Crushing News

Posted by Juliette

Hi everyone.

We received some bad news some days ago which felt like a huge blow to our hearts and stomachs. To some families living with CF this may be very normal and may, unfortunately for them, happen regularly but for us this is our first time and it made us feel like we were crashing back to earth. That CF is real.

Sammy's cough swab results came back from the lab showing there is a bacteria living inside him. It's called Haemophilus Influenzae. Having a bacterial infection is very bad for Sammy as it could permanently damage areas of his lungs. He is on an antibiotic which is designed to kill this bacteria and we hope so much it will kill the bacteria before it does any damage.

This is a big blow for us as we felt that Sammy had made it to a year (nearly, he's 11 months old) without getting anything more serious then a cold. We have been reassured by the CF nurses that of all the bacterial bugs that could have grown this one is not particularly strong and we are trying to hold onto that thought.

Sammy is doing ok and trying to play despite feeling lousy, he is more cling then usual which is to be expected. We will give him an extended course of the antibiotic used to fight this bacteria and have another cough swab taken in a weeks time. We pray that the Haemophilus Influenzae is gone then.

A little less time

The only non-blurred photo

Posted by Juliette

We have been kept very busy by little Sammy and have not had the time to post a blog recently. Friends keep saying sorry for not keeping up with the blog and ask how we are doing but there has been nothing posted to have kept up with so no one should feel bad. We know lots of you think of us. Thank you for doing so.

So what have we been up to? Well fortunately we have just been kept busy with fairly normal baby things rather then sickness things. Sammy started weaning at 6 months, crawling and using a baby walker at 7 months, has had a bit of fruitless teething and now he is 10 months old he is starting to take his first independent steps! He has also started talking. Can you see why we've been so busy now? We are doing great though.

So about the weaning: it is going extremely well, Sammy just eats and eats. His portions are often bigger then Joseph's. I thought perhaps it was a CF thing but now just think he currently needs a lot of fuel to run his almost constant activity. Sammy and I are doing 'baby led weaning' which essentially means Sammy is given whatever we eat and he self feeds too. Its either something he can hold in his hand like a sandwich or piece of pear or I give him a loaded spoon of things like soup, flaked fish or yoghurt that he puts in his mouth. It's a messy business for several months as he learns exactly how to get the spoon in his mouth without losing its contents on his cheeks and chin but his 'Yoghurt Beard' or sometimes 'Green Pea Soup Beard' is getting smaller as he gets more proficient. You won't catch me steaming, pureeing, freezing, defrosting and reheating food for Sammy, I don't have the time or energy for that so he just eats the same as us and we don't have any fuss over whether something is finely pureed or has the odd lump in it plus it is a delight to watch Sammy turning a piece of food over and over in his hands peering intently as it as he explores the food he is eating.

One major difference with weaning a baby with CF is that for every gram of fat Sammy eats we have to make sure he has the correct dose of enzymes (Creon). This means lots of label reading on packets and calculations at every meal which we're getting the hang of too. (Well we are at home anyway, we haven't braved eating out at friends or restaurants yet - will I have to ask people to measure how much oil they added to this and tell me how many eggs went into that and please show me the packet for the desert? I don't think I can, or if I do we'll never be invited back! No, I have just now decided to ask people the day before we go to tell me what we will be cooking and I'll calculate the fat and Creon ahead of eating there. It's got to be worth a try.)

As a result of his CF, Sammy needs to eat about 50% more fat plus loads of salt and fluids too. I'm waiting for the time when I'm out in a cafe adding salt to Sammy's fatty pizza and some busybody comes and tells me I'm poisoning my child or worse. Don't worry, I'll blog about it when it happens!! I heard from the CF nurse that one mum was so fed up with everyone staring that she just said to all the strangers in the restaurant "My child has a medical condition which means she needs twice as much fat as other kids and a huge amount of salt just to be healthy. I'll happily answer any questions you have if you want to come and ask me but if you don't have any questions then stop --- staring and mind you own --- business" (you fill in the blanks). Hooray for that mum. Another parent told me you just have blinkers on and don't even notice the people around you who watch you and tut or talk about you. I'm cultivating my new thick skin!

Sammy has a milestone of his very own. I know not many other kids can do this. At 10 months Sammy will take a syringe full of medicine and not only put it in his mouth but suck all the medicine out. Should I be proud of this? Half of me is and half of me wishes he'd never seen a syringe. However I can now believe the nurses when they say that 2 year olds swallow Creon capsules!

So Sammy is hitting all his milestones head on and we are running after him just trying to keep up but absolutely loving it.

