NURS 415: Integrated Practicum III
Pediatric Case Study: Methylmalonic acidemia
University of Illinois at Chicago College of
... [Show More] Nursing
Recent Medical History and Course of Hospitalization
The patient was a three-year-old female born at 38 weeks of gestation with poor weight gain who presented to the University of Illinois Hospital (UIH) in a metabolic acidosis crisis secondary to poor PO (by mouth) intake and upper respiratory infection, leading to catabolic state and breakdown of native proteins due to patient known history of methylmalonic acidemia. Patient was in her baseline state of health prior to emergency department visit, though with poor PO intake over several days. Father described the patient’s presentation as similar to previous hospital admissions for metabolic acidosis most recently on October 6 and October 12 at Advocate Condell Hospital. Her most recent admission was at OSH (outside hospital) due to emesis and dehydration.
She initially presented to Advocate Condell Emergency Department after two episodes of NBNB and persistent tachypnea. She then was transferred to pediatric intensive care unit fat UIH from Advocate Condell emergency department where she patient recently presented with emesis, decrease PO intake, tachypnea and tachycardia, and severe metabolic acidosis state. Labs were obtained and notable for a pH level of 7.02, pCO2 of 19, bicarbonate level of 5, and an ammonia level of 117. She received one dose of bicarbonate 14mEq, two 20 mL/kg normal saline boluses and was started on D10 ½ NS at 1.5 maintenance at OSH prior to transfer.
Upon transfer to UIC respiratory rate was in the 140s with Kussmaul breathing. After transfer to UIH a venous blood gas was obtained that showed a pH level of 7.08, pCO2 of 14, and ammonia level of 311. The case was discussed with nephrology and urgent dialysis was not necessary at this time. She received a total of two additional doses of 14mEq sodium bicarbonate. Fluids were changed from to D10 0.45% sodium chloride and 0.45% sodium acetate with 20mEq potassium acetate at 1.5 maintenance. Ammonia down trended to 218 in three hours after initial ammonia level drawn at UIH followed by 147 an additional six hours later.
Peripherally inserted central catheter line was placed by radiology and patient was started on total parenteral nutrition (TPN) with low protein content. Acidosis was resolved throughout hospital day one and mental status returned to baseline. She was continued on TPN and IV (intravenous) fluids through hospital day three. She was offered formula PO starting on hospital day two but was not interested in eating and had some non-bloody/non-bilious (NBNB) emesis so decision was made to not place nasogastric (NG) tube at this time. On October 18, patient was put on contact/droplet percussion due to positive results of three viruses (parainfluenza, entero/rhino and adenovirus). Patient lost her PICC access in the early morning of October 19. TPN was stopped due to lack of central access. She continued with IV fluids, intralipids, NG was placed for formula.
During day four on October 21st, the student nurse was able to assess the NG tube and IV site with no breakage or drainage leakage noted. Patient pain level was also noted using a FLACC scale that revealed a pain level of zero. At 0730 patient vital signs were assess by the student nurse and where within normal limits (Tempture:37.0oC, Pulse:140, Respiration: 31, Blood Pressure;109/74, Oxygen saturation: 99%). Breakfast arrived around 0740 but patient was not interested in eating. At 0800 patient was given her dose (10mL) of lactulose via her NG tube. IV solution was continuously running with potassium chloride at 74mL/hr and intralipid fat emulsion at 3mL/hr via NG tube. A full physical assessment was done by the student nurse around 0900, that revealed some moderate distress, but alert and awake appearance. No discharge from eyes were observed. NG tube secured and placed. Mucus membrane moist and pink. Neck was supple with no noses or masses present. Chest/Respiratory symmetric. Lungs sounds clear and equal in all lobes bilaterally. No crackles, wheezing or rales. Abdomen soft and full with no masses or organomegaly. Non tender to light and deep palpation. Extremities normal, well perfused and warm, moves all extremities equally, peripheral IV-dressing clean, dry, and intact. Cardiovascular regular, rate, and rhythm, normal S1 and S2. No murmur, pulse 2+ x4. Skin warm and dry, cap refill <2sec, and no jaundice.
