Hello All,
One of our PPNI Support Group
members asked about the prognosis for those of us with Periodic Paralysis. I
decided the best way to answer her question was to use my original writing from
our first book, "Living with Periodic Paralysis" before we edited it.
This article was changed and streamlined for the book.
At this time, I have lived eight years beyond what we ever thought I would. For that I am thankful and owe it to following the plan outlined in our book; the pH balanced diet, discovering my triggers, avoiding exertion, avoiding stress, using oxygen and taking no drugs.
Prognosis
As I began to research the
prognosis for Periodic Paralysis, I found an inadequate amount of information
provided, both in the number of articles and the amount written. I decided to
include in this chapter the actual passages to prove my point. So, the
following snippets of information are all I was able to locate in my research
regarding what to expect now that we know we have Periodic Paralysis. Most of
the passages are simply written by professional people who have no form of
Periodic Paralysis. There are only two to three sentences, except for one,
detailing the rest of our lives!!!! I did, however, find one article that gives
an honest evaluation in just two sentences. It is the last quote below.
"Prognosis"
While the disability can
range from minor, occasional weakness to permanent muscle damage, inability to
hold a normal job and use of a powerchair, most people function fairly well
with drugs and lifestyle changes. 1
What is the
Prognosis?
The prognosis
for the familial periodic paralyses varies. Chronic attacks may result in
progressive weakness that persists between attacks. Some cases respond well to
treatment, which can prevent or reverse progressive muscle weakness. 2
Prognosis
The prognosis for periodic paralysis varies. Over activity, bad diet or
simply an unfortunate gene mutation can lead to a type of chronic, low level
weakness called an "abortive attack," or to permanent muscle damage.
Abortive attacks often respond to extra potassium, cutting carbohydrates,
getting plenty of rest, increasing doses of medication and gentle daily
exercise such as short walks. Permanent muscle weakness is just what it sounds
like, permanent, irreparable damage to the muscles. Vacuoles and tubular
aggregates form and destroy healthy muscle tissue. This type of damage should
show on a muscle biopsy. Not even anabolic steroids can bring these damaged
muscles back.
Life span is expected to be normal, but attacks can drop potassium to
levels low enough to cause life threatening breathing problems or heart rhythm
difficulties. Patients often report muscle pain and cognitive problems during
attacks. Migraines occur in up to 50% of all hypokalemic periodic paralysis patients
and may include less common symptoms like phantom smells, sensitivity to light
and sound or loss of words. Medical literatures states that muscle strength is
normal between attacks, but patients tell a different story. "Normal"
for them is not exactly the same as "normal" for everyone else.
Because there are dozens of possible gene mutations, some drugs and
treatments that work fine for one patient will not work for another. For
example, most patients do well on acetazolamide, but some don't. Some patients
will do well with extra magnesium (the body's natural ion channel blocker) or
fish oil, while these same nutrients will make other patients worse. Patients
and care givers should take extreme caution with all new drugs and treatment
plans. 3
Outlook
(Prognosis)
Hypokalemic periodic paralysis responds well to
treatment. Treatment may prevent, and even reverse, progressive muscle
weakness. Although muscle strength is initially normal between attacks,
repeated attacks may eventually cause worsening and permanent muscle weakness
between attacks.
Possible Complications
- Kidney stones (a side effect of acetazolamide)
- Heart arrhythmias during attacks
- Difficulty breathing, speaking, or swallowing during attacks (rare)
- Progressive muscle weakness 4
Prognosis
While the disability can range
from minor, occasional weakness to permanent muscle damage, inability to hold a
normal job and use of a powerchair, most people function fairly well with drugs
and lifestyle changes. On a lighter note, people with periodic paralysis are
sometimes lovingly called Possums because they play dead when startled or
stressed.5
Expectations
(prognosis)
Chronic attacks may eventually result in progressive muscle weakness
that is present even between attacks. Hypokalemic periodic paralysis responds
well to treatment. Treatment may prevent, and even reverse, progressive muscle
weakness. 6
Previously, primary PPs were regarded benign diseases as frequency and
severity of attacks decrease with age (1).
However, with advanced age, quite a number of patients develop permanent
weakness and muscle degeneration with fat replacement (1).
In individual cases, fatal cardiac arrhythmias may be a severe complication of
the paralytic attacks…. (1). 7
How Bad Will it Get?
The Truth
When I began to research I wanted to know what to expect.
