ONE MAN’S BATTLE FOR QUALITY OF LIFE
▶ Doctors often misdiagnose HAE, a potentially lethal condition, but Rashad Matraji wants that to change
For 25 years, Rashad Matraji could not find an explanation or treatment for his unusual condition, which causes his limbs and organs to swell up to three times their usual size.
“I inherited the condition from my father and grandfather before him,” says Mr Matraji, 34, from Lebanon. “No one knew what I had. Doctors would diagnose randomly but no one could find a cure.
“I even had my appendix unnecessarily removed. But I knew there must be a treatment and I vowed to find it.”
The oedema, or swelling attack, happens in different body parts each time and usually once or twice a month. It can be triggered by stress, hormonal changes or a minor injury.
“If you hit your hand against a table it could become red for a while, but for me that triggers an attack and my hand would grow to three times its size,” the industrial engineer said.
“If it was my right hand, I wouldn’t be able to write any more. If the attack hit my foot, I would walk with a limp.”
After what he described as the most upsetting attack yet, Mr Matraji was determined to find out what he is suffering from.
“I was in London in 2008 for a training course and the attack hit my face. I could not skip the course because my company had already paid for it,” he said.
The episode lasted four days, throughout his trip.
“It took days to swell to its maximum, and two days to deflate. I was traumatised. I couldn’t talk properly and I couldn’t eat or drink because my lips were massively enlarged.
“At that time I did not know of HAE and that there were people who could help me.”
After some online research, he found that his troubles could be the result of HAE, or hereditary angioedema, a potentially life-threatening genetic disorder.
The condition is a result of a defect in a blood protein known as a C1 inhibitor. It leads to a biochemical imbalance that makes the tissue around an organ swell. If an oedema affects the airway, it can be lethal.
“When I suffered abdominal attacks the swelling put pressure on my intestines, which causes diarrhoea, and I would start throwing up the acid released by my stomach.
“When I would have these episodes – described by mothers with HAE as being as bad as labour pain – I could not do anything. I would lie down and wait for the pain to pass, which takes more than 24 hours.”
There is a medication that stops the swelling but it is not often covered by insurance companies.
UAE nationals can acquire it through public hospitals but expatriates must place a special order with the supplier and ask their insurance company to cover it each time.
Mr Matraji has made it his mission to make the medication as accessible as possible to patients in the region and to educate doctors, and patients, about the condition.
In 2010, he became the Middle East and North Africa advocate for HAEi, the international patient organisation for C1 inhibitor deficiencies.
There are no accurate records for the number of patients in the region who suffer from the condition because countries keep no register, except for Iran, which has 55 cases.
Mr Matraji personally knows of five sufferers in the UAE, about a 100 cases in Saudi Arabia, three in Oman, three in Kuwait, one in Bahrain, six in Lebanon and five in Qatar. They plan to start their own register.
As HAE affects only one in up to 50,000 people, very few doctors in the region are aware of it and patients seldom receive an accurate diagnosis.
Dr Ravi Gutta, a consultant in allergy and immunology at Mediclinic City Hospital, said there could be up to an eightyear delay to diagnose HAE. He has come across 12 HAE patients in the UAE.
“The greatest challenge in the region is awareness of HAE in physicians and emergency doctors and awareness of medications and insurance cover,” Dr Gutta said.
“I have been trying my level best to convince insurance companies to approve Berinert [an FDA-approved treatment for acute HAE abdominal or facial attacks] for patients because it can be life saving if a laryngeal attack happens.”
This occurs in about 50 per cent of patients, he said.
After he became an advocate, Mr Matraji contacted three pharmaceutical companies to ask about importing the medicine to the UAE, but only CSL Behring, which supplies Berinert, would do so.
He organised regional conferences between doctors, patients and experts in the field to spread awareness and make the medicine “accessible in a normal manner”.
“The medicine is not available in a normal way and sometimes we are cut off for three months until the order arrives,” Mr Matraji said.
“My mission is for everyone with HAE to have access to medication because it is possible for us to live a normal life. The solution is out there.”
He also co-ordinates with physicians in the region, such as Dr Gutta, who listed Mediclinic City Hospital as a centre for HAE.
One shot of Berinert, however, costs more than Dh3,000, and a patient’s need depends on their weight and the severity of the attack.
Now the challenge is to have it entirely covered by insurers.