More often than not, we call them autistic as “may sariling mundo” (confined in their own world). But unknown to us, there is a lot that is going on inside their brain that makes them behave as such…

Carly(photo courtesy of

I have come across this video about an autistic child who was able to breakthrough from her body and is now able to express intelligently what transpires within her body. Those we perceive as hurtful purposeless flinging of extremities, banging of the head to the floor or wall, apparently has an explanation…

“You dont know what it feels like to be me, when you can’t sit still because your legs feel like they are on fire or it feels like a hundred ants are crawling up your arms. People look at me and assume I am dumb, because I can’t talk. It’s hard to be autistic because no one understands me. What do I want? I want to be like every other kid. But I cant. Because I am Carly.

I am autistic, but that’s not who I am. Take time to know me, before you judge me. I am cute, funny and like to have fun. I think people get a lot of information from so-called experts, but if a horse is sick, you don’t ask a fish what is wrong with the horse. You go right to the horse’s mouth.”

When asked why she bangs her head to the floor, she said: Because if I don’t, I feel like my body is going to explode. It’s just like when you shake a can of coke. If I could stop it I would, but it is not like turning a switch off. I know what is right and wrong but it’s like I have a fight with my brain over it.”

“I want to be able to go to school like normal kids, but not have them getting upset, or scared if I hit a table or scream. I want something that will put off the fire.”

“I want people to know that no one is telling me what to say and I don’t have a hand at my butt like a puppet.”

Therapist asks her, Carly, why do autistic kids cover their ears, flap their hands, hum and rock?

Carly replied: “It’s a way for us to drown out all sensory input that over loads us all at once. We create output to block input.”

“Doctors would tell … that we have a hard time processing information. It’s not really true, our brains are wired differently. We take in many sounds and conversations all at once. I take over a thousand pictures of a person’s face when I look at them. That’s why we have a hard time looking at people.”

“Dear Dad, I love when you read to me. And I love that you believe in me. I know I am not the easiest kid in the world… Father, you are always there for me holding my hand and picking me up. I love you.”

“I think the only thing I can say is don’t give up. Your inner voice will find its way out. Mine did.

Carly, you truly are inspiring. Your voice was very significant in helping me change my perceptions of how autistics behave, what autistics are having in their brains. Now, I would say you’re not confined in your own world. You just have a lot of stimulation that makes you counteract it by those pervasive movements. Now I know why… Thank you Carly.

Carly: Autistic, Intelligent

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Posted by on April 23, 2013 in Uncategorized


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sotto (a) verb – copying one’s work and claim it as your own. (synonym: plagiarize); (b) noun, the act of crying on national television to claim a son died because of wife’s intake of contraceptive pills that came out to the public 3 years after the death of the son; (c) noun – a person who claims he was sent by God and whose mission in life is to oppose passage of reproductive health and responsible parenthood bill into law (synonym: bigot)

mitos magsawsaw (a) verb – the act of commenting on all administration-related issues; (b) noun – the state of denying once a close ally in order to advance own personal agenda (eg, “I am not close with GMA… we only see each other during house sessions”); (c) adjective – an attempt to sell yourself as feisty, fearless, ready to fight anyone. in other words, mongrel; (d) the act of claiming you follow the administration’s tuwid na daan but back lashes after being categorically denied of joining the administration bandwagon

enrile (a) noun, the act of claiming your ambush was staged to support passage of a martial law but in later life deny it in your memoir

lacson (a) noun – male with a macho first name but adapts a sissy nickname and categorically denies he is gay… and will never be gay; (b) adjective – a policeman who is cleared after being embroiled in rubout and deaths of people of influence

arroyo – (a) adjective, the butt of all misfortunes that beset’s a country; (b) noun – a petite woman who claims she will not run for election after unseating a popular president but still runs and wins through cheating; (c) noun – the original “amalayer”

dick (a) verb – to stand erect when stimulated…. by calamities, head a national organization, mobilize people and later on use this as a propaganda to woe votes during a national election; (b) noun – a tool used to penetrate an imperforate anus… just so your campaign slogan would rhyme

noynoy (a) noun – a balding head of the state; (b) verb – to delegate a task to your subordinates and take all the blame if they did not deliver; (c) adjective – someone who blames his fault to a predecessor, including the arrest and crucifixion of Jesus (via @hecklerforever)

miriam (a) noun – a person who asks God for a second life to be able to investigate erring public officials, including God; (b) noun – a person accused of using public funds to buy grocery, pay salary of maids and pay rent to own building

chiz (a) noun – an arrogant, alcoholic man who sprawls on his girlfriend’s house’s sofa; (b) adjective – an ambitious man who asks for a campaign fund but denies he needs a haciendero to be able to pursue a presidential campaign; (c) noun – a person who uses neuroleptic, hypnotic technique when talking in front of the press to captivate an audience; (d) adverb – talking in verbally-rich sentences that only translate to two to three English words after each sentences

llasos (a) adjective – a person who is gay but deprives himself of sexual gratification because of vow of purity and celibacy… ahhhhhh shet! adu la amin!

rh bill (a) noun – the reason for all the calamities in a Catholic country. (antonym – wrath of God)

nancy (a) noun – any person who served as an executive assistant of a parent that becomes instantly qualified for a senatorial seat

jamby (a) noun – a person who offers a contest with luxurious prizes (eg, ipad) to woe votes; (b) noun – someone who claims to be pro-poor but doesn’t know the prize of the food poor people eats

sonny (a) adjective – gwapo. ’nuff said. (hahaha bias… e definition ko to no, may angal?)

eric (a) verb – to dance as a campaign for any health related programs


Fr. Dakay of Cebu criticized the new pope for taking a bus,. He said the new pope (as a cardinal in Argentina) would cook his own food or take public transport. “just because he knows how to cook doesn’t mean he has to cook his food. And I don’t think Buenos Aires will allow him to take a bus. He has a car. Moreover he added that “sobra ra sab na kaayo nga pasakyon na lang mi tanan og Ceres (bus) — (It is too much to have us ride a bus. That is no longer right.) (From philstar the freeman editorial, March 18, 2013 12:00 am.)

