Contents:
Osteoporosis and Myasthenia Gravis
Siblings' Myasthenia Gravis Undiagnosed for Years
A Message of
Hope in the Desert: 2006 Annual Meeting
Osteoporosis and Myasthenia Gravis
By Robert L. Baldwin, M.D.
Osteoporosis is a skeletal disorder characterized by reduced bone strength that increases the risk of fracture or distortion of bones. Osteoporosis is usually caused by loss of bone, and in most cases, is treatable years before it becomes obvious to the patient or doctor by a fracture occurring. The disorder affects females more than males, whites more than blacks, and is most common in women that have passed through menopause. In fact, 40% of white post-menopausal females are affected. Other factors put one at higher risk for developing bone loss and osteoporosis, including inactivity, the aging process, lack of adequate calcium and vitamin D in the diet, and small body frame and a history of fractures in the family without any trauma to cause it. The latter situation is typically a hip or a vertebral fracture. Cigarette smoking, alcohol abuse, excessive caffeine, and excessive salt intake also predispose to osteoporosis.
Fortunately, osteoporosis is now a
preventable and treatable condition. Unfortunately, since there are usually no
symptoms until there is a fracture, most patients go undiagnosed and therefore
untreated, in spite of new diagnostic means and medications that are effective.
It is usually more advanced
Osteoporosis is related to many disease states, but we are particularly interested in how it affects us as MG patients. Two primary risk factors for MG patients are the lack of physical activity and the use of cortisone (prednisone) and immuno-suppressants (Imuran, Cytoxan, methortexate, Cellcept, etc.). We will forget about aging for now! Physical activity, particularly weight-bearing exercises, help to keep bones strong and is both preventative and therapeutic. Even walking is of benefit. The problem is that MG patients cannot always do much exercise, or if we do, feel bad afterwards, and discourages further activity.
Many MG patients either take or have taken cortisone. Cortisone makes one lose bone by interfering with the normal, ongoing process of building new bone, as old bone is lost naturally. Most of the bone loss from cortisone comes in the first 4-6 months of treatment, and therefore it is very important that a patient's risk factors be elevated and preventative measures begun early in the treatment period. Likewise, many of us require ongoing cortisone treatment for many years, and that coupled with inactivity and the aging process, significantly increases our risk for development of osteoporosis and subsequent fractures. If you are taking more than 7.5 milligrams of prednisone a day, you need to be evaluated by your physician for bone loss and osteoporosis. Appropriate management should follow.
The best method to diagnosis
osteoporosis is the DEXA scan, a simple test wherein the bone is scanned,
usually the hip, spine or forearm. The test is not painful and takes only a
short time to perform. The heel or finger scan is not always an accurate
measure, but they are available in physician's offices, health fairs, malls and
other places- The normal DEXA score (t-score) is: +1 to -1; if it is between
-1.6 and -2.5, bone loss is present and if it is higher than -2.5 (-2.8 for
example), osteoporosis is present. The latter two scores require that
prevention and/or treatment measures be instituted. Tell your doctor you want
to be tested for osteoporosis using a DEXA scan. Especially
if you take Prednisone or other immunosuppressant medications. Ask for
your test score and compare it with the numbers above. Caution: DEXA scans
should not be performed on pregnant women!
AMPS: Conquer,
Siblings' Myasthenia Gravis Undiagnosed for Years
By MARIKA BATES
In
1963, I first heard the words "myasthenia gravis," and I was happy to
add them to my vocabulary.
For
eight years or more before then, "MG" already had been a part of my life,
but I mistook it for laziness, awkwardness and clumsiness.
The words come from both Greek and Latin and translate into "serious
muscle weakness." I was all too familiar with serious weakness, but I
didn't know there was name for it.
Current literature on myasthenia gravis will describe a rare neuromuscular
disorder causing rapid fatigue and muscle weakness made worse by activity and
improved with rest. It results from an autoimmune attack against the
nerve-muscle junction. Even rarer forms of the illness are caused by
architectural changes of the nerve-muscle junction. Symptoms may include
generalized weakness, which limit a patient's ability to walk or stand. Typical
symptoms include double-vision, drooping eyelids, sometimes difficulty in
chewing, swallowing, slurred and nasal speech, even breathing.
