We Need To Talk (A Guest Blog by Luke, Sam's Husband)

We need to talk.

Those four words the head consultant said to me last Wednesday will resonate with me for as long as I live; for reasons I can only begin to explain.

Entering our fourth week at the Brompton and the few days running up to the major ward round on Wednesday, Sam had become increasingly unwell. She was requiring a great deal of morphine in a sub-cutaneous (under the skin) syringe pump to manage her chest (rib) pain, which at this stage was so agonising that it had begun to wake her at 4am. In addition Sam was being given a cocktail of intravenous antibiotics in various forms (injections and drips), some as many as four times-a-day, together with anti-sickness medications - the kind used with cancer chemotherapies - and anxiolytics (anti-anxiety) to quell the distress. However in spite of all this, the infection was still not yielding. Sam was overwhelmed with exhaustion from fighting and tired of all the pain and did what her body instructed her to do - and that was to sleep.

The ward round team came to see us first. The team gowned up and came in to see that Sam was on her full-face mask with the non-invasive ventilator in a deep and unresponsive sleep. In addition, her oxygen requirements through the machine were vastly increased (from 4l/min to 10l/min) and she did not look well at all. Seeing all this and having discussed the team's struggle to manage Sam's infection the head consultant, Di Bilton, asked me if there was somewhere private we could talk. My heart sank below my stomach. I was under no illusion what this meant but I still didn't quite believe it. We sat on a brown leather couch in the relative's room. The conversation that ensued was difficult to swallow. Di explained that she hadn't expected Sam to have survived the last major hospitalisation during the summer of last year and this time Sam had deteriorated. She said that there was a fine line between life and death it could go either way for Sam and that the next few days were critical. I was strongly advised to not go to work and in fact not to leave the hospital. The treatment options for Sam at this stage were equally risky; the blood work had suggested the current regime wasn't controlling the infection and continuing may not improve things. However a change of antibiotics would provide a window for the infection to take hold and and make Sam worse. Both scenarios had potentially deadly consequences for Sam. Obviously it was a lot to take on board but I couldn't process the news immediately. It was, however, brought into sharp focus the minute I saw Sam again. The conversation with the consultant had now coloured how I looked at her, seeing Sam as fragile and that she could slip away at any moment. The cliches are absolutely true in these circumstances; my world had literally stopped. The nurses asked if I had got hold of Sam's Mum. I told them I would ring, but I couldn't find the words in my mind to describe the situation. Thankfully they took that responsibility from me and rang Sam's parents so they could come up to be with her. All I could do is sit by Sammy and pace around, unable to decide what to do. The room had somehow concentrated my grief and thoughts of how this might play out dominated my mind. I really wanted Sam to wake up for just a few moments to say something, or anything. I needed that feedback to quell my fears, but all I could do was cry in the bathroom when I became overwhelmed.

Sam's mum, Ros, rushed to the hospital, with Alan (Sam's Dad) and brother Ian waiting at home poised to travel up if the situation became critical. To be honest I was grateful for the company. We could only sit there and spectate as Sam battled her CF as she slept. We prayed that God would spare her life and I found great comfort in Psalm 16. Sam continued to sleep for 2 days, although it felt like weeks. The team kept giving Sam her intravenous drugs and I know that she was fighting valiantly as she slept. We all braced ourselves for the worst. we wondered if Sam would ever see her 28th birthday later that week. The nursing staff, who are very fond of Sam would periodically pop in to see her, obviously having heard the news. The gravity of the situation weighed heavily on my mind and I was sharply reminded of it when the nursing staff kept offering me cups of tea - this was very kind, of course, but the fact that they continued to asked how I was doing meant that Sam wasn't fairing very well at all.

Despite all of this Sam began to recover. Sam woke up (thank God). At first it was for a few moments to use the bathroom. But then those moments evolved into short periods of time. Sam wasn't very lucid in these periods of wake but that was enough for our hopes to be raised. Day by day Sam became more conscious for longer periods. On Saturday Sam managed to enjoy her birthday. We marked it with a small celebration with the families and decorated her hospital room.

Today, Sam had her first day of full consciousness. She is stable now and we are more hopeful. Clearly, we have a long road ahead of us and we may never leave hospital - unless we get that transplant. Sam is very tough and we take great hope from the knowledge that God has spared her life again.

It's a Long Hard Slog!