So I'll leave you with a little mental image. Sammy is walking more and more each day, he loves practising his new skill. His walking style is brilliant to watch, back straight and arms up in the air for balance, then with each step he punches the air with both hands. Its like every step is his entrance to a party. If you can't quite picture it think Tevye in 'Fiddler on the Roof' singing 'If I were a Rich Man' ... Ya ba dibba dibba dibba dibba dibba dibba dum... Yidle-diddle-didle-didle-man.
Got it?

Sammy at CF Clinic

Posted by Juliette

This is a very quick update because all I really wanted to do was add some photos.  We went to CF clinic last Friday.  We saw Sammy's team of professionals: a CF consultant, a CF nurse, a physiotherapist and a dietitian.  Everyone is very happy with him as he is doing great.  No-one is more happy than Mark and me as Sammy is meeting every milestone with enthusiasm and a cheeky grin, he's a joy.  These pictures were taking while we were waiting for Sammy's team to come and see him.  He is sitting in the consultants chair and playing with Daddy.

Wheeeeeeeeeee!

  

PA role: PA to upwardly mobile young man, includes other responsibilities

Posted by Juliette

In one of my old posts I mentioned the many new roles we have had to take on as parents now we have a child with Cystic Fibrosis. I thought I'd elaborate here. I would just like to add before I start that neither of us begrudge doing any of this. The way we see it, if we can do anything to make sure Sammy is as healthy as possible, we do it. As a result of this Sammy is indeed a healthy boy, in fact in some ways he's in better shape than us as he gets good food, daily vitamins, antibiotics and physio to prevent lung infections and as much sleep as he wants. We on the other hand don't eat so well, forget to take any vitamins and are sleep deprived because it turns out that Sammy doesn't want as much sleep as we'd like him to have, nor does he choose to take it in the middle of the night! 

So let's see what extra things we do in addition to being a parent. 

Physiotherapist: From the very beginning Mark and I were taught how to give physiotherapy. On a normal day we give 5 minutes twice a day. If Sammy is ill we do more physio each day. I think the amount of physio will increase when he's bigger as his lungs will be bigger. 

My P.A. role: We go to clinic every 3 weeks so I make sure there is either someone to take or collect Joseph from school or request an appointment where I can do the school runs and go to the clinic. Mark often takes Leave from work and comes too, but if not, I might organise that someone comes with me. 
 
As Sammy is on several medications, I make sure we always have stock of these, order more from the GP, collect prescriptions, take them to the pharmacy and collect the medicines. Sometimes I warn the pharmacist about a prescription so that they can order it in for him. I used to be all over the place with this and would be at the GPs every other week or more requesting something I hadn't realised was going to run out. Now I've sorted it so there is a monthly prescription. 
 
Sometimes there are questions for the CF team in between clinic dates. I email them and we often have to change medication, doses or physio. 

Nurse: I nearly forgot this one, it's become so normal that I forgot it's not a normal parent role. We prepare and give at least 19 doses of medicine a day. Most are given by oral syringe but the ones that are granules are given on a spoon with apple puree. 

 
Pharmacist: Once a week we take a bottle of powdered antibiotic and reconstitute it with the precise amount of cooled boiled water. 

Dietician: This is a new addition to our roles as we have started weaning Sammy. We have to work out the dose of an enzyme called creon for every meal Sammy eats. There is more about this in my last post. On the move

Well 5 extra roles! Of course I'm not saying we do any of these things anything like the professionals, just that we have taken on a little bit of all of these roles. As a result though Sammy is really thriving and does everything full pace. 

A special thank you has to go to our wonderful team of real CF professionals and our pharmacists for teaching us how to do a little bit of what they do. 

On the move

Posted by Juliette

It looks like Mark and I have fallen down that common bloggers' pitfall of not blogging when things in life are good. Well to rectify that, here is an update on how we are all getting on. 

Mark is very busy at work and busy helping and joining in with us when he is home; Joseph is loving school and we are loving that he's learning to read; I'm busy at home but finding my feet more and more in our CF and day to day routine - the CF specialist nurse even told me I was 'very organised'! I nearly fell off my seat and wished all my ex bosses had heard that one; and Sammy, well Sammy must be the busiest of us all. He's growing very well and is continuing to put on good weight and length which will help him fight infections in the future and is keeping him firmly on the centile chart. He is currently infection free having fought off a horrid cough last month. When Sammy gets ill (under the direction of our CF team) we immediately change his antibiotic from his regular Flucloxacillin to a rescue course of Co-Amoxiclav and increase his physio to 3 or 4 times a day and for double the duration. This time we did this for 3 weeks and it cleared up the cough beautifully and prevented the virus from developing into a bacterial infection in Sammy's lungs. Preventing bacterial infections is what it's all about! The BIG NEWS is that Sammy at 7 months is crawling, pulling himself up, cruising round the sofas on his feet and if you hold his hands he walks everywhere. HE'S MOBILE and he is also learning the word NO! He frequently crawls towards the radiators, wires, toilet or other hazards we'd rather he stayed clear of. He initially turned and laughed at Mark the first few times he shouted NO because he had never heard the tone Mark was using. We think/hope he's getting it now though. 

