I love having guest writers at Hearing Elmo because it helps all my readers understand and stay informed about various disabilities that, frankly? I have very little information and certainly a lack of firsthand knowledge. Ruth has written for Hearing Elmo before and I always welcome her contributions. For this post her husband, Gary, contributed as well.
Mitochondrial Disease is in the news again, because of the battle between the parents and the English justice system over the treatment of little Charlie Gard. The form of Mitochondrial Disease that Charlie Gard has is extremely severe and very rare. It has damaged everything in his body to the extent that he requires medical life support to survive.
The core of mitochondrial disorders is energy. Mitochondria is in every body cell (minus red blood cells) and is responsible for converting food to energy. When this does not work properly, it is call Mitochondrial Disease (or Mito for short). Mito affects everyone differently, depending on where and how the person’s mitochondria are affected. Any body system that has defective mitochondria will malfunction and possibly progress to complete failure.
As a person with Mito, people have asked me what the effect of mitochondrial disease is. For me, it means working with multiple medical specialist to maintain the function of weakened organs. Because the degree of energy and fatigue determines the rate at which my organs deteriorate, managing that energy level is my greatest challenge.
Some people use the spoon theory to explain life with Mito fatigue. However, my husband and I wrote another narrative that makes more sense to us. Instead of spoons, we picture a zero balance bank account. Just about, everyone knows what it means to have a bank account and how the balance of that account affects his or her activities. They also know the pain of an overdraft charge.
A healthy person makes energy deposits into their account from food, rest and exercise and has energy left in their account at the end of the day to pass over to the next day. People with Mito do exactly the same thing, but because their energy output is very low, their account drops to zero at the end of the day with nothing to share with the next day.
People with Mito know that all of their body’s involuntary organs like their brain, heart, lungs, ears, eyes, and digestive system need a huge portion of the limited energy they are able to put into their account. The average healthy person is typically unaware of the energy use by their involuntary body systems.
After meeting the energy needs of their involuntary functions, using energy for self-care activities is next. This includes things like bathing, brushing teeth, dressing, or eating. Some people with Mito do not have enough energy in their account to do these things independently, and some can do them independently, but often need to rest to build up their energy account between tasks.
After basic self-care comes necessary health related appointments to the dentist, the primary care doctor, the endless specialists, therapy appointments, etc. The person with Mito has to anticipate ahead of time how much extra energy these appointments will demand. To avoid having their energy balance slip into the red, which means a Mito crash where involuntary body systems are short of energy and struggling to function, they spend days ahead resting more, and building up their account to cover that extra expenditure of energy.
Everyone gets health benefit from exercise. People with Mito have difficulty with repetitive movement, as their energy accounts are often too low to replenish the energy used by their muscles fast enough. Yet getting some exercise, even passive exercise provided by therapists, results in strengthening. It takes considerable effort for people with Mito to balance their energy account with exercise.
Like every other functioning human being, a people with Mito, have the same desire to live independent lives as healthy people do. This can mean parenting children, holding down a job, keeping up their home and yard, shopping, preparing meals, participating in religious activities, attending educational classes, enjoying leisure activities, and keeping up with friends and family. Participation in these ordinary things are difficult, or even impossible, for people with Mito because their energy account balance is too low.
People with Mito can increase the balance in their account by careful budgeting of their energy. They can chose to sit to perform their self-care routines, instead of standing as most people do. They can use a mobility device to save the energy they would use to walk, to do something else. They take advantage of seating that tilts to avoid using the extra energy that it takes to sit upright against gravity. They can chose the timing of their activities to avoid extremes of temperatures, which will drain much energy from their account.
Finally, there are those unexpected life events that may totally empty a Mito person’s energy account: mental and emotional stress (both positive and negative), illness and surgery. A simple cold can be life threatening for a person with Mito, depending the amount of energy left in their account to fight it. It can take days and even weeks for the person with Mito to replenish their energy accounts.
Though I cannot do everything, I want to do, and have struggles with my limitations; life is good, because I have learned by years of practice how to budget my energy. My husband helps too by taking on the more energy consuming activities, while I do the less energy consuming things in our household. This way we can enjoy more of what life has to offer, together.
Ruth and Gary Fox
L. Denise Portis
© 2017 Personal Hearing Loss Journal