Hereditary haemolytic anaemias and parvovirus infections in jehovah's witnesses

Authors


We are interested in the article by Drs Marsh and Bevan (2002) on the treatment of Jehovah's Witnesses with acute haematological problems. We would like to add two case reports reflecting the problems caused by acute parvovirus B19 infections in patients with hereditary haemolytic anaemias, where respecting patients' beliefs caused problems for health professionals (Finfer et al, 1994).

Case 1.  A young man, born in 1968, was admitted aged 18 years. Homozygous sickle cell disease was diagnosed when he was 10 years old. He and his parents were devout Jehovah's Witnesses. On admission he was febrile, with abdominal pain and jaundice.

His haemoglobin was 5 g/dl (normal steady state 8 g/dl), without any reticulocytes. The working diagnosis was that of an acute parvovirus crisis, confirmed by antibody sero-conversion. Transfusion was discussed but he had an advance directive refusing transfusion, correctly signed and witnessed (The Watchtower Bible & Tract Society, 1995). He became extremely confused and toxic and his haemoglobin fell to 2·9 g/dl. Treatment was supportive with fluids and oxygen. A week after admission reticulocytes were seen and he recovered slowly.

He has subsequently refused transfusion to help treat priapism.

Case 2. This case has already been reported in this journal (Mabin & Chowdhury, 1990), but bears repeating. In essence, a child with hereditary stomatocytosis (the daughter of the patient V first described in 1962: Lock et al, 1962) was admitted with a haemoglobin of 2·7 g/dl, and was both pyrexial and jaundiced. Because of her clinical condition, the procedure outlined by Marsh & Bevan (2002; 1, Emergencies) was followed, two consultants discussed with the parents the need for an urgent blood transfusion, which they accepted.

Parvovirus DNA was identified in the child's presentation sample. Her brother and mother, both of whom also had hereditary stomatocytosis, were shown to be susceptible to parvovirus.

The mother was admitted 14 d after her daughter with similar symptoms. She refused transfusion and died 4 d later. Her last recorded haemoglobin was 2·4 g/dl. Again parvovirus DNA was detected.

The brother remained well.

These cases had marked and long-lasting repercussions on the staff dealing with these patients, and many unofficial discussions took place to try and help relieve the emotions experienced by ward staff, nurses and doctors. Anger and frustration were common feelings.

The effect on staff of caring for the second family was most marked. The mother had asked to see her children, having decided not to be transfused, to tell them that if she accepted blood she would go to Hell. The hospital chaplain was invaluable at allowing staff to come to terms with the situation (E. Jackson; personal communication).

There was further distress when news of the girl having experienced a period of ‘disassociation’ when her father remarried and the family moved became known.

Both the patient and her brother have now left the sect, and each has been transfused. She has a daughter, also with hereditary stomatocytosis (G. W. Stewart, personal communication).

Similar reactions in staff have been mentioned in other settings (Cullis et al, 1998) and proactive staff meetings may have been helpful. In retrospect, we were slow to realize the problem.

It would also have been helpful to have a pre-emptive policy for dealing with such situations, as suggested in the recent article. If the parvovirus status of patients was known, advance planned management can be decided. For a non-immune child, medical staff and parents can discuss what blood products would be acceptable in an emergency and review this as necessary, with the help of the Jehovah's Witness Liaison Committee or other church elders (Simpson, 2002).

The recombinant vaccine undergoing clinical trials in the States may be acceptable to Jehovah's Witnesses as definitive protection.

Ancillary