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WELCOME


Welcome to "the Porphyria forum", a website designed to give you the basic information you need to know as you research this usually manageable disease. If you are new to Porphyria, or if you are just doing research on the latest findings, the latest research, and the latest group of online friends that serve as a support group -- well you are likely to find a listing here.

For the absolute latest information and Porphyria findings, please visit :
the Porphyria blog

After some serious consideration, I have decided to give up my blog and The AIP Forum. I had planned to merge the two and start posting again as of March 1st. Obviously, that hasn't happened.

I am looking someone responsible & trustworthy to take over The AIP Forum. If that person also chooses to take over my Porphyria blog, they can have that too. If that person is you, please email me (aipforum_at_sbglobal _dot_net).
I spelled that out so spammers don't use their spiders to start spamming me to death. Just type it the way it sounds.

The AIP Forum will stay intact until someone else takes it over. I initially thought of just closing both sites for good. But both sites have gathered a rather substantial base of regulars, and their top placement in the search engines when people search for Porphyria information simply don't come over night. It's taken me years to get ranked that high. So whoever takes this over will have an immediate impact on the Porphyria community with a huge number of hits.

Just since JANUARY 1, 2004:, this is the story on The AIP Forum:

We have received 41792 pages views since 01.2004, 890 of them just yesterday. Our busiest day so far was Thursday, February 26, 2004 (1547 page views), while Saturday, February 14, 2004 (119 page views) was fairly quiet. Most people visit us on Monday with a total of 7328 page views, while Friday is a slower day, with a total of 4974 page views. On average, our busiest hour (with 2526 page views ) is at 10 :00, while only our dedicated fans show up at 4 :00, (with only 1157 page views).

So realize this is a HOT commodity that I'm walking away from!
Also: the web domain
www.porphyric.com is for sale. Just email me on that too.

I've enjoyed the ride.

-- Brent

 


What is Porphyria ? What Are The Porphyrias ?
Acute intermittent porphyria is a particularly severe form of porphyria which results from a deficiency of porphobilinogen deaminase. In common with the other acute porphyrias the clinical course is one of clinical latency followed by an acute episode following a precipitating event. The main clinical features of an attack are gastrointestinal disturbance and neuropsychiatric disorders. Acute intermittent porphyria is an autosomal dominant deficiency in porphobilinogen deaminase. This results in an excessive production of the haem precursors aminolevulinic acid (ALA) and porphobilinogen (PBG). Most individuals remain clinically latent until a precipitating factor triggers an acute attack. Porphobilinogen deaminase is an enzyme used in haem synthesis; its deficiency results in acute intermittent porphyria.

What are the "precipitating factors" ?
The most common precipitants are drug ingestion, alcohol consumption, and endogenous and exogenous steroid hormonal factors. AIP, VP and HCP may be precipitated by:

  • alcohol

  • drugs, especially barbiturates, and sulphonamides; enzyme inducing anticonvulsants, for example carbamazepine, phenytoin, phenobarbitone, primodone, oral contraceptive pill; also, diphenylhydantoin, rifampicin, chlordiazepoxide, griseofulvin and ergots.

  • fluctuations in female sex hormones may also precipitate acute porphyrias

  • infections

  • starvation

Clinical features are similar to those seen in lead poisoning (abdominal pain, constipation or vomiting, peripheral neuropathy, confusion or psychosis, tachycardia, hypertension).

Medications and drugs are often the worst triggers. For a comprehensive list of contraindicated drugs consult one of the safe/unsafe drug lists in the Links area of this page. Here is a list of the most commonly used unsafe medications: alcohols, barbiturates, sulphonamides, carbamazepine, phenytoin, rifampicin, pyrazinamide, chlordiazepoxide, griseofulvin, ergot, imipramine, meprobamate, lead poisoning

How common is Porphyria ?
Acute intermittent porphyria rarely presents before puberty. Females are affected slightly more than males; 1.5 to 2.0:1. The incidence in most parts of the world is 1.5 per 100,000; rates in Sweden are 1 per 100,000.

