the p o r p h y r i a blog
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the p o r p h y r i a blog


Look's like this may be my hotel real soon ...

My hospital


In case you can't tell, I'm still sick. Not only is my Porphyria acting up, but I'm retaining water around my feet, ankles and in my legs so bad that I can hardly walk. I'm on Lasix for that. But the scary part is they believe it may be my heart causing this. Tests have been done. We're just waiting for the results.

So add heart problems to my Porphyria !

Top that off with Diabetes has attacked my eyes, and I'm now wearing glasses for the first time. This sux.

And today, I am going to a pain management specialist to get some help on this back problem, the Porphyria pain that has entered my life (this is rather unique to me), and the P.N. in my legs.

I'm falling apart.

Merry Bah-Humbug Christmas !

-- Brent

PS. Thanks everybody for posting comments. Bad eyes and all, I can still read. And they are much appreciated!

I will try to update later today or on Tuesday Dec 2nd.


Sorry

Sorry about no posts. I know I promised to do better, but I'm still pretty sick. Please keep checking back. This site is a priority for me once I get some strength. -- Brent


Tests you need if you suspect Porphyria:

PROTO blood test (measures porphyrins, blood)

Enzyme assay for:
Uroporphyrinogen decarboxylase
Protoporphyrinogen oxidase
PorphobilinogenPBG deaminase
Coproporphyrinogen oxidase
ALA dehydratase
Uroporphyrinogen III cosynthase
Ferrochelatase


What abnormal Porphyria blood test results may mean:

Increased levels of coproporphyrins may indicate:
congenital erythropoietic porphyria
sideroblastic anemia

Increased protoporphyrin levels may indicate:
infection
thalassemia
sideroblastic anemia
lead poisoning
iron deficiency anemia
erythropoietic anemia
increased erythropoiesis

Increased uroporphyrin levels may indicate:
congenital erythropoietic porphyria
erythropoietic protoporphyria


From a message board:

Subject: Possible porphyria??
Topic Area: Misc.
Forum: The Gastroenterology and Liver Diseases Forum
Question Posted By: IsabellaD on Monday, April 07, 2003

I am 26 years old. An RN from my family recently suggested that my ongoing symptoms remind her of porphyria. Here's what's happening:
--Light sensitivity: Sunlight hurts my eyes. I am very fair skinned and burn even when I wear sunblock. My hands are red and chapped, and sometimes actually crack and bleed. When I am in the sun for a few hours, I tend to feel very drained and exhausted. My mother is also extremely sensitive to light.
--Abnormal periods: I go 30-40 days between periods. My actual period lasts for 24-36 hours, and is accompanied by severe cramps.
--I have unexplainable stomach, leg and back pain.
--I have frequent migraines.
--I frequently feel sick to my stomach, no matter what I have eaten.
--I have frequent bouts of diarrhea and constipation.
--I have excessive fine hair on my face, hands and other areas.
--Tingling in the feet and hands
--I constantly crave food with sugar.
--I have had bad reactions to several medications. I cannot take any penicillin based antibiotic, zithromax, cipro, keflex or ceftin. When I had my tonsils removed, the anesthesiologist tested IV Ceftin before putting me under. I had convulsions and vomited on the table.
I have allergies and asthma which are kept completely under control with Singulair, Zyrtec and albuterol as needed. I have had my tonsils, appendix, umbilical hermia and an infarcted appendix epiploicum removed. I had scarlet fever at age 7 and Rocky Mountain Spotted Fever at age 6, chicken pox at 19 (treated with zovirax to prevent shingles) and mononucleosis and pneumonia in college. I have also been treated for anemia and many ear/throat infections.

Between 1998 and 2000 I have had a huge battery of tests, including EKGs, X-Rays, ultrasounds, bloodwork, urinalysis, etc. They have checked for Crohn's, gallstones, etc. All of the tests have had normal results. My blood pressue is normal--usually 110/70--and I do not have high cholesterol. In 2000 my doctor ordered a number of blood tests to check for Epstein-Barr, Cyclomegavirus, etc.--these came back negative. I do not suffer from depression and lead an active life.

I am uninsured and I do not have the financial means to pay for unnecessary tests. I would like to know if I should actually pursue testing for porphyria. Does it seem likely? If so--does anyone know of a doctor in the New York area that might be willing to see me on a sliding fee scale?

Thank you for any help you can offer.

Following is the Answer Posted By: Forum-M.D.-KYP on Monday, April 07, 2003

Hello - thanks for asking your question.

The incidence of porphyira is approximately 1-2 per 100,000. The disorder is expressed clinically almost invariably after puberty, and more commonly in women than in men. Abdominal pain, which may be generalized or localized, is the most common symptom and is often the initial sign of an acute attack. Other gastrointestinal features may include nausea, vomiting, constipation or diarrhea, abdominal distention and ileus. Urinary retention, incontinence, and dysuria are also common, and tachycardia, hypertension, and, less frequently, fever, sweating, restlessness and tremor may be seen. In up to 40 percent of patients, hypertension may become sustained between acute attacks.

Peripheral neuropathy is a common feature of porphyria. Muscle weakness often begins proximally in the legs but may involve the arms or the distal extremities. Motor neuropathy may also involve the cranial nerves, or lead to bulbar paralysis, respiratory impairment, and death. Sensory, patchy neuropathy may also occur.

Psychiatric complaints such as hysteria, anxiety, apathy or depression, phobias, psychosis, organic disorders, agitation, delirium, and altered consciousness, ranging from somnolence to coma are also associated.

