A life of four days can appear to some, to have little consequence. It can appear to be to some, insignificant. What can one achieve in four days? My daughter Ariana, lived just four days. And as a result of her short life, she saved the life of our third child, our miraculous and unexpected son Oliver.
Ariana Rose was born into our world at 7.34pm, on Friday the 1st of January 2010. She was born via emergency c-section at just 29 weeks gestation weighing only 1.2kils, and measuring a tiny 34 cms long. She had brown curly hair, blue eyes and a fighting spirit never seen before.
Ariana was just 18 weeks “on the inside” when the doctors at a routine Morph scan said the words “something is wrong, we think it’s hydrops”. Hydrops Fetalis is a devastating condition where fluid pools around the organs of the baby, most regularly causing severe lung and heart development delays, often causing heart failure. Our daughter was also diagnosed with severe anemia. At the time of her birth it is estimated she had only half the amount of blood in her body as a healthy baby of that gestation. But despite her being diagnosed with Hydrops and anemia, the doctors had no idea why she was so sick. Hydrops is a side effect of illness, not the cause – so we were all stumped!
We were offered a termination of the pregnancy, and an amnio to find out why our daughter was terminally ill. We refused both. After trying for eight months to conceive our daughter, after having just told our first daughter Lucy all about her new baby, and after spending a lifetime believing abortion is wrong – we could not accept a termination. We also refused the amnio on the grounds that knowing what was making Ariana sick would never make her better. She would still be sick, only we might know why. Providing of course that the procedure did not cause a miscarriage. The risks of miscarriage outweighed our need for knowledge, and so we watched her, through multiple ultrasounds.
The doctors believed Ariana would pass away, inside of me, before she reached 24 weeks gestation. How were they to know they were dealing with my daughter, someone as stubborn as I.
For eleven weeks we were monitored twice a week by ultrasound. I was given steroid shots, counseling and as much support as we deemed necessary. We discussed delivery dates, ranging from 35 weeks, best case scenario, to anywhere from 28 weeks at the worst. At our scan just before 29 weeks we found Ariana with dangerously low levels of amniotic fluid. Ironically her Hydrops appeared gone, and her Anemia appeared stable.
Two days later, our littlest angel stopped moving about inside me. I felt sick with worry, but was sure it was ok. After much deliberation, I went to the hospital for what I thought would be routine monitoring. After making us wait for an hour, I was finally attached to the machines – and all hell broke loose.
I was admitted. Immediately. I was asked about my hospital bag which made me laugh – I didn’t have one! She was not due for 11 weeks and had looked so great just two days earlier! It was decided that since she was laying on her umbilical cord, she was cutting of her supplies of blood and oxygen when she moved. Initially I was booked for a C-section the following day. Matt left to get me some things, so I could at least have a change of underwear. In that ten minutes he was gone, things went from serious, to immediate crisis.
Ariana’s heart dropped off the monitor. I watched her heart rate go from a reasonably healthy 162 BPM, to 30 BPM, to nothing. For the longest few seconds, there was nothing but silence. Right as I was about to scream for help, someone walked into the room. They took one look at the monitor and hit the emergency buzzer. A portable ultrasound machine was bought in, I was shaved, gowned up, and had sexy stockings put on. I frantically called Matt back to the hospital, things had gone bad, she was coming out. Now.
We found her again. She had not moved, it wasn’t that she moved from the monitor. Her heart stopped, and by some miracle, started again. I was monitored continuously for the remaining hour of my pregnancy. While Matt was on his way back, the doctor came to see me. He told me she would die, before they got her out. He wanted me to be prepared that she would not be alive, that we were minutes away from losing the baby we had been fighting for.
He was wrong.
Her eyes opened, once, briefly before she was rushed to the side of the room, where they intubated her, hooked her up to life support machines and told me, amazingly that she was in fact a little girl. We all thought we were having a boy – what an amazing surprise!
I was taken, briefly, to see her in the NICU after the surgeons had finished putting me back together. I lost a lot of blood. They debated over giving me a transfusion. It was decided I could wait, but that the lines should stay in, just in case. Those lines caused an infection in my arm that made me incredibly sick. I was lucky not to end up back in the hospital. I never did need the transfusion.
Ariana spent four days in the NICU as doctors tried desperately, to keep her going. She had blood transfusions to combat her anemia, but in the end, they just couldn’t do enough. At just four days old our littlest angel had a grade four intracranial hemorrhage that left her paralyzed, unable to move, or grasp our fingers as she had been doing, just a few hours earlier. Her kidneys could not be started, she had not urinated in what we guess to be almost two weeks, which caused a massive build up of hydropic fluid under her skin, and around her organs. The only way to remove the fluid, was to wee. Ariana couldn’t.
We asked all our family to come to the hospital, to say goodbye. We knew, that our baby had fought long enough. Our daughter Lucy got to hold her. We have so many photos of the special people in our lives with Ariana. Loving her, holding her and crying over her.
In the very early hours, of the 5th of January 2010, Matt and I held our daughter as she slipped peacefully away. We had asked the staff to remove her ventilators, and all of her drugs, to let her be pain free as she went. Our baby opened her eyes, for a fleeting second, sighed, and left us.
It’s been a year since Ariana came into our lives, and left again. In that time, life has moved forwards, but a part of our hearts will forever remain, in that hospital, with our precious daughter. When we received her autopsy results and the things we learned were staggering. Our precious baby was so much sicker than anyone, including doctors from all over the country knew. Ariana had something called “neonatal hemochromatosis”, an incredibly rare and lethal disorder caused by something in the mothers blood, possibly an enzyme attacking the developing baby’s liver enzymes. This condition causes the liver in the baby to breakdown, and as a consequence Iron that should be processed through the liver is re-directed and stockpiled in the major organs of the baby.
Essentially, it is Iron Toxicity, which in severe cases such as Ariana’s, can show as hydrops and anemia. The chance of it happening again in subsequent pregnancies is as high as 90%. A terrifying statistic for someone who has already been dealt the loss of a much wanted, much loved child.
Ariana didn’t survive the various things attacking her tiny body. But she gave us the gift of love. She gave us a deeper understanding of our family, of our hearts, of what a life can mean. She saved the life of Oliver, the little brother who she will never know, who will never know her. Because of Ariana we knew that my body would attack our child. Because of Ariana, we were able to save him.
NH is incredibly rare. Our hospital has treated just 24 cases, in almost 40 years. Even the most advanced medical professionals refer to Ariana as a miracle. Four days is such an achievement for a little person so sick. Our home is full of her photos, her memories and our love for her. We talk about her, we cherish her and we remember her. And one day, we will see her. She’ll be heavenly beautiful, and heavenly healthy. Until then, we live, we laugh and we love, because we promised her we would.