Dear Scleroderma, You Take My Breath Away…
Twenty years young, I was living life and having fun, with not a care in the world with my best friend and the love of my life. It couldn’t get any better than that. Then suddenly, on one chilly day, things I would have never dreamed of to happen would change my life forever. I had noticed a strange numbing/ tingling sensation in my hands… they were blue! I knew they were cold, but I’ve never seen them turn like that before. Was this normal? It was a little surprising, but I brushed it off and didn’t worry about it too much. As days passed, weeks, to months, I kept getting random attacks of this strange blue tint and numbing to my hands. Not only was it the strange color changing, but I was noticing that my hands were becoming weaker and weaker. Once before I could open a pickle jar no problem, it became an impossible task. I knew I needed to be seen by the doctor… there’s only so much of WebMD one can handle before thinking the worst-case scenario!
As weeks go by, symptoms were getting worse and really becoming a problem for me. It felt as if my muscles were a very short stretchy band that I could only stretch so far. I ached, I was fatigued, I kept having the sensation of wanting to pass out at random moments. My arms, shoulders, and fingers hurt so bad I couldn’t even raise my arms high enough to wash, dry or style my hair! The dressing was close to impossible. I couldn’t button any dang buttons on my clothes! My fiancé, Manuel, had to help me with a lot of chores I was once able to do no problem. I finally took my butt into the doctors, ready for them to tell me that I had rheumatoid arthritis (that was my self-diagnosis thanks to WebMD). I told the doctor all my symptoms, so he ordered me to get a blood test. A few weeks went by, I got my test results back. The rheumatoid factor was negative! I felt so defeated, so confused, so lost… The doctor referred me to a local rheumatologist, and that was just the beginning of it all.
I was finally established with my rheumatologist. Lots of blood work done, x-rays, ultrasounds, all kinds of tests to narrow down the cause of my extreme pain and fatigue. In January of 2011, I was then diagnosed with systemic lupus, and scleroderma. I had an autoimmune mixed connective tissue disease. Lupus is an inflammatory disease that affects the joints, skin, kidneys, blood cells, brain, heart, and lungs. The immune system attacks your organs because it sees them as foreign substance or infection. Scleroderma/ CREST is also an immune disorder that attacks the body, where there is an overproduction of collagen causing scar tissue on the organs and blood vessels, and narrowing of the blood vessels: GERD, digestive issues, heart, lung, and kidney problems, and Raynaud’s phenomenon (the bluing of the fingers due to cold and stress) are all part of the package.
Treatment started right away, as I was put on steroids, and immunosuppressant medication to slow my immune system down from doing any more damage to my body. The first medication my rheumy put me on, I had an allergic reaction within one week… very itchy rash spots, so we had to discontinue that one and try another, methotrexate. The steroid and methotrexate were making me feel sicker than anything! My hair was starting to fall out, and I was constantly feeling nauseous and tired all the time. I literally felt like I was dying! I knew that my body wasn’t used to such strong medication and that I would just have to be strong and suffer through it until I may wake up one day feeling better. Then, amid the starting of the treatment, the doctor visits, and pushing through one day at a time…. Surprise!!!! Manuel and I had a baby on the way! With being pregnant, I knew that I wouldn’t be able to be on the medications I was on because it would cause serious birth defects. I wasn’t going to risk it.
So, I talked to my doctor, and he suggested that it would be fine coming off the medication, we would just keep a close eye on it. Most of the time Lupus/ Scleroderma have been known to go into “remission”, where your symptoms kind of go into hibernation mode. Sure enough, by the grace of God, my symptoms were going away and the pain was pretty much gone! Wow, who could have thought to get pregnant would have made me feel better?! It was time to start the OB-GYN visits, the baby shopping, baby showers, and preparing to bring this little one into the world!
