INDIVIDUAL CASE STUDY

NATIONAL CHILDREN’S HOSPITAL - HEMATOLOGY AND ONCOLOGY WARD:

BONE MARROW FAILURE SYNDROME

Angela Eres Balingbing

3NU01 - RLE Group 6

OCTOBER 20, 2022

 I. PATIENT PROFILE

SEX: FEMALE
AGE: 8 mos. and 2 days old
BIRTHDAY: 02/20/2022
ADMISSION DATE: 10/14/2022
CHIEF COMPLAINT: PALLOR, FATIGUE AND LOW HEMOGLOBIN
ADMITTING DIAGNOSIS:BONE MARROW FAILURE SYNDROME
HISTORY:
At age 6 mos old, the guardian noticed that her baby was fatigued and pale for the past 2
weeks. On August 2022, Reyame was then checked and admitted to her first hospital wherein
she was not diagnosed. The patient was transferred to numerous hospitals which were not
recalled before getting to National Children’s Hospital. On October 14, 2022, the patient was
admitted to the Hematology-Oncology Ward of NCH with a working diagnosis of Bone Marrow
Failure Syndrome. Baby Reyame’s blood work shows that she has low hemoglobin. No bleeding
was present. When asked about family history, the guardian verbalized that she and her
husband have no family history of any diseases. She had no pregnancy complications and the
baby had a complete EPI vaccination.

On October 17, 2022 patient showed signs of bleeding through bruising of the skin in the head
and extremities (ecchymoses & petechiae) and gum bleeding which was accompanied by fever.

Vitals Signs

Date Time RR CR TEMP 02 SAT

10/20/22 8 AM 46 bpm 164 36 99

10 AM 33 bpm 168 36.4 98

10/21/22 8 AM 37bpm 128 36.4 98

10 AM 43bpm 122 36.3 99

II. DISEASE INTRODUCTION

Bone marrow failure syndromes are the inability of the bone marrow to produce sufficient
quantities or abnormal state of the three main types of blood cells in the body:
○ white blood cells, which work to ward off and fight infection.
○ red blood cells, which contain hemoglobin, carry iron and deliver oxygen to
tissues throughout the body.
○ platelets, which help form clots and stop bleeding.
 These syndromes are usually detected thru a low peripheral blood count indicating a
bone marrow failure. Bone marrow failure syndromes may be present at birth or develop
later in life. Some bone marrow failure syndromes are due to an underlying inherited
genetic condition, while other bone marrow failure syndromes are due to an acquired
cause such as viral or drug/toxin exposures. In many cases of bone marrow failure, the
underlying cause cannot be identified and the term “idiopathic” is used. The bone
marrow failure can manifest as one isolated cytopenia (one blood cell type decreased) or
pancytopenia (all three blood cell types decreased). Some samples of these syndromes
are the following:
● Amegakaryocytic thrombocytopenia
● Pearson syndrome
● Shwachman diamond syndrome
● Thrombocytopenia with absent radio plastic anemia, idiopathic or post-infectious
● Diamond blackfan anemia
● Deficiency of ADA2 (DADA2)
● Dyskeratosis congenita
● Fanconi anemia
● MIRAGE syndrome
● Paroxysmal nocturnal hemoglobinuria (PNH)

Treatment of bone marrow failure is heavily dependent on the underlying cause of the bone
marrow failure but can include careful monitoring of blood counts and supportive care such as
blood transfusions to temporarily relieve symptoms, or bone marrow transplant also known as
stem cell transplant and/or immunosuppression where patients undergo conditioning therapy.
This may include chemotherapy and/or radiation, to prepare the body for the transplant. The
goal of conditioning therapy is to remove the diseased bone marrow cells to make space for the
new healthy ones to grow. Conditioning therapy also suppresses the immune system so that the
body doesn’t reject the donor’s foreign stem cells. In some patients, bone marrow failure can
increase their risk of developing aggressive blood cancers such as acute myeloid leukemia
(AML) or myelodysplastic syndrome (MDS). The risk for developing blood cancers varies among
bone marrow failure disorders. .

