www.360SCDHub.org

Together, we can improve quality care through education, empathy and understanding.

www.360SCDHub.org

School personnel and caregivers play an important role in a student’s health and academic success.
For students living with a chronic health condition like SCD, communication between parents and school officials is essential in supporting positive academic outcomes.
As a result, we have developed this customizable booklet.
We encourage teachers, students, and caregivers to read all sections and tailor the information that relates specifically to their situation.

THIS BOOK IS TO HELP GUIDE SCHOOL NURSES ON HOW TO BEST HANDLE STUDENTS WITH SICKLE CELL DISEASE

This booklet is a companion to the course in the app 360 SCD Hub.

Both are intended to advance the training of school nurses in the clinical manifestations of sickle cell trait and disease, how to manage it as part of the care team, and how to help educate your patients, parents, staff and community.

Please download the app here to fully participate in this training as well as have access to unique resources only offered here.

The app is also appropriate for your staff, parents and students. Encourage them to download and use it.

LEARNING MANAGEMENT SYSTEM (LMS)

Each of your patients may have their own training, learning, tracking and rewards area.

Encourage them to use it with you and their primary care doctors.

SICKLE CELL FOUNDATION OF ARIZONA

DEVELOPMENT TEAM

• ROBERT GILLIO - CHIEF MEDICAL OFFICER
• LUCY HOWELL - PROJECT DIRECTOR
• KACEY NYQUIST - ASSOCIATE DIRECTOR

EXECUTIVE TEAM

• MOSES AKPAN - SCFA PRESIDENT
• SARAH SPIEKERMEIER - SCFA BOARD MEMBER
• KEVIN JOHNSTON - SCFA BOARD MEMBER

CONTRIBUTING ORGANIZATIONS

• SICKLE CELL FOUNDATION OF ARIZONA
• RED CROSS OF SOUTHERN ARIZONA
• ARIZONA DEPARTMENT OF HEALTH
• BANNER HEALTH PLANS
• AZ BLOOD ALLIANCE
• THE FORCE FOR HEALTH® NETWORK

CONTRIBUTING ADVISORS

• FRAN ALTMAIER
• KATHERINE FULLERTON
• MELISSA DOMINGUEZ
• DR. NAOMI NEWMAN
• DR. SANJAY SHAH
• AKILA
• FATOUMA TALL
• YVETTE MILLER
• MAURICIO TIPPETT
• COURTNEY SLANAKER

The 360 SCD Hub Project is funded by the Health Resources & Service Administration (HRSA) grant and administered through the Sickle Cell Foundation of Arizona.

The views expressed in this platform, and video content are solely those of the creators and do not represent the views of the state of Ohio or federal Medicaid programs.

Content based on evidence based practices from Subject matter experts. These are the primary sources for the information shared in this manual and online app and course:

• Shook LM, Mosley C, Farrell CB, Connelly A, Jones CL. Educational Needs of School Nurses Regarding the Evidence-Based Management of Sickle Cell Disease. Int J Environ Res Public Health. 2021 Nov 5;18(21):11641. doi: 10.3390/ijerph182111641. PMID: 34770153; PMCID: PMC8582712.
• CDC Fact sheets on SCD

• 1. ABOUT SICKLE CELL DISEASE
• 2. TIPS FOR TEACHERS, NURSES, AND SCHOOL ADMINISTRATORS
• 3. TIPS FOR PARENTS AND OTHER CAREGIVERS
• 4. MANAGING COMMON AND CRITICAL SCD MANIFESTATIONS
• 5. FORMS
• 6. CONCLUSION

EDUCATION | RESOURCES | COMMUNITIES | TRAINING | TOOLS & TRACKERS | CONNECTIONS

Sickle cell disease (SCD) is an inherited blood disorder (a blood disorder that runs in families). People with SCD produce an abnormal type of hemoglobin (called hemoglobin S (HbS) or sickle hemoglobin).

