Nutritional management following bilateral sequential lung transplantation in an 18-year-old female with connective tissue disease–associated interstitial lung disease

Ishathri PV *, Selvamani N , Sangeetha R

Department of Clinical Nutrition and Dietetics, Kauvery Hospital, Vadapalani , Chennai, Tamil Nadu

*Correspondence

Abstract

Lung transplantation is a life-saving therapeutic option for patients with end-stage lung disease. Nutritional management plays a critical role in optimizing recovery, reducing complications, preserving lean body mass, and improving overall clinical outcomes following transplantation. This case report describes the nutritional management of an 18-year-old female diagnosed with interstitial lung disease (ILD) associated with connective tissue disorder and CRZ mutation, complicated by chronic respiratory failure and severe pulmonary hypertension. The patient underwent bilateral sequential lung transplantation and received comprehensive nutritional support through parenteral nutrition, enteral nutrition, oral feeding progression, and oral nutritional supplementation. Continuous monitoring of biochemical parameters, nutritional intake, and drug–nutrient interactions facilitated successful recovery and discharge. The patient successfully progressed from parenteral nutrition to enteral nutrition and finally to an oral high-protein diet, achieving nutritional goals without nutrition-related complications prior to discharge.

Keywords: Lung transplantation; Interstitial lung disease; Clinical nutrition; Enteral nutrition; Parenteral nutrition, Medical nutrition therapy

Introduction

Interstitial lung disease (ILD) is a group of chronic pulmonary disorders characterized by progressive inflammation and fibrosis of the lung parenchyma. In patients with connective tissue disorders, ILD may progress rapidly, leading to respiratory failure and significantly impaired quality of life. Lung transplantation remains the definitive treatment for selected patients with end-stage pulmonary disease.

Patients undergoing lung transplantation experience significant metabolic stress resulting from major surgery, systemic inflammation, prolonged intensive care stay, immunosuppressive therapy, and increased risk of infection. These factors substantially increase energy and protein requirements, making individualized medical nutrition therapy an integral component of postoperative care. This case report highlights the role of individualized nutrition intervention in a young patient who underwent bilateral sequential lung transplantation.

Case Presentation

An 18-year-old female was admitted on 02 May 2024 with progressive shortness of breath, fatigue, and persistent dry cough. Clinical evaluation revealed acute exacerbation of usual interstitial pneumonia (UIP), interstitial lung disease associated with CRZ mutation, chronic respiratory failure, severe pulmonary hypertension, and recurrent pulmonary infections.

Owing to progressive deterioration in pulmonary function and failure of conservative management, the patient underwent bilateral sequential lung transplantation. Following surgery, she was transferred to the intensive care unit for close monitoring and multidisciplinary management.

The patient remained hospitalized until 24 May 2024 and received comprehensive nutritional care throughout the perioperative period.

Disease Background

Connective tissue disorders are autoimmune conditions capable of affecting multiple organ systems, including the lungs. Progressive inflammatory injury may result in interstitial lung disease characterized by fibrosis and reduced pulmonary function.

In this patient, connective tissue disorder associated with CRZ mutation contributed to progressive pulmonary fibrosis, ultimately leading to respiratory failure and the requirement for lung transplantation.

Nutrition Assessment

Table 1. Patient Demographics

Parameter	Details
Gender	Female
Age	18
Date Of Admission	2-5-2024
Date Of Discharge	24-5-2024
Procedure	Bilateral Sequential Lung Transplantation

Table 2. Anthropometric Assessment

Height	Value
Height (cm)	147
Weight (Kg)	45
BMI kg/m2	20.83
Ideal Body Weight (IBW) (kg)	41-51
SGA Rating	A
Nutritional Status	Well Nourished

Note: BMI was within the normal range, the patient remained at high nutritional risk because of severe chronic respiratory disease, systemic inflammation, anticipated postoperative hyper catabolism, and prolonged ICU stay.