After assessment, the physical therapist (PT) arrived and was able to access the patient’s mobility. Patient was able to walk with no assistance more than 250 feet move and up and down the stairs. Upon further assessment, the student nurse noted some developmental delays for her age. Patient was not able to form small coherent and fluent sentences, clearly not in the expectant developmental level of Piaget preoperational thinking. Patient was also experiencing some separation anxiety from mother. Similarly, patient was not in full control of her bodily function and was very dependent on grooming, dressing and feeding, clearly in the Erickson stage of Autonomy vs. Shame and doubt. Later Patient was rounded on by the nutritionist and pediatric team around 1030, who iterated the need to offered foods to assess her ability to hold down foods Throughout the day patient was offered PO foods and was able to take in some crackers and some formula but had one episode of emesis. During assessment at 1145, no remarkable findings were observed during the student nurse assessment beyond the previously noted. Patient care continued with IV fluids and NG feedings and careful monitoring for episodes of emesis.
During day five, ammonia levels rose to a maximum of 257, which decreased to a goal <100 as feeds were adjusted to meet necessary intake without TPN. She was also placed on schedule lactulose due to elevated ammonia levels. There were multiple electrolyte abnormalities as well during the first three days of hospitalization, which resolved as electrolyte were repleted. Patient labs stabilized as she received her full NG feeds of genetic formula.
She was started on IV levocarnitine every three hours initially, which was transitioned to NG levocarnitine every twelve as medication for home. Formula feeds were initially continuous, then transitioned to bolus feeds with increasing volume. IV fluid was titrated down as NG feeds increased. Lactulose was discontinued as ammonia levels normalized. After discussion with the parents, the decision was made for the patient to be discharge with an NG tube October 25, 2019. The goal feeding was 900mL/day of formula. Patient was able to tolerate feedings prior to discharge.
Past Medical and Surgical History
The patient was diagnosed at birth with methylmalonic acid enzyme deficiency. She has been known to have poor weight gain and a poor PO feeder. At home, she is treated with a low protein special formula diet. According to father, it is not the first time she presents with metabolic acidosis. Father stated the most recent admission was on October 6 and October 12 at Advocate Condell hospital. Patient also has a history of developmental delay and is receiving El services for ST (speech-language) and OT (occupational) therapy. Patient has no remarkable family or surgical history and lives at home with parents. Currently, patient is admitted for metabolic crisis with moderate dehydration and hyperammonemia.
Medical Diagnosis
The patient had no new medical diagnosis than the one related to her previous and current hospital admission for methylmalonic acidemia and electrolyte imbalance. All previous and current diagnoses were interrelated and caused by poor PO intake, upper respiratory infection, dehydration, excessive vomiting, and high ammonia levels all of which are related to her genetic disorder of methylmalonic acidemia.
Home Medication
The patient had no home medications but had a protein restricted diet that was supplemented with a specialized medical formula (propimex-1/prophree) that was setup by the genetic team and given PO by parent who were educated in how to feed the infant.
Physical Assessment at 0730 on day four of admission
Vital signs and Baseline Data
Temperate (axillary): 37.00C
Heart rate: 140
Respiration rate: 31
Blood pressure (left leg, sitting): 111/74
O2 saturation: 99%, on room air
Pain: (0) access via FLACC scale
Body mass index: 17.23, 89th percentile
Allergies: No known allergies
Height: 90.5cm, 11th percentile
Weight: 13.9 kg, 50th percentile
Reference growth chart on next page
General assessment
The patient was a three-year-old female with a past medical history of methylmalonic acidemia who was assessed on the fourth day of admission. During assessment patient was alert, awake, and cooperative with no involuntary movement. Dress and hygiene appropriate to setting. Affect and verbal response appropriate. Able to answer questions appropriately with examiner. Thought process appropriate for age. Appears calm and in no acute distress.
Spine and back
The patient had a normal spinal profile, with no tenderness, deformity or curvature present. Similarly, no scoliosis was present.
Extremities
The patient extremities appeared pink in skin color with smooth appearance and with no redness, cyanosis or lesions present. No varicosities or thickening of nails in both upper and lower extremities were found. Patients lower and upper extremities were equal in size bilaterally without swelling or atrophy. Capillary refill were less than two seconds in both upper and lower extremities. All pulses (brachial and dorsalis pedis) present, 2+ and equal bilaterally. Similarly, extremities were warm to touch, and no lymphadenopathy were present.
