What is MPS IV

I realized today that I have been telling you about my boys and just put up donation information but most of you reading this blog don’t know what MPS IV or Morquio syndrome is. It is an autosomal recessive syndrome. Meaning both me and my husband carry the recessive form of the mutated gene. Currently there is no cure or treatment for this syndrome, but there are clinical trials that were started in 2009. What is happening to the boys is that they are missing an enzyme that most of us have that breaks down natural sugars in our body. It has nothing to do with the sugar we eat. Because they either don’t have any of these enzymes or not enough of them these sugars build up in their joints. There is normally no signs at birth but has they get older and as the cells break down and the sugars don’t the left over sugars start to build up in the bodies. It affects bone development resulting in many surgeries though out their lives. Because of the bone development these children usually slow down growing around 18 months and usually stop growing at around 8 years of age. It can also affect their organs. Examples would be enlarged livers and splines. Also can affect the heart by being enlarged and having irregular beats. Luckily, children with MPS IV have normal intelligence. As of right now, the boys’ organs are normal and we can hope they stay that why. They do have many of the bone deformities that come with this syndrome. As of right now we do not know if the boys will have a shorten life span. Because their organs are normal I can hope that they will live into their 50s and 60s. But because of bad organ development some can pass around their 20s and 30s. This syndrome affects children differently. We may not experience some of the symptoms and some we may not experience as severe as others. We just have to wait and see how it affects them. This is why I set up a fund for my children. It is not done after this last surgery. This bill will not be our last. They could be facing upwards to 20 surgeries a piece. We will be in and out of the hospital whether it is just for annual check ups or extended stays. I do not like asking for help but I know that we can not do this financially alone. I also have realized in the last month that there are wonderful people out there that want to help in any way they can even if its not in a monetary form. I thank everyone who helps out and in any way they can. I don’t even know if the reality has hit me with what is going on and what lies ahead. Maybe it is best that I feel that way and take this day by day and step by step.

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About Alison

Mother of twin boys whom both have morquio syndrome
This entry was posted in Jack and Hunter. Bookmark the permalink.

2 Responses to What is MPS IV

  1. Hi Allison,
    My name is Danette Baker and I live in Eastern Washington state. I am 37 years old and also have Morquio like your boys do. I found your site via Annabelle Bozarth’s CaringBridge page and I see in your links that you also know Bella Burton. I wanted to let you know that we also have a Yahoo group for those with Morquio and you can join that group by going to http://www.yahoogroups.com/morquio . Welcome to the family of Morquio!

    Danette Baker

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