FEV1
Birthday Wish
This time last year all I wanted for my birthday was Kalydeco.
Each year my birthday wish has been for a CF cure. Last year was the first time that it felt close, frustratingly close as I knew it would be a while before it was approved in Australia. Luckily the named patient program meant I was able to start Kalydeco in September. The change has been astonishing, my lung function has improved 30%, now my highest in 3 years, I am at my highest weight ever, and my sweat test is now below the diagnostic point for Cystic Fibrosis.
So what could I possibly want this birthday?
1. Fast approval/listing in Australia so the 200 people with G551D can access Kalydeco.
2. Fast research for everyone with F508del and other rare mutations, so we can all have a medication like Kalydeco.
3. Someone that can do my uni assignments for me so I can relax on my birthday 😉
229 days of Kalydeco
Felt like being creative with the title so went with 229 days rather than almost 8 months.
This is 458 magicbluepills. Pretty amazing what many small pills in a row can do.
I had clinic yesterday and my FEV1 was 69%. Whilst not in the special 70s, it was 2.36L last time and 2.35L this time, so it really is an insignificant difference! I was expecting it to be lower as I have been quite stressed and busy with uni recently. My grandpa also passed away. Despite all of these stressors my lung function has been stable. Previously, when I was very stressed during my final year at school, I was quite unwell and varied between 60 and 75%. Now, 11 years later, I’m still at 69% 🙂
This means I have been stable between 69-71% for 8 months.
It has now been 8 months without inhaled or intravenous antibiotics. I think the last time I went this long was back in 1994. But even back then, my pfts varied. My cough is slightly productive in the morning but I rarely cough during the rest of the day. It is also a mostly dry cough. My sputum cultures have not changed, the pseudomonas is still there. My sputum tests don’t show the amount of pseudomonas, but I am sure the amount has significantly decreased.
It has been 6 months with no chest physiotherapy now, but I am still doing pulmozyme and bronchitol regularly. This combination, along with the Kalydeco, is definitely helping to keep my lung function stable.
I was looking back through a few folders that have copies of my pfts/admissions:
Number of admissions (respiratory- intravenous antibiotics):
1994: 1, 1995: 2, 1996: 2, 1997: 4, 1998: 3, 1999: 4, 2000: 3, 2001: 4, 2002: 4, 2003: 3, 2004: 2, 2005: 3, 2006: 4, 2007: 3, 2008: 3, 2009: 3, 2010: 3, 2011: 4, 2012: 3 and… 2013: 0! (none since sep last year)
Total= 58 admissions (plus others for sinus surgeries, gastro blockages and a stroke in 1993…) This equals about 150 weeks. Thats 3 years.
Thats enough for my lifetime 🙂
My FEV1 is stable at around 70%, my highest in 3 years. My BMI has plateaued at 22.
7 Months of Kalydeco
Uni is going well and unlike last year I completed 3 exams on time. I was able to study all day for the last few weeks, something I could not have done last year. Last year I thought it was unlikely that I would be able to complete the subjects this year as they involved so many contact hours, so its pretty exciting that one term is over, just three more to go.
After the exams I took a break and visited Tasmania. We went 4wdriving in the north west, took a slide into a swamp (!) and hiked in the cradle mountain national park. Last year I visited cradle mountain but decided to only do the ‘flatter’ less steep walks, this year I conquered one of the more difficult walks. It was still quite difficult and I was puffed out climbing to 1200m with 70% lung function, but thanks to the magicbluepill I made it to the top!
Not much else has changed. I had another sweat test today so will post about the results soon.
Between Arthur River and Sandy Cape.
For some reason there is a big 110m slide down into the Tarkine swamp. Much faster than walking down!
Cradle Mountain National Park: Keith in front of Crater Lake, half way to the top.
Almost at the top…
Resting in a pretty comfy chair, 1200m high. Didnt rest for long though as the weather was coming in…
Cradle Mountain in the background, Tasmania really is beautiful!
6 months of Kalydeco: stable at 70%
The constant productive cough, the overnight cough that disrupted sleep and the breathlessness from walking quickly and up stairs are now distant memories. Instead I have noticed some new changes; my hair is in better condition, my skin has more colour, the dark circles under my eyes have mostly disappeared, even my nails have less small ridges. I can’t remember the last time someone told me you look sick or tired, I was starting to get this daily before Kalydeco. Instead most people now comment about how well I look. I looked back at some photos, and the last time I looked well (not pale & tired) was in May 2010 at my 25th birthday.
