Rare Disease Day – 28February 

I’ve always been driven by positive stress. It has given me the drive to work my butt off in education, in supporting husband’s businesses, in parenting. Before I stopped teaching Secondary school, I’d start the year at about eight stone, but by school year’s end I’d be about six and a half to seven stone. I left teaching, commuted for three years to another city to study for an IT degree. Then the stress of finding no IT work in my home town, so into a lecture room at a tertiary institute, teaching IT. And throughout, stress.

On three separate occasions, I trip over nothing; on the last two occasion I break a toe (one in each foot). I put it down to clumsiness, or failing to take note of where I was stepping. One day in the supermarket, while searching the shelves for a particular product, starting at the bottom shelf and scanning each higher shelf for what I wanted, I realize that I’m feeling dizzy, and I fall back and down. Bang my head on the metal lower shelf behind me and dent it. I put that one down to being hungry.

Then comes the day when, as I walk through the kitchen to the dining room, intending to turn left into the lounge, my legs plant themselves and stiffen. I fall and cannot lift a foot to step in any direction to brace myself. Down I go, as straight as a powerpole, landing on and fracturing the neck of stem of the hip. Hubby just at that moment pulls up in the back yard and cuts the motor. He hears me go down and rushes to raise me. I scream in pain, and he realizes it’s Ambulance time.

At the hospital they ignore my description of freezing in place (they ask hubby do I drink during the day!). They replace the hip joint, and next morning ask me to leave the bed and walk, propping on a high frame. I feel like screaming, it is so sore. My legs are trembling, but I am told not to be so stupid and get on my feet and walk. Repeat later in the day.

The next day, repeat – this time hubby is with me. I tell him “I don’t care what they say, it feels as if it’s still broken!” My stress level is in the clouds by now, I am so scared to try and stand, let alone walk. But I do, and my legs stiffen and my feet root themselves to the floor.

I am sent for another x-Ray which reveals that, as typical are men, they have rammed home the stem of the replacement too hard, and have split the femur another six inches. Another replacement, and an extra feature- a spiraling wire to hold the femur together. As I come around, and they explain, stress again.

Next day, back to try the walker. I ask for the waist-high one with handles, and after arguing, they allow me that. I start to manage walking.

But the stress of the actual fracture and operations now has become anxiety, and I am stiffening up, hunching down into a near foetal position over the walker. By day’s end, I am having shaking spasms, as I try to walk, and as I lie in bed.

The tremors and spasms worsen over the next few days. The ward doctor doses me up with biodiazepines, and eventually they send me home to recover from the hip fracture.

But the spasms continue, and and I have episodes of such extreme stiffness I cannot speak, not move willingly, nor control spasmic movements of my arms, flailing beyond my control. Sometimes I black out.

I get taken to A & E, by either hubby or an ambulance. When hubby has to take me, he’s alone, and has to use a forearm to hit me across the groin to make me bend, to sit on the car seat. As I spasm into a stiff log, he has to swivel me around to face forwards, and again hit me, this time in back of the knees so my feet will get into the car.

At A & E they leave me in a bed, barriers up for me to hold onto (I am terrified I am going to fall off the side), with or without a sedative – according to the mood of the staff of the moment. They send me home as soon as they feel I am back in control.

As soon as I have to put a foot to the floor, back into spasm I go. I am yelled at, cursed (once), and grumped about, but ignored. Hubby is ordered to take me home, no matter what. On two occasions they refuse to allow us a wheel chair to the car.

On one day at home, my daughter came to visit at about midday. She found me in full spasm, and spent three hours sitting on my bed, watching me, and whenever I black out, she breathes for me. No phone within reach, and she’s too scared to leave the room.

Hubby arrives home, relieves her, and I spasm again,

A few days later, with someone home, I spasm about four times. Each time the ambulance arrives, we decide that as the A & E only leave me to myself and send me home after a while, the Ambos won’t bother taking me to hospital. On their fifth call out, the darling Ambos call my doctor and describe how they have found me during the day. My doctor calls A&E and demands they admit me to the Medical/Surgical Ward, so at last I am admitted.

Not good news as at first thought. The Ward Doctor has no idea of where to start with a diagnosis, what to consider as a cause. He refers me to Psychology. Their two leading staff come to my room, and find me asleep. I don’t respond to any stimulus so they sit beside me as I sleep.

Suddenly, I am six inches above the mattress! A full spasm, unable to breathe, arms flailing, unable to speak, all abductors and adductors in full tension. I’m arching – my heels and back of my head are all that are on the mattress. As they said, psychological conditions don’t eventuate from a dead sleep. It’s Neurological.

So I’m off to Wellington for MRI, x-Ray, and CAT scans. As I complete the last, the nurse who travelled with me assists me to the toilet, and as my bare feet touch the floor, I’m away – full spasm, face down over the loo pan. Luckily, before I’d used it.

Nurse calls the neurologist and others come too, and watch this weird manifestation of … What?

