Multiple obstacles make hereditary angioedema diagnosis, treatment difficult
Click Here to Manage Email Alerts
Key takeaways:
- Respondents saw an average of nine patients with hereditary angioedema (HAE) each year.
- 82% of respondents said HAE symptoms are often misdiagnosed.
- 86% said they occasionally use telemedicine for appointments.
Clinical and economic factors make hereditary angioedema difficult to diagnose and treat, particularly among patients who live in rural areas, according to a study published in Annals of Allergy, Asthma & Immunology.
However, providers can take action to improve care at the individual and systemic level, J. Allen Meadows, MD, past president of the American College of Allergy, Asthma and Immunology, and colleagues wrote.
Diagnosing, treating disease
The researchers surveyed 138 members of the ACAAI, with a mean of 15 years in practice, including 80 physicians, 48 physician fellows and 10 allied health professionals such as nurse practitioners and physician assistants.
“Previous surveys were done before many products came on the market,” Meadows told Healio. “We were curious about the challenges residents in rural areas faced.”
The respondents saw a mean number of nine patients (67% female) with hereditary angioedema (HAE) each year. Also, 53% of these patients had moderate disease, and 79% of them were aged 18 to 64 years. Classifications included type I (66%) and type II (15%) HAE as well as HAE C1nl-INH (19%).
“A very high number of patients, 19%, had HAE C1nl-INH, representing a much higher proportion than has been suggested historically,” Meadows said. “By comparison, in a 2019 survey of physicians treating patients with HAE, most physicians reported having no patients (38%) or one to five patients (52%) with HAE C1nl-INH.”
On average, the researchers said, 12% of these patients lived in rural areas, defined as populations with less than 20,000 people. Also, 22% of the respondents who saw or treated more than five patients with HAE each year (n = 43) and 8% of those who saw or treated fewer than five reported patients from rural areas.
Approximately 55% of the respondents said they diagnosed more than half of their patients, and 45% diagnosed less than half. On average, referrals came from primary care or the patients themselves (31%), other specialists (18%), ED providers (12%), pediatricians (5%) and gastrointestinal specialists (2%).
Most of the respondents said they managed their patients’ disease with prophylaxis with on-demand treatment as needed, although 34% on average were treated only with acute on-demand treatment.
When selecting treatment for their patients, providers considered frequency of HAE attacks (90%), attack severity (88%), patient preferences (67%), impact of HAE on the patient’s daily life (67%) and distance the patient must travel to an ED in case of a life-threatening attack (41%).
Treatment goals included improving overall quality of life (68%), reduced attack severity (51%) and frequency (49%), attack-free status in prolonged periods of time (33%) and reduced or eliminated ED visits (33%).
During approximately half or more patient visits, 91% of respondents said they discussed HAE symptoms and when patients should seek medical attention, 87% discussed living with HAE, and 62% discussed costs associated with care.
Also, 88% of the respondents who saw more than five patients with HAE each year and 62% of those who saw fewer than five said they discussed how to administer treatment at home during approximately half or more of patient visits.
The researchers further noted that 98% of respondents said that HAE diagnosis was challenging and that it often was not recognized due to its rarity. Common challenges in diagnosis included misdiagnosis as other conditions (82%), a lack of common indicators (66%), a lack of familiarity with HAE among providers (64%), a lack of understanding of symptoms (41%) and awareness of the need for diagnosis (45%) among patients.
The high proportion of patients with HAE C1nl-INH illustrates another one of the challenges of diagnosing HAE, Meadows said, as most patients with this variant of the disease are diagnosed clinically.
“Although genetic testing is available, it does not test all known variants. A negative genetic test result does not rule out HAE C1nl-INH,” Meadows said. “We encourage industry to move forward with developing point-of-care testing kits to aid the diagnosis of HAE.”
Misinterpretation of test results was a barrier to proper diagnosis for 51% of respondents as well, as 30% reported problems with their laboratory such as unreliable or false results or mishandled samples. Additional obstacles to diagnosis included the patient’s insurance not being accepted (18%) and patients who declined testing due to high deductibles (16%).
Obstacles to care
Another challenge was lack of patient access to allergists or immunologists (43%), with 42% of respondents agreeing that “there aren’t enough specialists who can treat HAE in my area.”
More specifically, 56% of the respondents who saw more than five patients each year and 36% of those who saw fewer than five patients said there were not enough specialists in their area. Similarly, 24% of those who treated patients from rural areas and 15% of those in suburban areas strongly agreed that there were not enough specialists in their area.
