A 19-year-old man with sickle cell disease presenting with spinal infarction: a case report

-April Edwards, E Leila Jerome Clay, Valerie Jewells, Stacie Adams, Regina D Crawford and Rupa Redding-Lallinger

Introduction

Vasculopathy of the large vessels commonly occurs in sickle cell disease, and as a result cerebral infarction is a well characterized complication of this condition. However, spinal infarction appears to be rare. Spinal infarct is infrequent in the non-sickle cell population as well, and accounts for only about 1 percent of all central nervous system infarcts.

Case presentation

In the present work, we report the case of a 19-year-old African-American man with sickle cell disease who experienced an anterior spinal infarct and subsequent quadriplegia. He was incidentally noted to be a heterozygote for factor V Leiden. We also reviewed the literature and found two previous cases of spinal cord infarction and sickle haemoglobin. Our literature search did not demonstrate that heterozygocity for factor V Leiden plays an important role in spinal cord infarction.

Conclusions

The paucity of cases associated with sickle haemoglobin does not allow us to postulate any particular risk factors with sickle cell disease that might predispose patients to spinal cord infarction. Our patient’s case raises the question as to whether spinal cord infarction is being missed in individuals with sickle cell disease and neurologic symptoms.

Introduction

Cerebral infarction is the most common neurologic complication that occurs with sickle cell disease (SCD); it can be either overt or silent and it can be associated with significant morbidity. Overt stroke in SCD was first characterized in 1923, and histopathologic studies later revealed large vessel narrowing with superimposed thrombosis as the underlying cause. Though cerebral infarction is the most frequent neurological complication, a number of other potentially devastating central nervous system (CNS) sequelae have also been described. These include: intra-cranial hemorrhage, isolated neuropathies, transverse myelitis, auditory and ocular manifestations, and spinal cord involvement. In the spinal cord there has been a description of cord compression by extramedullary hemopoietic tissue in addition to rare case reports of spinal cord infarction.

In the non-sickle cell disease population it appears that spinal infarct is much less frequent than cerebral infarction as well, and accounts for only about 1 percent of all CNS infarcts. Of those with spinal infarction, most appear to be from traumatic or surgical aetiologies than other organic causes. Aortic disease is a frequent culprit with many case reports detailing adverse sequelae following surgical repair of aneurysms, but also aortic thrombosis, and aortic dissection. Other non-traumatic, non-surgical aetiologies of spinal cord infarct include: global hypotension and/or arterial insufficiency, often after cardiac arrest; transient ischemic attacks; fibrocartilagenous emboli; arterial vascular malformations; syphilitic arteritis and adjacent spinal disease. In a 2006 study, Novy et al. noted that 12 of their 27 patients with spinal infarct had pre-existing spinal disease including compression fractures, spondylolisthesis, chronic arachonoiditis and chronic cervical disk protrusion, and of those 12 patients, 11 had an infarct at the level of their pre-existing disease. However, the cause of spinal infarct is frequently cryptogenic.

There is considerable evidence that sickle cell disease represents a hypercoagulable state. It appears that nearly every component of haemostasis is altered to some degree in SCD. Studies have indicated that in sickle cell disease there is increased platelet activation and aggregation, increased levels of D-dimer and fibrinogen and fibrin-fibrinogen complex while there are simultaneously decreased factors V, VII, VIIa and proteins C and S. There is good evidence that there is externalization of phosphatidylserine (a phospholipid normally found in the inner monolayer of red blood cells) in SCD, which is thought to play a significant role in promoting macrophage recognition in erythrophagocytosis and thus triggering a signal for the coagulation process. Increased phosphatidylserine exposure is also thought to be associated with increased tissue factor expression. However, it remains unclear how or to what extent those abnormalities contribute to disease complications such as cerebral and spinal infarcts.

