1) Introduction
Ms. VR is a 28 year old female was diagnosed with gastrointestinal dysmotility syndrome with intestinal failure in February, 2018. She had intestinal transplant of lower gastric, small intestine, and partial large intestine from 7 year old male Epstein-Barr virus (EBV) at Mount Sinai on 2/3/2018. On day 30th post-transplant she developed EBV infection and post-transplant lymphoproliferative disorder (PTLD) treated with Rituximab and intravenous immunoglobulin (IVIG). She was transferred to Memorial Sloan Kettering Cancer Center (MSKCC) for further management of her PTLD on 3/31/2018 and received six doses of rituximab and EBV cytotoxic T lymphocytes (CTLs) targeting donor graft HLA Cycle 1 (4/13/2018, 4/20/208 & 4/27/2018) and cycle 2 of EBV CLTs using the same donor (5/18/2018, 5/25/18 & 6/1/2018). Her course was complicated by cytomegalovirus (CMV) viremia managed initially with Valganciclovir, but developed resistance and was changed to Marabavir. MRI done on 4/12/19, s/p abnormal mental status that revealed new bilateral ventricle ependymal and right peritrigonal white matter diffusion restriction suspicious for CMV reactivation, ventriculitis/encephalitis. She was started on foscarnet for CNS penetration, IVIG 7 doses, and CMV CLTs two doses given and third one held on 5/28 for worsening mental status. Her course complicated by hemolytic anemia (multiple autoantibodies including severe cold agglutinins) requiring packed red blood cells transfusions, steroids, whole blood exchange, plasmapheresis and rituximab. She was admitted to ICU from 6/3/2019 – 6/14/2019 with respiratory failure, pneumonia, metabolic acidosis, renal insufficiency, pancytopenia, delirium, hypertension, hypernatremia, seizures requiring vasopressors. On 06/10/19 patient was obtunded, STAT CT was performed that showed worsening hydrocephalus. Hypothermia observed, sepsis work-up initiated. Bedside Ommaya performed and 40 cc drained without incident. 6/30/19 seizure activity noted, worsening neuro exam, pupils dilated 8mm and non-reactive. Ommaya clogged and was removed on 7/1/19 at bedside and external ventricular drain (EVD) placed. EVD remained open until 7/10/19 to drainage at 0 with about 7 cc drainage per hour and intracranial pressure ranging 12-18. Patient remained non-verbal, pupils dilated 4-5 mm and withdrawal to pain stimulus. On 7/11/19, EVD replaced, left clamped, not transduced and internal ventricular shunt placed. Since then she has been obtunded, tachypneic, tachycardiac and hypertensive. Neuro exam continues to wax/wane.
2) Pathophysiology
“Post-transplant lymphoproliferative disorders (PTLDs) are potentially fatal disease that results from and an uncontrolled growth of lymphocytes after a solid organ transplant (SOT) or and allogenic hematopoietic stem cell transplant (Sawalha et al., 2018).” Post intestinal transplant procedure, patient received immunosuppression therapy as part of routine therapy to suppress patient’s own immune system so it doesn’t recognize transplanted organ as foreign and start attacking and rejecting. The disadvantage of immunosuppression therapy is EBV rapid reproduction, a type of herpes virus. It is termed as “kissing disease” that is transmitted via kissing, sharing drinking glasses or utensils or coughing. Research studies shows that mostly people get EBV virus when they are young and it remains in their B lymphocytes throughout the life span, but they do not get sick because T lymphocytes keeps it under control by suppressing it from multiplying (Xu et al., 2018). In case of Ms. VR, immune system was suppressed post-transplant, therefore EBV caused B lymphocytes to grow and multiply uncontrollably. These uncontrolled growth can be benign or potentially fatal malignant lymphomas. This fatal malignant lymphoma is PTLD caused by EBV in this patient. PTLD can be caused if the patient already had EBV and it got activated post-transplant, EBV from donor is reactivated or patient gets infected with EBV for the first time after transplant. The chances of patients getting affected with EBV related PTLD post transplantation increases if the donor is not matched well, because stronger immunosuppressant will be required (Morscio & Tousseyn 2016).
3) Assessment
Patient denies alcohol, illicit drug or tobacco use. Mother reports history of constipation and no other medical condition. Father with PMHx of hypertension and hyperlipidemia. Patient was Hispanic. She was unmarried and used to work as Certified Public Accountant in an accounting firm. She has a 26 years old brother, according to whom she loves travelling with friends, reading, and is very spiritual. Her parents were very supportive and caring of her. They want patient to be full code. She met the criteria for constant care.
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On physical assessment it was observed that patient was obtunded, opens eyes spontaneously, no verbal output and unable to follow commands. Both the pupils were very sluggish, right pupil dilated to 5 mm and left pupil dilated to 4 mm. Dysconjugate gaze observed which could be due to seizures. Responded only to peripheral painful stimuli. Since the patient withdraws her hands and legs when pain is applied, it is interpreted as better brain functioning, as it was able to receive the impulse, interpret it correctly, and send out a proper response. Unable to assess gait or coordination due to altered level of consciousness. No ulcers or soreness observed in her mouth, but full of thrust white crust formation were noted, for which oral suctioning was done. Patient was non-verbal so could not gather any subjective data. Read from previous notes that she was responsive and interactive until a month ago when she expressed extreme depression, fear and sadness due to multiple diagnosis. She had confined herself to room due to concerns of acquiring infections. She was tachypneic (RR 30), tachycardiac (HR 145) and hypertensive (BP 140/100). Her feet were turning inwards, thus along with venodynes, another padded boots used that prevented clubbed feet as well as pressure ulcer since she was bedridden. Diminished respiratory sounds at bilateral bases, no cough.
Laboratory and Diagnostic studies
Ms. VR’s white blood cell (WBC) count on 07/18/19 was 12.4 K/mcL, which is elevated. She was diagnosed with PTLD related to EBV. The distinctive nature of PTLD is uncontrolled growth and spread of lymphocytes i.e. WBC. Her low red blood cell count 3.06 M/mcL, low hematocrit (20.5) and low hemoglobin (Hgb) 7.0 g/dL was due to autoimmune hemolytic anemia along with severe cold agglutinins. Goal for Hgb was to maintain more than 8.0 g/dL which was achieved PRBC transfusions using blood warmers. Continues on hydrocortisone for autoimmune hemolytic anemia. Her platelet count was very low as well 84 K/mcL. Thrombocytopenia is also attributed to PTLD, due to which bone marrow production of platelets decreases. Goal was to maintain her platelet level more than 100 K/mcL, for which platelet transfusions were ordered. PT (13.0 SEC) & INR (1.03) were within normal range. Venous pH was 7.27 (acidic), pCO2 39.0 mm Hg (normal), bicarb 17.9 (acidic), pO2 41.0 mm Hg (normal), O2 stat 72.2 (normal). Patient was in metabolic acidic state as result of which to compensate her respiratory system became tachypneic which was evident by her RR 30 bpm. Elevated BUN 23 mg/dL was attributed to renal insufficiency. Plasma sodium 147 mEq/L (hypernatremia) was from hypertonic earlier in the hospitalizations and lack of free water intake and plasma potassium 5.1 (hyperkalemia) could be in the setting of decreased glomerular filtration rate, tacrolimus drip and/or hemolytic anemia. Plasma chloride 107 (normal), creatinine 1.2 (due to AKI), plasma glucose 95, and plasma calcium 8.5 (normal).
4) Medications/Fluids and Blood products
| Medications/Fluids/Dosage | Frequency/Route | Rationale |
| Amphotericin/Nystatin – 1ml | Q6h/IVPB | It is an antifungals used to treat progressive and potentially fatal fungal infection |
| D5W Bolus – 500ml | Q24h/IV | To provide hydration and calories and prevent hypoglycemia |
| Famotidine INJ – 20mg | Q24h/IVPB | It is a peptic guard to manage GI symptoms since patient is NPO, on TPN |
| Fentanyl (Duragesic) 25mcg/hr | Q72h | High risk opioid analgesics used for continuous opioid analgesics therapy used for an extended time at a dose |
| Foscarnet Sodium – 2000mg | Q24h/IVPB | To treat CMV viremia along with ganciclovir |
| Ganciclovir – 140mg | Q24h/IVPB | To treat CMV viremia along with foscarnet sodium |
| Hydrocortisone – 10mg | Q24h/IVPB | Anti-inflammatory drug used for allergic, neoplastic, autoimmune disorder & septic shock |
| Labetalol – 10mg | Q6h/IV push | Beta blocker used for management of hypertension |
| Levetiracetam – 500mg | Q12h/IVPB | Anticonvulsants used to prevent seizures |
| Micafungin Sodium – 100mg | Q24h/IVPB | Antifungals used to treat candidiasis |
| Phenytoin – 100mg | Q8h/IVPB | Anticonvulsants used to treat/prevent tonic-clonic seizures and complex partial seizures |
