PEDIATRICIAN Spring 2003 - AAP-CA

Ten-Year Experience
with Pediatric Bilateral
Living Donor Lobar Lung
Transplantation
Marlyn S. Woo, M.D. and Vaughn A. Starnes, M.D.
running out of options
for this kid,” said the voice
“We’re
on the phone. My heart
sank at that statement from an experienced
physician. He had called to discuss his patient
referral that had arrived at our office only a few
days earlier. He had been unable to keep the
ten-year old boy out of the hospital for more
than a few weeks at a time, even though the
child had been on almost continuous intravenous
antibiotic therapy for several months. The
young boy was oxygen-dependent and could
not walk more than a few steps without dyspnea.
He could only speak two to three words
without pausing for breath. The medical team
had exhausted all conventional medical and
surgical options. What about lung transplantation
for this dying patient?
In the past, children with end-stage lung
disease were not likely to survive to receive
cadaveric lung transplantation unless they
were referred as soon as they entered the terminal
stage of their disease. Unlike heart or
liver transplant candidates, cadaveric lungs
available for transplant are allocated based
upon the blood type, size needed, and how
long the candidate has been waiting on the
list. Although lung transplant candidates with
idiopathic pulmonary fibrosis get three months
of time added at their initial listing, cystic
fibrosis and primary pulmonary hypertension
patients with type O or A blood types must
wait over a year (at times, five years!) before
a suitable cadaveric organ becomes available.
This situation puts these fragile children at a
disadvantage compared to the adult lung transplant
candidates (the primary adult diagnosis is
chronic obstructive pulmonary disease), who
comprise the majority of the cadaveric lung
transplant candidates. Thus, it is not surprising
that children are far more likely to die than
adults while awaiting lung transplantation.
The majority of pediatric lung transplant
candidates have cystic fibrosis as their primary
diagnosis. Hence, these patients with purulent
lung disease require double lung transplantation,
which also contributes to their increased
delay in obtaining suitable cadaveric organs. It
was in this milieu that the first human bilateral
living donor lobar lung transplantation was
performed. The patient was a 21-year old girl
with end-stage cystic fibrosis lung disease,
who had been listed for cadaveric lungs for
several months. She had been hospitalized for
several weeks and was not expected to survive
for more than a few weeks. Her parents asked
the transplant surgeon (Starnes) if they could
donate portions of their lungs to save her.
Dr. Starnes had already performed successful
human single lobar lung transplantation. After
clearance with the hospital Ethics Committee
as well as the Institutional Review Board, the
successful surgery took place January 1993 at
USC University Hospital. A few months later
(May 1993), the first successful human pediatric
bilateral lobar lung transplantation took
place at Childrens Hospital Los Angeles. The
pediatric patient was a 13-year-old male who
also had end-stage cystic fibrosis lung disease.
Since those first cases, over 150 living
donor bilateral lobar lung transplants have
been performed throughout the world. While
the majority have occurred in adult and pediatric
cystic fibrosis patients, this procedure has
also been successfully utilized for patients with
primary pulmonary hypertension, non-transplant
bronchiolitis obliterans, primary ciliary
dyskinesis, and severe bronchiectasis. As of
January 2003, Childrens Hospital Los Angeles
has performed 45 bilateral living donor lobar
lung transplants in children. Although living
donor lobar lung transplant recipients are generally
more ill than our cadaveric candidates,
there has been no significant differences in
length of intubation, post-operative ICU stay,
total length of hospitalization, or in perioperative
mortality between these two groups.
Although both cadaveric and living donor
recipients receive the same triple immunosuppression
therapy, pediatric living donor lobar
lung transplant recipients have better one year
survival and a significantly lower incidence of
chronic rejection/bronchiolitis obliterans syndrome
compared to cadaveric lung transplant
recipients at our institution. So should living
donor lobar lung transplantation be preferentially
performed in all pediatric lung transplant
candidates? There are several obstacles to performing
living donor lobar lung transplantation
in all pediatric candidates.
Living donor lobar lung transplant candidates
must meet the same criteria as those
being considered for cadaveric organs (Table
1). They must be healthy enough to survive
major surgery. Importantly, they must also
have at least two healthy donors acceptable
to the Transplant Team (Table 2). Our Center,
as well as the other United States Transplant
Centers who have performed more than living
donor lobar procedures, do not accept living
donor lobar candidates who have no emotional
attachment to the proposed recipient or family;
solicited or “stranger” volunteers; nor donor
candidates who have been coerced (selling
organs). Interestingly, over half of the living
lobar lung transplants have utilized lobes
donated by healthy adults who are not related
to the recipients.
CONTINUED ON PAGE 20
CALIFORNIA PEDIATRICIAN — SPRING 2003/ 13