Backlog journals - First fracture

Evelyn with her grandma

Last year Nov 23, at her 18 months old. Evelyn fell off the high bed and broke her 6 spine vertebrae. She has had 6 compression fractures at T5 to T8, L1 to L2.

It was Friday evening at 6.30pm. She was extremely tired after her full day nursery. As usual, we let her sleep in our 2.5 feet bed not knowing she will awake in the middle of her sleep and scoot around the bed.While Francis and I were watching TV in the living room, we heard a big scream and abnormal crying from the bedroom. We quickly ran into our bedroom and noticed she was lying her back on the ceramic floor. She was stiffed in that position for a long while. The first thing in my head was she must have broken her skull or spine or legs !!! Oh my god! How can we leave her alone in the bedroom. I tried to carry her with my gentle hands, unfortunately she had cried even louder & abnormally. We quickly packed her stuffs and went to emergency have her checked. The doctors checked her whole body to see if she has any swollen or bruise part, because those are fracture signs. She didn’t have any of those. So They have ordered a skull xray but see no fractures so they allowed us to go home right away.

That night she hardly sleep, she cried throughout the night. She cant move her limbs or body, her whole body still stiffed like a wood. She can only lie on her back. All her movements were very slow and gentle. I remember the OI Parents forum said rub her with hot/ cold pack will help to release her pain. So that whole night we were busy with the rubbing and caring her.We played her favourite songs and cuddle her to comfort her. 3days later, she can sit up abit but her body and legs were still stiff.

 

The OI Parents said she must have broken her spine. So a week later we went back to emergency for spine xray. It was confirmed that she has had 6 compression fractures at her spine vertebrae - T5 to T8, L1 to L2. No cast or brace can be prescribed for her but painkiller and more lying on back rest. The spine doctor called for seeing them in 2 months time.

It took over a month's time to heal. Evelyn was able to get back to her routine movement and mobility. However, the fall has made a remarkable curve to her spine. That's why I called it the most "scary" incident ever. We should not underestimate her mobility ability, that she can scoot freely not long ago.

Evelyn is such a fighter. I know she were in pain but she still smiling while lying on her back. She is the strongest and brave girl ever! We love her so much! 

 "Some people think that to be strong is to never feel pain. But in reality, the strongest people are the ones who feel it, understand it, and accept it... "

Evelyn weighed at 18 months old : 5.9kg, 71cm

Backlog journals - 15m to 17m

15 months old

Our fighting for starting pam with the geneticist still not being approved. We have seen spine doctor this month, her upper curve had progressed to 33 degree. The comments from the OI Parents forum was - if Evelyn started the pam drug treatment earlier she would not have this curve at that age. She would have been a moderate type, and not severe. We were super sad to learn this.  I have no idea why Malaysia has such a rule to delay the drug treatment given that the study and research have showed the positive side of the drug. In US, an OI baby started the drug at 3 months old or earlier right after the OI diagnosis. I was truly mad with the doctor here, so I have started to look for overseas help on pam drug treatment.

They still said nothing can be done, she was too young for surgery, so will "watch & wait". The only thing we can do is to watch her posture, do not let her lead to one side, this will make the curve progress badly. So we have came out an idea with sofa as her table and cushion as her chair so she can sit on them with body straight. She loves them very much.

However, when she sits on floor to play freely, we have to watch her posture and never let her sit more than 30 min. This month, Evelyn started to scoot around to reach toys. Her movement always start backward, we are fine with this as long as she keep progressing.

16 months old

Wow, cant believe that we have came a long way since her birth. Evelyn had free of sick for the past 3 months from July-Sept 12. We really deserved a celebration and break. So I booked a vacation to Terengganu beach. This was the first family vacation. It is 5 hours drive from KL, all we have in our mind was the challenge drive as Evelyn had never travelled such a long journey. We were lucky that Evelyn didn't make much noise on the journey to Terengganu. The beach was quiet with no crowd. She enjoyed the beach; the wind; the sand; more importantly, the pool. We have had a good time there.

Just a week after our vacation, Evelyn suffered for flu and cough again. She has had a very dry cough started about a week ago. We brough her to Ped emergency again. The doctors were quite impressed with her condition that her immune system had improved so much. Based on her past sick history, she would have cough badly till suction or admission for pnuemonia.  

Another proud moment this month!!! Evelyn started to sit up by herself from tummy.

17 months old

Evelyn switch from home pool to the big outdoor ones. She had been constantly gainning muscles and core strength. She started to buttock-scoot forward, so she could do backward and forward now. She was very happy that she finally able to do her own mobility. Still, we need to watch her movement closely to avoid fall and bone breaks. Another milestone accomplished. I have recorded each of her milestone. Her progress momentum gave us some positive hope. We have the gut feel that very soon she will be able to pull to stand. It said "have faith at everything you do, because "there is a life, there is a hope". So we have only "positive hope".

We're very proud of Evelyn's achivement. She had no pam treatment, just daily PT at home, and yet she had progressed so well. 

"The struggle you are in today is developing the strength you need for tomorrow".

