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C is for CGD

This is not one of my normal blogs, normally we provide amazing updates on the progress of our boys through this long process, this blog is more about a recent experience that left me speechless and started making us think that some people that we meet along our journey really have no idea what it means to have children with a primary immune deficiency.

It all started last week, I had ventured out the relative safety and security of our isolation apartment to grab some filtered water for Levi as he is not able to drink normal tap water until his immunity is 100% operational. Now it’s important to note that Ronald McDonald House is a respite for many families in need with a variety of varied problems, some are terminal and others are not, regardless though, we are all there with a problem of some sort. In the process of filling water, it is common for people to start conversations with you, mostly its idle chatter, though sometimes the conversation gets a little on the heavy side with discussing why we are there and how long we have been there etc. On this occasion, the conversation had turned to the heavy side with the person starting to tell me their life story before asking why I was here. I summarised our situation by simply saying that our little boy was admitted at the beginning of January to have a bone marrow transplant to attempt to cure his genetic disease called Chronic Granulomatous Disease and that we would be doing it again with our second born sometime in the near future. I didn’t expect to be on the receiving end of what happened next….

They looked me in the eye and said “well you must be one of the lucky ones to have a chance at a cure, my kid has leukemia and we have just started chemo for a bone marrow transplant”. For anyone that knows me, you will know that I am hardly even speechless but I was, I had nothing. I simply finished filling my bottles of water and walked back to the safety and sanity of the isolation apartment. That evening it got me thinking, why do some people think that cancer is the trump card of all diseases.

What this person failed to understand about our situation is that where some cancers can lay dormant for years or even a lifetime, most people that have CGD will not make it past their teenage years if left undiagnosed and without prophylactic medication. If I went down to the horse races and placed a $100 on two different horses, one with CGD and one with cancer, I would walk away with $20,000,000 if the CGD horse won, but only walk away with $2,500 if the cancer horse won, those odds are only good if you want to become an instant millionaire, not be diagnosed with a potentially fatal genetic disease.  It doesn’t make me feel lucky. There are very few organisations established to support people with genetic diseases, however there are more than 100 established to support people with cancer. It doesn’t make me feel lucky. Post bone marrow transplant, the boys will be in a higher category of having a cancer later in life than what a normal child would be. It  doesn’t make me feel lucky. Levi won’t have an immune system for the next year and there is a chance that his body will reject the bone marrow transplant resulting in his CGD returning and having to start this process all over again. It doesn’t make me feel very lucky.

It wouldn’t have bothered me if it was an isolated incident as I would have chalked it to ignorance and left it at that, but when I was staying on the oncology ward for his bone marrow transplant it actually happened twice before, with people telling me that it would be far easier to only worry about a genetic disease than cancer, though this was the first time someone told me that I was lucky. Rather than chalk it to ignorance, I am going to give the benefit of the doubt and say that it is more likely to be a misunderstanding in that what people don’t realise is that the majority of cancers are actually caused by a defect in the bodies genetic structure. Yes some are considered to be hereditary, but even then, you could say that is genetic as well. The treatment for CGD is not dissimilar to that of a leukemia patient, with the exception that Levi did not require total body irradiation (TBI) prior to the transplant, he had a partial body irradiation. However he is required to stay on some of the harder medications for a lot longer to minimise the chance of graft versus host disease (which is as it sounds, the host graft fighting with the donor graft as it is invading the hosts body) which is encouraged in leukemia patients but can be detrimental to CGD patients. These medications place a burden on vital organs and cause a swagger of other issues as we progress along his path of recovery. In addition, a patient that is receiving a Bone Marrow Transplant that is not considered an oncology patient does not have the same access to pain medication that would normally be available to an oncology patient which requires the doctors to jump through more hoops to make the process bearable for the patient.

Unlike a cancer patient, Levi won’t have the luxury of playing in a backyard for some time or have normal interactions with other children with the exception to his little brother due to the risk of infection, that could easily compromise his graft and result in a rejection. I am sure if Levi understood luck, he wouldn’t feel very lucky right now.

It shouldn’t be a case of “my dog is bigger than your dog”, or “my cancer is more news worthy than your genetic disease”. As one of our doctors told us at the start of this whole process, “people may try to talk up their situations, but rest assured, everyone here is in just as much of a crap situation as the next, it is all relative”. I wish that we were out of the woods, but it is a long road to recovery and ensuring that the boys can have a normal life, with the exception of the monthly visits to Sydney Children’s Hospital for the rest of their lives. It is imperative for the message to go out that children with genetic diseases, that include having a primary immune deficiency, are just as hard off as a child with cancer. They don’t want it, nor should they have to have it. It is not fair, but one thing I have learnt through this process is that you have to roll with the punches and play the hand that you have been given.

With all of this aside, there are something’s that we feel lucky about, our amazing donor that gave his bone marrow expecting nothing in return, our wonderful families and friends that have given us love, comfort and warmth through this process, and mostly, the love that our little family unit has for each other for every minute that we have together.