What is Cystic Fibrosis?

Throughout the month of April, our charity window will be proudly hosting
The Ian Ritchie Foundation in support of Cystic Fibrosis.

Cystic Fibrosis, an inherited condition caused by a faulty gene, is a life-shortening and complex disease, for which there is no cure. Secretory glands are responsible for producing the body’s mucus and sweat, but in a CF sufferer, these glands are unable to function correctly. Instead of producing the slippery and watery mucus required to line and moisten the body’s organs, thus avoiding them drying out or becoming infected; these glands, as a result of CF, produce thick and sticky mucus. This thick and sticky mucus then builds up in and around the organs, clogging the lungs in particular, encouraging the growth of bacteria and causing chronic respiratory tract infections, as well as repeated lung infections and obstruction of the airways, progressively limiting the ability to breathe.


The thick and sticky CF mucus also sits heavily in the ducts of the pancreas, preventing digestive enzymes from travelling to the small intestine, where the body would ordinarily absorb its vital nutrients from. These enzymes, for a CF sufferer, must instead be taken orally in the form of capsules, with all foods and drinks consumed, providing some support for their digestive system. As a result of these digestion difficulties, CF commonly causes vitamin deficiency and malnutrition, leading to bone related conditions such as Osteopenia (decreased protein and mineral content in bone tissue) and Osteoporosis (brittle and fragile bones); as well as commonly causing the severe pain and discomfort of excessive intestinal acid reflux and bowel obstructions.
CF causes very salty and increased levels of sweat, and is also usually accompanied by many other debilitating and life-limiting symptoms, including the development of CF-related diabetes, infertility in CF males, tiredness and regular headaches, as well as adrenal insufficiency.


Management of CF involves a life-long and increasingly aggressive combination of oral, nebulised and intravenous antibiotic treatment for chronic and increasing infections, which throughout the life of a CF sufferer, cause extensive and permanent damage to the lungs; until eventually, and inevitably, a lung transplant is required. Regular physiotherapy helps to loosen and clear the mucus build-up, for which exercise is also encouraged, where the body will allow. Currently, each week in the UK, 5 babies are born with CF. In the 1980’s, most CF sufferers failed to reach adulthood; but improvement in care and treatments continues to increase the life-expectancy of people with this condition, which sadly still remains at around just 35 years old.
 
About Us
 
The Ian Ritchie Foundation was formed in 2015 by Ian’s closest family and friends, following his life-long and difficult battle against CF, which he sadly lost at the age of just 25. We work on Ian’s behalf to continue helping families in the CF fight that we didn’t win; whilst also keeping his beautiful memory alive.
 
We are a registered charity (1165534) based in Weston-super-Mare; set up to support, promote and protect the wellbeing of those suffering with Cystic Fibrosis, and their families, across the Southwest of England. We achieve this through the provision of financial assistance, medical equipment, practical support and advice, as well as raising public awareness in all areas relating to Cystic Fibrosis.
 

Ian’s Story
 
Ian Ritchie was a determined and inspirational young man, born with Cystic Fibrosis. He lived in Weston-super-Mare and attended local schools; first St. Marks Primary, and in later years, Priory. He fought a life-long battle against the condition, until at just 25 years old, his aggressive treatment was no longer effective, and with his body not responding to medicine, Ian underwent his last hope of a better quality of life; a double lung transplant.
The transplant journey for Ian and his family was a painfully difficult one, and sadly, following 5 months in Critical Care, doctors concluded he could not be saved. Fulfilling Ian’s final wish, his devastated family arranged for Ian to be returned home, so he could spend his remaining hours in the place he was most comfortable, surrounded by those who loved him dearly. Ian lost his fight in the ambulance, with his mum by his side, 40 minutes from reaching home.

The Work that we do

We recently purchased a £2000 portable spirometer (lung function machine) for the CF team at Bristol Royal Infirmary. This allows lung function tests to be carried out in CF patients’ homes; something which could not previously be done, helping to relieve not only some of the discomfort of hospital visits for CF patients, but also some of the financial strain that regular trips to and from hospital can impose.
We are also very pleased to have purchased exercise bikes for the physiotherapy team caring for Cystic Fibrosis patients at Bristol Royal Infirmary. Physio and exercise both play a primary role in the management of CF, but the risk of spreading life-threatening CF-related infections when in hospital, often means those with the condition cannot interact with one another, leaving them confined to their hospital rooms, with exercise opportunities somewhat limited. The bikes we have purchased can be moved between their rooms; improving access to in-hospital exercise equipment, whilst reducing the infection spreading risks.

Events

We host a variety of local community events, which help us not only to raise the funds required for meeting our objectives, but also to meet with CF patients and their families, making them aware of our work, and to raise awareness amongst the general public of the devastating effects of living with CF; as a patient, as a parent, and as a close relative or friend of a child or young adult with the life-limiting condition. So far, with our hard work and commitment to helping Cystic Fibrosis families, along with the overwhelming support of the general public, we have managed to raise in excess of £15,000.

A huge supporter of Wolverhampton Wanderers FC, one of Ian’s greatest loves was football. As a boy, despite his gruelling and relentless daily regime of Cystic Fibrosis treatments, he loved to play the game his heart belonged to.

The journey from our home town of Weston-super-Mare to Wolverhampton’s Molineux Stadium is 135 miles long, and was one Ian made regularly with his Dad, to watch their mighty Wolves play.

In Ian’s loving memory, to raise funds for The Ian Ritchie Foundation, Ian’s sister and his 14-year-old nephew cycled all 135 miles of this very journey, from Weston’s Grand Pier to the football ground Ian held so very close to his heart. Priory School head teacher Neville Coles also supported, riding part of the way alongside them.
 
Wolverhampton Wanderers FC kindly donated match tickets for this fundraising event, allowing Jen and James to watch Wolves take on Blackburn at the finish line.
Here you can see some of the highs and lows of their difficult but determined ride, which saw them set off on April 7th and reach Molineux 2 and a half days later. A more painful challenge than they ever imagined, but Ian taught everyone who loved him just how important it is to fight on until the end; and that’s exactly what they did.

Thanks to so many generous sponsors, Jen and James have so far raised over £1,500 for their cycle ride, with donations still coming in! THANK YOU!

The Ian Ritchie Foundation

Visit our website

www.theianritchiefoundation.org