Nang dahil sa yo Fr Dakay, mas lalo dadami pa ang magiging Katoliko. Nice job!

Have a solemn reflections on this Holy Week. In other words, enjoy your vacation.

Follow me on Twitter: @simply_clinton

DICK-shunari, DAKAY

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Posted by on March 27, 2013 in Personal, Politics


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— Politics: Devil’s Game

Lucy Torres: “This strengthens my resolve to continue to fight, ganito talaga ang pulitika. Laro ng demonyo,”

Taning: hiyang hiya naman ako sa yo oy! Nananahimik ako dito, bakit na naman ako nadamay, porke nadethrone ka lang. E di ba champion ka?

— Collateral Damage

Magulang ni Heart Evangelista, pinakiusapan si Chiz, live on TV, na makipag cool-off sa anak.

Chiz Escudero: “Hindi ako showbiz indi ko pinagsasama at di ko pinaghahalo ang trabaho ko sa personal kong buhay at lalong hindi ko po pinapayagan na manghimasok ang personal kong buhay sa aking trabaho.”

Chiz Escudero, pinakiusapan ng mga Ongpauco na lubayan na si Heart Evangelista. Hiyang hiya naman kami sa standards nyo.

Halla! 28 na yang anak nyo, di pa ba nagme-mens at pinanghihimasukan ang love life? Pang-ilang relasyon na niya ang pinakialaman nyo? Mamaya, mag-faint ulit siya dahil sa makapal na usok. Charot!

— Award Winning

Eddie Garcia, hinirang na “People’s Choice Favorite Actor” (sa pelikulang Bwakaw) nitong nakaraang 7th Asian Film Awards sa Hongkong.

Samantala, si Nora Aunor naman ang nag-uwi ng Best Actress trophy para sa movie na Thy Womb.


— Ambush

Reliable source: Convoy of Jack Enrile in Nabunturan, Compostela Valley were fired at in Mawab, Davao Oriental | via @marizumali

Karamihan sa nagrereact, sinasabing peke ang ambush; like father, like son. (Me: Kawawa ka naman, di ka na pinaniniwalaan ng tao, kahit pa siguro totoo.)

Kris Files TPO vs James Yap

Kris Aquino, nagfile ng TPO laban kay James Yap dahil umano sa isidenteng nangyari sa bahay ni Kris noong Disyembre. Ayon sa reklamo ni Kris, umano ay gustong makatikim ng “human rights” aka “marital obligation” ni James kaso nabigo siya, dahilan na siya’y makapagbitaw ng mga salitang hindi katanggap-tanggap. Nakita raw ng kanilang anak ang pangyayaring ito at at pilit pinapaalis ang ama.

James Yap, pinabulaanan ang akusasyon ni Kris. Gusto lang daw niyang halikan siya ng bata bago umalis pero pag hindi niya ginawa, si Kris ang hahalikan niya. Noong umayaw si Bimby, saka niya nilapitan at tinangkang halikan si Bimby.

Chopsuey 2: Politics, Showbiz

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Posted by on March 19, 2013 in Personal, Politics, Showbiz


I got an interesting referral from one hospital. It is not really that difficult of a case but it is quite puzzling for the untrained ones, nevertheless interesting.

The mothers’s history is generally unremarkable. She only had some flu-like symptoms during the last trimester of the pregnancy. There was no history of hypertension nor diabetes. Mother’s prenatal visits were timely and regular as this is very much wanted pregnancy. Generally, she never had symptoms. She came to the hospital in labor. She had an ultrasound which stated that the baby is in fine condition, there was adequate fluid. About 12 hours into delivery the bag of waters ruptured. The mom recalled that she noted the fluid to be yellow-stained (normally it should be clear). There was no foul odor.

She gave birth by normal vaginal delivery. The baby was not that big, weighing only 2.6 kgs. There was no difficulty during baby’s delivery. There was no cord loop around the neck (that may have strangulated the baby). The fluid was viscid, thickly saturated with meconium (baby’s first stool). Few minutes thereafter, the baby began to have respiratory distress requiring oxygen support.

Baby had an xray of the chest revealing pneumonia. He was then started on empiric broad spectrum antibiotics. Baby was nursing well from the mother while on oxygen supplementation. There was no progression of the respiratory distress. Despite requirement for oxygen, baby remained comfortable. On the 5th day of life, baby still cant be weaned off from oxygen, breathing was still fast although comfortable. Repeat xray of the chest revealed significant clearing of the pulmonary infiltrates initially seen on the previous xray. An arterial blood gas analysis was done but it was unremarkable. So why then is the baby requiring oxygen despite the comfortable breathing, normal blood gas analysis and clearing of xray picture?

