On the way to the hospital in Dar es Salaam there’s a sight that slightly jars. This is not to do with the minibus trip which starts at “I left the stove on and what if my house burns down?” pace and usually hits a point where the cars are so stuck you’d imagine they’d wedged themselves in a tar pit.
On the way there is a collection of shops, open for the bustling people in the small city that spreads out from the front gates of the hospital. This is before you get to the “Curare Pharmacy” which seems a bit curiously named if they actually know that curare is the root form of drugs that paralyse your muscles and would kill you if you were left to your own thwarted attempts to breathe.
These are shops, decorated in the preening advertisements of big American soft drink corporations, which are part of a cemetery. The old tombstones hold their air under shading low trees and swooning blades of grass, seeming to slowly crumble in that space. We never have the time to stop for an abstract reminiscence among those graves but we can see where they rub up against the stalls selling Coke and chips to eat first so you need something to wash them down. Like Coke.
The tombstones arrest all thoughts for a moment as the minibus picks up speed closer to the hospital, before clearing the security gates on the way to the Cardiac Institute and our waiting patients.
The other reason the first shop stands out is the name.
“The Rich Also Cry Shop”.
The major operation we’ve been asked to help with this time around is for a particular condition called Tetralogy of Fallot. This naming dates back to a time when industrious people involved in medicine could claim signature diseases in a way that would stick.
Fallot was a French doctor who managed to put together a sequence of particular findings and have them named after him because he did that first little bit of detective work and put all four findings together. It’s possible he wasn’t the first to make the observation but he was the doctor who attached his name to the constellation.
To understand his four findings, maybe a refresher on heart anatomy would help. This is not to do with the poetic sort of heart anatomy that finds space for soaring highs and crushing, broken lows.
At some point while developing the heart develops into chambers. Some of these chambers are receiving areas and are called the atria. This is also not a real estate section. These atria receive blood. From the atria the blood passes through into the ventricles to hurry on to somewhere important.
When the blood returns at first it arrives at the right side of the heart, enters the right atrium, moves on to the right ventricle and from there jets out to the lungs to pass by the air sacs that provide space for the outside world. The layers between blood and air are so thin it’s as if a sheet of blood hangs suspended in the atmosphere, entering dark and spent and being painted bright red by an inrush of oxygen.
The blood, now embarrassed with its riches, returns to the heart but this time on the left side to be received in the left atrium. From there the next stop is the left ventricle, and then it flees onwards to the aorta.
So it’s fair to imagine whichever cartoon version of a heart catches the back of your mind and imagine a crucifix dividing it up into those four chambers.
The Four Stars of Fallot’s Constellation
The plumbing of the heart has many described variations. Sometimes the rooms are not walled off quite right and there is an additional doorway between two chambers where the blood shouldn’t be mixing. Any of the valves that act as doors between the chambers can be undersized, narrowed or have less swing in their function as a door. Whole blood vessels can be joined to the wrong ventricle.
In Tetralogy of Fallot there are four distinct findings. The right ventricle, usually a not particularly muscular muscle, has an overabundance of fibres. That’s the first part of the grouping. The area where the right ventricle leads up to the vessels to the lungs shows a degree of obstruction to the flow of blood, either below the valve or at the door or just about anywhere else. This is the second part. This makes the flow off to the lungs that much harder.
To go along with these two, the two ventricles have an opening between them, known as a ventricular septal defect. Part three. For many patients with such a hole in the heart, blood would mostly head back from the left ventricle to the right ventricle, circling back around the lungs.
For these patients though, the obstruction to those lungs means some of the blood feels it is easier to head to the left ventricle. This shunting of blood leaves it short of oxygen and the resultant mixing with the blood that did make it to the lungs that is in the left ventricle ready to head to the body drops the total amount of oxygen heading out to the body.
The last sign? The whole of the aorta, the big highway for blood to the body is shifted a little to the right, sitting a little over that hole in the heart.
