In the summer of 2013, both Cliff and Amber were excited to learn that they were finally going to have a baby!
Unfortunately that excitement quickly turned to fear. While at their 14 week ultrasound, the technician noticed something that didn’t seem right, they were informed that their baby had been diagnosed with an omphalocele.
An omphalocele is a birth defect that occurs within the abdominal wall of the fetus, forcing the organs in the belly to develop in a sac outside of the body. There were approximately 775 omphalocele births in the US last year (roughly 1 out of every 5,400).
Through the following months they did their best to prepare for Paige’s arrival. They refused to do any omphalocele research on the internet, instead focusing their energy on the fun things; they decorated her room, completed a baby registry, and documented her weekly growth by posting pictures of themselves on Facebook with the fruit or vegetable that corresponded to Paige's current size! Even through the fun, it was very tough for them not to constantly think about what they were up against.
The big day finally arrived on March 10th, 2014! They woke up on a cold Monday morning, grabbed their bags, kissed the dogs good-bye, and anxiously drove to Women and Infants to meet their baby in person (still not knowing they were having a boy or a girl!). At 8:04am they welcomed Paige Elizabeth Alston to the world!!
Again the excitement was cut short, they only had a few seconds to see her before she was whisked away by the medical team. It was several hours until they were able to see Paige, but when they finally did, they learned that Paige’s omphalocele was bigger than they originally thought. Paige’s omphalocele contained her stomach, intestines, and partial liver. It was technically classified as a “Giant Omphalocele”......
They spent the next two weeks at Women and Infants as doctors attempted to stabilize Paige’s condition. Paige had been having a difficult time breathing and was presenting a very high respiratory rate - most likely due to under-developed lungs as well as the extra weight that was now residing on her stomach. Ultimately, the decision was made that Paige needed to be intubated to help her breathing. Shortly after intubation, Paige was transferred to Hasbro Hospital’s Pediatric Intensive Care Unit (PICU) where she would remain in the care of the Surgical Team.
Now it was time to focus on her omphalocele and start to transition her organs back where they belong. The first step in doing so was to have surgery to insert a tissue expander in Paige’s abdomen. The expander would be slowly inflated over the next several weeks in order to create more room. This surgery went well and they thought they were officially on the road to recovery!
Approximately two weeks after this surgery Cliff and Amber were approached by Paige’s cardiologist. Its seemed as though Paige had some additional complications. This time it was with her heart. Paige had what is called an Atrial Septum Defect (ASD). This is when there is a hole in the wall of the upper two chambers of the heart.
For Paige however; it seemed as though this was more than just a hole - the wall was almost non-existent. They were told that with most babies this hole could possibly close on it’s own. Unfortunately, this was not the case with Paige. She was going to require bypass surgery in order to correct this. As a result of the ASD, Paige was also diagnosed with pulmonary hypertension. Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of your heart. Pulmonary hypertension begins when tiny arteries in your lungs, called pulmonary arteries, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, and raises pressure within your lungs' arteries. As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and eventually fail. The combination of omphalocele, ASD, and hypertension left Cliff, Amber, and their surgical team, with a very difficult decision as how to proceed. Which surgery to do first? What could Paige handle?
Paige continued an up and down course for the next several weeks. She battled pneumonia and a collapsed lung leaving her in a hypertensive state. She was unable to maintain her oxygen saturation so Cliff and Amber were forced to have several conversations with the medical team about how to proceed. These were conversations that no parent should never have to do, they were running out of options. They were left with only one more option before ECMO (a medical device that works in place of the heart and lungs) which was to flip Paige on her stomach. Paige had never been on her stomach before because the pressure of the omphalocele would prove to be too much for her and cause her vitals to drop, but the belief (hope) was that by flipping her on her stomach, this would force her lungs to naturally expand and improve her oxygen saturation. Prior to Paige being flipped they were told that her numbers would get worse, but should level out, and then improve. She was flipped and her numbers in fact, got worse. It took several minutes but her numbers finally leveled off. Several more minutes passed and then they began to improve! Paige proved once again how tough she is and actually started showing some small improvements.