Because cerebral infarcts occur only in a subset of the sickle cell population, it has been postulated that there may be identifiable features of this subgroup that exacerbate the hypercoagulable state of sickle cell disease. In the search for possible characteristics of this subpopulation, some have begun to explore factors that predispose the general population to coagulation abnormalities and thrombophilia. Specifically, there have been case reports of persons with SCD who developed CNS infarcts and were found to have the factor V Leiden, a prothrombin gene variant, a methylenetetrahidrofolate reductase gene mutation, or some combination of those mutations.

These studies were conducted in Brazil and Israel; notably the prevalence of the factor V Leiden and the prothrombin gene variant are known to be very low in African-Americans. Also, there have been a few single nucleotide polymorphisms (SNPs) in persons with SCD that have been found to be associated with increased stroke risk: ANXA2, TGFBR3, and TEK were noted in a study including these SNPs. However, further validation is needed before these can be used to prospectively guide recommendations for molecular genetic testing or treatment. There is no known identifiable thrombophilic abnormality that predicts cerebral infarction in sickle cell disease.

Case presentation

On the morning of admission, our patient, a 19-year-old African-American man with sickle cell anaemia, felt himself to be in his usual state of health, although he had just been discharged the previous day from a hospitalization for acute chest syndrome. He ate breakfast and spent the day watching television. However, at approximately 5:45 p.m. when he used the bathroom, he noticed that he could not pull up his trousers due to weakness in his left arm.

As he walked out of the bathroom, he noted that he was having difficulty walking because of weakness in his right leg. As his mother was helping him to his bed, his left leg also became weak. He began experiencing ‘shocking’ pains on both sides of his neck, which were unlike his usual pain, and also noted that he had an erection. These events transpired rapidly, within about six minutes, at which point his family called Emergency Medical Services (EMS) and our patient was transported to our hospital.

On arrival at our hospital, he was alert and oriented and cranial nerves II to XII were intact. He had flaccid paralysis of the bilateral upper extremities and the left lower extremity, and normal tone with 5 out of 5 strength in the right lower extremity. He had areflexia in the biceps, triceps, and brachioradialis bilaterally, hyper-reflexia at the left patella, and sustained clonus at the left Achilles. Sensation was intact throughout. The results of the rest of his physical examination were normal.

Relevant medical history included asthma, recurrent acute chest syndrome (>10 episodes), and intermittent attempts at hydroxyurea treatment with poor compliance over the previous 10 years. Following the identification of silent cerebral infarcts, he was treated for the three years between 2005 and 2008 with exchange transfusions to maintain haemoglobin S < 30 percent; during this time he did very well. At 10 days prior to presentation, he was hospitalized with an acute chest syndrome. During that hospitalization he had an initial PO2 of 76, a haemoglobin (Hb)/haematocrit (Hct) nadir of 5.8/17, and was found to have a methicillin-resistant Staphylococcus aureus (MRSA) pneumonia. He was treated with antibiotics and a transfusion. His discharge haemoglobin was 6.6 and oxygen saturation 96 percent. He was without symptoms at the time of discharge.

Admission laboratory test data included a white blood cell count of 12 × 103/uL Hb 8.7g/dL, Hct 26 percent, platelets 449 × 103 cells/mm3 with a haemoglobin electrophoresis of HbA 86 percent, HbS 7 percent, and HbC 7 percent. He had a lumbar puncture that demonstrated unremarkable cerebrospinal fluid findings and no evidence of IgG oligoclonal bands. The results of peripheral blood and urine cultures were negative. A chest X-ray showed patchy consolidation in the right upper lobe suspicious for pneumonia. The results of computed tomography (CT) angiography of the head and neck were unremarkable. Given concern for spinal cord involvement, 1.5T T1, T2, and fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) studies of the brain and cervical spine was performed showing an abnormal T2/FLAIR signal in the cervical spinal cord, which was thought at that time likely to be due to artifact.

