Understanding Median Predicted Survival

This year’s UK CF Registry Report shows that the median predicted survival for people with cystic fibrosis is a bit lower this year. To explain this change, Dr Janet Allen, Director of Research and Care, explains what we mean by median predicted survival and how “wobbles” happen.

You will have noticed in the annual report for the Registry that the median predicted survival is 36.6 years and that this is lower than the 43.5 years reported last year.

First of all, it is important to understand that median predicted survival is not the same as life expectancy. Median predicted survival is calculated from the registry data and represents the age beyond which half of the people in the UK CF Registry today would be likely to live. The annual values for median predicted survival tends to wobble around from year to year as the calculation is sensitive to the number of deaths recorded in any one year and there is a tendency to over-interpret the yearly wobble. This year the value appears to have dropped because the number of deaths recorded rose to 146 cases (of over 10,330 people on the Registry, representing around 1% of the UK CF population). A similar downward blip was observed in 2009 when the median predicted survival was 34.4 and the deaths recorded at 141. Apart from these dips, the trend in median predicted survival continues to rise and it may be better to report the overall trend rather than the year to year variation.

By way of an analogy, it may help to think of a country’s economy.

Over the past 50 years the UK economy has greatly, and largely consistently, increased, and yet there will be patches where, for a number of reasons, it contracts.

However when it does this, it never goes back to the start. It’s a case of always taking several steps forward, but every now and then one or two back.

The median predicted survival for people with cystic fibrosis has done the same and increased over the past 50 years, and yet, as happened in 2009, last year there was a noticeable but not statistically significant decline, even though the trend remains upwards.

Remember that when the Trust was founded in 1964, 90% of people with cystic fibrosis died before the age of 10.

Life expectancy answers a different question; for a child born today how long could they be expected to live?

These are complex calculations and are not calculated every year. The last report for the UK was over 20 years ago when it was calculated that life expectancy for a newborn child with cystic fibrosis in 1991 was in the order of 40 years. It is now estimated to be over 50 years for a baby born today, and the Trust is exploring having these calculations done again to show how far we have come since 1964.

You can find the UK CF Registry Report over on the Trust’s website both in Summary Form and as a Full Data Report.

Keeping Cool With Cystic Fibrosis

As temperatures soar, Public Affairs Officer Lynsey Beswick presents her own guide to keeping cool with cystic fibrosis.

So the British summer has finally arrived. But with it the government has issued some important health warnings for those of us who may suffer with long-term health conditions.

So whilst I can confirm that I have been frantically routing to the back of my wardrobe to find my shortest shorts I have also been contemplating some of the advice and what it will mean for me and my cystic fibrosis.

Whilst the government have put together a heat wave plan I have been carefully considering my own CF-proof plan.   

Not only am I armed with inhalers (in every handbag I own) I also plan to ensure that I am extra careful about remembering to take my doses along with any other nebulisers or breathing medication.

We all know that the sun causes sweat – and people with cystic fibrosis love to sweat – so I have also been frantically digging out the salt tablets which are usually only reserved for holidays abroad; to avoid any heat-induced cramps which can be common for people with cystic fibrosis.

 

I have also started to take an antihistamine to act as an anti-inflammatory and to help ensure that any allergies are kept at bay as this can also play havoc with my chest and make me feel worse.

As well as ensuring I have the obvious – lots of fresh water and a good dollop of sun cream – there are some other things that might not be quite so obvious. For example certain antibiotics that I take for my cystic fibrosis can cause photosensitivity – this could mean the difference between a glorious tan and a slightly less glamorous shade of beetroot.

I will certainly be taking things at a slower pace and avoid over exerting myself – a great excuse to get out of going for my evening run – that will have to wait until the weather is cooler I’m afraid!

Instead I will be remaining inside during the hottest hours of the day with my feet up (hopefully), windows open and a fan flowing to keep me as cool and as comfortable as possible. I will also keep all medications in the shade too – particularly digestive enzymes as the heat can apparently damage them and make them ineffective. 

