DYSPHAGIA - WHAT IS IT?

This is the name for a medical condition that most people are aware of…..unless you have it.  It means that you have difficulty swallowing.  Yes, it is one of the conditions that accompany some forms of muscular dystrophy.  It is also very common in older people.  It is also a condition, if severe enough, that can be terminal.

The first time that I experienced dysphagia…..I thought I was going to die.  Yes, I thought I was going to choke to death.  I was eating some Chinese pad thai, which is a dish that has thick rice noodles.

I swallowed and the food stuck in my throat.  I had never experienced that problem before, and did not know what to do.  I broke into a sweat, with my heart racing.  I was trying to get someone to help but I could not speak.  I was finally able to swallow after about twenty minutes.   Needless to say, I don’t eat pad thai anymore.

If you have Dysphagia here are some solutions that I have found to help. 

(1)  Increase your muscle mass, as the muscles in the throat are impacted by the muscular dystrophy.  There are a number of posts that I will do on this subject.

(2)  Cut your food into small pieces.

(3)  Chew all your food well before swallowing.

(4)  Do not try to swallow large amounts of food at one time.

(5)  Do not eat when you are in a hurry….. take time to enjoy your food.

(6)  Do not eat pasta dishes, breads or any other food that has caused you to have a problem.

(7)  Always have a glass of water with every meal.

(8)  Do not drink really cold drinks as that cause the muscles to cramp, which can be painful.

(9)  If the dysphagia is severe enough you should have your food processed in a blender.

RESOLARIS - THE CURE FOR FSHD?

As I have mentioned in my previous posts, there are no current approved medical treatments that cure any type of muscular dystrophy.  There is truly exciting news  about a clinical trial for Resolaris, a potential solution for people with facioscapulohumeral muscular dystrophy (FSHD).   FSHD typically starts in the face and upper body and progresses to the lower body.  People with FSHD typically experience debilitating fatigue and chronic pain.   FSHD can be fatal, especially with those who have an early onset.  FSHD is typically diagnosed by the pattern of muscle weakness, DNA testing and other symptoms.

Resolaris is a new drug developed by aTyr Pharama,  a San Diego based company.  Resolaris is derived from protein released into the blood by human skeletal muscle cells.  Resolaris is administered intravenously.  aTyr believes that Resolaris will provide help to people with rare myopathies with excessive immune cell involvement.

The clinical trial is the first administration of a naturally occurring protein derived from physiocrines.  Pysiocrines are a new class of physiological modulators that promote the process of restoring stressed or diseased tissue to a healthier state.  The clinical trial is scheduled for 44 FSHD patients in the European Union.  The trial is designed to evaluate the safety, tolerability, pharmacokinetics and biological activity of adult patients.

For more information about Resolaris please visit the aTyr websitehttp://www.atyrpharma.com.

PERCEPTIONS

People have perceptions that doctors know everything about muscular dystrophy.   A general practitioner will not be as knowledgeable about the disease due to the broad variety of issues that they deal with.  If you have any questions that you might possibly have a form of muscular dystrophy go directly to a neurologist who deals with muscular dystrophy and the related diseases.

Obviously, neurologists who specialty is neuromuscular diseases do know quite a lot about the various types of muscular dystrophy.  Actually, they are quite adept at diagnosing this disease.

Since there are no known cures available, I need to outline that this is your medical issue.  This is your fight, obviously you doctor can help, but you need to take the responsibility to beat this disease……no one else can do it.

This disease will beat you or you can beat this disease.   In other words…..blaze your own trail.  Do the things that are going to help you deal with this crippling rehabilitating disease.  Don’t believe that there is nothing that you can do to help you live with this!

DEPRESSION

Depression impacts nearly everyone that has muscular dystrophy (MD).  Yes, most of us have to deal with it since MD impacts our lives so significantly.  Once you understand that you have this disease it is difficult to deal with the reality.  Especially, once you understand all the ramifications and that your life will never be the same again.
   

