A BIG FANTASTIC UNEXPECTED SURPRISE

     Ava was born as quite a surprise to Lewis and me. What was thought to be appendicitis with severe abdominal pain sending me to the hospital, was actually this brilliant and most beautiful child. To our complete shock, on September 25, 2021, at 9:25pm, Ava Louise was born in the toilets at A & E. A true miracle baby! 

     After being resuscitated shortly after birth, Ava was taken straight to the neonatal unit for tests and bloods. Whilst coming to the realisation that I was now a mummy, I went to see my new daughter. She was connected to wires and machines and was being poked and proded by needles. Ava was in an incubator because she couldn’t maintain her own body temperature. My mum, Sarah (Ava’s grandma), stayed with me the whole time and shortly after swapped places with Ava’s daddy, Lewis, who then came to meet Ava for the first time. 

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LEARNING TO BE NEW PARENTS THROUGH MEDICAL CHAOS

     Ava was extremely jaundiced when she was born. Because of this, she was put under the UV light for a few days which meant minimal chance to hold her, although we were able to hold her hand through the small incubator doors. Lewis and I spent a few weeks at the hospital with Ava watching nurses do bloods, tests, and imaging. They cared for her and were such a big support to me and Lewis being new parents. They taught us as we went along. 

     Ava started to slowly gain weight and took milk through her mouth rather then the NG tube she had in her nose. This gave us a slight chance of hope that we would be able to go home and meant that, in terms of development in the NICU, Ava was slowly improving and showing signs of "normal".

ALL SIGNS LEAD TO RARE LIVER DISEASE

     Ava’s jaundice didn’t improve though, and blood tests showed she had high bilirubin levels which meant issues with her liver. There, hospital staff got into contact with the closest liver specialist which happened to be at Leeds General Infirmary. We were held back from going home a while longer and instead transferred there. This is when we had the hard realization our child would have long-term medical needs and that her medical issues were more severe than we had known.

     At Leeds, Ava was taken for a HIDA scan. This test is used to check how well the liver and bile ducts are working and can be used to diagnose cholestasis. The doctors suspected biliary atresia, a rare cholestatic liver disease where bile ducts inside and outside of the liver do not develop normally and prevent bile from reaching the intestine. While the HIDA scan didn’t confirm any diagnosis, Ava was taken to the theatre for a very invasive Kasai procedure at only 6 weeks old. This is a big surgery performed specifically on biliary atresia patients in infancy. The doctor's intention was to remove blocked bile ducts and the gallbladder and replace them with a segment of her intestine. But, it was clear to the doctors during surgery that Ava did not have biliary atresia, so they closed her up and sent her back to the NICU ward. They then gave Lewis and me the devastating news that she would need a liver transplant due to Ava's diseased liver. We had no diagnosis again, but knew it was a form of cholestatic liver disease.

TESTING CONTINUES, LIVER TRANSPLANT IS EMINENT, EMOTIONS RUN HIGH

     While back in the NICU, Ava had a blood transfusion and lots of medication, but quickly recovered. She was taken to the liver and kidney ward where we spent a couple of weeks and also met with the specialist nurses and doctors on L50. It had now been 8 weeks since she was born. In that time, we fought through some mental battles as parents. The devastating news was hard. Our little girl faced surgery so very young. Ava’s consultant, Murumbo, noticed how we had become disheartened and gave us the gift to go home and a chance at normal life with Ava while we waited to move to the new chapter. We we will forever be grateful for that.

HOME FOR THE HOLIDAYS AND LIVING LIFE TO THE FULLEST

     Ava was given just over 2 weeks at home. She got the chance to meet her family and our friends whilst being on many meditations and a feeding plan. We were extremely grateful to also have her first Christmas at home. 

     Ava’s care stayed under the liver specialists in Leeds and a dietician whose main focus was to help improve Ava’s weight gain. We had a local health visitor who kept in contact with Ava’s dietician and the hospital after weighing her weekly. We took Ava to see Father Christmas, to the aquarium and did so many other things during this time of having her at home, but it was cut short after a phone call from the hospital telling us that Ava needed to be readmitted and that this stay would be long term until she was ready for her transplant. 

