The problem is, the human body doesn’t deal well with shock.

It would take a perfect storm of poor circumstances and bad decisions for McArdle’s disease to kill me.  Most people don’t even know they have the disease until they reach their third or fourth decade.  Every disease has its very own sinister quality, and when it comes to McArdle’s, that quality is the ability to hide.  McArdle’s hides from the mother who can’t figure out why her son is so lazy.  It hides from the gym teacher who hates quitters.  It hides from the group of friends who are always one step ahead.  You see, if you’re lucky enough, people expect great things from you.  The mother expects you to adopt her work ethic.  The gym teacher expects you to work hard and run hard.  Your friends expect you to be one of them.  These qualities are instilled in us from our earliest moments.  If we’re lucky.  If we’re lucky, people want us to be great.  They encourage us to be successful.  They demand that we do the best we can.

The problem is, the human condition doesn’t deal well with disappointment.

I chose to swim.  I practiced every afternoon.  I chose to enter the pool.  Sometimes I would swim until I vomited.  I chose to race people who were sometimes twice as fast as me in front of a crowd of strangers.  Every time I stood on the blocks, I knew I was going to be the slowest person in the race.  The guy on the left and the guy on the right would beat me every single time.   People in the stands laughed at me.  My girlfriend laughed at me.  My friends laughed at me.  It should have been humiliating.

It wasn’t.

After the first violent blow of hitting the water started to wane, the first step was to find a rhythm.  There was a count or a beat, some kind of timing mechanism that made me kick and breathe and stroke and process at the same time.  I would settle in and wait for it.  Soon enough the thighs would burn like a stretched rope baking in the sun.  My shoulders would develop this sickening ache.  Hamstrings and calves and forearms would tighten and flex and give and take and shrink and grow and groan and groan and the wall was right in front of me.  I could just stop.  I knew, I just knew that pain was going to come.

It was the only time the battle seemed fair.

In the foggy blue haze, McArdle’s couldn’t hide.  There was no way I could kick and reach and push without it coming out and facing me.  There was no way I could finish a race without facing it.  No disguises.  No secret places.  It was a level playing field.  Just as sure as the pain would come, it would faithfully go.  The ache would turn to wear, the wear would diminish to a dull fatigue, and the dull fatigue served as a familiar plateau.  McArdle’s didn’t shock me when it greeted me during my first lap.  It didn’t surprise me when it felt like it was dragging me to the bottom of the pool.  I didn’t surprise myself when the second wind came and carried me home.  Everything had its place in the water.  Everything played its part.

I only competed for one year.  I didn’t compete in the conventional way.  I knew I wouldn’t qualify for districts.  The only way I’d see the state swim meet was to buy a ticket.  I worked and pushed and had to confront an enemy that didn’t like to fight its battles in public.  After one season, I felt like we had an understanding.

Pain is intimate.  Shock is disturbing.  Disappointment is natural.  Humiliation is a state of mind.  When you learn to keep up with yourself, the answers seem to be easier to come by.  When I jump in the pool these days, I find it to be a peaceful and relaxing experience.  The water holds my form.  The pattern of the stroke and the sweet quiet has a meditative quality.  Taking a break to breathe almost interrupts the trance.  It feels so good to just propel myself to the wall and back with nice slow strokes.
- Ryan, GSD Type V, McArdle’s disease.

    Ryan was born November 1976 in  Iowa.  While he enjoyed the same activities the other kids in his neighborhood enjoyed, it was obvious from an early age that he simply couldn’t keep up.  This inability to run with the pack, coupled with bouts of significant leg pain, led him to the University of Iowa Hospitals and Clinics.  At the age of 11, he was diagnosed with Limb-Girdle Muscular Dystrophy.  This diagnosis came as a shock, and the prognosis was dire.

Armed with this initial diagnosis, his mother took him to Mayo Clinic for a second opinion.  The visit was a blur of voices and white coats, appointments and lab tests, but it culminated in a brand new diagnosis:  McArdle’s disease.  The name was unfamiliar, but the prognosis was welcome.  It was life-altering, but it wasn’t life threatening.

Thanks to the support of his family and expert medical care, he has been able to enjoy a relatively normal life.  Like any disease, McArdle’s does assign unique challenges to those who are affected.  Through this writing, he hopes to illustrate some of those challenges through a personal account of his own experiences.

