All my bags are packed
I'm ready to go
I'm standing here outside the door
I hate to wake you up to say goodbye....
(Excerpt from Leaving on a jet plane by John Denver)
No, I'm only going away for a week,
As a parent supporter for my kids.
They are both off to KL to represent their school
in a chess tournament organised by the
Malaysian Ministry of Education.
So, to keep you occupied when I am away
Here's another chapter of A Gift from God....
(yeah, there's more to come)
A Gift from God (part 2)
The first 2 weeks of the Baby's life was pretty normal. The couple felt terribly stressed as they still did not know what was wrong with the child. Suddenly, starting in the 3rd week of the baby's life, the baby started crying every time her diaper was changed, as well as every night from 1 to 5am. This went on for weeks. The couple did not know what was wrong! The wife was really stressed as she was up all night every night trying to soothe the baby, and during the day, her life had to go on, taking care of her other two kids. They considered hiring a night nurse, but finally decided against it as they were not used to having others take care of their children, much less a new born. A call to the paediatrician was made, and he suggested that it could be colic. Being Asians, they did not personally make a visit to the doctor as the mother was supposed to be in confinement.
Meanwhile, the gynae and paediatrician were pondering over baby's birth x-rays. The gynae's husband, who is an orthopaedic surgeon chanced upon the x-rays one day, as it lay on their study table at home. He immediately told his wife that the thigh bone was a "healing bone" i.e one that had been fractured and was now in the process of healing. The paediatrician on the other hand, forwarded the x-rays to another doctor who came up with the same diagnosis, and suggested that the x-rays be forwarded to a visiting professor.
The mother and child made visits to both the gynae and paediatrician at the end of the confinement month, and more x-rays were ordered for the Baby. That was when they found out the reason behind those crying spells. The Baby had fractured her femurs again!! (The first time was in utero.) The visiting professor responded with the answer for the fractures. The baby was diagnosed as having Osteogenesis Imperfacta (OI for short).
They were then referred to a geneticist and a professor at the university hospital. Meanwhile, the couple did their own research on OI.
Osteogenesis imperfecta (OI and sometimes known as Brittle Bone Disease) is a genetic bone disorder. People with OI are born without the proper protein (collagen), or the ability to make it, usually because of a deficiency of Type-I collagen. People with OI either have less collagen than normal or the quality is poorer than normal. As collagen is an important protein in bone structure, this impairment causes those with the condition to have weak or fragile bones.
As a genetic disorder, OI is an autosomal dominant defect. Most people with OI receive it from a parent but it can also be an individual (de novo or "sporadic") mutation.
(Here's an article on OI in yesterday's newspapers)
The more they read, the more confused and worried they got. The family had no history of OI. So they concluded that it had to be an individual gene mutation that caused it. There are many categories of OI, ranging from very mild to the very lethal, where they can die within a year from birth. Bones can even break from just a sneeze. The couple was at a lost how to treat their precious baby. They also found out that at present time, there is no cure for OI. Treatments are aimed at increasing overall bone strength to prevent fracture and maintain mobility. Physiotherapy could be used to strengthen muscles and improve motility in a gentle manner, while minimizing the risk of fracture. Alternatively, metal rods could be surgically inserted in the long bones to improve strength. But all this was not advisable at such a young age.
So began a series of visits to the specialists. Every visit to the specialists was a heart wrenching experience for the parents especially the mother. This was because she saw what could become of her precious baby - as there were a few other children with OI who had appointments at the same time. Some, as old as 10, years were going for their appointments in baby strollers, as they could not sit up. Some younger ones were carried in for appointments on mattresses, so as to minimise contact (for fear of fracturing bones). Many had to use wheelchairs to get around.
The specialists advised them to minimise any possibility of a fall. They also had to be on the look out for fractures (even if there were no falls). To observe the child carefully, for eye related problems (deteriorating eyesight and eventually blindness could result depending on severity), dental problems (poor dental health is also a major problem) as well as hearing problems (deafness could result). The list was endless. Whilst the husband tried very hard to follow the doctor's advise, almost to the extend of keeping the child in isolation and limiting those who could even carry her, the wife, wanted the child to have as normal a childhood as possible. Basically the husband was more cautious, whilst the wife was - to put it mildly - clueless as she really did not know how to handle the situation. She just wanted a normal childhood for her child.
The couple worried about how their other 2 children would feel when they found out about their little sister. Would they despise her and consider her a burden? Would they be angry with her, because their parents would be devoting more time to the baby because of her physical disability? Would they be upset because family activities would in the future have to be of the non-dangerous type - ie no soccer playing, no rock-climbing, no skiing etc. Or, would they warm up to their baby sister and love her just the same, or even more.
So whilst the couple decided to keep the truth from their children (as they were unsure how severe the OI was, nor how to explain all these to their two young children), they had to tell them to be very, very careful and gentle around their little sister. They had to enforce a “no hugging" rule for fear that it could cause fractured ribs. The children were really puzzled as they couldn’t really understand what the fuss was all about! Friends and family were also discouraged from carrying the baby. Many could not understand, as the couple did not explain to them what was actually wrong with their new baby. The wife just could not get herself to talk about it. Actually, she really couldnt accept the situation. A question that keep popping up in her mind was - why is God testing me like this? Is this the cross that I have to carry?
Meanwhile, the husband plunged himself into work. He had to make sure that there would always be more than sufficient for their children, especially the precious youngest child. He needed to make sure that the unknown medical bills that they would face, could always be paid. He wanted to be able to get the best medical help around when the time came. The couple even reviewed their insurance coverage as their needs were now different.
Every turn, cough and even sneeze the baby made was observed with trepidation. It was worse when she started to learn how to crawl (at 6 months) and walk (at 10 months). They just could not risk her falling!! What if more bones broke? They were told that it would be best if she did not suffer any more fractures! Every fracture suffered would further weaken any healing bones and would indicate a more severe diagnosis of OI. They were also told that the child would in the future not be able to participate in activities like gymnastics, physical sports, playing on monkey bars, ballet, and they were even told to discourage the child from going on the slide!! The child, when she grew up, would have to depend on her brains for future employment, and any activity of a physical nature was to be ruled out.
The unthinkable happened one day when the baby was about four months old. As the wife was preparing for lunch, she put the baby in a baby carrier on the chair next to her. As she turned to get a plate, something happened. No one knew how it happened, but the child and the carrier toppled off the chair and the baby fell out of the carrier! The baby was not crying. The wife, petrified, bent slowly over, and she picked the baby up. Suddenly, the baby started crying and the mother became hysterical. Fortunately there was no blood nor bruise.
Were there any broken bones? If so, how many? How was the wife going to explain what happened to her husband??!!?
....to be continued.....