Cuti-cuti Zoo Melaka dan Bayou Lagoon Park Resort

It was a last minute plan.

Satu hari tu, saya rasa macam dah lama tak berjalan-jalan. Almaklumlah pada cuti sekolah hujung tahun yang lepas, kami berganti-ganti tak sihat. Mula-mula saya, lepas tu Aqil chicken pox, lepas tu jangkit kat Aqlan pulak. Last-last tengok cuti pun dah habis.

Lalu saya pun survey-survey la kat internet. Jumpa tempat yang agak menarik di Melaka. Hotel baru, ada apartment, ada waterpark, harga pun macam murah je. Terus tanya suami. Suami kata ok, terus book hotel. Settle.

So hujung minggu lepas kami telah bercuti-cuti ke Melaka... hehehe dekat je pun...

Zoo Melaka

Macam bawak rombongan budak sekolah hehe

Dah lama dah nak bawak Aqil Aqlan jalan ke zoo. Yelah diorang pun masih kecil so rasanya zoo lah yang paling diorang dah boleh appreciate. Kalau bawak pergi kidzania ke, legoland ke, petrosains ke macam tak boleh nak enjoy sangat la kan. Memang la diorang seronok sangat. Tapi pagi tu Aqlan macam sakit perut pulak. 2 kali muntah. Alahai kesian anak mama, apalah salah makan agaknya. Pagi tadi semasa bersarapan di warung makan nasi lemak berselera sekali (kami semua lah). Nasib baik mama pun bawak sling. so selesa lah sikit dia tidur dalam tu.

Zoo? macam biasalah macam zoo. Sampai-sampai dekat pukul 11 terus kami tuju ke ampitheathre nak tengok birdshow. Lepas tengok birdshow berkejar pulak ke Elephant feeding. Lepas tu baru kami round zoo tu. Aqlan masa birdshow dengan elephant feeding dia ok lagi. Lepas tu dah tertidur dah di dalam kendongan mama hehhe.. Aqil hebat gak dia macam tak ngantuk je steady je walaupun dah tengahari. Tapi masa dah nak habis round tu baru dia tidur.. di dalam kendongan bibik pulak.

Burung merpati hinggap kat tangan Maisara

Tekunnya Aqil tengok apa tu..

Selesai round zoo dalam pukul 2. Belum boleh check in hotel lagi ni. Kami pun round nak cari makan. Cuma masa tu kesian kat Aqlan macam tak berapa larat dia. Jumpa tempat makan just kat belakang Bayou Lagoon. Order Nasi Goreng Pattaya bungkus nanti makan kat bilik je.

Bayou Lagoon Park Resort

Pukul 3 kami check in, receptionist kata bilik tak ready lagi. Sabor je lah. Kan dah pukul 3. Dia kata tunggu dalam setengah jam. Hmmm... nasib baik la tak sesak nak checkin macam masa kat Morib Gold Coast hari tu. Dalam pukul 3.20 gitu kami redah je bilik. Eh tengok elok je dah bersih. Kalau tau terus masuk je tadi. Budak-budak dah tak sabar dah nak terjun kolam.. isk isk...

Kami ambik apartment 3 bilik. Saya rasa memang selesa dengan living area yang sangat luas. Yelah family kami pun dah besar, ada bibik lagi, memang kalau nak cari tempat nak menginap kalau boleh memang saya cari yang ada apartment. Harganya sangat lah berpatutan. Kami dapat rate RM350++ satu malam untuk apartment 3 bilik yang luas ini. Saya book melalui Agoda. Mungkin juga sebab masa kami pergi tu bukan peak season. Saya tengok kalau book untuk raya cina minggu depan mau naik sampai RM500++ untuk apartment 3 bilik ni.

Luaskan? boleh main bola. memang budak2 main bola pun.

Aqil Aqlan keletihan melawat zoo

View waterpark dari balcony apartment kami

Nice... Teringin nak cuba slide tu tapi takuuuutt...

Syok main air. Tak sangka raihana pun dah terrer berenang

Angin pun sangat kuat. Memang betul-betul ditiup bayu lah.

Saya rasa memang puas hati duduk kat sini. Bilik apartment yang besar dan selesa, waterpark yang nice... Cuma slacknya satu breakfast tak best. Tak variety dan tak berapa sedap. Pastry bun pun tak ada. Selalunya itulah yang saya tapau bawak balik bilik hehe. But other than that memang berbaloi lah for the stay. Lain kali boleh ke sini lagi bawak mak ke. Tapi timing kena betul juga. Kalau tak kena masa, harga boleh melambung-lambung dan takut crowded pulak macam masa pergi Gold Coast Morib hari tu yang saya tak sempat nak bercerita lagi sebab tak de ambik gambar sangat sebab tak berapa sihat masa tu.

Ikan Bakar Umbai

Petang tu kami dah plan dah pukul 5.30 budak2 kena naik siap-siap kita nak pekena ikan bakar Umbai. Selalu dengar je orang cerita, first la ni nak rasa. Apa tu kedai nama Parameswara? hehhe... Tapi Aqlan masih tidur pulak. Kesian dia. Luqman pulak tak nak pergi sebab dia baru habiskan nasi goreng tadi sebungkus. So terpaksalah tinggalkan diorang dengan bibik.

Lebih kurang 20 minit jugalah perjalanan dari Bayou Lagoon ni ke Perkampungan Ikan Bakar Umbai ni. Kami sampai dalam pukul 6.30. Dari jauh dah nampak Parameswara tapi dia gerai yang paling hujung kat tepi laut sana. Sampai sana mak ai ramainya orang. Kami ni pulak ramai budak-budak. Meja pun dah penuh. Rasanya macam tak sanggup je nak menunggu. Jadi kami berjalan patah balik ke deret gerai-gerai ikan bakar yang banyak lagi kat sebelah sini. Tak tau mana yang sedap, kami redah masuk Gerai Anda, gerai nombor 10.

Tempat ikan naik

Pilih sendiri ikan mana mau dimasak

Sementara tunggu lauk sampai,
kita makan Aqil dulu hehheh

 Saya pilih ikan siakap bagi dua - sebelah masak 3 rasa, sebelah lagi bakar biasa, ikan pari bakar sambal, sotong goreng tepung, udang butter, kerang bakar dan sayur campur. Makan lauk je tak oder nasi pun. Imran je makan nasi lemak. Favourite dia. Alhamdulillah memang kenyang dan sedap dan terasa manisnya ikan fresh dan  sotong goreng tepung dia memang sedap dan hmm.... makan je lah. Cuma kerang dia macam tak berapa masak sangat kot macam masih berair tapi sedap je makan hehhehehe.... Kami makan tak abis so tapau bawak balik untuk bibik dan luqman dan Aqlan. Tambah lagi order udang goreng tepung. Bil kami RM110 tak silap termasuk air buah/horlick 6 gelas. Saya tanya suami, "Murah ke?". Suami jawab, "Murahlah!" Heheh... mana saya tau... Yeay, akhirnya dapat merasa juga ikan bakar Umbai yang famous tu. Lain kali boleh datang lagi lah. Bila la gaknya ye. Cuma kalau dia buka tengahari kan bagus. Memang boleh singgah selalu tu. Ini buka pukul 6 so ada masalah logistik sikit lah kalau nak menyinggah-nyinggah ni.

Kenyang alhamdulillah

Esok paginya kami sambung main air lagi. Sempat jugalah saya berendam juga dalam kolam tu. Memang sejuk tak tahan punya. Aqil Aqlan pun semalam tak dapat mandi sebab tidur. Pagi ni baru dapat merasa. Oh ye, sebab kami ambik apartment 3 bilik so kami dapat tiket free utk 6 orang so saya tambah tiket utk 2 orang kids berharga RM15 sorang. Dewasa tak tanya pulak. Anyway, Aqlan mula-mula excited main air kat kolam cetek tu tiba-tiba dia terjatuh. Terkena air sikit la muka dia. Lepas tu terus tak nak main. Kesian dia. Satu lagi keajaiban, cewah, Aqil Aqlan sebelum pergi tu dok selsema. Bila selsema hidung diorang selalu berdarah. Tapi lepas mandi kolam tu hilang terus selsema. Ajaib tu hehhe..

