Management of Children With Hematologic or Oncologic Disorders In The School Setting. Scott Rathjen, RN School Nurse, Hays CISD

Transcription

1 Management of Children With Hematologic or Oncologic Disorders In The School Setting Scott Rathjen, RN School Nurse, Hays CISD

2 Scott Rathjen, RN School Nurse, Hays CISD

3 A Subtle but Complicated Student Walks In The Name: Hem N. Onc

4 Objectives Identify Hematology/Oncology students that can be in your school population. Learn how to recognize complications that a current cancer patient may present to you. Classify the many different Heme conditions and triage into 2 groups! Briefly discuss long term issues of cancer survivorship and chronic disease that may affect school.

5 What makes a hem/onc student? Any condition that involves the blood making system and/or involves neoplasms. 3 types of students to be discussed here: the student who is currently being treated for cancer, the hematology condition student, and cancer survivors. Each has their own unique set of issues, but share many of the same too.

6 How many kids have cancer? 15,000 children are diagnosed with cancer every year- over 40 a day with incidence rates on a continuous incline. Average age at diagnosis is 6, which is prime school age. Survival rates are almost 90 percent when taken overall. This leaves a lot of students dealing with this life altering condition.

7 Cancer can affect at any age, but certain cancers are more prevalent at certain ages. In the elementary population, leukemia will be the most prevalent and is the most frequent of all childhood cancers. Central nervous system (CNS) cancers are the next most frequent and affect across all age ranges. Solid tumors account for rest.

8 Lymphomas especially Hodgkin and bone/soft tissue tumors tend to appear more in adolescence. Some of these end up being treated by adult treatment regimens despite overwhelming evidence that pediatric protocols have a much higher survival rate. Hodgkin's has a well documented relationship to Epstein Barr virus (EBV) infection; thus, higher rates are seen among teens and young adults.

9 For the most part all students with cancer can be viewed as one when it comes to side effects of disease and the treatment.*** Surgery remains the main treatment for the majority of neoplasms with the exception of leukemia/lymphomas Chemotherapy and radiation are adjunct treatment with the former being systemic and latter local control. They create many of the complications that may arise.

10 Complications that may present to the nurse s office Chemotx has many different mechanisms; thus many different side effects. However, a couple are very common across the board. Bone marrow suppression- anemia, thrombocytopenia and leukopenia. The nadir is average 5-12 days post tx. This is usually much more intense in leukemia tx, although lessened when in maintenance. GI symptoms to include nausea, vomiting, mucositis, diarrhea and constipation with some drugs very emetic inducing.

11 Bone Marrow Suppression Anemia - RBC s are the least affected of this group due to their lifespan. They tend to decline more gradually, but in return increase more gradually too. Transfusion guidelines vary but usually will be allowed to drop below 7-8 gm HGB unless symptomatic. Many are in that 7-10 range.

12 Bone Marrow Suppression Fatigue is the most common complaint and this will be the most likely reason for nurse office visit. Is this fatigue just the overall fatigue of tx or is the student showing signs of anemia. HR, resp rate/sob and general pallor may help to differentiate. What was last known CBC and current tx regimen

13 Bone Marrow Suppression Thrombocytopenia Can be quite aggressive, but transfusion guidelines are generally <20,000 as they have faster recovery after nadir. Many are going to be in the thousand range. This is where good communication with teachers and relationship with student come to play - any head bump should be assessed considering risk.

14 Bone Marrow Suppression Neutropenia The most common serious complication arising from chemotx. Leukemia again affected more and recovery meds (neulasta) not used. Neutrophils are the key here, as they are our front line response to infection. Most frequent admission for all oncology patients is febrile neutropenia. Neutropenia is leading cause of severe complications and death after disease itself (sepsis, pneumonias, typhlytis.)

15 Bone Marrow Suppression Neutropenia In neutropenia our immune response is compromised. The body defends itself by thermoregulation in response to any threats. Fever > F with accompanied neutropenia (ANC<500) is pretty much an across the board automatic admission to hospital for supportive therapy. This, again, is when good communication IS KEY to a good outcome. Remember that with neutropenia there are no reliable signs of infection, except fever.