Skin and Nails
The patients’ skin was pink, warm, dry, and intact, outside the presence of a peripheral IV in the lower right forearm, which had no presence of redness, swelling or drainages. Similarly, no presences of scars, rashes or lesions were observed. Skin temperature was warm and equal bilaterally. No excessive perspiration or swelling present. Texture was smooth and firm. No presence of clubbing or deformities observed in nail beds. Nails were pink with a prompt capillary refill of less than two seconds
Hair and Head
The patients hair was curly and dark brown with an even distribution and thick texture. Head was normocephalic, with no lesions, tenderness, flakes, dandruff, or scaling of the scalp.
Face
The patients cranial nerve V (trigeminal) were intact as evidence by sensitive to light touch and equal in strength, and with no pain in masseter muscle. Similarly, patients’ cranial nerve VII (facial) was intact as evidence by symmetric facial movements. There was no presence of tenderness or pain in the frontal and maxillary sinuses and no locking, popping, or pain in the temporomandibular joint.
Eyes
The patients’ cranial nerve II (optic) were intact as evidence by patient’s ability to name letters from a book. Similarly, patients’ cranial nerve III (oculomotor) was intact as evidence by patient’s ability to constrict eyes upon light, eyes being well rounded and equal, and accommodation (PERLA). Patient cranial nerves III (oculomotor), IV (trochlear), and VI (abducens) were intact as evidence by smooth extraocular movements. Patients conjunctiva and sclera were clear and white. Likewise, patient’s cornea were smooth and convex.
Nose
The patient nose was symmetric with clear patent nares, outside from the nasogastric tube in place in the right nostril. Patient mucosa was pink and with no discharges or obstructions present. Similarly, patient had no septal deviation or perforation present. Likewise, patient nose sinuses were non-tender.
Mouth
The patient lips were pink and moist, and with no lesions. Patient had a good dentation with no caries present. Similarly, patient mucosa and gingivae were pink and with no presences of lesions or masses. Patient tongue was pink, smooth, and even. The patients’ cranial nerve IX (glossopharyngeal) and X (vagus) were intact as evidence by the rise in the midline on phonation and movement of palate. Likewise, patients’ cranial nerve XII (hypoglossal) was intact as evidence by patient symmetrical movement of the tongue.
Neck
The patient’s trachea was at midline. The patient’s cranial nerve XI (accessory) was intact as evidence by patient equal strength in movement of head. Patient neck was symmetric with no masses or tenderness present. Similarly, patient exhibit no lymphadenopathy in all lymph nodes (anterior/posterior neck, head, and supraclavicular region). Patient also had full range of motion of the cervical spine with no pain present.
Ears
The patients’ ear was equal in size bilaterally with no swelling, lesions, or masses present. Patient skin was consistent with facial skin color. Patient cranial nerve VII (vestibulocochlear) was intact as evidence by patient able to hear soft spoken sounds bilaterally.
Heart
The patient precordium exhibit no pulsation, heaves, lifts, or retractions. Patient had a regular rate and rhythm pulse. Patient apical impulse was present and strong, with no thrills on auscultation. Patient S1 and S2 was crisp, not diminished or accentuated and no presence of S3, S4, or extra sounds. Patients carotids were 2+, upstroke brisk and equal bilaterally with no presences of bruits. Similarly, good capirally refills were observed at less than two seconds. Likewise, patients’ peripheral pulses were palpable and equal bilaterally with no pedal edema present.
Thorax and lungs
The patients anterior and posterior thorax appeared symmetric with no tenderness, lesions, or lumps present. Patient was warm to touch and moist. Similarly, patient lung sounds resonant to percussion bilaterally. Likewise, patient breath sounds were clear over lung fields and equal bilaterally. No wheezing, crackles, or rhonchi present.
Musculoskeletal
The patient had no limitation to motion. Patient muscle strength (+4) intact with full range of motion against resistance and equal strength bilaterally in both upper and lower extremities. No clubbing, cyanosis or edema present in either upper or lower extremities. Patient also appeared stable and in no pain upon walking.
Genitourinary and Gastrointestinal
The patient abdomen was symmetric with no apparent lumps or lesions. Patient umbilicus was at midline and inverted, with no sighs of discoloration, inflammation, or hernia. Similarly, skin appeared smooth and even with no apparent scars, jaundice, or moles. Patient bowel sounds were present in all four quadrants. Likewise, patient abdomen appeared soft, non-tender, non-distended, with no guarding, masses, or rigidity present. Patient last bowel movement was on 19th two days after admission. Patient had little to no appetite and was connect via an NG tube for total parental nutrition. Patient had one wet diaper with no stool present and skin appeared pink and intact. No edema or rashes were observed.
