Nursing Care Plan (NCP) for Hemophilia

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Outline

Pathophysiology

Hemophilia is a genetic bleeding disorder that is characterized by a deficiency of clotting protein. Patients with hemophilia experience longer bleeding time than others because their blood clots much slower. The amount of clotting protein available in the blood determines the severity of hemophilia and categorizes it into three levels: mild, moderate and severe. Complications of the disease include bleeding into the joints, hemorrhage into the central nervous system or vital organs, and aspiration from bleeding into the airways.

Etiology

Hemophilia is genetically (X) linked with females being the carriers, and males being affected by the disease. There are two types of hemophilia. Type A (classic) is caused by a deficiency of Factor VIII (8) clotting protein and is the most common, affecting 1 in every 10,000 males.  Type B (Christmas disease) is caused by a deficiency of Factor IX (9) and affects 1 in every 30,000 males.

Desired Outcome

Patient will be free from bleeding complications; patient will be educated on how to prevent injury and bleeding; patient will have optimal physical mobility

Hemophilia Nursing Care Plan

Subjective Data:

  • Irritability (infants)
  • Pain or tightness in the joints
  • Sleepiness or lethargy
  • Double vision

Objective Data:

  • Large or deep bruises
  • Unexplained nosebleeds
  • Blood in urine or stool
  • Excessive bleeding from cuts or after dental work

Nursing Interventions and Rationales

  • Assess patient for signs of bleeding, cuts, scrapes, bruises, swollen joints

 

Patients often experience deep bruising from minimal contact or minor injuries. The deep bruising may lead to bleeding into joint spaces and vital organs.

 

  • Assess patient for evidence of pain (non-verbal cues)

 

Patients may report painful joints or aching muscles; younger children may not know how to express pain and exhibit symptoms through guarding or irritability and fussiness.

 

  • Provide passive ROM exercises

 

This is not advised during acute phase or with active bleeding, but encouraged when patient’s condition is stable to maintain or improve joint and muscle mobility

 

  • Provide assistive devices as required

 

Physical deformity of joints may occur due to bleeds into joint, which may cause limited mobility. Provide assistance and devices as necessary.

 

  • Initiate bleeding precautions per facility protocol

 

Small and simple injuries may cause excessive bleeding. Client should avoid blade razors (use electric razors), use soft-bristle toothbrush, and avoid blowing nose during nosebleeds

 

  • Monitor coagulation tests; hemoglobin and hematocrit levels

 

Monitor for effectiveness of interventions and therapeutic levels of medication and supplements

Expect bleeding times to be reduced from the client’s baseline

 

  • Anticipate need and administer blood products as required

 

Excessive blood loss may require transfusions; have O-neg blood immediately available in case of hemorrhage

 

  • Avoid unnecessary invasive tests and techniques

 

Avoid heel or finger sticks and IM injections if possible

Be prepared for excessive bleeding with administration of vaccines (opt for subcutaneous routes if available).

Apply pressure to site for several minutes to allow time to clot

 

  • Administer medications orally as available; administer replacement clotting factors as necessary

 

Replacement of clotting factors is the primary treatment for hemophilia; other supplements, antibodies and antifibrinolytics may be required

 

  • Educate patient and caregivers on safety and prevention of injury

 

Prevention of injury is most important; patient should avoid physical contact sports and high-risk activities; use soft toothbrush, give appropriate (not sharp-edged) toys, use helmets and padding for sports, supervise playtimes

Writing a Nursing Care Plan (NCP) for Hemophilia

A Nursing Care Plan (NCP) for Hemophilia starts when at patient admission and documents all activities and changes in the patient’s condition. The goal of an NCP is to create a treatment plan that is specific to the patient. They should be anchored in evidence-based practices and accurately record existing data and identify potential needs or risks.


References

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Transcript

We are going to focus on a care plan based on hemophilia. The pathophysiology behind hemophilia is that it’s a genetic bleeding disorder that is characterized by a deficiency in clotting protein. Patients with hemophilia experience longer bleeding times than others because their blood clots much slower. The amount of clotting protein available in the blood determines the severity of the hemophilia. It is categorized into three different levels. There’s mild, moderate, and severe complications of the disease including bleeding into the joints, hemorrhage into the central nervous system or the vital organs, and aspiration from bleeding until the air res. Some of the things we want to consider as nurses is we want to assess the patient for bleeding. We want to get those bleeding precautions initiated. We want to assess the stool and urine for blood, and we want to assess and manage pain. Our desired outcome for this patient population is that the patient is going to be free of infection. We’re going to manage and reduce the pain and inflammation, regain optimum mobility and prevent complications from bleeding. 

These patients with hemophilia are very irritable during their events; that can mean a variety of things, but with this particular patient population, they are irritable. They also have pain and tightness in their joints. They always complain of pain or tightness, and that’s primarily because these patients are susceptible to blood pooling into their joints. They’re typically sleepy and lethargic. They have double vision. And some of the objective data that we might observe are deep or large bruises. These patients tend to have unexplained nosebleeds. They may have blood in the urine or stool, and they may have excessive bleeding from cuts or after dental work. So excessive bleeding. Okay. As nurses, there are a few interventions that we can do. The first thing we want to look at is we want to assess the patient for any type of signs of bleeding, whether it be by cuts, scrapes, bruises, or in it’s swollen joints. Deep bruising may lead to bleeding in joint spaces and other vital organs. So, we want to do an assessment of that. We want to assess for bleeding. 

The next thing that we could do is initiate those bleeding precautions because these patients, if left in the right circumstances will bleed. So, we want bleeding precautions. A small injury that will cause a normal person no harm can make this patient bleed excessively. So we want to be careful of that. We also want to avoid any unnecessary invasive techniques. So this kind of leads into the blending precautions. We want to avoid heel and finger sticks, we want to avoid IM injections, if at all possible. Be prepared for excessive bleeding. When we administer vaccines, we can try to opt for sub Q routes when those are available as well. Be sure that with these patients, that we apply pressure to any site for several minutes to allow time to clot, because they take a lot longer to do that. 

One of the treatments for hemophilia is more of a therapeutic and that is when we administer replacement clotting factors. So, that’s the next goal of ours; we want to replace clotting factors and that’s just an infusion that we can give IV. Replacing clotting factors is the primary treatment for hemophilia. Other supplements, antibodies, and antifibrinolytics may be required for this patient population. We’re going to monitor the coagulation tests. We’re going to look at their hemoglobin and hematocrit levels because we need to know if this patient is bleeding. We need to know where they’re bleeding. So, we want to do hemoglobin and hematocrit. We want to monitor the effectiveness of the interventions and the therapeutic levels of the medication; expect bleeding times to be reduced from the patient’s baseline. So, we’re going to also look at the PT, PTT, INR. Just like I said, different bleeding and coagulation blood levels that we’re going to monitor. 

Finally with this patient, let’s focus on pain. We are going to assess the patient for evidence of pain, but very specific pain. We are looking at pain that is showing up in the joints or muscles. Patients often report very painful joints or aching muscles. And again, that’s due to the blood that pools, maybe they bumped their elbow somewhere. And where on the surface, you may not see anything internally. There could be blood pooling into those joints causing pain. So the key points that I want you all to focus on for hemophilia is that hemophilia is a genetic bleeding disorder characterized by a deficiency in the clotting proteins. The subjective data that the patient is going to present is they’re going to be irritable. They’re going to have some joint pain. They may be sleepy,lethargic, and they may have some double vision. 

Well, we’re going to be able to see in the patient, our objective data that we’re going to have, is there is going to be some blood and urine or stool. They may have some large or deep bruises and some unexplained nosebleeds, small and simple injuries. We’re going to put them at a bleeding risk. I’m going to put them on bleeding precautions, because like I said, small and simple injuries can cause excessive bleeding, bleeding precautions, and we want to avoid any unnecessary lab sticks or insertions. We want to make sure we replace their clotting factors. Remember clotting factors are the only way to treat these patients. It’s the main treatment that these patients will receive. Remember that there are two different types of hemophilia and depending on which type A or type B it would depend on what clotting factor is needed. And lastly, we love you guys; go out and be your best self today. And, as always, happy nursing.

 

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