Fatigue and Impaired Quality of Life Are Significant Consequences of Hemolysis in PNH Regardless of Transfusion Requirements1
Abdominal pain, fatigue, and other quality-of-life issues are commonly reported in PNH.
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Severity of fatigue and impact on QoL can be independent of anemia/transfusion requirements1,2
Fatigue and impaired quality of life may be underestimated in PNH.
- Physician probing focuses more on clinically evident signs vs. fatigue and QoL
- Some symptoms such as dystonias are underappreciated
- Optimal management is individualized and includes careful assessment of:
- Degree of fatigue
- Full spectrum of possible symptoms (i.e., dystonias)
- Impact on daily living/functioning
Paroxysms: Clinical evidence for contributions of hemolysis to fatigue.
- Many patients note a feeling of fatigue that may be disabling during periods of hemoglobinuria2
- This is not related to hemoglobin level (anemia), as it continues when the hemoglobinuria stops
- PNH patients frequently complain of disabling fatigue that is often out of proportion to the degree of anemia3
Burden of fatigue and QoL is extensive in a study of 29 PNH patients:1
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96% of patients report fatigue1
- 59% of patients transfusion-free for at least 6 months1
- 76% of patients forced to modify their daily activities due to their PNH1
- 17% of patients unemployed due to PNH1
- 57% of patients report abdominal pain1
- >50% reported abdominal pain, headache, and dyspnea
- Patients commonly reported dysphagia (41%) and erectile dysfunction (47% in males)
- Most symptoms rated as moderate to very severe, and a substantial majority reported distress associated with the symptoms
- Fatigue, QoL, and abdominal pain are linked by underlying hemolysis and the threat of ischemia1
The current availability of an effective PNH treatment option warrants testing appropriate patients.
References: 1. Meyers G, Weitz I, Lamy T, et al. Disease-related symptoms reported across a broad population of patients with paroxysmal nocturnal hemoglobinuria [ASH abstract]. Blood. 2007;110: Abstract 3683.
2. Rosse WF, Hillmen P, Schreiber AD. Immune-mediated hemolytic anemia. Hematology (Am Soc Hematol Educ Program). 2004:48-62.
3. Brodsky RA. Paroxysmal nocturnal hemoglobinuria. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2005:419-427.
4. Hill A, Richards SJ, Hillmen P. Recent developments in the understanding and management of paroxysmal nocturnal haemoglobinuria. Br J Haematol. 2007;137:181-192.