Our CF Story

Here is our Cystic Fibrosis Story- I hope it raises awareness for the condition and perhaps helps another family out there who have to come to terms with the diagnosis for the first time.

pregnant!

Josephs first day

Our son was born 9lbs 9 ounces 15 days over due, he came out in a hurry with a blur of doctors, nurses, midwives and alarms around him- a dramatic entrance for a little man! His heart rate was dipping in labour and when he came out he wasn’t breathing but the staff soon got him in full working order and I couldn’t believe he was finally in my arms after all this waiting!

He was perfect. We got him home and soon started to learn all there was to know about each other.

He slept great, looked adorable and seemed to be the picture of health! And just like the rest of our family boy was he hungry! I tried to breastfeed but I found it difficult and felt like I couldn’t satiate his hunger so quickly moved on to bottle feeds. He cried often as babies do and I diligently fed him whenever he needed it. But I was finding it difficult to cope with his demands. He just wouldn’t stop crying for food. I felt like I was doing something wrong and I often sat and cried in desperation and frustration. The midwife thought I may have postnatal depression and so organised extra visits. One day my mom came to stay not long after he had been born and she noticed something a little strange that I as a first time mom just overlooked.

little Joe

“How often are you feeding him?” she asked “He can’t possibly be hungry again?!” as she watched him guzzle hungrily at another bottle. I just thought babies fed a lot so I just fed him and fed him and it turned out I was feeding him every hour throughout the day, upwards of fifteen bottles a day. And he still seemed hungry. No wonder I was frustrated! I had thought nothing of it as I just assumed that he was a hungry baby being a big boy and all that and I wasn’t really sure of how much he should be drinking. Then he started projectile vomiting after every feed and still crying out for more food. The midwife came and weighed him and he’d lost a little weight as most babies do in their first few weeks, I expressed my concerns about his feeding and vomiting but she didn’t seem very concerned and put me at ease so I continued to feed him as normal- baby led bottle feeding.

Joe Baby black and white

We had the routine heel prick test during this time and thought nothing of it until I got an unexpected visit from the health visitor one Tuesday morning with another lady in tow who I had never met. They came in and sat down and the new lady produced leaflets from her bag and started saying things like “have you ever heard of cystic fibrosis” and “abnormal blood results” and “Have you got anyone you can take to the hospital with you” in her calm soothing voice.

I could see sympathy in her eyes as I sat there trying to take it all in but feeling numb and fuzzy and outside of myself all at the same time. Luckily my Rob came home for lunch from work about ten minutes after they arrived so we could process this news together.

It’s possible your son may have cystic fibrosis. You need to take him to hospital for a sweat test tomorrow and speak to a consultant. We can’t confirm that he does have the condition, you need to get him tested first.” And the other thing they said was “don’t look it up on the internet. Wait until you have spoken to the doctors.” Alarm bells rang in my head and all I wanted to do was research research research!

So we clung on to the idea that it wasn’t a confirmed diagnosis. He couldn’t possibly have it. It must be a mistake! Not that we even really knew what it was. All I could remember was there was a little girl who used to live next door to a friend of mine when I was little who had it and I can vividly remember her with a canister of oxygen and a face mask and I remember she was very poorly. I pushed the panic down inside and began to ring my close family. We needed someone to come with us to help process the information we were going to receive. The Health visitor was adamant we took someone with us. And who better than my mom. She didn’t hesitate and traveled the one and a half hour journey up to us to offer her support.

Tigger Joe

So off we went to the hospital to see the consultant. We were taken into a room where there were three health professionals and I held my little baby on my lap. This is when they told us “your son does have cystic fibrosis we are very certain of this. The sweat test is just confirming what we already know, I’m very sorry” I could feel myself looking down at the room from a high up place. I could see my mom with tears in her eyes, my husband with a thousand questions on his lips and me holding my perfect baby oblivious to it all. I couldn’t hear what they were saying after that. I held my son down while they did the sweat test and watched him struggle and cry. I fed him his bottle and all the time I was just numb. Now I know why the health visitor was so adamant we take someone with us to help. I couldn’t process this information. I could barely remain conscious. We were given a prescription for us to pick up and start using straight away for antibiotics. It wasn’t until I got into the chemist in the hospital and I saw all these older poorly people sat there that it hit me. My perfect baby has a life shortening disability and there’s nothing I could do about it. There is no cure. I began to sob. My mom took me out of the room and we went and sat on a bench in the cool air. I couldn’t stop crying. It was so unfair! Why had this happened to him? He didn’t deserve this! They must have made some kind of mistake! My mom held me and soothed me. We went and sat in the car. And we went home.

little Joe

So, our child has cystic Fibrosis. We were told he was to start prophylactic antibiotics straight away to help reduce the risk of catching an infection so he now takes 2.5ml flucloxacillin 4 times a day. We were sent home from the hospital with a folder of information and a link to the CF Trust website which you can see here. Our Joe who had seemed perfectly fine just a day or two before now had a life shortening condition, a consultant paediatrician, a specialist lung nurse, a physiotherapist and a dietician. We were to go to the hospital every few weeks to see his team of health professionals so they could check on his progress.

They told us that people with cystic fibrosis often have a non functioning pancreas and he had to have a blood test but because our child was not underweight they did not rush his test results through and they initially said they didn’t think his pancreas was affected. I held on to the hope that maybe in fact his pancreas was fully functioning and continued to feed him as normal- maybe he was just a hungry baby? But the next visit to the hospital confirmed that his pancreas has near to no function. This means he cannot digest nutrients from his food particularly fat, protein and carbohydrates hence his continual need to feed. “He’s done really well to gain weight” the paediatrician told us “how often do you feed him?” “all day” was the answer- the sheer amount we were feeding him meant that a little bit of nutrients were getting through, but it could never be enough.

So he was given his next lot of medication- dalivit multivitamins that he must take everyday and creon micro which is an enzyme replacement that he has before all food that contains fat, carbs or protein.  These are tiny granules that have to be mixed with apple puree so the baby can swallow them. So in effect at 6 weeks old baby had to start using a spoon and eating solids! He was to be weighed at every visit and his weight is still monitored very closely now on a monthly basis. We were also to start physiotherapy twice a day. At the time it consisted of just using two fingers as he was just a tiny baby and involved firmly tapping his chest and his back whilst he lay in different positions.

Then the problems began with his eating. He struggled to take milk from his bottle, he projectile vomited out all of his milk and we would just have to feed him again. This happened at every feed. He would suck hungrily and then writhe and scream in pain. So at the next visit to hospital he was diagnosed with gastro intestinal reflux. He was prescribed thickeners for his milk to help him keep it down.  We started using the thickeners but things didn’t improve with the feeding. So he was prescribed mezzopram which is a tablet that he has to have everyday crushed onto apple puree. This combats the reflux.

And then the coughing started. Another visit to the hospital. He had caught a virus, so in addition to all other medication and physio he was prescribed more antibiotics to take three times a day for two weeks. This concoction of milk and medicine gave him chronic diarrhea and several times at night he would wake with a dirty nappy leaking all over him and the cot which required changing him, all his clothes and bedding and giving him a bath in the early hours of the day. He finished the antibiotics but he continued to cough. The diarrhea stopped but now he had constipation. A week passed without a dirty nappy, so off we went to the hospital again. This time he was prescribed lactulose which he has twice a day.

In the meantime the health professionals had started visiting the house to check up on us and to see how we were coping and to provide more support. Every week we would be at the hospital or have the physio or lung nurse at the house or extra visits from the health visitor. During this time we discovered that we could apply for disability living allowance but the form was pages and pages long with questions on it that could not possibly relate to a newborn baby. Luckily we have a great health team behind us and a specialist lung nurse came to our house to help us fill out the forms.

His breathing still wasn’t right and they continued to prescribe more and more courses of antibiotics. His physio was increased to four 10 minute sessions a day. I took him into hospital one day as I was concerned about his breathing and his persistent cough (luckily he is allowed 24 hour access to the childrens hospital). This is when it started to get scary. “If this set of antibiotics doesn’t work then I’m afraid we are going to have to admit him to hospital.”

They then had to take a cough swab and check for viruses up his nasal passage so they stuck tubes up his nose to suck out the mucus and sent it off for tests. They also gave us a nebuliser inhaler to use on him four times a day to ease his breathing. We continued to give him another course of antibiotics. Two weeks passed and his eating and breathing problems remained the same.