Without examining you myself, I cannot comment on the probability of whether you have porphyria. Diganosis is obtained via urinary tests for porphobilinogen (PBG) and delta-aminolevulinic acid (ALA).

If you are having constant stomach pain, you may want to consider an upper endoscopy since this can evaluate many diseases that can cause that symptom.

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Thanks,
Kevin, M.D.

Bibliography:
Sassa. Acute intermittent porphyria. UptoDate, 2002.


Heme and Iron and Porphyria

http://www-medlib.med.utah.edu/NetBiochem/hi.htm


Basic Porphyria Information

I found this on the web and thought it might be a bit useful for you. With the holidays coming up and the likelyhood you will be having to explain this disease during family gatherings, this one might be a great way to break it down into simple terms that a knothead relative might be able to understand. Get prepared for those lovely family dinners and tons of shitty fruitcake! :)
Porphyria is a name given to a group of metabolic disorders. These disorders cause the individual to accumulate "porphyrins" or "porphyrin precursors" in their body...which in turn causes an an abundance of the porphyrins.

These porphyrins are normal body chemicals however, they normally do not accumulate. The type of chemical that builds up in their body depends upon the type of porphyria that the individual has. There are several types of porphyria that have been given an official "medical term." There are also mutations of this disorder that have yet to be given a name. Each day doctors are learning more about this disorder. It is my belief that many individuals are not being properly diagnosed with porphyria due to the lack of knowledge and because it's symptoms are similar to other, more common diseases.

Below is a list of some of the forms of porphyria.
Acute Intermittent Porphyria
Variegate Porphyria
Hereditary Coproporphyria
Protoporphyria
Porphyria Cutanea Tarda
Congenital Erythropoietic Porphyria
ALA-D Porphyria
Symptoms:
The following are some of symptoms associated with these disorders. Please note this is not a complete list of symptoms and that these are the most common one's noted. An individual may have a couple or several of these syptoms depending on the type of porphyria and the severity of their condition. Some may be cyclic in nature and appear only during severe attacks and some may occur frequently between attacks.
- abdominal and/or back pain
- urine of "some" patients is a purple-red color.
- urine "may" change color once exposed to light
- mood swings
- increased hyperactivity
- nausea and or vomiting
- constipation (at times)
- sensitivity to every-day chemicals
- increase allergies & infections due to decrease immune system
- tachycardia
- hypertension
- pain and or cramping in limbs, neck and/or chest
- muscle weakness
- sensory loss
- fine tremors
- restlessness
- excess sweating
- disorientation or paranoia
- may have skin lessions
- excess hair and fingernail growth.
- anxiety
- insomnia
- depression
- sun sensitivity
- seizures-partial and or Tonic Clonic
- brain Fog ~feeling~
- headaches


Lower Back Pain

Includes Porphyria information and prescribing information.
You have a choice of which document to view. An easy to read PDF document, or the document which has been converted into a webpage that is a mess to read. Please pick the PDF document! But if you don't have Adobe Acrobat Reader (which is free and becoming a web necessity just like your browser), then go ahead and click the HTML link. It will just be harder to sort it out.

PDF Format: http://www.pec.ha.osd.mil/Updates/99%20PDFs/9903.pdf

HTML Formatted mess: http://216.239.39.104/search?q=cache:vKnPG1xhu_0J:www.pec.ha.osd.mil/Updates/99%2520PDFs/9903.pdf+porphyria+back+pain&hl=en&safe=vss&ie=UTF-8


I'm back from the 'almost' dead

I'm alive and kicking. Still recovering and having to take it slow. But getting back on the computer and updating this page regurlarly isn't too taxing on my body. So start checking back in. Updates will be frequent now.

Thanks for all the well-wishes I got during my 3rd major Porphyria attack of 2003. I'm hoping it will be the last and that maybe I can enjoy the holidays this year.

-- Brent


Neurogenic Pelvic Pain

http://www.harcourt-international.com/e-books/pdf/170.pdf

If you have any kind of pain in that region, this is an excellent article and a must read. It's a PDF document, so you will need Adobe Acrobat Reader. But you should have that anyway!

Finding this document was by request. If you need assistance in finding something, please leave a comment or email me directly. I'll do my best to help find the material you are looking for.


Royal deaths and diseases

Tainted blood
The history of monarchy is littered with, strange unexplained illnesses, madness and premature death – all the result of royal inter-marriage. In trying to preserve their blood lines, royal families throughout the world have spread genetic disease.

***

Prince Carlos of Spain (1545-68) was severely damaged at birth (see Royal childbirth). But even if he had been a perfect physical specimen, his life would probably have been blighted by mental illness inherited from one of his great-grandparents.

This inheritance was much more likely because of a series of incestuous marriages carried out by his immediate ancestors. Instead of the usual eight great-grandparents, Carlos has only four and two of those were sisters – Maria of Castile and Juana La Loca (The Mad), countess of Flanders and queen of Castile.

Juana, as her nickname implies, suffered from severe mental illness, as had her grandmother, Isabel of Portugal. It was through Juana that inherited insanity entered the Habsburg dynasty. Following the death of her husband in 1506, Juana and her youngest daughter Catalina were incarcerated in the castle of Tordesillas, first by Juana's father and then by her son, the future Holy Roman emperor Charles V. Carlos was Charles V's grandson.

