Nursing Care Plan: Sickle Cell Crisis and Acute Chest Syndrome.

Provide a synopsis/summary of the salient information you have learned about your patient – as an individual patient and as a person

Patient was a 7-year old child who had been admitted to the unit with an acute pain crisis related because of acute chest syndrome to his diagnosis of sickle cell disease. Sickle cell disease is common genetic blood disorder that is prominent in certain ethnic groups, particularly in people of African or Hispanic descent. In this case, the patient and their family were of African- American descent (Solovieff et. Al, 2011). The child’s weight was noted to be 23.9 kg. During their admission in hospital the patient received a blood transfusion where 240 mL of pRBC was transfused within 4 hours.

The patient was noted to have no known allergies and development was normal for their age group. During their admission into the unit the patient experienced fever, breathing difficulties and pain in their lower back and chest.

The patient was placed on 1L of oxygen per minute due to his breathing difficulties while they were in crises. Symptoms of acute chest syndrome include breathing difficulties, chest pains, and fever (Jain, Bakshi, & Krishnamurti, 2017). Children typically present with a fever, cough, tachypnea, hypoxemia, and abdominal pain (Indiana Hemophilia & Thrombosis Centre, 2018). The patient was subsequently placed on an IV of D5NS which initially ran at 35mL/hr but was later reduced to 10 mL/hr. The patient was also receiving IV morphine which was initially set at 21 mcg/kg/hr (0.05 cc/hr) and later reduced to 12.5 mcg/kg/hr (0.03 cc/hr).

While caring for this patient, I could talk to the mother who is currently a personal support worker (PSW). Literature for sickle cell disease shows that

Vitals

 Medications

Vitamin D:  1000 UI (or one drop) given PO once daily. The recommended dose for vitamin D is 600 to 1000 UI/day for a child between 1 to 18 years of age. Vitamin D is recommended for children who have sickle cell disease as vitamin D deficiency is common regardless of the patients age or the season (Soe, Abas, Than, Ni, Singh, Said, & Osunkwo, 2017).

Ceftriaxone: 2000 mcg IM or IV, once daily. It is a broad-spectrum antibiotic used to treat gram negative bacilli. It is often used to treat infections in the lungs, skin, abdomen, lining of the brain, and urinary tract (Rexall Pharma Plus, n.d.).

Penicillin V: 300 MG PO, twice daily. Used to treat and prevent infections caused by pneumococcal bacteria in sickle cell patients (ie. Antibiotic prophylaxis) (Medicines for Children, 2011).

Hydroxyurea: 500 mg, PO once daily. Used to reduce the frequency of pain episodes and the need for blood transfusions in those diagnosed with sickle cell anemia (Multum, 2018). In children 2-18 years of age the initial dose that is recommended is 20 mg/kg per day (Multum, 2018).

Folic Acid: 1 mg, once daily.  Is a type of B vitamin that helps the body produce and maintain new cells. It is often recommended to be used as a prophylactic supplement for hydroxyurea (Cheng & Walkom, 2008). It is also used to prevent severe anemia (Stanford Children’s Health, 2018).

Clarithromycin: 2000 mcg, once daily. In addition to ceftriaxone, children who are experiencing severe respiratory distress should also be treated with doses of clarithromycin for coverage of atypical organisms (Carcao, Cook, Allen, Friedman, & Chorostil, 2012, p. 3).  

Polyethylene Glycol: 17g, two times daily PO. Used to treat occasional constipation. For infants, children and adolescents the recommended dose is 0.2-0.8 g/kg/day (not to exceed 17 g/day) (MedScape, n.d.).

PRN medications:  Benadryl, gravol, Ventolin, salbutamol (5 puffs x 4 hours), naloxone, acetaminophen (for fever and pain, 325 mg), ibuprofen (240 mg)

Assessment Findings:
Vitals were stable and within normal range throughout the shift. There were no significant findings upon inspection of the patient. Skin turgor was noted to be good, however, upon auscultating the lungs there were some crackles and wheezes in the patients’ upper chest. Urine output was adequate throughout the day and the patient had one bowel movement.

