Seanset

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[seanset]

My bike is CFAware

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Wearing 2 bands for CF Awareness month.

[ebfayth]

WATCH THIS »» http://pdl.vimeocdn.com/39215/933/153600660.mp4?token=1365516417_fb1b7f33371556a16c70fba5c1797b50

Not enough people are educated about this life threatening condition… Please help me spread the word by reblogging this.

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Lauren's Kalydeco has arrived. Happy days!

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Uploading data

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Modelling the new cfaware wrist bands

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Kalydeco!

A very special moment for us, the first dose of Kalydeco for Robyn and hopefully it shouldn't be too long until Lauren gets hers as well.

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I-neb Insight Online

Robyn has been using the I-neb for about 6 years now to neb various medicines including Pulmozyme, Tobi & Promixin. One of the features of the I-neb is that the hospital could download data from it to monitor the treatments to make sure the correct dosage is being administered, but they could only do this when you attended clinic in Sheffield, which for us is probably only 1 or 2 times a year.

Phillips Respironics have now developed the software to allow the patient to download the data to your own laptop and then upload that data to a website that the hospital and themselves can access. You can do this as often as you like, we will probably do it every 2 weeks. I guess during times that PFT's are down they will probably want the data more regularly. 

The above photo is of the treatment view tab and shows I-neb use, nebulization time and daily nebulizations both in graph form and also as a %. So no way of hiding missed treatments from your CF team!

This second photo is of the Devices View tab. In this view it basically shows mesh performance, Inhalation time and rest time. Philips monitor the mesh performance and can see when and if the mesh needs changing which is good especially with using Tobi through the neb that is sticky. The other 2 graphs that show Inhalation and rest times can help highlight potential oncoming infections, i.e. If the Inhalation time comes down and the rest time goes up.

The first download is scheduled for tomorrow and it will be interesting to see the results.

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Newspaper Articles.

I thought I would post 2 articles where the girls have featured in the press. 

http://www.thestar.co.uk/news/local/we-applaud-our-readers-1-4055003

http://www.thisisgrimsby.co.uk/Inhaler-breathing-new-life-futures/story-13520146-detail/story.html

[seanset]

'Tis the Season to do IV's!

Yes we're into that time of the year that the CF community hates! And we as a family are no different.

The first signs started about 3 weeks ago for Robyn at a routine clinic appointment in Grimsby with the Sheffield CF team, she normally blows a good 100% with no trouble, but that day she could only manage 89%!! Que puzzled looks from me and Jo, there hadn't been any signs to justify this result. I mean there hadn't been a cough or wheezing, no loss of appetite and still very active.

The Doctor listened to her chest, took a cough swab and it was decided to put her on oral antibiotics return in 10 days to see Dr.Samy to do another blow and repeat the cough swab. The first cough swab came back all clear and we returned to see Dr.Samy and repeat a blow, again it was only at the same level as before.

So for only the second time in over 12 years it was decided to go for IV's to nip any potential infection that could be brewing in the bud. Last Tuesday we arrived at hospital for Dr.Samy to put a long line in Robyn's arm, she was very nervous but with the help of some gas & air and a Doctor who is excellent at doing the procedure it was in in less than 5 minutes. 

Fast forward to now, Sunday, and our home IV auto pilot function has kicked in. I guess doing them for over 20 years helps in a way, although it's something I would rather not have on my CV. I swear I could draw the drugs up blindfolded just like a scene from a movie where the soldier strips his weapon down and reassembles it!

The good thing is although we couldn't initially see anything wrong with Robyn there does seem to be an improvement in her general well being and of course she is just like her big sister and taking it all in her stride.

We wish you all a Merry Christmas and Happy New Year.

[seanset]

Were These My Perfect Days?

On Thursday I spent the day painting the bathroom and a good portion of Friday tiling, perfect! Now some of you might be thinking I've been at the cooking wine and been influenced by a certain madman who lives in LaLa land, "how can that be classed as a perfect day"? I hear you say. I'll explain my reasoning.

