NHS Survival – reflections and suggestions.

As many Patient Leaders know I have been very frustrated by the lead up and launch of NHS Survival. From the first tweet my suspicions were raised but I had been keeping my own council whilst supporting others brave enough to question and challenge.

David Gilbert, raised many good points in his blog http://futurepatientblog.com/2015/08/20/nhs-survival-why-i-wont-be-joining-the-club-yet/ However, my view is slightly different so I feel it is time to share my thoughts on what has happened and what needs to happen next in the hope that they will further the debate and allow the campaign to move forward.

In my role with the CCG I am responsible for ensuring that patients and public are included in the commissioning of local services. This has made me very aware that this isn’t something that comes naturally to many NHS staff! In many cases they just don’t know how to do it, some have had experience of poor tick box involvement and just don’t believe it is worth the effort. So my concern with the launch was not just that there was no genuine patient engagement and collaboration in the start up, but that the main players in this campaign fall into the categories above and don’t already see that patient and public involvement brings value. I hope they will all reflect on this and consider how they include their own patients in this campaign and in their own service reviews in future.

I was glad to see the call for patients to get involved with NHS Survival, http://www.nhssurvival.org/points-of-view/22-nhs-survival/63-go-on-tell-us but, as many others have pointed out, there is still a lot to be learnt about how to admit you have made a mistake. The HSJ launched the first 50 top Patient Leader awards in July, and there are many Patient Groups and charities supporting patients who could have been approached, so there was absolutely no excuse. In the words of the wonderful @Betabetic an honest ‘we screwed up” would beat “dog ate my homework”.

Had there been wider involvement from the start I believe the mission statement would be very different, the tone is paternalistic “we are trained in this, we know best” in the way that many well-meaning clinicians display every day. I don’t believe many patients will get involved unless the mission is revisited so that it is representative of the vision of all core stakeholders – Patients, Politicians, Clinical & Non Clinical NHS Staff.

I am passionate about the NHS and want to see it available free at the point of need to anyone who needs it regardless of their status. However, it is time to look at what is really needed and how that should be staffed and funded, what services should be delivered and from where. The reviews must include every part of the service, including all back offices services which are frustratingly bureaucratic, cumbersome and do not support efficient, effective, long term planning. More effort should be focused on preventative measures and wellbeing and it’s time to move away from the negative and jargonistic language that excludes and alienates many people. I’m not sure if a Royal Commission will achieve this but if the membership is changed to include politicians, patients and the public this groups will have a better chance of making a difference and I wish them well and will offer support, and advice if asked, but like David I won’t be joining up just yet.

In the Vein . . . . . . . . I Hope!

I’d like to tell you about my experience of living with Hereditary Angioedema (HAE) and to explain some of the issues that I am facing in trying to be a responsible patient, engaged in my own care. I guess it’s an attempt to improve awareness of the condition whist trying to process my very mixed emotions as tomorrow ,1st August 2013, I’m having my first training session for self administration of Berinert (C1 esterase inhibitor, human) which I am hoping will give me greater control and a better quality of life, hence the title of this blog!

As I explained in my first blog HAE  is a rare condition; incidence 1/10,000 to 1/50,000 of the population, caused by a defect in the gene that controls a blood protein called C1 Inhibitor (C1 INH), part of a complex cascade of reactions that control the release of fluids from capillaries into the surrounding tissues, thereby causing oedema (swelling).  It is an inherited condition and if a parent has HAE, there is a 50 % chance they will pass it on to their children, and luckily I haven’t! Family members who have been tested and who do not have HAE will not pass the disease on to their children. However , HAE can also occur with no family history as a spontaneous gene mutation, I am one such mutation.  Attacks can start at any time from early childhood, in adolescence or later in life and the frequency of attacks can vary from once or twice a year to every few days in the most severely affected patients, with nearly every patient being slightly different. These swellings can affect many areas of the body and usually last from 3 to 5 days,  the types of attack are as follows;

Localised subcutaneous swelling – affecting hands, feet, limbs, genital areas and trunk.

