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PAIN & PORPHYRIA
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Pain
Understanding & Controlling Pain
Psychological Aspects of Pain
Porphyric PN Pain
Leg Pain in Porphyria
Pain Drugs & Treatment

PORPHYRIA FACTS:

PAIN & PORPHYRIA


One prays that pain will go away.

Sometimes it does but often it will linger.

Sometimes pain will intensify in one place and then go into remission to surface
at another location.

One comes to the conclusion over years of endurance that one just learns to live
with pain.

SOIURCE:
Mary Ellen Williams MNS
Pain Therapist
++++++++++++++++++++

Pain is an unpleasant sensory and emotional experience associated with actual
or potential tissue damage or described in terms of such damage.

SOURCE:
Robert Johnson MD
++++++++++++++

During onsets of acute attacks the pain is mostly experienced in the upper right
quadrant of the abdomen.

SOURCE:
D. Montgomery Bissell MD
+++++++++++++++++++++

Because of the intensity of .u.r.q. pain the pain found elsewhere, seems to be
trivial.

Try not to give in to the pain by taking pain medications.

One can use hot tub, exercise, TENS, physical therapy and relaxation
techniques whenever possible.

Some porphyria patients even use acupuncture to relieve chronic pain..

SOURCE:
Robert Johnson MD
+++++++++++++++


Porphyria Pain Scale

0 - Pain Free

1 - Very minor annoyance - occasional minor twinges. No medication needed.

2 - Minor Annoyance - occasional strong twinges. No medication needed.

3 - Annoying enough to be distracting. Mild painkillers take care of it.
(Aspirin, Ibuprofen.)

4 - Can be ignored if you are really involved in your work, but still distracting.
Mild painkillers remove pain for 3-4 hours.

5 - Can't be ignored for more than 30 minutes. Mild painkillers ameliorate
pain for 3-4 hours.

6 - Can't be ignored for any length of time, but you can still go to work and
participate in social activities. Stronger painkillers (Codeine, narcotics) reduce
pain for 3-4 hours.

7 - Makes it difficult to concentrate, interferes with sleep You can still function
with effort. Stronger painkillers are only partially effective.

8 - Physical activity severely limited. You can read and converse with effort.
Nausea and dizziness set in as factors of pain.

9 - Unable to speak. Crying out or moaning uncontrollably - near delirium.

10 - Unconscious. Pain makes you pass out.

SOURCE:
Ron Kelly
HCP Porphyria Patient
++++++++++++++++++
Pain measurement scales, questionnaires, tests, and other methods have been
used to assess pain severity and duration in patients or experimental animals to
aid in diagnosis, therapy, and physiological studies.

SOURCE:
Robert Johnson MD
+++++++++++++++++


Abdominal pain

Abdominal pain is a sensation of discomfort, distress, or agony in the abdominal
region.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++++++++

The symptoms of acute porphyria involve the nerves to the gastrointestinal tract
where severe abdominal pain, often severe enough to be confused with acute
appendicitis can develop and lead to exploratory surgery.

SOURCE:
The Canadian Porphyria Foundation Inc.
Neepawa, Manitoba, Canada
++++++++++++++++

Severe abdominal pain without signs of perittonism is seen in almost all attacks
of acute porphyria.

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
+++++++++++++++
The most common symptom in acute porphyria is abdominal pain (85 to 95% of
attacks)

These statistics were confirmed in studies undertaken by Herbert Bonkovsky as
early as 1982.

SOURCE:
Dr. Robert Johnson M.D.
Retired Clinician
+++++++++++



It has been stated in various listings of statistics on the acute porphyria and
especially in regard to acute porphyria that 90% experience abdominal pain

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++++++++++

Unexplained abdominal pain still remains one of the most constant indicators
of porphyria.

SOURCE:
Melissa Wesner FNP
Internal Medicine
++++++++++++

Back in 1957 in a study by Waldenstrom 85% of the porphyria patients indicated
abdominal pain.

Two years later in 1959 Goldberg found in his study that 94 % of the porphyria
patients indicated abdominal pain.

Twenty years later, in 1979 Drs. Stein and Tschudy found that 95% of all
porphyria patients indicate having the abominal pain.

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++++++++++


Chest, back, and limb pain may also occur either in the presence of or absence
of abdominal pain.

SOURCE:
Melissa Wesner FNP
Internal Medicine
++++++++++++

While not everyone experiences abdominal pain, and while it may not be present
with every acute attack, abdominal pain stil remains one of the major symptoms
of acute intermittent porphyria.

SOURCE:
Dr. Robert Johnson M.D.
+++++++++++++++++++

It is believed that an imbalance in the autonomic innervation of the gut leads to
abdominal pain which is commonly associated with the attack.

The reasons are still unclear as to why the pain is present, however it is present
in almost 90 percent of all porphyric attacks.

SOURCE:
Lifelines:
Journal of Emergency Medicine
October 1998
123: 437-443
Emergency Treatment of the Porphyric Patient
Kirsch, N,.E., M.D.
+++++++++++

The absence of abdominal pain in a patient with hepatic porphyria is unusual.

Without abdominal pain this usually will lead to a delayed recognition of the
illness.

SOURCE:
Arthritis and Rheumatism
March 1997 Vol. 40 No. 3
page 586
+++++++++++


Acute pain

Acute pain is that which goes away immediately after illness or injury and
resolves after healing.

SOURCE:
Robert Johnson MD
++++++++++++++++

Allodynia

Allodynia is an altered sensation in which normally nonpainful events are felt as
pain.

SOURCE:
Jennifer Evans DNP
Pain Management
+++++++++++++

Back pain

About half of all AIP, VP and HCP patients experience back pain as a symptom
associated with their porphyria.


SOURCE:
D. Montgomery Bissell
Hepatic Porphyrias
Chapter 31
Diseases of the Liver
6th Edition / Lippincott
+++++++++++

Chest wall pain

Chest pain that originates from a noncardiac cause.

Chest wall pain typically involves an inflammatory condition of the muscles,
bones or joints that comprise the thorax.

Chest wall pain can occur during acute attacks of porphyria.

The origin of the pain is not well understood.

SOURCE:
Robert Johnsoin MD
+++++++++++++++++++


Chest pain has many causes.

One celebrated cause is angina which results from inadequate oxygen supplyto
the heart muscle due to coronary artery disease or spasm of the coronary
arteries.

Treatment of angina includes rest, medication, angioplasty, and/or coronary
artery bypass surgery.

Often this chest pains mimics the upper right quadrant pain associated with
acute hepatic porphyria.

SOURCE:
Helene Frederickson
Pain Management
+++++++++++++++

Chronic pain


Chronic pain is pain that persists beyond the time of normal healing and can last
from a few months to many years.

Chronic pain can result from disease, such as arthritis, or from an injury or
surgery.

Chronic pain can also can occur without a known injury or disease.

Chronic pain has been found to present in porphyria and porphyric PN.

SOURCE:
Columbia Health Systems
2001
+++++++++++

Various types of pain frequent porphyria patients

Most porphyria patients have abdominal pain and will also experience other
intermittent pain especially during porphyria attacks.

SOURCE:
The Porphyrias:
A. Kappas, 1995
+++++++++++++++

Limb, head, neck, or chest pain have all been indicated in the symptomology of
hepatic porphyria patients.

SOURCE:
Robert Johnson MD
++++++++++++++++

In 1959 Goldberg found in his poprhyria research studies that 52% of all hepatic
poprhyria patients experience these forms of pain separately from the abdominal
pain.

SOURCE:
The Porphyrias:
A. Kappas, 1995
+++++++++++++++++++
Low back pain

About half of all AIP patients experience back pain as a symptom associated
with their porphyria.

SOURCE:
D. Montgomery Bissel
Hepatic Porphyrias
Chapter 31
Diseases of the Liver
6th Edition / Lippincott
+++++++++++++++++

Low back pain is a continuous pain in the lower back or lumbar region and is
often associated with porphyria.