To learn more about McArdle’s Disease, visit:

GSD Life Type V

Links provided by the Association of Glycogen Storage Disease

Avery’s Hope Fund was established to raise public awareness about Glycogen Storage Disease and to support fundraising efforts to find a cure for this disease.

Take a moment to check them out!

In the coming days and weeks you will see some visual changes to our different sites as well!

It is one of the many exciting things that have been happening behind the scenes with so much more to come!

Table of contents for Feeding Therapy from a Glycogen Storage Disease Type 1a Perspective

1. Part I – What is a Feeding Disorder?
2. Part II- Negative Food Associations
3. Part III- Power and Control Behaviors
4. Part IV- Attention Seeking Behaviors
5. Part V- Caregiver Attitudes
6. Part VI- How Feeding Therapy with GSD is Different

Feeding Therapy Basics
Feeding is a task.  Most people without feeding disorders like eating, they find it pleasurable. Having more food is generally preferred. People with a feeding disorder tend to view eating as a “job.”  A full plate is often just too much work to tackle.  In the beginning stages of therapy food is presented in taste-size bites, then one normal-sized bite at a time, and on up.
Feeding is social.  Many people are uncomfortable eating alone when others are present.  Imagine being expected to put something you know little about into your mouth while everyone in the room sits and watches you.  How uncomfortable! That’s not how every-day eating works.  People eat together and we often try unfamiliar foods only after we see someone else enjoy them.   How brave the first person to eat an artichoke must have been.  Eating the same foods as the child and sharing background information and personal experience with the food items present can encourage food acceptance.
Feeding is easier when foods are familiar.  Feeding therapy often starts with an assessment of what the child is currently taking by mouth (if anything) and therapy moves through “like” foods.  Foods can be alike in texture (liquid, puree, mashable, crunchy, chewy, hard).  They can be alike in color, smell, or flavor.  Therapy can move from already accepted foods to foods that are very similar to accepted foods, to foods that are somewhat similar and slowly expand to include a wide food repertoire of all textures, colors, and flavors.
Feeding can be easier with a new trusted person.  For some reason many children will eat for a feeding therapist and not for their parents using the same strategies.  Even feeding therapists sometimes need to send their own children to an outside feeding therapist.  That’s just how it works sometimes.  Occasional breaks from therapy or switching therapists when progress plateaus are done from time to time to maintain the “specialness” of the feeding therapist and facilitate better progress.
How feeding therapy is different with GSD1a
There are several points, specific to GSD1a, which should be considered when developing a feeding therapy treatment plan.  Firstly, the goal may differ.  The goal of feeding therapy for most children with tubes is to improve oral feeding skills so that the feeding tube can be removed.  That is probably not the goal for a child with GSD1a, at least in the short term.  Therapists may not be aware that the child will have to reliably drink large quantities of raw corn starch, on a strict round-the-clock schedule, in order to have the tube safely removed.
The next thing to consider is the food used for therapy.  Cheese spray, canned frosting and other “un-friendly” foods are feeding therapy staples because of their fun factor, good taste, and extended shelf-life.  Therapists may need information about the GSD1a diet, and the importance of it.  Parents, with advice from the child’s physician and/or nutritionist, should work with the therapist to select foods that will meet therapy objectives.  As with everything in life with GSD, there are no absolutes.  It is possible that for an individual child with GSD1a, chocolate frosting may be the only thing that works in the beginning stages of therapy but, the choice to use frosting should be child centered and not based on therapist habit or convenience and should be cleared with the child’s physician.
Therapists, as a group, are used to largely ignoring “healthy” foods in favor of getting the child to eat; an effective strategy for typical feeding therapy kids.  Eventually, children learn to accept other foods and eat a healthy diet.  GSD1a presents an additional challenge in the form of extensive dietary restrictions. A consideration when choosing therapy foods is functionality.  How functional (or useful in everyday life) is it for a child with GSD1a to eat frosting?  How many GSD friendly foods have a similar look, taste, or texture to frosting?  With functionality in mind, therapists might choose different foods.
Another GSD specific difference is the need to do feeding therapy when the child is not hungry.  A typical feeding therapy practice is to delay or skip tube feedings before therapy sessions so that the child experiences hunger and is therefore more likely to eat.  This strategy could be dangerous for a child with GSD1a and there is no reason to take that risk.  Needing to eat when not hungry is a fact of life at some point for every child with GSD1a.  Feeding therapy performed around this obstacle functionally prepares a child with GSD1a for everyday life.
Conclusion
The factors that cause feeding disorders and perpetuate them are varied and there is never just one quick tip that will solve the problem.   Establishing functional feeding skills is hard work.  Families can help a child to overcome feeding disorders and develop healthy patterns, usually with the help of a feeding therapist.  It is hoped that the information presented here will empower parents to take an active role in the therapy process.

