Forum Replies Created

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  • tim-blowfield

    Member
    March 19, 2024 at 6:03 pm in reply to: CF is more than just lungs

    Hi Kay,

    my wife attends the CF Clinic in the Alfred Hospital in Melbourne. She was found to have G542X and R75Q when her gene was sequenced by the Canterbury, NZ lab in 2009. Her sweat test was equivocal but her list of symptoms were 20 of 25 for CF. I would reply to your Dr who said it is not worth finding your other mutation “Bulldust! It might be rare BUT it could be one of the 180 variants that Vertex has shown should respond to Ivacaftor, Trikafta etc. ” If it is then being on TK may be the best treatment for you. You may have some unpleasant symptoms at first as your body adjusts but in most those symptoms peter out. Go fot it!

  • tim-blowfield

    Member
    March 14, 2024 at 6:19 pm in reply to: CF is more than just lungs

    Unfortunately, it seems that still far to many of our CF Experts are experts in lungs only, appearing to be in denial that any other organs than mucous membranes are affected by the faulty CFTR protein and Chloride channel. Except they do recognize that sweat glands are affected. Even the registries fail to record the extent of CF co-morbidities, most of which do appear to be CF caused. And Vertex; ……….. enough said.

    And researchers concentrating on replacing the faulty gene in the airways only I fear will leave pwCF short changed – in other words up the creek without a paddle! We are also seeing this in pwCf who have had lung transplants. They haven’t been cured of their co-morbidities though some may have their Adrenal failure improves by the anti-rejection drugs given post-transplant.

  • tim-blowfield

    Member
    March 13, 2024 at 3:27 am in reply to: CF is more than just lungs

    Hi Kay. No I am not in a CF Clinic just a humble husband of a wife who does have CF and has done so for nearly 82 years but only diagnosed 15 years ago. She has one of the 100 or so mutations, R75Q; of which just 9 are recorded in the Australian CF Registry and is probably the only person in Australia with that variant and not F508del on her other chromosome.

    As a retired Vet who has had to nut out some curly problems in my patients over a 50 year career I am not averse to posing curly questions. With CF I see that there is much unknown especially as to why pwCF have so many co-morbidities. Abnormal intracellular chloride and other electrolytes seem to be a reasonable explanation. I fear that just treating the lungs is shortchanging pwCF.

    CF Physicians, while needing to be experts in the lungs, need to be expert general physicians!

  • tim-blowfield

    Member
    March 11, 2024 at 4:29 pm in reply to: CF is more than just lungs

    Are they going to try you on a lower dose of Trikafta, Orkambi or on Lumacaftor (an earlier drug)? That often reduces the liver damage. It appears your sarcoidosis is an inflammatory response to past infections.

  • tim-blowfield

    Member
    March 7, 2024 at 3:56 pm in reply to: Struggles with CF-related diabetes

    The CSIRO, Australia’s government owned and funded research organization, has developed a diet that is recommended for Diabetes and obesity – indeed most persons. It is published in a number of their books, one of which I have attached.

  • tim-blowfield

    Member
    March 5, 2024 at 4:38 pm in reply to: Struggles with CF-related diabetes

    This seems common with CFRD (probably CFRD is called Type 3C by some). My wife’s blood sugar (BG) swings widely sometimes even when she has eaten nothing. Usually dips overnight and may be blow 3mmol/L about 3-6am. At times has been difficult to get up. We have found adding glucose to an electrolyte drink seems to work best. It seems abnormal intracellular electrolytes plays a significant part in CFRD. Where? Does it play a part in the production of Insulin in the Islet cells or in the activation of that Insulin? It seems people on Trikafta have better control of their BG. Do they? Comments please?

  • tim-blowfield

    Member
    February 13, 2024 at 4:31 pm in reply to: Sunday Morning (61): From Infection to Inflammation

    Aspirin (Acetyl Salicylic Acid) is an anti-inflammatory and reasonable pain killer, but not usually classed amongst the NSAIDs and is not a steroid such as Prednisolone and Cortisol are. It is an extract from the bark of the willow tree. It has many benefits but also like all drugs some issues. it is irritating to the stomach and can cause bleeding. In low doses (100mg a day) does reduce clotting and the propensity for heart attacks.

    I once had a dog with severe gastric bleeding – a colleague had put him on Aspirin and the naturopathic Willow Bark Extract together. At I knew how much Acetyl Salicylic Acid was in the Aspirin tablets – How much more was in the WBark Extract was the unknown. This is why I am wary of naturopathic/alternative medicines. I like to know what I am taking and how much!