The baby was then referred to me at this time. At first I went with the line of unresolved infection so I suggested shifting of antibiotics as well as determination of c-reactive protein (an indicator of inflammation – often due to infection, that may as well be used to monitor response to treatment).

The CRP was reactive, meaning it indicated presence of an ongoing inflammation/infection, but the value was not congruent with the distress of the baby. So I tried to play along with some of the facts that the baby presented. First, baby was born throught thickly meconium stained amniotic fluid. Second, the baby’s initial xray findings highly suggested pneumonia. Third, the baby remained oxygen dependent despite ample time for antibiotics to have worked, granting this was supposed to be an isolated pneumonia. Fourth, despite improvement of x-ray picture, the baby remained, clinically, unimproved; was still dependent on oxygen support.

With these, I came to rationalize that baby might be having meconium aspiration pneumonia (MAP). (While meconium is supposed to be sterile , meaning it is free of bacteria, antibiotics was a rational modality of treatment as CRP was reactive). But on top of the MAP, I considered that baby might be experiencing as well a complication.

I then requested to obtain blood gas analysis from the right and the left arms, with emphasis on the pulmonary oxygen between the two sites.


The aorta, the main vessel that arises from the left ventricle (red vessel creating a loop on the above illustration) gives rise to three large vessels that supply the upper part of the human body. The first branch immediately divides into two, one serving as right subclavian artery (that which supplies our right arm) and right carotid artery (that branch supplying to the blood). The second main branch becomes the left carotid artery which also supply our head on the left side whereas the third main branch is the left subclavian artery which supplies our left arm.

There usually is a ductus arteriosus that exists in the fetus and closes permanently about 10 days after the baby is born. It usually arises after the right subclavian and carotid arteries, and before the left subclavian artery. Thus, by origin, the right subclavian artery is usually termed preductal while the left subclavian artery may be, most often, post-ductal. Since preductal vessels include those vessels that send blood supply to the brain, it is also then safe to assume that preductal blood picture also reflects the same blood picture that goes to the brain.

In cases of pathology when the pressure in the right side of the heart is higher than that of the left, and the ductus artery is still patent/open, the unoxygenated blood in the right side may dilute the already oxygenated blood in the left through the ductus arteriosus. In cases therefore involving increased right-sided pressure, the baby tends to be cyanotic from this explanation.

One way of determining whether there is a significant shunting from the right side to the left side via the patent ductus arteriosus is performing a 2-D echocardiography, which exactly measures the pressures between the two sides of the heart. Just compare your obtained pressure from the right side of the heart with the normal values for age and you can immediately say there is elevation of pressure, or simply put pulmonary hypertension. Another way of determination is to obtain blood gas from preductal (right arm) and post ductal (left arm, right foot, left foot) extremities. Then, compare the partial oxygen tension/pressure (pO2). A gradient between right and left of more than 20 mmHg is highly suggestive of right sided pressure, in this case, persistent pulmonary hypertension, of the newborn (also known as persistent fetal circulation).

The baby’s right arm blood pO2 was 81.7mmHg, whereas the left arm pO2 was 42.16mmHg. There was a gradient between right and left arm of 39.1mmHg, way higher than 20mmHg cut off, clearly suggesting that the baby has a pulmonary hypertension. Pulmonary hypertension usually arises when the small supposedly thin capillaries in the lungs that carry blood for oxygenation thickens. With thick pulmonary vessels, the transit of oxygen from lung alveoli to the blood vessels is rendered more difficult and hence the slow or lack of oxygenation of the blood that returns to the heart, making the baby a little bluish. And if the pulmonary hypertension is severe, this “resists” the incoming blood from the heart and is shunted directly into the aorta via the ductus arteriosus, unoxygenated, already diluting the blood that may have successfully went into the lungs for oxygenation (but not sufficiently). This can turn into vicious cycle until the baby’s demise.

In cases however of pulmonary hypertension, where the wall between the right and left atrium has a large communication known as patent foramen ovale, since at the level of atrium there is already mixing of blood between right (unoxygenated) and left (oxgenated) sides of the heart, there may not be an appreciable gradient of pO2 between preductal and postductal blood gas analyses.

Respiratory Distress?


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Carly Rae Japsen pulls out of concert for the Boy Scouts because the latter do not support gay rights. According to Perez Hilton’s blog, “Carly Rae Jepsen was set to perform at the Boy Scouts of America National Scout Jamboree concert in Mount Hope, West Virginia this summer, but considering that they’re very closed minded when it comes to gay rights — she decided to cancel her appearance.”

CRJ tweeted the following:




“The group Train was also going to perform at the summer concert, but after Carly dropped out of the lineup, they said the hell with the Boy Scouts… unless they change their anti-gay policy.” (source:

Carly Rae Jepsen Advocates Gay Rights


Posted by on March 5, 2013 in LGBT Rights, Personal


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(Image courtesy of

During my training, I was lucky to witness a case of hydrops fetalis undergo a rare form of management done in our institution.

During the prenatal course, the obstetrician noted the baby to have hydrops fetalis. Serial ultrasound established the diagnosis and at the time the baby was nearing birth, the condition persisted.