Table of contents for Feeding Therapy from a Glycogen Storage Disease Type 1a Perspective

1. Part I – What is a Feeding Disorder?
2. Part II- Negative Food Associations
3. Part III- Power and Control Behaviors
4. Part IV- Attention Seeking Behaviors
5. Part V- Caregiver Attitudes
6. Part VI- How Feeding Therapy with GSD is Different

Caregiver attitude toward feeding and food is a subject that is not always addressed by therapists because it can be uncomfortable to discuss but, it can have a tremendous impact on a feeding disorder. It’s an understatement to say that being the parent of a child with GSD is hard. It’s fair to say that every family raising a child with GSD has a degree of increased stress. Part of the management of GSD1a often includes eating prescribed food types at specific times and, when you have a child who is resistant to feeding, parental stress is a predictable consequence. Managing the stress and not allowing the stress to manage the situation is the key to a better outcome. That’s much easier said than done but is tremendously important. The presentation titled “Coping With GSD, What’s the Secret” by Lisa Merlo, Ph.D. M.P.E. available on the GSD Life web site, is a good resource for suggestions to dealing with family stress related to GSD. Parents should remember to model the behavior they want to see in their child. Adults can eat the same foods the child is encouraged to eat (GSD friendly) and show how much they enjoy eating. Making the GSD diet a “big deal” to the child (I’m so sorry you can’t have the “good” foods) could be detrimental. Having a plan of action to take the place of an emotional response is important.

Table of contents for Feeding Therapy from a Glycogen Storage Disease Type 1a Perspective

1. Part I – What is a Feeding Disorder?
2. Part II- Negative Food Associations
3. Part III- Power and Control Behaviors
4. Part IV- Attention Seeking Behaviors
5. Part V- Caregiver Attitudes
6. Part VI- How Feeding Therapy with GSD is Different

Attention Seeking Behaviors
Like all children, kids with GSD seek attention to one degree or another.  The facts of GSD life can influence what specific behaviors a child chooses to get attention.  For example, a typical attention seeking child might use a loud voice and jump around pulling on her mother’s hands to interrupt a phone call.  A child with GSD might lie still and quiet in a corner.  A parent of a GSD child cannot ignore a “too quite” child as they can an overactive loud one, making “too quite” a very effective strategy to get attention.  Adults can recognize attention seeking behavior by asking the question “Am I irritated, annoyed or scared?” If the answer is “yes,” chances are the behavior is motivated by attention seeking.
Eating, or more specifically not eating, can garner more attention for a child with GSD1a than for a typical child because of the character of the disease.   Not eating can prolong the amount of time caregivers spend with a GSD child “getting him to eat” and that extra time is rewarding when attention is the goal.  Something to note is that attention seeking children typically do not distinguish between positive and negative attention.  Attention (adult focus on the child) is the goal, not causing the parent to be pleased or getting a “good” reaction. Any adult reaction will do, so long as it is focused upon the attention seeker.
When attention seeking motivates feeding behavior, giving attention to the preferred behavior (eating) and not to non-preferred behavior (not-eating) can help.  In the beginning over-enthusiastic praise for eating and ignoring non-eating is needed.   For example, a mom might seat Tommy at the table with his prescribed corn chip snack.  Tommy knows he needs to eat his snack so his mother doesn’t talk to him or interact beyond opening the bag. As soon as Tommy puts a chip into his mouth, mom is right next to him talking about how good the chips are or how grown-up he is getting.  Mom stays as long as eating continues but turns attention away if feeding stops before the prescribed snack is eaten.  All behaviors that are not eating (dropping the chips on the floor, saying “mom,” crushing the chips to powder) are not “noticed” or given attention.
A child may eat more with physical parent contact and full focused attention. Sometimes getting to sit on Dad’s lap while eating a snack, knowing that Dad will stand up as soon as non-eating appears, is enough.  A feeding therapy goal may be to accept food within a set time limit (usually 5 or 10 seconds).  A toy and enthusiastic interaction occur when the child eats within the time limit.  No response, beyond placing another bite on the plate, is given when the child does not eat “in time.”
Just as for power motivated behaviors, having a contingency plan to deal with not eating is key.  Knowing ahead of time what to do if a snack or meal is skipped or delayed makes it easier for a parent to avoid the “not-eating circus” that can occur around a GSD child.
Something to note when first using any of the strategies described in the last two sections is that the feeding behaviors are almost always long-standing patterns.  They cannot be changed over night with a “quick tip.”  Behaviors often get worse before they get better as kids try more intense versions of the “old way” before using new patterns.  Persistence and patience are musts.  All caregivers consistently following the same plan is paramount.

