Planning a Holiday Abroad? … Are You Fit to Fly?

Flying in an aeroplane is the equivalent to being 5000 to 6000 feet up a mountain.  Think of a mountain twice as high as Ben Nevis, or being a third of the way up Everest.  The air is ‘thinner’ because the pressure is less and, as a result, there is less oxygen.  In fact, the concentration of oxygen falls from 21% at sea level, to about 15%.  Most people will tolerate this, but, if you already have problems breathing, this will become worse.

For those respiratory patients who do not normally need oxygen: Patients who can walk 100 metres on the flat without needing oxygen, and at a steady pace without feeling short of breath, are very unlikely to have a problem in-flight.  These patients are considered safe to fly according to the guidelines issed by the Civil Aviation Authority.  If you can not do this, you should talk to your doctor about the safety of travelling by air.  They will be able to carry out studies where they simulate the oxygen concentration in the aeroplane cabin.  You will be sitting comfortably wearing a close fitting mask that delivers oxygen at a reduced concentration of 15%  They will see what happens to your oxygen saturations over a period of time.  They can then recommend whether or not you need oxygen during the flight.  Short haul flights are less hazardous than longer ones.

For those respiratory patients that already need oxygen: Talk to your doctor about how much oxygen you will need in-flight.  This may be a higher flow rate than you normally use.  You must also remember to arrange oxygen to be available at your holiday destination.  Your local respiratory nurse will be able to offer you advice on this.

For all those patients that need oxygen on the flight: Remember to let the airline and/or travel agent know in good time, ideally a month before you fly.  Some, but not all airlines, charge for the oxygen and it might be worth planning your flight with an airline thay does not charge.  Do not rely on the airline having oxygen avalable at the last minute; the oxygen the airlines carry is for emergencies and would not last very long.

Final checklist once oxygen is sorted … don’t forget:

  • Check your insurance policy covers you for medical costs and return home.  The British Lung Foundation has a list of insurance companies that will insure patients with lung diseases.
  • Bring your EHIC [European health insurance card] if travelling to the EEA or Switzerland.  Phone 08456 062030 or visit www.dh.gov.uk/travellers.
  • Arrange disabled assistance at the airport if required.
  • A letter in your hand luggage detailing your condition and medications.
  • Medication in your hand luggage plus one spare week’s worth.
  • And, most impotantly, don’t forget to have fun! 

 

Charity Bike Ride, 8th June 2014, Round-up

The sun was out, the picnics were plentiful, the sunbathing was rife …. oh yes and there was a charity bike ride going on …. in Richmond Park early this month.  Each year, we wonder if the rain will stay away; this year, we almost fried with the heat of the sun!

Almost 100 riders cycled seven miles around beautiful Richmond Park on Sunday 8th June 2014 in support of five UCLH Charity funds; Breathing Matters, Dementia Care, Leukaemia and Lymphoma Unit, Rays of Hope and TTP Research.

Cyclists included patients, relatives, doctors, researchers and families with children as young as four years old – along with our resident dog, Dexter.   Two of our regular cyclists ran the route this year instead, “I won’t be doing that again” stated Greg, before collapsing under a tree. 

Theresia, Jo, Donna and Manjiry

Theresia, Jo, Donna and Manjiry

Our Patron and Medical Director, Manjiry Tamhane and Dr Jo Porter, along with friends, formed a team called ‘Girls on a Mission’ for the challenge and smashed their fundraising target of £1000.  Here is Manjiry and Jo along with Donna Basire (organiser) and Dr Theresia Mikolash (BM Clinical Fellow).

We are once again very grateful to our fabulous volunteers including a few mum and dads, without whose help this event could not have taken place.  We are indebted to you all.

Over £11,000 has been raised so far for this event for Breathing Matters alone, which is almost triple last year’s total.

Look out for the 2015 date!

Support our Summer Stall 11.7.14

Summer is here!  And our Summer Stall is almost here too!!

The lovely Jane Walker is organising the Breathing Matters’ Summer Stall at UCH Atrium on Friday, 11th July 2014.

The talented Jane has designed kids’ summer picnic/holiday bags, cards and coasters.  Also on sale will be fans, colouring books, games and this year’s must-haves … loom bracelets.

