First draft of this update
2015 April – IPF – Idiopathic Pulmonary Fibrosis – Where I’m at & where the IPF community is at with IPF researchers
IPF – Idiopathic Pulmonary Fibrosis – What’s it like if you’ve got it?
VIDEO NO 1- Please watch the first 5 minutes-ish
There is some hope – My ‘Roger’s IPF story’ and approach to healing is at the foot of this evolving research page.
Life is still sweet: I don’t feel sorry for myself. My suffering is minimal compared to several dear friends who are seriously ill or who have died in the recent past.
I’m ‘winning’ in the sense that more than half of those diagnosed in the same year as myself are now dead.
I have the support of Marion, my wife, I have faith, I have an education, & for however long I might have left I have a purpose – celebrating & exploring inter-spirituality. Life is still sweet – every breath, every day.
However I also try to rehearse death with every breath and leave the decision to my Maker.
As the saying goes, “Trust in God – but first tie your camel.”
VIDEO No 2. 30secs Shortly before succumbing to the disease, Evel Knievel recorded this –
SOME KEY FACTS
PLEASE HELP RAISE AWARENESS OF THE PLIGHT OF UK’s PULMONARY FIBROSIS PATIENTS – basic information for healthcare professionals, social care professionals, politicians – and patients
IN THE UK 15,000 people have Pulmonary Fibrosis (PF). Five thousand die each year. HERE for USA
ANYONE CAN GET PF – but adults between ages 30-80 are at higher risk .
CURE: None – 50% of those diagnosed die within 36 months. PF is relentless, progressive & terminal.
WHAT HAPPENS? Patients gradually lose the ability to breathe as their lungs fill with scar tissue.
PF IS INCREASING Since 1999 the number of patients with PF has increased by 156%.
MISDIAGNOSIS More than 50% of cases are misdiagnosed for a year or more.
WORSE PROGNOSIS THAN MOST CANCERS – see this 5 year survival rate comparison chart;
”SOME PATIENTS SAY ‘I WISH I HAD CANCER!’” says consultant Dr Toby Maher in BBC article (RP I don’t! – There are only two forms of cancer, of the ones listed here, that have a worse prognosis that IPF)
PF PATIENTS ARE NEGLECTED compared to cancer patients. One example – the UK government invested only £600,000 in PF research last year – leukaemia research alone received over £32m.
PF receives nothing like the same level of prioritisation as cancers of comparable mortality, eg leukaemia – yet PF – kills more people each year than this one form of cancer alone.
UK GENERAL HELP: currently it is difficult to get comparable, appropriate help from most agencies. IPF is a rare disease & many doctors never come across a single case. Ask your MP to help.
This site might be useful – https://sites.google.com/a/pulmonaryfibrosis.org.uk/pulmonary-fibrosis-uk/
For USA patients see www.coalitionforpf.org
June 2014 – https://www.youtube.com/watch?v=0JWAeQDql7A
Idiopathic Pulmonary Fibrosis Update: Bench to Bedside – ACCPCHEST – CHEST2012 –
IPF SUFFERERS STORIES
IPF HITS THE FAMOUS AS WELL AS ORDINARY GUYS LIKE ME
US Governor (retd) Olene Walker – https://www.youtube.com/watch?v=rhV4zgRdLUI&feature=youtu.be
Evel Knievel & Marlon Brando
Living with IPF – Colin’s story
There are many such stories, many on YouTube. Mine is at the foot of this article.
Published on 26 Sep 2014
Colin was diagnosed with idiopathic pulmonary fibrosis, IPF, after he became breathless on a walking holiday in Cornwall. Colin was sent for tests and scans and eventually saw a consultant who diagnosed him with IPF. IPF is a build-up of scar tissue that makes lungs thick and hard, making breathing difficult. IPF is incurable and will progressively get worse. About half of people with IPF only live for three years after their diagnosis. In this film, Colin talks about how he is living with IPF and how he has been supported by the British Lung Foundation.
Top 151 IPF videos – http://youtu.be/OICp4hEHTMo?list=PLltMCkzmVXiftJ5ciA7Df6Rm_Z3U_wq_j
Very short vid – http://youtu.be/WVjgR2NTshA
‘CURES’/Stuff that might help – not supported by conventional medicine but which might have helped me
NB The key to my approach is that it is spiritual, psychological, social and physical
Examples of stuff that might help include 1) Nattokinase, 2) Serrapeptase, 3) NAC
Stuff that I haven’t tried yet
Cannabis and/or Hemp Oil & IPF – Must have 30% + of CBD
A FEW EXAMPLES OF ASSOCIATED DISEASES which may or may not be causative
1 Sleep apnea and IPF –
2 2/3 of IPF sufferers have OSA
3 GERD/Acid Reflux and IPF – May occur in up to 90% of patients
4 Viruses, bacteria, fungi etc
Various forms of Herpes and IPF – http://jcm.asm.org/content/41/6/2633.full
5 Autoimmune disease – to follow
OTHER INFORMATION SOURCES – probably won’t tell you anything you can’t get from NHS Direct & other sites dedicated IPF sites
NOTES/EXTRACTS TO HELP ME ASK QUESTIONS – NB I’m still not sure if IPF is an autoimmune disease or whether autoimmune diseases are frequently associated with it.