Output: 6.466 mL/kg/hour

Labs

CBC: 93L
Glucose: 5.3 mmol/L
pH: 7.30
Na: 135L
Leukocytes: 3.00 L
Potassium: 4.3
Red Cell Distribution: 16.1 H
Hematocrit” 0.262 L
Mean Cell: 31.0 H
Eosinophil: 0.6 H
PCO2: 53
HCO3: 26

Describe the relationships between the pieces of information and why they are significant for your patient at this time.

Identify the possible alternatives to choose from in caring for your patient.

Acute chest syndrome is a complication of sickle cell disease that is characterized by respiratory symptoms, fever and pulmonary infiltrate on chest x-ray (British Society for Haematology, 2015). Conversely, sickle cell related pain typically presents in the arms, chest, lower back, and legs due to a vaso-occulsive crisis (Stanford Children’s Health, 2018). In this case, the patient pain crisis originated in the patient’s lower back and chest likely due to the flow of blood being obstructed by sickled cells.

Since the patient was diagnosed with acute chest syndrome due to his falling HgB levels, a simple transfusion was administered, it would be expected that over time their HgB levels would begin to increase, which in this case went from 64 (Nov 12) to 93 (Nov 14). The patient’s lab values also showed low sodium levels which is often due to dehydration caused by factors such as severe vomiting, excessive sweating, and inadequate fluid intake (Williams- Hooker, Hankins, Ringwald-Smith, Stockton, & Shurley, 2013).

One important value in sickle cell patients is the reticulocyte count. In sickle cell patients suffering through conditions such as hemolytic anemia, it is expected that the count would be high. This is due to the anemia causing increased destruction of the red blood cells, conversely, the bone marrow increased red blood cell production as a compensatory measure leading to a high reticulocyte count (Weatherspoon, 2017).

It would be expected that the patient would be on an IV to help with dehydration and/or if they were in moderate to severe pain and unresponsive to oral treatments (Carcao, Cook, Allen, Friedman, & Chorostil, 2012, p. 3). Following the protocol at SickKids, cardiopulmonary monitoring would be initiated and a CBC, reticulocyte, and differential count would be obtained through lab work (Carcao, Cook, Allen, Friedman, & Chorostil, 2012, p.3).

Acute chest syndrome often occurs very suddenly and is a life-threatening complication of sickle cell disease (Stanford Children’s Health, 2018). Sickle cells begin to stick together, which consequently blocks the flow of oxygen in small vessels located in the lungs. The complication often arises when the body is under stress from fever, infection or dehydration and symptoms include pain, fever and a violent cough (Stanford Children’s Health, 2018).

As the patient was also receiving an opiate (ie morphine) it was expected that PEG would be given to help prevent constipation, and that acetaminophen and ibuprofen would be administered alongside the morphine if required to manage pain (Carcao, Cook, Allen, Friedman, & Chorostil, 2012, p. 5).  

Options for care

Typically, options for care will depend on several factors including the child’s overall health, the age of the child, how sick the child is, and how well the child is able to tolerate certain treatments, therapies or medications (Finke & Gersten,2018). Investigations for acute chest syndrome include obtaining a chest x-ray, ordering biochemistry tests and a full blood count.

Basic management of patients going through acute chest syndrome include pain relief, treatment of bacterial or viral infection and in some cases incentive spirometry (British Society for Haematology, 2015). In hypoxic patients, blood transfusions should be considered, particularly a simple transfusion early during treatment (British Society for Haematology, 2015).

Transfusions are used to help dilute the HbS (a destructive hemoglobin) with normal hemoglobin (HgB) in treating chronic pain, anemia, and acute chest syndrome to prevent stroke (Finke & Gersten,2018). To help manage pain associated with pain crisis and acute chest syndrome, hydroxyurea is prescribed. This medication is noted to also help reduce the number of blood transfusions patients receive (Stanford Children’s Health, 2018). Other medications typically prescribed to help manage infection and prevent severe anemia include penicillin and folic acid.

