Abstract
Complex regional pain syndrome (CRPS) is a debilitating, painful condition in a limb, associated with sensory, motor, autonomic, skin and bone abnormalities (Veldman et al., 1993). However, it is not common, and therefore, not widely understood or recognised by many in healthcare.
There are two types of CRPS, type I (CRPS I), which arises after minor injuries or without any preceding events, and type II (CRPS II), which arises after direct severe trauma to the peripheral or central nervous systems (Veldman et al., 1993). Approximately 15% of sufferers will have unrelenting pain and physical impairment 2 years after onset and are considered to have a long term condition (Royal College of Physicians, 2018). Early diagnosis and treatment are best practice in order to avoid other physical problems associated with CRPS. These physical consequences are linked to long term psychological challenges of living with undiagnosed chronic pain.
I want to share my experience of CRPS, not only to give insight to other young people that maybe affected by CRPS, but also to highlight a diagnosis to be considered when a patient presents with unexplained pain following a soft tissue injury. It was the recognition of this condition, and subsequent referral to a specialist pain clinic, that was pivotal in my road to recovery.
The initial injury
I was attending a gymnastics session and caught my first toe on the mat. Although it seemed like a minor injury, the pain and swelling that developed over my forefoot over the next hours, seemed disproportionate to the level of injury and I was completely unable to put any weight through my foot.
I attended the emergency department that day. The X-rays revealed no bony injury and I was provided with a care plan for a soft tissue injury. Over the next day the level of pain intensified and I was beginning to show the early symptoms of dystonia with my foot and ankle developing a plantar flexion position. I was reviewed by the orthopaedics team and my lower leg and foot were placed in a ‘back slab’ with the aim of controlling the pain and swelling.
Emerging CRPS signs and symptoms
I was followed up by the orthopaedic team and my back slab was removed. By then I had developed more severe CRPS-related dystonia of my ankle and foot, with increased plantar flexion and inversion of my ankle.
Further examination revealed an absence of motor function to my foot and ankle, and paraesthesia from my knee down to my foot. This new presentation confounded the orthopaedic team and further investigations were undertaken including a magnetic resonance image) of my ankle, which was normal and nerve conduction studies.
My family became skilful, in manipulating the position of my ankle, so that I could place my foot in an air cast boot. This enabled me to mobilise with crutches, and although I had no spontaneous motor function to move my foot, my pain at this point was controlled.
I was later reviewed by a specialist neurological paediatric physiotherapist who was the first professional to raise the possibility of CRPS. Unfortunately, I made negligible progress, my foot remained dystonic, and I was given a Botox injection into my posterior calf, aiming to improve the dystonia.
My care was then transferred to a tertiary children’s hospital, where I was assessed by an orthopaedic surgeon and a neurologist who both confirmed that I had CRPS. The initial management plan was to have three further Botox injections with graded casting to correct the dystonia under anaesthesia.
Although I had been warned that the procedure could result in a worsening of pain control, I was not prepared for the level of pain that followed the procedure. I developed severe neuropathic pain in my lower leg which I described as a ‘raging fire burning, which was interspersed by someone pouring more coal on the fire and then stoking the fire with a red-hot poker, leading to excruciating exacerbations’. It was intolerable and only intensified after each episode of surgery. Oral morphine did nothing to control the pain.
After this 6-week course of treatment, the removal of the cast revealed only a very minor improvement in the position of my foot, but a marked intensification of my pain. My disappointment was immense. Up to this point I had tried to remain positive; I had believed that the procedures would be successful, but now my future seemed uncertain, and likely to be monopolised by uncontrolled neuropathic pain.
Coping with CRPS
I could never have anticipated the journey that would unfold after my initial injury. I had expected a full resolution of symptoms and a return to normality, as you would expect after any soft tissue injury. It was only with time that I realised this was not to be the case. At the time of my original injury, I was in my first year of GCSEs and enjoyed attending gymnastics.
Although my parents met with my teachers, it was difficult for my friends and teachers to fully understand the extent of my condition. To the outside world it just appeared that I was someone on crutches. They could not appreciate the intensity of my neuropathic pain. The only visible abnormality apart from the cast was that my foot would spontaneously become very mottled in colour. I had also developed marked alloyndia, and if someone caught my leg with their school bag the pain became overwhelming.
I was aware that my sleep pattern had deteriorated, which compounded the problems I had with pain control. Resting my leg on the bed, was intolerable, as was any contact with the bed clothes and therefore hanging my leg out of the bed was the only measure to offer temporary relief.
This poor sleep pattern and the medication I was taking, affected my thought process, made it harder to concentrate and left me feeling fatigued. This added a sense of frustration. Although I understood that the pain would be intolerable without the medication, the adverse effects affected me both physically and emotionally. There were days when the pain was too severe to attend school, and balancing my outpatient appointments, investigations and physiotherapy with school attendance was a challenge.
Moving forward
Our local tertiary children’s hospital did not have a specialist paediatric pain clinic, and therefore, I was referred to another children’s hospital. I still see this as a pivotal point in my recovery and will always remain extremely grateful for their input.
The pain clinic was based around a multidisciplinary model, including pharmacological and psychological input and support from physiotherapists, orthotic and occupational therapy departments. I was initially very sceptical about any input from the psychologists, but they improved my understanding of how my foot/ankle was overreacting to external stimuli. This, combined with gabapentin and amitriptyline to improve my sleep, enabled me to tolerate some physiotherapy.
By this time my dystonia was extending proximally with a flexion posture developing in my knee and hip. Initially the physiotherapists concentrated on mirror therapy, because I was unable to tolerate them touching my foot or ankle. I became familiar with desensitisation therapy, and learnt initially to tolerate the brush of cotton wool against the skin on my leg, and then gradually increasing my exposure to more abrasive materials.
I had been left with marked muscle wasting in my entire left leg, and it took over a year of weekly physiotherapy sessions, to complete my recovery. The improvements were very gradual, and the most memorable first sense of achievement was being able to place my foot flat to the floor.
How it has changed me as a person
It has certainly given me strength, perseverance and resilience that I did not possess before. To everyone’s surprise and delight I have returned to gymnastics. However, over the period of my recovery I lost a lot of my upper body strength and conditioning. I made the decision to transfer my interest to trampolining.
I never contemplated as a healthy teenager how chronic pain and chronic illness could affect not only the patient, but the entire family unit and it has given me a level of insight and empathy that I know I would not have otherwise gained. This experience and exposure to the NHS has led me now to consider medicine as a future career. I will never be able to express how thankful I am to the team at the pain clinic and my physiotherapists, but their support and valued input has allowed me to make a full recovery.
