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PORPHYRIA FACTS
PORPHRIA & IV ACCESS
There are many types of iv accesses for infusion therapy inporphyria.
Nobody wants to have a central catheter put in their body, but if your health care
provider determines that you need one, knowing which catheter best fits your
medical and personal needs will help you to adjust to the new catheter in your
daily routine.
PERIPHERAL VENOUS ACCESS LINES
Peripheral Venous Access lines are the typical "hospital IV" line put in your
hand or forearm when you are admitted to the hospital. It is a short catheter,
usually 3/4 to 1 inch long, inserted into a small peripheral vein and designed to
be temporary.
These catheters need to be changed every three days, or more often if they
dislodge from the vein.
Because the veins used are small and have less blood flowing past the
catheter, many medications can irritate a peripheral vein.
There is a plastic dressing over the catheter, which has to be kept clean and dry
at all times.
These peripheral IV lines work well in the hospital, where there are nurses to
monitor and change them frequently, but are impractical for extended
home use because of the potential for dislodging the small catheter from the
vein.
You have to be careful when moving your arm and daily activities become a little
more difficult.
You have to be careful when moving your arm anddaily activities become a little
more difficult.
You have to be careful when moving your arm and daily activities become a little
more difficult.
Blood cannot be drawn for lab tests from a peripheral catheter.
A peripheral catheter needs to be flushed with a saline and heparin injection
after every use or at least twice daily if not in use.
Saline is a salt solution used to clean or "flush out" the catheter and heparin is
flushed into the catheter to prevent blood from clotting in it between uses.
MIDLINE PERIPHERAL CATHETER
Midline Peripheral Cathetersare a type of catheter
is inserted into your arm near the inside of the elbow
and threaded up inside your vein to a length of 6 inches.
It is no more painful than having a peripheral IV inserted because you don't feel
the catheter moving up your vein.
These catheters typically last about six weeks -- a perfect catheter for a short
course of antibiotics, but not really practical for long-term intravenous therapy.
The end of the catheter will reach a much larger vein with more blood flow and
will cause less irritation of the vein.
Because the catheter is so soft and the end is well inside the vein, the chances
of it dislodging are much less than with a peripheral IV. It will still need to be
covered with a plastic dressing which needs o be kept clean and dry.
That means wrapping your arm in plastic wrap before a shower, or taking a bath
instead, holding your arm out of the tub.
Your nurse will clean the dressing and clean the site once a week or more.
Because it is near the elbow, this type of catheter shows under a T-shirt, but it is
hidden under a long-sleeved shirt.
You can do most normal activities with this type of catheter, except swimming, as
long as you are careful with the arm.
This type of catheter also needs to be flushed with saline and heparin after each
use or at least once daily if not in use.
Blood may not be drawn for lab tests with this catheter.
CENTRAL CATHETERS, PERIPHERALLY INSERTED
These catheters are centrally placed, meaning the tip ends up in the Superior
Vena Cava, which is the largest vein leading directly to the heart after collecting
blood from the rest of the body.
Even irritating medications can be given through a central catheter because
there is enough blood flow past the catheter to dilute the drug.
"Peripherally inserted" means it goes into your body at your elbow and the tip is
threaded up into your vein.
The Groshong type catheters have a valve at the tip, preventing blood from
backing up into the catheter, so heparin is not necessary.
Groshong catheters are usually thinner and more
flexible than other types of catheters and don't require a clamp, which adds bulk
to the others.
These types of catheters are inserted by a nurse, and are usually no more
painful than a peripheral IV insertion.
After the catheter is inserted, a chest X-ray is required to make sure the tip is in
the right location above the heart.
There is a plastic dressing at the elbow which must be kept clean and dry at all
times; it is changed and the site cleaned once a week or more by your nurse.
These types of catheters usually last for six weeks to six months, but frequently
last even longer than that.
With this type of catheter, you can do most normal activities, except swimming or
other extreme movements of the arm.
You can also have your blood drawn from a central catheter instead of being
"stuck" each time for lab tests, if your doctor allows.
The Groshong needs just a saline flush after each use or once daily if not in use,
while the Intrasil needs both saline and heparin flushes.
CENTRAL CATHETERS, TUNNELLED
Central Catheters, Tunnelled are catheter with tips that also end up in the
Superior Vena Cava, but the other end is tunnelled about six inches away under
the skin on the chest.
On the catheter, inside this skin tunnel, is a Dacron cuff which your skin seals
around, preventing bacteria from crawling along the outside of the catheter into
the blood stream.
Two popular brand names of this type of catheter are Hickman® and
Groshong®.
The Groshong catheters have a valve at the tip, tend to be lighter and more
flexible and don't require a bulky clamp.
They are usually more comfortable to "wear."
These catheters are surgically placed as an outpatient procedure under local
anesthesia.
Afterwards, the shoulder area is somewhat sore for a few days but is tolerable.
The catheter will usually exit the skin near the nipple area and since the end
dangles out, it's always available for use.
This makes it very easy to use for daily medications.
You will usually be responsible for cleaning the catheter exit site and changing
the the dressing daily.
For the first two weeks after a tunnelled catheter insertion, the gauze dressing
must remain dry and intact even in the shower which means covering it with a
plastic dressing or plastic wrap or taking a tub bath while keeping your chest dry.
After two weeks, the dressing can be changed immediately if it gets wet in the
shower.
The gauze dressing changes are easy to do, and once you get the hang of it, it's
a quick process.
Once your tunnelled catheter is healed completely (usually by six to eight
weeks), the catheter site should just need a bit of soap in the shower and
a plain bandage when you get out.
Instead of daily gauze dressings, some patients
prefer to have their nurse change the dressing once a week with a transparent
plastic dressing -- but it has to be kept dry and intact.
During the day, the catheter should be taped to the chest to prevent dangling or
catching it on something.
It won't be noticeable under T-shirts or even tank tops most of the time.
You can usually have some say in the location of the exit site by discussing your
preferences with the surgeon in advance.
You might prefer it to exit on the left side if you always sleep on your right side,
for instance.
Remember, though, that the surgeon will need to choose the placement that will
work the best with each patient's veins and chest.
You can perform all normal daily activities when the shoulder is no longer sore,
except for swimming.
When the catheter is fully healed, some doctors will allow their patients to swim
in a clean pool if the catheter site is covered with a plastic dressing meant to
keep out water.
Swimming in rivers, lakes or oceans is usually not allowed, and some doctors
don't allow their patients to swim (or hot-tub) at all.
Though these catheters are designed to be permanent, they are easily pulled
out by your doctor with just a slight stinging sensation.
The Groshong tunnelled catheter needs a saline flush after each medication or
every one to seven days if not in use.
The Hickman tunnelled catheter needs a saline and heparin flush after every
medication or at least once daily if not in use.
Blood can be drawn from a tunnelled catheter if your doctor consents.
CENTRAL CATHETERS, IMPLANTED PORTS
Implanted ports have all of the advantages of a central
line except they are not always immediately available for use.
A port is a small titanium reservoir with a rubber "stopper" that is attached to the
catheter entering your vein below the collarbone.
The whole thing is implanted under the skin in an outpatient procedure with local
anesthesia and IV sedation.
These catheters are usually not noticeable under your skin, but may sometimes
show as a small lump.
