long catch up! with pics!

Edit: So this post looks ok on Facebook but for those that didn't know every highlighted word has a link or picture attached to it :-)
So I'll start my catch up with lunch with Holly. Every get together from holidays to lunch I think we have ever arranged has fallen through because I've not been well enough. Well on April 8th I was well enough and we met, mums and all at TGI Friday's. A place I've been to before and knew Holly would love. We had a lovely lunch and the food didn't disappoint :) I gave Holly a little gift I'd bought a while back on the late Jessica Wales' birthday. TMD had a significant meaning to Holly and Jessica, Holly has TMD tattooed on her foot and I think it was also played at Jess's funeral. How strange it was that I just happened to see the gift on Jessica's birthday, I knew I had to get it and knew instantly who to give it too, I almost felt like Jess was trying to tell me something!

Myself and Holly at TGI Friday's
Next I was at the Newstart gathering. That was on April 10th it was the Newstart 'Christmas' party, held on a warm sunny day at the marriott hotel just beside the airport. It was so nice to be able to go, my first get together since being home with my fellow transplant patients that are now friends. We all sat around large tables and just chatted, there was around 150 people (patients and relatives) there and apart from myself a couple of people in wheelchairs and also another on oxygen you would just never know what some of us in the room had been through to get where we are today, to be alive and well. One guy on our table was 20yrs post heart transplant, just amazing! I got a picture with my special man :) and collected my transplant lapel, a small pin with the Newstart logo in gold and silver awarded exclusively to recipients with pride. I felt proud to be alive and a recipient that day.My transplant lapel
On April 15th my brave and foolish (!) little (17yrs old) cousin brought the newest addition to our family into the world. Bailey (how beautiful is that name?) was born after an overnight labour weighing 6lb 13oz. I'm very close to my cousins, I think because my mum is so close to her brothers and sisters and I'm an only child. So when we heard he had arrived I nagged mum to take me out so we could spoil him :) I sent a photocard to my 36yr old auntie congratulating her on becoming a nanna and one to my nanna to congratulate her on becoming a great nanna. My auntie is the youngest in her line of brothers and sisters and so her becoming the youngest nanna is an oddity! But there you go 'anything but ordinary!'Bailey
It leaves me feeling somewhat out of place, I'm the eldest female of the next generation in the family and I feel like I should be the first or one of the first having the children, not my younger cousins. But then I guess that's just not the way my life has mapped out.
Obviously when Bailey was born the rest of the family were keen to meet him so one of my auntie's came over to stay, most of my family live in North Wales. She got to us late afternoon and we ordered in Chinese and we settled down in front of the TV to watch Britain's Got Talent, glass of wine in hand (not me though mind!) A couple of the neighbours came over, it was Manchester Derby Day and so the drink was flowing nicely. Towards 11pm mum jumped up and said "omg your IVs!" She had premixed them and they were all ready to go I just needed attaching. Mum put on her gloves and went to flush the line and it wouldn't flush... She tried and tried but it just wasn't going so instead we tried drawing back on it. Usually my port has a good backflow, blood comes out of it very easily. Not that night, it wasn't budging, I got about 2mls of blood then just air. We phoned the ward and they advised us to go to A&E. They phoned the duty manager ahead due to the complexity of my case and advised us to go straight to triage when we got to A&E and a chest doctor would have a look at the port. I was reluctant to try flushing it again due to the air in the line but knew if there was blood in the port by the morning it would have clotted and so I'd have lost the port and would need a new one putting in. Everyone in the house par myself had had a drink! Awesome, any other night of the week (and most weekends) they'd all have been sobber! By this time I was tired and felt like I'd spoiled the evening for everyone, I started to get upset by the whole situation. My heartrate was already racing from my late evening nebulisers and as it started to race more with me getting upset my hands and eyes started to feel a bit floppy. I've giggled at it before when I've read it on the back of an 'directions for use' label on the back of an epipen label but an 'impending feeling of doom' was one I've experienced before (I've had anaphylaxis before) was the best way to describe it and I barked at mum to get me an ambulance. By the time she'd got through to ambulance control and my auntie had reassured me and helped me concentrate on just breathing I was fine. I was infact about to slip into one big panic attack. Pre transplant I never had panic attacks, just recently I've had one big one and get feelings I manage to breathe through at least once a day since I got home. Something that could do with better control I think, anyway if not for my auntie at that moment I think I would've had one big panic attack. I love my family.Myself and auntie K
Anyway by the time a sober family friend had been found to take me to A&E it was 11.45pm. To cut a very long story short the chest doctor managed to get hold of one of the night nurse practitioners (NNP) that knew me well and one flush with heparin, an anticoagulant was all it needed. The NNP gave me a vile of heparin to hep-lock the port overnight. I got home at 3am, tired, stressed and emotional. What a night.
On the health front I had clinic today. I've just felt generally run down breathing wise since getting home, OK in myself just not as good as I was in hospital. I raised the question recently over my antifungal treatment. Out of three bugs I've grown since transplant two of those we're fungus. My antifungal treatment was until I left hospital Caspofungin IV because I grew a simple Candida in my lungs whilst in hospital. I'm unable to tolerate the other commonly used conazole drugs (Itraconazole and Voriconazole) due to the side affects so when I went home my antifungal cover stopped. But there is another drug on the market now that's becoming more commonly used, Posaconazole. It's said to be well tolerated and is easier on the body than the other conazoles. Today my consultant decided going on that would be a good option, he sent an email to, I'm going to call him the 'bug man' because off the top of my head I can't think of his actual title! But he's the guy that has to give the ok to start this drug, in the mean time and since I've been feeling a bit off he restarted my Caspofungin IV to see me through to the cross over to Posaconazole.
Well I hope your still with me after that long and varied catch up! I'll leave you with a snap of me enjoying the late afternoon sun in the garden the other day.

