Back in the ICU, but still fighting

Hello More Than Conqueror followers.

It has been some time since we've posted an update largely because, thanks be to God, we haven't been in crisis, however our family finds itself once again in need of your prayers and attention. As you may have learned by now Ariella is back in intensive care, this time at Yale New Haven Hospital. She has been sick for several weeks. Prior to the hospitalization we thought it was just another cold, however, when she continued to worse and her blood oxygen level began to slowly dip over time we knew something more severe was happening. She was initially given antibiotics on December 23rd. This was a general antibiotic that was meant to treat a number of possible infections. However, after three days on this antibiotic and no improvement we followed up with our pediatrician on Saturday the 26th. During this doctor visit we learned that Ella had a double ear infection. Up this point our baby has occasionally had fluid in her ears, but it has never turned into a single ear infection let alone two. We were given a new antibiotic meant to specifically treat the ear infection. Before we could give her a dose on the new antibiotic she got much worse. Saturday during her nap she was not getting enough oxygen in her blood and by Saturday night we were in the emergency room at Connecticut Children's Medical Center. Due to over crowding at CCMC Ella was transported to the pediatric ICU at Yale New Haven. She was admitted just after midnight Sunday to Yale and within a couple of hours we have learned that in addition to the double ear infection Ella had pneumonia and RSV or respiratory syncytial virus. RSV is a virus that causes infections of the lungs and respiratory tract. RSV is a fairly common virus that effects most children, however for someone in Ella's condition with profound muscle weakness and an already compromised respiratory system RSV can be vicious when you add pneumonia on top of RSV you get a perfect respiratory storm that put our little warrior in the most critical state of health since we received her diagnosis and she was hospitalized at 6 months of age. Since her admittance last Sunday, thank God the double ear infection has been cured, however the pneumonia and RSV continue to present the most difficulty for our little conqueror.

For those of you that have followed the blog in the past you know that Ella's respiratory system is already compromised by the nemaline myopathy.  When Ella is healthy there are two things that we pay the most attention to, her physical safety because she is a fall risk and her respiratory system. Either could put our angel in the and most critical health state. Because she does not have the muscle strength to brace herself even a small fall could get result in a severe injury. And in terms of her respiratory health if her lungs get too clogged with mucus and secretions than she will not be able to effectively breathe on her own. Furthermore, we have learned during this hospitalization that she has trouble exchanging oxygen and carbon dioxide, which means Ella tends to retain more CO2 than it should.

Having said that as a result of this perfect respiratory storm the lower portion of both her lungs are clogged. Since last week we have tried a number of non-invasive therapies to help Ariella recover and expel the mucus to clear her lungs, however over the past five to six days Ariella has had multiple critical events that have resulted in her oxygen levels in her blood falling below 70% and in one of the more severe episodes reaching 60%. When you consider that when someone is healthy their blood oxygen level is usually between 98% and 100%, falling below 80% represents a real problem and for someone like our little angel falling below 80% is a very severe event. The first two times Ariella was able to recuperate with some more intense non-invasive therapies, however, while I was staying with her on New Year's Eve she had the most severe episode and could not recover on her own as a result a more invasive measure was needed to help her breathe and clear the secretions. Just before the new year our baby had to be intubated. She now has a breathing tube that has been inserted into her mouth down into her airway that gives us direct access to her lungs. This way they're able to give her more concentrated and controlled pressure support to help her lungs remain inflated and it also provides direct access to suction out the mucus. Yesterday the doctors also performed a bronchoscopy, which is a procedure that takes a tiny camera attached to a very narrow suction tube directly into the lungs and into the airways to try and target the areas where there are pockets of mucus to remove them. The pulmonologist felt that this procedure went well and that they were able to remove a good amount of mucus however, her lungs are not yet clear and her respiratory treatment regimen has been increased to every 2 hours which makes it very difficult for her to rest and recover between each treatment. Ella is fighting and hanging in there. We have not had any more critical events since Thursday and her numbers continue to hold at a positive level. We appreciate all of your support and concern. At this point truly the most important thing you can do is pray that are baby recovers and that her body gets healthy enough to fight off these infections and expel the mucus and secretions out of her lungs. Thank you so much for following the blog, check in daily for updates and any news about our little warrior. Thank you Jesus we are all more than conquerors!