What are the symptoms / clinical features of AIP ?
Generally, the clinical features of AIP relate to the autonomic nervous system. There are acute attacks characterised by:

  • respiratory failure - bulbar paresis

  • motor neuropathy in acute intermittent porphyria

  • cerebellar signs may develop

  • hypothalamic dysfunction - inappropriate ADH secretion

  • hyponatremia - from inappropriate ADH, or GI or renal sodium loss

  • patients with AIP often have psychiatric disturbances, particularly with features of delerium - confusion, agitation, disorientation and hallucinations

  • syndrome of inappropriate antidiuretic hormone secretion

  • systemic hypertension

  • postural hypotension

  • cerebellar signs

  • confusion

  • agitation

  • hallucinations

  • severity and frequency of attacks varies widely between individuals

  • autonomic neuropathy: abdominal pain: in 80-90% of cases; diffuse or localised; often intermittent and spastic, constipation, vomiting, hypertension, postural hypotension, peripheral neuropathy, localized pain to a complete generalised flaccid paralysis

What investigation should be done if Porphyria is suspected?

  • biochemistry - hyponatraemia, hypomagnesaemia and hypovolaemia may occur during acute attacks

  • definitive test is porphobilinogen deaminase in erythrocytes which is decreased by 50%

  • urine - usually normal colouration when fresh but becomes brown, red or black on standing; marked increase in porphobilinogen (PBG); slightly elevated coproporphyrin and uroporphyrin

  • stool - coproporphyrin and protoporphyrin normal

  • liver function tests - sulfobromophthalein retention

  • increased serum cholesterol, serum iron and T4

  • hyperbetalipoproteinemia

  • abnormal glucose tolerance

What is the Ehrlich Aldehyde Test ?
Ehrlich's aldehyde test is used to confirm a diagnosis of acute intermittent porphyria. Ehlich's aldehyde reagent consists of p-dimethyl amino benzaldehyde in acid solution. Equal volumes of urine and Ehrlich's reagent are mixed. If a pink colour is formed this indicates a raised urinary concentration of either porphobilinogen or urobilinogen. In cases of raised porphobilinogen, as in acute intermittent porphyria, the pink precipitate is observed to be insoluble in chloroform.

Is there a treatment for my Acute Intermittent Porphyria ?
Currently, there is no specific treatment. Avoidance of precipitating factors is advised. Glucose loading and haematin infusion have some benefit but haematin may cause transient renal insufficiency and coagulopathy. Specific symptoms should be treated during an attack.

What does the APF say about all this?
"AIP is a rare disease that is inherited from one parent. It is almost always latent (i.e. does not produce illness) in childhood and is usually latent in most adults throughout life. When the disorder is active it can cause intermittent attacks of abdominal pain as well as a variety of other symptoms which range from mild to life threatening. After the correct diagnosis of AIP is made, simple precautions can be taken to prevent attacks. It is important to check all relatives of porphyria patients for the genetic defect, so they can take the same precautions and avoid becoming ill from porphyria. Even relatives who have never had symptoms should be tested."

source: The APF


So what does all this mean ?
That is an oversimpilization of our disease and attacks, and it's intended to give you the "medical mumbo-jumbo that might inmpress your friends or doctors or emergency technicians. In reality, only YOU can truly describe this illness as each case presents a variety of symptoms that magnify in one person, and hide-away and never to be seen in other people.

You would be doing yourself a great favor by sitting down during one of your lucid moments and list all the physical and mental troubles you experience due to Porphyria. Always keep that for future use. And that is also the day that you should start a brief journal about Porphyria. List all your daily activities (difficulties doing them), medications taken, what were your limitations, what was your pain scale (and what hurt), were you hampered from doing chores, miss work/school/church, etc ... In other words, list everything about your health and your Porphyria. That will be a basis for a great Porphyria journal that you can later share with your doctor, an insurance company, or the SSA and it's disability board.