:-)

Doesn't everything seem so much easier when the sun's out and its warm? This temperature, around 12'c-15'c suits me perfectly at the moment, not too hot and not too cold.
My life really is a full time job of planning at the moment. Everywhere we want to go takes a day to plan in advance. I made it out to the Trafford Centre for I think a well earned shopping trip the other day. I have inherited a mobility scooter from my grandad who passed away recently and I have to say it's a gem. I despised them pre transplant and refused to use one, I have no idea why, I just didn't like the stigma attached to them. They seemed to make me feel 'more disabled' yet I was happy to sit in my wheelchair? The mind boggles!
We planned and measured the night before and managed to get a large 2ft oxygen cylinder (weighing 15kg!) sat between my legs and my liquid oxygen cylinders on the arms of the scooter which gave us more than enough time. I had teething trouble with the accelerator getting caught on the cylinder (scary moment) and managed to take out a rack of scarves in Accessorize (embarrassing moment) but had a great day. We also had lunch out, something that a few months ago would have been a challenge for me. I still require highflow oxygen so eating is still a challenge but I've managed to solve this myself by purchasing a Biflow mask. (I must buy more of them now because otherwise I won't be able to eat!) But my nausea since the transplant has been the main problem, I'm PEG tube fed (or at least topped up) because at one point it was so bad I was retching up NG tubes and kept very little diet wise down. Since going on a subcutaneous Nozinan infusion it feels like a switch has been flicked, I feel 'normal' after eating and the thought of going out to eat doesn't scare me. The difference has been amazing and as long as the pump doesn't leak I'm rarely sick now.
I feel so much more positive being at home and although at times it is far from easy, I can say the lungs are damaged and my god there are moments I can tell (by that I mean at times the symptoms are very noticeable!) it's worth the hard work just to be around the people I should be and be in the place I should be in.
I saw the team (or one of the doctors anyway) on the ward on Monday for a review. At the weekend I contacted the ward after 'not feeling right' on Friday night. I couldn't say exactly, just I was panting more and my heartrate was raised. I feared my CRP (marker for infection) had risen again and I wanted bloods doing to be safe. After some persuasion it was agreed I could go to the ward and have bloods done from my port. Come Monday we had the results, of course I was right, my CRP had risen only slightly but enough to warrant an IV change. So now I'm on IV Tazocin and feel better already for the swap.

There's always hope...

