Abstract

Background: The barriers to adherence with chelation therapy for chronically transfused and iron overloaded patients with sickle cell disease (SCD) have been described only anecdotally.

Objectives: To describe barriers to home deferoxamine (DFO) administration adherence among adults with SCD. It was hypothesized that barriers would include limited patient education on the importance of chelation and perceived aversiveness of the regimen.

Methods: Medical records were reviewed for 189 adult patients seen at a comprehensive sickle cell center. Patients with transfusion induced hemosiderosis, defined as a serum ferritin ≥ 1500 ng/ml, were administered a four item interview asking if iron overload had ever been discussed with them; if they had been informed they were iron over loaded; if chelation therapy had been offered; and if not currently home chelating, why not. Patients not interviewed were deceased (3); unavailable (10); or declined (3). A study coordinator who did not provide clinical care conducted the interviews.

Results: 54 of the 189 patients (29%) had a history of intermittent or chronic transfusion, or pheresis. 45 of these patients were iron overloaded. 29 of these patients agreed to complete the interview; 22 (76%) were female. Average age was 41.5 years (range 22.4 – 58.4 years) and average serum ferritin was 4240.8 (range 1547 – 9420). 23 of the 29 patients (79%) reported that their physician or nurse had discussed iron overload and chelation with them. 16 of these (55%) reported that they were currently receiving home DFO therapy. Reasons given for not administering home DFO included:

Reason Number (%) 
“Don’t want to stick self” 3 (23) 
No longer being transfused or being exchanged 3 (23) 
Awaiting clinical trial for oral chelator 2 (15) 
Home situation too complex 2 (15) 
Don’t want to (no further explanation) 2 (15) 
Too many adverse effects 1 (8) 
Reason Number (%) 
“Don’t want to stick self” 3 (23) 
No longer being transfused or being exchanged 3 (23) 
Awaiting clinical trial for oral chelator 2 (15) 
Home situation too complex 2 (15) 
Don’t want to (no further explanation) 2 (15) 
Too many adverse effects 1 (8) 

Discussion: Life threatening levels of iron overload were observed in intermittently transfused adult sickle cell patients. Contrary to expectations, iron overload and its treatment had been discussed with most patients. However, just over half were currently chelating at home. Toxicity of DFO and misunderstanding that iron overload is no longer a problem if chronic transfusion therapy stops are the most common reasons for non-compliance. Repeated patient counseling are essential in order to prevent progressive iron toxicity in sickle cell disease.

Reason Number (%) 
“Don’t want to stick self” 3 (23) 
No longer being transfused or being exchanged 3 (23) 
Awaiting clinical trial for oral chelator 2 (15) 
Home situation too complex 2 (15) 
Don’t want to (no further explanation) 2 (15) 
Too many adverse effects 1 (8) 
Reason Number (%) 
“Don’t want to stick self” 3 (23) 
No longer being transfused or being exchanged 3 (23) 
Awaiting clinical trial for oral chelator 2 (15) 
Home situation too complex 2 (15) 
Don’t want to (no further explanation) 2 (15) 
Too many adverse effects 1 (8) 

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