guidebook. - Fanconi Anemia Research Fund

74
Fanconi Anemia: Guidelines for Diagnosis and Management
such practices may lead to increased risks of fungal
infections and antibiotic resistance.
Sedation and analgesia for invasive procedures
Given the need for frequent evaluation of the bone marrow,
adequate sedation and analgesia should be offered
to every patient undergoing bone marrow aspiration and
biopsy. The use of local anesthetic alone may not be
sufficient to alleviate the anxiety and pain that is associated
with frequent, repeated bone marrow aspirations.
The use of intravenous propofol, fentanyl and midazolam,
or similar regimens used in accordance with
the guidelines established by the American Academy
of Pediatrics, is strongly recommended. Such regimens
may make it easier for families and patients to accept a
yearly bone marrow examination as a routine part of the
care for FA.
Acknowledgements
The author gratefully acknowledges the following
colleagues: Melody Cunningham, MD and Matthew
Heeney, MD, both of Children’s Hospital, Boston for
critical reading of the iron overload section and Susan
Rose, MD, Cincinnati Children’s Hospital Medical
Center, Cincinnati, for commentary on the endocrine
sections.
References
1. Butturini A, Gale RP, Verlander PC, Adler-Brecher B, Gillio
A, Auerbach AD. Hematologic abnormalities in Fanconi anemia:
an International Fanconi Anemia Registry study. Blood 1994; 84:
1650-1655.
2. Rosenberg PS, Greene MH, Alter BP. Cancer incidence in persons
with Fanconi anemia. Blood 2003; 101: 822-826.