Even as a child, Carlos was a handful. He didn't begin talking until the age of five, and always remained hard to understand. He was extremely strong-willed, with a wild, unpredictable temper. By nine, he was torturing little girls, servants and animals, at one time maiming a stable of horses so severely that 25 had to be destroyed. He also liked to roast small animals alive – especially hares.

Carlos's education was sporadic and then fizzled out through lack of interest. Following a serious fall, his conduct became increasingly erratic, violent and sadistic. In the royal account books are entries concerning money given to the fathers of girls 'beaten by order of His Highness'.

It was generally believed that the prince would be unable to perform sexually, at least well enough to produce an heir. He underwent a 'cure' carried out by apothecaries and physicians, and boasted that he had successfully passed the final test. The doctors, however, thought that the results were inconclusive.

Carlos seems to have daydreamed about ruling part or all of the realm of his father, Philip II of Spain – over the Netherlands, where a Protestant uprising raged, or the entirety of Spain with the help of his illegitimate uncle Juan. There are suggestions that he may have actually plotted with some of the Dutch leaders or with his uncle. In any event, he told his confessor that he wanted to kill 'a man' and it appeared very much as if that 'man' was Philip, who – perhaps understandably – had become increasingly hostile towards his son.

On 17 January 1568, Philip arrested Carlos and locked him in the tower of Arévalo castle. The prince went on hunger strike; he was force-fed soup. On 9 July, he was found guilty of treason for having plotted the death of his father and conspired to become ruler of the Netherlands. He was sentenced to death, but there was no need to prepare an execution. Carlos was suffering from raging fevers and vomiting, and by 24 July he was dead – from slow poisoning, it was said.

***

While she was queen of France in 1559-60, the future Mary Queen of Scots (1542-87) first showed signs of the recurring illness – abdominal pain, vomiting and bouts of hysteria – from which she would suffer until the end of her life. Some historians now believe that these were symptoms of porphyria, an hereditary condition that would afflict a number of her descendants.

Its effects on Mary's ability to rule were disastrous, and she often appeared close to death. In 1566, her courtiers ordered mourning dress as their queen lay seemingly dead at Jedburgh. However, her surgeon Arnault revived her by tightly bandaging her big toes, legs and arms, pouring wine into her mouth and giving her a 'clyster' (enema).

After her husband Lord Darnley's murder in 1567, Mary had a complete mental and physical breakdown, during which she married the earl of Bothwell. Her army deserted her, Bothwell fled and she was imprisoned in Loch Leven castle, where she miscarried twins.

Later, as a state prisoner of the English, she was prescribed cinnamon water, unicorn's horn and bathing in wine (Queen Elizabeth complained of the cost). She was also allowed to 'take the waters' at Buxton Spa.

***

Prince Carlos of Spain was not the only Spanish heir to be struck down by the streak of Habsburg madness. Perhaps the most 'perfect' example of the terrible results of inbreeding was Carlos II (1661-1700), who became king of Spain at the age of three in 1665 – his father and mother were uncle and niece.

Witnesses at the time described Carlos as a monstrosity. His Habsburg jaw was so big that the two rows of teeth could not meet; therefore, he could not chew. His tongue was so large that he could barely speak. Mentally he wasn't much better off. He was breastfed by wet nurses until the age of five or six, and could hardly walk until he was almost fully grown. But he never developed properly and remained an invalid child and, later, a childish invalid.

Carlos was subject to the regency of his mother Mariana. When he was 14, in 1675, he was presented with a decree continuing her rule because of his own incapacity. He refused to sign the document, but after a two-hour meeting with his mother, he gave in. It was his last act of rebellion.

It was obvious from his birth that the prince would be unable to consummate any marriage he might be part of, but this did not stop the powers-that-be from choosing brides for him. His first was Marie Louise of Orléans, niece of Louis XIV. She struggled to become pregnant for 10 years, to no avail. Eventually she sought comfort in food and died at the age of 27. Within three months, Carlos was a groom again, this time to Maria Ana, the daughter of the Elector Palatine. She went through an exorcism to promote her fertility, but with equally unsuccessful results.

As well as any inherited afflictions, Carlos may have been suffering from acromegaly, a genetic disease that leads to great overgrowth of bone, which would have explained his over-large head. Other conditions from which he suffered – ulcers, diseased bone and teeth, nervous disorders – could have resulted from him contracting syphilis at birth, the result, perhaps, of his father's many trips to the Madrid brothels.

As he entered his 30s, Carlos became increasingly disabled. He was lame, suffered epileptic fits, was bald, had lost most of his teeth and was extremely myopic. At 37, he had a series of fits, which left him deaf. Two years later, he was dead. His death led to the War of the Spanish Succession, which involved another Habsburg unfortunate, Philip V.

***

From an early age, Frederick William I (1688-1740), king of Prussia, was afflicted by migraines and stomach cramps, attacks of which were usually very violent. During attacks in 1734 and 1739/40, the king's doctors recorded that his urine was 'very red' – a sign of the porphyria, that he had inherited from his mother, Sophie Charlotte of Hanover, who was descended from Mary Queen of Scots. Several of his children's medical histories suggest that they, too, suffered from this genetic disorder.

Frederick William suffered his first attack in 1707, at the age of 19, with a sudden increase of temperature, colic, skin rash and fainting fits. He was depressed and had outbreaks of rage. From the age of 39, he became increasingly irascible. He would get into blind rages over the least important things, striking at all within reach, breaking teeth and noses, then sit silently weeping for hours on end. An insomniac, he would spend whole nights wandering aimlessly.

