Hemophilia

Hemophilia Nursing Diagnosis Interventions and Care Plans

Hemophilia NCLEX Review Care Plans

Nursing Study Guide on Hemophilia

Hemophilia is a rare blood disorder characterized by prolonged clotting. It is usually an inherited condition affecting men.

It is caused by inadequate amount of either the clotting factor VIII or factor IX. These clotting factors are blood proteins that bind with platelets to form clots when there is bleeding.

Hence, low levels of either of these clotting factors can cause prolonged clotting time which lengthens bleeding time.

The severity of bleeding depends on how low the levels of clotting factors are.

There are several types of hemophilia.

The two most common types are Hemophilia A (classic hemophilia) and Hemophilia B (Christmas Disease).

Hemophilia A is characterized by the lack or low levels of clotting factor VIII.

Hemophilia B is characterized by the lack or low levels in clotting factor IX.

Signs and Symptoms of Hemophilia

  • Excessive bleeding from accidental cuts or planned procedures that may involve bleeding
  • Bruising
  • Bleeding unusually after vaccinations
  • Bleeding into the joints which may be characterized by tightness and/or pain often in the knees, elbows, and ankles.
  • Presence of blood in the urine and / or stool.
  • Unexplained epistaxis or nosebleed that may be hard to stop.
  • In babies, unexplained irritability
  • Bleeding in the head of the newborn after a difficult delivery may also be noted.

Causes and Risk Factors of Hemophilia

Hemophilia is a genetic disorder wherein there is mutation or changes in the genes that determine the body’s clotting factors. The mutation causes inadequate or total lack of the clotting factor VIII or IX. It is carried in the X chromosome. Most of the case of hemophilia is inherited although about 30% of the reported cases show no prior family history of the disease. 

The risk factors include:

  1. Gender. Hemophilia occurs mostly in men because it is carried by the X chromosome. Women are usually the carriers of the defective genes and can pass it onto their offspring. However, women rarely develop signs and symptoms of the disease, if they ever get the defective X chromosome. Their symptoms will rely mostly on the level of their clotting factors.
  2. Family History. Having a family history of hemophilia serves as a risk factor for developing the disease.

Complications of Hemophilia

  1. Internal bleeding. Bleeding internally is more difficult to take note of and therefore address in hemophilia. It can lead to serious organ damage and severe pain.
  2. Damage to joints. Joints mostly experience friction therefore making them prone to bleed in a person suffering from hemophilia. Bleeding in between joints can cause serious pain, arthritis, and permanent damage to the joints.
  3. Infection. Blood transfusion is mostly required for people suffering from hemophilia. This brings forth the possibility of getting infections.
  4. Adverse reaction to treatments. In some cases of hemophilia, the immune system gets compromised causing the body to react differently or inadequately to treatment.

Diagnosis of Hemophilia

The diagnosis of hemophilia can be made by testing the levels of clotting factors in the blood.

  • Umbilical cord blood test – for individuals with family history of haemophilia; used to determine the levels of clotting factors VIII and IX. However, a low level of clotting factor IX immediately after birth may not necessarily suggest hemophilia as it isn’t fully developed until the newborn is about six months old. 
  • Complete Blood Count – hemoglobin levels can be low in a person with hemophilia due to the bleeding
  • Activated Partial Thromboplastin Time (APTT) test – blood test to measure the time it takes for the blood to clot. A person with hemophilia will have longer time for their blood to clot.
  • Prothrombin Time – to assess the time it takes for blood to clot. It measures the ability of the clotting factors I, II, V, VII, and X. An individual with hemophilia will mostly test normal in these parameters.
  • Fibrinogen test – to measure the blood’s ability to form clots. It is usually performed in conjunction with the other blood clotting test. Fibrinogen also refers to clotting factor I.
  • Clotting factors test – also called factor assays; a test performed to diagnose bleeding disorders. It shows the type of hemophilia and its severity.

Treatment of Hemophilia

  1. Blood Transfusion. Since hemophilia involves a low level or total lack of a blood product, replacing it is the most common treatment given. Blood transfusion is performed when there is ongoing bleeding that can’t be stopped, or it may be given regularly to prevent bleeding in the future.
  2. Medications. Pharmacologic treatments related to haemophilia include:
  3. Desmopressin. It is a hormone that can either be injected or given as per nasal spray. It is given to stimulate the production of clotting factors.
  4. Clot-preserving medications. These medications can reduce the likelihood of clot breakdown.
  5. Fibrin sealants. These are mostly useful in dental therapy. It is a medication that can be applied directly on wounds to promote clotting.
  6. Physical therapy. Therapy can ease pain involved in joints caused by bleeding in these areas.
  7. First aid for minor injuries. Immediate treatment is necessary to stop any bleeding or prevent possible bleeding.
  8. Vaccination. It is recommended for people with hemophilia to get hepatitis A and B vaccines as they will normally receive regular blood transfusion.

Nursing Care Plans for Hemophilia

Nursing Care Plan 1

Nursing Diagnosis: Risk for Bleeding related to inadequate or total lack of the clotting factor VIII or IX

 Desired Outcome: To prevent any bleeding episode.