When I was informed that I had muscular dystrophy, it was as if I had been run over by a truck.  The doctors indicated that FSH runs in families.  So, I thought that the doctors had to be wrong again (denial). I am the exception to that rule, as I did not have FSH or any other type of muscular dystrophy on either side of the family.

That is when depression set in for three days…it was like a black hole that I could not get out.  I was depressed about the difficulty of the situation.  Then, I decided that I was going to find a solution and BEAT this disease.  So, I started researching MD and any possible solution.

So, the first thing to do is to recognize it for what it is!  Here are some of the classic symptoms.

1.    Feeling of sadness

2.    Feelings of worthlessness

3.    Lack of interest

4.    Lack of focus

5.    Thoughts of suicide or death

 SUMMARY:

Depression is a normal emotional condition with any major disease.  If it becomes more pronounced you should seek competent medical care.  I chose to not discuss it with the doctor or take any type of medication.

SUGAR - THE SILENT KILLER

In my previous post on Ph Balance & Muscular Dystrophy, I mentioned that a low Ph level, which is acidic, can have a very negative impact on muscles.   Ok, I admit that I have a sweet tooth….I am sure that many people do.  One of the worst foods that you can consume is sugar.  Excessive sugar intake has the effect of making the body acidic.  The muscles do not perform well in an acidic environment.

In fact, excessive sugar intake is bad for everyone.  Since it causes the body to become acidic, an acidic environment encourages the breeding of fungus, mold, bacteria and viruses.  This is in addition to the other well know concerns such as obesity, type 2 diabetes, high blood pressure, heart disease, stroke and tooth decay.

Obviously, most people know that there is sugar in cakes, deserts, jelly, sodas since those foods are sweetened with either sugar or high fructose corn syrup.   Sugar is in more food products that most people would realize.  There is sugar in cereals, juices, ketchup, many sauces like pasta and barbeque sauces, mustard, bread and many other products.

You have to read the labels to determine how much sugar is in the processed foods that we use every day.  When reading labels, these are the names of various sugars high-fructose corn syrup, corn syrup, fruit juice concentrate, molasses, honey, dextrose, fructose, glucose, lactose, maltose and sucrose.  Usually they are grams, a teaspoon of sugar equals to 4 grams.

The American Heart Association recommends strict limits on added sugars….150 calories (about 9 teaspoons) a day for men, and 100 calories (about six teaspoons) a day for women.  This is a general guideline for added sugar.  For comparison purposes, a regular size 12 oz. soda contains eight teaspoons of sugar or about 130 calories.

So, it is important to limit the amount of sugar intake that you do daily.  I reduce my intake of sugar products by (1) reading labels when shopping and eliminating purchases with high sugar, (2) cutting portions of anything with sugar and (3) using Stevia as a sweetener for tea, coffee, and other liquids.  I do not recommend the other sweeteners due to the controversial safety claims.

PH BALANCE & MUSCULAR DYSTROPHY

As I have mentioned in previous posts, one of the best things that you can do is tune up your body.  If you keep your body in optimum shape, this will slow or reduce muscle deterioration.  Ph balance is very important to people with muscular dystrophy.  When the body is acidic it takes out alkaline minerals like calcium, magnesium, and potassium from your bones, teeth, and organs to neutralize the acids.

These minerals are essential to proper muscle function.  Therefore, if these minerals are depleted in your body, you will experience faster muscle breakdown.

Magnesium is another mineral that is required for proper muscle function.  It also helps with over 300 other reactions in the body. It works along with potassium to support proper muscle function.   The lack of magnesium will result in symptoms of muscle weakness, muscle twitching and muscle contractions.   Magnesium is also important to muscle relaxation, which is important for healing after exercise.

Magnesium absorption is important so, magnesium glycinate, citrate and aspartate are highly absorbable forms of magnesium.   Patient studies have shown that magnesium aspartate helps relieve muscle pain.

