Carson's Journey with PH+

    Carson Smith was diagnosed with Pulmonary Hypertension (PH) and Pulmonary Veno-Occlusive Disease (PVOD ) her freshman year of high school and has not let the disease slow her down. She is currently enrolled in a Masters of Public Health degree program.  Upon graduating this will be her second Master’s degree.

   

    Since we last heard from Carson in her “PHA Journey’s” article, she has faced a number of great achievements, difficulties, and eye-opening experiences, all allowing her to grow as an individual.

   

    In 2009, Carson graduated from Washington University with a degree in Anthropology. She worked for a year and used this time to adjust to her limits. During this time she was faced with the stress of having her files transferred from a children’s, to adult hospital, and meeting with new doctors who were not aware of her medical history.  Being a natural born athlete however, physical activity helped her cope. Soon, Carson was alerted that she was no longer considered sick enough to remain on the lung transplant list. The news left her with bittersweet feelings. While there was a breath of relief to know she was healthy enough to be kept off of the list, it also left her with a lack of security. Carson had already experienced the process of being added to the transplant list. With this background she knew that if one day she did need a transplant, she would have to go through the strenuous process of getting back on the list, and waiting for a donor.

  

  In August of 2010, Carson enrolled in Morehead State University in Kentucky where she pursued a Master’s degree in Public Administration.  By December of 2010, she found herself being rushed to an emergency room and later airlifted to a hospital in St. Louis. Doctors had discovered an accumulation of fluid in Carson’s lungs, leaving her sedated for 6 days and in the hospital for a total of ten. Carson had always considered herself lucky because she did not experience a lot of the things other PH patients did. She was never reliant on oxygen, and was always able to take oral medication. Upon being discharged from the hospital, Carson realized once again that while she was not on oxygen, and was able to continue in certain physical activity, she still suffered from a severely dangerous disease. Even with this setback, she graduated in 2012 and enrolled in a Master’s in Public Health program in August of 2012.

   While faced with the complications of living with a chronic disease, Carson has sustained a positive attitude, and has learned to appreciate the little things in life. She has maintained an attitude of resilience, and believes it is a trait that all PH patients should adopt. She states “it is so much better to try and have to quit, than to not try at all. You have to continue to fight, and use your support network.” For doctors, Carson believes you should “allow patients to test things.”

Kullie's Journey with PH+ (as told by her daughter, Lakesha)

“Continue to fight and educate, get involved and make life comfortable.”

Kullie Sanders was diagnosed with Pulmonary Hypertension and Pulmonary Veno-Occlusive Disease (PVOD) in November of 2010. Her journey was one of determination and fight.

    In efforts to cure through surgery what doctors thought to be a pulmonary embolism, Kullie Sanders and her daughter, Lakesha Jones were flown out to California by the University of California Hospital and flight for life. After 10 minutes of surgery, the doctor approached Lakesha, explaining that her mother had something much more serious than a pulmonary embolism. Kullie was now diagnosed with PH and PVOD. The doctor went on to explain that he would be surprised if Kullie survived another month.

    In the months succeeding surgery, Kullie faced a number of challenges. Having been such an active individual who was so full of life, and often worked multiple jobs, it was difficult to adjust to becoming so short of breath so quickly. The disease had essentially stopped her life. Kullie was now on 22 liters of Oxygen and had trouble breathing after taking only two steps. She was now dependent on a wheelchair, and needed transportation to be mobile.

    Although Kullie was faced with these challenges, she did not give up her fight. She had a strong determination, so strong that her doctor stated “I have never seen a patient fight so hard.” With the progression of her disease, it was now an accomplishment to take four steps without becoming short of breath, or to be able to get dressed on her own. One of Kullie’s biggest accomplishments took place at Church one Mother’s Day morning. She stood out of her wheelchair for a prolonged period, singing and clapping her hands along to the music.

    Throughout her fight, Kullie found an extreme amount of strength and support from her family. Although she was facing a life threatening disease, her biggest concern was always her children, and six grandchildren. They kept her going and fighting, she always wanted to make sure that before anything, they were okay.

