2017 - A year of the dust settling

For all my plans of sending Sarah-Hope to school in 2017 with a facilitator, they didn’t materialise. The wonderful lady who had been helping us at home in 2016 became ill and wasn’t able to return to work in January. Fortunately providing a facilitator wasn’t a requirement of the school, and so Sarah-Hope continued to attend. There was now a separate class for the older children. Sarah-Hope was in this class and enjoyed the increased instruction as is age appropriate, as well as some protection from the younger and ‘wilder’ preschoolers.

Sarah-Hope amazed everyone with how well she coped. Now in a healthier space, it was as if she was a different child. As her health increased, so did her independence. She was interacting with others, even taking initiative and leading play at times demonstrating a huge increase in confidence. I also deliberately started the year by sending Sarah-Hope and Libby to school on different days to stretch them more socially. Their default had become playing together in the presence of others, and the previous year of poor health meant that we had had no capacity to foster our children’s social lives.

What had also become increasingly apparent was how Libby was becoming physically affected – with Sarah-Hope’s mobility impairments settling as a more permanent reality and Rich being so physically weak, we didn’t get out much because just having everyone home and out of hospital seemed to be a treat! The missed opportunities for age appropriate development for Libby were weighing on me. And so when Sarah-Hope went to school, I took Libby out to do those things that were difficult to do with Sarah-Hope around... like climbing! Actually, Sarah-Hope wants to do what everyone else does but then I need to climb with her which makes it difficult for me to help or stretch Libby physically. So while Sarah-Hope is physically well enough to be at school or cared for by others, I try to make the most of my individual time with Libby. Because when Sarah-Hope gets sick, I find it difficult to leave her. She didn’t have any gut related issues in 2017, which was wonderful, but she did have some chest trouble. This significantly slowed her down and resulted in her wanting to be carried more and the inevitable moaning from losing the energy required for and agency provided by independence. We invested in a nebuliser to help manage her chest, and apart from two nights spent in hospital later in the year for extra oxygen, she recovered well at home during these periods.

And then she stopped walking

And then in April 2017 Sarah-Hope stopped walking on her feet. Just like that. That precipitated a whole lot of investigations – was it a matter of strength, neurology, pain or stability? X-rays, an MRI, and consultations with specialists ensued. With her underlying condition of TAR Syndrome, the absence or displacement of the patella (knee cap) is common. This is something that was on the radar but knee caps only ossify between three and four years of age so we knew we had to wait and see. Sarah-Hope was now five and the radiologist couldn’t identify knee caps on the X-ray, and the surgeons and physios and orthotists weren’t entirely convinced about what they could feel around her knee either. Either way, she had radically unstable knees and was choosing not to walk on her feet as it made her too vulnerable to being knocked over. On her knees she was participating, stable, able to move and talk. Even in the presence of the stairlift and wheelchair we had invested in, she would often choose to walk upstairs on her knees. She was strong and mobile, as far as walking on her knees could take her and as long as her health could sustain it. With a strong mind, she would usually push her body until it just couldn’t go anymore. Hearing her femurs knocking into the floor, a similar sound to high heels on a wooden surface, made me wish she wasn’t moving! I was now caught between trying to encourage independence and begging her to sit still on the couch so that we could preserve her knee joints as much as possible and get her body to rest. And unfortunately the only way I could get her to agree to sit still was to put her in front of a screen.

The conclusion the specialists came to is that she was choosing to walk on her knees. This made sense from a stability point of view since the wheelchair and stairlift provided additional mobility support. But this has other consequences. The muscles used for walking are wasting away. She isn’t bearing weight through her legs. This means her feet bones and muscles aren’t developing except for the amazing dextrous way her feet are also used like hands. Her knees are raw from the contact with the ground. She is losing range in her legs which are now always bent. And so in this moment, I realised that while we have always been open to letting Sarah-Hope lead us in terms of how her body could be used, we also had to weigh her immediate solutions with long term realities. If we don’t get her back onto her feet somehow, would she lose the possibility of walking again in the future? Two of the paediatric orthopaedic surgeons we consulted said they didn’t think she should be touched as she has incredible function and any surgery would stiffen the legs and take away all she had gained through natural adaption. Another offered a different perspective saying that in terms of the academic literature, the remodelling potential of the legs would diminish after the age of eight. And that when you start these surgical journeys, they are not usually once off. You try to fix the knee, and then realise the foot needs mending, and so it goes. In the end we decided to first see her through the transition into a bigger school environment. With more and bigger children moving around her and doing exciting things, I’m confident that she will want to participate. I’m hoping that will give her the motivation for the journey. It will definitely include hydro therapy, which has already begun. It could include surgery. Or standing frames. Or adaptive devices. Or all of the above.

