Vivienne L.

My beautiful Vivienne was born on December 30,2019. She weighed 8lbs.and 6 oz. We were all very happy with her arrival. Vivienne’s sister was very happy and excited that she was finally going to be a big sister (big age gap difference 12 yrs). We noticed the day that she was born; she wasn’t very interested in the bottle, they told us that it was normal,  that many newborns are not interested in their bottle. We were  discharged from the hospital and they told us to follow up with our pediatrician for Vivienne’s check up. We followed up with our pediatrician and they told us that they were worried because Vivienne was losing weight very rapidly. They tested her for jaundice, and they checked her glucose levels in the office, and they all came back normal. They told us to come back the following day, so they could check her weight, again. They stated, if she loses weight again in one day, you need to take her immediately to the hospital; or, if you feel that something is not right then take her to the emergency room tonight.

I took Vivienne that night to the hospital. I knew in my heart something was not right. She was already vomiting frequently, lethargic, and not very responsive. They were running many blood test on the baby. They pulled me to the side and told me that they thought that she had meningitis. They said that they were going to do a lumber puncture (spinal tap) similar to an epidural. Next thing we know we are in the NICU! Once they transferred us to the NICU they said to us that my daughter had very high ammonia levels. They told us that nothing looked favorable for Vivienne and that most likely she was going to pass away. They asked, If we wanted to get a priest to give her holy sacraments. She was in need of a line for dialysis and that the first step was to  take  her to an anesthesiologist to put in a line, and they were not sure if she would even get past that point. She made it out and they transferred her to the PICU. That’s when they told us that our daughter had Propionic Acidemia. They gave me a folder with all of the information. Vivienne had a team of doctors (neurology, kidney, cardiology, genetics, and pediatricians). She was on dialysis for a couple of days and thank God her ammonia levels went down and it was time to disconnect her from the dialysis machine. All of her teams signed off that it was okay to take her off the machine. Once they took her off the machine she didn’t respond. She started bleeding internally, she started having pulmonary hemorrhage, they didn’t know why. All the alarms from the hospital started ringing. All the doctors started coming into her room trying to revive her and intubate her, because now she couldn’t breath on her own. As a parent seeing your child losing her life in front of your eyes it’s extremely difficult; you feel impotent, useless, helpless. It was a traumatic experience to go through as parents; to see in front of your eyes that your daughter is losing her life.She goes back on dialysis and the medical team decided to put her enough blood product; so she wouldn’t bleed out and coagulate (she ended up with a blood clot). She was sedated due to the intubation so she wouldn’t feel pain.They say babies don’t cry but I saw a tear out of her eye that day. They would tell us to go to the Ronald McDonald Charity house many times to rest;  but, I couldn’t leave my child. I was with her at all times. Then, a couple of days later she comes off the dialysis machine without any problems and the breathing tube was removed too; then, they said that she would need another operation. They said that they wanted to perform an operation for a g-tube. They said that could be a backup plan, if she ever didn’t want to eat by mouth. At first, I didn’t want to go forward with the operation and my husband did want to go start the process. Then we talked and decided to go forward with the g-tube operation and it was the one best decision that we took for her.The whole month of January 2020 she was hospitalized. When she was sent home we had to learn how to inject her Lovenox for her blood clot( it was one of the hardest things in my life to inject my baby twice a day), and give all of her medication and prepare her formula.


Then, in February, she was in the hospital again she had a metabolic crisis. In March, she was hospitalized again due to a metabolic crisis.  By this time, I started to recognize the symptoms that would drive Vivienne to a metabolic crisis.She would spit up, vomit, not interested in her feed, very sleepy, lethargic,hiccups, gagging and dehydrated lips. By March, my husband and I had to organize ourselves. He would take our oldest daughter to school everyday and he would take care of things. I would stay with Vivienne in the hospital. Due to Covid-19 many things changed,it was difficult for us; hospital policies and visitation rules changed. Overall,  Vivienne got tested for COVID-19, six times because that’s the hospital’s  policy any time you get hospitalized you get tested (testing had to be done to keep our hero’s doctors, nurses, and staff safe). In April, again she was hospitalized due to a metabolic crisis. In May,  she was in the hospital for three weeks for a metabolic crisis, and they also thought that she had another rare disorder called hirschsprung’s. They decided to do a biopsy and she tested negative, and the whole month of June she was hospitalized until July 03,2020.

It’s the first time in Vivienne’s life that she has been out of the hospital for more than a month! It’s been amazing! We have been going to genetics appointments weekly and then bi weekly. There have been formula changes every week, lab tests every week, but Vivienne has been home without any IV lines going bad and no daily lab work for ammonia levels, which is great! I am very thankful to God that Vivienne has been much better! Her dietician is amazing, very proactive, very attentive, and professional (dieticians are very important in our babies lives). The Propionic Acidemia Foundation web page has been very helpful! The prop nutrition management toolkit was very helpful! There’s plenty of helpful and educational material on the foundations web page. I also wanted to thank Jill Chertow… She has been my support since the beginning of my journey! She has guided me and has always listened to me. I have cried and she has comforted me. I needed advice and she has provided it! She gave me courage to keep going in my hardest moments.

updated 9/2020