New revelations that have
appeared in the New York Times and the
Atlantic Monthly, about John F. Kennedy's
health have raised questions about his physical
condition during his presidency. Robert Dallek,
in the December Atlantic Monthly,
described in "The Medical Ordeals of JFK" long
standing medical problems that started in
childhood. In Kennedy's adolescence,
gastrointestinal symptoms, weight and growth
problems as well as fatigue were described.
Later in life, he suffered from abdominal pain,
diarrhea, weight loss, osteoporosis, migraine
and Addison's disease. Chronic back problems,
due to osteoporosis resulted in several
operations and required medications for chronic
pain. He was extensively evaluated in major
medical centers including the Mayo Clinic and
hospitals in Boston, New Haven and New York.
Among the multiple diagnoses were ulcers,
colitis, spastic colitis, irritable bowel
syndrome, and food allergies. His medications
included corticosteroids, antispasmotics,
Metamucil and Lomotil. However it is not clear
that his physicians obtained a definitive
diagnosis.
Review of this medical history raises the
possibility that JFK had celiac disease. Celiac
disease is caused by ingestion of gluten, which
is the main protein component of wheat and
related cereals, rye and barley. The small
intestine develops villous atrophy that results
in difficulties in the absorption of nutrients.
Diarrhea and abdominal pain are common symptoms.
Elimination of gluten from the diet results in
resolution of the inflammatory condition in the
intestine and the associated symptoms and
prevention of the complications of the disease.
A life-long gluten free diet is then required.
People with celiac disease, providing they
adhere to the diet have normal longevity.
Celiac disease can present at any age. In
infancy and childhood it may cause chronic
diarrhea, abdominal pain, and growth, behavioral
and development problems. In older individuals
the presentation of celiac disease is frequently
due to the development of complications of the
disease. These include anemia, osteoporosis,
skin rashes or neurologic problems. The
neurologic problems include neuropathy,
epilepsy, ataxia (balance disorders) and
migraine. While the disease is more common in
females, men are affected as well. Osteoporosis
is common in patients with celiac disease, men
often are more severely affected than women.
Gastrointestinal symptoms in celiac disease
persist for many years prior to diagnosis and
are often attributed to an irritable bowel
syndrome or spastic colitis. Patients typically
see many physicians prior to the diagnosis of
celiac disease.
Autoimmune disorders occur more frequently in
patients with celiac disease than the general
population by a factor of ten. Frequently the
autoimmune disorder assumes greater clinical
significance than the celiac disease and as a
result is diagnosed first. The associated
autoimmune disorders include thyroid
dysfunction, psoriasis, dermatitis herpetiformis
(an intensely itchy skin rash), Sjogren's
syndrome, and Addison's disease. Relatives of
patients with celiac disease have a greater
risk, not only of celiac disease, but also of
other autoimmune diseases.
THE IRISH CONNECTION
Celiac disease was formerly considered a rare
disease of childhood. It is now recognized as
being very common in those of European descent,
one of the most common genetically determined
conditions physicians will encounter. Recent
studies have demonstrated the country with the
greatest prevalence to be Ireland. In Belfast
one in one hundred and twenty two have the
illness.
The prominent familial association of the
disease indicated by the occurrence in one of
ten first degree relatives and in 80 percent of
identical twins points to a genetic component of
the disease. However the actual genes
responsible for the disease have not been
discovered though there are many groups working
on the problem. It is known that there is a
strong association with specific HLA genes that
are required for the disease to occur, but are
themselves not sufficient for the disease to be
manifested.
Kennedy's Irish heritage, long duration of
gastrointestinal complaints (since childhood),
diagnosis of irritable bowel syndrome and
migraine, presence of severe osteoporosis, and
the development of Addison's disease all lead to
a presumptive diagnosis of celiac disease.
Kennedy was given steroids for his problems.
Steroid use is associated with the development
of osteoporosis and Addison's disease. However
steroids were initially used in clinical
practice in the 1930s and 1940s for many
indications, not considered appropriate now. In
the case of Kennedy, if he did in fact have
celiac disease, the steroids would have
suppressed the inflammation in the intestine and
reduced his symptoms, making diagnosis of celiac
disease less likely to be established. The
occurrence of Addison's disease in his sister,
however, argues for a familial cause of his
Addison's disease, rather than an iatrogenic
one.
Could celiac disease have been diagnosed in
Kennedy during his lifetime? Possibly. The
disease was first recognized in 1887 as well as
its treatment with an elimination diet. It was
recognized to occur at all ages. However, it was
not until the 1950s that the shortage of bread
during the Second World War and its subsequent
reintroduction in Holland prompted recognition
of the role of wheat as a cause of this
malabsorption syndrome. While it was in the
1970s that physicians became aware of the more
subtle presentations of the disease. The
diagnosis of celiac disease initially requires
consideration that it may be present in an
individual patient, even now many physicians do
not consider the diagnosis.
It would however be possible to diagnose celiac
disease in JFK now, if biopsies taken during his
life, or autopsy material of the small intestine
had been archived and was now made available.
Frozen blood samples could also provide
diagnostic material for there are serologic
tests now available that are sensitive and
specific for the condition..
A diagnosis of celiac disease, if it had been
made could have been treated by diet alone. This
would have prevented all the manifestations of
the disease and its complications. Because of
the strong genetic component of celiac disease,
Kennedy's family may well be interested in
obtaining the diagnosis as well.
By Peter H R Green Dr. Green is
Professor of Clinical Medicine, Director of the
Celiac Disease Center at Columbia University
College of Physicians
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