Vera 3 years old: a look back
Vera is 3 1/2 years old now. Three years filled with many hospitalizations and stays, therapies and examinations. But also three years filled with love, bonding, development and happy moments together as a family of 4.
Vera is still a cheerful little girl full of laughter. She loves music, watching other children, going outside and cuddling with mum and dad. And let’s not forget: watching videos. The Lotte en Max DVD’s for instance, a great tool for teaching her sign language to help her communicate. Vera really likes signing and is actively using several signs, such as ‘going outside’, ‘sleeping’, ‘eating’, ‘done’, ‘getting dressed’, ‘biking’, and several others. It is wonderful that she can express herself this way and we can understand her better. With this limited vocabulary we are able to have a lot of contact and communication. Big sister Mieke joins in enthusiastically and because of this we can switch between spoken and sign language all day long. Slowly Vera is making new sounds with her mouth and she is even able to form real words. Just before her third birthday Vera could say: ‘mama’ 🙂
Vera still receives 100 % of her nutrition, fluids and medication through her Mic-key button, a feeding tube that goes directly into her stomach.
Since February 2014 Vera has been eating a so called blended diet. This means we do not give her formula anymore but home made porridge and purées made of vegetables, fruits, grains, etc. For a tube feeding this is not very common in The Netherlands. When we spoke to other parents of children with Costello Syndrome (in particular in the United States) we heard about the potential health benefits of the blended diet and decided, after consulting with our paediatrician and dietician, to give it a try. For Vera this makes a big difference in her quality of life. For months Vera would spit up and had diarrhoea, but all of that went away when we started her new diet. Her skin is calmer and she has increased energy to play and develop. Thanks to donations we were able to purchase a powerful blender that helps us make her food very liquid to make sure the feeding tube doesn’t get clogged.
Vera does not eat by herself yet. But she is very interested in food and when offered a piece of food she will suck, taste and bite it. Occasionally she may swallow some. At this moment her favourites are: crisps, shortbread cookies and ice cream (my, my, that sounds promising…).
Since September 2014 Vera has been attending a therapeutic preschool (offered by Sophia Revalidatie) in Gouda for 1 1/2 days per week. Vera really enjoys going to ‘school’. Here she receives lots of physical therapy, speech therapy and intense support. In the past Vera hated physical therapy and screamed at the therapist. But now she loves it. She loves to ‘get to work’, as they call it. She is busy learning getting to a sitting position from lying down, to a standing position from sitting and walking with her walker. Vera loves anything with wheels. Besides her walker she has a small red scooting car and a pretty pink scoot bike. Even though she can’t fully walk by herself without assistance yet, with the help of all of these she can get where she wants to go. Her semi-orthopaedic shoes are of great help in all of this. She has super cool red ones. They are firm like hiking boots and have a special foot bed that is custom made for her. It corrects the position of her feet and supports her ankles. Which then helps to position her knees, legs and back. This is important so she doesn’t grow into the wrong posture. This is why Vera also has a custom high chair, car seat and buggy.
Because it is important that we as parents keep correct posture as well while caring for Vera, she now has a custom bed which can be lowered and raised and has a high railing with gates to prevent falling out of bed. We carry and lift Vera a lot on a daily basis. With a bed and chair that can be raised and lowered we put less strain on our backs.
In March 2014 Vera received a port-a-cath (PAC), see this blog post.
This was necessary to be able to administer her bisphosphonate IV-drip on a regular basis to help strengthen her bones. It is now much easier to access Vera’s veins in case of an emergency or for taking blood samples. The downside to the PAC is that we cannot lift Vera by her left underarm (this is where the PAC is located), which requires new lifting techniques for us. You can never just lift Vera by her underarms like you would normally do. It is very important that everyone who cares for Vera remembers this as the PAC could shift out of place by incorrect lifting.
The good news in late 2014 was that Vera’s bone strength had improved tremendously and treatment could be diminished by half.
It is nice to know her bones are stronger now that she is developing her motor skills and sometimes takes a fall.
