Saturday, May 23, 2020


     In our life we say this not too infrequently. We apply for a job  and the interview goes well and then we have second interview  and then we get rejected  and we are back to square one .Sometimes people date  and things are going well and then there is engagement and then they break up , again back to square one . In medicine we also see this sometimes . After extensive work up we are still at same level of confusion or state as we were before the work up. This brings me to today's patient.
   I had a call from a cardiologist requesting to see one of his patients . She was 71 years old female  and has progressive shortness of breath, He saw her  and the cardiac work up and the heart was 'fine' . She was also seen by a lung specialist for 3 years  and was told that she had some damaged bronchi and may be COPD . She was started on medications  and she was not getting any better . She had 3 CT scans  and several pulmonary functions studies . At one time the cardiologist thought of a condition of Polymyalgia rheumatica, and started her on steroids  and had her see specialist - rheumatologist , Some additional tests were done  and the steroids were continued with weaning schedule for the steroid dose. She had gained weight due to steroids  and she was still short of breath  and also needed some oxygen . She came to see me with her husband .Their frustration was that they did not know the diagnosis  and she was not better and may be worse . She was now on oxygen that she did ot have a year ago .They had all the records from previous pulmonalogist - This is very unusual -most patients come with no records  and then I have to request them . So I reviewed the records .
        I saw the CT scan reports  and the notes  and the lung function data . She had several of them . The Ct scan had mentioned some scars  and also the breathing test had shown that the mechanical function of the lungs - taking breath in and forcing it out - was almost normal -about 80 % -Nothing to suggest ASTHMA or COPD , but the diffusion - how oxygen is transferred from air sacks to blood - was markedly reduced  and also it had gotten worse in last 6 months . No wonder she needed oxygen supplementation. I though of 2 conditions - one where ver tiny bronchi get inflammation and get clogged up called Bronchiolitis Oblitarance  or Pulmonary Fibrosis.
    I did a High Resolution CT scan  and that did show FIBROSIS . So I told her that we could start her on medications which will help . It does not CURE nor does it PREVENT PROGRESSION , BUT IT REDUCES RATE OF PROGRESSION .I alos told her that only way to get exact diagnosis was to do OPEN LUNG BIOPSY.
  They wanted to know the diagnosis . So I sent her to a surgeon and she had biopsy. The biopsy showed that she had fibrosis , but the local pathologist sent the specimen to Mayo clinic for opinion
I had a call from Mayo clinic pathologist as he was not too sure on the diagnosis  and wanted to KNOW THE CLINICAL PICTURE . So I spoke to him in great detail describing him the CT scans  and the Pulmonary Function tests  and also the other problems . After the discussion he concluded that MOST LIKELY SHE HAS FIBROSIS OF UNKNOWN CAUSE  and so from my stand point the treatment would be what we had prescribed  and got on hold due to the open biopsy.
                        SO WE ARE BACK TO SQUARE ONE !!

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