ImageAbdominal angioedema – symptoms include severe abdominal pain, nausea, vomiting and diarrhoea and abdominal distension. Because of large shifts in body fluids, symptoms of physical shock may be present. Many patients have received incorrect diagnosis, treatment and surgery for abdominal attacks in the past.

Laryngeal oedema – Facial or lip swelling may progress to affect the larynx. If untreated swelling of the airway (larynx) may result in death from asphyxiation. Voice changes and difficulty in breathing are very serious symptoms. It is very important that swelling of the face and airway are treated as soon as symptoms occur

Don’t I look wonderful ! Facial swelling Sept 2011

ImageSome patients can identify warning symptoms (Prodomal symptoms) in the 24 hours before an attack. These symptoms can include extreme tiredness, a rash or tingling over the affected site

An example of a HAE rash from HAE UK website.

There are some things that are known to make a HAE patient more vulnerable to an attack of swelling. I don’t get the rash and rarely get any warning but in my case I know that hormones, stress & infection are my main triggers, and it’s usually a combination of things that trigger a major attack.  Some other known triggers are

  • Minor trauma e.g. repetitive daily activities when gardening, or in the gym.
  • H.pylori (a bacteria that can be present in the stomach).
  • Dental treatment – swelling typically occurs about 6 hours after treatment.
  • Intubation when having a general anaesthetic.
  • Surgery
  • Drugs – ACE inhibitors, oestrogen in the contraceptive pill or in hormone replacement therapy.

Like many other people suffering with rare long term conditions, I consider myself to be a patient expert, qualified by my own research and the daily experience of living with HAE. Whilst I try my best to stay positive and keep healthy, these attacks can come on from nothing to acute in a matter of minutes, which has a vast impact on the quality of my life.

My first attacks occurred when I was 3 years old which were mainly face, hands or feet swellings and continued randomly throughout my childhood.  I also had frequent stomach aches, diarrhoea and  vomiting but no one linked the symptoms , and all allergy testing drew a blank.  I felt I was just seen as a sickly child who often missed school. In my early teens when puberty hit I also had to deal with the break up of my parents marriage, and my condition worsened. I had frequent acute stomach attacks, often passing out with the pain.  I know I had a reputation of being an attention seeking neurotic, and remeber several occasions when I was told it was all in my head.  I was taken to many different hospitals & clinics for various tests and was finally diagnosed in July 1976,  aged 15, by the wonderful, and very caring, Immunologist Dr Rita Brown at The Royal Berkshire Hospital (RBH) in Reading .  It was a massive relief, although back then the conditions was referred to as Hereditary Angioneuroticoedema (HANO), which didn’t help with many health care professionals attitude!

I was started on the normal prophylactic treatment which was, and still is, Tranexamic Acid tablets daily.  After years of taking these regularly, playing with the dosage and keeping diaries I don’t think it makes any difference to the number or severity of my attacks. Alternative treatments are steroid based Danazol or Stanozolol  but again they have had limited efficacy and although I did try them in my twenties I had a significant number of side effects when taking them for any length of time ( I turn into something like a Tazmanian Devil as my poor long suffering husband can confirm).

From diagnosis through to my early 20s I had regular attacks that co-incided with my menstrual cycle. The pain was so acute that I was hospitalised several times a year, often via ambulance, and usually ended up spending at least 3 days in hospital,  far more if the attack happened on a Friday or over the weekend , with my longest hospital stay being 9 days!  Back then the treatment for acute attacks was  Fresh Frozen Plasma and pain relief.  Things did settle down a bit in my late 20’s until my pregnancy , then everything went very wrong for me.  I was admitted to hospital several times up to 20 weeks and then stayed in hospital permanently for the last 4 months, but that is a story for another blog!

After having my daughter, in May 1992 , my attacks improved for about 5 years . Up to this point I had been treated locally at RBH, but when Dr Brown retired  they closed the immunology department and I was transferred to the John Radcliffe Hospital in Oxford, a 45 minute trip each way on a good day!

