As many of you know, I was born with a genetic disease cystic fibrosis (CF). If you are new here, CF causes extra thick and sticky mucus throughout my body. You wouldn’t know that mucus can cause so many problems but it’s actually found in every major organ of our bodies. Particularly in the lungs, making it harder to breathe, sinuses, causing chronic sinus issues, & digestive systems, making it hard to digest and absorb nutrients.
Because of cystic fibrosis, I have fought for every breath I have ever taken and every pound I have ever gained. While CF can be an awful disease, I have chosen to choose joy through it all and remain positive. CF has tried to take my life many times in my short 26 years. And one time, especially, it almost succeeded. On June 21, 2016 I received an emergency, life-saving double lung transplant. My life was hanging by a single thread but I’m a fighter and I fought with everything I had (and a lot of help from my amazing doctors) to stay alive.
I was invited by the Cystic Fibrosis Foundation to participate in a program called “DC’s Finest.” DC’s Finest honors the area’s best and brightest young professionals. I was selected because I am not only making a positive local impact but because I am committed to supporting the CF Foundation’s mission, a cure for cystic fibrosis. Through this 17-week leadership program I will have the opportunity to attend professional development classes and meet other like-minded individuals.
The Cystic Fibrosis Foundation is part of the reason I am still alive today. As the world’s leader in the search for a cure for CF, virtually every approved cystic fibrosis drug therapy available now was made possible because of the Foundation and its supporters, people like you! The Cystic Fibrosis Foundation will not stop until a cure for ALL with cystic fibrosis is found. And that cure will come sooner rather than later because we are CLOSER THAN EVER.
It has become one of my personal missions to raise awareness about cystic fibrosis and support the CF Foundation in any way possible. I want SO BADLY for CF to stand for Cure Found. I am honored to be selected as a Finest this year. Here are a few ways you can join me in my fight against cystic fibrosis, to be part of finding a cure.
Purchase a ticket to the Breathe Easy Bash, our final DC’s Finest event. A night full of food, drinks, silent auctions and FUN! It will be held on August 22, 2018 at District Winery in Washington, DC. Tickets may be purchased HERE.
Donate to the Cystic Fibrosis foundation through my Finest campaign HERE.
Ask your company to become a corporate sponsor for our Breathe Easy Bash. (Please contact me if your company would like to become a corporate sponsor.)
In 22 months I have had over 7 surgeries, have spent over 200 days in the hospital and have been to over 100 doctors appointments. And hopefully on April 23 I will have my last surgery for a long while. I feel like I am just feet away from the end of a very long tunnel I have been in. I know there will be other challenges ahead, but nothing can compare to the last 22 months.
I went in for a hip aspiration today. Basically, the doctor sticks a very long needle (after numbing of course) into the joint of your hip and pulls out fluid. The fluid is then sent to the lab and tested for infection. The hip on your “average joe” should not have fluid. Because of this fungal infection in my hip, there has been fluid. But today? Today he could barely even get one drop. Even when he injected fluid into my hip to then remove it right away, my hip sucked up all the fluid and he could not get any back. He said this was a really good sign…hips shouldn’t have fluid and mine no longer seems to. Which means the spacer has worked. I have put all of my trust in my doctors and they have continually come up with plans that work.
Not seeing any fluid come from my joint gave me SO much joy today and A LOT of hope. Hope that after my replacement in 9 days (Monday, April 23) I will be able to get to the top of the mountain that I have dreamed of since my transplant.
There are three things that have gotten me to where I am today…my donor, choosing joy, and dreaming.
I want to honor my donor, Samantha, and the gift she left behind for me. I know that these are my lungs now, but I will never forget who they came from. I feel like it is my duty to LIVE to honor her.
I also try to keep a positive mind and choose joy. Of course, there have been many times since my transplant where I’ve felt sad. But I pick myself back up and I just keep swimming. I find things that bring me joy and I do a lot of it…art, hanging out with my friends, shoes, shopping, food.
And lastly I have dreamed a lot…And I’m not talking the dreams you have in your sleep. Day dreams where you picture yourself places, picture yourself accomplishing things. I had a lot of times of uncertainty, but at the end of the day…I never stopped dreaming of what I could accomplish with my new lungs, my new hip and my second lease on life.
Today my doctor said, “I am in awe of you, Jackie.” And I couldn’t help but think, “I am in awe of myself for getting to this point.” This summer I am traveling to Colorado, Alaska & California. This summer I am going to do a lot of living and some of those dreams I’ve had, they are going to come true.
It’s been a while since I’ve posted, and I know many of you have wondered where I went. I post a photo to my Instagram account everyday with more frequent updates. You can see my Instagram feed on the right sidebar under my photo. If you click on the picture, it will redirect you there. I am going to try to figure out another way to post the daily updates to my blog so you are notified. But until then here is another health update.
A lot has happened since my last health update. I wrote it all out and realized it was way too long and boring so I’m going to give the short version. I spent 6 weeks in the hospital (with a 2 day break) from October – December. I had sinus surgery, 2 surgeries on my hip, had pneumonia and was in a medically induced coma for 3 days, got rid of the pneumonia, recovered and left the hospital in time to visit the White House. I’ve been feeling really good the last couple of weeks and have been getting out to see friends and have some fun.
My hip has been the biggest issue. Originally, from MRI scans, my doctors believed I had Avascular Necrosis (AVN or bone death due to lack of blood supply). But we’ve discovered that the bone died because the fungal infection is in the head of my hip bone and has been in the joint fluid surrounding the bone. The infection has likely been there since my transplant or even before, no one knows because I had absolutely no symptoms until March 2017. Then after the washout in March 2017 I had no symptoms until September 2017. During my last hospitalization I had 2 washouts and a large hematoma (accumulation of blood) in my pelvis muscle (SO painful, let me tell you) removed.
Each time they do a surgical washout and put me on an IV antifungal, after stopping the IV antifungal the infection comes back with more force. They need to get this fungus out of my body to prevent it from spreading. And the thing about this fungus is that it is extremely rare. We are talking only 13 reported cases of it in the lungs and a little over 100 reported cases ever. When a doctor hasn’t come across something, they research in medical journals and get information from what other doctors have done. In my situation, there isn’t any of that…so they’ve been working with little to no information. They’re doing an amazing job and they’ve kept the infection confined to my hip.
Yesterday my entire transplant team and orthopedic surgeon met to discuss a plan of action. I was anxiously waiting a call from my infectious disease doctor after the meeting last night. The plan is for me to finish my course of IV antifungal then get another hip aspiration in the beginning of March. (The aspiration only tests the joint fluid. If the fluid is infected that means the fungus has spread from the bone to the joint.) If that comes back clear they are going to put a spacer in. A spacer is a temporary hip replacement that has the IV antifungal stored in it. Slowly, it will release the antifungal directly into my hip. The reasoning behind this is to 1. Get the infected bone out of my body (they cut off the femoral head just like they would in a hip replacement) 2. Make sure the fungus is completely eradicated. I will have that for 4-6 weeks. By the time the spacer is ready to come out it will be April. Then comes the actual hip replacement. I’m seeing my orthopedic surgeon on Monday for more information about the spacer and hip replacement so that’s all the information I have for now.
I’m not gonna lie, on the phone with my infection disease doctor, Dr. K, and after we hung up, I cried. Why? Because I am missing my best friends Bachelorette party, plans to visit my brother in Boston and aunt in Colorado. My life is again being put on hold and plans cancelled. It was hard to hear and very upsetting. But I know in my heart that this is the right plan. I know this fungus needs to get out of my body and I’m not going to risk it spreading (and risk my life if it does). It really sucks but I let out all of my tears last night and I’m ready for what’s to come. I’m going to stay positive and find things to keep me busy. Before I know it, I will be walking with my new hip this spring. I’ll be OK…I have been until now after all.
Have you ever wondered how a lung transplant happens? My transplant surgeon answered some questions about the transplant process from beginning to end. This is a general overview & is not the exact process taken for all transplants. This process may also vary depending on the hospital where the transplant is taking place. He explained this process in an understandable way. Whether you have had a lung transplant or not, I think you will find it interesting.
