Adel has been having this nagging cough for more than 2 weeks now (since his last transfusion) and I had only brought him to Ms Lai 2 days ago for a checkup! I can't believe I can be so complacent! What was I thinking?
When he started coughing, I was right to think it was a virus, because he got better after few days of nebulizer. Normally, his cough would stopped if he has no wheeze, but this time it wouldn't go away. When I listened to lungs (I somehow through experience, mastered the art of telling if his lungs are well or not, even without using the stethoscope! hah!) On top of that, his appetite was normal, he drank well, pooed like his usual self, so I didn't think it justified going to the doctors.
When I came back from Tioman Island, I noticed he was coughing more, very chesty and had less appetite plus didn't sleep well, immediately I sprang into action!
Called Ms Lai, saying Adel might need antobiotic this time..it could be a bacteria infection! Ms Lai, checked his throat and said "inflamed"!!! Omigosh, poor child, how long did he suffer for this? I suppose catching a bug like this doesn't bother him as much as it would bother other normal kids. Did his recent progress lulled me into a false sense of security? I'll never let it happen again, I promise..

How did we do it? "It must have been really hard for the family and yourself"  that the comment frequently heard from relative and friends when they learn about what happened to us the last 2 years. Actually, it's now become like a natural part of us to take care of him, which we can't do without. Sounds strange. Of course, to get to where we are, hasn't been easy at all. It started out like I'd been left in the middle of the sea without a life vest and the only way out was to swim back to shore. Then it was followed by anger and denial. Slowly, it became acceptance and healing.

Perhaps, a year ago, I was trying very hard to "heal" Adel completely, wondering did I do this right, or not, and craning my neck waiting for the day Adel gets off oxygen & CPAP. I became so stubborn, that if I stumble, I'll get extremely frustrated. I demanded answers for my questions, hoping to get to the root of the problem. Don't get me wrong, it's not like I've stopped trying.. just that the mission has become somewhat passive. Yes, I want Adel to be healed completely, but if he doesn't I'm not going to get frustrated. I'll keep trying..and I know that he will get better..Is it what you call FAITH? I'm starting to understand what is like having faith. I've stopped being so hard on myself. I'm beginning to appreciate that with Adel, he has given me so much love, knowledge and friends that I've gotten to know, whom I never would have if not for Adel.

I'll always remember what a mom to a child with disability whom I met in the hospital shared with me : She said to me, "These special children are God special children and He has guaranteed them a place in heaven. Our special children refused to be separated from their parents, so as parents to these special children, we get a first class ticket to heaven to be together with them."

Whenever I feel the chips are down, I'll be reminded of what she has told me and I'll feel much better. It's through Adel that I've finally found the "way". The way of all religion, universe and all divide. I can't explained it, it's a personal way of knowing that you'll leave a legacy (not in the material sense) and that you'll be remembered long after you're gone.

We need to recognise that every child is different, and appreciate their individual talent. God is fair, He gives some and takes some from us. I'm very blessed to have very independent children. I suppose it's God's way of compensating for the time that I need to spend with Adel.

Adam's a Mr. Popular in school. I reckon he aspires to be a rockstar and footballer rolled into one? He's a all rounder, good in studies and sporty. Sometimes he gets carried away with the attention he gets in school and I have to remind him constantly that his schoolwork should be his priority. Adlan on the other hand, is Mr. Know-All, an intellectual "sponge bob". He doesn't need any prompting for schoolwork and he excels in them effortlessly. He cracks me up too. Just yesterday, his friend at school fell on him and injured his right foot. I received a call from his class teacher informing me on his swollen foot. When he came home, I took him to the TCM for a rub and a bandage then called his teacher to let her know that Adlan would go school the next day with sandals. Guess what, I found out last night that he wore a sandal on his injured foot alone and wore a shoe on the other! No wonder, I couldn't figure out in the morning why he left one side of his unused socks on his
desk! I laughed so hard and asked if anyone thought he looked silly, he just remarked casually that no one noticed! Nayli is an eager learner. Where her peers are still learning to read and write, she can read everything and anything (including my emails or text messages) and writes short letters. She reads all her school circulars and tells me the gist of them. I hardly had to ask her if she finishes her school homework or her Kumom Maths worksheets. She does them as soon as she gets them, sometimes on her way home from school. More than a year ago, while I was busy with Adel, popping in and out of hospital, she was playing with an interactive globe which I had bought for the older boys. She could point to at least 50% of the countries on the globe by self-learning. It was such a "party piece" that I would show her off to people that came to visit. She could pinpoint within seconds where most Asian countries, she could even point to Bosnia, Georgia, Guatemale, Honduras, Czech Republic, Poland and a whole lot of others in South America!! I was impressed. I hope she keeps up with her spirit of learning!

