At the start of March, I had four weeks of unexplained illness. For a month, I lay fatigued on the sofa, my oxygen saturations dropping every time I moved. My PH consultant had seen me during this time, so was keen for me to be admitted to the Brompton for more detailed tests, to gauge the condition of my heart and lungs, and work out if I was starting to permanently decline. Apparently the Brompton staff were expecting a very poorly me to enter the ward doors; however happily, in the interim weeks I’d made a dramatic recovery. As quickly as I’d nosedived down, I’d shot back up again. Nonetheless, even though I was no longer as symptomatic, I still had two very hectic days of tests lined up, looking for clues to explain my puzzling month of decline.
Once again, with my husband working, a wonderful voluntary driver took me to London. It was my first ever overnight hospital stay alone, so I sent Phil continuous whatsapp photos, in case he was wondering about my current view, or what I was eating! 😉 Happily, I’d been allocated the best bed in the ward: next to an inch opened window. Essential to counterbalance the permanently pumping radiators, and great for people-watching from high. In the first two hours there were swabs and blood tests, vitals checks and ECGs, questions and forms, consultations and chats. Hectic and exhausting.
First of the big assessments was the CardioPulmonary Exercise Test, where detailed heart and lung recordings are taken whilst running or biking. I’d last done it a year ago, when I wasn’t on oxygen… but this time I was. Cue much confusion! They’d never had an oxygen user before, so were unsure how I should do the test. Normally the patient wears a sealed gas mask to collect all the expelled gases, however if I did so then I would be unable to wear a nasal cannula for my oxygen, so would be completing exercise on room temperature, which is potentially dangerous. However if they gave me my oxygen throughout, then the gas mask would be unsealed, thus would affect the results. A debate ensued. For fear of making me ill, the physiologists wanted to abandon the respiratory part, and collect heart readings only. But knowing PVOD is a lung disease, and keen to get accurate results to compare with last year, I was determined to do both the heart and lung tests… without oxygen. Luckily, a PH consultant agreed to supervise me so I could do it all. So with electrodes on my chest, a gas mask around my face, I peddled continually as they increased resistance. Amazingly, I managed seven minutes before I was gasping for breath and feeling faint! Immediately afterwards, still wearing the claustrophobic mask, the consultant grabbed my shaking arm and stabbed a needle in my artery to collect some arterial bloods. Stabbed whilst breathless! The life of a PH patient! 🙂
I didn’t have sleep problems until I got ill. However over the last four years, like many PH patients, I’ve not since had a restorative nights sleep. The quantity is sufficient- a solid nine hours nightly, however the quality is lacking! I now toss and turn repeatedly, wake frequently, dream too much, and biweekly awake unable to breath. Normally a nose sleeper, my body sometimes struggles to get enough oxygen this way, yet forgets to open my mouth to get more air… until I awake gasping! Very strange! So at 9pm, a sleep doctor arrived on ward to fit me with gadgets for a sleep test. Chest wires measured my lungs movements, a neck probe recorded sound, chest electrodes checked my heart rhythm in the night. A nasal cannula recorded the air breathed in and out, an oximeter took continuous oxygen saturation recordings, and a large box attached to my chest collated all the information. Despite the uncomfortable equipment, despite being only able to sleep on my back because of it, despite being in a bright noisy ward… I actually fell asleep fine. However a 3am toilet stop in a luminous ward awoke me, so I spent the next few hours wide awake. I never get insomnia, but on the night of a sleep test I did! 
The next day, I had an Echocardiogram of my heart, and Lung Function Tests. As an inpatient, instead of being allowed to scooter myself, I was pushed around in wheelchairs by porters. Relaxing and less exhausting, but very time consuming as I had to keep waiting for porters or ambulances to take me around the site. Wonderfully, my friend Zoe and baby Agnes visited, so we naughtily escaped the hospital grounds to take the little one to play in the park over the road. The nurses kept ringing me to return for the next item on the agenda, but I really needed to escape hospital world for a while!
So happily it was all good news. PH normally causes heart failure, and indeed when first diagnosed my heart was double it’s size so functioning badly. However, amazingly, over the last four years, and in particular this last year, my heart has completely returned to normal. Apparently you wouldn’t know I had PH from looking at my heart. As my lungs have deteriorated due to the PVOD, they have probably taken pressure off the heart, allowing it to return to normal. On the main, the Exercise Test was comparable to last year; meaning although my lungs are very inefficient and functioning poorly compared to my peers, they have not deteriorated recently. Happily the Ve/VCO2 score, which measures inefficient carbon dioxide ventilation, has actually improved from 59 last year, to 44 this time (ideally needs to be below 30). Presumably due to the steroid and immuno-suppressant medication taken since the last test. The TLCO score in the Lung Function Tests, showed my lungs are still functioning at 30%, so I continue to be stable. Again, despite the more detailed tests, and despite the expertise of the principal PH lead at the hospital, there was still no explanation for my puzzling deterioration. It is not a normal clinical trait of PH, but as I’ve done it a number of times throughout my illness, it is therefore probably related to my lesser understood PVOD. I continue to be “a mystery”. And predictably the sleep test didn’t identify any major problems- probably as I was awake for a good chunk of it! 😀
For the first time I was discharged before rush hour. However as oxygen users are not allowed in voluntary driver cars when leaving hospital, I felt very guilty as I was chauffeured home in the back of a private ambulance! Lottie was very excited to see me- she will be very confused when I am in hospital for a few months with my transplant! So I’m still plodding along, I’m still stable, I’m still a mystery… and I’m still waiting and hoping for my call for new lungs one day soon.
We Can Adjust The Sails 