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Merry Christmas and good night

December, a month of looking back at the year in review, counting challenges and fortunate breaks we had. We have a lot to tell you about 2019. But by far, the number one notable thing has been: getting a good night’s sleep. Because (spoiler alert!): We are sleeping! I want to frame this phrase and hang it on our walls.

Last January, we noticed that seven years of poor sleep was starting to take its toll. And not just for us, but for Vera herself as well. She was having a hard time, crying more often and having behavioral issues. In consultation with Dr. Pillen of the Kempenhaeghe Centrum voor Slaapgeneeskunde (Center for Sleep Health), we started a medicine called Gabapentin that is usually used for epileptic seizures. Vera does not have epilepsy, but this medicine helps numb the sensory stimuli. And that was the main reason Vera was unable to have deep sleep cycles, along with some throat, nose and ear issues.

Vera was admitted to the hospital to start the medication and regulate the dosage because this medication can have an affect on heart rhythm. And Vera’s heart rhythm is being held under control by two separate heart medications. We did not want any disruption to an already hard to manage irregular heart rhythm. So we had a couple of intense nights… Would it work? Would it not interfere with her heart? We almost didn’t dare hope it would be successful. But it’s working. It’s helping I could say also. Because, as with all medication that Vera receives, with it we try to get to normal values. And now Vera’s system can finally relax at night. You can even tell her body is less restless physically in bed. And since Vera has been sleeping better, things are going better. She is eating better, there are fewer behavioral issues (even though that is still something we are working on) and we have received reports from school that she is more alert and present in class. Is everything going well all the time? No, Vera is still an early riser. And when a lot is going on in her life (such as a teacher being sick or having different drivers that take her to school and not one consistent one) we notice this in her sleep. But it certainly has not been as bad as it was before the medication. As Dr. Pillen said: “You have to see Vera’s sleep as her weak spot.” It is not atrocious anymore, we now have a basic night’s rest, but when life gets unpredictable or there is a lot of change, her sleep will not be as good. So that’s where we are at. We have been getting a normal night’s sleep for months now. It’s almost like emerging from a storm cellar after a hurricane has blown through and we are looking around us thinking: What now? Well, there is plenty to do, but now we have the energy to do it.

For instance, we have been working with the city of Gouda for an extended period of time to get reimbursed for the stairlift we had installed for Vera. It took a lot and many kind friends helped to get this finalized. And if all works out, there will be some accommodations in the shower so Vera can be more self-sufficient with our supervision. This will also really help us spare our backs and arms. Unfortunately, I hurt my back this year (hernia) and Niels has been struggling with shoulder and arm pain for months now. So, accommodations and other aids are no luxury for us.

And then, we received the news that we no longer qualified for KinderThuiszorg (at-home medical care). Vera no longer fit the profile to receive this long-term care. This was very hard for us. Now we no longer had the professional help we needed, no childcare or moments for much-needed self-care and we were on our own. It took a little while before we gathered the courage to ask for a PGB. A PGB is government funding in the Netherlands that long-term caregivers of elderly or people with long-term illness or disabilities can receive to help cover the cost of all that is involved in this care.

It took a lot of red tape and paperwork and Niels took care of most of this. Request forms, creating a budget plan, descriptions of care, care agreements, contracts, an at-home interview and an interview at the care office in Eindhoven. Altogether it was a lot. At this moment it is looking like everything will come together and we will receive PGB. This will give us many possibilities. We can hire people again to help care for Vera. And because I myself am one of Vera’s main caregivers I will receive an income. It amazes me how good that feels. That there is acknowledgment for the fact that taking care of Vera is very different from taking care of an average 7-year-old. Because no matter how well Vera does in her development, she needs assistance in almost every aspect of her life: bathing, changing diapers, getting dressed, eating, playing, moving around. And then there is all the medication, the tube feedings, hospital visits and contacting doctors and therapists. It is a job in and of itself and now that is acknowledged.

And so, we are looking back on a year in which we are finally sleeping, have been able to arrange for accommodations and we get to look forward to receiving the care funding. We are also looking back on wonderful family moments, vacations, holidays and also just the average day-to-day. Those moments you could take for granted but that are so valuable. We are thankful for each other. And we are thankful for all the people that think and care about us.

We wish you all Happy Holidays and a Happy New Year filled with love and hope!



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!


Roller coaster

We have received such sweet and overwhelming reactions to Vera’s website. Thank you! Several donations have come in already for which we are so thankful and happy. Thank you again! Unfortunately, we have had to switch quickly from all the positive attention to big concerns for Vera these past few weeks.

Two weeks ago we received a phone call from the pediatrician about some results that had come back for the many tests that had been done to figure out why Vera has been having ongoing issues with her bowels. It was bad news: raised values in 2 components of Vera’s urine that point to neuroblastoma. Vera has a greater chance of developing neuroblastomas, a form of cancer, due to her syndrome and the ongoing diarrhea could be caused by a neuroblastoma in her body.
Two days later an x-ray was ordered for her chest and we were called that evening with bad news. The x-ray showed several anomalies that could be tumors. Everything started to point in the direction of neuroblastomas and we were starting to get really, really worried.
Two days after that new tests and exams followed at the Academic Medical Center in Amsterdam where the pediatric oncology specialists are.

Our talk with the pediatric oncologist at the end of the day took a completely different turn however. The anomalies that were seen on the x-ray turned out not to be tumors, but two broken ribs and all of Vera’s bones looked very brittle. Apparently, Vera has quite porous bones and with that another medical issue. Vera will be examined for this in Utrecht this week to find out why her bones are so porous and what would be the best treatment option.
Now that the spots turned out not to be tumors, the neuroblastoma suspicions are a lot less and we will find out if further examination is necessary or whether a new check will be done in 3 months to monitor her for raised values.

And then there are still the long term ongoing issues with her bowels that have not been diagnosed yet. We will continue the search for answers after her bone examinations.

The past couple of weeks have been an emotional rollercoaster, we were very concerned about cancer and the doctors seemed to be preparing us for this. As it turns out, cancer appears not to be the case, but our girl does have another added issue: porous bones. And the bowel problems continue to exist.wkzbrieven
In the coming week things will still be tense with 5 appointments in the hospital. We hope and pray for a good outcome and effective treatment.  In the mean time we buckle down tightly again for the next rollercoaster ride.