Also see Neeta's 2006 Conference Story
My health update:
It has been almost a year since we moved to Rochester, MN to be close to Mayo Clinic for the treatment of my idiopathic pulmonary arterial hypertension (IPAH) and its complications. Despite being very happy to be alive and, feeling much better than when I wrote my first story for Our Journeys, my prognosis does not look quite promising unless our researchers come up with a cure.
I was diagnosed with Thrombocytopenia soon after the diagnosis of IPAH due to a platelet count of 48K. Good news being, it had been holding steady for the first 4 years and recently gone up to 70+k. Last year, between the Yale Hematologist and Mayo Clinic Coagulation specialists, Acquired Von Willenbrands with insufficient factor VIII was added to my diagnosis from blood test results. Recently, Hereditary Hemorrhagic Telangiectasia (HHT) has also been added to the list by Dr. Karen Swanson (HHT Specialist) from the Mayo clinic; with a confirmation via a CT scan of the liver showing AVM's. Dr. Robert Frantz, my PAH Specialist at Mayo Clinic, was suspicious of HHT, after Mayo ENT, Dr. Blum found a couple of telangeictasia in the nostril which caused many nose bleeds with a few uncontrollable, gushing epistaxes that stopped only with ER intervention. HHT, fortunately is not as risky in my case, due to telangeictasia found only in my nostril and AVM's only in the liver. Gushing nose bleeds on the other hand have been a pain in the neck with a major bleed that lasted 42 days summer of 2009 making me anemic; PAH meds sustaining the bleeds. However, positive about it has been that, this bleed led the physicians to the discovery of Acquired Von Willenbrands and later, confirmation of HHT.
Once again although, it is good to have a diagnosis, unfortunate to not have much in the way of treatment! Today, with Amicar tablets, a medication that helps clot where it bleeds, bloody nose is a manageable situation; coupled with Avastin, a nasal spray compounded by Mayo Clinic's pharmacy with the hope to completely stop the epistaxis at the end of a month since use. If Avastin works, I will be able to wean off of the Amicar tablets. I am grateful to all the Mayo clinic specialists from HHT, PAH, ENT and Hemophelia center, to have come up with this combination of treatment, overcoming insurance denials for coverage since Avastin, a cancer medication is not yet FDA approved for HHT or the nose bleeds; although, found very effective. Read news story for more information
About 10 days ago, Mayo Clinic has obtained my blood sample to clinically test HHT via genetic testing with the hope of accurately diagnosing if it was inherited or am I the first in the family to have this genetic mutation on the same gene ALK 1 as the PAH mutation. I have been involved with Vanderbilt’s HHT study for the last couple of years, ever since Dr. Robert Frantz, my PAH specialist was suspicious of HHT. As per Dr. William Nichols, Associate Professor of Medicine & Laboratory Medicine; at Mayo Clinic, I am one of the three interesting patients in the nation to be diagnosed with IPAH and Acquired Von Willenbrands.... We hope to do all we can to stop this disease from moving on to the next generation by co-operating with research studies!
“All truths are easy to understand once they are discovered; the point is to discover them.” ~ Galileo Galilei
Pulmonary Hypertension is a simplified name for a complex health problem- continuous high blood pressure in the pulmonary artery in the lungs, resulting in an enlarged heart which can also lose its ability to pump. There is no cure. With proper treatment, half the fortunate patients live five years from diagnosis, with a few managing to live longer. PH, called the ‘other high blood pressure’ has altered my life dramatically.
In April of 2004, I noticed that I became short of breath anytime I walked up an incline or climbed the steps to our second floor townhouse. Over the course of a few weeks, I got worse with daily episodes of dizzy spells, bloody nose, and a few fainting episodes with more breathlessness. I had to struggle to speak in smooth, full sentences. I was unable to climb the stairs to my own home without taking frequent breaks. Soon even common tasks, like bending over to tie my shoes would cause terrible dizzy spells and nausea. Chest pain and pressure became my nightly visitors. To add insult to injury, I began gaining weight in strange, unpredictable ways. Over the course of a single day my comfy, lounge clothes would become very tight and almost unbearable, but miraculously fit me again the next morning! All too quickly, I became almost enfeebled by my illness in spite of looking perfectly healthy and no doctor could understand why.