Severe pain can be associated with the peripheral neuropathy that presents in
the acute porphyrias.

SOURCE:
Robert Johnson MD
++++++++++++++++
If peripheral neuropathy, such as pain in the back and legs or parathesias
occurs, it is almost always preceded by abdominal pain.

SOURCE:
Acute Intermittent Porphyria
Anne LeMaistre, M.D.
1995 TMC
++++++++++++++
Back pain symptoms in the low back can relate to the bony lumbar spine, discs
between the vertebrae, ligaments around the spine and discs, spinal cord and
nerves, muscles of the low back, internal organs of the pelvis and abdomen, and
the skin covering the lumbar area.

SOURCE:
Ellie Gabbon
Physical Therapy Department
+++++++++++++++++

Neuropathic pain.

Neuropathic pain which is better known as PN, is a pain that originates from a
damaged nerve or nervous system.

Neuropathic pain often accompanies the acute porphyrias and diabetes.

SOURCE:
Robert Johnson, M.D.
Retired Clinician
+++++++++++++++++++

PN pain also experienced in acute porphyria.

Pain is the most disturbing symptom of peripheral neuropathy.

Gabapentin monotherapy appears to be efficacious for the treatment of pain
and sleep interference associated with peripheral neuropathy and exhibits
positive effects on mood and quality of life.

SOURCE:
Gabapentin for the symptomatic treatment of painful neuropathy
Backonja M, et. al.
Department of Neurology
University of Wisconsin
Madison Wisconsin 53792
JAMA
1998
Dec 2;280(21):1831-6
++++++++++++++++++

Always be sure to have an open-ended pain prescriptions which to use for
varying degrees of pain, but try not to use such medications until absolutely
necessary.

SOURCE:
Robert Johnson MD
+++++++++++++++


Editor's note:

***Gabapentin is the generic name for Neurontin


Neurontin treats PN pain

The antiepileptic drug Neurontin has been used successfully in cases of
porphyric neuropathic pain.

Its mechanism of action is still unknown, but seems to be related to a positive
effect on the synthesis and liberation of the neurotransmitter GABA.

SOURCE:
Review of Neurology
1998 Apr;26
(152):618-20
Treatment of neuropathic pain with gabapentin
Sanchez-Valiente S., M.D.
Neurology Service,
Hospital de Calatayud,
Zaragoza, Spain.
+++++++++++++++++

Pain scale.

A pain scale is a system of rating pain.

A pain scale is often based on a scale of 0 to 10, with 0 being no pain and 10
being the worst imaginable pain.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++

Pain threshold.

A pain threshold is that point at which pain is noticeable.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++


Pain tolerance level.

A pain tolerance lvels is the peak amount of pain that a person can endure.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++

Pain presents with PN

" Peripheral neuropathy is manifested as pain in the extremities. and it may
progress to a severe motor neuropathy.

SOURCE:
Journal of Photodermatololy, Photoimmunology
and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
Helsinki, Finland.
+++++++++++++++++++++


Opioid analgesics

Opioid analgesics are centrally acting agents.

These opioids provide fast pain relief by either binding or blocking opiate
receptors in both the brain and the spinal cord.

An agonist effect is known as binding. The blocking effect is known as an
antagonist effect.

Opiods are also known as narcotics.

Opiods can play a role in the management of some chronic pain conditions and
this includes many of the acute hepatic porphyric pain.

SOURCE:
Jeffrey Balsberger RPh
+++++++++++++++++

It is thought by many chronic pain specialists that non-addictive personalities of
porphyria patients who use the opiods specifically for their analgesic effect have
a very low possibility of addiction.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++

Patients r who use such opiods because of the their euphoric effects have a far
greater possibility of becoming addicted to such drugs.

Nonetheless, because of the social stigma and also in many places the legal
issues that of opiods, there continues to be a barrier to both the patient's ability
to comply and the physician's ability in prescribing.

For these reasons non-narcotic analgesics are often preferred as the first-line of
therapy for porphyric patients for their often chronic pain.

Since January1, 2001, all physicians must address the treatment of pain control
in all patients presenting with pain. Opiods are just one of several options.


SOURCE:
Robert Johnson, M.D.
Retired Clinician
+++++++++++++++++++

Pain center.

A pain center is a facility with a group of physicians and other health care
providers whose collective expertise allows for the management of a variety of
pain problems.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++


Pain clinic.

A pain clinic is a medical facility with one or more physicians and other health
care providers who specialize in the treatment of painful conditions, such as
back pain or headaches.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++


Pain pumps

A pain pump is a device that's surgically implanted in the lower abdomen,
where it provides a steady stream of medication - typically an opioid - to
the spinal column.

A Pain Pump generally is used to control pain associated with a chronic illness
or severe nerve damage.

SOURCE:
Pain Control
United Health Systems
1999
++++++++++

When oral medications and/or nerve blocks do not sufficiently control the pain,
advanced pain therapies or implantable systems may be effective.

These systems are designed to interrupt transmission of pain signals from the
spinal cord to the brain.

If the pain signals do not reach the brain, then the patient does not actually feel
the pain.

SOURCE:
Spine Health
++++++++++

A pain pump is generally used to control pain associated with a terminal illness
or severe nerve damage.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++

Pain scale

A Pain scale is a system of rating pain.

A pain scale is often based on a scale of 0 to 10, with 0 being no pain and 10
being the worst imaginable pain.

SOURCE:

Mayo Education & Research
Mayo Foundation
Rochester, Minnesota
++++++++++++

Patient controlled analgesia (PCA)

PCA’s are a system that allows patients to control the amount of pain medicine
they receive.

The patient pushes a button and a machine delivers a dose of pain medicine into
the blood stream through a vein.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++

Pain rehabilitation

A Pain rehabilitation program is a program that provides comprehensive,
rehabilitative therapy for people suffering from chronic pain.

SOURCE:
Pain Control
United Health Systems
1999
++++++++++

Limb pain

Fifty-one percent of acute porphyria patients experience pain in the limbs.

SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++


Motor weakness

Abdominal pain may be accompanied by motor weakness in the limbs,

SOURCE:
Acute Intermittent Porphyria
Differential Diagnosis of Acute Pain
Stanley L.Wiener, M.D. et. all
McGraw-Hill Inc. 1993
+++++++++++


Rebound pain.

Rebound pain is that which occurs when regular use of a pain medication
makes a person’s pain worse instead of better.

SOURCE:
Pain Control
United Health Systems
1999
++++++++++


Tolerance

Tolerance is the point at which a person adapts to a specific substance, so
larger amounts of the prescribed medication or a new medication is needed to
achieve the same results.

SOURCE:
Mayo Education & Research
Mayo Foundation
Rochester, Minnesota
++++++++++

Transdermal pain treatment

Transdermal is any substance entering the body via the skin, such as a
medicated lotion being absorbed through the skin.

SOURCE:
Mayo Education & Research
Mayo Foundation
Rochester, Minnesota
+++++++++++++

Treatment
Mild pain can be adequately controlled with aspirin, paracetamol or
hydrocodeine.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
+++++++++++


Biofeedback

Biofeedback. is a behavior-training program that teaches a person how to gain
some control over autonomic reactions, such as heart rate, blood pressure, skin
temperature and muscular tension.

Biofeedback also helps in teaching a patient how to control pain.
SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
++++++++++++++++++

Preventive therapy

Treatment to relieve symptoms of the disease but not to cure it.

Frequently takes the form of making the patient more comfortable through pain
management.

Sometimes is referred to as pallative treatment.

SOURCE:
Sally Blackwell MNS
Pain Management
++++++++++++++++++





Tricyclic antidepressants.

Tricyclic antidepressants are a group of drugs used to relieve symptoms of
depression.

These drugs may also help relieve pain.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++++


Trigger points
Trigger point.s are places on the body where muscles and adjacent fibrous
tissue (fascia) are sensitive to touch.