In order to use this catheter, the nurse must locate and clean the site, and place
a special needle through the skin and into the rubber stopper.
This can be done for each dose of medication or left in place with a plastic
dressing and weekly changes.
Ports that remain in place between usages are usually bulkier and more
cumbersome to "wear"than a tunnelled catheter, and the dressing needs to be
kept clean and dry.
The ports are made to withstand 2000 needle entries, but this does not irritate
the skin over the port.
Most people develop a callus that quits hurting when the needle is placed
through the skin.
As a patient, you can be taught to clean the site and access the port with a
needle, but it is difficult to learn and complicated to do.
Because of the procedures involved in accessing the port for use, these
catheters are usually not recommended for daily or more frequent medications.
They are perfect for someone who gets a medication only once a week or for a
week every six weeks or some other intermittent schedule.
One type of implanted port is actually placed in the arm near the elbow and the
catheter line threatened up the vein to the Superior Vena Cava, but they
offer no particular advantage over a chest port, tend to have more complications,
and are harder for the patient to self-access since two hands are almost required
for the procedure.
When the port is not accessed, it is hardly visible and requires no care other
than a once monthly access for flushing with heparin.
Patients with unaccessed ports can swim, though sometimes a doctor will
recommend covering the site with a waterproof plastic dressing.
Since the skin is an excellent barrier to bacteria, unaccessed ports rarely
become infected.
However, frequent accessing of a port, or eaving the access in place for
extended periods can make the odds of infection greater than with
a tunnelled-type catheter.
Blood can be drawn from a port for lab tests, if you doctor consents.
SOURCE:
The STEP Program
Seattle WA
1996
+++++++++++++++
IV GLUCOSE TREATMENT
A true acute attack can be a very serious problem,but if it is recognized and
treated correctly right from the start, it is possible in nearly every instance to
abort the attack and have the patient discharged from hospital within three or
four days.
This requires a willingness to:
Recognize the acute attack immediately and seek quick confirmation with a urine
PBG test
Stop all drugs which may be precipitating the porphyria Immediately start
infusion.
SOURCE:
The Porphyria Center
University of Cape Town
South Africa
+++++++++++++++++
What is the basic treatment for the acute porphyrias.
Ingestion of high carbohydrate intake with glucose infusion for 48 hours.
Avoidance of alcohol, toxic drugs, and infection, along with carbohydrate intake
will resolve attacks.
SOURCE:
The porphyrias.
Kappas A, Sassa S, Galbraith RA.
The Metabolic Basis of Inherited Diseases,
Scriver CR, Beud AL, Sly WS, Valle D eds
6th edn.
New York:McGraw-Hill,
1989; 1305 65.
++++++++++++++++++++++++++
Bolus injection
The injection of a drug (or drugs) in a high quantity (called a bolus) at once, the
opposite of gradual administration (as in intravenous infusion).
For hepatic porphyria patients receiving glucose infusion, quite often a D-50
bolus will be given at the onset of intervention care.
It is given quickly and in one large syringe.
SOURCE:
Robert Johnson MD
+++++++++++++++++++
What is the first choice of treatment to suppress the biosynthetic pathway
for heme?
Glucose given intravenously is most frequently recommended to suppress the
biosynthetic pathway for heme."
SOURCE:
"The Porphyrias"
Diseases of the LIver and Biliary System
Dr. Joseph R. Bloomer, 1993
*Dr. Bloomer is a member of the APF
Medical Advisory Board
++++++++++++++++++++
Intravenous administration of glucose which is a pure form of carbohydrate, is
the standard treatment of acute attacks of porphyria.
Glucose is given by vein because the stomach and intestine usually do not
function properly during an attack and material taken by mouth is not properly
propelled through these organs.
SOURCE:
Diet and Nutrition in Porphyria
Dr. Karl E. Anderson
American Porphyria Foundation
Houston, Texas
++++++++++++++++++
Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++++
Clinical findings have proven that infusion of glucose and high carbohydrate
intake will bring normalize porphyrin levels and bring a patient into remission.
SOURCE:
The effect of carbohydrate feeding on the induction of -aminolevulinic acid
synthetase.
Tschudy DP, et. al.
Metabolism 1964; 13: 396 406.
+++++++++++++++++
Glucose is most commonly used therapy in the intervention of acute porphyria
attacks.
The "glucose effect" reverses or aborts acute porphyric attacks by reducing the
rate of porphyrin synthesis using normal endogenous energy metabolism.
SOURCE:
"Use of glucose in acute porphyria"
Harris, A.L. et. al.
Journal of Family Medicine
++++++++++++++++++++
Administration of carbohydrate is the first choice of therapy in the treatment of
attacks of acute porphyria.
If it does not respond after 48 hours of glucose administration or if neurologic
manifestations develop or progress, treatment by hjematin is indicated to
suppress the ALA synthetase activity [over production of porphyrins in the liver.]"
SOURCE:
Treatmebnt of Acute Porphyria
Columbia Medical Newsletter
2002 page 23-24
York, L et al.
++++++++++++++++++++++
Symptoms of porphyria respond to intravenous carbohydrate loading.
SOURCE:
Acute Intermittent Porphyria
Differential Diagnosis of Acute Pain
Stanley L.Wiener, M.D. et. all
McGraw-Hill Inc. 1993
+++++++++++++++++++
Carbohydrate ingestion blocks d-aminolevulinic acid (ALA)-synthase, as has
been demonstrated in numerous clinical and experimental studies.
However, the mechanisms by which carbohydrates modulate the components of
porphyrins and heme synthesis are highly complex and only partially elucidated
to date.
SOURCE:
Beneficial Effect of Diabetes on
Acute Intermittent Porphyria
Folke Lithner, MD, PHD
Department of Internal Medicine
University Hospital, Umea, Sweden
Diabetes Care
2002; 25:797-798
++++++++++++++++++++
Nutritional management of acute attacks of porphyria require Intravenous
administration of glucose (a pure form of carbohydrate). It is part of the standard
treatment of acute attacks of porphyria. Glucose is given by vein.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++
What is the benefit that glucose gives?
Rapid infusion of glucose slows heme synthesis.
SOURCE:
Treatment of Hypertension in a
Patient With Acute Intermittent Porphyria?
Bruce Gardner, MD
Associate Clinical Professor of Family Medicine
University of Washington, Seattle
Attending Physician, Family Medicine
Swedish Hospital and Medical Center, Seattle
+++++++++++++++++++
Is glucose beneficial?
Glucose given intravenously can be beneficial.
SOURCE:
Merck Manual
1996
+++++++++++++++++++++
Heme deficiency in the liver of AIP patients stimulates an increase in
ALA-synthase which triggers an escalating metabolic chain reaction, leading to
an increase in the porphyrin content.
This reaction can be reduced by treating AIP patients with glucose."
SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria.
Andersson C, Bylesjo I, Lithner F
Primary Health Care Centre,
Arjeplog, Sweden.
+++++++++++++++++++
Glucose given intravenously is most frequently recommended to suppress the
biosynthetic pathway for heme.
SOURCE:
"The Porphyrias"
Diseases of the LIver and Biliary System
Dr. Joseph R. Bloomer, 1993
*Dr. Bloomer is a member of the APF
Medical Advisory Board.
