He was treated for blood clots but still felt fatigued and breathless

Mr. Porter’s wife drove him to the infirmary, where they were forced into an examination room. They panicked when they heard the doctor say to someone on the phone: We have an ambulance ready to transport you. ”

Doctors also told Porter, then 47, that he had two life-threatening blood clots. One is deep inside the thrombus. his calf vein And the second rarest and most dangerous is saddle lung An embolism had obstructed the blood supply to the lungs. Porter had to go straight to a hospital in Idaho Falls, about 30 miles away. The doctors were waiting for him there.

“It was surreal,” said Porter, who recalled being shocked by the news but not completely surprised.

The discovery of the clot, which was treated with anticoagulants to dissolve it, was only the beginning of Porter’s ordeal. The extreme backcountry ski enthusiast, who was in good health, spent the next 16 months trying to figure out why he was so debilitated that he could not cross the room without stopping to rest.

“I made it,” said Porter, who underwent intense treatment in August 2022 and now feels “amazing.” He said he wished he had sought a second opinion sooner. Instead, he made repeated visits to his family doctor, who said he could find no reason for Porter’s worsening shortness of breath, chest pain, and fatigue, and did not refer him to a specialist. ..

“I don’t like waves, I don’t like fights,” said Mr Porter, worried he would anger a long-time family friend and doctor.

Porter’s blood clots had probably formed for months. In July 2020, he began feeling increasingly short of breath, despite a daily exercise regimen that included weightlifting and water skiing. His doctor suspected that Porter had a history of bronchitis and pneumonia and that he had developed the disease. exercise-induced asthma I was prescribed steroids and an inhaler.

Neither helped. Several times Porter saw a doctor, but the doctor did not recommend anything new. In early November, weeks before being rushed to hospital, Porter’s right calf became so painful and swollen that it became difficult to walk. He didn’t remember hurting it and thought the problem might be something like this: sciatica, a compressed nerve caused by injury to the intervertebral disc in the lumbar. When the pain got worse, he turned to the internet. No search results found. deep vein thrombosis, A possible cause is a blood clot in the leg.

A startled porter immediately made an appointment with a doctor. He apologetically told the doctor that he was convinced he was overreacting. Doctors examined his leg and immediately sent Porter for an ultrasound, followed by a CT scan, which revealed a potentially fatal blood clot.

While in hospital for two days, Porter began taking anticoagulants to dissolve blood clots so doctors could determine the cause of the clot. They were surprised when he told them all that he hadn’t hurt his leg, never been on a plane, never had the coronavirus. trigger. And they were particularly surprised by Porter’s age, lack of risk factors, and level of fitness. Most people who develop blood clots are very old, overweight, sedentary, or have an underlying clotting disorder.

Porter was shaken at the brink. “By the time a disease is discovered, nurses usually Saddle PEthe patient is already dead, ”he said.

Doctors told him he should be fine after six months of blood thinners.

But seven months later, she was still short of breath. By August 2021, Ms. Porter began to feel pain in her chest and felt so exhausted that she took daily naps.

Worried that a new blood clot had developed, Porter consulted her family doctor in late November. The doctor ordered an ultrasound and a CT scan, cardiac stress test. No new blood clots were detected, and Porter passed a stress test that measures heart function during exercise. Doctors told him his heart was fine and he shouldn’t be short of breath. Doctors say his chest pain may be due to acid reflux. He allowed the porter to ski.

“After many years of misdiagnosis, he came to me like any other patient.”

— Allen Salem, Doctor

Porter knew what acid reflux felt like, but was skeptical. But the self-professed worrier said he wondered, “Maybe a lot of this was in my head.”

In late December, Porter took one of his three sons on a long-planned backcountry ski trip to British Columbia. “I just endured it and suffered,” he said. He was having trouble breathing at times and the pain in his chest was so severe that it woke him up in the middle of the night.

When I got home, I found out that my doctor was on vacation. Ms. Porter made an appointment with a new doctor to see a cardiologist.

The cardiologist put him back on blood thinners as a precaution, several tests (negative) to detect genetic factors that contribute to the formation of blood clots, and VQ scan, Two nuclear medicine tests that evaluate air and blood flow in the lungs. Scanning is considered essential for detection. Certain lung problems. Because the scan was abnormal, right heart catheterizationThis test, which measures pressure in the heart and lungs, showed the possibility of pulmonary hypertension, high blood pressure that affects the lungs.

The cardiologist then referred Porter to a pulmonologist in Idaho Falls. Allen Salemhe met in March 2022.

“After many years of misdiagnosis, he came to me like any other patient,” Salem said. Like Porter, many people have asthma, heart failure, or have been falsely told there is nothing wrong, he added.

