Porphyria Educational Services
Monthly Newsletter
October 2002
Disclaimer
All information published in the Porphyria Educational Services Monthly Newsletter is
to provide information on the various aspects of the disease porphyria and it's associated
symptoms, triggers, and treatment.
Columnist and contributors and the information that they provide are not intended as a
substitute for the medical advice of physicians. The diagnosis and treatment of the
porphyrias are based upon the entire encounter between a physician and the individual
patient.
Specific recommendations for the confirmed diagnosis and treatment of any individual
must be accomplished by that individual and their personal physician, acting together
cooperatively.
Porphyria Educational Services in no way shall be held responsible in part or whole for
any injury, misinformation, negligence, or loss incurred by you. In reading the monthly
newsletters you need to agree not to hold liable any contributing writers.
Controlling Pain in Multiple Conditions Collectively
For many acute porphyria patients there is the pain that accompanies the acute
attacks. With 48 hours of glucose infusion the pain usually goes into
remission as does the acute attack itself.
However for many acute porphyria patients they become ridden with the PN
pain, a deep bone pain in the limbs that becomes chronic.
Fentanyl patches have worked for many but skin sensitivities often find the
patches cast aside, leaving the patient having to make multiple trips to
clinics or hospital emergency rooms to get the break through pain
under control.
Of course the best treatment for pain is one that is constant, the right
strength and is given in a timely fashion to avoid any break through pain
which makes it that much harder to get pain under any type of
control.
Added to the pain dilemna for many porphyria patients is pain from other
medical conditions such as MS, FM, migraine, arthritis, and many other
conditions.
Originally designed for use in cancer patients, but now a "given" in pain
control for countless others, are the inplanted pain pumps.
With the inplanted pain pumps porphyria patients no longer have to use the
pain pumps attached to the iv poles. They can avoid patches, trips to the
clinic for injections, or being hooked to iv pain pumps. Most of all they
can be relatively pain free.
As documented with many porphyria patients, the pain from other medical
conditions often becomes the trigger of acute porphyria attacks.
An example is that of a person with extremely painful FM, migraines, PN
secondary to the porphyria and porphyria its self.
FM is painful and without timely or proper medication = stress
Stress = Migraine headache onset
Migraine = acute attacks (nausea, vomiting, abdominal pain and PN)
PN = Peripheral neuropathy with deep bone pain
All four conditions now excerbate extreme pain. How does one treat these
painfful conditions presenting with pain?
Do you treat each pain cause individually? Do you treat pain in a combined
pain treatment? If so, how so?
Many porphyria patients have skin sensitivities which do not allow for long
term use of Fentanyl patches. Many also have logistical problems in receiving
treatment especially in rural areas due to the great distances the patient
must travel.
Even during hospitalizations pain reaches breakthrough before additional
dosages are given, causing the situation to be worse than needed and
allowing for the porphyria to kick in.
Because all four medical conditions present with severe pain, PAIN is the
focal point of concern.
With proper pain control the likelihood of triggering further porphyria acute
attacks will most likely drop significantly.
WIth many other patients with multiple medical conditions all exhibiting pain
the use of an implanted pain pump has produced relative pain free living and
greatly reduced acute attacks of porphyria.
Combining the use of the implanted pain pump along with the use of home
infusion of glucose and electrolyte supplementation (as needed) acute
porphjyria patients can now be free of repeated hospitalizations and
continuous suffering due to uncontrolled pain
.
Dr. Robert Johnson M.D.
Retired Clinician
The Different Carbohydrates and Porphyria
If you have a diagnosis of an acute hepatic porphyria then you need to learn
as much as possible about carbohydrates.
Low intake of carbohydrate is known to trigger acute attacks of porphyria.
Acute attacks are caused when an overproduction of porphyrins occurs within
the liver for the forms of AIP, HCP and VP. While the exact science or reason
for glucose is not fully understood, it is known that the intake of glucose
(the purest form of carbohydrate) will stop the over production of porphyrins
and thus bring an acute attack into remission..
Carbohydrates are your body’s main energy source. Carbohydrates are used not
only in your liver to maintain the right level of porphyrin production which
takes place there, but carbohydrate are used throughout the entire body.
