Thank you for your query at DoctorSpring.com
Unfortunately, Pulmonary fibrosis it's a relentlessly progressive disease, that has a five year morality worse than cancer.
However, does all pulmonary fibrosis fare the same? - no. It depends if it's steroid responsive or not, progressive or not and whether it's progression can be arrested.
From your e mail I understand that she is severely breathless, has severe pulmonary artery hypertension. In that case I presume that the disease might have started 3- 4 years back and progressed gradually.
What would I have done? Well I need history of joint pain, any previous medication in past( recent or remote past), exposure to pets dust or anything in peculiar.
If everything was negative, I would opt for cough suppressant, N acetyl cysteine, steroids,calcium, antacids, and dytor. Deriphylline might help, but not very convincing.
Assurance- no big role.
Continue oxygen. Perfinidone might help. Onbrez has nothing to offer being so expensive. It offers nothing more than duolin.
However overall progress remains grim. Continue bipap and oxygen.
Feel free to discuss further,
Patient replied :
she had TB at her age of 40 and that was not cure properly at that time. Dr. Samir Garde from Mumbai is docot who is attending her. He has not recommended steriod to her.
Please find attached prescription and recommend any additional which will help us to imporve her health.
I would have been more happy to have a ct image than a doctor prescription.
Duration of illness-
Any joint pain
Smoker ( for completion sake, no offence meant)
Present condition - distance she can walk,
Any swelling of legs.
Plus ct images of lung, if you have it.
Pulmonary fibrosis has many causes. So we need to know what's causing it, to arrest it.
I think I would not just keep on giving all medicines available in market, just because you are willing to buy our because your mother is still symptomatic. I will refrain from onbrez, montair plus ( as you haven't given history of asthma), and select one route for inhaled drugs, either nebulization or inhalers.
I also will not be giving 3 drugs- bosentas, assurans, dytor for pul hypertension.
All these said and done without seeing the patient.
I don't see much to add, but do see need of reduction in treatment, increase time on oxygen and bipap.
Patient replied :
Duration of illness- From last three years facing lot issues with leg swelling, short breath and weakness. Any joint pain : Not yet Any pets: No Occupation : Housewife Smoker ( for completion sake, no offence meant): No Present condition - distance she can walk, : Hardly three steps. Any swelling of legs: Yes, earlier was high but now improved lot. Dytor 10mg two times suggested by doctor. Today she is feeling better but lot weakness. Any medicine or suggestion for it. Because of that she cannot able to walk or stand also.
Thank you for your follow up.
Deriphylline, and dytor causes muscle weakness by various mechanisms. Suggest to monitor potassium and sodium. Add calcium, vitamin e, N acetyl cysteine also will help
Duration of oxygen may be increased.
Otherwise treatment remains same.
Dr. Jacob George P
MD IDCCM FCCP