The most frequent victim of his outbursts was his son Fritz, the future Frederick the Great. Whenever they met, Frederick William would seize him by the throat and throw him to the ground, force him to kiss his boots and beg forgiveness. He would then end the encounter by saying: 'If my father had treated me like this, I would have put an end to my life long ago. But you have no courage.'

In 1730, August II of Poland invited Frederick William and Fritz for an immense military extravaganza in Saxony. Amid the jousting, Frederick William seized the 18-year-old Fritz, kicked him, beat him, dragged him along the ground by his hair and sent him off, bleeding and dishevelled, to make an official appearance.

Fritz made plans to flee to Britain, but the scheme failed and he was imprisoned at Küstrin on a charge of desertion. Frederick William would have executed him but for the intercession of the Holy Roman emperor. However, Fritz was forced to watch as his lieutenant, friend and accomplice Hans Hermann von Katte was beheaded. Frederick William also wanted to disinherit Fritz, but in the end, he pardoned and released him. The young man laid low until his father's death in 1740.

***

There was a history of insanity in the family of Ludwig II (1845-86), king of Bavaria. His aunt, Princess Alexandra, thought that she had swallowed a grand piano made of glass, while his younger brother Otto suffered weeping fits and barked like a dog – he was incarcerated in about 1876.



I M P O R T A N T - - - I N F O

I am a friend of Brent's and he asked me to post a message to inform everyone that he is again sick and in the hospital. They intend to keep him this time until they get the infection completely cleared up and he can safely come back home with no worries and maybe a portion of his health back. That may be late this week, it may be next week, it may be next month.

Brent will be taking a break until he is fully recovered. In the meantime his mom and myself will make an effort to update this website a couple of times per week. Please bare with us and Brent is very sorry that there have not been consistent updates in the past few weeks.

If you would like to call Brent he is in his regular hospital, CCU Room #219 with visiting and phone hours from 9-11am and 6-8pm Corpus Christi, Texas time. You can email his mom if you need more information or leave a comment and one of us will contact you.

K E L L Y


A patient study: 14 year old Porphyria patient

Wow !
http://www.mribhatia.com/braintf19/braintf19text.html


1Up Health

Another good summary page.
Make sure you click all the relevant links for more information.
http://www.1uphealth.com/health/porphyria_symptoms.html


Porphyria, Episodic Muscle Weakness

Excellent outline of all the Porphyrias!
You need to print this one out and save it.
http://www.neuro.wustl.edu/neuromuscular/nother/porph.htm#aip


AAFP Photo Quiz

Interesting, and good:
http://www.aafp.org/afp/20021001/photo.html


(AIP) How does the disease present?

SOURCE: Canadian Porphyria Foundation CPF

There may be a family history and this must be a blood relative. Abdominal pain, nausea, vomiting and loss of appetite are the most frequent symptoms and are often accompanied by constipation and a fast pulse rate. The urine may be dark or it may darken on standing, particularly in sunlight. The abdominal pain is difficult to describe and does not fit close by descriptions for other diseases. It is important to avoid any unnecessary surgery in an attempt to diagnose the cause of the pain.

Most patients with AIP never experience anything more severe than the symptoms listed above: let's call this "Stage 1". Beyond stage 1 the events to be described are quite rare.

Stage 2
There will be a change in the level of consciousness and various abnormalities of mental function. Problems may arise with salts and water in the body (technically called "hyponatremia"). The abdominal pain, vomiting, etc. may become worse.

Stage 3
Muscle weakness may develop. Nerves supplying both muscles and sensation do not conduct properly and the disturbances of brain function listed under stage 2 become more severe. Finally, the muscles that govern breathing may become paralyzed and under these circumstances, death is possible unless the patient is supported in a hospital.

Stage 4
This is the stage of recovery. Recovery from stages 1 and 2 (above) will be complete. However, if the peripheral nerves have been affected, i.e. if muscle weakness has developed during the acute attack of AIP, some residual paralysis may remain and this may take from one-half to three years for recovery to be complete. In the occasional patient it may never be complete.

It cannot be overstressed that the events described in stages 2, 3, and 4 are uncommon today and your chance is small of suffering such severe illness once you know you have AIP, are properly treated, and take reasonable precautions.


Causes of Porphyria

Cause of Porphyria: Excess production of heme and porphyrins
Triggers list for Porphyria: The list of triggers mentioned in source as possible causal factors for Porphyria includes:
Barbiturates (subtype of Drug abuse)
Tranquilizers
Birth control pills
Sedatives
Chemicals
Certain foods
Sun exposure
Alcohol
Acute infections
Surgery
Missed meals
Poor diet
Stress


Reviewing the Meaning of Chronic Smoldering Porphyria

Chronic illness is not just one illness, or necessarily a classification of illness. Chronic comes from the word "chronos" which refers to the word "time"

In some of the porphyrias "chronic" has been used by various porphyria specialists to denote an "on-going" smoldering pattern of the disease rather than just being "latent" without any symptoms, or being "acute" when many many symptoms seem to exacerbate.

Chronic illness in general terms is a medical condition that just does not go away. It can last from weeks to months to literally years. Often chronic illness is genetic, such as with the acute porphyrias.

With chronic illness due to acute porphyria, you have to live with the condition for long periods of time, and avoid triggering factors for the entire length of you life.

Such chronic illness may limit what a person can do. A porphyric may not always be sick, but they will never get rid of their condition altogether. A porphyric will learn ways of managing their disease. This means avoiding some lifestyles, foods, environmental treatments and pharmaceuticals including prescriptions drugs.