    On May 21st 2011 Kullie Sanders lost her fight with PH and PVOD. Through her battle, she continued to keep her faith in God, and determination to keep fighting. Her daughter, Lakesha shares, “The most frustrating part of this experience was to find out that there is a PVOD specialist right here in Colorado after being sent to California. Make sure you talk to doctors and find out information. I think we put a lot of trust into doctors, but we need to make sure doctors are giving us the best care. Continue to fight and educate, get involved, and make life comfortable.”

                                          

Improper Diagnosis: The Challenge of PVOD

Improper Diagnosis: The Challenge of PVOD

Pulmonary Veno-Occlusive Disease (PVOD) is a disease of pulmonary venules and small veins, characterized by fibrous thickening and abnormal intimal cellularity, leading to vascular obstruction and pulmonary hypertension (2013, Istanbul, Pulmonary Vascular Research Institute Workshops and Debates). It is an incredibly rare form of pulmonary hypertension. With doctors knowing so little about the disease, it is often improperly diagnosed, or completely undiagnosed. Patients and caregivers share their stories and reflect on their journey.

Mysterious Symptoms—

“I started being seen by numerous doctors who all had different diagnoses. I spent hours sitting through medical tests and waiting for results. I felt like a lab rat always being poked and prodded. My freshman year (of high-school) the doctors’ suspected severe asthma. Today, they, and I know they were mistaken. I actually have Pulmonary Veno-Occlusive Disease (PVOD.” –Carson; St. Louis, Missouri    

 “..At every move, I was still gasping for breath. In late August we all knew I had reached a plateau as far as getting better. I was terribly worried since I could not breathe if I did anything strenuous – just walking and talking were exhausting. Finally in November, after many medical tests, and visits to various specialists, a biopsy and heart catherization needed to be done. Armed with those results they sent me to Boston to see another specialist. By the end of November, we had a diagnosis – Pulmonary Hypertension Veno-Occlusive Disease.” –Marie; Maine

“In November of 2010, my mom and I were flown out by Flight for Life and the University of California hospital for surgery of what doctors thought to be a pulmonary embolism. After 10 minutes of surgery, the doctor said it was something more serious.” –Kullie (as told by daughter, Lakesha; Colorado)

“We had spent the last year of her life (2009-2010) trying to sort out why she did not feel well, why she was so thin, why she didn’t want to eat, and why she complained of not being able to get her breath. We watched her slow down physically, finding new hobbies like reading lots of books instead of riding her bike or playing soccer. She had many appointments with several doctors... wrongly she was given a clean bill of health.” –Aine (as told by family)

Challenges and frustrations…

“Adjusting from having my parents around to take care of me, to taking care of myself during my first year of college was difficult. I struggled to do things for myself, and realized that I needed to take better care of myself. I also struggle with knowing when to tell people of my disease, or who I should tell and who I should not.” –Carson

 “For years, life had been too busy – I had lived on a diet of promises to myself that my time to relax would come when I retired; so I was ready and eager to start my new life. Instead on May 2 I found myself in the hospital emergency room.” – Marie

 “We know now that had the doctors looked further into just that one symptom—shortness of breath with exertion—or had the cardiologist seen her for a follow up; or had they not ignored her low blood states; or had they not labeled her with anxiety; or had the pediatrician followed up where the specialist left off, they would have found pulmonary hypertension...a lung transplant could have saved her life had they not ignored her.” – Aine’s family

Coping…

“Being physically active helped me adjust with stress.” –Carson

“My mom turned to her family, specifically my brother and I; we were her pillars, her strength and support.” –Lakesha

Why do you believe it is important to keep looking for answers?

“I think we put a lot of trust into doctors, but we need to make sure doctors are giving us the best [care]. We need to press doctors to keep working and finding information. It was a nightmare to find out there was a PVOD specialist right here in Colorado after being flown out to California for treatment.” –Lakesha

 Advice for Others…  

“Always keep a positive attitude and appreciate the little things. Cherish being able to do things and be optimistic. Keep trying to do things, it is so much better to try and have to quit, than to not try at all. You have to have an attitude of resilience.” –Carson

“My attitude was good and maybe that’s what helped me cope. I discovered there was life after diagnosis. This acceptance does not mean I liked it, I can dislike something and still accept it.” –Marie