(Pictured: A 2 part title image. The first features a ventilator where the display screen instead of graphs and breath rates reads; “5 Years to Acceptance.” Next to it is a photo of me with my vent mask on with long tubing, over it is a comical SnapChat filter where it puts cartoon cat ears and nose on me on my vent)
I didn’t think I’d ever be writing about myself using NIV (Non-Invasive Ventilation), never mind felt bold and at peace enough with it to pubically display a picture of me on it! This may in fact be the ultimate key to Acceptance by hitting publish for this blog post now that I think about it….
Before I go into my particular journey, I’ll just clear up what NIV is and why many with Neuromuscular Conditions use it. Just incase you’re not in our world 🤓
What is NIV?
NIV stands for Non-invasive ventilation. Meaning it’s a type of breathing support that is usually delivered via a nose/full face mask or something called a “sip” mouthpiece (which closely resembles sipping from a straw each time you need a breath). It is Non-invasive as it requires no surgical intervention or permanent fixture to the body, whereas someone who may need a tracheostomy (a surgically created stoma in the throat in which you insert a breathing tube) that is attached to a ventilator would be considered as having “invasive” ventilation support.
Why do a % of people diagnosed with a Neuromuscular disease end up needing NIV?
There are over 60 NMDs and these all cause muscle weakness and wasting in different muscle groups, at various ages, from mild to severe, with different progression rates EVEN in the same type of NMD, some don’t affect life expectancy whilst others do. For the types that do have respiratory and/or cardiac muscle involvement, the course is usually bleak without intervention. Before NIV was available, commonly these individuals would die prematurely due to breathing insufficiency, related to their progressive Neuromuscular disease.
NIV, particularly using a ventilator known as BIPAP (where both inhale AND exhale pressures are supported by the machine) is now giving people like me a new lease on life. With a portable machine and mask people with NMDs are living 1-2 decades longer than textbook. The therapy is so life changing that it can alleviate symptoms such as migraines, daytime sleepiness, chronic fatigue, memory and concentration problems and eccelerated pressure on already weak cardiac muscles, meaning people (as long as they use their BIPAP machine as advised) are able to participate in various activities and ways of life they’ve been unable to in a long time due to gradual respiratory failure.
What is it like and how long do you have to wear it for?
What is it like? The BIPAP machine senses you taking each breathe, so as you do, it will inflate your lungs beyond your capability. So giving you a deeper, more efficient breath. Every breath! Inefficient breathing also causes you to be unable to rid the body of carbon dioxide cO2 fully upon exhaling. So BIPAP also assists with a bit of extra force behind every exhale to help prevent cO2 build up, which if high levels of cO2 are trapped and building due to the inability to get rid of it yourself…. It can lead to sudden, acute respiratory failure. So people who use NIV and invasive ventilation are monitored closely to make sure their machine settings are a good fit for their degree of respiratory weakness. This isn’t a cure for us, it does extend our lives but it will not stop a progressive muscle wasting disease. It may slow things down and buy a person time and another option than to be trached and ventilated invasively.
How much do you have to use it? Commonly people start off by using it at night. Mainly because when you’re asleep your breathing and heart rate are on auto pilot, unconscious breathing is normally lower than your daytime breathing. When patients with NMDs start experiencing morning headaches, nausea, chronic fatigue, memory and concentration issues etc, those are the tell-tale signs of inefficient night time ventilation. People can go for many years or in more slowly progressing NMDs, decades on just nightly NIV support. While a good majority will over time benefit from using their BIPAP when they’re ill or having break through symptoms that call for needing to use it in the daytime aswell. Unfortunately many people do eventually graduate (for lack of better words) to invasive ventilation, commonly via tracheostomy placement to be hooked to a stronger vent like a Trilogy full time. For some people switching to invasive ventilation is a choice, while for others it’s their only option.
Now we’ve got all the medical jargon out the way. Let’s get into what this post is actually about..
It wasn’t until I was half way through my 1st pregnancy that Dr Bentley wanted me to be admitted to trial BIPAP NIV, originally to prevent complications of late pregnancy where weight of baby may cause me respiratory distress. You can read more about that here in my post Life as a Disabled Parent | Q & A | Pregnancy and Child Birth
I was originally put on a type of machine called a Vivo APAP with a full face mask. I had a “know it all” kind of respiratory tech who was setting it up/teaching me everything. Off the bat she put the settings way too high. She insisted I needed a FULL face mask as I was a mouth breather. I hated it. I was getting light headed, I was claustrophobic with the mask and couldn’t even have my glasses on because the mask was attached to a head strap up the bridge of my nose. I politely expressed my concerns but she was adamant I would “adjust” and to just give it time. Keep busy! Though I found it hard to keep busy when I’m unable to see anything so couldn’t even read text messages, never mind do the sudoku puzzle books and read magazines people gave me. All I could concentrate on was how claustrophobic I felt in that mask, I could hardly see and as my mouth was covered people could barely understand me. I was miserable. The next morning thankfully the blood gases revealed the settings were over ventilating me so she tapered it down. I was released after a week of trying to get used to this machine in the daytime for the ultimate goal to be tolerating it enough to fall asleep with it on every night.