As in my previous articles, hydrops means the generalized swelling of a fetus. The skin becomes edematous and very taut, disabling a good expansion of the chest when breathing. The pleural space, where the lungs float is also filled with fluids, compressing the lungs. So once the lungs becomes occupied by air after birth, there will be difficulty of the lungs to expand. The pericardial cavity, which the heart occupies, may also be fluid filled. Abdominal cavity may also contain fluid, termed ascites. At least 2 of these 3rd spaces must be involved before diagnosis could be entertained in an individual.

On the baby’s latest ultrasound, it was noted that the right lung has pleural effusion, the left lung was spared. In order to manage the baby optimally at birth, a pre-natal conference among all parties involved in the delivery of the baby was called – the perinatologist, neonatologist, and pediatric surgeon, to discuss how the delivery process will take place. At that time that this was done, the unang yakap was still in conception so it was not employed in this particular delivery.

The perinatologist wanted to employ EXITextrauterine intrapartum treatment. This means that the treatment will happen when the baby is out of the uterus but is still within the confines of the delivery procedure (umbilical cord remains uncut).

1.The baby was delivered via emergency section. He was brought out of the uterus, the umbilical cord still connected to the placenta.

2. The pediatric surgeon inserted a needled through the right pleural space, drained fluid as much. (The purpose of doing this is to allow maximum expansion of the lungs when it gets aerated; the fluid, if not evacuated, will limit the expansion of the lungs leading to poor tidal volume, lesser oxygenation of the blood, which will aggravate the already compromised baby).

3. Umbilical cord was then cut and baby was handed to the neonatology team.

4. Neonatologist then intubated the baby.

5. Chest x-ray was done to document the level of the tip of the endotracheal tube, check how much of the fluid was drained, and check how expanded the lung after the procedure.

In short, the procedure was successful. We were able to ventilate the baby afterwards. Baby however had other pressing problems, and as expected he when through persistent pulmonary hypertension, or persistent fetal circulation. PPHN or PFC usually results from a thickening of the walls of the arterioles/capillaries that surround the lung alveoli. Because of this thickness, oxygen travels slowly from the alveolar space into the bloodstream, thus leading to lesser oxygenation of the blood. PPHN can be primary (no identifiable cause) or secondary (a result or effect of another primary disorder). In this case, if the baby, who has hydrops fetalis, also has PPHN, it would only mean than the hydrops was a chronic disorder that it has affected the development of the lung tissue and vessels. If the PPHN was primary, then the arterioles and capillaries are thick to begin with, and there’s no way to reverse this anymore. If it is secondary, it means that the arterioles or capillaries have thinned out for postnatal life but then became thicker as a response to/result of an insult/injury. Despite proper ventilation and support given to the baby, he succumbed to PPHN after several days.



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I was again given the opportunity to deal with a very interesting, intriguing, puzzling case in my career as a neonatologist.

I was called for the delivery of a baby presenting with hydrops as seen during prenatal ultrasound. (Hydrops refer to the generalized swelling of the baby, and is defined as the presence of fluid accumulation in third spaces including pleural, peritoneal, pericardial spaces, edema of the skin, as well as swelling/thickening of the placenta). Accordingly, the ultrasound detected fluid in the abdominal cavity and testicular sacs, but none wheresoever. The consideration of hydrops is dubious but still it was highly considered. I called on the different personnel who will be involved in the baby’s care later on. Meanwhile, the obstetrician has already spoken to the mother (the husband is overseas) and explained the possible outcome of the baby’s birth. (Hydrops usually is dreadful, some expire within an hour). They were preparing that the baby might indeed expire immediately after birth.

Upon baby’s birth, we noted that the abdomen was distended, scrotum was bulging, as big as an apple. The skin was not edematous however. It seems that baby did not satisfy the criteria for hydrops. Baby had a weak breath, thus I had to intubate him. In less than an hour, I took an x-ray to confirm presence of fluid in other body cavities. Liver was palpable on examination (though it may be normally appreciated about 2 finger-breadth below the right subcostal margin, baby’s liver was about 4 finger-breadth palpable); there was also a soft mass I palpated at the right side, seemingly an enlarged spleen. Pleural space (where lungs are) is free of fluid. This at least ensures that baby’s breathing would not be difficult, unlike when there’s pleural effusion (fluid in the pleural space), the fluid will prevent full expansion of the lungs with every breath, letting to less oxgenation of the blood, which will make the heart work double time. There was a single bubble (signifying air entry into the stomach), but none for the rest. This is understandable as the x-ray was obtained just minutes after birth. It will takes hours before the air swallowed will pass into intestines, then about 24 hours until the air passes out of the anus.

I requested for ultrasound to see what in the abdomen is causing this disorder in him. But to my and the sonologist’s surprise, we saw nothing but a large liver. There was no tumor, the intestines were being pushed down though, ebbing and bobbing into the scrotal sac. The mass I palpated indeed was the spleen. But these don’t seem explain why there is fluid in the abdomen, and the scrotal sac enlarged, as seen in the prenatal ultrasound. With a liver enlarged, I tried to work up the baby along that line. I tested for blood samples reflecting liver functions, decided to repeat the x-ray of the chest and abdomen 6 hours after birth, this will give ample time for air to pass through to the rest of the intestines distal to the stomach.

Repeat chest and abdominal x-ray showed passage of gas into the distal intestine. This can eliminate pyloric stenosis (part of the stomach before duodenum) as a possible entity. Albumin was low, so I corrected.

