Marco's story differs from those of many other SCI/SCD patients; while not unique, it is an unusual case. He is 23 years old and had, for some years, worked as an electronics technician, fabricating complex devices. Over the course of a winter, he took four months off of his full work schedule to work and travel in India and Thailand. The journey was exciting, and without any adverse incidents; Marco returned feeling invigorated and ready to resume his job.
About four weeks after his return, he came down with a sudden fever along with headaches and angina. His physician was not able to make any specific diagnosis; he was treated with antibiotics and immediately improved. Another month passed and a new and strange symptom arose: a twitching and feeling of “pulling” at Marco’s left heel. He tried to disregard it for a number of days and went about his work and life. However, the odd feeling began to expand to a pain in his leg, traveling slowly and steadily up his calf. One week after this started, he was having back pain that extended up to the area of the thoracic spine. At this point he admitted himself to a nearby hospital; whatever he had – it seemed to him to be both serious and worsening.
The odd feeling began to expand to a pain in his leg, traveling slowly and steadily up his calf.
On admission, his doctors found extensive edema around the thoracic spinal cord and began treating him with high-dose steroids. Marco’s condition nevertheless deteriorated and one day later progressed to paraplegia AIS A at the level of Th-10. As a secondary condition, Marco developed acute urinary retention (due to a lack of sphincter tone) and loss of muscle tone in the rectal sphincter. Additionally, the vision in his left eye began to blur due to occular inflammation (uveitis and retinitis). Serology tests (including tests for a range of viral and bacterial infections) were negative. While the signs and symptoms were clear, the doctors could make no specific diagnosis; there was however a suspicion that Marco might have had a condition known as Behçet’s Syndrome (see Box 2); this despite the fact that Marco did not fulfill the strict criteria required for a case definition.
At the first hospital it was difficult to understand or accept my health condition because of the unclear diagnosis; I didn’t know what to expect. It was a very unpleasant situation with all of the uncertainty.
Box 2 | Behçet’s Syndrome
Named after the Turkish dermatologist who first described the condition, Hulusi Behçet, Behçet’s syndrome (or Behçet’s disease) is a recurring, inflammatory disease that affects multiple body systems. In the past, viral or bacterial infections were thought to cause the syndrome, but no direct evidence has yet been found and it is now assume that the disease has an autoimmune etiology.10 The body systems affected may include eyes, skin, mucosa, joints, the gastro-intestinal and central nervous systems, blood vessels and lungs. It is commonly considered a diagnosis of exclusion where a patient’s condition is of unknown etiology and other common causes have been ruled out. The diagnosis of Behçet’s disease is based upon a grouping of symptoms; the strict clinical diagnosis must include 1112recurrent oral ulcerations and two of the following:
- Recurrent genital ulcerations
- Eye lesions
- Skin lesions
- Positive Pathergy test
While uncommon in Europe and North America, the condition is more prevalent in the “Silk Road” region, stretching from the Middle East into Eastern Asia. There is presently no cure for Behçet’s syndrome and treatment options generally focus on alleviating symptoms through medications such as corticosteroids.
His prognosis was far from certain. No one was able to predict if, when and to what degree he would regain his functioning.
A decision was made to continue steroid therapy and Marco did show some improvement over the following days. The edema lessened, some sensitivity restored and with that his overall condition was somewhat better, though still far from normal. However, three weeks later, Marco still had no control over his urination or bowel movements and required catheterization, colon massage and manual evacuation of the bowel.
It was at this point that Marco was transferred to an SCD unit for further rehabilitation and medical management. There, the initial urethra catheter was changed into a suprapubic catheter that enters the bladder through the abdomen.
I really couldn’t stand the urinary catheter. It was painful when putting it in and then it felt like some foreign object inside of me; it was really unpleasant. The supra-pubic catheter was even worse. I felt like I had a hole in my stomach and couldn’t bear to look at it.
At this time-point, despite both the unknown etiology of his condition and prognosis, efforts were undertaken to support his independence, increase his mobility and improve his self-care skills. However, his prognosis was far from certain. No one was able to predict if, when and to what degree he would regain his functioning.
After six weeks of supra-pubic catheterization, Marco developed a detrusor sphincter dyssynergy, requiring a new intervention to manage his urination. A urinary condom was fitted to passively collect urine as needed.
For the first two months at the Rehabilitation centre Marco regained muscle power in his legs and trunk. Consequently he was able to move his legs to some extent and to eat without resting or holding on to a table/chair. He also made great improvements in handling the wheelchair. Two months following the onset of the disease the health care team decided to undergo a comprehensive assessment as the basis for the next rehabilitation phase.