Do come along and support us!

Spring 2014: Quiz Answers

Question 1: What was the name of the film the original Jamaican bobsleigh team inspired?

Answer: Cool Runnings.

 

Question 2: How many medals did Team GB win in the 2014 Winter Olympics?

Answer: 4 medals consisting of Gold x 1, Silver x 1, Bronze x 2.

 

Question 3: In which Winter Olympics did Eddie the Eagle compete in?

Answer: 1988, Calgary.

 

Question 4: Which country was top of the medal board in 2011?

Answer:  Canada with Gold x 14,  Silver x 7, Bronze x 5 medals.

 

Question 5: Upon which Sea is Sochi located?

Answer: The Black Sea.

 

Question 6: In which year was the first Winter Olympics held, and where?

Answer: 1924 in Chamonix, France.

IPF: The Game Is On, The Game Is Changing …

Until recently, we have had very little to offer patients with Idiopathic Pulmonary Fibrosis (IPF).  The disease is on the increase, tends to affect men in their late middle age, and has a prognosis worse than most cancers (50% of patients will die within three years of diagnosis).

The approach to the patient with presumed IPF, appears primitive compared to the sophisticated approach to the cancer patient. No-one would treat a cancer patient without a biopsy, but only a tiny minority of IPF patients undergo lung biopsy because of the morbidity and mortality associated with this surgical procedure; as a result, most patients receive a diagnosis of IPF on the basis of a history and typical CT scan results. There is a wealth of drugs on offer for the patient with cancer, but until now, there has been limited and conflicting evidence that any drug can alter the course of IPF. 

Until now  ……….  Last week at the American Thoracic Society, new data was released from a number of clinical trials that are published in the latest edition of the New England Journal of Medicine, the most established and reputable international medical journal. 

The studies published included the INPULSIS™-1 and -2 trial (Nintedanib, previously BIBF-1120 in IPF) and the ASCEND trial (Pirfendione in IPF).

Nintedanib is an investigational small molecule tyrosine kinase inhibitor in development by Boehringer Ingelheim for IPF.  It targets growth factors, which have been shown to be potentially involved – the vascular endothelial growth factor receptor (VEGFR), fibroblast growth factor receptor (FGFR), and platelet-derived growth factor receptor (PDGFR).

The two Phase 3 INPULSIS™ Trials of Nintedanib involved a total of 1,061 people with mild to moderate IPF treated for 52 weeks, and showed that Nintedanib reduced the lung function decline compared to placebo. The studies did not follow enough patients for long enough to comment on effects on mortality, but there was a trend towards reduced death rates in the Nintedanib group. The most frequent adverse event was diarrhoea, affecting 61.5% of patients on Nintedanib. 

However, before we get too excited, the patients in INPULSIS™ all had reasonable lung function (average lung capacity was 80% of that predicted and around 2700mls); the lung volume lost in the treated group over a year was 114ml compared with 228ml in the untreated group.  We are talking about small volumes, 114ml ~4% of the total lung volume saved over a year – or the same volume as one third of a can of coke out of a total of nearly 3 x 1 litre bottles. Will this translate into a serious impact on exercise ability and life expectancy?  Will there be effects on mortality given that there were no effects on acute exacerbation frequency? What will be the effect on patients with moderate to severe disease, the ones that are not caught early and perhaps have more progressive, aggressive disease?

The ASCEND trial was the fourth Phase 3 study to look at the effect of Pirfenidone (Esbriet) in 555 patients with IPF.  The American FDA has not licensed this drug, thought to act by inhibiting TGF β1, because the results of the first three trials in IPF were mixed. In the ASCEND trial, Pirfenidone reduced the one year rate of decline in FVC, with evidence, from pooling data from all four studies, that it may also reduce mortality. The patients in ASCEND had worse disease than in INPULSIS™ which may explain why they declined more quickly. Pirfenidone saved ~140mls of lung function per year.