Wow when people tell you transplant is a rollercoaster, they really aren't lying. The last 2wks have been a crazy ride of emotions. Today in particular.
In my last blog I talked about clinic and going back a few days later to see if the increased doses of the anti-rejections had made any difference. I did go back to clinic a few days later in a similar state, probably slightly worse off. It was decided there and then I needed a bronchoscopy to see what was going on. Seen as I was out of breath, on my doctor's request I agreed to have the bronch without any sedation (yes I'm crazy like that). He was hoping, as we all were, the problem would be something immediately obvious and fixable. Not long after my transplant my Anastomosis (complicated medical word for where the donor lung is joined to recipient tissue, in this case airway) in my right lung had narrowed. I had it surgically stretched and it made me feel better almost instantly. But this time it wasn't the case, the Anastomosis looked almost unchanged and I had a strong feeling that was the case anyway, something felt different. The only thing the bronch really showed was some sticky 'jelly like' mucus in the bases of both lungs. Not really a nasty, dirty colour like you'd see in infection but it was just there and didn't really seem like it was supposed to be. So the samples were sent off and the only thing we could really do was cross our fingers (as strange as that sounds) that something grew from it because if nothing did there was nothing more to be done.
Two days later following a temperature, a very unhappy tummy and a junky cough my mum called the ward and I was readmitted. At first it didn't seem obvious, even to me what exactly was wrong, I just didn't feel 'right'. The team looked into every possible test whilst keeping me comfortable and hydrated, they were still waiting for the cultures from the bronch. Then after the weekend the results came back, they had found a fungus in my lungs called Aspergillus. I had heard of Aspergillus, it was something I was tested for regularly with my old lungs because of my symptoms but I'd never grown it. I've been on treatment for it (Amphotericin nebulisers, Itraconazole and Voriconazole (Vfend) prophylactically, since my transplant to avoid catching it but Aspergillus like most bugs is changing all the time, becoming clever and immune to certain drugs and it seems that's maybe what's happened. Given how breathless I was, how I'd already been on treatment and how I wasn't able to tolerate Vfend which would have been a good opition to switch back to it was decided I was to start on IV AmBisome (basically the same as Amphotericin but alot kinder to the kidneys, something very important post transplant as the kidneys get a battering from all the drugs). AmBisome is a good drug but it will take it's time to work and I'll be on it IV for a minimum of 6 weeks, thank god I got my port-a-cath when I did. It's not so much having a fungal infection in the lungs that's the biggest issue, my CRP (marker for active infection) is less than 1 the Aspergillus acts as more of an irritant making the lungs inflammed and generally a bit unhappy. (see ABPA for more info).
The big question now is obviously will I get loads better? Now this has been found and I've started treatment? My team are an excellent team and they're doing everything they possibly can and I believe that. They're good though at times at painting a rosy picture, telling patients what they want to hear to keep them positive and happy they do this because in transplant a positive attitude is at times half the battle. I like to know what I'm facing so I can work out how best to deal with it, the team have learned now that's how I am and they have been very straight, and, well, a bit blunt. In a nutshell they're 'reasonably' confident they can improve me overall by 5-10% with the AmBisome and fingers crossed getting rid of the Aspergillus. Back to my pre-admission self. But will not give me false hope and say I'm going to get much better than that and cannot say what the future will hold. They aren't confident I will come off oxygen again (at the moment I'm on 8-10LPM) and I may still find moving around just as difficult as it was. And I think I need to believe that, I cannot afford to set myself up for anymore disappointment, I know doctors have been wrong in the past and hell at times I've gone out of my way to prove them wrong but I'm going to believe what I'm being told, any improvement beyond what is expected will be a bonus. The thing that sprang to my mind was was it worth it? Possibly months of IVs for a 5-10% improvement? IVs are alot of work, in hospital they just get made up in pharmacy and administered, at home the chances are that won't happen (mainly because of the nature of the drug) and we will have to mix them at home. On top of that everything has to be sterile because the port line and drug are being given directly into my bloodstream (ports can be a major source of infection) and we will have to be trained on an IV pump because the AmBisome has to be given over 2hrs to avoid any reactions. But I did not battle to get these lungs and come this far with them to settle for anything other than the best I can be.
ANY improvement will be worth it and so I will do the IVs.
My doctor's words will stay with me "Your one tough cookie, you've proved us wrong in the past and I wish we were having a more optimistic conversation but there's always hope..."
Indeed there is.

are you ok?

