Miriam:

Swimming in the sea of chronic serious illness, my twenty-something year old daughter Tríona, who has idiopathic pulmonary capillaritis, had a childhood characterized by acute hospital admissions, spending night after night in yet another hospital bed, as an inpatient with acute pulmonary haemorrhaging. She passed day after day as a patient on day-wards receiving regular infusions of, over time, intravenous steroids, cyclophosphamide, rituximab and/ or immunoglobulin. Subsequently, we were trained to give her home infusions of IVIg (intravenous immunoglobulin) initially via PICC lines (peripherally inserted central catheter) and then it changed to via a portacath. This took several hours as Tríona needed a very slow infusion rate. She developed aseptic meningitis-like symptoms the first time she had IVIg, and lived experience showed that her body needed a very slow infusion rate to help decrease side effects. Then, last year, we were trained in administering subcutaneous infusions of immunoglobulin instead of intravenous. This means that instead of the infusion going into a vein, it goes into the tissue just under her skin with a number of sterile special needles over about an hour. This was a near miraculous development for us— home infusions that her body tolerated better, were easier to administer, and had a massively reduced infection risk. Tick. Tick. Tick. Tríona’s illness journey was punctured by repeated absences from school of varying length, from just hours for outpatient attendances, to days and weeks for admissions, to recurrent admissions over a period of months with profound vomiting, dramatic progressive weight loss and hypokalaemia from Cryptococcus-induced vomiting. Such episodes were bolstered by cherished periods of normality—the sumptuous, understated, and underappreciated bliss of ordinary life, family occasions, car drives, visits to the cinema, children’s parties and, in recent years, of attending college.

Maya Angelou said: “I’ve learned that people will forget what you said, people will forget what you did, but people will never forget how you made them feel.”

Over the years, we have received time, care and treatment from skilled, compassionate, and patient-centred doctors, nurses, and other health and social care professionals. These experiences were needed healing balm and remain with us. Hospital cups of tea filled to the brim with compassion and humanity were sustaining; gifts of chocolates cannot express our gratitude to them. The legacy of these actions remains embedded within us. Looking back, perhaps those experiences were the yin to the yang of other soured and unwanted clinical ordeals. The yang included serrated words and comments that pierced with surgical precision and are flavourings of memories I wish we didn’t have.

One of the most devastating yang experiences on Tríona’s illness journey was the diagnosis at twenty years of age of non-cirrhotic portal hypertension with oesophageal varices from mercaptopurine use. She underwent oesophageal banding twice to treat the varices and was due to have another scope, a gastroscopy (OGD/EGD, (o)esphagogastroduodenoscopy) to assess and manage oesophageal varices if identified again. This involved the insertion of a camera scope into her mouth, down into her stomach to check for oesophageal varices. Then, once identified, another tube was added that circumscribed the scope and was used to treat any varices. She had several gastroscopies over the years even before developing varices. As a child, though, they were done under general anaesthetic, so she was asleep for the procedure. As an adult, the scope was done under sedation. In more recent years, unfortunately, she has had some very difficult experiences with gastroscopies, experiencing nightmares after the procedure, and in the weeks preceding and anticipating the next one.

Tríona:

I’ve always hated OGDs, along with any other procedure where I’m only under sedation and not proper anaesthesia. But OGDs are definitely the worst. It’s hard to make someone whose never experienced it understand just how terribly traumatic it is.

The sedation confuses me and paralyses me, but it doesn’t knock me out. I’m trapped, unable to move as a tube is put down my throat, choking me. And because I’m sedated, I can’t remember what’s happening, why I’m being choked, and I can’t get the tube out. I’m given even more sedation, which only makes me more confused and disorientated, and does nothing to calm down the panic I feel at being unable to breathe. I’ve always breathed more through my mouth than my nose.

I was terrified leading up to my most recent OGD. More terrified than usual, though I’m always sick with fear when the procedure date starts to loom over me. This time, though, I was definitely more upset than usual. Maybe I had a sixth sense that something was going to go wrong, or maybe it was because we received the date later than usual, so I had less time to prepare myself mentally. Either way, I was practically faint with nerves by the time the OGD rolled around.