++++++++++++++++++++
How can one suppress the overproduction of porphyrins causing the acute
attacks?
A high intake of glucose or other carbohydrates can help suppress disease
activity and can be given by vein or by mouth.
SOURCE:
Dr. Karl E. Anderson
University of Texas Medical School
Galveston, TX
++++++++++++++++++
Does glucose affectively stop porphyria damage during acute attacks?
Early use of carbohydrate [dextrose] is mandatory because porphyria damage
must be avoided. If this does not
bring an attack under control then hematin should be employed.
Clinical remission hinges on early employment of this intervention.
SOURCE:
Dr. Claus Pierach M.D.
"The Porphyrias"
Conn's Current Therapy
*Dr. Pierach is the former chair of the APF medical/
scientific advisory board.
+++++++++++++++++++++
Administration of carbohydrate is the first choice of therapy.
SOURCE"
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++++++
In acute attacks administration of carbohydratecombined with supportive care
will bring about a remission.
SOURCE:
Hepatic Porphyrias
Dr. Dwight Montgomery Bissell M.D. et al
Diseases of the Liver
Sixth Edition 1987
Lippincott Co.
++++++++++++++++++
Carbohydrate loadings represses hepatic ALA synthase and therefore the
reason that glucose in used as treatment during acute attacks.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
++++++++++++++++
Attacks of acute porphyria may be treated with intravenous glucose infusions,
glucose injections, and special high glucosedrinks.
SOURCE:
Disorders of Porphyrin Metabolism
A Goldberg et. al.
Plenum Medical Book Company
New York
1987
++++++++++++++++++
How much carbohydrate intakeis needed for bringing an acute
attack of AIP under control?
If the patient is able to consume food, a diet containing a minimum
of 300-400 grams of carbohydrate is ideal along with intravenous
infusion of glucose for up to 48 hours to bring the attack under control.
SOURCE:
Diagnosis of acute intermittent
porphyria in northern Sweden:
an evaluation of mutation analysis
and biochemical methods.
Andersson C, Thunell S,
Floderus et al
Journal of Internal Medicine
1995; 237: 301 8.
+++++++++++++++++++
Induction of ALA synthase is abolished by glucose.
This is known as the glucose effect.
SOURCE:
“Glucose effect" and rate limiting function of uroporphyrinogen synthase on
porphyrin metabolism in hepatocyte culture: relationship with human acute
hepatic porphyrias.
Doss M, et. al.
++++++++++++++++++
Treatment of AIP is based on large amounts of glucose administration for 4
days.
It is important to avoid precipitating factors such as drugs and fasting.
SOURCE:
The Porphyrias
Anderson, Karl E
Cecil Textbook of Medicine,
13th ed. Mc Graw Hill,
1994.
++++++++++++++++++++++
Heme deficiency can be stabilized by glucose infusion and by high carbohydrate
consumption .
SOURCE:
METABOLIC LIVER DISEASE
Neville R. Pimstone, MD
Porhyria Specialist
University of California Medical School
Davis, California
++++++++++++++++++++
Is glucose effective in treating an acute attack?
Glucose is effective in treating attacks of porphyria.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++++++++
Administration of carbohydrateis the first choice of therapy.
If it does not respond after 48 hours of glucose administration or if neurologic
manifestations develop or progress, treatment by hematin is indicated to
suppress the ALA synthetase activity [over production of porphyrins in the liver.]"
SOURCE:
Treatment for AIP
Clinica Chimica Acta
1995 page 171-5
Yutaka Horie et al.
+++++++++++++++++
In the symptomatic phase, glucose is effective in reversing the metabolic
processes responsible for the exacerbation.
SOURCE:
Lakartidningen 1998 Jun 24;95(26-27):3045-50
Floderus Y, Harper P, Henrichson A, Thunell S,
Andersson D Medicinkliniken
Sodersjukhuset, Stockholm.
++++++++++++++++++
Most acute attacks, if correctly recognized, settle with supportive treatment
which includes dextrose infusion and high carbohydrate intake.
SOURCE:
The Porphyrias
Stein and Tschudy
1970.
+++++++++++++++++
Administrtaion of carbohydrate must be given to reverse carbohydrate fasting in
the liver in order to stop the triggering of an acute attack."
SOURCE:
Disorders of Porphyrins or Metals
Dwight Montgomery Bissell M.D.
# 203
pp1182-7
Cecil Textbook of Medicine
Volume 1 18th Edition
+++++++++++++++++
By using preventive glucose you can prevent an attack or by using theglucose
as Intevention therapy you can bring about the recovery from anyone one of the
acute hepatic porphyrias AIP, VP or HCP"
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++++++++++++
What is the action of gloucose during acute attacks?
Glucose can diminish excess excretion of heme precursors
which in turn, can prevent an attack or hasten recovery froman attack of the
acute porphyrias.
Some physicians has prepared a standing order for patients who are prone to
porphyria attacks to help facilitate administration of intravenous glucose.
SOURCE:
The porphyrias.
Kappas A, Sassa S, Galbraith RA.
The Metabolic Basis of Inherited Diseases,
Scriver CR, Beud AL, Sly WS, Valle D eds
6th edn.
New York:McGraw-Hill,
++++++++++++++++++++++
Carbohydrate loadings represses hepatic ALA synthase and therefore the
reason that glucose in used as treatment during acute attacks.
SOURCE:
"The Porphyrias"
Karl E. Anderson M.D.
HEPATOLOGY:
A Textbook of Liver Disease
W.B. Saunders Company
Philadephia 1996
+++++++++++++++++++++++
By using preventive glucose you can prevent an attack or by using theglucose
as Intevention therapy you can bring about the recovery from anyone one of the
acute hepatic porphyrias [AIP, VP or HCP"
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++++++++++
Intravenous infusions or high oral consumption (300-400 g/d) of glucose may
abort attacks if given early.
Glucose reduces the activity of hepatic aminolevulinic acid synthase, the
rate-controlling enzyme of hepatic heme synthesis.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
++++++++++++++++++++
Have there been any studies that prove the use of glucose iv infusion to be
beneficial?
Several research studies have proven the benefits of glucose infusion in both
clinical studies and expiermental porphyria.
SOURCE:
The 'glucose effect' in acute hepatic porphyrias and in experimental porphyria.
Doss M & Verspohl F.
Klin Wochenschr
1981; 59: 727 35.
+++++++++++++++++++
Effective management requires prompt and accurate diagnosis.
Determination of urinary porphobilinogen,urinary and faecal total porphyrins,
and total porphyrins in erythrocytes and plasma must be made in order to
achieve a correct and complete diagnosis.
After determination is completed immediate infusion of glucose should begin.".
SOURCE:
Journal of Clinical Pathology
2001 Jul;54(7):500-7
ACP Best Practice No 165:
Front line tests for the investigation of suspected porphyria.
Deacon AC, Elder GH.
Department of Clinical Biochemistry
King's College Hospital
Denmark Hill, London UK.
+++++++++++++++++++++
Glucose can and does diminish the excess or overproduction of heme
precursors in the liver.