Later the initial MRI was read to also show swelling of the cord in the same area. He was admitted to the neurologic intensive care unit where he received an exchange transfusion with no significant improvement in his symptoms; subsequent haemoglobin electrophoresis showed HbA 85 percent, HbS 9 percent. While in the intensive care unit (ICU) he experienced episodes of hypotension that were initially managed with vasopressors. After his blood pressure stabilized he was transitioned to fludrocortisone and midodrine. He never had respiratory insufficiency. Two days after admission he had a repeat MRI, which showed T2 hyperintense signal extending from C2 through to C7 (Figure 1A). In addition, diffusion-weighted imaging demonstrated restricted diffusion consistent with a focus of infarction in addition to cord oedema and swelling in the grey and white matter of the right side of the cord.

There was associated enlargement of the spinal cord consistent with oedema from the anterior spinal infarct. A hypercoagulability investigation performed during his hospitalization included a polymerase chain reaction (PCR) study that demonstrated that he was heterozygous for the factor V Leiden 1691 G>A mutation. Other studies performed were for factor VIII, fibrinogen, functional anti-thrombin, lupus anti-coagulant, anti-cardiolipin, all of which were within normal limits. His erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were both elevated, and proteins C and S were found to be low but within the expected range for someone with sickle cell disease. He was anti-coagulated with a heparin drip during his stay in the acute care facility, but this was discontinued on discharge. A monthly exchange transfusion regimen was instituted with the goal of keeping his haemoglobin S level < 30 percent.

Figure 1. A,B T2 hyperintense signal extending from C2 to C7 with oedema of the grey and white matter of the cord. The arrows point to the oedema. As with all infarcts, the area of infarct is bright on B1000 and dark on apparent diffusion coefficient sequences.

Although initially there was almost complete paralysis of his extremities, over the four days he spent in the neurologic ICU, our patient demonstrated slow but steady progress in regaining some motor function of his affected limbs. He was transferred from the ICU to the wards on day five and began working with physical and occupational therapy. On day 10, he was transferred to a rehabilitation facility, where he made gradual but steady progress in regaining motor function. He was discharged home after three weeks.

Five months after the acute onset of paralysis, he had some residual left arm and leg weakness and spasticity, but was able to walk unassisted and perform most activities of daily living without assistance. A repeat MRI scan showed a persistence of slight T2 signal abnormality in the cervical cord, consistent with previous spinal cord infarction. There was no spinal cord atrophy (Figure 2). Our patient continued to make progress, regaining much of his strength and function, and was maintained on a regimen of monthly scheduled exchange transfusions.

Figure 2. A,B Follow-up magnetic resonance imaging study demonstrating no spinal cord atrophy with residual signal from myelomalacia, months after infarct. Arrows point to the decrease in oedema.

At 18 months post-infarct he presented with complaints of three hours of generalized weakness, worse in his lower extremities in association with a pain crisis. His symptoms of weakness had largely resolved by the time he arrived at our Emergency Department. On examination he had 4 out of 5 strength in his left lower extremity and 5 out of 5 in right lower extremity, and 3 out of 5 grip strength bilaterally with a slightly unsteady gait; these findings were not substantially different from his post-spinal cord infarction baseline. His hemoglobin S was 51.5 percent at that time. Repeat imaging studies of his brain and spine at that time were unchanged from his prior studies. He was admitted and had an exchange transfusion achieving a post-transfusion HbS of 8.3 percent. He was given daily low-dose (81mg) aspirin. Currently, at 20 months post-spinal cord infarction, his condition is unchanged.

Discussion

Spinal cord infarct is infrequent compared to cerebral infarction in the general population, and most commonly occurs as a result of a dissecting aortic aneurysm or aortic surgery. In persons with sickle haemoglobin, significant spinal cord infarction appears to be an even more rare neurologic complication. To the best of our knowledge, there are only two reported cases of other persons, both now deceased, detailing this pathology . Of note, the radiographic findings from our patient have been previously presented in a radiology journal with emphasis on the diffusion-weighted images, but in this report we describe the clinical details and our patient’s subsequent course.