III. SIGNS AND SYMPTOMS

Since bone marrow failure syndrome affects healthy blood cell production, patients often
experience
● Fatigue
● Shortness of breath
● Frequent infections
● Easy bruising or bleeding
● Paleness
● Petichiae
 IV. BLOOD TRANSFUSION

Date Order Nursing Responsibilities

10/15/22 1 UNIT OF PRCB TYPE B+ 70 Before:

CC ● Confirm doctor’s order, availability,
consent, and CBC result. Confirm if you
10/16/22 ` UNIT OF PRBC TYPE B+ 80 need pre-med before infusion.
CC ● Assess blood product against order.
10/17/22 Check for blood type, Rh factor,
10/16/22 expiration, color, leak, and consistency.
1 UNIT OF PLATELET ● Calculate for flow rate with macro set (20
APHERESIS gtts/min) for 4 hours
10/19/22 ● Assess through guardian previous
1 UNIT OF PLATELET history of BT, allergies and previous
APHERESIS reactions.
10/20/22 ● Confirm blood typing & cross-matching
PRBC 90 TYPE B+ 90CC ● Monitor Vital signs
● Make sure it is not too cold

During:
● Check blood product again against
order. Check for latest blood test.
● Gather all equipments. Explain the
procedure to the patient
● Check IV patency.
● Monitor rate, color, consistency, amount
infused, swelling during infusion
● Infuse slowly during first 15 minutes to
detect any early blood reactions.
Administer on actual calculation after 15
minutes.
● Stop infusion immediately if BT reaction
occurs and keep patient in in KVO
PNSS. Assist patient in Fowler position.
Monitor vitals every 5 minutes and
document accordingly.
● If itching occurs, administer blood slowly.
● Do not administer too fast to prevent
circulatory overload. Neither should you
administer too slow which may damage
the blood product and infected.

After:
● Remove blood bag and flush with PNSS
● Monitor the vitals signs for any reactions.
● Repeat CBC
● Document assessment, findings, any
reaction, and time finished.
 V. NURSING MANAGEMENT (NCP)

ASSESSMENT NURSING PLANNING NURSING INTERVENTIONS RATIONALE EVALUATION

DIAGNOSIS

SUBJECTIVE: Ineffective STG INDEPENDENT: INDEPENDENT: GOAL NOT MET:

peripheral After 24 hours of 1. Assess the patient’s 1. Identify other patient symptoms STG
Mother tissue nursing signs and symptoms such as the pattern of breathing, After 24 hours of
verbalize perfusion interventions, the of ineffective tissue appearance, dehydration, and nursing
“parang laging related to patient’s perfusion. the like. interventions, the
pagod po siya severe circulation will 2. Measure capillary 2. Rule out dehydration, shock, and patient’s circulation
at anemia improve as refill time. hypothermia. did not improve as
namumutla” secondary to evidneced by 3. Monitor CBC ( HGB 3. CBC and RBC are blood tests evidenced by
Fatigue bone marrow improvement of & RBC) primarily in charge of the body’s fatigue and pallor.
failure fatigue and oxygen delivery. Determine other
OBJECTIVE: syndrome as pallor. affected blood components. :
manifested 4. Monitor the patient’s 4. To determine if the oxygen level LTG:
10/18/22 by pallor, LTG: arterial blood gases is adequate. After 2 days of
HGB: 74g/L fatigue, low After 2 days of (ABG) levels. nursing
(120-160) hemoglobin, nursing 5. Monitor patient’s 5. Worsening of severe anemia may interventions, the
RBC: RBC, and interventions, the vital signs. lead to hypovolemia, shock and patient did not
0.2210^6/uL presence of patient will the early sign is through the vital maintain adequate
(3.93- 5.22) skin maintain signs. peripheral tissue
bleeding. adequate DEPENDENT: DEPENDENT: perfusion as
10/20/22 peripheral tissue 1. Administer PRBC as 1. To alleviate the signs and evidenced by
HGB:70g/L perfusion as ordered by symptoms of anemia. hemoglobin of 70g/L
RBC: evidenced by physician. and RBC of 2.19
2.2010^6/uL improvement in 2. Provide oxygen 2. To improve the gas exchange of 10^6/uL
the ecchymoses therapy, as per oxygen and carbon dioxide.
Pallor and petechiae, doctor’s oder.
hemoglobin, and COLLABORATIVE:
Ecchymoses red blood cell, 1. Refer to a COLLABORATIVE:
and petechiae hematologist and 1. To perform in-depth hematologic
on the upper phlebotomist. assessment and care specialized
extremities for the patient.
 VI. FDAR

DATE AND FOCUS DAR

TIME

10/20/22 Severe Anemia DATA:

10 AM ● “parang laging pagod po siya at namumutla”
● 10/18/22
HGB: 74g/L
RBC: 0.2210^6/uL
● 10/20/22
HGB:70g/L
RBC: 2.2010^6/uL
● Pallor
● Fatigue
● Ecchymoses
● Petechiae

ACTION:
● Monitored patient’s vital signs.
● Administered PRBC as ordered by physician.
● Provided oxygen therapy, as per doctor’s oder.
● Referred to a hematologist and phlebotomist.