It was around this time that Berinert was prescribed to me as a named patient for the first time.  I still had to present at RBH A & E and wait for the drug to be authorised,  usually after a phone call to an immunologist at JRH. Once prescribed it had to be  found from Pharmacy, or on one occassion another hospital, and administered.  However, once I was given a dose I was usually out of hospital within 2 days possibly 3. This was a massive improvement and things improved even further when I was allowed to keep my own supply at home,  enabling quick administration as soon as I arrived at A & E,  though this did depend completely on the doctor I saw on admission.  It is only prescribed for use in acute abdominal attacks or those above the neck,  as it is a very expensive treatment (my current dosage is 3 vials at a total cost of approx £2,800)  and I occasionally need two doses to get an acute attack under control. However all the research, anecdotal evidence from fellow sufferers and my own experience shows that early administration improves efficacy, reduces the need for a second dose and significantly reduces length of an attack.

Attacks can build slowly or can come on in a matter of minutes so over the next ten years I got more practice at knowing when to take myself to hospital. It is a very personal decision and involves balancing the need for quick, in many cases life saving treatment against all the other considerations of presenting at A & E with a rare condition. i.e. Travelling to hospital  25 minutes away in extreme pain, will the flag still be on my record or will it have disappeared meaning delays before being triaged and the increased uncertainty of how quickly I might get treated, the understandable but very frustrating ” lets just rule out other pain causes” route taken by A & E staff with no experience of HAE , the vagueries of night & weekend shift staffing, the risk of being admitted to avoid overstay of the 4 hour target , the awful feelings of guilt that I’m adding pressure to already overstretched staff.

My husband has by necessity also become an expert !  He is able to explain the condition very calmly, can advise how best to mix up the drug so it dissolves fully, ready for injection.  He has saved me from many delays by firmly pointing out that “yes he is sure this is an HAE attack, so  please just give the  C1 so we can avoid any risk of asphyxiation!”    I also built up a file of documents to take with me, first those provided by the now defunct PIA (Primary Immunodeficiency Association) then documentation developed by the JRH Immunology and more recently the wonderful patient association HAE UK.  However, even if  I don’t encounter any problems getting treated it is nearly always  a draining experience, not least as I have dreadful veins. After years of  blood tests, canulas and injections they are very scarred and it often takes two attempts for the professionals to site a canula, as my veins just run away from the needle.   So for a few years I preferred to stay home and take opiate pain relief rather that face the added stress that a trip to A&E entails. However that all changed in 2009 when I had my first laryngeal oedma, requiring a very dramatic blue light ambulance ride from my GP, and since then I have had a 3 further emergency throat swellings. So now I always attend A & E for all swellings above my neck. My records show that I haven’t treated a stomach attack in the last three years, though I know I have had at least 2 major episodes each year.  Sometimes this means spending a day or two in bed but it also means that an attack usually takes longer to fully resolve , and untreated attacks can last for 5 to 7 days and subsequent localised/rebound swellings often occur.

Over the years I have missed work regularly, which has caused extra stress, and have lost count of the family & social occassions I have missed out on and the money I have lost in deposits or tickets never used! However,  over my lifetime the majority of my attacks have been mild, and for large periods of my life they have been infrequent, so I know that I am lucky. I didn’t fully appreciate this until  2011 when found the help and support of two wonderful women Ann Price & Rachel Annals who run the amazing patient association HAE UK (check out their website  http://www.haeuk.org).  I now get support from the website and from their secure Facebook page. In November 2011, at the age of 50 , I finally had my first meeting with another HAE sufferer, when I attended their patient conference in Manchester. I can honestly say it changed my life. To finally be able to discuss this very complex condition and know that someone understood was a massive weight off my shoulders. I was able to explain my concerns about self administration, which I had finally been offered this year due to the increase in my attacks, and it was through their advise and support that I have finally gained the confidence to accept the training. For the last month I have been very excited at the prospect, because it gives me real hope that I will gain a degree of control over the severity and length of my Hereditary Angioedema attacks, which should improve the quality of my life and I’ll be less reliant on the amazing NHS A&E services.   Whilst I don’t relish being my own personal pin cushion I have no fear of needles and I’m not worried about the thought of actually injecting a needle into my skin. However sitting here now I’m also quite scared as I’m deeply concerned about the emotional effects it will have if

a) the training brings on an attack.

