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Update, Christmas wishes and thanking sponsors!

Dear people,

A short look back on 2016 for Vera and us as a family:

Saying goodbye to the Sophia Rehabilitation preschool: After more than one and a half years, it was time to move on from the therapeutic preschool. We look back with thankfulness for the wonderful support, time, love, attention and endless patience from the employees of Sophia Rehabilitation in Gouda.

Vera had her 4th birthday! (this blog postFor preschoolers, this is like growing up: doing more things yourself and moving on to… elementary school. It was also confrontational for us, because Vera can’t do a lot of things by herself yet. But off she went to elementary school!

Mytylschool de Brug (The Bridge, school for children with special needs): after consulting with Sophia Rehabilitation, we applied for this special education school in Rotterdam. It is a big school and Vera has to travel a bit to get there, but now that she has been there for a while, we do not regret it. Vera is really going to school now: she gets assignments, sits in circle time, learns numbers and colors and interacts with classmates. At school she gets support from the neighboring Rijndam Rehabilitation center. We are glad that Vera feels at home at this school, we get the sense she really gets the time and space and stimulation she needs to grow and learn. She doesn’t have the energy for full days yet, she currently goes to school for 4 half days a week. She gets picked up in the morning at 7:30 by special taxi and around 1:30 PM, one of us picks her up from school. At the Rijndam Rehabilitation center, lots of capable therapists are working with Vera on developmental goals that we have determined together.

Medical: 2016 was also the year of the Chiari Malformation operation, a procedure to create more space in Vera’s skull for the cerebellum that had slipped down. Everything went well and we have noticed an increase in Vera’s mobility since then. Unfortunately, we found out during her last MRI that the cyst in her spinal cord has not disappeared yet.
We started growth hormone treatment over the summer. You can read more about this in this blog post.
In the fall, we had an ENT-procedure on the calendar: Vera’s tonsils and adenoids were removed and she received new ear tubes. Before the procedure, Vera had been snoring loudly and had developed sleep apnea. This has improved tremendously since then. Unfortunately, she is still not a great sleeper and still needs sleep medication to sleep. Even with sleep medication she does not sleep through the night.
Lots of medical concerns and problems still remain. Her tachycardia is difficult to treat, different treatment options have been explored this year, but to no avail. Additionally, the way her feet and hips are formed and positioned are still cause for concern.
But Vera has grown also. In the literal sense slightly but definitely in her development. She is speaking better and better, is able to play independently better and she is better able to fight off colds and viruses on her own. Hospital visits are still frequent, but they are all planned visits now.

Wheelchair: Because Vera is such a big girl now, she was ready to say goodbye to the stroller. She has had her manually operated wheelchair for a couple of weeks now and is quite capable with it. And she uses her walker at home as well.

Moving: Because of the wheelchair, we have started looking for a different house. It would be nice if Vera had a bedroom and bathroom on the main floor so we don’t have to carry her up the stairs several times a day (for changing, sleeping, bathroom use) and she can retreat to her room independently. We currently also have a big step up to the front door, which requires us to lift the wheelchair out of the house daily. If anyone knows of a great place in Gouda that would make all of this possible, please let us know. Preferably within walking distance of the city center, so Vera can use her wheelchair to get around easily.

It was a difficult year for the international Costello group. Several people with the syndrome passed away and even though they lived far away, the loss feels very close to home…

Mieke had her 9th birthday, the final year before we have a tween in the house. She is enjoying school and her local scouting group. She has joined a musical performance class this school year, she loves to sing and dance. Next year, Mieke will join a so called “bristers” (brothers and sisters) group at Vera’s school. The group is intended for siblings of children with a handicap to connect and share experiences.

Mieke and Vera get along really well together (other than the occasional sibling quarrel). Even when Vera behaves in a challenging way, Mieke is an incredibly sweet sister to her.

Finances: Thank you so much to all the people who send us donations on a regular or one time basis. This year we have used your donations for:

  • Fuel cost to/from school in Rotterdam and the hospital in Utrecht, as well as lots of parking at the hospital
  • Copayment for daily special taxi service from home to school
  • Meds that are not covered by insurance such as sleep medication, nasal sprays etc.
  • Membership for the play library that has special toys (Vera has hyper flexible fingers which makes it hard to manipulate regular toys)
  • Cooler for medication and food
  • Lots of baby wipes (luckily, diapers are now covered)
  • Copayment for custom shoes
  • Custom equipment to help with learning to eat & extra large bibs
  • Faucet adapters that allow Vera to reach the water stream to wash her hands on her own
  • Some healthy food, because we still make all Vera’s tube feedings ourselves
  • And lots more…

When the sponsor added a special note to use some of it for something special for Mieke or for family outings, we have done so.