But this wasn’t of a significant help. It won’t still explain why there was herniation of intestines into the scrotal sac, nor the large liver. It may explain the fluid accumulation, but that’s just it. I was at a loss. I was at a loss what next step to take. There were no other organ systems showing abnormalities, which might suggest a syndrome. It was a clear cut gastrointestinal system derangement. I didn’t shame to call on a gastroenterologist to help me solve this puzzle. I didn’t want to miss a thing for the sake of the baby, who I am being asked to do everything possible to make him survive. (A herculean request).

The gastroenterolist also seemed puzzled by the baby’s case. A toss between malignancy and… it was hard to come up with differential diagnoses… On baby’s second day of life, his abdominal girth dramatically increased, but remained soft. There was feces meconium coming out of the baby’s anus, though the color was like that of a caramel; there was no vomiting. This tells you that there seems no obstruction of the intestinal tract. But we wanted to sieve and scan through and don’t want to miss anything. Even if it was initially mentioned that CT scan might not be of help, we still did it. Surprisingly, the CT scan of the abdomen revealed only a large pneumoperitoneum (air inside abdominal cavity, but outside the intestines – which should never happen in a normal individual), fluid accumulation, but nothing more. This then puzzled me all the more; air can only escape into the abdominal cavity if there is a perforation along the intestinal tract. I then referred the baby to a pediatric surgeon, who scheduled baby for an emergency explore laparotomy.

At the operating room, the stomach, duodenum and jejunum were intact. At the middle of ileum however, there was a perforation, and an atresia (obstruction due to non-recanalization of the intestinal cavity during the development). The atretic portion, together with the perforated segment was then excised, washing and evacuation of the spilled fluid and meconium into the abdominal cavity and creation of ileostomy were done. We marveled at the actual operative findings in this baby because we never expected it to be so. Gladly, it wasn’t a cancer or a tumor. So, in retrospect, the perforation occured even before the baby was born. The reason and when it happened, we dont know. This lead to leakage of fluid and meconium into the abdominal cavity that incited reaction from nearby organs, including the liver which became enlarged. There were adhesions also of the intestines due to inflammatory reaction to the meconium that was spilled. The liver then produced low albumin as well that lead to the edema formation.

Baby did well during the operation. No untoward complications took place. We will have to play close attention to the function of the intestines as we have yet to commence feeding.


One week after the discharge, he came back for his return visit to the office. I was surprised upon seeing him. He was so malnourished, his skin was wrinkled and he looked severely dehydrated. In the medical parlance, that is a condition we refer to as “failure to thrive.” Painful as it may be, I had to admit baby again, to “rehabilitate” him from his dreadful status. It seemed that while on ileostomy, most of the milk he has been taking was not absorbed. Instead, he was dumping them and thus the resultant situation. (This is a condition known as short bowel syndrome).

I referred him back to the surgeon for further management. Finally, we agreed to have baby operated on again, to reunite the intestinal segments that were disconnected before to create an ileostomy. After preparing baby for the said procedure, the operation was finally carried out. It took about 72 hours when we noted that baby has patent intestinal segments, he was having regular passage of stools, hence we started feeding baby, initially minimally, then gradually increasing. After he was able to achieve his full enteral feeds, the venous line was discontinued. We even documented this by doing a fluoroscopy, to really check if the intestines are patent. And yes, the dye passed through without impediment through the intestines, no retention whatsoever. This made us then confident that baby’s problem has been resolved. So, for the remaining days of baby in the hospital, he was being fed, he was moving his bowels regularly, his weight noted to be increasing daily. And we were happy to send him home.

He was asked again for follow-up return after a week. And again, baby surprised me. His abdomen was now bloated like a balloon. Again, this puzzled us once more. He seemed to be having Hirschsprung’s disease and this just contradicts how he behaved during the last hospital stay. Well, we had to abide by the baby’s presentation. The surgeon again created an ileostomy, repeated fluoroscopy and obtained biopsy specimen. Then baby was back to his problem of short bowel syndrome. It was not a challenge how to resolve this but with the help of gastroenterologist, we were able to minimize him dumping watery stool. Baby was discharged once more with confidence that his problem has been resolved. Our aim then is to nutritionally build baby and then when bigger enough, will do the corrective surgery.

For now, baby is stable, with a slow weight gain… I really hope it works this time so baby will recover fully before his next surgery.

A Puzzling Abdomen


Posted by on September 7, 2012 in Congenital Anomalies, neonates


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(Image courtesy of’s_curse)

Have you ever heard of the legend “Ondine’s Curse?” Would you believe it could happen in real life?

Myth says that Ondine, a water nymph had a mortal lover. He swore to her that “his every waking breath would be a testimony of [his] love.” Unfortunately, one day, Ondine found out that her lover was unfaithful, and thus cursed him: “should he fall asleep, he would forget to breathe.” Eventually, he fell asleep from sheer exhaustion, and his breathing stopped.

Seven years ago, when I was still a neonatology fellow, we received a call from south of Manila, referring a patient for transfer and further care. The baby was male, born full term from an apparently unremarkable prenatal background. I was the one on deck to fetch baby from the referring to the hospital, and it was an exhaustive three hours trip (one way, as SLEx was under construction that time).

The baby had distended abdomen. He also had several episodes of cessation of breathing (apnea) (which is unusual for term babies, unless there is a problem elsewhere, particularly the brain or lungs). Initially, infection was suspected but despite adequate antibiotic coverage, it did not resolve, hence the referral.