These areas are generally in the upper and lower back muscles, but they may
occur elsewhere.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++++

People living with chronic pain often compare their lives to a roller coaster ride.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++++++

Porphyria patients can have good days when they feel uplifted and in control.

Unfortunately, most porphyria patients find that good days are followed by bad
days when their mood drops and they feel helpless.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

Rarely does pain stay at an even level — it fluctuates.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Most porphyria patients state that their pain waxes and wanes and is very
unpredictable.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

Pain also doesn’t have any boundaries.

When a part of you is in pain, your whole body reacts.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Porphyria patients need to try to understand, accept and manage their porphyria
and the pain that evolves from porphyria.

Porphyria patient's behavior and emotions may go through a series of ups and
downs dealing with the pain that they must endure.

This is especially true the porphyric has debilitating pain.

Behavioral and emotional changes follow a predictable pattern in porphyia
patients who are dealing with chronic pain.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

One of the first noticeable effects of chronic pain is the change it brings in your
day-to-day activities. Regular tasks often become more difficult, even
impossible.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++++++



Because of pain, many porphyria patients decrease their activity.

Rather than "farming out" tasks to others to complete for them, they let things
pile up because they don’t like the idea of watching others take over your
responsibilities.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

Many porphyria patients then wait for a day when they feel better and then all of
a sudden they have a big increase in activity.

The result is too much activity ends in more pain and back to decreased activity.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Many porphyria patients belittle themselves for trying to do too much at one time
and spend the next few days resting and trying to get rid of the pain and feel
better again.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++

When the porphyria patient begins to feel better, and they become more active,
the pain worsens.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

For many porphyria patients dealing with pain leads to a loss of strength and
physical deconditioning.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

The time you spend lying around is making you tired and weak and less able to
finish up those leaves.

Because of your long stretch of inactivity, your stamina is leaving you.

You get fatigued easily, and even the thought of physical labor is daunting.

SOURCE:
Mayo Medical Education'Mayo Foundation
Rochester, MN
++++++++

It has been found that porphyria patients who do not learn to deal with their pain
often find themselves facing withdrawal and isolation.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++

You find yourself spending more time alone and less time with those who care
about you.

Because you’ve stopped going out with your friends, they’ve stopped calling.

They figure that you’d just turn them down anyway, so why bother?

Your family has become accustomed to doing things without you.

They’ve started going out to dinner or attending social events without you.

They think they’re accommodating you by not forcing you to go.

You retreat even further from your family, friends and favorite activities.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++

Porphyria patients often find themselves on an emotional rollcoaster because of
lack of control over their cycles of pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++

Just as your behavior fluctuates when you’re in pain, so do your emotions.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++

AMA chronic pain media briefing Journal of the American Medical Association

Release of NEW Study from Pain Medicine, the journal of the American
Academy of Pain Medicine: Chronic Persistent Pain After Severe Burns - A
Survey of 358 Burn Patients Little research has been done to examine pain's
long-term effects on the lives of burn victims, according to Dr. Daniel Carr,
medical director of the New England Medical Center Pain Management Program.

Far more needs to be done to reduce the physiological, psychological, and
emotional pain endured by burn victims.

Rx Laughter - Using Humor to Help Treat and Prevent Pain

Dr. Margaret Stuber, professor of Psychiatry & Biobehavioral Sciences at the
Neuropsychiatric Institute & Hospital at the UCLA School of Medicine, describes
this integrative and innovative approach of studying and using the potentially
healing power of humor to reduce pain and stimulate immune function in children
with cancer, AIDS, or diabetes - and in children receiving organ transplants,
bone marrow treatments, or dialysis.

Dr. Stuber will release new data on cortisol levels and pain tolerance at the
briefing.

Disparities in Access to Pain Medications and Cultural Considerations

When Establishing a Pain Management Plan Dr. Stacie Pinderhughes, assistant
professor of Geriatrics at the Mount Sinai School of Medicine, offers a number of
successful strategies to overcome access barriers to appropriate analgesia:
"When a patient resists treatment for pain, the physician needs to understand
and ask, 'What are your concerns about taking these pain medications? Are you
fearful of addiction? Are you fearful that this means you're giving up? Does this
have something to do with valuing suffering or a valuation of stoicism?'"

Future Approaches to Pain Management

Accelerating advances in neurophysiology and neurochemistry are yielding new
therapies -- with fewer side effects -- for those with chronic low back pain,
chronic headache pain and chronic nerve pain, according to Dr. Russell
Portenoy, chairman of the Department of Pain Medicine and Palliative Care at
Beth Israel Medical Center.

Non-Operative Treatment Options for Low Back Pain

When addressing low back pain, non-specific treatment for a non-specific
diagnosis is going to yield non-specific results, according to Dr. Joel Press,
medical director of the Center for Sports, Spine and Occupational Rehabilitation
with the Rehabilitation Institute of Chicago. Effective treatment for back pain is
individualized therapy.

Preemptive Approach to Managing Pain: Evidence of Its Effectiveness and
Guidelines for Its Use

Even though COX-2 inhibitors are not indicated for pre-emptive pain (they're
indicated for acute pain), administering COX-2 inhibitors prior to surgery may
minimize the amount of postoperative pain control needed, according to Dr.
Mark Lema, chairman of the Department of Anesthesiology & Pain Medicine at
Roswell Park Cancer Institute

Newer COX-2 selective drugs may offer a new option to physicians and patients
if studies continue to demonstrate their effectiveness in reducing both the pain
and the need for more opioid medication.
Recent Trends in State Policies Governing Pain Medications: An Annual Review
2001

Although patients and physicians harbor a variety of concerns about opioid pain
medications, including fears of addiction, investigation, or discipline, Dr. Aaron
Gilson, assistant director of the Pain and Policy Studies Group at University of
Wisconsin- Madison, predicts that increasing collaborations among those in
medicine and drug regulation bodes well for patients needing effective pain
management in the future.
+++++++++++


Associations that can help patients with pain include:

American Chronic Pain Association, Inc.
PO Box 850
Rocklin, CA 95677
(800) 533-3231
www.theacpa.org

Chronic Pain Foundation
19744 Beach Blvd.
PMB 245 Huntington Beach, CA 92648
www.chronicpainfoundation.com

American Pain Society
4700 W. Lake ave.
Glenview, IL 60025
(847) 375-4715
www.ampainsoc.org

American Academy of Pain Management
13947 Mono Way #A
Sonora, CA 95370
(209) 533-9744
www.aapainmanage.org

+++++++++++++++++
PORPHYRIA FACTS:

PAIN - UNDERSTANDING PAIN CONTROL


After years of uncertainty, you’ve finally learned what’s causing your discomfort.

Whether it is just the porphyria or the PN associated with porphyria, the result
is the same. It’s chronic.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++

Knowing the source of your pain isn’t enough to alleviate your discomfort.

There aren’t any quick fixes for chronic pain.

And often, there’s only so much doctors can do.

You’re the key ingredient.

If you want your life to improve, you need to take steps to manage your pain.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++










The first and most important step in controlling your pain is accepting the fact
that you may always have pain.

Some people can significantly reduce or eliminate their pain.

But if you’re like most people with chronic pain, your pain always will be a part of
your life.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++

A positive attitude is a key factor in dealing with chronic pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++

Managing chronic pain isn’t about making your pain disappear.

It’s about learning how to keep your pain at a tolerable level.

It’s about enjoying life again, despite your pain.

And it’s about accepting that only you can control your future.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++



There are both natural painkillers and pain enhancers within the human body.


Pain is a universal experience.

The degree to which you feel pain and how you react to it, however, are the
results of your own biological, psychological and cultural makeup.

Past encounters with painful injury or illness also can influence your sensitivity to
pain.

SOURCE:
Mayo Medical Education
Maro Foundation
Rochester, MN
+++++++++++

When pain persists beyond the time expected for an illness to end, it can
become a chronic condition.