Table of contents for Feeding Therapy from a Glycogen Storage Disease Type 1a Perspective

1. Part I – What is a Feeding Disorder?
2. Part II- Negative Food Associations
3. Part III- Power and Control Behaviors
4. Part IV- Attention Seeking Behaviors
5. Part V- Caregiver Attitudes
6. Part VI- How Feeding Therapy with GSD is Different

Power and Control Behaviors
The source of feeding disorders in some children is assertion of power and control; “You can’t make me eat.”  One of the facts of having a condition like GSD is an increase in the number of non-preferred things a child “has to” do.  It is understandable then that some of these little ones will exert control when and where they can, often at meal-time.  Such assertions of power tend to cause caregivers stress and anxiety and the resulting reaction can make meal-times unpleasant for all concerned.  Negative food experiences further reinforce the pattern of poor feeding and the cycle continues.
To combat this, parents can build in as many choices as possible for the child throughout the day, including at mealtime.   A variety of food choices, favorite plates and cups, choice of where to eat, and more can be offered.  Food choices can be presented as a choice between items or as a rejection of all but one.  “No,” is a powerful word and using it with boundaries can be motivating and effective at encouraging a selection (I have four snack choices; chips, pretzels, corn chips, or toast.  You can say “no” three times).
Avoiding a power struggle is always better than engaging in one if the goal is a pleasant meal-time.   A good way for a parent to recognize a power struggle is to ask the question, “Am I angry?”  If the adult is angry, chances are that “power” is underlying the child’s behavior.  The only way to “win” a power struggle is not to have one.  Parents can develop a contingency plan that addresses what to do if the child chooses not to eat a prescribed snack.  The child’s doctor should be consulted and the plan ready to go to when the situation arises.  Caregivers should avoid making the food refusal a “big deal.”
Punishment or consequences (natural or otherwise) that are effective for children without feeding disorders and GSD, such as letting the child go hungry, are simply not safe options for kids with GSD.  Parents can make behavior plans and contracts that include feeding behaviors but, any  consequence should be “delayed” and not occur in physical proximity food or during mealtime.  Sticker charts and point systems that include feeding tasks often work but should be completed outside of the kitchen, away from food unless a reward is earned.
Parents should also be aware that sometimes praise can be a form of “punishment” for a child with power motivated behaviors.  “Yeah! Tommy ate three chips!  Hurray!” is appropriate when putting chips into the mouth and swallowing them is a relatively new skill.  That same sentence can mean, “You did what I wanted you to do; you lose,” to a child who is exerting power over feeding.  Any responsible caregiver of a child with GSD1a will take note of what the child eats, but sometimes doing so unobtrusively is a good idea.  A father may hand Tommy his snack and then make a phone call.  The snack is adequately supervised but the pressure a child might feel from being watched is lessened.
The eventual goal is that the child will notice that it’s snack time and feed himself without adult help.  Encouraging independence from an early age, to the degree safety allows, can head off power struggles.  A sense of power and positive feelings of accomplishment come with responsibility. A family might store GSD friendly snacks in child accessible areas for independent retrieval and choice making, teach time-telling, or create a health plan in simple language the child can read and understand.
In persistent cases, a therapist might suggest activities that “turn the tables” on the parent-child dynamic.  During such therapy sessions, the child directs all feeding behaviors.  She may tell her mother what to eat and how, or may even spoon feed her parent.  During prescribed snack time, the child may choose between two items with the understanding that “Daddy has to” eat the other choice.  Such activities can be helpful in breaking the power and control cycle and create the opportunity for the parent to model good feeding skills.