Nutrition Problem

PES Statement

P- Problem	E- Etiology	S- Symptoms
Inadequate energy and protein intake -Interstitial lung disease (ILD) with acute exacerbation and chronic respiratory failure.	Related to decreased oral intake, respiratory distress, and increased metabolic demand secondary to advanced interstitial lung disease- Genetic CRZ mutation associated with connective tissue disorder.	As evidenced by significant mild weight loss, poor oral intake,Shortness of breath, dry cough, fatigue, low oxygen saturation, and mild physical finding of small hand size.

Table 3. Nutritional Requirements

Parameter	Requirement
Energy	1800@35Kcals/kg/IBW
Protein	[email protected]/kg/IBW
Carbohydrates	270 g @ 60% of total calories
Fats	40g @ 20 % of total calories
Fluids	Liberal
Salt	5 g/day
Vitamins	As per RDA
Minerals	As per RDA
Diet recommendation	High-Calorie High-Protein Neutropenic Diet

The nutritional goals were to promote wound healing, preserve lean body mass, support immune function, maintain electrolyte balance, and minimize medication-related nutritional complications.

Nutrition Intervention

Nutritional support was initiated immediately following surgery. Feeding was progressively advanced according to gastrointestinal tolerance and clinical condition.

Nutrition Progression during Hospital Stay

On Postoperative Day (POD) 0, only Ryle’s tube water flushes were administered every 4 hours to maintain tube patency, as enteral nutrition was not yet initiated.

During POD 1–2, parenteral nutrition support consisting of Albumin 20% and Amino hepa was provided to meet the patient’s early postoperative nutritional requirements while gastrointestinal function recovered.

From POD 3, continuous enteral nutrition (EN) was initiated using a semi-elemental (peptide-based) formula at 50 mL/hour in combination with parenteral nutrition. Enteral feeding was gradually advanced according to tolerance. During this period, the patient underwent intermittent bronchoscopy procedures as clinically indicated.

By POD 6, parenteral nutrition was discontinued, and the patient continued on exclusive enteral nutrition with the peptide-based semi-elemental enteral formula. Although a transition to another enteral formula was planned, it was deferred due to the patient’s clinical status. Simultaneously, oral semi-solid feeding was introduced while maintaining enteral nutrition.

Between POD 7 and POD 12, oral intake was progressively advanced from a semi-solid diet to a soft diet, while enteral nutrition was continued to supplement energy and protein intake. Episodes of constipation were managed successfully through dietary modification and the addition of fibre supplementation. Nutritional intake improved steadily during this period.

On POD 13, enteral tube feeding was gradually weaned and replaced with oral nutritional supplements (ONS) using a peptide-based semi-elemental enteral formula., administered five times daily along with a soft oral diet. By POD 15, the patient successfully achieved the prescribed energy and protein targets, demonstrating good tolerance to oral intake and nutritional supplementation.

From POD 18 onwards, the oral nutritional supplement was changed to a polymeric formula, while the patient progressed to a high-protein normal oral diet. Polymeric ONS was continued to support adequate nutritional intake, and by POD 22, the patient was consistently meeting 100% of the prescribed calorie and protein requirements through oral feeding with supplementation.

Overall, the nutrition management followed a stepwise progression from parenteral nutrition to enteral nutrition, followed by a gradual transition to oral feeding with oral nutritional supplementation. This multidisciplinary nutrition strategy facilitated optimal postoperative recovery, supported wound healing, preserved lean body mass, and enabled achievement of the patient’s nutritional goals prior to discharge.

Follow-up

Following discharge, the patient received regular nutritional counselling and outpatient follow-up. At the 6-month follow-up, the patient demonstrated good adherence to dietary recommendations and was consistently consuming approximately 65 g of protein per day, with satisfactory nutritional status and continued clinical recovery.

Biochemical Monitoring

Serial biochemical investigations were monitored throughout hospitalization.