Clinical Manifestations
The patient initially presented to Advocate Condell Emergency Department due to emesis, decrease PO intake, tachypnea, and tachycardia. She was then admitted to PICU from Advocate Condell Emergency due to metabolic crisis. Her most recent presentation included metabolic acidosis, moderate dehydration, and hyperammonemia all of which are clinical manifestations of her diagnosis of methylmalonic acidemia (Sheppard, Ficicioglu, Hahn, & TePas, 2019). Upon transferred to UIC PICU, labs at OSH were notable for low levels of pH at 7.02, low levels of pCO2 at19mmHg, low levels of pO2 at 69mmHg, high levels of ammonia at 117, low levels of bicarbonate at 5, increase anion gap at 30, high levels of bun/creatine ratio of 47, high levels of white blood cell count at 19.1mm3, high platelet count at 667, and presences of ketones in the urine at 80 ("Selected Normal Pediatric Laboratory Values"). All abnormal laboratory values were correlated to her diagnosis of methylmalonic acidemia (Keyfi, F., Talebi, S., & Varasteh, A. R., 2016)., and the elevate white blood cell count could be related to her upper respiratory infection.
Significant labs and test results
Reference values (normal and abnormal) and clinical implications for each test was provided by Fischbach & Dunning (2015) and Wilson (2008). These lab tests included blood gases, electrolytes, metabolic panel, and liver function. All tests were part of a routine laboratory evaluation in patients with positive newborn or clinal features suggestive of methylmalonic acidemia (Sheppard, Ficicioglu, Hahn, & TePas, 2019). Labs obtained at UIH for October 20th, 2019.
Lab tests Normal Values Patient values Rationale
Blood (arterial blood) pH 7.32–7.42 in pediatrics 7.46 Generally, the pH is decreased in acidemia (acidosis) because of increased build of acids or an excessive loss of bicarbonate, or by a buildup of carbon dioxide in the blood that results from poor lung function or depressed breathing (respiratory acidosis), and pH is increased in alkalemia (alkalosis) because of a loss of acids, fever, hypovolemia, severe ventilation-perfusion abnormalities or ingestion of excess base. In my patients’ case, the pH was high because of the two doses of bicarbonate given to her due to her initial low pH and low bicarbonate level.
pCo2 (arterial blood): 30–40 mmHg (4.0–5.3 kPa) in pediatrics 33 mmHg Cerner flagged this result as low, though Wilson (2015) indicated this result as normal for children. In general, an increase in pCO2 (hypercapnia) usually is associated with hypoventilation (CO2 retention), COPD, chronic bronchitis, nerve or muscular disorders, hypothermia, or obesity; a decrease is associated with hyperventilation (blowing off CO2). In the case of my patient, the pCO2 was low because she was having kussmaul breathing, which is a common feature of acidosis, so in an attempt to remove the buildup of CO2 (acid) she began to hyperventilate causing the slight drop in pCO2.
Magnesium Children: 1.7–2.1 mg/dL (0.70–0.86 mmol/L) 0.57 mmol/L In general, a decrease in magnesium levels can be caused by chronic diarrhea, malnutrition, malabsorption syndrome, and celiac disease. An increase in magnesium levels are usually associated with renal failure, hypothyroidism, oliguria, or dehydration. In my patient case, her low magnesium levels where due to her poor PO intake and excessive vomiting.
Glucose 60–100 mg/dL or 3.3–5.6 mmol/L (fasting) in children 2-18 years old. 135 mg/dL Generally, a high glucose level is associated with diabetes, malnutrition, prolonged inactivity, renal failure, and liver disease. In contrast, a low glucose is usually caused by malabsorption, liver damage, and enzyme-deficiency deficiency. In the case of my patient, her high level of glucose was associated with her condition of methylmalonic acidemia and poor nutrition intake.
Ammonia (NH3) 40–80 mcg/dL (28–57 µmol/L SI units) in children 257 mcg/dL An increase in ammonia levels is usually caused by enzyme deficiencies, liver disease, hyperalimentation, and renal disease. While a decrease in ammonia is associated with malignant hypertension. In the case of my patient, her elevated ammonia levels were associated with her condition of methylmalonic acidemia, an enzyme deficiency disorder.
