Celiac disease - Nursing Case Study

Pathophysiology

• Primary mechanism: Celiac disease is an autoimmune disorder triggered by ingestion of gluten, a protein found in wheat, barley, and rye. Once gluten is consumed, the immune system of a person with Celiac disease reacts abnormally, damaging the small intestine's villi (tiny, finger-like projections that absorb nutrients).

• Secondary mechanism: The damage to the villi results in malabsorption of nutrients. Regardless of the amount of food consumed, the individual may become malnourished due to the inability of the damaged small intestine to properly absorb nutrients into the bloodstream.

• Key complication: Over time, the continuous inflammation and damage in the small intestine can cause complications like osteoporosis, infertility, nerve damage, and seizures due to anemia or vitamin and mineral deficiencies.

Patient Profile

Demographics:

47-year-old female, elementary school teacher

History:

• Key past medical history: Diagnosed with Celiac disease 5 years ago

• Current medications: Gluten-free diet, multivitamin supplement, regular intake of calcium and vitamin D

• Allergies: No known drug allergies

Current Presentation:

• Chief complaint: Persistent diarrhea, fatigue, and weight loss despite adhering to a strict gluten-free diet

• Key symptoms: Abdominal pain, bloating, mouth ulcers, joint pain, and an itchy rash (dermatitis herpetiformis)

• Vital signs: Blood pressure 115/75, pulse 92, temperature 98.6°F, respiratory rate 18, BMI 18.2 indicating underweight

Section 1

Change in Patient Status:

Over the next week, the patient's condition deteriorates, with increased fatigue and weight loss, and she returns to the emergency department. She reports that her abdominal pain has worsened and she now experiences occasional bouts of nausea and vomiting. Her joint pain has also escalated, affecting her mobility. On physical examination, the patient appears pale and has lost another 3 pounds since her last visit. Her BMI is now 17.9, further indicating malnutrition. She also exhibits signs of dehydration with poor skin turgor and dry mucous membranes. Her vital signs are blood pressure 105/72, pulse 100, and temperature 98.4°F, and respiratory rate 20.

This deterioration suggests that the patient's condition is worsening despite maintaining a gluten-free diet, indicating possible refractory celiac disease, which is a severe form of celiac disease that doesn't respond to a gluten-free diet. Malabsorption of nutrients appears to be progressing, with signs of malnutrition and dehydration. The escalation of joint pain might indicate the development of arthritis, another complication of celiac disease. This change in patient status calls for further diagnostic testing and a review of her current management plan.

Section 2

New Diagnostic Results:

The medical team orders a complete blood count (CBC), serum albumin, and erythrocyte sedimentation rate (ESR) to further evaluate her nutritional status and inflammation. The CBC results reveal a hemoglobin of 9.2 g/dL (normal: 12-15.5 g/dL), indicating anemia. The albumin level is low at 2.5 g/dL (normal: 3.5-5.0 g/dL), suggesting protein malnutrition. The ESR is elevated at 50 mm/hr (normal: 0-22 mm/hr), reflecting systemic inflammation.

The team also orders a repeat biopsy of the small intestine, which reveals persistent villous atrophy, crypt hyperplasia, and increased intraepithelial lymphocytes. These findings are consistent with refractory celiac disease type 1 (RCD1), indicating that the patient's illness is not responding to a gluten-free diet.

These new diagnostic results provide concrete evidence of the patient's deteriorating condition and suggest that her celiac disease has become refractory. The medical team will need to consider a new management plan to address the patient's malnutrition, inflammation, and persistent damage to the small intestine.

Section 3

Change in Patient Status:

The patient's condition continues to deteriorate over the following days. She reports increasing fatigue, weakness, and a decrease in appetite. She also complains of chronic diarrhea and abdominal distention, which is consistent with her malabsorption syndrome. On examination, her abdomen is distended with visible peristaltic waves, suggestive of bowel obstruction. Her heart rate is elevated at 110 beats per minute (normal: 60-100 beats per minute) and blood pressure is slightly low at 100/60 mmHg (normal: 120/80 mmHg), indicative of possible dehydration.

Her weight has also decreased by 5% over the past month, further demonstrating the severity of her malnutrition. Furthermore, the patient shows signs of peripheral edema, indicating hypoalbuminemia. She is also pale and fatigued, which is consistent with her anemia. Despite her adherence to a strict gluten-free diet, her symptoms have not improved, suggesting that her celiac disease is refractory and is not responding to dietary changes. The medical team must now adjust the management plan to address these evolving complications and consider more aggressive treatments for her refractory celiac disease.

Section 4

New Diagnostic Results:

The recent laboratory results reveal some significant abnormalities. The patient's albumin level is low at 2.5 g/dL (normal: 3.5-5.5 g/dL), which explains the peripheral edema due to hypoalbuminemia. Her hemoglobin is also low at 9.1 g/dL (normal: 12-16 g/dL for women), indicating anemia, likely due to poor nutritional absorption. The patient's electrolyte panel shows hypokalemia with a potassium level of 2.9 mEq/L (normal: 3.5-5.0 mEq/L), a common complication in those with chronic diarrhea. Her total protein is also low at 5.1 g/dL (normal: 6.0-8.3 g/dL), further confirming her malnutrition.

A CT scan of her abdomen shows marked thickening of the small bowel wall, raising concerns for refractory celiac disease, and potential complications such as lymphoma or ulcerative jejunoileitis, a severe form of celiac disease characterized by ulcers in the small intestines. The medical team decides to conduct a capsule endoscopy and take a biopsy for further evaluation. These new findings necessitate a change in the treatment strategy, and the team begins to consider more aggressive approaches like immunosuppressive therapy or parenteral nutrition to manage her refractory celiac disease and its complications.

Section 5

Response to Interventions:

The medical team initiates the patient on a strict gluten-free diet, along with parenteral nutrition to address the malnutrition. The plan also includes potassium supplements to rectify the hypokalemia and iron supplements for the anemia. Despite these interventions, the patient's conditions do not improve as expected. After one week, her albumin level remains low at 2.8 g/dL, and her potassium level only slightly increases to 3.0 mEq/L. The hemoglobin level is still below normal at 9.3 g/dL.

The persistent hypoalbuminemia and hypokalemia, along with her unimproved anemia, suggest that her refractory celiac disease is not responding to the dietary changes and supplements as expected. The lack of response raises concerns about a possible complication such as lymphoma or ulcerative jejunoileitis, as suggested by the CT findings. Given this, the medical team decides to proceed with the capsule endoscopy and biopsy to further investigate the cause of the refractory nature of her celiac disease. This scenario requires a higher level of clinical reasoning and pushes towards a more aggressive treatment approach.