Agnes

Agnes

Co-Founder
Executive Director

My Sickle Cell Disease (SCD) Story

My name is Agnes, co-founder and Executive Director for ASCA.

Our daughter was born with sickle cell disease (SS). She was born at King Edward Memorial Hospital in Perth. At the time, my husband and I both were unaware that we carried the sickle cell trait until she was diagnosed at 14 months old. We have three other children who do not have this condition. The warning signs were present when I was pregnant and I became very sick requiring admission to the intensive care unit for two weeks. I was in the hospital for almost two months without a conclusive diagnosis and was treated with steroids and different vitamins, trial and error antibiotics and medications to treat what seemed like sickle cell crisis symptoms. Even though the hospital detected that I had the sickle cell trait, they didn’t go further to test my husband to rule out the possibility of our daughter having the disorder. I should mention, however, that, King Edwards hospital has now put guidelines to manage mothers and at-risk babies by doing sickle cell pre-natal testing.

Baby Joy’s SCD Symptoms

When our little girl was born, on the surface she looked healthy. But after about 6 months she started having symptoms that turned out to be sickle cell symptoms. At six months she had dactylics (Foot and hand syndrome), followed by her first case with pneumonia at 7 months. Around the time she turned nine months old, she had (BRUE) a brief resolved unexplained event. At the time all we saw was that she became pale, unresponsive, sweaty and this lasted for about a minutes.  In medical terms, this is when an infant younger than one year stops breathing, has a change in muscle tone, turns pale or blue in color, or is unresponsive. I have since heard of other parents of children living with SCD that their kids also experienced this episode. Other symptoms included her suffering incessant coughs and colds, which led to a second pneumonia attack at twelve months.

At 12 months, two days after her immunization injection, Joy ran a high temperature that lasted for days. She was taken to hospital on three occasions, but the doctors insisted that it was a reaction to the injection. The day her temperature went over 39 degrees Celsius and she could not cry anymore, we took her to the after-hours GP whose surgery was right next door to a hospital, and it was there that the GP literally had to present her to the emergency department for review. However, by this time she had developed severe pneumonia and before we could comprehend what was happening, we were being transferred to the main Children’s Hospital in Perth. The following day we were told that our little girl aged 14 months had sickle cell disease (SS) and at the time her sickle cells were 87%. Her left lung had collapsed, and she needed to have a drain connected to the lungs to drain the fluid that had built up. That was November 2009.  Joy has since undergone several surgeries and has been on different disease management treatments. Until January 2019,  she was on a four weekly Red Cell exchange at The Royal Children’s Hospital in Melbourne. In February 2019, she had a bone marrow transplant receiving the donated bone marrow from our third child who was a 100 % match. Today she is cured from SCD more than 10 years since she was diagnosed.  

SCD Advocacy Journey

The diagnosis of baby Joy was a shock and quite a depressing moment for our family. For the first five years, we only told a few friends and family that our baby had SCD. In 2012, I decided to go and study Nursing to understand baby Joy’s condition. After two years of full-time study, gaining a Master’s degree in Nursing, the journey became easy in terms of understanding this condition and hospital procedures. In 2014, I started a Facebook page as a coping mechanism. Through researching and posting different articles about SCD, understanding this condition became easy. In October 2018, together with other parents, friends, and clinicians, this advocacy association was officially registered.