For 6 months my lung function has been stable and I have not needed antibiotics. My weight is still increasing. For the first time in my life I need to adjust my CF diet and start to cut down portion sizes. I already eat reasonably healthily (never really liked/agreed with the high fat diet that was promoted when I was younger), but I am hoping some small changes will help. I have started running more regularly, now that it is finally below 35 degrees, which should also help. I am planning to write an in depth post about my exercise in the next few weeks.
Now that I have been busier with uni every day, I have noticed something that is really pretty obvious, that sleep is very important for my lung function. I have been pushing it a little with 6-7 hours of sleep during the week, which starts to catch up with me. My FEV1 can drop 2% or so when I am really tired (a minor drop I know compared to my previous 20% drops, but given how stable I now am 2% starts to concern me!). I am trying to gradually change my body clock and routine as the early starts are going to continue all year.
I have still been noticing a gradual decrease in sputum now that I have been on both Pulmozyme and Bronchitol for a few months. The combination seems to be working really well. I have now reached my goal of being stable and IV free for 6 months, so my new goal is 1 year 🙂
Start: FEV1: 41%, Weight 55.6kg
1 month: FEV1: 70%, Weight 58.5kg
2 months: FEV1: 71%, Weight 60.0kg
4 months: FEV1: 70%, Weight 61.5kg
6 months: FEV1: 70%, Weight 61.8kg
Four months of Kalydeco
I went to clinic a few weeks ago and my FEV1 was 70% 😀
It’s the first time I’ve been out of hospital for five months in the last few years and the first time I’ve been stable without antibiotics in about 15 years!
Start: FEV1: 41%, Weight 55.6kg
4 weeks: FEV1: 70%, Weight 58.5kg
8 weeks: FEV1: 71%, Weight 60.0kg
4 months: FEV1: 70%, Weight 61.5kg
Kalydeco helped me bounce back from my cold, helped stop my PFTs from dropping and helped stop me from getting sicker. With my last three overseas trips I’ve returned home and my FEV1 has been at 49, 50 and 41%. The only way I improved back then was with IVs. Now my body’s ability to cope has improved dramatically. Being stable whilst pushing the limits (travelling, plus the cold temperatures, pollution, smoke and no nebuliser) and still staying at 70% is amazing!
I am planning to stay on Bronchitol and see how this goes for the next few months. I love how convenient it is and it is still helping me clear my lungs well. My cough is back to my new ‘normal’- meaning I am not coughing very much and if I do it is yellow in colour and easy to clear. My energy levels are improving, but I discovered recently that I still struggle with full time university (9-5 every day). Last year I struggled with part time uni, so this has improved a lot, and I am hoping it will continue to improve.
Over the last 10 years I have kept my lung function results in an excel document, so I decided to make some graphs showing my FEV1 and weight. The graphs are pretty amazing, they show my new stability and the very large initial improvement.
I am currently at my highest FEV1 in 2.5 years and only 5% off my highest in 8 years.
This is my highest weight ever. My BMI has changed from 20.0 to 22.1
For anyone who is interested in stats, just for fun (I have been studying stats & evidence based practice for 3 weeks at uni) I had a look to see if my weight and FEV1 were correlated and found there is a strong (r=0.64) relationship between my FEV1 & Weight. There is also a very strong relationship (r=0.92) between my FEV1 & FVC.
Amazing China and the return of the CF cough
China was amazing! We visited Beijing and Shanghai, hiked on the great wall on christmas day, visited the terracotta warriors in Xian, hiked on a high mountain with dangerous paths and sheer cliffs, saw pandas in Chengdu, enjoyed 26 hour train journeys with the geckos tour group, partied in Yangshuo for new years eve, rode a bike on crazy streets in Yangshou, hiked on a high mountain through rice fields and ended the trip in Hong Kong and Macau! It was an action packed 4 weeks that was over too soon! I’m already back at uni now and dreaming about future holidays already!
As you can probably tell from the title, not everything went to plan in China. The first two weeks went well. We had two days in Brunei and it was nice to be able to not lose all my salt in a hot & humid place. I had no salt on my skin even though it was about 90% humidity and over 30 degrees. After this the next 2 weeks in China went well, I coped well with the smog, the cold temperatures and the busy days.
According to my pico1 my lung function was about 69-72% before I left. I did my PFTs about once a week whilst away and during the first two weeks I was at 70%. I then had a bad week, we slept overnight in a room that was below zero (the water bottles froze) on the tour and I used a lot of energy keeping warm over these cold days (-15 degrees celsius). There was also a lot of smoke in the restaurants and public areas, plus the smog. I did not sleep fantastically on the overnight trains, and some of the days were quite busy, so I was getting more tired. I also found it hard to have fatty food with the tablet every 12 hours, there was no cheese over there (my normal night time snack with Kalydeco) so I had the tablet with breakfast and dinner. This meant it was a little less than 12 hours and sometimes the meals were not particularly high in fat. I also did not have a nebuliser with me, but I kept up with all my tablets, ventolin and bronchitol.