Hemoglobin is a protein in red blood cells that carries oxygen from the lungs to the organs and tissues in the body. The abnormal hemoglobin in SCD can cause the red blood cells to have a sickle or banana shape under certain conditions. People with SCD often have a decreased number of red blood cells, a condition called anemia, which can cause lack of energy, breathlessness, and pale color of the skin and lips.

There are many forms of SCD and the most common type is Hb SS, known as sickle cell anemia, which is inherited when a child receives two “S” genes (one from each parent). Hb SC is a form of disease that is inherited when a child receives one sickle cell gene, “S” from one parent and from the other parent, a gene for an abnormal hemoglobin called “C”. Another type of SCD, sickle beta-thalassemia, occurs when a child inherits one sickle cell gene and one gene for beta thalassemia (another type of abnormal inherited hemoglobin that causes anemia).

In the United States, SCD is most commonly found among African‑Americans or persons of African descent; however, people of all races and ethnicities can have SCD.

About 1 in every 365 African-American babies in the United States is born with SCD; and worldwide, approximately 300,000 babies are born with SCD each year. As more people move from areas highly affected by SCD to the United States, schools will become more diverse and there is a higher chance that teachers will encounter a student with SCD in their classrooms. As with any student with a chronic health condition, students with SCD may experience health problems during the school day.

SCD can have a significant impact on the quality of life and may require comprehensive medical management. Newborn screening programs in the U.S. aim to identify affected individuals early, enabling timely interventions and support. While advancements in treatment and care have improved outcomes, individuals with SCD still face challenges, making ongoing research, education, and access to healthcare vital in addressing this inherited blood disorder.

1. Ensure adequate access to water/hydration. Staying well hydrated by drinking plenty of water can help prevent pain episodes and other health problems.
2. Allow frequent bathroom breaks. Children with SCD produce large amounts of dilute urine even when they are dehydrated. Do not restrict students with SCD from bathroom breaks.
3. Allow accommodations during extreme temperatures and conditions. Cold or hot weather can trigger pain crises. Teachers should not assign a student with SCD a seat in drafty locations, directly in front of fans or under air conditioner vents.
4. Allow accommodations during physical education and recess activities. Most children with SCD can engage in moderate exercise, however, teachers may modify curricula so that a child experiencing health problems related to SCD can participate in physical education in roles that are less strenuous.

5. Take special care of injuries. Never apply a cold pack to an injury or pain site. First aid measures that should be provided when necessary include applying direct pressure for bleeding, wrapping with an ace bandage, or elevating a hurt limb.
6. Watch for signs of stroke. Strokes may be difficult to detect when they affect a small portion of the brain, but they are extremely important to watch for because they are relatively common in the early school years among children with sickle cell disease.
7. Be aware of emotional well-being. Not all children with SCD have outward signs of illness. These sometimes subtle outward signs may make children living with SCD targets for teasing and bullying.
8. Maintain open lines of communication with parents. Teachers can help create a positive relationship with school and build confidence when asking for help.

Staying informed and up to date on current information for treatment and responses for sickle cell is vital for ensure children's safety in school.

It doesn’t stop there. Keeping records of when students are taking medication on campus, what they were doing when a pain crisis occurs, as well as how you took action and how the treatment plan worked can affect future treatment.

Having a response plan in place is also essential for ensuring the child receives effective treatment. This plan may include emergency contacts, doctors information, and preferred hospital. All of which are important for if something were to happen.

Each child's needs may be different. Work with parents and providers to decide how to best support the child. Document what changes and accommodations are needed in the classroom and in activities.

