Abu Dhabi-based Indian expat was shocked when he was told that his abdominal pain is not a common health issue but rare thrombotic thrombocytopenia purpura (TTP).
Having heard those words for the first time in his life, doctors explained to the 52-year-old patient that TTP is a rare and life-threatening disorder that causes blood clots to form in small blood vessels throughout the body, which could limit or block blood flow to organs like the brain, kidneys, and heart.
A multidisciplinary team of 12 doctors at Lifecare Hospital, Mussafah, have successfully treated him through plasma therapy as Harkesh spent 18 days in the ICU.
“I had pain in my abdomen for around a few weeks. I ignored it, thinking it was typical gas trouble. But then I developed a mild fever. So, I got worried. We keep hearing about new infections and viruses, so I decided to go for a checkup.
“And as I feared, I was diagnosed with a rare disease. I realised it was a life-threatening one and was terrified. But doctors and other hospital staff supported me mentally and physically. All thanks to the Almighty and doctors. This is my second life,” said Harkesh, a foreman at a private company in Abu Dhabi.
Fatal disease if left untreated
Doctors said that TTP is a rare disorder of the blood coagulation system that usually affects 4-6 persons per 1,000,000.
Dr Ashraf Abdelrahman, head of the department of critical care services, said it was the first case of TTP at the hospital.
“During the initial evaluation, he seemed to be suffering from acute pancreatitis. But he had low red blood cell and platelet counts that did not correlate with the preliminary diagnosis. The ADAMTS 13 test confirmed he had TTP, a rare disease that can be fatal if left untreated.”
Dr Mathew Vadakoot, a specialist gastroenterologist, 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.”
Patients with TTP require close monitoring as they are at high risk for altered conscious levels, seizures, hemiplegia and multi-organ failure.
A 12-member medical team at work
Harkesh was quickly shifted to the ICU. A 12-member multidisciplinary team of doctors was formed to analyse his health condition. The team designed the treatment protocol and started therapeutic plasma exchange (TPE) – a process of replacement of plasma obtained from healthy individuals.
“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,” said Dr Abeesh Padmanabha Pillai, specialist nephrologist.
Harkesh remained on mechanical ventilation till complications were controlled and his condition improved.
No cause or ways to prevent TTP
It took around 18 days for Harkesh, who hails from India’s Himachal Pradesh, to recover from this flare-up.
“As he has completely recovered from this initial attack, Harkesh should have a normal quality of life in the future,” said Dr Mudhasir Ahmed, specialist medical oncologist. “Since relapse can occur at any time, he should do regular follow-up and continue the treatment as recommended. Most patients will have a complete recovery, but the literature says the relapse can occur in 30 per cent of patients.”
Doctors added that early detection and treatment of this flare are crucial in preventing irreversible damage to vital organs and death.
The medical team also included Dr Divya S. Nair, consultant neurologist; Dr Sajid Syed, consultant neurologist; Dr Debasish Mishra, specialist rheumatologist; Dr Karthikeyan Chinniah, specialist intensivist; Dr. Sahithi Surapaneni, specialist pathologist, and Dr Kayane Mheidly, consultant haematologist.
Dr Baiju Faizal, specialist internal medicine, added that there is no clear cause or known method to prevent TTP or its relapse.
“What we know about TTP is that it is a true medical emergency, and without treatment, 95 per cent of patients succumb to death. However, 80-90 per cent can achieve remission with treatment.”
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.