Evelyn weighed at 17 months old : 6.25kg, 71cm

Backlog journals - 12m to 14m

12 months old

I have brought an indoor baby pool for her with a neck float. The OI Parents forum said water exercise is good to build muscle strength. Evelyn enjoyed splashing & kicking in the water. We can see that she had gained some strength since then.

After a few session of pool exercise, she finally able to sit independently. Another big milestone had accomplished. Unfortunately, sitting may harm to her spine. we noticed her uneven shoulder. Her right shoulder was higher than the left, that means her spine have a curve to the right.

We were referred to a spine doctor right away. Prof Kwan and team measured the curvature degree and it was exactly shown at 22 degree. The T6 vertebrae was in triangle shaped, this deformity had made the spine curve started. They said nothing can be done at the point but to "watch and wait". It needs to be monitored in every 6 months.

We celebrated her 1st birthday with her grandparents and my sister's family.

 

 

13 months

Evelyn had gained some core strength on trunk and thighs, she can push her legs while sitting in baby walker. Woo hoo….

Living with OI is like a roller coaster. There are always shock news followed by a good news. Evelyn had admitted for pnuemonia again for a week. Thank god it was not as bad as those 2 admission before. It is only viral infection with no fever. I was all well-prepared and know what to expect from the pnuemonia protocol. The antibiotic of a week had been given, they did suction, nebuliser, physio as usual. They have given some oral antibiotic after the discharge. After a week, Evelyn had fully recovered. Looks like she can go back to her pool again :)

 

14 months old

It had been almost 2 months since Evelyn started water exercise. She loves water very much. She had getting more active after the water activities. Her arms and shoulders became stronger. This month, she started to sit with both legs in cross W position with both hands on floor. We're proud of her, she is our strong and brave girl. My tears off seeing her achievement.

The genecicist suspected that Evelyn might have Cole-Carpenter Syndome (CCS) on top of OI. CCS is a very rare syndrome that manifests with bone fragility, craniosynostosis, ocular proptosis, hydrocephalus, and distinctive facial features. It represents a form of congenital brittle bones resembling osteogenesis imperfecta. Simply said - on top of OI, CCS has additional symptons such as water in brain and abnormal shape of brain, head, facial etc.

the left xray taken at 9 months, the right was taken at 13 months old with curve at 22 degree

So a brain MRI was ordered to rule out if she had the above said symptons. Good news - everything were perfect in the report. That proved to the geneticist that he was totally wrong! 

It is proven from the OI research, that it is beneficial for a baby to start pamidronate as early as possible to strengthen bones and mucles, so the child can meet their milestones earlier, improve bone mineral density so bone fracture can be prevented. They also proved that the drug is good for her spine curve too, because the drug will flow into the spine to correct the vertebrae wedge-shaped perfectly so the spine may look good. We have always discussed with the geneticist to start the pamidronate drug treatment but our request have always being rejected with the 2 reason :- 1) Evelyn is too young to take up the drug, she might have sufferred from the side effects. 2) It is a standard rules in Malaysia that the drug can only be started at the age of 2 if no fracture occur or no bone pain happened. Evelyn had no new fracture since birth, at least a good news so far.

 

Quote : "Once you replace negative thoughts with positive ones, you will start having positive results" - Willie Nelson

Evelyn weighed at 14 months old : 5.98kg, 71cm

New milestone but admission again

Evelyn had getting more active, her arms were stronger, she did tummy time for the first time at 10 months old. She can only hold it for a minute or two. Although it was a slow process but we were very much happy to see her accomplishment.

Tummy time at 10 months old for the first time

We put her in infant car seat for feeding, she loves seating on it for watching TV too. However we were not sure if that was the right posture for her at that point. It maybe harmful to her spine. She had been sick for lying down on her back at all time. So we thought sitting with this support gave her a better viewing.

Evelyn sitting in the infant car seat

At her 11 months old, she admitted to UMMC for measles. It started with fever for 10 days, then followed by rashes all around her body. We initially thought the rashes was because of medication alleegy. So we brought her to few pediatric clinic, as usual, paracetamol and antibiotic have been given but we did not see any recovery still.   On top of these, she was having a bad cough and flu too and breathing difficulty. The fever had gone up high at 38.5' celcius. We brought her to pediatrician emergency  at UMMC. The doctor advised to admit right away.

Daddy is feeding her through tube

During her 10 days stay in the hospital, she received a nebuliser and phlegm suction 6 times daily. An oral antibiotic was not useful at that point, so she required antibiotic infusion 4 times daily. Due to her breathing difficulty, she was given an oxigen tube through nose. Another tube had inserted to her throut through mouth to provide milk intake. Body temperature was being monitored closely every 3 to 4 hours. We had wiped her with wet towel on and off to keep her body hydrated to avoid high fever. Few blood and stool had been taken for bacteria and virus test. 

It broke my heart to see Evelyn in such a pain and suffering. She has had enough, with pnuemonia admission at 6 monts old, and meascle attack again at 11 months old. We prayed for her speed healing.

"Suffering becomes beautiful when anyone bears great calamities with cheerfulness, not through insensibility but though greatness of mind". -- Aristotle

Evelyn weighed at 11 months old : 4.95kg, 69cm