Some persons will eventually go into remission for a time by avoiding all of their triggers, but most will have just slight lingering symptoms which are a constant reminder of the disease porphyria.

Ruth Whitestone R.N.
Long Term Illness Specialist
More info in the PES Newsletter



Center For Science In The Public Interest

http://www.cspinet.org/


Two views on supplement health claims

The September/October issue of the Journal of Nutrition Education contains two articles on health-related claims that can be made in advertisements for dietary supplements. The first is written by Annette Dickinson, Ph.D., Vice President of Scientific and Regulatory Affairs for the Council for Responsible Nutrition, a trade association that represents the dietary supplement industry. 1 Marion Nestle, Ph.D., M.P.H., Chair of the Department of Nutrition and Food Studies at New York University, wrote the second article. 2

Dickinson simply reviews some major elements of a document issued by the U.S. Federal Trade Commission (FTC) in October 1998 that provides industry with guidelines on advertising dietary supplements. 3 (The FTC is charged with protecting consumers against deceptive advertising for products and services.)

Nestle's article, in contrast, is both opinionated and highly informative. Nestle takes a critical look at these FTC guidelines and compares them to the U.S. Food and Drug Administration's regulations governing health-related claims on supplement labels. She notes that politics rather than science is behind the fact that health-claim policies for both foods and supplements "are converging on the relatively undemanding approach used by the FTC." The growing deregulation of health claims, she fears, signifies "a trend toward the pre-1906 days of rampant quackery" before the first national food and drug law was enacted.

The rules and regulations that govern health-related claims made in the advertising and labeling of dietary supplements will be discussed in future issues of TDS.


Shah Porphyria Article

Here's an article for you on Acute Intermittent Porphyria.
This one by Mubashir A. Shah, MD and others.

Click here for the article


Weiner Wear !

Your little wiener deserves the best, and at wiener wear we know what wieners really want when it comes to clothing.

http://www.wienerwear.biz/


Mullet Haiku

Mullet Haiku
A poetic tribute to the mullet.
http://www.beerchurch.com/mullet_haiku.htm


Drugs Affecting Men's Health and Sexuality


Yes, there is a Porphyria reference!
Click here for the information.

And here's a joke to accompany that rather serious subject:
A young man truly in love with his girlfriend decided to have her name tattooed on his penis. Her name was Wendy, and the tattoo was done while the penis was erect, so when it was not erect all you could see was W Y.

Shortly after the couple was married they were honeymooning in Jamaica the man was in a bathroom in Jamaica, and standing next to him was a Jamaican man who also had a W Y on his penis. The American said to him "Oh is your girl named Wendy too?"
The Jamaican replied, "No, Mr. that says Welcome to Jamaica Have a Nice Day".


Estrogen and Porphyria

Wetterberg L, Olsson MB, Alm-Agvald I. Estrogen treatment caused attacks of porphyria. Lakartidingen ;92:2197-8,2201.

Two female patients with acute intermittent porphyria, who received oestrogen skin pads as supplementary treatment for postmenopausal discomfort, developed severe psychiatric disorders with persistent confusion, aggression and paranoid reactions. Some decades earlier they had reacted with symptoms of acute porphyria following oral contraceptive usage. There is well documented evidence of the advisability of restrictiveness in the use of oestrogens in conjunction with acute porphyria, particularly in cases of patients with a history of hormone-related symptoms of acute porphyria. The putative mechanisms by means of which oestrogens may exert effects on neurotransmitters and peptides are discussed in the article. The authors would be grateful to hear from colleagues abroad who have treated patients with similar symptoms following postmenopausal treatment with oestrogens.


What is P.N.?

So the moron who used the "comments" function below about non-Porphyria topics can find the reference easily, I made it RED ! -- Brent
The peripheral nervous system is the network of nerves that connect the brain and spinal cord to all the other organs. Damage can occur to these nerves that interfere with their ability to function. Not only can it impair conduction to and from the central nervous system, but nerve damage can also lead to many pain syndromes. There are many causes for peripheral neuropathy. The symptoms can come on rapidly or may develop over many years.

There are numerous causes of peripheral neuropathy. They can be localized, affecting one or several nerves, or generalized, affected the entire peripheral nervous system. The most common causes of peripheral neuropathy are diabetes, alcoholism, vitamin deficiency and some inherited disorders. Other forms of generalized peripheral neuropathy can arise from reactions to drugs or chemicals, including emetine, hexobarbital, chlorbutanol, sulfonamides, phenytoin, nitrofurantoin, heavy metals, carbon monoxide, solvents or industrial poisons. Other causes of peripheral neuropathy include malabsorption disorders, autoimmune reactions, decreased thyroid function, acute porphyria and complications of dialysis. Localized peripheral neuropathy can develop as a complication of nerve compression from tumors or bone growths. Sometimes, nerve canals can become narrowed, such as in the carpal tunnel syndrome. Cancer can invade the nerves or cause indirect peripheral neuropathy through other mechanisms. Disease of the spine, including ruptured discs and spinal stenosis, can compress the peripheral nerves as they originate at the level of the spine.