Once baby reached my training hospital, one thing we focused on was his enlarging abdominal girth. It seemed that baby has a form of intestinal obstruction — if baby feeds, the milk is retained thus he will vomit; on the other end, there was no passage of meconium – baby’s first stools. Work-up revealed that he seemed to have Hirschsprung’s disease (segment of the intestine does not have innervation/nervous network – thus the obstruction is not anatomic but rather functional). Usually, babies with Hirschsprung’s disease will only have a segmental involvement of the large intestine (colon); few though may entire colonic affectation (termed microcolon). Unfortunately for our patient, he indeed had microcolon.


Fig A. Microcolon on barium enema

(Image courtesy of


(Image courtesy of p://

Fig B. Normal Barium Enema (Note the contrast on the diameter of the large intestines between Fig A and Fig B).

Microcolon is a form of aganglionosis (meaning, the entire large intestine is devoid of nerve ganglia that’s responsible in controlling its movement/peristalsis, so that food can be moved from the stomach to the anus. Without such ganglia, the intestinal segment becomes obstructed, food remains at the stomach or other parts of the intestine, cause distention and retrograde efflux/vomiting. This is what our patient had.

He was referred to a pediatric surgeon, but operation to take out affected intestinal segment was no longer considered beneficial to the patient. If the entire large intestine is removed, it will eventually lead to malabsorption syndrome, possibly short bowel syndrome also, conditions detrimental to the baby. So only a segment was removed, and a biopsy of the proximal segment of the colon was obtained confirming absence of ganglia. If ganglion cells were absent in the proximal as well as the distal segments, then most likely the entire colon was devoid of ganglia as well.

After baby’s operation, we wanted to remove the respirator that had been assisting his breathing the past days, so he could breath by his own already. We were able to do this after a few days. Then while baby was already spontaneously breathing, meaning without the respirator, we noted that there were episodes of him becoming bluish (cyanotic), and when blood gas was done, he had been retaining carbon dioxide in his blood. At first it was a baffle, but then we noted that these episodes particularly happened when the baby was in his deep sleep. HE WASN’T BREATHING (apnea)! Curious that we were, we all trooped to our textbooks and looked for the definition of congenital central hypoventilation syndrome (THE CURSE OF ONDINE). We worked up baby further to rule out other possible causes why he always had apnea but all were normal.

CCHS is a condition wherein when awake, patients seem normal, they breath normally. But when asleep, they stop breathing, with progressive retention of carbon dioxide (hypercapnea) and low oxygen in the blood (hypoxemia). In normal individuals, when they have hypercapneic episodes, this will stimulate them to breath faster to expel the unwanted carbon dioxide. Unfortunately, patients with CCHS are not stimulated to rebreath, hence this may eventually lead to their death, if not awaken. Therefore, these patients will require ventilatory assistance while asleep, but not when fully awake.

But what is the connection between the baby’s Hirschsprung’s disease (Total Aganglionosis) and this CCHS? Could they be a manifestation of just one disease entity? Or is the baby just unfortunate to have both disease at the same time?

I was so lucky to have a very diligent resident. She was able to find a disorder pertaining to the abnormality of neural crests, or small nerve tissue, that migrate from the central nervous system to peripheral location, including the intestines. It seems that there is abnormal or no migration of this ganglion cells to the intestines, and thus, resulting to Hirschsprung’s disease. But if the absence of ganglion cells involve the entire colonic segment, then that leads to total aganglionosis (a worse variety of Hirschsprung’s disease). Some of these neural crests too eventually end up as chemoreceptors for our breathing. As chemoreceptors, it is their function to detect abnormal concentrations of carbon dioxide and oxygen in our blood. If it detects hypercapnea, then that triggers our brainstem to send signal and increase the work of breathing. In patients with CCHS, there seems to be absence of these chemoreceptors. A worst case scenario, is thus a combination of both aganglionosis, and CCHS in a syndrome called HADDAD SYNDROME. This is what out patient have. At the time my resident was checking if there’s registry for this condition, she saw about 44 patients worldwide so far. That makes our patient possibly 45th. In the Philippines, our case was the first reported one.

My mentor then explained the condition of the baby. He will be needing a pacemaker that will help him regulate breathing while asleep, but the technology is not yet available locally at that time. Alternatively, baby required to be on respirator when asleep, but that would mean intubating baby every now and then. The parents are young and well off, but they knew that this will eventually drain their finances, with no guaranteed positive outcome. They then decided bring baby back to the previous hospital that referred him to us, and discontinued other aggressive treatment measures for the baby. In less than 48 hours, baby expired.

Ondine’s Curse, Is It Real?


