The word "catastrophic" in the name of catastrophic antiphospholipid syndrome is a clear indication of the power of the disease – catastrophic. The pathogenesis of catastrophic antiphospholipid syndrome is not quite the same as that of common antiphospholipid syndrome. In general, antiphospholipid syndrome is dominated by macrovascular thrombosis, which progresses slowly and generally does not involve multiple organs.

But catastrophic antiphospholipid syndrome is quite different, it is a serious disease with a clinical mortality rate of more than 50%. Involving more than three organs within a week of onset, with arterial venous thrombosis and microvascular thrombosis as the main manifestation. Most patients eventually die of DIC symptoms due to pulmonary embolism or abnormal blood clotting.

The most frightening thing is that there is no ideal treatment for catastrophic antiphospholipid syndrome.

"If it's CAPS, it's bad. Yuan Ping'an's face sank like iron, and he said to Sun Lien, "The patient has just received thrombolytic and anticoagulation therapy, but his avascular necrosis of the small intestine has not been completely reversed. If the necrotic area continues to expand, excision surgery is performed at the risk of heavy bleeding during surgery. ”

"This risk is more acceptable than the risk of necrosis of the small intestine. Dr. Pascal wasn't too worried, "Let's get an APL antibody test first." Diagnosis of CAPS is confirmed, and immediate initiation of hormone pulse and immunosuppressive therapy, followed by adjunct plasmapheresis. This allows antibody aggregation to be controlled in the shortest possible time and reduces the risk of abnormal clotting in the case of deheparinization. As long as the plasma is replaced in a timely manner, he should be safe to undergo surgery. ”

The treatment proposed by Dr. Pascal is the standard coping method. For the current situation, this is the only thing doctors can do for Xia Hongyuan.

"Let's get a serious illness notice. Sun Lien sighed, he really didn't want to see this situation. A teacher who is loved by students, a young and energetic first-class athlete, is now lying in the emergency room and dying. Even after surgery, blood clot removal, and thrombolysis therapy, the chances of him surviving are still half and half.

In this case, how could Sun Lien be relaxed?

"I'm going to talk to the family. Seeing that Yuan Ping'an brought the notice of serious illness, Sun Lien stood up and took the notice, and turned to Dr. Pascal and said, "After the family agrees, the treatment will start immediately." ”

Sun Lien left the most difficult work to himself.

Xia Hongyuan's parents waited outside the door for four hours, their only child had already undergone an operation, but seeing the doctors as if they were facing a great enemy, Xia Hongyuan's parents couldn't figure out why the doctors were so nervous. Shouldn't the child get better after the surgery?

"Doctor Sun!" Xia Hongyuan's father saw Sun Lien from afar, he quickly stood up, walked two steps forward and asked, "How is he doing?"

Sun Lien looked around with some embarrassment, this is the rescue hall, and there are many people nearby. He said in a low voice, "His condition is quite stable now, please come with me with your wife first, this is not the place to talk about things." ”

Xia Hongyuan's father suddenly sank, and he tried to hold his wife's hand. But the uncontrollable tremor of the whole body can be seen as long as the eyes are not blind.

Sun Lien sighed softly and led the two to the small conference room.

Dr. Pascal had expected Sun to take the parents to other conference rooms, but he didn't expect him to bring people here directly. Looking at the middle-aged couple who looked like ashes, Dr. Pascal also sighed. He stood up and found two paper cups and poured some tea into them.

"Please be seated, these are all colleagues from our treatment team. Sun Lien invited the two of them to sit down, and then handed over the notice of serious illness that he had been holding in his hand. "Please sign this notice of serious illness. ”

Xia Hongyuan's father suddenly wiped a large pool of water from the water cup he had just picked up, "What's going on?"

Sun Lien sighed again. "As you two know, we have already operated on Xia Hongyuan. But his problem isn't just what we can solve with surgery. He pointed to the whiteboard with the order of symptoms written on it, "Xia Hongyuan's disease is causing all of these symptoms, and based on the information we have so far, we believe that the name of this disease is 'catastrophic antiphospholipid syndrome'." ”

"What do you mean...... You haven't been completely sure?" Xia Hongyuan's father was keenly aware of the problem in this statement.

"We are still waiting for the final and decisive evidence of the examination. "But based on the current situation, this disease is the most likely." ”

Xia Hongyuan's father was silent for a moment and then asked, "If this is the disease, how can it be treated?"

"Let me explain that. Sun was about to speak, but Dr. Pascal stepped up on his own. "It's my professional content. ”

"I'm a doctor in the Department of Rheumatology and Immunology, Pascal. Dr. Pascal briefly introduced himself to the parents, "According to our diagnosis, Xia Hongyuan's disease is a relatively rare autoimmune disease, and the main feature of this disease is abnormal blood clotting in the blood vessels of the body. ”

"First of all, the cause of this disease is still not certain. But according to research, trauma can be a major trigger for this serious reaction. But given his previous experience as a professional athlete, there's no way to narrow it down to a specific traumatic experience. Dr. Pascal's voice was very steady, sounding a little slower than he normally would. But his disease was very insidious, and not many organs were affected at first. He pointed to the whiteboard, "The first organ involved should be the small intestine." Dr. Pascal glanced at Xia Hongyuan's mother, who was already crying, with some sympathy, "That's when you received a call from him before, saying that you had an upset stomach. ”

Later, he was admitted to the hospital with severe pain all over his body, which we now believe was caused by a blood clot in his brain. Dr. Pascal continued, "The clot was not supposed to be large, so he didn't show symptoms of a severe stroke, but rather unexplained pain." ”

"When we entered the hospital, we first relieved his pain symptoms. The rest of the story is what Xia Hongyuan's parents knew, but Dr. Pascal's explanation was still very detailed. "And we immediately recalled Dr. Sun. He pointed to Sun Lien, who was listening carefully to the lecture on the side, and praised him a little, "He is the leader of our treatment team, and he is also the doctor with the best diagnostic technology in us." ”

Of course, Lao Pa's praise is not to pat Sun Lien's ass, but to soothe the emotions of these two people a little - after learning that the doctors who treat their children are of high quality, it can indeed relieve the pressure of many patients' families.

"After several tests, we gradually locked his disease on the current 'CAPS'. Dr. Pascal said a great deal of more before he continued, "Because of the severity of this disease, we have issued you a serious notice. ”

The rest of the content should be Sun Lien's coming, "First of all, let me tell the two of you that Xia Hongyuan's illness is very dangerous. ”