Diagnostic tests and procedures
The patient had a computed tomography (CT) scan of her abdomen and pelvis area following up her recent abdominal x-ray (radiography) during her previous hospitalization stay at UIH about a year ago for a possible ileus. Considering that x-rays use light or radio waves as radiation for scanning effected areas such as fractures, bones dislocations, lung infections, and tumors. A computed tomography uses a combine x-ray with computer technology to provide a much more cross-sectional detailed structure of the effected body part such as organs, tissue, and tumors ("X-rays, CT Scans and MRIs", 1995). For this reason, a computed tomography was the preferred choice to allow for a more precise diagnosis. Findings in the computed tomography were noted for a small left pleural effusion and trace right pleural effusion with atelectasis in the dependent portion of the right and left lower lobes of the lungs. Similarly, bowels were noted for nonspecific mural thickening of the colon, predominately at the splenic flexure and descending colon. The colon loops of bowel were also prominent. These finding in combination with her presented symptoms which were bilious emesis, fever, and kussmul breathing led to a definitive diagnosis of ileus (paralytic). Given that her primary diagnosis is methylmalonic acidemia this can explain how she developed ileus, which is a disruption in the normal peristalsis of the intestine (Ansari, 2018). Since methylmalonic acidemia can cause of buildup of acid and ammonia and can be triggered by an infection this can explain why she developed and an obstruction and a fever ("Methylmalonic Acidemia - Acute Illness Protocol"). Because of her small stature the buildup of fluids could have accumulated quickly and surround her lungs, which can explain why she was having kussmul breathing.
The most recent procedure required for the patient was a peripheral inserted central catheter (PICC) line placement. This was required because the patient had high levels of ammonia with an altered mental status that needed immediate administration of IV fluids and medications. She also required blood draws every twelve hours to monitor her ammonia and electrolyte levels. Essentially a PICC line is a thin soft tube that is inserted into a large vein in either the arm, the leg, or the neck (Children's Hospital, 2014). Typically, a PICC line is usually placed by using an ultrasound to evaluate the vein and then placed by using an x-ray by an interventional radiologist or a nurse. The procedure takes about 30-60 minutes, generally painless, that may or may not require sedation. In the case of my patient she required anesthesia. The procedure is considered low risk, but potential complications include bleeding, infection, and blood clot. Normally, if properly care a PICC line can stay for weeks or months. Overall, my patient had no complications and the PICC line was successfully placed.
Treatment
The primary goals for my patients given her status is to correct her biochemical abnormalities (hyperammonemia, metabolic acidosis), provide hydration with a high caloric supplementation, and eliminate toxic metabolites ("Methylmalonic Acidemia - Acute Illness Protocol"). The standard management for methylmalonic acidemia (MMA) includes correcting biochemical abnormalities and essentially resolving acidemia symptoms. The nursing implications for correcting biochemical abnormalities include ensuring the patient is receiving the right proper nutrition that is by either PO or total parenteral nutrition, ensuring patient is receiving correct medication based on her weight and status, constant monitoring labs for any up trending/down trending abnormalities and ensuring the physician is inform , specially ammonia levels, and constant neuro to prevent seizure and further complications. In my patient case, she had a PICC line placed and was started on TPN with a low protein content. Additionally, she was given two boluses of 14mEq of bicarbonate upon transferred to UIH PICU to stabilize her pH levels (Hockenberry, Olshansky, & Perry, 2018). Throughout her stay, she was also given lactulose at 10ml via her NG tube every six hours to help stabilize her ammonia levels. Another standard management for MMA includes proving hydration with a high caloric supplementation.
The nursing implication for proving proper hydration includes assuring that the patient IV line is running smoothly and patient is receive the fluids, if the patient IV fluid is not continuously running making sure the IV line is flush every two hours to ensure patency, ensuring the right rate for patient is running to assure the correct amount of fluids are being administered, and ensuring that the medication given is compatible with the IV solution to prevent any reactions and clogging of the IV line (Girdwood, 2019). In the case of my patient, she had a peripheral IV line running continuously with potassium chloride + 20 mEq+D10%/NaCl 0.45% at a rate of 74mL/hr. Along with the patients peripheral IV line, she had intralipid fat emulsion (20%) running at 3mL/hr via her NG tube. Providing hydration and a high caloric supplementation was necessary for this patient to reach an anabolic state and prevent further exacerbation of the metabolic decompensation state.
Given the accumulation of toxic metabolites during a metabolic crisis state in patients with MMA the number one goal is to eliminate the toxic metabolites and reestablished homeostasis. The nursing implication for eliminating toxic metabolites include providing medications to eliminate toxins, monitoring urine input and output to ensure proper elimination, and monitoring urinalysis and blood gases to access the level of toxicity in the body and see if the interventions provided are working. In my patients’ case, she was given levocarnitine, a supplement used to treat low levels of carnitine cause by high levels of acid metabolites. When levocarnitine it supplemented it can conjugate and detoxify the buildup of acids ultimately reducing the acid levels ("Methylmalonic Acidemia - Acute Illness Protocol"). Initially patient was given 350mg of levocarnitine IV push every three hours, which was then transitioned to NG every twelve hours as medication for home. Reference of medications are provided for more information on following page regarding the patient.
Medication Dose Indication and mechanism Nursing considerations Adverse effects
Lactulose 10mL, oral solution, NG tube, Q6HR Promotes increased peristalsis, bowel evacuation; decrease serum ammonia concentration.
Inhibits diffusion of NH3 into blood by converting NH3 to NH4+
Conduct neurological exam in patients with elevated serum ammonia levels, assess hydration status, encourage adequate fluid intake, Monitor daily pattern of bowel activity Severe diarrhea, electrolyte imbalance, laxative dependence, chronic constipation, loss of normal bowel function
Levocarnitine 350mg, injection, IV push, Q3HR It is used to treat carnitine deficiencies, acute myocardial infarction, supplement to total parenteral nutrition
Carnitine is a small protein that binds to and helps transport fatty acids into the mitochondria, the site of energy production within cells. In the mitochondria, carnitine binds to and removes toxins from the cells. Monitor serum triglycerides, fatty acids, and carnitine levels Nausea, vomiting, abdominal cramps, heartburn, gastritis, diarrhea
Potassium chloride (additive) 20 mEq+D10%/NaCl 0.45% 1, 000mL Peripheral line, 74mL/hr, 1000mL, first dose Hydration and fluid replenishment, treatment and prevention of hypokalemia
Maintains normal renal function, acid-base balance Assess for hypokalemia, monitor serum potassium, monitor daily pattern of bowel activity Hyperkalemia, nausea, vomiting, diarrhea
Intralipid fat emulsion 20% 108mL central catheter, 3mL/hr, 108mL per bag, x 24hr, first dose Patients requiring parenteral nutrition for extended periods of time and source of essential fatty acids
Mechanism unknown Observe patient closely. Acute reactions tend to occur within the first 2 ½ h of therapy, Determine baseline values for hemoglobin, platelet count, blood coagulation, liver function, plasma lipid profile
Headache, dizziness, flushing, drowsiness, nausea vomiting
Psychosocial Subsystem
The patient’s psychosocial subsystems (family, spiritual, cultural) were evaluated by Hockenberry, Olshansky, & Perry, 2018, in conjunction with clinical assessments tools provided by the American Academy of Pediatrics (American Academy of Pediatrics, 1996). Patient is a three-year-old female who has a history of developmental delay and is receiving speech and occupational therapy from El services, who requires around a clock care. Patients main caregiver are her parents. Her family constellation at home consist of her mother and father, as well as three older brothers. Her family currently lives outside of Chicago and requires a social consult to discuss accommodations options near hospital. Additionally, patient requires total support from parents when it comes to managing her disorder and controlling paperwork and billings.
Given the patients age and cognitive development, patient was unable express or understand the concept of spirituality. This may due because child has not yet learned about faith or religion from their guardian. According to the article written by Ben Mardell and Mona Abo-zena (2010) called Kindergartners Explore, it stresses the need to guide exploration about difference in children’s beliefs in order to help develop healthy attitudes about spirituality and cultivate meaningful relationship among diverse families. Spirituality has also been used in ways to help children cope with their illness. In a study conducted by Cotton, Grossoehme, & Mcgrady (2011), they found that children who use prayer gain a better sense of control when sick or in the hospital and felt less stress and discomfort when in pain or distress. Indeed, I believed this child will benefit from learning about spirituality since she’s been in and out of the hospitals and has not cope well being separated from her family.
