These were from a talk by Dr. S..... she gave handouts later. She said the studies are limited.
Evaluation of low blood counts
r/o other treatable causes of low blood counts
anemia-- blood loss, antibodies, erythropoietin levels
neutropenia - infections or medication
G-CSF increases neutrophils and not all patients respond. Indications for using G-CSF:
neutropenia with persistent or serious bacterial infections or fungal infections
neutropenia w/ history of recurrent bacterial/fungal infections, gingivitis or mouth sores
some centers recommend G-CSF prophylactically for persistently low neutrophil counts (<200-500)
need to weigh potential risks
G-CSF potential side effects:
bone pain
enlarged spleen (associated with chronic use)
?osteopenia (seeing it in SCN patients-not sure if it is the underlying disease or from G-CSF)
Anectdotally- kidney problems
Bone marrow exam with cytogenetics should be done prior to initiating therapy with G-CSF. No causal relationship between cytokine therapy and leukemia has been demonstrated to date, but can;t rule it out, either.
Leukemia patients have used G-CSF to get through chemotherapy and have not seen an adverse effect. Not sure if you can extrapolate this to SDS population.
Supportive care for Anemia
Transfusion
Indications for transfusion: symptomatic anemia, fatigue, exercise intolerance, rapid heart rate and breath rate, poor growth
Rough guide: transfuse when hemoglobin is <8 -- this varies from patient to patient.
Risks of transfusion
allosensitizatization: patient develops antibodies against transfused red cells or platelets such that transfused cells are rapidly destroyed (makes transplant harder)
iron overload secondary to red cell transfusion (also makes transplant more difficult) (can use chelating agents)
transfusion reaction
infection (blood borne)
high body iron stores puts you at risk for transplant complications.
supportive care for thrombocytopenia
Indications for transfusion: symptomatic, bleeding, bruising and prophylaxis prior to surgery
20,000 is usually uses, but studies show 5,000-10,000 can be okay. Must consider the patient....for instance, a toddler who is always bonking his head.....transfusion would possibly be considered at a different number.
must also check Vit K levels....low vit K levels with low platelets can cause worse problems. Also, liver function can be a cause of bleeding problems.
Risks of platelet transfusions-- infections
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