Air kat sini sejuk gilerr!!

Kolam untuk baby. Air paras buku lali je. 

Kami checkout pukul 12 tgahari. Budak-budak request nak makan nasi pattaya semalam sedap sangat katanya. Easy meh.. gerai belakang hotel ni je. Saya pun teringat-ingat ABC kat gerai sebelah tu masa order bungkus semalam macam best je. So kami terus menuju ke gerai tersebut. Namanya Nurin Maz Station. Ianya terletak di medan selera di taman perumahan betul-betul belakang Bayou ni. Tapi memang tak banyak gerai buka pun kat medan selera tu ada 3-4 je. Luqman, Imran, Raihana dan bibik order pattaya, maisara order mihun sup, saya dengan humaira makan nasi campur. Saya sebab nak rasa asam pedas dia. Suami pula share habiskan pattaya raihana makan tak habis. Nak dijadikan cerita semalam kan sedap sangat nasi goreng pattaya dia. Hari ni nasi dia ada mentah sikitlah. Spoil je. Tu yang ramai tak habis tu.

Order ABC kat gerai sebelah sorang satu. Hmmm... sedapnya. Semangkuk RM1.50 saja... Alhamdulillah kenyang...

Selesai menjamu selera, kami pun bertolak balik ke KL.

Sebenarnya ada satu lagi impian tak tercapai untuk percutian kali ni, Coconut Shake Pantai Kelebang. Kalau ikutkan katanya tak jauh pun. Suami kata 11 minit je (ikut kat Waze). Tapi sebab dah kenyang makan ABC maka ditangguhkanlah dulu ke satu masa yang tak tau lagi bila heheh..

Alhamdulillah syukur di atas segala nikmat dariNya.

Congo Bars

This is my latest obsession. Very easy to make and the whole family like it!

2 3/4 cups flour
2 1/2 tsp baking powder
1/2 tsp salt
2 cups brown sugar
150g butter (softened to room temp)
3 eggs
1 tsp vanilla extract
250g chocolate chip/chopped dark chocolate
1 cup pecan/almond/hazelnut/walnut (optional)

1. Sift flour. Add in baking powder and salt. Set aside.
2. In one large bowl, mix together butter and brown sugar using whisker or wooden spoon until butter disappear.
3. Add in eggs and vanilla extract. Continue mixing.
4. Add in flour mixture, a little at a time. Until evenly mixed.
5. Add in chocolate chip/chopped choc and nuts.
6. Pour the batter into a baking tray.
7. Bake in the oven for 25 minutes at 175 degree Celsius.

Original recipes here.

Bila Waktu Telah Berakhir

Apa yang anda faham tentang gambar di atas?

Ye, mati itu boleh bila-bila masa sahaja.

Ada yang diberikan rezeki umur yang panjang. Ada yang masih muda, umur 20-30-an telah ditarik nyawanya. Ada yang masih di bangku sekolah telah dipanggil olehNya. Ada juga yang baru beberapa hari melihat dunia, namun Allah lebih menyayanginya. Semuanya telah tertulis di Luh Mahfuz.

Beberapa orang kawan saya yang agak dekat, telah dipanggil menemui Ilahi.. Arwah Farsze' - dormmate di sekolah menengah, juga sama-sama berkursus sebelum melanjutkan pelajaran ke luar negara di bawah tajaan Telekom, masuk kerja dengan Telekom sama-sama usrah semasa tinggal di Vista Angkasa walaupun tak serumah. Arwah meninggal kerana kanser rahang sewaktu umurnya 25 tahun. Arwah Karl, sama-sama belajar di New College Cardiff junior setahun juga kerana cancer. Arwah Azlina (Ina) - teman sekerja sama-sama buat product development dan juga jiran setaman, juga disebabkan cancer, meninggalkan anak yang masih kecil-kecil.