We returned to hospital for his check up. And it was bad news. Due to his infection not clearing they had decided he was going to have to have intravenous antibiotics which meant a two week stay in hospital starting straight away. I was devastated. I couldn’t believe it had come to this so soon. He was only a few months old. The worst time of our lives so far had just begun.

As before mentioned Joe has gastrointestinal reflux disease which is common in babies with Cystic fibrosis. The paediatrician suspected that the reflux had helped cause the infection in his lungs. He was bringing up milk in the night and this was going into his airways and settling in his lungs causing an infection. So as well as admitting him to hospital for intravenous antibiotics he was also given another medication called domperidone which he has three times a day.

Seeing our baby in hospital and suffering was the hardest thing we have ever had to do and it really pushed us to the limits emotionally and physically. Due to his cystic fibrosis the hospital staff decided it would be best if he stayed at home during the day and came into hospital every night to stay for his evening doses so as not to risk him picking any other bugs up in hospital and also to help us keep a grasp on normality. This was a blessing being able to spend time at home, but it was difficult spending every night in hospital with a little poorly baby-and me and my Robin found it difficult being separated. He would have two doses of IV antibiotics in hospital and two doses at home (community nurses would come out to administer it). He had to have a cannula inserted which was quite distressing, initially they sedated him and tried to put a long line in but his little veins were too small and he woke up during the sedation. That time we didn’t go in with him but we could hear him screaming. After this his cannula came out around 12 times and every time since I went with him when he had it re-inserted. It was horrible to see him so distressed but I much preferred being there with him.

During the stay in hospital he got serious diarrhea again due to the medication and he would need changing several times a night. One night it was so bad that I ran out of nappies and we both ended up covered in diarrhea, I didn’t know what to do because it just wouldn’t stop pouring out of him and I couldn’t leave him on the bed alone to go and get help. I considered pressing the alarm but I was covered in poo and didn’t know if it was enough of an emergency! In the end he stopped and I managed to get some more nappies off the wing after cleaning us both up.

One time his cannula came out when I was at home with him. Luckily my friend had come round to cheer us up and bring me some lunch. I went to pick him up from his nap and carried him into the kitchen when I realised I was covered in blood.

Blood was dripping on the floor and all over my clothes. It took a few seconds to realise that it wasn’t mine and realising it was my baby bleeding like that was one of the most terrifying and horrible feelings of my life. We rang the hospital and they told us how to staunch the bleeding then when we managed to stop it I sped off back to hospital to get another cannula inserted into him. We arrived covered in blood, I had hardly slept and my hair was crazy I must have looked like a wild woman! My little man throughout this fiasco hardly made a peep! He was not bothered at all!

poorly Joe

He missed his last dose of IV antibiotics because they couldn’t find another place to insert a cannula as he had had so many. It was devastating to see all these little bruises all over him and bandages and tubes coming out of him. But he did so well he was a little angel.

Since then touch wood he hasn’t had another stint in hospital but he has had several more courses of antibiotics. He also has had the palivizumab injections (once a month for 5 months over winter) to help build up his immune system.

He is doing great and most days if you ignore the medicine and physio you wouldn’t think there was anything wrong with him.

The first few months of his life were tiring, stressful and scary but also wonderful. We have learnt so much about life and love since he came into our family. So if you are struggling with the news that your child or someone in your family has cystic fibrosis don’t fear- its hard and scary but even though you think you’re not strong enough to cope-you are. And whatever struggles may lie ahead in the future make CF a small part of your life and theirs, cherish every moment and remember life is beautiful whatever guise it may take.

13 thoughts on “Our CF Story

  1. mssharonmullins says:

    Oh Rachel what a sad beginning to your little mans life. Our cf soldiers are so brave and put up with so much. It’s only other cf mommies and daddies know Jst how much we all go through. I’m glad robin hasnt needed too many ivS in hospital long may it continue. God bless from another cf mommy xx

    • Rach says:

      Thank you very much for your comments and for taking the time to read my blog, I have started following you and have to say you seem to be an incredibly strong woman and your blog is a beautiful testament to your lovely daughter Orla, I am so sorry that you have had to go through such an incredibly difficult time, by sharing your story with others you are not only keeping the memory of Orla alive, but helping other parents who have had to endure the unspeakable too,it is very courageous. Kind thoughts from me, Rob and Joe x

