Mirabelle was born on April 5 2018 with a rare congenital heart defect known as Hypoplastic Left Heart Syndrome (HLHS). Congenital means "from birth". An anatomical defect.
She touched the lives of many people despite a gradual decline in her state of well being after enduring two open heart surgeries in the first year of life, as her single right ventricle had poor function and only got worse with the passing of time.
HLHS was an uphill battle from the start, with her heart stopping twice during surgeries, invasive cardiac catheratizations that left her on her back for weeks, the stent moving out of place after her Hybrid surgery, filling with scar tissue, and the comprehensive stage 2 Glenn-Norwood surgery failing to address her failing right ventricle.
Unbeknownst to most "cardiac parents" (parents of children with congenital heart defects), repeat surgeries also cause a decrease in heart function due to the build up of scar tissue. Mirabelle most likely was in the subset of HLHS children living with a "single letter" defect in her DNA which causes the filament of cardiomyocytes to malfunction, all of this adding up to a weak heart as a whole, even beyond the fact she only had one right ventricle. (See: Mayo clinic HLHS single letter defect)
For her second surgery, I wanted to take her to Boston Children's hospital very badly as they pioneered new therapies, and so I demonstrated my knowledge of HLHS so far and made acquaintances with Dr Sitaram Emani. Emani and I talked on the phone and was at first on board with taking Mirabelle in. As we talked, I came up with an idea for a new medical procedure he could use to "recruit" atretic HLHS patients into his biventricular conversion procedure. He said it was a great idea, and thanked me for thinking of it! Looking into possible solutions, I noticed at Boston, they put stem cells in the heart during open heart surgery which strengthens the hearts of children with heart defects. It could have been great for Mirabelle. Unfortunately, after a blunder in my communication with Dr Emani, he told me "don't get on that plane". We had no choice but to go to Texas for her surgery, where they don't do stem cells. How much longer she would have lived will always remain a mystery.................I also had spoken to other Drs from Boston and they had mentioned mitochondrial therapies would not help her. It does seem that now in 2022 this is not necessarily the case, as shown by newer research papers.
In addition to open heart surgeries and procedures, Mirabelle needed to take oral medicines for her heart Every twelve hours since she was a baby, and after so long, it was a challenge to get her to take the meds. As time progressed, she was unable to receive any treatment due to the advanced state of heart failure she had, preventing her from getting any treatment that involved sedatives.
During her first year of life she still looked normal and could still walk. We enjoyed beautiful moments together. She absolutely loved ducks at Paradise park in Reno NV. She loved flowers, especially roses (like her name), mozzarella cheese sticks she called "fish" (because fish sticks look like mozzarella sticks and the name stuck), and towards the ends she became obsessed with beads and....Vivaldi, four seasons-Spring. Funny as she was born in the spring.
Mirabelle did not seem to be a regular child. At the age of two she was obsessed with classical music, and played a small piano with extreme precision despite no teaching of the instrument. She would play note by note with one finger going up the keys then go down the keyboard in the same fashion. She was sensitive and observed details around her with maturity. She would understand things that happened around her. She had a sense of humor. She loved her parents, her toys, and nature. She was special outside her heart condition. If you spent time with this little girl you too would notice her awareness, curiosity, and affinity for music. We would head out and conquer the world as a team, exploring things, driving with songs, blowing big bubbles and singing while swinging, being surrounded by a hundred birds taking off in flight simultaneously by the lake. A marvel to behold. Moments lost in time but gathered in the mind. A mosaic of memories. We loved each other very much as mother and daughter. I was a very involved parent, truly sacrificing myself to make my little angel happy.
Unfortunately these happy moments were numbered.
As her other organs started failing following her failing heart, there was nothing we could do to help her. She started to look badly.
I was doing my best to make her happy but misinformed and nosy people at the store would call cops on me thinking she was abused due to her appearance. She was bluish/ashen, and the skin beneath her right eye would always have a tendency to be more discolored than the left. I assume that's because her right ventricle was failing and accumulating fluids in her tissues. The little failing half-heart was holding up a fort twice its size. The cardiologist would up her oral meds but to no avail. Additionally, some of the oral medicines had undesirable side effects such as swelling and flushing out electrolytes, exacerbating some issues.
I had cps called on me, cops harassing us, not believing a word I said, despite showing papers from the hospital. I had to put up with harassment I never deserved, adding insult to the injury. The rude cops made Mirabelle cry when they flashed a flashlight at her. I had to get in the way of the light and tried to explain, but they still didn't believe me, until they heard from her Dr. It was ridiculous! After this, Mirabelle would cry if a cop appeared on TV.
The CPS girl Laura Capriolli was extremely unprofessional, stepping into our apartment, calling grocery bags filled with bought food "garbage" and acting unprofessional with Mirabelle's father Miles right in front of me. Additional CPS people did nothing but harass us once again, asking to open our cabinets and questioning her oral medicines. We were even followed by police inside Target. It was absolutely ridiculous.
