Evelyn is 2

Time has passed by so quick. I cant believe that Evelyn is 2 now.

The past 2 years was terrified, from the moment the pediatrician told us her deformities, followed by her OI diagnosis, then the knowledge on how to care OI baby, her developmental delay, the frustration we have dealt with doctors, therapist, orthotics, specialist, physical therapy (PT) training on our own, pam drug treatment/ doctor appointments routine, and many more. OI is really a scary thing. There are lots of uncertainties so we really don’t know what to expect next. Her scoliosis (spine curve) is the most scary diagnosis, from zero degree at birth to 38 degree at 18 months old in S curve, and yet the doctor said it would be a progressive curve - this is something bad to hear. We have no other option but to live with it and accept the way it is.

We were told that she would never walk - but just recently (22mo), Evelyn started to cruise furniture with support, pull to stand with support, stand/ walk with support. Her daily PT is something that we cant be ignored, because every little effort do helps. We are hoping that she keeps progressing and gets stronger each day in meeting her major milestones. A good quote from Master Zheng Yan "Never under-estimate yourself, because human have unlimited potential". We are proud of her learning attitude, shes love learning and keeps learning everyday. She discovered fun in learning. We truly believe that she would do better in her path with her own way.

To Evelyn :" On the 23th May 2011, you enter into our world and our live changed forever..... You teach us to be strong, be patient, be brave, be happy, be positive, be gentle, be calm, be broad-minded, be courage, be energetic, be loving, live life to the fullest, and more importantly - appreciate small little thing in life. Your change to us is just countless, and we are what we are now to be a better person". WE LOVE YOU FOREVER!!!

We celebrated her birthday 2 weeks earlier with my family together with mother's day. It was a great party, we enjoyed ourselves very much.

Look at her birthday cake & presents - She was excited!!!

Birthday party with her girly cousins

Here is a video clip I created for her to recap from birth to 2. Enjoy!

Evelyn weighed at 24mo : 6.95kg, 75cm




Backlog journals - Scoliosis



 

Front view of Evelyn's leaning to her left



Human spine made up of vertebrae, a normal vertebrae is in a cube shape, There are 7 cervical vertebrae (C1 to C7), 12 thoracic vertebrae (T1 to T12), and each connects to the 12 pairs of ribs. The lower spine made up of 5 vertebraes called lumbar spine (L1 to L5). So when all cube shape vertebrade line up straight it becomes a straight spine at front/ back view. One of reason for scoliosis associated with OI developed is because of vertebrae loosen liagaments (means the vertebrae in between is loosen os they cant connect firmly to each other).  Evelyn has 10 triangle-shape vertebraes (T1 to T8), (L1 to L2)  that made her spine cant stand straight. Her upper spine curve to the right made the right ribs twisted to right up; the lower spine curve to the left made the left ribs twisted to left down. It is a very complicated S curve. We are hoping that pam drug treatment could help to strengthen the triangle-shape vertebrae so it would not get collapsed further.

Evelyn tends to lean her body to left side due to the twisted right up ribs, and twisted left down ribs. She received her back brace support since 20 months old. .  The brace is to support her body posture so that she will have a straight body and to avoid leaning to either side. It needs to be wore 24/7 no matter what position she is in, ie: sitting, standing, walking, sleeping.

Evelyn on her brace, with this brace she cant really move her body in sitting posture, so we let her stand with support



Back view of Evelyn's spine curve, upper T1 to T8 twisted to right up resulting the right shoulder to be uneven, lower lumbar L1 to L2 twisted to left down.



Once Scoliosis associated with OI started to develop, it will not be straighten by itself. A mild curve can be corrected through brace or pam treatment or therapy. A curve > 30 degree needs to be monitored closely; it is highly likely the curve will progress rapidly. The brace is not for spine correction, but to help to prevent her curve progression. For curve > 50 degree, surgery maybe required if the internal organs such as lungs and heart are affected by its function.

Evelyn had 37 upper degree curve at 18 mo. They had never seen this bad curve at such a young OI toddler. Her lower spine curve was less than 10 degree, we have been praying for the lower ones to stop progressing.

almost meal time

"Happiness is to stop worrying things beyond our control"

 

Evelyn weighed at 22mo : 6.8kg, 75cm





Backlog journals - First PAM treatment

The geneticist had finally approved Pam drug treatment after E's heavy spinal compression fractures. They initially used the term "Possible OI", and now they can confirmed it is a "real OI". At least we dont have to travel overseas for this treatment like what we have planned earlier. So Evelyn received her first pam drug treatment at 19 months old.

Pamidronate is given by a drip (infusion) into a vein through a fine tube called a cannula.  The infusion is given over 3 to 4 hours. After the infusion, the doctor will monitor and check if E has any side effects such as "flu like" symptoms, or fever. Usually baby will get these side effects on their first treatment. We were lucky that Evelyn had been doing great during & after several hours of the infusion. However, we still need to stay overnight for close monitoring. Evelyn discharged the next day without any side effects still. Woo hoo….



Evelyn sleep throughout the 4 hours pamidronate infusion



There are 2 tests to be monitored yearly along with the pam drug treatment:-

1) DEXA scan to check her bone mineral density. This is to monitor the progression of her bone density after the treatment received.

2) Kidney ultrasound to check if her kidneys are normal, because a long term drug treatment will cause to kidney stones.

The researchers proved that children who received this treatment have made a remarkable improvement in quality of life. Many of the children gained greater bone density, reported reduced bone pain, gained mobility, and had fewer fractures during the treatment.

Evelyn can bear weight at 20m - still shaking her legs

We just love the pam treatment. Evelyn had gained so much strength after her first treatment. She can bear weight at 20mo. Her appetite had improved gradually too. Her weight stop at 6.2kg over the past 6 months (15mo to 21mo), she gained 0.5kg to 6.8kg at 22mo. she was still far behind her peers. We cant force-feed her. Severe OI children have tiny and fragile bones and poor muscle tones so the expectation on their weight & height are totally different to the normal growth chart. In OI world, they would not look at the "normal" chart, instead they said "Let them grow at their own pace".

"No matter what you're going through, there's a light at the end of the tunnel and it may seem hard to get to it but you can do it and just keep working towards it and you'll find the positive side of things". - Demi Lovato

Evelyn weighed at 22 months old : 6.8kg, 74cm