The decision was made that to maintain Paige’s safety, she would need to be heavily sedated and medically paralyzed. Over the next month Paige laid motionless in her bed. Every time an attempt was made to wean her pain meds, Paige would become angry and begin to have issues. Her body had become dependent on the meds and we were unable to get them off. Paige remained in a hypertensive state and they were forced to return to Boston Children’s for another cardiac catheterization. The specialists felt as though she would be stable enough for surgery to repair her ASD. Two days later, she was in surgery. The whole procedure took approximately 7 hours. Paige did well but was still in very serious condition. The following morning Paige continued to do well. Cliff and Amber decided to head to their parent room to try to get some rest when not long after falling asleep they were woken by a call from Paige's nurse. Paige started to develop complications and required emergency bedside surgery. It turns out Paige had blood pooling around her heart that was not draining. A chest tube was inserted and her chest remained open in case of swelling. Another terrifying event, but she again pulled through.
The decision was made to consult Boston Childrens Hospital and have a cardiac catheterization. This is a procedure that is used to diagnosis cardiac conditions by threading a long thin tube through the groin and into the heart. Terrifying to say the lease. The first step in the process was transporting to Boston. Unfortunately, Paige had become very sick prior to transport and required increased sedation medication to keep her hypertension under control. Very soon after our arrival to Boston, Paige suffered a major setback.
While getting checked in, and right in front of Cliff and Amber, Paige choked on her endotracheal tube resulting in a code needing to be called. In a matter of seconds, the room was full of doctors and nurses and they began CPR. Several minutes later, a pulse was found and Paige regained consciousness. This was the most terrifying experience they had ever experienced. The next several days Paige was hooked up to electrodes to monitor her brain function and was kept on a cooling blanket to lower her body temperature. Paige finally warmed up, woke up, and the cardiac catheter was completed. The test revealed systemic to super systemic pressures. They made the trip back to Hasbro but when they returned Paige, again, was very sick. She had contracted pneumonia,a blood infection, and a partially collapsed lung. It took several weeks before Paige was back to a “somewhat” stable condition. It was at this point that the decision was made to take Paige’s tissue expander out from her belly. This went well and Paige’s omphalocele actually shrunk slightly! However, there still wasn't enough room created for everything to fit back where it belonged.
Paige had amazingly made it though multiple major events. Having started the omphalocele procedures and having now completed her heart surgery we were left with one more major hurdle. Paige was still intubated, breathing through a tube in her mouth, but leaving her very uncomfortable. It was difficult for Cliff and Amber to hold her, difficult for her to meet her milestones, and she was still having bradicardic responses to the tube (sudden dropping of her heart rate due to the tube hitting her vagal nerve). It was recommended that Paige now have a tracheostomy. This is when a tube is placed in the windpipe, giving her a secure airway and allowing her to breathe with the help of a ventilator. They were told that this would be the best thing for Paige and would give them the best chance of getting her home. Paige completed the procedure and has thrived ever since.
Cliff and Amber weren’t allowed to take Paige home until they completed the hospital mandated training. This would take them at least another month. The training consisted of trouble shooting, emergency care, understanding and working the ventilator, performing CPR, packing an emergency bag, taking your child off grounds, working an entire overnight shift alone (without any nursing support), giving medication, and of course, changing the trachs - they were basically turned into nurses. The training was rigorous and scary! They had to make changes to their home in order for the hospital to allow her to go home. They needed to have electrical work completed to support her machines, they needed to make physical changes to their home, including a ramp, an addition, and the installation of a generator (to ensure they would never lose power to her vent). Once they completed all the above they talked about a discharge date, something Cliff and Amber always dreamed about.
On September 3rd, 2014, after 179 days in the hospital, they were going home! They were so excited and nervous walking out those hospital doors. They were so used to knowing their nurses and doctors would be there for them if anything happened. They had become a part of Cliff and Amber's family.
A lot has changed since they’ve been home. Paige is on a lot less medication, she spending time off her vent, and she beginning to meet milestones - she has learned to sit up on her own, started walking with assistance, she is communicating using basic sign language, and she’s really developing her own wonderful personality. But there are still obstacles that they are facing. There may still be multiple surgeries ahead of them, not only her stomach, trach maintenance but possibly for her eyes. They currently receive early intervention, physical therapy, and speech therapy on a routine basis. Any illness is an immediate stay in the intensive care unit. Plus there are the ongoing expenses of having a child with special needs.
Cliff, Amber and Paige have been through so much that most people don't ever have to deal with in a lifetime. Thank you for taking the time to read Paige's story. Now let's rally to keep her on a happy and healthy path so that she can live her life to the fullest!