b) I do pass the training –
i) how I will have to deal with the difficulties of how replacement of the Berinert will be managed across Trust boundaries ? There are already issues now when trying to get a replacement prescription as I leave A&E, which causes a strange and
unhealthy mix of frustration & self justification, with associated feelings of anger & guilt. I can see that this will be far
harder when I have to hold a bigger stock for repeat prescriptions, especially if I treat all acute attacks.
ii) will it change my relationship with my consultant if my usage does go up
dramatically?
iii) will I actually being able to repeat the process one handed whilst in pain? Not something that can be
replicated in training, knowing how darn difficult the professions find it with two hands!

c)   I don’t pass the training  – my disappointment and feelings of failure could exacerbate my condition in the short term and
I will have to continue to balance the need for treatment with the anguish of going to A & E.

These thoughts have been building up and causing me sleepless nights all week, but somehow typing them out has put them back into perspective. So instead I’m going to concentrate on all the positives

1) The cost savings at only having to attend A & E if I have a throat swelling.

2) The expected reduction in the number of acute attacks by promptly treating all acute attacks.

3) A reduction in the duration of attacks.

4) Regaining some control of my own health.

5) A massive improvement to my quality of life.

So I must finish off now and get this published so I can get a good nights sleep, and dream of nice juicy veins all eager and ready to provide perfect target practice. Wish me luck !

These shoes are made for walking . . . . .

Encouraged by  @anniecoops and @whoseshoes I had intended to begin blogging by sharing my delights and fears at the prospect of shortly being able to self-administer Berinert – C1 esterase inhibitor (C1-INH). This is the intravenous medication I take for acute attacks of Hereditary Angioedema (HAE). It’s a very rare long term condition (1/50,000 of population) that puts me in the Frequent Flyer category for many NHS services.  I value the NHS care & services highly, and normally receive excellent care but I also believe it means I am well placed to comment on my experiences of it’s weaknesses too. This was the main reason I took on my most recent challenge, and career change, and agreed to become Lay Member of Public & Patient Involvement for the newly formed Bracknell & Ascot CCG and this role will doubtless feature heavily in future blogs. . . .  However, when I looked back over my tweeting history I noticed that in addition to my main passions  –  singing, my daughter, the NHS – there are a large number of tweets about shoes! It made me realise that shoes have indeed been an enduring passion throughout my life and so I decided changed the topic of my first blog!

I adapted the title from the Nancy Sinatra song These Boots are Made for Walking, which I loved and sang along to in my youth . . . . . as it seemed particularly fitting because this weekend 20th/21st July, for the third year running , I hope to be taking part in the 24 hour Relay for Life at Ascot Racecourse . . . .  I say hope as with HAE I can never take anything for granted. Relay for Life is a wonderful community event that takes place over many weekends all over the country. It’s the lesser published sibling of Cancer Research UK’s  ‘Race for Life’ and sees teams of 6/8 or more people come together to walk (or run) round a track from 12.00 noon on the Saturday until the closing ceremony at same time on the Sunday. With the aim to raise as much money as possible for research into cancer treatments and cures. The Ascot Relay for Life is now in it’s 6th Year, held at wonderful setting of the Silver Ring of the famous Ascot Racecourse, and this weekend they are expecting to bring the total sum of money raised past the half a million pounds mark! Wellington boots have been the required footwear for the last two years  . . . . .  so this year I am hoping my sparkly Fit Flops will be up to the challenge.