With the syndrome that Vera has, she unfortunately has an increased chance of cancer. She is screened for this 4 times per year. Previously this was done at the Academic Medical Centre in Amsterdam, but now the screening is done at the specialized Maxima Medical Centre in Utrecht. In November 2014 her urine analysis was cause for alarm. Her values indicated the possible presence of a neuroblastoma. Several values were much too high. We were told this was bad news. An extensive examination took place to try and find the neuroblastoma and included MRI and radioactive scans. These were very challenging times for us, but fortunately they couldn’t find anything. The high values in her blood are now considered something that is typical for Costello Syndrome. The preventative screening (ultrasounds and urine analysis) continue to take place four times a year.
Medical appointments are still filling up our calendar. We visit the paediatrician, cardiologist, ENT doctor, dermatologist, rehabilitation doctor, eye specialist and oncologist on a regular basis. And we are regulars at the pharmacist as well. A large group of specialists who help find out what’s best in Vera’s treatment and daily life. Our most recent examination was a sleep study. Vera had not been sleeping well (for her entire life). She would wake up often at night, sometimes every 15 minutes or every hour or hour and a half. Lots of medical causes have now been ruled out and the pragmatic solution of sleep medication was chosen. We had always considered sleep medication as a last resort, but given ours and Vera’s extreme exhaustion we had now arrived at this point. And what a remedy it has been! All of us are so much better rested now and Vera has a lot more energy to work on her development.
Luckily KinderThuisZorg (at home children’s care) still supports us in the care for Vera. They help us with medical procedures that can be done at home, but also to give us as parents some respite. And in those moments when the special nurses care for Vera, we can focus on our other daughter Mieke and spend time with her. Now that Vera is attending preschool and is sleeping better, we rely less on the at home care, but we are still extremely grateful for the hours they help out in our family.
Vera, 3 years old. We are almost scared to say this, but Vera has been doing better than ever lately.
From the very fragile little baby she was, she has now grown into an opinionated toddler who can handle a small bump or two. But of course she still remains a daughter that requires a lot of care.
Three years: life has never been this intensive. But we would like to underline again what was written on Vera’s birth announcement: “Desired, received, loved”!
Below you can find our updates from her first and her second year, so you can see the progress.
Vera is now 1 1/2 years old. She has a cheerful personality and loves to make raspberry sounds. She likes funny sounds, music and going out with the stroller. From her safe spot she knows how to charm the people she meets along the way.
She is clearly very attached to daddy, mommy and big sister Mieke. She is very happy to see us and reacts to us. Mieke and Vera have a lot of fun together, a great thing to witness.
Despite her periods of illness Vera is developing. She can mostly sit by herself, stand on her feet with some help and even take a few steps at times. She can clap her hands and explore her toys.
Vera receives all her nutrition through her PEG tube, but she is definitely interested in food. She likes to feel it, smell it, suck on it, taste it and spit it out. She prefers pickles, ketchup and ice cream. During our meals, Vera joins us in her chair and has her own plate of food to work with. This is a very positive development and gives us good hope that she will start eating on her own in due time.
Vera receives physical therapy. Because mom and dad are doing such a good job, this is only once a month. Which is fine by Vera because all those exercises she has to do with a stranger are not her favorite. So usually the physical therapist gives tips for mom and dad to work with.
Vera has speech therapy about once a month also. Here, she learns different ways of communicating, gets challenged to make different sounds and is stimulated in the basics of dealing with food.
Unfortunately we had to stop her “snoezelen” sessions (controlled multisensory environment (MSE) therapy). As much as Vera enjoys it, the other children also love to drool and suck on the objects in the room and this poses a danger for infection of her airways.
Vera has a busy schedule visiting the pediatrician, ear, nose and throat specialist, cardiologist, eye doctor, dermatologist, ultrasounds every 4 months to screen for tumors, dietician, occupational therapist and rehabilitation specialist.
Her girlriends from the at home care team come and visit on a weekly basis to come and care for her for a couple of hours or keep her company during the night so mom and dad can get some much needed rest. Vera likes structure and is very attached to mom and dad, so when the nurses arrive Vera may object for a (long or short) while.
We now have a lot of custom equipment around the house; a custom stroller, high chair, bathing seat and car seat. Recently Vera acquired a “Vera mobile”, a special walker to help her stand and walk. She got to practice with the floor model in the showroom and is getting quite a kick out of moving herself from place to place. So beware!
For an impression of Vera’s first year you can watch this personal video (warning: lots of hospital images).