Catherine and I have always been very pro-active with the care for Sophia. Over the first 2 years their have been some really scary moments. We have always made choices as Sophia’s parents as to the best course of action for our little princess. We have come very close to losing her …..but remained calm and continued to work on her and eventually brought Sophia’s oxygen and heart rate back up. One of those instances was last May when we were returning from seeing Dr. Schroth in Wisconsin. We were stranded in a hotel and Sophia crashed….it was very clear we did not have time to wait for help to arrive…….Sophia was motionless and the sparkle in her eyes were gone, we were left with a blank gaze. Catherine and I continued to work on her and after what seemed like an eternity we were able to get her numbers back up and put the bi-pap back on her and she closed her eyes and fell asleep . As an SMA parent over time you gain this uncanny ability to assess the situation to make the best choice for your child. We have been able to battle many times at home……….Saturday it was clear would be different.
Saturday morning we all woke up fine , started our typical ritual gave Sophia a saline nebulizer to break up her secretions, did chest pt and cough sets and put the Bi-pap back on her. Sophia’s sats were fine. I started to do some chores around the house and Catherine called for me to come right away. I ran into Sophia’s room her Oxygen levels were dropping she was in the 80’s her Hr was 210-215 and trending up. I quickly grabbed the oxygen and put it inline with her Bi-pap. Sophia’s body was shaking with every heartbeat. Her jugular vein was pulsating out of control…….I was afraid her engine would give out. With the addition of oxygen her sats came back up to tolerable levels………… 97 and maintaining but her heart rate was still out of control. We grabbed Sophia’s stethoscope and her lungs were clear. Catherine grabbed the thermometer and took Sophia’s temperature the most accurate way. She was 102.6 and burning up. We knew she was sick but had no idea of what it was. We were able to maintain Sophia’s oxygen levels with a bleed of O2 so we decided to take Sophia in her own van. I had recently upgraded the inverters to handle Sophia’s equipment and her van is essentially a portable E.R. We have back-ups of Sophia’s equipment that always stay in the van ready to go. Had we not been able to maintain Sophia’s numbers with the O2 bleed we would have called an ambulance with intubation abilities. For our family that would have told us Sophia needed a secure airway, thankfully that was not the case ……….we felt the safest option for Sophia would be to transport her ourselves. I called Dr. Schroth and described the situation and where we were going so she could open lines of communication for Sophia’s care. Our next call was to the Chief Pediatric Pulmonologist at Winthrop hospital to inform them we would be coming in. They would be readying a team to meet us at the ER and transition us to the Pediatric Intensive Care Unit once Sophia had been admitted.
The ride to the hospital was uneventful and Sophia was maintaining 97 on her oxygen, during the drive I received a call from the hospital informing us that everyone was ready. When we arrived at Winthrop we went to the Emergency Room and were ushered into a private isolation room. Preliminary cultures and labs were ordered to screen for all infections and viruses. Sophia is a very hard stick…..and unless the person is highly skilled with pediatrics it can be torture. Sophia has extremely low muscle tone and her veins float. Luckily the 2 RN’s chosen for Sophia were amazing! They were able to perform the catheterization on the first shot as well as insert an IV and draw blood from that port prior to administering fluids. Sophia was only stuck once! Based on the cloudiness of the urine sample and some irritation the initial assumption was a UTI. The team in the ER prescribed an IV antibiotic and called Dr Schroth to keep her informed on their findings. Shortly after we were brought to the Pediatric Intensive Care Unit.
I had previously been to the PICU at Winthrop on multiple occasions to help another local SMA family. This was the first time Sophia would be admitted. The PICU rooms at Winthrop are heads and shoulders above any of the local hospitals we had been to. Every child has their own private room. The outer walls are glass so the nurses can always see in, but there is a privacy curtain that can be drawn across the entire wall when parents are their with their child. The rooms are new, spacious and very clean. Sophia’s room had a brand new Stryker bed with an onboard scale and automatic inflation system that continually adjusts to relieve pressure points. On the other side of Sophia’s bed was a couch that folds down to become a full size bed for parents who stay with their children in the PICU around the clock. We were thoroughly impressed with the level of professionalism shown by the entire staff from the nurses all the way up to the Chief of Pediatric Pulmonology. We had become accustomed to battling with staff whenever we had brought Sophia in for her surgeries, this was the first time we did not have to. Every step of the way the hospital stayed in touch with Dr Schroth for Sophia’s care. The staff saw how we cared for Sophia and the way we performed CPT and Cough sets on her. Instead of trying to impose their will, they were more interested on how they could support us in the best care for our daughter. The co-operation was such a relief in what was already a stressful situation and it immediately became clear we would all work together as a team to provide Sophia the best care. Prior to leaving Winthrop another set of labs had to be done on Sophia, this time the staff was unsuccessful in drawing blood on Sophia they tried 3 times. We did not want to see Sophia continue to suffer from multiple attempts at a catheterization and blood draws so we requested the original team from the ER to come and perform the required labs. The PICU obliged and made the call………the ER team was successful on the first try with both and Sophia did not cry at all.
We are very thankful that our first hospital visit for an illness went as well as it did. Winthrop was a wonderful facility for us and we would recommend them to our local SMA families for a care option on Long Island. Thank you to all of our friends and family for the prayers and kind thoughts during this situation, we truly appreciated the support and it helped to uplift our spirits while we were in the hospital. Sophia is now home and happy to finally be in her own bed and missed her brother terribly.