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The event starts with a Survivors Lap, which provides great motivation for the 24 hour event, and the fact that so many count themselves as Survivors is very uplifting and a great comfort to many who are newly diagnosed. This is followed by a Teams Lap and then Relay continues with a member of each team walking the track at all times until the event ends. The only halt to proceedings is for the Candle of Hope Ceremony held at 10 pm, where decorated Candle Bags in support or in Memory of Cancer Patients line the track.  It is a very moving ceremony, which always makes me cry as I reflect on the loss of my Mother, the wonderful Zola Wilkinson, to Bowel & Liver Cancer over 21 years ago, aged just 48. The Candle of Hope bags then provide illumination and inspiration for the walkers who continue through the night.
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So going back to the shoe thread . . . . . I got my passion for shoes from my Mum, who was a prolific shoe shopper. It a pleasure we shared, and was passed on to her from my Grandmother, who had very dainty size 3 feet. As anyone with “normal” sized feet will know size 3 feet are always well catered for in sales racks and she did love a bargain. When she died she had over 200 pairs of shoes, most of which had been well worn even if it was only by the grandchildren for dressing up.  She also absolutely loved music and she played the piano, sometimes accompanying my Granddad who played the ukulele , and would burst into song at the drop of a hat, my Mother was the same so I guess it was natural that a love of music would pass on to me too. I have many great memories of singing with both of them as a child including a very dramatic rendition of ‘Hey Big Spender’ tottering around in oversized high heels! However the main thing I remember when I reflect on these two amazing role models is that they always made the best of every situation and showed caring, empathy & kindness to everyone! I only wish I had told them both that I valued these strengths even more than I loved their shoe collections!

My own shoe collection has changed dramatically with the fashions.  I did my growing up before the digital age so there is no photographic evidence of these fashion statements or mistakes, and I am glad and sad about that in equal measure. In the late 60’s & early 70’s I took great pre-teen pleasure in wearing white PVC pull up boots with bright red Hot Pants, promptly moving on to amazing 5 inch Platform shoes in late 70’s, which I thought compensated for my vertically challenged 4’10 ½ body! In the 80’s I delighted in every possible colour of bright neon court shoe to complement my shoulder pad filled wardrobe and I continued to seek out beautiful high heels of all designs & colours right through the 90’s. However the strength of this passion and quantity of shoe purchasing have changed considerably in the last 10 years thanks to my health.  Foot swellings are just one of many possible presentations faced by HAE patients, not something that affected me much in my youth but far more prevalent now, so as a result my current shoe collection is now clearly divided into three

1) Comfortable shoes that won’t cripple me if my feet start to swell without warning.

In addition to me HAE, since I had knee surgery I can’t walk far in shoes with heels higher than 2inches so every day shoes now are largely of the sensible variety, a woe I have shared often on Twitter!

2 Shoes I loved and thought would be comfortable but aren’t !

Now called Restaurant Shoes, these aren’t comfortable to wear for any longer than two hours and are mainly for decoration purposes i.e. occasions where I’m confident I will be mainly sitting and that walking will only be door to car, car to restaurant table and same journey in reverse, possibly with a short period of standing in a bar, but if this period is prolonged for any reason I have even been known to face my dread of bar stools in social settings!

3) Beautiful shoes I can’t wear but can’t bear to be parted from

Very high heels that put pressure on the balls of my feet, an absolute no-no which can now cause swellings if worn for longer than 5 minutes. These are gathering dust in my shoe rack, kept in the vain hope that after Menopause my HAE swelling patterns will change and allow me to return to this guilty pleasure . . . . .  not sure who I’m kidding, wearing of high heels is a skill that takes years to master and pull off with elegance and is a skill that is unlikely to return as my knees and back are not really up to it anymore but I still can’t bear to part with them!

So I still scour the high street in search of my Holy Grail – to find a pair of killer heels that look like this

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and feel like this

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 M&S foot-gloves just don’t do it, so all you shoe-makers out there please take note . . . .  there is a vast untapped market out there! I know that I’m not the only person who has the desire to point to a pair of Jimmy Choo’s or Manolo Blahnik’s and say . . . . . These shoes really are made for walking  . . . . .   but until then I’ll put my best foot forward and keep searching ,  while getting enjoyment from high heels like these!

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