Emma has been lucky so far as she has not experienced a single issue with her shunt. This is not the norm as more than half of the shunts placed fail within two years. That statistic has been in the back of my mind since Emma was born. Other statistics show about 40,000 shunt-related surgeries are performed each year costing over $1 billion in the United States alone. These numbers are staggering when you take into account the limited knowledge most people have about hydrocephalus. This can make it a little more challenging to learn about the condition without the support of doctors and websites like Hydrocephalus Association (both of which I am grateful for).
As we approach her second birthday, I am doing my best to make sure I am prepared for when (not if) she does have her first shunt malfunction. I know it is coming and I know that time is extremely important when it does happen as mere hours can be there difference between a revision surgery and brain damage or death. Some of the symptoms of a malfunction, for a child that can not verbally communicate how they may feel differently, are very subjective. If you have spent enough time with Emma and know her personality as well as her normal eating and drinking habits, you are more likely to notice a change in them. Decreased appetite or lethargy are symptoms that are difficult to gauge as every child does have a normal change in these habits over time and even day to day. The same goes for a fever - it can be a symptom of a malfunction, but it can also be a sign of so many other things that it is hard to know if the fever should just be treated with Tylenol or a cause for concern.
Shunt malfunctions are most commonly caused by a partial or complete blockage of the shunt. An obstruction can occur in any part of the shunt, but in children it is most common in the part that is in the brain. You also have to consider the possibility that parts of the shunt can become disengaged or broken due to wear and growth. No matter the cause, there are symptoms to watch for. If the child is young enough to still have a soft spot (or in Emma's case, still does due to her increased head size before the shunt was placed), then you can watch that area. If it becomes full and tense, the likelihood is that there is an increase in pressure from fluid that is causing it. Once the soft spots are closed on children, this sign is no longer valid. Other physical signs on the head to watch for are swelling or redness of the skin near the shunt as well as bulging veins. Other symptoms include headaches, vomiting, irritability, and tiredness.
To correct a shunt malfunction, revision surgery will be required (and possibly more than one). After any shunt-related surgery, there are risks for shunt infections. However, they usually happen within six months post-op. Other complications to watch for include the shunt system draining the cerebrospinal fluid (CSF) at the wrong rate. Not draining enough and draining too much are both cause for concern.
In an effort to monitor her shunts' functionality, Emma has rapid MRIs done on a routine basis. Her next one will be on March 7th so we are keeping our fingers crossed for good news. With all of her development, I couldn't be more proud of how far she has come already. So until her birthday, we'll continue to play with her new favorite toy: BALL!
