Day + 160 Turning Two with a Temperature…..

Today was Orla’s second birthday and wow what a 2 years it has been, especially the last 12 months. Although today had its challenges we do feel somewhat less anxious than we did on this day last year…
Unfortunately, Orla developed a temperature last evening and we had to take her to the emergency department at the royal children’s hospital. Ed has had a nasty cold/flu and unfortunately Orla has picked it up. We were allowed to bring her home last evening after her bloods were checked and a couple of IV antibiotics infused. We then had to go back to the hospital late this afternoon for her to have another IV antibiotic and be reviewed by the BMT doctors. She has had somewhat of a bad run over the past couple of weeks as her ear infection turned out to be fungal.This is now clearing with appropriate drops.
She has started to eat a small amount in the past couple of weeks which is great and enjoyed some puree roast chicken tonight with us. Grandpa George and Nan are here and we have had a lovely evening despite the challenges of the day.
We thank everyone for their kind birthday wishes, cards and gifts…She is very unlucky in many ways but extremely lucky to be so cared for and spoilt too!!

Day + 150 In the news!

Yesterday a story about Orla and fertility preservation appeared in all the News Ltd papers across Australia.

Below is a couple of different links featuring the 2 different photos. Orla was very cooperative when the photos were taken here on Friday. It was quite a tricky story for the journalist but she did a great job.

Day +148 Ears, tubes, tests and therapy!

It has been just over a month since I last posted and shared the fabulous news of Orla’s bone marrow aspirate result showing 100% engraftment. A friend who lives in Switzerland emailed last week with his news and commented, “there haven’t been any blogs in a while?” It is lovely that people look out for them. My initial thought was, I don’t really have much to say. Whilst we have been busy, it’s doing much the same each day/ week. On reflection, there really has been a bit happening to write about but can generally be summarised with the 4 words in the title of this post.
Orla has been attending the hospital each fortnight for blood tests and a review with the haematologist. She continues to do well from a BMT perspective, is gaining a small amount of weight and growing but continues to receivie all her nutrition via the nasogastric feeds. As of Thursday this week, through consultation with the dietician, it was decided to trial more bolus style feeds whereby she is given a bolus amount of feed every 4 hours through the day and then the feeds are turned off overnight. Currently each bolus lasts for approximately 3 hours but over time, we hope to increase the rate if she tolerates it. She still has quite nasty diarrhoea, which has been tested and shown not to be harbouring any bugs or viruses…We are told the gut takes the longest to recover after a BMT and with time it will. It is somewhat draining though and must be uncomfortable for Orla.
Tubes, Orla pulled out her nasogastric tube as she went off to sleep the Wednesday night before last. A trip to hospital was required for a new one to be inserted the next morning, which was meant to be our week off hospital visits. This was trouble-free albeit a pretty uncomfortable procedure. The reason however that Orla pulled her tube out was because she was pulling at her right ear which bled through the night and again the next night, so back to RCH on the Friday to find she had infections in both ears. The ENT registrar suctioned the “gunk” from Orla’s ears which wasn’t much fun, the noise terrifying her.
I was also suffering from a cold amongst this, really no big deal, however with Orla’s immune system still less mature than that of a newborn it was quite stressful in an effort to try to make sure she didn’t catch it, we seem to have been lucky. It was a simple head cold however it rendered me shattered, my resilience somewhat lower than normal, I think mainly due to fatigue.
Orla had yet another hearing test a few weeks ago too. This time, with her aids on, to measure her brain activity in response to low volume sounds across all frequencies. The good news is that Orla can hear all sounds with her aids on. The audiologists will continue to test her hearing to try to get the most precise prescription for her aids possible. In a child Orla’s age, only small amounts of testing can be performed at each appointment as their tolerance is low.
The haematologist is also referring Orla to a opthamologist to have her eyes checked. Yet another specialist to cover all the areas affected by Mannosidosis….!
Therapy, Orla continues to have physio every week and is making good progress physically. Our wonderful physio, Belinda recommended another incredibly patient lady who specialises in early childhood development for the early years who is also visiting Orla weekly now. In addition, Orla is also eligible for government funded early intervention as she has more than one area of developmental delay. We have chosen an organisation called Villa Maria. She is assigned a key worker from one of numerous disciplines. Orla’s is a very experienced speech therapist who has engaged her extremely well already.
We have been to see Orla’s general paedatrician which was a challenge for a few reasons. Firstly, Orla woke from a nap covered in poo to the point she needed to be bathed before the appointment so I was stressed to make it there on time. Once we arrived, Orla was very anxious and didn’t even allow the doctor to examine her without screaming so we didn’t bother. The paed and I also had a fairly confronting discussion around Orla’s future. Our paed has been a great support to us through Orla’s diagnosis, the decision of to do the BMT or not and then through the BMT itself. We discussed the fact that although the transplant has been a great success, the reality is that Orla was born with a neurodegenerative disease. It is hoped that the transplant will halt this degeneration but we don’t know and there is no way of knowing how much and what damage is irreparable. And yes, she feels Orla will be left with a disability but how great this will be is unknown and whilst we hope and pray it to be mild, there is no way of knowing. Preparing for this unknown is hard. Everyone has their uncertainties in life this is ours..