The procedure seemed to go off without a hitch, but afterwards I felt off: small, vulnerable, upset. I felt like a little girl again, and all I wanted was to cling to my mother, where I was safe. My mind felt slow and confused, even after the sedation had worn off, and I felt sad.

Those were the first signs that something was wrong.

The next day, I still felt upset and clingy, and I had started having trouble speaking. Thinking felt like trying to move through waist-high sludge; not impossible, but slow and difficult.

That was the most terrifying part of the whole mess. I’ve been sick longer than I can remember. I can deal with a damaged body. But my mind has always been safe. Everyone’s been telling me I’m clever since I was a little girl. My body may be broken, but my mind made up for that in spades. If I lost that, what did I have left?

At any rate, things weren’t too bad on Friday, the day after the OGD, and on Saturday I seemed to improve a bit. Mam and I went out to a craft fair. We won a prize in the raffle, and the word-finding difficulty seemed to ease, even though my mind still felt slow. But it seemed like I was okay, that the sedation had just affected me worse this time. We thought maybe they’d used a higher dosage or something, and that was why I’d been so affected, but I’d be fine in the morning.

Miriam:

Friday, the day after Tríona’s scope, I noticed that she had some word-finding difficulties. I found it curious, but logically I thought that it must have been related in some way to the OGD and sedation the day before. So in true medical-parent style, I downplayed it and decided to take the time honoured watch-and-wait approach. The next day, her speech fluctuated throughout the day. It actually improved for a while in the afternoon and we had a lovely pre-Christmas excursion to a local craft fair. Tríona mooched around happily as we admired different stalls. We “ooh-ed” and “ahhhh-ed” appropriately and enthusiastically as we chatted with various artists and jewellery-makers.

Tríona:

Then morning came, and I had turned a corner, but not the “Improvement” corner. Rather, I had turned the corner to complete stammering, and blankly staring as I tried and failed to comprehend what my mother was saying to me. The words on the page of my book made no sense. It was just a jumble of nonsense. It was hard even to understand what the lyrics of the songs I put on were.

We went out for a treat. I had a cake, I think, but I don’t really remember. I just remember being so confused, and upset that I couldn’t seem to get my thoughts across. I didn’t understand what was happening. I barely understood what Mam was saying. Mostly I just nodded when she talked, because well, honestly, I don’t remember. I think it was just instinct. Frankly, I wasn’t even aware enough to be frightened at that point. I just felt so weak and tired and slow. Trying to understand what was going on was impossible.

I slept in the car on the way back, and Mam woke me up as we were nearing our house. She told me we were going to the hospital, that she was calling an ambulance.

In hindsight, the ambulance shows how serious Mam was taking it. I’ve been hospitalized over fifty times in my life (I think). Even when I was five years old and coughed blood for the first time, I’m pretty sure Mam just drove to the hospital. That she chose to call an ambulance so I’d be seen quicker… it didn’t say good things about what was going on.

Of course, I wasn’t able to comprehend that at the time. I was confused that she said she was getting an ambulance. I think it was around the time I was escorted into it by the lovely ambulance team that I started getting frightened. They were asking Mam and I questions, and I think I answered some of them, but honestly I was mostly confused; the unfamiliarity of being in an ambulance, people talking about strokes, frightened me. It took me a while to understand what they meant, but once it sunk in, I was terrified.

Like I said, my mind has always been my safe place, and if I didn’t have that, I have nothing.

Miriam:

Tríona’s symptoms became more bewildering as the weekend progressed. I still believed the precipitating factor must have been the OGD in some way, though I was attributing it to the sedation for the procedure. Inaction was not an option, initially I hoped to get an urgent outpatient MRI scan of her brain somehow. However, an experienced family doctor recommended managing Tríona in accordance with stroke protocols in view of her complicated medical history. And so, we found ourselves at home waiting for the arrival of the ambulance.

Tríona:

We were in the ambulance for a while. I think we went to a different hospital first. They did a CT and a doctor examined me. I don’t remember what they said, but afterwards we went to our local hospital. I was in the A&E (accident and emergency department) there until the next day I think.

My sister and her fiancée came to see me in the A&E. They brought Mam and I some food because we hadn’t eaten since the morning. My Dad called me on my mobile phone, but I couldn’t understand what he was saying.

