An expatriate in Dubai who was diagnosed with a rare, life-threatening blood disorder that affects just a handful of people has survived the platelet-suppressing condition after weeks in ICU.
When Indian expat LalTelu Ram Harkesh, 52, experienced abdominal pain, he first ignored it but days later developed a mild fever and sought medical help.
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Things took a serious turn when, after laboratory and radiology investigations, doctors at Lifecare Hospital, Mussafah, diagnosed Harkesh with a Thrombotic Thrombocytopenia Purpura (TTP) – a rare disorder of the blood coagulation system that usually affects about four people in a million.
TTP is a life-threatening disorder in which the blood clots that form in the small blood vessels throughout the body can limit or block blood flow to the major organs. This, in turn, can damage the organs like the brain, kidneys, and heart and prevent them from functioning correctly.
“During the initial evaluation, he seemed to be suffering from acute pancreatitis,” said Dr Ashraf Abdelrahman, head of the hospital’s critical care department, in a media statement.
“But he had low red blood cell and platelet counts that did not correlate with the preliminary diagnosis.”
“Tests confirmed he had TTP, a rare disease that can be fatal if left untreated. This is the first case of TTP we are coming across in our hospital… such patients require close monitoring as they are at high risk for altered conscious level, seizures, hemiplegia and multiorgan failure.”
Doctors said diagnosing TTP was the main challenge they faced. TTP is a rare disease with very vague symptoms, and the confirmatory test is performed only in a few laboratories in the country.
Dr. Abeesh Padmanabha Pillai, specialist nephrologist, said: “Harkesh received a total of 10 sessions of TPE daily with almost 30 litres of plasma exchanged. He was also treated with steroids and monoclonal antibodies medications.”
As he had neurological symptoms of seizures and loss of consciousness, doctors deeply sedated and endotracheally intubated him. He remained on mechanical ventilation till complications were controlled and his condition improved.
It took around 18 days for Harkesh, who hails from India’s Himachal Pradesh, to recover.
The expat, who works as a foreman in a company in Abu Dhabi, said: “I had pain for around 20 day… when found that I was diagnosed with a rare disease I got scared. Now, this is my second life.”
According to the doctors, a few questions remain unanswered, including why TTP happens, why patients relapse after achieving remission, and how to cure the disease.
“What we know about TTP is that it is a true medical emergency, and without treatment, 95 percent of patients succumb to death. However, 80-90 percent can achieve remission with treatment,” said Dr Baiju Faizal, a specialist in internal medicine, at the hospital.
There is no method of preventing TTP or its relapse. According to doctors, when a patient is diagnosed with this disease, it is better to follow a healthy lifestyle by avoiding smoking and ensuring adequate hydration, a low-carbohydrate, low-salt, low-fat diet, and proper sleep.
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