By using preventive glucose you can prevent an attack or by using the glucose
as Intevention therapy you can bring about the recovery from anyone one
of the acute hepatic porphyrias [AIP, VP or HCP]
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++
Glucose therapy is simple. For those who have only occasional episode they
can be iv accessed through a Hickman.
For those who regular run preventive glucose or are chronic porphyrics, and
need regular interventioncare, the place of a PICC or a PORT is most desirable.
Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake. A steady daily amount is necessary to suppress
disease activity.
SOURCE:
CIndy Matteson NPA
IV SPecialist
+++++++++++++++++++
Remember that daily requirements run 300 mg or better of carbohydrate daily.
During an acute attack a porphyric's requirement runs 500 mgof carbohydrate
total including both consumed and iv infusion of carbohydrate.
SOURCE:
Sheryl WIlson [HCP] MSN RD
+++++++++++++++++
Quite often porphyria patients when they go to the clinic or hospital while
experiencing an acute attack often will relate that they have not felt well enough
to eat or have been unable
to eat. Ironically it is the very treatment they need, high consumption of
carbohydrate containing foods.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++++
Often it is felt that if the porphyria patient had not skipped over the consumption
of carbohydrate containing foods, that they would have aborted their own acute
attacks and not needed to present for ermgency interventional care.
Preventive therapy is most cost effective, as well as most importantly a healthy
move.
SOURCE:
Eleanor Wilkinson MSN RN
IV Therapy Specialist
+++++++++++++++++++
Preventing acute attacks, a porphyric reduces the risks of renal failure, liver
failure, scarringof the liver resulting in cirrhosis or hepatic cancer.
SOURCE:
RObert JOhnson MD
Internal Medicine
++++++++++++++++++
Using preventive glucose therpay helps prevent the possibility
of respiratory failure and death.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++++
How does the use of glucose work in stopping an AIP acute attack?
Heme deficiency in the liver ofAIP patients stimulates an increase in
ALA-synthase which triggers an escalating metabolic chain reaction,leading to
an increase in the porphyrin content.
This reaction can be reduced by treating AIP patients with glucose.
SOURCE:
Effects of diabetes mellitus on patients with acute intermittent porphyria
C. Andersson, I. Bylesjö & F. Lithner
Journal of Internal Medicine
Volume 245 Issue 2 Page 193 -
February 1999
++++++++++++++++++++++
Glucose reduces the activity of hepatic aminolevulinic acid synthase, the
rate-controlling enzyme of hepatic heme synthesis.
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
++++++++++++++++++
Infusion of glucose slows heme synthesis.
SOURCE:
Prognosis of acute porphyria
Kauppinen R, Mustajoki P.
Medicine. 1992;71:1-13
+++++++++++++++++++++++
What does glucose do to stop acute attacks?
Glucose reduces the activity of hepatic aminolevulinic acid synthase, the
rate-controlling enzyme of hepatic heme synthesis."
SOURCE:
Medicine Journal
August 6 2001
Volume 2, Number 8
Maureen Poh-Fitzpatrick, MD
Department of Internal Medicine
Division of Dermatology
University of Tennessee
College of Medicine
+++++++++++++++++++
Glucose and other carbohydrates can repress the pathway for synthesis of heme
in theliver. As a result, the overproduction of porphyrin precursors and
porphyrins is repressed by carbohydrate administration.
Heme therapy (intravenous administration of hematin or heme arginate) has a
similar but much more potent effect.
However due to coagulation problems with heme, most clinician order the use of
glucose for up to 36 hours after which they consider hematin.
SOURCE:
Nutritional Guidelines For People With Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++
Has the use of glucose been found to stop the over production of porphyrins?
The use of glucose in chicken emryo studies has confirmed that the use of
glucose stops the elevated porphyrin levels in the heme pathway.
It also seems to show the ability to stabile the insulin levels and stop
hypoglycemic responses.
SOURCE:
Fischer WF, Stephens JK, Marks GS.
Effect of varying the insulin to glucagon ratio on porphyrin biosynthesis in chick
embryo liver cells.
Fischer WF, Stephens JK, Marks GS.
Molecular Pharmacology
1978; 14: 717 21.
++++++++++++++++++++
What is the definition of "glucose effect"?
The ability of the sugar glucose to block sugar metabolism by keeping the genes
which make the enzymes involved in the early steps of sugar metabolism from
making those enzymes.
SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++
The "glucose effect" was investigated in human acute hepatic porphyrias
(acute intermittent porphyria, variegate porphyria, coproporphyria and
porphobilinogen synthase defect porphyria) and in avian liver cells.
Patients with acute abdominal-neurologicalporphyria syndrome were treated
with high carbohydrate intake mainly in form of intravenous glucose infusions.
The biochemical response with a decrease of metabolites of porphyrin
biosynthesis was highly significant, accompanied by clinical improvement.
Patients with delayed detection of the disease under the condition of
paralysis died after temporary clinical improvement due to ventricular
arrythmias in one case and septicemia in the other.
The importance of early diagnosis and glucose therapy, as well as an omission
of drugs and alcohol cannot be overemphasized.
Complementary studies show the "glucose effect" in drug -mediated induction of
porphyrin synthesis in liver cells grown in culture: delta-Aminolevulinic acid
synthase and protoporphyrin synthesis are repressed.
SOURCE:
The "glucose effect" in acute
hepatic porphyrias
Doss M, Verspohl F.
Klin Wochenschr
1981 Jul 1;59(13):727-35
+++++++++++++++++++
Does glucose stop the overproduction of porphyrins in the liver?
Glucose and other carbohydrates can repress the pathway for synthesis of heme
in the liver. As a result, the overproduction of porphyrin precursors and
porphyrins is repressed by carbohydrate administration.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
+++++++++++++++++++
In what forms of porphyria has the glucose effect been found beneficial?
The "glucose effect" was investigated in human acute
hepatic porphyrias (acute intermittent porphyria, variegate porphyria,
coproporphyria and porphobilinogen synthase defect porphyria) and in avian
liver cells.
SOURCE:
The "glucose effect" in acute hepatic porphyrias and in
experimental porphyria.
Doss M, Verspohl F.
+++++++++++++++++++
Treatment with parenteral glucose was beneficial on the plasma ALA levels
inALA-D case studies.
SOURCE:
Journal of Neurological Sciences
1990
January 95(1):39-47
Porphyric neuropathy and hereditary ALA-D
Verstraeten L, et. al
Department of Neurology
University Hospital of Antwerp
Belgium.
+++++++++++++++++++
What do research studies show in regard to glucose effect?
Complementary studies show the "glucose effect" in drug -mediated induction of
porphyrin synthesis in liver cells grown in culture: delta-Aminolevulinic acid
synthase and protoporphyrin synthesis are repressed.
SOURCE:
The "glucose effect" in acute hepatic porphyrias and in
experimental porphyria.
Doss M, Verspohl F.
++++++++++++++++++
Levulose is another form of glucose infusion.
Levulose a simple sugar found in honey and in many ripe fruits and is
soemtimes called a fruit sugar, or fructose.
Another name for this is ketohexose.
Levulose is is labeled under this name throughout Europe and is available in
the UK under this name.
SOURCE:
Sally Stromberg RPH
Pharmacology
+++++++++++++++++++
Why is slow infusion of iv glucose important in porphyria patients?
