There is currently no cure for Cystic Fibrosis. While there are a lot of medical breakthroughs, treatment mostly involves trying to stop the progression. In South Africa, we do not have access to the latest modulator treatments that slow down the progression (yet). But we do have access to the important basics.
The treatments to maintain Cystic Fibrosis are preventative, which also means they are part of everyday life. This includes moving mucus out of the lungs daily and ensuring the body is as strong as it can be – by providing it with the nutrition it needs through food and supplements.
Firstly, the daily routine looks different for each individual with Cystic Fibrosis. Below is the treatment regime for a little girl of 4 years old, as shared by me, her mom:
She is nebulised with 5% saline. This is very salty stuff – used in hospitals for IV administration – it helps loosen the mucus by adding salt to the lungs pathways so that it can move more easily. As it can irritate the chest, so some individuals use an asthma inhaler before treatment to open the chest. We don’t have nebuliser from the local pharmacy, we have a top end (read expensive) PariJnr nebuliser. It is more powerful than the average nebuliser. It takes about 15 to 20 minutes to nebulise with the Pariboy Jnr. Then there is a 20-minute wait (sometimes this is not possible but most days this is routine). Then 14 minutes of percussion physio (cupped hand striking across the back/shoulder in a rhythmic manner), 7 minutes per side. This is part of airway clearance, moving the mucus out of the lungs as much as possible. Then there are breathing exercises (blowing bubbles for example, or using apparatus like a bronchovibe or bronchopep specially developed for airway clearance). The blowing exercises help move the mucus out of the lungs, and can trigger coughing to get it out. It is also extremely important to stay active throughout the day to ensure the lungs stay clear.
Nebulising and physio are done twice per day and takes up about an hour at a time. In between, I have to make sure her nebuliser parts are cleaned and sterilised properly. While it is only necessary to sterilise once per week, I prefer sterilising after each use.
As her pancreas is insufficient it means she cannot digest food properly. To help her, she gets Creon (enzymes) with each meal or snack. Either half a tablet or one tablet, depending on how much she eats. It comes in a tablet form, but luckily the tablet is filled with granules, and I give this to her with the help of a Squishy so that it doesn’t get stuck in her throat or mouth. This was a challenge initially but has become almost second nature.
And talking about food – because her body works harder than ours due to the sticky mucus, she has to eat almost 3 times more than an average child her age. This is tough! Her diet needs to be high protein and high in fats. She also needs more salt – the root of the problem lies with the genes that instruct the salt channels. She loses most of her salt through her sweat – which means dehydration is a real risk on hot days.
Lastly, she gets a high regimen of vitamins and an immune booster.
When she is sick, the above treatments are intensified to 3 to 4 times per day and could lead to admission in the hospital to keep an eye on her breathing. Kids and adults with CF also often have to get IV (Intravenous Antibiotics) for two weeks in hospital (sometimes at home) to clear out a build up of bacteria in the lungs. This is called a “tune-up”.
Regular Checkups with a CF Team
To maintain health, Cystic Fibrosis patients have a team looking after them. This usually includes a dietitian, a pulmonologist, a respiratory physiotherapist and in some cases a doctor that specialises in gastric issues.
Our little girl, as any other individual with CF, also goes for standard checkups (currently every two to three months), at our local CF clinic at the Red Cross Hospital. Her sputum is checked for bacterial growth, her pulmonologist listens to her chest, her weight and length are also checked (it is important that she grows and gains well).
As mentioned, each individual with CF is different. And therefore treatments may vary. But the basics are always the same – airway clearance and nutrition.
Learn more about the latest Cystic Fibrosis treatments here: https://www.cff.org/Life-With-CF/Treatments-and-Therapies/
This blog contains extracts from https://piecesofjolene.wordpress.com/2018/11/04/what-the-cf/