9 years ago

9 years ago, at almost exactly this time of night, I sat in a hospital cubicle with my 3 yr old daughter Anna, phoning those who needed to know to tell them that Anna had leukaemia. The story from then until now is in the archives of this blog.

Anna, 12 yrs old, is now in her first year at secondary school and living life to the full. She is a happy, kind and thoughtful girl who loves being with her friends and doing all the same kind of stuff as other 12 yr olds. Leukaemia is becoming an increasingly distant memory. SMA now rules our lives.

Mostly life is good, but just sometimes the challenge of raising a child with SMA becomes almost unbearably difficult. Not because of the help Anna needs with every part of her day to day life, not because of the interrupted and sleepless nights, not because of the endless meetings, appointments and assessments nor even because of the limitations on my own life. But because some of those very organisations who are supposed to help Anna instead use their statutory powers to make our lives even more difficult than they need to be. Their lack of transparency, accountability and at times rational thought makes it almost impossible to challenge their actions and they cause us more stress and distress than anything else. They try to disable us more than we can ever be disabled by SMA.

Luckily we have the love and support of our family, friends and many others who help us in every way possible. As always, we are truly grateful.

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Day 7 – home

Anna’s surgeon took everyone by surprise and allowed her to come home today. Day by day she has had all her tubes and lines removed (final cannula removed from her hand before leaving hospital this afternoon). She had met all the physiotherapist and OT criteria (I passed my own physio assessment too!), her wound is healing nicely and everything seems to be working properly. He said there was no reason to stay.

Nobody was quite ready for that so there was some fast rearranging of her cough assist update (had been planned for Friday morning), as well as checking we had all equipment needed at home and various other hospital admin stuff. But we definitely wanted to come home if we were allowed.

Anna is still very tired and not able to spend long periods in her wheelchair yet. We have some way to go in terms of recovery. But this evening we are home, in the sunshine, and as always I’m just glad to be here with both my children. Together. x

Smiles

Lots of smiles and chat today. Anna is much better. She’s been in her wheelchair, done some physio, had x-rays, eaten lunch/tea and had the line in her neck removed. She’s still in intensive care but they’re going to try her tonight without her overnight breathing support. If she manages okay then it’s likely she’ll go onto the ward tomorrow. She had such a busy day that she was absolutely shattered by teatime and her smile faded a bit. But definitely a much better day and getting back to her usual self.

Thanks for all the lovely comments and support. I read them out to Anna and I think it makes her happy to know so many people are wishing her well. X

Making good progress

A much quieter and better day. Anna is gradually reducing the number of tubes and intensive care interventions and letting her body get back to normal. Feeds through her nasal tube have now been reduced to overnight only, top up pain and anti-sickness medications are needed less often and her body is adapting to food going in and out more like normal.

She’s been sitting up in bed and in her wheelchair, had a bed bath with her hair washed and spent time watching her phone and reading messages. It’s all going in the right direction.

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Jam sandwich

Anna’s had a tough day. She is still making really good progress, more than they would expect on day 3 post-surgery, but today she has found it hard. I think it’s because she did so well yesterday and spent around 45 minutes in her wheelchair, using her phone and doing some activities. It meant that today she expected to be able to do much more and that just wasn’t possible.

This morning it was agreed that she could keep her mask off during the day but still keep it on overnight to help with her breathing. Although she had her arterial line removed yesterday, she still has an NG tube, intravenous line and various other attachments to make it easier to get things into and out of her body. All of these tubes and lines pull on her skin, which she hates, and they get in the way when she is being moved in the bed or when trying to get her out of the bed. And that’s apart from her wound which goes from the bottom to the top of her back and is covered in various dressings.

The Physiotherapist and nurses tried unsuccessfully to get her from her bed into her wheelchair this morning. It’s difficult not least because of all the various attachments but because she was very uncomfortable (despite good pain relief). I think she is frustrated at not being able to sit up properly, not being able to move her head easily, not being able to eat normally and not being able to do all the things that she wants to do. Her muscles are all a bit stiff and her body isn’t used to this new position yet. It’s going to take time.

I don’t want her to lower her expectations but she may need to be a little bit more realistic in terms of the pace. She will be able to do all of these things again but just not yet.

This afternoon she asked for a jam sandwich. She wanted to eat it normally. But she didn’t have enough strength in her arms to lift it from the plate to her mouth. Once she had finally managed to get the sandwich to her mouth she couldn’t swallow it properly because the NG tube was troubling her throat. She was upset and frustrated because all she wanted to do was eat a jam sandwich. She’s not happy and she’s not smiling.

She remains in intensive care tonight and for another few days. But she is now in her own cubicle so it’s a bit more peaceful. Despite today’s difficulties she really is recovering well and everyone is pleased with her progress. And she did manage to get into her wheelchair after a second attempt this afternoon.

I’m hoping for a better day tomorrow. And maybe a smile. X

Sitting up straight

Less than 48 hours after surgery, Anna is sitting up straight in her wheelchair. It’s absolutely incredible to see the difference. Not just is she sitting in her wheelchair but she is messaging friends on her phone and doing some arts and crafts!

