Wheels and High Heels

As my CF has progressed, the most noticeable impact it has had on my life is the way it limits my daily activities. My parents were very good at making sure my CF interfered with my life as little as possible whilst growing up. Of course, we were very compliant with treatments and so on, but they certainly didn’t wrap me in cotton wool! Although I often had IVs at home and lots of physiotherapy, I pretty much lived a normal life. Many people didn’t even realise that I had CF. However as the disease naturally progresses, things do change and one has to adapt to them. Since 2008, my relatively normal life became limited and restricted, almost overnight as the result of a devastating chest infection. Twenty three years of normal living was then reduced to a life of imprisonment – my mind and soul are still of an outgoing, scrappy lass, but my body just cannot match that. I still struggle with these feelings of incredible frustration from time to time. 

 As I discussed in my previous blog my lung function is, at best 18%. This means that not only am I tethered to oxygen tubing twenty four hours a day, I now have very limited exercise tolerance. In real terms this means I can just manage a flight of stairs and general pottering around the house and the garden. I didn’t always use to be like this. I used to be very active. I used to jog daily, walk to uni from my student digs (approximately 2 miles) hold down a busy teaching job, partake in additional evening activities and still have energy to go out socialising at the weekends. Now walking 100 metres feels like jogging for half an hour. I don’t necessarily get as sweaty, but it’s the equivalent in terms of how breathless I feel. My oxygen saturation levels (sats) also drop dramatically when I exercise, which puts more stress on my body and for prolonged periods of time is dangerous. To give an analogy, it’s a bit like being at a high altitude. My sats are already lower than average so when I exert myself they drop fairly quickly.  It’s a really difficult position to be in, as I need to do a certain amount of exercise as part of my daily physiotherapy, but I need to be careful to get the balance just right so I do not overdo things and exhaust myself. 

Over the years it’s become more and more apparent that breathlessness is really invading my life. Routine chores, including the vacuum cleaning, are now too strenuous to manage. Whilst I’m not the biggest fan of housework (who is?), it is frustrating to not be able to get on with such a mundane task! However, I am able to cope with the lighter housework, so I’m not off the hook entirely! Another humdrum task to succumb to my breathlessness is the weekly shop.  Walking around the supermarket would require me to make regular stops to sit down to get my breath back. Medically this is quite dangerous, but realistically this is a bit impractical and shopping in this manner would take decades! The doctors at the Brompton suggested investing in a wheelchair, so that I could keep living as normal a life as possible. I also need someone to push me at all times, as using a self propelled chair would defeat the object of not exerting oneself! Luke’s parents very kindly bought me a chair, which has enabled me to ‘get on’ as my family would say! 

The early days of being pushed around in my chair (or ‘Wheelie Chair’ as she’s been unimaginatively christened) were odd. The most striking oddity was getting used to talking to people’s mid-drifts unless I looked up. Being a petite lady I often have to look up anyway, but shrinking from 5 feet to 4 feet is quite noticeable! More oddities include, not being able to see over the counter of some shops as well as relinquishing control of my movement to a trustworthy (!?) driver.  Oh yes, and the comedy that ensues from negotiating the pavements and various surfaces of our fair towns and cities. Most places are accessible by wheelchair but I feel the term ‘wheelchair friendly’ is a bit of a euphemism in some circumstances! The smallest lip or slight step can be quite difficult to navigate. I have to say, I was somewhat taken aback by the different and often frustrating challenges posed by the terrain to wheelchair users. Cobbled streets, steep hills and gravel to name but a few pose some real logistical nightmares. I love visiting historical houses, but I seriously doubt that the architect of properties such as Highclere Castle had wheelchair users in mind! We discovered this when we couldn’t access the upstairs rooms! However, registered disabled persons do get discounted entrance prices. Now we know why! Having a wheelchair has really helped me to try to live a more normal life, during this time of waiting. Luke and Ian were kind enough to take me to Marwell Zoo the other day. I had a marvellous time. On the other hand, Ian and Luke were exhausted... Largely from having to push me around the entire park and up and down rather steep inclines! 

Onwards and upwards!