Carbohydrates are used in the brain as its primary source of fuel. That
gives meaning to the old caption stating "Brain Power".
And what makes carbohydrates? At the base of all carbohydrates are sugar
components. There are different kinds of carbohydrates and this important for
porphyria patients to remember.
Depending on the number of components and how they’re linked, a carbohydrate
is classified as a simple carbohydrate (sugar) or a complex carbohydrate
(starch).
After being consumed the body breaks down complex carbohydrates into simple
sugars.
And what makes up simple sugars? Sweets, milk, fruit and some vegetables
contain simple sugars. .
And what makes up complex carbohydrates? Grain products and certain
vegetables contain complex carbohydrates.
Another name for the complex carbohydrates could be that of starches.
Starches are complex carbohydrates and include bread, cereal, rice, pasta,
beans and certain vegetables, such as corn, potatoes and squash.
Simple sugars are contained in fruit and dairy products. Every form of fruit,
from the familiar apples, bananas and oranges to pears contains simple sugars.
Milk and milk products contain simple sugars.
The vegetables with simple sugars includes all nonstarchy vegetables, such as
lettuce, asparagus and zucchini.
When planning your meals for throughout the day is best to eat a mixture of
complex and simple carbohydrates. The advantage of complex carbohydrates is
that it takes your body longer to break them down into sugar. This means
sugar enters your bloodstream at a prolonged rate. With some simple
carbohydrates, sugar may enter your bloodstream quickly.
For porphyria patients this is excellent for maintaining a continuous intake
of carbohydrate into the liver. For those porphyria patients who are also
diabetic, this continuous flow of carbohydrates is excellent in maintaining a
rather constant blood serum glucose level. .
The more fiber the food contains, the more slowly it’s digested and the more
slowly your blood sugar level rises which benefits diabetes, but is not
beneficial in carbo-loading pending the onset of an attack, however it is
good in maintaining a constant carbohydrate level for controlling porphyrin
production as long as there is a continuous carbohydrate intake.
By spreading carbohydrate intake every 2-3 hours through your waking
hours and also including a carbohydrate "middle of the night" snack, you will
effectively achieve maintaining a continuous carbohydrate level in the blood
stream and liver. At the same time you will achieve maintaining a steady
blood serum glucose level which will keep your diabetes under
control.
The best way to maintain porphyrin production in the liver and at the same
time to control blood serum glucose levels is by eating the same amount of
carbohydrates at similar times throughout the day.
Nutritional Guidelines for Porphyria
AIP Medical Guide
Sheryl Wilson (HCP), MSN, RD
Porphyria Patients with the Added Hepatitus C (HCV)
Having various forms of porhyria is a whole learning experience and
something that one needs to deal with medically and most likely for the rest
of their lives.
For some poprhyria patients, especially those with PCT, many find themselves
with chronic hepatitis C (HCV) as well.
Often these porphyria patients are surprised to find out that they harbor
this virus, because for the most part symptoms are usually absent.
The absence of symptoms is even true in the more advanced stages of the
disease manifestations.
Even porphyria patients who have progressed to cirrhosis find themselves
with HCV and are shocked to find out this diagnosis.
And just like with many other liver diseases, if the symptoms of HCV are
present, they are usually non-specific. Porphyria patients are very
familiar with the terminology "non-specific" because porphyria in any forms
is generally very non-specific.
It is noted that only about 20% of people with chronic HCV experience
symptoms. Those symptoms most generally noted include fatigue and
generalized weakness, and are often masked as a generalized weakness and
fatigue often accompany the symptoms of porphyria as well.
And as hepatic porphyria patients complain of abdominal pain, it is easy to
be unaware of the vague abdominal discomfort, often in the area over the
liver that is a part of HCV.
Weight loss, which is contraindicated for acute hepatic porphyria patients,
is often a sign of HCV. Decreased appetite, weight loss, and depression all
have been noted in HCV patients.
WIth HCV there is liver damage and inflammation of the liver itself. With
the usual signs and symptoms of poprhyria the signs and symptoms of HCV are
often masked.
Physical findings are often normal in people with chronic HCV.