As for Adel, there hasn't been any expectations at all (not that I have high expectations of all my kids). I just want him to be with us for as long as possible. *holding back tears* My greatest fear in life is: (hate to say this).. that should any of my children precedes me... I just want to be there before them. So I can greet them and say, do not be afraid, mama's here..

Okay, I'm starting to ramble...to pen-off today's blog, I'm going to show off one of Adlan's essay "A Blackout" which I so kindly got from his teacher. I was in the dark about this unpolished gem of a writer. p/s can't get the essay to come down here..

Motherhood and Adel

Procrastination in a trait I’m most guilty of. I should have done this earlier. With so much happening in these 2 years, I’m won’t harp on it. I’d promise I would keep a blog on the joy and pain of motherhood, focusing mainly on my special no.4 and now I’ve done it!

I used to be driven career woman, now a full-time mom cum nurse to Adel, full-time chauffeur to my kids and a part time lawyer. Who would have known of the destiny that was to come and my destiny to provide and care of a child like Adel?

Adel is my fourth child and we are Malaysians, living in Johor Bahru (JB), the southern most city of Malaysia, bordering Singapore. My first two pregnancies were uneventful, so I took it for granted that every child is a healthy child. First, I have 2 beautiful boys, eldest Adam, 14 years old and Adlan 12 years old in my twenties.

Then I took a long break, in 2003 I became pregnant with my third child Nayli. Nayli was not an easy pregnancy. At 18-19 weeks I was referred to Singapore hospital from JB as the ultrasound scan showed Nayli’s stomach to be in her chest area, the gynae suspected Congenital Diaphragmatic Hernia. In Singapore, at Y Hospital Nayli was immediately checked and investigated for chromosome abnormality and the results were negative. It was confirmed that she was indeed a case of Congenital Diaphragmatic Hernia (CDH). We were given the option to terminate and a prognosis of 50-50 at birth, should we proceed with the pregnancy. By the time the chromosomes results were out, I was already in advance pregnancy-22 weeks. It was hard during that few days of deciding to terminate or not. In the end, I had not gone for termination although I was booked for it. After that, it was a harder 5 months pregnancy and I remembered crying every night to sleep and constantly asking God, “why me?”100x.

On 27th January, 2004, Nayli was born in Y Hospital, Singapore by normal delivery. I was 34 years old. She was delivered in the presence of a team of doctors and specialists and was immediately intubated and sent to ICU. Contrary to our expectation, she did extremely well after the surgery to her diaphragm at 2-3 days old, she was extubated 3 days post surgery and was soon in room air. She had a little trouble bottle feeding initially, but she latched on well, so she was sent home at 3 weeks old! She never went back. I breast fed her exclusively for a year. She is as normal as can be, very active and no one would have guessed that she was a CDH baby.

After what I had gone through with Nayli, there is a somewhat soft spot for babies. So, I didn’t think I should take precaution not to any more children.

When I conceived again with Adel, I was 37 years old, a little old, well what the heck, let it be our last baby!

