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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



Disclosures Dr E.J. van Beers Company name Research

support Employee

Consultant Stockholder Speakers bureau Advisory board Other

Novartis x



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



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



Global causes of anemia YLD

Kassebaum et al. Blood 2013



• How severe is mild?

Anemia What kind of disability is it?



Icons: Creative Stall Aha-soft from noun project

: anemia = dyspnea

: dyspnea = anemia

: ?



Blood function

Blood distributes oxygen to the organs

Grotcott et al. NEJM 2009

CaO2 in ml/L, Hb in g/dl, PaO2 in mmHg



• How severe is mild? How

Severe is

Mild?



Musallam et al. Acta Haematol 2013

Hb 9 g/dL = Hb 5,6 mmol/L



Effect of high altitude

Grotcott et al. NEJM 2009



Grotcott et al. NEJM 2009|*estimation @Hb 9 g/dL

Oxy

gen

(m

l/L)

*



http://ucrtoday.ucr.edu/3827/oh-oxygen



• How severe is mild? What about

dyspnea and

anemia?



Purdue University photo/Steven Scherer

Let’s do a simple

experiment



Experiment

• Group A

• All with anemia

• Group B

• All others

• Hold your breath

for 90 seconds

• Record you Borg dyspnea scale

• Relax



Experiment



• 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



Conclusion

• Acute poor ventilation causes dyspnea

• Chronic anemia does not cause dyspnea



• How severe is mild? What about

excercise and

anemia?



Maximum cycling in SCD

E.J. van Beers et al. Am J Hematol 2014



Anemia is related to exercise tolerance





Exercise tolerance is related to lung function tolerance





Reasons to quit cycling

Icons: Tahsin Tahil from noun project E.J. van Beers et al. Am J Hematol 2014


= dyspnea



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



• How severe is mild? What about the causes of

dyspnea in

anemia?



Factors associated with dyspnea in SCD

Delclaux et al. Chest 2005/Fletcher et al. Proc R Soc Med 1952



Factors associated with dyspnea in SCD

Delclaux et al. Chest 2005



Schrezenmeier et al. Haematologica 2014

Dyspnea in PNH



Dyspnea in PNH

Hill et al. BR J Haematol 2009



Parent et al. NEJM 2010



SCD

TM/TI

Parent et al. NEJM 2010 Derchi et al. Circulation 2014



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



• How severe is mild? Thank you

Q?


Organ damage and treatment in TI

Taher et al. Blood 2010


Cause of heart failure in TI

Aessopos et al. Blood 2001


• 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



Weaver et al. NEJM 2009

5% 3%

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