I don't wish to bore you with a long detailed update, but I know that many of you like to know how I'm getting on!

Last weekend was a bad one. Things took a turn for the 'worse' on Friday. My chest pain had become so unmanageable that I was put back onto a morphine syringe driver. However, the good news to accompany this is that subcutaneous anti-sickness drugs can also be added, so all my feeling of nausea are gone (yay) and I am generally able to eat well. My sputum remained thick and stubborn, so we decided to increase my usage of a drug called DNase which breaks down the sputum at a molecular level, making it easier to cough up. I also began using saline to hydrate my airways. In addition to this, physio was increased from three sessions to four slightly shorter sessions. Needless to say this was exhausting, so time spent not doing nebulisers or physio was spent sleeping on the NIV. We got through it! I felt so much better by Monday which was good, as it meant I could enjoy spending time with my brother, Ian, when he came up to visit!

However, I did continue to feel tired and when Mum came to visit on Tuesday I spent quite a lot of the time asleep (sorry Mum!). However, it transpires that there was an explanation for this... You know my lovely CRP infection markers had come right down to 40? Well Tuesday's tests showed them to have jumped right back up to 140! Grrrrrrrrrrr!! Stuuuuuuuuupid CRP/CF/body! Therefore I had another change of IVs on ward round (only one of the drugs, so I'm now on Meropenum, Colomycin and now Septrin). Again it's going to be a case of waiting and seeing whether this combination works. Obviously I'm annoyed, but we'll have to just wait and see if this Septrin IV kicks some bacterial butt! Oh, and nebulised Tobi has decided to make me tight chested, so that's a no-go for the moment!

Another recent development is that Luke has now started staying with me (glamping in Chelsea, you know!). This has helped me immensely and he just travels to work from the hospital as opposed from Eimi's. Hopefully towards the end of my admission when I am feeling stronger he can go back and enjoy spending time with his friends! An illustration of how helpful Luke is here is that last night I had a particularly poor night, for some reason my sputum decided to mobilise itself at about 2.30am. It just meant with Luke being here, he could help me clear and just generally comfort me and I hacked up about half a sputum pots worth of joy! Obviously had Luke not been here I would have been wonderfully looked after by the nurses and on call physios, but Luke knows my chest so well (sounds dodgy!) that we could just get on with it efficiently and as quickly as possible.

On a bit of a different note, my diabetes is being a bit of a pain. Obviously my steroid dose is still high, so that plays havoc with blood sugar levels, but I've been getting high readings first thing in the morning which isn't great. It's an indication of the progression of my CF related diabetes. So I will need to now take some long acting insulin at night and see if that helps. What worries me about other things going 'wrong' is that is I so desperately need new lungs, I don't want anything else to start failing before I (hopefully) receive my transplant!

I'm so overwhelmed by people's responses to the ITV Meridian news piece that I featured in on Wednesday, as it makes you feel quite vulnerable putting all your emotions out for the general public to see! Thanks for all the lovely messages and I just hope and pray that people are encouraged to sign up to the organ donation register and tell their loved ones their wishes as a result of this huge ITV From the Heart campaign. The last figures I read were that 100,000 people have signed up since the beginning of the week!

I must also urge all of you to visit Rhod and Caroline's 'Team End of my Tether' fundraising page! These amazing people are running the Reading half marathon to raise money for the CF Trust, so any donation will be gratefully received!

I am still hoping that my call for my double lung transplant will come very soon. It just makes me sad that all this fighting and effort to beat this exacerbation is basically what my life is now. Just treading the water until I get that call. But I will do it with a smile on my face and as much positivity as I can muster!

Time to Press Reset...

One week on and nothing much has really changed (unfortunately). It has been necessary to play the role of the 'patient patient'. Usually I do not play the role well, but as I have been feeling a rubbish-y it has been a bit easier! It is really difficult to put into words my hospitalisation and recovery. As I have said in many previous blogs my admission is not going to make me 'better' per se. The main aim is control my infection, as I am never going to be infection free due to the nature of my lungs, but the level of infection can be dramatically reduced and managed. Therefore the aim of my treatment is to reduce infection markers (CRP provides an indication of the magnitude of infection, whereas white blood cell count is always elevated as I am always colonised with Pseudomonas) and to increase my lung function and other clinical observations.

