Benjamin was born in September 2014 and fed constantly, yet was not gaining the weight which would reflect such prolonged feeding. He also had green oily stools to which no visiting midwives, health care visitor or A&E doctor could explain.
Then one Monday morning when he was 3 weeks old we had a phone call from a health visitor which would rock our world to its core. The lady asked could they come round to discuss the results of Benjamin’s heel-prick blood test, the routine blood test which had been carried out when he was 5 days old. Although shattering news was to follow, ultimately, receiving this phone call led to Benjamin’s life being immediately put on the best possible path and we remain forever grateful that this diagnosis came early. So, the worst but the most important phone call we’d ever had.
Just an hour later the health visitor and two other ladies whom I later knew were specialist nurses knocked on our door and we were told the devastating news that our precious new born baby had Cystic Fibrosis. This came completely out of the blue as neither I nor Benjamin’s Dad had any family history of CF nor did we know we were both carriers of the mutated gene which causes CF. Suddenly, there was an explanation – his body wasn’t able to digest the fats in milk because CF causes the internal organs, especially the lungs and digestive system, to become clogged with thick sticky mucus resulting in chronic infections and inflammation in the lungs and difficulty digesting food. By luck, he hadn’t yet shown any respiratory exacerbations.
The very next morning we attended a hospital in London which has a dedicated team specialising in the lifelong care and treatment of children with CF. A sweat test was carried out followed by a blood test to confirm. A few days later we spent time with the CF team on a two-day parent’s education programme (respiratory doctors, nurses, physios, and clinical psychologist) where we were taught more about CF, reducing risks of infections, how to give Benjamin chest physiotherapy, what medicines he would need to go on straight away, how to estimate and give pancreatic enzymes to our tiny newborn etc. It was a lot to take in but the fight began there and then.
Life for him and us is a delicate juggling act between living life to its fullest but still reducing risks to his health, and fitting in the medication and physio around family life and work. This can be difficult for people to fully understand because he looks well, but a lot goes on ‘behind the scenes’ in order for him to be well. His lungs are an ideal breeding ground for bacteria which are easily trapped by this thick mucus and able to colonise and cause infections. Repeated infections can cause permanent scarring and reduces lung function. It’s recommended that Benjamin try to avoid or take great care when in contact with people with coughs, colds, chest infections and even things like hay, stagnant water, outdoor sand pits – things which can collect water and be breeding grounds for bacteria or fungal spores.
Benjamin takes a prophylactic antibiotic twice daily and needs to take pancreatic enzymes with all meals, snacks and milk (anything which contains fats), along with a vitamin preparation which is tailor-made for cystic fibrosis. He needs a high fat diet for life. At the moment, he takes around 12 capsules of the enzymes throughout the day, which are opened up and poured onto apple puree for him to swallow. Soon, he will be taught to swallow the capsules whole. His chest physio is twice daily, and extra sessions if he gets a cough as it means we have even more mucus to move around. This is currently done by firmly patting his chest for around half an hour each session, and using a PEP mask to force mucus to move, and bouncing on a gym ball. He is seen regularly at the CF centre and every few weeks has a routine cough swab to see if any bacteria are present in his lungs, usually this means extra antibiotics.
Giving various medications to a baby was (and still can be) very difficult and distressing for him and us. He does not understand why, and as he’s got older he has become more headstrong and cheeky. He is such a happy little boy and I know he will take things in his stride as we’ve seen him do so from a very early age.
At three months old, Benjamin had a bronchoscopy and pH study at his hospital to look at his lungs and he was also diagnosed with gastro-oesophageal reflux disease which meant more medication for him. At the start of 2016, a persistent cough ended up with him being admitted for another bronchoscopy and two weeks of intravenous antibiotics through a long line in his arm. He was incredibly brave and he melted the hearts of staff. There, he also had to start using a nebuliser which contained a solution which could thin down the mucus secretions to help him recover from the cough. When a cough lingers or if a certain bacteria is detected on his cough swab it can mean two weeks in hospital on IVs. Earlier this year, a routine cough swab tested positive for the pseudomonas aeruginosa bacteria which meant more oral antibiotics and nebulising another antibiotic twice daily for three months. The extra mask was distressing for him when he would much rather be playing with his toy cars but seconds later he would be back to his happy little self.
At only two years old, he’s taught me more about life than I ever could have imagined and I feel honoured to be his mummy as he’s here to make an impact in this world.