Table of contents for Feeding Therapy from a Glycogen Storage Disease Type 1a Perspective

1. Part I – What is a Feeding Disorder?
2. Part II- Negative Food Associations
3. Part III- Power and Control Behaviors
4. Part IV- Attention Seeking Behaviors
5. Part V- Caregiver Attitudes
6. Part VI- How Feeding Therapy with GSD is Different

Depending on the history of the individual, particularly when he or she was diagnosed and other co-occurring illnesses, eating orally may have caused illness and even physical pain in the past.  Human beings naturally avoid pain.  Behavior can change as a natural response to these negative experiences, even if the person cannot truly remember or articulate “why” they don’t like to eat.
Behaviorism teaches that it takes many positive experiences to counterbalance one negative experience.  If there are negative food associations, it is exceptionally important to make eating orally a positive experience.   This is why therapists teach children to play with their food.  Parents can encourage these positive associations by making meal-times child centered.  They can structure the day to allow for low pressure “no rush” meals, save a special toy for meal times only, cut foods into interesting shapes, and use food coloring if the child can have artificial colors.
Some children with negative food associations and/or oral aversion may even gag at the sight or smell of food.  Feeding therapy for these children begins with desensitization.  Depending on the individual, therapy may start with tolerating pictures of food items, then tolerating real food items in closer and closer proximity, touching and manipulating food, allowing food to remain on the body and eventually to touch the face and lips, accepting food into the mouth and spitting it out, and eventually progress to accepting food and swallowing it without gagging.
Replacing the gagging behavior with a tongue posture that is not compatible with volitional gagging sometimes helps. Children can be encouraged to stick their tongue hard to the roof of the mouth if they feel that they might gag.  This strategy should be used only when there is no food in the mouth.  Over time, that “no gag” posture can be shaped into a swallow with the tongue held on the bumpy ridge behind the teeth.

Table of contents for Feeding Therapy from a Glycogen Storage Disease Type 1a Perspective

1. Part I – What is a Feeding Disorder?
2. Part II- Negative Food Associations
3. Part III- Power and Control Behaviors
4. Part IV- Attention Seeking Behaviors
5. Part V- Caregiver Attitudes
6. Part VI- How Feeding Therapy with GSD is Different

Definition of Feeding Disorder
Feeding disorders include problems getting food into the mouth, holding it there, forming a cohesive ball of food that’s safe to swallow, and choosing to do these things effectively.  Lots of children are “picky eaters” but a disorder exists when it impacts the health of the child.  With health impact in mind, a child with GSD1a may be considered to have a feeding disorder when another child with the same feeding behaviors is just a “picky eater.”

Factors Related to Feeding Disorder in GSD1a
Feeding tubes, negative food associations, power and control behavior, attention seeking behavior, and caregiver attitudes can all impact feeding.  There is never one cause or reason a feeding disorder persists, but rather a mix of many factors.  Understanding these underlying factors can help to encourage good feeding skills.
There are also aspects of GSD1a that influence how feeding therapy is performed.  Because GSD is a rare disease, few speech pathologists and occupational therapists will have background knowledge related to the disease itself.  Families can help by partnering in the therapy process and understanding a bit about the differences between “usual” feeding therapy and feeding therapy with GSD1a.

Feeding Tubes
Feeding tubes are a controversial topic in the GSD world and a personal decision.  Many GSD parents find feeding tubes, particularly for a GSD child who is reluctant to eat, indispensable.  As many children with GSD use feeding tubes and feeding tubes do have a relationship to feeding disorders they are an important part of this discussion.
A consequence of not using the mouth to get all nutrition is that there are a limited number of opportunities to practice using the mouth to eat.  Eating is a skill few people think about but, it is a tremendously complex process.  It might be easier to imagine how walking, another complex but mostly automatic skill for almost everybody, would be delayed if a toddler had casts on both legs before he learned to walk.  In that case, he would not have developed the balance, coordination, and strength to walk like other children his age.  We would not expect him to run and play on the playground the day the casts were removed.  Feeding is a developmental skill, just as walking is.  Children with limited opportunities to practice feeding do not develop the same coordination and muscle strength as other children their age without extra help and practice in that area.
There are a few things that can be done to help prevent feeding problems related to feeding tube use.  These strategies cannot take the place of feeding therapy, when it is indicated, but are general suggestions.  First, stimulate the mouth when the stomach is being filled.  This is especially important for infants.  Infants should be given a pacifier or other oral stimulation during feedings. Older children can complete a tube feeding much faster than eating by mouth so; increased practice using the mouth at times other than meal-time is advisable.  Including “Chewy Tubes,” chewing gum, chewing on drinking straws, using an oral vibrator (z-vibe), or nuk® brush in the daily schedule is a good idea.   Such activities decrease “oral aversion” (the dislike of having things in the mouth) and can increase muscle strength.