Singer Celine Dion and British man Ian Rawlins both live with Stiff Person Syndrome

 

Singer Celine Dion recently acknowledged that she has an extremely rare neurological disorder called stiff person syndrome (SPS). According to a recent article in The Metro, the disease occurs when a person’s immune system attacks that person’s own tissues. The result is an attack on the brain and spinal cord. Celine has regrettably been forced to cancel a series of upcoming performances.

The same disorder has affected former cyclist and British lorry driver Ian Rawlins, age 58. Before Ian’s illness, he had competed in “motorbike trial events” throughout the world. He appeared on the BBC biking show “Kick Start.”

Ian has been living with SPS for 18 years. It is aptly termed ‘one in a million,’ as estimates are that only one or two people have SPS out of one million individuals.

SPS causes painful spasms and stiffness in limbs and other parts of the body. When Ian is having spasms, he develops a ‘statue like” appearance.

Treatment for the rare disorder was only recently made available to SPS patients. Until then, no effective medication could keep the disease in check. As yet, there is no cure.

Ian’s Diagnosis

In 2005 Ian began to experience stiffness and pain in his legs. He was mistakenly diagnosed with gluten ataxia. But the symptoms worsened, and he had to resign from his job.

Then about three years ago he was diagnosed with SPS. He is now taking medication to control the spasms, but his overall condition has deteriorated. Ian is experiencing painful symptoms almost daily.

About SPS Recognition

Ian’s wife Andrea, age 54, describes the difficulty in attempting to administer medication if Ian is having a spasm. When in a convulsive state, Ian’s jaw tightens, his teeth are clenched, and his body is rigid – like a statue. Seizures may last for hours.

The spasms can be triggered by various disturbances such as sudden motions or sounds. Ian will either stiffen or begin to have spasms that may last for hours.

Andrea describes one Christmas when Ian had uncontrollable seizures. She immediately called for an ambulance. The paramedics were not familiar with the disease and tried to search for the information as they drove to the couple’s home.

Once in the home they acknowledged that they were unfamiliar with the disease and had to call for assistance. When the backup team arrived, they were also unfamiliar with the illness.

Andrea warned them that if they moved Ian during a spasm, they could rupture his muscles or dislocate his bones.

The Power of Love

Ian and his wife receive mail from Celine Dione’s fans usually referring to her as the Power of Love singer. Many fans want to know more about her illness. Andrea can only reply that it will be a long time before Celine returns to the stage. And yet the fans still believe or hope that she will be performing again in the near future.

Andrea mentioned that one option for Celine may be a stem cell transplant. According to The Canadian Press (2014) one of the first patients to receive a stem cell transplant for SPS was a personal fitness trainer named Tina Ceroni of Toronto. Tina says the transplant she received in 2011 has brought her back to a normal life.

When the attacks were most frequent, not only were Tina’s legs and arms affected, but the muscles controlling her respiration would contract and she was unable to breathe properly. Tina was rushed to the hospital 47 times the year before she had the transplant.

Researchers are currently focusing on the cause of the disease and the connection to anti-GAD antibodies. A high concentration of GAD antibodies can be found in the cerebrospinal fluid indicating a link with GAD.

Additional SPS information may be found in the Genetic and Rare Diseases Information Center of the NIH.

Rose Duesterwald

Rose Duesterwald

Rose became acquainted with Patient Worthy after her husband was diagnosed with Acute Myeloid Leukemia (AML) six years ago. During this period of partial remission, Rose researched investigational drugs to be prepared in the event of a relapse. Her husband died February 12, 2021 with a rare and unexplained occurrence of liver cancer possibly unrelated to AML.

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