6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
6th EUROPEAN SYMPOSIUM ON RARE ANAEMIAS 1st Dutch-Belgian meeting for patients and health professionals
21st - 22nd November 2015
Amsterdam - The Netherlands
Shortness of Breath Benauwdheid
Dr. E.J. van Beers
Van Creveldkliniek, UMC Utrecht, the Netherlands
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Disclosures Dr E.J. van Beers Company name Research
support Employee
Consultant Stockholder Speakers bureau Advisory board Other
Novartis x
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
This talk is applicable for:
Definite Probable
Thalassemia’s x
Sickle cell disease x
Membrane disorders (e.g. sferocytosis)
x
Enzym defects (e.g. PKD, G6PD) x
PNH x
Other forms of hemolytic disease x
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
This talk is applicable for:
Definite Probable
Thalassemia’s x
Sickle cell disease x
Membrane disorders (e.g. sferocytosis)
x
Enzym defects (e.g. PKD, G6PD) x
PNH x
Other forms of hemolytic disease x
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Years Lived with Disability (YLD)
Lancet Dec 13, 2012 Icons: C Shannon, J Cabesaz, A Coscovelnita, M Vanco.
6,916,000,000
772,000,000
2010
21,000,000 29,000,000
68,000,000
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Global causes of anemia YLD
Kassebaum et al. Blood 2013
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• How severe is mild?
Anemia What kind of disability is it?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Icons: Creative Stall Aha-soft from noun project
: anemia = dyspnea
: dyspnea = anemia
: ?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Blood function
Blood distributes oxygen to the organs
Grotcott et al. NEJM 2009
CaO2 in ml/L, Hb in g/dl, PaO2 in mmHg
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• How severe is mild? How
Severe is
Mild?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Musallam et al. Acta Haematol 2013
Hb 9 g/dL = Hb 5,6 mmol/L
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Effect of high altitude
Grotcott et al. NEJM 2009
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Grotcott et al. NEJM 2009|*estimation @Hb 9 g/dL
Oxy
gen
(m
l/L)
*
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
http://ucrtoday.ucr.edu/3827/oh-oxygen
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• How severe is mild? What about
dyspnea and
anemia?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Purdue University photo/Steven Scherer
Let’s do a simple
experiment
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Experiment
• Group A
• All with anemia
• Group B
• All others
• Hold your breath
for 90 seconds
• Record you Borg dyspnea scale
• Relax
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Experiment
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• Oxygen consumption
+/- 200ml/min
• Start:
+/- 1000ml Oxygen
• After 1.5min:
+/- 700ml Oxygen
Kwan et al. Int J Food Sci Nutr 2004
Experiment
• Hb 9 mg/dl/5.6 mmol/L: +/- 625ml Oxygen
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Conclusion
• Acute poor ventilation causes dyspnea
• Chronic anemia does not cause dyspnea
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• How severe is mild? What about
excercise and
anemia?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Maximum cycling in SCD
E.J. van Beers et al. Am J Hematol 2014
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Anemia is related to exercise tolerance
E.J. van Beers et al. Am J Hematol 2014
Maximum cycling in SCD
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Exercise tolerance is related to lung function tolerance
E.J. van Beers et al. Am J Hematol 2014
Maximum cycling in SCD
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Reasons to quit cycling
Icons: Tahsin Tahil from noun project E.J. van Beers et al. Am J Hematol 2014
Maximum cycling in SCD
= dyspnea
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
conclusions
• Chronic anemia limits exercise capacity mostly not limited by dyspnea
• Exercise capacity is important for daily life
• Degree of exercise capacity is related to degree of anemia
• Don’t use dyspnea as the only measure to estimate transfusion treatment effectivity
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• How severe is mild? What about the causes of
dyspnea in
anemia?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Factors associated with dyspnea in SCD
Delclaux et al. Chest 2005/Fletcher et al. Proc R Soc Med 1952
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Factors associated with dyspnea in SCD
Delclaux et al. Chest 2005
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Schrezenmeier et al. Haematologica 2014
Dyspnea in PNH
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Dyspnea in PNH
Hill et al. BR J Haematol 2009
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Parent et al. NEJM 2010
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
SCD
TM/TI
Parent et al. NEJM 2010 Derchi et al. Circulation 2014
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
Chronic dyspnea in chronic anemia
• Look voor factors other than anemia
• A(late) presenting symptom of PH
• Associated with lung function abnormalities
• Chronic anemia causes very diverse complaints
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht
• How severe is mild? Thank you
Q?
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Organ damage and treatment in TI
Taher et al. Blood 2010
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Cause of heart failure in TI
Aessopos et al. Blood 2001
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
• thromboembolic events in 1.65% of 8,860
thalassaemia patients (TI - 24.7% or TM - 75.3%)
from the Mediterranean and Iran.
Thromboembolism occurred 4.38 times more
frequently in TI than TM (p < 0.001),
• more common in
– females,
– splenectomized patients
– profound anaemia (<9 g/dl).
Thrombosis in thalassemia
Taher et al. Thromb Haemost 2006
6th European Symposium on Rare Anaemias - 1st Dutch-Belgian meeting for patients and health professionals
Van Creveldkliniek UMC Utrecht