In preventing acute chest syndrome (ACS), patients admitted in pain crisis are at increased risk to develop ACS, particularly when back or chest pain limits the depth of inspiration (Meier & Miller, 2012). Regular use of incentive spirometry is recommended as well as the use of transfusions, which are generally reserved for patients who have increased oxygen requirements.

Patients with acute chest syndrome are often too unwell to maintain adequate oral hydration, so IVs are recommended. Fluid requirements are guided by the patients fluid balance and their cardiopulmonary status (Howard, Hart, Roberts-Harewood, Cummins, Awogbade & Davis, 2015).

What are the pharmacological management options, why are these appropriate?

Treatment strategies to address ACS include the use of broad-spectrum antibiotics (ie cephalosporin), IV fluids, and pain medication (Meier & Miller, 2012). Patients who suffer from asthma are also noted to be at an increased risk of ACS, and measures to control the asthma should be implemented (ex. inhaled corticosteroids and beta agonists) (Meier & Miller, 2012).

In treating pain in the hospital setting, it is important that IV access is obtained in cases of moderate to severe pain. Prior to this, initial lab work (CBC, Na, K, Cl, BUN, Ca, HCO3, PO4) should be obtained. If a fever is also present, blood cultures should be ordered. Typical IV fluids include the use of a NS bolus and D5NS (1x maintenance in cases of acute chest syndrome). In addition to the administration of pain medications, medications for narcotic side effects may also be ordered (ex. dimenhydrinate) as well as an order for stool softeners (Eckman & Platt, 2016).  

Depending on the level of pain the patient is experiences there is an analgesic ladder (Eckman & Platt, 2016):

1. Acetaminophen à Dosed at 15 mg/kg PO q4 (up to a maximum of 75 mg/kg/24hr)
2. Ibuprofen à Dosed at 10 mg/kg PO q 6 hours (max 20 mg/kg/24 hr)
3. Morphine à 0.01 mg/kg/dose  IV
4. Morphineà 0.05 mg/kg/dose IV

Guidelines also recommend the use of antibiotics in cases of acute chest syndrome. Antibiotics should cover atypical organisms, regardless of if the blood work cultures are negative. It is also recommended that patients are offered penicillin V prophylaxis. Evidence as indicated that in many cases it is simply not possible to distinguish between the different causes of acute chest syndrome, therefore it is assumed that infection is present Howard, Hart, Roberts-Harewood, Cummins, Awogbade & Davis, 2015). Patients with sickle cell disease are also vulnerable to infection, so prophylaxis treatment is administered to help reduce the risk by taking a daily dose of penicillin (NHS, 2016).

Describe the decisions you made in caring for your patient, the factors that influenced your decisions, and how you determined the priorities of your care.

Describe any adjustments or changes you made to your care based on follow-up assessment of your patient

1. My main priorities for today’s patient included prompt pain relief and distraction. Assessing the child’s comfort level before and after any medication was administer or q4 (unless otherwise indicated) was also important to ensure the dose being given was still effective in helping to manage the child’s pain. Measuring the patient’s level of pain was done through both the FACES pain scale as well as done verbally as the child could communicate their thoughts effectively. I also used distraction techniques in encouraging the patient to play with video games to help ease any pain they might have been experiencing.
2. Since the patient was still receiving continuous oxygen administration and opioid infusions, it was also important to monitor the cardiac and O2 saturation for signs of deterioration (Carcao, Cook, Allen, Friedman, & Chorostil, 2012, p. 6). There is evidence to show that while oxygen administration is not supported for all cases of vaso-occulusive crisis, if the child is experiencing hypoxia, lack of oxygen administration can lead to increased sickling (Carcao, Cook, Allen, Friedman, & Chorostil, 2012, p. 7). As the patient was receiving opioid infusion, monitoring the respiratory rate and pulse was important (Eckman & Platt, 2016).
3. Monitoring the child’s ins/outs. Children receiving IV fluids should have a fluid balance chart to document their ins and outs (Royal Children’s Hospital Melbourne, n.d.).. As the child was receiving a continuous IV, it was important to make sure that the output and input of the child was monitored in case adjustments were needed. Therefore, it would important to check hourly that the IV pump was still running and the site was clear, and that the intake and output of the patient were being recorded by the patient’s mother whenever he was to eat food, drink, or use the washroom.   