I've had so many people ask recently how I'm doing and besides "ok I got a port put in today and I'm to have a peg soon" I haven't really been able to explain how I'm actually doing. I guess the best part of all of what I'm about to say is I'm doing better than I was 5wks ago. Before I went into hospital I didn't even have the breath to stand up at the sink for long enough to brush my teeth and wash my face, the day I got admitted mum had to put a stool in front of the sink for me. I was bad, I just hadn't realised how bad.
Now I'm going to try and explain what's going on, as best I can anyway. A few weeks ago I had a lung biopsy to confirm if it was rejection, I didn't think it could be anything else but the biopsy was negative, NO rejection. The biopsy (and CT scans) showed inflammation and scarring or fibrosis. Basically something has injured my lungs (we aren't 100% sure what and we may never know now) and it caused inflammation which in turn caused permanent scarring. This isn't going to go away, nothing can be done for fibrosis. The hope is now that whatever caused the injury was acute and it isn't something that is going to continue injuring the lungs. The problem I'm left with is my lungs are once again scarred, they're never going to be normal, scarring doesn't go away. All I can do is build up my exercise tolerance as much as possible to maximise what I can do with them. I'm still on oxygen, I don't know when or if I'll come off it.
I guess what I'm focusing on through all this is this isn't ideal, far from it but it's better than the situation I was in pre transplant. I feel better than I did pre transplant, I have my days but everyone does, my life has already been extended as far as I'm concerned it's already been proved that transplant was the right decision. I don't know what my life is going to be like from here on out but I don't really care because I'm alive and that's all that really matters...

Back in

Well I've been feeling a bit crappy for a couple of weeks. I started to notice a gradual dip in my exercise tollerance, an increase in breathlessness and lack of appetitte plus my cheeks were constantly glowing! Related or not I don't know!
I had a CT scan last week and I'm quite glad it came along on a day I was feeling really quite bad as it reflected how I was feeling in the following clinic appointment when I got the results. It showed I had a small pneumothorax (collapsed lung) in my pesky right lung, it seems to be the mischievous one! And 'scraggy' white patches which looked like inflammation over both lungs. My consultant said he'd seen alot of this before and although there was more than he'd have liked to seen he wasn't too worried but did want to get to the bottom of what they were, for sure, asap. A bronchoscopy and biopsy were planned for the following Thursday, just 2 days later. Wednesday saw me lounging in pjs all day with no energy or breath to do anything, I was back on oxygen and I couldn't even stand up long enough at the sink to wash. I had a few teary moments, out of frustration, feeling helpless and the worry that this is just how things were going to be, that this was it. Everything was an effort and poor mum was waiting on me hand and foot, I felt so guilty. I turned up for the biopsy yesterday and my consultant (different one to Tuesday) was weary. I admit I was worried about how I'd cope with the bronchoscopy as I was feeling really quite bad but I wanted it done so they could get to the bottom of it and I could get back to feeling better. After a chat and a check of my oxygen levels it was decided I wasn't well enough and it was cancelled. They sorted a bed out on the ward and I was sent up. I'm being treated for a bout of rejection, my second bout now (I had a bout during recent pneumonia) so they're looking at changing around my antirejection medication to prevent further bouts. Rejection is common during the first year of transplant, its takes a while for you to adjust to the treatment. But I find it so worrying, rejection brings back all the memories of my old lungs and my struggles I had with them, the struggles I don't want to be reminded of.
Anyway now the treatment has started I'm already starting to feel a bit brighter and hoping not to be in too long.

Front page News...

I Know I/we said this blog would be the thoughts and journey of a transplant couple and believe me in time it will be but for now it's other peoples thoughts... Rachael made front page news today. Below is the article but you can see it for yourself here:

‘Help me before it’s too late’

by Adam Derbyshire (For the Tameside Advertiser)
15/10/2008

A TEENAGER whose crippling lung disease was triggered by the family’s pet cockatiel desperately needs a transplant to survive.

Rachael Wakefield, 20, is fighting for her life in Willow Wood Hospice and admits time is running out.

Specialists in the UK have refused to offer her the lung transplant she desperately requires, telling her she doesn’t "fit the criteria".

Rachael was struck down by the mysterious disease at 13.

She said: "I am dying — this is the last roll of the dice. My only other alternative is a wooden box. I’m on morphine and oxygen round-the-clock. Every single breath is a struggle."

The family needs to raise £400,000 to pay for a transplant at the world’s leading lung clinic the Duke University in North Carolina, America.