Peripheral neuropathy usually produces symptoms that appear gradually and slowly progress. Most commonly, they produce tingling and numbness that begins in the hands and feet and progress inwards, towards the body. This type of sensory change is often described for its location, stocking and glove sensory changes. Frequently, the symptoms tend to be symmetric. Weakness of the involved muscles may occur. Pain in an extremity may occur, which is frequently worse at night. Loss of sensation may develop in the hands and feet. This can lead to unexpected injuries and burns, because the bodies natural defense systems are not intact. The involved areas of the skin often become dry and increasingly sensitive to touch. Unusual burning sensations in the area of sensory loss can occur. Some forms of peripheral neuropathy may affect the nerves that supply rectal, urinary and sexual function. This can lead to incontinence and the loss of sexual function, particularly in men. Some of these changes can be confused with diseases that affect the spine and spinal cord. Involvement of the sympathetic nervous system can also lead to problems with orthostatic hypotension. Here, the blood vessels cannot contract in response to changes in position and lowered blood pressure may occur.


Medical Brief in Opposition to the Mandatory Contraception Bill S.104

How EPICC Would Threaten Women's Health and Reproductive Freedom
Many in the various women's health and family planning specialties are alarmed about S. 104, the ''Equity in Prescription Insurance and Contraceptive Coverage Act of 2001'' (EPICC) bill being considered in the Senate. Essentially the bill would require all health plans to cover prescription contraceptives (the pill, IUD, Norplant, Depo-Provera, diaphragm, and so-called ''emergency contraception'') as well as outpatient contraceptive procedures, as a ''basic health care right'' for women. In support of this bill, various testimonies were given on Sep. 10, 2001 before Sen. Barbara Mikulski, who chaired the committee for Sen. Kennedy. (1) The most important argument advanced by proponents of the bill is the “unintended pregnancy” argument. Dr. Anita Nelson, representing the American College of Obstetricians and Gynecologists, testified that 50 percent of U.S. pregnancies are unintended, and that 50 percent of these unintended pregnancies are aborted. Thus she urged the Senate to mandate coverage in the belief that prescription contraceptives can solve this problem. A lesser argument centers on alleged ''noncontraceptive health benefits'' of prescription contraceptives. This argument alleges that oral contraceptives are associated with reduced rates of certain diseases and therefore improve women's health apart from their contraceptive effects. (2)

What does ''unintended pregnancy'' mean?
Increasingly researchers question the meaning of ''unintended pregnancy''. Noting a troublesome subjectivity, changeability, and arbitrariness, few researchers agree on the definition of the terms ''unintended'', ''unwanted'', ''mistimed'', and ''unplanned''. The most prominent of these researchers are now calling for more careful study of this confusing question, especially before any practical policy decisions are made. (3) The EPICC bill would be a classic case of imprudent policy if based on this elusive concept. Why would the Senate take precipitous action based on such an ill-defined term, when the experts cannot agree as to what it means? The 1995 report of the Institute of Medicine carries this fuzzy thinking to an extreme level, requiring that the nation adopt a ''new social norm. 'All pregnancies should be intended -- that is, they should be consciously and clearly desired at the time of conception.' '' (4) This is absurd. Most pregnancies throughout recorded history could not meet this standard. Moreover, the imposition of new social norms regarding pregnancy intentionality is contrary to reproductive freedom and gives the medical profession too much social control over the reproductive choices of women. This bill would become an instrument affording physicians an extraordinary and unprecedented level of social control of women. Quietly in development now are far more longer-acting ''contraceptives'' which become very hard to distinguish from sterilization, and which would essentially put the nail in the coffin of reproductive choice. We want to ask the bill's proponents why they are not clamoring for the Senate to mandate full coverage of surgical reversal for post-sterilization regret. Often poor and uninsured patients succumb to vasectomies and tubal ligations based on a subtle form of fiscal coercion, regretting them later but sadly not being able to afford the expensive and noncovered reversal.

And it is not the wealthy or the adequately-served who would find themselves under the control of physicians and planners; primarily, it is poor and underserved women, especially of certain ethnic groups -- those who currently cannot afford prescriptions -- who would be effectively giving up their right to refuse and choose. Once these draconian measures are fully covered in the insured, they would be inserted into public assistance programs and coercively marketed to the poor and uninsured, who still would be without health insurance for the diabetes, nicotine addiction, blood clots, strokes, birth defects, and other disastrous complications which are inevitably associated with these methods. Any cost analyses purporting to have found no increased costs with mandated coverage cannot have included appropriate assumptions about these common complications. Why would anyone call this reproductive health?

The EPICC approach does not work (contraceptives do not reduce unintended pregnancy rates)
It is sad to see this contraceptive ideology consistently resurface. In layman's terms this repeatedly- failed belief system could be expressed thus: there are too many unwanted pregnancies, therefore we need more funding for contraceptives and indoctrination in their use. This indoctrination occurs irrespective of existing cultural, social, familial, moral, and religious norms which in many instances are violated.
In 1981 Susan Roylance, testifying before the United States Senate Committee on Labor and Human Resources showed conclusively that federal expenditures on family planning consistently produced greater, not fewer adolescent (unwanted) pregnancies. Given the extremely high correlation coefficient of 0.882, we could say in a statistically accurate way that expenditures for contraceptives are advisable only if unwanted pregnancy is the goal. (5) Similarly, Jacqueline Kasun offered corroborating testimony showing that these contraceptive programs actually increased the total pregnancy rate among adolescents, and that declining adolescent birthrates were only accomplished by a drastic increase in the abortion rates, rather than through any claim to reduction in unintended pregnancy. (6)