More doctors came and went. I think they did more tests, but it’s all a blur. I was basically non-verbal by the end of the day. I was communicating via text, but the words were coming out written almost phonetically and hard to comprehend. My thoughts were so confused and slow that I wasn’t scared anymore. I was just confused. I didn’t understand what was going on. I couldn’t remember the names or faces of the doctors and nurses who were coming in and out.

I was moved to a room the next day. The clinical staff on the ward were very kind. They did more tests, but they were all negative. It was decided to wait for the neurologist to come on Friday, but they were giving the medical team advice in the background in the interim.

A speech and language therapist (SLT) came to see me. I’ll call them “my SLT.” They were lovely, but it was hard for me to understand what they wanted me to do. They asked me questions and I answered as best I could, but I felt very lethargic and indifferent. Trying to interact with people was giving me a headache. I couldn’t meet their eyes because it was physically painful. So I watched their eyebrows or lips instead.

Miriam:

On that rain-free Sunday afternoon in December, Tríona walked into the ambulance mobilizing with her walking stick as usual. However, she felt weaker on one side, which was new. The two ambulance team members had that wonderful cocktail-type blend of humour, skilled competence, and compassion. On arrival to the nearest hospital with acute stroke on call, Tríona had a CT brain with contrast and a CT angiogram. Thankfully, both were normal. After being examined clinically, as Tríona did not have a stroke she was discharged to go to our local acute hospital for ongoing care. As it happens, I also work there. Usually she is extremely articulate with an easy use and flow of language. Now she struggled to speak and her texting became disjointed and phonetic. She was dizzy on standing and for a day or so needed a Zimmer frame to mobilise to the ensuite toilet. She was too unwell to shower unaided and needed assistance, which is very tough for a young adult. All of which was a dramatic change from the week before.

Tríona received individual sessions with the speech and language therapist during her hospital stay. In parallel to ongoing investigations, she was also advised to do exercises on a particular app. Meanwhile, an MRI brain and electroencephalogram (EEG) were both negative. Cause identification and seeking reversibility are deeply engrained and almost unconscious instincts for a clinician. So, as a physician, my belief that the event was somehow related to her recent scope and accompanying sedation was an unintended comfort. That intuition helped and supported me as a mom, too, when navigating this new manifestation of hell.

Tríona:

I watched a lot of shows on my laptop. Reading, my favourite activity, was practically impossible. I couldn’t understand what I was reading, and the effort stressed me out and frightened me. I couldn’t write either, which was bad for my mental health. I’ve always used writing to get my frustrations out, so with no way to properly communicate with anybody, no writing and no reading to hide from the world, my thoughts started to spiral.

I wondered if I had some sort of permanent brain damage that would leave me stuck like this forever. I wondered if I had gone crazy, or if my subconscious was making up my symptoms somehow.

Miriam:

From the age of 7, Tríona was a voracious and enthusiastic reader. Before developing this FND, she was very articulate, could read three hundred pages per day, and had an almost photographic recall of the information. She is also a keen writer, regularly uploads her own fanfic stories and loves attending college. But now her thought processing seemed slowed. Meanwhile, we tried to juggle visiting in order to maximise having a family member or close friend with her as a support and company given this new restriction in communication.

Tríona:

See, the thing about having an ultra-rare disease is that it’s hard for some people to believe you. How can I have chest pain when there aren’t any nerve endings in that area to cause it? How can I be coughing up blood when there’s nothing on the x-ray? It’s enough to cause even me to doubt myself sometimes. If there’s nothing wrong on the tests, surely there’s nothing wrong at all? That’s what I feared with the Functional Neurological Disorder (FND). I couldn’t talk, no matter how hard I tried, but if there was nothing they could see on the x-rays, would I be discharged and left that way forever? When my thoughts became clear enough for me to think those things, that’s when I started to really get frightened.

Miriam:

We have encountered doubt and disbelief during our twenty-something years navigating Tríona’s serious-illness journey. These were definitely not universal experiences but sadly not unusual too. The impact of these encounters is that I tend to wonder: what do I, and we, need to do to get believed by clinicians? How do we convince them to help her and act?

