Hereditary Spherocytosis

Hereditary Spherocytosis

Is the condition that James has (not his fault) and now, we believe, Josh has too. He still needs another confirmation blood test but it certainly seemed likely from what the doctor told me earlier from prilimary results.

Should we be worried? Well, I am not the expert. From what I understand it could well involve blood transfusions, food supplements and eventually, a Splenectomy.

 As a comfort we can look at James and his dad .André and see that it is possible to live quite a reasonably normal life with this condition. My concern is that, if not given the right treatment, it can cause behavioual issues later on which will effect his education. This is not from the spherocytosis itself but from the Anemia that it can cause. If it develops into Chronic anemia it may result in behavioral disturbances in children as a direct result of impaired neurological development in infants, and reduced scholastic performance in children of school age, this could, and I hope he doesn’t mind me saying so, explain a lot with James. I hope it may raise a smile when I say ‘It’s not your fault’.

Sean says, “Whatever we need to do we’ll do it”, Daisy says “We’ll deal with it” and, although I have ot yet heard, I am sure James will be positive and supportive too.

In a nutshell, what is it?

It is where the red blood cells are the wrong shape. The immune system in the splene detects this as them being abnormal and attacks them. This is just the splene doing what it is meant to do except, there are no ‘normal’ blood cells so effectively, it is destroying all the red blood cells in the body if left unchecked.

As the red blood cells are what carry our oxygen, the consequences can be bad.

What does this mean for Josh and the family?

Probably loads of hospital visits, more tests, worry and general feelings of helplessness. Both this family and James family are used to that sort of thing, them more specifically with this condition, us more with ‘conditions’ in general.

We all like to think of baby as being healthy, everyone says it don’t they? “It doesn’t matter, as long as it is healthy” it almost an attack on anything which could possibly deviate away from ‘normal’.Well, little Josh will be OK, he’ll have a good and happy life. Maybe now would be a really good time for everyone concerned with him to put aside their differences? Work really hard at getting along. Josh may have a very long and happy life but, if his time with us is, for whatever reason, limited, can’t we all at least try to make sure it is the absolute best possible? I am not trying to scare anyone here but, as with Jermaine, we have to accept all possibilities.

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