We Can Adjust The Sails

I’ve been stable for the past two years. 🙂 I’ve been incredibly lucky, and am incredibly thankful. I’ve crammed in excitement, filled up my photo album and created many many happy memories. Despite the uncertainty when first diagnosed, amazingly I’ve carried on living and loving life with Pulmonary Hypertension and Pulmonary Venous Occlusive Disease. However in recent weeks my stability has been rocked. New symptoms, new problems, new struggles… but no obvious reason why. I’m worried that it’s beginning to point towards a new developing illness. And the thought of the battle ahead -to get a diagnosis, and regain my previous health- is daunting.

Since the last days of sunny September I have started experiencing some worrying new symptoms. The scariest -a feeling of tiny lungs- restricts my breathing even more than normal. Although I have occasionally experienced something similar, this is in another league. More severe, longer lasting. My lungs shrink for a few days, then improve for a few days, then shrink again, then improve again. I have had seven episodes of ‘tiny lungs’ in the past seven weeks. The longest lasted for five awful days, the shortest for 36 hours. The worst left me completely unable to move or talk due to lack of oxygen, the least severe left me moving very slowly so my breathing could keep up. It is horrid. Distressing. Frightening. Worrying. When I’ve got ‘tiny lungs’, I’m just living hour by hour. Getting through the day. Yet when I awake to find improvement, and have a few days respite from the breathing difficulties, I’m a happy bunny. Optimistic that it won’t happen again, trying to forget what I’ve just been through. But then it comes back a few days later…

A fortnight ago I completed a barrage of heart tests for some of my PH team at Oxford. Then last week I visited my Respiratory consultant at the Royal Brompton for lung function tests and a strange arterial blood gas test from my ear! So far none of the assessments have shown any recent deterioration, or highlighted any new issues. On one hand this is wonderful fabulous news… whatever is causing my breathing issues, has so far not caused permanent damage to either my heart or lungs. However on the downside, we therefore have no clues or simple answer to explain my current problems. And if we’re no closer to identifying the cause, we’re no closer to identifying the solution. We’re once again facing the unknowns.

Lung function tests

My Spirometry graph!

My clamped ear after the arterial blood gas test

So we’re just left with a list of possibilities and maybes and guesses. Perhaps it is an infection covertly running amok through my body (the immune-suppressant drugs mask external symptoms); though this becomes less likely as the weeks pass. Perhaps it is due to a rise in my pulmonary arterial pressures (worsening of PH) which has not yet damaged my heart; this can only be checked with a Right Heart Catheter. Perhaps it is due to an increase in blocked pulmonary veins (worsening of PVOD), as on occasion it takes time to damage them enough to affect lung tests. Perhaps it is a common breathing disease like asthma, however my symptoms do not match and my oxygen saturations fall. Perhaps my lungs have become hyper-reactive or have developed random allergies, however I’ve never had allergies before, and no obvious triggers can be identified. Perhaps it is due to a mild obstruction I have had for a few years in the small airways of my lungs (swelling? scarring?), however why would it be causing issue now after many years. It’s all guesses still. In plain, we just don’t know what is going on.

So Plan A is to start a daily steroid/bronchi dilator inhaler, to see if it helps. We’re just throwing a medicine at my lungs and watching to see what happens! My favourite TV doctor, House MD, would have approved! 🙂 I will be discussed at the Multi Discipline Team meeting next week, to hopefully identify a plan B. Maybe a Right Heart Catheter. Maybe allergy testing. Maybe a steroid increase. Maybe a swap of PH medicines. I’m keeping in contact with Harefield, in case I need to be re-activated on the transplant list again. But for now it is just waiting and guessing and hoping.

So we don’t yet know all of the answers. We can’t yet explain what is wrong or why. We haven’t yet figured out if it’ll worsen or improve. We’re facing the unknowns. I’m at the start of the journey to find a diagnosis and solution to the problems. And it’s daunting. Really daunting. And although I’ve faced this before, whilst searching for my PH and PVOD diagnoses, it feels psychologically harder this time, as I know what is ahead. I’m very aware of how much work it takes to get an answer and a name. I remember the number of tests and examinations and appointments and consultations. I recall needing to chase up and pester and email and ring. They’ll be starting and stopping medication, scary and frank conversations, disappointments and false diagnoses. And they’ll be plenty of shrugged shoulders and “we don’t know!”. It takes months, or even years, to find out what is going on… and all whilst feeling ill. So I’m slightly daunted by the journey ahead.

And I’m also pretty scared. The worsened breathlessness with ‘tiny lungs’ is frightening. Like I’ve got a bag on my head, or I’m breathing through a straw, or there’s not enough air in the room. Sometimes I feel like I’m mere seconds away from having a full blown panic as I can’t breathe quite right. I’m constantly trying to distract myself and remain upbeat, ’cause I know that if I let panic creep in then it will overwhelm and disable me, making the situation many times worse. I remember trying caving once, and feeling so claustrophobic crawling through tunnels. For the hour underground I had to constantly calm and reassure myself, as was always one second away from having a complete panic. It’s like that… but for hours or days at a time. I’m also slightly nervous about the future. Will we solve it before it damages my heart and lungs? Will it worsen? Will we find a new disease that prevents a future transplant?

I also feel kinda powerlessness. I’m no longer solely in control of my destiny. I need to rely on doctors and nurses and consultants. I’m just a lowly blade of grass in the vast field that is the NHS. 😛 So despite wanting the answers now, I have to wait alongside the other blades of grass. And as there are millions of us needing the attention and expertise of a few, everything takes time. Consequently, a week after being told to start the inhaler, I’m still waiting to be prescribed it.

Although illness and uncertainty are difficult to experience, I’m trying to remind myself that I’ve been here before and come out the other side. I’ve faced rocky roads and then gone on to have stability. Winter always gives way to Spring. I have everything crossed that my wonderful body finds a way to solve this once more.

But until then I’m just waiting and guessing and hoping. 🙂