Tríona:

The neurologist came to see me on Friday afternoon. Dad had finished work and come to spend some time with me by the time they arrived. They asked a bunch of questions first, and Mam answered them. Then they sent my parents out of the room and did some tests. I don’t remember most of them, except for the one where they asked me to touch my nose and I poked my glasses and got them smudged, which made me overly upset. The next thing I remember, they went out to talk to my parents privately, which I found frightening. I thought maybe it really was brain damage, and they wanted to break the news to them first.

It was terrifying, to imagine being trapped like that forever. I was still basically non-verbal, but Mam said my spelling had improved a bit. For myself, I couldn’t feel any real difference, other than I was frightened more than I was confused. They came back in and the doctor told us they thought it was something called a “Functional Neurological Disorder,” FND for short. They explained it, but I just couldn’t comprehend what they were saying. What I did understand was that there was no medicine or anything that they could use to cure it. It was a matter of waiting for it to fade, and that could take anything from weeks to months. I honestly felt more afraid when they left than before they came.

I was in hospital for another week, being visited daily Monday to Thursday by my SLT, and I was seen by other various interdisciplinary team members. My SLT, bless them, hit on a good treatment for my speech problems when they had me try out an app for treating apraxia. On my second Friday in hospital, the neurologist came to see me again, and confirmed the FND diagnosis. It was decided I could be discharged, because all that could be done was give me Speech and Language Therapy.

I was actually upset at being discharged. The hospital provided SLT for inpatients but does not have outpatient SLT facilities. I knew, especially as we were so close to Christmas, that it would be early January at least before we’d be able to arrange for outpatient SLT. I was afraid of being alone when Mam was at work because if I had an emergency, how would I get help?

But I went home anyways.

Miriam:

Tríona received amazing care in the acute hospital. However, public access to SLT on discharge was unavailable. I am a palliative medicine doctor. In palliative care, we are passionate about interdisciplinary care because it benefits patients. I felt burdened by the seemingly elusive access to SLT for my daughter as a person living at home. As the Christmas and holiday period progressed, many non-urgent healthcare services were on leave. I penned, that is to say typed, a detailed email summarising her presenting symptoms, the investigations, the diagnosis of FND, and of the treatment given and care received to date. It gave me a feeling of doing something, of moving forward somehow. Then I turned to the magic of the internet, seeking out a private speech and language therapist in person or online in Ireland, North or South, and perhaps even online in the UK. I wondered about the option of going to England for a number of weeks—would that be a way to access intensive SLT for her? I was feeling desperate, and I knew it. Tríona had lost so many educational opportunities through physical ill-health and treatment schedules, and here was yet another impediment to her education looming.

The tension flowed out of my body as I received two positive responses to my email requests for private speech and language therapy. I started to consider and tentatively plan the practicalities, but we had liftoff! We had real options for accessing speech therapy for her. The following morning I was contacted by a very experienced SLT who was not only based in the same county, but in the same town, and who worked from home nearby. The next morning, Tríona walked over to them on the gently snow-laden footpath. She walked onwards towards her speech returning, towards hope and to the restoration of her full young adult life.

There is a twist in our story, though. Shortly afterwards, Réaltín (Irish for little star), one of our beloved doggies, became increasingly ill over about ten days. She had developed a bowel obstruction and required urgent surgery. As Tríona focused on caring for Réaltín, her stammer melted further away, her speech became clearer, and she became more confident. She transformed back into her pre-FND self.

Tríona:

Within a few sessions, my stuttering had faded to once or twice a day. It became basically non-existent, behind the occasional word-finding difficulty when I was stressed. But the FND didn’t go completely. My reading became much slower than it used to be, I struggled to remember things I had just said or read. I was more prone to headaches and confusion, and found it harder to meet people’s eyes. But I was back at college, I was able to talk and read, and I cannot express how grateful I was for that. I will never be able to take the ability to communicate for granted again.

Miriam:

All was well for a little while until the relapses began. It’s hard to describe the feelings of sadness and disappointment when this happens. Tríona, who is highly intelligent and articulate, now suddenly, depending on the severity of the FND relapse, struggles to speak, has dilated pupils, and her texts become phonetic again. She appears stoned despite not using any recreational drugs, and decision-making is very difficult for her during these flares.

We were delighted when Tríona returned to college in the autumn. She continues to learn more about the triggers, and how to manage them.