::: Star Weekend Magazine :::

Straight Talk

In Times of Adversity

I can hear the laughter of my children ringing throughout the house. Their latest sport is to slide down the stairs on a sleeping bag. They call it Kamikaze crash --- I call it madness. The name should give you an impression how of crazy this particular game is, I doubt you will not find my husband or I trying this any time soon! Apart from a wave of nervousness that hits me every now and then, making me feel that I should intervene and tell the children to stop their game, I cannot help smiling to myself especially when I hear them in uncontrollable fits of giggling. As a parent the sound of happy children is unbeatable. But that feeling is tinged with a heavy heart as I know that right now my six year old niece is in hospital recovering from a bone marrow transplant.

Six years ago if someone had asked me what Diamond Blackfan was, I would have had no clue whatsoever. However, when my niece, only twelve weeks old at the time went into the Accident and Emergency unit of the hospital, the doctors were concerned with the acute anaemia she seemed to be suffering from. According to them, she had been brought in just in time and even went as far as saying that her blood level was so low that it was “nearly incompatible with life”. Extensive tests revealed that she suffered from a very rare blood disorder called Diamond Blackfan Anaemia (DBA) and that DBA is one of those diseases that is so uncommon that it only occurs in around 5 to 10 babies out of one million births. I cannot envisage what a parent must go through when they hear something as life changing as that.

DBA is actually what can be described as a bone marrow defect where the marrow does not produce any red blood cells. Without red blood cells it is impossible for the blood to carry out its primary function which is to transport oxygen around the body. Normally these red blood cells are continually produced and destroyed in “a natural cycle in which iron is recycled from old to new cells”. However, in my niece's case this was not happening.

There was the inevitable agony and helplessness that accompanies the knowledge of a diagnosis as rare and unusual as Diamond Blackfan Anaemia and months of trying to come to terms with the full extent of what was in store for their daughter and for my sister in law and family. The Treatment for DBA is said to be possible with medication (steroids), blood transfusion, bone marrow transplant or use of the umbilical cord blood which is a rich source of the bone marrow stem cells from an unaffected sibling identified prenatally.

Sometimes children with DBA show physical features such as an extra thumb joint, cleft palate or some sort of characteristic facial appearance. In some cases, children with DBA have a slow growth rate and are very short for their age which could also cause delayed puberty. Otherwise they develop normally, and do not show any signs of learning difficulties. Looking at my six year old niece, there is nothing to suggest that she is any different to any of my children or her friends in school. She is a happy, fun loving little girl who seems to have accepted her fate with a brave face. You would never think that every month she has to go into hospital for a blood transfusion to maintain an acceptable haemoglobin level. On top of that she is also administered a drug at home via a needle for 5 nights every week, 12 hours a night which helps remove the excess iron in her system which would otherwise build up due to the transfusions. I can remember the time when my eldest daughter broke her arm or when my youngest had to be taken to hospital --- I was a mess. The thought of having to go through this kind of agony day in and day out is unimaginable.

Over the years our niece received a whole host of treatments including a drug trial to try and cure the symptoms and the condition but sadly none of them worked. For a child suffering with Diamond Blackfan Anaemia, the only long term cure is a bone marrow transplant which in itself is a difficult and painful procedure. As parents it is unbearable to watch our children in pain or to see them suffer in any way. But the decision had to be taken to try and ensure the long term wellbeing of their daughter and my sister in law and her husband took the only step available to them.

The stem cell and bone marrow transplant took place last week and the chemotherapy that goes hand in hand with an operation such as this will mean that this little girl will gradually lose her hair and have to undergo levels of pain that is unthinkable. Due to the fact that the chemotherapy temporarily destroys the immune system she has to be confined to a sterile environment with a specially designed, air-filter for her room in the hospital. This includes not being able to have any visitors or be able to interact with her siblings for over an extended period of time. Even when she returns home it will be a long time until her immune system recovers which in turn means that she will not be able to go to school or interact with too many people. I can only pray for a speedy recovery.

The reason for I have written this article is to pay tribute to all those parents and children who have had similar experiences or are living through an illness or debilitation. The inner strength and courage people show in times of adversity can only make me bow my head in admiration...