Potassium is important as a nutrient since it helps to build proteins which are the building blocks of muscle.  The lack of potassium in the diet will result in similar symptoms to lack of magnesium.  Bananas are a good source of potassium as well as other minerals and vitamins.  Potassium is also important in controlling the acid – alkaline balance.

It is important that you keep your body in a positive ph range of 7.2 – 7.4.  This can be done ideally thru eating nutritious alkaline foods and proper vitamin and mineral supplementation.   It is relatively easy to accomplish with a diet of vegetables, leafy greens, sprouts, avocados and green juices.  If we flood our bodies with vitamins, minerals, oxygen and chlorophyll we will create healthy cells.  Conversely, an acidic diet high in animal products, processed carbs, and refined sugar will accelerate muscle deterioration.

Another alternative method of maintaining ph level is to drink alkaline water.  Alkaline water is available at some grocery stores or you can purchase water filter dispensers.

The alkaline levels can be measured by purchasing ph testing strips.  You can test your urine at home with litmus paper strips, which can be purchased in drug stores or online for under $15.  It is best to test your second urine of the day to get an idea of your ph. Your urine should fall in the 6.8 to 7.5 pH range.   Please keep in mind that the ph changes depending on what you eat and when.

References:

National Institute of Health – Calcium in diet         http://www.nlm.nih.gov/medlineplus/ency/article/002412.htm

National Institute of Health – Magnesium in diet: http://www.nlm.nih.gov/medlineplus/ency/article/002423.htm

National Institute of Health – Potassium in diet:     http://www.nlm.nih.gov/medlineplus/ency/article/002413.htm

PROTEIN

One of the best things that you can do to help with muscular dystrophy (MD) is make sure that you are eating a proper diet.  Your diet should include a high amount of protein.  The muscles use protein to build muscle, so therefore a lack of protein will make the breakdown of the muscles accelerate in someone with muscular dystrophy.

It is estimated that the average man should consume at least 56 grams of protein per day, and the average woman should consume at least 46 grams per day.   People with muscular dystrophy should consume additional protein above the daily suggested limits.

Since people with muscular dystrophy are typically less active it is best to have a high protein diet.  This high protein diet will help with the muscular dystrophy and reduce weight gain.  Here is a list of high protein foods.

1.    Chicken/turkey

2.    Fish

3.    Cheese

4.    Lean pork

5.    Lean beef

6.    Tofu

7.    Beans

8.    Eggs

9.    Yogurt, milk, coconut or soy milk,

10. Nuts

With our busy lifestyle, it is easy to not eat properly.  Another method of obtaining the proper amount of protein daily are protein shakes, drinks and bars which are available at many retail locations.

MUSCLE BUILDING SUPPLEMENTS

One of the most important things that you can do to deal with muscular dystrophy is to take muscle building supplements.    The most widely accepted muscle building supplement is creatine,  Creatine is a natural substance in the body, it also comes from some protein rich foods such as meat or fish.

Creatine is typically used to improve strength, increase lean muscle mass and help the muscles recover after exercise.  It is widely used by athletes from high school to professional sports.  It is estimated that approximately 50% of college and professional athletes use creatine.

Not everyone responds well to creatine, it is not tolerated by everyone and may not work for every person.  So I would suggest that if you try creatine…. research side effects and start at a low dosage of one gram and build up from there.  Also, purchase only high quality German  creatine, otherwise your wasting your money.

It is recommended that you drink at least a pint of water when taking creatine….water helps the muscles absorb the creatine.

I have found that taking HMB – Hydroxyi-Methybutyrate-Monohydrate works well.  HMB is a natural compound that is found in the body, it is created in the body from leucine.  Studies have shown HMB to decease muscle damage after exercising.  A good time to take HMB is after a strenuous exercise.

Creatine or HMB should be taken as part of a total supplement program including L- Arginine, and L-Glutamine.  If you are younger than 40, HMB may not be as effective as creatine.