Arwah Abg Pauzi - sepupu suami, meninggalkan isteri yng tidak bekerja dan 9 orang anak! ketika itu umurnya awal 40-an kerana serangan jantung. Arwah Abg Din, abang ipar saya, suami K zun, juga awal 40-an meninggal kerana sakit. Tak pasti sakit apa tapi rebah dan masuk ICU satu malam dan terus meninggalkan K zun dan Ammar.

Saya tidak dapat menatap wajah terakhir ayah saya. Arwah Bak meninggal semasa saya menuntut ilmu di tahun akhir di UK. Begitu juga dengan arwah nenek sebelah mak saya. Beliau pergi semasa saya dalam perjalanan dalam bas ke Terengganu untuk mengikuti kursus induksi sebelum ke luar negara. Arwah atuk sebelah mak juga begitu. Beberapa tahun terlantar sakit tua dijaga mak bergilir-gilir dengan pakcik dan makcik yang lain. Akhirnya beliau pergi semasa saya sedang kursus di Kuantan.

Yang pasti, betapa dekatnya kita dengan kematian. Tak kira umur. Sewaktu saya sakit hari tu, saya sungguh takut kalau saya mati. Saya terbayang-bayang apa akan jadi jika umur saya tak panjang. Bagaimana dengan anak-anak saya terutama Aqil dan Aqlan yang masih kecil lagi. Adakah sudah cukup bekalan saya. Cukupkah amalan saya? Adakah Allah redha dengan saya? Adakah saya akan menyusahkan orang-orang yang saya tinggalkan. Puasa masih belum habis ganti. Penama di KWSP saya tak kemaskini lagi. Borang wasiat sudah diambil tapi masih belum diisi. Dosa-dosa saya.. dengan Allah dan juga dengan sesama manusia. Hingga saya nekad untuk tebalkan muka mengaku kesalahan saya kepada seorang sahabat saya yang telah saya pendam lebih 10 tahun. Alhamdulillah beliau memaafkan. Syukur... kerana Allah telah bukakan hatinya. Saya rasa saya ada menyinggung hati mak tidak lama dulu lalu saya titipkan permohonan keampunan dalam kad harijadi mak. Saya fikir saya tak mahu tangguh lagi kerana saya takut tak sempat. Dosa-dosa saya yang lain yang saya tak ingat, tak perasan menyinggung hati sesiapa, hati suami, adik beradik, kawan-kawan, jiran? Ya Allah, ampunkanlah dosa-dosaku... Bagaimana dengan tanggungjawabku adakah semuanya dilaksanakan dengan jujur dan amanah? Sebagai anak, isteri, ibu, pekerja, bos, ahli dalam jemaah, ahli dalam masyarakat? Oh banyaknya soalan yang perlu dijawab. Ini baru soalan sendiri. Belum lagi soalan yang akan dilontarkan oleh Mungkar dan Nakir.

Bila diri dah mula sihat. Alhamdulillah.. Namun kesan tarbiyah dari Allah itu masih terkesan. Mati itu boleh bila-bila masa dan tidak kira umur. Adakalanya saya sangat berdebar bila melihat kesihatan Aqil yang kadang-kadang menurun. Adakah saya benar-benar bersedia untuk menghadapi sebarang kemungkinan? Saya semakin curious tentang pengurusan jenazah. Adakah saya bersedia untuk memandikan jenazah mak saya atau anak-anak saya atau adakah mereka yang akan memandikan jenazah saya? Saya rasa saya tahu kenapa suami saya tak berapa support keinginan saya untuk berhenti kerja, salah satunya kerana jika berlaku apa-apa kepada beliau saya masih boleh berdikari. Siapa akan pergi dulu, saya atau dia?

Huhuhu... sungguh mengerikan apabila membicarakan tentang kematian. Dan selalunya soalan-soalan ini akan berlalu begitu saja kerana kita amat takut untuk memikirkannya. Namun saya rasa memang perlu kita fikirkan sebab kita tahu mati itu pasti.  Dan ianya datang tanpa dapat kita duga. The question is "ARE WE READY?"