No longer is the pain viewed as just the symptom of another disease, but as an
illness unto itself.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

The brain and spinal cord of a person can produce their own painkillers.

Such painkillers are similar to the narcotic drug morphine, used to treat severe
pain.

Two of these morphine-like pain relievers are called endorphins and
enkephalins

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

When endorphins and enkephalins are released, these substances attach to
special receptors in a person's brain, producing stop-pain messages.

Other substances in the human body do just the opposite.

These substances can intensify the pain that a person feels.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

Pain basically results from a series of exchanges involving three major
components: your peripheral nerves, spinal cord and brain.

SOURCE:

Mayo Medical Education

Maro Foundation
Rochester, MN
+++++++++++

A protein called substance P stimulates nerve endings at the injury site and
within the person's spinal cord, increasing pain messages.

Other pain enhancers work by activating normally silent nerve cells in the injured
area.

The activation of the silent nerve cells prompts the cells to discharge pain
messages even when the stimulation they detect isn't painful.

This not only worsens the pain but also enlarges the area of sensitivity.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

An important aspect about pain has to do with a process called sensitization.

Do to sensitization, chronic pain can be so severe and such pain may seem out
of proportion to the evidence of disease in the affected body tissues.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++

Sensitization can also explain why specific treatments directed at pain relief may
provide only limited benefit.

Although the neurobiology of sensitization is complex, the basic idea behind it is
straightforward.

When pain signals are transmitted from injured or diseased tissues, these
signals can then activate (sensitize) pain circuits in the peripheral nervous
system, spinal cord and brain.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++

The process of sensitization in pain control can sometimes amplify pain and
sometimes distort the pain message.

The result is a painful condition that is severe and out of proportion to the
disease.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

Sensitization may affect all regions of your nervous system that process pain
messages, including the sensing, feeling and thinking centers of your brain.

When this occurs, chronic pain may be associated with emotional and
psychological suffering.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++

Scientific evidence now confirms the presence of sensitization in various pain
conditions that don't involve amputation including the abdominal pain
experienced in porphyria which is not connected to any existing organ or the
removal of any organ.

When treatments in such cases are directed at injured or diseased tissues
themselves, they have no effect on the sensitized pain pathways in the spinal
cord and brain.

As a result, little benefit is experienced.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++



There is scientific research focused on identifying the molecular and cellular
processes that cause sensitization.

Hopefully the results of this research are likely to provide new and better
treatments for many types of chronic pain.



SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

The cause of chronic pain is not well understood.

There may be no evidence of disease or damage to your body tissues that
doctors can directly link to the pain.

This is especially true with the abdominal pain that acute porphyria patients
experience.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

There are a lot of theories about why chronic pain exists, but very little clinical
evidence exists.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

Sometimes, chronic pain is due to a chronic condition, such as arthritis, which
produces painful inflammation in your joints, or fibromyalgia, which causes
aching in your muscles.

Most often for porphyria patients chronic pain will be associated

with the porphyric neuropathy which causes deep bone pain.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++



Cronic pain may stem from a damage to a peripheral nerve such as experienced
in acute porphyria.

This type of nerve pain that lingers is called neuropathic — meaning the
damaged nerve, is causing the pain.

Neuropathic pain can also result from diseases such as diabetes or alcoholism,
both which are often associated with various forms of porphyria.

Once damaged, the nerve may send pain messages that are unwarranted.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

Little is known about why injured nerves sometimes misfire and send painful
messages.

However, one reason is that when a nerve cell is destroyed, the severed end of
the surviving fiber can sprout a tangle of unorganized nerve fibers (neuroma).

This bundle of nerve tissue then starts sending spontaneous pain signals.

These fibers also refuse to follow normal checks and balances that control the
rest of your nervous system, keeping pain at bay.

SOURCE:
Mayo Clinic
Rochester MN
+++++++++++

It used to be thought that pain transmission pathways in the peripheral nerves,
spinal cord and brain were hardwired circuits that simply communicated pain
signals from injured or diseased parts of the body to message centers in the
brain.


But based on recent scientific research, there is new knowledge of how pain
transmission actually works and how the conscious experience of pain is created
in the brain.

SOURCE:

Roxanne Asbury

Pain Management Centre

++++++++++

Years ago, people who complained of pain that had no apparent cause were
thought to be imagining the misery or trying to get attention.

Doctors now know that’s not true.

Chronic pain is real.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++

Unlike acute pain, with chronic pain you may not know the reason for the pain.

Chronic pain can also occur without any indication of injury.

Often this is the case with porphyria patients.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

People who condiiton themselves to endure pain that would incapacitate others
find that they can live a seemingly normal lifestyle.

Such individuals may experience less discomfort than people who focus on their
pain or who are more prone to complain.

Attitude has a lot to do with being able to cope with pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++++

Acute pain is triggered by tissue damage. It's the type of pain that generally
accompanies illness, injury or surgery.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++

Acute pain may be mild and last just a moment, such as from a sting.

Or it can be severe and last for weeks or months, depending on the injury or
infection causing the pain.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

When you have acute pain, you know exactly where it hurts.

In fact, the word acute comes from the Latin word for "needle," referring to a
sharp pain.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++++

Chronic pain hangs on.

Pain is generally described as chronic when it lasts 6 months or longer.

This is reflected in the word itself. Chronic comes from the Greek word for "time."

SOURCE:
Mayo Clinic
Rochester, MN
++++++++

A porphyria patients personal makeup plays an important role in how they can
cope with pain.

A person's emotional and psychological state, memories of past pain
experiences, and one's upbringing and attitude also affect how you interpret
pain messages and tolerate pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++

Cronic pain spans the full range of sensations and intensity.

It can feel tingling, jolting, burning, dull or sharp.

The pain may remain constant, or it can come and go, like a migraine that
develops without warning.

SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

A person's emotional state can also work in their favor improving even a severe
pain experience.

A positive attitude goes a long way in winning the battle in dealing with pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++

Pain comes in many forms: sharp, jabbing, throbbing, burning, stinging, tingling,
nagging, dull and aching. Pain also varies from mild to severe.

Severe pain grabs your attention more quickly and generally produces a greater
physical and emotional response than mild pain. Severe pain can also
incapacitate you, making it difficult or impossible to sit or stand.

The location of your pain also can affect your response to it.

SOURCE:
Mayo Clinic
Rochester MN
+++++++++

It’s important to be informed about your health, but don’t overdo it.

Spending too much time reading about your condition or discussing your pain
can be counterproductive.

Some pain helplines turned into pain pity parties.

It draws your attention to your pain, instead of away from it.

You need to stay positive and focus on others things besides you pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++++++


Most importantly, a porphyria patients needs to take responsibility for their pain.

A porphyria patient can not become emotionally dependent on their physician.

He or she should be compassionate but not overly sympathetic or enabling.

The doctor or pain physician can help the porphyria patient to learn to manage
their own pain, but ultimately the individual patient is the one in control.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++
People around people with pain generally react in one of two ways to pain
behaviors.

They become annoyed by them — "Not this again!" — or they become overly
attentive to the behaviors — "Here, let me do that."

Either response creates an unequal relationship in which people tend to focus
more on your behaviors than on your thoughts or feelings.

Pain behaviors also consume a lot of energy that could be channeled into other,
more productive ventures, such as taking steps to manage your pain.

The bottom line is that pain behaviors don’t help your situation, and they can
harm your personal relationships as well as your self-esteem.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++++

Common pain behaviors include:

Limping
Crying
Groaning
Grimacing
Limiting activity
Staying in bed
Using protective posture
Talking about pain or surgery
Withdrawing from others

SOURCE:
Neuropsychiatric Center
Mayo Clinic
++++++++++


When you’re in pain, others often can tell it by your actions.

These actions, called pain behaviors, refer to the things you do or say to signal
people that you’re experiencing pain.

They’re a way of calling attention to your pain — either consciously or
unconsciously.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++

Pain behaviors are a natural response to pain.