Culturally, patient presented as young polite child saying please and thank you after each gesture. At first shy, but once she warmed up, she was social with the RNs and staff showing them the activity she was participating in. Patient was also fairly cooperative and compliant with the nurse’s care. Similarly, patient showed good social engagement with the therapist and increased language quantity and quality and laughing. Patient expressed feeling sad from being separated family but was reassured that she would see them soon. Patient currently attends El service public school where she expressed missing her teachers and playing with friends. In terms of stress and coping, patient has some limitation in social-emotional regulation, although age appropriate, has contribute to her difficulty coping with hospitalization.
Nursing Diagnosis (in priority order)
1. Deficient fluid volume related to active fluid volume loss as evidence by patient excessive vomiting.
2. Imbalanced nutrition less than the body requirement related to emesis and poor nutritional intake as evidence by patient’s imbalance electrolyte levels
3. Nausea related to gastrointestinal irritation as evidence by patient decreased pH in the blood.
4. Risk for infection related to malnutrition as evidence by patient’s poor dietary intake.
5. Risk for injury related to disorientation as evidence by patient’s irritability and agitation
6. Ineffective coping related to chronic condition as evidence by patient excessive crying and sadness.
7. Risk for caregiver role strain related to discharge of family member with significant home care needs.
Plan of Care for Three Nursing diagnosis (two physical and one spiritual, cultural, or psychosocial):
Ineffective coping related to chronic condition as evidence by patient excessive crying and sadness.
Goals Interventions Rationale Evaluation
Verbalize ability to cope
Determine patient’s ability to cope
Explore previous coping strategies Encourage patient to verbalize feelings, fears, and concerns
Evaluate available resources and support systems
Encourage the patient to describe previous stressor and the coping mechanism used. Open discussion can facilitate the identification of causative and contributing factors
The environment could be aggravating ineffective coping so it’s important to determine the source of the problem
Allows for identifying ineffective strategies and developing new ones appropriate for her case. Compare previous verbalization to current and determine patient expressiveness changed
Compare patients different support systems and determine ineffective support
Compare patient’s previous response to coping to current and assess efficacy to current coping
Risk for caregiver role strain related to discharge of family member with significant home care needs.
Goals Interventions Rationale Evaluation
Caregiver will identify resources available to help in giving care
Caregiver will maintain physical and psychological health
Increase family caregiver’s knowledge of effective and safe caregiver strategies and improve their emotional coping skills
Encourage the involvement of other family members to relieve pressure on the primary caregiver.
Introduce stress-reducing methods to the caregiver.
Encourage caregiver to participate in support groups Allowing other members to care for the patient can decrease burden and allow for successful caregiving
It is important for caregivers to relax and reenergize emotionally to assume care responsibly
Support groups have shown to be advantage in proving education and anticipatory guidance
Assess caregiver’s role in identifying available resource based on patient needed care
Use an evaluation tool to determine caregiver coping and strain if needed, such as the Caregiver Strain index (Ribeiro, et al., 2019)
Compare caregiver previous knowledge in emotional coping to current and assess efficacy
Imbalanced nutrition less than the body requirement related to emesis and poor nutritional intake as evidence by patient’s imbalance electrolyte levels
Goals Intervention Rationale Evaluation
Patient will display nutrition ingestion sufficient to meet metabolic needs
Patient will show no signs of malnutrition
Patient will weight within normal range for height and weight Administered appropriate feedings as indicated by physician order based on patients’ nutritional needs.
Encourage resting periods within feedings
Determine healthy body weight for age and height Allows for correct administration of feedings and minimizes patient discomfort and complications
This could help with conserving energy and minimizing calories requirement
This would allow for better evaluation of patient require nutritional intake Reassess patients’ vital signs after each feeding and determine patient’s status improvement
Assess patient’s ability to tolerate feedings
Compare patient previous and current and determine correct weight is met
Discharge Needs
Given patients diagnosis of methylmalonic acidemia, parents require extensive education regarding this condition and potential complications. The primary education to focus on in the discharge planning for this patient would be how to properly setup a feeding pump and how to insert an NG tube and check for proper placement, how to administer the feedings via the NG tube, and general health management and medication education.
NG tube management were covered in depth by the Nurse in charge with patients’ father at the bedside. Handouts regarding proper insertion and placement of NG tube were provide and discuss at the bedside with patient (https://www.childrensmn.org/educationmaterials/childrensmn/article/15553/nasogastric-tube-feeding/). Proper health management in case of emergencies and medication regiment should have also been discussed with parents.