Tempering the enthusiasm of the positive studies, is the realisation that the drugs didn’t make people feel better, stop disease progression, cure the disease or reverse the fibrotic damage – the ultimate therapeutic goals.  Coming with these sobering thoughts was the report of PANTHER-IPF.  Disappointingly, N-acetylcysteine or NAC was shown not to slow the rate of decline in FVC (although it may reduce the toxicity of prednisone and Azathioprine in IPF). However, one drug did offer some promise. FibroGen presented their Phase 2a study of FG-3019, an investigational monoclonal antibody that inhibits connective tissue growth factor (CTGF).  The trial recruited 37 patients with mild to moderate IPF of whom 33 completed 48 weeks of treatment. After 48 weeks of treatment, 12 of the 33 patients (36%) had improved fibrosis (unheard of in IPF before now) and two patients had stable fibrosis. This means that 14 patients (42%) had improved or stable fibrosis, improved lung function and felt better. Eighteen of the 33 patients continued FG-3019 treatment for a second year, and four continued to improve, but the rest worsened.

So where does that leave the patient with IPF and his clinician as the nihilistic approach begins to disperse?  What should we do with the mounting evidence that immunomodulation (with Cyclophosphamide and Rituximab) although beneficial in inflammatory pulmonary fibrosis (PF), such as that associated with rheumatoid arthritis and scleroderma, may increase mortality in IPF?  

More than ever before, we believe that it is essential to make a confident diagnosis in all patients with PF, to separate out those with IPF from those with inflammatory pulmonary fibrosis, and, in many cases, this requires a biopsy of the affected lung.   Our own work establishing non-invasive bronchoscopic cryoscopic lung biopsy to rigorously identify those with IPF, from those with other forms of PF, is set to be a game changer.

Breathing Matters has funded the research that has set the stage for a full application to the NIHR for a proper study on the role of cryoscopic lung biopsy (CLB) in the management of PF of unknown cause. It no longer seems fanciful to imagine a time when a patient with PF will be worked up with a scan and a CLB that will provide an individualised diagnosis; this result will guide the clinician on how best to manage their patient.

 

Although we are behind our cancer colleagues, (the standard of diagnosis and treatment options that they offer their patients is superior to that offered to patients with PF), the distance is closing, and it will not be long before they feel our toes on their heels!   The game is on……!

 

 

Professor Brown reports from the American Thoracic Society

Professor Brown was an invited speaker at the recent American Thoracic Society meeting in San Diego, talking about the recent findings his research group has made into how the immune system protects the lungs from protection. 

In addition, his clinical PhD students, Dr Jose and Dr Periselneris, presented some of their research data, and Professor Brown attended major symposia on bronchiectasis and pneumonia. These covered a lot of different areas of lung infection, including the increasing problems of antibiotic resistance and how this may be prevented, the use of longterm antibiotics in patients with bronchiectasis (especially azithromycin), and a new scoring system developed in Edinburgh for the severity of disease in patients with bronchiectasis which will help identify those patients requiring particularly intense treatment and will also help with clinical research.

Professor Brown reported that “The conference was very stimulating and provided plenty of ideas for future areas for improving the care of patients with lung infection.”

 

 

My Amazing Father, David John de Warrenne

We recently lost my Dad, David John de Warrenne, to an awful disease called Idiopathic Pulmonary Fibrosis, and pneumonia.  

Dad was such a lovely and truly wonderful man loved by so many. I couldn’t have asked for a better father. He was also an extremely talented musician, winning top competitions throughout his life and dazzling many with his “spine tingling” performances. He studied with the famous Julius Katchen, the famous concert pianist in his Paris apartment overlooking the Eiffel Tower. Dad, and his fellow pupil and dear friend of over 50 years Anthony Green, at 19 years old were the first white people to perform in Bermuda, casting aside the racial segregation of the times; something they were both very proud of.  

Following this, Dad worked hard all his life, teaching at Trinity School in Croydon for 40 years, arranging, accompanying and writing music for Trinity Boys Choir and the much coveted ”DW” choir, the public face of Trinity Boys Choir.

Not only this, but along the way he helped his students and family, not just musically, but with everyday obstacles life threw at them. Especially me, as his son, I always confided in him whenever I needed to and always depended on him.

Dad always approached life in a positive way and never let his illness affect him. Unfortunately, Dad was diagnosed with IPF not long after his retirement which restricted his ability to carry out every day tasks. After many years teaching, it’s so sad that he never got a fair crack at retirement.

