The best Christmas gift I ever received.

Like many, I haven't quite wrapped my head around the fact that Christmas is in three days. It feels like every year, the days get shorter, the shopping lists get longer and the Goodwin tribe grows in numbers. This year was no exception with my brother Jeff's engagement. His fiancee, Brittany, has been spending Christmas with our family for a while now, but saying fiancee rather than girlfriend definitely makes it more official. Next year, she and my brother Jeremy's fiancee, Erica, will both be Mrs. Goodwin which is even more official and I am beyond excited. Now, to convince one of my older sibling to give me adorable nieces and nephews to buy (way too many) super cute presents for. Hm...

Speaking of, they say the best presents can't be bought at the store. Which I wholehearted agree. The best Christmas present I ever received wasn't something anyone purchased; it was the gift of life and it all started on December 19, 2011.

Have you ever thought you were going to die?

I don't mean a cliche, passing thought of everyone dies or the times where near-miss accident make your heart race inside your chest. And honestly, I'm not even talking about the looming thoughts of death that come with having an incurable, chronic illness.

I'm talking about experiencing a situation that was so terrifying and so overwhelming that you truly believed it was going to kill you. A situation that ripped through every rational thought and paralyzed every notion you had that you were in control.

I have. One ordinary Monday morning in 2011, I woke up and for the first time in my life, genuinely believed I was dying. I woke up and life as I knew it came to a screeching halt. Every Christmas gift I had procrastinated on buying, the plans I had for that weekend or whatever else seemed important to my 19 year old self fell away and in a matter of hours, the only thing left... the only thing that matter was trying to stay alive.

Four weeks before, I called my pulmonologist to schedule a hospitalization. It was pretty routine for me to ask to be admitted.  I was feeling run down and I knew I needed the extra boost from IV antibiotics. At the time, I didn't have a central line or a port, so I have to go inpatient to get a PICC line placed. We agreed that I would go in on December 2nd and stay for ten days. I had planned to go out of town on December 13th, so this would let me get a tune up, then go home with my PICC to finish antibiotics outpatient through my home nursing company and not miss out on my vacation. This was all pretty normal for me and is no where near what most call a near-death experience. I've been doing these tune ups 2-4 times a year since I was 15 years old. I've had literally thousands of doses of antibiotics pumped into my veins to fight the chronic infections living in my lungs. The admission had a few hiccups, but overall went smoothly and I went home as planned with no problems.

I left on Tuesday, December 13th to spend two nights at a Bed & Breakfast in North Carolina with my mom for a girls' trip. I felt okay for the trip but more tired than usual. I figured my body was still just getting use to being up and active again. By the weekend though, things were getting much worse. This wasn't normal for being post-admission. Normally, the weeks following an admission, I feel bulletproof! Instead, I was extremely fatigued and experiencing severe episodes of lightheadedness. On Sunday, I was Christmas shopping with my little brothers and I became so lightheaded that I keeled over right in front of the cash register with my head between my knees to keep from passing out right then and there. By Sunday night, I was spiking a low grade fever. I knew it wasn't another lung infection, because I was still on a strong IV antibiotics regime, so I began to suspect a bigger problem. I went to bed early with plans to call my doctor first thing in the morning.

The next morning, I woke up feeling sicker than I can ever remember. And I've felt really sick before. I had a fever and was experiencing chills so intense that I was violently shaking, yet my head and body was burning up to touch. I was extremely lightheaded, even laying down. My vision blurred and my ears were ringing. But that's not what scared me... what terrified me more than anything was the throbbing pain in my chest.

I felt like my heart was literally on fire and burning me from the inside out. The excruciating pain increased every time I took a breath.  

I tried, unsuccessfully, to get out of bed. I couldn't move. My body felt like each appendage weighed 400lbs. Instead, I fumbled with my cell phone and called my mom. I couldn't even grasp the phone so I just dialed then hit speaker phone, leaving the phone laying on the bed beside me. I still remember her answering with a cheerful "good morning sweetie." My teeth were chattering so hard from the chills that when I tried to speak, nothing came out. I remember laying there trying to talk and hearing the cheerfulness quickly flee and worry set into her voice as she asked "Jill, what's wrong? Are you okay?" I finally managed to get out a breathless "I need help".

Within seconds, my mom and my then thirteen year old brother, Jordan, were in my bedroom. Jordan carried me downstairs and laid me on my mom's bed. I managed to take some ibuprofen and sip on water while I half consciously told my mom what was going on. It didn't take long for us to agree that I needed to go to the Emergency Room. My mom called my doctor, my brother packed me an overnight back and we were on our way.

I vaguely remember the drive to hospital but the all too familiar turns and scenery felt foreign. I remember seeing the green road side for the Midtown Tunnel and thinking "what road are we on? why is she driving this way?" when in reality, I've passed that sign a hundred times before. It was a struggle to just focus and concentrate on what was going on around me. We got to the ER and I couldn't stand, so she got a wheelchair to take me inside. My mom took me to the desk to check in and almost immediately, the nurses took me into triage. I remember thinking "well that was fast." Apparently, presenting with "extreme chest pain" will do that. I was taken to a bed and what seemed like 300 doctors, nurses and other medical personnel were attending to me, asking dozens of questions. I don't think I answered most of them. By this point, the Motrin had helped with some of the pain but I was lethargic to the point that I struggle to stay coherent and kept dozing off. I just wanted to sleep. The rest of the morning and afternoon, if I was awake for them, are pretty hazy. One of the last things I clearly remember is the doctors and my mom telling me that I was getting a blood transfusion.

I had no idea what was happening. I knew something was very, very wrong but I didn't know what. I couldn't see, I couldn't hear, I couldn't think. I don't think I was crying, I was just numb. I remember laying in that Emergency Room, hearing the commotion around me, terrified to a point that I felt paralyzed. I could hear the fear in the voices of the staff and more importantly, in my mom's voice. I remember thinking to myself over and over again, "this is it, I'm going to die."

Some hours later, I woke up in the Pediatric ICU, hooked up to more wires and tubes that should be considered legal. 

Someone, I couldn't tell you who, explained to me where I was and that I had experienced a very rare and very severe drug reaction called drug-induced hemolytic anemia. They told me I had several blood transfusion and would continue until my labs were normal. Drug-induced immune hemolytic anemia occurs rarely (1 in 1 million population). It is auto-immune reaction characterized by a sudden decrease in hemoglobin (red blood cells) after treatment from the drug in question. 

Yeah, you read that right. It occurs in literally 1 in 1 million individuals. Only a handful of case studies even exist on it and almost all of them are fatal. Every medical professional that was involved with my care that week was in shock that it was even happening, and that I lived through it. Ironically though, since experiencing all of this, I've learned of at least 3 other cystic fibrosis patients that developed this exact drug reaction to this exact medication, so go figure.

The drug reaction caused my antibiotic, Zosyn, to coat my red blood cells with a protein that made them appear foreign to my immune system. Being a good little immune system, my body then began destroying these "foreign bodies." When I arrived at the ER that morning, my hemoglobin level (red blood cell count) was a 4.2. For the record, healthy is 15+. After seeing my labs, the doctors in the ER immediately gave me a blood transfusion to replenish my red blood cells. After a pint of blood, my hemoglobin was down to only a 2.6. This meant that in the time between my first blood draw and the end of the transfusion (approximately 2 hours), my body had destroyed every single one of my native red blood cells and that 2.6 was only the transfused blood.

Red blood cells are responsible for carrying oxygen from your lungs to the rest of your body. The human body cannot function without red blood cells because the body, and more importantly, the brain, cannot function without oxygen. My body was destroying my red blood cells so rapidly that if I gone to the ER just one hour later that morning, I wouldn't be alive to tell this story.

All my symptoms were from the lack of oxygen. The lightheadedness, the fevers, the blurry vision and ringing in my ears, the fact that I literally could not think.... it was all my brain's response to not having oxygen. And the pain in my chest? It was the beginning stages of heart attack. My heart rate was skyrocketing to try and compensate for the lack of oxygen in my blood and was putting my heart muscles into cardiac arrest. Yep, not even twenty years old and I have had a heart attack.

Over the next three days in the ICU, I received 8 blood transfusion to stabilize my hemoglobin counts. My antibiotics was immediately discontinued and identified as a high risk allergy in my records. Which, speaking of.... this whole experience had absolutely nothing to do with my prior medical history or my cystic fibrosis. It could happen to anyone who took the medication, I just happened to be that anyone.

I spent a total of five days in the ICU, but on December 24rd, I was able to go home. 

I could end my story here and say that getting to spend my Christmas at home with my family was the best gift I ever had, but that's not true. The best gift I ever received was 8 pints of blood, from over a dozen selfless, strangers. The blood transfusions I was given were packed red blood cells, meaning the blood bank removed all the other parts of the blood (white blood cells, platelets, etc) and left just RBCs. So to get a full pint of packed RBCs, you need multiple blood donations. 

A lot of people connected to cystic fibrosis are strong advocates for organ donations. Many individuals with CF will eventually be listed for a double lung transplant and will die without it. Not all receive transplants and transplants are not a cure for CF, but that's a whole different blog post. I am definitely a fan of organ transplant and encourage everyone to register at organdonor.gov, but there is a certain sadness that goes with organ donation. In order for someone with CF to get a lung transplant, or anyone in need of an organ, the donor has to have passed away. Yes, some living donors happen (such as kidneys donors), but the vast majority are deceased. And that sucks. It breaks my heart that someone has to die for others to live. 

Blood donation, however, is a much different situation. Any healthy individual can donate blood and in many cases, myself included, blood transfusion save lives just as much as organ donation. Actually, one blood donation can save up to three lives. Blood transfusion are needed in so many situations. Everything from tragic or violent accidents, to chronic disorders, to acute episodes of illness such as my drug reaction. In each though, medical professionals depend on blood banks such as the American Red Cross to provide blood that will save their patients lives. 

I'll be honest.... before I needed a transfusion on December 19th, 2011, blood donation was something that rarely crossed my mind. I had never donated and never even looked into what was involved or why it was so important. Today, I am not physically eligible to donate, but I try to get involved financially, as a volunteer or even just an advocate by sharing information online about upcoming blood drives that my friends are hosting. I think like most charitable causes, it's not something you think about until you're experiencing it first hand. But who's to say that tomorrow, you or your loved ones won't be the ones who need it. Blood drives happen all the time all over the country, so please visit the American Red Cross to learn how you can get involved.

Which is where I'll wrap things up.... I wrote this blog because it shares a part of my medical history that most people don't know about. My friends and family know about the breathing treatments and pills, they've seen me check my blood sugar... but most could never imagine the experience I had with drug dependent hemolytic anemia.   

It was bizarre, unexpected and scary. It's something that even now, four years later, I feel a pit in my stomach thinking about. I was told back in 2011 that if I was ever given the antibiotic again, I would be dead without hours. Additionally, this wasn't a new medication. I had taken it half a dozen times before and that particular December, had been taking it several times a day for over two weeks before developing the reaction. Because it's an immune response, not a true "allergy", it can happen with any medication, at any time. So I'd be lying if I said I don't worry about the future and if it will happen again. I'm just so thankful that this time, it had a happy ending.

Thanks for listening and I hope you all have a very Merry Christmas!