OPICK – BILA WAKTU TELAH BERAKHIR 

Bagaimana kau merasa bangga
Akan dunia yg sementara
Bagaimanakah bila semua hilang
Dan pergi meninggalkan dirimu

Bagaimanakah bila saatnya
Waktu terhenti tak kau sadari
Masihkah ada jalan bagimu
Untuk kembali mengulangkan masa lalu

Dunia dipenuhi dengan hiasan
Semua dan segala yang ada akan kembali padaNya

Bila waktu tlah memanggil,
Teman sejati hanyalah amal
Bila waktu telah terhenti
Teman sejati tinggallah sepi

Selamat Tahun Baru 2014

Oh dah 7 haribulan hari ini. Selamat Tahun Baru saya ucapkan walaupun terlewat sikit. Almaklumlah minggu lepas sibuk dengan persiapan anak-anak memulakan persekolahan, sibuk membantu guru PASTI memulakan sesi persekolahan yang baru, lepas tu kami balik kampung pulak. Minggu ni baru masuk office balik. Keadaan di office pun masih agak lengang. Meeting pun tak ada. Kerja pun tak banyak sangat.

Alhamdulillah dengan 2013. Bagi saya, tahun 2013 agak mencabar. Penuh dengan dugaan dan ujian. Pertamanya kerana anak-anak saya didiagnose dengan PPH. Saya kena ambil unpaid leave selama dua bulan untuk jaga Aqil. Oh nanti bonus kena prorate lah! Keduanya kerana saya sendiri jatuh sakit yang agak teruk. Banyak pengajaran yang saya dapat melalui ujian yang saya lalui ini. Mungkin saya akan ceritakan lain kali.

Alhamdulillah saya rasa saya dah sihat sepenuhnya. Cuma adakalanya tangan saya ni terutamanya di pergelangan tangan ada rasa sakit macam salah urat dan tak berapa kuat sangat kalau nak angkat barang yang berat. Sekarang masih teruskan dengan calcium dan glocosamine untuk kesihatan tulang dan sendi-sendi.

Alhamdulillah untuk Aqil dan Aqlan. Aqil walaupun keadaannya masih belum pulih, tapi at least dia selalu ceria dan sihat. Dah jarang batuk dan selsema. Perkembangannya juga normal.. berjalan, berlari, melompat, main bola, menari dan dah semakin banyak cakap sekarang. Cuma mungkin badannya kecil sikit. Aqlan pula tetap aktif macam biasa. Badan pun semangat! Sebab suka makan.

Ops makan lollipop, abislah gigi!

 Alhamdulillah anak-anak semua dah mula bersekolah semula. Selamat memulakan alam persekolahan yang baru buat Raihana Huda! Mama dan ayah taklah risau sangat sebab ada 4 orang bodyguard! hehehe.. First day sekolah pun mama hantar naik bas je. Oklah takde incident nangis-nangis ke apa. Dah naik darjah satu ni dah tak boleh main-main pasal solat. Kena solat betul-betul tau.

First day of school 2014. Berderet!

Alhamdulillah atas nikmat yang Allah berikan. Semoga 2014 menjanjikan kehidupan yang lebih baik dan lebih dirahmati Allah.

A different kind of pressure

http://www.thestar.com.my/story.aspx/?file=%2f2013%2f5%2f12%2fhealth%2f13079422

A different kind of pressure

BY DATUK DR AIZAI AZAN ABDUL RAHIM AND DATUK DR DAVID CHEW

   

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How pulmonary hypertension develops.

More than 25 million people who suffer from a disease that is not easy to diagnose and can be fatal – pulmonary hypertension – commemorated World Pulmonary Hypertension Day last Sunday.

MANY are familiar with the disease called hypertension (high blood pressure), which is an elevated blood pressure measured by a cuff around the arm. This reflects the systemic pressure throughout the body that is generated when the left ventricle of the heart contracts.

A less commonly known condition is called pulmonary hypertension (PH), which is high blood pressure that occurs in the arteries of the lungs.

