Always different

Sometimes I look at Vera and all I see is her big smile. And even though she looks different from an average child, I see a small, perfect girl. vera-lachtstrip

I make sure to enjoy those moments. Just like the times when strangers in the street think I am pushing a large baby in the buggy and I do not correct them.

Because even though we are (fortunately) currently not dealing with health crisis after health crisis like in the first two years of her life, there is still so much to take into account.

I do not like to complain and most of the time I manage to stay positive and focus on everything that is going well and enjoy the moment. And I don’t want to be an overly concerned mother either, there are times I feel I may be exaggerating in my alertness. But, unfortunately we are constantly confronted with the fact that Vera’s body does not function like the body of a healthy child. When Mieke falls, has a bruise, coughs or is cold, we take care of her and she recovers. But with Vera, our brains immediately open up a large file with areas of attention, possible scenarios and treatments. Luckily, nowadays Vera can take a fall without breaking a bone and we can weather through a cold with home care. But we cannot take this for granted, we are always alert when something is going on.

Since I am/was an art therapist I recently made a drawing of Vera and her body. For each body part I wrote down the issues we need to pay close attention to. It helps me to have an overview. And I am sharing the drawing here, so people can understand why we make certain choices and why we have to be careful. Perhaps you can light a candle for Vera and the body she has to live in. It also helps to give you a visual of all the things that are going on and we may not always talk about. Images are very helpful this way.

When you look at the drawing, understand that this is part of our lives. But when you look at Vera, make sure you mainly notice her big smile.


costello syndroom meervoudige handicap


Operation successful

Lead up

We have known for about two months now that Vera would need an operation. We have been anticipating this with mixed feelings of looking forward to it and dreading it at the same time.
We were looking forward to it because the operation would solve and prevent issues. And when we knew it had to happen, we just wanted to get it over with as soon as possible. Preferably at a time when Vera was doing well, so she would withstand the operation better.
We were dreading it because it is not a nice experience for Vera and for us it means chaotic and tiring times ahead. This week it was go time. We received a phone call on Tuesday and were asked if we could report to the hospital at 8:00 AM the next day; the only time slot available where all physicians involved would be present at the same time. It wasn’t even that bad to know with such short notice. Thankfully, grandpa and grandma de Zwarte were able to watch Mieke, things could be arranged at work (like always, fantastic employer!), bags were packed and we were ready to go.
Three specialists were needed in the OR. First, a port (portacath) was placed, second, the throat-nose and ear doctor placed ear tubes in Vera’s ears and last, the dermatologist removed a wart from her cheek. The ear tubes are needed because Vera has a narrow ear canal and has had several bad ear infections, which led to sleepless nights because of pain lying down. The ear tubes will prevent future ear infections.

Port (or portacath)

The port requires some explanation: schematische weergave PAC
a port (portacath) is the main form of a central venous access device. The term: totally implantable venous access system (TIVAS) is also used. ‘Port-a-cath’ is a brand name. A port (or portacath) is a small medical appliance that is installed beneath the skin, in Vera’s case on the side of her chest right under her left arm. A catheter connects the port to a vein. Under the skin, the port has a septum through which drugs can be injected and blood samples can be drawn many times. schematische weergave PAC The reason Vera needs this is because Vera’s veins are very hard to find and starting an IV-line has been very troublesome. During her last day treatment for her porous bones, Vera ended up needing general anesthesia to get an IV-line in (see this blog post). General anesthesia is not desirable because it is not without danger for Vera because she has a narrow trachea. Every time Vera needs treatment and a vein cannot be found quickly, it is risky. With the port this risk is taken away, there is always direct access. And since Vera needs an IV-treatment for her bones (with so called bisphosphonates) several times a year, it is a good thing that the port was placed. So, it was an unpleasant procedure once to make it possible to give her medication through an IV or even take blood more easily and repeatedly in the future in a much more comfortable way. In the future, when treatments are no longer necessary the port can be removed.

