Updated 22 June 2016

Motor-neuron disease patient from Krugersdorp saved with custom-made breathing device

A Krugersdorp patient is recovering well after doctors found a way to improve his breathing difficulties caused by motor-neuron disease.

A Krugersdorp man with motor-neuron disease, the same debilitating medical disorder suffered by ex-Springbok rugby player Joost van der Westhuizen, was saved recently thanks to the actions of his caring wife and collaboration between local and international medical teams.

Fifty-six-year-old Deon Nel, who has been on a ventilator since 2008 to assist him to breathe, and can now only move his eyes, was admitted to Netcare Krugersdorp Hospital’s intensive care unit (ICU) with severe and potentially life-threatening respiratory distress. 

The specialist who has been treating Deon for many years, ear, nose and throat (ENT) specialist, Dr Andre Hough, quickly established that, after so many years of being ventilated Deon’s anatomy had changed; his trachea had become enlarged, and his ventilation tracheostomy tube was no longer fitting properly, resulting in it ‘leaking’ air  which resulted in him struggling to breathe.

Deon’s wife, Bernice Nel, says that she is able to communicate with her husband to some extent through his eyes. “Deon ‘talks’ to me in this way, and I could see he was distressed. The problem was that there was no ventilation tracheostomy tube available in the country that was large enough to fit his enlarged trachea,” she adds.

Being a thoroughly devoted partner to her husband and taking care of him around the clock, Bernice immediately began researching where the medical team could find a ventilation tube that would fit Deon’s trachea. Dr Hough and Bernice soon established that Smiths Medical in the United States was able to custom design tracheostomy tubes to meet the needs of patients in such situations. 

Dr Hough immediately got to work taking measurements, life-size x-rays and scans, and liaising with doctors in the US and France, as well as with Smiths Medical and their local representative Babcock Healthcare, in order to ensure that they got the design of Deon’s trachea tube absolutely right first time.

Help from the US

The measurements and design were sent to the US where a special tube with two cuffs was especially manufactured for Deon. According to Dr Hough, the custom designed tracheostomy tube proved to be a perfect fit. Deon has recovered well from the procedure and his breathing has been restored to normal. 

“This is the second time that Dr Hough and his team at Netcare Krugersdorp Hospital have saved Deon and I am extremely grateful to them. The doctors and staff at the hospital are like a second family to us. Dr Hough also fitted Deon’s original tracheostomy in 2008, which has given him seven years of life so far. I am certain that Deon is thankful for that too. 

“While Deon is bed-ridden and relies on a ventilator to breathe, he has an incredibly strong will to live, and I know he still wants the privilege of meeting his grandchildren one day,” Bernice says.

Motor neuron disease, which is also known as amyotrophic lateral sclerosis (ALS), is a relatively rare, progressive medical condition which results in damage to the nervous system. Specialist nerve cells in the brain and spinal cord degenerate to the point that a sufferer may find it increasingly difficult to perform daily activities and even become bed-ridden, as happened to Deon. Public awareness in South Africa of this debilitating condition has increased in recent years mainly because of the publicity regarding Joost van der Westhuizen’s condition.

“I think it is important that ALS caregivers are aware that this option of developing a custom-designed tracheostomy tube is available as it could save other patients’ lives. While motor neuron disease is considered relatively rare, I come into contact with a number of sufferers and their families from around the world,” observes Bernice.

“Since Deon became ill, I have made it my business to find out as much as I can about ALS, and I provide support to and also receive support from sufferers and their families from around the globe. The doctors and staff at Netcare Krugersdorp Hospital know us well by now and are aware that I am there to assist in any way I can. I have watched what they do to care for him closely over the years and, with the knowledge I have acquired, I am able to ventilate and care for him at our home in Krugersdorp.”

“I can now see in Deon’s eyes that he is once more completely at ease, and that the operation was a success,” concludes Bernice.


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