Lily Parra Needs a New Heart

Here’s a familiar story. Four-month-old Lily Parra won’t be put on the transplant list for the heart she needs because of disability. Long-time readers may remember that I’ve written about two similar stories: Children’s Hospital of Philedelphia’s refusal (eventually reversed, after a long fight) to perform Amelia Rivera’s kidney transplant because of her intellectual disability, and the Hospital of the University of Pennsylvania’s refusal to put Paul Corby on the transplant list for a heart because of concerns stemming from him being autistic.

Content Note: Ableism, Child Illness, Organ Transplant, Medical Model of Disability

Dr's prescription pad with "Heart Transplant" written on it. Keyword: Lily Parra

Image Description: Dr’s prescription pad with “Heart Transplant” written on it

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Stephanie Parra was 32 weeks pregnant when doctors informed her about Lily Parra’s heart defect and how it meant that she probably wouldn’t live long past birth. They suggested that Stephanie have an abortion, but she refused.  Lily Parra was born on December 8th, 2015 and went home a month later, after surgery to have 2 stents put in her heart. Her parents took her back to the hospital a week later because of concerns that she was having a seizure. Doctors quickly saw that she was in respiratory distress, and  determined that she’d picked up a bacterial infection from being in the NICU that had settled in her lungs. After an echocardiogram also showed that Lily had mitral valve regurgitation in addition to her other heart problems, doctors determined that she needed to be put on the transplant list.

Read more about Lily Parra’s health problems

However, an MRI showed that Lily Parra had water on the brain, causing a condition called diffuse cerebral dysfunction that might result in developmental disability. Stephanie Parra says that she was told by doctors generally that “because of this diagnosis, her daughter would have a poor quality of life and therefore, they would not list her for a heart transplant” and by Dr. Shamel Abd-Allah specifically that “I don’t have a bag full of hearts that I can give to everybody and I’m not going to waste a heart on a kid who might have disabilities.

A letter from Loma Linda University Children’s Hospital posted on the online petition that Stephanie Parra has started states Lilly Parra isn’t being considered for a transplant because chromosomal abnormalities that will affect longevity. Stephanie says that she only heard about this when she heard doctors and nurses in the PICU discussing how Lily Parra was missing genetic material on chromosome 2p21. Individuals with this deletion are usually developmentally disabled.

I Get It

Donated organs are in short supply and tough decisions need to be made about how they’re allocated. Doctors should be reasonably assured that recipients are committed to caring for themselves and the organ that they’ve received – taking the anti-rejection medications, following up with doctors, and staying in good health to the greatest extent possible.

I get bringing up some disabilities as a consideration when evaluating people for transplant. If I needed a transplant, I would get it if doctors asked me questions like:

When you’re depressed –

  • Do you realize that you’re depressed?
  • How well do you take care of yourself?
  • Do you ever stop taking your medications?
  • Do you have a plan that you put in place to help you get back to baseline again?
  • How likely are you to put that plan in place by yourself? What kind of help do you need to get it going?
  • How long does it take you to get it going?

If I indicate that every couple of years I get so depressed that it’s hard for me to get out of bed and to work and my appointments, and that I stop taking all my medications, I imagine that this would be concern for a transplant team.

I think is fair it’s that disabilities with the potential to affect how well a transplant recipient  adheres to the regimen that will endure the success of the transplant be a concern in the evaluation for the recipient’s suitability for a transplant.

However…

Disability on its own shouldn’t dictate whether a person is excluded from transplant (especially very young children). Lily Parra may very well end up developmentally disabled. The reality is that we can’t know the extent of her disability at this point, and that, just as it would be fairly easy for me to put a plan in place with support from others that ensured I was taking my anti-rejection meds and seeing my doctors even when depressed, Lily Parra can have supports put in place around her as well, if needed, to ensure that she cares for herself as an organ recipient.

I worked with an intellectually disabled woman who never forgot to take her meds. She’d tell me, “I need to take my meds now.”

It’s not fair to decide when a baby is four months old that he or she will not be able to live with the demands of being an organ recipient, even if developmental disability is involved.

Lily Parra – Deeper Issues

Doctors want people who have the best chance of high “quality of life” to receive donated organs, but their conception of “quality of life” is often narrow and ableist. If doctors can’t imagine a recipient getting a typical education and ultimately contributing to society through paid work, family, community roles, etc., then they assume that donated organs would be better off with individuals who can do these things in the stereotypical ways that they envision.

This bias toward the stereotypical “normal life” (and emphasis on how potentially “functional” in society a person is as a criteria for being being an organ recipient), is highly ableist. It’s perfectly possible to have high quality of life while having significant disabilities, and almost certain that quality of life would be even higher without the physical and attitudinal barriers blocking disabled peoples’ full access to society. The view that disability is something that’s in the individual and needs to be cured is so prominent within the medical community that it’s actually called the medical model of disability, and its refusal to acknowledge that disability is a social creation (the social model of disability) doesn’t leave any room for the idea biology doesn’t predict destiny.