  • tim-blowfield

    Member
    February 13, 2024 at 3:01 pm in reply to: CF is more than just lungs

    The latest news from Aus:

    1. Ivacaftor has been recommended by the PBAC (The Pharmaceutical Benefits Advisory Committee – a Gov Committee) that it should be available to any pwCF who has a mutation where there is evidence it should help. This will make it available for about 10 persons in Aus. that can’t get it now, incl Reva. Just have to wait for the Health Minister/Gov to act!

    2. Trikafta is being considered in March for kids aged 2 to 6 years by the same committee .

  • tim-blowfield

    Member
    February 13, 2024 at 2:52 pm in reply to: CF is more than just lungs

    One may question the cause of your sarcoidosis. little is known about it in ‘normal’ people though like Sjogren’s it is thought to be autoimmune. In CF both these conditions appear to be inflammatory reactions in the damaged lungs. Could be a sign of the presence of Mycobacteria, a bacteria not uncommonly seen in pwCF.

  • tim-blowfield

    Member
    January 29, 2024 at 5:28 pm in reply to: Exercise and CF

    “More vigorous exercise boosts life quality, lung health with CF” was posted today. It is well known the benefits of exercise is great for pwCF as with all people but the conclusions of an article such as this are flawed by the question ‘which is the chicken and the egg?’ The conclusion made was that those who exercised better had better health and lungs but is it that those with better lungs and health could exercise better. A pwCF with poor health and lungs may be depressed in that they cannot achieve what this article suggests they should. While encouraging the best for our pwCF we need to be compassionate in understanding and awareness when interpreting results such as these.

  • tim-blowfield

    Member
    January 12, 2024 at 9:43 pm in reply to: CF Therapies Symdeko and Orkambi Seen to Also Ease Inflammation

    High fat diet is proving to be unnecessary even harmful in persons who have responded to one of these modulators or Trikafta. And the drugs seem to be having benefits in the function of many other organs, Adrenal and other endocrine glands, heart, mental illness and others.

    Reporting of these effects are needed not just as side effects but there needs be discussion about them. Are we seeing improvements in Osteoporisis?

    Headaches are reported as a common adverse side effect. But what is really happenning here? In most they seem to wane with time. Are they evidence that the brain is adjusting to changed intracellular electrolyte levels?

    Many pwCF with CFRD have reported improvements. This suggests that the Islet cells are responding.

    Abnormal intracellular electrolytes appear to be the cause of many of the so called co-morbidities seen in pwCF. Logical? It certainly appears that the faulty CFTR is causing high chloride within cells with corresponding high cations (K, Ca, etc). Some cells may be little affected but in others the effect may be profound: Adrenal and Parathyroid Glands, Muscles incl Heart muscles, etc.

    The study of CF needs much lateral thinking about this. While the lung issues will long be most important, Intracellular electrolyte abnormalities may be causing a host of ignored issues.

  • tim-blowfield

    Member
    December 7, 2023 at 6:06 pm in reply to: What are you looking forward to in 2024?

    I am looking forward to:

    1: Trikafta being available to those who have mutations other than F508del that Vertex has shown to respond.

    2: Greater awareness of the effect of the faulty CFTR as the cause of the so many ‘co-morbidities’ seen in pwCF. It appears that these are caused by abnormal electrolytes within cells caused by the Chloride ion being retained and high in the intracellular fluid necessitating high cations to balance it. High Calcium may be expected to affect Parathyroid function causing hyperparathyroidism and osteoporosis. It may also affect muscle function including cardiac causing myocardiopathy and poor response to many cardiac drugs esp. Calcium Channel Blockers. High Potassium may also be the cause of Adrenal malfunction and Hypokalemia, cardiac muscle and even nerve malfunctions. So much to learn and currently being ignored.

    3: More information being gathered on observations of what Trikafta and the other modulators are doing in the body apart from the lungs (not ignoring the lungs). It appears that many of its ‘side effects’ are from it ‘correcting’ the intracellular electrolytes.

  • tim-blowfield

    Member
    November 21, 2023 at 5:03 pm in reply to: Trikafta and Severe Constipation

    As William said – Talk to your Doctor.

    Your diet as a person with CF has been designed to cope with your gut not absorbing food well. With starting TK that changed though some damage may persist – take time to heal or even be permanent. Fibre is important in the functioning of a normal gut but how normal is yours at this time. Other drugs can also cause constipation. Dapagliflozin is a diabetic drug of the SGLT2 inhibitor group. It prevents the reabsorption of glucose in the distal kidney tubules causing increased urine production and loss of water. This group of drugs can cause severe dehydration as the gut absorbs water to replace that lost in the urine. Increased fibre in the diet does retain water and modulate the softness of faeces – makes hard faeces softer and fluid faeces thicker. Metamucil is good – I take Benefibre 1 heaped desert spoonful with my breakfast muesli. Apart from increasing the volume of poo I know of no nasty side effects.

    Trikafta by changing the dynamics of Chloride transport may be expected to change the transport of salt and water across the gut wall. Therefore we should expect the consistency of poo to change. How much it will change will depend on the condition of the gut itself – a pwCF who is more than a few years old is likely to have gut damage that may or may not be reversible. Globlet cells may have been lost so reduction in the amount of protective mucous may be present. Scaring from repeated infections too.

    We may expect TK and the other modulators to improve gut function but there may be some damage that is permanent. But increased Fibre should help most people.

  • tim-blowfield

    Member
    November 16, 2023 at 4:40 pm in reply to: CF is more than just lungs

    Yes the benefits to the lungs is amazing and MUST not be sidelined. But in assessing Lumacaftor, Trikafta & other therapies for pwCF the co-morbidities also should not be ignored. Too often the effects on other body systems are reported as adverse and undesirable side effects without any attempts of trying to understand why they are occurring. Often they are just ignored in the reporting.

    I postulate that many of the ‘side effects’ are the drugs correcting (lowering) the Chloride levels within cells to levels similar to that of a ‘normal’ person thus lowering balancing cation levels esp Calcium & Potassium. These lower cation levels should be expected to beneficially affect not just Adrenal and Parathyroid gland functions but also muscle and neurological function. WE may even find Diabetes improves with these electrolyte improvements.

    In assessing treatments a wholistic approach is needed assessing not just lung function but the effects on the function of all other body systems.

  • tim-blowfield

    Member
    November 3, 2023 at 5:15 pm in reply to: Any Recommendations on Steroids for muscle growth?

    Don’t!

    Unless your Dr prescribes and closely supervises them.

    Premature death and heart failure are all too common .

  • tim-blowfield

    Member
    November 2, 2023 at 5:03 pm in reply to: CF is more than just lungs

    The last section of my post yesterday was was rendered somewhat incomprehensible by formatting instructions. I commented on the report received yesterday:

    “”Trikafta leads to clinical gains for 2 adults with rare M1101K mutation”

    I commented that while wonderful, it was an example reporting on the lungs but nothing as to whether improvements had occurred in other organ systems.

    We need pwCF being assessed as whole persons – not just a pair of lungs!”

  • tim-blowfield

    Member
    November 2, 2023 at 2:33 am in reply to: What are you looking forward to in the Fall?

    No. Not much snow in Melbourne – we do get the occasional flurry on the Dandenongs – hills on our eastern border but very rare in the city. Do have snow on the Australian Alps.

    As I have said before:

    Beautiful one day – perfect the next!

    Though some say we have all four seasons in one day – can have. Stops us getting bored.

  • tim-blowfield

    Member
    November 2, 2023 at 2:27 am in reply to: CF is more than just lungs

    An update:

    3 Months ago Reva was admitted ’cause she had splenic infarcts causing severe pain and caused by clots been thrown off from the left atrium. Was in hospital for 7 weeks. She now has a device blocking off the left Atrial Appendage which should reduce clots being formed by 90%. The Alfred Hospital which has the CF Unit just wanted to treat her palliatively so she transferred to Cabrini where the LAA was blocked off and she gradually recovered. But throughout her ordeal we had to be very vigilant and assertive to ensure her multiple co-morbidities of CF were not neglected.

    It is amazing how usual it is that doctors treat the problem in their field and refuse to consider other co-morbidities and the CF that underlies it all.

    We need

    1: a better understanding of CF in our wider medical fraternity

    2: CF Physicians that see CF as being a problem that affects more than just the lungs.

    There are some wonderful reports of the benefits of Trikafta on the lungs and respiratory tracts but in all the research effects on other organ systems are unreported at best reported as ‘side effects’ to be avoided. Are we seeing benefits in Adrenal function? Cardiac & Muscle function and strength? Diabetes? Vascular health?

    What is happening in Mental health? Headaches have been reported when pwCF start TK and is a reason many stop. But is it a result of intracellular electrolyte changes caused by improved Chloride transport?

    A recent report <b style=”background-color: var(–bb-content-background-color); font-family: inherit; font-size: inherit; color: var(–bb-body-text-color);”>Trikafta leads to clinical gains for 2 adults with rare M1101K mutation<font color=”rgba(0, 0, 0, 0)” face=”inherit”> while wonderful is an example reporting on the lungs but nothing as to whether improvements had </font>occurred<font color=”rgba(0, 0, 0, 0)” face=”inherit”> in other organ systems. </font>

    <font color=”rgba(0, 0, 0, 0)” face=”inherit”>We need pwCF being assessed as whole persons not just a pair of lungs!</font>

  • tim-blowfield

    Member
    September 25, 2023 at 9:09 pm in reply to: What are you looking forward to in the Fall?

    I prefer not to fall. falling in hospital causes too much paperwork! We have a beautiful spring day here in Melbourne. Lovely day – Beautiful one day – perfect the next!

  • tim-blowfield

    Member
    February 13, 2024 at 4:13 pm in reply to: Sunday Morning (61): From Infection to Inflammation

    Hi J-P,

    Lyrica (Pregabalin) is a rather strong pain killer that works best in neurogenic pain. Was originally developed for epilepsy. Needs reasonable supervision with a particular check for suicidal ideation. Otherwise is very good but can be addictive.

    With all drugs we should read the Product Information sheets. In Australia we have 2 forms, a CMI which a consumer Information sheet that should be either in the packet or supplied by the Pharmacist when dispensing and a much more detailed PI (Product Information). Both can be accessed through the TGA (Therapeutics Goods Administration) – Australia’s controlling organization in the Health Department. Search TGA Australia. It can be a bit daunting searching for the information but I usually go through the public TGA Information. You may have to tick a consent box or so. The US has a similar source of Info.

  • tim-blowfield

    Member
    February 13, 2024 at 3:16 pm in reply to: Sunday Morning (61): From Infection to Inflammation

    The drugs you mentioned are quite varied: some are anti-inflammatories and some anti-allergy . Both have their place – we have found low doses of Ibuprofen do improve breathing. Usual dose is 2x200mg 4x a day but we find just 1 capsule once of twice a day is adequate. It is a Nonsteroidal Anti-inflamatory (NSAID). Need to watch kidneys & Liver with NSAIDS. Aspirin can help but again need to watch side effects as also Panadol.

    Re: Anti-allergy drugs there is a plethora of Antihistamines and the like for Asthma – all have their peculiarities/side effects – some worse /some better than others. Most relatively safe.

  • tim-blowfield

    Member
    February 13, 2024 at 3:02 pm in reply to: CF is more than just lungs

    One may question the cause of your sarcoidosis. little is known about it in ‘normal’ people though like Sjogren’s it is thought to be autoimmune. In CF both these conditions appear to be inflammatory reactions in the damaged lungs. Could be a sign of the presence of Mycobacteria, a bacteria not uncommonly seen in pwCF.

  • tim-blowfield

    Member
    January 12, 2024 at 9:18 pm in reply to: What are you looking forward to in 2024?

    Kay, Sorry your experience has been so poor. You should be asking for further genetic testing as the unknown mutant may be one that responds to a modulator (eg Trikafta). If it does then that would improve your treatment immensely.

  • tim-blowfield

    Member
    September 8, 2023 at 7:14 am in reply to: Dating and relationships: When do you mention CF?

    Hi All,

    The last 2 months have been just horrendous. First mid June my wife had a viral infection that put her in hospital for 4 days, then 2 weeks later her chronic discomfort became severe pain so sought out an emergency GP to get better pain relief. During the triage exam the nurse called in the GP who on examining her immediately referred her across the road to A&E. By days end they found multiple infarcts in the spleen which they attributed to an inadequate dose of Apixaban. After 2 weeks in the CF ward trying to get the pain under control she was transferred to Cabrini where further tests showed clots had blocked not just arteries in the Spleen but legs as well. Pain was slowly got under control and Yesterday a device was placed in her Left Atrial Appendage to block it off (That was the source of the clots). It has been a testing time for us both, a huge test of my love. Very difficult watching my love suffer so much, being supportive through all. Our faith has been tested, yes but has been so very important. Cabrini has a hugely supportive Christian ethos and wonderful pastoral support.

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