Spoon Theory for Late-onset Pompe Disease Patients
If you have late-onset Pompe disease, you’re likely dealing with muscle weakness, aches, and cramps, and possibly headaches and fatigue. Many people may not understand how overwhelming all this can be, and it may not always be easy to explain. However, an analogy called the spoon theory can help family and friends understand what you go through regularly.
What is the spoon theory?
The spoon theory is an analogy conceived by a lupus patient, Christine Miserandino. She used it to explain to an inquiring friend what it’s like to live with a chronic disease.
According to the theory, you start each day with 12 theoretical spoons. You have to give up one spoon for each task you perform: brushing your teeth, putting on clothes, visiting the doctor, making dinner, etc. When you finish using up all the spoons, that’s it. You are done for the day.
Healthy people have all the energy necessary to do whatever they need to do on a given day. They, unlike you, have a seemingly infinite spoon supply.
The spoon theory illustrates how those with a chronic disease — as well as their caregivers — have a finite amount of energy that must be carefully rationed. Opting to perform one errand or task can limit what you can do for the rest of your day.
How does it apply to Pompe disease?
Pompe disease is characterized by the abnormal buildup of a sugar molecule called glycogen inside cells. This buildup impairs the working of different organs and tissues, especially the heart, respiratory, and skeletal muscles.
The disease is caused by mutations, or errors, in the GAA gene, which encodes for an enzyme called acid alpha-glucosidase. Acid alpha-glucosidase normally breaks down glycogen into glucose. As a result of the mutations, glycogen builds up in tissues and causes damage.
The three main types of Pompe disease each differ based on the age of onset and disease severity. Two-thirds of patients have the late-onset type.
Symptoms of late-onset Pompe disease can include:
- progressive muscle weakness, especially in the legs and trunk
- mobility problems, including difficulty walking and exercising, and an increased chance of falls
- breathing problems and shortness of breath
- frequent lung infections
- morning headaches
- weight loss
- difficulty swallowing
- difficulty hearing
- scoliosis, or abnormal curvature of the spine
Because you can manage some of these symptoms to stay somewhat active, those closest to you sometimes discount or overlook the disease’s toll on your life.
How do I put spoon theory into use?
Understanding that you have only so much energy renders daily prioritizing and planning crucial. Show yourself compassion if you don’t complete everything you set out to do. When you’ve spent all your energy, you are done for the day.
Practicing self-care is vital. If part of that means “using a spoon” for, say, practicing with a walking aid in lieu of another get-together with friends, then so be it. You know your body and needs best.
When you’ve exhausted your spoon set, ask for help from others.
Last updated: Nov. 10, 2020
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