Slow infusion of hypertonic solutions (glucose/dextrose/sucrose) is essential to
insure proper utilization of the dextrose.
By infusing slowly, this helps avoid production of hyperglycemia in patients, and
at the same time avoids hypoglycemic response with mental confusion, fatigue,
weakness.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++
Have there been any valid medical findings regarding B-6 therapy in conjunction
with glucose therapy?
Vitamin B6 and glucose therapy initiated resolution of symptoms.
SOURCE:
Acute intermittent porphyria
with atypical neuropathy.
Goren MB, Chen C.
Department of Medicine
Fairfax Hospital, Va.
Southern Medical Journal
1991 May;84(5):668-9.
+++++++++++++++++++
Treatment with parenteral glucose was beneficial on the plasma ALA levels in
ALA-D case studies.
SOURCE:
Journal of Neurological Sciences
1990
January 95(1):39-47
Porphyric neuropathy and hereditary ALA-D
Verstraeten L, et. al
Department of Neurology
University Hospital of Antwerp
Belgium.
++++++++++++++++++++++++
Can glucose metabolism be affected in PCT?
Research work been undertaken to analyze the effect of HCB on some aspects
of glucose metabolism, particularly those related to its neosynthesis in vivo. HCB
treatment decreased PC, PEPCK, and G-6-Pase activities.
The effect was observed at an early time point and grew as the treatment
progressed.
Loss of 60, 56, and 37%, respectively, was noted at the end of the
treatment when a considerable amount of porphyrins had accumulated in the
liver as a result of drastic blockage of uroporphyrinogen decarboxylase (URO-D)
(95% inhibition).
The plasma glucose level was reduced (one-third loss), while storage of hepatic
glucose was stimulated in a time-dependent way by HCB treatment.
This derangement of carbohydrates leads porphyric hepatocytes to have lower
levels of free glucose.
These results contribute to our understanding of the protective and modulatory
effect that diets rich in carbohydrates have in hepatic porphyria disease.
SOURCE
Hexachlorobenzene impairs glucose metabolism in a rat model of porphyria
cutanea tarda.
Mazzetti MB,
Arch Toxicol.
2003 Jul 29
++++++++++++++++
Is abnormal glucose tolerance found in PCT patients?
Abnormal glucose tolerance and serum antinuclear antibodies are found more
frequently among PCT populations.
SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD,
Professor Emerita and Special Lecturer,
Department of Dermatology,
Columbia University College of Physicians and Surgeons,
Clinical Professor of Medicine,
Division of Dermatology,
University of Tennessee College of Medicine
eMedicine Journal
+++++++++++++++++++++
Is glucose intolerance observed in PCT patients?
An involvement of the lipid metabolism, observed by the raised levels of plasma
NEFA and glycerol, is evident in PCT patients.
SOURCE:
Insulin resistance in porphyria cutanea tarda.
Calcinaro, F., et. al.
Journal of Endocrinology Investigation
+++++++++++++++++
Blood glucose levels are often above normal as well in PCT.
SOURCE:
Porphyria cutanea tarda. Don't forget to look at the urine.
Rich MW
Department of Internal Medicine
Northeastern Ohio Universities
College of Medicine
Rootstown Ohio
+++++++++++++++++++
Blood glucose levels are often above normal as wellin PCT.
SOURCE:
Robert JOhnson MD
Internal Medicine
+++++++++++++++
++++++++++++++++++++
How is HCP treated?
Heme therapy (to replace missing heme in the body) and treatmentwith
intravenous (IV) glucose are used to relieve the symptoms of acute attacks.
Medications to treat pain, nausea, and vomiting can also help.
Attacks can be prevented in some cases by avoiding factors that trigger
symptoms, such as certain drugs, alcohol, and dieting.
Doctors recommend that people who experience skin symptoms avoid sun
exposure as much as possible.
SOURCE:
Lister Hill National Center for
Biomedical Communications
National Institutes of Health
Department of Health & Human Services
++++++++++++++++++++++++++
Does glucose show benefit in treatment of ALA-D patients?
Treatment with parenteral glucose was beneficial on the plasma ALA levels in
ALA-D case studies."
SOURCE:
Journal of Neurological Sciences
1990
January 95(1):39-47
Porphyric neuropathy and hereditary ALA-D
Verstraeten L, et. al
Department of Neurology
University Hospital of Antwerp
Belgium.
++++++++++++++++++++
Treatment with parenteral glucose was beneficial on the plasma ALA levels in
ALA-D case studies."
SOURCE:
Journal of Neurological Sciences
1990
January 95(1):39-47
+++++++++++++++++++++
Can the use of glucose improve the memory?
The glucose levels in the hippocampus of rats fall significantly during complex
cognitive tasks.
Researchers measured the brain levels of glucose while the animals ran mazes.
The glucose levels in older rats, those more than 2 years old, dipped nearly 50
percent in the hippocampus and needed a half-hour to replenish. The rats'
decisions about which way to run apparently were impaired by their brains'
inability to retrieve glucose from nearby cells.
Younger rats, those 3 months old, had only a 12 percent decline in glucose
levels and recovered more quickly. When the older rats were given glucose
supplements before running the maze, they didn't show the same sharp drop in
brain sugar levels and did the job as well as younger rats.
Lest you think the human brain has little in common with rats, previous
experiments showed that both college students and people with Alzheimer's
disease do better on memory tasks after drinking sugary lemonade than when
drinking saccharine-sweetened beverages.
It does caution that sugar's effect on memory is fleeting.
However too much sugar, like too much stress, also can impair memory.
SOURCE:
Glucose Improves Memory
Dr. Paul Gold
Neuroscience
University of Illinois
Journal of Gerontology and Neurobiology of Learning and Memory
+++++++++++++++++++++
What is glucose therapy?
Glucose therapy is simple. For those who have only occasional episodes he
can be iv accessed through a Hickman.
For those who regular run preventive glucose or are chronic porphyrics, and
need regular intervention care, the place of a PICC or a PORT is most desirable.
Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake.
A steady daily amount is necessary to suppress disease activity."
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++++++
Glucose therapy is simple.
For those who have only occasional episodes glucose therapy can be delivered
through a temporary cather.
For those porphyria patients who are chronic or use preventive therapy, iv
infusion can be iv accessed
through a permanent PICC or a PORT.
A steady daily amount is necessary to suppress
disease activity."
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
++++++++++++++++++
What is glucose infusion solution?
Dextrose is better known today as glucose, this sugar is the chief source of
energy in the body.
Glucose is chemically considered a simple sugar.
It is the main sugar that the body manufactures.
The body makes glucose from all three elements of food, protein, fat and
carbohydrates,but in largest part from carbohydrates.
Glucose serves as the major source of energy for living cells. It is carried to each
cell through the bloodstream.
Dextrose is a sirupy, or white crystalline, variety of sugar, occurring in many ripe
fruits.
Dextrose and levulose are obtained by the inversion of cane sugar or sucrose,
and hence called invert sugar In commercially preapred glucose solutions there
can be added
nutritional supplementation including various electrolytes such as sodium,
potassium
or magnesium or calciumadditives as needed to bring deficient blood serum
level back to a normal range.
The key to glucose infusion is a slow methodical pace in which the body is
allowed to properly absorb the solution and bring biochemical balance about.
SOURCE:
Roy Jenkins, RPhm.
Pharmacology Department
++++++++++++++++
In a straight DS (saline) glucose iv that is run at 150 ml per hour for a 6.5 hour
run for a 1,000 ml bag, the hourlycaloric intake would be 56 calories total.
In this same iv the patient received 7.5 grams of carbohydrate an hour.
in 20 hours of iv infusion the patientwould receive 150 grams of carbohydrate.
The total caloric value for 20 hours wouldtotal 1,120 calories.
This is why a patient must continue to orally consume carbohydrates.
IV alone does not meet the minimal carbohydrate necessary intake.
SOURCE:
Sheryl Wilson MNS RD [HCP]
+++++++++++++++++++++
What is “preventive” therapy?
Preventive glucose can bedone through home infusion or outpatient clinic visits,
or of course done inpatient in a hospital setting.
Home infusion is mostpopular because you can do it at night in your home and
sleep in your own bed.
The whole principle of carbohydrate loading is tostop overproduction of
porphyrins in the liver whichhappens when a perosn does not have
enoughcaloric/carbohydrate intake or has exposure to chemicals which trigger
overproduction of porphyrins.
Carbohydrate is the natural preventive ...it prohibits over production, thus if you
maintain 300 mg or better of carbohydrate, overproduction of porphyrins does
not occur.
By infusing 2 or 3 times a week that maintains the carbohydrate level in the
blood and liver thus preventing overproduction of porphyrins to begin.
No overproduction of porphyrins, no acute attacks.
The standard protocol is 1,000 ml of glucose (dextrose) run at 150 ml per hour.
Thus one bag takes 6.5 hours
to run.
For prevention it is most productive run 3 times a week.
Many porphyics fail to realize the importance or ignore the importance of their
carbohydrate [sugar] intake.
A steady daily amount is necessary to suppress disease activity.
This is the basis of regular preventive glucose infusion.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++++++++
Preventive therapy is most cost effective, as well as most importantly a healthy
move.
Preventing acute attacks, a porphyric reduces the risks of renal failure, liver
failure, scarringof the liver resulting in cirrhosis or hepatic cancer.
More over safe guarding onesself from the possibility of respiratory failure and
death is essential by use of preventive therapy.
SOURCE:
"Stopping Porphyrin Overproduction
and the Acute Attack"
The Medical Forum
Allan Vasques MD et al
145-8 6:1999
+++++++++++++++++++++
Blood clot
The conversion of blood from a liquid form to solid through the process of
coagulation.
A thrombus is a clot which forms inside of a blood vessel.
If that clot moves inside the vessel it is referred to as an embolus (embolism).
The presence of atherosclerotic plaque lining blood vessel walls is a significant
stimulus for clot formation.
Porphyria patients receiving intervenous infusion often experience blood clot
formation.
SOURCE:
Rita Ballard RN
IV Therapy
+++++++++++++++++ +++
The consumption of a diet with high free glucose can promote the development
of oxidative stress that we tentatively attribute to hyperglycemia.
SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++
High levels of glucose can produce permanent chemical alterations in proteins
and lipid peroxidation.
SOURCE:
Oxidative stress is dependent on the free glucose content of the diet.
Folmer V, Soares JC, Rocha JB.
Departamento de Quimica
Centro de Ciencias Naturais e Exatas
Universidade Federal de Santa Maria
97105-900 Santa Maria, RS, Brazil.
Int J Biochem Cell Biol.
2002 Oct;34(10):1279-85
++++++++++
IV INFUSION - PICC’S
When experiencing repeated acute attacks of AIP it soon becomes apparent that
veins will no longer be accessible. Repeated insertions of cathers and phlebitis
take a toll on veins.
Also for simplifying the avilability of iv infusion as a preventive measure as well
as intervention through home care administration generally necessitates a PICC
or a Port access.
In this Fact we deal with the PICC.
PICC lines considered best
The Peripheral Inserted Central Catheter has been found to be the PICC of the
Bunch.
In terms of clinical use or in individualized home care the PICC in terms of
efficiency and risk management far outweighs its closest competers.
When placed is the forearm it allows for easy access for the individual patient to
perform maintenance.
Infection ratios for the PICC are minimal in comparison to all other catheters in
use today, and trail some 82 percent behind the long time used Hickman
catheter.
SOURCE:
Nursing Times
August 1998
19-25;94(33):70-3
"PICC of the bunch: Peripherally inserted central catheter."
Timmis L
++++++++++++++++++
PICC'S ideal for those with poor veins & regular blood draws
PICCs are ideal for persons with a history of blown veins and poor vein access.
PICC's are ideal for those who need regular routine blood draws such as PCT
patients, and those with coagulation problems.
SOURCE:
Nancy McAdams RN
IV SPecialist
+++++++++++++++
Advantages of the PICC
"Peripherally Inserted Central Catheters better known as [PICC] are designed for
the administration of I.V. fluids, blood products such as hematin or heme
ariginate, medications and parenteral nutrition solutions, as well as blood
withdrawal.
PICCs are ideal for persons with a history of blown veins, poor vein access and
those who need regular routine blood draws such as PCT patients, and those
with coagulation problems.
The catheter is placed into one of the large antecubital veins and is threaded
into the superior vena cava above the right atrium.
There are several kinds of PICCs available. The Bard Access Systems offers the
Per-Q-Cath® line of open-ended PICC s BARD also offers the very popular
Groshong® PICC
The GROSHONG comes in a variety of French sizes including single and dual
lumen configurations. Bard also offers a variety of tray configurations including
the full procedural tray making PICC placement easy and convenient.
Place of the Bard PICCs is done under a CT scan by a Interventional
Radiologist."
SOURCE:
Nursing Times
August 1998
19-25;94(33):70-3
"PICC of the bunch: Peripherally inserted central catheter."
Timmis L
Department of Gastroenterology,
City General Hospital,
Stoke-on-Trent. UK
+++++++++++++++++++
PICC's are surgically implanted
PICCs which are surgically implanted under fluroscope or CT scanning reduce
bacteruial infection rates by 40% over other types of peripheral intravenous
placements."
SOURCE:
Nancy McAdams
IV Specialist
++++++++++++++++++
PICC's easy for patient self use
In terms of patient self use and maintenance PICC's are also the easiest and
requires less work than other similar catheter lines.
Placement of the PICC in the forearm makes easy access for the individual
patient to perform the daily routine maintenance of flushing.
With today's needleless syringes, PICC lines are very simple to use."
SOURCE:
Betty Vinson RN
IV Infusion Instructor
+++++++++++++++++
Explanation of PICC
"Peripherally Inserted Central Catheters (PICC) are designed for the
administration of I.V. fluids, blood products, medication and parenteral nutrition
solutions, as well as blood withdrawal.
For those that do home infusion it is particular easy to care for and has a lifetime
of 5 years if one maintains it regularly.
The advantage of the PICC is that you can access it yourself and it does not
mean having to be stuck.
All lab testing can be drawn through it, which really saves on digging around
trying to find veins that are all ready been through a crisis.
The catheter is placed into one of the large antecubital veins and is threaded
into the superior vena cava above the right atrium.
The catheter is buried beneath the skin and there is no scarring at its access
point should you change ocation.
SOURCE:
Nancy McAdams RN
IV Specialist
+++++++++++++
Blood clots are rare in PICC's
"Phlebitis is PICC is rare."
SOURCE:
Nursing Times
August 1998
19-25;94(33):70-3
"PICC of the bunch: Peripherally inserted central catheter."
Timmis L
Management of IV Lines
IV Lines need careful management
Most important for porphyria patients is the careful management of Infections in
IV lines and ports. is Today many AIP patients enage in home infusion for the
prevention as well as the intervention of acute porphyria attacks.
It very important to remember that AIP patients may simultaneously experience
more than one type of catheter infection.
The use of oral antibiotics will often take care of the exit site infections or any
infections that may be found at the local site. Cleaning the site before dressing
the site is vital. Use of alcohol followed by betadyne is essential.
SOURCE:
Mary Ellen Crosby, MSN, RN
Infusion Therapist
++++++++++++++++
Lab tests need to be completed on any iv infection
Lab testing needs to follow all PICC or PORT live infections. Infections are
considered corrected when there is normalization of neutrophil and white blood
cell counts.
In general, port pocket infections will respond to antibiotics, but isolation of S.
aureus usually requires port removal.
SOURCE:
Rita Wickham, MS, RN; et. al.
College of Nursing
Rush-Presbyterian-St. Luke's Medical Center
Chicago, IL;
Catheter infection
++++++++++++++++++
There is a great variance in catheter and line infections from very few to
well over 60%
There are many reasons for such varying reports in catheter and line infestions.
Most likely these variances are due to a number of interrelated factors.
Such factors can include the protocol used by the attending clinic or hospital, or
by the home infusion provider.
Another factor include the immunocompetence of the patient themselves. Some
people just give way to infection more often
than others. Another factor is the patient's coagulation factors.
Many AIP patients also have a diagnosis of a Factor, protein C, protein S, or
Antithrombin III deficiency which need to be considered in clotting problems.
Moreover such statistics are often due to the way catheter infections are
reported by medical professionals."
SOURCE:
Jocelyn Adamson, RN
Hematology & Oncology
Recognizing catheter and line infections is most important.
For many things in porphyria a check list would be most welcome. Recognizing
catheter and line infection is one of those.
The main things in a catheter infection or line infection is that of pain and
erythema.
If the iv site is slow in healing, taking a full week or more, then that should be
cause for alarm.
SOURCE:
CIndy Delisle MSN RN
Infusion Therapist
+++++++++++++++++
Pain and erythema at the exit site or even with tunnel are all
of concern and should be indicated to an iv specialists immediately.
A simple laboratory test to check for neutrophils levels is appropriate.
Fever is an indicator however less specific.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++
There may be localized skin reactions ranging from erythematous rash to severe
blistering secondary to transparent dressings, plastic tape, cleansing solution, or
creams.
These reactions, which are often mixed-up with local infections. Such reactions
are significent because they alter skin integrity and can cause loss of the
catheter.
These complications can be corrected or reduced with the use of alternative
cleansing and/or dressing techniques.
SOURCE:
Mary Ann Hodgson RN
Infusion Therapist
+++++++++++++++++++
Bacteremia often indicates line clots
PICC line thrombosis should be suspected in patients with bacteremia.
SOURCE:
Nancy McAdams RN
IV Specialist
++++++++++++++++++++
Catheter care is essential
"One risk factor that nurses may influence significantly is the manner in which
catheter care in rendered.
Specific in-service training in catheter procedures and care should be mandated
in all policies and procedures. Special attention needs to be made of the
handling of iv catheters.
Studies support the notion that when a catheter care team or nurse provide
standardized, meticulous care, infection rates are significantly reduced, on
average from about 25% to 33% to about 4%."
SOURCE:
Mary Ellen Crosby MSN, RN
Infusion Therapist
+++++++++++++++++++++++
What about clots forming near the heart?
It is possible for a blood clot (thrombosis) to form in your vein at the tip of the
PICC.
Some people are given Coumadin to prevent this from happening.
Your PCP will determine whether this is appropriate for you based upon your
own individual coagulation history.
If you do develop a blood clot the catheter and line may have to be removed.
You will then be given medicines to dissolve the clot.
SOURCE:
Reginald Petry PA
Interventional Radiology & Vacular Medicine
++++++++++++++++
Central venous catheter
A central venous catheter is a small, flexible plastic tube inserted into the large
vein above the heart, through which drugs and blood products can be given and
blood samples withdrawn painlessly. A CVC is also called a central line, or
Hickman catheter.
++++++++++++++++++++
Central venous line --central venous catheter
A central venous line is a small, flexible plastic tube inserted into the large vein
above the heart, through which drugs and blood products can be given and
blood samples withdrawn painlessly. A central venous line is
also called central line, or a Hickman catheter.
++++++++++++++++++
Peripherally inserted central catheter [Temporary PICC]
A catheter inserted into an arm vein and used for periods of up to three months.
This catheter does not need to be surgically implanted and can be inserted at
home by a trained nurse.
+++++++++++++++++
PICC line
A catheter inserted into an arm vein and used for periods up to four years. This
catheter is implanted usually by an interventional radiology using a scope to
trace the line placement through the access vein and into the chest.
++++++++++++++++
I V INFUSION - PORTS
How common is infection in PORT usage?
Infection is a well-recognized complication of all types ofcatheters.26,39-41
Infections are potentially life-threatening, particularly in patients with neutrophil
counts of 500
cells/mm, in those with rapidly declining neutropenia, or those with protracted
neutropenia.
Local infections, including exit site and port pocket, and tunnel infections can
occur, as well as systemic infections from colonized thrombi or fibrin sleeves or
from intraluminal or extraluminal catheter colonization.
Partly due to the increased use of central venous catheter over the past 10
years, the causative organisms identified
used to include Escherichia coli, Klebsiella, Pseudomonas.
Today these cause 25% to 33% of infections, while gram-positive aerobes from
the skin (i.e., Staphylococcus aureus, S. epidermis, and streptococcus species)
are implicated in more than 50% of all infections.
Candida species are isolated 5% to 7% of the time.
Coagulase-negative cocci, which are normal skin flora, have a high pathogenic
potential when introduced through catheter insertion or other procedures that
violate intact skin.
These organisms are particularly difficult to eradicate because they can
preferentially bind to catheter surfaces and some are capable of producing a
slime-like glycocalyx that may resist antibiotics and host defense mechanisms.
SOURCE:
Long-Term Central Venous Catheters:
Rita Wickham, MS, RN; et. al.
College of Nursing
Rush-Presbyterian-St. Luke's Medical Center
Chicago, IL; and
Hurley Medical Center, Flint, MI.
Seminars in Oncology Nursing
1992;8,2(May):133-147.
++++++++++++++++++++++++++++
If you have edema should you stick to DW rather than DS in iv fluids?
Remember that during acute attacks often some of the glucose IV will contain
saline solution, while other may be only water.
This is important to remember if you have problems with edema.
Then you should use the water based glucose only.
SOURCE:
Nutritional Guidelines For Porphyria
AIP Medical Guide
Sheryl Wilson, [HCP], MSN, RD
++++++++++++++++++++++++++
If you can not eat enough carbs do you have to have iv?
Where vomiting precludes an adequate oral intake of carbohydrate, it is
imperative to admit the patient for intravenous therapy.
SOURCE:
Porphyria Information Centre
MRC/UCT Liver Research Centre
University of Cape Town S.A.
++++++++++++++++++++++++
Central venous thrombosis should be suspected in patients with bacteremia.
SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++
PORT pocket infections most often respond to antibiotics.
Isolation of S. aureus in PORTS requires PORT removal.
SOURCE:
Janelle Hurley MSN RN
Infusion Theraopy
++++++++++++++++++++++++
Management of Local Infections in ports is most vital.
It must be remembered that patients may simultaneously experience more than
one type of catheter infection.
Oal antibiotics and local site care is the usual treatment for exit site ifections.
Resolution of these infections usually occurs with normalization of neutrophil
and white blood cell counts.
In general, port pocket infections will respond to antibiotics, but isolation of S.
aureus usually requires port removal,
SOURCE:
Venous Catheters:
Rita Wickham, MS, RN; et. al.
College of Nursing
Rush-Presbyterian-St. Luke's Medical Center
Chicago, IL;
+++++++++++++++++++
Exit site infections often resolve with oral antibiotics and local site care.
SOURE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++
Management of Tunnel Infections is difficult at best.
Tunnel infections, especially those caused by Pseudomonas species, are
difficult to manage because they are commonly resistant to antibiotic therapy.
Although a trial of IV antibiotics may be helpful, this is not usually successful
unless accompanied by catheter removal.
SOURCE:
Long-Term Central Venous Catheters:
Rita Wickham, MS, RN; et. al.
College of Nursing
>Rush-Presbyterian-St. Luke's Medical Center
Chicago, IL; and
Hurley Medical Center, Flint, MI.
Seminars in Oncology Nursing
1992;8,2(May):133-147.
++++++++++++++++++++
Reported catheter-related infection rates vary greatly, from 2.7% to 60%.
These differences are likely related to a number of interrelated factors including
the immunocompetence of the patient, the number of catheter lumens or type of
device placed, the development of extraluminal thrombosis, the patient's
primary diagnosis, and the protocol of catheter care.
Furthermore, there are differences in the manner in which catheter infection
rates are reported.
The most common measure is based on the number of infective episodes per
1,000 days of catheter use.
SOURCE:
Long-Term Central Venous Catheters:
Rita Wickham, MS, RN; et. al.
College of Nursing
Rush-Presbyterian-St. Luke's Medical Center
Chicago, IL; and
++++++++++++++++++++++
Do transfusion work in stopping CEP?
Attempts to reduce erythropoiesis and lower circulating erythrocyte transfusions
have been successful in reducing the expression of the disease.
However, the complications of a chronic transfusion regimen are potentially
severe.
Severe hemolytic anemia with subsequent splenomegaly is one of the most
pronounced consequences of CEP.
Splenectomy decreases the hemolytic anemia by increasing the lifespan of
erythrocytes; however, the benefits are short lived.
SOURCE:
Erythropoietic Porphyria
Jeanette Hebel Matthews, MD,
Department of Dermatology,
The Skin Surgery Center
eMedicine Journal,
May 22 2002,
Volume 3, Number 5
++++++++++++++++++++++++++
Are CEP patients dependent on transfusion?
Severely affected CEP patients are ransfusion dependent and have utilating
cutaneous manifestations.
SOURCE:
Molecular Genetics & Metabolism
1998 Sep;65(1):10-7
++++++++++++++++++++++++
IV INFUSION TERMINOLOGY
Administration Set
Tubing. Used to connect solution or medicine to the catheter or needle.
Alcohol Swab
A small piece of gauze soaked in alcohol. Used to wipe off an area to make it
sterile.
Ambulatory Care Center
A treatment site where a patient walks or moves a wheelchair into the location to
receive their therapy, rather than being an inpatient in a facility or remaining in
the home.
The Infusion Suite may be in the Option Care facility or in a separate facility
such as a medical office building.
Antibiotic
Medicine used to fight an infection.
Aseptic
Free of germs or sterile.
Catheter
A piece of soft plastic tubing placed in a vein. Used to give fluids or medicines.
Central Line
A catheter placed into a large vein near the heart.
Connector
A device used in place of a needle.
Contaminated
Dirty, should not be used.
Dacron Cuff
A piece of fuzzy material around the part of the catheter that is under your skin.
It helps hold the catheter in place and keeps germs from moving up the catheter.
Electrolyte
A chemical in your body necessary for cells to work properly. Examples are:
Sodium, Potassium, Calcium, Magnesium, etc.
Entry Site
The place where a catheter goes into a vein.
Exit Site
The place where a catheter comes out of the skin.
Expiration date
The month and year written on medicines, solution, TPN bag labels and other
product containers. The product should not be used past the date.
Glucose
A sugar also called Dextrose. It is the main sugar in blood and body fluids.
Glucose also provide calories in TPN.
Heparinization
Flushing the catheter with a syringe of heparin so it does not plug or close off.
Infuse
To put a medicine or solution into a vein through a needle or catheter.
Infusion Pump
A pump used to move the medicine or solution through the tubing and into the
body.
Infusion Therapy
Infusion of nutrients and drugs directly into veins, muscles or under the skin
makes for more direct and efficient treatment.
Inject
To put a medicine or solution into a vein through a needle or catheter. Some
drugs may be injected directly into the skin or muscle.
Injection Cap
Rubber stopper on the end of the catheter used to put medicine or solution
through.
Injection (medicine) Port
A small, rubber stopper on the solution bag or catheter where medicines can be
added.
Intermittent
Off and on, not all the time.
Intravenous (I.V.)
To put into a vein using a catheter or needle.
Liter
Used to measure liquid. Almost equal to one quart.
ML (milliliter)
Used to measure liquid. 1ml is the same as 1cc.
Needleless Connector
See Connector.
Nutrients
Parts of food that nourish the body-proteins, carbohydrates, fat, vitamins,
minerals and water.
Occlusion
Clotting, plugging or closing off the tube so nothing goes through.
Occulsion Clamp
A special clamp used to pinch the catheter or tubing.
Parenteral
Parenteral may also be referred to as intravenous (I.V.). Receiving food or
medicines through your bloodstream.
Peripheral
Area on arms, hands, legs. I.V.s are placed in veins in the arms and hands.
Povidone-Iodine Swab
A small piece of gauze soaked in povidone-iodine. Used to wipe off an area to
make it sterile.
Prime
See Purge.
Purge
The word means “clean out.” In I.V. therapy, liquid is forced into the tubing to
“clean out” the air.
Rate
How fast the solution goes into the catheter/vein.
Sepsis
Infection in the blood stream causing chills and fever.
Sterile
Free of any bacteria or other germs.
Subcutaneous (S.C., sub-q)
To put under the skin using a catheter or needle.
Tapering
Slowing down an infusion of solution to prevent low blood sugar.
TPN (Total Parenteral Nutrition)
Another name used for intravenous hyperalimentation, providing food by IV.
Vial
A small bottle which has a rubber top and holds more than one dose of
medicine.
SOURCE:
Care, Inc.
485 Half Day Road, Suite 300
Buffalo Grove, IL 60089
2000
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