Again, especially when a potential organ recipient is very young, it’s terribly unfair to totally base a decision about whether or not someone gets organ on, “Disability always comes with low quality of life.” We should not be making decisions transplants based on whether we think a recipient could or couldn’t reach the quality of life that we’d consider acceptable because:

  1. What doctors consider a good quality of life may not be what the recipient considers a good quality of life.
  2. An individual generally should not be able to dictate what a good quality of life is for someone else.
  3. As much as I respect doctors and clinicians and their abilities, they are frequently wrong about what disabled people end up being able to do. My doctor at my first stroke rehab centre didn’t think that I’d ever really be able to move my left arm again. I have considerable movement in my left arm and am still getting return in my left hand after 15 years.

I Also Realize…

…that we don’t have the full story from Loma Linda University Children’s Hospital. Garrett Caldwell, Executive Director for Public Affairs at Loma Linda, wrote a letter in response to Special Olympics Chairman Timothy Shriver’s op-ed about Lily Parra in the Washington Post, but it was very general (as responses usually are). There are aspects of the story that don’t make sense – like why the Parras were not told about the 2p21 deletion, and why one doctor told them that Lily Parra was in kidney failure and another then said that she wasn’t. It’s totally understandable that parents under extreme stress might misunderstand information or might not remember something that was said to them. There may be other factors behind the decision to remove Lily Parra from the transplant list.

However, there’s enough consistency in Stephanie Parra’s story across media accounts to make me believe that it was said that Lily Parry would not receive the transplant because of a developmental disability or the potential for one, and this is not fair. It’s ableism that needs to be called out and that Loma Linda University Children’s Hospital needs to respond to.

I didn’t like most of Shriver’s op-ed, to be honest – I thought that he relied too much on emotion and not enough on fact to make his argument. But I do think he nailed it with this:

“This is an infuriatingly familiar story, and people with intellectual disabilities know it well. Too many medical professionals see them only in terms of “function” or “dysfunction.” The vast diversity of gifts and challenges that make up each of us are reduced to a binary conclusion. You’re either able or disabled, valuable or not. And when you’re not, you’re hopeless.”

Please sign Stephanie Parra’s petition to have the Transplant Board at Loma Linda University Children’s Hospital reconsider putting Lily Parra back on the transplant list.

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Intellectual Developmental Disorder and the DSM-V

Graphic of a man's head, clear; the brain is visible, each of its sections a different colour. Keyword: intellectual developmental disorder

Image Description: Graphic of a man’s head, clear; the brain is visible, each of its sections a different colour.

The deadline for public input on revised diagnostic criteria for autism in the DSM-V is June 15th. There’s been a lot of controversy on this, so be sure that you’re well-informed and that you voice your opinion about any of your concerns. See this blog post for a starting point on where to research what’s been going on with controversy

Also bear in mind that there’s been controversy over the revised diagnostic criteria for mental retardation, starting with renaming it Intellectual Developmental Disorder.

Yes, “Mental Retardation” is Diagnostic Criteria

And bravo to the American Psychiatric Association for finally moving away from it in the DSM-V.  Service providers in Canada and the US have been using the less emotionally-loaded “intellectual disability” for at least as long as I’ve been involved with developmental services, and the US government has recently made a commitment to use “intellectual disability” in its disability legislation. It’s going to be much easier to get people to stop using “retard” and “retarded” in reference to people with disabilities when there’s no clinical term with the word “retard” in it to give those slurs any false air of legitimacy. Like most things, however, this particular redefinition of diagnostic criteria isn’t that simple:

  • “Mental retardation” changes to “intellectual developmental disorder” (as I mentioned).
  • The criterion that the disability begin before age 18 disappears, replaced by one that the disability begin “during the developmental period”.
  • The IQ criterion, now 70 or under, becomes more flexible, shifting the focus more to  the individual’s level of adaptive functioning.

“Intellectual Developmental Disorder” Doesn’t Roll off the Tongue…

Advocates at organizations such as The Arc and the American Association on Intellectual and Developmental Disabilities fear the DSM-V’s use of “intellectual developmental disorder” and the departure from the more standard “intellectual disability” will confuse people. I do agree that this could potentially happen. However,  it’s not like the official diagnostic term hasn’t differed from the everyday language for years.  I have never in my career, unless I was speaking specifically in clinical terms regarding the official DSM diagnosis with a colleague who understood my context, spoken about “mental retardation” in my work. I use “intellectual disability” with the people I support, with their families, and, the overwhelming majority of the time, with colleagues.  I probably still will, unless a superior directed  me to use “intellectual developmental disorder”. I doubt I’ll be alone.

Now for the Famous “That Being Said…”

I’m fascinated by the “developmental” element in “intellectual developmental disorder”, particularly in light of the fact that there will be more flexibility around the IQ standard. A lot can happen to a child before age 18. They may not be born with a condition that traditionally causes an intellectual disability, but what if a traumatic brain injury caused by a car accident leaves a teen with the severe deficits in adaptive functioning that a teen with an intellectual disability might experience? Or impairs ability to perform on an IQ test to the point where it looks as if the teen’s IQ has suffered a drop into the zone associated with intellectual developmental disorder? The developmental element provides a way to assist clinicians to differentiate between what is truly an  intellectual developmental disorder and what disabilities require a different diagnosis and perhaps more appropriate treatment and supports.

Because Diagnosis Determines Supports

For a long time, Ontario’s definition of developmental disability (often used interchangeably with “intellectual disability” in the province) was IQ-bound (as is the DSM-IV’s diagnostic criteria for mental retardation).  I’ve seen how this can make decisions tricky about who gets and doesn’t get support within the developmental services sector, particularly for people with an IQ in the low 70s. People in this “borderline” zone got cut from services that they needed because of a couple of IQ points.

Watching how the IQ criterion sometimes prevented people from getting services that they need, creating more cracks through which people could fall, was very frustrating for me and for others in social services. Knowing that Ontario’s definition of developmental disability is now less IQ-bound and more skills-based is a relief. I was relieved the see the APA making a similar shift in its proposed diagnostic criteria for intellectual developmental disorder. Unfortunately, I haven’t been in a position to get any hard data about how that shift is working for people with developmental disabilities in Ontario, but the move does make intuitive sense to me.

The Bottom Line

I do have concerns:

  • I’d prefer that the APA stay away from the word “disorder”
  • I think that the developmental period in which a person needs to develop the signs of  an Intellectual Developmental Disorder in order to receive the diagnosis needs a description.
  •  I think that diagnosticians are going to want to know how much latitude they have with the IQ criterion, but I assume that they will receive information about that.
We’ll see how this goes.

 

 

March is Developmental Disabilities Awareness Month

The title says it all. March is Developmental Disabilities Awareness Month. What should that mean for us?

Keyword: Developmental Disabilities Awareness Month
Autism is a developmental disability.

Image Description: “Autism” in brightly coloured block letters against a black background.

What is a Developmental Disability?

The definition of developmental disability changes slightly from state to state (and province to province, in Canada), but the definition used by the Developmental Disabilities Act of the United States is as follows:

“The term ‘developmental disability’ means a severe, chronic disability of an individual 5 years of age or older that:

1. Is attributable to a mental or physical impairment or combination of mental and physical impairments;
2. Is manifested before the individual attains age 22;
3. Is likely to continue indefinitely;
5. Results in substantial functional limitations in three or more of the following areas of major life activity;
(i) Self-care;
(ii) Receptive and expressive language;
(iii) Learning;
(iv) Mobility;
(v) Self-direction;
(vi) Capacity for independent living; and
(vii) Economic self-sufficiency.

5. Reflects the individual’s need for a combination and sequence of special, interdisciplinary, or generic services, supports, or other assistance that is of lifelong or extended duration and is individually planned and coordinated, except that such term, when applied to infants and young children means individuals from birth to age 5, inclusive, who have substantial developmental delay or specific congenital or acquired conditions with a high probability of resulting in developmental disabilities if services are not provided.”

(From the Maryland Developmental Disabilities Council)

Here are some examples of developmental disabilities:

  • Cerebral palsy
  • Epilepsy
  • Autism
  • Hearing loss
  • Down syndrome
  • Spinal injury
  • Brain injury

Developmental Disability Fun Fact, in Honour of Developmental Disabilities Awareness Month

Having a developmental disability doesn’t necessarily mean that a person has an intellectual disability.

I’m surprised by how many people who work in my field don’t know this. Many of the people with conditions on the list above are very intelligent. There’s even a debate within some agencies that support people with autism whether they should be supporting individuals who have it without supporting documentation that these individuals have intellectual disabilities as well.

What Will Developmental Disabilities Awareness Month Mean for You?

I think that, for me, Developmental Disabilities Awareness Month will me doing pretty much what I try to do already, with a focus on developmental disabilities – raising awareness of what they are, celebrating the achievements of people who have them, and continuing to bust stereotypes where I can of people with developmental disabilities. Because it would be really nice if society got to the point where we didn’t need a month for people with developmental disabilities, because they’re just…people. Don’t you agree?

 

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