On Wednesday Lauren came home after spending 10 days in hospital trying to get to the bottom of her drop in lung function. The hospital is in Sheffield which is about a 75 minute drive from our home so it can take a lot of planning when we visit, it's not just the journey we have to consider it's also having to organise for Robyn getting her nebs and medication before she goes to school and also in the afternoon when she gets home from school, and making sure that both her and Beth get dinner and some attention from both myself and Jo.

This is where I'm so lucky to have a brilliant wife who is so organised, (I'm sure she was a military planner for the Roman Empire in a previous life!)  Because Jo also works and doesn't drive I usually go through to see Lauren on my days off while she holds the fort at home. Robyn had an appointment in Sheffield on Tuesday at the Children's hospital, so Jo had the day off work and came through with me, we went to see Lauren first and then went back after Robyn's appointment and took her out for dinner. 

So for a family who don't have to deal with all the ups and downs that living with CF brings might not agree with my idea of a perfect day, but just to do the normal things mean's that we are winning the battle against CF. To quote Josh from Joshland, 'CF will not win, because CF is dumb!'

Work in progress.

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One step forward two steps back. Again!

You would think after living with CF in our lives I would learn not to brag and tweet about good things that happen to the girls because it has a habit of coming back to bite you!

It all started nearly a fortnight ago with Robyn's annual review feedback clinic and Lauren finishing a routine course of IV's. Dr.West was very happy with how Robyn had progressed though the year, her PFT's had been at a constant level around the 100% mark. Her Chest x-ray hadn't significantly changed from last year and Dr.West even took the time to explain to Robyn all about it because she has started to ask questions about her treatments and how CF affects her. The bloods showed her Pseudomonas antibodies were a little high but because she hadn't actually grown anything for a long time it was decided to leave her on Promixin. All the good results meant that her score had gone up 8% to 79% from the previous year. Dr.West even talked to us a little bit about the potential of the VX-770 drug because the girls have the G551D gene mutation. So we left the hospital with a big smile and I even tweeted the good news of how well Robyn was doing.

Two days later a nurse from the Northern General came across to do a post IV's PFT for Lauren, now this is where we start to take two steps back. Her blows had gone down to 39% from 44% pre IV's! The nurse said she would report back to the doctors and they would be in touch, later that day they rang and said that they wanted Lauren to go in on the Monday for a week of intensive physio to clear some of the gunk from her chest. We arrived about lunchtime on the Monday and found that the plan had changed, 2 more weeks of IV's but on a different combination of drugs. They have also decided to start her on Promixin on her off month and also to start Hypotonic saline 7%, they are also changing her from her eFlow to an I-neb, the plan is for her to do another PFT on Monday and then hopefully be let home to finish the IV's.

Later on the Monday afternoon Jo rang me to say that the Children's Hospital had rung to say that Robyn had grown PA from the cough swab she had done at clinic and they were prescribing 3 months of Cipro. Friday of this week they rang again to say that further tests now showed that the PA was resistant to Promixin, seriously how many steps back are we going to be punished for one little tweet? We now have to take Robyn across to Sheffield on Tuesday so they can start her on Tobi, as she's never had it before they want to do a pre and post PFT to check that she doesn't have a reaction. So that's been our last 2 weeks but at least I've been off for most of the week to keep Lauren company, even if we have both fallen asleep in the afternoon a couple of times.

Hey Beth! let's see how many different positions I can get this bed into?

The only good thing about Lauren being in this week is that she gets to stay in the 5* CF Hilton ward, I think she's had at least one massage and a few goes in the private CF members only gym on the ward. They have got a VIP guest going on Monday to officially open the ward so I'm sure she'll tweet something about it if she's still there.

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Lauren demonstrating how easy it is when she uses the Tobi Podhaler. 

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Running on the Spot!

Even after nearly 20 years of living with CF in our lives I still can't help but feel totally helpless and useless sometimes when one of the girls is not feeling very well, the past couple of days both of them haven't been at their best so this has only served to multiply these feelings. It's like the bad dream you have when you are running to help someone but you don't go anywhere, you just stay where you are Running on the Spot! 