Keep in mind the goal of this page is to prevent Porphyria attacks, especially those "life threatening attacks". Hopefully you will find useful information at this site, including the links on this page.

Facts you probably didn't know...
Type II does not show up in the blood serum.In other words the PBG-deamanase which in Type I will be diminished by 50% wil be normal in Type II and Type III.

Type III is known to have some cutaneous elements with itching and rash of the skin.

Type II has been referred to as "pseudo" cutaneous.....whatever that is supposed to mean.

14% without the diminished PBG_D  do have skin problems along with the CNS symptoms.





Porph-Group On Yahoo offers an email support and education group dedicated to helping you fine tune your Porphyria research. We are celebrating our 3rd year of serving the online Porphyria community.  Our membership hovers around 300 active and helpful people either with Porphyria, or caring for a loved-one who has this disease. Their greatest source of help and support comes from an email exchange between members.  But they have much more to offer...Click to subscribe to the Porph-Group email list, or visit their homepage at... http://groups.yahoo.com/group/Porph


NUTRITIONAL GUIDELINES FOR PORPHS...
The following dietary guidelines are recommended for Porphyrics...

  • Energy intake should be prescribed at a level to maintain a desirable body weight.

  • Carbohydrate intake should be 55 to 60 percent of total energy intake.

  • Protein intake should follow the RDA. (Recommended daily allowance.) This may be increased in elderly subjects, and reduced if there is kidney impairment.

  • Total fat intake should be less than 30 percent of total calories. (Particularly in individuals with high blood cholesterol levels, saturated fat should be less than 10 percent of total energy intake, polyunsaturated fat 6 to 8 percent, and the remainder monounsaturated fat.)

  • Cholesterol intake should be less than 300 milligrams per day.

  • Artificial sweeteners are acceptable.

  • Salt intake need not be restricted unless it is important for controlling hypertension. (The management of hypertension (high blood pressure) may include salt restriction. This is not discussed here because most patients with porphyria do not have persistent hypertension.)

  • Intakes of vitamins and minerals should meet the RDAs. Porphyria is NOT due to a vitamin deficiency! It can NOT be cured with vitamins.

  • Calcium intake in women should be at least one gram daily.

  • Iron intake should be adequate to avoid iron deficiency. Women with heavy menstrual blood loss and patients who have had frequent blood drawings due to illness and hospitalization may require greater intakes of iron. (Iron is a component of heme. Iron deficiency can compromise heme synthesis and may exacerbate porphyria. Therefore, iron deficiency should be avoided in porphyria. Early iron deficiency occurs before there is anemia (low blood count). Early iron deficiency can be detected by tests such as serum iron and iron-binding capacity, and serum territin.)

  • Alcoholic beverages should be avoided. Alcohol stimulates the heme biosynthetic pathway in the liver and can itself exacerbate porphyria. Alcohol has other harmful effects and can lead to weight gain. Some experts feel that small amounts of alcohol are not harmful in porphyria while others feel that even small amounts should be avoided.

  • Fiber intake should be about 40 grams per day, but should not be increased above 50 grams per day. (A high-fiber diet may increase the requirements for calcium, iron and trace minerals. High dietary fiber intakes should be avoided in patients with upper gastrointestinal problems (abnormalities in the esophagus or stomach) because sometimes excess fiber can accumulate in the form of "bezoars." Increasing dietary fiber intake sometimes causes abdominal cramping, diarrhea and flatulence. These can be minimized by increasing fiber intake gradually.)

  • Foods contain many natural chemicals that can stimulate the heme biosynthetic pathway. Although none have been definitely linked to attacks of porphyria, the possibility that these chemicals might contribute should be kept in mind especially when attacks of porphyria recur in the absence of a definite inciting factor. Some of the dietary factors that might have an adverse effect on porphyria include charcoal-broiled meats (which contain chemicals similar to those found in cigarette smoke), certain vegetables (such as cabbage and brussel sprouts which may contain chemicals that in large amounts can stimulate heme and porphyrin synthesis), and high intakes of protein. Probably, none of these foods need to be completely avoided in porphyria. However, it is important to consume a well-balanced diet and not to consume any particular type of food in excess. (The best way to maintain a well-balanced diet is to learn to eat a variety of foods from what are commonly referred to as the four major food groups. Detailed advice on how to do this should be sought from a dietitian.)