During an initial period of acute pain, they may help reduce your pain.

But over time they become ineffective.

For people with chronic pain, pain behaviors often become a habit.


SOURCE:
Roxanne Asbury
Pain Management Centre
++++++++++

PAIN - PSYCHOLOGICAL ASPECTS

People living with chronic pain often compare their lives to a roller coaster ride.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++++++

Porphyria patients can have good days when they feel uplifted and in control.

Unfortunately, most porphyria patients find that good days are followed by bad
days when their mood drops and they feel helpless.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

To accommodate pain, many patients change their life, often in ways that they
don't like.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++++


A pain scale is a system of rating pain.

Often based on a scale of 0 to 10, with 0 being no pain and 10 being the worst
imaginable pain.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

When porphyria patients are experiencing pain they need to realize their
pain threshold.

A pain threshold is the point at which pain is noticeable.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++


A pain tolerance level is the peak amount of pain that a person can endure.
:
SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++


Chest, back, and limb pain may occur in addition to abdominal pain associated
with acute attacks.

SOURCE:
The Porphyrias
Kappas, A. et. al.
The Metabolic Basis of Inherited Disease
7th Edition 1995
++++++++++


Rarely does pain stay at an even level — it fluctuates.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Most porphyria patients state that their pain waxes and wanes and is very
unpredictable.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

For some porphyria patients they may have to contact a pain rehabilitation
center for effective control of their pain.

Pain rehabilitation programs provides comprehensive, rehabilitative therapy for
people suffering from chronic pain.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

Pain in porphyria may last for hours or days.

Pain can become continuous in chronic cases.

SOURCE:
Acute intermittent porphyria in a
children's psychiatric hospital.
Boon, F. F. and C. Ellis
J Am Acad Child Adolesc Psychiatry
1989; 28(4): 606-9.
+++++++++++++

A chronic condition is one that is long-standing, not easily or quickly resolved.

SOURCE:
David Webner, M.D.
Department of Family Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
+++++++++++++

Much of pain in porphyria becomes chronic.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

Severe pain is often treated with narcotic analgesics.

Pain should be treated only under a doctor's supervision.

SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
++++++++++++


Pain also doesn’t have any boundaries.

When a part of you is in pain, your whole body reacts.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Abdominal pain experienced by people suffering from porphyria may be one of
three things.

a.. Pain unrelated to porphyria, and benign, e.g. pain caused by constipation,
'winds', irritable bowel, etc.
b.. An acute attack of porphyria, mild or severe, and potentially dangerous.
c.. Pain indicating a more serious problem, e.g. appendicitis or a peptic ulcer.
It is important to distinguish these three causes as the treatment is quite
different.

SOURCE:
The Porphyria Center
University of Capetown
South Africa
+++++++++++++


The initial and commonest manifestation of acute porphyria is abdominal pain.

Abdominal pain can be diffuse or localized and can be colicky.

SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
++++++++++++

Peripheral neuropathy is manifested as pain in the extremities. and it may
progress to a severe motor neuropathy.

SOURCE:
Journal of Photodermatololy, Photoimmunology and Photomedicine
1998 Apr;
14(2):48-54
"Management of the acute porphyrias."
Kauppinen R, M.D.
Department of Medicine,
University Hospital
Helsinki, Finland.
+++++++++++++

Fifty-one percent of acute porphyria patients experience pain in the limbs.

SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++


Pain and hope go in cycles.

Gradually, or perhaps suddenly, you feel better.

You're optimistic that your condition finally is improving or the new treatment
you're trying is working.

Excitedly, you start getting back into your old routine.

But after a time, the pain returns and you become deeply disappointed and lose
all hope of recovery.

You feel depressed and find that you can hardly make it out of bed in the
morning.

Things that used to matter to you, such as appearance or attending family or
social activities, don't seem as important.

SOURCE:
Mayo Foundation
Rochester, MN
++++++++


Porphyria patients need to try to understand, accept and manage their porphyria
and the pain that evolves from porphyria.

Porphyria patient's behavior and emotions may go through a series of ups and
downs dealing with the pain that they must endure.

This is especially true the porphyric has debilitating pain.

Behavioral and emotional changes follow a predictable pattern in porphyia
patients who are dealing with chronic pain.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++
The key to living with chronic discomfort is to control the pain, instead of letting
the pain control you.

SOURCE:
Coping With Chronic Pain
Sefrea Kobrin Pitzele
A Caring Place
Abbey Press
++++++++++++


One of the first noticeable effects of chronic pain is the change it brings in your
day-to-day activities. Regular tasks often become more difficult, even
impossible.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
+++++++++++++



Because of pain, many porphyria patients decrease their activity.

Rather than "farming out" tasks to others to complete for them, they let things
pile up because they don’t like the idea of watching others take over your
responsibilities.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++

Many porphyria patients then wait for a day when they feel better and then all of
a sudden they have a big increase in activity.

The result is too much activity ends in more pain and back to decreased activity.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Many porphyria patients belittle themselves for trying to do too much at one time
and spend the next few days resting and trying to get rid of the pain and feel
better again.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric
+++++++++

When the porphyria patient begins to feel better, and they become more active,
the pain worsens.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++
Some porphyria patients think that the cycles of chronic pain will never end.

However, a patient needs to learn how to break free of pain cycles.

A porphyria patient needs to have a positive attitude, and become involved in
life.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++

The downward spiral that often accompanies chronic pain occurs when all of
your attention is focused on your pain.

Learn to control your disease and chronic pain.

Develop a full life with a variety of activities on which to focus.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++++


For many porphyria patients dealing with pain leads to a loss of strength and
physical deconditioning.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

Many porphyria patients dealing with chronic pain begin to feel as though they
are no longer loved or needed.

Many porphyria patients with severe chronic pain find that their self-esteem hits
rock bottom.

Some may even talk of suicide.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++

Some porphyria patients begin to wonder if they are deserving of love and
attention.

As the patient draws deeper inside themself, their pain becomes the focus of all
of their attention.

Fear, isolation and depression, combined with days with nothing to do, make the
pain feel even worse.

The severity of your pain finally forces the patient to look for other forms of
treatment, setting the patient up for more pain.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++++

Porphyria patients need to remember that the way the patient reacts to their pain
also affects their family.

Your pain, and . Their responses to the patient's behavior and emotions can
take on parallel cycles of their own.

SOURCE:
Jocelyn Rogers PA
Pain Management
+++++++++++

When chronic pain first becomes a problem, family members generally show a
great deal of support.

They're often increasingly attentive to you, and they do more tasks around the
house so that you can relax and get better.

Family members also become vigilant in assessing your pain and keeping track
of the activities that seem to make it better or worse.

They monitor you closely in an attempt to help lessen your pain and help your
doctor make a diagnosis.

When your pain doesn't improve, your family's patience may start to wear thin.

At this stage, they begin to resent the extra burden they've been handed.

And though most family members realize that it's not your fault, they may find it
becomes difficult to separate the person from the pain.

They might begin to withdraw themselves and pay less attention to you.


SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++++


The time you spend lying around is making you tired and weak and less able to
finish up those leaves.

Because of your long stretch of inactivity, your stamina is leaving you.

You get fatigued easily, and even the thought of physical labor is daunting.

SOURCE:
Mayo Medical Education'Mayo Foundation
Rochester, MN
++++++++

It has been found that porphyria patients who do not learn to deal with their pain
often find themselves facing withdrawal and isolation.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
++++++++++

You find yourself spending more time alone and less time with those who care
about you.

Because you’ve stopped going out with your friends, they’ve stopped calling.

They figure that you’d just turn them down anyway, so why bother?

Your family has become accustomed to doing things without you.

They’ve started going out to dinner or attending social events without you.

They think they’re accommodating you by not forcing you to go.

