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Merry Christmas and good night

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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!

Leonie

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How are you?

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“How are you?”
I never know what to answer when people ask me this question. Not because of the person showing interest, but because of what happens in my head at that moment. I go over everything in our life, try to take an average and make up a total score. I can’t do this very fast and so I usually end up saying: “Pretty good.” Because can anyone really say life is 100% good? In our case it’s never really 100% good. In our normal, a family with a special needs child, there is always something going on. Not that that is necessarily always a bad thing. Some things you get used to, such as still changing diapers and giving tube feedings. Other things we never get used to, such as poor sleep or Vera’s temper tantrums.

portret of Vera, October 2018

Not getting enough sleep is one of the trickiest. After having visited two sleep clinics, we now have a lot of information, but no real solution yet. Vera wakes up crying and talking and tossing and turning many times during the night. And she is done sleeping very early in the morning, between 5:00 am and 5:30 am. While I write this, our pediatrician is looking into a different sleep medication and we are hoping to get some relief soon. Because we can handle everything better when we are well rested. And Vera herself feels so much better when she is rested. When she has had a reasonable night, we see a cheerful, goofy, talkative 6-year-old girl, who wants to be independent. If we have had a bad night, Vera can be quite defiant. There may be kicking, hitting, throwing things and biting and it will take a lot of coaching and guiding to get her on the school bus in a good mood. Off to school where she is now in the Blue Group. Since summer, Vera is no longer a Kindergartener, but an elementary school student. And just like at a regular school, this means sitting at desks in a row, working more independently and learning ABC’s. She is fully a part of this class. But it is still new and takes some getting used to. Some deficiencies are more noticeable now. Such as Vera’s vision. We know she has limited eyesight, but at home this doesn’t interfere with things as much. Now that Vera is in her new grade, she gets to go to recess in the big schoolyard. This means more kids, bigger bikes and carts and a larger space. If you are not that stable on your feet and you can only see things clearly that are 1 meter away, it can get a bit dicey. The teachers noticed and try to take Vera to the schoolyard when things are calmer, or have her play in a quieter corner. In the classroom she has been placed in front and when something is put up on the digital board (which still proved hard for Vera), she and the girl next to her have an iPad that shows what’s up on the board, so they can see up close.

It seems like small adjustments, but we have found that small adjustments can make a big difference. Sometimes we feel that we have made too many small adjustments in our lives and our own energy and we have to take a step back for a bit. I recently experienced this when I went to a museum with Vera and the adjustments were missing, see my Facebook post about it.

And sometimes we notice that small adjustments at home, in schedule, in approach, in tools, help Vera thrive more. That’s when she is most fun, and we are more fun and things are more fun for Mieke. And then things are pretty good 😉

It didn’t become an overview, this story. There is so much more to tell. About Mieke, who is becoming such a teen, about still diapers and tube feedings, about casts again, new splints, new shoes, new wheelchair. About Vera’s glasses that do help her see better, but will not completely correct her vision. About sensory processing and a trampoline in the living room. But for now, we’ve given you a bit of an update on how things are going over here.

Love,
Niels & Leonie

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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: