S. Booma

Assistant Nursing Superintendent, Kauvery Hospital, Trichy-Cantonment, India

 

Nursing management of a multi-organ transplant (Liver and Kidney) on an adolescent

Abstract

Primary hyperoxaluria type 1 (PH1) is an uncommon and progressive genetic disease with debilitating and life-threatening clinical manifestations due to increased hepatic oxalate production.

The metabolic defect in PH1 results from a deficiency of the liver-specific peroxisomal enzyme alanine-glyoxylate aminotransferase (AGT), which converts the oxalate precursor glyoxylate to glycine. With absent or deficient AGT activity, glyoxylate is oxidized to oxalate, leading to increased plasma oxalate levels. Hepatically produced oxalate, excreted largely by the kidneys, is the toxic mediator of end-organ damage in PH1.

Patients with PH1 commonly present in childhood with kidney stones, nephrocalcinosis, and end-stage kidney disease, If plasma oxalate levels rises sufficiently systemic oxalosis results, with deposition of oxalate in tissues such as bone, retina, heart, and skin.

The condition may remain undiagnosed or incorrectly diagnosed for years, 43% of patients diagnosed with PH1 already had end-stage kidney disease at the time of diagnosis.

Keywords: Primary hyperoxaluria type 1 (PH1), Alanine-glyoxylate aminotransferase (AGT), nephrocalcinosis, End-stage kidney disease

Current strategies and their limitations

Conservative measures are limited and should be applied as soon as the disease is diagnosed. These measures include:

(1) Massive fluid intake (>2-3 L/m2 BSA per day or through the tube or gastrostomy feeding in infants).

(2) Calcium oxalate crystallization inhibitors (oral potassium citrate and magnesium citrate or sodium citrate in case of impaired renal function to maintain urine pH above 7).

(3) Pyridoxine (starting dose of 5 mg/kg per day that may be progressively increased up to 20 mg/kg per day). For pyridoxine (vitamin B6), an excellent (inexpensive) chaperone molecule acting on protein stability, catalytic activity, and peroxisomal import of AGT.

(4) When estimated GFR (eGFR) declines under 30 mL/min per 1.73 m2 BSA, dual liver-kidney transplantation is currently proposed. Because oxalate retention increases at this stage of renal dysfunction, the evolution to ESRD nearly inevitable.

(5) In chronic kidney disease (CKD) stage 4, early combined liver-kidney transplantation is preferred.

(6) In CKD stage 5 or chronic dialysis, when systemic oxalosis is more intense, sequential transplantation can be another option: first the liver followed by hemodialysis to decrease systemic oxalate storage and then the kidney. However, no clear benefit from this strategy has been reported, and choosing between combined and sequential liver-kidney transplantation remains controversial.

Presenting history

A 17-year-old adolescent, known to have primary hyperoxaluria, with CKD on regular follow up. He was evaluated for liver + kidney transplant and was found to be fit for the procedure. He was admitted and underwent the transplant surgery on 04.05.2022.

Past history

Known to have CKD – S/P Renal transplant done 3 years ago.

On examination

Conscious, oriented, afebrile (Temp, 97.4°F); PR, 86/min; BP, 120/80 mmHg; SpO2, 98% in room air; CVS, S1 S2 (+); RS, BAE (+); P/A, Soft; CNS, NFND.

Procedure done on 04.05.2022, Combined liver-kidney transplantation (deceased donor)

Patient was evaluated and taken up for the procedure on 04.05.2022. Surgery proceeded for 16 hrs. Patient was later shifted to transplant ICU.

POD 1: He was stable overnight and was extubated. Doppler study of liver and kidney showed normal vascular flow. Initially he had no urine output. Patient was started on renal replacement therapy (RRT) in view of anuria/to prevent systemic oxalosis. His other vitals were stable during the entire post-operative period. RRT was continued intermittently based on his clinical condition. Urine began draining, which then gradually improved as he recovered from acute tubular necrosis.

Urine output the day before discharge was 3.17 L. Serial follow up with hepatic and renal doppler showed normal vascular pattern but showed perinephric collection (lymphocele). The collection, on follow up, showed decrease in size. Patient was managed with appropriate antibiotics, intravenous fluid supplementation, analgesics, proton pump inhibitors and other supportive measures.

Serial surveillance cultures were negative. Procalcitonin showed decreasing trend. He was started on immunosuppression with Tacrolimus that was tailored as per institution protocol. Last Tacrolimus trough level was 11.5 ng/ml on 16.05.2022. Additionally, Mycophenolate Mofetil (MMF) and steroids were given.

Renal drain was removed on 14.05.2022. Chest spirometry, physiotherapy and mobilization were given effectively during hospital stay.

Patient was discharged with drain tube in situ (right – cut colo applied). Graft function was stable throughout the post-operative period. He improved symptomatically, and was clinically stable. Urine output improved.

Nursing challenges in post-operative period.

Respiratory concerns

Patient was extubated the next day.

However, patient was troubled by the thought that he had undergone a renal transplant earlier. However psychological support, encouragement and music therapy brought him out from his problem and he started co-operating.

When he developed respiratory distress, he was supported with High Flow Nasal Cannula (HFNC); he was monitored carefully and weaned off early.

Fluids and electrolytes balance

To maintain his electrolytes normal, output and intake were carefully monitored and adjusted accordingly. Glucose, potassium and serum proteins levels were monitored closely. As blood sugar was elevated insulin infusion was started, and Potassium level was adjusted carefully.

Medication management

Providing immunosuppressant medicine was a challenge as it needed to be approved by the multidisciplinary team, but was successfully managed by good team work. Usage of Albumin required close monitoring to prevent fluid over load.

Hematology concerns

Proper administration of blood and blood products, without volume overload, to maintain Hb and coagulation was also a challenge but was well-managed

Acute rejection

Monitoring of, and on time evaluation for, possible acute rejection was in place.

This involved watching out for temperature spikes, elevation of SGOT and SGPT, and evaluation by doppler. Proper administration of pulse steroids and adjustment of immunosuppressants were our responsibilities

Maintaining CVC/HD/PICCO (Pulse index Continuous Cardiac Output):

Adherence to HICC protocol, proper handling of tubes and drains, judicious administration of high alert medications, careful withdrawing blood for investigations and early removal of invasive catheters without infection called for the greatest attention from the nursing staffs.

Nephrotoxicity

With potentially nephrotoxic drugs, and immunosuppressants (Cap. Prograf/Tab. Cellcept), nephrotoxicity was avoided by dose adjustments.

Vascular occlusion

Early diagnosis and proper administration of anti-coagulant and anti-platelet aggregators to prevent vascular occlusion were successfully managed by the expert team

Early ambulation

Ambulating the patient, who has received multi organ transplant, and was on tubes and drains required much support and was managed very well with team effort.

Psychological and emotional support

Managing a patient who had multiorgan transplant by providing emotional and physical support, with very good outcomes, was our greatest achievement.

References

[1] Garrelfs SF, Frishberg Y, Hulton SA, et al. Lumasiran, an RNAi Therapeutic for Primary Hyperoxaluria Type 1. N Engl J Med. 2021 Apr 1;384(13):1216-26.

 

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