You retreat even further from your family, friends and favorite activities.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++

Porphyria patients often find themselves on an emotional rollcoaster because of
lack of control over their cycles of pain.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++

Just as your behavior fluctuates when you’re in pain, so do your emotions.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++

Uncontrolled porphyria and chronic pain commonly causes a pattern of
emotions.

It is not uncommon for porphyria patients to be fearful and concerned when
dealing with chronic pain.



SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++

Family members often go through the very same emotions you do. Initially, they
fear the cause of your pain.

Later, when your treatment doesn't seem to be working and they're shouldering
more responsibilities, they become angry and begin to ask, "Why us?"

Just as you do, family members often feel a loss of control over their daily lives
and normal routines.

This frustration can lead them to withhold affection because they're angry at the
situation facing them.

They may unintentionally take this anger out on you.

They feel bad about being angry with you and, in turn, start to feel bad about
themselves and how they're acting. "I'm not a good person" and "I should be
able to handle this" are common thoughts.

Their guilt often leads to increased attentiveness and care, beginning the
behavioral cycle again.

Unfortunately, even though you and your family share common feelings, you
may find these feelings difficult to talk about.

Your family members fear it will sound like they're blaming you for your pain.

They also don't want to come across as being selfish and inconsiderate.

You have many of the same fears.

The silence, however, often brings more resentment and frustration.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++++

Often when family members do not understand the disease and can not
physically see the pain, they tend not to accept such pain and sometimes even
doubt the disease and associated conditions.

Because of this the porphyria patient tends to withdraw more and feel more
isolated at the same time the family is withdrawing from them.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++

Many porphyria patients feel that their families have withdrawn unto themselves
and somewhat ignore the porphyria patient themselves.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++

Learn how to manage your pain so that it's no longer the focus of your attention.

Once you have pain under control you can concentrate on the things that give
you pleasure and satisfaction.

This renewed feeling of control over your life will help you end the pain cycles.

SOURCE:
Mayo Medical Education
Mayo Foundation
Rochester, MN
++++++++++

PAIN - PORPHYRIC PN

Rarely PN neuropathy develops apart from the abdominal symptoms of acute
porphyria.

SOURCE:
The Porphyrias
Dr. Karl E. Anderson M.D.
Cecil Textbook of Medicine
20th Edition 1996
+++++++++++

When peripheral neuropathy occurs, it is usually preceded by abdominal pain.

SOURCE:
The Hepatic Porphyrias
Scmid, R.
Seminars in Liver DIsease
2:87 1982
+++++++++++

Pain associated with porphyric peripheral neuropathy is known as Neuropathic
Pain.

SOURCE:
The National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Bethesda, MD 20892
++++++++++

PN especially at night, may be almost unbearable.

SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
+++++++++

Dysesthesia, a numbness, tingling, and burning sensation can be present during
an acute attack.

SOURCE:
Acute Porphyrias: Pathogenesis of
Neurological Manifestations
Urs. A Meyers M.D. et. al.
Seminars in Liver Disease
Vol. 18, Number 1
January 1998
++++++++++++

Peripheral neuropathy is manifested as pain in the extremities, and it may
progress to a severe motor neuropathy.

SOURCE:
Photodermatology, Photoimmunology and Photomedicine
1998 Apr;14(2):48-51
Management of the acute porphyrias.
Kauppinen R.
Department of Medicine,
University Hospital of Helsinki,
Finland.
++++++++++

For some people symptoms are constant.

SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
+++++++++

PN pain may include:the sensation that you're wearing an invisible glove or sock
.
SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
+++++++++
;
There may be a Burning or freezing pain; or a sharp, jabbing or electric pain
associated with porphyric PN.

SOURCE:
Angela Reece FNP
Neurology
+++++++++

Extreme sensitivity to touch, even light touch

SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
+++++++++

Some patients say they don't have PN pain but have unpleasant and irritating
sensations, which may include "buzzing," "like bugs crawling," and "aching."

SOURCE:
Beth Israel
Continuium Health Partners
Department of Pain & Pallative Care
++++++++++




Some patients have constant pains, day and night, whereas others only have
noticeable pain at bedtime.

SOURCE:
Beth Israel
Continuium Health Partners
Department of Pain & Pallative Care
++++++++++


PN Pain can be caused by pressure on a nerve, touch, temperature changes,
and
poor blood flow.

The pain of peripheral neuropathy can vary from day to day.

SOURCE:
Peripheral neuropathy
Nerve
1996
++++++++++++

Depending on the nerves involved, symptoms can range from a mild tingling or
numbness in the fingers or toes to searing hand or foot pain.

SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
+++++++++++

There may be pain in the back, arms and legs.

SOURCE:
Toene, Jess G. et. al.
Physician's Guide to Rare Diseases
1992 Dowden Publishing
+++++++++

Pain in the legs is a common complaint or symptom.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++



Pain may be described as aching, burning, jabbing, searing, and a tightness.

SOURCE:
Peripheral neuropathy
Nerve
1996
+++++++++

Patients who develop pain with polyneuropathy describe the pain using a
variety of words, including "burning," "raw skin," "skin sensitivity," "sharp,"
"electric-like," "deep ache," "freezing cold," "like walking on ground glass,"
"itchy," and others.

SOURCE:
Beth Israel
Continuium Health Partners
Department of Pain & Pallative Care
+++++++++++



At times your symptoms may be barely noticeable.

At other times, especially at night, they may be almost unbearable.

For some people symptoms are constant.

These may include:

The sensation that you're wearing an invisible glove or sock
Burning or freezing pain
Sharp, jabbing or electric pain
Extreme sensitivity to touch, even light touch"

SOURCE:
Mayo Health News
The Mayo Clinic
Rochester, MInnesota
++++++++++++

Some patients experience much pain associatedwith their PN and may
complain that the pain interferes with their sleep.

SOURCE:
Beth Israel
Continuium Health Partners
Department of Pain & Pallative Care
+++++++++++

Muscle ache, muscle pain; myalgia or pain in the musclesis common in acute
porphyria.

Any pain in the muscles including muscle spasms, and joint pain, have been
evidenced in association with acute porphyria, even during times of remission.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++++




In some patients, the pain does not spread beyond the toes or feet; in others,
the progression to calves and hands occurs in months; and yet in others the
spread is very gradual, over many years.

SOURCE:
Beth Israel
Continuium Health Partners
Department of Pain & Pallative Care
+++++++++++



Treating the underlying condition can relieve some cases of peripheral
neuropathy.

In other cases, treatment may focus on managing pain.

SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
++++++++++

Neurontin (Gabapentin) has ben found to be beneficial in treating PN pain as
well as repressing seizure activity.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++


Therapy for peripheral neuropathy differs depending on the cause.

SOURCE:
The National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Bethesda, MD 20892
++++++++++++

Some PN may require physical therapy and/or splints may be useful in
preventing contractures (a condition in which shortened muscles around joints
cause abnormal and sometimes painful positioning of the joints).

SOURCE:
The National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Bethesda, MD 20892
++++++++++++

Using a TENS apparatus has shown to be beneficial in reducing PN pain.

SOURCE:
Robert Johnson MD
Internl Medicine
++++++++++



Use of TENS may help with nerves and numbness.

SOURCE:
The National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Bethesda, MD 20892
++++++++++++

For reducing PN pain at night the use of an electric mattress (not a blanket) has
been found to be beneficial.

Long used in the treating of arthritus sufferers, the electric mattress cover
radiates heats upward to the limbs most effected with PN pain.

SOURCE:
RObert Johnson MD
Internal Medicine
++++++++++

Electric mattress or jot tub or hot packs may help with deep bone pain.

SOURCE:
The National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Bethesda, MD 20892
++++++++++++

While doctors can be reluctant to prescribe pain killers, many studies have
shown that people don't take too many or get addicted when they need them for
pain relief.

SOURCE:
Mount Sinai Neuro Research Program
++++++++++++

Pain in the extremities is often described as muscle pain.

This pain may be a manifestation of early peripheral neuropathy."

SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++

Some patients with polyneuropathy experience pain contunously.