The true effects of contraception (contraceptives increase abortion and unintended pregnancy)
Proponents of contraception had expected modern contraceptives to reduce abortion rates, especially since abortions constitute truly unwanted pregnancies. This hypothesis has been tested repeatedly in demographic analyses. These surveys consistently show that contraceptives increase abortion rates, thereby exacerbating the abortion/unwanted pregnancy syndrome. (7) If we look at U.S. abortion statistics, we will see that while prescription contraceptives have been available since the 1960s, and most eligible women had obtained them by 1975, U.S. abortion rates still continued to climb until 1990. Among African-American women, rates continued to climb until 1997, and African-American pregnancies were aborted 30 to 100 percent more frequently than other U.S. pregnancies. This is a highly selective racial disparity and a clear inequity, all the more disturbing considering that before this trend African-Americans had by far the highest rates of fertility, thus the most to lose, of all U.S. ethnic groups. (8) The same statistics overall show sustained increases in U.S. pregnancy rates until 1990. Declining birthrates were achieved only until 1982, predominantly because of large increases in abortion rates rather than contraceptive efficiency. Regardless of any theoretical efficacy, on the practical level contraceptive technology appears ineffective even for the relatively simple task of overall reduction in pregnancies. The more difficult task of selectively targeting only unwanted pregnancies would therefore seem beyond the grasp of this technology. We are not aware of any Western nation showing reduced abortion ratios in the years following the ''contraceptive revolution'', and would challenge the bill's proponents to provide such proof.

Why contraceptives fail
The failure of contraceptives can be more easily understood if we avoid narrowly focusing only on their pregnancy prevention characteristics, and instead recall their more hidden feature -- obstruction of and interference with reproductive function. This antagonism is most effectively highlighted when contraceptive methods are considered alongside methodologies which harmonize with, rather than antagonize, reproductive health. The evidence that exists suggests that not all pregnancy avoidance methods increase abortion rates -- only contraceptives do. Modern natural family planning (NFP) methods, while associated with unplanned pregnancy rates comparable to those seen with contraceptives, seem to have much lower abortion rates, (9) and preliminary data suggest high NFP satisfaction rates even when unplanned pregnancy occurs. (10) Pregnancies occurring in the contraceptive context seem more likely to be aborted, and this is not likely to be entirely explained by a pre-existing intentionality toward fertility control. (11) Studies evaluating abortion decisions show pregnancies to be accompanied by shock, panic, and even denial; a nearly automatic and non-reflective abortion decision frequently is the result. In the minds of aborting women unwanted pregnancies can be the ''death of self''. Often, the decision is made even before the pregnancy test. (12) This suggests the influence of primitive, atavistic, and possibly preconscious motivations and points to the possibility of neurobiologically patterned or conditioned behavior in response to the pregnancy challenge. While clearly needing adequate study, an inclination toward abortion may be a decisive characteristic of contraception which places a wide gulf between it and noncontraceptive pregnancy- avoidance methods (NFP). Such an enormously important contrast would have to be acknowledged honestly because of its impact on both maternal and child health. Certainly mammalian research shows that maternal behavior is not automatic, but rather must be prepared in advance by certain neurobiological and hormonal factors associated with ''attachment''. (13) If the most important of these factors are associated with sexual intercourse, as is likely, it is conceivable they are impeded by anticonceptive interventions. This would not be the case with NFP, since there is no interference with sexual and reproductive mechanisms.

Noncontraceptive health benefits?
EPICC's proponents point to alleged noncontraceptive health ''benefits'' such as prevention of anemia, ovarian cancer, dysmenorrhea, benign breast disease, and acne. (14) However, by definition contraceptive interventions can have no such ''health benefits''; those who say otherwise violently distort basic concepts of health and illness, reducing them to utterly meaningless concepts. According to this concept we would have to begin to consider disease and even death itself to have ''health benefits''. After all, the deceased never develop cancer or diabetes. For each of the diseases allegedly prevented, contraceptives have introduced a new disease which cancels out the possibility of the ''prevented'' disease, both diseases being at opposite ends of a single spectrum of function. As an analogy, the thyroid gland can secrete too much hormone or too little hormone, both disease states at opposite ends of the spectrum of thyroid function. However, we never consider hyperthyroidism to be a health benefit because it ''prevents'' hypothyroidism. So, where the pill causes normal bleeding to stop there is a new disease, the absence of menstruation (secondary amenorrhea). A woman who cannot menstruate at all cannot menstruate excessively, but this cannot be considered healthy prevention of excessive or painful bleeding and anemia. Otherwise polycythemia (having too much blood) would be considered healthy prevention of anemia (having too little blood). (15) Similarly, the pill causes interruption in the cyclical ovarian function of ovulation, which includes multiplication of ovarian epithelial cells. Cells that cannot replicate normally also cannot replicate cancerously but this is ovarian insufficiency and premature senescence, rather than healthy prevention of ovarian cancer. This is the likely reason that users far more often require hormones in the menopause, (16) and this also explains cases of persistent amenorrhea which often occur after discontinuation of hormonal contraceptives. (17) Oral contraceptives are associated with breast cancer and fibrocystic disease, hardly a situation of benign breast disease ''prevention''. (18) Similarly, when the pill causes horrific and potentially fatal skin diseases such as porphyria, neurofibromatosis, and malignant melanoma, it is obvious skin health has declined. If less acne occurs, it is because the normal function of acne-producing glands is interfered with. (19) The concept of ''benefit'' presented here is well illustrated by another analogy. Numerous studies could prove that removing both kidneys in healthy individuals would reduce the incidence of kidney cancer, but in no way would this intervention be a ''health benefit''. But perhaps a terminally lazy patient who preferred undergoing dialysis rather than undertake the effort to urinate might rationalize that this was beneficial. However, such a patient would be denied this ''therapy'' regardless of his personal preference and lifestyle choice. The noncontraceptive, so-called ''health benefits'' of modern contraceptives represent just this sort of rationalization. When patients become unhappy with normal sexual function and want to disturb reproductive health, the clinical situation demands counseling, not contraceptives. Physicians should encourage patients to understand and accept the meaning and consequences of sexuality, rather than punish the body in order to nullify the human significance of freely-chosen acts, while evading reality.

