REPORTING OF COVID-19 IN HAEMOGLOBIN DISORDERS Survey

The purpose of the registry is to collect clinical data on thalassaemia patients who have been infected with the SARS-CoV-2 virus.

Currently the effects of this infection in thalassaemia patients is not known. It is suspected that patients with low iron overload and with few or no vital organ complications are not in danger of having a worse clinical outcome than the general population. This, however, is not certain. Patients with complications such as heart disease, pulmonary hypertension, liver disease, diabetes may have more serious development during the course of the infection. This also is not known.

Such knowledge is important to be known since increased precautions for vulnerable patients and more timely treatment may be necessary.

We request that all treating doctors fill this questionnaire so that global data, and the experience of as high a number of cases is recorded and analyzed.

Information must be without patient identification.

This form is to be completed by a health care professional caring for patients with beta thalassaemia and documented coronavirus (COVID-19). Please complete the form after the patient has had COVID-19 for a long enough duration to experience partial or complete recovery, hospitalization, or death.

Obtain IRB approval before completing if this is in accordance to your country's regulation

Note: Data management, storage and transfer will be secured according to institutional policies. Data will be saved on excel spread sheet (>=128 bit key AES encryption) in password protected files on secure, password protected computers with updated antivirus/malware protection.

1.Reporter Information

Name of person reporting the case

Email address of person reporting case

Name of physician providing care for the patient case if different from the reporting person

Name of center/practice providing care for thalassaemia

2.Patient Information

Age of patient, in years

3.Country of residence

4.Gender

Male

Female

Other

6.Ethnicity

State ethnic group if different from country of residence

Disease factors:

7.Transfusion Dependent Thalassaemia

Beta thalassaemia major

Haemoglobin E beta thalassaemia transfusion dependent

Other transfusion dependent thalassaemia

8.Non transfusion dependent beta thalassaemia

Thalassaemia intermedia

Haemoglobin E beta thalassaemia non transfusion dependent

Haemoglobin H disease

Other (please specify)

9.Sickle Cell Disease

Sickle Cell Anemia (homozygous HbS)

Sickle/β0 thalassaemia

Sickle/β+ thalassaemia

Other types of SCD(please specify)

10.Was the patient evaluated/diagnosed in a hospital Emergency Department?

Yes

Unknown

11.Was the patient evaluated/diagnosed in the day transfusion centre?

Yes

Unknown

12.How was the diagnosis confirmed?

By nasopharyngeal specimen using RT-PCR?

By other test

Please name the test

13.Has the patient been hospitalized due to COVID-19?

Yes

Unknown

If yes, length of hospital stay in days

Thalassaemia & SCD Medications:

14.Hydroxyurea

Yes

How long has the patient been taking this medication and what dosage

15.Penicillin (currently)

Yes

16.Desferrioxamine. If yes, what is the current regime?

Days per week

Daily dose

17.Deferiprone. If yes, what is the current regime?

Yes

Days per week

Daily dose

18.Deferasirox. If yes, what is the current regime?

Yes

Days per week

Daily dose

19.Combination

Yes

What is the combination of iron chelating agents?

What is the current regime: days per week

Daily dose of drug 1

Daily dose of drug 2

20.Insulin

Yes

21.Thyroxin

Yes

22.Oestrogen replacement

Yes

23.Testosterone replacement

Yes

24.Other treatment

25.Did the patient take any NSAIDS during this COVID-19 infection?

Yes

If yes, please name the medication

26.Were any of the above thalassaemia or SCD medications discontinued due to COVID-19?

Thalassaemia or SCD disease-related history, prior to the COVID-19 infection:

27.Splenectomy

Yes

Age of splenectomy and indication

28.Cardiac arrhythmias

Yes

Is the patient on medications? Specify

29.Cardiac failure

Yes

Last ejection fraction before the onset of the infection

30.Cirrhosis

Yes

31.Liver failure

Yes

32.Hepatocellular carcinoma

Yes

33.Acute painful episodes

Yes

Is the patient on medications? Specify

34.Stroke

Yes

Last TCD findings before the onset of the infection

35.Acute chest syndrome

Yes

36.Acute splenic sequestration crises

Yes

37.G6PD deficiency

Yes

38.Pulmonary hypertension

Yes

Last mPAP before the onset of the infection

39.Sickle Cell hepatopathy

Yes

If Yes, please specify the type of hepatopathy

40.Past Hepatitis C infection

Yes

41.On-going Hepatitis C infection

Yes

If yes, is the patient currently on anti-viral therapy? If yes, please name the medication

42.Past Hepatitis B infection

Yes

43.Ongoing Hepatitis B infection

Yes

If yes, is the patient currently on anti-viral therapy? If yes, please name the medication

44.Hypothyroidism

Yes

45.Diabetes

Yes

46.Current iron loading

Average serum ferritin over past year

Last liver iron concentration (if within past 12 months) mg/g dry wt

Last myocardial T2* (if within past 24 months) MS

Average pre-transfusion Hb over the last year

Number of blood transfusions (unit) in the month before infection with COVID-19

Have other cases of COVID-19 been reported from the patient’s usual treatment centre?

Does the patient have any of the following comorbidities? (for ages 19 or higher)

47.Asthma

Yes

48.Other Chronic Lung Disease (NOT asthma/COPD)

Yes

49.Hypertension

Yes

Please list medication

50.Cancer

Yes

Please specify

51.Current cigarette smoker

Yes

52.Past smoker (long term effects of smoking – related perhaps to ACE-2 enzyme)

Yes

53.Other comorbidities

Year of COVID-19 diagnosis:

54.Specify the approximate number of days from onset of symptoms to confirmed COVID-19 diagnosis

55.What was the level of Hb at the time of diagnosis?

56.White cell count at the time of diagnosis

The total of white cell count

The total of neutrophil count

The total of lymphocyte count

57.Please choose a severity of COVID-19 category:

Asymptomatic-no clinical signs or symptoms during the positive COVID-19 period.

Mild- symptoms of acute upper respiratory tract infection, including fever, fatigue, myalgia, cough, sore throat, runny nose, and sneezing or gastrointestinal symptoms of digestive symptoms such as nausea, vomiting, abdominal pain and diarrhea.

Moderate-pneumonia, with or without clinical symptoms, no hypoxia

Severe-early respiratory symptoms or gastrointestinal symptoms followed by dyspnea and hypoxia (O2 saturations less than 92%)

Critical-ARDS, respiratory failure, encephalopathy, shock, coagulopathy, multi-organ impairment (lung, heart, kidney, brain) that may be life-threatening

Did patient develop any of these complications at the time of COVID-19 infection?

58.Difficulty in breathing

Yes

59.Liver failure

Yes

60.Acute painful episode

Yes

61.Pulmonary hypertension

Yes

62.Heart arrhythmias

Yes

63.Heart failure

Yes

64.Kidney failure

Yes

65.Bacterial infection

Yes

If yes, what organism?

66.Have patient's symptoms resolved at the time of this report?

Yes

Unknown (lost to follow up)

Patient never developed symptoms (just tested positive)

67.Did the patient require a ventilator?

Yes

68.Did the patient receive blood transfusion, during the course of the infection?

Yes

69.Was there a marked drop in Hb levels during the period of infection?

Yes

70.Did patient die of COVID-19 or other complications caused by or contributed to by COVID-19?

Yes

Unknown

71.Official reported cause of death