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We are devastated to live without our Riley, but we are so proud of his legacy! #OurRareWarrior

Riley was born on the 1st of November 2012. He was so precious. Riley sailed through his first weeks as a new born, he had a little trouble feeding but we put it down to reflux. By 6 weeks old he was battling so much that we took him to the paediatrician who sent us to a nursing clinic where he was treated for reflux. At his 8 week injections, the nurse asked if he lifts his legs at all, like when we change his nappy. He moved them so we didn’t notice anything, but then realized he didn’t lift them at all, he also couldn’t hold his head up. We took him to his paediatrician and was diagnosed

with low muscle tone. We did some baby boot camp with him, some exercises to try strengthen his neck and back.

By 3 months old he still wasn’t holding up his head or lifting his legs. The breast-feeding clinic nurse held him and told us he didn’t feel like a 3-month-old baby, he felt like a new born, she sent us straight to a physiotherapist who told us he was on the low side of low muscle tone. She called the paediatrician and sent us straight back to him. He then did blood tests that confirmed raised CK levels. The next day he was booked into hospital for a series of tests to try figure out what was causing his low muscle tone. By this stage, Riley had mastered the art of smiling and charming everyone he came across and was quickly nicknamed Smiley Riley. Not much got him down. He dealt with what he had to with a tear and finished it off with a beaming smile. By 4 months old Riley’s feeding became a huge problem, he was losing weight and was not able to drink from the breast or the bottle, he chocked and splattered his milk and cried through every feed.

He was losing his ability to suck and swallow. He had a nasal tube put into assist with his feeds. At 6 months old his diagnoses, as officially confirmed at Red Cross Memorial hospital, was Spinal Muscular Atrophy severe type 1. Our worst fear was confirmed. He was given a year to live, without intervention he wouldn’t live to his second birthday. There was a dark cloud over our lives but Riley’s life shone brighter than the sun. He radiated happiness and we appreciated every minute with him. At 7 months old we took the first step in intervention and had PEG placed, a feeding tube direct to his stomach, this enabled us to have full control of his feeding and all nourishment needs where sorted. A month later Riley was struggling with a temperature and gagging,we took him in and he was booked into hospital.

In the early hours of the next morning his lungs collapsed,he went into respiratory failure and was immediately intubated on the ventilator. We didn’t think he would make it home, every time they tried to extubate, his lungs collapsed again. He was transferred to Christian Barnard under the care of apaediatric pulmonologist. After numerous tries and all the odds stacked against him he was successfully extubated onto Bipap and eventually off ventilation completely. We proudly took him home after 4 weeks in ICU to his own room. Accompanying him home was an oxygen machine, Bipap (a form of ventilation), sats monitor, cough assist machine, feeding pump, a suction machine and a cupboard full of medical supplies. At this stage Riley had lost all his ability to move. He missed all his mile stones, he never held up his head himself, he was not able to sit, roll, crawl, or ever walk.

He lay down most of his day as being in an upright position put too much pressure on his breathing and also caused him to choke, he could not swallow his secretions, he needed to be suctioned throughout the day. Riley managed to keep a balance between sleeping with assisted ventilation and being off Bipap in the day. Riley continued to have breathing problems and he was having a hard time keeping his sats up himself. He choked often and although he could not talk it was clear he was wanting his Bipap on more and more. He eventually lost his ability to smile, the mouth is controlled by muscles. But he continued to smile with his eyes. He also learnt how to frown and give a very mean cross face. By a year and a half he had become fully dependant on the Bipap ventilation.

He had lost the ability to breath on his own completely. This did not stop him from living the fullest life he could. His mind was not affected and he learnt how to communicate in his own way and he it was very clear what his favourite things were. He loved us reading to him, he loved Barney, loved playing but could not move his arms so we would hold his hand and help him play with very light toys and shoot things like nerf guns. He loved bathing and the pool, gravity did not affect him as much when he was in water so he moved his arms and legs a little. We took him out as often as we could. Outings took a lot of planning, luckily his machines had battery power. Some of our favourite outings were to the aquarium, the beach, the games centre and walks with our dog stitch. At 2 years old Riley welcomed a little sister into his life. He was not so convinced in the beginning, he frowned constantly at her, but we could see he loved her.

As she grew she quickly learned not to pull and tug at his pipes and she knew to be gentle with him. She took him toys and placed them in his hands for him, she would put little tea cups or cars at his head. She wanted to sit next to him and sleep on his bed with him all the time. Riley’s love for Barney quickly changed to a love for Mickey Mouse club house and he pretty much only wanted to watch mickey all day every day. The following 2 years became tough, as he got bigger he became heavier and could not move his body or head at all. His lungs started collapsing often and he was hospitalized a number of times. Just before his 3rd birthday he was intubated again and spent another month fighting to get back onto the bipap and eventually home. He was tired but we followed his ques.

The next couple of months took a huge toll on him, his body was paining and he was resisting being moved, he hated being carried and he started crying through cough treatments, he was hospitalized every 2 months and he became progressively more tired. He was now not only bipap dependent but he was oxygen dependent too. He was sleeping more and more. And his only care in the world was that Mickey Mouse was on the TV. Riley reached the point where we could no longer intervene. He passed away in our arms, asleep and completely comfortable, he had no pain, he seemed to be in the most peaceful sleep. Forever asleep. Forever 3 years old. Forever in our hearts. Riley endured more in his nearly 4 years than anyone could ever imagine. We shared his life but we spared the details. Riley’s life was an inspiration to so many people and he will never be forgotten.

He lived his life to the best of his ability and when SMA attacked he bounced back with such grace, he never let his disability get him down. He persevered and taught us so many life lessons. We are devastated to live without him but we are so proud of his legacy. He will always be our hero. Spinal muscular atrophy (SMA) is a muscular disease passed on genetically. One out of 40 people are carriers of this recessive gene. Where both parents carry a copy of the recessive SMA gene, there is a 25% chance that the pregnancy could result in a SMA child. You may never have heard of SMA before, however it’s important to realize that SMA is the number one genetic killer of children under the age of 2. What is SMA? A disease that is caused by an abnormal or missing gene known as the survival motor neuron gene (SMN1), which is responsible for the production of a protein essential to motor neurons.

Without this protein, lower motor neurons in the spinal cord degenerate and die. There is no cure for SMA, but in December 2016 the FDA approved the first treatment. Spinraza is the 3rd most expensive drug in the world, it came into the market at $750 000 for the first year of loading doses and $375 000 thereafter. A scary price to pay for the life of a child. There are various types of SMA (type 1, type 2, type 3 and type 4). Riley was diagnosed with type 1, the most severe. Type 1 symptoms usually occur before 6 months of age,more often before 3 months of age. Symptoms may even start in the womb. They are not able to hold up their heads, roll over, crawl,sit up without support, or walk. All of their muscles are extremely weak, with the

weakest muscles being the legs, upper arms, and neck. Their chest may appear con

cave, or very skinny at the top, with a big belly.

SMA affects all muscle systems, including sucking and swallowing. Common problems are, not being able to control excessive drooling (secretions) and getting proper nutrition for proper weight gain. A common cold can easily turn into pneumonia which is what usually takes the lives of these special children; along with “respiratory failure” (no longer have the lung or chest muscles to support breathing). Current statistics show that the average lifespan of a child with type I is only 8 months of age, with 80% dying by the age of one, and the rest by age 2 (if not put on permanent ventilation or “life support”). But these children often have bright, expressive faces and eyes. The brain is not affected, and they have been tested to have at least average to above average intelligence.

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