In PH, the blood vessels in the lungs constrict and become narrowed due to cellular proliferation, remodeling and obstruction of smaller pulmonary blood vessels. They become structurally and functionally abnormal. This makes it harder for the right ventricle of the heart to pump blood through the lungs to reach the left side of the heart and to the rest of the body.

This increases stress to the right ventricle, resulting in its enlargement and subsequent weakening, eventually leading to right heart failure.

When this happens, not enough oxygen gets delivered to the rest of the body, resulting in symptoms and eventually premature death from progressive right heart failure or an abnormal heart rhythm.

The gold standard in confirming the diagnosis of PH is right heart catheterisation, where a small plastic tube is advanced into the right side of the heart and pulmonary vessels to measure pressures and oxygen saturations.

For diagnosis to be proven, people need to undergo an invasive procedure called right heart catheterisation (RHC).

By definition, PH exists when the mean pulmonary artery pressure (PAP) is more than 25 mmHg in the presence of a normal pulmonary capillary wedge pressure (PCWP) of less than 15 mmHg.

The RHC is usually performed in specialised cardiac catheterisation laboratories by cardiologists in Malaysia.

How common is PH and who gets it?

Although PH is an uncommon condition, it is widely accepted that it’s under-diagnosed. The occurrence of the disease in the general community has been estimated to be anywhere between 30 and 50 cases per million population.

There is no data for Malaysia at the moment.

In childhood, the condition affects both genders, but after puberty, it is more common in females (approximately two to one ratio).

There is a heritable form of PH and 70% of these individuals have a mutation in the gene for Bone Morphogenetic Protein Receptor II (BMPR II). This condition is inherited and people with this mutation have a 10-20% chance of developing PH.

PH is also common amongst people with connective tissue diseases such as scleroderma spectrum of disease (up to 27%), systemic lupus erythematosus (up to 14%) and rheumatoid arthritis (up to 20%).

Uncorrected congenital “hole in the heart” diseases with large shunts such as atrial septal defects, ventricular septal defects and patent ductus arteriosus can also act as trigger factors.

Users of appetite suppressants such as aminorex, fenfluramine phentermine (Fen-Phen) and dexfenfluramine phentermine (Dex-Phen) for longer than three months have a 23-fold higher risk of developing PH, hence the reason why these drugs are now banned.

Other causes include blood disorders such as sickle cell disease, obstructive sleep apnoea, survivors of deep vein thrombosis and pulmonary embolism, advanced chronic obstructive airways disease and even HIV infection.

It is very important to screen patients with these conditions for PH as early detection and treatment may provide significant benefits.

Classification of PH

PH was first described by Dr Ernst Von Romberg in 1891. It is a broad name for a group which currently consists of five distinct types of chronic disease that affects the heart and lungs:

Group 1: Pulmonary Arterial Hypertension (including idiopathic, heritable, drugs/toxin induced, congenital heart disease, HIV, connective tissue disease)

Group 2 : PH associated with left heart disease

Group 3 : PH associated with respiratory disease

Group 4 : PH due to chronic thromboembolic disease

Group 5 : PH due to miscellaneous cause

Individuals with PH can experience a range of symptoms depending on the severity and underlying cause of their disease. In general, the most common symptoms are:

·Breathlessness

·Weakness

·Dizziness and sometimes syncope on activity and when standing up

·Sensation of racing heart beat (palpitations )

·Chest pain, particularly on activity

·Cough

·Swollen legs and ankle

Early symptoms of PH are often subtle and common to other conditions such as asthma, chronic pulmonary disease and even systemic hypertension and are frequently overlooked. Diagnosis is made when other causes have been excluded first.

Often, this results in delayed diagnosis and treatment. The right heart continues to fail and this leads to worsening symptoms such as increasing fatigue, abdominal distension due to ascites, enlarged and tender liver, ankle swelling, coughing up blood stained sputum, having to sleep in a propped up position with several pillows (orthopnoea) and bluish lips and nails (cyanosis).

How is PH diagnosed?