When I was in my 16 weeks, an amniocentesis was scheduled for him in same hospital as Nayli’s. On that day, the scan showed Adel had pleural effusion (fluid) in his left lung, the gynae whom delivered Nayli whispered, “Thalassemia”. I told him I was not screened for it, since no one suggested for me to do so. In Singapore, mandatory screening is done at 8 weeks. Since I’m Malaysian, and my primary care doctor was in Malaysia and I had had 3 normal children already, we didn’t think we needed that screening. Anyway, it was still a cause for concern. Having called some doctors friends and read some articles on the internet, we thought we would seek a second opinion. We got in touch with Prof G, an expert in maternal fetal medicine. Apparently, he is the only expert equipped to handle ante-natal tap on feotuses in South East Asia. He is based in X Hospital in Singapore, so we transferred there. They suggested intra-utero “tapping” of the fluid. We went ahead with the "tapping" procedure through a very long insertion via my abdomen. I had to be admitted for a night for monitoring and during the procedure, I had to be very still for 1/2 hour or so, I even had to stifle a cough or a sneeze! After monitoring on daily basis, the fluid resolved after a week or so. We were pleased. Amniocentesis results from Y Hospital did not show any abnormality-good! So we thought we would have a normal pregnancy after all. But our joy was short-lived.

After that, my husband and I were asked to do screening for Thallassemia as the gynae in X noticed my blood count wasn't great. When the results came out, it was shock for us to find out that both of us are carriers of Beta-Thallassemia gene. So, another amniocentesis was scheduled urgently to check Adel again. The bigger shock came when we were told that the Thallasemia test result on Adel was positive. I was then 19 weeks. About the same time, we were also told of Adel having a big hole in the heart (VSD). Given that Adel is a Beta-Thal major, with a heart defect and had history of pleural effusion, I was unsure if I could do it again. On the other hand, I believed in the medical advancement which had given Nayli new a lease of life. My husband was adamant and he was totally against termination. Naturally, I was depressed, but I went ahead and continued the pregnancy.

Comparing the time when I found out about Nayli and Adel, I guess for Adel I was more composed. We started to meet parents whom have Thallassemia children, and learnt more about Thallassemia. It doesn’t seem so bad after all and I was also beginning to accept my fate to care for a Thallassemia child. The part on his heart defect or lung problem, we hoped for the best.

On 27th May 2007, I agreed to be induced for delivery in X as Adel was showing signs of Intra Uterine Growth Retardation. Adel was born at 36 weeks weighing only 2 kgs. He cried extremely loudly and breathed in room air! - They had expected him to need some help since his left lung was under-developed due to the fluid earlier. To us he looked normal, was as normal as my other children, he could latch-on my breast, he fed very well but was limited by the doctors on his intake due to his heart problems. He was confirmed to have 2 heart defects, PDA, a large VSD which needed repair and a possible coartation of the iorta, had pulmonary hypertension and bearing in mind he is also a Thal-Major. They ran all necessary tests on him, cranial scan, MRI, hearing test and the usual metabolic tests, he passed all the tests. We were eager to take him home, so we scheduled his PDA ligation together with coartation repair when he was 2 weeks old.

On 11th June 2007, he was wheeled out of the OT with only PDA ligation, the surgeon decided not the do the coartation repair as the gradient measured before and after PDA ligation did not justify a repair. Our joy was shortlived, as a day later, his blood pressure started to drop. On the 13th June 2007, they had asked to reopen Adel’s earlier wound to do a coartation repair, we agreed. However, in the middle of the surgery, they called us and informed us that an emergency open heart surgery was conducted in an attempt to save him. We later learnt that the surgeon couldn’t achieve the gradient after 2 attempts via the previous opening from his back.

The open heart surgery took forever, when he was wheeled out from the OT, we couldn’t bear to look at him, he was wired and hooked up to tens of drips and tubes. Adel was intubated for total of 28 days, he had pulmonary hypertension which needed nitric oxide, had 3 chest tubes inserted in his lungs to drain fluid due to numerous infections to his lungs. Miraculously, he overcame all of these, was extubated and put on CPAP (non-invasive ventilator) for a further month or so and finally was on oxygen cannulla at 0.1 litre per minute, which was very negligible. However, his voice was not audible although he cried lustfully. We were informed of his “vocal cord palsy”, which is a known-risk associated with coartation repair. It was hard to accept that our child’s voice may not be as normal as we wanted, but we were consoled and were thankful that his brain function is normal. His gross motor skills didn’t match a 2-3 month old at that time, but he seemed alert, focused well and most importantly he cooed when mummy came to visit and he seemed in touch with his surroundings. We were ready to take him home on oxygen, so we can move on with our lives.

Since his traumatic heart surgeries, he has been on regular blood transfusion at 5 weeks interval.