Amusingly Sammy managed to lock Mark in the shower one day whilst Joseph and I were out. While Mark was in the shower cubicle Sammy crawled over to take a look and put his little hands on the bottom of the shower door in such a way that Mark couldn't open it without crushing his fingers. Mark started shouting 'NO!', 'SAMMY, GO TO OVER THERE!' and anything he could think of to persuade him to move but Mark probably became more interesting and intriguing to Sammy at this point. Eventually Mark realised he had to wait for little Sammy to get bored and move away on his own. He finally did, at least the shower water remained hot the whole time. 

The other great new thing in Sammy's life is food. As with Joseph we are doing baby-led weaning. Sammy has taken to it well and likes a big variety of food, especially baked beans and Greek yoghurt (though not together - but maybe we'll give it a try). He is also very good at spooning food into his mouth. I, on the other hand, am perhaps not doing quite as well. You may remember from other posts that Sammy needs to take an enzyme called creon every time he eats something with fat in it. This means I have to work out how much creon to give for a food depending on it's fat content. For example 50ml of whole milk and 1/2 a Weetabix contains about 2g of fat so I give 3/4 of a scoop of creon granules. (2.5-3g fat = 1 scoop) Sound complicated? Try working out the fat content for tuna mayonnaise (tuna = 6.8g for half a tin, mayo = 10.2g for 15ml) now work out how much fat is in the 6 spoons Sammy actually ate and then work out the creon dose for that!! Don't worry you don't have to do this, Phew! And I'm getting the hang of it :-)
 
So to round up, we are all fine, busy but having lots of fun oh and there is food all over Sammy, me and the floor at all meal times but thank goodness for wipes and washing machines. 

Camp it up. The louder the better!

Posted by Juliette

So I thought I would blog about the fun we all have when we give Sammy his physiotherapy. It might sound a bit odd to you that it is fun as perhaps you will see it as one of a number of chores we have to do to help Sammy stay one step ahead of his CF but if we saw it like that then for one, we would be miserable and two, Sammy could grow to see it like that.

 Therefore as Sammy will/may need physio for the rest of his life we choose to make it fun and in this post I want to explain why and how we give Sammy his physio and then how we camp it up!*

WHY?
Everybody has mucus in their lungs, it's there to help protect the airways. The problem CF causes is that the mucus is much thicker and it therefore doesn't move around in the same way making it difficult to shift and cough up. An added danger is that it is a warm, moist, sticky environment, so bacteria love it, therefore any one with CF has an increased risk of developing chest infections.

So what? you may think. Everyone gets chest infections and they just get on with it, don’t they? But this this the depressing thing; with CF, each chest infection has the potential to kill off some lung tissue. Once dead this tissue will never recover resulting in reduced lung capacity and function. Every infection has the potential to cause this irreversible damage and low lung capacity and function will either effect quality of life or will reach a state where the amount of functioning lung is not even compatible with life itself. Yes you know what I'm saying. I don't want to actually put it into hard text because I don't want to read it back. 

So I guess if you didn't already understand why I'm obsessive about trying to keep Sammy free from colds and infections, you do now.

Right, sorry if I brought you right down; let me pick you back up again. We do two main things to help Sammy clear the thick mucus from his lungs and stay free from infections. We give him an antibiotic every day to help prevent bacteria from growing in his lungs and we give him physiotherapy. The physio is normally given twice a day for five minute at a time. We increase the amount we give if he has any sign of an infection. Currently he has a cold (Panic! Panic!... No he's doing fine) so we do two lots of 10 minutes until the infection has gone. We can do physio up to four times a day if necessary. The physio helps to move and clear the mucus and when he has a cold there is more mucus to clear, hence more physio. 