InterventionsRationales
Assess the patient’s vital signs and perform a focused physical assessment, looking for any signs of bleeding.Early signs of bleeding include gum bleeding, epistaxis, and unexplained bruises. Low blood pressure, low temperature, and dizziness may result from excessive bleeding.
Obtain blood samples and monitor coagulation levels (INR, PT, and PTT).To measure the risk of bleeding by knowing the coagulation levels of the patient.
To check for the need for blood transfusion.
Administer blood transfusion as prescribed.Blood transfusion is performed when there is ongoing bleeding that can’t be stopped, or it may be given regularly to prevent bleeding in the future.
Administer medications for hemophilia as prescribed.Pharmacologic treatments related to haemophilia include: Desmopressin. It is a hormone that can either be injected or given as per nasal spray. It is given to stimulate the production of clotting factors. Clot-preserving medications. These medications can reduce the likelihood of clot breakdown.Fibrin sealants. These are mostly useful in dental therapy. It is a medication that can be applied directly on wounds to promote clotting.  

Nursing Care Plan 2

Nursing Diagnosis: Fluid Volume Deficit related to blood volume loss secondary to bleeding as evidenced by hematemesis, HB of 70, skin pallor, blood pressure level of 85/58, and lightheadedness

Desired Outcome: The patient will have an absence of bleeding, a hemoglobin (HB) level of over 100, blood pressure level within normal range, full level of consciousness, and normal skin color

InterventionsRationales
Assess vital signs, particularly blood pressure level.Hypovolemia due to bleeding may lower blood pressure levels and put the patient at risk for hypotensive episodes that lead to shock.
Commence a fluid balance chart, monitoring the input and output of the patient. Include episodes of vomiting, gastric suctioning, and other gastric losses in the I/O charting.To monitor patient’s fluid volume accurately.
Start intravenous therapy as prescribed. Electrolytes may need to be replaced intravenously.     Encourage oral fluid intake of at least 2000 mL per day if not contraindicated.To replenish the fluids and electrolytes lost, and to promote better blood circulation around the body.
Educate the patient (or guardian) on how to fill out a fluid balance chart at bedside.To help the patient or the guardian take ownership of the patient’s care, encouraging them to drink more fluids as needed, or report any changes to the nursing team. 
Administer blood transfusion as prescribed.To increase the platelet count and hemoglobin level and manage hemophilia, anemia and hypovolemia related to bleeding.

Nursing Care Plan 3

Nursing Diagnosis: Risk for Injury related to abnormal blood profile

Desired Outcome: The patient will be able to prevent injury by means doing activities that can be done without spending too much energy, and by modifying environment to adapt to current capacity.

InterventionRationale
Assess the patient’s energy level and fatiguability, as well as his/her usual activities of daily living (ADLs).To explore the patient’s energy levels and how these can affect ADLs. To gain useful information before modifying the patient’s environment.  
Place the bed in the lowest position. Place the call bell within reach (if there’s any), and keep the visual aides and patient’s phone and other devices within reach.To prevent or minimize injury of the patient.
Promote adequate lighting in the patient’s room.To promote safety measures and support to the patient in doing ADLs optimally.
Ensure that the floor is free of objects that can cause the patient to slip or fall.To promote safety measures and support to the patient in doing ADLs optimally.

Nursing Care Plan 4

Nursing Diagnosis: Fatigue related to bleeding and blood loss secondary to hemophilia, as evidenced by verbalization of lack of  energy, verbalization of tiredness, and generalized weakness

Desired Outcome: Post surgery, the patient will verbalize improved energy levels and will demonstrate active participation in necessary and desired activities.

InterventionsRationales
Ask the patient to rate fatigue level (mild, moderate, or severe fatigue).     Assess the patient’s activities of daily living, as well as actual and perceived limitations to physical activity. Ask for any form of exercise that he/she used to do or wants to try.To create a baseline of activity levels, degree of fatigability, and mental status related to fatigue and activity intolerance.
Encourage progressive activity through self-care and exercise as tolerated. Explain the need to reduce sedentary activities such as watching television and using social media in long periods. Alternate periods of physical activity with rest and sleep.To gradually increase the patient’s tolerance to physical activity.
Encourage the patient to follow a low cholesterol, high caloric diet. Refer to the dietitian as needed.To increase energy levels.
Teach deep breathing exercises and relaxation techniques.   Provide adequate ventilation in the room.To allow the patient to relax while at rest. To allow enough oxygenation in the room.
Refer the patient to physiotherapy / occupational therapy team as required.To provide a more specialized care for the patient in terms of helping him/her build confidence in increasing daily physical activity.

Other possible nursing diagnoses:

  • Activity Intolerance
  • Powerlessness

Nursing References

Ackley, B. J., Ladwig, G. B., Makic, M. B., Martinez-Kratz, M. R., & Zanotti, M. (2020). Nursing diagnoses handbook: An evidence-based guide to planning care. St. Louis, MO: Elsevier.  Buy on Amazon

Gulanick, M., & Myers, J. L. (2017). Nursing care plans: Diagnoses, interventions, & outcomes. St. Louis, MO: Elsevier. Buy on Amazon

Ignatavicius, D. D., Workman, M. L., Rebar, C. R., & Heimgartner, N. M. (2018). Medical-surgical nursing: Concepts for interprofessional collaborative care. St. Louis, MO: Elsevier.  Buy on Amazon

Silvestri, L. A. (2020). Saunders comprehensive review for the NCLEX-RN examination. St. Louis, MO: Elsevier.  Buy on Amazon

Disclaimer:

Please follow your facilities guidelines and policies and procedures.

The medical information on this site is provided as an information resource only and is not to be used or relied on for any diagnostic or treatment purposes.

This information is not intended to be nursing education and should not be used as a substitute for professional diagnosis and treatment.

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