She’s still in intensive care and being monitored very closely, especially her breathing, although she has been allowed to take her mask off while she is in her wheelchair and it will go back on when she gets back into bed. She is still having food and fluids through a tube but has begun to take some drinks orally. Her arterial line (used to monitor her blood pressure directly) has been removed but she still has her intravenous line to administer medication and fluids as well as draw blood when needed.

I can tell she’s not particularly happy as she’s hardly smiled at all today and yesterday. She’s uncomfortable and at times sore despite a really good pain relief regime. She has a large dressing covering her wound which goes from top to bottom on her back. But she doesn’t complain (other than about the breathing mask which she hates).

The final photo is from near the beginning of lockdown so you can see the difference.

Just a quick update…

Anna remains in ICU tonight. She’ll probably be there for the next few days until they’re happy that she’s breathing completely unassisted.

She was ventilated overnight but they removed her tube this morning. She went straight onto a breathing mask which is fitted around her head and not that comfortable. Anna was awake, conscious and alert through all of this and trying hard to speak even though neither the tube or mask really allowed her to do that. By the time I arrived this morning she was already communicating via whiteboard with the staff and making her feelings very clear. I didn’t need to tell them that Anna will express her thoughts as they had already found out!

She’s had two sessions of physio today to make sure her airways are clear. Everyone is pleased with her in terms of how well surgery went yesterday. I think she exceeded all expectations. Her blood loss was low and bone strength was much better than expected. Her anaesthetist, surgeon and neurology consultant all came to see her today and all were delighted with her progress even in this short time.

She was a bit teary and emotional as her meds wore off mid morning especially as she was struggling with the mask and beginning to feel quite uncomfortable. After a couple more doses of pain relief she was settled and slept for a couple of hours during the day.

I’m always amazed by how quickly she seems to recover even after major events like this surgery.

She wasn’t happy for me to share any photos of her today so instead I’m showing you a picture of her playing in the garden with Isla, our cat. I think Anna would be pleased with this as an alternative.X

3 inches taller

I’ve just had a call from the surgeon. Anna is out of theatre and it all went well. He says she is at least 3 inches taller. No bone cement was needed as her bones were stronger than they thought. She will now go to high dependency unit overnight as she needs help with her breathing and then they will probably wean her off ventilator tomorrow.

This photo is from this morning. It’s been a long day.

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Posterior instrumented spinal fusion +/- femoral head bone grafting +/- cement augmentation + intrathecal nusinersen

Anna is safely tucked up in bed at Edinburgh Sick Kids hospital tonight ready for her spinal fusion surgery tomorrow. She will have two metal rods inserted into her back to straighten her spine which will help relieve the pressure on one side of her lungs and also improve her posture and sitting balance. They will probably have to use a bit of additional bone and maybe even cement to strengthen any particular weak areas. It’s a major operation with significant risks but we all agree that the potential benefits to Anna are great enough to outweigh these risks.

Anna was in sick kids all day on Tuesday for pre-admission. There were various tests (Covid 19, bloods) and procedures (ultrasound to check veins for a line post surgery) and we met the team who would be looking after her throughout all of this – anaesthetists, physiotherapists, occupational therapists, surgeons, ward staff… It will take a lot of people to get her through this successfully but as always we know she’s in the best place possible.

At the same time she will have her final intrathecal dose of spinraza. The fusion will close up future intrathecal access. We have to hope that oral substitutes will become available in the near future. That in itself was part of the difficult decision making process as it was such a hard battle to get access to spinraza in the first place.

Although we had assumed that she would have spinal fusion surgery in her early teens, we have had quite mixed messages over the last 12 months about whether this would be suitable for her or not. We finally came to a decision to go ahead with it just before lockdown when of course all elective surgery was postponed but then 3 weeks ago got a call saying surgery would be on Thursday 18th of June.

It’s planned that she will go to theatre around 8:30 am tomorrow morning. It’s likely she will be in until around teatime. Before they can even start the procedure there is a lot of work in positioning her, checking anaesthetic levels et cetera. After surgery she will go to ITU for the night and most likely be on a ventilator until they are happy that she’s breathing by herself. They will probably allow her to wake on Friday, all going well, and then be moved back to the ward where she will stay for possibly 1 to 2 weeks. That’s the plan but of course it could all be completely different.

I’ll keep you updated.

Spinraza day 1

This is day 1. Anna has just returned from theatre having had a general anaesthetic to receive her first dose of Spinraza (nusinersen) by lumbar puncture. She feels sick and is likely to be sore. Possibly a spinal headache.

She will repeat the procedure on days 14, 28 and 63 and then 4 monthly afterwards. Several days before each procedure we have to come in to get her bloods taken.

These are traumatic events for Anna. But it’s all worth it. Because this is the treatment that could potentially change Anna’s life. At the very least it should stop the deterioration in her strength that we see as she grows.

Scotland approved this treatment to begin April 2019 (Anna is one of the first as a result of the new agreement) as have many other countries. But shockingly it’s not yet available elsewhere in the UK

https://www.musculardystrophyuk.org/news/news/access-to-spinraza-an-update-2/

We are so grateful that Anna can have this but so disappointed for those who are not yet able to.

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