Day + 118 Can’t get any better than that….!

Orla was seen at the hospital today and we received the results of her bone marrow aspirate from last week. Orla’s bone marrow is 100% male and therefore 100% donor…! So as you can imagine, we are delighted, the hard yards feel very worth it when we receive such amazing news as this. We simply could not have hoped for anything better than 100% engraftment. The previous chimerism results looked at Orla’s blood where as this looked at her bone marrow and, as the doctor hoped, gave us a better result but more precise result too!!
Orla has also put on some weight since last week too. Her weight has now returned to that which it was when she was discharged from the BMT unit. The dietician and speech therapist reviewed Orla today regarding her feeds and where she is at with eating. We hope she will continue to gain a little more weight in the next few weeks using a mix including some high calorie feed and then it is hoped soon she will be able to have some time off the feeds and transition to bolus rather than continuous feeds and she might get hungry in between!!!
Orla doesn’t need to go back to RCH for a whole 2 weeks now….!!

Day + 111 Home and resting again….

Orla had a big morning today. We arrived at the hospital for 8am this morning for her to have her numerous procedures. The staff were all very nice and worked together so Orla had all her procedures done swiftly. The anaesthetists arranged for her to have some paracetamol prior to the procedure to limit any discomfort. They also used her Hickman line to inject anaesthetic to send her to sleep rather than the mask and gas which makes things a bit traumatic for everyone involved. The ENT doctor was very helpful, removing some wax from inside Orla’s right ear which appeared to be blocking her grommet. Her Hickman line was removed without any trouble, some bone marrow aspirated and her nasogastric tube changed.
Prior to her transplant, when Orla would wake after an anaesthetic we could give her a bottle which would be guaranteed to comfort her. Unfortunately we are not able to do this anymore as she won’t take one! She wasn’t happy today after waking and especially so when we made our way up the day oncology area for her appointment with the BMT consultant following her procedures. We ended up being put into one of the quiet rooms and the door shut as she was screaming so much and unfortunately Mum didn’t check the DVD cover we took…it was minus the DVD and the day oncology wards DVDs were not of Orla’s particular standards!!
We are yet to receive the results of the bone marrow aspirate. Her bloods today were all good, except for the Cyclosporin level which is low, not surprisingly as she has vomited a few doses in the past week. The consultant agrees that we need to give her some anti-nausea medication from now on prior to her doses to avoid her vomiting and the need to hold or slow her nasogastric feeds. Orla has unfortunately lost some more weight which again I’m not surprised by, it was concerning me. The solution will be to add a small amount of high energy feed into the mix of her other feeds. This feed can apparently help reduce nausea and vomiting which would be great for Orla. The only issue might be that the new feed is not as broken down as the feed she is currently on as her gut hasn’t been able to tolerate the less refined feeds yet.
So we were hoping we might be able to go to the hospital once a fortnight from now on as her bloods have been stable but since her weight isn’t Orla still needs to be seen weekly.

Day + 110 Daily challenges….

There were issues the doctors could never have prepared us for prior to Orla’s transplant as every child is different and every transplant is too. Orla continues to do well in terms of her blood results from the transplant perspective. The daily challenge is related to the balancing act of her feeds, medication, preventing her from vomiting and trying to get her to eat!!

Her gut is still recovering and thus we are not able to increase the rate of her nasogastric feeds to allow her any time off. She still appears to be experiencing some nausea and is also becoming quite anxious regarding her medications, in particular the immunosuppressant, Cyclosporin. This is of course the most important medication and the only which must be taken orally and can’t go down her nasogastric tube. It tastes revolting, despite us disguisng it with a chaser of an antifungal full of sugar, and the side effects of it are why she requires all the other meds…So the daily challenge is to manage the administration of her medications so she doesn’t vomit them up while making sure she gets enough feeds. At her hospital appointment last week, she had lost a small amount of weight, so we were losing the weight battle a little, due to her not receiving enough feeds. This is partly due to the need to put the feeds on hold or slow the infusion rate around the administration of her medications to try to prevent her vomiting. If her feed rate is too high while she sleeps, she wakes up nauseated. We can’t dial the rate of her feeds up to catch up, as this will also cause her to vomit or lead to her persistent diarrhoea becoming worse and more frequent. Amongst all this, Orla has zero interest in eating still and I possibly wouldn’t either!!  As you can see, from the photo below, there are a number of medications (this is her morning lot) she must have and this picture doesn’t include the Cyclosporin (nor the accompanying chaser) which is given at a different time in case she vomits.