This meant my CF cough returned for the first time since September (please stop reading now if you do not want to read about sputum colour!). I started coughing up thick green mucus in moderate amounts (but only about 30% of what I used to cough up). I did not feel great, I was quite tired, I was getting more puffed out and also had a blocked nose (also green, first time since September). My FEV1 dropped to 64%. I tried to get more rest to get on top of this but after 3 days I gave in and started cipro. This helped to stop me getting worse but over the next 10 days I still continued to feel like I was not 100%. On one of the days I remember thinking if I was back in Adelaide I almost felt like I was sick enough for IV antibiotics.
I was surprised that my FEV1 only dropped to 64%. Previously when I have felt like that I have dropped much lower, down to the 40s. By the end of the holiday I was a little higher at 65%. Within 3 days of being home, with rest, no smoke/pollution, high fat meals with kalydeco, and pulmozyme nebs I was back at 69%. My cough improved very quickly, I am back to coughing up small amounts that are light green/yellow. I will hopefully continue to improve slightly over the next few days to get back to where I was before China.
I have official PFTs later this week. It is hard to tell exactly how well bronchitol worked for me given that I was in China, so I am not sure whether I prefer pulmozyme or bronchitol at this stage. I need to improve 10% from the start of my one month trial to be able to stay on both bronchitol and pulmozyme (the PBS in Australia only lets you have one of these medications, however there is a program through Pharmaxis where some people can access both if they improve 10% with bronchitol).
Overall it was much easier travelling without a nebuliser and pulmozyme in a fridge, it definitely would not have stayed cold and it actually probably would have frozen in some parts! Kalydeco and Bronchitol made it much easier to travel and also helped my FEV1 stay pretty high despite 2 weeks of feeling unwell! I am now very hopeful that I will be able to recover quickly from colds this year, just with oral and inhaled antibiotics. I am hoping that I will spend very little time in hospital, which will be a nice change from my previous 6-8 weeks of hospital IVs per year. It seems weird to me not to be planning IVs around uni, work and holidays, definitely a nice to change to have more free time and one less (major) thing to schedule in to the calendar. I have a very busy year ahead with uni, so I am looking forward to keeping healthy with Kalydeco 🙂
The difference one year can make
This time last year I only just managed to return home from overseas when I became very unwell. I was very close to being hospitalised in Vietnam, not surprisingly I did everything I could to avoid this option.
It all started when I was travelling on my own in Laos and Vietnam. I was volunteering in Laos, teaching english to children, when I got a very bad chest infection. I had never coughed up more sputum before! There was a lot of smoke in the restaurants and pubs, plus amazing amounts of dust on long bus trips. The result was a pretty bad chest infection. I started cipro but there was only so much this could do.
I was not going to let this stop me from continuing my holiday, so I continued my holiday to Vietnam where I started a tour. Over the two days where I transferred from Laos to Hanoi to Saigon, everything seemed to go wrong. I decided to climb a mountain (called a mountain but really a big hill) on the first day and I lost a lot of salt, whilst I had plenty to drink there was then nothing to drink in the airport waiting area in Laos. I then had all my money stolen by my taxi driver in Hanoi, but luckily I still had all my luggage and medications. My hotel in Hanoi was in the middle of nowhere, but I managed to find some rice and some drinks, nothing more. The next day the ATMs were not working in the airport in Hanoi, so I could not get any money out for drinks when I was very thirsty. I then arrived in Saigon, by now I was dehydrated and had very little food over the last two days. I started to feel quite sick, nauseated, dizzy, and could not eat. I would put a small amount of food in my mouth and swallow it with water, this was the only way to avoid the gag reflex. I was tired, but I could not sleep. I remember being worried that if I did sleep I might not wake up, thats how bad I felt.
I thought this was a combination of my chest infection and dehydration, but I knew I had to go home or I would be in a hospital. I changed my flights and then started on what felt like an extremely long journey. Each plane flight was so difficult, I would get up every 15-20min and walk around as this made me feel less sick & nauseous. I kept counting down every 15min on the clock, and I thought I never wanted to be on a plane ever again. I was very happy when we crossed the coast in northern australia, I knew if something happened to me on the plane that at least I would get to an australian hospital.
