CLAN's report on the 2006 CAHSAPI Meeting
What an encouraging and inspiring 2nd CAHSAPI Meeting!! With the support of the outstanding Section of Paediatric Endocrinology (part of the Department of Paediatrics at the Philippines General Hospital (PGH)), the Filipino CAH Support Group CAHSAPI held its second-ever meeting on November 10th, 2006. Sincere thanks must go to Drs Lorna Ramos-Abad (Head, Section of Paediatric Endocrinology), Sioksoan Chan-Cua, Eve Fernandez and Cynthia Feliciano. Congratulations also to CAHSAPI President Mr Alain Yap and his wife Leriz for their vital role in the smooth running of day.
With 36 families attending (it is estimated that there are around 80 children in the Philippines who have CAH), CAHSAPI experienced a 50% growth in 12 months, no doubt reflecting the influence of an expanding Newborn Screening (NBS) Program in the country. There are now two NBS Laboratories in the country, with a plan to expand to 11 in the future! A tour of the NBS facilities in the Manila headquarters at UP-PGH (University of Philippines - Philippines General Hospital) on November 9th was very interesting, and CLAN wishes to thank Dr Charity Jonmento for her time in showing us around her Laboratory.
NBS rates have increased from 5% to 20% in the last year, but with over 70% of all Filipino births still occuring in the home, there is still enormous scope to capture more babies in the screening program. It is for this very reason that the Paediatric Endocrinologists at PGH were inspired to conduct a parallel program directed specifically at Health Care Professionals (HCP) in a nearby lecture theatre on the day of the CAHSAPI meeting. Midwives were specifically targeted, given they are the people most likely to attend births outside of hospital maternity units. Two case studies were presented to the attendees: one of a baby girl born with ambiguous genitalia, and another of a baby boy with failure to thrive at 2 weeks of age. Educational programmes like this offer the best hope for babies born with CAH who slip through the NBS net, and the pivotal role of the Philipines Society of Paediatric Metabolism and Endocrinology (PSPME), and its current President Dr Melinda Millares-Atienza, must be gratefully acknowledged.
Current estimates suggest that about 1 child every month is diagnosed with CAH in the Philippines. This certainly explained the high number of little babies and their parents attending the CAHSAPI meeting on the 10th! The CAH population is still relatively young at PGH, but it was also great to see so many "old friends" there from last year, and delight in the children taking up with one another again so easily. AJ, Honey and Mika formed an especially tight gang very quickly!
After an opening prayer and welcoming remarks, Dr Lorna Ramos-Abad gave a comprehensive presentation covering the physiology, pathophysiology, genetics and general management of CAH. A brief question and answer session followed, with some of the parents of newly-diagnosed children taking the opportunity to clarify some points.
Dr Eve Fernandez followed, with a report on the findings of the survey of parents launched at the CAH meeting in 2005. This survey has been continued through the last 12 months, with new families invited to participate, and everyone was most interested to hear the findings:
- Survey Population
- 31 parents responded to the survey, representing some 39 CAH patients.
- 38 had 21-hydroxylase deficiency; 1 had 11-hydroxylase deficiency
- 32 had Salt Wasting (SW) CAH (11 males and 21 females)
- 6 had Simple Virilising (SV) CAH (all female)
- age at diagnosis ranged from 10 days to 5 months for those with SWCAH (mean age 45 days) and 2 weeks to 15 years for those with SVCAH (mean 8 years; median 1 year)
- 28 of the 32 children with SWCAH were diagnosed through NBS (only 1 of 6 with SVCAH were picked up with NBS)
- ambiguous genitalia was a feature of all the girls at diagnosis, but the majority also had vomiting and failure to thrive; 9 of the 11 boys presented with vomiting.
- 7 siblings of children with SWCAH had died in the neonatal period
- 4 families had more than one child with CAH; 3 families had 2 children with CAH, and one family had 3 children with CAH.
- 44% of children were using Prednisone (this reflects period prior to CLAN's donations)
- 41% of children lived in Manila
- texting is the best means of communicating with families. Only 42% reported access to the internet.
- the mean time to travel for consultation was 2.2 hours (range 30 minutes to 10 hours), and for travel for emergency treatment mean time was 45 minutes (range 15 minutes to 2 hours).
