Rare Blood Disease Fatal after Slow Labs Review, Narrow Focus

Description

A 40-year-old female died of a rare blood disease several weeks after abnormal laboratory results (including a low platelet count) became available.

Key Lessons

Clinical Sequence

Allegation

The patient's estate alleged that her PCP negligently failed to diagnose TTP, which led to her death.

Disposition

This case was settled for more than $1 million.

Analysis

Clinical Perspective

1. Symptoms were not followed to a definitive diagnosis and the diagnostic delay may have made recovery less possible. This patient had many of the subtle symptoms (rash, fatigue, abdominal pain and rectal bleeding) of TTP weeks, possibly months, prior to her diagnosis. Given the patient’s continued symptoms, along with the results of lab tests done prior to her death (e.g., fall in platelet count from 302 to 59, abnormal liver function tests, etc.), the differential diagnoses should have been expanded and the seriousness of her condition better appreciated.
   
   Certain presentations are more worrisome than others and should be explored until a definitive diagnosis is made, even if a suspected condition is unlikely. Providers should consider the seriousness of the worst likely outcome when considering problems that don't have a clear source. This is a factor in deciding on possible follow-up tests to address the symptoms and monitor the patient’s status. When two disease processes are in close proximity of each other, the challenge is to recognize when a single complaint may be masking a second problem, and then fully evaluate, document and follow both concerns. TTP is a rare disease with an annual incidence in the United States estimated at 4 to 11 cases per million people. If it goes undiagnosed/untreated it has a very high mortality rate, but if caught in time, the disease responds well to plasma-exchange treatment. Recognition can be difficult though, as its most common symptoms are non-specific, including such things as malaise, weakness, nausea, vomiting, abdominal pain and changes in the skin (e.g., purpora, petechiae, jaundice). Associated risks of TTP include obesity and gender, as females have a 2 to 1 higher incidence than males.
2. The PCP’s narrow diagnostic focus had several sources: underappreciating the seriousness of this patient's continued symptoms; adherence to a belief that they were the result of a previously treated condition (rash); and the patient’s non-compliance (fatigue due to poor control of her diabetes).
   Analysis of physician error is consistent with research showing that people tend to embrace either the first solution that comes to mind, a familiar solution, or one that worked last time. This can delay a realization that it might be time for a new approach. In 54 percent of diagnosis-related malpractice cases, the provider did not order the appropriate diagnostic test or imaging—often despite an unresolved symptom or complaint. Physicians who over-rely on prior diagnoses, unconfirmed diagnoses, or intuition are especially at risk. Non-resolving symptoms can be a cue for providers to reassess clinical indicators and laboratory findings and pursue appropriate new studies and consultations.

Patient Perspective

1. Facing a long-time physician’s failure to diagnose their loved one's rare disease, the family focused on imperfections in the process of care, such as a lack of follow-up of the low platelet count several months before the ED admission and death.
   
   Patients and families may believe that medical providers should always get the diagnosis right. They are understandably driven to know if anything could have been done to prevent the bad outcome. A provider’s well-documented actions to seek a resolution of complaints can help reassure dissatisfied patients/families—and the plaintiff attorneys they may turn to—that the appropriate effort was put forth to do the right thing.

Risk Management Perspective

1. The patient’s history of non-compliance helped mask a serious unsuspected disease process.
   Appreciating the seriousness of a patient's condition can be difficult at times, especially when the patient is known to frequently not follow through with the health team's recommendations. Physicians and nurses can become frustrated and less responsive to a patient’s concerns. Providers need to be aware when this is happening, and focus on diligence in reinforcing the care plan, providing the patient with information as to the importance of following through, and documenting those discussions.
2. Although it may not have had direct bearing on the outcome, this case showed that the primary care doctor's office struggled with making sure that test turnaround time and test result management were timely and efficient; e.g. the PCP did not have a good follow-up system for ensuring that recommended tests such as guaiac stool cards were returned or that test results were reviewed in a timely manner when they were done (i.e., blood test results available two days after they were drawn but not reviewed for an additional seven days).
   
   A clinician's ability to make a timely and accurate diagnosis is reliant on proper test reporting. Best practices for physician offices include systems that allow them to reconcile outstanding labs and referrals, to ensure that the provider is aware of the results/referral and that appropriate action is taken. Some offices designate a qualified person to monitor which tests are outstanding for all patients. Paper or electronic “tickler” systems are common, along with protocols for routing and reviewing results as soon as they return.

Legal Defense Perspective

1. The physician’s documentation regarding this patient’s abnormal test results was sparse at best (i.e., “labs awful”).
   
   A claim is more defensible if the documentation supports the clinician's decision-making process. Information not in the medical record is often more troublesome than what is recorded. Include a diagnostic rationale, that includes the thinking around significant lab results and differential diagnoses. Keeping records up-to-date allows providers to provide the best care for their patients, while also providing evidence that the care was appropriate and timely.
2. The plaintiff’s expert argued that the patient's physician failed to properly follow up on the abnormally low platelet count, either by repeating the laboratory tests or referring the patient to a hematologist for consultation.
   
   A decision not to pursue unresolved diagnostic questions makes a case more difficult to defend. Weaknesses in test-reporting systems frequently factor in liability claims. Juries will be encouraged to find that, had the condition been properly identified, appropriate treatment could have been initiated, potentially resulting in the patient’s survival.