Wednesday, November 26, 2014

A Year to be Thankful PHor

Throughout the year, these blogs are an opportunity to tell you about what PHA is doing with and for you.  As we approach the end of the year, this blog is a little different…

With Thanksgiving a couple of days away and the Winter holidays fast approaching soon after, I’ve spent some time recently reflecting on this year and all that we in the PH community continue to achieve. From celebrating our most successful International PH Conference and Scientific Sessions to date, to launching PHA’s accreditation program for PH Centers, to rapidly increasing PH awareness through our new PHAware program, to the continuing growth of our 245 support group network, to shining a new spotlight on Chronic Thromboembolic PH (CTEPH), and new PH research programs and grants, it is amazing to see what we have achieved together in 2014.

More than these successes though, I’m so grateful to be connected to this amazing community. You give so much to those affected by pulmonary hypertension. No matter how you have been touched by this disease, you go above and beyond to sustain our efforts to fight PH and support the PH community. Today I’m asking you to give back in a different way, through a tax-deductible donation to PHA’s end of year appeal that will support our continuing fight against PH.

Another way I’ll be getting into the spirit of the holiday season this year is by participating in #GivingTuesday, a global day dedicating to giving back to the causes that mean the most to you. I invite you to join us by making a donation to PHA’s end of year appeal on Tuesday, December 2 and continuing to spread hope for this community.

Now is a great time to give to PHA, because your donation will be doubled! Thanks to a matching gift from Actelion Pharmaceuticals, the first $25,000 in donations will be matched dollar for dollar. Make a gift today to double your impact.

If you have not already, please take a look at PHA’s 2014 Annual Report to see some of the ways this community put Hope in Motion over the last 12 months and our plans for the next 12. It is your support that makes all of this possible.

As I’ve said before, and I’m sure to say again, this was an extraordinary year for the PH community. Though there is still much to be done, we can be so proud of all that we accomplished this year, together.

Please make a tax deductible gift today, or mark your calendar for #GivingTuesday and support the programs that are sustaining hope and changing lives every day.

With hope for 2015,

Wednesday, November 12, 2014

Busting Clots and Myths: Why Early Diagnosis Matters

Early and accurate diagnosis is the key to improving patient treatment outcomes and quality of life. To save lives, doctors must be able to recognize symptoms and understand the correct process of diagnosis. PHA’s Early Diagnosis Campaign: Sometimes it’s PH is actively working to break down the barriers to accurate diagnosis through physician and patient education and awareness. By decreasing the time to diagnosis, we save lives. 

The following guest post by Dr. Manreet Kanwar tells the story of Angel, a chronic thromboembolic pulmonary hypertension (CTEPH) patient who went through the complexity of the health system to finally reach her diagnosis months after symptom onset. Her story shows the struggles patients must go through to get answers and proper treatment. In honor of PH Awareness Month and CTEPH Awareness Day on Nov. 18, please read and share Angel’s story for Better Understanding, Screening & Treatment (BUST) of CTEPH. Also, tune into the CTEPH Q&A webinar with CTEPH specialists Dr. Bill Auger, Dr. Gustavo Heresi-Davila and Crystal Weber, RN, on CTEPH Awareness Day (Nov. 18) at 5:00 p.m. ET. Register now for this webinar.

Until she is ready to share this story in her own words, let’s call her Angel…

I recently met Angel and her husband Bill in my clinic, and their story reminded me of why the focus on early diagnosis in CTEPH remains key in offering a potential cure for this deadly disease.

Angel lives in a small town in the Midwest with her husband, Bill, and their teenaged son. When Angel turned 40 a couple years ago, it was only the occasional migraine that could slow her down, but that would soon change.

It started slow. At first when Angel noticed that she had to stop and catch her breath every once in a while going up a flight of steps, she didn’t really think much of it, and gradually, it became a thing of routine. She promised herself that she would join a gym, guessing that she was just out of shape. Surely, she thought, that must be it.

Weeks went by and everyday activities started seeming like chores, until finally she could not ignore her symptoms any longer. After a visit to the family doctor, chest x-rays and a round of antibiotics, she thought she would be good as new. But the breathing just did not get better, and now Angel was starting to get and stay tired all the time. After another trip to the family doctor and some blood tests, there were still no answers to be found. She underwent a breathing test (she had some exposure to second hand smoke at work) and was prescribed a trial of nebulizers. After a few weeks, it was clear that these were not going to do the trick either. Since all her test results looked ok, it was decided that her symptoms were "probably just related to anxiety." Another three months went by, and now Angel was convinced that something was really wrong. After yet more blood tests and reassurances, Angel asked for a second opinion. This time, a CT scan of her lungs was ordered.

Shortly after undergoing the scan, Angel received a call to schedule an urgent follow-up appointment. She was told that her scan was "abnormal" and that she may have a condition called sarcoidosis of the lungs. This could be, she was told, the explanation for her symptoms. Since there are no blood tests to confirm sarcoidosis, Angel was referred to a surgeon for lung biopsy. She spent the next week reading all about sarcoidosis on the Internet.

On the day of her appointment, Angel and Bill arrived at the multi-specialty clinic only to realize that they had accidently made an appointment with another physician who happened to share the same last name as her surgeon! Luckily for them, he was a pulmonary specialist. He reviewed Angel’s CT scan and his words left them stunned. He told Angel that she did not have sarcoidosis but rather another rare condition called pulmonary hypertension. The next step was a series of tests to confirm the diagnosis.

Angel and Bill returned home more confused and concerned than ever and started reading up on PH. As she read the symptoms, she felt that they described her experience; but how could she have acquired this condition? They were determined to find out and made another appointment with yet another specialist.

The weekend before her appointment, Angel found herself feeling more tired than usual. As she got up from finishing a meal, she suddenly felt a wave of dizziness pass over her; and then, Angel passed out. She soon found herself in the ICU undergoing a flurry of new tests. An echocardiogram showed that her pulmonary pressures were more than 100 mmHg (normal is less than 25), and Angel’s pulmonary hypertension was soon confirmed via a right heart catheterization.

Angel was fortunate that the center where she was being treated knew to order a V/Q scan to rule out the presence of CTEPH. In her case, the scan confirmed that she had a number of old blood clots in both lungs. Angel finally had a diagnosis: chronic thromboembolic pulmonary hypertension (CTEPH). Angel was promptly started on blood thinners and IV drugs to lower her pulmonary pressures, and for the first time in a while, she felt “like her lungs could finally get some air.”

A week later, Angel was back on her feet and had been referred to our clinic to be evaluated as a potential candidate for pulmonary thromboendarterectomy (PTE) surgery, a surgical intervention that would potentially offer her a chance for a cure from this otherwise progressive and potentially deadly disease. As part of the evaluation, Angel also underwent several tests to rule out other potential causes for her PH and her blood clots.

Angel is currently scheduled to have PTE surgery this month.

Along the way to her CTEPH diagnosis, there were a lot of “what ifs” for Angel. What if the initial CT scan had been done with contrast? (In order for a CT scan to detect clots, IV contrast has to be used.) What if she had not accidently seen the “wrong” doctor who had her on the right path? What if she had not persisted in her quest to seek out the right answers?

But despite all this, Angel tells me that she is focused on the future, thankful that she finally understands why she felt the way she did and glad that she has a shot at a possible cure.

Last, but not least, Angel has promised that after she’s recovered from her surgery, she will share her journey in her own words, as a follow-up to this blog.

So, stay tuned!