When I come to again, I’m back in the consult room.The lead Neurologist comes into the consult room, and does the physical manipulation tests all over again. They draw blood and send it off. And they diagnose Stiff Person’s Syndrome.

The cocktail of Meds is changed, and this time I do improve. After weeks, I leave the hospital dependent on hubby and home-help to shower, dress, toilet, prepare a cup of tea. Hubby copes as well as he can. My youngest daughter helps him and me.

With physiotherapy, I manage to learn to walk, but only around the block at first, and with a walker. One barking dog and I’m back to curling over Ito the near foetal position over the walking frame. A therapist walks with me, and she too admits that dog scares the b’jasus out of her. I graduate to walking around the block and to the corner store. One day, after turning the corner having  passed the place with the dog, I hear a screech of brakes, a squealing yelp, and silence. The dog had leapt over the fence, and been run down. Does anyone blame me for feeling … absolutely nothing for that dog. I return to the driver’s seat, and drive myself to supermarkets, parks…just for the sake of the independence.

Until another stress trigger. My son is involved in a major car crash, near fatal, at the other end of the island. He may be dead before we can get to him. But we make it, he makes it, and starts a long recovery. Hubby is with me, until his boss demands he return to work to compete end of academic year tasks. So I am on my own, crossing wide open spaces between family hostel and ward building, and worse, crossing third and fourth floor gangways from building to building, with glass balustrades. I walk the centre line, and feel the anxiety pulling me down into a crouch as I go.

Back home with a surprisingly, wonderfully recovered son, the stress takes over again. Back into the hospital for R & R. This time, knowing we would be relocating to a new city for hubby’s new position, the hospital give me a motorized hospital bed with air mattress, a bedside commode, a commode chair to get me to the shower, a tea trolley for trying to make snacks, bed pans, a leaning stool for propping as I try to work at the sink…who knows what else.

The movers shift everything in one day, setting up the hospital bed exactly in the best place possible – beside a garden view, and able to see and interact with family in the lounge, dining and kitchen.

But, the spasms become uncontrollable again. An ambulance takes me to the Rotorua hospital A&E, and when hubby tells them “it’s SPS” they don’t bat an eyelid. I’m in a four bed room, and the specialist arrives. He reads my meds list from the previous town, and asks (be prepared for asterisks) “What the f*** have they got you on this s*** for!” He tears up hubby’s list, sends a nurse straight to the pharmacy, and tells hubby to dump everything we’ve had me on up to now.

New meds, new understanding, new attitude from nursing staff. I leave the hospital with a supply of Fortisip – my spasms had made it impossible to eat solid food. Even the sight of a drinking straw coming at me would make me jerk backwards, and I’d be feeding the pillow or wall. My weight on final release was six and a half stone.

Since then, I have recovered weight (more than I really wanted), am able to walk distances, catch buses, and work at a desk job I created for myself. I do get pains in the back, tremors in legs when in bed, moments of dizziness if I forget my limitations and try to boogie, cramps in my calf muscles or toes.

But I have been so lucky compared to so many more SPSers. Yet, there are not “so many” more. There are so few of us, that one sufferer was able to place all FaceBook SPS group members onto a Google map. Thus I learned of someone living in a nearby city.

We are ONE IN A MILLION!

Researchers need funding to learn more about causes, positive identification, effective medication combinations, therapies and support funding.

We suffer. The range of manifestations of SPS is broad, and SPS accompanies or brings with it other syndromes, diseases and problems. There is no single cure or treatment. Medications alone helped me, and I helped myself with meditation and Tai Ch’i. Other SPSers face regular transfusions, stem cell transplants, or high and increasing dosages of medications without full release from their debilitating pain.

Our families suffer – some families split because of the strain of caring for a partner who is fine one day and gibbering, screaming, writhing in pain the next. Our children suffer – many miss out on sitting on a parent’s lap to read a story, or to tell a secret…or to have a moment of hugs. My elder daughter could only freeze in panic and withdraw without helping if I was in a spasm needing help.

Our friends suffer – what do they do if an SPSer goes down in front of them? What can they say? How can they understand the ups and downs from day to day? My friends see me at my current state and I know they have forgotten how only five years ago I was bed- and wheelchair-bound.

I am writing this at 9:00 pm on Saturday 28th in New Zealand time. Please publish, for the sake of this disease so rare you didn’t even have it in your list!

_______________________________________

This was originally written for Rare Diseases Day, in an Internet text box which was so small I couldn’t read back to correct errors. Until it was published at http://www.rarediseaseday.org/stories/5193 I had no idea how fatigue had affected my typing skills! Hence this corrected version, at last readable.

Please also realize that some details may be not in the correct time order. Brain outages can do that–foul up one’s memory. Thank you

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3 thoughts on “Rare Disease Day – 28February 

  1. Thank you for sharing,making me remember your giggle, your laugh,your bright shining eyes and your determination! Give your dear hubby a well-deserved cuddle .

  2. Wow, I can’t begin to put to words how I feel reading this. It is unimaginable what you went through and are facing. Your courage and spirit to even write about it is admirable. Thanks for sharing.

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