Sunday, October 8, 2017


      Being in medical practice for so many years , I come across the patients that I can not fully understand the diagnosis. So sometimes I send them to Mayo Clinic or Shands Hospital at UF. I am in a way happy when they come back with no additional diagnosis or different treatment. But then I also feel bad that we can not CHANGE the disease. But sometimes I know the problem and I refer patients to other consultants and the patient comes back with some additional tests and no answers. I can understand the frustrations that patients go through. But I can not not tell my frustrations to patients or the family . I have also a problem when I explain the problems ans still the questions are the same. This brings me to the the patient of today.

      I saw this lady , 72 years old for chronic cough . It started many months ago and then the PCP gave her some antibiotics and then the cough medicines and the inhalers that are uses for asthma .It was OK and she still did have the cough and so she came to see me . She looked comfortable and did not have big or severe bouts of the cough when she was in my office . She had good oxygen saturation at rest. She had couple of family members with her. Her lungs had crackles on examination and that suggested to me that she had some fibrosis -scar tissue in the lungs . Whenever there is injury to lungs -from infection or chemicals or some unknown causes -there is chance of the healing leaving patients with scar tissues . The first symptom of the scar tissue  in the lungs is cough . The fibrosis can get worse quickly --6 months -was at one time called vanishing lung syndrome -or it can last many years and does not shorten the life . So it is unpredictable.
    So I explained them my initial impression . We did the CT scan and the breathing test and the oxygen check on walking . The scared  lung does not allow the oxygen transfer quickly when the demand go up and the circulation is rapid . So her CT scan was consistent with fibrosis and the breathing test did show that the diffusion capacity - the process of oxygen transfer was low at 32 % out of 100%. The walking also dropped the oxygen . So now I had to decide if there is any cause for the scars . The fibrosis for which there is NO CAUSE is called idiopathic or IPF , There are 2 new drugs that were approved only in 2015 and the cost is ONLY $50000 per year. They are not approved for the scar tissues that have a cause .So I did the work up . One of the causes of the fibrosis is conditions like scleroderma or rheumatoid arthritis or lupus. Some times in older patients there may be a problem with swallowing and the food or the liquid can go in to lungs and not in food pipe or esophagues . This can lead to chemical injury on repeated basis and that can lead to fibrosis .
       So I did the blood tests for the autoimmune diseases and did the swallow study. The autoimmune blood tests were strongly positive and the swallow study was abnormal , but not that bad . I did the tests that measures the contractions of the food pipe , as that is abnormal in some autoimmune diseases and so that leads to aspiration . This was severely impaired and the report stated 'consistent with connective tissue disorder '-one of the auto immune diseases . So in my mind the circle was complete . She had some auto immune process which impaired the contractions of the food pipe and that lead to aspiration - food and the liquids going in the lungs and causing the injury which healed with scar tissues and that caused the cough. Some of these auto immune diseases also can cause the scaring in the lungs without the aspiration .
    So I explained the situation to the patient and the family . I told them to see the Gasroenterologist and the Rheumatologist . I sent all the reports to both . The patient came back stating to me that Gastroenterologist did the endoscopy and it was normal . The rheumatologist did some tests and told her she was OK . The endoscopy can not diagnose the aspiration and the scars tissues and the strongly positive blood tests and the esophageal motility problem -all were pointing to the auto immune process. I did show them the reports and the explained them the whole things . And I still had the question -so why I am coughing ?

    I can not give an expensive medicine that has not been approved and proved to help scar tissues in this condition . So I my only way was to call them or send them my notes and my conclusions. 

No comments:

Post a Comment