Conclusion
The preceding information has been a very brief primer, a medical refutation of the arguments of EPICC's proponents, prepared by The Edith Stein Foundation. It is by no means exhaustive, and strong evidence of increased rates of cardiovascular death, blood clots, suicides, accidents, domestic violence, divorce, birth defects, prolonged and incurable infertility, STDs, autoimmune diseases, and some 60 other conditions which afflict women and stem gratuitously and unnecessarily from prescription contraceptives, could have been provided.
EPICC would have disastrous consequences for the health of America's women and children, and would disproportionately afflict African-Americans and the poor. Why would we mandate health coverage for a toxic intervention which impairs the health of women? From a different perspective, if prescription contraceptives constitute basic health care, why has the medical profession and the medical insurance industry traditionally considered them not to be so? If contraceptives constitute legitimate intervention, why is legislation now necessary?

Why can't the insurance industry be persuaded of their legitimacy?
We propose a program of study and investigation as to the real role of contraceptives in women's health, a program which would have to resist an ideological attachment to contraceptives, and embrace an openness to noncontraceptive alternatives. EPICC would impose by finance what has not been successfully implemented by ''choice'' -- a debilitating surrender to the fear of pregnancy which is already the greatest threat to women's reproductive health and freedom, and we think this bill should be roundly rejected. We respectfully suggest to legislators that they demand scientific proof for the unrealistic and impossible claims made by EPICC's proponents.
This was written by Dr. Dominic Pedulla M.D., and they might or might not represent my opinion. Regardless, you should be informed. For more information, visit The Edith Stein Foundation.



Fibromyalgia (with info about Porphyria)

Deals a large bit about muscle pain for Fibro (and Porphyria):
http://author.emedicine.com/PMR/topic47.htm


Wrong DX: Porphyria

Nice Website.
Good Porphyria Information.

http://www.wrongdiagnosis.com/p/porphyria/intro.htm


ALAD deficiency porphyria

What is ALAD deficiency porphyria?
ALAD deficiency porphyria is a very rare type of porphyria. The disorder results from low levels of the enzyme responsible for the second step in heme production. Heme is a vital molecule for all of the body's organs. It is a component of hemoglobin, the molecule that carries oxygen in the blood.

ALAD deficiency porphyria is a subtype of which condition?
Porphyria

How common is ALAD deficiency porphyria?
This disorder is very rare; fewer than 10 cases have ever been reported worldwide.

What are the signs and symptoms of ALAD deficiency porphyria?
Symptoms, which have varied in the few people diagnosed with this disorder, include weak muscle tone (hypotonia) in infancy, acute attacks of abdominal pain and muscle weakness, and muscle pain or numbness. Symptoms can begin in infancy, childhood, or adulthood.

What are the genetic causes of ALAD deficiency porphyria?
Mutations in the ALAD gene cause this type of porphyria. ALAD makes an enzyme called delta-aminolevulinate hydratase, which is critical to the chemical process that leads to heme production. If gene mutations prevent sufficient activity of this enzyme, heme cannot be produced normally. Instead, a byproduct of the process called aminolevulinic acid (ALA) builds up in the body, which can cause the symptoms seen with this form of porphyria. Low levels of delta-aminolevulinate hydratase can also increase the risk of developing lead poisoning.

How do people inherit ALAD deficiency porphyria?
ALAD deficiency porphyria is inherited in an autosomal recessive pattern, which means two copies of the gene must be altered for a person to be affected by the disorder. Most often, the parents of a child with an autosomal recessive disorder are not affected, but are carriers of one copy of the altered gene.

How can doctors determine if a person has ALAD deficiency porphyria?
Doctors diagnose this form of porphyria by measuring the activity of delta-aminolevulinate hydratase in red blood cells. The enzyme's activity is reduced to less than 10 percent of normal in people with this disorder. The condition also increases the levels of ALA in urine and blood. Unlike other acute forms of porphyria, ALAD deficiency porphyria does not cause elevated levels of porphobilinogen (PBG, another byproduct of heme production). A urine test can help diagnose acute hepatic porphyria by measuring the pattern of ALA and PBG levels.

What treatments are available for ALAD deficiency porphyria?
Heme therapy (to replace missing heme in the body) and treatment with intravenous (IV) glucose have helped relieve symptoms in some patients with this disorder. Doctors recommend that people with acute hepatic porphyria avoid certain drugs, including antiseizure medications, some antibiotics, and tranquilizers, which may trigger symptoms.

What other names do people use for ALAD deficiency porphyria?
5-ALA dehydratase-deficient porphyria
5-aminolaevulinic dehydratase deficiency porphyria
ADP
ALA dehydratase porphyria
ALA-D porphyria
ALAD porphyria
ALADP
Amino levulinic acid dehydratase deficiency
Delta-aminolevulinate dehydratase deficiency porphyria
Plumboporphyria
Porphobilinogen synthase deficiency
Porphyria, ALA-D type
Acute hepatic porphyria