Idiopathic means “no reason for”, pulmonary “lung” and fibrosis “scarring” and thus has no explanation for its occurrence. But upon diagnosis, it’s life changing with extremely devastating statistics. Thus, research and testing needs to be continued and advanced and hence why the family and I have chosen “Breathing Matters” as the charity of choice to raise money in Dad’s name. 

All donations in Dad’s name are welcome and greatly received http://www.justgiving.com/AdeW4DdeW. I will also be undertaking a challenge, yet to be decided, in Dad’s name in due course. 

Sleep well my amazing father, love forever, Alex

My Amazing Father was written by Alex de Warrenne

 

Are you up for a Challenge?

We are excited to annouce that Breathing Matters have teamed up with Sport for Charity, which incorporates Run for Charity http://www.runforcharity.com/ and Cycle for Charity http://www.cycleforcharity.com/.

They have given us one free place at the Spartan Race and one free place at the Zombie Evacuation Race .  They have also given us a decreased price of £35 registration for both events all year round, subject to availability.  These events are infamous and are a challenge above all else. 

The Spartan Race http://uk.spartanrace.com/ integrates obstacles with the natural terrain to create the best racing experience possible. Each course is unique, so racers never compete on the same course twice.​ What happens if you fall off or fail to complete an obstacle? It’s simple, just drop and give us 30 burpees before moving on!

The Zombie Evacuation Race http://zombieevacuation.com/ is a beginners’ obstacle race.  It’s not just a race, it’s an experience.  Why watch a Zombie Apocalypse … when you can be part of it.  Their mission is to embrace both the athlete and the geek, and for you to leave their events with a smile.

These events are all over the UK – find one near you!

A free space at the Run To The Beat 10K in Wembley on 14th September 2014 has also been offered www.runtothebeat.co.uk Past guests at this unique music challenge event has been Jessie J, Tinie Tempah, Calvin Harris and DJ Fresh.

Email us if you are for the challenge on breathingmatters@ucl.ac.uk

Marie’s Ongoing Story of Optimism

Further to Marie’s article on our website at the end of 2013, Marie still keeps her chin up despite ongoing obstacles.  Here is an update that she wanted to share with you for International Day of Happiness, 20th March 2014.

 

By Marie Maldonado

I was admitted to UCLH on January 27th 2013 and stayed on Ward T08 until February 13th 2013. During this time, my meds were totally changed as we are trying to discover what the ‘nodule’ is that’s been found by CT scan in my right lung. This nodule may be cancerous and has led to Papworth removing me from the transplant list. My extensive research and contacts with lung transplantees all over the world told me that it could be fungal in origin and both Dr Porter and Prof Brown took that on board, and the lab did indeed grow a strange fungus on my sputum. During my first few days on the ward, the team consulted with an expert microbiologist in Bristol and decided which antifungal they would give me. Unfortunately, this new drug (Voriconazole) would be impacted by the Omeprazole which has been shielding me from gastric reflux, part of the Scleroderma package.  The doctors tried removing the Omeprazole from my drug regime and I have been suffering ever since with the most horrible acid reflux/heartburn/indigestion, something that has led to a complete change of diet for me (so perhaps not all bad!) I am now taking Lansoprazole and Ranitidine and the reflux has abated a little, though I am still woken in the night and have to sit chugging Gaviscon for an hour or so.

I asked for a frank talk with Prof Brown about my prognosis and he admitted that I am indeed in end stage fibrosis and basically that I was looking at ‘weeks’. I was discharged with a full care package and now have a carer come in to the flat three times a day as my mobility is limited. I have a wheelchair for hospital visits and my very own ministering angel, who masquerades as my friend, neighbour and ex-colleague, who pushes it for me. To begin with, it was extremely hard to adjust to people letting themselves into my home and doing, very slowly, things that I knew I could do at twice the speed had I only the breath to do them with. Organising the meds has been another challenge as I have nine medications to take in various combinations and at various times throughout the day. However, as time has gone on, it has become much easier as I have been able to get into a routine and as my regular carer has learned what I need doing and how I want it done. (I have turned into an anal mini-Hitler, I’m afraid, sitting like the Queen of Sheba ordering her about.) My friends have been amazing (I never knew I had so many) and I think there have only been two days when I haven’t received a visit, if not from a friend then from a health professional. My social calendar has never been so full. Sometimes, I am grateful when no one is coming as I can then chill out and relax a little more.