Unfortunately my lung function is being uncooperative and is currently about 13% despite two and a half weeks of IVs. My sputum is also still being really stubborn, thick and an effort to move. This means I have been attached to IV fluids 24 hours a day to help keep it moving and much easier to cough up and spit out. Another issue which has (unsurprisingly) reared it's ugly head is my chest pain. I suffer from chronic chest pain due to the constant inflammation and infection, but when I have an exacerbated infection the pain obviously increases, not particularly helped by super intense physiotherapy! Luke says that I am a different person when I am in pain - drained of energy, irritable, very tired and it affects my sleeping, eating and obviously my breathing. Everything is a constant effort, even taking a simple breath. Hence my increased need for the non-invasive ventilator. It also knocks my confidence a bit. So the doctors have increased my background morphine dose as well as my 'breakthrough' doses. They have also said that if I want to I can go back on a continuous subcutaneous morphine pump to ensure the pain is continually managed. I am a bit reluctant to do this, but the higher dose of oral morphine is making me a bit drowsy and nauseated, so perhaps it may be the best option just for the short term. Another little niggle is that my oxygen saturations are playing around a bit in the early hours of the morning, sitting at around 87% to 91% despite being on the ventilator... Hoping that this will get better once the infection subsides further!

As my infection markers have reduced a little, but nothing else has really improved, the doctors have decided to completely change all my IVs. So now I am back on Meropenum and Colomycin but they have also added Chloramphenicol (which is four times a day, boo!). They have stopped the IV anti-fungal and 24 hour Ceftazidime (one less 24 hour appendage, yay!). This means i can also restart nebulising Tobramycin which I've always found effective. In essence we have hit the 'reset' button so I am assuming I will be in here for another two ish weeks to see what this cocktail does to me!

We are playing a long game here and the main thing is that I remain fit for transplant. Harefield are fully aware of my current condition and who knows, perhaps I may get a call whilst in the Brompton! I'm still living with that hope of receiving that life changing and saving phone call. My whole family are.

Oxygen tubing and fluids

Fluid drip

Ceftazadime infusion... no longer!

An Update From the Brompton

Lukey and Me!

This last week has been pretty difficult. Since being in hospital my lung function has actually declined a bit, but that can be explained by many different factors, which I hope will be reduced when the infection begins to subside further. At the beginning of this week the Meropenum intravenous antibiotic (IV) has been replaced with a continuous Ceftazidime infusion, which delivers the drug constantly. This is to basically keep the bacteria-killing levels high throughout the day, rather than it peaking and troughing with having the dose three times a day. I'm still on tobramycin and ciprofloxacin IVs as well as IV caspofungin (antifungal).

All of these IVs are wreaking havoc with my electrolyte levels, so have to have regular magnesium and potassium infusions to replace what has been depleted by the medication. I also need to have quite a lot of extra fluid to keep my sputum lose enough to cough up with the least amount of effort. This means I am attached to three different drips all day and night.

Sorry if you're squeemish, sub-cut terbutiline

As I have still been quite tight chested, I am also having terbutaline (a drug which opens up the airways) subcutaneously (a little needle underneath the skin of my stomach). However this could cause me to become more tachycardic (fast heart beat) as I'm quite tachycardic naturally, so that's yet another thing to keep an eye on!

I'm relying quite heavily on my non-invasive ventilator (NIV) as you can imagine, with my infection markers being high and so on. It's a bit disheartening to be so reliant on so much machinery, but at the same time I feel incredibly blessed to have such expert help available, to help keep me as fit as possible for transplant. I am feeling very frustrated that although some of my infection markers have dropped a bit (CRP is now 145) they are still far from ideal. Mornings are also so hard for me, as my chest just feels so tight and heavy with sputum. It basically takes forever for it to wake up. However, I must not let myself slip into the spiral of self pity and negativity, otherwise things are ten times as hard to face! I keep praying and hoping that my transplant call will come soon and although it's going to be another hard road to travel, it's a lot more hopeful than the one I'm on at the moment.

Me and my wires!

I did have a very exciting start to the week! ITV came in and filmed me as part of an organ donation campaign they are going to be running in February. I was very pleased to be able to do it, I felt incredibly unwell on the day of filming, so I don't think I looked my best at all! However, vanity aside, I really hope and pray that the message about organ donation reaches many during that week and people make the decision to sign up to the organ donation register!

Please remember to visit Rhod and Caroline's CF fund raising page!! Any donation gratefully received for a good cause!