August 2, 2020
“Do not fear, little flock, for it is your Father’s good pleasure to give you the kingdom” Luke 12:32
Friday, January 4, 2019, I was driving back home by myself and my phone rang. It was the cardiologist for Sophia. He usually calls me after the echo each year to give me the result of the echo and tell me that everything is normal, but this time was different. I don’t know how I drove home while I was talking to him, I only remember him saying, “Sophia has heart failure, the ejection fraction is not normal, her numbers came back at 40 and the normal numbers should be between 55-60. Sophia will need 3 medications for the heart twice a day. One of them we will have to increase the dose gradually. Pay attention to her very closely because 2 of the medications will lower her blood pressure and may cause her to get dizzy or faint. If she gets dizzy or can’t breathe take her and run to the Emergency Room. I want her to start her medications today, so I will put the order to the pharmacy right now, so you can pick it up within an hour.”
After I hung up, I stayed in my car for 5 minutes because I couldn’t believe what I just heard. I didn’t know what to tell John and Angelina when I walk in. I couldn’t believe this is it. We reached a dead end. Sophia was just diagnosed with cardiomyopathy. Cardiomyopathy means your heart muscle is weak. In most cases cardiomyopathy is curable or manageable with medication, but not in Sophia’s case. Once Sophia’s heart muscle gets weak, it can’t be reversed. The muscular dystrophy disease will weaken the heart muscle until her little heart is too weak to continue beating. The medicine was literally just to buy us some time with her, but no one can predict how much time.
As soon I walked in to the house John and Angelina can tell right away there was something wrong with me. I was thinking to tell John alone but I broke down in front of both of them and said Sophia’s heart got effected with the disease. I explained to him what exactly the doctor said and he said let’s get the medicine. I don’t think at that moment John fully understood what the doctor said or what it really meant. Sophia didn’t know what was going on. While we were picking up the medicine from the pharmacy, Angelina recorded a video of Sophia singing a beautiful hymn (see Video below). It was her first time ever singing a hymn. We didn’t even know that she had memorized any hymns. We felt while we just got the most devastating news about our child, our child was glorifying God. At this point we understood God’s message to Sophia. We also understood why the surgery was moved from March 2019 to October 2018 and the cardiologist appointment was moved from October 2018 to December 2018. Nothing was planned by us. It was all God’s plan and timing. It was His will.
The very next Monday I pulled her medical file and called every doctor on her team to schedule an appointment with each one of them. I knew the one who will give me the bottom line was her genetic doctor. Her orthopedic doctor said don’t bother with her braces because he wanted Sophia to be comfortable at this point. Her GI doctor said don’t force any food but make sure she has something before you give her the medications. Her Pulmonary doctor said we need to do the sleeping test because her lungs muscle might have been affected also and she will need a breathing machine during her sleep. Her genetic doctor confirmed everything that I was scared of. John asked her straight forward how much time do we have? She said I can’t give you an answer, but she asked Sophia where she wanted to go. Sophia said, “I want to go to the zoo.” The doctor looked at us and said, “take her to the zoo as soon as you can.” Also, she suggested that we see a palliative care doctor. At this point, I knew where we were heading. When people get referred to palliative care doctors, it means it is time to get the patient comfortable their last few months.
Her cardiologist said he will treat her as a regular cardiomyopathy patient. The medicine might work and improve her ejection fraction and might not, only time will tell. He wanted to see her every week so he can increase the dose and will do another echo at the end of February.
We were able to get an appointment with a pediatric palliative care doctor. He was so sweet and kind. He knew exactly what we are dealing with, and how to treat us. The first thing he said, “there are only 50 cases of LMNA muscular dystrophy documented in the medical archives in the world and your daughter is the youngest one whose heart is affected from this disease on record. The youngest one before her was 7 years old when his heart got affected.” At this point, he said that my contact will be with him only and he will be the one to contact any other doctor from her team, to make it easier on us.
What got our attention was that Sophia attended all of those meeting with us and she never asked anything. It’s like God closed her ears around certain words. Both the genetic doctor and palliative care doctor advised that we should talk to Angelina and explain what was going on with her sister and prepare her. Was it easy to do that? No, but we didn’t have another choice. Our life at this point turned upside down and we were so lost and scared of how life will be without her. John and I both agreed that the only way we could go through this without being broken is by sticking to God. Prayers and communion were our only protection. Were our days going smooth and easy? Definitely not! It was going worse than we imagined.
Around the end of February, we did another echocardiogram and the ejection fraction went down even lower than before. It reached 36. At this point, Sophia started having a hard time swallowing and eating. We also did her sleeping test and her Pulmonary doctor said she will need a BiPAP machine during her sleep because her lung’s have been slightly affected.
Her genetic doctor contacted me after she received all the updates and urged me to take Sophia to the zoo, because that was Sophia’s wish, and she was concerned that we might not have enough time. She also sent me an article about LMNA muscular dystrophy end stage, so I can get an idea of what to expect. The article stated once the heart is affected the average life expectancy is between 12 to 18 months. According to all the symptoms, the genetic doctor and the palliative care doctor didn’t expect Sophia to make it to May of this year, so only 3 months from now.
Lessons Learned:
- When life gets too hard to stand, kneel in front of God
- It is in your pain that God is closest to you
PLEASE NOTE – for all of the “lessons learned”, we did not always see it that way during the events that were occurring which are mentioned in the posts. Many of these lessons were learned weeks, months, or even years later when we looked back and reflected on the circumstances.
