Waiting And Guessing And Hoping

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.


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.

photo 1n

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. 🙂


6 thoughts on “Waiting And Guessing And Hoping

  1. A great blog as always. Your attitude is wonderful, I so understand the complex avalanche of feelings you are going through and you are in my thoughts daily. We are so lucky to have such wonderful specialists at our disposal who must feel huge frustration and concern whilst also dealing with the grinding and groaning machine called the NHS. I stay confident with you, looking forward to seeing you enjoying wonderful trips to France. Your scooter looks great, where on earth did you find such a good one? Xx


    1. Thanks Lucy. I love my scooter, it is called a Luggie. It keeps me the same size as a normal person so I can go in and out of doorways and around shops without knocking too much over!!


  2. Hi there,
    I’ve had a worsening during the autumn months for several years myself. I am coming to find that I had several allergies which occur with the damp and the mold. They were never really that noticeable until my lungs functioned at a lower threshold and thus my tolerance for just about any nuisance infection/ virus/allergy was not good. (A silly rhinovirus which may have made me carry a tissue before will send me to the couch for days) It does happen with a bit of cyclicality. I chalk it up to hormones and my body getting used to inflammation associated with infection/allergy. It is annoying because you cannot predict from day to day how you are going to feel. My doctors have mixed emotions on this, but I am using prednisone to manage the worse days. I am already on a low daily base dose 10mg and bringing it up to 20mg has brought me relief. At the very least, it has lessened the effort to breathe into tiny lungs. Hope this finds you on a good day or at the very least, gives you an avenue to explore.
    Take care and puzzle&tea on!
    PS. Also I’ve notice that wood fire smoke seems to send me to a very bad place even if it is coming from my neighbors chimney.


    1. Hi Tracey. Thank for your thoughts, that’s so interesting, and it sounds so similar to me. I’m on prednisolone at the moment (7.5mg), but we have discussed putting it up again if this continues. Do you stay on the highest dose of 20mg all year around, or just in the Autumn months? And yes wood fire seems bad for me too- I’ve got all of my windows closed at the moment as lots of the neighbours have fires!! Do you know what your specific allergies are- have you been able to pinpoint them? Have you ever had allergy testing? 🙂


      1. Hey there apologies for the delay! I was thinking a response notification would come through email. I am on about 10 mg prednisone for most of the year until fall or I get a cold. I have been tested. Ragweed is a big trigger which starts here around the end of August. Then come the molds created by the damp leaves yeesh! The inflammation seems to act a lot like PH and PVOD when it comes to producing breathlessness on exertion. It’s funny my DLCO was measured at 52% in early Nov last year and in Dec it popped back up to 84%. Might make sense to try to treat an allergy even if you don’t know what it is? As far as I can see, aside from the annoying side effects of a steroid, most other meds that help out with allergies are pretty benign to PH/PVOD. It’s funny that we both ended up on low dose Prednisone. I cannot find any medical research which supports it, but of the Very few people I have encountered


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