Repeated hospitalizations : Sept 2007-February 2008

On 23rd August, 2007 when he was almost 3 months old, we took him home. I was trained to insert Nasal Gastric (NG) tube and CPR etc, we also hired a home concentrator to generate oxygen for Adel and bought an oxygen tank from the vendor. He was termed Beta-Thal major with Chronic Lung Disease secondary to prolong ventilation. I also noticed then that his abdomen was a little fuller than normal. We were told of his enlarged liver and spleen probably due to the multiple infections he had after his heart surgery. Nothing was done except to follow-up regularly. We were told that his lungs would mend by 1 year of age and that his heart repair was satisfactory, no gradient, no leak to the VSD patch.

When he came home, we struggled to look after him. We were assisted by 2 extremely brilliant neo-natal nurses from the local hospital. Despite all our efforts and me giving him expressed breast milk, he was hospitalized almost every month for chest infection/exacerbation of his Chronic Lung Disease and often had audible wheeze. Finally after 5 hospitalisations in X, we decided that there was something was not right and asked for his lungs to be checked thoroughly.

Adel was referred a pulmonologist and undergone a bronchoscopy at 9 months old under general anesthesia, at the same time X also took the opportunity to repair his hernia. He did well, we came off the ventilator immediately after that and went back to his nasal oxygen and didn’t need to be sent to the ICU. The pulmonary specialist confirmed bronchomalacia (floppy airway) and also Adel’s left main bronchus to be compressed and ordered for a CT scan of his heart and lungs. She suspected heart compressing on the left lung. However, the cardiologist overruled her. So, we also got a CD copied of his CT scan and sent to the Malaysian National Heart Institute for review. In Malaysia, the findings of his heart was consistent to X, no adverse finding on his heart, in fact their feedback were positive of his heart, however, the reason that his heart was enlarged and has not shrunk was probably due to his compressed left lung. After the bronchoscopy, X suggested to put Adel on non-invasive ventilator (CPAP) instead of the nasal cannulla Adel was using at that time. So, we complied we bought ourselves a new CPAP machine in addition to his home concentrator which we had also purchased about the same time. Adel did well after that and for the next 6 months, (i.e between Feb 2008-August 2008) we didn’t get hospitalized. During that period, he only went for his 4-5 weeks regular blood transfusion and pre-transfusion hemoglobin count was usually 8+ or at worst 7+. During that period too, his review were good. Physically he was not up to date, fine motor skills were up to date and his oral feedings were poor. He developed oral aversion, because we somewhat relied on Nasal-Gastric (NG) feeding, as advised during those monthly hospitalization for fear that his occasional choking or breathlessness may caused his milk to aspirate into his lungs causing aspiration pneumonia. However, we maintained a degree of oral feedings, because he was a keen drinker, always asking for his bottle by gesturing. He refused spooning. Around this time, his nurse also pointed out that his lower spinal bone was a little arched. We thought was a Thallassemia trait. We didn’t query. We celebrated his first birthday, and he came off CPAP & oxygen for almost 2 hours!! We thought we were on road to recovery…

At 15 months old, we decided to give him a gastrostomy, since the NG tube might be hampering his oral development as we were told by the feeding specialist.

Downhill period : October 2008-April 2009

On 11th August 2008, Adel undergone another general anaethesia for key-hole assisted gastrostomy and at the same time the ENT specialist checked his vocal cords via laryngoscopy. Left vocal cord palsy was confirmed. He did well, he came off the ventilator immediately and wasn’t sent to ICU. The paediatric surgeon made a comment about his very large spleen and liver, however, it was left at that since his blood transfusion was within his baseline of 4-5 weeks.

Things seemed to go downhill after his gastrostomy. It seemed like he was only well for less than a month after his gastrostomy. To be fair, Adel’s oral skills did improve after we gave him the “button”. He drank 50-80mls in less than 5 minutes about 4-5 times a day without choking. But it wasn’t enough for him to grow.