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New Global Causes of Child Mortality Data Released in Lancet, May 2012. (Reference: Global, regional, and national causes of child mortality: an updated systematic analysis for 2010 with time trends since 2000. (2012) The Lancet, Early Online Publication, 11 May 2012 doi:10.1016/S0140-6736(12)60560-1)

  1. The burden of mortality in children younger than 5 years varied widely across WHO regions in 2010, with the largest number of deaths seen in Africa (3·6 million) and southeast Asia (2·1 million). Breastfeeding has and is playing a crucial role in the survival of the infants.
  2.  In 2010, neonatal mortality was highest in southeast Asia (1·096 million deaths, 52·3% of regional deaths in children younger than 5 years), with 19·2% of deaths (0·402 million, UR 0·262—0·558 million) attributable to preterm birth complications, and 21·8% of deaths (0·457 million, UR 0·359—0·551 million) attributable to pneumonia in the neonatal and postneonatal periods.Of the WHO


The majority of mortality is being contributed by the newly born population. Perinatal mortality has been defined as mortality of either of the mother-infant dyad during the perinatal period, which is defined as the period from 20th week of gestation to seven days after birth. Infants dying in their first week of life then highly speaks of the quality of prenatal care their mothers received. On the other hand, infant deaths occurring between seventh to the 30th day reflects the quality of infant care and may not necessarily a sequela of prenatal care.

A good question to ask is, when do these neonates die? In a data review of the deaths of neonates by day locally, it showed that most infants do not survive more than 48 hours, with a declining incidence towards the end of neonatal period. This first 48 hours of life still coincides with the perinatal period, which then clearly underscores the fact that majority of our mothers are not receiving optimum and adequate prenatal care thus leading to their demise.


Deaths of Neonates by Days, Philippines 1998-2003. (MA Silvestre, 2009).

There has been increasing preterm birth in the last few years, and preterm birth complications have contributed significantly to mortality burden among children before their fifth birthday. Locally, pregnancies are terminated prematurely because of urogenital infection (triggering preterm labor), uncontrolled maternal hypertensive disorders, abnormal bleeding (placenta previa, abruptio placenta). Having adequate access to mother-child care providers will significantly improve this saddening statistics. The PhilHealth recently reported that out of the 1400 plus cities and municipalities, only 607 cities and municipalities have mother-child care facilities; LESS than 50%.


The peak incidence of mortality from pneumonia is the first six months of life. Pneumonia is a vaccine-preventable disease. There are currently two conjugated and one polysaccharide pneumococcal vaccines available in the market. Of these, it’s the conjugated vaccines that can protect the infant from pneumococcal death during the first six months of life, as the polysaccharide vaccine can only be administered when the child is at least 2 years of age. Unfortunately, the conjugated pneumococcal vaccine is not available in health centers due to its high cost. Majority of the population that flock to the government vaccination centers, who cannot avail of the said vaccine, are therefore vulnerable to succumb to this disease.

The newly born infant’s immune system is inexperienced, thus any infection immediately at birth may lead to infant’s death. During pregnancy, whatever antibodies the mother has through vaccination or active infection are shared to the fetus through the placenta. (Problem lies when the mother never had infection nor vaccination, there would be nil to share). These antibodies may be the only armamentarium the infant has, however do not confer absolute protection after birth, and wanes by around six months of life. However, mother also continues to provide additional antibodies to the baby after birth during breastfeeding; a process cow milk formula can and will never duplicate.

A person has two manners of acquiring antibodies – active: by producing during vaccination or active infection; and passive: through infusion of already formed antibodies during pregnancy, breastfeeding and infusion of immunoglobulin concentrate. Vaccination is a process whereby a weakened antigen (whether the organism itself of its by-product) is introduced to the individual in an attempt to trigger his immune system to produce antibodies against the offending organism, but the antigen is not strong enough to cause infection or disease by itself. Pneumococcal vaccination should then be accomplished during the infant’s first six months of life to preclude this peak incidence of death from pneumonia during this period.

What has been noticeable though, according to the article, was the mention of how breastfeeding is playing a crucial role in the survival of these infants.


Delaying the initiation of breastfeeding is HARMFUL!!! In an observational cohort done at Ghana, observing 10,492 infants who survived beyond their 48th hour from birth, the following were the findings:

  • there is an increased risk of death from infection with increasing delay of initiating breastfeeding;
  • if the breastfeeding was initiated only after 24 hours of life, there is a 2.6-fold risk of death;
  • moreover, if initiation was delayed, the infant was partially breastfed (meaning baby was also given cow milk formula), the risk increases to 5.7-fold. (Reference: Edmond KM, et al. Effect of early infant feeding practices on infection-specific neonatal mortality: In rural Ghana. Am J Clin Nutr.  2007; 86:1126-31.)

In Southern Nepal, frequent home visits were done among 22,838 breastfed newborns, who survived beyond 48 hours. It was noted that

  • in the first hour of life from birth, only 3.4% were breastfed;
  • within 24 hours of life, 56.6% of infants were breastfed;
  • babies who were partially breastfed (72.6%) were at higher mortality risk than exclusively (purely) breastfed infants- the longer the delay of initiating breastfeeding, the higher mortality rate;
  • mortality was higher among late (>24 hours) than early (<24 hours) initiators
  • neonatal death may be avoided if breastfeeding is initiated within first day or 24 hours of life (7.7%), but more when initiated within the first hour of life (19.1%). (Reference: Mullany LC, et al.  Breast-Feeding patterns, time to initiation, and mortality risk among newborns in Southern Nepal. J Nutr, 2008; 138(3):599-603).

