This article was previously published in the IDF Advocate.
Primary immunodeficiency diseases (PI) can be difficult for many, but it also may only be one of several diseases that they are trying to manage simultaneously. Over the past decade, people dealing with multiple chronic conditions (MCC) has escalated dramatically. MCC is defined as two or greater chronic conditions simultaneously occurring. This has become a public health problem which offers significant medical challenges to our healthcare system.
The most common reason for this dynamic change to MCC being more prevalent is due to an increase in life expectancy, which contributed greatly to our aging population. Several examples include a decrease in childhood fatalities because of better infant care through the use of vaccines and cancer screening, such as mammograms, colonoscopies, and PSA testing in adults. Other contributing factors include cholesterol screening, management of hypertension, smoking cessation, and diabetes screening.
By definition, a chronic illness is a condition that lasts more than one year. In my practice, a significant majority of my patients with an underlying PI have other related or unrelated chronic conditions that make managing their underlying PI more difficult. See figure 1.1 for a list of other chronic conditions.
Unfortunately, many that have MCC may suffer from suboptimal care according to an article published in Public Health Reports 2011 titled Managing Chronic Medical Problems: A Strategic Framework in Health Outcomes and Quality of Life. As stated in the article, healthcare systems have been dependent on outdated medical models. In these medical models, each medical condition is handled separately in a linear fashion with no regard for how these other chronic conditions may impact other chronic conditions the patient has or vice versa. This leads to many frustrated patients. Despite getting treatment they still cannot achieve the quality of life they expect.
As stated in the article, this is a significant problem affecting at least one in four Americans alive today, and it’s estimated that at least two-thirds of older adults suffer from MCC. Problems that result from MCC are directly related to the number of conditions that individuals are dealing with. Complications include “increased mortality, poor functional status, unnecessary hospitalization, adverse drug events and conflicting medical advice.”
Recently, several of my patients who had biopsy-proven autoimmune diseases had their findings dismissed by other specialists because serologies for their particular disease state were negative.
Another area of concern is the designs of clinical trials today, especially when it comes to the use of new medications. In many of these clinical trials, exclusion criteria may leave out certain patients from the study pool because of an underlying condition or disease. What happens in these instances is that the study population is small compared to what is being seen in the general population. This may give an incomplete picture of how effective a certain treatment regimen may be in the general population.
For example, several years ago our practice was involved in a clinical trial for a new treatment for C. difficle, the pathogen involved with antibioticassociated diarrhea and colitis. Several patients had developed C. difficle but were excluded from the study because the patient had an underlying PI. The question now becomes just how effective is this medication in those with PI and will insurance companies understand if there is treatment failure initially.
Over the past several years, the Department of Health and Human Services (HHS) has offered some modeling to improve integration of healthcare and patients dealing with multiple medical chronic conditions. Listed below is the framework that The HHS Interagency Workshop on Multiple Chronic Conditions has developed in dealing with patients with multiple medical problems. These include:
“(1) foster healthcare and public health system changes to improve the health of individuals with MCC; (2) maximize the use of proven self-care management and other services by individuals with MCC; (3) provide better tools and information to healthcare, public health, and social services workers who deliver care to individuals with MCC; (4) facilitate research to fill the knowledge gaps about and intervention and systems to benefit individuals with MCC” (Parekh et al., 2011).
As can be seen from this modeling, the challenges are lofty and will become greater as time goes on and we identify more individuals with chronic illnesses who will live longer. The challenges for the PI community, and organizations like IDF, will be to implement some of these changes to provide better guidance for patients and their providers who are living with PI and other chronic medical conditions that are making the management of their PI more difficult.
Chronic Conditions Associated with PI
These are examples of secondary chronic conditions that have been found in my patients with a diagnosis of PI.
|Chronic Bronchitis Obliterans|
|Allergic Fungal Sinusitis|
|Gastro-Esophageal Reflux Disease (GERD)|
|Inflamatory Bowel Disease (IBD)|
|Mast Cell Dysfunction|
|Paroxysmal Orthostatic Tachycardia Syndrome (POTS)|
|Gammanopathies including Multiple Myeloma|
|Fatty Liver Disease|
|Alpha 1 antitrypsin Deficiency|
|Obstructive Sleep Apnea (OSA)|