Lauren started complaining of a small headache on Thursday night that seemed to still be there on Friday morning. After a big coughing fit, physio, more coughing, treatments, more coughing and a little bit more physio she looked absolutely knackered but she assured me & Jo that she was ok to still go on her driving lesson. When she returned from the lesson she said she was starting to feel a little better and would be alright to come into town with us, I think it was because Dad was buying drinks and cake that helped her feel better.

We left Lauren in town with Beth and went to pick Robyn up from school, by the time they came home the headache and coughing were back stronger than ever and to top it all off Robyn had started with a sore throat. I was keeping my fingers crossed that we didn't get any blood streaks every time a cough came on. For the rest of the evening Lauren just became so lifeless laying there on the sofa, she was even talking about cancelling her night out on Saturday which isn't like her at all. CF had reared his ugly head again from where he was hiding and was giving us a good working over.

Well CF I'm afraid you'll have to get up pretty early to defeat us Setterfields! FUCF!

Saturday morning arrived with a better looking Lauren, some extra doses of Pulmozyme and a little bit of extra special manual percussion from Jo had seemed to have done the trick. So it looks like the anticipated call to Sheffield on Monday has gone on hold for now, Robyn has still got a little bit of a sore throat but with some doses of cough medicine it should go without the need of any Oral Antibiotics. Lauren has even managed a full day at work and has now gone out with Matt and their mates for a few drinks. 

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Another Milestone.

One of the biggest worries for us when Lauren transferred over to adult CF services in Sheffield was about how we would get her Port flushed, we found ourselves in this position because Grimsby doesn't have any adult CF services. Lauren had always got it done at home by one of the children's community nurses from the local hospital in Grimsby. 

Next Thursday will be another milestone in our journey living with CF. I will be performing my last supervised Port Flushing on Lauren, thanks to our brilliant Community Nurse who has taught me and above all else given me the confidence to stick a large needle into my daughter. It now means that we don't have to do a 150 mile round trip to the clinic in Sheffield for them to do the flush which takes about 10 minutes.

I must just add that of course none of this could happen without the cooperation of Lauren who was willing to let me do it. She has always been very good with needles right from an early age, even having Long Lines inserted without any numbing cream. I can remember one time when she was about 4 yrs old and we were in the treatment room waiting for the Doctor to put a line in, the nurse was getting a blanket ready to wrap Lauren up so she wouldn't wriggle while the procedure was happening, I told her that it wouldn't be necessary and she would be quite alright sat on my knee. Lauren sat very still and just watched the Doctor do his stuff without so much as a flinch, the nurse couldn't believe her eyes! She's one tough cookie our Lozzy. 

I wonder what the next milestone will be?

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Time For an Update.

It's been a month since my last post, Wow!, where did that time go to? This is really just a post to update on what has been happening over the last few weeks with the family.

I suppose the first place to start would be with Robyn, she is continuing to grow and put on weight at a good rate. Around Boxing day she started to develop a small cough, nothing major just enough for me and Jo to start noticing, with Lauren being that much older she has a full on choking cough and we sometimes don't notice if  Robyn is coughing because we are so used to hearing Lauren's, and hers can sometimes mask other peoples. By New Years Eve it still hadn't gone so we decided to give Grimsby community nurses a call on the Tuesday after the bank holiday to see if we could take a cough swab in just as a precaution, we keep some at the house just to save time. We got a call back on the Friday evening to say that all was clear and no need for any Oral Antibiotics. A good start to the new year, I guess that doing Promixin twice a day has really helped to keep the PA at bay, along with her regular twice daily physio using the PEP. Over the last year she has only grown Pseudomonas twice and Staphylococcus on one occasion, although her bloods do show a raised antibody level to Pseudomonas.

