Kara Ayik’s sons have now grown up and flown the nest, and a new phase in her life is beginning. Here, she reflects on how ASMD (acid sphingomyelinase deficiency) has shaped her sons’ relationship, and looks forward to a special Christmas gift that will be a reminder of something much greater…

An empty nest

2022 marked my transition into an empty nest, with both my sons, Evren and Erol, now in college and living away from home. In honouring this new phase of life, I decided that this Christmas I would create a special gift for myself. I am having a mother’s ring made.

For those unfamiliar with mother’s rings, they are typically created with one birthstone representing each child’s birth. Coincidentally, my sons’ birthstones are a garnet and an emerald. Yes, that’s right. My mother’s ring will have Christmas colours, and I will proudly wear it year-round.

When I contemplated the stones together, red and green, it dawned on me that the gemstones’ colours are on opposite ends of the colour spectrum, and therefore complementary. It is a fitting symbol of my two sons, who are also quite different but whose relationship has helped them to bring out the best in one another.

My sons ended up on opposite ends of a spectrum largely because of genes. Genes are funny, and I never stop marvelling at the roll of dice that is genetic inheritance. Both of the boys had just a 25% chance of inheriting two copies of the genes that cause ASMD (acid sphingomyelinase deficiency). One inherited both copies, and the other, neither.

Bringing up rare brothers

Many who first learn that the boys are brothers are rather shocked because Evren and Erol do not look alike at all. Not only does Evren have a different hair colour and skin tone than Erol, but Evren’s ASMD also made his outward appearance somewhat different, including his facial features. Their personalities are also quite distinct. Evren is a quiet, calm and dreamy boy, whereas his brother Erol is mercurial, active and intense. But both boys are kind and caring people who seek a meaningful and fruitful life.

I would call them rare brothers. Now, rare brothers have many things in common with ordinary brothers. Over the years, they have supported each other, fought with each other and certainly stressed their mother out on countless occasions. But Evren’s ASMD has shaped their relationship in some very distinct ways, as would any rare or chronic disease. For example, I had to work to make sure that the typical sibling fighting never became too aggressive, and the very few times it did meant cause for immediate and assertive parental intervention. Because Evren has ASMD, he has an enlarged liver and spleen, so typical brother behaviour of rough-housing or a punch in the stomach could prove fatal.

In fact, that tragic fate happened to a pair of brothers, one of whom had ASMD. While playing too aggressively, the brother with ASMD received a blow to his spleen and passed away from the complications. When I learned of that story from a research paper, I was terror-stricken. Although I wanted to keep the story a secret to preserve normalcy and to avoid instilling fear in my sons, I had to share a version of it with Erol, who was a little hot-tempered, so he would know how dangerous it would be if he lost his self-control.

A hidden burden

So the end result of Evren’s ASMD was that Erol grew up with undercurrents of fear and anxiety, especially during the times when his brother was hospitalised. He grew up fearing for Evren’s health and mortality. I didn’t know this though, until fairly recently. Erol never so much as mentioned a word about how he worried about his brother’s health while growing up.

In hindsight, I should have known. I could see Erol observing Evren’s life from the time the boys were both very young. Erol grew from early childhood to young adulthood witnessing the suffering his brother experienced on many levels, physical and emotional. The physical impacts of ASMD are many, and just as hard, children and teenagers are not always nice to anyone who is different. Evren also had endless strings of appointments and medical interventions, many of which Erol attended, especially when they were younger. Once Erol surprised me by telling a friend that Valley Children’s Hospital was “where we spent half our lives growing up”. Although his words were a gross exaggeration of our actual reality, it struck me to realise how deep an impression our visits to the hospital had left on Erol.

With the adversity that accompanies rare disease, though, comes the opportunity for an exceptional sibling relationship. Rare siblings have something extra special, something that deepens the sibling relationship and bonds them even more closely than most typical siblings are. And not only do rare siblings mature faster, but their worldviews expand in impressive ways, too.

While in high school, the boys took a genetics class together, Erol as a sophomore and Evren as a senior. When the teacher assigned the students to prepare a presentation on a rare disease, Evren and Erol paired up for ASMD, although Evren more or less took over the project, which surprised Erol, who is usually the leader in any cooperative assignments. Erol quietly told me that he understood Evren’s assuming almost total control over the project. Erol intuitively knew that his brother’s rare disease was a significant facet of his brother’s identity, and Evren needed to have control over how the ASMD was shared with the class—what information to share, and what to hide. At that time, Erol was just fourteen, so his empathy and insight was remarkable.

Some time ago, I asked Erol, “Do you feel like you were kicked to the curb because of Evren’s ASMD while you were growing up?” Erol replied, “Of course I did. But what other choice did you have?” I thought about his answer for some time, wondering what I could have done differently.

Months later, in a follow-up conversation, Erol explained that he did not mean to say that he felt neglected or cast aside, but that he often felt like he didn’t know what was going on with Evren’s health, and that left him worried. “Maybe I was just too young to understand anyway,” he said. I simultaneously felt relieved that Erol did not grow up feeling invisible, but also troubled that I was not more perceptive about his feelings of fear and anxiety.

The moral of the story, for me, is to practise what I preach. And that is, tell children the truth in a way that they can understand it. 

Even if it is uncomfortable or painful, it is easier to process the truth than it is to be left in a vacuum, a sort of black hole without any information to begin processing the reality of a brother’s or a sister’s chronic disease.

The brothers’ deep and loving bond

Ultimately, both boys drew what they needed most from each other. Erol found great inspiration in his brother’s brave perseverance, which also gave him insight and perspective. Erol is a young man who does not succumb to self-pity, peer-pressure or behave in shallow, excluding ways. And Evren has certainly found a source of great support and joy in Erol, his loyal younger brother who loves him deeply and unconditionally. Evren is proud of his brother’s achievements, and together they have had some wonderful adventures. This summer, Erol attended his first-ever in-person conference for ASMD with Evren. Two years prior, I had to nudge Erol to attend his first family conference online during COVID, and he was astounded to learn how much it meant to meet other people his age whose siblings also had ASMD.

I am so excited to see my Christmas ring. I believe that when I look at it, it will call to mind the joy I feel over the greatest gift I could have ever asked for: my two beautiful boys. The icing on the cake is that the two boys I love so much also share such a deep and loving bond with one another.

---