International Use

About SID

About Primary Immunodeficiency Diseases

Secondary immunodeficiency (SID) is a weakening of the immune system by a treatment, disease, or environmental factor. There are several subtypes of SID, the most prevalent being secondary antibody deficiency. Common causes include hematologic malignancies such as chronic lymphocytic leukemia, lymphoma, and multiple myeloma, HIV, diabetes, immunosuppressant drugs, malnutrition, severe burns, and cancer treatments, such as chemotherapy.1

SID often involves multiple causes related to both the underlying condition and its treatment, including a growing range of treatments targeting B cells. SID occurs across a wide range of diseases and is therefore important to both primary and secondary care providers.1

The signs and symptoms of SID are similar to symptoms of primary immunodeficiency (PIDD) which mainly include frequent, prolonged, or unusual infections.2,3 

Prevalence

SID is significantly more prevalent than PIDD. Unlike PIDD, however, SID may be reversible in some cases if the underlying cause is resolved.1

Diagnosis

The prompt diagnosis of SID is key in reducing infection burden and is dependent on appropriate screening and an appreciation of risk factors for disease development. SID is diagnosed by reviewing the patient history and conducting a physical exam, as well as laboratory testing to assess antibody levels.1

 

Treatment Options

Treatment Options

Recommended treatment depends on the nature of the deficiency and includes treatment of underlying disease, antibiotics, antivirals, prophylactic vaccination, and immunoglobulin (Ig) replacement therapy. For those patients being treated with Ig, there are subcutaneous or intravenous administration options to prevent infections.1,4

Learn more about the differences between IVIg and SCIg

References

  1. Patel et al. The Expanding Field of Secondary Antibody Deficiency: Causes, Diagnosis, and Management. Front Immunol. 2019; 10:33.
  2. Immune Deficiency Foundation. About Primary Immunodeficiencies. primaryimmune.org/about-primary-immunodeficiencies  Accessed June 30, 2020
  3. Sánchez-Ramón et al. Primary and Secondary Immunodeficiency Diseases in Onchohaematology: Warning Signs, Diagnosis, and Management. Immunol. 2019; 10:586.
  4. Windegger et al. Subcutaneous Immunoglobulin Therapy for Hypogammaglobulinemia Secondary to Malignancy or Related Drug Therapy. Transfusion Medicine Reviews. 2017; 31:45-50.

 

Related Publications

Subcutaneous Immunoglobulin Therapy for Hypogammaglobulinemia Secondary to Malignancy or Related Drug Therapy

Immunoglobulin replacement for secondary immunodeficiency after B-cell targeted therapies in autoimmune rheumatic disease: Systematic literature review

Utilization of intravenous or subcutaneous immunoglobulins in secondary immune deficiency (ULTIMATE): A retrospective multicenter study

The Expanding Field of Secondary Antibody Deficiency: Causes, Diagnosis, and Management

About Iron Overload

About Iron Overload

Several conditions such as beta thalassemia, sickle cell disease, myelodysplastic syndromes, and some forms of cancers can cause anemia. Patients with these conditions require repeated blood transfusions. However, transfused blood contains about 250 mg of iron in each unit, and this excess iron can accumulate in the heart, liver and other major organs causing potential damage.1

It is important to remove the unnecessary iron before damage can occur. Iron reduction is accomplished with chelation therapy, which removes iron from the body pharmacologically with an iron-chelating agent.2

Beta thalassemia is one of the most common causes of transfusion related iron overload. It is an inherited blood disorder in which the body makes an abnormal form or inadequate amount of hemoglobin, the protein in the blood that carries oxygen. The disorder results in excessive destruction of red blood cells, leading to anemia or low red blood cell count.3

Prevalence

Symptomatic beta thalassemia occurs in approximately 1 in 100,000 people globally. It is most common in people from South Asia, the Middle East, Africa, and Mediterranean countries such as Greece and Turkey.3

Symptoms include:3

Fatigue

Weakness

Slow growth

Dark Urine

Abdominal swelling

Diagnosis

Thalassemia is diagnosed via blood tests for anemia, abnormal hemoglobin, and red blood cell shape. Another test known as hemoglobin electrophoresis separates the different molecules in the red blood cells, allowing them to identify the abnormal type. Depending on the type and severity of the thalassemia, a physical examination might also help.4

 

Treatment Options

Treatment Options

Iron Chelation therapy, such as Desferal®, removes the excess iron from the body. It binds to iron and together they leave the body in urine and feces. Desferal® is administered via subcutaneous infusion with an infusion pump like the KORU FreedomEdge or FREEDOM60. It is infused between 3-7 times per week, depending on the amount of iron overload present.1

Slow subcutaneous infusion of Desferal® over a period of 8 to 12 hours is considered effective and especially suitable for outpatients, but treatment can also be given over a period of 24 hours.1 

References

  1. Remacha et al. Guidelines on haemovigilance of post-transfusional iron overload. Blood Transfus. 2013; 11(1): 128-139.
  2. Brittenham et al. Iron-Chelating Therapy for Transfusional Iron Overload. N Engl J Med. 2011; 364(2): 146-156.
  3. National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention. Thalassemia. https://www.cdc.gov/ncbddd/thalassemia/facts.html Accessed April 15, 2020
  4. Healthline. Everything You Need to Know About Thalassemia. https://www.healthline.com/health/thalassemia Accessed Aug 19, 2020

 

Related Publications

Iron-Chelating Therapy for Transfusional Iron Overload

 

Resources

Genetic and Rare Diseases Information Center

Cooley’s Anemria Foundation: Leading the Fight Against Thalassemia

About Dehydration

About Iron Overload

Dehydration is a condition that results when the body loses more fluids than takes in and does not have enough water and other fluids to carry out its normal functions. Anyone may become dehydrated, but the condition is especially dangerous for young children and older adults.1

The most common cause of dehydration for young children is severe diarrhea and vomiting. Older adults have lower volume of water in their bodies and may have conditions or take medications that increase the risk of dehydration. Thus, even minor illnesses, such as lung or bladder infections, can result in dehydration.2

The symptoms of dehydration differ depending on whether the condition is mild or severe. Symptoms of dehydration may begin to appear before total dehydration takes place. Severe dehydration is a medical emergency.

Symptoms of mild to moderate dehydration include:2

Fatigue, dizziness, or lightheadedness

Headache

Dry mouth, increased thirst

Decreased urination

Dry skin

Constipation

 

Treatment

Treatment Options

The only effective treatment for dehydration is to replace lost fluids and lost electrolytes. The best approach to dehydration treatment depends on age, the severity of dehydration and its cause.2

 Subcutaneous hydration, also known as hypodermoclysis, is a useful and easy hydration technique suitable for mildly to moderately dehydrated patients who are unable to take adequate fluids orally or in whom it is difficult or impractical to insert an IV line. The most frequent adverse effect is mild subcutaneous edema that can be treated by local massage or systemic diuretics. Subcutaneous hydration can be administered at home by patients, family members or a nurse.3,4

 Approximately 3 liters can be given subcutaneously in a 24-hour period at two separate sites. Common infusion sites are the chest, abdomen, thighs, and upper arms. The preferred solution is normal saline, but other solutions such as half-normal saline, glucose with saline or 5 percent glucose, can also be used.3,4

References

  1. Dehydration. http://www.nhs.uk/Conditions/Dehydration/Pages/Introduction.aspx Accessed 19 August 2020
  2. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/dehydration/symptoms-causes/syc-20354086 Accessed 19 Aug 2020.
  3. Smith L. Hypodermoclysis with older adults. Nursing. 2014; 44:66
  4. Caccialanza et al. Subcutaneous Infusion of Fluids for Hydration or Nutrition: A Review. J Parent Enteral Nutr 2018; 42: 296-307

 

Related Publications

Hypodermoclysis: an Alternative Infusion Technique

Volume and Site Preferences for Hypodermoclysis: A Review of Clinical Practice Guidelines

Hypodermoclysis: an alternative to I.V. infusion therapy

About Our Flow Rate Controller

About Our Flow Rate Controller

The KORU Precision Flow Rate Controller is intended for use in the administration of 3 centipoise viscosity infusion fluids with the flow rate up to 300 ml per site per hour with the 24G HigH-Flo Subcutaneous Safety Needle Sets. The Flow Rate Controller enables patients to follow drug manufacturer’s Package Insert and the rates prescribed by healthcare professional.

Please note: Precision Flow Rate Controllers are not for sale in the US Market.

Part of the System

Flow Controller

Key Benefits

Patients can administer drug products and change the flow rates as prescribed without changing tubing during the infusion. Patients can administer comfortably at home eliminating the need to go to a hospital or infusion suite.

Patients can infuse HyQvia® monthly, fitting infusion therapy into their schedule. The Freedom Infusion System with the Flow Rate Controller requires no batteries or electricity, assuring self-sufficiency and patient independence.

How to Use

1 Set Controller
1. Set Controller

1. From OFF position, rotate controller to the setting determined by your healthcare professional.

2 Begin Infusion
Begin Infusion

2. Begin infusion by turning pump ON (if using the FREEDOM60®) or by closing the top lid (if using the FreedomEdge®).

3 Set Rate
Set Rate

3. Select flow rate as directed.

4 Emergency
Emergency

4. To immediately stop flow, in an emergency, pull the tubing through the slide clamp.

 

Related Publications

Enabling self-treatment through a novel patient-driven facilitated subcutaneous immune globulin (fSCIg) administration

A New Patient-Driven Facilitated Subcutaneous Immune Globulin (fSCIg) Administration – A Case Study on Successful Self-Treatment