Bean's meds

Last Tuesday night, Orla started to vomit as we put her in her cot – not long following her cyclosporin. When this happens she becomes quite upset but it passes relatively quickly.  This time it was different. She was gasping for air and a bit flat following the vomit followed by drowsy and just not herself….After deliberation, Ed initially ringing the ward at RCH, we made the decision to call an ambulance – after everything she and we have been through we are not prepared to take any chances. The paramedics were very nice, but, of course when they arrived and we told them about Orla’s condition and that she recently had had a bone marrow transplant, the only advice they could give us was to head to the Royal Children’s Hospital!! We asked them if they would mind checking the level of oxygen in her blood and heart rate, which thankfully were both ok. We waited for a bit and then Orla seemed to return to herself and as a bonus got to sleep with Mum that night! Although the paramedics suggested it would be a good idea to take her to RCH they were however happy for us to make the call. We watched and waited and then felt it would be more risky to take her into the emergency department full of bugs. When speaking with the transplant coordinator the next day, she suggested from our description, Orla choked and the energy she used to protect her airway exhausted her and made her drowsy. The experience however did make her even more sceptical of her medication for a few days. We have since devised a few strategies and she is doing ok for the moment although we are using anti nausea medication regularly which the doctors would prefer us not to!

Tomorrow, Orla will have a needle inserted into one of her hip bones to aspirate some of her bone marrow so it can be examined closely under the microscope. This will hopefully confirm the great results we have received a little while ago which measured how much of her blood cells are male (from the donor) compared to female (her own which survived or regenerated following the chemotherapy). Looking at the marrow itself will give an even clearer indication at what is going on. As the bone marrow aspirate involves a general anesthetic, we thought we would take the opportunity to do a few extra things, it would be a waste of an anaesthetic otherwise! So tomorrow Orla will have her Hickmans line removed and her nasogastric tube replaced. Removing the Hickman line is a big milestone. This has been decided with careful consideration by the BMT doctors as if she needs an IV infusion, more than likely blood transfusion or immunoglobulin, now she will need a line inserted into one of her veins to do so. The doctors and I agree that the risk of infection is greater with the Hickamans line in than her needing intravenous access for anything else. It will be a great relief for Orla to have a bath without the concern of the Hickman site getting wet and not having to wrap it in glad wrap and tape it at bath time time. We decided to replace the nasogastric tube as it seems she may need it for a few months yet and 4 or 5 months is the lifespan of a nasogastric tube normally. Usually, both the Hickman removal and replacing nasogastric tube would not be performed under anaesthetic but Orla would thanks us if she knew I’m sure. Finally, the ear nose and throat doctors are going to have a look in her ears while she is asleep to check her grommets, again an easier task while she is unaware!

Day + 100…..A Big milestone!

Today marks 100 days since Orla’s transplant!

The first 100 days are thought to be the most risky especially in regard to acute graft v’s host disease (GVHD). GVHD occurs when the new lymphocytes (white blood cells) of the donor recognise the cells of the recipient, attack and reject them. The tissues in the liver, skin and gut are the most at risk. Orla seems to have been very lucky and has avoided any significant acute GVHD. She has experienced some intermittent rashes along the way which the doctors kept a close eye on and she also needed a gastroscope at one point to confirm if the persistent diarrhoea she was experiencing was GVHD which fortunately it wasn’t.

There is such a thing as chronic GVHD which can occur after the 100 day mark but as Orla hasn’t had the acute version she is low risk.

At 100 days, there are some changes in Orla. The first and most noticeably is her breathing, she now sleeps much more soundly and her breathing is no longer uncoordinated. Her teeth have also grown considerably. Her teeth had erupted when expected however they failed to grow but seem to be now making up for lost ground. Orla also appears stronger in her muscles, and is not such a sack of potatoes to carry now, this makes her scoliosis look better. The doctors also no longer find her liver and spleen to be enlarged when examining her.

