Harriet’s Seven Month Update

I can’t believe Harriet is seven months already. The time really has passed so quickly despite everything that has happened.

At the moment she is doing well. We had one hospital appointment this month with her surgeon and he was so pleased with Harriet that he discharged us, which is fantastic.

Nancy had a heavy cold so it was inevitable that Harriet would pick it up too. In all honesty, Nancy has probably suffered the most. I wish I had a pound for every wail of ‘maaaaam I have a snotty nose!’ Harriet just had a cough which, at its peak, was awful. It was only one night but she literally coughed none stop, no break for hours. It was awful but she was on extra antibiotics which helped it to clear. I have to keep reminding myself that all babies will have approximately ten colds in their first year and not everything is related to Harriet having cystic fibrosis.

We have really got going with weaning now. I was reluctant to go too fast too soon after her surgery but I feel confident that she can try most things now. I dug out the old, slop splattered Annabel Karmel and set about batch cooking to fill the freezer as I used to with my older daughter. The only difference now is I added some butter into vegetable purees for extra calories and some salt. After some reading I bought some Himalayan pink salt as it is said to have lots more health benefits than just normal table salt. If you’re going to have it you might as well get the most out of it you can! I purchased a large bag online but only a short while later both Aldi and Lidl started selling it at a fraction of the cost. Typical!

Our dietician did a home visit to discuss creon doses in relation to the fat content in food. Not going to lie, it all seems a little complicated right now but as with everything along this journey, I know it will become second nature at some point. Harriet now weighs sixteen pounds and ten ounces, and looking through Nancy’s baby book she weighed seventeen pounds at this stage so all things considered I am over the moon with that!

This month there has been some exciting new developments in the cystic fibrosis field. First up is a drug called Orkambi which is specifically for Harriet’s mutation of DdF508. I have been reading about this for a long time but to see it hit media spots like the BBC just made it seem all so official. The combination drug is the first to actually address the underlying cause of CF rather than just treating its symptoms so it is groundbreaking. I have spent way too much time watching patient testimonies at the conference in America who trialled this drug and it really is emotional. Results were said to be disappointing at first but at least it is something. It is a start. The way I see it is that these huge pharmaceutical companies are only going to keep on making improvements. The CF Trust are announcing the results of Gene Therapy next weekend. There is quite a buzz around this and I have signed up to watch it live online – sad I know! Maybe sometime soon I won’t be Googling ‘cure for cystic fibrosis’ at 3am.

Development wise Harriet is almost sitting up unaided. She is getting stronger and stronger everyday and loves to sit propped up with cushions or people – see above! Sleep wise she still naps twice a day, morning and afternoon, then does another twelve hours at night – the dream! We bought her a walker which she loves – well, she is such a happy little thing that there’s not a lot she doesn’t like.

Fingers crossed for some warmer weather – a hot summer would be so beneficial for Harriet and hopefully she will have built up her immune system by next winter!

Cystic Fibrosis FAQ’s

May is cystic fibrosis awareness month so I thought I would answer some of the questions we have been asked recently. They do range from fairly sensible to completely ridiculous but to be fair we knew very little about the condition before our daughter’s diagnosis so any question is a valid one!  I am still learning every day so apologies to any CF experts; please correct me where needed.

The questions are a mixture of what we have asked our CF team and what others have asked us. Some will be directly related to Harriet and the rest applicable to the cystic fibrosis community in general.

What is cystic fibrosis?

Most people believe that CF is all to do with the lungs and a build up of thick, sticky mucus. This is true but is only one part of the condition as it affects the digestive system and other parts of the body too.

How do people get cystic fibrosis?

Cystic fibrosis is an inherited genetic condition. One in twenty five people are unaware that they are carriers of the faulty gene. My husband and I both carry the most common type which affects around eighty percent of CF patients. Harriet has two copies of Delta F508, and there are thousands of other gene types for cystic fibrosis and the discovery of this came not so long ago. Because of this, our families now have the option of having their own genetics tested if they want to find out their carrier status.

If we have another baby what are the chances of passing on cystic fibrosis?

There is a one in four chance. One in four that the child will have CF, two in four that the child would be a carrier like my husband and I, then a one in four chance the child would be free of any faulty gene. Our eldest daughter will have the option when she is older to have her carrier status checked. We have been offered IVF should we decide to have another baby but we are still unsure. If I could skip the pregnancy part I would have a house full!

Are there different types of cystic fibrosis?

There are different gene types but CF is a very individual disease. The picture below shows how the cells do or don’t work for the various mutations. As you can see, Harriet has a class two mutation. Class one being the worst through to five being the least severe. A sufferer has to inherit two copies of a CF gene but they can come in any combination. The majority of people with CF have the same mutation as Harriet but even if they have the same type their symptoms can wildly differ. I have often asked if there are any indicators symptom wise to tell us how Harriet will be affected, but apparently there is not. For example, Harriet was born with meconium ileus so I asked whether she will now be prone to bowel issues but seemingly no. Our CF team explained that they have seen babies have bowel surgery then go on to no future issues. In contrast they have had children not born with a blocked bowel and go on to have digestive problems so it is all very unknown.

How is cystic fibrosis treated?

At the moment treatment is mostly preventative. Patients are seen regularly at clinics where cough swabs and bloods are taken to make sure there is no signs of infection. Harriet is on daily medication for life which includes antibiotics, sometimes a few different types, vitamins, pancreatic enzymes, salt replacement, medicines for her liver and stomach function. Harriet also has daily percussion physiotherapy to help move the mucus clogging her lungs. She has two twenty minute sessions a day at present.

What is the current life expectancy for a person with cystic fibrosis?

Currently around forty years old although I read a different figure almost on a daily basis. The way we look at it is that over the last twenty years life expectancy has gone up dramatically and it’s only going to continue on that upward trend.

What does the future hold in terms of a cure for cystic fibrosis?

There are so many exciting developments at the moment and we are very positive for Harriet’s future. There is a wonder drug available to patients with a class three mutation and the company who produce this are launching a similar drug for Harriet’s mutation soon. It hasn’t proved to be as effective but they will continue to make improvements. There have been other breakthroughs in terms of potential cures but funding is needed to further the research.

Does Harriet need any further operations?

No. Hopefully her bowel issues are all over now and she will not require any further surgery. Only one in ten babies with cystic fibrosis are born with meconium ileus so most patients will not need any operations. Sometimes as adults a cystic fibrosis sufferer may require a lung transplant but that is something I can’t think about at this stage of our journey even though there are some amazing positive stories out there.

What can/can’t Harriet do?

There are very little restrictions – Harriet is just like any other child. There are precautions we can take e.g. not letting her come into close contact with people who have bad colds and she shouldn’t be around stagnant water but other than that she is fine. Exercise and keeping fit will be massively important for her to keep her healthy.

Will she be able to walk?

A valid question we were asked and the answer is yes. Cystic fibrosis affects a persons internal organs and there are no outward signs of illness.

And finally, my personal favourite…

Can you have a real Christmas tree?!

This one was asked by me to our consultant as I had read online that we shouldn’t get one because of spores the tree would give off. Needless to say it got a laugh and we did get a real tree!

If you have any questions about cystic fibrosis please ask and I will do my best to answer them for you!





Bonus Blog!

Just a quick one for those living locally. We are holding a quiz night at St. Matthew’s Church Hall on Friday 5th June 2015 with all proceeds going to The Stuart Gordon Fund for cystic fibrosis research.

Tickets are £5 and the night will include a quiz, raffle and entertainment.

The last one we had was a great night so please message me if you would like to come and support our fundraiser.

My beautiful baby and many others are in desperate need of a cure so please help. If you can’t make it and have anything you would be willing to donate as a raffle prize let me know.