There is a case report from 1970 of a 59-year-old Jamaican woman with presumed sickle cell trait who deteriorated over the course of several years to near complete paraplegia and who was subsequently found to have a slightly swollen spinal cord in the cervical region and atrophic thoracic and lumbar spine cord segments on autopsy. The authors noted that her vasculature and neural tissue was otherwise without the stigmata of significant atherosclerotic or degenerative disease, and while no thrombosed vessels were found in relation to the areas of necrosis in her spinal cord, there were however, many arteries and veins distended with abnormally shaped sickle red cells.

A 1980 case report describes a 19-year-old African-American man with sickle cell disease who developed sudden-onset quadriplegia and in post-mortem studies was found to have multiple, old, focal and confluent infarcts involving the cortex and subcortical white matter in the brain, and also of the cervical, thoracic, and upper lumbar spinal cord. There are no data from these case reports in the literature concerning other potential risk factors including any thrombophilic abnormalities, as these were not commonly looked for in 1970 and 1980.
From the available reports that have looked for an association between factor V Leiden and complications of sickle cell disease, there is no evidence of an obvious relationship.

Kahn et al. studied a cohort of 82 patients with different sickle cell states, 19 of whom had had a stroke. Only one of the 82 was heterozygous for factor V Leiden (there were no homozygotes), and this was not a patient who had experienced a stroke, priapism or any other vascular-type. Andrade et al. similarly examined a cohort of 73 patients with sickle cell disease in Brazil, of whom five had a stroke. One of the five was a heterozygote for factor V Leiden; of the patients who had not experienced a stroke, none were positive for the factor V Leiden mutation. Interestingly, that patient had a sister who also had sickle cell anaemia and stroke, but the sister did not carry the factor V Leiden mutation. We conclude that our patient’s heterozygosity for factor V Leiden did not contribute to the occurrence of the spinal cord infarction.

Our patient has severe sickle cell disease as manifested by multiple bouts of recurrent acute chest syndrome and the presence of a silent cerebral infarction. As a comorbidity which predisposes to more severe disease, he also has asthma. However, he would not be considered to be very unusual in having this degree of illness. Therefore, the question arises as to why he developed the rare complication of spinal cord infarction. It occurred during the recovery from an episode of acute chest syndrome, which is known to be a time period of increased risk for cerebral infarction, but this is clearly not a full explanation given the frequency of acute chest syndrome and the rarity of spinal cord infarction.

His hypoxemia had resolved when the spinal cord infarction occurred, and his worsened anaemia had been corrected. In addition, his sickle haemoglobin percentage was quite low. Although our review of the literature does not suggest that his infarct can be explained by the factor V Leiden heterozygosity, he was not tested for any of the other genetic variants that have been recently found to be associated with stroke in SCD such as ANXA2, TGFBR3, and TEK. It is possible that a combination of factor V Leiden heterozygosity and another mutation may increase his risk for this complication. However, in order to determine risk factors for this complication, its true incidence in SCD must be known.

Conclusions

It is possible that spinal cord infarction may occur more commonly than previously recognized in sickle cell disease and is missed or misdiagnosed as cerebral infarction. Although in our patient’s case there were clear findings suggestive of spinal cord involvement, some presentations could be more subtle, and many clinicians may not think of the spinal cord when a patient with sickle cell presents with neurologic deficits. We hope that this report may lead others who care for people with sickle cell disease to be vigilant to the possibility of central nervous system infarction involving the spinal cord.

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Geralyn Spollett, MSN, C-ANP, CDE

The specialized role of nursing in the care and education of people with diabetes has been in existence for more than 30 years. Diabetes education carried out by nurses has moved beyond the hospital bedside into a variety of health care settings. Among the disciplines involved in diabetes education, nursing has played a pivotal role in the diabetes team management concept. This was well illustrated in the Diabetes Control and Complications Trial (DCCT) by the effectiveness of nurse managers in coordinating and delivering diabetes self-management education. These nurse managers not only performed administrative tasks crucial to the outcomes of the DCCT, but also participated directly in patient care.