- the biggest worries for families related to the need for lifelong treatment, the cost and availability of medications (over 25%); adrenal crises (over 25%); the child's development; and possibility of death
- the biggest burdens were financial; accessing medicine; and the need for lifelong treatment
of the girls with CAH, only 6 had had surgery (5 of the parents were happy with results)
v - the biggest worries parents had for their daughters related to future marriage and reproduction (over 50%); growth, fertility and adrenal crises.
Dr Fernandez finished her talk with some suggestions for future plans in the Philippines:
- Start a CAH Registry in the Philippines, and collect data about CAH
- Develop local books and resources on CAH
- Establish reliable availability of drugs
- Provide more information on CAH to families (website, newsletters, books and meetings)
- Christmas party next year for CAHSAPI
Claire Henderson spoke next, and shared a bit about her life. Claire lives in Australia, works as an Endocrinology Nurse and also has CAH. Claire's talk had earlier been transated into Tagalog by Dr Lorene Cimacio, and families read along as Claire shared her life-story. Claire was a huge inspiration to the children and families attending that day - it is one thing to be told that life with CAH should be happy and healthy, it is another to meet someone who has achieved this goal. The gathering was stunned to hear about Claire's self-management with hydrocortisone injections and stress dosing and asked questions about this at the end!
Morning tea was then served, and after travelling such long distances to attend, many of the families were grateful for the excellent food and drink provided.
Kate Hansen of CLAN spoke once everyone had partaken, and shared some of the basic rules her family follows to help their child with CAH enjoy the highest quality of life possible. Some of the fundamentals of CAH management were covered in a power-point presentation.
Dr Lorelei Elma-Chua (Child Psychiatrist) shared a powerful talk on the complicated issues CAH can raise around gender, and her interactive style encouraged discussion amongst parents as they shared their own experiences. The majority of CAH patients in the Philippines are still female, and parents were obviously happy to be able to meet others who could empathize with their own stories.
Dr Dante Dator (Paediatric Urologist) gave an informative talk on the surgical issues relating to CAH, and responded to many of the questions posed by parents. The complexity of some situations was handled sensitively, and it sounded like several of the families were keen to follow-up with him afterwards.
Lunch was held under tents in the hospital courtyard, and sincere thanks must go to the PGH caffeteria staff who did such an outstanding job catering for the CAHSAPI meeting. Families divided into geographical regions, and sat with others from their home-town areas. It is hoped these relationships will continue to grow and forge additional support networks in the future. Children and parents alike enjoyed the excellent food and refreshments, and the play equipment provided a chance for the kids to burn off some of their energy! Next year it is planned to book a play-room at the hospital, as the increasing number and age of the children makes for quite a full lecture theatre now!
After lunch, CAHSAPI held a formal meeting, and all parents attended and participated. Representatives were elected from each region, and opportunities discussed regarding how people could grow CAHSAPI links in their own areas. It is hoped that families might be able to "carpool" to PGH, and even share drugs in the event of running out of supplies at home. The distribution plans for medication - including times that parents can come to PGH to collect medications - were also addressed.
The closing talk was by Jeff Cagandahan, an inspiring young man with CAH who now works as head teacher at a private school. Jeff has been kind enough to share his story, and CLAN would encourage everyone to take the time to read it. As the first Filipino to legally change their gender from female to male, Jeff's story is one of heartache and suffering, but also courage, strength and compassion. Unfortunately not everyone is as strong as Jeff, and we must remember that his story reflects the reality of too many lives in resource-poor countries where the current standard of diagnosis and treatment of CAH is inconsistent with a high quality of life. Jeff's mission in life is to help others living with CAH, and prevent them from having to experience the trauma and hardship that he has endured over the years.
At the end of the meeting, free medication was donated to each child, dispensed by the treating doctors in the nearby Outpatients Department. This was clearly "a happy ending" that met with the approval of the families!
In closing, CLAN would like to extend its sincere congratulations to CAHSAPI and the wonderful doctors at PGH and of the PSPME. They are effecting huge change in the lives of children and families living with CAH in the Philippines, and CLAN is proud to work alongside these amazing people. Thank-you also to all of the sponsors and donors who make CLAN's work possible, particularly Alphapharm, Bristol-Myers Squibb and MedicAlert. And last but not least, Ms Claire Henderson, whose warmth and compassion prompted her to share so generously of her time and self by visiting Vietnam and the Philippines, with the hope of helping others with CAH enjoy happy and healthy lives.
PHOTOS COMING SOON!!