To Sink or Swim? My Testimony…

Cystic Fibrosis is a condition that make it feel like you are sinking…inside your own body.  Plagued with recurring infections that attack the lungs can feel like sinking underwater with no air.

First, I will say that my testimony is not complete because God is ALWAYS WORKING THROUGH ME.  God will always be changing things in my life and giving me new experiences that I will add to this from time to time. 🙂

First, I will give you an in-depth description of Cystic Fibrosis. It is an inherited ONLY life-threatening disorder that damages the lungs through swelling and infection, and the digestive system with little to no functionality of the pancreas. It causes mucus blocked airways, which are perfect environments for dangerous bugs and superbugs. In Cystic Fibrosis, the mucus is extremely thick and sticky, so it is hard to breathe and causes a lot of coughing. The infections love CF mucus, because it can hide so easily. The GI side of Cystic Fibrosis can be extremely challenging as well. The pancreas ducts are clogged with thick mucus once again, so there is little to no absorption of food and calories. This causes malabsorption and frequent diarrhea (even sometimes with enzymes!) It is also a gateway for CFRD (cystic fibrosis-related diabetes) which is a form of both type one and type two diabetes in the CF pancreas. A lot of people assume since CF is a lung disease, it only damages the lungs. CF effects the whole body, the abdomen, lungs, throat, entire gastrointestinal system, sinuses, delayed development, chronic fatigue syndrome, deformity of nails, nasal polyps requiring surgery, weight loss, salty sweat, reproductive issues, pulmonary hypertension and severe exacerbations (flare ups).

So… that was a lot about CF. Let’s start at the very beginning of my life, shall we? On October 24th, 1998, I entered this world. I was born weighing seven pounds and one ounce. I was very happy and content with my arrival on earth, and I was a baby who always laughed, smiled, and loved people and new things. I was thriving and at a healthy weight, with chubby cheeks and fat rolls, until I turned three months old. I started having uncontrollable stools that became “explosive” and I was going through two diapers every single time. At this point in my life, I dropped weight so drastically that I looked like a different baby. This is also when my skin became salty, and it would burn the lips of anyone who kissed my cheek or head. I would demand to eat every hour of the day, I never felt full, because all of my food went directly through me. Multiple doctors dismissed these three key symptoms of CF as “normal infant growth”. No one in my family had heard of CF, so my parents didn’t think much of it.

Lets fast forward to April of 1999, when I was 6 months old. It was a sunny day with a breeze, and temperatures were in the low 70’s. I was in pants and a jacket, on my swing outside just enjoying the day with my grandma. I was sweating, and became overheated fast. Every time I sweat, even now, I lose salt, and it causes electrolyte imbalances. Since I was not diagnosed, I wasn’t getting any additional salt. On the swing, I started to have a seizure because my electrolytes were off and I was severely malnourished. Shortly, I was rushed to the local hospital, where later that day I was admitted for testing. One of the tests I had done was a sweat test, and it came back positive for Cystic Fibrosis. My parents were surprised, and also mad at the doctors who dismissed my symptoms for 6 months. I was very malnourished, so they did a nasogastric intubation for nutrition. Fast forward a little more time, and the specialists were insisting on doing surgery to placing a gastrostomy tube (G-Tube) into my stomach for a more direct way of nutrition. My pulmonary doctor, who I still have now, stepped in around this point. He argued against a gastrostomy tube in such a small baby. After that, I stayed on nasogastric intubation (NG tube) for a year, only getting a break from it when it was changed every Sunday. I started on my enzymes in applesauce,  I took the small smooth feeling beads every time I had fat or calories. Eventually, my weight went up, and I was strong enough to leave tube-feeding.

When I got a little older, around 6 or 7, I contracted Methicillin-resistant Staphylococcus aureus (MRSA) from an unknown source. This form of Staphylococcus is hard to treat, and can cause a major decline in lung function. This is when I was first introduced to the horrible antibiotic called Ciprofloxacin. Cipro is not recommended for people that age, as it is a high power, joint and growth damaging antibiotic. I went on it anyways, and by the grace of God, I had no serious complications. MRSA cleared up fairly quickly, and it has not been in my lungs since. During my childhood, I had few lung infections and a very high energy level with high lung function numbers. There are over 2,000 mutation variations in CF. My form of Cystic Fibrosis is called a nonsense variation, or nonsense/stop mutation. It is only 10% of the Cystic Fibrosis population. I have R1162X and DDF508. Delta F508 is the basis of CF, the most common mutation to have. R1162X is more rare. My variation is more pancreatic than lung.

Until I was 15, I hadn’t had a hospitalization since diagnosis. My first hospitalization was August of 2014. It was also my first PICC (peripherally inserted central catheter) line, which I was more than nervous about. I was being treated for symptomatic Staphylococcus and Pseudomonas and spent the first three days in the hospital to make sure I had no reactions to the antibiotics. During those 3 days, I was pretty bored, but also three days in the hospital flies by- even though it seems like it wouldn’t. It was quite a new endeavor for me, but I was well-equipped. The rest of treatment was at home, and I was on a strict schedule. I had that PICC line for only two full weeks, and by the end of the second week, I was feeling like a new person.