Table 4. Nutritional Interpretation of Key Biochemical Parameters

Parameter	Observation	Interpretation
Serum albumin	Improved from 3.0 → 4.0	Improvement in serum albumin reflected recovery from the acute inflammatory response together with adequate protein provision.
Total protein	Improved to 6.2 g/dL by discharge	Improvement in protein nutritional status.
Creatinine	Stable at 0.20–0.25 mg/dL	Stable renal function during nutrition therapy.
Urea	Stayed within normal range (50–62 mg/dL)	Balanced protein intake without overload.
Sodium	~129–132 mEq/L	Mild hyponatremia; monitored with IV fluids.
Potassium	Mostly normal; one peak 3.98 mEq/L	Electrolytes were closely monitored throughout hospitalization.
Bilirubin	All within range	Stable liver function.
Calcium phosphorus	All within range	Calcium and phosphorus levels remained within the normal range during treatment.
Hb	Varied slightly 9.3 → 10.2 g/dL	Mild anemia; iron + protein supported.
WBC	10,888 → 20,000+ (transient)	Transient leucocytosis was attributed to postoperative inflammatory response and corticosteroid therapy.
Neutrophils	Increased during therapy (80–90%)	Reflects infection or steroid effect; uncooked foods completely avoided.
Platelets	Normal range throughout (116 → 328)	Indicates no bleeding or thrombocytopenia issues.

Drug–Nutrient Interactions

Table 6. Drug–Nutrient Interactions and Nutritional Management

Drug	Purpose	Nutrition interaction	Nutrition Plan
Tacrolimus (Pan Graf)	Prevents organ rejection (Immunosuppressant)	Absorption is reduced with food (especially high-fat meals). Avoid grapefruit. Can cause hyperkalemia and hypomagnesemia.	Scheduled before food, consistently at 7 AM & 7 PM. Avoid grapefruit because inhibition of CYP3A4 may increase tacrolimus concentrations. Monitored electrolytes and supported magnesium through food/medicine.
Azathioprine (Imuran)	Immunosuppressant (prevents rejection)	May cause GI upset, anemia and low WBC. Can reduce appetite and nutrient absorption.	Administered after food to reduce nausea. Ensured a protein- and iron-rich diet.
Prednisolone	Anti-inflammatory steroid	Causes muscle breakdown, fluid retention and hyperglycemia. Increases calcium loss and appetite.	Planned high-protein, moderate-carbohydrate, low-sodium meals. Included calcium-rich foods and ensured adequate vitamin intake. Monitored weight gain and blood glucose pattern.

Medication-specific nutritional counselling was provided throughout hospitalization.

Outcome

The patient demonstrated successful nutritional progression from parenteral nutrition to full oral intake. Protein intake gradually increased to approximately 90 g/day by discharge.

Key outcomes included:

Successful transition from PN to EN and oral feeding.
Achievement of protein and energy targets.
Improvement in serum protein markers.
Stable renal and hepatic function.
No major nutrition-related complications.
Discharge on normal oral diet with oral nutritional supplementation.

Discussion

Lung transplantation induces significant metabolic stress, increasing nutritional requirements and susceptibility to complications. Early nutrition intervention is associated with improved recovery, reduced infection risk, and preservation of lean body mass. In the case, individualized nutrition support facilitated achievement of protein-energy goals despite multiple challenges, including bronchoscopy procedures, immunosuppressive therapy, and fluctuating oral intake. Continuous monitoring enabled timely adjustments in feeding modality and supplement selection. This case highlights the critical role of clinical dietitians in optimizing outcomes among lung transplant recipients through structured nutritional assessment, intervention, and monitoring.

Conclusion

This case demonstrates that individualized nutrition support, delivered through a structured progression from parenteral to enteral and ultimately oral nutrition, can facilitate recovery following bilateral lung transplantation. Close biochemical monitoring, management of drug–nutrient interactions, and multidisciplinary collaboration were essential in achieving nutritional adequacy and favourable clinical outcomes.

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Nutritional management following bilateral sequential lung transplantation in an 18-year-old female with connective tissue disease–associated interstitial lung disease

Nutritional management following bilateral sequential lung transplantation in an 18-year-old female with connective tissue disease–associated interstitial lung disease

Ishathri PV *, Selvamani N , Sangeetha R

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