The student nurse was not present at the time of discharge, but discharge instructions should have included scheduled follow ups, such as follow up care with primary physician within a week a 1 and genetic and surgery consult. The discharge should have also included proper nutrition regimen that includes how much NG formula should be given and how much regular food/drinks can be given. Similarly, a nurse should have provided a review of the home meds and what they are used for. Overall, if parents have any questions or concern instructions should have be printed and handed with all emergency contact in it.
References
Adapted, with permission, from Kemper KJ, Kelleher KJ. 1996. Family psychosocial screening: Instruments and techniques. Ambulatory Child Health 1:325–339. (Ambulatory Child Health published by Blackwell Science, http://www.blacksci.co.uk.)
Ansari, P., C. (2018). Ileus - Digestive Disorders. Retrieved from https://www.merckmanuals.com/home/digestive-disorders/gastrointestinal-emergencies/ileus.
Children's Hospital. (2014, March 31). Peripherally Inserted Central Catheter (PICC Line). Retrieved from https://www.chop.edu/treatments/peripherally-inserted-central-catheter-picc.
Clinical Assessment Tools. (n.d.). Retrieved from https://www.aap.org/en-us/advocacy-and-policy/aap-health-initiatives/resilience/Pages/Clinical-Assessment-Tools.aspx.
Cotton, S., Grossoehme, D., & Mcgrady, M. E. (2011). Religious coping and the use of prayer in children with sickle cell disease. Pediatric Blood & Cancer, 58(2), 244–249. doi: 10.1002/pbc.23038
Fischbach, F. T., & Dunning, M. B. (2015). A manual of laboratory and diagnostic tests (9th ed.).
Girdwood, S. (2019). Clinical Guideline Highlights for the Hospitalist: Maintenance Intravenous Fluids in Infants and Children. Journal of Hospital Medicine, 14(3), 170. doi:10.12788/jhm.3177
Hockenberry, M. J., Olshansky, E. F., & Perry, S. E. (2018). Maternal child nursing care (6th ed.). St. Louis, MO: Elsevier.
Keyfi, F., Talebi, S., & Varasteh, A. R. (2016). Methylmalonic Acidemia Diagnosis by Laboratory Methods. Reports of biochemistry & molecular biology, 5(1), 1–14.
Kizior, R. J., & Hodgson, B. B. (2017). Saunders Nursing Drug Handbook 2018 - E-Book. Saintt Louis: Elsevier Health Sciences.
Mardell, B., & Abo-Zena, M. M. (2010). Kindergartners Explore spirituality. YC: Young Children, 65(4), 12–17. Retrieved from http://search.ebscohost.com.proxy.cc.uic.edu/login.aspx?direct=true&db=ehh&AN=52889906
Methylmalonic Acidemia - Acute Illness Protocol. (n.d.). Retrieved from https://newenglandconsortium.org/for-professionals/acute-illness-protocols/organic-acid-disorders/methylmalonic-acidemia/.
Nelson, S. L. (2019). Methylmalonic Acidemia: Brief Overview of Methylmalonic Acidemia, Etiology and Neuropathology, Evaluation of Methylmalonic Acidemia. Retrieved November 13, 2019, from https://emedicine.medscape.com/article/1161799-overview.
Ribeiro, O., Brandão, D., Oliveira, A. F., Martín, I., Teixeira, L., & Paúl, C. (2019). The Modified Caregiver Strain Index: Portuguese version. Journal of Health Psychology, 135910531988393. doi: 10.1177/1359105319883933
Selected Normal Pediatric Laboratory Values. (n.d.). Retrieved from http://wps.prenhall.com/wps/media/objects/354/362846/London App. B.pdf.
Sheppard, S. E., Ficicioglu, C. E., Hahn, S. E., & TePas, E. E. (2019). Methylmalonic acidemia. UpToDate. Retrieved November 13, 2019, from https://www.uptodate.com/contents/methylmalonic-acidemia
Wilson, D. D. (2008). McGraw-Hills manual of laboratory & diagnostic tests. New York: McGraw-Hill, Medical Pub. Division.
X-rays, CT Scans and MRIs - OrthoInfo - AAOS. (1995). Retrieved November 15, 2019, from https://orthoinfo.aaos.org/en/treatment/x-rays-ct-scans-and-mris/. [Show Less]