Participating on her knees
One of the short term interventions we have agreed is necessary is to try and protect her knees. An orthotist recommended an imported knee protector, the type that might get used by a sportsperson - its flexible enough to allow for a lot of movement but also provides a layer of protection. This is a work in progress - as they come they are comfortable to put on but then slip down. As you adjust them, they can easily become too tight or stiff to allow comfortable movement. With this inconvenience, a busy little girl wants to just get on moving and long term knee damage not a concern to her in the moment! But a brilliant idea came - one night Sarah-Hope was talking to me about how she wanted to dance like all the other children. Libby had been doing some ballet at school. I checked with the teacher and then we agreed that Sarah-Hope could join the class as long as she wore her knee pads! She agreed. And loved it. 

Lifestyle adaption as a family

What has become certain is that we have to adapt our lifestyle to mobility constraints. It seems as though I could say with a fair bit of certainty that there will be periods of independence and adaption, there will be periods where much more hands on support is needed, and there may be periods of significant set backs. The more set up we are for these different modes of being, the easier it is to transition between them. With the healing of Sarah-Hope’s gut and her gaining weight again, the fragility of her frame became more apparent. It was hard for her to carry her weight when it exceeded 14kg. And it also became hard for us to carry her. This was the first year that I went to a physio for strain in my arms and neck. Despite my being relaxed about Rich and Sarah-Hope needing physical support, I was over anxious about myself! I knew we couldn’t afford for me to get into a situation where I couldn’t manage the kids – picking up children was how I kept myself sane in amongst their frustrated moans. So I needed to get things in place which meant less carrying was required, ensure I picked them up properly when it was needed, and get stronger. This was more pronounced in periods of ill health where the comfort of being carried was what Sarah-Hope wanted. It didn’t help that the more I carried her, the more Libby wanted to be carried. And so I often carried them both. With the stairlift and wheelchair and the bus to transport it now all in our lives, we were getting other options in place. There were times when Sarah-Hope wanted us to help her walk upstairs or carry her into certain places – and we’re trying to explain that she needs to ask us and also respect if we don’t feel we can, therefore being open to using other devices. I don’t have any hard and fast rules, like ‘you will always go upstairs in the stairlift’. We navigate different energy levels all the time and sometimes we ask her to sit on our lap as we go up in the stairlift! What we try to keep as consistent is the request for help and respect of the options we give her at the time depending on where we are at.

School choices

While absorbing this new mobility constrained reality, I was aware that Sarah-Hope was approaching school going age. I spoke to a number of principals to explore the possibility of Sarah-Hope attending their schools but in the end only applied to one of them. And thankfully, Sarah-Hope was accepted. This school is in Pinelands and is the forerunner in inclusive education in the city. The fact that Pinelands is flat is definitely a help – the schools in town are generally on an incline which makes movement that extra bit challenging. But the main thing was the sense of excitement and possibility as well as uncertainly that they were able to embrace. I don’t hold anything against those not being sure if they can cope with Sarah-Hope’s needs – I have felt that much myself. Afterall, no-one knows how she will cope and what to expect from her. But the willingness to make multiple adjustments along the way as the need arises was such a relief to me as such flexibility had become part of how we have learnt to operate as a family but I know most people and institutions aren’t able to offer that straight up. And so it seems as though this is a good place to start the schooling journey because I am confident that all the parties will do whatever they can to see if Sarah-Hope can be schooled there. This journey will involve the practicalities of how she will move from place to place as well as how she will express her knowledge and be assessed. Technology should be able to assist in both of these, although that journey would be as much for us at home as for her at school. So again, I’m grateful for a responsive and adaptive environment in which we can figure these things out during these formative years.