When we headed out, I had to start wearing a "special T-Shirt" explaining the situation in writing (see pics) so people would stop calling cops on me. I had to get stickers for my car about CHD so they "get it". Even still, most people were very rude and would not believe me. I would have to frequently show her chest scar to prove it. It was absolutely Heinous. We were never treated normally. Except the very few who truly understood her condition. I quickly and painfully noticed that society has nowhere nice for terminally ill children to go to to live their short lives. We didn't belong anywhere, and nevada governor steve sisolak closed down Mirabelle's only favorite indoor playground Jump Man Jump during this time.
The only stimulation I could offer Mirabelle was going grocery shopping and the park. There was simply nowhere else to take her. At the park, strangers tried taking her picture right in front of me. She was only 2 and with a failing heart and this was so much unnecessary extra stress to deal with. I made her a fantastic indoor play area but she still always enjoyed heading out.
We continued heading out because she asked to go out every day as it made her happy, and I kept doing my absolute best to make her happy even until the very end. She received the absolute best diet possible which also passed on antibodies to her. Immunity was not a concern, it was the fact her heart was failing and she was not a candidate for a transplant*. "She would just die on the table" her cardiologist Dr. Joseph Ludwick said.
"I have no idea how she's still alive!!!" he once exclaimed, saying she was a "one of a kind patient in the entire nation" (2021).
We were noticing a steep decline in her well being as she developed massive ascites and a protruding liver from heart failure. Even her kidneys appeared to be failing. I helped her breathe one day by lifting her off her own fluid-filled belly. My heart bleeds writing this.
*In the woes of desperation, I thought of a wonderful solution which could save her life. I filed my invention as a PPA with USPTO. Unfortunately, I didn't get a chance to try this invention out..........
On the Eve of March 25 2021, my adored Mirabelle woke up, and asked for cookies and fish as usual. She was on her bed. When I brought them to her she started eating, sitting up. Moments later, she cried and fainted by falling forward from a sitting position.
I caught her in my arms, panicking, patting her back, shouting baby? baby please? baby?! Mirabelle was emitting a gurgling sound. I blew air into her mouth but nothing happened. My own mom and Mirabelle's dad Miles ran to the scene. My mom did CPR and laid her down on the bed to do chest compressions. I saw her eyes roll back into her head and I knew she was gone. I touched her chest and felt no heartbeat. I screamed "AAA her heart stopped AAAAA!!!" After a few compressions blood started coming out of her mouth. My mom cried "oh no". I was screaming and grasping to the wall with both arms screaming GOD PLEASE NO PLEASE GOD NO and sobbing wildly. Miles and I held each other screaming. I vomited all over myself. The dogs were barking. Miles called 911 but I knew in my heart she was already gone.
Cops flooded our apartment threw her on the ground did more CPR and took her away in an ambulance. I changed clothes and got in the car with my mom and Miles, her dad. I couldn't drive so my mom drove us to the ER.
We were in the waiting room, pleading, hearts pounding, sweat dripping. After a long and laborious attempt, the ER could not bring her back. A woman came into the waiting room, grabbed on to the edge of the door, looked at us and said quite nonchalantly, "she's dead".
When I came to see her in the Emergency room, a team of personnel stood looking at me with shock. She was pretty blue and had a tube coming out of her mouth and pads glued to her little chest. It looked like she had gotten lines placed on her ankle and wrist then removed and fitted with gauze pads. It was horrifying to see, all of it. We asked them to please remove the tube out of her mouth and let us spend time with the body. One good nurse told us that her body was just her "shell" and that she didn't feel anything and not to worry because she wasn't "there".
I was able to spend time holding my beloved Mirabelle's "shell" in a private room alongside my mom. When I held her body, blood came pouring out of her mouth and drenched the right side of my shoulder and bra strap. She felt heavy and long for a two year old, almost three year old. Her long white legs dangled down my body as I comforted this "shell". I held the body close to me and said "there there..... there there". Unbelievably, my mother was there and captured this horrendous, chilling moment. From the angle, you can't tell she's long gone.
When we returned home it was already sunrise. I somehow managed to sleep. When I woke up I was in such physical pain I could not walk properly. I felt like I was walking on shattered glass. I felt weak and limp.
Days went by and I was faced with a decision having to choose a mortuary. This felt like adding salt to my wound. Eventually I chose one and thankfully a special club called the Aubri Brown foundation covered all the burial and casket costs. I thanked them but never heard back.
The next day March 26, at night, as I was laying fully awake on the floor with closed eyes, I suddenly saw something in my mind's eye. I saw a figure rising from the dry hills in northern Nevada, up to the sky, filling a space between clouds with its figure and lighting up the sky. After the sky flashed with light, I saw that this figure remained put. I woke up gasping because this was scary and took me by surprise.