Rachael, Dukinfield, Cheshire, underwent years of tests but her illness continued to baffle doctors who diagnosed everything from childhood asthma to pulmonary fibrosis.

Two years ago, it emerged the culprit was cockatiel Penni who had lived in the family home since Rachael was eight.

Specialists at a top London children’s hospital told Rachael she has hypersensitivity pneumonitis, more commonly known as ‘bird fancier’s lung’.

Dust from the bird’s droppings wafts into the air like an aerosol. It is harmless to most people but can trigger chronic breathing difficulties in a minority of people.

Throughout her teenage years, Rachael was hooked up to oxygen 24-hours-a-day. While her pals enjoyed nights out she was "trapped in the body of a pensioner" — a virtual prisoner in her own home.

Regular stays in Hospital led to her contracting MRSA twice. And last November she "died" during surgery and had to be revived after antibiotics were given to her too quickly.

She even underwent a course of chemotherapy in January in a bid to "wipe clean" her immune system.

But she deteriorated in August and after yet another spell in Hospital she was transferred to the hospice three weeks ago.

Rachael added: "No one who can help me is willing to do so — I feel like I’ve been left. It is so rare they just don’t know how to treat it. But every day I’m getting worse. And if we don’t raise the money I will die."

Mum Lynnette, 41, said: "It’s heartbreaking. They have told us there is no shortage of organs at the institute in America. But we have to find the cash."

Rachael has set up a website where people can donate to her fund at www.breathingislife.com

Although Paypal is a quick, trusted and easy way to donate they're taking their fees. Out of a £1.00 donation they will take 37p. So cheques can now be made payable to 'Rachaels Future Hope' and sent to: 32 Rosehill Road, Ashton-Under-Lyne, Lancashire, OL6 8HR. A Seperate bank account has been opened for the transplant fund and account details will be published on the website ASAP.

I promise tomorrows blog will be a personal one!

Rachael

A desperate plea

I was going to blog tonight about how horrible I have felt most of the day and the local newspaper coming to take my story, then Katie, my best friend sent me something she had been working on whilst in hospital so I'll leave this blog to her:

Over 20 different medications, nebulisers, inhalers and other numerous aggressive treatments, 24hr oxygen, and non-invasive ventilation, is a scary prospect for anyone let alone a young 20 year old girl. This is the norm in the life of Rachael Wakefield, battling to keep herself breathing, living and enough oxygen in her body. This was her (pictured) only a few months ago, enjoying life as best as she could, not letting things get in her way. But over the last few months things have got much worse for Rachael very quickly, and very dramatically.

My best friend Rachael Wakefield has a rare interstitial lung disease that has unfortunately left her so unwell that she desperately needs a lung transplant in order to continue living. Over the years her lung disease has progressively got worse and her lung function is now less than 15% of what it should be. This is not a great for anyone, let alone a young 20 year old woman who has her whole life in front of her. She has battled for many years doing everything that she can to try and improve her condition; in the mean time she has raised the awareness for others with rare lung conditions. Providing families with a friendly understanding ear, sympathy and often much needed advice. Her latest mile stone is setting up a website; www.breathingislife.com, which is helping to further share the stories of sufferers and explain to others the trials and tribulations of living with a rare lung disease.

Unfortunately all medical treatments available for the treatment of her lung disease have been tried and regrettably have not worked, the only way for her to continue living is through this much needed lung transplant. Sadly, all options within the UK have been exhausted and there is nothing left for the doctors to even suggest except being able to refer her for receive this lifesaving treatment, a lung transplant, in America. In order for her to get to America to receive this much needed transplant she needs to raise as much money as possible. This is where YOU can help...

Therefore I am asking you from the bottom of my heart to donate money or anything you can to this special person so that she can get the treatment she desperately needs. She is my best friend, and watching her deteriorate so quickly over the last few months has broken my heart. I can’t stand by see her struggle to live anymore knowing that there is a hope of dramatically changing her life, through this transplant. Unfortunately I, her family and friends can not cover the costs for this treatment, and I find it heart wrenching to think that it is only money that will stop her from dying and save her life.

Please visit www.breathingislife.com to donate and also for further information on this unique and truly special girl who deserves a chance at life.

Many Thanks from a desperate best friend,

Katie Mason

I think that pretty much sums up this blog

Rach

P.S Happy Birthday Katie x