Shopping used to be a favourite past time of mine (I have a particular penchant for shoes!) however, needing a wheelchair to do any hardcore shopping has somewhat dampened my enthusiasm for the activity. Shopping for clothes has become a bit of nightmare, especially with a husband who doesn’t really understand the way women shop for clothes in tow. As I am of petite stature (and am picky), shopping for jeans has always been a challenge, but now it’s at a new level! Many ladies will know that when clothes shopping you often visit a range of shops and look at styles and maybe try a few pairs on... Then, inevitably some shops are revisited to ensure the best fit and of course the biggest bargain has been achieved. Needless to explain, poor Luke, tired from pushing me and loathing shopping, was not impressed when I bought jeans from the very first shop visited!

Shopping for food is usually less eventful, as supermarkets are incredibly wheelchair friendly. The wheelchair trolleys are a bit cumbersome but you do get used to them! However, we have often made the mistake of opting for a basket where a trolley really should have been chosen. It is on occasions like these I become a trolley myself, with a little head peeping over the top of the broccoli and bread! It is a bit disheartening to have to relinquish so much control to someone else whilst choosing something like your food. It has been difficult to let go of being able to have a proper rummage through the veggies and various items on the shelves. However, Mum usually takes me food shopping and she has a canny eye for a bargain, so it’s not all bad. I also have to remind myself that hopefully, this is only a temporary situation and it won’t be too long until I am able to rummage independently again. 

Occasionally I have what I like to dub ‘down days’. I don’t like to bathe in self-pity, as I feel that it is not a healthy state of mind to have. However, sometimes when I’m pushed around town and have my oxygen tubes stuffed up my nose I can’t help but feel a bit down. Many people gawp and stare, sometimes out of pity with sorrow written on their faces, others are just curious or even nosey. It’s a million miles from where I used to be and it’s difficult to get used to. I often feel robbed of an identity as a woman and a wife, when I have to rely on people to help with things that I should be doing myself. I miss holding my husband’s hand whilst we walk around our town centre. Often, when out with Mum I get mistaken for a child (I have always been young looking, which I may be thankful for in years to come!) and this really upsets me. I simply crave normality. Nevertheless, I must push forward and have faith that this is a temporary period. One day I will get my transplant and will be running about doing a million and one things again and I will be able to vacuum as much as I like! So for now, I will make the most of ‘wheelie chair’ and wear the most ridiculous heels as I can find without having to worry about walking in them!

Another Tether

Unfortunately I am now adorned with another tether, which I hope is temporary! After feeling a bit on the ill side for a couple of days, I rang the Brompton Hospital to get some oral antibiotics, as I want to jump on any infection as soon as possible. However, the antibiotics decided not to work and to make me sick instead (thank you very much!), so I had to go to the Royal Brompton Hospital as a day case and start a course of intravenous antibiotics (IVs for short). As I mentioned in my first blog, the sticky mucus in the lungs of CF sufferers is a breeding ground for bacteria. Most CF sufferers are permanently colonised with a type of bacteria, in my case this is Pseudomonas aeruginosa (or to give it it’s full title ‘a pain in the ar*e’). This can never be fully eradicated, but is managed using a regime of inhaled antibiotics. Over the years during infective episodes where the bugs become too much to handle, I am given IVs to kill the uprising infection. Over time it has become resistant to a large number of antibiotics, so only a handful of IVs will manage it. It’s been a fine balance throughout my life to manage the infection in my lungs. On one hand I’ll never be rid of all infection on the other we don’t want the bacteria to grow uncontrollably. It is these episodes of infection which have wrecked my lungs. This is why I need a double lung transplant, not only due to my horrendous lung function but if I only had one lung transplanted, infection would cross from my old lung to the new one. So I need a new full set. 

On arrival at the ward I had my observations taken and I blew a lung function test. The hospital generally look at 2 readings; FEV1 which is the amount of air you can force out in 1 second and FVC which is ‘forced vital capacity’ this gives a reading of the total volume of the lungs. The FEV1 is the most indicative of infection so it’s the result most often looked at, although the FVC is useful to know too. My result was abysmal... my FEV1 at the best of times in the last couple of years has been only 18%, so to blow half my average is not a laughing matter!  Fortunately my blood oxygen saturation levels had remained unchanged (93%), so the doctor agreed to let me go and start my IVs at home, obviously with the strict instructions to contact the hospital if I don’t begin to feel better in a couple of days. 