SOURCE:
Beth Israel
Continuium Health Partners
Department of Pain & Pallative Care
+++++++++++++

You may lose some of the feeling in your feet or hands.

SOURCE:
Understanding neuropathy
Adams, R.D. et. al.
Nerve
+++++++++++++


There may be pain in the back.

SOURCE:

The Hepatic Porphyrias
Scmid, R.
Seminars in Liver DIsease
2:87 1982
++++++++++++++

Half of all acute porphyria patients experience pain in the back.

SOURCE:

Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++++++
Limb, head, neck or chest pain can manifest in
acute porphyria.

SOURCE:

Dr. Karl E. Anderson
The Porphyrias
Hepatology: A Textbook of Liver Disease
Volume 1 Third Edition
1996 W.B. Saunders Company
++++++++++++++

Extremity pain is often noted in acute porphyria.

Both legs and arms can demonstrate pain associated with porphyria.


SOURCE:
Acute Porphyrias
Sleisenger & Fordtan
Gastrointestinal & Liver Disease
6th Edition Volume 2
1998 W.B. Saunders Company
++++++++++++++
Limb, head, neck or chest pain can manifest in acute porphyria.

SOURCE:
Dr. Karl E. Anderson
The Porphyrias
Hepatology: A Textbook of Liver Disease
Volume 1 Third Edition
1996 W.B. Saunders Company
+++++++++++++++

Extra magnesium may help reduce chronic lower back pain.


SOURCE:
Dr. Mildred Seeling
University of North Carolina
Might Magnesium
Eat Smart
USA Weekend
+++++++++++++++++

Gabapentin monotherapy appears to be efficacious for the treatment of pain
and sleep interference associated with peripheral neuropathy and exhibits
positive effects on mood and quality of life.

SOURCE:
Gabapentin for the symptomatic treatment of painful neuropathy
Backonja M, et. al.
Department of Neurology
University of Wisconsin
Madison Wisconsin 53792
JAMA
1998
Dec 2;280(21):1831-6
++++++++++++++

Mild pain can be adequately controlled with aspirin, paracetamol or
dihydrocodeine.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K
++++++++++++

PAIN - LEG

Leg pain can present in different ways and go by different names
including leg pain, and leg cramps.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

Pain is an unpleasant sensory and emotional experience associated with actual
or potential tissue damage or described in terms of such damage.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++


Leg pain involves any pain in the leg.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++

Leg pain is specific to the leg while other pains may be specific to
the foot, hip, knee, joint or muscle.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
Pain in the legs is a common complaint or symptom in the acute porphyrias.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++\

Cramping pain in the legs is often caused by muscle fatigue, strain, or
depletion of certain minerals.

Electrolyte panels should be run on porphyria patients with leg pain to detect
any deficiences such as potassium, calcium, sodium, and magnesium.

Such leg pain often will occur when a patient is taking diuretics.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

If a porphyria patient is on iv infusion and experiences leg pain
it is possible that blood to the muscles of the leg is being blocked
and that phlebitis has developed.

Phlebitis is another name for blood clots which are common with
blood coagulation problems and with blood clots due to iv accesses.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

Neuropathy can cause nerve damage in the leg which in turn will present with
leg pain.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++

Leg pain can be common in diabetics, smokers, and alcoholics.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++
Elevate the leg when resting or sitting.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++
Gentle massage may improve comfort.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++
If pain persists or swelling develops, call your health care provider.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++

There can be swelling of the ankles - feet - legs in acute porphyria.

Such swelling can be accompanied by leg pain.

SOURCE:
David Webner, M.D.
Department of Family Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++++



Peripheral edema is an abnormal buildup of fluids in ankle and leg tissues.

Edema can be accompanied by severe pain.

David Webner, M.D.
Department of Family Medicine
University of Pennsylvania Medical Center
Philadelphia, PA.
+++++++++++


Myofascial pain is a ain and tenderness in the muscles and adjacent fibrous
tissues (fascia).

Sometimes such pain occurs in the leg.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++

Leg pain can b acute or chronic.

Pain that occurs immediately after illness or injury and resolves after healing.

Chronic pain. Pain that persists beyond the time of normal healing and can last
from a few months to many years. Can result from disease, such as poprhyria.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++

Heat (warm soaks or a warm heating pad) or cool soaks may help improve
comfort.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++

Compression hose with elastic bandages or support hose can help.

SOURCE:
Steven Angelo, M.D.
Assistant Professor of Medicine
Yale School of Medicine
New Haven, CT.
+++++++++++

PAIN MEDICATION & TREATMENT


When a porphyria patients is suffering pain, the patient will likely do whatever it
takes to make it go away.

Treatment options can be varied.

What works for one porphyria patient, will not necessarily work for another.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++

Medication may seem like an easy way to control your pain, and it’s often the
best approach for acute pain, such as from a toothache or pain after a surgery.

But for many chronic pain problems, medication may not be the answer.

Even pain medications that are considered safe can cause side effects.

Medications can be costly.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++++++

Pain medications can become a crutch or distract you from more effective,
safer, long-term solutions.

Some patients become psycchological dependent on pain medication.

SOURCE:
Dr. Kenneth Carlson
Neuropsychiatric Medicine
+++++++++++++

Some people take them because they feel they need to, not because the
medications help.

These people are often surprised to find that stopping their medication isn't as
difficult as they anticipated.

They also often find that not using drugs gives them a greater sense of control
over their pain and life.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++++




Pain Management
What have studies shown in regard to effective pain management?

Virtually all patients with pain (both acute and terminal) as a real problem
have good outcomes with effective pain management.

SOURCE:
Robert M. Lowrey RPh
Pharmacology
+++++++++++++++
Pain Treatment

Do doctors have to treat pain?

Legitimizing pain management and palliative care for those with acute, chronic
and terminal pain, needs to be foremost in caring for chronically ill patients.

Legislation has been past mandatingthat pain must be treated.

Encouraging medical practitioners to become knowledgeable, and to universally
and regularly use good pain management tools and skills is a must.

SOURCE:
Robert M. Lowrey RPh
Pharmacology
++++++++++++++++++
Prescribing for Pain

Why are doctors against prescribing pain medication?

There are the concerns of the average physician who with the patients who have
long-term chronic pain and become addicted to their prescriptions.

Frequently, these patients simultaneously see a number of practitioners in order
to meet the medication needs of their addiction or to sell the excess drugs on the
street.

Pharmacists often have the same concerns in filling prescriptions for consumers
with chronic pain who are being prescribed enormous quantities of certain
medications.

SOURCE:
Robert M. Lowrey RPh
Pharmacology
++++++++++++++++++

All medications have risks and benefits.

The key to using medications to manage your pain effectively is to tailor them so
that the benefits outweigh the risks:

SOURCE:
Mayo Clinic
Rochester, MN
++++++++++

Why are so many doctors unfamiliar with pain management?

Historically, pain and its management have been a fairly low priority in
receiving time and attention from healthcare professionals.

Medical and nursing schools have devoted very little time to this
topic, leaving professionals with only the most fundamental skills.

SOURCE:
Robert M. Lowrey RPh
Pharmacology
++++++++++++++

Is it true that terminal patients have better pain management than other
patients?

In the '90s, pain management and palliative care for people with a terminal
diagnoses began to be recognized as important specialties.

Unfortunatly though, the same attention was not given to those with chronic pain
such as those with diseases such as the porphyrias.

SOURCE:
Robert M. Lowrey RPh
Pharmacology
++++++++++++++++++++++++
OTC NSAIDS

Are OTC NSAIDs good for treating chronic pain?

Non-prescription NSAIDs are not intended to treat chronic pain caused by
conditions that affect many older persons such as arthritis or migraines.

SOURCES:
Harris Interactive Poll
Dec. 16-19, 2002
National Consumer's League
Marie Griffin, MD,
Professor of preventive medicine
Vanderbilt University et. al.
++++++++++++

Acute Porphyria Pain

Two years later in 1959 Goldberg found in his study that 94 % of the porphyria
patients indicated abdominal pain.