The following year, August 2015, I went back for the same symptomatic infections, and what CFers would call a “clean out”. Clean outs are going in, being admitted, and working hard to get your numbers up while receiving full power antibiotics to kill the infections. Clean outs are a maintenance thing for us, but we also have a clean out if we are feeling sicker than normal for a long period of time. In 2015, I received Meropenem IV, and  Ceftazidime IV for the first time, and Ceftaz caused some pretty intense nausea at first. I was being given Phenergan which I wasn’t sure if it was really worth it. Phenergan made me spaced out and made my muscles weak, so they switched me to Zofran, which made the nausea somewhat bearable. After being released 3 days later for my home IV’s, I was still feeling crappy. With high-power IV’s, it makes you feel absolutely horrible before feeling better.

It wasn’t until May of 2016 when things started to crash fast. I remember feeling great for my New York City class trip in April 2016, and having lots of energy! I was figure skating lots, and maintaining good grades in high school. Once May hit, it was like a light-switch flipped. I had gone from the happy-perky Jennifer, to someone who slept every day after school, and cut all the extra-cirriculars for sleep. I was falling asleep in classes. I felt like the life had been sucked out of me, and I had no energy for anything. Going out felt like hiking a mountain, and even things like driving felt too tedious. By beginning of June, my lung culture came back with Stenotrophomonas Maltophilia and later appearing Burkholderia Cepacia Multivorans Complex. We got the call on June 4th, and I was admitted for a two and a half week stay on June 6th. The reason I was in there for 2 1/2 weeks was because my antibiotics were hospital given only, due to their strength and possible complications. My PICC was placed just 3 hours after being there, an evening placement which is only done in emergency situations. It was my first time being awake for a PICC line procedure, because they don’t usually do anesthesia in the evenings. It was very nerve wrecking, and the novocaine shots in my upper-inner arm did not feel nice. I did not feel the PICC placement itself, I only felt pressure, because it was numb. After having it placed, I had my first doses of IV Colistin and IV Ciprofloxacin. Two powerful drugs for two superbugs. These superbugs were only able to be treated with these two antibiotics, out of all the antibiotics in the world. Unfortunately, I am very allergic to Cipro, that drug is very hard on my body.

I had no choice. I had to have IV Cipro, and let me just tell you it was BRUTAL. I thought I had totally zapped energy BEFORE, well I was wrong. Cipro had me bed ridden for days, my body was trying to adjust to it so quickly. I woke up in the middle of the night on day 3, and tried to run to the bathroom, but forgot I was plugged into my IV pole. It made me vomit in such forceful ways I hope to never vomit again. The terror of that nausea went beyond any nausea I’ve ever experienced. It was a nausea that makes your muscles and bones ache and tingle. It made my brain so foggy I couldn’t make sense of a lot of things, I felt so lost. After that, I was pre/post medicated for Cipro so I could get the full 14 days of it, which made me feel even worse. It felt like a fight for my life, and I didn’t feel as if I was winning when I was injected with bags of Cipro. IV colistin wasn’t much better, it made my heart and kidneys go hay-wire. They had to stop Colistin for a few days because my Creatinine levels were all over the place. My heart rate dropped to 50 BPM one night, and I had the cardiology team standing in my room, running constant EKG’s, and they were about ready to wheel me to the intensive care cardiology unit (note the fact that it was a night I was by myself). During this hospital stay, I really found out what it meant to fight. I had blood sugar drops that were very scary, and I ended up doing a glucose tolerance test, which came back with my diagnosis of Hypoglycemia. I might as well mention how LONELY two and a half weeks in a hospital room is. I watched, and re-watched, all of the pre-downloaded movies on the hospital TV. I had tried everything on the menu, except the fish dinner. I had met all of the nurses, respiratory therapists, nutritionists, and volunteers on floor five. I felt too sick to walk around, so I watched the sunset against the dull brick walls that my room was facing. I had a few visitors, that I was extremely grateful for, because I felt human again. The smell of the hospital started to become a homey feeling, and I even had a nurse steal a really good Hill-Rom bed out of an empty room and switched mine (mine was not a Hill-Rom remote controlled bed).

Since May 2016, I have cultured Burkholderia Cepacia Multivorans (which I will abbreviate to BCM now) and that has been quite the fight. I got my PICC line out June 30, 2016, I believe. Well, that was short-lived because on July 15, 2016, I was in the pediatric surgical unit waiting on a new PICC line placement because my symptoms had become so severe again. This time, I tried a PICC in my left arm, which I probably won’t do again. It was so painful for 3 days and I felt like I had gotten punched repeatedly. This round of treatment was at home (thankfully). I was on two home safe IV medications a day, and I was grateful this time the superbug changed its mind so that it can be treated with other things besides those 2 horrible ones. I had that PICC line in for about a month, and then I got it pulled because I was finally feeling stable again. I then entered my senior year of high school.