It may be because of the meds or it may be the new dietary regime, or it may just be my long held spiritual beliefs bubbling up to the surface again, but I actually feel great!  Indeed, if it weren’t for the fact that I’m very ill, I would be very happy. Hell, I AM very happy! I imagine the doctors think I’m in denial, but I’m truly not; I just have the feeling that, if everything falls into place and the nodule can be seen to be non-cancerous, then I have a very, very, very slim chance of persuading Papworth to put me back on the transplant list and heigh ho, another challenge will begin. (There’s a sense that this period is something of a dry run for what life will be like post transplant, if I ever get that far.) Unfortunately, I don’t think everyone sees it quite this way. The Haringey Palliative Care Nurse whom I have seen has been tremendous as far as practicalities are concerned; she’s obtained bits and pieces of equipment for me and even got my Disability Living Allowance almost tripled. However, her whole focus is on my dying. Let me give you an example: the physio at UCLH arranged for the pulmonary rehab physio from the class I was attending for several months before Christmas to come to my house once a week. He’s another bright young man and he takes me through half an hour or so of gentle exercise with weights, culminating in my ‘walking the hall’. I keep note of how much I do, and each week I increase my score a little or go up a weight. I’m supposed to do this every day, but I truly don’t seem to have the time, plus it is hard to motivate myself when I’m alone. It does make me feel great! When I told the Palliative Nurse about this, she was almost disapproving. I could hear a ‘tut’ and see a shake of the head. I felt she wanted me to stay on the sofa and up the morphine and be comfortable. Unfortunately, Haringey Palliative Care does not go in for any kind of complementary therapies, unlike the Palliatives at UCLH, so my requests for massage, reflexology, etc. have fallen on deaf ears. Luckily, the universe heard my pleas and sent me an aromatherapist who gave me an incredible massage, and yesterday my hairdresser (from Mr Toppers in Tottenham Court Road) came all the way from her home in Brixton to crop my hair. Everything falls into place, like a jigsaw that does itself.

My dietary regime revolves around the reflux and the need not to trigger it. I have taken advice from fellow sufferers and am having some success. No more coffee, chocolate, cheese … in fact, as money is now no object (well, I may as well spend it rather than save for my old age!) I am shopping (or being shopped for) at Planet Organic and Whole Foods Market. I eat only organic, totally healthy food in small portions and I now focus on my food rather than multitasking as I eat. Some of my friends bring me home cooked food to go in the freezer. In fact, the friend coming later today to take me out to Epping Forest is bringing portions of her amazing chicken casserole which we’ll have for lunch before we set off, with a few extra pots to go in the freezer for another day.

I have also taken advantage of having helpers (carers and friends) to tidy and organise my flat, in a very amateur Feng Shui way, to free up energy flow, etc. It works for me, as does surrounding myself with things of beauty. The corners, piled with bits and bobs for years, are being cleared and everything is slowly becoming more organised. It does make a difference. As does practising the Buddhist precept of ‘mindfulness’: organising in my head, for example, what I need to pick up from each room, so that I only have to make one journey.

As far as I can see, end stage fibrosis usually means admission to hospital, possibly an induced coma and then a very quick death. And I wonder if it has to be that way. If more people were able to count their blessings rather than turning their faces to the wall in despair, would their endings at least be easier, gentler and, hard though it is to believe, as enjoyable as mine is proving to be.

Breathing Matters’ Easter Stall, UCH Atrium, 4.4.14

All the fours!  What are you doing on 4.4.14?  How about supporting Breathing Matters’ Easter Stall. 

Jane Walker is kindly organising this Easter Stall in the UCH Atrium between 11am-2pm on Friday, 4th April 2014. 

On sale will be cards, coasters, mini easter eggs and bunnies.

Jane is raising money for Professor Brown’s research into bronchiectasis.

Do come along to say ‘hi’ and support us.