"When providing our services, we respect each client's right to choose and experience a broad range of opportunities and to take calculated risks in an environment that provides relevant information, support and care." (GDAV Client Policy 1.2a)

Adriana is a 56-year-old woman with optic neuritis and Guillain-Barré Syndrome. She is an example of a client taking calculated risks and living willingly with the consequences. I worked with Adriana for a month during 1999, and have had only occasional contact with her since then, but I have come to admire her tremendously for the choices she continues to make about her health care and mobility. Her attitude is unusual: in the face of extraordinary loss of vision and movement, she has remained cheerful and optimistic. She expects healing to occur, and it is.

Since Adriana's diagnosis was made retrospectively, I will describe her experience first, before giving a more clinical description of Guillain-Barré Syndrome.

Adriana's CV
In 1999, when Adriana began to experience visual and health changes, she was well equipped to make informed decisions about her own health care. During her General Nursing Certificate training she was awarded the ophthalmic prize. She has done further studies in midwifery, herbal medicine and palliative care, a Bachelor of Education, a Masters in Nursing Studies and begun work on a PhD in Nursing. During the course of her illness, she kept meticulous notes about her visual and health changes, appointments, actions suggested and decisions taken.

Visual Changes
On January 26, 1999, Adriana noticed a sudden loss of vision in her right eye. Two days later, on the prompting of a neighbour, she visited her GP. She was referred on to an optometrist, then an ophthalmologist within the day. Her visual acuities were R: 6/18 and L:6/5. Automated Perimetry testing showed a residual quadrant of vision in her upper nasal field in the right eye. She had slight loss of peripheral vision in the nasal field of her left eye. Her optic discs were swollen. The pattern of field loss indicated that the cause was neurological.

Adriana was offered a diagnosis of optic neuritis/papillitis and warned of the likelihood of losing vision in her left eye also. Suggested causes included multiple sclerosis, cerebral aneurism and tumour. A CT Scan the next day established that there was no infection or infiltration.

Adriana was advised of the usual treatment for optic neuritis, which is an MRI Scan and hospitalisation for the intravenous injection of 'massive doses of hydrocortisone'. She was told that a possible side effect of the hydrocortisone was cardiac arrest, and sent away to think about it. Her vision continued to deteriorate rapidly in her right eye to the point where one week later, she had light perception only.

Surprisingly, Adriana's response to her loss of vision was not despondency, but instinting optimism. She chose to bypass hospitals, tests and treatments. She steadfastly refused the MRI Scan and hydrocortisone treatment.

Verbal and written feedback from the ophthalmologist indicated that he thought Adriana was "far too cheerful, and that a person with a possible frontal lobe tumour was not capable of making good decisions for herself". In a report to her GP, he "felt that her emotional response to her illness was rather bizarre" and recommended that she see a psychiatrist.

Support
Three weeks after her initial vision loss, Adriana moved from her own unit into her parents' home. The ophthalmologist asked to see her parents and strongly recommended the MRI Scan and hydrocortisone treatment. Adriana's mother supported Adriana's decision to refuse treatment, and her belief in the need for rest. Adriana was told she would never read again, would find it difficult to go to church and would need a Guide Dog.

At psychiatric assessment, Adriana was deemed to be of sound mind and intellectually capable of making an informed choice. She was "not clinically depressed, but sad at what was happening to her". She signed a disclaimer for the ophthalmologist, confirming her refusal of treatment, and she has not seen him since.

Finding medical opinion demoralising, Adriana chose to focus her energies on developing her mobility skills while she waited for healing to take place. In April, she referred herself for mobility intervention. At assessment, Adriana was adamant about refusing further investigation of her vision loss. Her fitness for training was confirmed and a long cane training program was recommended.

Adriana tackled long can training with enormous drive and determination, practicing several times a day. She made rapid progress. Within a few weeks, she was able to walk through the CBD of her town and negotiate quite busy road crossings with prompt, safe traffic decisions.

Balance and communication changes
Less than four weeks into her program, Adriana's vision was continuing to deteriorate, her speech was unsteady and balance problems were becoming evident. When learning cane techniques for use on stairs, she reported that her legs were feeling numb from the knees down. Her long cane program was suspended until such time as her balance difficulties were resolved.

Adriana's balance deteriorated over the next week, to the point where she could no longer walk independently. She rested at her parents' home and continued to refuse medical intervention. Her GP had been sympathetic to her original decision to refuse treatment for optic neuritis. The instructor advised Adriana to keep her GP informed of her circumstances and progress for two reasons - primarily to act as a support to Adriana's parents who were caring for her and secondarily, to access domiciliary physiotherapy services when Adriana felt ready to become mobile again.

During the next six to eight weeks, Adriana was confined to her bed with a variety of symptoms including profound loss of hearing, light perception only in both eyes, headaches, numbness in first her right side, then her left side and loss of bowel control.

After two weeks, the domiciliary physiotherapist taught her to use an Oscar Frame (with wheels) to walk around the house with the help of her father. Later she graduated to a pick-up frame. Her father communicated to her using large cut-out cardboard letters and Adriana could speak her replies.

Recovery
It took six months for Adriana to recover hearing, vision and balance equivalent to the time when her mobility program was suspended. She re-referred herself for a review of her long cane skills in December, 1999. By that time she could travel into the CBD independently by bus from her parents' home, but was not yet ready to live independently.

In July 2000, fourteen months after the onset of balance problems, Adriana returned home to her own unit and was soon confidently managing her activities of daily living. She could walk independently into the CBD from her home using her long cane. She had occasional mobility sessions to update her cane skills.

Automated Perimetry testing in June 2001 indicated a nasal quadrant of residual vision in both eyes. She expressed frustration with Automated Perimetry testing, because of the time restraints imposed on her responses and claimed she could see a lot more looking out than others could looking in! She was informed that there are other methods of field testing which may give a more accurate measure of her current visual fields.

Adriana reported that her vision had continued to improve slowly and steadily without any plateaux. In August 2001, she could read two inch high lettering in black texta. She has been gradually regaining her colour vision since July 1999, starting with blue, followed by red, yellow and mauve. She cannot yet see green.

Adriana has recovered her hearing and balance but both are less stable when she is agitated. Under stress, her hearing becomes "hollow sounding" and she staggers a little when walking. She takes these signs as a cue to rest.

Adriana' GP made a retrospective diagnosis of Guillain-Barré Syndrome. He surmised that instead of the usual debilitating impact on the respiratory system, the condition seems to have targeted Adriana's sensory system.

Guillain-Barré Syndrome
Various Internet sites state that Guillain-Barré Syndrome is a rare disease of the peripheral nerves, affecting about one person in 100 000 . GBS usually occurs a few days or weeks after symptoms of a respiratory or gastrointestinal viral infection.

With GBS, the body's immune system attacks and starts to destroy the myelin sheath that surrounds the axons of many peripheral nerves, or even the axons themselves . The function of the myelin sheath is to speed up the transmission of nerve signals and allows the transmission of signals over long distances. When the myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently and muscles cease to respond to signals sent from the brain.

This attack on the peripheral nerves results in varying degrees of weakness or tingling sensations in the legs. It typically works its way up the body, with the weakness and sensations spreading to the arms and upper body. When GBS is severe, the patient is almost totally paralysed and may need a respirator to assist with breathing. Most patients recover from even the most severe cases of GBS, although some continue to have a certain degree of weakness. Less than 5% die from the condition . GBS can develop in any person at any age, regardless of their gender or ethnic background.

Guillain-Barré Syndrome is also known as acute inflammatory demyelinating polyneuropathy or Landry's ascending paralysis . There is a more chronic version of GBS known as chronic inflammatory demyelinating poly[radiculo]neuropathy (CIDP) .

Orientation and Mobility
Adriana's case study raises questions about the limitations of the medical model for effective client work. She wondered in hindsight, whether her driven approach to long cane training may have triggered her loss of balance and resulting immobility. On the other hand, she may have become bedridden anyway. She has no regrets about the decisions she made regarding her health care and mobility training. She is naturally delighted that her vision is continuing to improve, and has recently graduated to an ID cane.

O&M instructors must make a judgment about how much medical information we need at the commencement of a client's program. It can be a struggle to get pertinent medical information about a client. Or the information, once we get it, may seem vague or unintelligible. There is no substitute for listening to the client's own story. We need to trust their insight into their own circumstances and respect their right to take calculated risks. A prescriptive attitude on the part of the instructor is not necessarily helpful.

Of course, the maxim: Do No Harm, still applies. A meticulous approach to documenting our actions and recommendations to the client, and their responses, is essential if we are to avoid litigation. Where necessary, a disclaimer can be signed by the client to release the instructor or agency from responsibility for decisions taken by the client.

The likelihood of coming up against Guillain-Barré Syndrome again is fairly slim. Nevertheless, I have learnt from Adriana something more of they mystery of the brain, the value of assertiveness when dealing with the medical system, and the impact of courage and hope on the healing process.