I tried really hard at home to get used to it. I did. I’d lay down on the couch and wear it, with the radio on. No matter how hard I tried to persevere, I couldn’t tolerate it. Ultimately I didn’t use it as often as I was advised and handed the machine back shortly after Abbie’s birth.
Again I tolerated my daily symptoms and brushed it off with it just being the way I am, if I just learn to pace myself more everything would be fine. Jump 3 years ahead and I was pregnant again and they wanted me back on the bipap. I was dreading it but I knew I had to try for my health and the baby’s sake. Though this time I knew there was a variety of masks on the market and I was going to put my foot down about not being given a full face mask again.
The respiratory technician I was given this time around was a lot more approachable. She took into consideration the fact the settings were too high last time, the claustrophobia issue and how I need to be able to wear my glasses! That’s when she pulled out the Swift FX Nano mask. Pairing it with a new model of BIPAP called a NIPPY 3. The new mask looked a lot better than the full face one, she explained it was the smallest interface they had and I could wear my glasses. I really felt good about it this time, that I could potentially tolerate it. All the boxes were ticked…
Only now another problem arised. I was having excruciating pain were the straps from the nose piece lay over the top of my cheek bones. I have very little “padding” on my face as my type of NMD causes facial muscle weakness and wasting. I’d get to an hour wearing it before I’d rip it off in agony. I tried stuffing folded tissue paper and make up pads under the plastic straps but nothing seemed able to take the pressure of the plastic off my cheek bones. I’d tried loosening the headgear straps but that caused air to leak into my eyes. Which isn’t pleasant!
I’d give up for a few months then try again and this pattern carried on until about 2 months ago. I went to get my blood gases done as routine at Dr Bentleys clinic and for the second time in a row it read my body was retaining too much cO2 and a critical situation maybe in the works. This was scary to hear to be honest. He showed me a chart that showed how my respiratory function has been slowly declining especially over the past 2 years. They have you bring your BIPAP to clinic and take out the memory card to chart how well you’ve been using it. This got me into bother as obviously it’d been too painful for me to continue its use as advised. I hadn’t been taking its use seriously after my 2nd child was born as I didn’t think I REALLY needed it like others with NMDs do. Apparently I did unfortunately.
I was taken in the respiratory lab and she cued another technician to come over, explained I needed a different interface that didn’t sit on my cheek bones. As I was told previously that the Swift FX Nano mask and headgear was the smallest available on the NHS, I was prepared to be told it was either this painful thing or be claustrophobic and blind with a full face mask again. I was pleasantly surprised when she rummaged in a draw of spare masks and introduced me to what I now deem as the holy grail to NIV masks – the Phillips Wisp!!
We trialed it then and then and the face straps lay literally 1/4 of an inch lower on my cheeks and that made ALL the difference. She explained it was a very popular mask for children and if the rubber straps were bothersome, there’s a fabric version she could order in for me! Though I was still not convinced after years of not being able to get it right, I’d lost faith in it. Though I still found it in me to persevere.
Nobody was more shocked than me that 3 days in I was falling asleep soundly wearing the Phillips Wisp and waking up feeling like I’d slept like the most restful sleep in my entire life. The more nights I wore it, the more it became as part of me as my powerchair.
I never thought I would ever feel comfortable knowing I needed breathing support. I’ve seen people worse off having to wear their BIPAPs strapped to the back of their powerchairs and wearing the mask as they go about their lives out and about. I used to feel like that must be such a horrible thing for them. Though now I was reeking the benefits of night time support, I realised the benefits outway the stigma/embarrassment of being seen wearing a breathing aid. I don’t know if one day I’ll need to wear mine out doors, but I feel a whole lot better about wearing it period and have come to the conclusion that it isn’t the end of the world. People do lead full, active lives with breathing assistance. Plus it doesn’t look so bad with Snapchat filters!
I am still me, BIPAP is enabling me to lead a better quality of life and I need to embrace that. OK, it took me a total of FIVE years to get the mask, headgear and attitude towards it right to accept using it. But I got there in the end and I would advise anyone embarking on the journey on transitioning to NIV to speak up and not pussy foot around people if something isn’t working for you. Despite what some respiratory technicians may say, there are LOTS of different mask and headgear interfaces to try. Just because the technician advises a particular model doesn’t mean you have to deal with problems caused by it not being the right one for you. You are the one that’s wearing it so it HAS to be right! As for accepting using NIV… It is a process. It might take a few months or like me years to accept you need this. It may take reeking the benefits long term to realise the pros outway the cons. Take it one night at a time and remember every breath is a gift. If it wasn’t for Non-invasive ventilation a lot of us wouldn’t be looking too far ahead. It is what it is, a way to manage, extend and improve your quality of life and THAT’S what really matters…
If you or a loved one has recently been told you may need NIV OR like me, struggling to get things right. There is a INCREDIBLE support network of people with Neuromuscular conditions on Facebook. Please don’t hesitate to reach out to the “Breathe with MD” FB Page and the “Breathe with MD” private support group and fill in the short admin form.💙 I cannot thank this group of individuals enough 💙 as I needed support throughout my journey and will continue to for years to come. //s7.addthis.com/js/300/addthis_widget.js#pubid=ra-5a77aa84ae451cbe