The transplant pulmonologist and transplant coordinator (who is usually a nurse practitioner or physician assistant) receive a call from the organ procurement organization (an organization that evaluates and procures deceased-donor organs for transplantation) regarding an offer for an organ. They then determine whether or not the donor organs are sufficient for donation. Some organs may be viable, while others may not be. The transplant surgeon then receives a call from the pulmonologist and coordinator with basic information regarding the donor. The surgeon will accept, decline, or ask for additional testing to be done. If they accept, they proceed with the transplant. The procurement surgeon (a transplant surgeon can do procurement and the actual procedure but they never do both for the same patient) drives or flies with two operating room nurses to the other hospital to bring the organ back to the hospital where the surgery will take place. There are four operating room nurses for the recipient and two operating room nurses for the donor. Cardiac perfusion runs the heart/lung machine if it is used during the operation and they help initiate ECMO support post-operatively, if necessary.
The process of taking out old lungs is relatively straight-forward but depends on the recipient’s underlying condition. Cystic fibrosis lungs and sarcoidosis lungs are generally very difficult to remove due to chronic infection and inflammation. The lungs are quite literally fused to the walls of the chest cavity and have to be peeled away. (This was the cause for excess bleeding post transplant in my case.) Conversely, COPD, chronic obstructive pulmonary disease, and PPH, primary pulmonary hypertension, are generally quite easy. They dissect out the pulmonary artery (the artery carrying blood from the right ventricle of the heart to the lungs for oxygenation), the pulmonary veins (the veins that transfer oxygenated blood from the lungs to the heart; there are two or three per side) and the bronchus (any of the major air passages of the lungs that diverge from the windpipe). If they are doing a double lung transplant, this process is done for both sides through a clamshell incision (shaped like a curved ‘W”, and is typically cut just below the breasts). If a single lung transplant is being done then it is through a thoracotomy (incision on the side of the chest towards the back).
My surgeon does most of his single lung transplants and all of his double lung transplants on bypass support. There are many reasons for this – his feeling is that it minimizes blood loss, keeps the heart stable while they’re working, and lets him take both lungs out at the same time during a double lung transplant. The alternative is bilateral sequential lung transplant off of bypass but this means that at some point, the new lung and one of the old lungs is in the body at the same time. This leads to potential contamination of the new lung. Not many people do double lung transplants this way in the modern era.
Time is of the essence, in two senses. One, they try to time their explant procedure to coincide with the arrival of the donor lungs. They don’t want a patient sitting on bypass for hours waiting for an organ. Conversely, they also don’t want the donor lungs to arrive before they are ready to put them in…which leads to the second component. In general, the lungs need to be put in the recipient within 6 hours from the time they were removed from the donor. This is why donor organs are allocated on a regional basis. It would not be possible for a recipient in California to receive donor lungs from Virginia. There are centers that push this number up to 7 or 8 hours but the majority of centers look at 6 hours as the maximal tolerable ischemic time (a restriction in blood supply to tissues, causing a shortage of oxygen and glucose needed to keep tissue alive).
The most critical point in the surgery is the airway anastomosis (connection between the recipients airways and the donor lung airways). Blood vessels bleed so if there is a problem its pretty obvious. The bronchial anastomoses do not – if there is a technical error, they don’t know until sometimes weeks later.New lungs are put in by matching the bronchus to bronchus, pulmonary vein to pulmonary vein and pulmonary artery to pulmonary artery. They are all matched up and sewn end-to-end.
A dry run is when it is decided that the donated organ is not viable. This happens 25-30% of the time in lung transplants and less than 10% of the time in heart transplants.
My surgeon’s longest double lung transplant was 8 hours and shortest single lung transplant was less than 2 hours. This is the amount of time he performs the actual transplant with other medical professionals assuming roles in the operating room before and after.
The advances of modern medicine are quite remarkable. To think that one person can donate an organ to another person is just incredible. Seventy-five years ago, no one would believe heart & lung transplants would be happening. So far in 2017, my hospital has performed twenty-some lung transplants.