1. Stroke Risk Assessment: Transcranial Doppler (TCD) Screening: Schools should be aware if students with SCD have undergone TCD screening, a non-invasive test to assess the risk of stroke by measuring blood flow in the brain's major arteries. Regular screenings help identify those at higher risk, allowing for preventive measures.
2. Recognizing Stroke Symptoms: Training Staff: School staff, including teachers and nurses, should be trained to recognize the signs of stroke in children, such as sudden weakness, speech difficulties, or severe headaches. Immediate action is crucial for timely medical intervention.
3. Monitoring Academic Progress: Schools should closely monitor the academic progress of students with SCD, as they may face learning challenges due to complications, such as cognitive impairments resulting from strokes. Regular assessments and communication with parents and healthcare providers can help identify and address these challenges.

Set up a meeting to discuss SCD with your child’s teacher. It may be important for parents and teachers to meet at the beginning of each school year. Invite these school staff to an introductory meeting: your child’s teacher(s) including the physical education teacher, learning coordinator, school nurse, principal, or counselor.

During an introductory meeting, it may be helpful to describe what SCD is in broad terms (e.g., an inherited disorder that affects the amount of oxygen carried in the bloodstream) and more specifically how SCD impacts your child’s daily life. It may also be important to describe how your child copes with having SCD and to discuss clearly how you wish to be informed when your child has symptoms at school (e.g., call me immediately, when to call paramedics, what hospital you prefer).

Initiating an annual meeting with school staff is essential to ensure that everyone is on the same page regarding your child's SCD. This provides an opportunity to educate school personnel about the condition, share insights into your child's specific needs, and establish effective communication protocols in case of emergencies or symptom flare-ups.

Discuss the possibility of a plan if your child has special healthcare needs that impact their daily life. Work with school staff to develop a plan that includes accommodations such as extra books, frequent bathroom breaks, or access to water. Both an IEP and 504 plan should be updated yearly to meet the student’s needs.

A 504 plan is administered in schools that receive federal funds (i.e., public schools) and is a written document that outlines reasonable accommodations for individuals with disabilities. The 504 plan ensures that a child with SCD has equal access and is able to participate fully in school activities; however it does not outline plans for remedial instruction.

The IEP addresses remedial instruction. When health problems related to SCD negatively impact a student’s academic performance, special education services may be recommended. An IEP is a written document developed between school staff and families to assure specialized or remedial instruction. Additional federal funding is given to schools to provide a wide range of services, depending on the needs of the child.

Develop an individualized care plan. An individualized care plan is a written document that can be tailored to the specific healthcare needs of a student with SCD. An individualized care plan requires input from the teacher, school nurse, the child, and the family. Plans should include emergency contact information, any special needs the student may have, and instructions for giving pain medication, including who is responsible for giving the medicine and how to decide which medication to give.

An individualized care plan is a critical tool for providing consistent and tailored care to students with SCD. This plan should outline emergency procedures, medication administration guidelines, and any specific requirements your child may have. Regular updates are essential to keep the plan current and effective.

Comprehensive Care Plan: Work with the school nurse, teacher, and your child to create an individualized care plan that covers emergency contact information, special needs, and instructions for giving pain medication.

Regular Updates: Ensure that the care plan is updated at least annually or whenever there are changes in your child's healthcare needs. If the school lacks a dedicated nurse, coordinate with the school secretary or counselor for plan management.

Tell teachers about changes in your child’s health. Families can support their child’s school success by keeping in close contact with teachers. Parents should talk to teachers about plans in the event their child misses school before a period of illness occurs. When your child is sick, parents can inform the school, ask for lesson plans and homework, or ask for homebound teachers to prevent the student from falling behind in their coursework.

As children grow, their ability to express their needs evolves. In the early elementary school years, parents may discuss all of their child’s healthcare needs with teachers. As children get older it is important to support them in becoming their own advocate. Parents can help their child understand their condition, help them to understand the ways that the school is required to help them keep up with their coursework and reduce the risk of health problems related to their condition, and they can encourage their child to express his or her medical needs clearly.

Q: Is sickle cell disease contagious?
No, you cannot catch sickle cell disease like a cold. Sickle cell disease is a genetically inherited disorder, passed down from a person’s parents.

