I just learned about Wolf-Hirschhorn Syndrome, a rare genetic condition that can cause children to have a characteristic facial appearance, delayed growth and development, intellectual disability, and seizures.
Unfortunately, I learned about it as I read the tragic story about a child with Wolf-Hirschhorn Syndrome who needs a kidney transplant, but whose parents were made to understand that she "shouldn't have the transplant done because she is mentally retarded."
As there are reportedly several adults with Wolf-Hirschhorn Syndrome in their 30's and 40's, should a three-year-old be denied a transplant at Children's Hospital of Philadelphia, especially if the transplant is coming from another family member?
The hospital does state that the "transplant programs at CHOP have never declined a patient for transplant based solely on their cognitive status and we have performed transplants on many children with disabilities and impairments," but that was hardly the impression this child's parent seem to get in their initial meeting with the transplant team.
In general, contraindications to getting a transplant might include having a life-threatening infection, cancer, unstable cardiovascular disease, and/or being noncompliant with the post-transplant medical regimen. Nelson Textbook of Pediatrics does state that "another relative contraindication includes severe neurologic dysfunction, but the wishes of the parents and the potential for rehabilitation must be considered."
Since the wishes of the parents were clearly for the transplant to happen, it is kind of surprising that it wasn't considered unless there is another contraindication. That the child should likely have a transplant may be even more clear when you consider the 2006 study in the American Journal of Kidney Diseases, "Kidney Transplantation in Pediatric Recipients With Mental Retardation: Clinical Results of a Multicenter Experience in Japan," which found good results when they studied 25 children with mental retardation because of chromosomal abnormalities, genetic syndromes, and developmental brain abnormalities.
Related:
Follow us on Twitter and Facebook
Wolf-Hirschhorn Syndrome
4p- Support Group
Hospital Denies Kidney Transplant to Girl With Intellectual Disability
Kidney Transplantation Committee
North American Pediatric Renal Trials and Collaborative Studies
Dr. Iannelli,
A well-written and informative article. From the tone of the article, though, I sense you are missing the fact that this has exploded into a general public outrage at what is being perceived as a denial of civil and human rights by a hospital through its official, generally applicable policies that, by the hospital’s own statements, take IQ into account as “a factor” in determining eligibility for an organ transplant. Hundreds of messages of shock and horror are being expressed by individuals who do not know the patient involved. Over 14,000 people have signed a petition. It’s the amount of public outrage that is the remarkable story here.
There is a larger message here, which is that hospitals and their medical staff are not “above the law”, and above basic standards of human decency; that medical professionals are likely to carry their own prejudices against those with developmental disabilities to inform their own seemingly objective medical decisions.
The case also raises disturbing issues of eugenics. CHOP is being likened by many to the Nazi regime. Members of your profession in the US, as late as the 1970s, were sterilizing human beings on eugenics-related justifications.
This is a Rosa Parks moment. It is fitting that it is occurring around the time we celebrate Dr Martin Luther King Jr’s birthday. Any medical professional who refuses at least to condemn the actions of the physician involved (if they are true), and any medical professional who refuses to condemn discrimination against those with developmental disabilities, is on the wrong side. The people have spoken.
I do not understand what the original post meant by “Paper number one has the words, “Mentally Retarded” in cotton candy pink right under Hepatitis C. Paper number two has the phrase, “Brain Damage” in the same pink right under HIV. ” I am not familiar with the paper/form but does the child have hep C and HIV as well? Also are her seizure drugs nephrotoxic? What other factors are involved here? Don’t young transplants usually need to be repeated? Any comments are appreciated.
“I am not familiar with the paper/form but does the child have hep C and HIV as well? Also are her seizure drugs nephrotoxic? What other factors are involved here? Don’t young transplants usually need to be repeated?”
I think the papers with hepatitis C and HIV are the general forms that they use to evaluate a child for a renal transplant. Her point may have been that the words ‘mentally retarded’ were in the same part of the form as hepatitis C and HIV. I don’t think that she has either of those.
The need to get another transplant or being on medications to control seizures don’t seem to be contraindications for a transplant either.
Thank you very much for writing this article. It has been so good to read here and elsewhere all the support for this little girl.It is heartwarming to see how many people have spoken up to point out that developmental delays or “Mental Retardation” do not mean that it is appropriate to deny medical care.I read elsewhere that CHOP may be reviewing the situation and potentially allowing the transplant. If so great news.
My guess is that the papers she is referring to are a list of contraindications for the transplant and that they highlighted hers. Not that she has hepatitis or HIV.
Clearly mom is experienced in utilizing media to go forth in battle for what she feels her child deserves.
http://www.bayada.com/Press%20Releases/SenatorBeachPressRelease.pdf
It truly seems that something is missing from this story told only from the perspective of a highly involved, albeit very emotional, mother.
“It truly seems that something is missing from this story told only from the perspective of a highly involved, albeit very emotional, mother.”
It is possible something is missing from the story, as CHOP and their doctors can’t respond directly because of privacy rules, but since the Washington Post cited a report that 43 percent of transplant programs do consider a child’s neurodevelopmental delays when making a decision to approve a transplant, maybe there isn’t.
And the fact that parents like this have to go to such lengths to get what their special needs children need is part of the reason this story has attracted so much attention. I’m sure they would rather not have to go to the media to get appropriate treatment and insurance coverage for their kids.