The emergence and subsequent growth of advanced practice in nursing during the past 20 years has expanded the direct care component, incorporating aspects of both nursing and medical care while maintaining the teaching and counselling roles. Both the clinical nurse specialist (CNS) and nurse practitioner (NP) models, when applied to chronic disease management, create enhanced patient-provider relationships in which self-care education and counselling is provided within the context of disease state management. Clement commented in a review of diabetes self-management education issues that unless ongoing management is part of an education program, knowledge may increase but most clinical outcomes only minimally improve. Advanced practice nurses by the very nature of their scope of practice effectively combine both education and management into their delivery of care.

Operating beyond the role of educator, advanced practice nurses holistically assess patients’ needs with the understanding of patients’ primary role in the improvement and maintenance of their own health and wellness. In conducting assessments, advanced practice nurses carefully explore patients’ medical history and perform focused physical exams. At the completion of assessments, advanced practice nurses, in conjunction with patients, identify management goals and determine appropriate plans of care. A review of patients’ self-care management skills and application/adaptation to lifestyle is incorporated in initial histories, physical exams, and plans of care.

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Many advanced practice nurses (NPs, CNSs, nurse midwives, and nurse anaesthetists) may prescribe and adjust medication through prescriptive authority granted to them by their state nursing regulatory body. Currently, all 50 states have some form of prescriptive authority for advanced practice nurses. The ability to prescribe and adjust medication is a valuable asset in caring for individuals with diabetes. It is a crucial component in the care of people with type 1 diabetes, and it becomes increasingly important in the care of patients with type 2 diabetes who have a constellation of comorbidities, all of which must be managed for successful disease outcomes.

Many studies have documented the effectiveness of advanced practice nurses in managing common primary care issues. NP care has been associated with a high level of satisfaction among health services consumers. In diabetes, the role of advanced practice nurses has significantly contributed to improved outcomes in the management of type 2 diabetes, in specialized diabetes foot care programs, in the management of diabetes in pregnancy, and in the care of paediatric type 1 diabetic patients and their parents. Furthermore, NPs have also been effective providers of diabetes care among disadvantaged urban African-American patients. Primary management of these patients by NPs led to improved metabolic control regardless of whether weight loss was achieved.

The following case study illustrates the clinical role of advanced practice nurses in the management of a patient with type 2 diabetes.

Case Presentation

A.B. is a retired 69-year-old man with a 5-year history of type 2 diabetes. Although he was diagnosed in 1997, he had symptoms indicating hyperglycemia for 2 years before diagnosis. He had fasting blood glucose records indicating values of 118–127 mg/dl, which were described to him as indicative of “borderline diabetes.” He also remembered past episodes of nocturia associated with large pasta meals and Italian pastries. At the time of initial diagnosis, he was advised to lose weight (“at least 10 lb.”), but no further action was taken.

Referred by his family physician to the diabetes specialty clinic, A.B. presents with recent weight gain, suboptimal diabetes control, and foot pain. He has been trying to lose weight and increase his exercise for the past 6 months without success. He had been started on glyburide (Diabeta), 2.5 mg every morning, but had stopped taking it because of dizziness, often accompanied by sweating and a feeling of mild agitation, in the late afternoon.

A.B. also takes atorvastatin (Lipitor), 10 mg daily, for hypercholesterolemia (elevated LDL cholesterol, low HDL cholesterol, and elevated triglycerides). He has tolerated this medication and adheres to the daily schedule. During the past 6 months, he has also taken chromium picolinate, gymnema sylvestre, and a “pancreas elixir” in an attempt to improve his diabetes control. He stopped these supplements when he did not see any positive results.
He does not test his blood glucose levels at home and expresses doubt that this procedure would help him improve his diabetes control. “What would knowing the numbers do for me?,” he asks. “The doctor already knows the sugars are high.”

