Hello and thankyou for approaching DoctorSpring with your query. I understand your condition and would like to know more information so that I can give you an informed opinion.
You have mentioned that 'you are feeling very sick again'. Please could you specify the symptoms you are having.
1) Are you having breathlessness that has worsened? Do you feel that slight amount of work makes you breathless?
2) Are you having a persistent cough with no sputum?
3)Are you feeling extremely fatigued?
Systemic Sclerosis (Scleroderma) associated with lung involvement has to be staged according to CT Thorax and ECHO findings. To know how your lung is doing presently, I will need current tests (current CT/ECHO) before I can make a prognosis.
Please can you upload the tests if you have them with you? If not when was the last time you performed these tests?
You can reply as a follow up.
Thankyou.
Patient replied :
That is a resounding yes to numbers 1, 2 and 3. My breathlessness is worse and know happens with the slightest exertion. I have a terrible cough which seems to me to be getting slowly worse. I cough all day and through the night. I am easily fatigued and have to "recover" walking from one end of the house to the other. I had an echo last monday and my pulmonologist said it was "unchanged." I have a CT scan scheduled for tomorrow morning. I also had a chest x-ray which came back "unchanged."
Hello, thankyou for your reply. Cough in systemic sclerosis is difficult to manage, but you can try the following cough suppressants which may help, codeine/dextromethorphan/levocloperastine.
I would like to know if the ECHO showed that you had pulmonary hypertension? I would be able to help you more if you could post a few cuts of the chest CT / X-Ray.
It is difficult for me to predict the prognosis without having a look at your investigations.
However your condition does NOT look grim. The fatigability can be due to reasons pertaining to heart and lung alike, but still I do not find it limiting your daily activities. So again I believe the disease is not as bad as you fear.
Kindly upload images of your investigations.
Thankyou.
Patient replied :
Dear Doctor,
I appreciate your opinion on my health. I cannot upload any of my tests because the HMO I belong does not allow me to transfer my medical test results electronically. If you want the actual test images and reports I have to order the physical records and it takes some time to get them. I normally just receive an e-mail and a paper copy in the mail from my doctor summarizing the results.
Fortunately, I never developed pulmonary hypertension and my recent echo confirmed that I still do not have it. But I have "moderate to severe" pulmonary fibrosis with "honeycombing" at the base of my lungs.
It is the CT scan tomorrow that I'm worried about. My last CT, three months ago, indicated that there has been a minimal increase of fibrosis and inflammation in my lungs since my last CT (three months ago).
I will ask my pulmonologist to prescribe the cough suppressant you mentioned. I really need some relief for my cough...and I am positive that my symptoms have slowly gotten worse and my physical limitations have subtly increased since just before Christmas. I had that terrible pneumonia from the beginning of June until the 6th of July when I finally went to the hospital and was admitted for four weeks there and another 2 1/2 weeks in a rehab hospital. My lungs failed and I could not breath on my own. I also went into septic shock and had to be intubated and was unconscious for 19 days. It took me four months to recover but when I stopped all the medicines, oxygen, and breathing treatments, my symptoms slowly began returning...now I feel sick again.
I do find comfort by what you say about the disease probably not being as bad as I fear. How sick I feel today is simply not reflected in my latest test results and it is baffling all of us. This is my last message...thanks for your help.
Victoria
Dear patient,
The description of the CT report given is suggestive of Interstitial lung disease ( Systemic sclerosis might be a cause for the same). However lung involvement in that manner (UIP pattern) is bit rare for systemic sclerosis.
It is very encouraging and heartening that you don't have Pulmonary hypertension. That means that your oxygen level, especially in sleep is not that low.
I strongly recommend doing a thyroid function test also for the increased fatiguability.
You seem to be having more symptoms than signs, which is making life difficult for you. Patients with these symptomatology will definitely benefit from a pulmonary rehabilitation program. A pulmonologist / physio therapist can easily help you.
You seem to have done a remarkable job fighting a septic shock and coming out of a ventilator with such a lungs. The very fact that you could be weaned out of a ventilator also gives me a good relief, as it tells that the lungs are not in a bad condition.
You seem to have a good body with a reserve of steel. Don't give up, and I do believe you can fight this out.
Regards
Dr. Jacob George P
MD, FCCP