How does porphyria die




















The rain set early in to-night, The sullen wind was soon awake, It tore the elm-tops down for spite, And did its worst to vex the lake: I listened with heart fit to break. When glided in Porphyria; straight She shut the cold out and the storm, And kneeled and made the cheerless grate Blaze up, and all the cottage warm;. Which done, she rose, and from her form Withdrew the dripping cloak and shawl, And laid her soiled gloves by, untied Her hat and let the damp hair fall, And, last, she sat down by my side And called me.

When no voice replied, She put my arm about her waist, And made her smooth white shoulder bare, And all her yellow hair displaced,. And, stooping, made my cheek lie there, And spread, o'er all, her yellow hair, Murmuring how she loved me. But passion sometimes would prevail, Nor could to-night's gay feast restrain A sudden thought of one so pale For love of her, and all in vain: So, she was come through wind and rain.

Be sure I looked up at her eyes Happy and proud; at last I knew Porphyria worshipped me; surprise Made my heart swell, and still it grew While I debated what to do. That moment she was mine, mine, fair, Perfectly pure and good: I found A thing to do, and all her hair In one long yellow string I wound Three times her little throat around, And strangled her. No pain felt she; I am quite sure she felt no pain. As a shut bud that holds a bee, I warily oped her lids: again Laughed the blue eyes without a stain.

And I untightened next the tress About her neck; her cheek once more Blushed bright beneath my burning kiss: I propped her head up as before, Only, this time my shoulder bore Her head, which droops upon it still:. The smiling rosy little head, So glad it has its utmost will, That all it scorned at once is fled, And I, its love, am gained instead!

Porphyria's love:. And thus we sit together now, And all night long we have not stirred, And yet God has not said a word! Select any word below to get its definition in the context of the poem.

The words are listed in the order in which they appear in the poem. You might have what's called latent porphyria, and never have symptoms. This is the case for most carriers of the abnormal genes. Porphyria cutanea tarda PCT typically is acquired rather than inherited, although the enzyme deficiency may be inherited. Certain triggers that impact enzyme production — such as too much iron in the body, liver disease, estrogen medication, smoking or excessive alcohol use — can cause symptoms.

In addition to genetic risks, environmental factors may trigger the development of signs and symptoms in porphyria. When exposed to the trigger, your body's demand for heme production increases.

This overwhelms the deficient enzyme, setting in motion a process that causes a buildup of porphyrins. Although there's no way to prevent porphyria, if you have the disease, avoid triggers to help prevent symptoms.

Because porphyria is usually an inherited disorder, your siblings and other family members may want to consider genetic testing to determine if they have the disease, and get genetic counseling if needed. Mayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission. This content does not have an English version. This content does not have an Arabic version. Overview Porphyria por-FEAR-e-uh refers to a group of disorders that result from a buildup of natural chemicals that produce porphyrin in your body.

Request an Appointment at Mayo Clinic. Autosomal dominant inheritance pattern Open pop-up dialog box Close. Autosomal dominant inheritance pattern In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes autosomes. Autosomal recessive inheritance pattern Open pop-up dialog box Close. Autosomal recessive inheritance pattern To have an autosomal recessive disorder, you inherit two mutated genes, one from each parent.

Share on: Facebook Twitter. Show references National Library of Medicine. Genetics Home Reference. Accessed Feb. Learning about porphyria. National Human Genome Research Institute. Overview of porphyrias. That said, other subtleties of the poem have been, and will always remain subject to a gamut of interpretations ranging from deeply religious connotations that emanate from the last line of the poem to the absurd such as erotic sexual strangulation offered by those who see what they want to see or conceived by the publicity seeker for the popularity that can be gained from the bizarre.

How can I be so certain that my take on this great literary work is correct to the extent that nearly two centuries of readers, both expert and layman alike, are wrong as having missed the mark. Well, you be the judge as you follow my logic. First we present the poem itself. The rain set early in tonight, The sullen wind was soon awake, It tore the elm-tops down for spite, And did its worst to vex the lake: I listened with heart fit to break. When glided in Porphyria; straight She shut the cold out and the storm, And kneeled and made the cheerless grate Blaze up, and all the cottage warm; Which done, she rose, and from her form Withdrew the dripping cloak and shawl, And laid her soiled gloves by, untied Her hat and let the damp hair fall, And, last, she sat down by my side And called me.

When no voice replied, She put my arm about her waist, And made her smooth white shoulder bare, And all her yellow hair displaced, And, stooping, made my cheek lie there, And spread, o'er all, her yellow hair, Murmuring how she loved me — she Too weak, for all her heart's endeavor, To set its struggling passion free From pride, and vainer ties dissever, And give herself to me forever.

But passion sometimes would prevail, Nor could tonight's gay feast restrain A sudden thought of one so pale For love of her, and all in vain: So, she was come through wind and rain. Be sure I looked up at her eyes Happy and proud; at last l knew Porphyria worshiped me: surprise Made my heart swell, and still it grew While l debated what to do.

That moment she was mine, mine, fair, Perfectly pure and good: I found A thing to do, and all her hair In one long yellow string l wound Three times her little throat around, And strangled her. No pain felt she; l am quite sure she felt no pain. As a shut bud that holds a bee, l warily oped her lids: again Laughed the blue eyes without a stain.

And l untightened next the tress About her neck; her cheek once more Blushed bright beneath my burning kiss: l propped her head up as before, Only, this time my shoulder bore Her head, which droops upon it still: The smiling rosy little head, So glad it has its utmost will, That all it scorned at once is fled, And l, its love, am gained instead! Porphyria's love: she guessed not how Her darling one wish would be heard.

And thus we sit together now, And all night long we have not stirred, And yet God has not said aword! The first six lines tell of a dark and stormy night, which is helpful to the extent that it sets the stage for a dismal human mood. The next seven lines tell us Porphyria has been to the cottage many times before and is comfortable building up the existing fire within the fireplace.

That is significant because it demonstrates a relationship of some duration. The reason for her forthcoming death first begins to reveal itself within the following three lines,.

And, last, she sat down by my side And called me. When no voice replied, She put my arm about her waist. That the speaker is in a solemn mood is made apparent when Porphyria speaks to him but he says nothing in reply. So she sits by his side, reaches for his arm and places it around her waist.

Speaking is something he cannot or is not willing to do because his mind is preoccupied with what he is about to do. We are next told that he takes sensitive pleasure in bending over to lay and rub his cheek upon her yellow hair,. And, stopping, made my cheek lie there, And spread, o'er all, her yellow hair,.

The mental image is powerful proof of his romantic affection for Porphyria. He is doing more than running his cheek upon her yellow hair; he is literally bathing in her presence.

This scene alone does not portend of madness. The logic behind Porphyria's death first begins to reveal itself within line twenty-two where it is stated:. The speaker is letting the reader know that there is something wrong with Porphyria. She loves the speaker and wants to be sexually involved, "to set [her] heart's struggling passion free," but she is too weak to do so.

That Porphyria's weakness is of some duration is evident from the fact that, notwithstanding her condition, she still sometimes gave herself to the speaker anyway.



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