The disease strikes at any age, but it is more likely to strike younger women
and older men. With medication, most patients can work and maintain a nearly
normal lifestyle.
A few MG patients may experience a drug-free remission for years. Others, in a myasthenic crisis, may spend months on artificial life
support before beginning to recover. Thanks to modern medical research, MG is
not the death sentence it used to be. In fact, if you had to choose a neuromuscular
disease, myasthenia gravis would be the way to go. There are treatments and
medications to try. Although not all of them work for all patients and some
have serious side effects.
As with many disorders of the muscle and the nervous system, myasthenia gravis
can be very tricky to diagnose. It often mimics the symptoms of multiple
sclerosis and Lou Gehrig's disease. In support group
meetings, patients tell stories of how they suffered with undiagnosed MG for
months or years.
Our foundation has dubbed myasthenia gravis the "Snowflake" disease
because none of us is alike in the way we are affected. We can be a
neurologist's diagnostic nightmare. Once diagnosed, a myasthenic
may look back and see that they probably had MG for a long time before they
became very weak or fell into a myasthenic crisis
where breathing is seriously impaired.
The disease has been part of my life for some 50 years. I can't tell my own
story without including both of my brothers, Aubrey and Arne Hellstrom. We all have the same very rare form of this rare
disease. We have never had the typical symptoms of drooping eyelids or facial
and respiratory weakness, thankfully. The incidence of families such as ours -
where all siblings have MG - is very small, possibly a dozen cases in the
world, so I am told.
Fatigue and muscle weakness insinuated its way into my own life when I was in
sixth grade at
Problems
as a child
On the playground outside, running the bases on the
softball diamond got to be increasingly difficult, then eventually impossible.
I could still swing the bat and hit the ball, though, so someone else in my
class would run the bases for me. My friends never asked questions. They just
helped out and said nothing.
My poor brother Aubrey, four years younger than I, was worse at running the
bases as a little leaguer than I was. The coach put the little guy in the
outfield where he wouldn't get battered by a stray line drive. Nobody ever
questioned his physical shortcomings, perhaps because he spoke with a stammer
and squinted through a blind eye. His fatigue and weakness, in someone's view,
were probably all wrapped up with the blind eye and the speech impediment.
In Dorsey, school was my life. I walked there, most of my friends walked there,
then home and back during lunch. We learned to roller skate on the sidewalks of
the school. Dorsey even had a roller rink, where our youth fellowship church group
would meet on Saturday night. But skating eventually went the way of softball.
About as soon as I made some progress in roller skating, I had to quit because
my strength was starting to fail me. I couldn't understand why I could skate
just fine for a while, then stumble and fall, not being able to skate anymore
for the evening. That is how MG works. Up, then down. Strong,
then weak.
Rapid fatigue and muscle weakness would no longer simply insinuate itself into
my life after I moved up to the seventh grade. With my first physical education
class the reality of myasthenia gravis crashed head-on into me. Running laps
and shooting a basketball were impossible. I knew I was weaker than most kids,
but this was not playground stuff. Because I tried my best and was a very good
student, academically, my teacher realized I was not faking it and let me go
by. She asked no questions about my health.
Painful
memory
In the tenth grade I experienced one of the most
humiliating experiences of my life. The physical education teacher brought me
out of the ranks to make me do a squat-thrust. I could get down to the floor,
but never thrust my legs backward. She asked me why I couldn't do it, and I
ventured that maybe I was too heavy. That's what it felt like to me, anyway.
Then she called out another girl, one who was tall and very heavy. She weighed
both of us, then had the bigger girl do repeated squat
thrusts.
Having proven her point that my weight was not the problem, the teacher then
asked me to do a squat thrust again. I tried it, couldn't do it, then suffered even more indignities when I offered that my
arms felt too weak to hold myself up on them. She proceeded to make me push
against her own outstretched arms, which accomplished nothing except to make me
dance around the gym floor like a rubbery marionette. Her final solution to my
problem was to tell me I'd better know how to do it when I came back on Monday.
I was so confused about what was happening to me that I actually spent that
weekend trying to master the squat thrust. I hoped there was some kind of trick
to the squat thrust that I was missing, or that I
would break my leg and never have to see that teacher again.
Visit
to the doctor
The following Monday morning I marched into the guidance counselor's office and
told him my tale of woe. He allowed me to substitute choral music for physical
education in my schedule until I finished high school. He never asked whether I
had seen a doctor.