Expected Outcomes:

1. In the pain assessment, I would expect the patient to either be able to verbalize their level of pain and that it is being controlled or be able to use the FACES scale. As the patient was being administered analgesics, in this case morphine, for their painful crisis, it would be expected that the child would be experiencing little to no pain and an absence of complications.
2. Monitoring the child’s O2 sat was important, particularly as when the nasal prongs were outside of their nose, their O2 sat would drop below 95%. Monitoring the O2 saturation would also be indicative of how well the patients’ lungs are functioning (Indiana Hemophilia & Thrombosis Center, 2018). The patient was also receiving IV narcotics, so à If the patient respiratory rate were to become depressed a PRN order of naloxone was implemented and would be promptly administered to the patient (Eckman & Platt, 2016). As oxygen was being administered to the child, it would be expected that the child would show no signs of hypoxemia or shortness of breath.
3. As the patient was receiving IV hydration, strict ins/outs charting was required. If fluid intake were to decrease, dehydration could occur which would exacerbate the sickle cell crisis. By monitoring the child’s input and output I would expect to see adequate hydration levels. In this case, the patient’s volume per weight per hour was 6.466 mL/kg/hour (the minimal acceptable urine output in pediatrics is 1 to 2 ml/kg/hour).

 Respond to one of the following:
“If I could repeat this day in clinical I would . . . . .”

“I learned about myself . . .”

“I learned about nursing . . .”

Today, I learned about nursing how important the parent can be in caring for their child. Having the parent involved in the care of their child promotes family-centered care and helps develop a stronger partnership between the healthcare team and the patient’s parents (Abdelkader, Khalaf, Kridli, Arabiat, & Alrimawi, 2016). Involving the parents in the child’s care has also improved my confidence in being able to communicate more effectively with the patient, as oftentimes the child is most comfortable with their parents around or comforting them at the bedside. I’ve learned that listening to parents is also a vital tool when assessing their child and this has been supported in literature when caring for the pediatric population (Shuster, 2015). It is evident that parents to some degree, parents should also have a voice in the care of their child, be able to feel that they can engage in shared decision making, and want to be able to feel comfortable to ask questions and not feel overlooked (Shuster, 2015). As a nurse, I can help nurture this relationship in acknowledging that their child will also benefit from this.

In caring for my patient, I learned early on how vital it was to have the support of the child’s mother (a PSW) keep accurate track of the child’s input and output. It was evident in talking with the mother how important she felt her role was in helping care for her child and helped to put her own worries at ease when she could participate in aspects of his daily care. This desire to be part of her child’s care is supported in the literature with parents generally desiring and expecting to be involved when their child is hospitalized (Romaniuk, O’Mara, & Akhtar-Danesh, 2014). It was evident that the child was most comfortable when their mother was around, particularly during assessments or questions surrounding their overall wellbeing.

With this experience in mind, it is so easy to often get caught up in just focusing on the patient, but understanding the parents background (ie. Profession, education, living situation), also plays a key role in how care for the child can be developed. With the mother currently working as a PSW, it was reassuring to think that the both of us together could work as a team to take care of her child. Through partnership building I could invite the mother to help with certain aspects of the child’s care and felt comfortable to ask for her suggestions in implementing care. Conversely, it was evident that through strong communication skills the mother was also comfortable to ask me for assistance in certain tasks.