[3] Never Close Our Eyes

-- We got no time to lose --

It's a brand new day, and with it comes a brand new month. 

August is Spinal Muscular Atrophy awareness month. But for me, every single day is governed by SMA. 

When I was born, a panic erupted. My ears were flabby bits of skin, and everyone originally suspected that I had a recessive genetic disorder which caused facial and throat skeletal deformities. It was quickly confirmed that I did not have Treacher Collins Syndrome, but that I shared their ears due to a freak of nature. 

My family grieved for my voice as they were told that I may never talk, but that I would get all the support I needed to help me hear and say my first words. My plucky nine year old sister Martina, however, was doing laps of honour with glee. I had no ears, but I wouldn't get water or sand in my ears, so she was happy for my freak of nature. 

But there was a reason she saw what was and still is a profound disability as a blessing in disguise. She didn't run around doing laps of glee - she drove. She came to meet me from her own hospital bed in The Children's hospital in Belfast. Martina had Spinal Muscular Atrophy, a diagnosis which had turned what mum and dad had planned for their life as a family upside down. 

Martina had found out that I was coming in December 1989 on Barbour Ward. She was recovering from surgery to put rods into her spine to straighten her scoliosis. It's the most painful part of having SMA. And from the very beginning she hoped that I would share that gene with her. 

Exactly eight years after her diagnosis, I was brought back to hospital to see her neurologist, Dr Hicks, for the fourth time. The three previous times I hadn't shown any of the medical signs of SMA, and my reflexes were present. But mum knew the small signs, even though they weren't enough to diagnose me. The choking on bottles, the constipation and my eye tracking instead of moving my head all said that I likely had it. This visit with Dr Hicks was different, and although my fasciculations hadn't showed up to the party, my reflexes were gone.

 

Within the hour, I officially had Spinal Muscular Atrophy Type 2. My family mourned, except Martina, who celebrated with cheers up and down the ward as the news reached her. What she, nor I, understood was that if you told a GP in 1991 that you had SMA, they'd probably tell you to enjoy every second, because they couldn't guarantee you many over two years old. 

When I turned six in 1996, Martina bought me a nurses uniform. By this stage I had spent as much as 3/4 of my life in Paul Ward at The Children's, surrounded by drips which I could pause myself, antibiotics I hated unless they were pink, and kids with lots of neurological conditions. I was still obsessed with the idea of being a nurse. 

Martina was 14. She had broken her arm playing with our cousin, and therefore did her make-up with one hand and no mirror. It was still flawless. She had been tested for Cystic Fibrosis, a degenerative and often fatal disease which affects the amount of mucus secreted in the lungs and other organs. Although she tested negative, she had endured lots of nasty infections including a rust infection in which you cough up "rust". She also had to be fed directly into her bloodstream with TPN while she recovered from yeast cells in her lungs. Martina used steam tents, oxygen and all sorts of aggressive physiotherapy techniques. 

In the winter of 1996 Martina had her hair plaited in a homage to Lauryn Hill, her musical idol. For the three years previous she had performed in musicals at The Lyric Theatre in Belfast as part of the chorus. My love for music and theatre definitely comes from her. 

On Christmas Day, Martina fell sick, spending most of mass in an alcove receiving physio from mum and dad. She enjoyed the day with family, and we continued as we so often had to when either of us were sick. 

When Boxing Day arrived she was taken to hospital, and Martina went to the clouds on January 4 1997. She was 20 days shy of her 15th birthday. 

Spinal Muscular Atrophy is the number one genetic killer of children under the age of two. The most severe form claims 90% of its victims by the age of eight months. Just a handful of those live beyond two years of age. 

In many ways, I have been blessed with the good part of the genetic lottery with the intermediate form of SMA. Yet, I require a life support machine to breathe for me every time I sleep, I'm fed through a tube in my stomach, and if I'm not using a WREX mobile arm support I can't even scratch my own nose without help. This is my normal. It is probably your worst nightmare. To rely on somebody else to help you do everything, except to think.

This SMA awareness month, I'll be blogging about my day to day life with SMA. I'll be brutally honest, showing every part of living with an incurable, untreatable but manageable chronic disease. There will be bad days, but they'll be outnumbered by the good days. You can follow a month with me, from tears of laughter to tears of pain. 

I'll be blogging, tweeting and YouTubing my journey through August using #LifeWithHollywood - as you learn the facts, talk about it in your circle. Spread the message of SMA. Because although our bodies fail us, our minds are stronger than most. We're blessed with a voice, although we all must use it in different ways, sometimes using machines. Use yours and spread the story of SMA. 

The song of my month will be Never Close Our Eyes by Adam Lambert. The song speaks of living every last second of life. Dr Hicks once commented on the resilience and tenacity of those of us with SMA and related neuromuscular conditions. This song is, in the words of Paula Abdul, our truth.

http://youtu.be/u3dkVk3F57c

Please visit www.muscular-dystropy.org and support the Muscular Dystrophy Campaign as they have supported my family through our journey.