Life moved on. While I was living the married life and having babies, my
brother Aubrey was getting some attention at school because of his fatigue and
muscle weakness. He tells me he was able to hide his falls and other
difficulties because his friends knew something was wrong and they would come
to his rescue, picking him up from the ground, carrying books for him and
assisting him onto the school bus. In high school, physical education was
probably Aubrey's big hulking monster, such as it was for me. My parents
finally took Aubrey to our family doctor who sent Aubrey off to
At Aubrey's first appointment at Johns Hopkins, the physician gave him a
neurological exam, observing that Aubrey had fatigue and muscle weakness in his
arms and legs, but in no other areas. Because he had no obvious symptoms such
as weakness in the eyes and facial muscles, Aubrey went home without a
diagnosis, to return in six months. Then, before sending Aubrey off, the
neurologist asked the big question: "Does anyone else in your have family have muscle weakness?" Aubrey tells me he
doesn't remember if he said yes or if our mother said yes, but for the first
time in my life somebody's medical finger was pointing at me.
Finally,
a diagnosis
In May 1963, I accompanied Aubrey to
Meanwhile, after a minor car accident left me with a sore, stiff neck, I went
to see a doctor whom I had never met before, Dr. Hubert Manuzak
in
"What? Are you paralyzed?" he asked. I simply told him what I knew.
My legs and arms were weak. They had nearly always been that way. He
half-scowled at me and asked me to perform a couple of other small tasks such
as getting up from a chair, holding out my arms, the usual stuff. Then he
seemed to sing these words to me: "I have an idea of what your problem is.
Would you like to come back in a week for a test?" I would have crawled
back to find out what was wrong with me.
The test I was given a week later was an injection of neostigimine,
which within an hour allowed me to lift my arms and legs better than I ever
remembered. It was one of the most exciting moments in my life. This
wonderfully observant family practitioner had studied myasthenia gravis
somewhere in his training and had the courage to venture a diagnosis, even
though I didn't have the usual symptoms of droopy eyelids or double vision. He
wrote "myasthenia gravis" on the back of his business card and that
started the ball rolling when I took it with me back to Johns Hopkins on our
next appointment.
Very
rare occurrence
Aubrey and I were later admitted to
My brother Arne had a much different experience with myasthenia gravis. His
diagnosis came in 1979, 16 years after ours. Arne noticed that his shoes felt heavier
and that bags of groceries he carried would slip from chest to waist level
before he reached the car. He found it increasingly difficult to pick up his
kids and to get in and out of vehicles. Until then his strength seemed normal.
He served a split tour in
Because our form of MG is considered genetic and inheritable and not
autoimmune, we have never responded to drugs that are now lifesavers to some
patients, such as corticosteroids (Prednisone) and the immunosuppressants
Immuran, Cyclosporin and CellCept, drugs typically given to transplant patients.
Some patients benefit from plasma exchange and intravenous immunoglobin
therapy. Both of these procedures are invasive and except in a few cases,
require a day at the hospital.
Medication
helps
We three take Mestinon in
about the same dosage as we always have, although our strength comes and goes
unpredictably. Since 1989, we also take an experimental medication that boosts
the action of the Mestinon. We carry around
containers of pills and capsules everywhere we go and take two of each every
four hours throughout the day. If we forget a dose, then we're in trouble.
The meds work, but we can't predict how well they work and when, except you can
count on being stronger in the morning and weaker in the afternoon. I might set
out to shop, then find myself unable to step up onto a
sidewalk to get into the store. Aubrey has been stuck in his car because the
wind was blowing too hard against the door. All of us can drive a car, but we
usually have to pick up our legs one at a time to get in.
The three of us are extremely lucky to have a close-knit family who are very
supportive and helpful. If nothing else, we have each other to commiserate and
laugh with. Humor has been our salvation with this difficult and unpredictable
disease. All three of us have fallen and broken bones, but somehow we manage to
laugh it off thinking of how ridiculous we must look to others.
Even though MG has flared at times to the point we have trouble getting
dressed, turning over in bed or lifting a hairbrush, we remain very thankful
that our breathing, swallowing and speech are unaffected.
