Procedures For Students With Sickle Cell Anemia

Definition

Sickle cell anemia is a serious condition in which the red blood cells can become sickle-shaped (that is, shaped like a “C”). Normal red blood cells are smooth and round like a doughnut without a hole. They move easily through blood vessels to carry oxygen to all parts of the body. Sickle-shaped cells don’t move easily through blood. They’re stiff and sticky and tend to form clumps and get stuck in blood vessels. The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ damage.

Background

Sickle cell anemia affects millions of people worldwide. It’s most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.

In the United States, sickle cell anemia affects about 70,000 people. It mainly affects African Americans, with the condition occurring in about 1 in every 500 African American births. Hispanic Americans also are affected; the condition occurs in 1 out of every 1,000 to 1,400 Hispanic American births. About 2 million Americans have sickle cell trait. About 1 in 12 African Americans has sickle cell trait.

Signs and Symptoms

  • Pain
  • Jaundice
  • Low energy level
  • Enlargement of spleen
  • Abdominal pain (due to gallstones and large spleen)
  • Abdominal distention
  • Fever
  • Chest pain
  • Shortness of Breath
  • Weakness of arms and legs (manifestations of stroke)
  • Persistent painful erection (priapism)

MANAGEMENT

Meeting of Principal’s Team

The principal/designee arranges a joint meeting with the student’s teacher(s), the assigned school nurse, and the student’s parent/guardian at the beginning of each school year.

The purpose of the meeting is to discuss any change in the student’s health status, current medications and their side effects, health history, triggers, apparent times/seasons of episodes, emergency care needed, any health related information necessary to assist the student, how school staff will handle emergencies and in-service education.

The registrar should make sure that sickle cell disease is noted on the student’s health frame information. Get several different phone numbers to call in emergencies, i.e. mother, father, doctor, aunt, uncle, grandparents, etc.

Data from many sickle cell specialty centers have shown that sickle cell anemia can affect the development of the brain because of changes in big and small blood vessels causing impairment of cognitive functioning and other learning disorders.  Therefore it is recommended that these children should have a neuropsychological test battery to evaluate areas of deficiency so they can be addressed as a part of the comprehensive educational plan.  Parents should meet with school staff to consider the need for the development of a 504 educational plan each year.

Triggers/School Environment

Common triggers for pain are cold weather, dehydration and stress. A student may also have additional unique triggers.

Parents should notify the school of the significant medical problems (pain, breathing problems, and priapism) and any factors that trigger these conditions.

Exposure to extreme changes in temperature should be avoided; the room should be kept warm, frequent breaks during summer time for hydration and permission to go to the bathroom as these patients have inability to concentrate the urine.

Medication Authorization for Students

The parent must be given an Authorization for Medication/Treatment Form for all medication that must be given during school hours.

Should a physician prescribe the use of analgesics (non-opioids), he/she needs to complete the Authorization for Medication/Treatment Form and the parents will need to sign and date to give permission for trained school staff to administer the medication to the student at school.  The administration of narcotic medications to students is not permitted in school or on field trips. Parents are responsible for providing all supplies, medication and equipment needed.

Physical Education

Each student with sickle cell anemia has a different capacity to exercise. Persistent physical activity without adequate hydration can trigger a pain episode.  Students should learn to pace themselves. Most students can participate fully in physical activities, however, if the student complains of pain or the inability to continue physical activity, he/she should be allowed to rest and resume activity as tolerated.

The physician should also state any of the student’s limitations, such as avoiding prolonged running or avoiding sports in cold or extremely hot weather.

Recent reports have shown that patients with sickle cell trait are at increased risk for pain, muscle damage in conditions of extreme heat and humidity, which can lead to significant dehydration.  Therefore precautions should be taken for students with sickle cell trait.

Field Trips

Students wishing to participate on a field trip must have a current Authorization for Medication/Treatment Form for any medication needed during a scheduled field trip. If the student needs medication during a field trip the school will assign staff trained in medication administration  to administer the prescribed medication.

Buddy System

The best practice of school personnel for a sickle cell anemia student is to assign the student a buddy who can serve as a runner in times of an emergency. This can be particularly helpful for lunch, physical education, or outdoor activities. Teachers may wish to rotate buddies. Students with Sickle Cell Anemia who are in distress may not be sent to the clinic with a buddy. Have the appropriate person come to where the student is. Whenever there is an emergency situation, the student should not be left alone with other students, but rather in the company of school personnel.

Emergency Care Plan/Crisis Management/Health Concerns

The student’s healthcare provider should indicate on the Authorization for Medication/Treatment Form any emergency care that may be needed by the student. He should write down information that can help prevent unnecessary emergencies such as pain and dehydration.

Staff should be educated with the signs and symptoms of sickle cell anemia. Early detection of symptoms and an appropriate response can prevent the need of a 9-1-1 experience.

Some students are on such potent medications that their personalities may have severe mood swings or their energy levels may vary drastically. Teachers should be made aware of possible side effects of the opioid medications and to whom these observations should be reported.

Hospital Homebound/Dual Enrollment

If a student has a history of frequent absences, then the school should have the parent consider filling out an application for dual enrollment in the hospital homebound program. Most parents will know their child’s condition as to when to return to school without frequent absences.   By planning for dual enrollment at the beginning of the year, a parent can be reassured that the student will be assisted in keeping up with the student’s core courses.
In-service

At the initial meeting with the parent, the school nurse and the classroom teacher, a plan of action should be discussed about in-service on sickle cell anemia for the appropriate school staff and the specific information needed to assist the specific student. The assigned school nurse should plan to provide scheduled in-service education to school personnel including teachers, clerical staff, bus drivers, and food service personnel.

Sickle Cell Services such as in-service and education to help students, teachers and parents become more knowledgeable in sickle cell anemia and sickle cell trait and learn ways to manage different conditions are available at the following hospital districts for Broward County.  The Principal or the assigned school nurse can arrange for in-service by contacting:

  • Memorial Regional Hospital and Joe DiMaggio Children’s Hospital at 800-388-5192 or email: sickle@mhs.net or by visiting www.floridasickle.org
  • Broward Health Chris Evert Children’s Hospital, Sickle Cell Day Treatment
    Center at 954-712-2994 or email:tsaele@browardhealth.org or visit www.browardhealth.org and click on Community Health.

For additional information, visit:
The National Institutes of Health, National Heart, Lung, and Blood Institute http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html
The Sickle Cell Disease Association of America
http://www.sicklecelldisease.org
The principal or school nurse/health designee can arrange for in-service at the school by contacting Coordinated Student Health Services at (754) 321-2272.