CTEPH is a rare and progressive form of pulmonary hypertension that results in blood clots getting stuck in the lungs, clogging the arteries and causing the formation of scar-like tissue. As a result, the right side of your heart has to work harder. The longer the heart works at that increased level, the more likely that a patient may have heart failure. Oxygen levels in the blood can also drop too low because of the increased time it takes for blood to travel through the lungs. Without treatment, CTEPH can be fatal. Dolores shares her journey of being diagnosed and managing this difficult disease.
What symptoms did you experience that eventually led you to diagnosis?
In 2012, I could not walk across a room without becoming short of breath. I knew something was wrong, so I made an appointment with my healthcare provider, who referred me for more tests. I initially was sent back and forth to cardiologists and pulmonologists who were trying to figure out what I had. Symptoms of CTEPH are often similar to many other cardiopulmonary diseases which can sometimes make diagnosis challenging. It wasn’t until September 2013 that someone could finally tell me what was wrong. The specialist I went to see took one look at me and had me transferred right over to the hospital where they discovered that both lungs were filled with blood clots. I was told by the medical team that they had never seen anyone survive having this many blood clots. I eventually had a right heart catheterization, which led to my CTEPH diagnosis.
How has your CTEPH been treated?
Unfortunately, I was told I was not a candidate for surgery, which is the primary treatment for CTEPH, but luckily my doctor told me my disease was treatable. When I was first returned from the hospital, I had to be on supplemental oxygen, but eventually, I no longer needed it. My disease is controlled by following the treatment protocol that my critical care pulmonologist has prescribed to me. This includes taking my prescription medications and making sure I go to my regularly scheduled doctor appointments.
“I have very good days, and then sometimes I have bad days. On the bad days, I am fatigued and have no energy. These are the days that I just need to rest.”
What else do you do to manage your diagnosis?
Besides following my medication regime, I listen to my body and rest when I need to. I have learned I have limitations and know when I have reached them. I also let my friends and family know when I need to rest and can’t do any more. Sometimes it is hard, but I know I need to pay attention to how I feel. My dog, Gracie, knows when I am having a bad day and won’t leave my side! I also try to make nutritious choices and make sure I have healthy food options that are ready or easy to prepare for the days that I have no energy.
Do you continue to have symptoms?
I do and they can vary day-to-day.
What else would you like people to know about CTEPH?
I feel like more people need to be educated about what CTEPH is. Because it is a rare disease, many people don’t know about it. Even doctors might think you have something else because the symptoms are similar to other health issues. It is important to spread the word about what this disease is and how to get treated for it. I feel like the healthcare specialists that helped me were put into my life to save me so that I can tell my story to help other people.
"I feel like the healthcare specialists that helped me were put into my life to save me so that I can tell my story to help other people." - Dolores
Important Points about CTEPH
- Symptoms of CTEPH are frequently nonspecific and similar to many other diseases such as asthma, chronic obstructive pulmonary disease or heart failure. The most common symptoms include increased shortness of breath with activity and an increased intolerance to exercise.
- Medication management may be recommended by your healthcare provider if you are not a candidate for surgery or if you continue to have pulmonary hypertension after having surgery.
- Because CTEPH is such a rare disease, it is extremely valuable to get a referral to a specialist at an expert CTEPH center to ensure you are getting the most up-to-date treatment options.
Blog last updated: October 25, 2023