Again, thank you so much. A handicapped and medically complex child brings along extra cost and less income because one of us had to give up their job. It is wonderful to feel supported in this by the people around us. We also wanted to mention: if you support us on a regular basis, please do not feel obligated to keep doing this. We have noticed that when one sponsor stops, another one will pick this up. And we are managing well. We may request additional support in the future when we will have to navigate a move and possible renovation.

For now, we send our warmest regards to everyone, wishing you a Merry Christmas and a beautiful new year filled with love.
“And now these three remain: faith, hope and love. But the greatest of these is love.” (1 Corinthians 13:13)

Bye!

Niels, Leonie, Mieke and Vera

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Growth hormones

It’s bedtime, but first… the shot! Vera is prepared. Every evening before bedtime she gets a shot. In her buttock. Small buttock, because that’s why she gets these shots: she is small. Even small for her syndrome. But there are additional reasons for the shot.

groeicurveOne of the characteristics of Costello syndrome is slow growth or lack of growth. Adult women who are affected by Costello have an average height of 1.35 m. (4 ft. 4) Vera’s sister Mieke is already taller than that at age 9, so we can get a good picture of how little grown-up Vera will be. And currently, with a height of 85 cm (2 ft. 7), she is very small for a 4.5-year-old. We knew Vera would be small, it is part of her genetic condition after all. But Vera is even below the adjusted growth curve for Costello syndrome. Because many children with Costello syndrome have reduced levels of growth hormones it seemed only logical to have her levels tested at WKZ (Wilhelmina Children’s Hospital). We knew from many parents of children with Costello syndrome that many of them were receiving growth hormone treatment to supplement the shortage to normal levels. And so, we added a new specialist to our list: the endocrinologist.

humatroop-startpakket

starter

prikken-banaan

practice to give a shot on a banana

The trajectory started with a testing day (administering arginine), which showed that Vera does have adequate growth hormone levels (her pituitary gland does make growth hormones), but her body is not processing it. The cells in your body are supposed to make IGF-1 (insulin-like growth factor 1) in reaction to growth hormones, but this isn’t happening in Vera’s body. This not only affects her growth, but also her bone growth, muscle development, blood sugar levels and supports heart functioning. Many parents we talked to reported that their children slept better and had more energy after receiving growth hormone treatment.
After the testing day we started a trial period: administering growth factor IGF-1, that’s what’s in her shot. Luckily, Vera’s body responded well to this, her levels went up noticeably. But in order to start ongoing daily treatment, a lot of consulting had to be done by many doctors to make sure it was safe to give this to Vera. Because, due to the complexity of her condition there is always a bit more uncertainty and less experience with the long-term effects of the new treatment. The most important question was: could the reduced levels of growth hormones in Vera’s body be protecting her against something that would be undone if we started treatment? At the conference in Manchester we had already learned that there is no direct correlation between administering growth hormones and incorrect cell division. If this were the case, we wouldn’t want to do the treatment, because with Costello there is already a greater chance of developing tumors, we wouldn’t want to make this chance even greater. After reading many articles, talking to other parents and of course consulting the doctors, we came to the conclusion that administering growth hormones does not have any proven risks but that it does have proven benefits. With that, we could start her daily treatment – indefinitely, with check-ups at set points. Luckily, our insurer agreed, because it is a rather pricey medicine and we can’t just pick it up at our local regular pharmacy, we have to go to the hospital pharmacy in Utrecht.

humatrope-naaldAnd so, the shot in our refrigerator was born. It took some getting used to. We ourselves already had to learn so many medical procedures to care for Vera, and not all of them are pleasant, with this one in particular, giving a shot, it was the first one in which we have to cause our child a little pain for her own good. Of course, she has had dozens of shots and we always held her and comforted her, but now we are the ones administering the shot. It took some getting used to, but after two weeks, we all adjusted, including Vera. The shot is part of it, it only takes a second and Vera is enormously brave; usually she allows it to happen willingly and she doesn’t make a sound. She does like to have control over who can give her the shot (mom or dad?) and where she wants to lie down for the shot. Because she may be small, but she has a big personality.uitspraak-short

vera-ballonAnd, does it help? Vera has been receiving the treatment for 5 months now. The dosage has been increased once because she is still not  at normal levels of IGF-1. But Vera is growing and it does seem to go faster than before. She does seem to sleep a teensy bit better on average. We do not see an increase in energy levels yet, but that has a lot to do with her heart condition (more about this later). As with all things, we are patient with this and will see what will happen in the long term. So for now, we will continue with our small shot for our big little Vera.