  • Food intake should be consistent, but should take into account lifestyle and physical activity.

  • The total daily energy intake should be distributed consistently with at least three regular meals each day.

  • Total energy intake must be individualized, because it varies with age, sex, and body weight, and is affected by physical activity. (Dietitians employ standard methods to estimate daily energy requirements. One of these methods is the Harris-Benedict equation.) It can also be greatly altered by illness.

  • Nutritional management of acute attacks of porphyria:

    Intravenous administration of glucose (a pure form of carbohydrate) is part of 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. Glucose and other carbohydrates can repress the pathway for synthesis of herne in the liver. As a result, the overproduction of prophyrin 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, and probably leads to more rapid improvement. Therefore, heme rather than glucose is becoming more accepted as initial therapy for an acute attack. However, it is still important to administer glucose and other nutrients. Particularly if an acute attack is severe or prolonged, sufficient glucose can be given by vein to meet the total energy requirements of a patient. This is best accomplished by a catheter that is inserted into a large central vein. Additional nutrients, including vitamins, minerals, amino acids and fat can be given in the required amounts to maintain all requirements. Provision of total nutritional needs in this manner by vein is commonly called "total parenteral nutrition". After recovery from an attack a high carbohydrate regimen should be prescribed, as described above.

  • More Nutritional & Dietary Info Porphyrics...

    Fasting or dieting is not permitted in people who have porphyria, as it can provoke an acute attack.

    The extra intake of sugar or glucose that is required to cure an acute attack may lead to a problem of overweight. Dieting should be avoided.

    A diet should be rich in fiber as constipation is a common complication for people suffering from porphryia.

    Bran should be included daily, 3 tbsp is a suitable quanity. It can be taken with a glass of water or added to milk, pancake batter, bread, waffle batter, minced meat, or Juice. 3 tbsp. of bran is about 20 calories.

    The remains of insecticides plus heavy metal toxins are stored in the liver. Eating liver should be avoided especially from wild game and poultry.

    In order to avoid insecticides in green vegetables and root vegetables, always peel and rinse them properly. Potatoes should be peeled before cooking.

    Only fruits that are not sprayed should be eaten without peeling, peel all other fruits.

    It is recommended that you avoid all alchoholic beverages, including those with low alcohol content. You should also avoid cooking, baking or marinating foods with products that contain alcohol, because they leave trace amounts behind on your food.

    Taking strong spices in excess effect some people with porphyria in negative ways.

    It is recommended to consume 6-8 (8oz) glasses of water along with other beverages high in glucose per day. (gatorade and other sport drinks are great sources of beverages high in glucose) -- also see recipe below.

    Fruit and Veggies that produce high alkaline urine are recommended for someone with porphyria.

    For an acute attack, extra carbs as in sugar or glucose are required. Some suggestions of this are as follows:
           a) 4 lumps of sugar per hour
           b) glucose tablets
           c) ready prepared glucose solutions
           d) gatorade or other high glucose beverages
           e) fruit pectin (nectar) drinks
           f) caffeine-free soft drinks ( gingerale)

    NOTE: other suggestions are routinely offered and may be helpful ! Recent suggesstion of D-50 bolus push at the hospital in addition to normal IV glucose have been highly recommended and are highly encouragable. Discuss this with your doctor and possibly have standing orders at your local ER if needed.

    NOTE2: Without a doubt, get diet counseling. Ask your doc for a prescription and it will probably be covered by your insurance or government medical assistance.