How long will I live? How bad will I get? Can this disease be reversed if I get
proper treatment? Will I lose my ability to walk? Will I ever drive again? Will
I need to be in an assisted living program? Is there medication to stop the
total paralytic episodes? What are my chances of dying from the long QT
interval heart beat? Will my breathing continue to get more difficult until I
can no longer breathe on my own? Is there
any medication I can take if I get another bladder infection? What happens if I
need an operation and can’t use anesthetics? What can I do to stop the pain in
my shoulder and back since I cannot take any pain medications? When I go into cardiac arrest, is it worth
trying to save me? Will I end up on dialysis due to kidney failure? Can I
travel? What will happen if I end up in the ER again and they cannot help me
with any medications?
The short blurbs above are all I was able to locate. There are no doctors who can tell me. My renal specialist told me that he is unable do anymore for me since the diamox did not work. My neurologist tells me that he does not know what to do for me. My cardiologist says my heart condition, due to the PP, is “not treatable” for me. I am, “not a candidate for a pacemaker“, “possibly a defibrillator later”. My Primary Care Physician (PCP) will not treat anything that has to do with my disease. Even the MDA doctors I saw did not recognize PP nor did they know how to diagnose it correctly. I was told by one of the MDA Healthcare Coordinators, that they need me to educate the MDA doctors so they will know how to treat me. This is not at all comforting. No one can tell me how to treat my symptoms or what to expect.
Here is what I do know about
myself and can review in answer to the above questions:
I am 70 years old and was
diagnosed with Periodic Paralysis (PP) on February 7, 2011 at the age of 62.
The kind I have is Andersen-Tawil Syndrome. I have had episodes of
partial and total paralysis for many years. During the episodes, my potassium
shifts are low, high and within the normal ranges. Due to several misdiagnoses
and a lack of proper diagnosis and treatment for over 50 years, I have become
totally and permanently disabled with weak muscles throughout my body including
those involved with my vision, digestion, breathing and my heart. I must be on
oxygen constantly and cannot exert myself in any way. The electrical workings
of my heart are defective. I have had a heart loop monitor inserted in my chest
to monitor the tachycardia and arrhythmia, which include long QT interval
beats. I now spend my days in a recliner, unable to walk farther than across a
room. I must use a motorized wheelchair for anything farther. If I did not have
the help of my husband, I would have to live in an assisted living program. I
was misdiagnosed for many years. The medications given to me made me worse.
Through the past years of my
physical decline, I have had to give up my career as a special education
teacher, my hobbies to include hiking, walking, swimming, exercising, fishing,
camping, traveling, shopping, cooking and baking. I had to sell, and move away
from, a beautiful home in the mountains of Utah. I can no longer drive. I have lost many friends, because I could
not keep up with them or entertain any longer. I have lost contact with family
members who did not understand or did not want to watch my decline or who
thought I was a hypochondriac. I have lost the connection I once had with my
grandchildren because I can no longer keep up with them or continue a
meaningful relationship with them. The relationship with my husband has changed
from husband and wife to caregiver and patient. Most of the over 37 doctors I
have seen in the past have treated me poorly and like I was mentally
ill.
I have spent the past several
years working diligently to get a diagnosis and treatment for the ailment that
cruelly stole the quality of my life. The most difficult part of this, for me,
is knowing that I may not have became this seriously ill if just one of the
over 30 doctors I had seen in the 6 years in Oregon before my diagnosis and the
many years before, would have taken me seriously.
The following quotes taken from
the above passages give us a clue of what can happen, but the passages from
which I pulled them insinuate it is not “normally” what happens. I am in
contact with many people from around the world who would beg to differ with
this. The following is more in line with what is happening to people with PP
due to lack of diagnosis and treatment.
“Permanent muscle weakness is just what it sounds like, permanent,
irreparable damage to the muscles” 8
“Life span is expected to be normal, but attacks can drop potassium to
levels low enough to cause life threatening breathing problems or heart rhythm
difficulties” 9
“…some drugs and treatments that work fine for one patient will not work
for another.”
“…repeated
attacks may eventually cause worsening and permanent muscle weakness between
attacks” 10
Possible Complications
· Kidney stones (a
side effect of acetazolamide)
· Heart arrhythmias during attacks
· Difficulty breathing,
speaking, or swallowing during attacks (rare)
· Progressive muscle weakness” 11
“…permanent muscle damage,
inability to hold a normal job and use of a powerchair,” 12
In my opinion, the truth
is, the majority of people with this disease end up like me; very, very ill;
but they are misdiagnosed, under-diagnosed, called mentally ill, or
hypochondriacs. They are diagnosed as suffering from conversion disorder, or
having pseudo seizures. They are laughed at and scoffed at. They are told they
are “too old” or it is not possible because they are “black”. They are given
medications that make them worse. Doctors dismiss them and ridicule them and
lie about them in the medical records. They do not get the medication or
treatments they need. Then they die of things such as, “unknown” muscle wasting
disease, accidental drowning in a pool or bathtub, cardiac arrest at age 40,
stroke, or failure to thrive. The worse is from suicide; because if your
doctors don’t believe you, how can your family members be expected to believe
you. They just give up.