A.B. states that he has “never been sick a day in my life.” He recently sold his business and has become very active in a variety of volunteer organizations. He lives with his wife of 48 years and has two married children. Although both his mother and father had type 2 diabetes, A.B. has limited knowledge regarding diabetes self-care management and states that he does not understand why he has diabetes since he never eats sugar. In the past, his wife has encouraged him to treat his diabetes with herbal remedies and weight-loss supplements, and she frequently scans the Internet for the latest diabetes remedies.

During the past year, A.B. has gained 22 lb. Since retiring, he has been more physically active, playing golf once a week and gardening, but he has been unable to lose more than 2–3 lb. He has never seen a dietitian and has not been instructed in self-monitoring of blood glucose (SMBG).

A.B.’s diet history reveals excessive carbohydrate intake in the form of bread and pasta. His normal dinners consist of 2 cups of cooked pasta with homemade sauce and three to four slices of Italian bread. During the day, he often has “a slice or two” of bread with butter or olive oil. He also eats eight to ten pieces of fresh fruit per day at meals and as snacks. He prefers chicken and fish, but it is usually served with a tomato or cream sauce accompanied by pasta. His wife has offered to make him plain grilled meats, but he finds them “tasteless.” He drinks 8 oz. of red wine with dinner each evening. He stopped smoking more than 10 years ago, he reports, “when the cost of cigarettes topped a buck-fifty.”

The medical documents that A.B. brings to this appointment indicate that his haemoglobin A1c (A1C) has never been <8%. His blood pressure has been measured at 150/70, 148/92, and 166/88 mmHg on separate occasions during the past year at the local senior centre screening clinic. Although he was told that his blood pressure was “up a little,” he was not aware of the need to keep his blood pressure ≤130/80 mmHg for both cardiovascular and renal health.

A.B. has never had a foot exam as part of his primary care exams, nor has he been instructed in preventive foot care. However, his medical records also indicate that he has had no surgeries or hospitalizations, his immunizations are up to date, and, in general, he has been remarkably healthy for many years.

Physical Exam

A physical examination reveals the following:

• Weight: 178 lb; height: 5′2″; body mass index (BMI): 32.6 kg/m2
• Fasting capillary glucose: 166 mg/dl
• Blood pressure: lying, right arm 154/96 mmHg; sitting, right arm 140/90 mmHg
• Pulse: 88 bpm; respirations 20 per minute
• Eyes: corrective lenses, pupils equal and reactive to light and accommodation, Fundi-clear, no arteriolovenous nicking, no retinopathy
• Thyroid: non palpable
• Lungs: clear to auscultation
• Heart: Rate and rhythm regular, no murmurs or gallops
• Vascular assessment: no carotid bruits; femoral, popliteal, and dorsalis pedis pulses 2+ bilaterally
• Neurological assessment: diminished vibratory sense to the forefoot, absent ankle reflexes, monofilament (5.07 Semmes-Weinstein) felt only above the ankle

Lab Results

Results of laboratory tests (drawn 5 days before the office visit) are as follows:

• Glucose (fasting): 178 mg/dl (normal range: 65–109 mg/dl)
• Creatinine: 1.0 mg/dl (normal range: 0.5–1.4 mg/dl)
• Blood urea nitrogen: 18 mg/dl (normal range: 7–30 mg/dl)
• Sodium: 141 mg/dl (normal range: 135–146 mg/dl)
• Potassium: 4.3 mg/dl (normal range: 3.5–5.3 mg/dl)
• Lipid panel
     
  • Total cholesterol: 162 mg/dl (normal: <200 mg/dl)

     

  • HDL cholesterol: 43 mg/dl (normal: ≥40 mg/dl)