PROPER SUPPLEMENTATION OF VITAMINS AND MINERALS

There are a number of good reasons to take vitamin and mineral supplements.   Perhaps best known are the lack of vitamins and minerals in the processed and fast foods we eat.  We have really busy lives and have a tendency to eat fast and easy meals.   Another is that current agricultural techniques leave the soil depleted of important minerals, causing the foods that are grown to be deficient of important minerals.

Upon discovering that I have muscular dystrophy, I knew that I had to do something or else I was going to have major problems.  Since the doctors had indicated that there were no know treatments or cures for Facioscapulohumeral (FSHD) muscular dystrophy.   I decided to so get as fit as possible by eating nutritious foods and researching possible solutions.

Then I began a supplement program to get my body in good condition.  Muscular dystrophy depletes a number of vitamins and minerals from your body, so taking vitamins is important.

A good vitamin and mineral supplement was the first order of business.  Most vitamins on the market, especially the ones in the stores are synthetic vitamins.  Synthetic vitamins that are not absorbed by the body well, then there are differences in the quantity of the vitamins in the supplements.  So, you don’t know if you’re getting enough of the necessary vitamins and minerals.

There are a number of producers that provide a high quality product.  There are a number of sources for information on which are the best vitamins and mineral supplements to take.  One of the best is sites is www.consumerlab.com.   They test vitamins and herbs by brand and report the results of their tests.  I would strongly recommend that you subscribe to use as guide to your supplementation.

Your vitamin and mineral supplement should contain at a minimum 100% of the recommends daily dose of the following vitamins and minerals A, C, E, B6, B12, magnesium, calcium, zinc, and selenium.

As I do into more posts, I am going to bring out a number of supplements that have helped me deal the FSHD.

FSH, FSHMD, FSHD, FACIOHUMEROSCPULAR

This post is about FSH called  faciohumeroscapular) (FSHMD, FSHD or FSH), and it also known as Landouzy-Dejerine, This is a common form of muscular dystrophy impacting approximately 7 out of every 100,000 people.  This form of muscular dystrophy in inherited from one of the parents and effects the skeletal muscles of the face (facio), upper arms (humeral) and the scapula (scapula)

The disease was discovered in 1884 by French physicians Louis Landouzy and Joseph Dejerine. In their research paper of 1886, Landouzy and Dejerine drew attention to the fact that this form of muscular dystrophy ran in family groups.   Although, there are cases where there is no family history of muscular dystrophy.

Symptoms usually develop in early childhood.  Approximately, 95% of the individuals have symptoms are that are apparent starting in their teen years.  This disease advances with progressive weakness to the face, shoulders and upper arms.  It also may impact other skeletal muscles.

FSH is characterized at first by weakness in the facial and shoulder muscles.  Then over a period it can spread to other muscle groups.   Muscle weakness in the pelvis and hips can make it difficult to climb stairs or walk for extended distances.

The person in the picture above is a classic example of FSH.  Note that the weak shoulder muscles tend to make the shoulder blades protrude from the back, a common sign known as scapular winging.  Due to the weakness in muscles of the shoulders and upper arms it can be difficult to raise the arms over the head or throw a ball.

TYPES OF MUSCULAR DYSTROPHY

There are currently thirty types of muscular dystrophy.  Here is a list of the most common types and who they impact.

Duchenne (DMD) – the most common and severe form of MD among children, DMD accounts for more than 50% of all cases. DMD appears typically in boys between ages 3 and 5 and progresses rapidly. Most people with DMD are unable to walk by age 12 and may later need a respirator to breathe. They usually die in their late teens or early 20’s from heart trouble, respiratory complications, or infection.

Becker MD – Appears primarily in males between ages 11 and 25. Some people may never need to use a wheelchair, while others lose the ability to walk during their teens, mid-30’s, or later.

Myotonic MD – The most common adult form,  myotonic MD appears in two forms, type 1 and type 2.  Myotonic MD affects both men and women between ages 20 and 30.