16 GI Complications N/V is THE most common side effect of chemo and radiation therapy. May occur at anytime (more frequent in CNS disease disease versus side effect). Chemical receptor preventative medicine (Zofran, Kytril) HIGHLY recommended to be available. Adjuncts include corticosteroids, antiemetics, cannabinoids. I have found peppermint and ginger altoids to be extremely helpful.

17 Mucositis Diarrhea Constipation Loss of mucosal lining ANYWHERE in GI tract. May present with N/V, diarrhea or visible open sore in in oral mucosa. Key thought about mucositis is that it mainly occurs when the child is also neutropenic. Diarrhea may be the result of above or due to drug tx. Constipation frequent with certain drugs but may be sign of much more serious complications.

18 Drugs most common to cause GI issues Platinum based drugs. Cisplat and Carboplat Alkylating agents. Cytoxan and Ifosphamide Anthracyclines. Doxo, Dauno and Ida rubicins. Plant Alkaloids-vp16 vm26 High dose Methotrexate Vinca alkaloids--constipation All can be issue depends on the patient Cytoxan and methotrexate are frequently used in autoimmune disease treatment in lower doses.

19 CNS Disease CNS disease presents a whole different set of problems to assess, from the disease process to the differences in treatment (radiation is a standard). CNS symptoms may present anywhere across the neuro sensory/ cognitive spectrum. Having a good baseline assessment of a student with CNS involvement is crucial to being able to assess any complaints that arise.

20 Radiation Therapy While not used as widely as in the adult population, certain sarcomas and most CNS tumors will be treated to some extent with radiation therapy. The two most common complaints are N/V and fatigue. With Cranial radiation, N/V can be extreme, prolonged, and difficult to treat. Radiation can cause a local reaction similar to sunburn. Avoid any creams to affected area.

21 Other Considerations Vinca alkaloids and Platinum based drugs frequently cause neuropathies. Anthracyclines can be cardio toxic. Alkylating drugs and Platinum can cause hearing loss. Bleomycin can be pulmonary toxic. Corticosteroids are front line treatment in Leukemia and CNS tx and frequently used as an adjunct to antiemetics in others. All side effects come into play here.

22 So what can you do? KNOW THE STUDENT!!! Speak with the parents, as they are very in tune to process. Ask for copy of the treatment protocol to know where in the treatment they may be on any given day. Know the drugs they are taking and their complications, as you would for any chronic student. Reassure that your goal is to help the student SAFELY continue school to their maximal potential. Call for any fever so parents aware so they can make the MD office aware.

23 The Hematology Patient Hematological conditions are any that arise from the RBC, WBC, and platelet/coagulation cascade. These can be either inherited (chronic) or acquired (acute). We will look at the two most common inherited conditionsthe hemolytic anemias and the coagulopathies. Acquired conditions can be seen in same view, just usually short term condition. WBC conditions fall under immunology along with auto immune diseases- think if the immune system is not operating properly-infection becomes key focus.

24 Hemolytic Anemias - sleepers This refers to a dysfunctional RBC production in the cell itself or the hemoglobin it carries. Cells are defective, have shorter life span and production can t keep up with hemolysis. Either way the oxygen delivery system is compromised. Common hemolytic anemias include Sickle Cell Disease (SSD), the thalassemias, spherocytosis, G6PD and pyruvate kinase deficiency. In addition inherited disorders that affect bone marrow can be grouped here too (Diamond Blackfan and Fanconi anemia). Acquired hemolytic anemias arise more as an auto immune response.

25 Hemolytic Anemias - sleepers The two most common complaints you will be presented with are fatigue and abdominal pain. When assessing fatigue, the first thing I focus on are current illness symptoms. Any infectious process will speed up the hemolysis. Heart and respiratory rate are better assessments than pulse ox, but knowing baseline will help (SSD).

26 Hemolytic Anemias - sleepers Stomach aches are more likely to be caused by spleen versus GI disturbance. As hemolysis increases, so will pressure in the spleen. Many of these students spleens can be easily felt with a gentle abdominal exam. Knowing baseline can be a great addition to being able to assess current complaint. Jaundice may be another assessment tool to assess hemolysis. Again baseline is important, as most will exhibit some sign of jaundice.

27 Sickle Cell Disease (SSD) SSD is unique in the hemolytic anemias, as it can trigger a cascade (sickle crisis) that kind of feeds on itself. Hemoglobin S is a poor carrier of oxygen. This leads to proteins being produced that misshape the cell.

