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.

Tags | lauren | Beth | Robyn | Jo | CF | Perfect Day |
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.

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

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. 

Tags | lauren | Robyn | Pulmozyme | CF |
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?

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. 

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.

The Down’s and Up’s of Clinics.

It’s been 6 weeks since Lauren had her 2 week stay in hospital for a course of IV’s and all has been well, since she was released from klink she has joined a gym and has been going 3 times a week as well as doing all her treatment’s and Physio without fail. As a result she has felt as well as she has been for quite a while. So we were in a positive mood and looking forward to a very straight forward clinic today, especially as Robyn had such a good feedback from her Annual Review 2 weeks ago.

We should have known better after 19 years of fighting this fight against CF!

First up was the Dietician, no real issues with weight and appetite, the only thing that was agreed to do, was for Lauren to get in touch with the Diabetic nurse to see if she can tweek her long acting insulin on an evening because her blood sugar has been a bit high first thing in a morning, and maybe look at having a small amount of Humalog with her supper.

PFT’s next on the list, blow your sock’s off sunshine! The first blow really cleared out a massive amount of gunk, nearly half a sputum pot. So the next 2 blows were done and we sat waiting for the results. Well you could have knocked us over with a feather, a 10% drop down to 43%! WTF is your crafty game CF? I saw something that I don’t see very often and that was a dejected look on Lauren’s face, it made my heart sink even further. We sat and waited for Dr.W to come in and see us, we tried to fathom out the reason for this drop, how could it be? Surely if she was down to this level we would have noticed a 10% drop before now?

Dr.W listened to her chest, which he said had a few crackles, but nothing out of the ordinary. He was happy that Lauren was doing as much as she could to stay well and that she looked in good health. He did remark that he wished all his patients were as good at doing their physio and treatments, and she was a model patient. My heart lifted a little after hearing that. It was agreed that we will go back in 4 weeks and see where her lung function is at then. In the meantime they will contact us if the sputum sample grows anything.

The original plan when Lauren first moved over to adults a year ago was to see if she could get her lung function up to around the 60-65% mark. After having sometime to reflect on the results and discussing it with Lauren we have come to the conclusion that perhaps this might not be achievable and accept that her lung function is at a level of around the 45% mark and she now has to keep up the good work to maintain it. (After all it took 2 weeks of hospitalized IV’s, daily gym workout’s and extra physio just to get it up to 53%). I think that this helped raise the spirits and also letting her choose the music for the journey home. You can’t keep Lauren down for long CF, she is back with more determination than ever to kick your arse! 

On another note, I hope to have some very exciting CF related news about Lauren to pass on very soon, until then we continue to fight the fight until CF is defeated. 

Annual Review Feedback.

On Wednesday we had to take Robyn back to Sheffield for the Feedback from her Annual Review. It was a very early start as we had a 0845am appointment, but I’m pleased to say that Robyn managed to keep awake for the journey, (unlike Lauren who falls asleep as soon as we get to the end of the street!)

Before we went up to the CF Unit we made a quick stop at Growth & Measurement to get up to date height & weight, and the first bit of good news for the day was that since 21st September Robyn has grown 0.6cm and put 0.8kg of weight on. As this visit was being treated as a proper clinic we also went in to get a PFT done, and that is currently at a very good 101.1%. We then went in to see Dr.West to go through the scores. For the last 2 years she has scored 69 points. Robyn’s chest X-Ray’s were first on the agenda, she has shown no significant change in scaring from last years, good news! All the blood test’s came back with nothing unusual to report and on the whole Doc was very pleased with her progress. To top it all off this years score is 71 points! Yes that’s right, an increase of 2 points, a big fat 71. In your face CF. 

We think that this good year is partly due to Robyn being found to have Acid Reflux just after last years Annual review, and the other thing is that she is very good at doing all her treatments. It seem’s weird to think that one little capsule (Omeprazole) can sort out the Reflux and help her put on the weight.

While we were in the CF Unit we also picked up a new mouth piece for Robyn’s I-neb which is the new modified version that improves the time taken in administering the drugs. 

The small square in the bottom left of the mouth piece contains a microchip that tells the main part of the Neb how much of the drug to administer in each inhalation depending on the strength of the patients action. At present the CF team can download info from the Neb but with a new program that the manufacturer is trialling at the moment it will be possible for the patient to download the info to their own PC and the CF team can then access it from the hospital to keep a check that everything is working OK. It will also mean that the patient can see that they are getting the correct dosages and how long the treatments are averaging.

In other news I performed my first Port accessing and flush on Lauren last week. I’m pleased to report that both patient & nurse(me) came through it with flying colours. I have got 2 more to do under supervision from the nurse before I will be doing it solo. This means that we don’t have to travel to Sheffield which is about a 150 mile round trip once a month for the sake of a 10 minute job. 

TOBI Podhaler (TIP)

The new TOBI Podhaler (TIP), has been given EU approval this week which is good news for the CF community. 

Here is the link to Novartis’ website that explains about the drug.

http://www.novartis.com/newsroom/media-releases/en/2010/1446760.shtml

And here is the link to my Posterous blog that shows you the TOBI Podhaler and a short explanation of how it works and what it looks like. Lauren has been using it for a few months now and it really seems to be helping with her lung function as well as being patient friendly.   

http://seanset.posterous.com/tobramycin-inhalation-powder 

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