I also need to monitor my food intake – hot weather often means I lose my appetite so making sure I have snacks to hand or supplement drinks will help to ensure I’m still getting essential calories and that I keep my energy levels up.

Of course at the first sign of feeling unwell or breathing difficulty I do have my specialist CF team on speed dial – but I am hoping that by taking sufficient precautions I can avoid any drama and enjoy this great weather while it lasts!

As Lynsey suggests, if you do feel unwell it is important to contact your specialist CF team immediately.

Setting the Parliamentary Agenda on Transplants

James Barrow, Head of Public Affairs, talks about the success of yesterday's debate on organ donation and transplantation in Westminster Hall.

Yesterday’s parliamentary debate on transplantation was an important moment for our community.  Since the launch of our Hope for More report we have been campaigning to improve access to lung transplantation for people with cystic fibrosis. Some 4,500 supporters emailed politicians from across the UK, asking them to urge the Government to take action and our Public Affairs team has met with over 60 MPs, AMs, MLAs and MSPs to discuss the report. This activity triggered the debate in national transplant week.

During the debate MPs raised the use of extended criteria lungs, the ability of surgeons to downsize lungs and the importance of new technology in preserving and reconditioning lungs. Cystic fibrosis was mentioned no less than 20 times during the 90 minute debate.

NHS Blood and Transplant (NHSBT) is now reviewing its existing lung allocation policy and is modelling a national allocation system.  And the Minister for Public Health, Jane Ellison, has expressed concerned at the number of deaths on the waiting list. NHSBT is set to make an announcement on the outcome of its review in the autumn and we will continue to campaign hard on this issue.

Thank you to everyone who continues to support our campaign – your help has made a real difference.

And thank you to those MPs who took part in the debate: Jim Shannon MP, Margaret Ritchie MP, Caroline Nokes MP, Kerry McCarthy MP, Jason McCartney MP, Glyn Davies MP, Madeleine Moon MP, Kate Green MP , Luciana Berger MP, Jane Ellison MP.

If you missed the debate a full transcript is available here.

Seven Things You Learn on the Transplant Waiting List

Robyn has been on the waiting list for a lung transplant for nearly five months. She takes IV antibiotics to keep her as well as she can be, and sometimes has to be on oxygen for long periods of time. A single mum, Robyn is coping with the support of her wonderful daughters Sophie, 11, and Phoebe, 7. Here she shares seven things she has learned from life on the transplant list.

1) Make lasting memories

Last year my parents took us all to Disneyworld in Florida which was absolutely amazing. We wanted to ensure we had the holiday of a lifetime and a chance to build some everlasting memories in case we didn’t get that chance again.

2) You may want to keep the whole truth from the ones you love

The girls have always known that their mummy is not well but I keep the frightening statistic that one in three people with cystic fibrosis die on the lung transplant list hidden from them. It wouldn’t be fair and could damage what we have if they stopped acting normally around me.

3) Life will change

The one thing I miss the most is running around with the girls. We live in the country and up until I became this ill, I enjoyed a very outdoorsy lifestyle with our horses, dogs and long country walks.

4) It’s okay to ask for help

I really miss doing these activities with my girls and have had to hand over the reins to my long suffering parents who take them out all the time. They have been a brilliant support to me, especially since my husband and I are no longer together.

5) Improvise!

I do consider myself very luck, because my daughters are content when we curl up on the sofa and have movie nights instead. We do lots of indoor activities too, like baking, and they like to have their friends over for sleepovers.

6) Be prepared

I can’t travel further than two hours from the hospital, in case I get that life-changing phone call. My parents are on standby and when the time comes no matter what time of day it is my mum is ready to look after the kids and my dad is ready to drive me to the hospital.

7) Anything could happen

I have written the girls a letter each for when I go in to have my transplant, in case the operation goes wrong. I’ve also started to write letters for them to open further down the line. Things like special messages for all of their birthdays, their wedding day and even for when a boy might break their heart for the first time. I want to give them these letters so that when they read them they can feel close to me and know that I have planned ahead for them, but most importantly to let them feel my love when they will need it most.