Life-Limiting Illness

One of the things that has really hit home over the last few months, as I've spent so much time in hospital, is that my illness is progressing. I have known for a long time that I probably wouldn't live to be 100 to receive a telegram from the Queen (or perhaps King, by then!), but with each new problem, it seems increasingly likely that I may be lucky to make it to 50.

I've lived with severe 'brittle' asthma for almost all my life. I also have severe allergies. Either of those could kill me. While thatt makes it a bit scary when I have an asthma attack or anaphylactic reaction, most of the time I can forget about those and put the thoughts of death to the back of my mind.

More recently, as my GI tract has shut down, my autonomic dysfunction has become more pronounced and my blood sugars have become more difficult to control, I have needed more day-to-day medical support.just to remain stable. I have a jejunostomy tube for medications and low-volume feeding, and a Hickman line (permanent IV line) for medications and IV fluids. My team are gearing up to start me on parenteral (IV) nutrition, which carries with it many risks. Risks of bloodstream infection (septicaemia), liver damage and blood clots, to name just a few. I know that these risks are small in comparison to the guaranteed complications of starvation and malnutrition, but they are still significant.

I can accept the restrictions that my illness has placed on my life. I am still so grateful to be alive, and to have the opportunity to experience this wonderful world. But I don't feel ready to die. Not now, and not in five or ten years. Probably not even in 20 years. There are still so many things that I want to experience. I want to grow old with my husband. I want to see our friends' children grow up, and to have nieces, nephews and godchildren, and to be there for them. I don't want to miss the conversations, the celebrations; even the bad times. I want to be there to comfort my husband, friends and family; to grieve with them in the sad times and rejoice with them in the good.

I am not afraid of death. I just don't feel ready to stop living.

My husband and I are very open with each other about this. We both know that I have a life-limiting illness, and we live accordingly. We seize our opportunities when they arise - who knows what might be impossible for me by next year or the year after? We talk about funeral plans and end-of-life decisions for both of us. We discuss the things that are important to us about medical care, our thoughts on remarriage, what we want to happen to our bodies after death, and how we want to be remembered.

During one of these conversations, I asked my husband how he copes with the thought that I will die before I'm old. His response?

"I will love you for as long as I have you. And then a bit longer."

Action Plans

Simple definitions:


Acute illness:

• Rapid onset
• Severe symptoms
• Short course

Chronic illness:

• Of long duration
• (often developing slowly or insidiously)
• Variable severity

Seeing acute and chronic illness separated and delineated makes them seem clearly distinct from one another.

However, in real life, things are often not so simple. Let's take asthma as an example:

I have been diagnosed with asthma since 1988. That is a long time. I would feel comfortable saying that I have chronic asthma. I take lots of different medications to treat my asthma, including daily high-dose oral steroids (Prednisolone). I nebulise medications every day and get daily symptoms of asthma. Again, this is easy. I have severe chronic asthma.

So what about when I have a flare-up of asthma. Sometimes this comes on subtly, making each breath shorter and more difficult over a period of hours or days; sometimes it happens in minutes. This is clearly an acute reaction (especially the rapid reaction, which is often a response to some allergen or other). The acute reaction is easy: I am having an acute flare on a background of chronic asthma. It's less easy to spot the acute exacerbation when it happens less quickly, and it can be very difficult to know where to draw the line between these situations:

1. I can manage this situation at home by increasing my normal medications
2. I need to speak to my GP or asthma nurse about extra treatment to keep me at home (e.g. extra steroids or antibiotics)
3. I need treatment in hospital

The time to decide which situations fit into each of the above treatment plans is NOT when you are having an acute flare. Hypoxia does not help good decision making. For me personally, the desire to stay at home becomes overwhelmingly strong when I'm not well. Hospital, with the undignified gown, endless questions, needle-sticks and bright lights, is not nearly as appealing as my big, soft bed at home.