My GI symptoms flared up over the summer of 2016, in the midst of the lung infection chaos, which seemed worse at the time. So, we pushed aside the GI complications until August 2016, when I had to have an endoscopy and colonoscopy. It came back normal, although I couldn’t eat anything but soup. I had severe stomach swelling with varying constipation diarrhea syndrome for a long time. I endured some very uncomfortable testing that later showed nothing in my stomach, but odd gallbladder numbers, although it was deemed not urgent. It wasn’t until January 11th, 2017, when I had a barium swallow test and imaging. Just later that evening, we got a call that I have a extremely rare congenital gastric diverticulum attached to the back of my stomach wall on the outside of my stomach. It was large, and the doctors were unsure if they could remove it safely or not. Just three weeks later, I was in the surgical waiting room waiting on four surgeries to remove my gallbladder (which was white and far gone), my appendix, and the gastric diverticulum. I still have many complications today that are not yet resolved.

This June of 2017, I got my 5th PICC line to once again fight at BCM in my lungs. It was very hard this time, because I had IV bactrim and IV Ceftaz, and with those two together I was first feeling so drained. Note that I attended the Theatre on Ice Nationals for a figure skating competition eight hours away at the same time I was undergoing this round of antibiotics. Remembering and keeping a schedule of antibiotics when traveling is about one of the hardest things I’ve ever done. It was just me remembering medications, and even thinking back on it makes me want to go take a nap. I even forgot half of my heparin, so it had to be emergency ordered into a pharmacy by my teams hotel. Luckily there were very understanding and helpful moms on the team who were helping me get my heparin flushes!

I should mention that in April of 2017, I went from 79% lung function to 91% lung function, out of nowhere. I wasn’t on IV or oral antibiotics. That is what God does, He is a healer. Doctors were shocked, and my PFT came back with little to no evidence of disease. After what 2016 and early 2017 brought to me, I was floored myself. That didn’t stick around as long as I had hoped, but it was a sign that GOD IS ALWAYS THERE!!! He was showing me what is in store for me (Healing!).

On July 9th, 2017, at youth summer camp, I was prayed over and I felt God’s touch on my lungs. I felt a hand on my lungs and it felt really weird as you could imagine it would. A few minutes later, I was breathing in so deeply and I was breathing with ALL of my lungs. I felt the bottom of my lungs breathe, it was so magical. Such a surreal feeling that I was speechless. That was God healing my lungs. Sure enough, a few days later, my doctor called and said the PICC line could come out. So we removed it (at camp, yes, I wasn’t going to wait any longer). Since then, no PICC lines! I take oral antibiotics now for my infection. I may still be fighting this infection, but God is showing and letting me feel what HEALING is! I have felt it many times. The only way I was able to come to that point of feeling the healing, is by trusting in God. I had to give my full Trust and Faith to Him, and let Him lead me. If I can’t surrender and let Him take control, how would He be able to perform these medical miracles in my life? Yes, I still have to take medication and do treatment, but God does things on His own time. I was expecting the total healing to happen the next day after I felt the touch, but that isn’t always so. God is teaching me patience while giving me a powerful testimony to tell. I am being healed slowly, and I am going to reach the finish line very soon. God will heal ALL. A lot of people will be mad at God for giving them sickness. I was put here to inspire others. I am lighting the path for hope for others. I strongly believe I was given CF so I can show that God is good all of the time, even through complicated trials. I have been beaten down through my health, it has gotten very dark sometimes to battle an invisible illness. I won’t sit here and tell you it is all easy and a smooth ride once you pray to be healed. It still takes trials, time, and a LOT of faith. I have been told to stand on faith, it’s the substance of things hoped for but not yet seen, which is so true. But, once you pray to be healed and get a touch from God, things will become easier slowly. I know it. I’m experiencing it right now. You may feel like no one understands what you are going through, but God does. Every. Single. Pain. And. Ache…God understands and feels with you. He gives you powerful armor on the battleground. I am me, and CF is on the battlefield, on the other side. We are in a constant war together, but God has equipped me so that I can win against the battle with CF. My health is still not at a total healing, I said it up there but I’ll say it again. I’m not 100% better. But my fight has gotten less stressful once I found God and gave all of my pain and suffering to Him. It is not your battle to carry, remember, it is God’s, and He will put you in a place of peace and hope.

I hope this testimony gives hope and faith to others who are feeling beat down in their health, and like things won’t get better. Things ALWAYS get better, God will bring you to your knees before bringing you back up to your feet. That’s what He did with me, and when I was on my knees, I found Him. The only way back up to my feet was through Him. And now I am walking forward in faith. I am on a new path, one that has the word “HEALING” engraved in every inch of the sidewalk. After being shown and feeling what real lungs feel like, I won’t ever forget it.

June 12, 2018: Since this was updated, I have had 3 more picc lines and one heck of a journey. My longest hospital stay was this year, and it wasn’t easy.

I will always remain a fighter, and a Follower of God. 

This testimony is NOT finalized, and it will be edited from time to time as God shows me more amazing things in my health journey.

• Jennifer Nelson: October 2nd, 2017