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From tube feedings to tacos

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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!

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Update October 2017

Hello everyone!

It has been quiet on the blog for about 6 months, but we certainly haven’t been sitting still. On the contrary, a lot has happened and luckily, mostly good things. This blog post will mainly be informative to update everyone. In the end, it turned out to be 5 pieces of information, I could (or should) have written 5 different blog posts. At the same time, this is very indicative of life with a child that needs special care; it’s not that sometimes something is going on, something is ALWAYS going on – and often many things at the same time. So, once again, welcome to our world!

House

Vera ready to move

We moved! And even though we haven’t unpacked all of the boxes yet, we are very happy with the move. Our new house is exactly what we were hoping it would be: it’s more spacious, and Vera can get around easily with her walker and wheelchair, there is a changing station downstairs and she can use the front door she can use with her wheelchair without us having to lift her. There is a spacious bathroom and luckily, the house is also just a pleasant place to live. Lots of people showed up to help us with the move and we can’t thank everyone enough for lifting boxes, cooking, working on home improvement projects and just thinking of us in general. Both girls are getting settled nicely and so are we.

Heart

Vera with halter monitor

Prior to the move, at the beginning of summer, there was some concern regarding Vera’s heart. She was born with severe heart issues; a heart that beats very infrequently and at the same time at too high a frequency and can start racing. This was controlled fairly well with medication, although her heart was still beating too fast for her age. In the spring we found out that Vera acquired a new heart issue, not only does she have arrhythmia in the atrium, but now also in the ventricles. Which is even more dangerous, if the ventricles start racing, this could be life-threatening. This came as a shock. We practiced our resuscitation techniques again, just to be prepared. And we had a meeting with the cardiologist. The medication that will help with this is Flecainide. However, when Vera was only a few months old, her levels of this medication were too high and caused cardiac arrest. We were there when this happened at the intensive care in Amsterdam and we will never forget that moment. Luckily, she was in a place where immediate medical attention could be given and with resuscitation and medication, she narrowly made it. “Never again this medication” was always on her medication list in big, bold letters. But now, with the added issue of arrhythmia in her ventricles, we couldn’t just do nothing. And there was no alternative medicine. The cardiologist still felt it was the right course of action and the situation was different also now, 5 years later.

in hospital for ECG and blood tests – always with a cookie afterwards

And so, in a very controlled way, we did end up starting with the Flecainide.
We started off with a very low dosage and made a weekly trip to the hospital for an EKG and blood draw to identify medication levels.
At home, we had a heart monitor to monitor her heart at night. And even though it was quite intrusive to have a heart monitor at home with all the beeps going on at night, it also gave us some reassurance. We dared to go to sleep because if something was really wrong, the alarm would sound and we could take immediate action. Each week we had one night where Vera was being cared for by a nurse (as always) so we could sleep for 8 uninterrupted hours knowing Vera was in good hands.
Over the course of the next couple of weeks, we gradually upped the medication levels. And, as is often the case with children who have Costello Syndrome, Vera needed quite a high dose to help alleviate the problem. But once this happened, it was good. Really good even. Her latest heart test showed no arrhythmia in the ventricles and her overall heart rate went down by 30 beats per minute.

heart monitor at home

This news almost sounded too good to be true. With the fear of the heart issues she had at birth and the cardiac arrest episode engrained in our memories, we could breathe a sigh of relief. Thank God! Her values have never been this good in her life.

This saves her body a lot of energy, beating 30 beats less in an hour means the heart isn’t working so hard and is beating 43,200 beats less in a day… We felt comfortable enough to get rid of the heart monitor and we will continue her semi-annual check-ups with the cardiologist. Because, even though her heart is functioning a lot better, it still isn’t functioning normally.
People often ask us if Vera can have heart surgery. The initial answer is that for now she is still too young and she doesn’t weigh enough yet, but hopefully: yes, maybe one day.
Vera’s heart nodes give off false impulses to the heart. When adults have this condition, a procedure can be done where the abnormal tissue that is causing the false pulses can be ablated (destroyed). Except Vera has so many false pulses that they can’t ablate all of it. But, who knows? Maybe one day this could be an option to get her heart to quiet down. But for now, this is not expected for the near future.

Eating

Before the summer, we also started eating therapy at school. Of course, essentially Vera has received eating therapy her whole life. From birth on (with the guidance of speech and language therapists who also specialize in swallowing difficulties) we have been practicing with bottles, spoons, licking and sipping.