I made it back to Adelaide, and went straight to hospital. They did many tests, and discovered my creatinine was 460. Mine is normally around 60 and in a healthy person it is between 50-100. My kidney function was at about 5%. They told me at first it must be an error, they could not believe it was that high. But it was right, and I was in acute renal failure.
My FEV1 had dropped into the 40s for the first time. I spent the next 3 weeks in hospital, gradually my health improved as they gave me fluids and inhaled TOB (no IV tob allowed due to my kidneys). After 3 weeks the creatinine was back to 80. The doctors believed the combination of my dehydration, salt loss and lack of food plus a reaction to pantoprazole was the likely cause of the kidney failure.
I was not allowed to take pantoprazole after this, or take any similar PPIs. This contributed to an increase in reflux and my poor health this year.
If I look at where I am now compared to last year, the difference is astonishing. I am now at 71%, my highest FEV1 in 2.5 years, 5kg heavier, and have a sweat test of 54, which is below the point commonly associated with the diagnosis of CF. For the first time in my life my CFTR channels are working to 50% of normal levels. In biological terms I am returning to homeostasis, with less chronic infection and more stability. Homeostasis means my cell & organ function is sustainable, instead of the decline associated with CF.
A year ago I could not have possibly imagined that I would be this healthy now. For the first time in my life I am looking forward to seeing how my health goes over the next year.
Lets hope my China trip is less eventful than my trip last december 🙂
First dose of Bronchitol
I am digressing from my normal topic of Kalydeco to post about Bronchitol.
Bronchitol is a new CF medication made from mannitol powder that is taken in a small inhaler. Bronchitol promotes the movement of water into the airway secretions by osmosis, which means the mucus is thinner and easier to clear. This works in a similar way to hypertonic saline, which also thins mucus by promoting osmosis.
Bronchitol has been available on the PBS in Australia since August 2012. Bronchitol was developed in Australia and Australia was the first country to approve Bronchitol. It has also been approved by the EMA in Europe, but I am not aware of anyone there taking it yet. It is being reviewed by the FDA. There is also a named patient program overseas, I am aware of at least one person in the US who is on this program. I am not sure about the criteria, here is the link: http://www.pharmaxis.com.au/named-patient-program
Before someone can start Bronchitol in Australia, they have to undergo a challenge where a small dose is taken and FEV1 and oxygen saturation (Sp02) are measured. This is to check that Bronchitol does not have a negative effect on lung function, similar to the first dose testing with hypertonic saline. The normal dose of Bronchitol is 10 capsules, the challenge involves inhaling 1, 2, 3 and 4 capsules with testing in between each amount.
I dont think you are able to take Bronchitol if your FEV1 drops more than 20% (relative not absolute) with the testing and does not recover in 15 minutes. It is also very common for Bronchitol to make you cough straight away as the Bronchitol can irritate your throat/airways, in a similar way to hypertonic saline. I was told that about 9/10 patients that had taken the challenge recently had large coughing fits at the start.
I was expecting to have a massive coughing fit with the first dose as pre Kalydeco my throat used to be easily irritated by pulmozyme and hypertonic saline (I was coughing much more often so my throat was easily irritated). I took the first dose and I noticed that it tasted very sweet and it did not make me cough at all. I then took the next few doses and I did not have an instant irritated cough, but instead there was a moist clearing cough. I cleared that much with one dose of Bronchitol that my FEV1 went up 5% (the average in the trial was a relative improvement of 6.5% or about 120ml).
Prior to the challenge I stopped pulmozyme and hypertonic for a week as I was curious to see what Bronchitol could do on its own, particularly since I am considering just taking this to China. Stopping the meds meant I dropped from 71% to 65%, which tells me that all the treatment I have been doing is very important. I was very excited to see that after no nebulisers for a week, one dose of Bronchitol got me back to my 71% (its 70.5 and I am choosing to round up!!). My lungs felt good and I had no cough for several days after this dose of Bronchitol.
I will start Bronchitol next week and I plan to take this to China. Long term I am hoping that if Bronchitol continues to work well for me, I may only need Kalydeco and Bronchitol. This would mean no nebs! I honestly cannot picture not using a nebuliser! I would still need the nebuliser for periods where I am unwell, but not needing it every day would be amazing!
More Information: http://www.pharmaxis.com.au/bronchitol
http://www.pharmaxis.com.au/assets/pdf/BRO026_Bronchitol_AU_PI_1_HR.pdf http://www.pharmaxis.com.au/assets/pdf/BRO024_Bronchitol_CMI_1_HR.pdf
41% to 71% after 8 weeks of Kalydeco!!
