Cystic Fibrosis
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 🙂
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My FEV1 is stable at around 70%, my highest in 3 years. My BMI has plateaued at 22.
Sweat Test: From 102 to 40!
Two posts in one day! I couldn’t contain my excitement about my sweat test result so here it is:
Before: 102 (Na 110, Cl 102)
1 Month: 54 (Na 60, Cl 54)
7 Months: 40 (Na 49, Cl 40)
This means I am almost below the ‘grey area’ of 40-59! I am still hesitant to call this normal but it pretty much is! I haven’t really changed the food that I’m eating with Kalydeco or the timing, it just seems to have decreased with time. The maximum sweat chloride results were seen at 4-6 months in the phase 3 trial (about 5-7 points lower than at 2 weeks).
Some information about sweat tests:
For people older than 6 months of age, a chloride level of:
Equal to or less than 39Â mmol/L = CF is very unlikely
40 – 59 mmol/L = intermediate means that CF is possible
Greater than or equal to 60Â mmol/L = CF is likely to be diagnosed
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!
2013: With Kalydeco
Cant remember the last time I coughed overnight
Hard to do a sputum sample
Can run up stairs without being puffed out
Have to look into my superannuation
Can travel without a nebuliser
Have to change my diet from ‘CF’Â to ‘normal’
Don’t have to plan around IVs
Don’t have to plan around CF.
Climbing a 2000m high mountain in china, something that Kalydeco made possible!
On the north peak. The path behind me leads up the green dragon ridge to the other peaks. The west peak is the one on the right in the distance.
The green dragon ridge leading to the other peaks. Getting a little nervous at this stage as I knew we had to go up there to get to the other peaks. Luckily it had not snowed yet.
Looking down the green dragon ridge. 1-2km cliff to the left. I could not leave the rail but Keith took some pictures.
Nice safe path to the west peak. A small 1-2km cliff to the left. Plus snow. And a flimsy chain for support.
After visting the south peak and east peak, we headed back down to the north peak.
Why not climb a near vertical ladder in the snow. The lamp is vertical in the background.
Almost back to the start, decided to get some love heart padlocks.
Back to north peak, just in time to get the last cable car down.
Funny story, called mum from a phone to say hi whilst on the mountain and told her to google mount hua shan. Then the phone dropped out. The first thing she sees is worlds most dangerous hiking trail and gets a little worried. I call back and assure her that no we are not doing the optional cliff walk, we decided the normal walk was dangerous enough!
The safety cable/harness is new, even 10 years ago people did it without a safety harness! It was closed due to the snow but there was no way I would be doing that anyway!
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
Pulmozyme V Bronchitol
Before I started the magicbluepill I often read blogs by people on Kalydeco and I was interested in reading about what treatments they needed. I read about people who stopped needing treatments, and wondered if this could happen to me?
Realistically I was not expecting to need no treatment, and as I work as a physiotherapist I would not feel safe doing this. I have noticed that the effect of doing no nebs/physio is not that great now, previously I could become sick and my pfts could drop if I missed treatments. Interestingly right now if I do not do any treatment I barely cough, but with treatment I cough slightly. The treatment helps me to clear very thin, yellow sputum.
Since I started Bronchitol I have stopped Hypertonic Saline. I found the Hypertonic Saline was aggravating my throat (dry irritated cough), and basically wasn’t needed along with both Bronchitol and Pulmozyme. This has meant I have not done PEP recently as I always did PEP connected to the nebuliser with Hypertonic Saline. My lung function has been stable even though I have not been doing any chest physiotherapy for almost 3 months. Just in case my physio is reading this and gasping in disbelief- I will start physio as soon as I notice any changes!
I have now been taking Bronchitol and Pulmozyme for over a month since I got back from China, and the combination seems to be working really well. Bronchitol and Pulmozyme keep my lung function at about 70%, without these I am around 65-67%. I am now getting in to a better routine, this took a bit of adjusting as I have been much busier with uni this year. Both Pulmozyme and Bronchitol help me clear sputum each day, and I’ve started to notice that Bronchitol is possibly helping me to clear more. I have always been a big fan of Pulmozyme as it has worked well for me, so I am surprised that perhaps Bronchitol is now helping more…
So right now my medications consist of Kalydeco, Creon Forte, Azithromycin, Abdeck (vitamin), Ranitidine (reflux, this is much better and I now do not need surgery for this), Pulmozyme and Bronchitol (once a day instead of twice a day- as I am on Kalydeco the second dose doesn’t seem to make much difference). Since I have been on Kalydeco I also have not needed any inhaled or intravenous antibiotics, which is a 15 year record for me!
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 🙂
magicbluepill 