Adult in Disbelief: Part 2

This is Part 2 of the story from CommunityMember41a3b6

Discovering my cystic fibrosis

I had been battling chronic pancreatitis for so many years receiving the exact same care. Hospital admissions were a necessity to attempt to keep my pancreas calm. Hospital stays were always the same scenario, a complete pancreas rest and often times undergoing the horrific experience of torture from the ERCP procedure and having stents placed. I still experience PTSD because I woke up in the middle of one and will never forget having about four staff members jumping on top of me to stop me from removing the scope and harming myself. The smell of the oxygen mask sets me off into a panic attack. After about 10 years of commuting to Philadelphia at the wee hours of the night, I was introduced to a new GI specialist that just joined my local Delaware team. He was so amazing, compassionate and smart, a down to earth doctor that explained his new role in the practice. He was a highly skilled and trained GI brought on to perform the highly sensitive endoscopy ERCP procedures.

I honestly found him to be a very thorough and eager to make patients feel at ease and he was gifted at having the best bedside manners of any professional I had ever been in contact with. After the sixth ERCP, he obtained every single piece of my medical history and studied it and began to get a bigger picture. He finally shared with me why he was vesting so much time and resources on my case. He suspected that I didn't just have a cftr mutation gene, but wanted to refer me to a new practice within the hospital campus for cystic fibrosis disease. He was convinced after he first saw the thick mucous and sludge that my pancreas was damaged from CF. He would be by my side every step of the way guiding me through the steps that were necessary for getting established with the cystic fibrosis clinic. I was initially sent to the pediatric hospital to have a sweat test initiated and it was performed twice for definitive results. The nurse practitioner called me while I was by my dad's side in the ICU, he was in recovery from a post triple coronary bypass. She told me that it's very unlikely to be diagnosed with cystic fibrosis in adults but it's possible. Because I had tested positive on the sweat test, I could not be denied. I remember wanting to cry hysterically but could not bother my dad with this information at that moment.

I was scheduled for a consultation with the CF clinic so that I could get a lung function exam, sputum cultures and CT scans. That was in 2018. Fast forward to 2024, I am still shocked. How can such a very distinguishable disease go unnoticed my ENTIRE LIFE. I have had many feelings from: disbelief, anger, anxiety, hope, faith, happiness, discouragement, love, despair and neglect. But above all, I am undeniably grateful for the new doctors who praise me and lift me up every day at every visit, on every phone call and do not dismiss my symptoms just because they are not the every day hallmarks of CF. I'm living proof that every single CF story has a few twists and sometimes has unbelievable surprises along the way.

More complications

I've had several additional hospital admissions because of this unrelenting bacteria that needs special attention and unique treatment plans to keep me moving and safe during an unexpected pandemic. When I first started paying attention to what devastating news was coming from my husband's family in Italy, I felt overwhelmed with fear. Not only because of the news but because of this new disease that I was still learning about. I have great lung function but I also have this Pseudomonas infection that basically lives in my lungs, I thought how can my system protect me from two infectious invaders at the same time. I also had many positive AFB cultures and that was overloading my mind. I will never forget when I was visiting my husband in the hospital after he developed complications from shoulder surgery in March 2020. I was witnessing something odd. Unfortunately I am a frequent flyer around the hospital and began to question why was everyone wearing masks. I was used to the norm at the CF clinic that wear masks all the time. I called my nurse practitioner and asked if I was safe coming on campus and did she know something that she could share with me. She assured me that the hospital was getting a higher rate of flu patients and precautionary safety measures were being implemented. She said there was no harm for me to wear a mask as well if I was going to be visiting much longer. I felt a sense of security wearing a mask just like everyone around me was wearing.

I will never forget the first time I called for a sick visit and the nurse practitioner was asking me so many new questions. I blurted out something like don't tell me that virus is here in Delaware! I didn't believe it. She arranged for me to go to one of the many facilities that were no longer my doctor's office but a makeshift casualty triage station. I waited outside in the freezing cold temperatures coughing and feeling like I was going to faint. That experience was overwhelming, I got swabbed and as I was being directed away from the other people, I immediately began sobbing because I was certain that I was infected and would suffer an excruciating death. Thankfully I didn't test positive but was believed to have a virus and just needed to get through it with Tylenol and lots of fluids. I have been tested 25 times and never tested positive. My husband had covid three times and last Christmas he had the flu and covid together and passed out while I was sleeping. For whatever reason, I jumped out of bed and found him unresponsive lying against the hallway wall. I had to initiate CPR and never considered that I likely was going to be the next one sick. But I would not leave his side for the rest of his recovery and was aware of the risk. I was no longer that panic stricken CFer. I had been vaccinated and kept up to date with boosters and was reassured by my care team that covid was treatable. I began to live life a little easier.

I continue needing pancreas intervention on a regularly scheduled basis for stent exchanges. Unfortunately I do get hit with severe uncontrollable pain and will likely always require the unexpected and overly unpleasant hospital admission for pain control and iv fluids. A few years ago I developed another condition that is incurable and progressive, primary sclerosing cholangitis, because of my CF disease and the damage to my biliary ducts. I experience a very specific undeniable pain that differs from typical pancreatitis and it's directly up under my ribs. I have gone through six very expensive heating pads in the last 20+ years and have a small one packed in my "go bag." There are times when no matter how much pain medication I take there is no comfort like wrapping that extra large five setting heating pad around my midsection to give me some relief.

I am glad that I finally committed myself to relax and take the time to post here to share my personal story and I thank you all for extending this invitation to me. It's been a very long exhausting battle and I praise every single one of you living and taking on the challenges that you have been accustomed to for survival. I have an enormous amount of admiration and use your personal stories to help me realize that I could have things be worse. I am graciously aware that there will always be someone somewhere who is in dire health and some who can no longer finish the race. Thank you for spending time reading my story and allowing me to be a part of an extraordinary group of people.