Congenital MD – This form of MD appears at birth or by age 2. Congenital means “present from birth.” Congenital MD affects both boys and girls, who often require support to sit or stand and may never learn to walk. Some patients die in infancy, but others live into adulthood with only mild disability.

Emery-Dreifuss MD- Symptoms usually begin by age 10 in boys and can appear in patients up to their mid-20’s. People with this form often develop heart problems by age 30, and they may die in mid-adulthood from progressive pulmonary or cardiac failure.

Facioscapulohumeral (FSHD) – FSHD refers to the areas affected: the face (facio), the shoulders (scapulo), and the upper arms (humeral).  FSHD affects teen boys and girls typically but may occur as late as age 40. Most individuals have a normal life span, but symptoms can vary from mild to severely disabling.

Limb-girdle MD – This form of MD can appear in childhood but most often appears in adolescence or young adulthood. Limb-girdle can progress quickly or slowly, but most patients become severely disabled.  This form impact the limb-girdle area with progressive muscle damage.  It may impact the ability to walk within 20 years of developing the disease.

Distal MD – Distal MDs refer to a group of diseases that affect the muscles of the forearms, hands, lower legs, and feet.  This form typically appears between ages 40 and 60. Distal MD is less severe and progresses more slowly than other forms of MD, but it can spread to other muscles. Patients may eventually need a ventilator.

Oculopharyngeal MD – This form occurs in both men and women, and it can be mild or severe. This form of MD typically appears in a person’s 40’s or 50’s. Some people will eventually lose their ability to walk.   This form is common among Americans of French-Canadian descent,  and Hispanics from the Southwestern U.S.

SUMMARY: 

Each form of muscular dystrophy can impact people differently.  It is important to get a confirmed diagnosis, if you exhibit any of the symptoms.  In addition, there are other medical issues that can cause similar symptoms.

MUSCULAR DYSTROPHY

Muscular dystrophy is an inherited muscle disease which is characterized by weakness and atrophy in various muscle groups.   There are thirty different types of muscular dystrophy, with symptoms that range in rom mild to extremely severe.  The different types also manifest at different ages and impact different parts of the body. I will go into more detail on each type and the implications in separate posts.

The most common forms of muscular dystrophy in children are Duchenne and Becker, approximately 1 in every 7,000 males.  The most common adult form of muscular dystrophy is Myotonic type 1, which affects approximately 1 in 8000 adults.  The incidence of muscular dystrophy (MD) in the United States varies, because different kinds of MD are rarer than others.

There are a number of tests that can be run to make an accurate diagnosis.  Also, other family members can be tested to see if they have the muscular dystrophy gene.

There are solutions on the horizon that will conquer this disease, the foremost  is stem cell therapy.  Actually, there are a number of companies that are performing stem cell therapy outside the United States.  I would urge caution before paying money to anyone, as there have not been any proven therapies or cures.  I will dedicate a future posts to these potential treatments and cures.

Currently, according to contemporary medicine muscular dystrophy cannot be cured, but there are various treatments which can help.

WELCOME WORLD

Thank you for visiting my blog!  The purpose of my blog is to give you Hope that Muscular Dystrophy can be managed and even one day cured.   This blog is dedicated to help the people that have muscular dystrophy, their families and the caretakers.

I have done thousands of hours of research trying to find solutions.  So, I am going to give practical solutions to problems related to muscular dystrophy.

A treatment for muscular dystrophy will be developed that cures muscular dystrophy.    Muscular dystrophy will be diagnosed, treated and the patient will be cured of any reoccurring symptoms.

Currently, the great hope for a cure is stem cell therapy.  Also, there are also a number of other potential treatments and cures in the wings.   I will go into great detail in future posts about new developments for treatments and potential cures.  The concept is to help you live a full, happier and better life!

I welcome any comments and suggestions as I will be posting as often as possible. .