tasting everything

Vera has always joined us at the table during meal times and we have always given her food to participate. And since birth, things have improved. Initially, she couldn’t bear any sensations around her mouth (except her pacifier), gradually she started touching food, holding food, licking food and putting food in her mouth. And eating a little bit. But definitely not enough yet and not the right kind of food to give her sustenance. And so, she has received her nutrition through a feeding tube for all of her life. First through her nose, later and to this day, through a mickey button in her stomach. 7 times a day we administer food or fluids or medicine through her mickey with a syringe.
At Vera’s school, there is the so-called EAT team, they support her language and speech therapist and us to help her learn to eat. What this means is that we essentially go back to baby food, porridge, and yogurt. Vera used to always spit this out. With eating therapy, she has started taking a spoonful of food in this little room at school and really eating it. Success! Fast forward to 6 months later and we’ve gone from the one spoonful to 2 fluid ounces. She now eats this every day. And we keep practicing with different foods, we give Vera anything she wants to try and will really eat. Even if that means it’s a cookie with whip cream. So that eating becomes a normal part of life and so Vera learns to get used to different tastes and textures. But it’s also about the technicalities: learning how to move the food from the front of the mouth to the back and actually going through the motions of swallowing it.
On the one hand, 2 fluid ounces seems a lot for her to eat by herself. On the other hand, this is still a far cry from being able to eat enough to really feed herself adequately.
We will still have to give it more time and patience, but she does seem to make progress.
Additionally, Vera also receives occupational therapy at school. Her eating issues are in large due to sensory sensitivities and occupational therapy helps with this. Vera’s body reacts to different stimuli in an overly sensitive way. Sound, touch and visual stimuli can be overwhelming for her. And that ties into how she deals with food. So, in therapy, she practices to get to know different sensations and not feel overwhelmed by them. In this way, playing with shaving cream with your hands contributes to learning to eat.

Sleeping

…or lack thereof. Another issue that Vera has had since birth. Vera is a restless sleeper and often wakes up crying at night and is done with sleeping at an early hour. We have already tried many things: Making her room darker, lighter, no noise or white noise in her bedroom, not too much food before bedtime or more food before bedtime, a nice bath and massage before bedtime, a weighted blanket, letting her cry longer or letting her cry shorter, making the room temperature warmer, colder, etc. She now takes sleep medication, which gives her two stretches of sleep a night, but still restless and interrupted. She never sleeps through the night. Needless to say, we are very tired often and Vera was getting headaches from lack of sleep. The neurologist referred us to the sleep clinic in Ede. They are specialized in sleep issues in patients with mental disabilities. It took some effort to get in (because who was going to pay for this?). And once we were in, they gave us quite a bit of homework. We filled out extensive questionnaires, kept a sleeping diary for 4 weeks, Vera slept with a machine that measures her movements at night for a week, and we took saliva samples that went to the lab to measure melatonin levels.

melatonin research

Sleep issues are difficult, not only because they make you very tired, but as parents, you feel so powerless that you can’t help your child fulfil this basic need. You start wondering what you are doing wrong and why it’s not going away. What’s going on? Is she in pain/is she hungry/is she scared? Or are we exaggerating and it’s not that bad?
The outcome of all the testing was quite illuminating. No sleep apnoea, no epileptic seizures. But we did find out that Vera’s body does not make any melatonin in the evening. She falls asleep because she is tired from the day, but not because her body gets sleepy. And that’s why she can’t stay asleep. The results from the movement monitor showed that she only really sleeps 54% of the time that she is in bed. When I heard that I felt so sorry for her. we go to her often at night, calm her down, tuck her in. And now it turns out that she is awake even more than we thought. And she must be so tired during the day.
So now we give Vera melatonin before bedtime, to replicate the natural process. We later found out that she doesn’t break down the melatonin during the day, which can also create issues with sleeping through the night. So, we switched her stomach medication to one that breaks down melatonin which she receives in the morning. For a while, it seemed we had found the solution. But, unfortunately, Vera’s sleeping pattern still varies and she continues to need sleep medication to even have a couple of stretches of sleep. The melatonin does give us hope, it feels like a step in the right direction. We are hoping it will lead to a more well-rested lifestyle for all of us.

Feet

casting in hospital

Right around her 5th birthday, Vera was able to walk unassisted for a bit. She needed her custom shoes and a hula hoop for balance but was able to take small steps through the living room. Unfortunately, the positioning of her feet got worse after that, which made her less stable and walking became harder again. Thankfully, she did continue to walk well with her walker. It’s a common problem in children with Costello Syndrome; Vera has shortened calf muscles and her Achilles heel is too tight, which makes her walk on her toes, her heels were not touching the floor anymore. She also has splayed flat feet (sounds charming, doesn’t it?). The physiotherapy and orthopedic doctors referred us for casting. This means that every week, Vera’s feet are positioned as best as possible and then put in leg casts.

series of her casting; right was the first one

By the next week, her muscles and tendons are stretched a little bit and the process is repeated. We were not looking forward to this and were concerned it would impede her mobility or that it would be painful for her. But again, our little veteran is a trooper; she creates cheer for everyone in the casting room and she enjoys picking out a new color and glitter for her next casts. And at home and at school she is fully using her feet, she walks on the casts, does her physical exercises with her casts and accepts it fully. She only misses her nightly bath ritual and she is now heavier and clumsier for us to lift. She is currently in her 5th set of casts and we can see enormous improvement. Yay! We will continue this for a couple more weeks and after that Vera will get splints in custom shoes. This should help keep her feet in the correct position, so it doesn’t get worse again. And hopefully, she will walk better and better.

So in short

We are living in a great new house, with heart medication that is working well, we are busy eating baby food and are not quite sleeping at optimal levels and Vera keeps taking steps. Onwards we go!
Lots of love from all of us!

 

Some pics with her sister: