Xavier Alford told BBC News how he vividly recalls the day, twelve years ago, when he was told by his doctor that he had a very rare disease. His doctor explained that the disease is slowly causing paralysis. He was thirty years old.
First, his doctor gave him some encouraging news. He told him that his illness was seldom fatal. Then he told Xavier that there is no cure. He said that although he could not predict exactly how the disease would affect him, he must prepare Xavier for the eventual paralysis of his arms and legs.
The only way Xavier could cope at the time was to avoid any discussion of his disease and its symptoms with his wife or his children.
Peripheral Neuropathies
Xavier was diagnosed with multifocal motor neuropathy, one of a group of disorders under the heading of autoimmune peripheral neuropathies.
Motor neuropathy causes the immune system to disrupt the myelin sheath covering the nerves. The myelin sheath allows the transmission of electrical impulses across the nerve cells. Once disrupted, the nerve cells are no longer able to transmit the signals from the brain to the muscles. The result is paralysis.
Guillain Barre syndrome (GBS), although rare, is one of the most recognizable of these diseases. People with this disease may become fully paralyzed but their brains are still viable.
The majority of GBS patients are able to not only survive the disorder, but many emerge fully from the paralysis. Some though are still confined to wheelchairs or must rely on breathing apparatus.
In Xavier’s case his health will only decline.
Xavier’s First Symptoms
The first symptom was numbness in his toes, and then he was unable to use his right hand or hold his camera steady. Xavier has difficulty writing and tires easily.
Xavier worried about the effect this would have on his wife and children. His train of thought led him to remain silent and not discuss his disease with his family. His stock answers were: “I’m fine,” “Don’t worry,” or “I don’t know.”
Xavier said everyone eventually stopped questioning him. For years he kept his worries and fears to himself. However, about two years ago his condition worsened to a point that he could no longer avoid the obvious.
Encouraging Others
Xavier had produced BBC documentaries for about fifteen years. When he filmed stories of personal turmoil, he always told the contributors that the filming would in many ways be beneficial and give them a sense of self-examination.
His wife used that premise to suggest that Xavier direct a film about his own illness. She thought it might help him to confront his own feelings and hidden anxieties.
When he began work on the film entitled “Locked in: Breaking the Silence” he gradually realized that he truly did need support.
He began by searching for other GBS patients, hoping that they could help him understand what was happening to him. It was this connection with others that caused Xavier to fully comprehend his condition.
Most of the other GBS patients he had contacted had the type of peripheral neuropathy from which they would either fully or partially recover. Xavier’s condition would only decline.
Scott’s GBS Paralysis
Xavier describes his interaction with forty-year-old Scott who related his initial symptoms as tingling in his hands.
But only thirty-two hours later Scott found himself in the ICU on a ventilator. He was put on a ventilator and was completely paralyzed. His brain continued to function normally. His recovery took two years, but he is currently able to live on his own with the use of a wheelchair.
About Rob’s Paralysis
Xavier received a call from seventy-year-old Rob, a GBS patient, who also described an initial tingling in his hands. Within one week Rob became paralyzed and was unable to move or communicate other than to blink his eyes.
The Key Difference
Looking at both illnesses emphasized a key difference between Rob and Scott’s condition and Xavier’s illness. Although there is no cure for Xavier, there is a treatment that can, temporarily, slow or halt its progress.
About Intravenous Immunoglobulin (IVIG)
Xavier describes his “lifeline” as being composed of blood from thousands of donors. He receives an injection that is composed of millions of healthy antibodies that resets his immune system. The injections work, but eventually his arms and legs give way to extreme fatigue.
He is concerned that at some future date the injection will no longer be effective. For now though, Xavier is extremely grateful for the treatment.
The film has opened his eyes to the many questions his children have also kept to themselves. His son, Louis at age eleven, asked Xavier if he will get GBS. Xavier was able to put Louis at ease by explaining that Louis was not any more susceptible to GBS than others and that it is a rare disease.
Thanks to the film and the opportunity to be open about his illness, Xavier and his family can now discuss their future. Xavier admits he often gets angry about his condition, but he also says that now he can live with it.