RESPONSE:
The patient’s signs, and symptoms, CBC, RBC, ecchymoses, and
petechia still persists.
 VII. DRUG STUDY

DRUG MECHANISM OF INDICATIONS/ CONTRA- SIDE EFFECTS/ NURSING RESPONSIBILITIES

ACTION INDICATIONS ADVERSE EFFECTS

GENERIC ACTION: INDICATIONS: Hematologic: ● Be aware that although most patients

NAME: Pain relief may Mild to moderate pain thrombocytopenia, tolerate drug well, toxicity can occur with a
Paracetamol result from caused by hemolytic anemia, neutropenia, single dose.
inhibition of headache, muscle leukopenia, pancytopenia ● Know that acetylcysteine may be ordered
BRAND prostaglandin ache, backache, Hepatic: jaundice, to treat acetaminophen toxicity, depending
NAME: synthesis in CNS, minor arthritis, common hepatotoxicity on patient’s blood drug level.
Calpol with subsequent cold, Metabolic: hypoglycemic coma ● Activated charcoal is used to treat acute,
Tempra blockage of pain toothache, or menstrual Skin: rash, urticaria recent acetaminophen overdose (within 1
impulses. Fever cramps or fever Other: hypersensitivity hour of ingestion).
reduction may reactions (such ● Determine overdose severity by measuring
DRUG result from as fever) acetaminophen blood level no sooner than
CLASSI- vasodilation and CONTRAINDICATIONS 4 hours after overdose ingestion (to ensure
FICATION: increased : that peak concentration has been reached).
peripheral blood Hypersensitivity to drug ● Observe for acute toxicity and overdose.
Analgesic flow in Signs and symptoms of acute toxicity are
hypothalamus, as follows
which dissipates ○ Phase 1: Nausea, vomiting, anorexia,
heat and lowers malaise, diaphoresis
body temperature. ○ Phase 2: Right upper quadrant pain or
tenderness, liver enlargement, elevated
bilirubin and hepatic enzyme levels,
PEDIATRIC prolonged prothrombin time oliguria
DOSAGE: (occasional)
○ Phase 3: Recurrent anorexia, nausea,
10 ml/kg/dose vomiting, malaise; jaundice,
hypoglycemia, coagulopathy,
10x4kg= 40 encephalopathy; possible
ml/dose cardiomyopathy, renal failure
○ Phase 4: Either recovery or progression
to fatal complete hepatic failure.
● Caution parents or other caregivers not to
 give acetaminophen to children younger
than age 2 without consulting prescriber
first.
● Tell patient, parents, or other caregivers not
to use drug concurrently with other
acetaminophen containing products or to
use more than 4,000 mg of regular strength
acetaminophen in 24 hours.
● Inform patient, parents, or other caregivers
not to use extra-strength caplets in
dosages above 3,000 mg (six caplets) in 24
hours because of risk of severe liver
damage.
● Advise patient, parents, or other caregivers
to contact prescriber if fever or other
symptoms persist despite taking
recommended amount of drug.
● Inform patients with chronic alcoholism that
drug may increase risk of severe liver
damage.
● As appropriate, review all other significant
and life-threatening adverse reactions and
interactions, especially those related to the
drugs, tests, and behaviors mentioned
above.
References:

● Doenges, M.E., Moorhouse, M.F. and Murr, A.C. (2019) Nurse's Pocket Guide: Diagnoses, Prioritized Interventions
and Rationales (Nurse's Pocket Guide: Diagnoses, Interventions & Rationales). 15th Edition
● Kolecki, P., & Brenner B. (2022). Hypovolemic Shock Clinical Presentation. Retrieved October 14, 2022, from
https://emedicine.medscape.com/article/760145-clinical
● Complete blood count (CBC) - Mayo Clinic. (2020, December 22). Retrieved October 13, 2022, from
https://www.mayoclinic.org/tests-procedures/complete-blood-count/about/pac-20384919
● Kolecki, P., & Brenner B. (2022). Hypovolemic Shock Clinical Presentation. Retrieved October 14, 2022, from
https://emedicine.medscape.com/article/760145-clinical