Yesterday was my birthday. Last year I made a disappointed post about my recent diagnosis of osteoporosis, and how that impacted on my thoughts of turning 28. I intend to make a similar post this year, but my health has become far more complicated.
This entry is purely to document the health struggles of the last year of my life, to place my future musings into context.
And so we begin.
I travelled interstate in May 2016 to see a neurosurgeon regarding my Chiari Malformation, Cranio-Cervical Instability, and Tethered Cord Syndrome. There we discovered that my cerebellar (brain) herniation had increased in size, and I would require decompression surgery – an invasive procedure involving permanently removing the base of my skull, potentially cauterising the herniated and damaged brain tissue, and patching the dura (the brain’s protective membrane). We would also need to release my tethered spinal cord, attached far too low to the base of my spine. Pretty intense stuff.
During the neurosurgeon’s examinations, he also spotted an unusual neurological quirk. Before going down the surgical route, he wanted that thoroughly examined. I’m already a complex patient. We needed to know if anything else was going on, too.
Back home I went, to find a neurologist familiar enough with my unusual and rare conditions. Before I could settle on a doctor, I became very unwell.
I know, I know. Story of my life, right? It’s always something.
A period of a few weeks of serious fatigue and excessive sleepiness is normal for me. These ‘flares’ are usually accompanied by an increase in pain, but not always. I kept on truckin’ and waited it out.
And waited some more.
6 weeks after the ‘flare’ began, I realised something new was happening. This was no ordinary flare up. I was sleeping between 16-22 hours a day, every day. I was next to impossible to rouse when needed. I missed appointments, skipped meals, stopped socialising outside my few wakened hours. No chance chasing neurology was on the cards.
My GP (family doctor) realised that I was critically low in vit D and iron stores, with low readings also in folate, magnesium, and b12. I was already on iron and vit D supplements (8000iu a day for vit D!), so this was more than just nutrition.
She referred me to gastroenterology, who diagnosed me with micronutrient deficiency as a result of a malabsorption disorder. We looked for all the usual culprits (coeliac, autoimmune disorders, thyroid disease, small intestinal bacterial overgrowth), but every marker came up clean.
Ehlers-Danlos Syndrome strikes again.
We increased my vit D supplement to 12,000iu a day, and started IV iron infusions. Still the fatigue and sleepiness persisted, and it started to cause its own issues.
The single greatest tool in any EDSers treatment is physiotherapy, or some other form of exercise. Remaining active reduces pain, increases muscle tone, decreases dislocations, increases blood circulation, aids digestion, reduces infection risk (especially respiratory), breaks down adrenaline, and releases endorphins – the body’s chemically identical morphine.
Staying active is what separates the ‘unwell’ from the ‘sick’.
Over the next few months, the rest of my body began to struggle. I wasn’t awake long enough to get in adequate nutrition, which was contributing to the fatigue and causing weight loss I could ill afford. I lost muscle tone and my dislocations drastically increased. The reduction to circulation and low fluid intake made my Postural Orthostatic Tachycardia Syndrome worse, and I struggled with arrthymias and fainting as blood pooled in my abdomen. The increase in adrenaline from the uncontrolled POTS made my gastroparesis worse, making adequate nutrition even harder to obtain. I started to get frequent urinary tract infections from sleeping through the need to pee, and that further sapped my body’s meagre reserves. Finally, I tore the cartilage in my dominant shoulder from too many dislocations, and it refused to stay in the joint.
We tried to tackle it piece by piece.
Cardiology prescribed a medication to prevent blood pooling, and try to keep my circulation moving while I’m not.
Dietetics moved me to a liquid formula diet, removing all ‘normal’ food (solid, liquid, or otherwise).
Gastroenterology flailed, unsure of what to do, sending me back to internal medicine for full-body advice.
Orthopaedics operated Mid-March, anchoring my socket back to where it belonged. The regional block failed, causing severe post-op pain, and repercussions. It has been 3 weeks and I’ve barely left the house the pain is still so severe, and though it is slightly improving day by day.
Finally, internal medicine are referring me through to neurology. It’s all come full circle. I need a sleep study (as an inpatient) to look for cataplexy, narcolepsy, hypersomnolence, or some other sleep disorder. It’s entirely possible this is all linked.
I started sleeping my life away in June 2016. Today is April 1, 2017. On a good day, I sleep only 14 hours. Earlier this week I went to bed at 10pm and woke at 9pm the following day. I fall asleep in waiting rooms, during movies, even during appointments if my doctor steps out of the room and I’m left alone with my swamping fatigue.
My shoulder will take a long time to heal. I’m not allowed to start physiotherapy/any active muscle use until at least 6 weeks post-op, but that may be pushed to 8. A healthy person can expect to start using their shoulder after 6 months. It may take me double that due to poor wound healing, and fear that an early dislocation will tear apart all their good work. It will be months until I am allowed out of the sling, and at least another 3 weeks before I’m even allowed to shower.
And after all of this, I will still need brain (decompression) surgery, and spinal fusion for my constantly partially-dislocating lower spine.
I am trying to stay strong, truly. It is just such a monumental task that some days the best I can offer is simply not to quit.
On those days, it is enough.