Josephine's Story
- Written by Josephine Horton's Aunty
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One of Josephine's hospital admissions in her first 12 months
Josephine is like any other 8 year old; she has a cheeky sense of humour, enjoys playing with her 3 sisters, and loves her dance classes three times per week. What’s different about Josephine is that she has been fighting a disease that is not only generally not recognized in children but is also generally not present in the hotter climates of Australia (let alone Western Australia).
Bronchiectasis is a chronic condition where the walls of the bronchi are thickened and damaged, usually from inflammation and infection. It is a serious irreversible condition and, without treatment, can lead to respiratory. failure or heart failure.
Josephine’s first hospital visit was in September 2014 when she was 3 months old. Her mother rushed her to Kalgoorlie hospital from Coolgardie as Josephine was having trouble breathing and her lips were turning blue. She was admitted for 4 days and put on oxygen before being discharged with suspected pneumonia. It was after this event that her family relocated down to Perth.
Over the next two years Josephine had a persistent cough, and more episodes of blue lips and difficulty breathing. Every visit to her GP resulted in an urgent referral to PMH (and later PCH) ED, with once again suspected pneumonia; there were never any answers as to why, and no clear diagnosis. Part of this could have been due to Josephine lacking the familiar wet cough and crackly chest that is usually associated with lung diseases, and even though her lips would turn blue, and she would clearly struggle to breathe, there were no suggestions as to why. Her treatment was simply oxygen until she was stable and then sent home and told to return to her GP if it happened again.
In July 2016, at the age of 2, Josephine was referred to a private respiratory doctor by her GP, and a bronchoscopy was booked; her chest X-rays were noted as abnormal, and she was put on IV antibiotics to try to get rid of the cough.
Over the next 12 months, things really started to decline; Josephine had adverse reactions to several of the antibiotics trialled on her, and in March 2017 she underwent an antibiotic challenge. Josephine had adverse reactions to several of the common antibiotics used for treating lung conditions, which proved to be challenging for all involved; it was at this time that bronchiectasis was first mentioned, and although no clear diagnosis was made, her new team was openly concerned about her ongoing symptoms.
Just another quarterly hospital stay
Enjoying her chickens at home in 2022
In the 18 months following Josephine had a visible decline in her lung functionality, and the veins used for her PICC line, and IV antibiotics were no longer suitable for use. The decision was made and Josephine had a port inserted in November 2018. This port is used to deliver her medications continuously for several days, straight into the bloodstream via a large vein through her heart; when it is not it use, it needs to be flushed monthly to minimize the risk of infection.
In early 2019 it was confirmed that Josephine had early mild bronchiectasis. There was relief that a diagnosis had been made, followed by uncertainly as this disease is not commonly present in children. Josephine was now under respiratory, cardiology, immunology, and ENT specialist teams, and was put on 3-monthly hospital visits with IV antibiotics, regular oral antibiotics, and a daily physiotherapy regime. Josephine was originally sedated for the port needling, but over time has adjusted to the procedure and just takes a big deep breath. In 2022 Josephine was also diagnosed with chronic obstructive pulmonary disease (COPD, or lung disease).
Hanging in the waiting room at PCH for a review visit
A visit from Josephine's new little sister in April 2019