Looks Blue and Squats
So these kids will sometimes be obvious because they have a hint of blue about them, thanks to that slightly underoxygenated blood. On top of that every now and then the overzealous muscle in the right ventricle can twitch right into spasm and when that happens the blood really struggles to reach the lungs. Less flow to the lungs means more of that oxygen-depleted “blue” blood getting out to the rest of the body and the patient actually turns blue.
At that point you might assume the patient does what comes naturally when you have something happening to your heart and lies down. Well the “doing what comes naturally” is definitely the case but they don’t lie down. They squat.
The reason they squat is pretty simple. When you squat you raise the pressure in the areas where the aorta is heading. That makes the pathway up to the lungs that little bit more interesting to that blood because just shifting across the hole in the heart becomes a little bit harder. Hopefully the blue loses some of its depth.
That “turns blue, squats and goes less blue” thing sounds perilous. It can be. At the same time, the blood flow around the body is generally continuous because at least the blood doesn’t slow down dangerously on the way to the lungs and stop reaching the left side of the heart at the rate you need to keep getting it out to the rest of you. That hole between the ventricles allows that blood to hurry on, even if it’s still a little blue.
So many blue kids …
The local team have been seeing lots of these kids turn up. Of the 88 operations they’d done in this spot, about one quarter have been for Tetralogy of Fallot, which is a really high number. You’d expect relatively straight forward holes in the heart to be presenting in overwhelming numbers. They’re not.
The locals have a theory about this. Kids with a relatively simple hole in the heart don’t present with obvious changes in colour or a history of squatting suddenly. They tend to look a bit malnourished, not grow quite as quickly and get lots of chest infections. There are plenty of other explanations for that. If they don’t get seen by the right person, they might just be put in a different category and die.
The right person might be a local doctor or health worker, but sometimes it takes the specially trained to pick these things up. Tanzania has about 45 million people with 4 or 5 million of those in Dar es Salaam. There are three paediatric heart specialists. They are all in Dar.
So the current theory is that the kids with Tetralogy of Fallot survive to a point where their symptoms are obviously something heart-related, while the kids with a simple hole in the heart like a ventricular septal defect with no other associated heart issues don’t get a diagnosis.
That’s the Masterclass
Dr Sharau and team aim to develop their skills on the patients they are seeing now. They set up the plan for this trip – a masterclass in Tetralogy repair. Almost every day they’ve done another repair with a little support and guidance from our visiting team to try and make the repairs as good as possible. This is no small feat.
The surgeon needs to safely hook up the plumbing of the cardiopulmonary bypass system (sometimes popularly called the heart-lung machine) to keep blood circulating around the rest of the body during the operation. The team then needs to take the necessary steps to stop the heart and after opening a path to the outflow of that right ventricle they have to perform a number of steps to remodel that muscle and widen the outflow tract enough to make sure there is no ongoing obstruction.
All while the heart is an entirely different state. After all, the heart won’t be empty, open and still once the operation is finished. If any level along the way isn’t right when you ask the heart to do its work again the obstruction will still remain but the defect between the ventricles will have been closed. That would mean there’s only a hard pathway up to the lungs with no chance for the blood to skip over to the left heart and keep things going.
The kid has a repair, but they have different issues.
For this trip things look to be going pretty well. Godwin and team seem happy with what they’ve achieved. The next challenge is to figure out how to make sure those patients who aren’t even reaching them get picked up with their much simpler holes in the heart. That will require work across a whole system.
Because there are kids out there who are never reaching the front gate of that hospital. They’re stuck somewhere else,maybe near some shops or struggling to get through the day, waiting to be found. It should never come to that.
The first post from this trip is right here.
There are way more stories from this aid trip hitting the screens over at the Open Heart International blog too. I reckon it’s well worth a look. Of course, while you’re there, you could consider chipping in (*nudge, nudge).