My symptoms grew so bad; I was hospitalized twice, from being unable to breathe. I had gone through a battery of tests like Lung VQ scan, nuclear stress test, various X-rays, cat scans, echoes, all kinds of blood tests, without a diagnosis. After the second discharge in last week of August, the cardiologist pointed to the pulmonologist for answers and vice versa. I looked at my PCP who gave the discharge order for answers. She said, ‘I guess medicine has no answer to your condition’ with a sad face! I was ready to embrace death but, kept praying not to let anything happen to me until after my daughter’s wedding to not spoil her day. By my daughter’s wedding date in September ’04, I was barely able to move and unable to partake in the festivities. I couldn’t even dance at my own child’s wedding!
My family members, who were awesome and helpful at the wedding, suggested I look to family and friends in the medical field for answers. My husband and I telephoned a few of them and described the symptoms, tests performed with no answers either. Finally, Dr. Mohan Sarabu, a good friend and member of our spiritual community, a cardio-thoracic surgeon said he knew what was ailing me and I needed to have a right heart catheterization to diagnose it. I telephoned my cardiologist who at first disagreed with the idea of an unnecessary invasive procedure and then, having known and worked with Dr. Sarabu, listened to his arguments. Within 10 minutes, she telephoned me back to say ‘Get ready for your RHC on this Wednesday at Westchester Medical Center!’
My cardiologist, Dr. Dina Katz was finally able to diagnose my illness as PH and referred me to NYP at Columbia, where they further identified as Idiopathic Pulmonary Arterial Hypertension, in its final stage. After being thought of “anxiety prone” by all my doctors, it was a relief to finally have a diagnosis. But that relief was short-lived. After working 25 of my 31 years in the US, I went on long-term disability to spend rest of my days wearing an oxygen tube and riding a wheel chair to move more than 100 feet—at the young age of 50!
Prior to getting ill, I was a working woman, wife, and mother of two grown children. During the week, I was a District Sales Manager for Coca-Cola Enterprises Inc. meeting sales goals and managing a staff of 14 in a very high pressure atmosphere. But, work wasn’t everything to me. I also took long walks with our energetic, young Labrador retriever, Sandy, while also being active both in a spiritual organization and my community. I served as an ESL teacher, Lighthouse volunteer reading to the blind, raised funds via walks and runs for juvenile diabetes and breast cancer. I fed the hungry, thru my spiritual organization and at St. John the Divine Church, N.Y.
After leading an active, vibrant lifestyle, I suddenly found myself immobilized! I had to give up Sandy for adoption, heart breaking though it was, I was unable to give her 2 miles per day exercise; drop all my volunteer and community activities, and even stop working.
Two years later, thanks to Dr. Roxana Sulica who has been treating me since December 2004, with new medications Tracleer, and recently FDA approved Ventavis Inhalation treatments, with my family, friends’ support and prayers; I am alive, stable and better than I was at my daughter’s wedding. We moved out of our second floor townhouse to a condominium with 4 floors and elevators. I have learned to manage my life working around whether it is a good or a bad health day. I have a daily schedule of attending Pulmonary-Cardiac Post Rehab program, using ‘Tag’ transportation services (since I am still unable to drive in traffic). I have made a few good friends here. I am able to do some light housework, including cooking that I love and enjoy my life with my dear husband. By God’s Grace, I am one of few PH patients who have gotten off of oxygen after 9 months use and am able to walk more than 100 ft. I enjoy reading, knitting for fun, social life, going out to dinner and the movies with my friends. I have learned to enjoy my relaxed lifestyle after a hectic life of tight deadlines that I thrived on.
During all this roller coaster of emotions, my dear husband has been a rock of steady strength standing besides me, taking me to every doctor’s appointment, changing his work schedule dealing with more stress at work with one goal of seeing me alive and lead a life as close to normal as possible. There is no way I could repay him for all that he has been to me encouraging me to be strong both emotionally and spiritually to fight this disease with a positive attitude.
Although I am unable to do as much volunteer community work as before, I enjoy my PH activities. PHA’s message board is one website I visit daily and religiously. I am actively working on raising funds with ‘Circle of Friends’ project to aid research for a cure. I like supporting Katie Kroner with her advocacy actions in any small way that I can. I am waiting to start a support group in the southern CT area with our first meeting to take off this April. I love educating and spreading awareness of PH to all whom I come across in my daily life.
I count my blessings and live happily one day at a time, making the effort to remember:
‘The universe is change: our life is what our thoughts make it.’ ~Marcus Aurelius Antoninus~
Thanks for everything!
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