The nurse then came along to attach my ‘tether’ through which my IVs will be administered. I have a ‘Portacath’, which is a device situated under my skin at the top of my chest, just below the collar bone. It is, in essence a metal ‘port’ which connects a catheter to a vein. This is the second Portacath, which was implanted surgically many years ago. This is then accessed externally using a needle with a line attached, which dangles on the outside. As I have had so many lines and catheters inserted into the veins in my arms and hands over the years, they have pretty much given up the ghost and kind of collapse or generally kick up a fuss if the docs try to put a line in them. My Portacath makes delivering my IVs a lot easier. There was a lot of pinching and gripping involved finding the Portacath under my skin, which of course is painful. Then comes the ‘inserting of the needle’ bit. It is quite usual for many nurses to have multiple attempts at getting the needle into the target, which is a silicone disc in the face of the device the size of a five pence piece covered by my skin. I have to confess when I was (a lot!) younger I would bite nurses’ hands when they tried to ‘stab’ me. What a horrible child! 

I left the hospital with mixed feelings. Firstly I was really exhausted from the battery of tests and anxious as I felt so unwell. I wondered whether I’d made the right decision to come home, should I have opted to stay in hospital? Conversely, I was pleased to be going home! I was also incredibly disappointed, as we were meant to be going on holiday to Cornwall with our good friends James and Ruth in about a week’s time, which we aren’t going to be able to do now. However, I suppose it’s more important to get myself better. 

I must take this opportunity to point out that there would be absolutely no chance of me being allowed home if it weren’t for Luke, my Mum and the rest of my family. Being on home IVs is not easy – it requires 3 doses of antibiotics to be drawn up in a sterile environment at regular intervals throughout the day. On top of this, I need to eat an awful lot more to help my body fight the infection. This means eating 3 cooked meals a day. I also need more intense physiotherapy. Needless to point out, I am not really allowed to do too much myself, although once I begin to feel a bit better I’m sure I’ll be pottering around as usual! There’s no way to really describe the immense gratitude I feel for my family. Luke, who has had to extend his PhD by a couple of terms over the years (due to my hospitalisations and so on) and is currently writing his thesis, is such a devoted husband he just does everything without complaining or ever making me feel like a burden. Then there’s Mum, who throughout my life has cared for me so well that I was able to complete school, college and university and qualify as a teacher. Then there’s Ian, my brother who keeps me entertained in a plethora of ways, but most commonly, through multi-player online gaming on the Playstation 3 game ‘Call of Duty’! Dad works so hard to support the family and does so without complaining, he’s a real pillar for the family. Of course I must mention my Gran, who pops in every so often to keep me company and keep my mind from dwelling on the negatives. Although I may not have the team or doctors, nurses, physiotherapists, cooks, cleaners and many, many other staff members that I would at the hospital with the love and support of family and friends I can basically receive the same treatment I would in hospital in the comfort of my own home. 

I am so blessed in many ways. I know people reading this will think I’m mad to say this, but there are so many people in my situation who don’t have such an amazing support network and have to spend a large proportion of time in hospital. So, this blog is largely a big shout out to all those lovely people who spend a lot of time looking after me, cheering me up with chats over coffees, animated discussions about weddings and bridesmaid duties, pizza and DVD afternoons, wii and wine nights and people who are just there! I couldn’t do it without you! Again, I’ll leave you with another bible verse. Even when things aren’t looking so great, this verse helps me (many thanks to Fred for bringing this to us in church last week); 2 Corinthians 4 v16-18 ‘Therefore we do not lose heart. Though outwardly we are wasting away, yet inwardly we are renewed day by day. For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. So we fix our eyes not on what is seen, but what is unseen. For what is seen is temporary, but what is unseen is eternal.’  

The cat feels the need to rest too...

My Dress Rehearsal

Waiting for a transplant is a really odd situation to be in. The doctors and health care professionals urge you to live as full and as normal a life as possible. However, you do need to remember to have your mobile phone on you at all times – or at least be contactable, should ‘that’ call come. On some days life is fantastic and Cystic Fibrosis and being on the transplant waiting list is the furthest thing from my mind. Yet on other days it’s all I can think about. It is so easy to sit at home and become tearful, morose and upset. Pondering questions such as; will I ever get the call? How much more ill will I become before I get the call? People who have had transplants themselves and the team at Harefield and the Brompton say that when the call comes, it will be totally out of the blue. I have had one false alarm, which did indeed ‘come out of the blue’...