So with everything going great with Robyn we then had to turn our attentions to Lauren who had another clinic on Wednesday. Just to recap where Lauren was before Christmas and after the last clinic, they did some bloods to check her HBA1c levels for her CFRD, plus her Aspergillus levels, she was also put on a 2 week course of Cipro as a precaution. The HBA1c level has dropped slightly from 10.0 to 9.6, it's still a long way from where it should be around 7.0, but the Doctors think that this could take a few months with Lauren just changing insulin and starting to count her Carbs. The Aspergillus levels where increased from the last test but not at a level that they would start treating with steroids. And so we came to the PFT's, last clinic they had fallen 2% to 41%, over the years sat watching and listening to Lauren doing these tests I have come to be able to judge when she has done a good set of blow's or not, I was convinced this time that she had put in a good set but as always you sit on the edge of your seat while the machine does it's calculations, they came out at a massive 50%, WOW! a 9% increase. The combination of 2 weeks of Cipro, starting her month on of TIP and a reduced HBA1c has seemed to have had a good effect.

Last on the list but by no means least we come to Beth, she has been working hard at college and has just taken some exams in English, Psychology and Business that go towards her final A levels, fingers crossed that everything has gone OK with them. She has also started a part-time job at a small frozen food shop in Cleethorpes which keeps her busy a couple of nights and on weekends and also gives her some money in her pocket, that is the best part for Dad. :-)

This months refill of medicine has made us realise that we need to clear out another draw in the kitchen, we have now separated all of Lauren & Robyn's meds, there is now 5 draws, 3 cupboards and a large portion of the fridge all solely used for the drugs. More room was taken up in the fridge by 4 months supply of Pulmozyme that we got on Wednesday from Lauren's clinic, we were only supposed to get 2 months but the Doctor couldn't of read the notes properly and thought she was taking it twice a day! Ah well just saves us worrying about it for a while.

Anyway that's all for now and I would just like to wish you all a happy and healthy New Year. 

[seanset]

No IV's For Christmas This Year!

After Lauren's last clinic 5 weeks ago there was talk of the possibility of having a 2 week course of IV's over Christmas, it was partly dependant on what if anything was grown from her sputum sample and what her PFT's were today. Well nothing has grown but there has been a 3% drop to 40% in Lung Function. This could be due to a combination of her being on her off month of TIP's and also a slight head cold a couple of weeks ago. There has also been a drop in weight but that could be to do with her starting her regime of Gym work and also a change in Insulin and dosages. Anyway the Doc was happy that her chest was clear and said that there was no need to have a course of IV's, he has although prescribed a 2 week course of CIPRO just in case there is something brewing down in the depths of her lungs.

After having Robyn on IV's for the first time last Christmas this was a huge relief for us, as well as Lauren, she's not to happy about not being able to have a few Christmas drinks though!

He also decided to do some blood tests, the first was to check her current HBA1c level, Lauren's HBA1c level has for some time baffled the medics as to why it is so high when her daily blood monitoring levels are on the whole fine. The other blood test was to check her Aspergillus levels, this bug first showed itself about 6 years ago, after 2 courses of IV's in short succession with no improvement in PFT's they tested for the Aspergillus bug and found her levels to be sky high, after a short treatment of steroids her lung function returned back to it's usual level. The Doc spoke about possibly starting her on steroids again if her levels are increased but not necessarily at a level that they would usually treat the bug at, he seemed to think that it could be another factor in the reduced lung function. The only downside for me was that because Lauren is due for a Port Flush they decided to do it today so they could draw the blood through it, this means I will have to wait another month to do my final supervised Port accessing and flushing, but after that I will be able to do them myself unsupervised.

Lauren has also agreed today to take part in a study that will look at the Symptoms and Measurement of Reflux in Adults with CF, it is a very straight forward study that only involves providing 4 sputum samples at various times during the day. These samples will be tested for Pepsin, which is found in contents of the stomach and will help to detect reflux. The normal way of testing for Reflux has always been with a PH test which involves having a tube down your nose into your stomach for 24 hours and measuring the acid levels, but apparently this doesn't always work with 100% accuracy. The purpose of the study is to identify people that don't have the symptoms of reflux so that they can treat it with either Medication or a small operation which will ultimately stop the reflux acid from reaching the lungs and damaging them. It will be interesting to see what Lauren's results will be like because she had a PH test done just before leaving the care of the Children's CF team that showed a negative, Robyn had the PH test done at the same time and was shown to have Reflux. Since she has started on OMEPRAZOLE to combat the Reflux she has started putting weight on and has grown at a good rate.