Treatments helping to control HAE boost life quality: Study in India
Availability of first-line therapies to prevent or treat attacks seen as crucial
Keeping symptoms of hereditary angioedema (HAE) under control is the most important factor contributing to a better quality of life, according to a study carried out by scientists reporting from India and noting severe limits on access to first-line medications there.
“There is an urgent need for easy availability and affordability of first-line medications in resource-limited settings like ours to have better disease control and [quality of life],” the researchers wrote.
The study, “Quality of life in patients with hereditary angioedema correlates with angioedema control: Our experience at Chandigarh, India,” was published in Asia Pacific Allergy.
Treatment access and affordability can limit options available to patients
HAE causes sudden episodes of swelling that are unpredictable and can occur anywhere in the body. These episodes can lead to pain, fatigue, nausea, and vomiting, making day-to-day living difficult for both patients and their caregivers.
Various HAE treatments work to help prevent swelling, while others treat disease symptoms as they occur. In either case, “availability and access to the first-line treatment options in patients with HAE have resulted in better [quality of life],” the researchers wrote.
However, most developing countries — including India — often do not have sufficient first-line treatment options or easy access to them, they added.
Researchers largely at a medical institute in Chandigarh evaluated the quality of life, and what contributes to it, for adults living with HAE in India. They noted no such studies had been done before.
A total of 135 adults (71 men, 64 women; mean age of 40.9) with a diagnosis of HAE took part. Most patients (82.9%) had HAE type 1, and the others HAE type 2. Both types are caused by mutations in the SERPING1 gene, which codes for a protein called C1-inhibitor. Symptoms were reported to begin at a mean age of 15.7 years, with a diagnosis arriving much later, at a mean age of 34.9.
“We observed that the majority of patients in the present study had [a] positive family history” of the disease, and despite that history “there were significant delays in diagnosis,” the researchers wrote.
Swelling occurred most commonly in the face (93.7%), extremities (80%), and abdomen (62.1%). Over half of the patients (53.3%) had experienced laryngeal swelling, or swelling in the throat or voice box. In addition to most (91.9%) having a family history of HAE, some (19.2%) had lost at least one family member to the disease.
As prophylaxis (preventive treatment), 30.4% used both attenuated androgens (e.g., danazol) and tranexamic acid, 16.3% used only attenuated androgens, and 11.1% used only tranexamic acid. For on-demand treatment, fresh frozen plasma was used, again because of patients’ limited access to first-line medications at the time of data collection.
Reported quality of life reflected perceived levels of symptom severity
Quality of life was measured on a scale of zero-100 using the Angioedema Quality of Life (AEQoL) questionnaire and based on a recall period of four weeks, with higher scores indicating a worse quality of life. Patients scored a mean of 43.3 points. Over half reported moderate (23.7%) or severe (38.5%) impairments to their quality of life.
Overall, better symptom control was linked to a better life quality. Patients with poorly controlled symptoms reported a poorer quality of life compared with those with well-controlled symptoms, as demonstrated by their significantly lower scores on the AEQoL questionnaire.
Other key factors affecting life quality included a patient’s sex, the frequency of swelling episodes, and length of follow-up. Men reported a poorer quality of life than did women, as did people who had a higher frequency of swelling attacks. Conversely, patients who had been followed for more than two years reported a better quality of life compared with those with less follow-up.
Patients with a family history of HAE, deaths in the family due to the disease, or genital swelling also reported a poorer quality of life. Patients with HAE type 1 tended to report the worse life quality, to experience more difficulty in daily activities, have more difficulty managing their mood, and feel more fear and shame than those with HAE type 2.
These findings may help guide future treatment approaches and healthcare policies for better management of HAE, particularly in regions of the world where first-line treatment options may be unavailable or difficult to access, the scientists wrote.
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