In November 2011, about 2 weeks before my due date, it was my soon to be a father in laws birthday dinner at his house. I was getting down on some really tasty, hot salsa when BAM! It hit me, the first big contraction I had ever felt. It took me by surprise, but I wasn’t going to get too excited yet. 5 minutes passed and another contraction rocked me again, then another. This time, I knew it was time to start counting and keeping track! I told everyone, “I think this is it!” The contractions were coming in strong, with fewer and fewer minutes apart. I made it through a couple of hours more, when I decided that the pain was just too painful not to take myself into the hospital. Manuel and my best friend Allison grabbed my overnight and baby bag, and out the door, we went.
It was a ten-minute drive to the hospital that couldn’t have felt any longer. Once we got there, I was admitted. They checked my cervix and told me the one thing I was dreading to hear. “You’re not dilated enough, go home.” What!! With how much pain I was clearly in they were going to send me home?! So, with nothing else to do otherwise, we drove all the way back home. I laid down on my bed trying to fall asleep as best as I could. Every 5 minutes or less I was being woken up with the most painful contractions. I told Manuel, “We’re going in whether they like it or not and I’m not leaving!” In tears and excruciating pain, we loaded up into the car and drove that hundred miles back to the hospital.
It was obvious that I was in that much pain because once I walked through the doors, they admitted me once again and hooked me up to the monitors and started prepping the room for the birth of my beautiful baby daughter! Gosh, I couldn’t wait to hold her in my arms. Eight hours later, I finally gave birth to my little bundle of joy. My son, Heath, was two years old at the time and so excited that he got to be the big brother! My life was complete. I had my fiancé, Manuel, and two beautiful children, Heath and Jada. We moved in with Manuel’s parents, where I would have a ton of help. Everything was going great. I almost forgot about the Lupus and Scleroderma! I kept busy planning my and Manuel’s wedding and taking care of the kids. Until one day, a distant old friend came knocking at my door. Pain. Everywhere. Once again, my body wasn’t liking me very much and I was beginning to become tormented by the pain I once felt before, although this time it was centralized in my wrists and my ankles. The pain was so excruciating that I couldn’t walk on my feet! I literally had to hobble around and even use crutches just to get off the couch to go to the bathroom. I was so fatigued I would fall asleep for hours throughout the day. I did become pregnant again after a year but miscarried due to the horrendous pain that I was enduring at the time. It was time to take myself back to the doctors.
I went through two years of treatment with the local rheumatologist. He was an out of town doctor so he only came once a month, and tried to see all his medical patients in one day. I was seeing my doctor probably for 10 minutes’ max, every 6 months. I was having these horrible symptoms, but I felt like every time I went in to talk to him about it, he just breezed through the appointment giving me another medication to try out to see if it would “help”. I was fed up at not being heard at that point. I felt like I was not getting my needs met and I was not being listened to. I knew something was wrong that just shoving pills down my throat wouldn’t fix.
I made an appointment to see my primary care provider, to see if there was any other option available for me. I wanted to see a different rheumatologist! It was my lucky day and I had wished I had gone in to see her earlier. She referred me to the best rheumatologists in the state… UCSF San Francisco! I was so relieved, now it was just a waiting game to hear that I was accepted. Soon enough, 3 months later I was accepted to be seen by them and made my first appointment. What was so great about going down there was that they just brought a rheumatologist/ scleroderma specialist from the east coast just to open and run the new scleroderma department at their clinics! It was perfect timing.
My new rheumatologist was amazing! He knew exactly what he was talking about, he listened, and made me feel like my health was his number one priority. It was awesome. He got me started on the right treatment plan, and 3 to 6 months my husband and I would go down there for regular checkups. I was doing fine, taking my medications like I was supposed to. Had a pain here and there of course but nothing that a pain pill or hot bath couldn’t take care of to ease some of it. Manuel and I got married, we moved into our own home, I got a job working as an instructor taking care of intellectually disabled adults at a day program, I was taking it one day at a time. Things were starting to become smooth sailing for us as we went on with our life.