WHAT IS PULMONARY FIBROSIS?
The word “pulmonary” means “lung” and the word “fibrosis” means scar tissue – similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. But, pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.
Also, pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike (see “Causes and Symptoms” below). The PF family of lung diseases falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.
Autoimmune diseases – From http://www.pulmonaryfibrosis.org/life-with-pf/about-pf 30/03/2015
Autoimmune diseases are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. “Auto” means “self” and “immune” refers to your immune system. With autoimmune diseases, your own immune system is attacking your lung. If you have an autoimmune disease, your immune system can cause inflammation and scarring in the lungs. Examples of autoimmune diseases that can cause PF include:
- Rheumatoid arthritis
- Scleroderma (now called “systemic sclerosis”)
- Certain muscle diseases (polymyositis, dermatomyositis, and the anti-synthetase syndrome)
Approximately 10-15% of those with an “idiopathic” form of PF have another family member afflicted by the disease. This is called familial pulmonary fibrosis (FPF) or familial interstitial pneumonia (FIP). A number of genes and genetic variants have been identified that are associated with the development pulmonary fibrosis, but frequently genetic tests are not performed when PF is diagnosed, as we are only just beginning to understand the significance of these genetic abnormalities. It is important to discuss the potential risks and benefits of genetic testing with a qualified genetic counselor and your medical provider. To speak with a Certified Genetic Counselor free of charge, contact Janet Talbert, MS, CGC at 800.423.8891, extension 1097.
CLASSIFICATION OF COMMON INTERSTITIAL LUNG DISEASES
The most common symptoms of PF are cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.
Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to IPF and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth.
Other common symptoms of pulmonary fibrosis include:
- Chronic dry, hacking cough
- Fatigue and weakness
- Discomfort in the chest
- Loss of appetite
- Unexplained weight loss
RP – The ones I have big time are the first 3 – but not the last two!
A “pulse oximeter” is a device placed on the fingertip or earlobe that indicates the amount of oxygen in the blood. Normally, nearly all of your red blood cells are full of oxygen. When all the red blood cells are carrying oxygen, the blood is called “100% saturated.” A normal saturation level is between 95% – 100% when the test is performed at sea level. Low oxygen saturation values can help your medical professional identify the presence of lung disease. Early on in PF, oxygen levels may be normal while you are resting, but can decrease while exerting yourself. This drop in oxygen saturation (called “de-saturation”) can make you feel breathless. When oxygen levels drop below 89% during exertion, your medical professional may recommend using supplemental oxygen while active.
- Alveoli: Tiny air sacs in the lungs where carbon dioxide leaves the bloodstream and oxygen enters the bloodstream.
- Bronchoscope: An instrument used for inspecting the airways of the lungs.
- Comorbidity: A disease or other medical issue that occurs simultaneously with PF.
- Diffuse parenchymal lung diseases (DPLD): Another name for interstitial lung diseases.
- Diffusion capacity (DLCO): A measure of the ability of oxygen to diffuse into the bloodstream.
- Dyspnea: Difficulty breathing or shortness of breath.
- Exacerbation: An episode of rapid decline or the emergence of more severe symptoms.
- Fibroproliferation: Relating to the growth of fibroblasts (and fibrous tissue), one of the basic connective tissue cells.
- Fibrosis: An increase in fibrous scar tissue.
- Forced expiratory volume (FEV1): The amount of air you can blow out in one second, which is measured by spirometry.
- Forced vital capacity (FVC): How much air you can blow out of your lungs, which is measured by spirometry.
- Gastroesophageal reflux disease (GERD): A regurgitation of stomach acids into the esophagus and throat, causing heartburn, acid indigestion, and possibly injury to the lining of the esophagus. This is also called acid reflux disease.
- Hospice care: Palliative care for patients at end-of-life.
- Idiopathic: Of unknown cause.
- Idiopathic interstitial pneumonias (IIP): A type of interstitial lung disease. Idiopathic pulmonary fibrosis is a type of IIP.
- Interstitial lung diseases (ILD): A broad category of over 200 lung diseases that affect the lung interstitium.
- Interstitium: The space between the alveoli (air sacs).
- Palliative care: Non-curative therapy that treats symptoms and focuses on improving quality of life. It can be received at the same time as curative therapy.
- Pathologist: A physician specializing in disease-associated changes in tissue and organs. Pathologists aid in making a medical diagnosis.
- Pulmonary: Relating to the lungs.
- Pulmonary hypertension: Abnormally high blood pressure in the pulmonary (lung) arteries.
- Pulmonologist: A physician specializing in the lungs.
- Radiologist: A physician specializing in using radiology tests(e.g., X-rays) to diagnose illness.
- Rheumatologist: A physician specializing in rheumatic diseases, which may include arthritis, autoimmune diseases, and joint diseases.
