Ruth Bailey, a passionate advocate for effective healthcare for adults with CP, recounts a sequence of episodes which encapsulate the problems with the current provision.
Cultivating curiosity about CP in adulthood
Adults with cerebral palsy (CP) are caught in a bind. On the one hand, specialist NHS services tend to focus upon children with CP, forgetting that children turn into adults. On the other, the experience of adults, backed up by the findings of medical research, suggest they need services more than ever as they age prematurely and are more susceptible to conditions of older age such as cardiovascular disease. As a just turned sixty wheelchair using woman with CP, I discovered this bind first-hand when I broke my leg earlier in the year in a hotel room in Cheltenham. From the moment the ambulance was called to my final discharge from the fracture clinic, I was treated in the same way as anyone else. And that was the problem. There was a lack of curiosity as to how my cerebral palsy might complicate my patient journey. This led to some problematic situations where my care could have fallen far short of best practice had I lacked confidence or cash, which all too many adults with CP do.
Call handler’s script says no
The first problematic situation arose when I failed to qualify for an emergency ambulance to take me to hospital. On the basis of this exchange, it is not hard to see why:-
Call handler: “When and where did the injury happen…”
Me: “Four days ago … in a hotel room …in Cheltenham…I’m now in London …but…”
The call handler prohibited articulation of my ‘but’, as they appear to follow a script designed to apply ambulance eligibility criteria efficiently. This allows no space for any curiosity of CP – or indeed mention of one’s condition. Had it done, I would have explained my ’emergency’ was of the slow burn variety. Travelling from Cheltenham to London was made possible by three people lifting me into my chair, as I screamed. After that, my wheelchair got me on and off trains and buses, and with copious amounts of painkillers and muscle relaxants I rolled from chair to bed once home. Four days later, the pain had not subsided, and I was no longer able to move from my bed, even to use the bathroom, let alone get to hospital.
It was the GP that instructed that I ring 999 so I rung back to confess my failure. They say, ‘you do qualify for an ambulance…you are housebound…ring again’. I fail again. I ring the surgery again. They arranged a non-emergency ambulance, a good option. In the end we all get to the right place (except my power wheelchair, which ambulances of all kind spurn, they refuse to take them, but that’s another story). But my experience suggests that CP should feature in conversations about eligibility for ambulances.
Stories and underlying causes
The second problematic situation arose due to missed opportunities to diagnose osteoporosis as the underlying cause of my fracture. The first opportunity missed was a failure to be curious about my ‘what happened story’. Once in hospital, doctors asked “How did you fall and break your leg?” In response, I would state there was no fall. Then I would explain the injury occurred while I was trying to get up and out of bed, something that requires of me the energy and ingenuity of any Olympic sport. Having been assisted to a sitting position, I must concentrate to maintain my balance while using my upper body strength to shuffle my bum round the 90 degrees from bed to wheelchair. My legs usually just follow my bum. But in the Premier Inn Hotel, Cheltenham, they didn’t.
This was due to the mountainous height difference between bed and chair. To climb that 10cm required herculean effort and necessitated my legs being outstretched and resting on my chair’s footrest rather whereas usually they would be bent with my feet on the floor. As I shuffled my bum millimetres to the left, my right leg went nowhere: it simply rotated outward. As it did so, a crisp but muted snap emanated from my knee, the sound of my right femoral median condyle fracturing.
Despite – or perhaps because – of my detailed story, my medical notes state that I injured my leg as a result of a fall. To my mind, that symbolised a lack of curiosity about my day to day reality of living with CP. More to the point, it was missed opportunity number one to consider osteoporosis as the underlying cause of my fracture. That’s because a fracture like mine, sustained without the impact or force of a fall, is considered a fragility fracture and these are often a sign of osteoporosis. Missed opportunity number two was the lack of knowledge about CP itself being a risk factor for osteoporosis. This risk factor has been identified by epidemiological studies, and is thought to be due to abnormal bone structures and children with CP non-weight bearing in childhood. My age, sex and sitting status (ie non weight bearing) are also risk factors for the condition, along with my family history, as in her nineties my mother managed two falls, each time breaking a different hip.
According to NICE Guidelines on CP, having two or more risk factors for osteoporosis (I had at least four) coupled with a fragility fracture, should prompt a referral for a bone density scan. But the lack of curiosity about CP meant it was down to me request the referral. I did so because a friend with CP alerted me to the CP osteoporosis connection. Following the scan, I started medication for osteoporosis 5 months after my fracture. However, it is recommended medication should start promptly following a fragility fracture, because risk of re-fracture is highest immediately after a fracture and risk remains elevated (Clinical Guidelines for the Prevention and Treatment of Osteoporosis 2021 nat. osteo guideline group).
