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Suggest Treatment For Acute Intermittent Porphyria
Question: In September 1986 my wife (born 31 March 1937) was diagnosed to have Acute Intermittent Porphyria. Since then we ar living with this situation as shown in the chronicle attached. In the beginning of 2015 the usual attacks stopped suddenly and didn't return any more except for some red urine being visible. We are wondering now what might have caused this phenomenon. Thank you very much in advance for any answer. Regards, XXXX Via I. Porro 7, San Pietro Val Lemina, Turin, Italy.
Brief Answer:
POSITIVE MEASURES AND SPONTANEOUS REMISSION HELPED!
Detailed Answer:
Dear Sir,
Thanks for sharing the very well maintained medical/treatment history of contraceptive induced acute intermittent porphyria (AIP) of your wife as attached herewith!
After going through all details, I could consider the followings in her case:
1. 90% of AIP are due to a mutation LEADING to deficiency of the enzyme porphobilinogen deaminase
Additional factors must also be present such as hormones, drugs, and dietary changes (PILLS IN PRESENT CASE) that TRIGGER the appearance of symptoms.
2. Most patients are asymptomatic;
Symptoms of AIP may include abdominal pain, constipation, DEPRESSION, and muscle weakness.
3. Attacks of acute intermittent porphyria can be prevented by:
Maintaining good nutrition
Consuming a high-carbohydrate diet
Avoiding alcohol
Avoiding drugs that can cause an attack
Avoiding physical and emotional stress and exhaustion
Avoiding crash diets to lose weight rapidly
4. spontaneous remission may occur without treatment; this could be due to the above measures or just a phenomenal event...not understood clearly...
I think ponts 3 and 4 together helped your wife to have such attack-free time! But still she is having the disease in a silent state as her urine is sometimes red!
Hope you got the answer; please free to ask if anymore questions are in your mind;
Regards!
POSITIVE MEASURES AND SPONTANEOUS REMISSION HELPED!
Detailed Answer:
Dear Sir,
Thanks for sharing the very well maintained medical/treatment history of contraceptive induced acute intermittent porphyria (AIP) of your wife as attached herewith!
After going through all details, I could consider the followings in her case:
1. 90% of AIP are due to a mutation LEADING to deficiency of the enzyme porphobilinogen deaminase
Additional factors must also be present such as hormones, drugs, and dietary changes (PILLS IN PRESENT CASE) that TRIGGER the appearance of symptoms.
2. Most patients are asymptomatic;
Symptoms of AIP may include abdominal pain, constipation, DEPRESSION, and muscle weakness.
3. Attacks of acute intermittent porphyria can be prevented by:
Maintaining good nutrition
Consuming a high-carbohydrate diet
Avoiding alcohol
Avoiding drugs that can cause an attack
Avoiding physical and emotional stress and exhaustion
Avoiding crash diets to lose weight rapidly
4. spontaneous remission may occur without treatment; this could be due to the above measures or just a phenomenal event...not understood clearly...
I think ponts 3 and 4 together helped your wife to have such attack-free time! But still she is having the disease in a silent state as her urine is sometimes red!
Hope you got the answer; please free to ask if anymore questions are in your mind;
Regards!
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Above answer was peer-reviewed by :
Dr. Vaishalee Punj
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