Her body was being held at a mortuary, and was arranged for viewing. She looked less swollen dead than alive, my poor baby. My family flew in from all over the country to see the body. we all held her and kissed her even if that was just her "shell". Days later, the burial took place and my good friends showed up. Miles said this is the worst thing that's ever happened to me. When it was my turn to speak I shared Mirabelle's fight with HLHS, and her struggles, mentioning she will be loved for all eternity. We adorned her body with her favorite toys, and other meaningful items including a long snip of my own hair wrapped around her hand.
Over the next few weeks I would smell her sweet scent in the air, or wake up with her scent inside my nose.
On May 10th, she visited me in my dreams. I dreamt my family and I were driving in a car through a city with long roads, and that the further we drove, the more flowers the trees had. I knew I was getting closer to her by the amount of flowers. When we arrived at our destination, it was a victorian-style house. I knew she was inside waiting for me. I ran up the stairs and saw her waiting for me with a big smile. She looked normal, and taller. With a big smile. I ran up to her overcome by emotion, dropped on my knees and hugged her tightly with my full body. My mom and Melissa (my sister) stood nearby each other, smiling witnessing this encounter. As Mirabelle and I held each other close, the camera spun slowly around us for what literally felt like a full live hour. You could see and feel details of this living room with extreme precision. There was soft sunlight bathing the air through frosted or glazed windows with some coloring on it. The carpet was soft and cream colored. There was music playing, it was like a soft touching piano. We continued to hug each other tightly for this entire length of time until I woke up.
This experience was extremely healing and allowed me to metamorphose into an MIT student online studying molecular biology with the expectation that I can find the cure to HLHS genetically so nobody has to ever endure what she went through.
On July 2 2021 I heard from Kelly at the cemetery that her stone was laid over the burial spot. I find myself extremely emotional still because although I had that healing experience, the truth is that no matter what, nothing can bring her back.
I live in a way blending strength and pain ever since. I strive for something bigger than myself. I have been doing well to improve myself and gain knowledge to help mankind.
I long for my beloved daughter, her bright blue eyes, her cute red hair, her porcelain white skin which I loved to kiss. Her soft voice and her choice phrases, "happy heart, more fish? Paper? Box? ....." her smile is etched into my mind. I can see it even with eyes open. I fight flashbacks. The flashbacks of her death haunt and terrify me to this day and probably for the rest of my life.
I miss tickling her pushing her on the swing and getting her toys. If she could have been saved we could've done everything together, had so much fun, live life uniquely going on adventures...
We weren't just mom and daughter, we were truly friends from the start. She loved me very much I could feel it. I was her comfort, I was her rocking chair. All of the possibilities we had were taken away from us. There is absolutely no replacing a dead child and the pain is incomprehensible to someone who hasn't lost a child. I came into motherhood with original ideas that would have increased creativity and amplify talents. I wanted to "parent my own way" and now I find myself filing patents instead.
I live to create the cure, make it accessible, low cost, and would like to welcome other families affected by congenital heart defects such as Hypoplastic Left Heart Syndrome, and hold other "heart kids" close to me and let them know they are loved and special. I understand that most people are not aware of heart defects, much less ones such as serious as having half a heart like HLHS. CHD research is underfunded. I seek to fund it myself and be a new researcher. I understand these Heart children grow up with unspeakable anxiety as they too could face heart failure at some point themselves. No human heart functions normally and well with only one ventricle--Period. The right ventricle is not shaped to do the job of the left. It's more crescent-shaped, thinner, and smaller, designed only to pump to the nearby lungs, not the whole body like the piston-shaped left ventricle. The Fontan physiology is a great deviation from a normal heart and comes with so many problems, protein-losing enteropathy, persistent low oxygen levels, lifetime of testing and medical requirements. Eventually most if not all patients still require heart transplants but there is a scarcity of available hearts, and a terrible lineage of hoops to jump through just to have a chance at one. Even if you obtain a transplant, the body can reject it and you're back at square one. You would need a lifetime of immunosupressant medicines to tolerate the foreign tissue and those medicines lower your immune system. Each surgery carries risks. As a new HLHS parent, I was shown all the happy stories and fed the hope of survival in a sugary spoonful. But what they DO NOT tell you is that EVEN with the surgeries, survival in general is not guaranteed. In fact, there ought to be a protocol in which if an HLHS patient is born with weak ejection fraction, and not given stem cells, they should be disqualified from the "single ventricle road" (the reconstructive open heart surgeries) as they would only cause pain and suffering and not offer a life or a way of living for them. Comfort care should still be offered if the case is very severe like Mirabelle's. Her life was gambled with. They ASSUMED her failing ventricle would somehow turn around after the Glenn, which it did not. The heart needed strength, which it did not receive. How can a different result be expected?
I have studied HLHS on my own since 2018 and have taken additional courses in molecular biology. I would like to contribute. My goal is to provide an alternate course of treatment to Hypoplastic Left Heart Syndrome, one which involves zero open heart surgeries and provides the patient with his or her own full heart and is able to be released to go home in days to weeks after treatment. I didn't survive Aplastic Anemia as a child myself for nothing.
In grievance,
Kyram R. Singleton
Thank you for reading