Q: Are there specific TRIGGERS TO be aware of?
Extreme temperatures and dehydration can sometimes trigger symptoms. We are cautious about managing these factors and keeping our child well-hydrated.

Q: Why is your child out of school so often?
They need to be seen by a doctor more frequently than other students, so they may be at a doctor’s appointment. At other times, sickle cell disease may cause them to be in so much pain that he or she cannot attend school.

Q: Are there any limitations Physically?
While our child can participate in most activities, we've been advised to avoid extreme physical exertion. We can provide a note from our healthcare provider outlining any necessary modifications.

While normal red blood cells are round like donuts and move freely through blood vessels, sickled blood cells clog the flow of blood and can break apart as they move through blood vessels. Additionally, sickled red blood cells do not deliver oxygen throughout the body as well as normal red blood cells do.

Pain episodes, fever, weakness, and fatigue are common manifestations of SCD. It's crucial for school nurses to recognize when these symptoms are severe or indicative of a serious complication. Prompt action, including seeking emergency care or contacting parents, can help manage these manifestations effectively.

Pain Episodes (Vaso-Occlusive Crises): Pain is a hallmark of SCD and can occur anywhere in the body. When a student complains of severe pain, take it seriously. Allow the student to rest in a quiet, comfortable place and monitor their pain level using a 1-10 scale. If pain is severe or worsening, or if the student's usual level of pain significantly increases, seek immediate medical attention.

Weakness or Fatigue: If a student experiences sudden weakness on one side of their body (not due to pain), inability to speak, or difficulty with memory, these could be signs of a stroke. Act promptly by calling 911 and notifying the student's parents.

ACS and stroke are among the most dangerous manifestations of SCD. School nurses must be vigilant in identifying these symptoms and acting swiftly. Immediate medical attention is crucial to prevent further complications and ensure the safety of the student.

Acute Chest Syndrome (ACS): ACS is a life-threatening complication of SCD, characterized by chest pain, coughing, and difficulty breathing. If a student exhibits these symptoms, treat it as a medical emergency. Call 911 immediately and inform the student's parents. Avoid applying cold packs to the chest, as this can worsen the condition. In addition to recognizing symptoms promptly, it is crucial to provide supportive care while awaiting emergency medical assistance. Keep the student calm and comfortable, encourage deep breathing exercises, and ensure an oxygen supply is available if possible.

Stroke: SCD is a leading cause of childhood stroke. Signs of stroke in a student may include severe headaches, dizziness, visual changes, sudden weakness in one limb or side of the face, numbness, inability to speak, or seizures. Recognize these signs and call 911 without delay. Notify the student's parents and, if necessary, initiate a chronic care plan for stroke prevention.

Whenever a student with SCD exhibits severe or worsening symptoms, such as excruciating pain, high fever, or ACS, do not hesitate to call 911. Timely intervention is vital to saving lives in these critical situations.

Urgent Care Referral: For less severe but concerning symptoms, such as moderate pain or mild fever, contact the student's parents immediately. Suggest urgent care referral for medical evaluation to address the symptoms promptly and prevent escalation.

Chronic Care Plan Implementation: Work closely with the student's parents and healthcare provider to develop and implement a chronic care plan tailored to the student's specific needs. This plan should outline medication administration, emergency contact information, and guidelines for managing symptoms during school hours. A well-structured chronic care plan is a valuable tool for managing SCD in school. It provides clear guidance on how to handle routine care, medication administration, and emergency situations.

Work with the student's parents and healthcare provider to ensure the chronic care plan is comprehensive and tailored to the student's needs. Regularly review and update the plan as needed, especially if there are changes in the student's health status or treatment regimen. Educate school staff about the plan and provide training on how to administer medications or perform specific interventions outlined in the plan.

