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[embed][/embed]ALS 2015 Algorithm lecture

Boston Children’s Hospital

Dr. Heidelise Als is a researcher and clinician who has focused her life research on the behavioral organization of the newborn infant, especially the preterm and high risk infant. From neurobehavioral and neurophysiological studies performed by our group and others, it is clear that the preterm infant at school age emerges as significantly more at risk for attention deficit disorder, lower IQ, difficulties in social-emotional functioning and self-regulation, and increased need for specialized school services. Dr. Als is the author of the APIB and the originator of the Newborn Individualized Developmental Care and Assessment Program, an individualized, behaviorally-based developmental care model which is changing Newborn Intensive Care Units around the world. 

NIDCAP and APIB were developed for the education and training of staff and the support of parents in reading the infant’s behavioral cues in order to implement all care collaboratively with the infant. Study results are consistent and demonstrate improved lung function, feeding behavior and growth; reduced length of hospitalization, improved neurodevelopmental function, along with improved brain structure and function for the infants who received individualized developmental care, as compared to the control groups who received the respective NICU’s current best practice. The aims of Dr. Als and her colleagues’ research is firstly to assess the immature nervous system’s readiness for sensory input and to document the infant’s current active behavioral efforts towards developmental differentiation and thresholds to disorganization. Secondly, they aim to modify the environment and caregiving in keeping with an individual infant’s current thresholds to disorganization. 

Dr. Als has received over 15 grant awards from the U.S. Department of Education and the National Institute of Health for her research and authored over 60 articles and chapters. Her postgraduate training took place at the Child Development Unit at Children’s Hospital Boston. Dr. 

Als is a developmental, educational, and licensed clinical psychologist with over 30 years of experience in undertaking and managing major research efforts involving high risk and preterm infants and families in medical settings, and in longitudinal follow-up. She furthermore holds a research associate appointment in Newborn Medicine at the Brigham and Women’s Hospital, Boston, and an adjunct pediatric psychology appointment at the Spaulding Rehabilitation Hospital in Boston. In 1987 she received the Award of Excellence of the Boston Institute for the Development of Infants and Parents, in 1988 the Senator D’Amico Award of the Massachusetts Early Intervention Consortium, and in 1995 CINN-Elekta Decade of the Brain Award. 

Keywords: [“infant”,”Care”,”developmental”]

Immunoglobulin Therapy & Other Medical Therapies for Antibody Deficiencies

There are several specific medical therapies available for patients with primary immunodeficiency diseases involving the humoral immune system. Effective therapies for these disorders are a reality for most patients, and optimize their health, improve their quality of life and allow them to become productive members of society. These immunoglobulins in the serum or plasma are IgG, IgM, IgA, IgD and IgE. Individuals who are unable to produce adequate amounts of Ig or antibodies, such as patients with XLA, CVID, Hyper-IgM Syndromes, Wiskott Aldrich Syndrome or other forms of antibody deficiency may benefit from replacement therapy with Ig. Only the IgG is purified from the plasma to produce commercial Ig products, so Ig used for treatment contains very little of any of the other Ig types. 

To commercially prepare the Ig for patients with primary immunodeficiency diseases, the immunoglobulin must first be purified from the plasma. It is important to understand that the Ig that is given partly replaces what the body should be making, but it does not stimulate the patient’s own immune system to make more Ig. Since Ig only replaces the missing end product, but does not correct the patient’s defect in antibody production, Ig replacement is usually necessary for the patient’s lifetime. SCIG infusions may be given as often as daily, weekly, or as infrequently as every three to four weeks, depending on the specific SCIG product that is being prescribed, the patient’s age and the preferences of the patient and the prescriber. The choice of route of administration of Ig therapy should be a decision based on discussions between the patient and provider. 

Some patients with chronic sinusitis and chronic lung diseases, such as bronchitis, do better when given higher doses of Ig. Some patients, who lose IgG molecules from their digestive tracts or kidneys, may require more frequent doses and/or higher doses. The IgA on the mucosal surfaces of the respiratory tract is not being replaced, which may be part of the reason that antibody deficient patients remain somewhat more susceptible to respiratory infections, even though they are receiving enough immunoglobulin to maintain normal or near-normal blood levels of IgG. Prophylactic Antibiotic Therapy. Patient compliance with therapy is paramount to achieving this goal. 

This will be done by taking the patient off of therapy and reevaluating humoral immunity. Excerpted from the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases FIFTH EDITION Copyright 2013 by Immune Deficiency Foundation, USA. This page contains general medical information which cannot be applied safely to any individual case. 

Keywords: [“patient”,”antibody”,”infusion”]