HOW?
There are a number of different ways to do physiotherapy for someone with CF but currently we do percussion, also known as patting. Traditionally this is done using cupped hands but on a baby this is quite tricky so we use a small rubber cup (which is actually a paediatric oxygen mask) to pat Sammy firmly on his chest and back. We have discovered that Sammy loves it if we do it to music and it is the choice of music which makes it so much fun. Mark has made a playlist of largely fast tempo, upbeat tracks on his phone and we sing along and jump about whilst patting. I only hope Mark's terrible song choices don't rub off on Sammy as he develops a musical taste of his own. 

 Mark’s favourite songs for patting include:

Waterloo - Abba
Laura - Scissor Sisters
9 to 5 - Dolly Parton
Brown Girl in the Ring/By the Rivers of Babylon - Boney M
Rocket Man - Elton John. 

My new guilty favourites chosen from Mark's camper than camp playlist: 

Call Me Maybe - Carly Rae Jepsen,
Price Tag - Jessie J
The Clapping Song - Shirley Ellis. 

Have any of these song choices put a smile on your face? If so you can see why Sammy loves it, and his face really does light up when the music comes on. So which one is your ear worm for today? 

*You may notice that I couldn’t decide between writing that Sammy will need physio for the rest of his life, or that he may need it. As things stand right now he will. But we are hopeful that with enough money and research, scientists will be able to find a cure, or at least effective treatment that will allow a full and rich life expectancy and remove the need for physiotherapy. Such a treatment is already available for those people with the rare G551D mutation, so something to help people like Sammy is not out of reach. In case you haven’t spotted it, this is just my cheeky little plug to ask you to sponsor Mark who is running half a marathon for the Cystic Fibrosis Trust as they fund the research. – As always, our sincere thanks to those who have already donated.
http://uk.virginmoneygiving.com/markleecarter

U'ntaneh Tokef

Posted by Mark

To our non-Jewish readers, the title of this post probably reads like a random hitting of the keyboard. Jewish readers however, may well recognise the name of one of the most poignant and powerful parts of the Rosh Hashana (New Year) and Yom Kippur (The Day of Atonement) synagogue service. It is a beautiful piyyut (Liturgical poem) and has always managed to move me but I think even more so this year following Samuel's diagnosis. In particular, the central part which can be translated as follows:

On Rosh Hashana it is written and on Yom Kippur it is sealed: How many shall pass away and how many shall be born; who shall live and who shall die - who at the full length of his days and who before; who by fire and who by the sword. Who by wild beast and who by hunger ... who will enjoy tranquillity and who will suffer; who will become poor and who will become rich; who will fall and who will rise.

It is pretty powerful stuff (much more so in the original Hebrew) and whether or not you believe that such things are somehow 'written' and whether or not you believe the piyyut's hopeful exhortation that in spite of this predestination:

Repentance, Prayer and Charity avert the severe decree

It is nonetheless a deeply moving reminder that so much of the future is beyond our control. I always look around me at this point in the service, trying to really grasp and acknowledge that although we are here today, there is no way of knowing who will be missing when the words are repeated in services next year. Those who appear ill and frail may well be amongst us while those who seem the strongest and seem to have years ahead of them may no longer be here. Each year there are a few new faces from new people who have joined the synagogue, but there are also faces that should be there but just aren't any more.

This year I was also particularly aware of the parents sitting with their children who, when Juliette and I first joined the synagogue, were little kids and now are grown up. I thought of Joseph and Sammy and how much I hope that in many years to come they will sit with us, grown taller and stronger than us, and wonder how it was that they grew up so quickly.

But at the same time I know that this picture may not come true. Juliette and I both spoke about how hard it will be if one day we will have to go to Rosh Hashanna or Yom Kippur services and Samuel is no longer with us. But I suppose one of the points of the U'ntaneh Tokef piyyut is to remind us that we just do not know what tomorrow holds for any of us and we should be mindful of this fact. Yes it is true that Samuel has a life-shortening condition but there are some people with CF who have lived well into old age, and who is to say he won't be one of those? And who knows what advances will be made in research and treatment next year and the year after that and the year after that? And even without CF, how do we know who will die at the full length of his days and who before?

Here is hoping that we will all be inscribed in the book of life, blessing and peace for the coming year.

P.S. We have decided that Samuel should become a 'Baal Tokea'. One of the highlights of this time of year is the blowing of the shofar  (Ram's horn) during services. It needs a lot of puff to blow it well and a good Baal Tokea (person who blows the shofar) needs to develop good lung capacity to do it well so practising the shofar should be good for Sammy's lungs - although what bacteria could be lurking in a ram's horn probably doesn't bear thinking about!



UPDATE:
I just saw this awesome video on popchassid.com of a baal tokea blowing a shofar in all sorts of crazy places across Israel! Enjoy, and a Happy New Year!