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How Is Idiopathic Pulmonary Fibrosis Treated ?

Posted on Fri, 27 Apr 2012

Question: Dear Sir,
My mother (58 yrs) is suffering in Interstitial lung disease since last 3 months. Chest X-ray showed B/L infiltrates, XXXXXXX -ve, RA -ve, Colonoscopy showed rectal+recto sigmoid ulcer, HRCT showed interstitial lung disease. HRCT chest was reviewed to find extensive interstitial thickening with GGO NSIP like picture. Transbronchial biopsy revealed non-specific interstitial fibroisis. ECHO showed border line LVH, diastolic dysfunction, mild TR, IVC engorged, respiratory variation absent, turbulent flow seen at draining site of hepatic vein. Patient was having postural change in BP with reeling of head & tinnitus. She is taking the following medicines:
Tab Pan (40), Tab Thyronorm (25 mcg), Cap Gemcal (500 mg), Cap Alpha D3, Tab Stemetil MD (5), Tab Stugeron (25), Tab Nurvmax (75), Tab Dilzem CD (90), Tab Mucomix (600), tab Azuran (50 mg), Syp Looz, Tab Rivotril (0.5), tab Gemidro (150), Tab Prednisolone (40 mg), tab Vertin (24), Cap Orofer XT, calcirol Granules.
All the doctors are saying there is no medicine for this disease. I am really feeling hopeless. Please help me. If really there is no treatment for the same Please tell me.
Many thanks & regards
Sourajyoti Sardar
Mumbai

Answered by Dr. Dinesh Goyal (27 hours later)

Hello,

Thanks for the query.

From the information you have provided to me, it appears that your mother is suffering from Interstitial lung disease probably Idiopathic pulmonary fibrosis(IPF).

As for as diagnosis is concerned the above description is consistent with it. The main stay of treatment for Idiopathic Pulmonary Fibrosis is Intra venous or Oral steroids 1mg/kg/day for period of six weeks, it may be overlapped with Azothioprim or Cyclophosphamide.

Domicillary oxygen has to be consider if there is desaturation below certain levels. Echo report appears to be acceptable for tinnitus, giddness. I think they are either related to desaturation or has some cardiac origin, it would be better to consult your Cardiologist for that.

I will like to add that only 33% of Idiopathic Pulmonary Fibrosis responds to pharmacological treatment. Responders and Non Responder has to be labelled after 6 wks of therapy.

Hope this answers your query. In case of any doubts, I am available for the follow ups.

Wishing your mother a good health.

Regards.

Above answer was peer-reviewed by : Dr. Ashwin Bhandari

Follow up: Dr. Dinesh Goyal (3 hours later)

Dear Sir,
First of all many thanks for your clarifications & suggestions.

Would you please suggest the followings? It will be very helpful for me.

1. Whether there is any new research therapy or medicine which may help to cure this disease or atleast expand the time span of the patient in India?
2. Which are the top three hospitals in India where one get the best treatment for this disease? (you may not like to take the name of best 3 hospitals. But believe me, I just need it just to get the best treatment for my mother. Please help me.)
3. How can I realise that my mother is responding in pharmacological treatment after 6 wks of therapy?
4. I am now in mumbai & my mother is in Kolkata. Would you please refer one or two name of the Pulmonologist in Mumbai & Kolkata who has the best experience in this field & can help me for the same.

You may not like to give answer some of my questions. But my intension is to save my mother at any cost. I am requesting from the bottom of my heart to help me out by giving the answer. I should be very much thankful for the same.

With best regards
Sourajyoti Sardar

Answered by Dr. Dinesh Goyal (12 hours later)

Hello Sourajyoti,

Thank for your follow up query.

1.Out of many pharmacological agents used for treatment of Idiopathic Pulmonary Fibrosis, Steroids, Azothioprim, Cyclophasphomide are the best.

Other agents like Gamma Interferon, Methotraxate, Colchicines, etc have been used in the past with minimal or no significant advantages.

Keeping response rate of disease to the drugs and the side effect profile of the drug , I think Steroids, Azothioprim, Cyclophasphomide are the best drugs and recommended.

Perfinadene is new drug now a days available in the market for treatment of Idiopathic Pulmonary Fibrosis. This is not a new drug but re-appearance of old drug.

Other non pharmacological treatment are domicillary oxygen therapy and lung transplant.

2. Beside subjective improvement, clinical improvement in pulmonary function test helps in labeling responders or non-responders.

3. There are reviews written by our users about doctors in our listing, those can guide you for a better doctor. You can search for a doctor in your locality. WWW.WWWW.WW
Hope I have answered your query.

Regards.

Above answer was peer-reviewed by : Dr. Jyoti Patil

Follow up: Dr. Dinesh Goyal (2 hours later)

Dear Sir,

Thank you very much for your prompt reply.
May last question is that whether there is any treatment for this disease in alternative medicine (ayurvedic or homeopathy). Is there any thing you have heard on the same?

Please suggest, if any.

Regards
Sourajyoti Sardar

Answered by Dr. Dinesh Goyal (6 hours later)

Hi,

I am not sure about scientific basis of Ayurvedic or Homeopathic approach towards treating such rare disorders like Idiopathic Pulmonary Fibrosis. I am sorry and I should not mislead you.

I am sorry, I mis-spelt the name of the new drug in my earlier reply. It is Pirfenidone.

You may contact specialised Chest Institutes in India like Vallabhai XXXXXXX Chest Institute, New Delhi. Department of Pulmonary Medicine, PGI Chandigarh where they are research oriented towards such kind of diseases.

You may wish to gather more information here, WWW.WWWW.WW
Hope your mother recovers soon.

Thanks again for posting your query on XXXXXXX

Regards.

Note: For further follow up on related General & Family Physician Click here.

Above answer was peer-reviewed by : Dr. Prasad