So what are we doing to mark the 100 days….? I am about to enjoy a glass of bubbles with our good friends who have just arrived with Indian takeaway for dinner. It is rather fitting as the same friends came equipped with takeaway and wine the night we received the news of Orla’s diagnosis.

Day + 98 Hearing test

Orla attended Australian Hearing today for her first hearing test following her transplant. We had some hope that her hearing may have improved as she has seemed more responsive and receptive to sound of late. She has been startling to some louder noises with only one aid in hence we have left one aid off until we knew for sure. Orla’s hearing has definitely improved in the sense that she no longer has fluid in her ears referred to as a conductive loss in addition to her nerve or sensoneural loss. However, unfortunately the test today found Orla’s sensoneural hearing loss to be much the same as prior to her transplant.

Performing a hearing test in a child of Orla’s age is difficult, and can be affected by many things. The child has a probe put into the ear and a sound is projected through it at the same time a puppet appears which an audiologist holds behind a curtain. If the child hears the sound, it is hoped that they will turn their head to the puppet to confirm it. A second audiologist sits in front of the child to watch the child’s more subtle signs of hearing the sound like raising eyebrows or stilling. There is hence a fine line between the second audiologist maintaining the child’s interest so they are not constantly looking for the puppets but without engaging the child so much that they lose interest in the puppets!

Whilst we were hopeful Orla’s hearing had improved, it is positive that it hasn’t declined. Her threshold to high frequency, commonly referred to as high pitched, sounds may have worsened slightly. Orla’s hearing impairment is classified as moderate. She is able to hear some speech sounds in human conversation but possibly not all as some are presented at different levels as well as frequency. The audiologist agreed today that Orla’s reactions to sounds are quite sensitive as well as her being quite visually alert. This can often result in one sensing, especially a hopeful parent, that a child is hearing more than they are actually are. Orla was also somewhat nervous for the test today so her physio Belinda will be pleased to hear she is not the only one that she screams at! It is however pretty understandable given everything she has been through over the past while and the room where the tests are held are sound proofed and almost like a bunker. The audiologists said that many children who have been hospitalised for long periods respond like Orla did today. In addition to the hearing test, a test called a tympanogram is also performed whenever Orla attends her Aus hearing appointments. The tympongram uses a machine to check the air pressure in the ear and can thus tell if there is a presence of fluid, wax or in Orla’s case as it appeared today, a blocked grommet in her right ear! We will now need to contact her ENT doctor….!

To finish this post; below are a few stories of interest to us which may be to you too.

The first is a boy who was in the BMT unit in the room next to Orla

And this a family with twin boys who have a very very rare condition and we can relate to much of the feelings described

Day + 96 Cool catch ups with Orla!

Cool catch ups with OrlaMy gosh, there are a few more things that require me “doing” at home (predominantly washing – I REALLY miss the linen skip) rather than in a hospital room. However the quietness of isolation at home is a welcome relief from the stressors of the past few months but also lonely at times. We have been fortunate enough to have a few special visitors including Orla’s cousins, Oscar and Leo. This was a visit with a difference as we had to sit outside and it happened to be pretty chilly on this particular day. Orla found this to be quite a shock after 3 months in a temperature controlled environment, making her reluctant to smile nicely for her Aunt Lucy! The reason for sitting outside was so Orla could see her cousins as she isn’t able to have contact with children and their germs at the moment and only well adults are allowed into our house. Pa also came for a visit and Nan is staying this week. Grandpa George has also booked flights for a visit in July.

Orla remains well and happy. We have been home 3 weeks today, although it does feel like longer . It also feels like we have been in a time warp for the last few months, the only telling reason that so much time has passed being the change in season from the midst of summer when Orla was admitted and we are now lighting the fire every night! Orla attends the hospital just once a week currently and to date she is progressing well. She still enjoys her visits to RCH especially the social aspect as she is quite familiar with the environment there. On one occasion, she was shouting so loudly that the consultant and I couldn’t hear each other talk!!

As Orla draws closer to 100 days post transplant, the acute stress of whether or not she will survive is dissipating and hence it is only now that Ed and I are starting to really come to terms with her diagnosis. The bone marrow transplant will no doubt help Orla but there is currently still so much uncertainty in our lives. At 22 months this week, Orla isn’t eating (most likely due to the effects of the BMT), talking, walking or even crawling and whilst we have much hope she will achieve these milestones the unknown is hard to deal with. And yes, we do need to and try our best to take every day at a time and be patient however it is challenging.