  2. Emma corr says:

    Pleased to have found you all here! My daughter was born in October last year and diagnosed at a week old. I also have a blog with our story which is emmakatecorr.blogspot.com looking forward to following your lovely family xx

    • Rach says:

      hi, thanks so much for checking out our blog, I have just signed up to yours on bloglovin, looking forward to reading more about your families story, congratulations on the birth of your beautiful daughter Harriet- looks like she has had quite an eventful life already! xx

  3. The Bold Bluebonnet says:

    Thank you for sharing your story as openly and detailed as you have. I’m sure it is helping lots of folks out there that have been touched by CF. I am not a parent, but I married a CFer and reading your story gives me a whole new appreciation for my mother and father-in-law.

    • Rach says:

      Thank you so much that is a lovely thing to say, I’m glad our story so far has given you a different perspective I hope you and your husband and family are doing well, it’s great to know that people are reading.

  4. Jess Knight says:

    Hi Rach,
    I stumbled across your blog by accident when researching ‘cystic fibrosis children and nurseries’
    My son Leo just turned 1 on Monday 28th and has Cystic Fibrosis. So far I have pretty much just kept him at home with me and not really ventured out to many Mums and Tots groups or playgroups etc. we see a lot of my friends (some of whom have older children) and I have a HUGE family thatwe see all the time (I have a 2 and a 3 year old nephew) but I’m very aware we don’t socialise with any children the same age as Leo. I was thinking about starting him at nursery a few mornings a week and hence the search….. Your blog is lovely and really reflected all my feelings of having a child with CF. I mainly commented because we live about 10 mins away from ‘ Billy Bob’s’ ice cream parlour I’m Skipton and go there quite a bit! Glad to see you enjoyed it too.
    Joe looks like he’s doing fab, Leo has kept incredibly well this first year so we have high hopes for the future. Keep writing, I’m really enjoying reading it. As you’ve mentioned before in one of your posts… It’s only really other parents that can fully appreciate everything that goes into having a child with CF.
    All the best xxx
    Ps. Apologies for any spelling/grammar mistakes, Leo threw my phone the other the day and it’s smashed to smithereens- I can barely see the screen!

    • Rach says:

      Hi Jess,

      thanks so much for your lovely thoughtful comments! No spelling mistakes as far as I can see (can’t say the same about my reply though!) I’m so glad that our blog has been of use! You and Leo seem to be in a similar predicament to us- what with no other siblings or young children to play with regularly. I too kept Joe away from toddler groups really before the age of 18 months as I was worried about the affect it could have on his health, and I found a few of the groups to be too crowded and unhygienic! It is a really difficult decision to make and a very personal one for you and your family so don’t worry if you have to take a while to decide whether nursery is for you or not! We waited until Joe was two partly because he got 15 free hours, partly because we were scared and but we took the plunge as I was beginning to see him crave the company of other kids- plus it is great for YOU to get some time to yourself- looking after a child full time is hard enough without the added stress of CF! But it’s not right for everyone- remember you know what’s best for your child, go on your gut instinct! Joe loves nursery so far, the only problem we’ve had is that he won’t eat the meals there. He’s really benefited from the company of other toddlers, plus interacting with adults he’s not sure of has boosted his confidence with other adults particularly at hospital visits which is a massive plus! And touch wood his health hasn’t been impacted so far!
      I’m so glad to hear that Leo is doing great, long may it continue! Billy Bob’s is awesome, you guys are lucky to live nearby, Skipton seems to be a lovely area, we are based in the ribble valley in Lancashire so not too far away!
      And again thanks so much for reading and if you ever want a chat about anything feel free to contact me on here or via email ecstaticmonkey2000@yahoo.co.uk
      xxxxxx

  5. Elli says:

    Hi Rach,

    Thank you for sharing your story. I totally feel for you. My little boy was born in 2013 and just like yours was hungry all the time, I thought it was normal! and was actually breastfeeding as well. So when I got the diagnosis I was devastated but sort of relieved, as I knew there was something wrong. I am glad your little one is doing well, ours is doing well as well. My hope is that this is a very different world from the one 20 years ago, and technology and medicine is advancing all the time. Who knows? Maybe ins 25 years’ time, CF is history 🙂 Keep writing. I will keep reading.

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