In her first few days after she was born, people had commented on how quiet and peaceful she was, and that she hardly ever complained. As Montanna grew, it was thought that she was just a slow developer for her age due to her lack of movement.

At 3 1/2 months, her parents Ross and Julie were told the news that their little girl would never be able to sit stand and may not live to her first birthday. Her parents first became aware that something was wrong when Julie would compare Montanna's development with other children in her mother's group. Other babies were moving around while Montanna was just lying there.

Montanna was diagnosed through a blood test. The Pediatrician they were seeing said it was only the second case that he had seen in twenty years, that's how rare SMA is.

Ross and Julie were told that there was nothing that they could do, and to go home and enjoy the time that they had left with their daughter.

But they were determined not to give up so easily. Ross began his research into SMA, and although Julie was hesitant and in denial at first, she too began trying to gain as much information about the disease as they could. They found doctor's knew very little of the condition which made it even harder.

Infection control was a big importance, as something as simple as a common cold could send Montanna into respiratory failure. They avoided large crowds with her such as supermarkets and any place they couldn't have control of the people she may come into contact with. They didn't have her wrapped in a bubble, but they had to be cautious. During her hospitalizations she often had a small tag pinned to her saying "Please wash your hands before you touch me". To some it may seem over the top, but it certainly got the message across.

Julie found it very hard when Ross came to tell her that Montanna would soon loose the ability to suck, and therefore breastfeed. As a first time mum, this was just another blow to Julie. They spoke with her doctor and discussed putting Montanna onto solids, as there was a decreased risk of aspiration. This was okay for a while she soon required the insertion of a nasogastric tube. This however, gave Montanna a new lease of life with improved nutrition, and the ability for her parents to now give her medication more accurately.

With Montanna's swallowing reflexes degenerating, so was her ability to control her secretions. They acquired the use of a suction machine soon after which would become the most important piece of equipment for Montanna. It was used to suction both orally and nasally. At around 5 months Montanna started to loose the ability to sit supported in an upright position. Julie was now starting to understand why SMA was also known as the robbing disease; it just seemed to slowly take everything away. Oxygen was now a requirement for Montanna.

There were many hospital visits when Montanna got a cold, as it just affected her so seriously. Chest physiotherapy was now important to maintain her respiratory health, which was later aided by the use of BiPap. At seven months of age, she was now restricted to a lying position.

When I looked after Montanna in early June, she was about 9 1/2 months old. Her care was more or less 24 hours a day. On continuous oxygen, she would often obstruct due to her inability to clear her airway and needed frequent suctioning. She gave us a scare a couple of times when her O2 sats dropped quite low due to mucous plugs but she recovered with suctioning. The thing with Montanna was that even though she couldn't move her arms and legs, or a lot of her internal muscles, she was bright, alert and she always managed a smile. Ross and Julie were more focused on the quality of life for Montanna rather than quantity. They didn't want to cross that invisible line where they would put their own needs in front of their own.

Montanna returned home to Heywood after about a week. She had short periods of going home, but always seemed to return to hospital after a few days due to pneumonia or increased secretions. Ross and Julie were constantly at her side, ready to suction, do physio or put on her Bi Pap machine. She then returned to hospital, and Ross and Julie knew that they wouldn't be taking Montanna home with them this time. Montanna passed away on June 25th.

Although in some ways this is a sad story, it puts the disease into perspective. There was this beautiful little girl, who although she couldn't move or talk, had the brightest eyes and would still smile at you. Cognitively she was very alert, and was lucky enough to have amazing parents who cared for in the way that they thought was the best, without overstepping the boundaries.

During Montanna's illness Ross and Julie set up the "Spinal Muscular Atrophy Association of Australia". Their aim is to provide a support network for those in Australia and overseas, so they can all offer each other support and information on possible treatments and care options available in Australia. They also hope to provided financial support for research and equipment.

The site has valuable information for parents of SMA kids, and a forum where parents can discuss their children. They found it great dealing with Montanna's illness, just being able to talk with people going through what they were, and hope they can now help other families through research awareness thanks to their Association.

- Rhiannon Taggert -

Together we can make a real difference!

SMA in Perspective

By Rhiannon Taggert RN Div 1,Royal Children's Hospital , Melbourne, Australia.

Montanna's Story