Our main reason for creating our
website, the Periodic Paralysis Network, and writing this book, is for these
people. They need to be diagnosed and get the proper medications and treatment
before it is too late. We want to help them. We are hopeful that the doctors
who see our website and read this book will become more aware of this disease
and gain enough information to begin appropriately diagnosing their patients
with Periodic Paralysis before it is too late.
In conclusion, many people with
Periodic Paralysis will live normal life spans and their disability will be
minor with occasional weakness. Some may actually not even have episodes of
paralysis. Others will have moderate disability and receive proper medical
treatment and medication. They will respond well, and may even reverse some of
their weakness. But, others with Periodic Paralysis will have mild, moderate or
severe disability and they will not receive proper diagnoses or treatment. They
will become more disabled as the symptoms progressively worsen. They will
suffer needlessly and may die due to complications. So, for some individuals
Periodic Paralysis is a terminal condition.
Terminal?
Several years ago I became extremely ill. We had
to call our local home health care. We were hoping they could come to the house
and evaluate me by doing some lab work to see if I was in metabolic acidosis
(to be discussed in the next chapter) or had some other infection of some type.
I was hoping they could help Calvin take care of my needs. He is disabled too
and cannot lift me. I could do nothing for myself. I could not eat. I slept
most of the time and I was in and out of total body paralysis for weeks.
A nurse and physical therapist
came to our home. Upon evaluation it was decided that I did not qualify for
their services due to some technicalities with my insurance and Medicare,
mostly due to the fact that my symptoms were intermittent. We did get a visit
from the nurse a few times over the following weeks but never got blood drawn.
With each visit we discussed the ways we could get some services from them, as
I needed them. They personally thought we deserved and needed the help from
them.
It was decided that I might
qualify for services under the hospice wing of their services. Hospice is care
and support to individuals with life-limiting conditions or illness and their families during later stages of the disease. Hospice is chosen and begins when
comfort and management is chosen rather than attempting to reverse or cure the
condition or disease. This was and is absolutely the case for me.
I have a disease with no known cure. There is no treatment I
am able to use. I am progressively getting weaker and my breathing gets more
difficult. I could die at any minute from arrhythmia. Because I can use no
antibiotic, a simple infection can kill me.
We had to discuss this with my
Primary Care Physician. After recovering from the severity of the illness and
regaining some strength, I researched the issues and wrote a paper arguing the
possibility of receiving services through hospice. On my next visit, we
discussed these issues. The following is the paper I wrote and presented to
her.
Appointment with Dr. J W Oct 24, 2011
Issues
1. No other doctors are caring for me. They say they “Can do no more for me”.
2. I need a standing
order for blood work for metabolic acidosis, lactic acidosis, bladder
infections and anion gap, etc.
3. I do not qualify
for home health care but what about home services through Hospice?
4. I am technically
terminally ill.
Medical issues
1. I cannot take any meds for my conditions so they are just progressing.
2. I cannot take
antibiotics for infections.
3. My breathing
muscles are weak and getting weaker
4. I need oxygen for
heart issues, blood pressure and breathing.
5. I have long QT
interval heartbeats during paralysis.
6. My breathing stops
during paralysis.
7. I experience
choking during paralysis.
8. My blood pressure
increases during paralysis.
9. My heart rate
increases during paralysis.