The best solution to this is to get together with your GP (or consultant, or specialist nurse) and agree a plan. For asthma, you might use peak flow measurement as a guide; for diabetes it might be blood sugar measurements; for gastroparesis, it might be body weight in combination with certain symptoms.

My asthma plan:

• Peak flow more than 80% of normal: Carry on as normal
• Peak flow between 50% and 80%: Increase oral and inhaled steroids, use nebuliser as often as four-hourly if necessary; contact GP
• Peak flow less than 50%: Go to hospital. Go straight to hospital. Do not pass Go. Do not collect £200.

There are other signs that fit into this, that indicate clear deterioration: waking at night more often than usual with coughing/wheezing, inability to talk in complete sentences without needing to take a breath, pulse rate more than 120 per minute (this is a bit iffy, because I have autonomic dysfunction, which messes with my heart rate), shortness of breath at rest, needing to use the nebuliser more often than every three hours. 

Having these signs and symptoms written down in black and white means that the pressure of making a decision is lighter. Without such a plan, I would tend to continue to try all the drugs available to me at home, and wait, and wait, and wait.

I don't want to make a fuss. I don't want them to think that I'm overreacting. I have stuff to do. I don't want to be admitted to the hospital. I'll just try one more nebuliser...

And wait...

Until it's nearly too late, and I end up in the Intensive Care Unit on a ventilator.

Because I didn't want to make a fuss.

I have a chronic illness, which means that I live with daily, constant symptoms, and it can be very easy to become blase about them, even when they become quite severe, as in the case of an acute asthma attack. Having cut-off points agreed in advance with your specialists (GP, consultant, specialist nurses), written down and reviewed regularly, mean that you don't have to make a decision. I'm not making a fuss, I'm just following my action plan.

Action plans save lives.

Blaming Myself

Among other things, I have what is known as 'difficult asthma'. This means that my asthma is severe and persistent, despite maximal medical therapy. It is par for the course that things flare up and I run out of things that I can try at home. At this point, I either try to wait it out at home, in the hope that I can keep things stable enough and give my lungs time to get their act together, or I take myself off to my local hospital. The latter option usually wins, even if I try the waiting option for a bit first.

I take my medical care quite seriously, and am careful about taking my medications and avoiding allergens and things that trigger asthma attacks.

This is a good thing, as I'm sure it helps me to avoid unnecessary hospital admissions, and means that my doctors are happy to let me leave hospital that little bit earlier, as they know I'm capable of looking after myself.

I know that I'm a 'good patient', but there's always a nagging doubt that maybe I could be doing something differently. Something better.

Articles like this one (which was published in the American Journal of Respiratory and Critical Care Medicine) suggest that difficult asthma is often nothing more than a failure on the part of the patient to take their medications as prescribed.

While I agree that it's sensible to make sure that people are taking their medication before trying additional treatments with potentially serious side-effects, this view does make me feel inadequate, or even defensive. Most of the doctors that treat me are able to see that I am committed to my medical care, but there is the occasional one who treats me as though I know nothing about my treatments, and that I'm in hospital because I'm stupid. This view manifests itself in questions about my psychological wellbeing, whether I know about and take my medications, and questions about whether I smoke or inhale other inappropriate things. Peanuts and dust, for example. Or cocaine. Having patients admit to using hardcore illegal drugs makes this type of doctor very happy.

The sense of disappointment that I feel when it becomes clear that things are deteriorating despite my best efforts is hard to describe. I have such optimism when things go well, and feel so disheartened when they don't.

I think that today's lesson is that I have an illness that fluctuates. Part of the nature of difficult asthma is that it is hard to control, and I need to be forgiving of myself when, despite my best efforts, things deteriorate. However, the fact that it is an illness full of ups and downs does not give me an excuse not to try my hardest to control it. Yes, it's disheartening when I try hard and things go wrong, and I know that even when things go right it's as much due to luck as judgement, but every good day (even every good hour) is worth the effort, and I owe it to myself to give myself every opportunity to live life to the full.


This post was written as part of NHBPM - 30 health posts in 30 days: http://bit.ly/vU0g9J