OUR JOURNEYS

Patient story

Linda Sullivan

My official journey with pulmonary hypertension began in February of 2005. My daughter had shared her upper respiratory infection with me and I went to my doctor figuring that an antibiotic was in order. The doctor decided to have blood drawn and proceeded to write me a prescription for antibiotics. The next day, I received a call from my doctor who told me that my red blood cells were elevated and wanted to draw more blood just in case the lab made a mistake. I complied with her request and my second blood test also showed elevated red blood cells. She immediately sent me to a hematologist who in turn told me that I have polycythemia vera, which is a thickening of the blood. He sent me for some x-rays and a CAT scan to find out the root cause. The next day, I completed my tests and the hematologist called and told me that I need to see a pulmonologist as soon as possible. I arrived at the pulmonologist the following day and had a pulmonary function test done (by the way, this was not one of my favorite tests). The pulmonologist informed me that he believed that I have an ASD, which is a hole between the upper chambers of my heart, that is shunting left to right, and that I should see a cardiologist. He proceeded to pick up the phone and send me over to the cardiologist’s office. I was told to come back the next day to have a TEE, which is an echo that is done down your esophagus. When I arrived at the cardiologist’s office, they took my oxygen saturation level, which was 77 at the time, and called an ambulance.

What a whirlwind week! My mind was spinning trying to figure out what was going on. At the hospital, I had a left and right heart catheterization and was wheeled into the recovery room with heavy bags on my groin. The doctor came in and stated, “Mrs. Sullivan, you are a very sick woman. I am referring you to Columbia in Manhattan for a heart and double lung transplant, do you have any questions?” I replied, “Not right now, but am I allowed to have a cup of coffee?”

I was officially diagnosed with a large congenital heart defect called Arterial Septal Defect that cannot be repaired, which in turn caused severe pulmonary hypertension. My pulmonary pressure at the time was 137, when a normal pressure should be around 20. I still cannot believe that I walked around for 45 years and no doctor picked it up. I was very fortunate that in one week I had my diagnosis and an extremely great group of doctors.

My real journey began when I was born. I was not an athletic kid so my defect was able to continue un-noticed. In my twenties and thirties I had hormone issues, fertility issues and lost my gallbladder and appendix. I always felt tired but did not know that it was abnormal to feel that way all the time. I really began feeling fatigued about eight years prior to my official diagnosis. I shrugged it off thinking that life was just catching up to me. I was a working mother with two great children and a great husband and I was the family health advocate for all our family members. My dad, who I loved dearly, was battling prostate cancer that spread to the bone and he died in 1997. I was devastated. Men, please do not forget to get your PSA checked. My husband injured his back and my dear sweet mother-in-law battled gallbladder cancer with metastases to the liver and lost her battle in 1999. The gallbladder can be deadly if left untreated. My friend and sister-in-law was diagnosed with stage three colon cancer which reared its ugly head a few years later as stage four. She lost her valiant battle in 2007, at the young age of 49. Colon cancer is one of the few cancers that are preventable; therefore remember to get a colonoscopy. As she would say, “get your butt checked; the one you save may be your own.” My elderly aunt broke her hip and passed away a few years later. I left my job of twenty years because a major company bought us. I did not want to work too far away from my children and I hated the elevators in the World Trade Center anyway. That decision saved my life, as I would have been on the 100th floor on that horrible day of 9/11 when the world changed and many lives were lost. I lost too many friends to count, especially my dear friend and my daughter’s godmother. Memorial after memorial…it was devastating to me. My father-in-law had quadruple by-pass surgery and my husband had back surgery two weeks later. Yes, I was fatigued but I thought it was normal since I was on a continual roller coaster filled with many happy and sad times.

My journey towards the future is definitely different. When I was first diagnosed, I found an adult congenital heart specialist who told me that it is not recommended to repair the hole in my case due to its size and because it essentially works as a pressure relief valve for the pulmonary hypertension. He recommended that I see Dr. Robyn Barst who is a world-renowned pulmonary hypertension specialist at Columbia Presbyterian Hospital in New York. She was a breath of fresh air and gave me the hope and confidence to deal with this disease they call pulmonary hypertension. The funny thing about pulmonary hypertension is the name. The name is simplified and you would never know how deadly it is by the name. Now, if you hear ‘cancer’ you get a different reaction. Dr. Barst retired which was a devastating blow to me since my trust in her was solid. I had total faith in her judgment. I now see Dr. Berman-Rosenwig who took over for Dr. Barst and also gives me confidence and is very good. I am involved in a clinical trial for Cialis, to which I have responded very well so far. I currently take the following medications: Digoxin, Fourosemide, PotassiumCL, Spironolact, TriCor, MagOx 400, Warfarin, Tadalafil (Cialis), Letairis (ambrisentan), Ranitidine (Zantac), Nexium, Lantus SoloStar Insulin, Janumet, Humalog Insulin and oxygen. I decided to treat my pulmonary hypertension orally and use the IV medications as my backup.

So far, I have had a few challenges such as uncontrolled diabetes, nodes on my thyroid, and heart arrhythmias that were cardioverted. The cardioversions were just like rebooting a computer. I have seen an Electrophysiologist to help me with my heart arrhythmias. The biggest challenge that I have found so far is the development of diabetes. It remains uncontrolled and is very frustrating. These challenges will come and go but I consider myself very lucky. Although I have this devastating and incurable disease, I have many benefits.

There are some perks that I benefit from. One would be finally getting to park closer to the stores if there are any handicap spots available. Others are strong neck muscles from my dogs stepping on the oxygen hose and giving me whiplash and knowing the location of every bathroom on Long Island (you know how it is with that Lasix). What I find most frustrating is that I appear relatively healthy on the outside but my insides are a complete mess. It reminds me of that old cliché never judge a book by its cover. Climbing a flight of stairs is my big killer, grocery shopping, laundry and running a vacuum are all mundane tasks that on most days are very difficult. Most people have difficulty understanding something they cannot physically see.

What gets me through each and every day is the wonderful support of my best friend and husband, Stephen, who has been with me every step of the way and who also, by the way, cleans! Also, my two great kids who are experts at nagging, “mom did you take your meds;” “mom put your oxygen on,” “mom, mom, mom!” I love them all. My daughter is in her second year of college to ultimately earn her Master’s Degree and become a nurse practitioner. Helping mom has given her an edge and experience to help in her career. My son is in high school and plans to become a great aviator. He is such a great help around the house and is my master organizer. My mom is also a tower of strength who has passed that on to me. They truly are my inspiration. They inspire me to meet each challenge head on. I am not done driving them crazy just yet. Having my family and good friends in my life makes this journey much easier.

 

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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.