Porter’s history of blood clots for no apparent reason, persistent shortness of breath and chest pain, and the results of her VQ scan and other tests strongly suggested one diagnosis: chronic thromboembolic pulmonary hypertension (CTEPH).

This rare form of pulmonary hypertension is caused by a blood clot that clogs an artery, forming scar tissue that adheres to the blood vessels in the lungs, narrowing them and impeding blood flow.

Experts estimate that 2-5% of people with blood clots develop CTEPH that does not respond to blood thinners.

However, unlike other forms of pulmonary hypertension, CTEPH can be treated by pulmonary thromboendarterectomy (PTE). long, complex and demanding work Remove blood clots. Non-surgical treatments include drug therapy, but are not curative.

“For me, this is always on my mind,” said Salem, who diagnosed 10 CTEPH cases last year alone. Among them was a park manager whose age suddenly made it difficult to climb hills, and a doctor’s mother who was misdiagnosed as having asthma.

“There is a lack of awareness (among primary care physicians) about what happens after a blood clot,” Salem said. “This disease is underdiagnosed and underestimated.”

Some doctors are reluctant to refer a patient to a pulmonologist if they can’t determine what’s causing the patient’s persistent shortness of breath, he said. Some people have never heard of CTEPH.

In Porter’s case, the diagnosis was not difficult, Salem said. “He was only 49 years old and in very good health, but he was getting more and more short of breath.”

Salem advised Porter to reduce his physical activity, and Porter said he did 60 to 100 push-ups a day to stay fit and referred him to specialists at the hospital. University of California, San Diego. The UCSD surgeon pioneered his PTE surgery, having performed over 4,000 surgeries, more than any other hospital in the world.

“I probably waited too long to fight for myself.”

— Mark Porter

Several days of work-up in San Diego were required to determine if Porter was eligible for surgery. Michael Madani He is the Chief of Cardiovascular and Thoracic Surgery and performs about 4 PTE surgeries a week.

Porter said her initial reaction to the diagnosis was “relieved.” I’ve been told I’m fine, or that I don’t know what the problem is. ”

He quickly decided that surgery was necessary despite the risks, including overall mortality. approaching about 5 percent. Prices for individual centers vary. Porter’s UCSD records mention: overall mortality of two percent.

all day long surgery You need a highly skilled and experienced team.

The surgeon makes an incision in the chest to access the heart and lungs and places the patient on a cardiopulmonary bypass machine. The cardiopulmonary bypass machine is periodically turned off to create the bloodless surgical field necessary for visualization of thrombus attached to the arterial wall. The body is cooled to approximately 68 degrees to avoid damage to the brain and other organs. This is a process called cycle arrest. After the blood clot is carefully removed, the patient is gradually warmed, the chest is closed, and taken to the intensive care unit.

For Porter, the process of getting the records to San Diego took about two and a half months, while he anxiously awaited an appointment. He was given a date in late August. Surgery was tentatively scheduled for a week later.

In July, Porter had a mild case of Covid-19 after being required to undergo a coronavirus test a week before arriving in California. “I was really nervous,” he said, possibly delaying the operation. Luckily, the test was negative at the time of need.

Porter and his wife drove 1,000 miles to San Diego, where they had relatives. During a thorough examination at UCSD, which included repeated VQ scans and other tests, doctors told Porter that he may not be the obvious candidate. Would he want to have surgery if there was only a 50/50 chance of success?

“I said, ‘Absolutely, I have no choice,'” recalls Porter. At that point, he could only work three hours maximum in his day and needed long naps as well. “I told her she couldn’t live like this, but I was really depressed that maybe they wouldn’t provide it.” His apprehension turned into elation.

Porter’s Aug. 30 surgery, which took nine hours, “went very well,” Madani said. “He had significant blockages in both lungs.” Mr. Porter’s heart was in very good shape, but the surgeon noted that this is not the case for many patients.

CTEPH can recur, especially in patients with underlying clotting disorders, but that doesn’t happen in Porter’s case if he takes the blood thinners he’ll need for the rest of his life, Madani said. said.

After spending four days and another week in the ICU, Porter’s wife drove the couple home.

Recovery was more difficult than Porter expected, and some pain lasted for months.

In November 2022, three months after surgery, Porter was cleared to resume weightlifting. A month later he repeated the backcountry ski trip. It was a completely different experience without the chest pains and shortness of breath that had plagued him before.

Looking back, Ms. Porter said she wished she had been more aggressive in seeking answers and less concerned about upsetting doctors. “I probably waited too long to fight for myself,” he said.

Submit your solved medical riddles to Sandra.boodman@washpost.com. Please do not leave any unresolved issues. To read the mystery so far, wapo.st/medicalmysteries.