Rarity breeds dissent

By this time I hadn’t eaten or drunk anything for an entire week. I relished my cup of tea as if it was fine champagne.

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As before, IVIg started to pull me out of crisis. Unfortunately another bank holiday weekend was about to strike. The SALT team wouldn’t be working over the three day weekend and couldn’t see me that Friday. The neurology team informed me they weren’t happy for my NG tube to be removed anyway. They reminded me of the risks of aspirating food and fluid into my lungs, possibly causing an infection, and emphasised how that would delay my thymectomy they’d booked for the 22nd May. 

I had no choice but to keep the dreadful tube until the following Tuesday, at the earliest.

It nearly broke me. Of all the drama and procedures that had occured over the previous month, this seemingly benign thing brought me to floods of tears and woe. 

There was a routine in hospital that I quickly fell into during my previous stay. It centred around doctors and drugs rounds, having obs taken, visitors and mealtimes. Sitting there unable to eat, watching my roommates food being delivered, was tough. I hadn’t been started on the ‘feed’ that would be delivered directly into my stomach via the tube and was extremely hungry, having not eaten for three days. I couldn’t even drink water, as my doctor had made me nil by mouth, although I had been hooked up to a saline/dextrose drip and IV vitamins. 

I almost managed to convince myself that I’d undoubtedly be able to swallow soup. All I had to do was pull out the damned tube myself.

The four days until Tuesday stretched endlessly in front of me. 

Must. Meditate. More…

My IVIg continued over the long bank holiday weekend. That weekend my ever difficult veins decided to play up. My cannula started leaking immunoglobulin. It had to be replaced and none of the nurses on the ward were willing to try inserting a new one, such was my reputation for being tricky to cannulate. They called for back up.

A nurse from A&E arrived. He tried a hand and an inner elbow before admitting defeat. Off he went and returned with a phlebotomist. She peered and tapped at my hands and arms, eventually deciding to go for a vein halfway between my wrist and scarred elbow. 

As she slid the cannula into my vein, I gasped in pain. My husband gripped my other hand, as I grimaced. I’d never had a needle in that location before and assumed it was just a tender place to have one. The phlebotomist muttered about feeling a nodule as she tried to work the cannula in. She carried on regardless and went on to try flushing it before I insisted she removed it. She covered it with a large dressing, but I could see a large, lumpy bruise forming underneath it.

My vein had ruptured.

A doctor arrived and on her 2nd attempt got one into my other arm.

I improved a little as my IViG went on and as expected my spirometry readings improved and I moved out of crisis.
A SALT therapist came to do my assessment on Tuesday morning and deemed me sufficiently able to swallow soft foods. I had to keep the tube for a further 24 hours, and if I was able to eat enough, it could come out the next day. I was overjoyed.

Ordering food from the special menu was disproportionately exciting. By this time I hadn’t eaten or drunk anything for an entire week. I relished my cup of tea as if it was fine champagne.

When my lunch arrived, I ate tentatively. Small forkfuls, very slowly. It wasn’t easy, my stomach had shrunk and my throat muscles weren’t cooperating fully. I was determined to finish, and did eventually. Again at dinner time, I worked diligently through my two courses. I wasn’t going to risk the doctors deciding my nutritional intake inadequate enough to leave my NG tube in place.

Wednesday brought Dr D, along with the entire team of neurologists  to see me. He was happy to see me out of crisis but disappointed that again, the benefits were limited. The general hope was that I’d continue to improve gradually and be well enough to be discharged for a few days the following week. They planned to bring me back for some preventative IViG after that to get me in the best possible condition for my surgery.

He had answers this time too.

Unknown to me, when I’d been in the neurosciences centre previously, my blood had been sent to the specialist lab to test for some obscure antibodies in addition to the anti-MuSK antibodies.

Anti-striational antibodies

I had tested positive for one of this subset; anti-Kv1.4.

This explained a lot. Why my myasthenia was so severe and stubbornly unmanageable. I tried to find facts and struggled through the medical research data and reports. There was little to no information in layperson’s terms. What I did glean was ominous;

The presence of striational antibodies is associated with more severe disease in all MG subgroups. Anti-Kv1.4 antibody is a useful marker for the development of lethal autoimmune myocarditis.

Antibodies were found to bind in a cross-striational pattern to skeletal and heart muscle tissue sections.

Anti-Kv1.4 was found in only 12-15% of myasthenics with thymomas. It was well known that striational antibodies were associated with late-onset MG subgroup. Meaning I was again amongst the rarest of the rare, being under 50 years old. Given that thymomas were present in only 10% of myasthenics, the following data was mindblowing;

The frequencies of striational antibodies in T-MG (Thymoma MG) patients are generally high. Many reports have shown that anti-titin antibodies are detected in 49–95% of T-MG, with anti-RyR found in 70–80% of cases and anti-Kv1.4 in only 40–70% of cases.

And;

We also confirmed that some MG patients with anti-Kv1.4 antibodies had a risk for lethal arrhythmias including ventricular tachycardia, sick sinus syndrome, and complete atrial ventricular block. We emphasize that special attention should be paid to MG patients with anti-Kv1.4 antibodies.

Oh.

Having a reason for why I hadn’t stayed stable for long was eclipsed by this discovery. It put my tachycardia following PLEX in a worrying context too. I needed to ask about the new threat to my health, to my life, the next time a neurologist visited me. And scour the internet for more digestible facts. My mind kept replaying the words ‘sudden death’ that I’d spotted.

The dietician agreed my feeding tube could be removed. My joy over this took the edge off my other news. No more permanently aggravated sinuses and dripping nose! 

As the end of week crept up I noticed with familiar dismay that my swallowing was already deteriorating. I voluntarily chose to go back to pureed food only three days after starting to eat the soft, mashable food options. By the start of the following week I was struggling significantly again, going through multiple glasses of water in my effort to get each meal down. 

On his first weekly ward round of the next week, Dr D decided l couldn’t be discharged. I fatigued fast during the usual tests and my speech was mangled. He knew as well as I did that I was on another downward spiral. I’d only completed my last round of IViG a week earlier.

All plans to go home were cancelled. The IViG that I was supposed to have as an outpatient the following week seemed a long way off.

I’d been in hospital for almost six weeks, with the exception of a few short days at home. A distant memory now. 

Staying positive just got more challenging. I’d always been stubborn, but was my disease more obstinate than me? No matter how determined I was to bounce back from this, myasthenia remained a loud voice of dissent. 

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