28 Sickle Cell Disease This is a two edged sword- not only does this cell not deliver O 2, it can disrupt the flow through smaller vessels. This leads to tissue hypoxia which in turn can lead to more cells sickling. Any and all stressors will increase the sickle process with pain chemical response a key trigger.

29 Sickle Cell Disease Prevention is the key to good outcomes. A pain management plan is highly encouraged. Respond quickly to any pain complaints. Help the student manage all means of stressors, both physical and emotional. Headaches need to be followed closely. Keys are pain control, hydration and rest.

30 3 key concepts to take away Be aware of an on-setting illness- this may lead to more rapid hemolysis. Tummy aches may be an indicator of ongoing hemolysis as spleen becoming engorged. Be conscious about the seriousness of any abdominal trauma.

31 Coagulopathies - bleeders Again these may be inherited or acquired with the same principles involved; former is chronic latter is usually acute. Can be dysfunction of platelets, coagulation factors or a combination.

32 Coagulopathies - bleeders The most common inherited are the factor deficiencies: Hemophilia A (VIII) Hemophilia B (IX) B Leyden Von Willebrand Disease (VWD) The most common acquired is ITP (Immune/Idiopathic Thrombocytopenia purpura).

33 Coagulopathies - bleeders Hemophilia both A and B are primarily seen in males as X linked, but can be seen in females if mother is a carrier and dad has. Factor 7 deficiency is seen in both as autosomal recessive. VWD is seen across both sexes although more likely diagnosed in females. VWD is most common and may affect 1-2 % of the population. It is thought that 70 percent people go undiagnosed with VWD. Factor deficiency diseases are classified as mild moderate or severe depending on how much factor can be produced.

34 Coagulopathies - bleeders Key here is bleeding and limiting the process. Our main concern should always be the head. Reversing the bleed is key to limiting damage done to joints long term. Depending on the severity of the disease, you may be able to control with aggressive first aid and or RICE for suspected joint bleeds. Most common to see in clinic will be epistaxis. Mod-severe will probably require adjunct treatment.

35 Coagulopathies - bleeders Bleeding in VWD may be controlled with DDAVP (stimate) and it is used in some hemophilia A cases. Prior testing will confirm if effective tx (type 1 or any factor 8 available). It is usually given intranasal and can be easily used in school setting. Amicar is a antifibrinolytic and is frequently used for epistaxis. For factor dependent students, rapid notification so factor can be given is critical. Many students by age 7 are taught to self infuse at first signs of injury or pain in joints.

36 ITP: Idiopathic/Immune Thrombocytopenia Purpura A platelet disorder where the immune system produces auto antibodies to platelets. Production normal but count severely decreased due to splenic sequestering. Platelets that are there tend to be large and some evidence of increased functionality. Treatment is usually corticosteroids and or IVIG. Usually self limiting.

37 Coagulopathies - bleeders Regardless of what s causing the bleed the goal is to limit its damage. This is done with rapid assessment and knowledge of students condition. Stay calm and power on; after all, its only blood.

38 Cancer survivors and Chronic disease More and more children are surviving devastating diagnoses every year. Survival rates have peaked just below 90 percent in pediatric cancer. For these children the battle is just changing scenery from the hospital to the outside world. Conditions from treatment may become chronic life long issues for them.

39 Children are very resilient both physically and emotionally, if allowed to be. But, certain things can not be ignored and will have to be faced as they come along. Understanding the diagnosis and the treatment undergone will help you guide this student to a successful period of their life called school.

40 Some examples of things they face are cognitive changes due to cranial radiation or intrathecal therapy, the after effects of surgery to remove the cancer - be it wilms (kidney gone), retino (eye gone), or osteo (limb most likely gone), cardiac, pulmonary, and hearing changes due to certain therapies used, radiation recall triggered by the sun, secondary neoplasms from tx, the list goes on

41 Thank You Very Much from all of them, to all of you All students with a chronic disease need a little extra TLC. You can t do this without understanding their disease process Be supportive, be caring and most of all be the student s advocate. They have marched a road to hell, now all they ask is to be a kid again.

42 Acknowledgements COG NHLBI APHON WFH Dana-Farber Hospital

43 QUESTIONS?