Sometimes you never realise what you have until it’s gone.
The ability to walk, talk, or even eat. Most days we take these things for granted. But if you start to lose these abilities, you soon realise how lucky you are to have a body and mind that works.
For people living with NP-C, everyday tasks like eating can become more and more complicated. This is where interventions like a PEG can enter the picture.
PEG feeding is important to wrap your head around because it’s something most people with NP-C will eventually face.
So, what is PEG?
It’s essentially a feeding tube that can move food directly into the stomach.
PEG stands for the term Percutaneous Endoscopic Gastrostomy (like most medical terms, it’s a bit of a mouthful). The tube itself is either called a gastrostomy tube or a feeding tube.
Why is it useful?
PEG is an option for people who have problems swallowing.
In NP-C, when brain cells that control muscle movement die, it can impact the ability to swallow (swallowing difficulties are also known as dysphagia).
There is also the added complication of having food go down the “wrong way”. Meaning, instead of food going down the oesophagus and into the stomach, it goes into your airways and lungs (this is also known as ‘pulmonary aspiration’, or just ‘aspiration’). Not fun.
The person may try to cough up the food or liquid, but this is not always effective and if it gets stuck in their lungs, it can lead to pneumonia.
Sadly, respiratory failure is the most common reason people diagnosed with Niemann-Pick disease pass away.
Therefore, a PEG is an intervention that could be used to reduce the risk of aspiration and potential pneumonia.
But it’s not a choice easily made.
It involves accepting that the disease has progressed to a severe level, and a willingness to make the necessary changes.
In a way, it limits a person’s freedom. Limiting food consumption can impact social interactions and make it hard to be in environments where others may be eating.
There is also the surgical risk to consider.
The surgery to get the port inserted into the stomach can be risky, but should only take around 30 minutes under anaesthetic. The person needs to be physically strong enough to undergo anaesthetic without fear of major complications.
Having a PEG does come with the risk of certain complications, so support staff and carers will also need to be properly trained on how to clean and use the equipment.
Due to some of these reasons, when someone living with NP-C is considering PEG feeding, it’s usually a last resort option.
On a brighter note, a PEG may improve nutritional status and quality of life.
Once the tube is in, dietitians prescribe special food that provides the person with reliable nutritional support. Some research studies even found that people improved their overall nutritional status after they had a PEG put in.
An improved quality of life was also recorded in some research papers.
Beyond making accommodations for feeding times, this intervention is unlikely to prevent someone from doing what they enjoy outside of eating food - like going to see a band, watching a movie with a friend, or going to a garden.
In the end, a decision to insert a PEG is a personal one. However, I encourage you to start thinking about what this might mean for you and your family. As always, please consult your health professionals with any questions you may have.
Stay supportive and supported,
Ellie