| Piperacillin/Tazobactam – 4.5gm | Q12h/IVPB | Anti-infective used to treat/prevent appendicitis, peritonitis, skin &skin infections |
| Total Parenteral Nutrition (TPN) – 1200ml with specific amount of each electrolytes K>4, Mg>2, Phos>2.5, iCa>4.5 | Continuous – new bag once Q24h/IV | TPN used to supply all daily nutritional requirements since the patient was NPO |
| Tacrolimus IVCl Adult | Continuous | Prograf used as prophylaxis to prevent organ rejection since she had undergone intestinal transplant |
| Thiamine (Vitamin B-1) IV – 100mg | Q24h/IVPB | Vitamins used to supply dietary supplements due to her GI disease |
| Sodium Chloride 2% | Continuous | Mineral and electrolyte replacements/supplements as well as hydration |
| PRBC via blood warmer – ordered depending on hemoglobin level | Ordered 1 PRBC at a time based on hemoglobin level | Low hemoglobin >8. Blood warmer used because patient diagnosed with hemolytic anemia (multiple autoantibodies including severe cold agglutinins) |
| Lovenox | Q24h/subq | Prophylactic to prevent blood clots as she is bedridden. |
| Fat Emulsion 20% – 100ml | 2 times a week/IVPB | To provide her with source of calories and fatty acids. Used special filter with the IV line |
b. Patient was not currently on any radiation or chemotherapy and doesn’t have any wounds.
- Nursing care plan
| Nursing Diagnosis | Expected Outcomes | Interventions | Rationales |
| Risk for catheter associated urinary tract infection (CAUTI) related to long-term indwelling catheter due to multiple diagnosis and altered mental status as evidenced by her neurological & muscular assessments. |
1) To reduce the risk of achieving CAUTI by looking for early signs and symptoms and treating them before it gets infected.
2) To maintain strict intake and out to acquire normal urinary output and monitor for signs for retention, oliguria or dysuria. |
1) Clean the catheter and perineal area thoroughly with special wipes at the start and end of shift and PRN if soiled or any other reasons (Durant, 2017).
2) Ensure proper hand hygiene techniques and PPE before and after touching catheter (Durant, 2017). 3) Maintain adequate fluid intake as per tolerated depending on other diagnoses (Durant, 2017). |
1) Proper cleaning of catheter and perineal area helps in reducing the risk of any infection or contamination.
2) Proper hand hygiene techniques minimized the cross infection risks. 3) Adequate fluid intake improves renal blood flow. |
| Risk for altered family processes/functioning related to anticipated loss of family member as evidenced by long-term illness and multiple diagnosis. |
1) Parents will express their concerns and feelings freely
2) Encourage and allow parents to participate in patient care as appropriate for the situation. |
1) Talk to parents in a caring, warm and respectful manner. Give them all the required information (verbal & written), and be honest (Landier et al., 2016).
2) Encourage appropriate anger expressions & acknowledge difficulties of the situation (possibility of death) (Landier et al., 2016). 3) Refer parents to support groups & therapy (Landier et al., 2016). |
1) Promotes the sense of competence in ability to handle current situation and provides empathy.
2) Appropriate expression outflow and communication enables acceptance of reality & appropriate grieving process. 3) Additional help may assist in resolving issues of disorganization related to potentially terminal disease. |
| Deficient knowledge related to verbalizing inaccurate information as evidenced by mother stating inappropriate reasoning for patient’s low hemoglobin. |
1) Patient’s mother will show motivation to learn.
2) She will be able to verbalize the understanding of patient’s disease state and reason for low hemoglobin. |
1) Assess mother’s motivation and readiness to learn (Landier et al., 2016).
2) Acknowledging cultural/racial/ethnic influences on health teaching and knowledge (Landier et al., 2016). 3) Provide thorough, clear & understandable explanations & demonstration (Landier et al., 2016). |
1) Learning new information requires energy and right state of mind. Also it’s her right to refuse educational service.