     

  • LDL cholesterol (calculated): 84 mg/dl (normal: <100 mg/dl)

     

  • Triglycerides: 177 mg/dl (normal: <150 mg/dl)
  •    

  • Cholesterol-to-HDL ratio: 3.8 (normal: <5.0)
• AST: 14 IU/l (normal: 0–40 IU/l)
• ALT: 19 IU/l (normal: 5–40 IU/l)
• Alkaline phosphotase: 56 IU/l (normal: 35–125 IU/l)
• A1C: 8.1% (normal: 4–6%)
• Urine microalbumin: 45 mg (normal: <30 mg)

Assessment

Based on A.B.’s medical history, records, physical exam, and lab results, he is assessed as follows:

• Uncontrolled type 2 diabetes (A1C >7%)
• Obesity (BMI 32.4 kg/m2)
• Hyperlipidemia (controlled with atorvastatin)
• Peripheral neuropathy (distal and symmetrical by exam)
• Hypertension (by previous chart data and exam)
• Elevated urine microalbumin level
• Self-care management/lifestyle deficits

   

• Limited exercise

   

• High carbohydrate intake

   

• No SMBG program
• Poor understanding of diabetes






Discussion

A.B. presented with uncontrolled type 2 diabetes and a complex set of comorbidities, all of which needed treatment. The first task of the NP who provided his care was to select the most pressing health care issues and prioritize his medical care to address them. Although A.B. stated that his need to lose weight was his chief reason for seeking diabetes specialty care, his elevated glucose levels and his hypertension also needed to be addressed at the initial visit.

The patient and his wife agreed that a referral to a dietician was their first priority. A.B. acknowledged that he had little dietary information to help him achieve weight loss and that his current weight was unhealthy and “embarrassing.” He recognized that his glucose control was affected by large portions of bread and pasta and agreed to start improving dietary control by reducing his portion size by one-third during the week before his dietary consultation. Weight loss would also be an important first step in reducing his blood pressure.

The NP contacted the registered dietician (RD) by telephone and referred the patient for a medical nutrition therapy assessment with a focus on weight loss and improved diabetes control. A.B.’s appointment was scheduled for the following week. The RD requested that during the intervening week, the patient keep a food journal recording his food intake at meals and snacks. She asked that the patient also try to estimate portion sizes.

Although his physical activity had increased since his retirement, it was fairly sporadic and weather-dependent. After further discussion, he realized that a week or more would often pass without any significant form of exercise and that most of his exercise was seasonal. Whatever weight he had lost during the summer was regained in the winter, when he was again quite sedentary.

A.B.’s wife suggested that the two of them could walk each morning after breakfast. She also felt that a treadmill at home would be the best solution for getting sufficient exercise in inclement weather. After a short discussion about the positive effect exercise can have on glucose control, the patient and his wife agreed to walk 15–20 minutes each day between 9:00 and 10:00 a.m.

A first-line medication for this patient had to be targeted to improving glucose control without contributing to weight gain. Thiazolidinediones (i.e., rosiglitizone [Avandia] or pioglitizone [Actos]) effectively address insulin resistance but have been associated with weight gain.12 A sulfonylurea or meglitinide (i.e., repaglinide [Prandin]) can reduce postprandial elevations caused by increased carbohydrate intake, but they are also associated with some weight gain.12 When glyburide was previously prescribed, the patient exhibited signs and symptoms of hypoglycemia (unconfirmed by SMBG). α-Glucosidase inhibitors (i.e., acarbose [Precose]) can help with postprandial hyperglycemia rise by blunting the effect of the entry of carbohydrate-related glucose into the system. However, acarbose requires slow titration, has multiple gastrointestinal (GI) side effects, and reduces A1C by only 0.5–0.9%.13 Acarbose may be considered as a second-line therapy for A.B. but would not fully address his elevated A1C results. Metformin (Glucophage), which reduces hepatic glucose production and improves insulin resistance, is not associated with hypoglycemia and can lower A1C results by 1%. Although GI side effects can occur, they are usually self-limiting and can be further reduced by slow titration to dose efficacy.