In fact it was on Tuesday 1^st March at 3.50am. Wrenched cruelly from a lovely sleep my darling husband Luke grunted at me ‘was that the phone?’ (As if I had made the call!) My response was something like ‘Shh... sleeping’. Then the phone rang again. We instantly knew it would be Harefield who else would ring at that hour? When Luke passed me the phone and the transplant coordinator explained that there was the possibility of a set of lungs becoming available my mind instantly turned into slush. We had planned what we would do when the call came. Our original plan was:

1. Ring my Mum and tell her we would be picking her up in about 10 minutes
2. Get washed and dressed quickly
3. Get my pre-packed overnight bag from under the stairs whilst Luke filled up my portable oxygen unit for the journey
4. Ring Luke’s parents to keep them in the loop and then leave, get Mum and head up to Harefield.

What actually happened was:

1. I burst into tears and stood in the middle of my bedroom for what seemed like an eternity.
2. Couldn’t decide what to wear so ended up getting previous day’s outfit from the washing basket
3. Cuddled my cat a lot as I didn’t know if this would be the last time I would see him for a while (if ever)
4. Forgot to wash and brush teeth.... Fortunately Luke was able to complete the rest of the plan without me, so although I was a dithering wreck, Luke kept us on track!

Picking Mum up at ridiculous o’clock in the morning was awful. I hugged my Dad and brother (Ian) wondering if this would be the last time I would see them. Poor Ian had been hastily woken and anyone who knows Ian will understand what a feat this!! I honestly cannot remember the 55 mile drive to Harefield Hospital in Uxbridge. I think Mum and Luke were chatting away but it could just as easily have been the disembodied voice of the SATNAV.

After my initial meltdown and detachment in the car I was surprisingly calm and collected. We arrived at Harefield and were taken up to a ward where we were met by the transplant coordinator. I had a lot of observations and blood tests taken, to check for infection levels to make sure that I could actually undergo the operation. As you are given immunosuppressant drugs prior to and after the transplant, it is important to ensure that infection levels aren’t too high. During this time, the potential donor was also having similar tests as well as tissue typing tests to ascertain who would be the best match for the organs. This leads me on to explain the fact that the hospital will always call two potential recipients for the transplant, to ensure that the organs actually get used. We were told that the results of the donor’s tissue typing test would decide which of us would potentially receive the lungs. (I must point out that the two potential recipients do not meet at any point!) I was also met by the on-call anaesthetist, complete with pillow crease embossed into her face. 10 minutes later the transplant coordinator was back to say that the tissue typing was not a match to the other recipient so I was ‘officially’ offered the lungs. It became very real very quickly at this point. I was whisked away for an x-ray, then had to sign THE consent form.

 The consent form signing represented another nervous moment. The rather impersonal, detached doctor who delivered the long list of risks and one sentence of benefits in this form may as well have been wearing a long black hooded cloak and have been carrying a scythe... The ‘cons’ which were pretty difficult to read through the tears included ‘death 10%, stroke 5%, risk of further surgery, acute rejection’ I have to confess, I didn’t read any more as I felt the worst had been read! The pro(s) list read ‘increased quality of life and expectancy’. Although the pro list was a lot ‘shorter’ the implications of those words far outweighed the lengthy ‘cons’. Luke and Mum quickly reminded me that it IS what I want – an improved quality of life would be amazing (life without a tether, hardly seems possible!!) I have to write here something that in hindsight had Luke and Mum in stitches, after reading the form I said something along the lines of ‘you aren’t going to give me s**t lungs, are you?’ to this rather aloof doctor. He was NOT impressed (he looked almost as if I had personally insulted his intelligence!) 