Twenty years later, in 1979 Drs. Stein and Tschudy found that 95% of all
porphyria patients indicate having the abominal pain.

Chest, back, and limb pain may also occur either in the presence of or absence
of abdominal pain.

While not everyone experiences abdominal pain, and while it may not be present
with every acute attack, abdominal pain still remains one of the major symptoms
of acute intermittent porphyria.

SOURCE:
Robert Johnson MD

Internal Medicine

++++++++++++++++++

Fentanyl
Is Fentanyl considered safefor use as an anesthesia in acute porphyria
patients?

Fentanyl is a drug commonlyused in clinical anesthesia and is considered safe.

SOURCE:
Acute Intermittent Porphyria
J.W. Stevens et. al.
Anesthesia & Analgesia
Volume 82 No. 2
February 1996
+++++++++++++++++++
Hydrocodone

Is Hydrocodone porphyogenic?

Hydrocodone, oxycodone, and dezocine are only moderately or
weaklyporphyrogenic, whereas buprenorphine and morphine does not increase
porphyrin
accumulation.

SOURCE:
Effects of selected antihypertensives
and analgesics on hepatic porphyrin
accumulation
Implications for clinical porphyria
Herbert Bonkovsky et. al.
Biochemical Pharmacology,
1999, 58:5:887-896
+++++++++++++++++++++
Tramadol

Is Tramadol a safe drug?

Studies suggest that patients with acute porphyrias may be at greater risk for
developing porphyric attacks when treated with tramadol (compared with
the other analgesics).

SOURCE:
Effects of selected antihypertensives and analgesics on hepatic porphyrin
accumulation
Implications for clinical porphyria
Herbert Bonkovsky et. al.
Biochemical Pharmacology,
1999, 58:5:887-896
++++++++++++++++++++
Gabapentin / Neurontin

**** SEE DRUGS -PAIN & SEIZURES: NEURONTIN / GABAPENTIN
+++++++++++++++++++

+++++++++++++++++
Meperidine / Demerol

***SEE DRUGS - DEMEROL / MEPERIDINE
+++++++++++++++++


Many over-the-counter medications are actually more toxic than prescription
medications.

For example, Tylenol (acetaminophen) in appropriate doses is not a problem.
But when you get into excessive doses, it causes liver damage, especially when
taken in conjunction with alcohol.

The same could be true for aspirin and related OTC drugs.

As with many pain relievers, aspirin can cause GI and kidney problems when
taken in too high of a dose. But taken appropriately, they are not a problem.

SOURCE:
Sidney H. Schnoll, M.D.
Medical College of Virginia
Richmond, Virginia
++++++++++++++++++

Porphyria patients need to monitor their symptoms and keep a record in a log
book of all medication when taken and how much.

Side effects need to be noted.

If you take pain medication regularly, including OTC products, discuss your
medication and its benefits and side effects with your doctor.

You may be attributing your fatigue, stomach discomfort or sexual problems to
your pain, when it's really your medication that's causing these symptoms.

SOURCE:
Roberta Grasser
Pain Therapy
++++++++++++

It is most pertinent that all porphyria patients keep good records of all
medications that they are taking including OTC medications or any herbal
supplements.

Often side effects can occur when two different drugs are used at the same time.

SOURCE:
Bessie Mooreton
Pharmacology
+++++++++

Each person responds differently to medications and will need different amounts
or different types.

Once you've found the right medication, it's important to find the dose that works
best for you.

Your doctor will usually start you with a low dose of the medication and then, if
necessary, will gradually increase it.

He or she will monitor your progress to find the optimal amount for you to take.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++++

If you're taking a variety of medications, or if you're on a fixed income, drug
prices may become a financial burden.

The porphyria patients needs to find strategies that may help lower their
pharmaceutical bills.

Find out if there is a less expensive brand or a generic form of your medication
that you can use.

Always follow the directions carefully, including storing your medications as
directed.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++

Store drugs as indicated — usually at room temperature and out of direct
sunlight — and taking them as prescribed.

This will help to insure that they work properly and won't need to be replaced.

By following directions carefully a person can prevent nnecessary drug costs.

SOURCE:
Bessie Mooreton
Pharmacology
+++++++++

Medications often interact, and the chance of this happening is greater if you're
taking more than a few different drugs.

Keep records of medications that you take and in what doses. You may also
want to organize a system to help you remember when each medication needs
to be taken.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++++


Make sure that your doctor is aware of all of the medications that you're taking.

Such drugs include over-the-counter (OTC) products and herbal supplements
as well as all can have an effect on a pain medication.

Substances that you view as harmless, such as antacids, antihistamines or
alcohol, may interact with your pain medication.

Keep track of both your current medications and those you have used in the past
with these medication logs.

Take them with you when you visit your doctor.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++++

If you take an opioid or another potentially habit-forming drug, ask your doctor if
it would be in your best interest to taper use of the drug.

Before you decrease the dosage, ask about side effects that you may
experience.

Some side effects include anxiety and nausea.

SOURCE:
Roberta Grasser
Pain Therapy
++++++++++++

The doctor can discuss with the porphyria patient ways to lessen side effects.

A good doctor will probably want to see the porphyria patient regularly during
this time to check your vital signs and make sure that decreasing your use of the
drug doesn’t trigger other health problems.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++

Though medications are necessary for some people to manage their pain, they
aren’t without risks and shouldn’t be used indiscriminately.

Eventually, your doctor will probably guide you to try to manage your pain
without medications or to use as little as possible.

Once you’re able to control your pain with medications, your doctor may start
you on a rehabilitation program with a gradual increase in activities.

When you’re able to achieve your target activities, you then may be asked to
reduce the amount of medication that you take.

If the pain returns, you typically resume taking medications.

But by gradually decreasing the amount you take, you can determine the
smallest quantity of medication necessary for you to remain comfortable.

Under your doctor’s supervision, this process can be repeated every 3 to 6
months, gradually reducing your medications to see if the pain remains.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++++++



Depending on the severity of your pain and the type of medication you’re taking,
your initial goal may be simply to change to a safer medication or reduce the
amount of medication you take.

However, as you become more comfortable in your role as pain manager, you
may want to consider eliminating your use of all pain medications and rely on
other methods to manage your pain.

SOURCE:
Roberta Grasser
Pain Therapy
++++++++++++

Many porphyria patients have learned over time and with experimentation using
alternative forms of pain therapy that they can effectively control their pain
without the use of drugs.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++

Pain management may include changes in your lifestyle.

Some people need medication to treat a specific condition.

But may patients may be among those who can control their pain effectively
without drugs.

SOURCE:
Mayo Clinic
Rochester, MN
++++++++++

Seek help for payment of your medications.

Depending on your age and financial situation, you may be eligible to receive
help for procuring pharmaceutical.

Whenever possible, buy drugs that are on your health plan's list of medications
that are covered (formulary).

If you buy a drug that's not listed in the formulary, you may have to pay the
entire cost.

Many pharmaceutical companies offer special assistance programs for people
who can't afford their products.

Ask your doctor or pharmacist for information on obtaining free or low cost
drugs.

Where you live can make a difference too.

Many states have programs to help people with low incomes pay for prescription
drugs.

Contact your state's department of heath or social services for more information.

Some assistance programs are promoted only to health professionals.

Ask your doctor and pharmacist if they're aware of programs that can help with
the costs of your medication.

There are membership organizations which offer discount pharmacy services for
members.


The American Association of Retired Persons (AARP) is one such organizations
that offers discount pharmacy services for members.

Veterans have one of the best pharmaceutical programs.

Military retirees age 65 and over may be eligible to participate in the TRICARE
Senior Pharmacy Program of the Department of Defense.

Those who are eligible can obtain low-cost prescription medications.

SOURCE:
Mayo Clinic
Rochester, MN
+++++++

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