The photo has been used for illustrative purposes.
Mariecar Jara-Puyod, Senior Reporter
Lifestyle diseases which have plagued the world for years may lead to haemophilia—the inability of the blood to clot generally due to gene aberrations—and so the more people have to be more careful with their way and state of living.
“I am not saying that lifestyle diseases are a direct cause of haemophilia. But there is acquired haemophilia,” Dr Shabiha Rana said.
The Al Zahra Hospital-Dubai Haematology consultant shared with Gulf Today her observations on haemophilia and sufferers in the past 13 years, primarily in the UK on Tuesday, eve of annual World Haemophilia Day, celebrated on April 17.
Rana explained that haemophilia which comes in three forms identified in the first letters of the English alphabet is a lifetime blood disorder arising from the abnormalities of the xx chromosomes—one gene is non-functional—of the mother (carrier) who passes these on to her son who manifests it and on to his daughter with whom the disease becomes “silent” and so on down the family tree. She has seen more cases of young children and young adults afflicted, the youngest of which was an adopted eight-months-old girl who was rushed to the hospital for bleeding.
Rana had a 52-year-old patient diagnosed with lymphoma, cancer of the lymphocytes: “He has the genetic inherent type of haemophilia and has been on treatment ever since. We did some haemophilia tests when he came to my clinic because of profuse bleeding. The tests showed he already was stricken with lymphoma.”
On the 78-year-old colon cancer patient referred to her, Rana commented, “Cancer causes a lot of changes in our body. The tests we had with our patient on this case showed hidden factors (which demonstrated) that he had acquired haemophilia.”
People with haemophilia do not bleed faster than others. They just bleed longer because of the lack of or the deficiency of a protein that allows the blood to clot. It is best that they are provided with rich protein diet, green leafy vegetables and red meat. Most experience internal bleeding even at the slightest trauma, the amount of bleeding depends on the severity of haemophilia. Compared to the general population, their life span is limited.
Rana said that while haemophilia is not preventable as it is genetically-borne, what can be done is that couples who plan to marry may go for blood tests to help them eventually prepare for their parenthood in such a way that peri-natal (before and after birth) tests would also be performed on their babies.
Diagnostics on the unborn child would determine whether he or she has haemophilia. Once known, it is highly recommended that the expectant mother of a haemophiliac baby undergo caesarian section delivery.
Rana added, “Normal delivery may require the vacuuming of (the haemophiliac baby) which of course is dangerous.”
As it is a World Health Organisation standard that newborns be injected with Vitamin K—a group of fat-soluble vitamins necessary for blood clotting—Rana said the recommendation is for haemophiliac newborns to get it intravaneous-ly rather than having it injected in their buttocks or muscles.
According to Rana, available in major hospitals in the UAE are replacement therapy, “concentrates of clotting factor VIII for haemophilia A and clotting factor IX for haemophilia B which are slowly dripped or injected into a vein.
There are prophylaxis to prevent or reduce the frequency of bleeding episodes in adult and paediatric patients with haemophilia A with or withous factor VIII (FVIII) inhibitors, first approved in 2017.”
In the UAE is a Haemophilia Society. Gene therapy may offer potential cure.
World Haemophilia Day has been observed every year since 1989, an advocacy of the World Federation of Haemophilia founded by severe Haemophilia patient, Canadian Frank Schnabel in 1963, not only for global awareness but more importantly, to help haemophiliacs and their families cope.
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