I was quickly washed down with surgical scrub and put into a rather sexy gown and taken down to the theatre waiting area on a trolley. The transplant coordinator was in constant communication with the donor hospital and gave us regular updates on the situation. At this point I was basically waiting to hear about the status of the donor’s lungs and whether they would be viable for the transplant. As soon as the go ahead was given I would be taken through to the preparation room and prepared for surgery (drugged, etc!!). It is hard to explain in one paragraph the guilt and associated feelings of basically having to wait for someone to die so that I can have a bit longer to live myself. (I’ll discuss this in another blog.) I met the surgeon and some of the team who would potentially be performing and involved with the procedure. Of course my mother thought the surgeon was ‘rather dishy’! The nurses in the waiting area were lovely and kept Luke and Mum in tea and biscuits. They had not eaten since dinner the night before and it was now 10 am. After two hours of waiting the donor lungs were unfortunately deemed unviable.  Devastatingly I had to get my belongings from the ward, and then go home. I feel I must stress at this point that the staff at Harefield Hospital are incredibly kind and caring and worked so hard to keep us constantly informed and put my family and I at ease wherever possible.

It is really hard to describe how I felt leaving the hospital. On one hand I was pleased to be going home and relieved not to have to go through a life threatening operation. On the other hand, the transplant is going to (hopefully) give me a better quality of life and increase the length of my life compared to that without a transplant. I felt so sad, not only for myself, but for the donor’s family who couldn’t donate the lungs which is obviously a wish they wanted to honour. I felt the pain that the other recipient felt at being sent home. I could also sense my family’s disappointment. It was now well past lunch time, so I consoled myself by eating a 20 piece chicken nugget meal... I really do not want to dwell on negative thoughts and emotions, but it was hard to overcome ‘the time that almost was’. I kept hoping that the next call would come again soon, but time does heal, no matter how corny that sounds. With the love and support of family and friends I picked myself up and am getting on with life. 

More than 10,000 people in the UK currently need a transplant. Of these around 3 a day will die waiting as there are not enough organs available. Many people with CF decide against having a transplant and opt instead for palliative care. Neither decision is reached easily; especially in your early twenties (not that age ever really plays a part in these decisions). I decided to go down the transplant route as I believe it gives me hope. It is an emotional roller coaster, but it also gives me something to hope for, I don't know when or if my call will come, but I stay strong in the hope that it will and try to live each day to the full! My faith keeps me strong and I particularly find the following bible passage comforting. Romans 5 v3-5 ‘...we rejoice in our sufferings, because we know that suffering produces perseverance, perseverance character; and character hope. And hope does not disappoint us...’ 

A cuddle with Oscar

The First One...

Keep Smiling!!

I do live life at the end of a tether – this tether being metres and metres of oxygen tubing! Although it does pose the question “Am I at the end of my tether?” On some occasions I would certainly say ‘YES!’ Waiting for a transplant (double lung in my case) is not exactly at the top of one’s ‘to do list’... People who know me well will also know that I’m not exactly the most patient person! I was really reluctant to write a blog, as it makes the situation seem more real and in all honesty, I was a bit embarrassed. This may sound incredibly odd, but when you are attached to an oxygen tank and you meet old acquaintances and even old friends, they are obviously a bit shocked to say the least! Then comes the awkward social dance... do we acknowledge the oxygen tubes or not? Or do we ask about it outright? Personally, I prefer the latter, as at least it gets the issue out in the open straight away! However, I have been cajoled into writing down my experiences and it is my hope that these will educate people on both the disease Cystic Fibrosis and about organ donation; even if only one person learns something new then it’s all worth it. 

I suppose I’d better start at the beginning! I was first diagnosed with CF at about 6 months. CF is a genetic condition so although I had it since birth, the doctors didn’t manage to identify what was wrong with me until then. After the diagnosis I was put on a regime of drugs and treatment which included about a million tablets (I of course jest, probably closer to 10 drugs), nebulisers, inhalers and daily physiotherapy. The aim of physio is to bash out the thick sticky mucus from my lungs, firstly so I could breathe easier and also to reduce the risk of infection. The most well known complication with CF is the thick mucus which is difficult to remove, especially from the lungs and leads to recurrent chest infections. These infections then damage the lungs and in essence transform healthy lung tissue into scar tissue which means my lungs don’t work effectively. The aforementioned sticky mucus also affects other organs including the liver, pancreas and CF causes many problems in the gastrointestinal tract. As a young child I was in and out of hospital fairly frequently, but more so when I reached about 10 years of age. I would have regular two weekly stints in hospital where I would receive intravenous antibiotics to get fight infections. I remember spending the whole of my summer holiday in hospital between primary and secondary school! My hospital is in London, so I remember mum having to take me down the King’s Road to John Lewis (the cheapest shop there!!) to buy my uniform for secondary school! At this point I also had a gastrostomy tube fitted. This was a feeding tube inserted directly into my stomach for overnight (liquid!) feeding as I found it so difficult to maintain a decent weight. Another facet to CF is the constant weight watching, and in the opposite sense to the norm. Currently, I have to aim for a 3000 calorie-a-day diet to maintain a BMI of 20 (normal)! Every cloud, eh?! 