2) Being aware of cultural/racial/ethnicity influencing will enhance communication, establish rapport & assist in developing interventions. 3) Clear explanations will allows mother to understand and ask question when she has basic information on what to expect. |
Patient had indwelling urinary catheter because of altered mental status, AKI, prevention of urinary retention since she is bedridden. Ensuring proper care and hygiene in maintaining catheter was a priority to avoid CAUTI. She was on many different fluids and drugs that cane affect the urine output. Her urine output was strictly measured every hour and compared with the input and assessed for color and concentration. Drainage bag was positioned well below the level of the bladder and ensured there is no urine backflow, it is not kinked or twisted and was secured. Catheter and was cleaned at every start and end of the shift and whenever required with special cleaning wipes to protect it from any infection.
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Within a span of one and a half years patient’s condition worsened from being absolutely healthy to being obtunded and only response to painful stimuli to the extremities due to multiple diagnosis including CMV & EBV-PTLD. Parents were feeling powerless, experiencing lack of control over situation and grief of anticipated loss (possible death) of their 28 year old daughter. Parents were encouraged to vent their feelings and how are they coping to accept the current situation. Parents were encouraged to participate in their daughter’s care wherever appropriate to allow them to have some control over the situation. Patient’s care goals, care plans and interventions were honestly and empathetically discussed with parents.
Patient’s mother said to nurse “My daughter’s hemoglobin is low because you take out so much blood all day in the name of testing.” Knowledge plays crucial and influential role in making critical decisions related to patient care. It is an essential part of nursing care to determine with the patients and family what, when and how much to teach related to health concerns. Their misconception about reasons for low hemoglobin can be observed and noted as baseline of their knowledge on the subject. Information should be provided as required at their level of understanding and learning style to correct their misunderstanding. It is also crucial to be sensitive about their cultural influences on their knowledge. Interventions should be appropriate based on their cultural background. They should be made comfortable in a calm and peaceful environment. They should also be treated with respect and information should not be given in a condescending manner. Any doubts, questions and concerns should be encouraged and addressed appropriately. Using visual aids, audiotapes and internet websites can also be useful, as people have different learning techniques.
5) Summary
I believe by collecting all the qualitative and quantitative data that was collected in order to write this case study I gained more in-depth insight into the patient’s diagnosis, medical history and treatment relevance. It helped me in simplifying such a complex case. I took care of this patient for three shifts with my preceptor but to be very honest I understand this case much better after writing case study as compared to before, especially pathophysiology of the her diseases. I had a great learning experience skills wise in taking care of this patient and the knowledge I gained by researching every aspect of this study gives reasoning to those skills. It contributed towards developing my critical care thinking process. For instance, her tachypneic state was assumed to be possibly because of pain and she was administered with IV Tylenol, but now that I had chance to review her lab values I could critically think that her tachypneic state may not be linked with pain but instead she was in respiratory alkalosis state because her body was trying to compensate for her metabolic acidosis state. Overall the case study was designed to identify each nursing process steps (assess, diagnose, plan, intervention and evaluate) a patient-centered framework in which we use critical thinking to achieve short-term and long-term nurse/patient goals and solve problems.
References
- Durant, D.J. (2017). Nurse-driven protocols and prevention of catheter-associated urinary tract infections: A systemic review. American Journal of Infection Control, 45(12), 1331-1341. doi:10.1016/j.ajic.2017.07.020
- Landier, W., Ahern, J., Barakat, L. P., Bhatia, S., Bingen, K. M., Bondurant, P. G.,… Hockenberry, M. (2016). Patient/Family education for newly diagnosed pediatric oncology patients. Journal of Pediatric Oncology Nursing: Official Journal of the Association of Pediatric Oncology Nurses, 33(6), 422–431. doi:10.1177/1043454216655983
- Morscio, J., & Tousseyn, T. (2016). Recent insights in the pathogenesis of post-transplantation lymphoproliferative disorders. World journal of transplantation, 6(3), 505–516. doi:10.5500/wjt.v6.i3.505
- Sawalha, Y., McMichael, J., Rybicki, L., Sussman, T.A., Mejia Garcia, A. V., Dean, R. M., Pohlman, B., Hill, B. T., & Jagadeesh, D. (2018). Risk of post-transplant lymphoproliferative disorders (PTLD) in solid organ transplant patients with EBV viremia. Blood, 132 (Suppl1), 4202. doi: https://doi.org/10.1182/blood-2018-99-114686
- Xu, H., Forsythe, A., Barley, A., Rashid, N., & Watson, C. (2018). A systematic literature review of real-world evidence in post-transplant lymphoproliferative disorder. Blood, 132(1), 5839. doi:https://doi.org/10.1182/blood-2018-99-113583
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