Why are Downs Syndrome sufferers living so much longer these days?

Just saw this stat in an article this morning

That’s pretty impressive. Well, still a fair bit under the average obviously, but comparatively pretty bloody good.

How did they manage to make such a big change in such a relatively short space of time?

Much better general medical care. Since the kind of conditions from which they are likely to suffer more than the general population are known and many are relatively easy to detect, their doctors make sure to look for those; early detection leads to better treatment and to better life quality and better survival rates. For example, they tend to have more cardiopathies: knowing this, the GP of someone with Down’s is more likely to stick an ECG into a “general checkup” than the same GP treating someone without Down’s.

Not better general medical care. More like better specialized care. Most people with chromosome disorders tend to have odd stuff. Some of that stuff can be life threatening…eg congential heart problems or whatever. There are actually still Down’s babies that are miscarried or stillborn b/c of the severeity of their medical problems.
Also, Down’s kids were still being warehoused and forgotten in places like Willowbrook, which might have contribuated to them not living as long.
Down’s people don’t have normal lifespans b/c all of them (including mosaic) end up developing early onset Alizheimer’s Disease.

I have a cousin with DS. He is in his mid 60s. One upside to the shorter lifespans of old was that they tended not to outlive their parents/caregivers.

One of the more common complication with Down’s Syndrome is a condition named The Tetrology of Falot. It is a four phase congenital heart defect which affects circulation in many persons, among them a very high percentage of Down’s Syndrome patients. It causes low oxygenation, impaired digestion, poor distribution of nutrition, and early onset of cardiac disease. In the late twentieth century surgery techniques were developed, and improved to provide repair of the Tetrology of Falot in younger and younger patients. The improvement of all aspect of health is dramatic. Among those who got the surgery early are the first Down’s Syndrome person to gain a Bachelor’s degree, and the first one to hold elective office.

The fact is that the heart defect contributes to the vast majority of the developmental difficulties associated with the condition, and it can now be corrected in utero! I was privileged to talk with the surgeon who developed the technique when he was still begging for patients. Fascinating stuff. When I met him he was limited by hospital regulations to infants older than 12 weeks. He said that cut his patients chances at normal development in half.

Tris

I think additional factors are the attempt to integrate people with Down’s into society, and encouraging them to engage in healthy activities.

Time was, someone with Down’s was warehoused in a big building, fed institutional food, given minimal care, given nothing to do, and had nothing to look forward to. Now - society says they should be educated as much as possible (it turns out some of them can even handle college), made as independent as possible, given health care for their physical problems, encouraged to eat healthy food, and exercise (not all of them are in the Special Olympics, but that’s the sort of thing I mean, encouraging these people to exercise).

I could see where those changes could have a significant impact on life expectancy for anyone.

Among those who got the surgery early are the first Down’s Syndrome person to gain a Bachelor’s degree, and the first one to hold elective office.

Who are these people?

Triskadecamus not all people with T21 have cardiac defects (about 50% do), and of those who do the majority do not have ToF. While cardiac abnormality is the leading cause of death in people with T21 in their first 2 years of life it is by no means a factor for everyone.

http://emedicine.medscape.com/article/943216-overview

I think a lot of the increase in life expectancy comes from better diagnosis and treatment of the conditions people with T21 can suffer from, including considering them as being eligible for treatment in the first place.

People with T21 suffer increased incidence of thyroid problems (which can seriously lower IQ and life expectancy if left untreated), leukaemia (which has a much better cure rate now than it used to), Hirschprungs disease (which can be fatal if enterocolitis develops) and other gut malformations. People with T21 also have weaker immune systems and decompensate earlier if they are septic.

Part of the increase must surely be down to ante-natal diagnosis. Nowadays in the USA you don’t continue a pregnancy affected by T21 unless you have the resources (both financial and emotional) to give that child the best care, and many pregancies affected by multiple complications (e.g severe cardiac defects, gut abnormalities and renal abnormalities) are terminated.

Ireland, for example has a much higher rate of Spina Bifida, T21 and congenital heart abnormalities than the USA- not because they aren’t diagnosed antenatally but because even though they are, parents here choose not to end the pregnancy.