​READMITTED AND PREPARING FOR THE UNKNOWN

     Her weight gain had been poor and remained poor, weighing 2.90kg, less then she should for her age. We wanted her to be as big and strong as possible for transplant so she was put on Total Parenteral Nutrition (TPN), a special formula given through a vein used to provide most of the nutrients the body needs. We still had no diagnosis for Ava, we just knew she was still jaundiced and that at some point she would need a new liver. Straight after the day of admission she was taken to theatre where she had a broviac line placed in her chest. 

     We spent 5 months on the ward until transplant. Ave grew to 6kg and started to develop her character. She absolutely loved the play team and the nursing staff. She developed a sassy attitude where she expected attention first thing in the morning from the play staff when they walked through the door, and made it very clear she wasn’t happy if she didn’t get her way. The play team and nurses became our family. They cared for Ava, and for Lewis & I whilst we were having such a hard time on the ward and dealing with the overwhelming circumstances. 

​ALAGILLE SYNDROME, AND MILESTONES MADE SPECIAL BY SPECIAL PEOPLE

     Ava’s jaundice, her skeletal abnormalities, facial features, and blood levels pointed in the direction of Alagille Syndrome which was eventually confirmed by genetic testing. This is the condition we were hoping to avoid due to its rarity and the difficulty to treat, but with help from the ward staff we took it on. 

     Ava’s hemoglobin levels dropped and she required multiple blood transfusions. She fought off e-coli infections and was put on antibiotics, had an endoscopy done, constant blood tests and pokes for bloods. Her happy character took on absolutely anything in stride and she didn’t let any of it bother her. 

     I experienced my first Mother's Day on the ward. Lewis made it so special for me, and the play team gave me cards they made with Ava. Both Lewis and I had our 21st birthdays also on the ward, again made special by the staff who made great gifts with Ava. Under the circumstances, these life milestones, celebrated in the hardest of ways, were made so lovely for us both.

 LIVER TRANSPLANT ASSESSMENTS AND THE BIG DAY

     Ava went through her transplant assessment which included an MRI, CT scans, ultrasounds and bloods. The surgeons educated us on the risks of surgery and we spoke to the doctors who would put Ava to sleep during transplant. Both Lewis and I put our names forward to donate our liver and we underwent all the testing to do so. My liver was the best match for Ava. 

     After 5 long months waiting on the ward, Ava and I went into surgery on May 26th. I had 25% of my liver taken in the morning which went straight to Ava where she spent 12 hours in surgery to receive her transplant. The day was absolutely filled with emotion. I didn’t get to see Ava until I was discharged 4 days later. Lewis would tell me how she was doing and all was seemingly going well until the day after I was discharged. 

​AVA'S HEALTH TAKES A TURN

     While Ava’s new liver took really well, there were other complications that came to light. Going back to see Ava was hard as she was extremely swollen and almost triple the size she was before her transplant. Around 11am 5 days after surgery, Ava went into cardiac arrest for the first time. CPR was performed for 4 minutes. She began to breath again on her own, then the rest of the day went smoothly after that and there were no more serious signs that gave us any concern regarding Ava’s continued recovery. However, Ava went into cardiac arrest again 12 hours later. Yet again, she recovered,  but shortly afterward went into her third cardiac arrest. At 2:00am, June 1, 2022, after her 3rd and final cardiac arrest, the doctor and nurses told us our precious girl was gone and that there was nothing else they could do. 

     After 8 months of nothing but fighting for her life, our daughter was too tired to fight anymore. She had gone into acute kidney failure which resulted in the cardiac arrests due to sepsis and she hemorrhaged. She was a true warrior and will be forever 8 months old.

A LOVE THAT LASTS FOREVER, BEING GRATEFUL, AND RAISING AWARENESS FOR A CAUSE CLOSE TO OUR HEARTS

     Ava came to me at a point I needed her the most. I will love her for the rest of my life. She made a bad situation positive and her presence lit up any room instantly. She made aunties out of her play team nurses and anyone who came close to her would pick up her sassy energy and give her attention immediately and so easily. Lewis and I will forever wish for our daughter back, however it’s not easy to dream for a wish that will never come true. September 2022, she should have been celebrating her first birthday. 

     We are dedicating this time in our lives while grieving and finding our new path forward to raising awareness about Alagille Syndrome, celebrating those who made terrible circumstances manageable, and who showed us unconditional kindness in the scariest of times. We are forever grateful to all of those who made the little time we had with Ava the most magical and special time of our lives.

"Daddy and I will love you, Ava, with every inch of us for every moment of our life and we truly believe we are the luckiest parents in the world to have had you."