Guest Blog By: Meranda Honaker
Over the last several months my health has continued to decline despite being compliant and diligent with my healthcare routine. In July I developed a fever during a trip to Boston to speak to a biotech company about my journey with cystic fibrosis. I developed a fever and by the end of my visit, I was unable to walk up steps without severe dyspnea. I was so exhausted from feeling sick I would return to my hotel room to lay in the bed for hours to rest. My chest pain and shortness of breath became so severe in the coming days that I could no longer take a deep breath. I checked my SpiroPd home spirometer which displayed a definite decline in my lung function. I immediately contacted my CF clinic to see my CF doctor. Initially, despite feeling bad, I assumed I was feeling poorly and decided not to rush to CF clinic. Sometimes I have a bad day or few bad days health wise and begin to improve on my own. Once I saw my lung function had declined I knew I needed to be seen in CF clinic rather than waiting it out.
My PFT’s had declined substantially from baseline. My FEV1 and FEF25-75 were significantly lower than I had previously seen. Needless to say, this was incredibly alarming. My doctor prescribed IV antibiotics for a CF exacerbation. I continued to check my SpiroPD during therapy. My PFTs slowly began to improve. However, my severe chest pain and shortness of breath did not improve even after completing three full weeks of IV antibiotics. I was then prescribed a short burst of prednisone. Prednisone helped alleviate my chest pain and reduced my shortness of breath. While on prednisone I continued to monitor my home spirometry to track my progress. I was quite pleased to see my lung function finally returned to baseline. Unfortunately, days after finishing my prednisone burst I began to experience hemoptysis. I returned to my CF doctor and was prescribed another burst of prednisone at a higher dose. I responded well to prednisone once again, and my lungs began to feel better. I continued to experience mild hemoptysis during my course of therapy. It became apparent to me my health was not trending in the right direction despite various interventions with medications. There was no point during my three weeks on IV antibiotics or during my first round of prednisone that I felt back to baseline. My home spirometry results reflected this with the exception of a boost in my spirometry while on a higher dose of prednisone. My home spirometry quickly declined as my dosage of prednisone tapered down.
It is now October and I have continued to decline. Even as I type this blog post during my nebulized therapies I do not feel well. My airways remain tight, shortness of breath persists and I feel poorly overall. I am now waiting for my doctor to call me with information to start an off-label inhaled antifungal which I sincerely hope provides me the relief I need from a symptomatic standpoint. I will continue to monitor my home spirometry as it helps me determine trends in my health, a correlation between which meds are helping and which aren’t, and keep me better informed of my lung function. Having access to my home spirometry has proven useful for me in tracking my health. It has also provided my CF doctor valuable data as he continues to provide the best care possible for me. I highly recommend those with CF to consider a home spirometer and work with your CF doctor to find one with user function to share results directly with your CF doctor. This will help you in the management of your CF and potentially catch exacerbations earlier.