Vera ready for the operation

Daddy takes Vera to the OR

Operation successful

The operation went well. Although sad to see that the doctors had ‘poked’ Vera everywhere to find an IV-line for the general anesthesia: little holes in both hands, feet and on her head were visible. Another confirmation that placing the port was very necessary.
In the OR the doctors noticed how irregular Vera’s heartbeat is, a known issue for our pediatric cardiologist, but to a doctor who sees this for the first time it can be quite unnerving. Her blood pressure also dropped at one time, which is not usual for her. During recovery the anesthesiologist wanted to keep her a little bit longer in the recovery room to monitor this and to adjust her medication accordingly. It is known that children with Costello Syndrome react differently to pain medication and experience pain faster, so more is needed. Several hours later we were out of recovery. Vera’s breathing was quite noisy which was worrisome to us and the doctors. They were the same noises that landed her in Intensive Care several times due to breathing problems. At the same time, it wasn’t so bad when she was calm. We had a very good consultation with the pediatrician where our experiences with Vera were taken seriously in consideration. Because Vera has responded well in the past to the anti-inflammatory drug Dexamethasone and we figured she could have an inflamed trachea due to intubation for the anesthesia, it was decided to give her this medication. In the meantime, the ICU was notified just in case, which made us nervous.  room in hospitalBut soon Vera responded very well to the medication. And ended up sleeping better than ever that night (with me next to her, see photo of our nice room). And, like so often with Costello-children, the next day she had improved tremendously. A true miracle and big difference with the day before.

Let’s go home

Because we heard Vera’s normal breathing sounds again the next day, we consulted about taking her off the Dexamethasone again. They agreed, took her off and things went well. Vera started babbling again. When we were moved to a different ward with other children Vera was highly interested in the other babies.
At the end of the day we were released with some instructions on how to care for her further at home. Because, when it’s medically possible, home is where you recover best.
When Vera realized we were going home she started signing (thanks to the children’s sign language DVD’s from ‘Lotte en Max’) enthusiastically. She signed the signs for: ‘go outside’, ‘car’ and ‘home’ with a twinkle in her eye and her familiar ‘yyyyyes!’
And so we did. Go outside, into the car and home. Yes!


logo WKZ
In our eyes, the type of care at Wilhelmina Kinderziekenhuis (WKZ) (Wilhelmina children’s hospital) is fantastic. The fact that 3 procedures were possible for us in one hospitalization for instance. We always get solid preparation and explanation ahead of time, which gives us a lot of trust. Doctors welcome all questions and respond very well so that you feel very involved as parents. And during hospitalization they are always alert and take effective action. A top-class hospital!


Rare disease, exceptional support

On February 28 it was the international Rare Disease Day. Rare Disease Day is an observance held to raise awareness for rare diseases and improve access to treatment and medical representation for individuals with rare diseases and their families. Costello Syndrome is one of these rare diseases. Of course we keep track of medical news ourselves, but the day went by like any other day.


avatar Rare Disease Day

On Facebook and Twitter we noticed people changing their avatar to raise awareness for rare diseases and often for their own child with a rare disease. See an example on this page.
For us, raising awareness for the rare syndrome our daughter has is not a one day thing: through this website we are continuously trying to show others what it is like to have a child with a rare disease. We are extremely happy to have so many people around us who follow the site with interest and by doing so we feel supported by them. I am not sure if the term “digital caregivers” exists, but to us it is real. People who care and show support make a big difference in our lives, because just feeling acceptance helps a lot. It makes it so we don’t feel uncomfortable talking about Vera’s disease and it prevents us from feeling isolated.

One of the ways in which we have felt this and have come across exceptional support is by receiving donations from many sponsors. We have received financial support from a great variety of people. Because of this we were able to purchase several practical things that help make Vera’s life better: DVDs with sign language (Dutch version, see Lotte and Max) so she can communicate better (we will tell you more about this later!), a PT/play mat that’s easy to clean, a slow juicer to prep vegetables for her tube feedings, an extra small walker, a winter leg warmer/cover for the custom stroller, financing medication that is not covered by insurance and covering expenses that come with the many hospitalizations. For this we thank you! It has a huge positive impact our whole family benefits from.

And on the morning of Rare Disease Day two sweet sisters from our church rang our doorbell and delivered fresh apple pie, flowers, a 3 month supply of diapers and more presents. There was a card signed by many members of our church with encouraging messages and greetings. Incredibly heart-warming. A day we will never forget…
Besides a rare disease crossing our path we also come across exceptional support from many sides. People who walk along, help and encourage. Caregivers in many forms. Either digitally or in physical form. With warm words, practical help or a wonderful donation. A beautiful side of people full of love. We cherish this.

Vera op speelmat

Vera op speelmat (tevens voor fysiotherapie)

Vera op loopkarretje

Vera op loopkarretje

trappelzak voor aangepaste kinderwagen

trappelzak voor aangepaste kinderwagen

zeldzame ziektendag cadeaus

cadeau’s van de kerk voor Zeldzame Ziektendag