January 2016, I became ill. I was experiencing a terrible cough, shortness of breath, extreme fatigue, body sweats and fever, I thought yep, sure enough, I probably have pneumonia! My mother in law was worried about me, so she took me into the local hospital where I was admitted for a week, where they diagnosed me with sepsis (blood poisoning) and pneumonia. They gave me an antibiotic while I was staying in the hospital, and pumping me full of fluids to keep me hydrated. They didn’t give me any kind of Lasix or diuretic because they said that my kidneys were failing and that if they gave me the Lasix it would just damage my kidneys further. S, because of all the excess fluids they gave me, I walked out of the hospital with 25 pounds of extra water weight!! I felt like a balloon ready to pop. I felt like death but still took myself into work the next day. Halfway into the work day, I knew that I had to leave and admit myself back into the hospital. I mean, I really felt like I was going to die! I called my mother in law to come pick me up from work and take me back to the hospital.
The hospital admitted me, and saw sure enough that I needed that water off! After a week of Lasix treatment, the “big wig” doctor came in before I was released from the hospital, and told me that, from the tests they had run from my time of being in there, I never had sepsis or pneumonia, to begin with, and that they think it was something a lot bigger than that. He said that he thought I may have a possible blood clot in my heart. Whoa, okay now. So, we go from pneumonia to blood clot in the heart? I almost lost it. When was there ever going to be an end to hearing the unwelcome news! So, for a week more, I stayed in the hospital where they ran blood clot tests, and believe it or not, there were no clots to be found. Hmmm… so what could it possibly be then? That’s when they decided that they would rather I go down to UCSF and be seen by my doctors down there.
I waved goodbye to my husband, children, and in-laws as they saw me off to the helicopter, where they emergency flew me down to UCSF medical center. Once I arrived at the hospital, they started performing all kinds of heart tests and blood tests right away. From one of the tests, they discovered that I had a lot of pressure in the right side of my heart. They gave me a right sided heart catheter test to measure the pressures in my heart and lungs. That’s when they discovered that I had developed pulmonary arterial hypertension (caused by the scleroderma). (Pulmonary Arterial Hypertension “PAH” is a type of high blood pressure that affects the arteries in the lungs and heart. The tiny arteries in the lungs become narrow or blocked.) My life was crashing down all around me at that point. It had already been 3 weeks of being away from my family, I was already fighting lupus and scleroderma battle, now come to add more problems and grief to the pile?
The doctors were very kind and very understanding to how I must be feeling. Defeated, sad, hopeless. They reassured me that no matter what I’m going through I have a wonderful team and support group standing right behind me to make me be the best that I can. My treatment options were to give me oral medication, or an IV pump that would be attached to me 24/7 feeding me hypertension medication through a tube placed through my chest right to the heart. I cried, I was devastated! If I had to get that pump, that means no more swimming for me, no more life the way that it once was. I was going to strapped and tied down to this, machine?! NO way. I prayed and prayed to God that the pressures in my heart and lungs weren’t so severe that I would need to have that, and I could just go home on the oral medication.
God answered my prayers and I was sent home after a month in the hospital on a handful of new medications, but thankfully, not the pump! I strongly did not want that just because I knew that it would change my lifestyle dramatically. Well, the medications were working, and I was starting to feel like my old self again. I continued working, became supervisor of the day program, and overall felt generally better.
September 2016, my friends and I decided how fun would it be to plan a girl’s weekend getaway? I thought, you know, I deserve it. I work hard, I’ve been through a lot, I need a break! So, in October, we went up to our friend’s cabins up by Mt. Shasta. It was fall, so it was cold up there. Good thing I brought my winter clothes! Well, regardless of how bundled I was, I could sure feel the cold creeping in my bones! And thanks, due to the Raynaud’s, I am always cold all the time as it is, numb blue hands and all. I could already feel myself getting sick. I got a runny nose, cough, and general all overtired feeling. Dang, it! I had a feeling I would be getting sick.