We were saddened to hear yesterday that the child across the corridor from Orla in the BMT unit passed away on the Friday following Orla’s discharge. He unfortunately was just short of reaching his first birthday. Life is so cruel at times. His parents returned to the unit on Mothers day, his mother’s first, to collect their belongings where they had been living since their gorgeous son was 4 months old. He had a an immunological condition but he lost the battle due to infection despite his father donating his bone marrow twice.

Finally, I applied for a Musical Moments scholarship for Orla offered by the charity Challenge and we were informed on Monday that Orla was successful. The scholarship consists of $500 to spend on musical experiences and we plan to use it for musical therapy at home over the next while which I know she will thoroughly enjoy. We discussed the option today of combining music therapy with her physio as Orla has now clued onto who Belinda is and what it means when she arrives..!! There was some serious coaxing required here today and reluctantly we had to resort to Justine Clark on DVD to get Orla to cooperate or rather stop screaming at poor Belinda, a great sign that her memory is functioning!!

Day + 83 Going well…..

The past week since we arrived home has been pretty hectic. Ed had last Thursday and Friday off work plus the weekend which was lovely. He returned to work on Monday and Orla and I have been readjusting to life outside the 4 walls of her room at RCH. Funnily enough, Orla seems to be in some ways more at home on the occasions we have been to RCH since her discharge than she is here! Although she was in a room only, she had a constant flurry of visitors, in the staff, which she obviously enjoyed! She enjoys going out in the car and has developed a liking for speed humps which has found Mum driving routes where they exist rather than avoiding them!

We have been back to RCH twice since Orla’s discharge and have another appointment tomorrow. Orla has blood taken at these appointments, dressings to her nasogastric tube and Hickman line changed, weight and blood pressure check and a review by one of the BMT consultants and so far all remains good. The children who have had a BMT wait in their own room when they attend the outpatients area and are first to be seen for the day to avoid any exposure to bugs. As the consultant told us last week, an upper respiratory infection in anyone could potentially see Orla end up in intensive care. Her immune system is still developing and not yet complete with all the necessary components to fight and withstand regular viruses and infections.

Orla’s physio, Belinda came to see her yesterday and was impressed with her strength especially given what she has been through over the past few months. Her muscles in all her body seem to be stronger including her voice and thus her ability to scream at Belinda when she has her in a position which she would prefer not to be in! The plan is for Orla to continue her physio sessions every week from now on if she remains well enough.

Below is a photo of Orla enjoying a few at home delights…..

Bean wine



Day + 75 We made it!!!

Ethan's welcomeHome safely today!!

Since 31st January, Orla has spent 3 nights at home..So after 12 and half and almost 7 weeks after our initial attempt at discharge, an episode of viral meningitis and a bacterial sepsis but most importantly a successful BMT , thus far, we are together at home tonight and enjoying a glass of bubbles!!

We are very pleased and extremely relieved to be here although it was an emotional departure from the BMT unit. Orla, I think, will take a few days to readjust and we to unpack. It is amazing how much stuff  you can accumulate in one room! The haematologist who organised Orla’s pre BMT workup returned to work just yesterday from long service leave and came to visit us this morning. (She did come in yesterday but Orla and I were her asleep!!) Her last day at work was the day after Orla was admitted…She was very impressed at how well Orla looked.

A photo including a teary Mum to mark the important day that we wondered if it would ever happen

Home 1

Day + 72 Ticking on….

Yes time is ticking on, Orla will have been an inpatient of the BMT unit for 12 weeks tomorrow… There isn’t really much to report but we are grateful for that! Orla continues to be receiving intravenous antibiotics and antiviral medication twice a day and I’m another year older since I last posted. Orla appears to be happier and stronger each day. Fortunately the antibiotics haven’ t messed with her gut and bowels too much although she has been suffering from some nasty thrush. The antiviral medication will finish tomorrow and the antibiotic course should be complete on Wednesday. We expect to be able to come home pretty soon after this but the doctors haven’t mentioned the H word…for fear of jinxing us I’m sure. We are hoping for third time lucky and expect the team will review Orla one morning soon and announce we can leave that day!

Here is a photo of the happy little girl…

Happy Bean!