A high index of suspicion is important as the symptoms are common to many other diseases affecting the heart and lungs. It begins with a thorough medical and family history evaluation with physical examination.

Blood tests, ECG, chest x ray, lung function test, lung ventilation/perfusion scanning and 2D echocardiogram are also useful.

The “gold standard” in confirming the diagnosis is RHC, where a small plastic tube is advanced into the right side of the heart and pulmonary vessels to measure pressures and oxygen saturations. This small tube or catheter is introduced from a vein in the neck or groin under local anaesthetic. This procedure is carried out in the cardiac catheterisation laboratory.

Treatment of pulmonary arterial hypertension

A sub group of PH is called pulmonary arterial hypertension (PAH). Though there are a number of causes of PAH, a significant number of patients suffer from PAH without any known cause. This was previously called primary pulmonary hypertension but is now referred to as idiopathic pulmonary arterial hypertension.

In the 1980s, there was no specific medical treatment for PAH. The average survival after diagnosis was two years and 10 months, and the only therapy that could be resorted to when patients deteriorated was a heart and lung transplant.

Since 1996, a number of drugs have been studied that improve blood flow to the lungs in patients with pulmonary hypertension, and appear to relieve their symptoms and improve their survival. These drugs are referred to as pulmonary vasodilators.

Before the advent of these drugs, the physician was limited to general measures and conventional medications, which are still applicable in the treatment of patients.

Physicians need to strike a balance between encouraging low levels of exercise and prohibiting excessive physical exertion, which may exceed the capacity of the limited function of the heart and lungs.

Oxygen can be administered to those who develop low oxygen levels in their blood as a result of their disease. Respiratory infections can be prevented to some extent by the use of flu and pneumococcal vaccines. However, if patients develop infections, these should be promptly treated.

Female patients with child bearing potential should avoid pregnancy. The increase demand on the heart and lungs in pregnancy is often not tolerated and can result in death in up to 50% of PAH patients. Hence safe and effective contraception in women who can become pregnant is mandatory.

Medications that may be helpful include:

1. Warfarin – for prevention of blood clots from developing in the lungs. This can be prescribed in patients if their risk of bleeding (a side effect of the medication) is low.

2. Diuretics (frusemide, spironolactone) – this is useful to remove fluid accumulation in patients who have developed heart failure.

3. Digoxin – this medication is useful for heart failure, or to control fast and irregular heart rate.

Calcium antagonists are a group of drugs used to treat systemic hypertension, and has been used to treat patients with PAH. A small proportion of patients with PAH do respond to calcium antagonist. These drugs can dilate the pulmonary vessels in susceptible patients.

However, the identification of such responsive patients is from the cardiac catheterisation and the performance of a vasodilator challenge test. If the test is negative, then the use of this group of drugs is generally ineffective and disappointing.

In 1996, a study was published reporting on the use of a continuous intravenous infusion of a drug called epoprostenolol (currently not registered in Malaysia). It showed that patients who received it had a longer life span than those who were on conventional medications.

Many studies followed, and currently there are five drugs which are available to treat PAH in Malaysia. These are bosentan and ambrisentan (belonging to a group called endothelin antagonist), iloprost (a prostaglandin type of drug, similar to epoprostenol), and sildenafil and tadalafil (drugs approved for treating erectile dysfunction, but found to be useful for PAH as well).

These drugs are expensive (monthly cost of between RM3,000 to RM10,000) and it is almost impossible for patients with PAH to be able to afford them, especially on a long term basis.

Most patients will require government assistance or subsidy to be treated with these medications. Some patients have good response to one of these pulmonary vasodilators, but often after several years, the patients become unwell again as the disease progresses, and a second pulmonary vasodilator drug may need to be added.

A registry of patients with PAH from the US revealed that the average survival has improved to more than seven years with modern therapies, including pulmonary vasodilators.

Ultimately, if the patient has poor response, then a lung or heart-lung transplant may need to be considered provided that the patients are suitable for this procedure, and there is a suitable donor.

> Datuk Dr Aizai Azan Abdul Rahim and Datuk Dr David Chew are consultant cardiologists.