From tube feedings to tacos

Eight years ago, now. I still remember how surprised I was that my baby couldn’t eat. And how sad I was about that. Food is so much more than sustenance – food is also: togetherness, sharing, smelling, tasting, enjoying, discovering, quenching hunger, growing, celebrating…

A couple of hours after Vera’s birth, a feeding tube was put in from her nose to her stomach. After a couple of months, that feeding tube was replaced by a port in her belly that connects to her stomach. These feeding tubes were her lifeline because, like most children with Costello Syndrome, Vera wouldn’t eat. She tried, but her fine motor skills and sensory issues got in the way. Her cell signals are different and do not send a message to the brain that food is needed to survive. Tube feeding was the only way for years.

We have gone through quite a journey in this with Vera. For those who know us or have been reading along with us, you may remember we went from pumped breast milk through the feeding tube to special tube feeding formula that caused vomiting and diarrhea, to discovering the blended diet: simply putting our daily meals in the blender and using syringes to feed Vera. This brought an enormous improvement in Vera’s quality of life. She received real food through the feeding tube, precisely the same healthy food we were eating. Seriously, when I start talking about the blended diet, I sound like a total spokesperson. And in the meantime an entire team of therapists worked alongside: pre-speech and speech therapy (yes, speech therapists do not only help with speaking but also with learning to eat: the movement of mouth and tongue are crucial in learning how to eat), there were play sessions with food and Vera did all sorts of exercises to learn to deal with different textures.

When you have a child with disabilities, you quickly discover how complex seemingly simple and mundane everyday tasks can be. Learning to eat proved to be this complex system of feeling hunger, smelling food, tasting food, feeling brave enough to touch food, being able to endure the textures of food in your mouth, making the right movement with your tongue, chewing and swallowing and all of this without gagging or choking. And then to keep eating and not be done after just a couple of bites. Vera has also had feeding therapy at school. She would go to a special room and practiced eating one bite of a sandwich and then would return back to class. As parents, we also received great support from the EAT-team at Rijndam rehabilitation.

bij David’s Gelato

There were lots of fun moments: we may have gone to the gelato place in town way too often over the course of a couple of summers because ice cream was just about the only thing Vera enjoyed eating. There were some tricky moments: Vera could eat an entire piece of cake with whip cream at a birthday party, but when we tried handing her a boiled egg at home, she would gag. When she really wanted to join us in eating our dinners together, but simply couldn’t and would get frustrated. It was a long and slow process with some steps forward, but then with illness, a big slide backwards again.

But we were making progress. At a certain point, she got enough food in her so she no longer needed a tube feeding at school. In the mornings and evenings, she would still receive healthy homemade tube feedings with lots of vitamins to round out her caloric intake.

And then came the big stay at home period due to COVID-19. After some initial confusion, Vera seemed to be doing well with staying at home so much. Everything was very predictable, the family was always complete and Vera had more energy. To our big surprise, she used this energy to focus on eating. Eating more, eating new things and crucially, eating a big enough dinner. There were days with some trepidation that we could skip the evening tube feeding because she really did eat a decent amount of food. Days where we could skip the morning tube feeding because she ate enough breakfast. And suddenly we realized she hadn’t had a tube feeding in 5 days. All of a sudden, a week in which we didn’t use the blender to make tube feedings. And 5 days turned into 5 weeks without feeding Vera through her Mickey-button in her stomach. What a milestone! Even some of her medication Vera now swallows on her own. Some other meds are still too difficult (a pill that is too large and liquid heart medication that is critical and has to be an exact dosage).

There are still some bumps in the road here and there. But currently, we no longer regard Vera as a tube fed child, but as a difficult eater. Like with many children: fruits and vegetables are the hardest and we use tricks like hiding them in soup or muffins or with some whip cream on a banana. But that is okay, it is a new phase. Vera still has to gain lots of experience in eating normally. But it is good enough. And good enough is fantastic.