Remain
thankful
We are not employed any longer because our condition
has deteriorated over the years. Arne travels down here from
It is our hope that by sharing our stories about myasthenia gravis we may reach
out and offer hope and encouragement to others dealing with MG and other rare
disorders.
Marika Bates lives in
Published June 19, 2005,
The Capital,
By: Jacqueline
Held Executive Director,
Even a minor surgical procedure can
be a physical and emotional stressor for the patient with myasthenia gravis,
and in fact any surgery should be a serious consideration and preparations made
before the surgery is done. Surgery of any kind should always be considered a
"big deal" for the MG patient and thorough preparations should be
made.
During the past six months, I have
had the opportunity to experience not one, but three surgeries and I have
learned a lot about what some people don't realize about the problems that we
can encounter. (The hardest part for me, looking back at it now, was the fact
that I lost out on all of our summer months). Many times surgery is necessary
to improve the quality of life, even though it can be associated with
complications.
With the advent of new technological
advances in surgical procedures, and the development of safer and shorter
acting anesthetics, most patients with MG can safely undergo most surgeries.
Even so, there are a number of precautions that must be taken to ensure a
smooth and uncomplicated hospital stay.
First of all, be sure that you ask
your surgeon to explain the procedure in detail, as to how long you will be
under anesthesia, the length of hospital stay, how long recovery will take,
what limitations you will have, how it will affect your MG, and will the health
care workers listen to you and pay attention to your unusual symptoms. Most
importantly, ask your surgeon to request that the anesthesiologist knows about
your condition, and use the type of anesthesia that has the fewest complications
and is still suitable for your procedure. Make the request that the surgeon
contact your primary care doctor (either your neurologist or family care
doctor) and that he knows what medications you are taking and the status of
your general health.
The most complicated of my
surgeries was a total knee replacement following a totally unknown injury. This
was the last of the three surgeries and by far the most uncomfortable. I was
very fortunate in the fact that I have two extremely good physicians, a neurologist
and a critical care pulmonologist, both of which took
a very active part in the entire procedure. Due to the fact that
hospitalization can expose the myasthenic to many
factors, (malnutrition, inflammation, infection, muscle wasting, and the exacerbation
of MG symptoms), joint replacement patients are given private rooms to prevent
as many problems as possible during their hospital and rehabilitation stays.
Preparation for surgery should
include the best overall physical and emotional health possible. Knowing what
to expect is very important and any concerns should be discussed beforehand.
Often several sessions of plasmapheresis can help
prepare the patient with myasthenia and can help prevent even the onset of
symptoms.
Very little was known about MG
among the health care workers, and it was surprising to hear them say that it
was just something that they had "heard about". It was gratifying to
know that the rehabilitation personnel were willing to listen and learn about
MG, and this provided a marvelous opportunity to do a little education on the
subject. This has carried through to my outpatient rehab which is still
continuing.
I have learned that an orthopedic
unit is an extremely busy place. Most patients are in extreme pain and the
personnel can be either very caring or indifferent. There are very good pain
relievers that can be safely given to a myasthenic
and a person must realize that they have to speak up for themselves if there is
a problem. I expect my surgery to ultimately be a success, and while I never
expected to spend my summer like I did, I know that I am grateful for the
doctors, health care workers and especially my family and friends that were
here when I needed them most. Most of the time with proper care, the right
mental attitude and help when needed, many myasthenics
can safely undergo necessary surgeries and have a gratifying outcome.
Amps:
A Message of Hope in the Desert: 2006 Annual
Meeting of the Myasthenia Gravis Foundation of America, Inc.
May 4-6, 2006
Wyndham Buttes Resort,
Please visit the
MGFA Web site at
www.myasthenia.org
or
contact the national office at 1-800-541-5454 for more information.
In Memory of:
By:
Howard Morris
Ellie Ruth Whelchel
Essie L. Anthony
Juanita Buren
Ginger, Bill, Chelly
Hines
Cathy & Charles Leake
Randy & Carolyn Collins
In Honor of:
By:
Dianne George & Gayle Woods
Jason & Betty Hoffman
Mr. & Mrs. Art Williams
Dr. J. Willis Hurst
PLEASE NOTE: Any views expressed in this newsletter are those of the
individual author and do not reflect any official position of the Georgia State
Chapter, MGFA. Each person's situation is unique. If you have any medical
questions, please discuss them with your doctor as he/she knows your situation
best.