71% after 8 weeks of Kalydeco! I’m still improving slightly 🙂
This is my highest in 2.5 years and now only 4% off my highest in 8 years!
Before I started: FEV1: 1.41 (41%), FVC: 2.48 (62%) & MMEF: 0.52 (14%). Weight 55.6kg.
After 4 weeks: FEV1: 2.36 (70%), FVC 3.16 (79%) & MMEF: 1.71 (47%). Weight 58.5kg.
This means I have now gained exactly one litre of FEV1. I was looking at my water bottle today, a one litre bottle. This is how much more air I can now get out of my lungs in one second. It looks like a very large amount when you look at it that way! I can definitely feel the difference now that I can get air in and out faster, I am not puffed out with walking quickly or with stairs and I am less puffed out with running or sport. I am working hard to further increase my cardiovascular fitness so I can take full advantage of my new pfts.
My highest FEV1 in the last 8 years is 75%. Since 2005 I have been trying very hard to get back to 75% each year. I successfully did this from 2005-2010. I felt stable. I then dropped over the winter in 2010 and suddenly could only get to 65%. I tried very hard to get above 65% and back to my 75%, but I could not. I thought that 10% was lost forever. I then thought I had lost more permanently when I dropped lower this year. Getting to 70% last clinic was amazing, much higher than I ever thought was possible with Kalydeco. I felt like the chance of me getting even higher was slim, I had done so much physio and tried so hard with my pft test that I just didn’t think it was possible. 1% may not seem like much, but it proves I can still improve slightly. The goal that I have had for the last 8 years, 75%, is still my goal for the future 🙂
In other news my liver enzymes were normal before I started Kalydeco and had not changed at the 4 week point (rare potential side effect of Kalydeco). My gastrointestinal doctor was happy with my decrease in reflux (I rated it at about 20% of what it used to be, mostly due to a decrease in coughing). This means the surgery that was being considered, redoing my nissen fundoplication, is no longer needed. I also don’t need my routine pre travel IVs for the first time 🙂
I also noticed something interesting, that after taking my tablet about 3 hours late, I suddenly craved salty foods. I normally eat healthily and do not eat fast food, but I suddenly had a craving for macdonalds! I have noticed that where I used to add salt to my food, I do not want to now. The food tastes too salty if I do!
I’m trying Bronchitol next week as I am hoping that I can take this to China and not take Pulmozyme or Hypertonic. This would mean I would not need a nebuliser pump, which makes travelling backpacker style on trains much easier. I normally travel with Pulmozyme, but due to 26 hour train journeys and unreliable access to a fridge, I am not going to try to keep it cold. I have had problems before in Europe where the Pulmozyme got too hot, turned yellow, and stopped working. Hopefully Bronchitol may make the travel easier, otherwise Hypertonic, Ventolin and good old Cipro (as a back up) will have to do!
3 1/2 weeks until China 😀
7 Weeks on Kalydeco
Thought it was time for another update
My PFTs have been stable at about 70% according to my machine, which is very exciting as I normally would be dropping by now (2 months post IVs). I have also just broken the 60kg mark on the scales so that means I am about 5kg heavier!
My cough has been becoming less productive over the last 3 weeks. I cant remember a time when I have coughed less!
I went 4wdriving and spent lots of time in the sun (cars got stuck on the sand dunes), normally I would have lost heaps of salt and become sick, but I felt fine the next day! I am looking forward to not being restricted in summer- previously I have found it hard to socialise and I have struggled just driving/shopping/running errands when it is above 30 degrees. It has actually become quite restrictive as the summers in Adelaide are very hot, and the salt loss made me feel unwell for several days and often led to bad chest infections.
I generally dont sleep that well and over the last few years I have felt very tired. I have felt tired even with 10 hours of sleep, and I have forced myself to get up. Over the last 3 weeks I have started waking up after 8 hours of sleep and found that I cannot sleep anymore. This seems really weird to me, I am not used to waking up and not feeling tired! I still do get tired throughout the day, but I am hoping this will continue to improve as the inflammation settles and my body becomes more healthy.
I am going to China in December for 4 weeks. The pollution is quite bad in China, so hopefully my lungs will cope better now that I am on Kalydeco. I was not allowed to go on the school trip to China as it was thought that my lungs may not cope with the pollution, so it will be nice to finally make it there!
Before I travel I always have 2 weeks of IVs to maximise my health, particularly given that I can’t get travel insurance that covers CF (can get travel insurance for everything else though). I realised the other day that I had not thought about my normal IVs before travelling to China. It looks like Kalydeco will be the only thing I need before this trip!
Being stable, not declining and not needing IVs like usual really is amazing 😀
magicbluepill 