Unless a porphyria patient's physician is looking for an enlarged and tender
liver, such signs and symptoms of HCV go unnoted, allowing for further liver
damage to occur.
In porphyria cutanea tarda (PCT) in which there is a skin abnormality that
may present as easy bruising of the skin, in addition to blisters that are
sensitive to the sun and bleed easily. In addition there may be areas of
increased or decreased skin pigmentation and increased hair growth, known as
hirsuitism.
Both hypothyroid and hyperthyroid have been indicated to occur in
approximately 5% of the individuals with chronic HCV. These disorders often
worsen once therapy with interferon has been initiated.
Elevated blood sugar (glucose) levels – has been found to be present in many
people with chronic hepatitis C.
Gregory Jackson PA
Internal Medicine
Knowing the Specific Types of Pain
All porphyria patients experience pain. But not all pain is the same. Nor do
all porphyria patient experience the same types of pain.
Acute hepatic porphyria patients are all too familiar with the colicky
gnawing and sometimes severe abdominal pain in the lower right quadrant
adjacent to the liver and often mistaken for liver pain.
Pain is an unpleasant sensory and emotional experience associated with actual
or potential tissue damage or described in terms of such damage. Pain can be
acute or it can be chronic. It can be mild or it can be severe. Pain can wax
and wane.
When pain is acute it usually occurs immediately and then resolves quickly.
When pain is chronic like it is with many aucte hepatic porphyria patients who
are termed as chronic smoldering porphyrics, pain is there almost all of the
time. It may be mild, but you still know it is there. For other, all too
often the pain is severe during the acute attacks and then the PN pain comes
and can easily bring one to their knees.
Chronic pain persists beyond the time of normal healing and can last from a
few months to many years. For porphyria patients it mostly results from the
peripheral neuropathy that acompanies ongoing acute attacks. And many acute
porphyrias patients also become diabetic and in time experience painful
diabetic neuropathy as well.
The pain from PN or diabetic neuropathy is known as Neuropathic pain.
Neuropathic Pain originates from a damaged nerve or nervous system.
Myofascial pain is still another type of pain. Myofascial Pain has tenderness
in the muscles and adjacent fibrous tissues (fascia).
Some acute porphyria patients unfortunately also have the medical condition
known as fibromyalgia. Again this is painful and in normally healthy people
can be treated with anti-inflamatories. However in acute porphyria patients
the use of most anti-inflammatories is contraindicated.
One of the best things porphyria patients can learn is that of tolerance, and
keeping busy.
Pain most likely is here to stay, so one needs to makes the most of their life
and work around the pain.
There are many levels of pain medication, physical therapy, massage, TENS,
theurapeutic pools and relaxation techniques that can help with the pain.
For the most severe and chronic pain there is a implanted spinal pain pump.
Work through the options and find those most appropriate for your pain.
Georgia Littleton RNP
Pain Management Team
Monthly Drug Update
PES drug information does not endorse drugs, diagnose patients or
recommend therapy. PES drug information is a reference resource
designed as a supplement to, and not a substitute for, the
expertise, skill, knowledge and judgement of healthcare
practitioners in patient care. The absence of a warning for a
given drug or drug combination in no way should be construed to
indicate that the drug or drug combination is safe, effective or
appropriate for any given patient.
ALLEGRA is a brand name for the generic drug FEXOFENADINE
which is a non-sedating antihistamine The drug carries a warning
with it for those who have liver or kidney disease, or electrolyte
abnormalities or low potassium.
ZYBAN is the brand name for the generic drug BUPROPION. It is classified as
an aminoketone drug and is used as an anti-depresant and for smoking
cessation. Side effects of this drug include abnormal liver funtion,
photosensitivity, jaundice, hepatitus, pancreatitis, edema, peripheral edema,
leukocytosis, muscle rigidity, leg cramps, muscle weakness, depersonalization,
neuropathy, and liver damage. WARNINGS & PRECAUTIONS: Not
recommended for persons with liver disease.
REACTINE is a brand name for the generic drug CETIRIZINE
which is a non-sedating antihistamine The drug carries a warning
with it for those who have liver or kidney disease, or electrolyte
abnormalities or low potassium.