After reviewing these options and discussing the need for improved glycaemic control, the NP prescribed metformin, 500 mg twice a day. Possible GI side effects and the need to avoid alcohol were of concern to A.B., but he agreed that medication was necessary and that metformin was his best option. The NP advised him to take the medication with food to reduce GI side effects.

The NP also discussed with the patient a titration schedule that increased the dosage to 1,000 mg twice a day over a 4-week period. She wrote out this plan, including a date and time for telephone contact and medication evaluation, and gave it to the patient.

During the visit, A.B. and his wife learned to use a glucose meter that features a simple two-step procedure. The patient agreed to use the meter twice a day, at breakfast and dinner, while the metformin dose was being titrated. He understood the need for glucose readings to guide the choice of medication and to evaluate the effects of his dietary changes, but he felt that it would not be “a forever thing.”

The NP reviewed glycaemic goals with the patient and his wife and assisted them in deciding on initial short-term goals for weight loss, exercise, and medication. Glucose monitoring would serve as a guide and assist the patient in modifying his lifestyle.

A.B. drew the line at starting an antihypertensive medication—the angiotensin-converting enzyme (ACE) inhibitor enalapril (Vasotec), 5 mg daily. He stated that one new medication at a time was enough and that “too many medications would make a sick man out of me.” His perception of the state of his health as being represented by the number of medications prescribed for him gave the advanced practice nurse an important insight into the patient’s health belief system. The patient’s wife also believed that a “natural solution” was better than medication for treating blood pressure.

Although the use of an ACE inhibitor was indicated both by the level of hypertension and by the presence of microalbuminuria, the decision to wait until the next office visit to further evaluate the need for antihypertensive medication afforded the patient and his wife time to consider the importance of adding this pharmacotherapy. They were quite willing to read any materials that addressed the prevention of diabetes complications. However, both the patient and his wife voiced a strong desire to focus their energies on changes in food and physical activity. The NP expressed support for their decision. Because A.B. was obese, weight loss would be beneficial for many of his health issues.

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Because he has a sedentary lifestyle, is >35 years old, has hypertension and peripheral neuropathy, and is being treated for hypercholestrolemia, the NP performed an electrocardiogram in the office and referred the patient for an exercise tolerance test. In doing this, the NP acknowledged and respected the mutually set goals, but also provided appropriate pre-exercise screening for the patient’s protection and safety.

In her role as diabetes educator, the NP taught A.B. and his wife the importance of foot care, demonstrating to the patient his inability to feel the light touch of the monofilament. She explained that the loss of protective sensation from peripheral neuropathy means that he will need to be more vigilant in checking his feet for any skin lesions caused by poorly fitting footwear worn during exercise.

At the conclusion of the visit, the NP assured A.B. that she would share the plan of care they had developed with his primary care physician, collaborating with him and discussing the findings of any diagnostic tests and procedures. She would also work in partnership with the RD to reinforce medical nutrition therapies and improve his glucose control. In this way, the NP would facilitate the continuity of care and keep vital pathways of communication open.

Summary

Advanced practice nurses are ideally suited to play an integral role in the education and medical management of people with diabetes. The combination of clinical skills and expertise in teaching and counselling enhances the delivery of care in a manner that is both cost-reducing and effective. Inherent in the role of advanced practice nurses is the understanding of shared responsibility for health care outcomes. This partnering of nurse with patient not only improves care but strengthens the patient’s role as self-manager.

Footnotes

Geralyn Spollett, MSN, C-ANP, CDE, is associate director and an adult nurse practitioner at the Yale Diabetes Centre, Department of Endocrinology and Metabolism, at Yale University in New Haven, Conn. She is an associate editor of Diabetes Spectrum.






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