I got through the weekend, came back home and tried to doctor myself up the best that I could. I knew if I didn’t get better than I would end up in the hospital again… I was NOT about to be going back there again! I did everything I could think of to get better; soups, tea, vitamin c overload, rest… nothing was working! It went on a month of trying to doctor myself up and going to work on top of it. I was starting to get scared. All the symptoms that I was starting to experience, were the same symptoms that landed me in the hospital back in January! In the beginning of December, I finally left work and took myself into the hospital. I couldn’t go one more day of feeling so out of breath no matter what I did. Just walking down a hallway was getting me winded.
Around that time, there was horrible pneumonia and flu bug going around. All the beds in the hospital were taken up, they were even sending people home because there was nothing they could do for them! The hospital was a madhouse. Anyway, the triage nurses checked my oxygen saturation and noticed I was hanging around 85%, which is really low. Normal O2 readings are 92% to 100%. Because the hospital was completely full, and the next week was going to be Christmas, they sent me home with an oxygen tank to keep me stabilized. They told me to come back after Christmas unless of course, I felt like I was getting worse than to come back earlier.
I made it through Christmas. The very next day, I took myself right back to the hospital where they admitted me. Now, the hospital beds were still completely full, but they did keep me down in the ER to stay. A couple days later, I was on a helicopter being transported back to UCSF. It felt like Deja Vu. Once I arrived, they got me all settled in, told me that I was to get another heart catheter test done. They wanted to check to see where I was with pulmonary hypertension. They did the test, and the outcome was just what I was dreading to hear. It was worse. A lot worse. They said, “We’re sorry, but if you want to live you need to get the IV pump and breathe with an oxygen tank!” Well, my life depended on it! At that point, I didn’t even care anymore. I was so tired of being so tired and feeling like death, I wanted to do anything to make that feeling go away! They also told me I would need a double lung transplant because my lungs weren’t going to get better and that they were just going to get worse. At least I have my youth on my side! I knew it was going to be a dramatic life changing event, but I wanted to live more than anything! I could deal with it. Once the doctors told me I will die if I don’t, that just made up my mind right then and there.
A couple weeks went by, and I had surgery to get the port put into place in my chest. Once that was put in, I went to spend some time in the ICU where they could monitor me on the new IV medication. Honestly, I was so out of it I barely remember being in there at all! I know my parents came to visit- barely remember them being there, and my husband. He was there for me through the whole thing! After a few more days in ICU, I went back to my room on the other unit. From there, it was 2 weeks of the teaching of how to mix and prepare my medications, and education on lifestyle changes I would need to be making once I got home.
Finally, after a month in a half of being in the hospital, it was time to finally go home. I had to be transported by ambulance, all the way back up to Redding. It was the worst trip I had ever been on in my life! Not only was I strapped to an uncomfortable gurney for 4 hours facing the back of the ambulance, hooked up to their oxygen, bumping all over the place, but I had the worst migraine and nausea I had ever had in my life. I’m talking dry heaving the whole way home. I was a mess. We got into town finally about 10 pm, I was finally home! It felt so good to be home, although I knew that this was just the beginning of a whole new chapter in my life.
Now, 4 months down the road, I’m doing okay. I have my good days, and I have my bad days. A few incidents landed me in the hospital… but I recouped and continue on with my life. I have such a wonderful support system… my doctors, friends, family, even people I don’t even know are so supportive! We are all in this fight together. My mind keeps busy with planning fundraisers to raise money for my lung transplant and taking care of myself and my family. My life consists of taking one day at a time, being thankful every day for each breath that I take. I won’t go down without a fight! I may be breathless, but I am not hopeless. The lung transplant may fix my pulmonary hypertension problem, but I will always live with Scleroderma, and it will never again, try to take my breath away.