After being treated for two life-threatening blood clots, avid back country skier Mark Porter grew "so weak that he couldn't walk across a room without stopping to rest." For 16 months, no one could figure out what was wrong, Sandra Boodman writes for The Washington Post. 

His 'age, lack of risk factors and level of fitness' were not consistent with his symptoms

In July 2020, Porter, who was 47 years old at the time, started to regularly feel short of breath — despite his daily exercise routine. Because Porter had a history of bronchitis and pneumonia, his primary care doctor believed that he could have been experiencing exercise-induced asthma. 

However, by November his right calf became so swollen that it was difficult to walk. Porter couldn't think of anything that could have caused the swelling, but an internet search suggested deep vein thrombosis as a potential cause. He immediately made an appointment with his doctor, who examined his leg and sent him for an ultrasound and CT scans.

Following the appointment, Porter received a call telling him to immediately come back to his doctor's office. The doctor explained that he had two life-threatening blood clots — a clot in a vein in his calf and a rare, very dangerous saddle pulmonary embolism that was cutting off the blood supply to his lungs.

He was immediately rushed to a hospital in Idaho Falls, where doctors were waiting to address the potentially lethal clots.

During a two-day hospitalization, Porter took blood thinners while doctors worked to determine the source of his clots. However, he had not experienced any of the triggers they asked about, and his "age, lack of risk factors and level of fitness" were not consistent with his symptoms.

'He was only 49 and in very good shape, but he was more and more short of breath'

While the doctors could not determine the cause of his clots, they told him he would likely recover after six months on a blood thinner. Seven months later, he was still experiencing shortness of breath.

"By August 2021, Porter started experiencing chest pain and felt so exhausted he began taking a daily nap," Boodman writes.

Concerned that he had developed new clots, Porter returned to his doctor in November. His doctor ordered an ultrasound, CT scans, and a cardiac stress test — but everything came back normal.

After struggling through a ski trip in December 2021, Porter made an appointment with a new primary care doctor when his was out of town. The new doctor sent him to a cardiologist, who performed additional tests before sending him to Idaho Falls lung specialist Allen Salem in March 2022.

"He got to me the way all these patients do — after a long period of misdiagnosis," Salem said. According to Salem, many patients, like Porter, are erroneously told they have asthma or heart failure or told that nothing is wrong with them.

However, Porter's history of mysterious blood clots, his ongoing shortness of breath and chest pain, and his test results led Salem to one diagnosis — chronic thromboembolic pulmonary hypertension (CTEPH).

"There's a lack of awareness (among primary care doctors) of what can happen after a blood clot," Salem said. "This disease is underdiagnosed and underappreciated."

According to Salem, some doctors are reluctant to refer patients to lung specialists when they don't understand why they are experiencing ongoing shortness of breath. Some doctors have never even heard of CTEPH.

For Salem, Porter's diagnosis was simple. "He was only 49 and in very good shape, but he was more and more short of breath," Salem said.

CTEPH can be cured with a pulmonary thromboendarterectomy (PTE) — "a long, complex and demanding operation to remove clots," Boodman writes.

Salem referred Porter to specialists at the University of California at San Diego (UCSD), where PTE surgery was pioneered. Surgeons at UCSD have performed over 4,000 PTE procedures, more than any other hospital in the world. Porter quickly elected to have the risky, day-long operation.

'I probably waited too long to fight for myself'

On August 30, 2022, Porter underwent the nine-hour procedure. According to Michael Madani, UCSD's chief of cardiovascular and thoracic surgery, who performed Porter's surgery, the operation went "very well."

"He had quite a bit of obstruction in both lungs," Madani said. However, Porter's heart was still in very good shape, which is not the case for many patients.

After spending four days in the ICU and an additional week in the hospital, Porter was able to return home. His recovery was more difficult than expected, with some pain lingering for months.

Three months after his operation, Porter was cleared to resume his usual activities. While CTEPH can sometimes return, Madani said he does not anticipate that for Porter if he takes blood thinners for the rest of his life.

From the onset of his symptoms, Porter spent 16 months "trying to discover why he had grown so weak that he couldn't walk across a room without stopping to rest," Boodman writes.

In hindsight, Porter said he wishes he had been more insistent in pushing for a diagnosis and less concerned about upsetting his doctor. "I probably waited too long to fight for myself," he said. (Boodman, Washington Post, 5/13)