have possibly made it through this very difficult time without everyone's support!!!!