I went through secondary school with little trouble – the odd spell in hospital here and there. I had my feeding tube removed as I was maintaining my weight well. When I was older I was also able to have my IVs at home as Mum had been trained to do them. My CF didn’t cause me too any problems during my later school years, I got 10 good GCSEs, did my A levels and then went onto university where I trained to be a teacher.  Miraculously, I got through uni without a single day off due to CF! I met some great people there who have become friends and who have been so supportive of me during this tough time. Oh yes... I must not forget to mention my husband. I met Luke back in August 2002 and we have been married for four years. 

I started my teaching career and had a great time and a school full of really supportive colleagues. I adored teaching and taught year 5 for 2 years. I was pretty ill at the end of my first year and was hospitalised during the summer holiday. (What is it with me and being in hospital over the summer?!?!) This was in all honesty a horrific experience as I had not been an inpatient for 10 years. I was miles from home, in deep dark London (Ok, Chelsea...) and I felt so scared and alone. I was on a non-invasive ventilator for the first week of this admission which was scary, but I got through it with the support of a very devoted husband, a loving family and some pretty amazing friends who trekked up to see me...  After that admission I had to have therapeutic oxygen at home which really depressed me, as it felt as though CF was beating me. I knew that a transplant was the only way that I was going to be able to breathe on my own again, but I had a tough time admitting it. Despite this, I returned to teach another year at school and remained pretty well throughout after about half a term’s worth of rehabilitation. However, at the beginning of my third year of teaching I got another chest infection. It was at this time the doctors at the Brompton said that I should really consider being assessed for a transplant. It was here I learned that even though my lungs are crap enough to mean that I need a transplant, you actually need to be assessed to see if you can even be listed!!!! So here began a rigorous series of tests to see how the rest of my body was behaving. I had to have about 20 different types of blood tests to get my blood group, tissue type and to check for viruses and various other health issues. I had a heart echo, ECG, liver and kidney ultrasounds, saw an ear nose and throat specialist and even had to see a dentist to make sure I was dentally fit for surgery!! Of course, being an awkward so and so, I needed my bottom two wisdom teeth removed! Boo hiss.

However, halfway through this assessment admission I had a ‘little setback’. One night Luke and I were watching the TV drama series ‘House’ and I just couldn’t breathe. I had been feeling a bit funny all day, but my observations had been ok... Luke ran to get a nurse who did my ‘obs’ and discovered that my oxygen saturation levels had plummeted and my heart was racing at a whopping 170 bpm! After emergency an ECG and X-ray there was no sign of a collapsed lung causing this episode, so I had an intense 3 hour physio session and then back onto the non-invasive ventilator I went (there were a whole host of underlying complications that caused this set back and many others that followed that I won’t go into at this point). Luckily, after a couple of weeks, I was able to breathe on my own (well, with my old friends the nasal specs) again and could go home. Whilst this was a terrible experience, it did make me really want a transplant. Before I was incredibly reluctant to admit I needed one but when the choice was almost taken away from me, I soon realised that I do want to beat CF and if getting a transplant will give me a few more years of quality life, I’m all for going for it! 

I am happy to say that I was deemed ‘fit’ for transplant and have been waiting for my double lung transplant since January 2010. I go to Harefield hospital (which is the transplant centre) every 6 months for a check up and I’m at the Brompton every 4 – 8 weeks for a check up there too. I have been stable for the 18 months I’ve been waiting. I live in hope that I will remain stable until I receive that call. The main way I remain sane is through my faith and I would like to end my first blog by sharing my favourite bible verse:

Psalm 23 v4 ‘Even though I walk through the valley of the shadow of death, I will fear no evil, for you are with me; your rod and your staff comfort me’.