4- UNDER heavy attack

Ambulance BusYou need urgent medical reference, if an acute attack is coming, because this means that you already have underestimated the notice of minor symptoms and at any moment the situation can escalate.

This site is not a doctor: it does not and cannot substitute the advice of a medical professional, to whom you have always to refer, but it is important for a carrier to remember how much complex could be the treatment and the convalescence and to ask medical help as soon as possible.

As Merck’s Manual remembers, just as example, “the diagnosis is based on high levels of δ-aminolevulinic acid and porphobilinogen (porphyrin precursors) in the urine during the attacks. The attacks are treated with glucose or, if more serious, with heme infusions. Symptomatic treatment, including analgesia, is managed as necessary. “

Another good example is in the reccomendations of EPnet in Italian language, where remebered that “as soon as an acute attack is suspected, you must immediately contact your doctor. If the episode is severe you must seek immediate hospitalization”.

This allows you to:

  1. Run the acute attack biochemical diagnosis by measurement of the PBG in urine. Immediately begin the specific treatment of the acute attack: for example by administration of intravenous human hemin
  2. Administer medications needed to treat the various symptoms that arise during the attack. These treatments include medications to relieve pain and nausea and sedation. It is also important to maintain adequate caloric intake and this can be achieved with glucose drip.
  3. Human hemin is to overcome the lack of heme in the liver and prevents the body of the request to increase the demand of chemicals (porphyrins and precursors) needed for heme production. Treatment with human hemin is definitely the best care; if it is not available in an acceptable time, can be administered glucose, which has a similar effect although much less effective”.

Detailed infos in English language for healthcare professionals are avaiable:

  1. by European Porphyria Network (EPnet) about acute attack maangement  and pain management
  2. by American Porphyria Foundation (APF) about emergency guidelines for acute porphyria
  3. by Norwegian Porphyria Centre (NAPOS) about safe drugs
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