He seemed bloated after we supplement his intake through the button. He would cry incessantly when he saw us coming with milk to put into his “button”, he rather drank himself. But, the more he drank, the more energy got expended and he got less nutrition to grow. He got upset after every bolus feed through his button and arched. He settled down after we vent out all his milk from his button. It became a vicious cycle of feeding and venting. We started going in and out of X again. However, each time there had been no diagnosis, “just reflux and gas bloat due to his CPAP” we were told and we were asked to give smaller feeds but frequently. We questioned the doctors again and again, and asked why was “gas bloat” not and issue before he had his gastrostomy, as he had CPAP ever since he was 9 months of age and was actually getting better. In fact before his gastrostomy, we often freed him from his mask and took him for a walk lasting an hour or so, twice a day. Ever since Sept, we were unable to do that. Adel would get nervous getting off, just for a mere few minutes for bathtime. At that time, we used the gastrostomy as our “marker”. We thought that the gastrostomy had somewhat disturbed the balanced in Adel and quite regret going for it. We were given more medication each time we went back to X. It was during these period that Adel gave us 2 scares, both times during the times we took off his mask, moving his equipment into his room, he turned blue gasping for air, we revived him ourselves, using our own method. We learnt the hard way that Adel couldn’t come off oxygen, anymore!

In December, he also became so anemic, we noticed that he was pale even though he just transfused in less than a fortnight. At his scheduled blood transfusion in early December, we got to know his platelets were very low and his hemoglobin count 6! After his transfusion he would be quite happy for a few days and again became very anemic within 2 weeks or so. We put him on higher oxygen and CPAP setting ourselves and gave him frequent nebulizer. We struggled with barely few hours of sleep every night late last year (till April) and it became almost unbearable. At 2.00 am on a night in early January this year (I can’t remember the date) Adel cried uncontrollably and suddenly collapsed. It was the worst scare I ever experienced with Adel. I had seen him turning blue or had apnea (breath holding) episode on few other occasions, but this time it was "real", he had no pulse for a few minutes. I panicked, couldn’t decide if I should call the doctor, ambulance or what. Then instinctively, I grabbed the oxygen tank while my husband slapped both sides of his cheeks him to wake up. On driving the oxygen at full blast of 15 litres per minute, he came back! We were so afraid his brain was deprived of oxygen during that crucial few minutes. He seemed okay, what a relief!

We wanted to take him to the hospital, but we knew there is nothing X could do except accrue a new hospital bill. So, we waited patiently for his next review. Meanwhile, we purchased 3 more 13 litres oxygen tanks (oxygen tanks can drive 15 litres/m as opposed to the oxygen concentrator which delivers maximum 5 litres/m) so Adel could have pure oxygen throughout the night when he slept. That is to minimise the risk of desaturating or worse still another collapse.

On 12th January this year, the cardiac review showed that his pulmonary hypertension had escalated. That explained why it was impossible for Adel to sleep through the night. The cardiologist added that Adel’s abdomen had grown very large and splinted on his diaphragm causing him to be more breathless. It was that time when splenectomy was mentioned to resolve hypersplenicism (a condition where the spleen traps all the red blood cells causing anemia). Immediately we wrote to the X hospital expressing our disappointment in their inability to help us find the root of problem. We asked them to refer us to another hospital which can assist us to find the problem if they find Adel’s case too daunting. In fact, since Sept, we had been dismissed by them and resorted to our own method of comforting our sick child. We often refer to our good doctor friends who are at our back and call, helping us alleviating our child’s discomfort.

In February, a conference (the 2nd one already) was called attended by the X Hospital’s hematologist, pulmonologist and Adel’s paediatrician/neonatologist /paediatric surgeon and the hospital management. They were of the view that Adel’s spleen ought to be removed first. This time, we didn’t take their words as gospel truth; we asked to get their counterpart in Y Hospital Singapore to verify. Meanwhile, we also intended to take Adel to Australia, to get him thoroughly checked. Having spoken to the doctors in Westmead Children’s Hospital in Sydney, we were discouraged to take Adel there, we were assured that the Singapore doctors are well equipped to manage Adel.