The patient’s inability to afford treatment due to insurance coverage and out-of-pocket costs were challenges reported by 76% of the respondents. Also, 64% agreed and 24% strongly agreed that patients with HAE and government insurance coverage are at a disadvantage because specialists often do not accept it.
Rural residencies amplify these challenges, the researchers continued, as 59% of respondents agreed that some of their patients had to travel a great distance to see them, including 84% of the rural respondents (n = 55), 67% of the suburban respondents (n = 117) and 58% of the urban respondents (n = 76).
Specifically, 42% of respondents with rural patients, 30% of those in suburban areas and 23% of the urban respondents said half or more of their patients must travel an hour or more to visit their practices.
When respondents were asked how treatment could be better supported, 72% recommended treatments that could be administered at home, 63% suggested better partnering between community providers and allergists/immunologists and 30% indicated the need for available peer-to-peer consultations.
Other recommendations included live or virtual events about HAE (22%), education and support groups for patients (50%) and services that could drive to and from appointments (28%).
Funding could play a role as well, with respondents saying that better payments for drugs (57%) and better payments to providers (49%) from Medicaid and Medicare could help support patients in rural areas. Also, 24% said a payment system could pay primary care and specialist providers a monthly fee to provide preventive care and support rural patients.
At 93%, nearly all of the respondents said patients in rural communities should always have access to prophylaxis treatment due to their distance to hospitals, including 69% who strongly agreed with this statement. Plus, 45% said federal rules that allow more people to use telemedicine with onerous requirements would help rural patients as well.
Visit frequencies included 74% who said they see patients with HAE in person several times a year or more and 25% who said they see patients once a year or less often. Also, 74% reported using virtual visits, including 36% who said they see patients several times a year or more.
Almost all respondents (93%) said they provide telemedicine services to patients who live in the same state where their practice is located, and 82% said they do not provide out-of-state services.
However, the researchers said these numbers may be due to the COVID-19 pandemic, as 80% said they had never used telemedicine before the public health emergency, but 86% have been using it at least occasionally and 17% have been using it most or all the time.
Next steps
The researchers called for specific efforts that would improve care, beginning with universal availability of testing that is more rapid and accurate. Next, they encouraged medical organizations and Congress to expand access to telemedicine.
The researchers also said that patients with Medicare and Medicaid who live in rural areas need equity in payments for provider and hospital services. Steps would include re-evaluations of geographic price cost indexes and new payment guidelines based on time.
Finally, the researchers said that awareness and education remain great needs for HAE and all rare diseases.
Individual providers, meanwhile, should be aware of and address the changes that patients face in obtaining lifesaving drugs, Meadows told Healio.
“Recent coding changes allow for better payments to doctors that need to spend time with these types of patients,” he continued. “Too many doctors are unaware of or don’t use the codes. Too many insurance companies don’t cover the codes the way they are designed by the group that makes the codes.”
Additionally, providers should advocate for policies that protect the rights of patients with rare diseases, Meadows said.
The researchers next will survey a statistically significant number of providers who serve patients who live in rural areas.
Reference:
For more information:
J. Allen Meadows, MD, can be reached at jameadows@att.net.
Perspective
Back to Top
These findings are not surprising, but they are very significant. They also are consistent with what I see in clinical practice. HAE can be challenging in general, especially for those with HAE C1nl-INH (previously called type III). This all gets accentuated when patients live in a rural area without access to specialists or located far from emergency services.
Doctors need to get these patients to specialists who are experienced with this patient type. If you are a doctor and unaware of who has expertise in HAE, then contact the nonprofit Hereditary Angioedema Association (HAEA) at www.haea.org, which has a list of specialists who may be able to help.
This article describes the challenges of this patient type well, such as diagnostics and treatment as well as living in rural areas. On top of that, one of the biggest barriers to care of these patients is insurance. HAE exacerbations secondary to insurance obstacles are real.
Insurance companies continuously move the goalposts to make it ever more challenging and costly for doctors just trying to get their patients access to treatment.
Further, anxiety is a trigger for HAE attacks. When insurance causes delays and denials, making it almost impossible for the treating doctor to get the care the patient deserves, it provokes even more attacks in patients. Insurance frequently is a trigger and cause of suffering in these patients.
There is such minimal oversight of insurance and little recourse for doctors, it makes it costly and challenging, especially in the context of the profit margins of many insurance companies. Insurance companies are now making medical decisions about patients and dictating care when they do not have a license to do so. At a system level, this must change for the preservation of doctors and care of patients.
Collapse
Read more about
Click Here to Manage Email Alerts