During the latter part of 2010, through the efforts and cooperation from Dr. Mianne Silvestre and her colleagues from the Philippine Society of Newborn Medicine, the Department of Health and WHO-Philippines has come up with Unang Yakap, a sequence of essential procedures at the delivery room. This program include:
  1. provision of warmth and drying of the infant, simultaneously stimulating him to breath;
  2. uninterrupted skin-to-skin contact — this procedure allows baby to acquire maternal bacterial flora that will colonize his intestines that will serve to prime his immune system, delays onset of allergic disorder, and positive effect on the duration of breastfeeding (likelihood of prolonged breastfeeding);
  3. delayed cord clamping – helps prevent iron-deficiency anemia; and
  4. initiation of breastfeeding within the first 90 minutes of life.

(Unang Yakap video may be viewed in this link:

The DOH with Bureau of Food and Drugs has also strictly implemented the Milk Code. Among the provisions of milk code are:

  1. exclusive breastfeeding for 0-6 months;
  2. no substitute nor replacement for breastmilk;
  3. appropriate and safe complementary feeding should start from six months onwards in addition to breastfeeding;
  4. breastfeeding is still appropriate for young children up to two years of age and beyond;
  5. infant or milk formula may be hazardous to a child’s health and damage child’s formative development;
  6. advertising, promotions or sponsorships of infant formula, breastmilk substitutes and other related products are prohibited;
  7. other related product such as, but not exclusive of, teats, feeding bottles and other feeding paraphernalia are prohibited in health facilities;
  8. government and all concerned stakeholders must continuously accomplish an information, dissemination campaign/strategy, and do further research on the advantages of breastmilk and the hazards of breastmilk substitutes or replacements; and
  9. milk companies, and their representatives, should not form part of any policy-making body or entity in relation to the advancement of breastfeeding.
Another venue where breastfeeding is being contested by milk companies is the infant’s vision and cognitive development. Breastmilk naturally contains long polyunsaturated fatty acids (LCPUFA) in the form of docosahexanoic acid (DHA) and arachidonic acid (ARA). A breastfed term infant can synthesize his own DHA and ARA relative to the maturity of the enzyme required, in his liver, thus exogenous supplement is not necessary. Breastmilk as well already contain DHA and ARA, on a proper ratio, and works in ways not duplicated by milk formula supplemented with such LCPUFA. Studies have been done, comparing infants fed breastmilk alone vs those infants fed milk formula supplemented with LCPUFAs. After 39 months follow-up of these infants, it was found out that visual, cognitive and language did not differ among the different arms of the study. This then clearly indicates that addition of LCPUFAs to milk formula does not offer additional benefit compared to breastmilk feeding. (Reference:

Are You Aware? New Global Causes of Child Mortality & Breastfeeding


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A Gush Of Air

Premature baby, 25 weeks gestation, was referred to my service immediately only after he was delivered, as the mother arrived in the hospital in active labor already. With all my might, I drove hastily to attend to him as the first few minutes is very critical for baby’s survival – their breathing, their temperature regulation more importantly. Gladly, their referral was, baby is on 100% oxygen saturation with low flow oxygen via his nasal catheter.

Arriving at the NICU, I immediately attended to his needs. His x-ray showed a severe form of surfactant deficiency. Talked to the dad and explained the condition of the baby. He needed surfactant badly. Unfortunately, resources are limited, he cannot provide it as he recently resigned from work and no one else can provide support that very instant. Baby had to make do with mere ventilatory support… but with risks…

The first 24 hours of preterm was essentially unremarkable, he was stable. In fact, on the 25th to 30th hour of life, I began weaning off the ventilatory support. (amazing) But on the 36th hour of life, he started deteriorating. Ventilatory support had to be increased to the point of a peak inspiratory pressure at 40 cmH20. This was the only setting that he became stable… (but with a PRIZE!) When I noticed that he was stable, I decided to go home and have my dinner. But upon arriving at home, I got a distress call that his heart rate suddenly fell. It was gut-wrenching feeling… I immediately ordered x-ray while I rushed back to the hospital (a 10 minute drive without traffic for an amateur driver like me). Lo and behold, he had pneumothorax on both lungs! Not just pneumothorax, but TENSION pneumothorax. There was a point of rupture somewhere along the baby’s lungs where air escaped from into the chest cavity. The air leak kept on increasing volume, on an enclosed space, thus compressing the lungs like a tin can, as well as the heart. This compression disables the heart to pump blood effectively thus causing decrease in the strength of pulses, blood pressure, faint/distant heart tones.

(Not the actual patient. Photo courtesy of

I immediately inserted needle onto the chest of the baby, both sides, after proper preparation and giving baby analgesics, to drain the air from the chest, to stop compressing on the lungs and heart. But… it was not enough. There was continuous exodus of air, and the baby’s heart beat and oxygenation has not improved significantly; I thought that the air leak must be so large that’s causing continuous egress of air.

Needle thoracentesis. (Image courtesy of Of course, this was done with baby lying supine though.

Chest tube thoracostomy. (Image courtesy of

I called in the pediatric surgeon to insert a bigger tube to help drain the chest of air. This was indeed a pulse-raising scenario. Had I a heart condition, I could already have chest pain or maybe heart attack at that instant, that was how tension-filled the NICU was at that moment.

But… after all our concerted, heroic efforts to save the baby, he continued to deteriorate. We resuscitated him, but he didn’t make it. When the parents came in, the mother hugged me, crying. It was heart-breaking scenario, as baby was their first…

When all things were settled, I finally bid them everyone. I went to an open fast-food chain to buy myself a dinner. It was midnight. Then I drove back home, to rest… because in a few hours time, I have a scheduled CS delivery to attend to.

A Gush Of Air


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