About this time, we also started to feed Adel continuously using the feeding pump (the kangaroo pumps delivers milk by droplets through his gastrostomy) to minimise his abdomen distention hence discomfort, and also not to aggravate his already oversized abdomen. The continuous feeding will ensure he gets the nutrition so he can fight whatever disease it was and not lose his precious weight. Thus, our list of home care equipment got longer and not to mention, we also have additional task of getting his oxygen tanks refilled everyday. Around late February, we met up with 3 doctors in Y Hospital whom were kind enough to visit Adel in X (during Adel's February hospitalisation), they were a paediatric cardiologist, a paediatric hematologist and a paediatric pulmonologist from Y Hospital. They were so sympathetic with what we had gone through with our child. We raised our concerns on splenectomy on a young child like Adel, who needs his spleen to help him fight bacteria infection. We were told of a procedeure called embolisation. Unlike X, they also thought the size of Adel’s spleen and liver were out of proportion to a Beta-Thal major child. We immediately transferred Adel to Y and got him investigated. They took some time, as Adel’s medical history is so complex. It was the first time we heard of “storage disease- Gaucher” from the geneticist and the pulmonologist in Y whom were trained in John Hopkins University. However, we were unable to confirm this since, Adel often had blood transfusion and the blood test after each transfusion is inaccurate. At first, I was in denial, “Not possible, Adel’s is very clever boy, his understanding of his surrounding is superb, he can communicate with us, although limited in his speech, not due to anything but his left vocal cord palsy!”, you can hear me retorting to the doctors. The ophthalmologist checked his eyes, he is normal. Physically he is weak, but not mentally. But, when I started reading more on Gaucher in the internet, it became clear to me that it is possible that we might have missed it.

The frequency of Adel’s transfusion gotten worse around March this year, it became weekly and his HB would drop to an alarming 6+. We would go home after a transfusion and then admit within a week or days for another transfusion. While Adel was in hospital, they would get as much investigations done as possible. On 23rd April, this year, we went ahead with embolisation immediately after a CT scan of his liver under general anesthesia, a non-invasive procedure to “ligate” part of his blood vessels to his spleen in Y by Prof L (he is the most experienced radiologist in this region, I reckon and a kind, kind man). We were worried for him as his lungs condition had gotten worse and he may not take general anesthesia as well, like before. After embolisation while he was still under anesthesia, Prof D (a wonderful pulmonologist) checked his lungs thoroughly by bronchoscopy. I was told they had to give another round of sedation because Adel was waking up quite quickly. Two rounds of anesthesia, I was truly worried. True enough, it was hard for the first week after that ( I think the Paeds ICU staff kind of freaked-out how unexpected our little Adel is, he desaturated drastically within seconds and had to be bagged continuously, so they had to sedate him more to calm him), they also had to call in nitric oxide therapy to reduce his pulmonary hypertension. But, as always our little fighter came through, extubated after 10 days and back on his CPAP/oxygen mask. We are proud to say, Adel may be the first child to be successfully embolised in Singapore/Malaysia (maybe South East Asia) for hypersplenicism!

May 2009 till now: Better days ahead?

Ever since embolisation, Adel’s platelets shot up! He also started with a new drug, Sildenafil. (Viagra) for his pulmonary hypertension. Now, he is holding on to a 3-4 or even 5 weeks transfusion. Just this 18th May, before his blood transfusion, his blood was taken, flown to Australia to check for Gaucher. Hb before transfusion was 7.8 (not too bad) and platelets were high (previous transfusion 3 weeks).

Three and a half weeks later, his pre-transfusion Hb was 9.1. Wow, never before. Guess what last week, we went in to Y for a scheduled blood test (to draw blood to check for enzyme glucocerobroside in order to complete and rule out Gaucher) prior to transfusion hb was 9.8!! Last transfusion was almost 4 weeks! So we went home without blood transfusion! Yay! So now, we are hoping he can maintain a 5 weekly transfusion.

On his oral development, I'm glad to say, Adel, now supplements 1-2 bottles of 150mls milk daily, delivered via the feeding pump to his gastrostomy. Which means he takes 80% of his milk orally. He is now more agreeable to spooning and attempts to lick/tastes some food. His record is 13 spoonsful of diluted porridge.

We are hopeful and will do whatever it takes for our little precious fighter. Perhaps a year ago, I was sometimes angry and often asked God “why me, why my child”. I’m glad to say, this child has given me great strength and peace. My hardship and endurance pales in comparison to what he is going through. Although the journey is long and arduous, I’m willing to give up everything I have, just to give Adel a longer, more normal life.