- Spirometry: A test that measures the amount of air inhaled and exhaled over time.
- Usual interstitial pneumonia (UIP): A specific abnormal radiologic or pathologic pattern of interstitial lung disease.
I am fighting for my life because no one else will or can
‘Let food be thy medicine…’ is my general approach but if necessary I use modern drugs.
WARNING – if you have IPF this is for information only – talk to the best medics you can find
as well as complementary approaches. With IPF particularly you have to manage your life, treatments & death.
IT SEEMS EVERY PATIENT AND THE COURSE OF THE DISEASE IS DIFFERENT – March 2015
I have the cough
I have the velcro crackle in my lungs
I have crushing, cold pain in my chest
I have the devastating exhaustion
I now have SA – Sleep Apnea
I now have heart problems – various kinds of palpitations and arrhythmias – see the ‘HEART STORY’ – HEART Herbs, vitamins, minerals & foods for the heart
I have had GERD/Acid reflux
I have, as all sufferers do, the ‘psychological load’, including the frustration & depression of not knowing if I have 4 weeks or 4 years. The only other guy locally I knew with IPF died last year. He was fitter, slimmer, younger than me – five sets of table tennis he said then suddenly he went from ‘normal’ health to death in 4 weeks.
Ironically as yet the oxygenation and pulse are steady and normal 96% & 65 – weird but long may that continue. It must have something to do with my ‘regime’ ? NB Nothing is straightforward with IPF – no cause and effect is simple.
5,000 were diagnosed in the same year as me – more than 3,000 of them are now dead.
UPS & DOWNS
I was mis-diagnosed – probably for years. My IPF was discovered ‘accidentally’ because I went for a heart scan.
I had a devastating ‘flare-up’ that lasted for 3 months over Christmas – which was a step further down the un-primrose path – in that exhaustion sets in earlier and I need to rest and sleep even more.
The previous Christmas I had 3 months of a devastating virus/bacterial infection….. Had to sleep on my knees lying forward on sofa or bed.
Stay away from people with colds, flu etc.!
HEART:For the last year and a half arrhythmia has been extremely alarming = HEART Herbs, vitamins, minerals & foods for the heart
SLEEP APNEA: Thank God my heart consultant referred me to a sleep clinic. I was successfully treated in that they supplied me with ‘the gold standard’ RESMED 9 machine which I have to breath from each night.
MY PHILOSOPHY WHICH I STARTED TO DEVELOP AS SOON AS I WAS DIAGNOSED
STRATEGY 1 If there is anything specific that might help specifically with the IPF do that. STRATEGY 2 If there is anything that might boost your general health do that.
I take occasional take breaks from my two regimes to give my liver & kidneys a rest and to re-…..
STRATEGY 1 If there is anything specific that might help specifically with the IPF do that.
1 The first two specific things I did was to start taking two enzymes Nattokinase and Serrapeptase. Enzyme therapy isn’t accepted but quite a few use it. The second thing in the first year after diagnosis was to consultant a qualified medical herbalist whose primary approach was to reduce inflammation. After a while she added one or two herbs to the medicine she made up for me that are specific for lungs.
I also did some sessions with an acupuncturist.
I also did a session with a horse whisperer who was also a distance healer. Believe me when a doctor looks you in the eye and says, “You have as little as months to live. There is no cure, except a lung transplant and you can’t have one.” and another says “The drugs being tried are arguably worse than the disease. We won’t start poisoning you yet.”
In the shock of those first days, weeks and months after diagnosis you will eat horse-dung sandwiches if you think they would have a slight chance of helping.
The sheer cruelty of the way the NHS treats someone at this point is dreadful, but that is another subject.
The sheer cruelty of aspects of the govt agencies is dreadful – partly because no one helps you – also another story.
Our MP Norman Baker has been brilliant.
THE NOT KNOWING INCLUDES MANY VITAL KEY QUESTIONS
How can I slow down the progress of the disease? a. “We don’t know.”
What might cause the disease to accelerate? a. “We don’t have an answer to that.” ETC.
I took on the superhuman task of trying to create, as far as possible, my own therapy. The main problem is that the resources of one desperately ill person are limited. In flare ups or times of viral/bacterial infection I can barely get from bedroom to lounge.
I had two pieces of bad luck – a friend who could have helped with understanding research papers died after 7 years of hell with lung cancer – he was one of the world’s most eminent professors in the field of lung disease!
A family one of whose members died said they would help me – and then changed their minds. Cruel.
I research several hours every week.
STRATEGY 2 If there is anything that might boost your general health do that.
I research & take super-herbs, super-spices and super-foods plus food supplements. – details to follow
WHAT HELPS ME MOST? – DETAILS RE MY PERSONAL PROGRAMME to follow
HOW BAD CAN DRUGS BE? -to follow
WHO CONTROLS OUR DISEASE MODEL OF HEALTH? – to follow
Every breath is a life- affirmation but I also try to rehearse death with every breath and leave the decision to my Maker – or
as the saying goes, “Trust in God – but first tie your camel.”