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..

5 thoughts on “Day +148 Ears, tubes, tests and therapy!

  1. Flick, we’ve been missing your blogs and talking about you lots and just hoping all was going well for you. Orla’s birthday is approaching and we’re thinking about her. So hard to believe what you’ve all been through this past year. I hope you have a nice time with George visiting – maybe we can catch up on Skype. Big hug for Orla. Xx

  2. Hi Flick, thanks for the update! I’m thrilled to hear about Orla’s hearing test results!

    You and Ed are doing an amazing job for Bean. We are thinking of you and cheering from the sides at every challenge!

  3. Hi Flick., Great to get the blog, though I always presume no news is good news – which it mostly is. Well done.. News good about Orla’s hearing tests and the fact that she is getting stronger. But all the trips to docs must be so wearing, especially when nappies have to be changed at the most inappropriate times. You and Ed continue to be astounding in efforts to do all. And of course Orla is doing her level best. big hugs c and g

  4. Hi Flick, Ed & Orla,
    Glad to be able to read of the Progress, a long day by day process, but there are rewards. Thankyou for keeping us up to date. So much in 2 small years, eh? Love to you all.Carmel keeps us in the loop. hugs & Kisses, Marie & Bill xx

  5. Glad little Orla is making progress, especially her hearing results. She will enjoy Nadine forever!! Even tho’ Carmel keeps us up to date, it’s great to read the blog. I wonder how you find the time to send it. Keep on keeping on Flick, Ed and Orla. Love from Ros and Owen xx

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