For now, the mickey-button in her stomach stays where it is. We like to see how things will progress for a couple of months. To really see and experience that this is the new normal. And we will also have to see how things will go when she is tired, ill or tense. Whether things will continue to go well, and we can really start living without tube feedings. Vera has had the mickey-button for eight years now. It’s a part of her and we don’t need to get rid of it just yet. Vera also perceives it as a necessary part of her body even though she has now asked us a couple of times whether it can come out. Regardless: I think it’s time for a return trip to our local gelato place or for cake because we definitely have something to celebrate!


Positive developments

“Have some leniency with her”, Vera’s eye doctor said at the beginning of her life. What he meant is that we needed to be patient. That she really would develop, but that it would take time. More time than with a typical developing child. And he is right, slowly but surely we can see an upward trend with Vera.
One of the main characteristics of Costello syndrome is developmental delay. Everything is very slow (or in some cases doesn’t develop at all) and we see this in Vera also. But if we look at all the facts, we can see several jumps in her development.

The first jump was early in her life, it was the moment she was no longer in intensive care and came home with us for a couple of weeks. During those weeks we saw progress, until another hospital stay followed and things halted again.

Vera met sonde

Vera met sonde

Sonde Vera er uit!

Sonde Vera er uit!

A real jump came the moment the feeding tube came out of her nose because she received a PEG (feeding tube in her stomach). The negative sensations in her nose and throat were gone. This really benefitted her, in particular in making sounds for instance.

On vacation in the summer of 2013 we noticed she was feeling well and that her development continued. Being outside often with nice weather and always having people (without white coats) around to smile at were exactly what she needed and are things that she enjoys.

Zomervakantie met Vera

Zomervakantie met Vera

There have not been any more hospitalizations since October 2013 and a new development in play and discovering the world is taking place.
And then the biggest step to date; this February we made a change in her food. With tremendously positive effects. In the past there were problems with her bowels, she had cramps and ongoing diarrhea. A real solution had not been found. She would often spit up food and mucus several times a day; for sure in the mornings, but also often in the middle of the night so that we had to change all her bedding.
From our closed Facebook-group for parents with a child with Costello syndrome we got the tip that the special tube formula for children who are lactose intolerant caused problems in some children when used for a prolonged period of time. It is unclear why. After all, it is the most allergy free food in liquid form that contains all necessary nutrients. In consultation with our main pediatrician we stopped the special formula for a couple of days as a test and substituted with pure soy milk as her main source of nutrition.

The effect was that within a couple of days she stopped spitting up her food, produced less mucus and for the first time of her life had a solid stool. Her skin calmed down, was less splotchy and even somewhat softer.

Naast medicatie geven we ook groentensap via spuitjes over de sonde

Naast medicatie geven we ook groentensap via spuitjes over de sonde

A week after, she even slept through the night for the first time in her life. Unfortunately, the latter was just the one time.

It made such a big difference that we have switched to our own food. In the USA a so called ‘blended diet’ is used: you puree your own food in the blender so it can be administered through a feeding tube.
And so now we are in the kitchen for an hour every day weighing grams, calculating the calories, taking notes in a food diary and pureeing with the blender or the slow juicer (thanks to donations!). Grinding up sunflower seeds, mashing avocados, pureeing cooked rice, juicing vegetables: it all turns into beautiful porridge.
Instead of using the food pump with a very slow drip of two hours so she wouldn’t spit up, feeding her now only takes 20 minutes per feed.
Only the first and the last feeding of the day are still by food pump (soy milk with baby cereal), but we no longer have to get up out of bed in the middle of the night to turn the pump off and rinse all the equipment out…


Slowjuicer zodat groente en fruit vloeibaar over de sonde gegeven kan worden











It seems like making your own food would take more time, but not having to change her bedding and not having to change her clothes at least twice a day due to her spitting up actually saves us time. And above all: it gives Vera so much more quality of life that we gladly do this for her.
And because she no longer has diarrhea and is no longer spitting up, she is even more cheerful and likes to explore more. She is even more interested in solid food. So with every meal, she joins in and tastes, sometimes she chews a little and sometimes she even swallows a little. And, at two years of age, this is a very good development for someone with Costello syndrome.

Video food therapy: