CASE STUDIES

Initial visit with Dr.Wasserman at age 8

Laboratory findings

• May 26, 2015: IgG low (424 mg/dL [normal 673-1734 mg/dL*])
• August 3, 2015: IgG low (417 mg/dL [normal 673-1734 mg/dL*]), pneumonia antibodies 7/14 positive, pneumonococcal vaccine given
• October 19, 2015: IgG low (405 mg/dL [normal 528-2190 mg/dL*]), pneumonia antibodies 10/14 positive, pneumonococcal vaccine given
• IgA and IgM were normal
• Anti-S. pneumoniae antibody was marginally maintained but good response to pneumococcal vaccine
• CBC and chemistry panel were normal

Diagnoses: Hypogammaglobulinemia

CBC=complete blood count.

*Ranges vary because testing was performed at 2 different laboratories

Initial treatment at age 8

• Initially treated with a loading dose of 20% SCIG 5 g/d for 4 days
• Maintained with 20% SCIG 5 g/25 mL/once a week in 1 site
• Infusion time: 2.5 hr

Initial SCIG treatment results

• On day 4 during the initial infusion period, Hadlie Jo developed a typical headache that was treated with ibuprofen. Subsequently, headaches were an occasional problem
• Continued to experience malaise and fatigue for 1 day after infusions
• Local AEs of burning and itching with each infusion
• Systemic reaction of headache and vomiting 1 year after treatment initiation
• 1 episode of sinusitis; 1 or 2 URIs per year. No hospitalizations or surgeries
• Multiple trips to the physician's office to monitor AEs

CUVITRU treatment plan

Current regimen: 12 g/60 mL/every 2 weeks at 2 sites
Infusion time: <1 hour
Rate: 35 mL/h/site

• Initiated at 5 g/wk at a rate of approximately 19 mL/hr/site in a single site
• After 2 infusions, Hadlie Jo transitioned to 10 g every 2 weeks at a rate of approximately 35 mL/hr/site
• Approximately 6 months later, her dose increased due to a 20% weight gain
• Because Hadlie Jo spends less time infusing, she has more time for dance practice and other activities

CUVITRU treatment results^*

• No reported local side effects (including itching and burning at the infusion site)
• No systemic side effects (no malaise or fatigue following infusions)
• Her doctor reviewed the most common side effects: local adverse reactions (eg, mild to moderate pain, erythema, and pruritus) and systemic adverse reactions (eg, headache, nausea, fatigue, diarrhea and vomiting) and is monitoring for these reactions and other possible outcomes (eg, thrombosis, hemolysis).

URI=upper respiratory infection

*Results represent one patient's experience. Individual results may vary. Patients should consult their physician as needed. Click here for reported side effects with CUVITRU in clinical trials.

From her early childhood, Julie struggled with being sick and was constantly in the hospital for asthma and other health issues.

• Prescribed allergy and gamma globulin shots in 9th grade by an ENT
• Discontinued shots after freshman year of college
• Experienced declining health after giving birth to second child that left her unable to work
• Ongoing sinus infections and strep throat led to multiple operations
• Consulted Dr. Ankmalika Gupta at age 52 and was diagnosed with PI

Initial evaluation and diagnoses at age 52

Relevant medical history

• Recurrent severe sinopulmonary infections, osteomyelitis, skin and ear infections
• Multiple surgeries and debridements were required
• Comorbidities: asthma, allergic rhinitis, atopic dermatitis

Laboratory findings

• Normal lymphocyte subsets
• CH50 normal
• Normal tetanus and diphtheria antibody titers
• IgG: 1228 mg/dL
• Poor response to polysaccharide antigens:
  • Impaired response to Haemophilius influenzae vaccine
  • Poor memory response to Streptococcus pneumoniae vaccine
  • No pre-vaccination titers were done

Diagnosis: Specific antibody deficiency (SAD)

Initial treatment results

• Initially treated with 10% IVIG every 4 weeks for ~3 years
• Infusions would typically take over 6 hours
  • Slow infusion rates in an attempt to minimize systemic adverse effects but would prolong infusion time
  • She also required pre-medications such as antihistamines as well as NSAIDS and IV hydration for migraines
  • Despite these efforts, she would still experience 1 week of complete down time following every infusion due to debilitating fatigue and malaise
• Patient responded to IVIG treatment (IgG: 997 mg/dL [normal 700-1600 mg/dL]) and experienced a decrease in serious infections (~1 per year), none of which required hospitalization
• However, after years of treatment with IVIG, her veins collapsed and she and her healthcare provider discussed alternate IG treatment options

CUVITRU treatment plan

Current regimen: 14 g/70 mL/every 2 weeks at 2 sites
Infusion time: <1 hour
Rate: <55 mL/h/site
Volume: <35 mL/site

• CUVITRU was chosen for Julie because of the subcutaneous delivery of IG and the reduction in the time she spent infusing per session
• She underwent training from a healthcare professional on how to self-administer CUVITRU at home with the HelloCUVITRU program
• She initially received 1 weekly infusion, but due to the higher concentration of CUVITRU and her willingness to attempt increased volumes per site, she opted for less frequent dosing

CUVITRU treatment results^*

• Similar to her response to IVIG, she continues to respond to SCIG treatment and has had no further hospitalizations due to infections
• Uneventful post-surgical outcomes following oral and facial surgery
  • Favorable wound healing without infection complications
• Based on her response to SCIG treatment and the flexible administration of CUVITRU, Julie is optimistic she may return to part-time work
• She experienced mild redness at the infusion site for the first few treatments and had no other discomfort. For subsequent infusions she did not experience local reactions
• She has no experienced systemic reactions with her SCIG treatment
• Her doctor reviewed the most common side effects: local adverse reactions (eg mild to moderate pain, erythema and pruritus) and systemic adverse reactions (eg headache, nausea, fatigue, diarrhea, and vomiting) and is monitoring for these reactions and other possible outcomes (eg thrombosis, hemolysis)

*Results represent one patient’s experience. Individual results may vary. Patients should consult their physician as needed. Click here for reported side effects with CUVITRU in clinical trials.

Walter experienced recurrent infections such as sinusitis from the age of 16.

• Underwent a tonsillectomy and adenoidectomy, along with sinus surgery
• At 54, symptoms began worsening and pneumonia at increasing incidence
• Suffered 4 bouts of pneumonia over a 6-month period

Initial evaluation and diagnoses at age 54

2013-14

• Intermittent sinusitis, worsening asthma
• Started Xolair^® (omalizumab)
• Chest CT showed mild pulmonary fibrotic change

2014-15

Dramatic decline in health

• Almost monthly visits for infections and antibiotics
• Nebulized tobramycin for Pseudomonas
• Nebulized hypertonic saline
• Inhaled steroids
• Laboratory findings:
  • IgG, A, M, E normal
  • Hypersensitivity Pneumonitis panel – eosinophil count, ANCA, CF DNA, histo/blasto normal
  • Chest CT showed nodular changes in lungs
  • Cultures 4-6x/years – growing Serratia, Moraxella, Pseudomonas

Diagnoses: Recurrent pneumonia, recurrent sinusitis

Evaluation and diagnoses at age 57

2016

Presented with bronchiectasis/recurrent pneumonia

• Laboratory findings:
  • IgG subclasses mildly low at 399 mg/dL
  • Pre/post-immunization titers:
    • Suboptimal response to pneumococcal vaccine – only 7/14 titers protective
    • No response to diphtheria vaccine
    • Only a doubling of tetanus titer
• Comorbidities: allergic rhinitis, asthma, diastolic dysfunction, GERD, eczematous lesions on abdomen

Diagnoses: Immunodeficiency with subclass deficiency and impaired
response to antigen stimulation, lung nodules, and dermatitis

Initial SCIG treatment in July 2016

• Regimen: 20% SCIG 12 g/60 mL once a week at 4 sites
• Infusion time: 2 hours

Initial SCIG treatment results

• Oxygen saturation: 91%
• Sputum: heavy-growth Pseudomonas
• On chronic suppressive antibiotic: oral azithromycin 3x/wk
• Chronic cough producing up to 1 cup (240 mL) mucus/plugs daily
• Experienced side effects, including a thigh rash (not at infusion sites)
• After the second infusion, developed eczematous spots on abdomen (not at infusion sites)