Personal Information

• Full Name:
• Date of Birth:
• Grade:
• Gender: Male ☐ Female ☐
• Sickle Cell Type:

Emergency Contact Details

• Name:
• Phone Number:
• Relation:
• Health Care Provider Name:
• Provider Number:

Treatment Plan

• Medications (list dosage, frequency, and administration method):
• Other Treatments:
• Symptoms and Triggers (i.e., common issues the child experiences):
• Symptoms Management (i.e., when to administer medication, use comfort measures, or seek medical attention):

Action Plan

• What to do when child is experiencing signs of Acute Chest Syndrome (ACS):
• What to do when child is experiencing signs of a stroke:

I, ____________________, give consent for the school nurse and designated school personnel to administer the medications and treatments as outlined in this Individualized Health Plan.

Signature: ____________________ Date: ____________________

Personal Information

• Full Name:
• Date of Birth:
• Grade:
• Gender: Male ☐ Female ☐
• Sickle Cell Type:

Emergency Contact Details

• Name:
• Phone Number:
• Relation:
• Health Care Provider:
• Provider Number:

Medical Triggers

Known Triggers for Sickle Cell Crises and Typical Symptoms Exhibited by the Student:

Signs of Medical Distress to Watch For:

Accommodations

Physical Activity Limitations and Modifications:

Other accommodations the student may need:

Signature: ____________________ Date: ____________________

1. Severe Pain: Sudden, intense pain, often in bones or joints.
2. Unexplained Fatigue: Profound tiredness or weakness that is not alleviated by rest.
3. Fever: Elevated body temperature.
4. Shortness of Breath: Difficulty breathing or labored breathing.
5. Swelling of Hands and Feet: Noticeable swelling in the extremities.

Refer to child's emergency plan for any questions or concerns.

6. Pale or Jaundiced Appearance: Paleness or yellowing of the skin and/or eyes.
7. Dizziness or Fainting: Feeling lightheaded or losing consciousness.
8. Vision Changes: Blurred vision or other visual disturbances.
9. Difficulty Speaking: Slurred speech or difficulty forming words.
10. Abdominal Pain: Pain in the stomach or abdomen.

Refer to child's emergency plan for any questions or concerns.

In conclusion, Sickle Cell Disease (SCD) is a complex condition that can impact a student’s daily life and education. School nurses play a crucial role in supporting students with SCD by recognizing, managing, and preventing complications. This manual has provided essential information and practical tips for school nurses to navigate various aspects of SCD care in a school setting.

Understanding the fundamentals of SCD, including its different forms and prevalence among diverse populations, is vital for school nurses. Equally important is recognizing the most frequent manifestations, such as pain episodes and fever, and being prepared to respond promptly. Additionally, being vigilant for the most dangerous complications like Acute Chest Syndrome (ACS) and stroke is essential, as timely intervention can be lifesaving.

Collaboration with parents, teachers, and healthcare providers is a cornerstone of effective SCD management in schools. By following the guidelines and tips provided in this manual, school nurses can enhance the well-being and educational experience of students living with SCD. It is our hope that this resource empowers school nurses to provide exceptional care and support for these students, ultimately contributing to their academic success and overall quality of life.

6. Pale or Jaundiced Appearance: Paleness or yellowing of the skin and/or eyes. A sudden change in color may indicate anemia or liver involvement. Seek medical attention promptly.
7. Dizziness or Fainting: Feeling lightheaded or losing consciousness. If a student complains of dizziness or faints, it could be a sign of reduced oxygen flow. Seek immediate medical assistance.
8. Vision Changes: Blurred vision or other visual disturbances. Any sudden changes in vision should be addressed urgently, as it may be related to blood vessel complications.
9. Difficulty Speaking: Slurred speech or difficulty forming words. If the student experiences sudden difficulty speaking, it could be indicative of a neurological complication. Seek medical help promptly.
10. Abdominal Pain: Pain in the stomach or abdomen. Abdominal pain can be a sign of a sickle cell crisis or other complications. Evaluate the severity and involve the school nurse.

Refer to child's emergency plan for any questions or concerns.