This Blog is all about Porphyria and relevant health related information.   It also contains an all new message board / chat page where you can leave a message or actually chat with others visiting the board at the same time you visit.  The blog is updated throughout the day, every day, with the latest Porphyria and health headlines and internet finds. Click the following link to check it out ...
http://porphyria.blogspot.com/

OTHER IMPORTANT PORPHYRIA LINKS AND INFORMATION:

   
GENERAL HEALTH LINKS:
No computer, phone line and webpage can take the place good, solid medical care from a knowledgeable doctor and staff. But having resources on hand to explore and answer your questions isn't a bad thing either. Here are a handful of sources to help you in your search for medical (non-Porphyria specific) information:

Complete home medical guide: Created by The Columbia University College of Physicians and Surgeons, contains information  on the health care system, symptoms & diagnosis, first aid, disease prevention, and drugs.

Food Finder : Nutritional information on items sold by fast food places. by Olen Publishing.

KidsHealth.Org - Nemours Foundation : Contains sections for kids, parents and professionals; covers wide range of topics including conditions, behavior, nutrition and safety.

Hardin Meta Directory : Large subject listing of Internet medical sites.

Health Watch UT SW Medical Center, Dallas : concise articles on a variety of health topics.

Health World Online : A health resource center--virtual health village where you can access information, products and services to help create your wellness-based lifestyle.

Mayo Clinic Health Oasis  : updates each weekday and includes easy-to-read articles on a wide variety of medical conditions and medications.

Medical Matrix : includes Medline, journals, patient education, drug info, medical subjects; very comprehensive.

Medlineplus : provides medical information on various health topics, drug information, doctors and other resources related to health

Merck Manual : An online version of The Merck Manual of Diagnosis and Therapy attempts to provide useful clinical  information to health  care professionals in a concise, complete, and accurate manner.

National Center for Health Statistics (CDC) : NCHS is the Federal Government's principal vital and health statistics agency. NCHS data systems include data on vital events as well as information on health status, lifestyle and exposure to unhealthy influences, the onset and diagnosis of illness and disability, and the use of health care.

NOAH: New York Online Access to Health : "Health Topics" in English and Spanish.

Official Site For Medicare Information : Site contains info on the latest Medicare options.

PubMed - National Library of Medicine : Search Medline for abstracts & a few full articles

Healthsquare Drugs & Medicines : Comprehensive information about more than 1,000 prescription medications, including side effects and possible food and drug  interactions. Data supplied by the PDR Family Guide to Prescription Drugs.

RxList : A HealthCentral.com network site providing an internet drug index. Includes searching by drug name,  keyword, and/or imprint code as well as alternative medicine FAQ's. Database consists primarily of  products currently on the US market or close to approval.

Immune System And Allergy : How Some Forms of Chronic Illness Trigger Allergy Formation

The Latest On Unsafe Vitamins :  From USA Weekend. Includes "Safe" Quantities

Medline & Pre-Med Searchable Database :  Links to journals and various medical databases.

MedicineNet: Diseases, Treatments, Procedures, Drugs, Etc.

guide to medical terms
british medical journal
doctor's guide to the internet
healthgate, gateway to Medline
intelihealth - johns hopkins
http--laurushealth.com
jama
medical sites
merriam webster medical dictionary
national institutes of health
national library of medicine
onhealth
pubmed
reuters health service
drug index
mednets pharmacy database
pdr.net
drug names/cross reference
chemical injury information you are what you eat
metabolic liver disease
genetic disorders databases
blood tests in liver disease
unchanged blood analysis
cutaneous sensations
mcs referral & resources
chemically sensitive living
gastrointestal diseases
drugs toxic to nerves
halftheplanet.com-disability
social security disability


Please visit other sites with caution. Not everything you read on the internet can be trusted. Please verify all information you read, and please consult a doctor or pharmacist before attempting anything you read on the net. Anyone claiming to be a Porphyria expert is blowing smoke. There are no experts, otherwise we would have a cure or at least better treatment




2003 Brent's AIP Page and The AIP or Porph Forum. All Rights Reserved. Site users are subject to standard web user practices. Any attempt at tampering with this webpage will be reported to appropriate authorities. We also have a privacy policy not to share any information about visitors to our site. Any server statistics are maintained for security purposes only.