10. After paralysis I need assistance due to
total body muscle weakness
Definitions
of “Terminal”
"Terminal
Condition"—defined as "a condition caused by injury, disease, or
illness for which there is no reasonable medical probability of recovery and
which, without treatment, can be expected to cause death." 13
Terminal illness is a medical term
popularized in the 20th century for an active and progressive disease, which
cannot be cured easily by popular medicinal practice. Curative treatment is not
viewed as appropriate. 14
The term terminally ill in the
context of health care refers to person who is suffering from a serious illness
and whose life is not expected to go beyond 24 months at the maximum. 15
Terminal illness…an advanced
stage of a disease with an unfavorable prognosis and no known cure. 16
Terminal illness is a medical term popularized in the 20th century for an active and progressive disease, which cannot be cured easily by popular medicinal practice. Curative treatment is not viewed as appropriate. 17
The term
terminally ill in the context of health care refers to person who is suffering
from a serious illness and whose life is not expected to go beyond 24 months at
the maximum. 18
Terminal illness is a medical term
popularized in the 20th century to describe a disease that cannot be cured or
adequately treated and that is reasonably expected to result in the death of
the patient within a relatively short period of time. This term is more
commonly used for progressive diseases such as cancer or advanced heart disease
than for trauma. In popular use, it indicates a disease which will eventually
end the life of the sufferer. 19
I am 66 years old and was diagnosed with Periodic
Paralysis (PP) on February 7, 2011. The form I have is Andersen-Tawil Syndrome.
I have had episodes of partial and total paralysis for many years. During the
episodes, my potassium shifts are low, hypokalemia; high, hyperkalemia and
within the normal ranges, normokalemia.
Due to
several misdiagnoses and a lack of proper diagnosis and treatment for over 50
years, I have become totally and permanently disabled with weak muscles
throughout my body including those involved with my vision, digestion,
breathing and my heart. I must be on oxygen constantly and cannot exert myself
in any way. The electrical workings of my heart are defective. I have had a
heart loop monitor inserted in my chest to monitor the tachycardia and
arrhythmia, which include long QT interval beats. I now spend my days in a
recliner, unable to walk farther than across a room. I must use a motorized
wheelchair for anything farther. If I did not have the help of my husband, I
would have to live in an assisted living program. I was misdiagnosed for many
years. The medications given to me made me worse.
Complications and Symptoms of Periodic
Paralysis:
“Permanent muscle
weakness is just what it sounds like, permanent, irreparable damage to the
muscles”
“Life span is expected
to be normal, but attacks can drop potassium to levels low enough to cause life
threatening breathing problems or heart rhythm difficulties”
“some drugs and
treatments that work fine for one patient will not work for another.”
“repeated attacks may
eventually cause worsening and permanent muscle weakness between attacks”
“Possible Complications
Heart arrhythmia
during attacks
Difficulty breathing,
speaking, or swallowing during attacks (rare)
Progressive muscle
weakness”
“…permanent muscle
damage, inability to hold a normal job and use of a powerchair,” (These
quotes are referenced above)
The Results
The doctor decided that hospice
was an option for me. I did indeed fit the definition of being “terminal”. I am
in an advanced stage of a disease called Periodic Paralysis, it has an
unfavorable prognosis because of lack of treatment and medications and it has
no cure. It was decided that when I felt it necessary, I could begin the
hospice services. In the meantime, a standing order for blood work was set up
and the home health care could draw it when needed. The doctor also agreed, at
my request, to sign a “Do Not Resuscitate Document (DNR)”. This means that if
my heart stops or I stop breathing, there is to be no CPR or any other type of
life-saving measures used. This
document is displayed in plain view when and if an ambulance is called and
recorded at the nearby hospitals.
And so, we now know that Periodic
Paralysis can be a terminal condition for some individuals. It is in fact
killing me due to a lack of knowledge and proper treatment. I cannot at this
time tell anyone else what to expect or how bad it will get. I truly believe,
however, that the sooner an individual begins to follow the appropriate plan
for their individual needs and condition, they can and will do better and may
live a long and healthier life. Although there is no magic cure, there is hope
through following a plan of natural and commonsense methods to manage the
episodes and improve the symptoms.
Note:
March 15, 2019
Following all of the natural ways to manage our symptoms that we have outlined in our books, in the Blog Articles, on our Website and discussed in our Support Group, I have improved the quality of my life. I have lived eight more years and have been able to do many things I had not thought possible like travel, create the PPNI Forum and write seven books!
All of the above information in this article is true, but it is possible to have some improvement and extend our lives and to be productive.
Note:
March 15, 2019
Following all of the natural ways to manage our symptoms that we have outlined in our books, in the Blog Articles, on our Website and discussed in our Support Group, I have improved the quality of my life. I have lived eight more years and have been able to do many things I had not thought possible like travel, create the PPNI Forum and write seven books!
All of the above information in this article is true, but it is possible to have some improvement and extend our lives and to be productive.
http://onlinelibrary.wiley.com/doi/10.1111/j.1600-0404.2007.00963.x/full
http://medical-dictionary.thefreedictionary.com/terminal+condition
http://en.wikipedia.org/wiki/Terminal_illness
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