A Natural Approach To Health
Living With Pompe Disease
I had a question the other day about Pompe disease.
Pompe disease is a progressive, multisystemic, debilitating, and often fatal neuromuscular disorder.
It was first defined in 1932 by Dutch pathologist Joannes C. Pompe.
In 1963 the disease was linked to an inherited deficiency of an enzyme called acid alpha-glucosidase (GAA), which is responsible for the breakdown of glycogen to glucose.
The result is an accumulation of glycogen, mainly in muscle cells, that leads to a progressive loss of muscle function.
Pompe disease can vary widely in terms of severity of signs and symptoms, rate of progression, and age of onset.
Pompe disease is caused by a gene mutation.
Continuous accumulation of glycogen causes swelling and rupture within cells.
This in turn leads to progressive decay of skeletal muscles, including respiratory, and, primarily in infants, cardiac muscles, eventually resulting in loss of function.
In children and adults the disease usually progresses slower than in infants, with little or no cardiac involvement, but it’s always relentlessly progressive and associated with significant misery and/or premature death, as an abrupt and rapid decline can happen at any time.
All patients with Pompe disease exhibit progressive muscular weakness.
Limbs and respiratory muscles are the most commonly affected across all ages.
Disease management strategies may include medications and lifestyle approaches to symptom relief, as well as interventions to delay serious complications due to muscle deterioration.
The main focus of medical treatment is enzyme replacement therapy, which replaces GAA.
Non-medical treatment focuses on keeping your body as healthy as possible in an attempt to delay progression and ease the burden of living with the disease.
To deal with Pompe disease it’s beneficial to:
*Drink 6-8 cups of purified water daily.
*Eat fresh, raw fruits and veggies; organic when possible.
*Include some protein with every meal and snack.
*Eat several smaller meals during the day instead of 1 or 2 large ones.
*Choose whole-grains versus “white” products.
*Increase your fiber intake.
*Deep breathing; relaxation techniques.
*Consider liver, colon and arterial cleanses.
*Manage your weight; you can follow my healthy weight blog at blog.dickandlenay.com.
*Eliminate processed, instant, sugar-added, chemical-laden, and junk foods.
*Eliminate white flour products, high carbohydrates, and sweets.
*Eliminate caffeine, soda pop, alcohol, tobacco.
*Eliminate hydrogenated and trans fats.
*Changing the size, texture, or thickness of foods can make them easier to eat, and minimize the risk of inhaling them.
*You may benefit from an exercise program to increase muscle strength, have less stiffness, and improved mental health.