So I wanted to start a foundation and also a support group in memory of my little brother Joe’mari who passed away March 20th this is for families who may know or knew someone living with Hunter’s syndrome also know as MPS.
Hunter syndrome is a rare genetic disorder that occurs when an enzyme your body needs is either missing or doesn’t work properly.
Because the body doesn’t have enough of the enzyme to break down certain complex molecules, the molecules build up in harmful amounts in certain cells and tissues. The buildup that occurs in Hunter syndrome eventually causes permanent, progressive damage affecting appearance, mental development, organ function and physical abilities.
Hunter syndrome appears in children as young as 18 months. It nearly always occurs in males.
There’s no cure for Hunter syndrome. Treatment of Hunter syndrome involves management of symptoms and complications.
MPS II is inherited in what is called an X-linked recessive manner. The ‘X’ and ‘Y’ chromosomes determine the sex of a person: males have one X chromosome and one Y chromosome (XY), whilst females have two X chromosomes (XX). In MPS II the gene that codes for deficient production of 2-sulphatase enzyme is located on the ‘X’ chromosome. Thus, males who inherit the faulty X chromosome from their mother will be affected; females who inherit the faulty X chromosome will be carriers but generally do not develop symptoms as they have a second, unaffected X chromosome that compensates.
If a woman is a carrier for MPS II, in each pregnancy there is a:
50% (1 in 2) risk that any male born to her will be affected; and a
50% (1 in 2) risk that any female born to her will be a carrier (but generally not affected);
The sisters and maternal aunts of an affected male have a 50% (1 in 2) chance of being carriers and a 25% (1 in 4) overall risk of passing the condition to any male born to them.
If a male with MPS II has children, all of his daughters will be carriers and all of his sons will be unaffected.
Although not common, in some cases MPS II might arise in a generation without the mother being a carrier or there being any family history of the carrier
It is difficult to be precise about life expectancy because of variation in severity and age of onset. Some individuals whose brain is affected have lived into adulthood but this is usually accompanied by a decline in their quality of life as brain function deteriorates.
If the brain is not affected, a more normal life span can be expected, but significant physical problems can develop that, without treatment, may reduce life expectancy.
My little brother was 11 when he passed away. Doctors told us he wouldn’t live to see 5. I was very blessed to have him in my life. He taught me so many great things when it came to love, accepting people, family, and most importantly GOD. I want to celebrate his life not his death, I’m currently looking into universities that may be studying this disease. I’m still brainstorming this whole thing but I know I just wanna help other families and also RAISE AWARENESS of this disease. Ladies get tested for this
For Joe’mari 💜