Who is the expert?
The third problematic situation arose due to lack of CP expertise. Usually, I have expertise in how CP affects me day to day. Place a thigh to ankle cast on my leg and that expertise diminishes. My mistake was to think there would be a corresponding increase in expertise in the physiotherapist on the orthopaedic ward, the professional you most need to trust in the early days of rehabilitation. For my first outing from my hospital bed the physio asked. “Do you want to use the hoist or the transfer board?” That the latter was offered I read as confidence that I would get back to my normal ways of doing things. Accordingly, the physio came to direct the process of getting up and placed me in a sitting position over the edge of the bed,…at which point by brain was overwhelmed, upright and unsupported for the first time in two weeks, one leg in plaster, and without my feet on something solid. My body went into a complete spasm. According to the doctor who spoke to my niece, this rendered me ‘unresponsive’. “Do you mean Ruth is having a dystonic storm?’ she replied “Ah, Yeeees”. It took me several days to recover.
Had the physio an attitude of curiosity about CP, she may have asked for greater detail about how I transferred at home. Another option would have been for the physio to call upon the expertise of a neurophysio, when getting me out of bed for the first time. Both the physio and I were hopelessly optimistic thinking I could use my tried and tested technique so soon after injury. It would be three months before my body was recovered enough to enabled me to sit over the edge of the bed safely, and longer still until I would be able to use my transfer board and banish the hoist from my bedroom.
This lack of curiosity about CP was also apparent when the same physio, along with an Occupational Therapist colleague, trained my PAs how to hoist me. The session glossed over how to manoeuvre my legs when suspended in the sling, given one was plastered and the other spasmed. By the end of it, none of us felt confident about using the hoist safely, to the extent I seriously contemplated delaying my discharge planned for later that day That evening, I feared I had made the wrong decision as we were at a loss to know where to position the wheelchair in relation to the bed in order to use the hoist. Help arrived the next day, in the shape of a chance visit of an ex pa whose father is a hoist user. She experimented with different approaches for hoisting me, and then made an explanatory video. Personal experience trumped professional training on this occasion.
Who gets help to mobilise?
The fourth and final problematic situation arose in the final stages of my recovery process, during which I was offered no help or support. In the recovery journey a significant milestone is reached when the plaster cast is removed. My leg reacted to its newfound freedom by spasming. The consultant did not grasp why this compromised the quality of the X-Ray of my knee, yet, as I told him, the radiographers had been exceptional. With care and kindness, we discussed how to position my leg and when moving it did so slowly, stopping before I reached my pain/spasm threshold. I asked the consultant how I should mobilise my leg to which he replied, ‘just bend and straighten it’. He struggled to appreciate the fact that I lack muscle strength to move my legs intentionally or to resist them moving against a force. The latter, I realised on the way home, would make it impossible to use the hoist, which I was still reliant upon for all transfers. The problem was once trussed up and dangling in the hoist sling, gravity would bend my knee to less than 45 degrees, yet inside the plaster my muscles had held it at 50*.
The consultant’s lack of curiosity about my CP meant once again, I found myself stranded in my bed, unable to get up even to use the bathroom. Following a fracture and plaster removal most patients receive advice on mobility equipment and / or a therapy appointment. I was offered nothing, as if mobility matters only to those who walk. Fortuitously, a neurophysio specialising in CP was visiting the next day, a private appointment I had arranged being keen to begin my rehabilitation immediately. She was able to identify a brace which would hold my knee in position and Amazon delivered it two days later. Now my recovery could begin…but only because I could track down a specialist and had the money to pay. The same was true of my original hospital discharge. Anxious to get home as soon as possible rather than await a care assessment, I self-funded four extra hours of care a day as a second carer was required each time I was hoisted, which I arranged through an agency I regularly use.
Conclusion
Breaking a leg was a reminder of both my advancing years and the kindness and expertise of those who work in the NHS. But I also experienced firsthand that there is scope for the NHS to do even better when it comes to meeting the clinical needs of people with CP. Greater specialist knowledge of the effects of ageing with CP would be the gold standard. But curiosity about CP would count for something. It is that ability to stand back and say ‘there’s something different going on here, better start a conversation with the patient about this’. From such conversations come solutions and strategies emerge, whether they be non-emergency ambulances, collaborative working between orthopaedic and neuro physios or timely interventions in the final stages of the recovery journey.