FWIW a friend of mine who works in a residential home with the mentally handicapped (Down Syndrome people among them) since the early 1990s and who talked with older colleagues who were near retirement then says her impression is that the change is less in medical progress than in general standards of care - more money, and more general human decency being invested.

One is an actor, whose name I don’t remember, He played the “Blaster” in Tunderdome" I don’t know more about the second other than an article in a magazine about advocacy for institutionalized people that covered his election to the City Council in a small city in the US. Just news items of interest in my professional reading.

Yes, ToF is not the most common cardiac problem in T21 patients. It is the worst. But cardiac insufficiency of lesser syndromes are much more common than the general population. I was imprecise in my reference to ToF in my second comments, meaning rather that the entire range of cardiac medicine has greatly benefited the population as a whole for those with T21, and is a major contributor to the increase in lifespan mentioned in the OP. The endovascular techniques for cardiac surgery are certainly not the only medical improvements that have had an effect.

Tris

I wonder what effect the wide availability of prenatal screening and abortion of Down Syndrome fetuses has on the overall health and longevity of those Down Syndrome children who make it to term. By removing from the population all those children whose parents wouldn’t have wanted to do the ‘full court press’ with regard to medical intervention for a Down Syndrome child, we have now biased the sample.

My wife is a neonatologist, and often comments on a kind of feedback loop in decisionmaking about treatment of critically ill premature infants. If you expect that all such children will end up profoundly disabled, you will not pursue the most aggressive medical treatments for them, which can result in them having worse outcomes and results in a self-fulfilling prophecy. The poor outcomes are then used to justify the lack of aggressive intervention in the next set of patients; after all, you don’t want to just throw money away on useless treatment. If, on the other hand, some centers start to treat such children aggressively, and they can demonstrate acceptable outcomes, then that cycle can be broken.

An anecdote- our spina bifida kids get very good care in the area I work in. They’re delivered by elective c-section, their backs are closed day 1 of life, they are often shunted in the first week. There is a Community Paediatric Consultant whose sole job is co-ordinate the care for these kids- who are often under orthopaedic surgeons, plastic surgeons, spinal surgeons, neurosurgeons, urologists, general surgeons, neurologists, physiotherapists, occupational therapists, dieticians, continence advisers, psychologists and physicians.

I am aware of a child who was born in another country (first world, no universal healthcare):
His mother was not offered an elective section (she was told her baby would probably die during delivery, and that she’d be grateful later for the chance for a normal delivery with subsequent normal children)
His back wasn’t closed until he was a week old
He still wasn’t shunted when he came to us at 18months of age despite severe hydrocephalus
He had been managed entirely by the local paediatrician, a young doctor who had never had a patient with spina bifida before (due to terminations of affected pregnancies) and who was clearly out of his or her depth.

I have no doubt that the life expectancy of our spina bifida kids is better than that of the kids in the country that child came from.

Probably.

After all, I’ve often encountered people here in the US whose attitude is that if their child was found to have a defect like spina bifida prior to birth they’d unquestionably abort and “try again”.* The idea seems to be it’s easier to dispose of such a fetus than attempt to raise such a child. On the other hand, if you live in a society where abortion is very much less an option (due either to the law or social standards) the focus will be more on how to maximize the health and life of a such a child.

  • I encounter this meme sometimes when people discover my husband has spina bifida - the “I would abort any such child” usually comes up right after the “why did you marry a cripple?” question. Yes, they are usually more polite than that, but not always.

I rather suspect that the opposite is true; that the greatest focus on maximizing health and life of such children comes in a society where the vast majority of such children are born to parents who deliberately choose to keep them. Volunteers vs. conscripts, and all that. Strictly IMHO, of course.

Are you sure about that? Yes, those who choose to keep a child with a serious birth defect are presumably those most motivated to care for such a child… but they may not be people with the resources to care for such a child.

Particularly in regards to medical care in this country, if your child has a chronic condition and you aren’t wealthy your child will be at a serious disadvantage for his or her entire life.

My husband has “mild” spina bifida - he is, for example, able to walk unassisted (although he has a bad limp) and no hydrocephalus (so he never needed a shunt). Nonetheless, he has had to deal with medical issues stemming from his birth defect all his life. He maxed out (hit the lifetime limit) on at least one health insurance policy by 21. Getting him insured THESE days is a trial - he’s currently in a special state program, but that program has to be renewed year to year and there are always threats of it being cut. The last time we looked at the individual insurance market he was quoted a premium of *$1,200 per month! That’s … not possible for us to pay. We just don’t have that kind of money. AND on top of that no one would cover anything to do with his “pre-existing condition” which covers… well, a hell of a lot of him, really. $1,200 a month for insurance that wouldn’t cover any problem below the waist, anything to do with his back, his kidneys, his neck, his skull… WTF would be paying insurance for?

Part of the problem is that while people MIGHT be sympathetic to the plight of children they totally forget that a lot of these children grow up to be adults. And frankly, the US doesn’t a flying [expletive deleted] about disabled adults for the most part. They can’t get private insurance, and employers are reluctant to hire them, which leaves them with claiming disability and going on Medicare, which more or less insists on grinding poverty to qualify for healthcare. (Nevermind that my husband, a man who now not only has spina bifida and diabetes but also crippling arthritis due to wear and tear on joints compensating for a malfunctioning body) has been denied disability (we’re appealing). WTF?

Sorry - yes, I’m a bit… angry about certain things. But while there may be loving parents out there doing their best for their less-than-perfect children society as a whole does NOT support their choice.

Just wanted to pop back in to the thread to say thanks everyone for the very informative replies.

I was aware (but had forgotten, admittedly) that there was a link of some sort between Downs and Spina Bifida.

Have Spina Bifida life expectancies also improved significantly within the last few decades? Or any other common genetic syndromes?

Yes.

When my husband was born most children with spina bifida involving open lesions on the spine died either shortly after birth, or before adulthood. (He, obviously, was an exception - but at birth he was given a life expectancy of six weeks and no medical care, basically being left to die. Only the intervention of a grandfather willing to pay for then-experimental surgery enabled him to survive. Said surgery is now standard and, as Irishgirl mentioned, routinely performed the first day of life in most first world countries. I should point out that my husband is over over 50 - an extraordinary age for someone of his generation with SB) Most children with spina bifida are now expected to live to adulthood, and with generally much better outcomes. Many more than in the past are walking, and of those with hydrocephalus only a small percentage now suffer the profound retardation that used to be the usual outcome.

At one point some friends of the family had a child diagnosed prenatally with SB. Of course, they were hearing all sorts of horrible things, all the worst possible outcomes. “Hi - I want you to meet my husband, he was born with SB…” Sure, there are problems, but wow, yes, you can grow up, go to school, get a job, get married… basically have a pretty normal life in many respects. It was also an incentive, when their child was born, to really push for getting the proper care immediately. Last I heard their kid - born with a similar level of SB as my husband - was walking by age 4 (my husband was unable to walk until 9 and some significant surgery) and doesn’t even have a limp. Things have improved, but it’s vital in these cases to intervene as early as possible. See, my husband had to wait a couple weeks for his initial surgery - this kid was whisked from the delivery room right into surgery, in part because the parents, having done their research, insisted on it.

The best treatment protocol in the world is useless if you don’t follow it, or can’t gain access to it. That’s currently a big obstacle with some of these syndromes.

Well, cystic fibrous patients, who used to commonly be dead by 5 or 6 years of age, are now living into their 30’s on a fairly regular basis. It’s still a truly wretched disease but significant progress has been made. A bunch of other genetic diseases and malformations now have treatments or improved treatments that significantly improve lives and in some cases allow the kids to grow up to be pretty normal in many respects. Unfortunately, not all such syndromes have treatments, some are still pretty lethal. It’s not a perfect world.

remember Corky on Life Goes On? He had Downs Syndrome and was played by Chris Burke. Chris has downs in real life.

Chris Burke is 45 years old and doing fine.
his web site
http://www.chrisburke.org/

UGH! Just UGH!!! It does seem like a lot of people are clueless about milder disabilties, and think that ALL disabilty= profoundly disabled, deaf blind with ten million different medical problems.
I wish people would understand that disabilty isn’t that big of a deal.
Yes, having an abortion for a fetus who has been dx as died or will die soon after birth is VERY different from having an abortion for a relatively mild disabilty.